+D	Disease
#<h2><a href="/kegg/kegg2.html"><img src="/Fig/bget/kegg3.gif" align="middle" border=0></a>&nbsp; ICD-10 Disease Classification</h2>
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#ENTRY       br08403
#NAME        ICD-10
#DEFINITION  ICD-10 disease classification
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A1. Certain infectious and parasitic diseases (A00-B99)
B  A00-A09  Intestinal infectious diseases
C    A00  Cholera
D      H00110  Cholera
C    A01  Typhoid and paratyphoid fevers
D      H00111  Typhoid fever
D      H00112  Paratyphoid fever
C    A02  Other salmonella infections
D      H00113  Salmonellosis
C    A03  Shigellosis
D      H00299  Shigellosis
C    A04  Other bacterial intestinal infections
D      H00278  Enteropathogenic Escherichia coli (EPEC) infection
D      H00280  Enterotoxigenic Escherichia coli (ETEC) infection
D      H01311  Enteroinvasive Escherichia coli (EIEC) infection
D      H00277  Enterohaemorrhagic Escherichia coli (EHEC) infection
D      H01312  Enteroaggregative Escherichia coli (EAEC) infection
D      H00321  Campylobacter infection
D      H00298  Yersiniosis
D      H00300  Enterobacter infection
D      H00338  Pseudomembranous colitis
C    A05  Other bacterial intestinal infections, not elsewhere classified
D      H00339  Botulism
D      H00335  Foodborne Clostridium perfringens intoxication
D      H00307  Vibrio parahaemolyticus infection
D      H00329  Foodborne Bacillus cereus intoxication
D      H00308  Vibrio vulnificus infection
C    A06  Amoebiasis
D      H00360  Amoebiasis
C    A07  Other protozoal intestinal diseases
D      H00362  Giardiasis
D      H00356  Cryptosporidiosis
D      H01087  Balantidiasis
C    A08  Viral and other specified intestinal infections
D      H00975  Rotaviral enteritis
D      H01323  Acute gastroenteropathy due to Norwalk agent
B  A15-A19  Tuberculosis
C    A15-A19  Tuberculosis
D      H00342  Tuberculosis
B  A20-A28  Certain zoonotic bacterial diseases
C    A20  Plague
D      H00297  Plague
C    A21  Tularemia
D      H00312  Tularemia
C    A22  Anthrax
D      H00328  Anthrax
C    A23  Brucellosis
D      H00325  Brucellosis
C    A24  Glanders and melioidosis
D      H00316  Glanders
D      H00317  Melioidosis
C    A25  Rat-bite fevers
D      H01314  Rat-bite fever
C    A26  Erysipeloid
D      H01315  Erysipeloid
C    A27  Leptospirosis
D      H00355  Leptospirosis
C    A28  Other zoonotic bacterial diseases, not elsewhere classified
D      H00306  Pasteurellosis
D      H00326  Cat-scratch disease
D      H00298  Yersiniosis
B  A30-A49  Other bacterial diseases
C    A30  Leprosy (Hansen disease)
D      H00344  Leprosy
C    A31  Infection due to other mycobacteria
D      H01042  Buruli ulcer
C    A32  Listeriosis
D      H00332  Listeriosis
C    A33-A35  Tetanus
D      H00337  Tetanus
C    A36  Diphtheria
D      H00343  Diphtheria
C    A37  Whooping cough
D      H00319  Pertussis
C    A38  Scarlet fever
C    A39  Meningococcal infections
D      H00314  Meningococcal infections
C    A40  Streptococcal septicaemia
D      H00333  Streptococcal infection
C    A41  Other septicaemia
D      H00304  Haemophilus influenzae infection
D      H00340  Vancomycin-resistant enterococci infection
D      H01175  Staphylococcus lugdunensis infection
C    A43  Nocardiosis
D      H00345  Nocardiosis
C    A44  Bartonellosis
D      H00326  Bartonellosis
C    A46  Erysipelas
C    A48  Other bacterial diseases, not elsewhere classified
D      H00336  Gas gangrene
D      H00311  Legionellosis
C    A49  Bacterial infection of unspecified site
D      H00301  Klebsiella infection
D      H00302  Citrobacter infection
D      H00303  Serratia infection
D      H00304  Haemophilus influenzae infection
D      H00313  Pseudomonas aeruginosa infection
D      H00309  Acinetobacter infection
D      H00320  Helicobacter pylori infection
D      H00333  Streptococcal infection
B  A50-A64  Infections with a predominantly sexual mode of transmission
C    A50-A53  Syphilis
D      H00354  Syphilis
C    A54  Gonococcal infections
D      H00315  Gonococcal infections
C    A55  Lymphogranuloma venerum
D      H00348  Lymphogranuloma venereum
C    A56  Other sexually transmitted chlamydial diseases
D      H00347  Chlamydial urethritis
C    A57  Chancroid
D      H00305  Chancroid
C    A59  Trichomoniasis
D      H00812  Trichomoniasis
B  A65-A69  Other spirochaetal diseases
C    A66  Yaws
D      H01318  Yaws
C    A68  Relapsing fevers
D      H00427  Relapsing fever
C    A69  Other spirochaetal infections
D      H00353  Lyme disease
B  A70-A74  Other diseases caused by chlamydiae
C    A70  Psittacosis
D      H00350  Psittacosis
C    A71  Trachoma
D      H00349  Trachoma
B  A75-A79  Rickettsioses
C    A75  Typhus fever
D      H00322  Typhus fever
D      H00324  Scrub typhus
C    A77  Spotted fever (tick-borne rickettsioses)
D      H00323  Spotted fever
C    A78  Q fever
D      H00310  Q fever
C    A79  Other rickettsioses
D      H00327  Trench fever
D      H01140  Sennetsu neorickettsiosis
B  A80-A89  Viral infections of the central nervous system
C    A80  Acute poliomyelitis
D      H00376  Acute poliomyelitis
C    A81  Atypical virus infections of central nervous system
D      H00061  Prion diseases
D      H00370  Progressive multifocal leukoencephalopathy
C    A82  Rabies
D      H00377  Rabies
D      H00378  Lyssavirus infection
C    A83  Mosquito-borne viral encephalitis
D      H00379  Mosquito-borne viral encephalitis
C    A84  Tick-borne viral encephalitis
D      H00380  Tick-borne viral encephalitis
C    A85  Other viral encephalitis, not elsewhere classified
D      H00393  Enterovirus infection
C    A87  Viral meningitis
D      H00393  Enterovirus infection
D      H01324  Lymphocytic choriomeningitis
B  A90-A99  Arthropod-borne viral fevers and viral haemorrhagic fevers
C    A90-A91  Dengue (haemorrhagic) fever
D      H00381  Dengue
C    A92  Other mosquito-borne viral fevers
D      H00382  Mosquito-borne viral fevers
C    A93  Other arthropod-borne viral fevers, not elsewhere classified
D      H00383  Arthropod-borne viral fevers
C    A95  Yellow fever
D      H00384  Yellow fever
C    A96  Arenaviral hemorrhagic fever
D      H00385  South American hemorrhagic fevers
D      H00386  Lassa fever
C    A98  Other viral haemorrhagic fevers, not elsewhere classified
D      H00284  Crimean-Congo hemorrhagic fever
D      H00416  Omsk hemorrhagic fever
D      H01322  Kyasanur Forest disease
D      H00405  Marburg disease
D      H00283  Ebola haemorrhagic fever
D      H00389  Hemorrhagic fever with renal syndrome
B  B00-B09  Viral infections characterized by skin and mucous membrane lesions
C    B00  Herpes simplex infections
D      H00365  Herpes simplex infection
D      H00387  B virus infection
C    B01  Varicella (chickenpox)
D      H00366  Varicella
C    B03  Smallpox
D      H00372  Smallpox
C    B04  Monkeypox
D      H00373  Monkeypox
C    B05  Measles
D      H00394  Measles
C    B06  Rubella
D      H00395  Rubella
C    B07  Viral warts
D      H00374  Viral wart
C    B08  Other viral infections characterized by skin and mucous membrane lesions, not elsewhere classified
D      H00369  Exanthema subitum
D      H00404  Erythema infectiosum
D      H01052  Molluscum contagiosum
D      H00393  Enterovirus infection
D      H01326  Hand, foot and mouth disease
D      H01327  Herpangina
B  B15-B19  Viral hepatitis
C    B15  Acute hepatitis A
D      H00411  Hepatitis A
C    B16  Acute hepatitis B
D      H00412  Hepatitis B
C    B17  Other acute viral hepatitis
D      H00413  Hepatitis C
D      H00415  Hepatitis E
C    B18  Chronic viral hepatitis
D      H00414  Hepatitis D
B  B20-B24  Human immunodeficiency virus (HIV) disease
C    B20-B24  AIDS
D      H00406  Acquired immunodeficiency syndrome (AIDS)
C    B20  Human immunodeficiency virus (HIV) disease resulting in infectious and parasitic diseases
D      H00368  Cytomegalovirus infection
B  B25-B34  Other viral diseases
C    B25  Cytomegalovirus infections
D      H00368  Cytomegalovirus infections
C    B26  Mumps
D      H00396  Mumps
C    B27  Infectious mononucleosis
D      H00368  Cytomegalovirus infection
D      H00367  Infectious mononucleosis
C    B30  Viral conjunctivitis
D      H00393  Enterovirus infection
C    B33  Other viral diseases, not elsewhere classified
D      H00397  Ross River fever
D      H00390  Hantavirus pulmonary syndrome
C    B34  Viral infection of unspecified site
D      H00393  Enterovirus infection
B  B35-B49  Mycoses
C    B35  Dermatophytosis
D      H01316  Dermatophytosis
C    B37  Candidiasis
D      H00363  Candidiasis
D      H01109  Chronic mucocutaneous candidiasis
C    B38  Coccidioidomycosis
D      H01319  Coccidioidomycosis
C    B39  Histoplasmosis
D      H01062  Histoplasmosis
C    B41  Paracoccidioidomycosis
D      H01329  Paracoccidioidomycosis
C    B44  Aspergillosis
D      H01328  Aspergillosis
C    B45  Cryptococcosis
D      H00364  Cryptococcosis
B  B50-B64  Protozoal diseases
C    B50  Plasmodium falciparum malaria
D      H00361  Malaria
C    B51  Plasmodium vivax malaria
D      H00361  Malaria
C    B55  Leishmaniasis
D      H00359  Leishmaniasis
C    B56  Trypanosomiasis
D      H00357  African trypanosomiasis
C    B57  Chagas' disease
D      H00358  Chagas disease
C    B58  Toxoplasmosis
D      H00435  Toxoplasmosis
C    B60  Other protozoal diseases, not elsewhere classified
D      H01060  Babesiosis
B  B65-B83  Helminthiases
C    B65  Schistosomiasis (bilharziasis)
D      H01045  Schistosomiasis
C    B66  Other fluke infections
D      H01048  Liver fluke infection
D      H01107  Intestinal fluke infections
D      H01093  Paragonimiasis
C    B67  Echinococcosis
D      H01153  Human echinococcosis
C    B68  Taeniasis
D      H01047  Cysticercosis
D      H01152  Taenia saginata infection
C    B69  Cysticercosis
D      H01047  Cysticercosis
C    B70  Diphyllobothriasis and sparganosis
D      H01014  Sparganosis
D      H01085  Diphyllobothriasis
C    B71  Other cestode infections
D      H01138  Hymenolepiasis
C    B72  Dracunculiasis
D      H01044  Dracunculiasis
C    B73  Onchocerciasis
D      H01043  Onchocerciasis
C    B74  Filariasis
D      H01086  Lymphatic filariasis
D      H01104  Loiasis
D      H01061  Mansonelliasis
C    B75  Trichinellosis
D      H01099  Trichinosis
C    B76  Hookworm diseases
D      H01092  Human hookworm infections
C    B77  Ascariasis
D      H01090  Ascariasis
C    B78  Strongyloidiasis
D      H01100  Strongyloidiasis
C    B79  Trichuriasis
D      H01046  Trichuriasis
C    B80  Enterobiasis
D      H01091  Enterobiasis
C    B81  Other intestinal helminthiases, not elsewhere classified
D      H01058  Anisakiasis
C    B83  Other helminthiases
D      H01055  Giant kidney worm infection
D      H01056  Toxocariasis
D      H01057  Gnathostomiasis
B  B85-B89  Pediculosis, acariasis and other infestations
C    B85  Pediculosis and phthiriasis
D      H01054  Pediculosis
C    B87  Myiasis
D      H01178  Myiasis
C    B88  Other infestations
D      H01098  Pentastomiasis
D      H01179  Tungiasis
B  B90-B94  Sequelae of infectious and parasitic diseases
B  B95-B98  Bacterial, viral and other infectious agents
C    B96  Other specified bacterial agents as the cause of diseases classified to other chapters
D      H00301  Klebsiella infection
D      H00335  Foodborne Clostridium perfringens intoxication
B  B99-B99  Other infectious diseases
#
A2. Neoplasms (C00-D48)
B  C00-C14  Malignant neoplasms of lip, oral cavity and pharynx
C    C00-C06  Malignant neoplasm of oral cavity
D      H00016  Oral cancer
C    C11  Malignant neoplasm of nasopharynx
D      H00054  Nasopharyngeal cancer
B  C15-C26  Malignant neoplasms of digestive organs
C    C15  Malignant neoplasm of oesophagus
D      H00017  Esophageal cancer
C    C16  Malignant neoplasm of stomach
D      H00018  Gastric cancer
C    C17  Malignant neoplasm of small intestine
D      H00034  Carcinoid
C    C18-C20  Malignant neoplasm of colon and rectum
D      H00020  Colorectal cancer
C    C21  Malignant neoplasm of anus and anal canal
D      H00044  Cancer of the anal canal
C    C22  Malignant neoplasm of liver and intrahepatic bile ducts
D      H00048  Hepatocellular carcinoma
D      H00046  Cholangiocarcinoma
C    C23  Malignant neoplasm of gallbladder
D      H00047  Gallbladder cancer
C    C25  Malignant neoplasm of pancreas
D      H00019  Pancreatic cancer
D      H00045  Malignant islet cell carcinoma
B  C30-C39  Malignant neoplasms of respiratory and intrathoracic organs
C    C32  Malignant neoplasm of larynx
D      H00055  Laryngeal cancer
C    C34  Malignant neoplasm of bronchus and lung
D      H00014  Non-small cell lung cancer
D      H00013  Small cell lung cancer
C    C38  Malignant neoplasm of heart, mediastinum and pleura
D      H00043  Neuroblastoma
B  C40-C41  Malignant neoplasms of bone and articular cartilage
C    C41  Malignant neoplasm of bone and articular cartilage of other and unspecified sites
D      H00036  Osteosarcoma
D      H00035  Ewing's sarcoma
D      H00053  Extraskeletal myxoid chondrosarcoma
B  C43-C44  Melanoma and other malignant neoplasms of skin
C    C43  Malignant melanoma of skin
D      H00038  Malignant melanoma
C    C44  Other malignant neoplasms of skin
D      H00039  Basal cell carcinoma
D      H00040  Squamous cell carcinoma
B  C45-C49  Malignant neoplasms of mesothelial and soft tissue
C    C45  Mesothelioma
D      H00015  Malignant pleural mesothelioma
C    C46  Kaposi's sarcoma
D      H00041  Kaposi's sarcoma
C    C47  Malignant neoplasm of peripheral nerves and autonomic nervous system
D      H00043  Neuroblastoma
C    C48  Malignant neoplasm of retroperitoneum and peritoneum
D      H00043  Neuroblastoma
C    C49  Malignant neoplasm of other connective and soft tissue
D      H00049  Myxoid liposarcoma
D      H00037  Alveolar rhabdomyosarcoma
D      H00050  Synovial sarcoma
D      H00051  Alveolar soft part sarcoma
D      H00052  Clear cell sarcoma of soft tissue
B  C50-C50  Malignant neoplasm of breast
C    C50  Malignant neoplasm of breast
D      H00031  Breast cancer
B  C51-C58  Malignant neoplasms of female genital organs
C    C51  Malignant neoplasm of vulva
D      H00029  Vulvar cancer
C    C53  Malignant neoplasm of cervix uteri
D      H00030  Cervical cancer
C    C54  Malignant neoplasm of corpus uteri
D      H00026  Endometrial Cancer
C    C56  Malignant neoplasm of ovary
D      H00027  Ovarian cancer
C    C58  Malignant neoplasm of placenta
D      H00028  Choriocarcinoma
B  C60-C63  Malignant neoplasms of male genital organs
C    C60  Malignant neoplasm of penis
D      H00025  Penile cancer
C    C61  Malignant neoplasm of prostate
D      H00024  Prostate cancer
C    C62  Malignant neoplasm of testis
D      H00023  Testicular cancer
B  C64-C68  Malignant neoplasms of urinary tract
C    C64  Malignant neoplasm of kidney, except renal pelvis
D      H00021  Renal cell carcinoma
C    C67  Malignant neoplasm of bladder
D      H00022  Bladder cancer
B  C69-C72  Malignant neoplasms of eye, brain and other parts of central nervous system
C    C71  Malignant neoplasm of brain
D      H00042  Glioma
C    C72  Malignant neoplasm of spinal cord, cranial nerves and other parts of central nervous system
D      H00043  Neuroblastoma
B  C73-C75  Malignant neoplasms of thyroid and other endocrine glands
C    C73  Malignant neoplasm of thyroid gland
D      H00032  Thyroid cancer
C    C74  Malignant neoplasm of adrenal gland
D      H00033  Adrenal carcinoma
D      H00043  Neuroblastoma
B  C76-C80  Malignant neoplasms of ill-defined, secondary and unspecified sites
C    C76  Malignant neoplasm of other and ill-defined sites
D      H00043  Neuroblastoma
B  C81-C96  Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue
C    C81  Hodgkin's disease
D      H00007  Hodgkin lymphoma
C    C83  Diffuse non-Hodgkin's lymphoma
D      H00008  Burkitt lymphoma
D      H00011  Lymphoplasmacytic lymphoma
C    C88  Malignant immunoproliferative diseases
D      H00011  Lymphoplasmacytic lymphoma
C    C90  Multiple myeloma and malignant plasma cell neoplasms
D      H00010  Multiple myeloma
C    C91  Lymphoid leukaemia
D      H00001  Acute lymphoblastic leukemia (ALL) (precursor B lymphoblastic leukemia)
D      H00002  Acute lymphoblastic leukemia (ALL) (precursor T lymphoblastic leukemia)
D      H00005  Chronic lymphocytic leukemia (CLL)
D      H00006  Hairy-cell leukemia
D      H00009  Adult T-cell leukemia
C    C92  Myeloid leukaemia
D      H00003  Acute myeloid leukemia (AML)
D      H00004  Chronic myeloid leukemia (CML)
B  C97-C97  Malignant neoplasms of independent (primary) multiple sites
B  D00-D09  In situ neoplasms
B  D10-D36  Benign neoplasms
C    D12  Benign neoplasm of colon, rectum, anus and anal canal
D      H01023  Juvenile polyposis syndrome
D      H01025  Familial adenomatous polyposis
C    D21  Other benign neoplasms of connective and other soft tissue
D      H00804  Multiple cutaneous and uterine leiomyomata
C    D23  Other benign neoplasms of skin
D      H00829  Multiple familial trichoepithelioma
D      H00947  Pilomatricoma
C    D31  Benign neoplasm of eye and adnexa
D      H01149  Ring dermoid of cornea
C    D33  Benign neoplasm of brain and other parts of central nervous system
D      H00502  Pallister-Hall Syndrome
D      H01007  Choroid plexus papilloma
C    D36  Benign neoplasm of other and unspecified sites
D      H00895  Basal cell nevus syndrome
B  D37-D48  Neoplasms of uncertain or unknown behaviour
C    D44  Neoplasm of uncertain or unknown behaviour of endocrine glands
D      H01102  Pituitary adenomas
C    D45  Polycythaemia vera
D      H00012  Polycythemia vera
#
A3. Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
B  D50-D53  Nutritional anaemias
C    D50  Iron deficiency anemia
D      H01145  Atransferrinemia
D      H01196  Hypochromic microcytic anemia
D      H01278  Iron-refractory iron deficiency anemia (IRIDA)
C    D51  Vitamin B12 deficiency anaemia
D      H01190  Transcobalamin II deficiency
D      H01277  Vitamin B12 deficiency anaemia
C    D52  Folate deficiency anaemia
D      H01252  Hereditary folate malabsorption (HFM)
C    D53  Other nutritional anaemias
D      H00198  Orotic aciduria
D      H01183  Thiamine-responsive megaloblastic anemia (TRMA)
B  D55-D59  Haemolytic anaemias
C    D55  Anemia due to enzyme disorders
D      H00664  Anemia due to disorders of glycolytic enzymes
D      H00668  Anemia due to disorders of glutathione metabolism
D      H00674  Anemia due to disorders of nucleotide metabolism
D      H01096  Pyruvate kinase (PK) deficiency
C    D56  Thalassaemia
D      H00228  Thalassemia
C    D57  Sickle-cell disorders
D      H00229  Sickle cell anemia
C    D58  Other hereditary haemolytic anaemias
D      H00230  Hereditary spherocytosis
D      H00231  Hereditary elliptocytosis
D      H00232  Hereditary stomatocytosis
D      H01125  Hereditary pyropoikilocytosis (HPP)
D      H01214  Rh-null hemolytic anemia (RHN)
C    D59  Acquired haemolytic anaemia
D      H01053  Paroxysmal nocturnal hemoglobinuria (PNH)
B  D60-D64  Aplastic and other anaemias
C    D61  Other aplastic anaemias
D      H00237  Diamond-Blackfan anemia
D      H00238  Fanconi anemia
D      H00439  Shwachman-Diamond syndrome (SDS)
D      H00788  Hoyeraal-Hreidarsson syndrome
D      H01132  Aplastic anemia (AA)
C    D64  Other anemias
D      H00917  Congenital dyserythropoietic anemias (CDAs)
D      H00982  Sideroblastic anemia
B  D65-D69  Coagulation defects, purpura and other haemorrhagic conditions
C    D66  Hereditary factor VIII deficiency
D      H00219  Hemophilia
C    D67  Hereditary factor IX deficiency
D      H00219  Hemophilia
C    D68  Other coagulation defects
D      H00219  Hemophilia
D      H01254  Prothrombin deficiency
D      H00220  Factor V Deficiency
D      H00222  Afibrinogenemia
D      H00223  Inherited thrombophilia
D      H00560  Pseudoxanthoma elasticum
D      H00938  Factor XI deficiency
D      H00941  Factor XII deficiency
D      H00945  Factor XIII deficiency
D      H00983  Alpha-2-plasmin inhibitor (a2-PI) deficiency
D      H00995  Combined deficiency of vitamin K-dependent clotting factors (VKCFD)
D      H01078  Fletcher factor deficiency
D      H01106  Plasminogen activator inhibitor type 1 (PAI-1) deficiency
C    D69  Purpura and other haemorrhagic conditions
D      H00224  Bernard-Soulier syndrome
D      H00226  Glanzmann thrombasthenia
D      H00225  Thrombotic thrombocytopenic purpura
D      H00227  Congenital amegakaryocytic thrombocytopenia
D      H00578  Epstein syndrome
D      H00867  Radioulnar synostosis with amegakaryocytic thrombocytopenia
D      H00978  Thrombocytopenia (THC)
D      H01162  Scott syndrome
D      H01235  Bleeding disorder platelet-type
D      H01240  Immune thrombocytopenia (ITP)
B  D70-D77  Other diseases of blood and blood-forming organs
C    D70  Agranulocytosis
D      H00100  Neutropenic disorders
D      H00939  Neutropenia, severe congenital 4, autosomal recessive
D      H01218  P14 deficiency
C    D71  Functional disorders of polymorphonuclear neutrophils
D      H00098  Chronic granulomatous disease
C    D72  Other disorders of white blood cells
D      H00233  Macrothrombocytopenia
D      H00234  Pelger-Huet anomaly
C    D74  Methaemoglobinaemia
D      H00235  Methemoglobinemia
C    D75  Other diseases of blood and blood-forming organs
D      H00236  Congenital polycythemia
C    D76  Certain diseases involving lymphoreticular tissue and reticulohistiocytic system
D      H00109  Familial hemophagocytic lymphohistiocytosis
D      H01168  Sea-blue histiocyte disease
B  D80-D89  Certain disorders involving the immune mechanism
C    D80  Immunodeficiency with predominantly antibody defects
D      H00085  Agammaglobulinemias
D      H00086  Hyper IgM syndromes, autosomal recessive type
D      H00087  Other humoral immunodeficiencies
D      H00088  Common variable immunodeficiency
C    D81  Combined immunodeficiencies
D      H00091  T-B+Severe combined immunodeficiencies
D      H00092  T-B-Severe combined immunodeficiencies
D      H01244  T+B+Severe combined immunodeficiencies
D      H00093  Combined immunodeficiencies
D      H00984  Bare lymphocyte syndrome (BLS) type1
D      H00985  Bare lymphocyte syndrome (BLS) type2
C    D82  Immunodeficiency associated with other major defects
D      H00094  DNA repair defects
D      H00931  Growth hormone insensitivity with immunodeficiency
D      H01004  Velocardiofacial syndrome
C    D84  Other immunodeficiencies
D      H01006  Hereditary angioedema
D      H01136  Carboxypeptidase N deficiency
C    D86  Sarcoidosis
D      H01309  Sarcoidosis, early-onset
C    D89  Other disorders involving the immune mechanism, not elsewhere classified
D      H00206  Mevalonate kinase deficiency
#
A4. Endocrine, nutritional and metabolic diseases (E00-E90)
B  E00-E07  Disorders of thyroid gland
C    E03  Other hypothyroidism
D      H00250  Congenital nongoitrous hypothyroidism (CHNG)
D      H00650  Allan-Herndon-Dudley syndrome
D      H00913  Choreoathetosis, hypothyroidism, and neonatal respiratory distress
D      H01040  Bamforth-Lazarus syndrome
D      H01186  Abnormal thyroid hormone metabolism
C    E05  Thyrotoxicosis (hyperthyroidism)
D      H01269  Congenital hyperthyroidism
C    E06  Thyroiditis
D      H00081  Hashimoto's thyroiditis
D      H00082  Graves' disease
C    E07  Other disorders of thyroid
D      H00251  Thyroid dyshormonogenesis
D      H00249  Thyroid hormone resistance syndrome
B  E10-E14  Diabetes mellitus
C    E10  Insulin-dependent diabetes mellitus
D      H00408  Type I diabetes mellitus
D      H00766  Wolcott-Rallison syndrome
D      H00854  Wolfram syndrome (WFS)
C    E11  Non-insulin-dependent diabetes mellitus
D      H00409  Type II diabetes mellitus
D      H00719  Leprechaunism
D      H01228  Insulin-resistant diabetes mellitus with acanthosis nigricans (IRAN)
C    E13  Other specified diabetes mellitus
D      H00410  Maturity onset diabetes of the young (MODY)
D      H00942  Rabson-Mendenhall syndrome
B  E15-E16  Other disorders of glucose regulation and pancreatic internal secretion
C    E16  Other disorders of pancreatic internal secretion
D      H01267  Familial hyperinsulinemic hypoglycemia (HHF)
B  E20-E35  Disorders of other endocrine glands
C    E20  Hypoparathyroidism
D      H00244  Pseudohypoparathyroidism
C    E21  Hyperparathyroidism and other disorders of parathyroid gland
D      H00246  Primary hyperparathyroidism
C    E22  Hyperfunction of pituitary gland
D      H01294  Nephrogenic syndrome of inappropriate antidiuresis (NSIAD)
C    E23  Hypofunction and other disorders of pituitary gland
D      H00254  Pituitary Dwarfism (PD)
D      H00255  Hypogonadotropic hypogonadism
D      H00253  Neurohypophyseal diabetes insipidus (NPDI)
D      H01011  Adrenocorticotropic hormone deficiency
D      H01253  Isolated follicle-stimulating hormone deficiency (IFSHD)
C    E25  Adrenogenital disorders
D      H00216  Congenital adrenal hyperplasia
D      H00608  46,XY disorders of sex development (Disorders in androgen synthesis or action)
D      H00599  46,XX disorders of sex development (Disorders related to androgen excess)
D      H01111  Cortisone reductase deficiency (CRD)
C    E26  Hyperaldosteronism
D      H00239  Bartter syndrome
D      H00240  Gitelman syndrome
D      H00602  Glucocorticoid-remediable aldosteronism (GRA)
C    E27  Other disorders of adrenal gland
D      H00258  Aldosterone synthase deficiency
D      H00259  Apparent mineralocorticoid excess syndrome
D      H00260  pigmented micronodular adrenocortical disease (PPNAD)
D      H01163  Corticosteroid-binding globulin (CBG) deficiency
C    E28  Ovarian dysfunction
D      H00627  Premature ovarian failure
C    E30  Disorders of puberty, not elsewhere classified
D      H00794  Aromatase excess syndrome
D      H00937  Precocious puberty
C    E34  Other endocrine disorders
D      H00492  Isolated short stature
D      H00601  Hutchinson-Gilford progeria syndrome
D      H00608  46,XY disorders of sex development (Disorders in androgen synthesis or action)
D      H00719  Leprechaunism
D      H00931  Growth hormone insensitivity with immunodeficiency
D      H01274  Growth delay due to insulin-like growth factor I resistance
D      H00296  Defects in RecQ helicases
B  E40-E46  Malnutrition
B  E50-E64  Other nutritional deficiencies
C    E50  Vitamin A deficiency
D      H01266  Hypercarotenemia and vitamin A deficiency
C    E53  Deficiency of other specified B group vitamins
D      H00180  Holocarboxylase synthetase deficiency
D      H01182  Biotinidase deficiency
C    E56  Other vitamin deficiencies
D      H00981  Ataxia with isolated vitamin E deficiency (AVED)
B  E65-E68  Obesity and other hyperalimentation
C    E66  Obesity
D      H00417  Alstrom syndrome (AS)
C    E67  Other hyperalimentation
D      H01266  Hypercarotenemia and vitamin A deficiency
B  E70-E90  Metabolic disorders
C    E70  Disorders of aromatic amino-acid metabolism
D      H00167  Phenylketonuria
D      H00165  Tyrosinemia
D      H00163  Alkaptonuria
D      H00166  Hermansky-Pudlak syndrome
D      H00168  Oculocutaneous albinism
D      H00169  Ocular albinism
D      H00170  Piebaldism
D      H00171  Histidinemia
D      H00548  Brunner syndrome
D      H00759  Waardenburg syndrome (WS)
D      H01233  Urocanase deficiency
D      H01262  Formiminotransferase deficiency
C    E71  Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
D      H00172  Maple syrup urine disease
D      H00173  Isovaleric acidemia
D      H00181  3-Methylcrotonylglycinuria
D      H00175  Propionic acidemia
D      H00174  Methylmalonic aciduria
D      H00179  3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
D      H00162  Sjogren-Larsson syndrome
D      H00176  Adrenoleukodystrophy
D      H00178  Glutaric acidemia
D      H00525  Disorders of fatty-acid oxidation
D      H00654  Barth syndrome (BTHS)
D      H00754  3-Methylglutaconic aciduria (MGCA)
D      H00925  2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency
D      H01121  Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency
D      H01123  HMG-CoA synthase (HMGCS) deficiency
D      H01279  Isobutyryl-CoA dehydrogenase (IBD) deficiency
C    E72  Other disorders of amino-acid metabolism
D      H00275  Cystinosis
D      H00843  Hartnup disorder
D      H00692  Lowe syndrome
D      H00182  Cystathioninuria
D      H00183  Homocystinuria
D      H00184  Hypermethioninemia
D      H00164  Carbamoyl phosphate synthetase I deficiency
D      H00185  Citrullinemia
D      H00186  Hyperargininemia
D      H00178  Glutaric acidemia
D      H00188  Hyperlysinemia
D      H00189  Ornithinaemia
D      H00187  Ornithine transcarbamylase deficiency
D      H00190  Hyperprolinemia
D      H00191  Nonketotic hyperglycinemia
D      H00200  Beta-ureidopropionase deficiency
D      H00834  Guanidinoacetate methyltransferase (GAMT) deficiency
D      H00835  Succinic semialdehyde dehydrogenase (SSADH) deficiency
D      H00849  Creatine deficiency syndrome
D      H00899  Lysinuric protein intolerance (LPI)
D      H00901  Cystinuria
D      H00905  Iminoglycinuria
D      H00923  Congenital systemic glutamine deficiency (CSGD)
D      H01003  Dimethylglycine dehydrogenase deficiency (DMGDHD)
D      H01028  Argininosuccinic aciduria (ARGINSA)
D      H01032  N-acetylglutamate synthase (NAGS) deficiency
D      H01079  3-Phosphoglycerate dehydrogenase (3-PGDH) deficiency
D      H01076  Alpha-methylacetoacetic aciduria
D      H01119  Prolidase deficiency (PD)
D      H01198  Fanconi renotubular syndrome (FTS)
D      H01237  Sulfite oxidase deficiency
D      H01242  Saccharopinuria
D      H01257  GABA-transaminase deficiency
D      H01268  Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome
D      H01283  Malonyl-CoA decarboxylase deficiency
D      H01285  Methylcobalamin deficiency type G (cblG)
C    E73  Lactose intolerance
D      H00116  Congenital lactase deficiency
C    E74  Other disorders of carbohydrate metabolism
D      H00069  Glycogen storage diseases
D      H00150  Danon disease
D      H00071  Hereditary fructose intolerance
D      H00114  Fructose-1,6-bisphosphatase deficiency
D      H00070  Galactosemia
D      H00115  Congenital sucrase-isomaltase deficiency
D      H00072  Pyruvate dehydrogenase complex deficiency
D      H00073  Pyruvate carboxylase deficiency
D      H00117  Primary hyperoxaluria
D      H00552  Glycerol kinase deficiency (GKD)
D      H01065  Pentosuria
D      H01126  Familial renal glucosuria (FRG)
D      H01189  Transaldolase (TALDO) deficiency
D      H01261  Congenital glucose-galactose malabsorption (GGM)
C    E75  Disorders of sphingolipid metabolism and other lipid storage disorders
D      H00124  GM2 gangliosidoses
D      H00281  GM1 gangliosidosis
D      H00144  Mucolipidosis IV
D      H00276  Galactosialidosis
D      H00125  Fabry disease
D      H00126  Gaucher disease
D      H00135  Krabbe disease
D      H00137  Niemann-Pick disease
D      H00136  Niemann-Pick type C disease
D      H00138  Farber lipogranulomatosis
D      H00127  Metachromatic leukodystrophy
D      H00272  Multiple sulfatase deficiency
D      H00074  Canavan disease
D      H00438  Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL)
D      H00149  Neuronal ceroid lipofuscinosis
D      H00148  Lysosomal acid lipase deficiency
D      H00151  Cerebrotendinous xanthomatosis
D      H00679  Pelizaeus-Merzbacher disease
D      H00736  Dorfman-Chanarin syndrome
D      H00869  Leukoencephalopathy with vanishing white matter
D      H00871  Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL)
D      H00874  Leukoencephalopathy with dystonia and motor neuropathy
D      H00875  Megalencephalic leukoencephalopathy with subcortical cysts (MLC)
D      H00878  Cystic leukoencephalopathy without megalencephaly
D      H00883  Lipoid proteinosis
D      H01230  Adult-onset autosomal dominant leukodystrophy (ADLD)
D      H01239  Combined SAP deficiency
D      H01297  Neutral lipid storage disease with myopathy
C    E76  Disorders of glycosaminoglycan metabolism
D      H00128  Mucopolysaccharidosis type I (MPS1)
D      H00129  Mucopolysaccharidosis type II (MPS2)
D      H00130  Mucopolysaccharidosis type III (MPS3)
D      H00123  Mucopolysaccharidosis type IV (MPS4)
D      H00131  Mucopolysaccharidosis type VI (MPS6)
D      H00132  Mucopolysaccharidosis type VII (MPS7)
D      H00133  Mucopolysaccharidosis type IX (MPS9)
C    E77  Disorders of glycoprotein metabolism
D      H00143  Mucolipidosis II and III
D      H00139  alpha-Mannosidosis
D      H00140  beta-Mannosidosis
D      H00141  Fucosidosis
D      H00142  Sialidosis
D      H00145  Aspartylglucosaminuria
D      H00146  Alpha-N-acetylgalactosaminidase deficiency
C    E78  Disorders of lipoprotein metabolism and other lipidaemias
D      H00155  Hypercholesterolemia
D      H00156  Hyperlipoproteinemia, type III
D      H00154  Hyperlipoproteinemia, type I
D      H00157  Hyperlipoproteinemia, type V
D      H00153  Familial combined hyperlipidemia
D      H00152  Sitosterolemia
D      H00158  Lecithin:cholesterol acyltransferase deficiency
D      H00159  Tangier disease
D      H00160  Abetalipoproteinemia
D      H00927  Chylomicron retention disease (CRD)
D      H00930  Hypoalphalipoproteinemia
D      H01101  Combined lipase deficiency (CLD)
D      H01199  Hyperalphalipoproteinemia
D      H01270  Familial hypobetalipoproteinemia (FHBL)
C    E79  Disorders of purine and pyrimidine metabolism
D      H00194  Lesch-Nyhan syndrome
D      H00193  Dihydropyrimidine dehydrogenase deficiency
D      H00192  Xanthinuria
D      H00197  Adenylosuccinate lyase deficiency
D      H00964  Thiopurine S-methyltransferase deficiency (TPMT deficiency)
D      H00966  AICA-ribosiduria
C    E80  Disorders of porphyrin and bilirubin metabolism
D      H00201  Erythropoietic porphyria
D      H00202  Hepatic porphyria
D      H00203  Acatalasia
D      H00208  Hyperbilirubinemia
C    E83  Disorders of mineral metabolism
D      H00209  Menkes disease
D      H00210  Wilson disease
D      H00211  Hemochromatosis
D      H00212  Acrodermatitis enteropathica
D      H00213  Hypophosphatasia
D      H00214  Hypophosphatemic rickets
D      H00557  Cutis laxa
D      H01113  Acid phosphatase deficiency
D      H01143  Vitamin D-dependent rickets
D      H01210  Hypomagnesemia
C    E84  Cystic fibrosis
D      H00218  Cystic fibrosis
C    E85  Amyloidosis
D      H00282  Muckle-Wells syndrome
D      H00288  Familial Mediterranean fever
D      H00845  Familial amyloidosis
C    E88  Other metabolic disorders
D      H00419  Congenital generalized lipodystrophy (CGL)
D      H00420  Familial partial lipodystrophy (FPL)
D      H00147  Sialuria/ Sialic acid storage disease
D      H00469  Mitochondrial DNA depletion syndrome (MDS)
D      H00473  Mitochondrial respiratory chain deficiencies (MRCD)
D      H00273  Pycnodysostosis
D      H01022  Diseases of the tricarboxylic acid cycle
D      H01127  Glycosylphosphatidylinositol (GPI) deficiency
D      H01234  Trimethylaminuria (TMAU)
#
A5. Mental and behavioural disorders (F00-F99)
B  F00-F09  Organic, including symptomatic, mental disorders
C    F01  Vascular dementia
D      H00536  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL)
B  F10-F19  Mental and behavioural disorders due to psychoactive substance use
B  F20-F29  Schizophrenia, schizotypal and delusional disorders
B  F30-F39  Mood [affective] disorders
B  F40-F48  Neurotic, stress-related and somatoform disorders
B  F50-F59  Behavioural syndromes associated with physiological disturbances and physical factors
B  F60-F69  Disorders of adult personality and behaviour
C    F63  Impulse disorders
D      H00864  Trichotillomania
B  F70-F79  Mental retardation
C    F78  Other mental retardation
D      H00480  Non-syndromic X-linked mental retardation
D      H00577  Syndromic X-linked mental retardation with epilepsy or seizures
D      H00574  Coffin-Lowry syndrome (CLS)
D      H00658  Syndromic X-linked mental retardation
D      H00768  Nonsyndromic autosomal recessive mental retardation (NS-ARMR)
D      H00773  Non-syndromic autosomal dominant mental retardation
C    F79  Unspecified mental retardation
D      H00889  MED12-related disorders
B  F80-F89  Disorders of psychological development
C    F84  Pervasive developmental disorders
D      H00440  Rett syndrome
B  F90-F98  Behavioural and emotional disorders with onset usually occurring in childhood and adolescence
C    F95  Tic disorder
D      H00862  Tourette syndrome
C    F98  Other behavioural and emotional disorders with onset usually occurring in childhood and adolescence
D      H01223  Mental retardation-stereotypic movements-epilepsy and/or cerebral malformations (MRSME)
B  F99-F99  Unspecified mental disorder
#
A6. Diseases of the nervous system (G00-G99)
B  G00-G09  Inflammatory diseases of the central nervous system
C    G00  Bacterial meningitis, not elsewhere classified
D      H00304  Haemophilus influenzae infection
D      H00333  Streptococcal infection
D      H01313  Escherichia coli meningitis
C    G03  Meningitis due to other and unspecified causes
D      H00282  Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome
B  G10-G14  Systemic atrophies primarily affecting the central nervous system
C    G10  Huntington's disease
D      H00059  Huntington's disease
C    G11  Hereditary ataxia
D      H00067  Friedreich ataxia
D      H01284  Marinesco-Sjogren syndrome
D      H00981  Ataxia with isolated vitamin E deficiency (AVED)
D      H00060  Dentatorubropallidoluysian atrophy (DRPLA)
D      H00064  Ataxia telangiectasia
D      H00094  DNA repair defects
D      H00266  Hereditary spastic paraplegia (SPG)
D      H00749  Episodic ataxias
D      H00848  Ataxia with ocular apraxia (AOA)
D      H00999  Coenzyme Q10 deficiency
D      H01036  Posterior column ataxia with retinitis pigmentosa (PCARP)
D      H01038  Cerebellar ataxia cayman type (ATCAY)
D      H01170  Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)
D      H01204  Cerebellar ataxia, mental retardation (MR), and dysequilibrium syndrome (CAMRQ)
C    G12  Spinal muscular atrophy and related syndromes
D      H00455  Spinal muscular atrophy (SMA)
D      H00062  Kennedy's disease
D      H00058  Amyotrophic lateral sclerosis
D      H00656  Scapuloperoneal myopathy (SPM)
D      H00841  Infantile progressive bulbar palsy
D      H00856  Distal hereditary motor neuropathies (dHMN)
D      H00524  TRPV4-related peripheral neuropathies
B  G20-G26  Extrapyramidal and movement disorders
C    G20  Parkinson's disease
D      H00057  Parkinson's disease
C    G23  Other degenerative diseases of basal ganglia
D      H00077  Progressive supranuclear palsy
D      H00832  Core neuroacanthocytosis syndromes
D      H00833  Neurodegeneration with brain iron accumulation (NBIA)
D      H01177  Infantile bilateral striatal necrosis (IBSN)
C    G24  Dystonia
D      H00831  Primary dystonia
D      H01161  Aromatic L-amino acid decarboxylase (AADC) deficiency
C    G25  Other extrapyramidal and movement disorders
D      H00769  Hyperekplexia
D      H00860  Benign hereditary chorea
B  G30-G32  Other degenerative diseases of the nervous system
C    G30  Alzheimer's disease
D      H00056  Alzheimer's disease
C    G31  Other degenerative diseases of nervous system, not elsewhere classified
D      H00078  Frontotemporal lobar degeneration (FTLD)
D      H00072  Pyruvate dehydrogenase complex deficiency
D      H00066  Lewy body dementia
D      H00290  Aicardi-Goutieres Syndrome
D      H00063  Spinocerebellar ataxia
D      H01184  Familial dementia
D      H01295  Neurodegeneration due to cerebral folate transport deficiency
B  G35-G37  Demyelinating diseases of the central nervous system
B  G40-G47  Episodic and paroxysmal disorders
C    G40  Epilepsy and recurrent seizures
D      H00606  Early infantile epileptic encephalopathy
D      H00783  Febrile seizures
D      H00806  Benign familial neonatal and infantile epilepsies
D      H00808  Idiopathic generalied epilepsies (IGEs)
D      H00810  Progressive myoclonic epilepsy (PME)
D      H00577  Syndromic X-linked mental retardation with epilepsy or seizures
D      H01124  Pyridox(am)ine-5'-phosphate oxidase (PNPO) deficiency
D      H01247  Pyridoxine-dependent epilepsy (PDE)
D      H01258  Generalized epilepsy and paroxysmal dyskinesia (GEPD)
C    G43  Migraine
D      H00775  Familial or sporadic hemiplegic migraine
C    G44  Other headache syndromes
D      H00282  Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome
C    G47  Sleep disorders
D      H00688  Familial advanced sleep phase syndrome
D      H00916  Congenital central hypoventilation syndrome (CCHS)
D      H01293  Narcolepsy
B  G50-G59  Nerve, nerve root and plexus disorders
C    G54  Nerve root and plexus disorders
D      H01131  Hereditary neuralgic amyotrophy (HNA)
C    G56  Mononeuropathies of upper limb
D      H00798  Familial carpal tunnel syndrome
B  G60-G64  Polyneuropathies and other disorders of the peripheral nervous system
C    G60  Hereditary and idiopathic neuropathy
D      H00264  Charcot-Marie-Tooth disease
D      H01155  Roussy-Levy syndrome
D      H00816  Agenesis of the corpus callosum with peripheral neuropathy (ACCPN)
D      H01296  Hereditary neuropathy with liability to pressure palsies (HNPP)
D      H00524  TRPV4-related peripheral neuropathies
D      H00075  Refsum disease
D      H00848  Ataxia with ocular apraxia (AOA)
D      H00774  Nav1.7-associated congenital insensitivity to pain (CIP)
D      H01220  Congenital cataracts, facial dysmorphism, and neuropathy (CCFDN)
D      H01259  Giant axonal neuropathy
B  G70-G73  Diseases of myoneural junction and muscle
C    G70  Myasthenia gravis and other myoneural disorders
D      H00770  Congenital myasthenic syndrome
C    G71  Primary disorders of muscles
D      H00120  Dystroglycanopathy
D      H00493  Heparan sulfate proteoglycan gene defects
D      H00562  Dystrophinopathies
D      H00563  Emery-Dreifuss muscular dystrophy
D      H00565  Sarcoglycanopathies
D      H00566  Dysferlinopathies
D      H00567  Caveolinopathies
D      H00568  Myotonic dystrophy (DM)
D      H00585  Epidermolysis bullosa simplex with muscular dystrophy (EBS-MD)
D      H00590  Congenital muscular dystrophies (CMD/MDC)
D      H00591  Facioscapulohumeral muscular dystrophy (FSHD)
D      H00704  Oculopharyngeal muscular dystrophy (OPMD)
D      H00592  Calpainopathy
D      H00593  Limb-girdle muscular dystrophy (LGMD)
D      H00594  Distal muscular dystrophies
D      H00595  Myofibrillar myopathies (MFM)
D      H00596  Nonaka distal myopathy (NM)
D      H00657  Reducing body myopathy (RBM)
D      H00697  X-linked myopathy with postural muscle atrophy (XMPMA)
D      H00698  Nemaline myopathy
D      H00699  Central core disease
D      H00700  Centronuclear myopathy
D      H00701  Congenital fiber type disproportion (CFTD)
D      H00702  Cap myopathy
D      H00703  Myosin storage myopathy (MSM)
D      H00705  Myotonia congenita
D      H00743  Paramyotonia congenita (PMC)
D      H00744  Potassium-aggravated myotonias (PAMs)
D      H00898  Myopathy with lactic acidosis and sideroblastic anaemia (MLASA)
D      H01129  Brody myopathy
D      H01200  Fatal infantile cardioencephalomyopathy
D      H01229  Inclusion body myopathy 3 (IBM3)
D      H01291  Spheroid body myopathy (SBM)
D      H01310  Multi-minicore disease (MmD)
C    G72  Other myopathies
D      H00745  Hyperkalemic periodic paralysis (HyperPP)
D      H00746  Hypokalemic periodic paralysis (HypoPP)
D      H00747  Thyrotoxic hypokalemic periodic paralysis (TPP)
D      H00748  Andersen-Tawil syndrome (ATS)
B  G80-G83  Cerebral palsy and other paralytic syndromes
C    G80  Cerebral palsy
D      H01097  Cerebral palsy
B  G90-G99  Other disorders of the nervous system
C    G90  Disorders of autonomic nervous system
D      H01005  Dopamine beta-hydroxylase deficiency
C    G93  Other disorders of brain
D      H00836  GLUT1 deficiency syndrome (GLUT1DS)
D      H00839  Porencephaly
C    G98  Other disorders of nervous system, not elsewhere classified
D      H00998  Alternating hemiplegia of childhood
#
A7. Diseases of the eye and adnexa (H00-H59)
B  H00-H06  Disorders of eyelid, lacrimal system and orbit
B  H10-H13  Disorders of conjunctiva
B  H15-H22  Disorders of sclera, cornea, iris and ciliary body
C    H16  Keratitis
D      H01273  Autosomal dominant keratitis (ADK)
C    H18  Other disorders of cornea
D      H00726  Meesmann corneal dystrophy
D      H00951  Reis-Bucklers corneal dystrophy (RBCD)
D      H00952  Thiel-Behnke dystrophies (TBCD)
D      H00953  Gelatinous drop-like corneal dystrophy (GDCD)
D      H00954  Macular corneal dystrophy (MCD)
D      H00955  Granular corneal dystrophies (GCD)
D      H00956  Lattice corneal dystrophies (LCD)
D      H00957  Fleck corneal dystrophy (FCD)
D      H00958  Congenital stromal corneal dystrophy (CSCD)
D      H00959  Schnyder corneal dystrophy (SCD)
D      H00960  Fuchs corneal dystrophy (FECD)
D      H00961  Posterior polymorphous corneal dystrophy (PPCD)
D      H00963  Congenital hereditary endothelial dystrophy (CHED)
D      H01221  Epithelial basement membrane corneal dystrophy (EBMD)
C    H19  Disorders of sclera and cornea in diseases classified elsewhere
D      H00789  Keratoconus
B  H25-H28  Disorders of lens
C    H26  Other cataract
D      H01256  Foveal hypoplasia
B  H30-H36  Disorders of choroid and retina
C    H31  Other disorders of choroid
D      H01116  Choroideremia (CHM)
C    H35  Other retinal disorders
D      H00417  Alstrom syndrome
D      H00481  Cone-rod dystrophy (CORD) and cone dystrophy (COD)
D      H00527  Retinitis pigmentosa (RP)
D      H00589  Familial exudative vitreoretinopathy (FEVR)
D      H00579  Hereditary angiopathy with nephropathy, aneurysms, and muscle cramps (HANAC)
D      H00779  Usher syndrome (US)
D      H00805  Vitreoretinal degeneration
D      H00814  Vitelliform macular dystrophy
D      H00819  Stargardt disease (STGD)
D      H00821  Macular degeneration
D      H00825  Familial flecked retina syndrome
D      H00837  Leber congenital amaurosis (LCR)
D      H01036  Posterior column ataxia with retinitis pigmentosa (PCARP)
D      H01180  Sveinsson chorioretinal atrophy (SCRA)
B  H40-H42  Glaucoma
C    H40  Glaucoma
D      H00612  Primary open angle glaucoma
B  H43-H45  Disorders of vitreous body and globe
B  H46-H48  Disorders of optic nerve and visual pathways
C    H47  Other disorders of optic [2nd] nerve and visual pathways
D      H00068  Leber optic atrophy
D      H01201  Jensen syndrome
B  H49-H52  Disorders of ocular muscles, binocular movement, accommodation and refraction
C    H49  Paralytic strabismus
D      H00838  Congenital fibrosis of the extraocular muscles (CFEOM)
D      H01118  Progressive external ophthalmoplegia (PEO)
C    H50  Other strabismus
D      H00633  Duane retraction syndrome
B  H53-H54  Visual disturbances and blindness
C    H53  Visual disturbances
D      H00787  Congenital stationary night blindness (CSNB)
D      H00971  Achromatopsia
D      H00974  Blue cone monochromacy
D      H00976  Colorblindness
B  H55-H59  Other disorders of eye and adnexa
C    H55  Nystagmus and other irregular eye movements
D      H00776  Congenital motor nystagmus (CMN)
#
A8. Diseases of the ear and mastoid process (H60-H95)
B  H60-H62  Diseases of external ear
B  H65-H75  Diseases of middle ear and mastoid
B  H80-H83  Diseases of inner ear
B  H90-H95  Other disorders of ear
C    H90  Conductive and sensorineural hearing loss
D      H01201  Jensen syndrome
D      H00712  Keratitis-ichthyosis-deafness syndrome
C    H91  Other hearing loss
D      H00604  Deafness, autosomal dominant
D      H00605  Deafness, autosomal recessive
D      H01209  Deafness, X-linked
#
A9. Diseases of the circulatory system (I00-I99)
B  I00-I02  Acute rheumatic fever
B  I05-I09  Chronic rheumatic heart diseases
B  I10-I15  Hypertensive diseases
C    I15  Secondary hypertension
D      H00602  Glucocorticoid-remediable aldosteronism (GRA)
D      H00259  Apparent mineralocorticoid excess syndrome
D      H00242  Liddle syndrome
D      H00258  Aldosterone synthase Deficiency
D      H00603  Hypertension exacerbated in pregnancy
B  I20-I25  Ischaemic heart diseases
B  I26-I28  Pulmonary heart disease and diseases of pulmonary circulation
B  I30-I52  Other forms of heart disease
C    I40  Acute myocarditis
D      H00295  Viral myocarditis
C    I42  Cardiomyopathy
D      H00292  Hypertrophic cardiomyopathy (HCM)
D      H00293  Arrhythmogenic right ventricular cardiomyopathy (ARVC)
D      H00294  Dilated cardiomyopathy (DCM)
D      H01219  Restrictive cardiomyopathy (RCM)
D      H01216  Left ventricular noncompaction (LVNC)
D      H00567  Caveolinopathies
C    I45  Other conduction disorders
D      H00720  Long QT syndrome
D      H01154  Wolff-Parkinson-White (WPW) syndrome
C    I47  Paroxysmal tachycardia
D      H01019  Catecholaminergic polymorphic ventricular tachycardia
C    I48  Atrial fibrillation and flutter
D      H00731  Atrial fibrillation
C    I49  Other cardiac arrhythmias
D      H00728  Brugada syndrome (BRS)
B  I60-I69  Cerebrovascular diseases
C    I67  Other cerebrovascular diseases
D      H00536  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL)
D      H00877  Brain small vessel disease with Axenfeld-Rieger anomaly
B  I70-I79  Diseases of arteries, arterioles and capillaries
C    I71  Aortic aneurysm and dissection
D      H00800  Loeys-Dietz syndrome (LDS)
D      H00801  Familial thoracic aortic aneurysm and dissection (TAAD)
C    I73  Other peripheral vascular diseases
D      H00771  Inherited erythromelalgia (IEM)
C    I78  Diseases of capillaries
D      H00533  Hereditary hemorrhagic telangiectasia (HHT)
B  I80-I89  Diseases of veins, lymphatic vessels and lymph nodes, not elsewhere classified
C    I89  Other noninfective disorders of lymphatic vessels and lymph nodes
D      H00896  Lymphangioleiomyomatosis (LAM)
B  I95-I99  Other and unspecified disorders of the circulatory system
#
A10. Diseases of the respiratory system (J00-J99)
B  J00-J06  Acute upper respiratory infections
C    J02  Acute pharyngitis
D      H00333  Streptococcal infection
C    J03  Acute tonsillitis
D      H00333  Streptococcal infection
B  J09-J18  Influenza and pneumonia
C    J09  Influenza due to identified avian influenza virus
D      H00399  Avian influenza
C    J10  Influenza due to other identified influenza virus
D      H00398  Influenza
C    J12  Viral pneumonia, not elsewhere classified
D      H00401  Respiratory syncytial virus infection
D      H00400  Parainfluenza infections
C    J13  Pneumonia due to Streptococcus pneumoniae
D      H01321  Pneumonia due to Streptococcus pneumoniae
C    J14  Pneumonia due to Haemophilus influenzae
D      H00304  Haemophilus influenzae infection
C    J15  Bacterial pneumonia, not elsewhere classified
D      H00301  Klebsiella infection
D      H00313  Pseudomonas aeruginosa infection
D      H00333  Streptococcal infection
D      H00341  Mycoplasma pneumonia
C    J16  Pneumonia due to other infectious organisms, not elsewhere classified
D      H00351  Chlamydial pneumonia
B  J20-J22  Other acute lower respiratory infections
C    J20  Acute bronchitis
D      H00341  Mycoplasma pneumonia
D      H00304  Haemophilus influenzae infection
D      H00400  Parainfluenza infections
D      H00401  Respiratory syncytial virus infection
C    J21  Acute bronchiolitis
D      H00401  Respiratory syncytial virus infection
B  J30-J39  Other diseases of upper respiratory tract
B  J40-J47  Chronic lower respiratory diseases
C    J43  Emphysema
D      H01103  Alpha-1-antitrypsin (A1AT) deficiency
C    J45  Asthma
D      H00079  Asthma
D      H01191  Asthma with nasal polyps and aspirin intolerance
B  J60-J70  Lung diseases due to external agents
B  J80-J84  Other respiratory diseases principally affecting the interstitium
C    J84  Other interstitial pulmonary diseases
D      H01299  Idiopathic pulmonary fibrosis
B  J85-J86  Suppurative and necrotic conditions of lower respiratory tract
B  J90-J94  Other diseases of pleura
C    J93  Pneumothorax
D      H01110  Pneumothorax
B  J95-J99  Other diseases of the respiratory system
C    J98  Other respiratory disorders
D      H00564  Primary ciliary dyskinesia
#
A11. Diseases of the digestive system (K00-K93)
B  K00-K14  Diseases of oral cavity, salivary glands and jaws
C    K00  Disorders of tooth development and eruption
D      H00432  Hereditary dentine disorders
D      H00615  Amelogenesis imperfecta
D      H00652  Solitary median maxillary central incisor syndrome
D      H01292  Nance-Horan syndrome
C    K05  Periodontosis
D      H01317  Periodontosis
C    K06  Other disorders of gingiva and edentulous alveolar ridge
D      H01250  Hereditary gingival fibromatosis (HGF)
C    K10  Other diseases of jaws
D      H00497  Cherubism
B  K20-K31  Diseases of oesophagus, stomach and duodenum
B  K35-K38  Diseases of appendix
B  K40-K46  Hernia
B  K50-K52  Noninfective enteritis and colitis
C    K50  Crohn's disease (regional enteritis)
D      H00286  Crohn's disease
B  K55-K63  Other diseases of intestines
C    K59  Other functional intestinal disorders
D      H00936  Goldberg-Shprintzen megacolon syndrome
B  K65-K67  Diseases of peritoneum
B  K70-K77  Diseases of liver
C    K74  Fibrosis and cirrhosis of liver
D      H01001  COACH syndrome
D      H01133  Reynolds syndrome
C    K76  Other diseases of liver
D      H00628  Congenital bile acid synthesis defect (CBAS)
D      H01264  Hepatic venoocclusive disease with immunodeficiency (VODI)
B  K80-K87  Disorders of gallbladder, biliary tract and pancreas
C    K80  Cholelithiasis
D      H01213  Gallbladder disease
C    K81  Cholecystitis
D      H01213  Gallbladder disease
C    K83  Other diseases of biliary tract
D      H00624  Familial cholestasis
C    K86  Other diseases of pancreas
D      H00933  Hereditary pancreatitis
B  K90-K93  Other diseases of the digestive system
C    K90  Intestinal malabsorption
D      H00352  Whipple's disease
#
A12. Diseases of the skin and subcutaneous tissue (L00-L99)
B  L00-L08  Infections of the skin and subcutaneous tissue
B  L10-L14  Bullous disorders
B  L20-L30  Dermatitis and eczema
B  L40-L45  Papulosquamous disorders
B  L50-L54  Urticaria and erythema
C    L50  Urticaria
D      H00282  Cryopyrin associated periodic syndrome (CAPS)
B  L55-L59  Radiation-related disorders of the skin and subcutaneous tissue
B  L60-L75  Disorders of skin appendages
C    L63  Alopecia areata
D      H01158  Alopecia universalis
C    L67  Hair colour and hair shaft abnormalities
D      H00671  Netherton syndrome
C    L72  Follicular cysts of skin and subcutaneous tissue
D      H00684  Steatocystoma multiplex
D      H00780  Atrichia with papular lesions
C    L73  Other follicular disorders
D      H00681  Acne inversa
B  L80-L99  Other disorders of the skin and subcutaneous tissue
C    L81  Other disorders of pigmentation
D      H00823  ABCD syndrome
D      H00885  Hypomelanosis of Ito
C    L94  Other localized connective tissue disorders
D      H01133  Reynolds syndrome
#
A13. Diseases of the musculoskeletal system and connective tissue (M00-M99)
B  M00-M03  Infectious arthropathies
B  M05-M14  Inflammatory polyarthropathies
C    M05  Seropositive rheumatoid arthritis
D      H00630  Rheumatoid arthritis
C    M08  Juvenile arthritis
D      H00282  Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome
C    M11  Other crystal arthropathies
D      H01193  Familial tumoral calcinosis (FTC)
D      H01236  Familial articular chondrocalcinosis
B  M15-M19  Arthrosis
B  M20-M25  Other joint disorders
C    M24  Other specific joint derangements
D      H00872  Trismus-pseudocamptodactyly syndrome
B  M30-M36  Systemic connective tissue disorders
C    M32  Systemic lupus erythematosus
D      H00080  Systemic lupus erythematosus
C    M35  Other systemic involvement of connective tissue
D      H00557  Cutis laxa
B  M40-M43  Deforming dorsopathies
B  M45-M49  Spondylopathies
B  M50-M54  Other dorsopathies
B  M60-M63  Disorders of muscles
C    M60  Myositis
D      H00333  Streptococcal infection
C    M61  Calcification and ossification of muscle
D      H00430  Fibrodysplasia ossificans progressiva (FOP)
B  M65-M68  Disorders of synovium and tendon
B  M70-M79  Other soft tissue disorders
C    M72  Fibroblastic disorders
D      H00614  Infantile systemic hyalinosis and juvenile hyaline fibromatosis
B  M80-M85  Disorders of bone density and structure
C    M85  Other disorders of bone density and structure
D      H00486  Endosteal hyperostosis
B  M86-M90  Other osteopathies
C    M88  Osteitis deformans (Paget's disease of bone)
D      H00437  Juvenile Paget's disease
C    M89  Other disorders of bone
D      H00437  Familial expansile osteolysis
D      H00613  Infantile cortical hyperostosis
D      H00623  Hajdu-Cheney syndrome
B  M91-M94  Chondropathies
B  M95-M99  Other disorders of the musculoskeletal system and connective tissue
#
A14. Diseases of the genitourinary system (N00-N99)
B  N00-N08  Glomerular diseases
C    N04  Nephrotic syndrome
D      H00576  Pierson syndrome
D      H00626  Nephrotic syndrome and focal segmental glomerulosclerosis
C    N07  Hereditary nephropathy, not elsewhere classified
D      H01260  Glomerulopathy with fibronectin deposits (GFND)
B  N10-N16  Renal tubulo-interstitial diseases
C    N11  Chronic tubulo-interstitial nephritis
D      H00537  Nephronophthisis
B  N17-N19  Renal failure
B  N20-N23  Urolithiasis
C    N20  Calculus of kidney and ureter
D      H00195  Adenine phosphoribosyltransferase deficiency
B  N25-N29  Other disorders of kidney and ureter
C    N25  Disorders resulting from impaired renal tubular function
D      H00428  Distal renal tubular acidosis (RTA type 1)
D      H00429  Proximal renal tubular acidosis (RTA type 2)
D      H00241  Combined proximal and distal renal tubular acidosis (RTA type 3)
D      H00243  Hyperkalemic distal renal tubular acidosis (RTA type 4)
D      H00252  Congenital nephrogenic diabetes insipidus (NDI)
B  N30-N39  Other diseases of urinary system
C    N37  Urethral disorders in diseases classified elsewhere
D      H00347  Chlamydial urethritis
C    N39  Other disorders of urinary system
D      H00279  Uropathogenic Escherichia coli (UPEC) infection
D      H00694  Dent disease
B  N40-N51  Diseases of male genital organs
C    N46  Male infertility
D      H00890  Azoospermia
B  N60-N64  Disorders of breast
B  N70-N77  Inflammatory diseases of female pelvic organs
B  N80-N98  Noninflammatory disorders of female genital tract
C    N98  Complications associated with artificial fertilization
D      H01039  Ovarian hyperstimulation syndrome (OHSS)
B  N99-N99  Other disorders of the genitourinary system
#
A15. Pregnancy, childbirth and the puerperium (O00-O99)
B  O00-O08  Pregnancy with abortive outcome
B  O10-O16  Oedema, proteinuria and hypertensive disorders in pregnancy, childbirth and the puerperium
B  O20-O29  Other maternal disorders predominantly related to pregnancy
B  O30-O48  Maternal care related to the fetus and amniotic cavity and possible delivery problems
B  O60-O75  Complications of labour and delivery
B  O80-O84  Delivery
B  O85-O92  Complications predominantly related to the puerperium
B  O94-O99  Other obstetric conditions, not elsewhere classified
#
A16. Certain conditions originating in the perinatal period (P00-P96)
B  P00-P04  Fetus and newborn affected by maternal factors and by complications of pregnancy, labour and delivery
B  P05-P08  Disorders related to length of gestation and fetal growth
B  P10-P15  Birth trauma
B  P20-P29  Respiratory and cardiovascular disorders specific to the perinatal period
C    P22  Respiratory distress of newborn
D      H00913  Choreoathetosis, hypothyroidism, and neonatal respiratory distress
C    P28  Other respiratory conditions originating in the perinatal period
D      H00217  Pulmonary surfactant metabolism dysfunction (SMDP)
B  P35-P39  Infections specific to the perinatal period
C    P36  Bacterial sepsis of newborn
D      H00333  Streptococcal infection
C    P35  Congenital viral diseases
D      H00395  Rubella
D      H00368  Cytomegalovirus infection
B  P50-P61  Haemorrhagic and haematological disorders of fetus and newborn
B  P70-P74  Transitory endocrine and metabolic disorders specific to fetus and newborn
C    P70  Transitory disorders of carbohydrate metabolism specific to fetus and newborn
D      H00512  Permanent neonatal diabetes mellitus (PNDM)
D      H00513  Transient neonatal diabetes mellitus (TNDM)
B  P75-P78  Digestive system disorders of fetus and newborn
B  P80-P83  Conditions involving the integument and temperature regulation of fetus and newborn
B  P90-P96  Other disorders originating in the perinatal period
#
A17. Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
B  Q00-Q07  Congenital malformations of the nervous system
C    Q02  Microcephaly
D      H00269  Primary microcephaly (MCPH)
D      H00616  Bowen-Conradi syndrome (BCS)
D      H00990  Microcephaly, Amish type
C    Q03  Congenital hydrocephalus
D      H01034  L1 syndrome
C    Q04  Other congenital malformations of brain
D      H00120  Dystroglycanopathy
D      H00267  Holoprosencephaly
D      H00268  Lissencephaly (LIS)
D      H00270  Periventricular nodular heterotopia
D      H00271  Polymicrogyria
D      H00263  Acrocallosal syndrome
D      H00544  Septo-optic dysplasia
D      H00530  Joubert syndrome
D      H00590  Congenital muscular dystrophies (CMD/MDC)
D      H00756  Pitt-Hopkins syndrome
D      H00839  Porencephaly
D      H00792  Warburg micro syndrome
D      H00897  Pontocerebellar hypoplasia
D      H01001  COACH syndrome
D      H01160  Schizencephaly
D      H01251  Focal cortical dysplasia of Taylor
C    Q05  Spina bifida
D      H00262  Spina Bifida
B  Q10-Q18  Congenital malformations of eye, ear, face and neck
C    Q10  Congenital malformations of eyelid, lacrimal apparatus and orbit
D      H00677  Aplasia of lacrimal and salivary glands
D      H00826  Blepharophimosis-ptosis-epicanthus inversus syndrome
C    Q11  Anophthalmos, microphthalmos and macrophthalmos
D      H01027  Anophthalmia and microphthalmia (A/M)
C    Q12  Congenital lens malformations
D      H00676  Congenital primary aphakia
D      H00662  Ectopia lentis
D      H01202  Cataract
D      H01292  Nance-Horan syndrome
C    Q13  Congenital malformations of anterior segment of eye
D      H00635  Aniridia
D      H01029  Cornea plana congenita
D      H01075  Peters anomaly
D      H00620  Axenfeld-Rieger syndrome (ARS)
D      H01220  Congenital cataracts, facial dysmorphism, and neuropathy (CCFDN)
D      H01159  Anterior segment dysgenesis (ASD)
C    Q14  Congenital malformations of posterior segment of eye
D      H01026  Renal coloboma syndrome (RCS)
C    Q15  Other congenital malformations of eye
D      H01203  Primary congenital glaucoma (PCG)
C    Q18  Other congenital malformations of face and neck
D      H00817  Branchiooculofacial syndrome
B  Q20-Q28  Congenital malformations of the circulatory system
C    Q20  Congenital malformations of cardiac chambers and connections
D      H00550  Transposition of great arteries
D      H00918  Conotruncal heart malformations
D      H01263  Progressive cardiac conduction defect (PCCD)
C    Q21  Congenital malformations of cardiac septa
D      H00546  Atrial septal defect
D      H00547  Atrioventricular septal defect
D      H00549  Tetralogy of Fallot
C    Q23  Congenital malformations of aortic and mitral valves
D      H00554  Bicuspid aortic valve
D      H01272  Hypoplastic left heart syndrome (HLHS)
C    Q25  Congenital malformations of great arteries
D      H00553  Congenital supravalvar aortic stenosis
C    Q27  Other congenital malformations of peripheral vascular system
D      H00531  Venous malformations
D      H00919  Arterial tortuosity syndrome
B  Q30-Q34  Congenital malformations of the respiratory system
B  Q35-Q37  Cleft lip and cleft palate
C    Q35  Cleft palate
D      H00909  Cleft palate with ankyloglossia
C    Q36  Cleft lip
D      H00516  Isolated orofacial clefts
C    Q37  Cleft palate with cleft lip
D      H00516  Isolated orofacial clefts
B  Q38-Q45  Other congenital malformations of the digestive system
C    Q38  Congenital malformations of lips, not elsewhere classified
D      H00611  Van der Woude syndrome
D      H00677  Aplasia of lacrimal and salivary glands
C    Q42  Congenital absence, atresia and stenosis of large intestine
D      H00463  Currarino syndrome
C    Q43  Other congenital malformations of intestine
D      H00908  Mowat-Wilson syndrome
D      H00910  Hirschsprung disease (HD)
C    Q44  Congenital malformations of gallbladder, bile ducts and liver
D      H00545  Polycystic liver disease
D      H00551  Alagille syndrome
C    Q45  Other congenital malformations of digestive system
D      H00571  Johanson-Blizzard syndrome
D      H00861  Pancreatic agenesis
B  Q50-Q56  Congenital malformations of genital organs
C    Q51  Congenital malformations of uterus and cervix
D      H00460  Hand-foot-genital syndrome
C    Q54  Hypospadias
D      H00609  46,XY disorders of sex development (Other)
C    Q55  Other congenital malformations of male genital organs
D      H00609  46,XY disorders of sex development (Other)
D      H01033  Congenital bilateral absence of vas deferens
C    Q56  Indeterminate sex and pseudohermaphroditism
D      H00608  46,XY disorders of sex development (Disorders in androgen synthesis or action)
D      H00598  46,XX disorders of sex development (Disorders of gonadal development)
B  Q60-Q64  Congenital malformations of the urinary system
C    Q60  Renal agenesis and other reduction defects of kidney
D      H01026  Renal coloboma syndrome (RCS)
D      H00822  Renal agenesis and Renal adysplasia
C    Q61  Cystic kidney disease
D      H00261  Meckel syndrome
D      H00537  Medullary cystic kidney disease 2
D      H00541  Glomerulocystic kidney disease
D      H00542  Polycystic kidney disease
B  Q65-Q79  Congenital malformations and deformations of the musculoskeletal system
C    Q66  Congenital deformities of feet
D      H00903  Congenital clubfoot
C    Q68  Other congenital musculoskeletal deformities
D      H00461  FLNB-related disorders
D      H00611  Popliteal pterygium syndrome
D      H00872  Trismus-pseudocamptodactyly syndrome
D      H01246  Isolated congenital nail clubbing (ICNC)
C    Q69  Polydactyly
D      H01226  GLI3 morphopathies
C    Q70  Syndactyly
D      H00484  Other brachydactylies
D      H01095  Syndactyly
C    Q71  Reduction defects of upper limb
D      H00629  Acheiropodia
D      H00637  Ulnar-mammary syndrome
C    Q72  Reduction defects of lower limb
D      H00629  Acheiropodia
C    Q73  Reduction defects of unspecified limb
D      H00572  ESCO2-related disorders
C    Q74  Other congenital malformations of limb(s)
D      H00464  Small patella syndrome
D      H00521  Cleidocranial dysplasia
D      H00538  Senior-Loken syndrome
D      H00811  Distal arthrogryposis (DA)
D      H00855  Triphalangeal thumb
C    Q75  Craniosynostosis
D      H01207  Trigonocephaly
D      H00458  Craniosynostosis
D      H00475  Enlarged parietal foramina/cranium bifidum
D      H00491  Craniometaphyseal dysplasia
D      H00503  Weyers acrodental dysostosis
D      H00610  Treacher Collins syndrome
D      H00850  Frontorhiny
C    Q76  Congenital malformations of spine and bony thorax
D      H00517  Spondylocostal dysostosis
D      H00852  Klippel-Feil syndrome(KFS)
D      H00979  Caudal regression syndrome and Sirenomelia
C    Q77  Osteochondrodysplasia with defects of growth of tubular bones and spine
D      H00751  Asphyxiating thoracic dystrophy
D      H00207  Rhizomelic chondrodysplasia punctata
D      H01194  X-linked chondrodysplasia punctata
D      H00452  Melorheostosis
D      H00505  Achondroplasia/Hypochondroplasia
D      H00515  Diastrophic dysplasia
D      H00503  Ellis-van Creveld syndrome
D      H00519  Otospondylomegaepiphyseal dysplasia (OSMED)
D      H00766  Wolcott-Rallison syndrome
D      H00456  Melnick-Needles syndrome
D      H00466  Grebe dysplasia
D      H00470  Acromesomelic dysplasia, Maroteaux type
D      H00492  Leri-Weill syndrome
D      H00520  Type II collagenopathies
D      H00522  Metatropic dysplasia
C    Q78  Other osteochondrodysplasias
D      H00452  Buschke-Ollendorff syndrome
D      H00456  Frontometaphyseal dysplasia
D      H00450  Sclerosing bone dysplasias
D      H00447  HEM skeletal dysplasia
D      H00444  Osteopathia striata with cranial sclerosis
D      H00518  RMRP related disorders
D      H00434  Camurati-Engelmann disease
D      H00486  Sclerosteosis
D      H00461  FLNB-related disorders
D      H00491  Craniometaphyseal dysplasia
D      H00493  Multiple exostoses
D      H00494  Desbuquois syndrome
D      H00506  Osteogenesis imperfecta
D      H00514  Bruck syndrome
D      H00515  The DTDST-related disorders
D      H00479  Metaphyseal dysplasia
D      H00436  Osteopetrosis
D      H00122  Multiple exostoses
D      H00508  Blomstrand syndrome
D      H01018  Metachondromatosis
C    Q79  Congenital malformations of the musculoskeletal system, not elsewhere classified
D      H00121  Ehlers-Danlos syndrome, progeroid form
D      H00802  Ehlers-Danlos syndrome (EDS)
D      H01241  Congenital diaphragmatic hernia (CDH)
B  Q80-Q89  Other congenital malformations
C    Q80  Congenital ichthyosis
D      H00735  Ichthyosis vulgaris
D      H00134  X-linked ichthyosis
D      H00734  Lamellar ichthyosis (LI) and Non-bullous congenital ichthyosiform erythroderma (NBCIE)
D      H00691  Epidermolytic hyperkeratosis
D      H00733  Harlequin ichthyosis
D      H00712  Keratitis-ichthyosis-deafness syndrome
D      H00671  Netherton syndrome
D      H00693  Ichthyosis bullosa of Siemens
D      H00739  Ichthyosis with hypotrichosis
C    Q81  Epidermolysis bullosa
D      H00584  Epidermolysis bullosa, simplex
D      H00585  Epidermolysis bullosa, hemidesmosomal
D      H00586  Epidermolysis bullosa, junctional
D      H00587  Epidermolysis bullosa, dysprophic
C    Q82  Other congenital malformations of skin
D      H00403  Disorders of nucleotide excision repair
D      H00274  Papillon-Lefevre syndrome
D      H00296  Defects in RecQ helicases
D      H00507  Dyskeratosis congenita (DC)
D      H00535  Lymphedemas
D      H00558  Geroderma osteodysplasticum
D      H00560  Pseudoxanthoma elasticum
D      H00645  Incontinentia pigmenti
D      H00651  Ectodermal dysplasia, anhidrotic
D      H00638  Ectrodactyly-ectodermal dysplasia cleft-palate syndrome (EEC syndrome)
D      H00648  Ectodermal dysplasia, hidrotic
D      H00663  Restrictive dermopathy
D      H00695  Mal de Meleda
D      H00714  Vohwinkel syndrome
D      H00715  Darier disease
D      H00722  Epidermolytic palmoplantar keratoderma (EPPK)
D      H00723  Non-epidermolytic palmoplantar keratoderma (NEPPK)
D      H00750  Keratosis follicularis spinulosa decalvans
D      H00796  Dermatopathia pigmentosa reticularis
D      H00844  Hailey-Hailey disease
D      H00866  Trichothiodystrophy non-photosensitive type 1 (TTDN1)
D      H00949  Focal dermal hypoplasia
C    Q84  Other congenital malformations of integument
D      H00670  Monilethrix
D      H00667  Woolly hair
D      H00684  Pachyonychia congenita
C    Q85  Phakomatoses, not elsewhere classified
D      H00523  Noonan syndrome
D      H00539  Cowden syndrome
D      H00559  von Hippel-Lindau syndrome
D      H00666  Peutz-Jeghers syndrome
D      H00915  Tuberous sclerosis complex (TSC)
D      H01222  Cowden-like syndrome
C    Q87  Other specified congenital malformation syndromes affecting multiple systems
D      H00458  Craniosynostosis
D      H00076  Cockayne syndrome
D      H00478  Prader-Willi and Angelman syndromes
D      H00403  Disorders of nucleotide excision repair
D      H00418  Bardet-Biedl syndrome (BBS)
D      H00454  Oral-facial-digital syndrome 1
D      H00442  Campomelic dysplasia (CD)
D      H00443  Osteoglophonic dysplasia (OD)
D      H00433  Holt-Oram syndrome
D      H00641  ADULT syndrome
D      H00847  Al-Awadi/Raas-Rothschild syndrome
D      H00611  IRF6-related disorders
D      H00449  Oculodentodigital dysplasia
D      H00453  Branchio-oto-renal syndrome
D      H00461  FLNB-related disorders
D      H00464  Nail-patella syndrome
D      H00484  Other brachydactylies
D      H00492  Langer syndrome
D      H00493  Dyssegmental dysplasia, Silverman-Handmaker type
D      H00493  Simpson-Golabi-Behmel syndrome
D      H00496  Congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD)
D      H00504  Rubinstein-Taybi syndrome
D      H00205  Zellweger syndrome spectrum
D      H00509  3M syndrome
D      H00510  Feingold syndrome
D      H00161  Smith-Lemli-Opitz syndrome
D      H00516  Isolated orofacial clefts
D      H00519  Type XI collagenopathies
D      H00520  Stickler syndrome 1
D      H00522  Parastremmatic dysplasia
D      H00523  Noonan syndrome
D      H00529  Cranioectodermal dysplasia
D      H00530  Joubert syndrome
D      H00539  PTEN hamartoma tumor syndrome (PHTS)
D      H00569  Aarskog-Scott syndrome
D      H00556  CHARGE syndrome
D      H00561  Smith-Magenis syndrome
D      H00571  Johanson-Blizzard syndrome
D      H00573  Townes-Brocks syndrome
D      H00581  Alport syndrome
D      H00607  Denys-Drash syndrome and WAGR syndrome
D      H00608  46,XY disorders of sex development (Disorders in androgen synthesis or action)
D      H00617  Desmosterolosis
D      H00622  Hypoparathyroidism-retardation-dysmorphism syndrome
D      H00631  Cornelia de Lange syndrome (CdLS)
D      H00639  Ectodermal dysplasia, ectrodactyly, and macular dystrophy (EEM syndrome)
D      H00640  Limb-mammary syndrome
D      H00643  Tooth and nail syndrome
D      H00673  Weill-Marchesani syndrome
D      H00665  Mandibuloacral dysplasia
D      H00669  Naxos disease and Carvajal syndrome
D      H00711  Russell-Silver syndrome
D      H00713  Beckwith-Wiedemann syndrome
D      H00718  Sotos syndrome and Weaver syndrome
D      H00653  Marfan syndrome
D      H00660  Congenital contractural arachnodactyly
D      H00687  Fraser syndrome
D      H00752  Ankyloblepharon-ctodermal defects-cleft lip/palate (AEC) syndrome and Rapp-Hodgkin syndrome
D      H00811  Distal arthrogryposis (DA)
D      H00757  Dyggve-Melchior-Clausen disease and Smith-McCort dysplasia
D      H00797  Martsolf syndrome
D      H00785  Hypotrichosis, congenital, with juvenile macular dystrophy
D      H00889  MED12-related disorders
D      H00894  FG syndrome (FGS)
D      H00868  Stapes ankylosis with broad thumb and toes
D      H00886  Donnai-Barrow syndrome
D      H00922  Schinzel-Giedion midface retraction syndrome
D      H00935  Cold-induced sweating syndrome (CISS) and Crisponi syndrome (CRISPS)
D      H00940  Cohen syndrome
D      H00943  TARP syndrome
D      H00946  Arts syndrome
D      H00965  RAPADILINO syndrome
D      H00997  CATSHL syndrome
D      H00986  Multiple pterygium syndrome
D      H00987  Fetal akinesia deformation sequence (FADS)
D      H00993  Microcephalic osteodysplastic primordial dwarfism, type I (MOPD I)
D      H00991  Microcephalic osteodysplastic primordial dwarfism, type II (MOPD II)
D      H00992  Seckel syndrome
D      H01008  C syndrome
D      H01195  VACTERL/VATER association
D      H01265  Hydrolethalus syndrome
D      H01271  Hypoparathyroidism with sensorineural deafness and renal dysplasia
D      H01281  Lathosterolosis
C    Q89  Other congenital malformations, not elsewhere classified
D      H00570  Kabuki syndrome
D      H00950  Arthrogryposis, renal dysfunction, and cholestasis
B  Q90-Q99  Chromosomal abnormalities, not elsewhere classified
C    Q93  Monosomies and deletions from the autosomes, not elsewhere classified
D      H00478  Prader-Willi and Angelman syndromes
D      H00764  Cri du chat syndrome
D      H01223  Mental retardation-stereotypic movements-epilepsy and/or cerebral malformations (MRSME)
D      H01238  Phelan-McDermid syndrome
C    Q97  Other sex chromosome abnormalities, female phenotype, not elsewhere classified
D      H00607  46,XY disorders of sex development (Disorders of gonadal development)
C    Q99  Other chromosome abnormalities, not elsewhere classified
D      H00465  Fragile X Syndrome
D      H00598  46,XX disorders of sex development (Disorders of gonadal development)
#
A18. Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R99)
B  R00-R09  Symptoms and signs involving the circulatory and respiratory systems
B  R10-R19  Symptoms and signs involving the digestive system and abdomen
B  R20-R23  Symptoms and signs involving the skin and subcutaneous tissue
B  R25-R29  Symptoms and signs involving the nervous and musculoskeletal systems
B  R30-R39  Symptoms and signs involving the urinary system
B  R40-R46  Symptoms and signs involving cognition, perception, emotional state and behaviour
C    R31  Unspecified haematuria
D      H00579  Hereditary angiopathy with nephropathy, aneurysms, and muscle cramps (HANAC)
B  R47-R49  Symptoms and signs involving speech and voice
B  R50-R69  General symptoms and signs
C    R50  Fever of other and unknown origin
D      H00912  Tumor necrosis factor receptor-associated periodic syndrome (TRAPS)
C    R56  Convulsions, not elsewhere classified
D      H00783  Febrile seizures
B  R70-R79  Abnormal findings on examination of blood, without diagnosis
B  R80-R82  Abnormal findings on examination of urine, without diagnosis
C    R82  Other abnormal findings in urine
D      H01290  Acute recurrent myoglobinuria
B  R83-R89  Abnormal findings on examination of other body fluids, substances and tissues, without diagnosis
B  R90-R94  Abnormal findings on diagnostic imaging and in function studies, without diagnosis
B  R95-R99  Ill-defined and unknown causes of mortality
#
A19. Injury, poisoning and certain other consequences of external causes (S00-T98)
B  S00-S09  Injuries to the head
B  S10-S19  Injuries to the neck
B  S20-S29  Injuries to the thorax
B  S30-S39  Injuries to the abdomen, lower back, lumbar spine and pelvis
B  S40-S49  Injuries to the shoulder and upper arm
B  S50-S59  Injuries to the elbow and forearm
B  S60-S69  Injuries to the wrist and hand
B  S70-S79  Injuries to the hip and thigh
B  S80-S89  Injuries to the knee and lower leg
B  S90-S99  Injuries to the ankle and foot
B  T00-T07  Injuries involving multiple body regions
B  T08-T14  Injuries to unspecified part of trunk, limb or body region
B  T15-T19  Effects of foreign body entering through natural orifice
B  T20-T25  Burns and corrosions of external body surface, specified by site
B  T26-T28  Burns and corrosions confined to eye and internal organs
B  T29-T32  Burns and corrosions of multiple and unspecified body regions
B  T33-T35  Frostbite
B  T36-T50  Poisoning by drugs, medicaments and biological substances
B  T51-T65  Toxic effects of substances chiefly nonmedicinal as to source
B  T66-T78  Other and unspecified effects of external causes
B  T79-T79  Certain early complications of trauma
B  T80-T88  Complications of surgical and medical care, not elsewhere classified
C    T86  Failure and rejection of transplanted organs and tissues
D      H00083  Allograft rejection
D      H00084  Graft-versus-host disease
B  T90-T98  Sequelae of injuries, of poisoning and of other consequences of external causes
#
A20. External causes of morbidity and mortality (V01-Y98)
B  V01-V09  Pedestrian injured in transport accident
B  V10-V19  Pedal cyclist injured in transport accident
B  V20-V29  Motorcycle rider injured in transport accident
B  V30-V39  Occupant of three-wheeled motor vehicle injured in transport accident
B  V40-V49  Car occupant injured in transport accident
B  V50-V59  Occupant of pick-up truck or van injured in transport accident
B  V60-V69  Occupant of heavy transport vehicle injured in transport accident
B  V70-V79  Bus occupant injured in transport accident
B  V80-V89  Other land transport accidents
B  V90-V94  Water transport accidents
B  V95-V97  Air and space transport accidents
B  V98-V99  Other and unspecified transport accidents
B  W00-W19  Falls
B  W20-W49  Exposure to inanimate mechanical forces
B  W50-W64  Exposure to animate mechanical forces
B  W65-W74  Accidental drowning and submersion
B  W75-W84  Other accidental threats to breathing
B  W85-W99  Exposure to electric current, radiation and extreme ambient air temperature and pressure
B  X00-X09  Exposure to smoke, fire and flames
B  X10-X19  Contact with heat and hot substances
B  X20-X29  Contact with venomous animals and plants
B  X30-X39  Exposure to forces of nature
B  X40-X49  Accidental poisoning by and exposure to noxious substances
B  X50-X57  Overexertion, travel and privation
B  X58-X59  Accidental exposure to other and unspecified factors
B  X60-X84  Intentional self-harm
B  X85-Y09  Assault
B  Y10-Y34  Event of undetermined intent
B  Y35-Y36  Legal intervention and operations of war
B  Y40-Y59  Drugs, medicaments and biological substances causing adverse effects in therapeutic use
B  Y60-Y69  Misadventures to patients during surgical and medical care
B  Y70-Y82  Medical devices associated with adverse incidents in diagnostic and therapeutic use
B  Y83-Y84  Surgical and other medical procedures as the cause of abnormal reaction of the patient, or of later complication, without mention of misadventure at the time of the procedure
B  Y85-Y89  Sequelae of external causes of morbidity and mortality
B  Y90-Y98  Supplementary factors related to causes of morbidity and mortality classified elsewhere
#
A21. Factors influencing health status and contact with health services (Z00-Z99)
B  Z00-Z13  Persons encountering health services for examination and investigation
B  Z20-Z29  Persons with potential health hazards related to communicable diseases
B  Z30-Z39  Persons encountering health services in circumstances related to reproduction
B  Z40-Z54  Persons encountering health services for specific procedures and health care
B  Z55-Z65  Persons with potential health hazards related to socioeconomic and psychosocial circumstances
B  Z70-Z76  Persons encountering health services in other circumstances
B  Z80-Z99  Persons with potential health hazards related to family and personal history and certain conditions influencing health status
C    Z80  Family history of malignant neoplasm
D      H00539  PTEN hamartoma tumor syndrome (PHTS)
D      H00881  Li-Fraumeni syndrome
#
A22. Codes for special purposes (U00-U99)
B  U00-U49  Provisional assignment of new diseases of uncertain etiology
C    U04  SARS
D      H00402  Severe acute respiratory syndrome
B  U80-U89  Bacterial agents resistant to antibiotics
C    U80  Agent resistant to penicillin and related antibiotics
D      H00330  Methicillin-resistant Staphylococcal aureus (MRSA) infection
C    U81  Agent resistant to vancomycin and related antibiotics
D      H00331  Vancomycin-resistant Staphylococcal aureus (VRSA) infection
!
#<hr>
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#Last updated: December 19, 2012
#<br>Classification taken from the <a href="http://apps.who.int/classifications/apps/icd/icd10online/">World Health Organization</a>
#<br><br><a href="/kegg-bin/get_htext?jp08403.keg">&raquo; Japanese version</a>
