Details of Disease

General Information of Disease (ID: DISZ74WG)

Disease Name	Constitutional neutropenia
Synonyms	Kostmann syndrome; Kostmann disease; Kostmann neutropenia; infantile genetic agranulocytosis; congenital neutropenia; genetic infantile agranulocytosis
Disease Class	4B00: Neutropenia
Definition	A rare disorder characterized by recurrent infantile infections and absence of neutrophils in the peripheral blood.
Disease Hierarchy	DISGCAMX: Neutropenia DISAWDAB: Congenital hematological disorder DISZ74WG: Constitutional neutropenia
ICD Code	ICD-11 ICD-11: 4B00.0
Disease Identifiers	MONDO ID MONDO_0015134 UMLS CUI C3805116 MedGen ID 1785816 Orphanet ID 101987 SNOMED CT ID 1156300000

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 4 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Epoietin delta	DM9L0X9	Approved	NA	[1]
Lenogastrim	DMDFL5Q	Approved	NA	[1]
Mirimostim	DMRV0DT	Approved	NA	[1]
Nartograstim	DMIJP5W	Approved	NA	[1]
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This Disease is Treated as An Indication in 4 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Talabostat	DM1IZ5D	Phase 3	Small molecular drug	[2]
GORALATIDE	DMYWA0Z	Phase 2	Small molecular drug	[3]
ICA-17043	DMRFY7I	Phase 2	Small molecular drug	[4]
CTCE-0214	DM2HES0	Phase 1	NA	[5]
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This Disease is Treated as An Indication in 2 Discontinued Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Glaspimod	DM9TP56	Discontinued in Phase 2	NA	[6]
VX-366	DMH4U87	Discontinued in Phase 2	NA	[7]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

References

1	Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
2	Phase II trial of single agent Val-boroPro (Talabostat) inhibiting Fibroblast Activation Protein in patients with metastatic colorectal cancer. Cancer Biol Ther. 2007 Nov;6(11):1691-9.
3	Thymosin beta4 and its degradation product, Ac-SDKP, are novel reparative factors in renal fibrosis. Kidney Int. 2013 December; 84(6): 10.1038/ki.2013.209.
4	URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 2331).
5	Trusted, scientifically sound profiles of drug programs, clinical trials, safety reports, and company deals, written by scientists. Springer. 2015. Adis Insight (drug id 800021975)
6	Trusted, scientifically sound profiles of drug programs, clinical trials, safety reports, and company deals, written by scientists. Springer. 2015. Adis Insight (drug id 800002485)
7	Trusted, scientifically sound profiles of drug programs, clinical trials, safety reports, and company deals, written by scientists. Springer. 2015. Adis Insight (drug id 800006007)