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Idiopathic pulmonary hemosiderosis

MedGen UID:
9403
Concept ID:
C0020807
Disease or Syndrome
Synonyms: Alveolar hypoventilation syndrome; Pulmonary hemosiderosis
SNOMED CT: IPH - Idiopathic pulmonary hemosiderosis (40527005); Idiopathic pulmonary hemosiderosis (40527005); Brown induration of lung (40527005); Pulmonary hemosiderosis (40527005)
 
Monarch Initiative: MONDO:0008346
OMIM®: 178550
Orphanet: ORPHA99931

Definition

Idiopathic pulmonary hemosiderosis is a respiratory disease due to repeated episodes of diffuse alveolar hemorrhage without any underlying apparent cause, most often in children. Anemia, cough, and pulmonary infiltrates on chest radiographs are found in majority of the patients. [from ORDO]

Clinical features

From HPO
Recurrent intrapulmonary hemorrhage
MedGen UID:
812249
Concept ID:
C3805919
Finding
A recurrent hemorrhage occurring within the lung.
Iron deficiency anemia
MedGen UID:
57668
Concept ID:
C0162316
Disease or Syndrome
Anemia caused by low iron intake, inefficient iron absorption in the gastrointestinal tract, or chronic blood loss.
Hemoptysis
MedGen UID:
5502
Concept ID:
C0019079
Sign or Symptom
Coughing up (expectoration) of blood or blood-streaked sputum from the larynx, trachea, bronchi, or lungs.
Pulmonary fibrosis
MedGen UID:
11028
Concept ID:
C0034069
Disease or Syndrome
Replacement of normal lung tissues by fibroblasts and collagen.
Respiratory insufficiency
MedGen UID:
11197
Concept ID:
C0035229
Pathologic Function
Impairment of gas exchange within the lungs secondary to a disease process, neoplasm, or trauma, possibly resulting in hypoxia, hypercarbia, or both, but not requiring intubation or mechanical ventilation. Patients are normally managed with pharmaceutical therapy, supplemental oxygen, or both.
Transient pulmonary infiltrates
MedGen UID:
812250
Concept ID:
C3805920
Finding
Abnormality of the respiratory system
MedGen UID:
866322
Concept ID:
C4018871
Anatomical Abnormality
An abnormality of the respiratory system, which include the airways, lungs, and the respiratory muscles.
Decreased circulating IgA level
MedGen UID:
57934
Concept ID:
C0162538
Disease or Syndrome
Decreased levels of immunoglobulin A (IgA).

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Idiopathic pulmonary hemosiderosis in Orphanet.

Professional guidelines

PubMed

Karavelikova IP
Folia Med (Plovdiv) 2021 Aug 31;63(4):582-585. doi: 10.3897/folmed.63.e56088. PMID: 35851160
Yang CT, Chiang BL, Wang LC
J Formos Med Assoc 2021 Feb;120(2):838-846. Epub 2020 Jun 3 doi: 10.1016/j.jfma.2020.05.022. PMID: 32505591
Chin CI, Kohn SL, Keens TG, Margetis MF, Kato RM
Orphanet J Rare Dis 2015 Aug 20;10:98. doi: 10.1186/s13023-015-0319-5. PMID: 26289251Free PMC Article

Recent clinical studies

Etiology

Saha BK, Milman NT
Prague Med Rep 2022;123(2):65-81. doi: 10.14712/23362936.2022.7. PMID: 35507939
Saha BK, Bonnier A, Saha S, Saha BN, Shkolnik B
Clin Rheumatol 2022 Jun;41(6):1627-1640. Epub 2022 Feb 18 doi: 10.1007/s10067-022-06104-3. PMID: 35179664
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
Zhang Y, Luo F, Wang N, Song Y, Tao Y
J Int Med Res 2019 Jan;47(1):293-302. Epub 2018 Oct 2 doi: 10.1177/0300060518800652. PMID: 30278795Free PMC Article
de Silva C, Mukherjee A, Jat KR, Lodha R, Kabra SK
Indian J Pediatr 2019 Jan;86(1):7-11. Epub 2018 Jun 14 doi: 10.1007/s12098-018-2725-x. PMID: 29948735

Diagnosis

Uda K, Yashiro M
N Engl J Med 2023 Jun 8;388(23):2179. Epub 2023 Jun 3 doi: 10.1056/NEJMicm2214160. PMID: 37272525
Saha BK, Aiman A, Chong WH, Saha S, Song J, Bonnier A
Pediatr Pulmonol 2023 Feb;58(2):382-391. Epub 2022 Nov 11 doi: 10.1002/ppul.26230. PMID: 36324124
Tryfon S, Papadopoulou E, Psarros G, Agrafiotis M, Saroglou M
Postgrad Med 2022 Nov;134(8):732-742. Epub 2022 Aug 8 doi: 10.1080/00325481.2022.2109121. PMID: 35912848
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
Zhang Y, Luo F, Wang N, Song Y, Tao Y
J Int Med Res 2019 Jan;47(1):293-302. Epub 2018 Oct 2 doi: 10.1177/0300060518800652. PMID: 30278795Free PMC Article

Therapy

Saha BK, Aiman A, Chong WH, Saha S, Song J, Bonnier A
Pediatr Pulmonol 2023 Feb;58(2):382-391. Epub 2022 Nov 11 doi: 10.1002/ppul.26230. PMID: 36324124
Tryfon S, Papadopoulou E, Psarros G, Agrafiotis M, Saroglou M
Postgrad Med 2022 Nov;134(8):732-742. Epub 2022 Aug 8 doi: 10.1080/00325481.2022.2109121. PMID: 35912848
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
Saha BK, Milman NT
Clin Rheumatol 2021 Jul;40(7):2547-2557. Epub 2020 Nov 12 doi: 10.1007/s10067-020-05507-4. PMID: 33184706
Zhang Y, Luo F, Wang N, Song Y, Tao Y
J Int Med Res 2019 Jan;47(1):293-302. Epub 2018 Oct 2 doi: 10.1177/0300060518800652. PMID: 30278795Free PMC Article

Prognosis

Saha BK, Aiman A, Chong WH, Saha S, Song J, Bonnier A
Pediatr Pulmonol 2023 Feb;58(2):382-391. Epub 2022 Nov 11 doi: 10.1002/ppul.26230. PMID: 36324124
Saha BK, Bonnier A, Saha S, Saha BN, Shkolnik B
Clin Rheumatol 2022 Jun;41(6):1627-1640. Epub 2022 Feb 18 doi: 10.1007/s10067-022-06104-3. PMID: 35179664
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295
Zhang Y, Luo F, Wang N, Song Y, Tao Y
J Int Med Res 2019 Jan;47(1):293-302. Epub 2018 Oct 2 doi: 10.1177/0300060518800652. PMID: 30278795Free PMC Article
Godfrey S
Pediatr Pulmonol 2004 Jun;37(6):476-84. doi: 10.1002/ppul.20020. PMID: 15114547

Clinical prediction guides

Tryfon S, Papadopoulou E, Psarros G, Agrafiotis M, Saroglou M
Postgrad Med 2022 Nov;134(8):732-742. Epub 2022 Aug 8 doi: 10.1080/00325481.2022.2109121. PMID: 35912848
Saha BK, Chong WH, Saha S, Aiman A, Bonnier A
Lung 2022 Apr;200(2):205-215. Epub 2022 Mar 10 doi: 10.1007/s00408-022-00523-4. PMID: 35267072
Saha BK, Bonnier A, Chenna P, Milman NT
Clin Rheumatol 2022 Apr;41(4):977-990. Epub 2022 Jan 24 doi: 10.1007/s10067-021-06029-3. PMID: 35067768
Karavelikova IP
Folia Med (Plovdiv) 2021 Aug 31;63(4):582-585. doi: 10.3897/folmed.63.e56088. PMID: 35851160
Saha BK
Respir Med 2021 Jan;176:106234. Epub 2020 Nov 17 doi: 10.1016/j.rmed.2020.106234. PMID: 33246295

Recent systematic reviews

Chen XY, Sun JM, Huang XJ
Clin Respir J 2017 Nov;11(6):677-681. Epub 2016 Mar 14 doi: 10.1111/crj.12440. PMID: 26692115

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