Idiopathic Pulmonary Hemosiderosis in Pediatric in Patients: Report of Four Cases in Rabat, Morrocco
Benchidmi S. *
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
Lamghari Y.
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
Saad A.
Pathological Anatomy and Cytology Laboratory, UHC, Ibn Sina Hospital, Rabat, Morocco.
Maalmi N.
Pathological Anatomy and Cytology Laboratory, UHC, Ibn Sina Hospital, Rabat, Morocco.
Jaabouti G.
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
Aminou S.
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
Elqoraichi O.
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
EL Hafidi N.
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
Mahraoui C.
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
Benchekroun S.
Division of Pediatric Immuno Allergology and Infectious Diseases, Ibn Sina University Hospital, Rabat, Morocco.
*Author to whom correspondence should be addressed.
Abstract
Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage. Patients with IPH usually present with the classical triad: hemoptysis, and iron deficiency anemia, and pulmonary infiltrates on chest imaging, the diagnosis is often delayed by years.
We report four IPH pediatrics cases of children diagnosed at the children’s hospital of Rabat, Morocco. The aim of this study is to review the clinical manifestations, diagnostic tools and treatment of this affection.
All the four patients were presented with the classical triad, the diagnosis was based on the Clinical, radiologic and biologic findings. Bronchoalveolar lavage was performed to all the patients and confirmed the diagnosis by the identification of siderophages.
High doses of Corticosteroids, by an induction therapy with an intravenous methylprednisolone (20 mg/kg/day for 3 days) followed by oral prednisone (1 mg/kg/day) had shown a good therapeutic response. One patient was treated first with oral corticosteroid and switched over to synthetic antimalarial drugs, because he had side effects from long-term cortico therapy. Then the clinical condition has been improved. The evaluation of response to the therapy included the clinical symptoms and signs, laboratory data and chest x ray.
The prognosis of IPH is influenced by several factors, including the time of diagnosis, early initiation of treatment, and the presence of comorbidities.
Keywords: Golde score, hemoptysis, hemosiderosis, idiopathic pulmonary hemosiderosis, pulmonary infiltrates, siderophage
How to Cite
Downloads
References
Poggi V, Lo Vecchio A, Menna F, Menna G. Idiopathic pulmonary hemosiderosis: a rare cause of iron-deficiency anemia in childhood. J Pediatr Hematol Oncol. 2011;33:e160–2.
Morgan PG and Turner-Warwick M. Pulmonary haemosiderosis and pulmonary haemorrhage. Br J Dis Chest. 1981; 75: 225–242.
Ohga S, Takahashi K, Miyazaki S, et al. Idiopathic pulmonary haemosiderosis in Japan: 39 possible cases from a survey questionnaire. Eur J Pediatr. 1995;154: 994–995.
Saeed MM, Woo MS, MacLaughlin EF, et al. Prognosis in pediatric idiopathic pulmonary hemosiderosis. Chest. 1999; 116:721–725.
Kjellman B, Elinder G, Garwicz S, Svan H. Idiopathic pulmonary hemosiderosis in Swedish Children. Acta Pediatr Scand. 1984;73:584-588.
Corrin B, Jagusch M, Dewar A, et al. Fine structural changes in idiopathic pulmonary haemosiderosis. J Pathol. 1987;153:249–256.
Saha, Biplab K. diopathic pulmonary hemosiderosis: A state of the art review. Available:https://doi.org/10.1016/j.rmed.2020.106234
Milman, N. Idiopathic pulmonary hemosiderosis. Post TW, ed. UpToDate. Waltham, MA: UpToDate Inc. Available:http://www.uptodate. com Accessed Oct 21, 2020.
Le Clainche L, Le Bourgeois M, Fauroux B, Forenza N, Dommergues JP, Desbois JC, et al. Long-term outcome of idiopathic pulmonary hemosiderosis in children. Medicine (Baltimore). 2000;79(5):318–326.
Maldonado F, Parambil JG, Yi ES, Decker PA, Ryu JH. Haemosiderin-laden macrophages in the bronchoalveolar lavage fluid of patients with diffuse alveolar damage. European Respiratory Journal. 2009;33(6):1361-1366.
Yao TC, Hung IJ, Wong KS, Huang JL, Niu CK. Idiopathic pulmonary haemosiderosis: An Oriental experience. J Paediatr Child Health, 2003;39(1): 27e30.
Susarla SC, Fan LL. Diffuse alveolar hemorrhage syndromes in children. Curr Opin Pediatr. 2007;19(3):314e20.
Kabra SK, Bhargava S, Lodha R, Satyavani A, Walia M. Idiopathic pulmonary hemosiderosis: clinical profile and follow up of 26 children. Indian Pediatr, 2007;44(5):333e8..
Kiper N, Goçmen A, Ozçelik U, Dilber E, Anadol D. Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979–1994): prolonged survival with low-dose corticosteroid therapy, Pediatr. Pulmonol. 1999;27(3): 180–184.
Chin CI, Kohn SL, Keens TG, et al. A physician survey reveals differences in management of idiopathic pulmonary hemosiderosis. Orphanet J Rare Dis; 2015. DOI: 10.1186/s13023-015-0319-5
Cheng-Tsung Yang, Bor-Luen Chiang, Li-Chieh Wang. Aggressive corticosteroid treatment in childhood idiopathic pulmonary hemosiderosis with better outcome, Journal of the Formosan Medical Association. 2021;120(2). Available:https://doi.org/10.1016/j.jfma.2020.05.022
Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004;24(1):162e70.
Afzal N, Mushtaq A, Rahman A, et al. Idiopathic pulmonary haemosiderosis presenting as severe iron deficiency anaemia–a case from Pakistan. J Pak Med Assoc. 2012;62:845–847.