Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

Siu, K K; Li, Rever; Lam, SK

doi:10.12809/hkmj144237

Cited by 9 publications

(7 citation statements)

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“…22 However, iron deficiency anemia is also a common manifestation of IPH, and this condition may precede other symptoms and signs by several months. 23,24 Bhatia S et al. 25 performed an etiological analysis of 108 patients with microcytic hypochromic anemia, among whom 15 (13.9%) patients were diagnosed with IPH.…”

Section: Discussionmentioning

confidence: 99%

“…22 However, iron deficiency anemia is also a common manifestation of IPH, and this condition may precede other symptoms and signs by several months. 23,24 Bhatia S et al 25 performed an etiological analysis of 108 patients with microcytic hypochromic anemia, among whom 15 (13.9%) patients were diagnosed with IPH. Although IPH and nutritional iron deficiency anemia are characterized by microcytic hypochromic anemia, patients with IPH have normal serum ferritin levels.…”

Section: Discussionmentioning

confidence: 99%

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Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients

et al. 2018

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Objective This study aimed to analyze the clinical characteristics and prognosis of pediatric idiopathic pulmonary hemosiderosis (IPH). Methods Pediatric IPH cases that were diagnosed at West China Second University Hospital, Sichuan University between 1996 and 2017 were reviewed. Follow-up data from 34 patients were collected. Results A total of 107 patients were included (42 boys and 65 girls). The median age was 6 years at diagnosis. The main manifestations of the patients were as follows: anemia (n = 100, 93.45%), cough (n = 68, 63.55%), hemoptysis (n = 61, 57%), fever (n = 23, 21.5%), and dyspnea (n = 23, 21.5%). There were relatively few pulmonary signs. The positive rates of hemosiderin-laden macrophages in sputum, gastric lavage fluid, and bronchoalveolar lavage fluid were 91.66%, 98.21%, and 100%, respectively. Seventy-nine patients were misdiagnosed. A total of 105 patients were initially treated with glucocorticoids, among whom 102 survived and three died. Among the followed up patients, two died and 32 survived, among whom 10 presented with recurrent episodes. Conclusions The classic triad of pediatric IPH is not always present. The rates of misdiagnosis and recurrence of IPH are high. Early recognition and adequate immunosuppressive therapy are imperative for improving prognosis of IPH.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients

et al. 2018

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“…Se presenta principalmente en la primera década de la vida y los síntomas más frecuentes son anemia, tos, fiebre y disnea, con o sin hemoptisis (6,7,8,9) , por lo que muchas veces se hace el diagnóstico inicial erróneo de neumonía bacteriana y anemia por otras causas, especialmente en los casos sin hemoptisis (10,11) y se describe un retraso diagnóstico de 30 meses en promedio (9,12) . Por ello, es importante sospechar HPI en pacientes pediátricos con infecciones respiratorias recurrentes asociadas a anemia e incluso, en pacientes con anemia refractaria en los que se haya descartado otras causas (13,14) . Figura 1.…”

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Hemosiderosis Pulmonar Idiopática (Hpi): Presentación De Un Caso Y Revisión De La Literatura

2020

Neumol Pediatr

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Se presenta el caso de una lactante de un mes de edad, que se presentó en el Servicio de Urgencia con shock hipovolémico secundario a hemorragia pulmonar. Necesitó medidas de soporte vital, incluyendo drogas vasoactivas y pulsos de metilprednisolona. Egresó del hospital a los 13 días de evolución y reingresó 5 días después por nuevo episodio de hemoptisis con compromiso hemodinámico. La fibrobroncoscopía efectuada a los 4 días de evolución del primer episodio mostró una anatomía normal, sin sangrado activo, con 20% de hemosiderófagos en el lavado broncoalveolar. En la radiografía de tórax se encontró infiltrados difusos. Los estudios en busca de infección, cardiopatía, enfermedad inmunológica, trombofilia, enfermedad celíaca, trastorno de deglución, anomalías vasculares y alergia a la proteína de la leche de vaca resultaron negativos, por lo que se planteó una Hemosiderosis Pulmonar Idiopática (HPI). Se manejó con fórmula aminoacídica, prednisona oral diaria hasta los 6 meses de edad y después en días alternos y fluticasona inhalada permanente. En controles posteriores se constató crecimiento y desarrollo normal, sin recidivas hasta el momento de este reporte, con 1 año de edad. La evolución favorable en este caso se atribuye al diagnóstico precoz y tratamiento oportuno con corticoides sistémicos. Se presenta una revisión del tema de HPI en pediatría y se proponen algoritmos de estudio y tratamiento.

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“…Haemoptysis is rare in children because in most cases they are not able to expectorate [ 12 , 16 ]. Iron-deficiency anaemia could precede other signs and symptoms by several months in children with IPH and could be severe enough to necessitate a blood transfusion despite adequate iron supplementation [ 1 , 15 , 17 , 18 ]. Other frequent signs in IPH are recurrent lower respiratory tract infections and recurrent tachypnoea [ 14 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

Idiopathic pulmonary haemosiderosis in paediatric patients: how to make an early diagnosis

et al. 2016

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BackgroundIdiopathic pulmonary haemosiderosis (IPH) is a rare but potentially lethal condition in paediatric patients. This condition is considered an immune-mediated disorder, but its pathogenesis is still unknown. Idiopathic pulmonary haemosiderosis is characterized by the classical triad of haemoptysis, iron-deficiency anaemia, and diffuse parenchymal consolidation on chest radiology. Unfortunately, this triad of signs is not frequent in children at the onset of this disease, resulting in a delay in diagnosis and a negative outcome.Case presentationThis case report describes a 4-year-old girl who was admitted for an acute episode of lower respiratory tract infection associated with severe dyspnoea, polypnoea, and severe anaemia (haemoglobin levels, 5.9 g/dL). She had a history of previous similar episodes, with anaemia treated unsuccessfully with iron supplementation and managed through repeated blood transfusions in the acute phase. She did not experience haemoptysis. A computed tomography (CT) scan of the thorax showed ground-glass opacity suggestive of pulmonary haemorrhage. After other causes of intra-alveolar haemorrhage were excluded, IPH was confirmed by the presence of siderophages in bronchoalveolar lavage. Immunosuppressive corticosteroid treatment was immediately started with a good clinical response.ConclusionThis case highlights the fact that IPH should be suspected in children with recurrent lower respiratory tract infections who have a history of iron-deficiency anaemia who shows no signs of improvement with iron supplementation and may require repeated blood transfusions. The absence of haemoptysis does not exclude the diagnosis of IPH in children. An early and prompt diagnosis is recommended in order to start adequate immunosuppressive treatment.

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Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

Cited by 9 publications

References 12 publications

Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients

Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients

Hemosiderosis Pulmonar Idiopática (Hpi): Presentación De Un Caso Y Revisión De La Literatura

Idiopathic pulmonary haemosiderosis in paediatric patients: how to make an early diagnosis

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