Aggressive corticosteroid treatment in childhood idiopathic pulmonary hemosiderosis with better outcome

Yang, Cheng-Tsung; Chiang, Bor‐Luen; Wang, Li‐Chieh

doi:10.1016/j.jfma.2020.05.022

Cited by 10 publications

(14 citation statements)

References 29 publications

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“…In the pediatric population, the disease generally occurs before 10 years. This trend was also the case in the most recent pediatric papers 18,23,27,39,40 . There have been some controversies regarding the age at which IPH is initially diagnosed in adults.…”

Section: Clinical Aspects In Iphmentioning

confidence: 68%

“…Anemia (91.6%) and dyspnea (85.5%) are more common presenting symptoms in adults than hemoptysis 3 . Among pediatric patients the prevalence of anemia and dyspnea ranges from 64%−100% (average 92.2%) and 15%−85% (average 45%), respectively 18,23,26,27,39,40,46–51 . Cough is more common among children than adults (65.6% vs. 26.2%).…”

Section: Clinical Aspects In Iphmentioning

confidence: 99%

“…Chest pain is present in a minority of adult patients (10.7%) 3 . Nearly half of adult patients have systemic symptoms, 3 likely comparable to pediatric patients 39,47,49,52 …”

Section: Clinical Aspects In Iphmentioning

confidence: 99%

“…42 The overall survival in adult and pediatric IPH patients was similar (82% vs. 92.6%). 3,18,23,27,39,46,49 The higher survival is likely due to the universal implementation of immunosuppressive therapy.…”

Section: Prognosis In Iphmentioning

confidence: 99%

“…3 Among pediatric patients the prevalence of anemia and dyspnea ranges from 64%−100% (average 92.2%) and 15%−85% (average 45%), respectively. 18,23,26,27,39,40,[46][47][48][49][50][51] Cough is more common among children than adults (65.6% vs. 26.2%). Chest pain is present in a minority of adult patients (10.7%).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

See 4 more Smart Citations

Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

Saha

Aiman

Chong

et al. 2022

Pediatric Pulmonology

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This manuscript reports the recent advances in idiopathic pulmonary hemosiderosis (IPH), a rare cause of diffuse alveolar hemorrhage in children and adults. This narrative review of the literature summarizes different aspects of IPH, including proposed pathogenesis, patient demographics, clinical and radiological characteristics, treatment, and prognosis. Additionally, the association between Celiac Disease (CD) and IPH is carefully evaluated. IPH is a frequently misdiagnosed disease. The delay in the diagnosis of IPH is often significant but fortunately, appears to have decreased in recent years. IPH in adults and children have distinct demographic preferences. The autoantibodies are common in IPH but with a definite difference between the adult and pediatric populations. The definitive diagnosis of IPH requires lung biopsy and careful exclusion of all competing diagnoses, even with lung biopsy showing bland pulmonary hemorrhage. The presence of nonspecific inflammatory cells or lymphoid aggregates may suggest a secondary immunologic phenomenon and needs careful evaluation and follow-up. A substantial number of patients suffer from coexisting CD, also known as Lane−Hamilton syndrome (LHS), and all patients with IPH need to be evaluated for LHS by serology. Although strict gluten free diet can manage the majority of patients with LHS, other patients generally require immunosuppressive therapy. The corticosteroids are the backbone of IPH therapy. Recently utilized experimental treatment options include mesenchymal stem cell transplant, liposteroid and bronchial artery embolization. The immunosuppression should be adjusted to achieve optimal disease control. Patients may progress to endstage lung disease despite all measures, and lung transplantation may be the only viable option.

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Section: Clinical Aspects In Iphmentioning

confidence: 68%

Section: Clinical Aspects In Iphmentioning

confidence: 99%

“…Chest pain is present in a minority of adult patients (10.7%) 3 . Nearly half of adult patients have systemic symptoms, 3 likely comparable to pediatric patients 39,47,49,52 …”

Section: Clinical Aspects In Iphmentioning

confidence: 99%