Clinical features and risk factors for recurrence of idiopathic pulmonary hemosiderosis in children.

Wang L ，Li Y ，Zhang R ，Liu H ，Chen L ... -  《BMC Pulmonary Medicine》

Aggressive corticosteroid treatment in childhood idiopathic pulmonary hemosiderosis with better outcome.

Idiopathic pulmonary hemosiderosis (IPH) is a rare but fatal disease characterized by a triad of anemia, hemoptysis, and increased pulmonary infiltration. This study is aimed to review the clinical manifestations, diagnostic tools, medication and outcome of childhood IPH in Taiwan. We retrospectively enrolled the patients less than 18 years old in National Taiwan University Hospital in the past 30 years. The clinical data were collected and analyzed. All of the twelve children diagnosed with IPH had anemia and increased pulmonary infiltration, eight had hemoptysis, and ten were confirmed with detection of hemosiderin-laden macrophages. The mean age at diagnosis were 4.9 (interquartile range 2.5-6.3) years old. Patients with high dose corticosteroid (CS, ≥ 1 mg/kg/day prednisolone equivalent) treatment had lower odds ratio for ICU admission and significant higher Hb recovery rate than those with mild disease activity not receiving high dose CS treatment (p = 0.011). The only factor that is significantly associated with persistent anemia is the usage of high dose CS (p < 0.001) after adjusting for hemoptysis, fulfilling triad, serum ferritin level, and ICU admission by multiple regression. The only factor that is significantly associated with ICU admission is the presence of microorganism yielded in sputum (p < 0.001) after adjusting for fever, serum ferritin level, usage of invasive MV, and high dose CS treatment days. The aggressive high dose CS therapy might prevent ICU admission and improve anemia. Aggressive high dose CS treatment is suggested in IPH patients regardless of the disease activity.

Yang CT ，Chiang BL ，Wang LC 《JOURNAL OF THE FORMOSAN MEDICAL ASSOCIATION》

Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients.

Zhang Y ，Luo F ，Wang N ，Song Y ，Tao Y ... -  《-》

Anemia as the sole presenting symptom of idiopathic pulmonary hemosiderosis: report of two cases.

Chen KC ，Hsiao CC ，Huang SC ，Ko SF ，Niu CK ... -  《-》

Idiopathic pulmonary hemosiderosis in adults: review of cases reported in the latest 15 years.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology which can cause diffuse alveolar hemorrhage. IPH is found primarily in children. In adults, however, it is extremely rare. A systematic review was applied to identify the details of IPH in adults. Articles of English or Chinese language published between 2000 and 2015 were included. Data were extracted on the clinical features, examinations, treatments and clinical outcome. A total of 37 cases of adult-onset IPH were included (13 females and 24 males). IPH combined with coeliac disease was found in five patients, three of whom received gluten-free diet (GFD) only and got full remission. Upon diagnosis, median age was 34 years. The main manifestations were: hemoptysis (n = 30, 81%), dyspnea (n = 23, 64%), anemia (n = 20, 54%). Most patients were treated by corticosteroids initially. The mortality rage was 14% on acute phase. The adult patients in this study showed some differences from the previously characterized IPH. It is characterized by immunologically mediated, middle-age onset, male dominance, absence of anemia, high mortality on acute phase.

Chen XY ，Sun JM ，Huang XJ 《-》