Idiopathic pulmonary hemosiderosis is characterized by hemoptysis resulting from recurring alveolar hemorrhage attacks, iron deficiency anemia, and parenchymal infiltrations as seen on chest radiographs. The clinical course may consist of silent and asymptomatic attacks, or it may sometimes exhibit a fulminant course with rapidly developing anemia and hypoxemia. Celiac disease is an autoimmune enteropathy triggered by the consumption of gluten-containing foods in genetically predisposed individuals. Co-existence of idiopathic pulmonary hemosiderosis and celiac disease is defined as Lane-Hamilton syndrome. We describe a case of Lane-Hamilton syndrome with growth and developmental delay; complete remission of pulmonary symptoms was achieved with a gluten-free diet.

Background/Aim:We aimed to analyze serum hepcidin level in children with chronic liver disease (CLD) and its relationship with serum cytokines level, liver function tests, hepatic iron content, and liver fibrosis.Patients and Methods:The study included 34 children with CLD, and 15 age- and gender-matched healthy children. Serum hepcidin, ferritin, iron level, interleukin-6 (IL-6), transforming growth factor-β (TGF-β ), total oxidant status (TOS), and antioxidant status (TAS) were studied in all patients and in the control group. Liver iron content (LIC) was measured from the liver biopsy specimen.Results:Serum ferritin levels were higher in patients with CLD than control group (100.1 ± 98.2 ng/mL vs 50.5 ± 32.2 ng/mL, P = 0.016). No significant difference was found in hepcidin levels. Hepcidin levels in children with CLD was positively correlated with ferritin (r = 0.75, P = 0.001), pediatric end-stage liver disease (PELD) score (r = 0.56, P = 0.001), TAS (r = 0.42, P = 0.02), but negatively correlated with albumin level (r = −0.45, P = 0.008). Transferrin saturation and hepcidin:ferritin ratio were significantly low in patients with severe fibrosis compared with patients with mild/without fibrosis (15.5 ± 5.5 vs 34.3 ± 30.1, P = 0.017 and 1 ± 0.5 vs 1.9 ± 1.4, P = 0.04, respectively).Conclusion:Serum hepcidin levels in children with CLD reflect both liver functions and TAS, and severe fibrosis is associated with low hepcidin:ferritin ratio in children with CLD.

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