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.
Although Crimean‐Congo hemorrhagic fever (CCHF) is mild and self‐limited in children, some patients may develop excessive bleeding, massive liver necrosis, and multiple organ failure associated with secondary hemophagocytic lymphohistiocytosis (HLH) induced by cytokine storm. Treatment of CCHF is mainly symptomatic and supportive. The efficacy of ribavirin, which is the only antiviral drug in the treatment of CCHF, remains controversial. Although therapeutic plasma exchange (TPE) has been shown to beneficial in small case series with primary and secondary HLH, there is no pediatric patient with HLH secondary to CCHF treated with TPE in the literature. In this report, we describe the first pediatric patient who was successfully recovered from HLH secondary to CCHF with ribavirin, intravenous immunoglobulin, and TPE.
Background. Abnormal movements such as tremors and myoclonus may be observed during both treatment and deficiency of vitamin B12, particularly in infants. Infantile tremor syndrome (ITS) is defined by the tetrad of pallor, developmental delay/regression, skin pigmentation, and brown scanty scalp hair.Case. In this report, two cases with ITS aged less than one year who had myoclonic movements during vitamin peroral B12 treatment are discussed based on hematologic, neurological, and magnetic resonance images (MRI) findings, one of whom developed a whole-body tremor and rhythmic myoclonic movements, titubation, and restlessness in the hands and feet as well as diffuse cerebral atrophy on brain MRI.
Conclusion.The infants of mothers with nutritional vitamin B12 deficiency may develop sudden abnormal movements following peroral vitamin B12 therapy and that the differential diagnosis of these disorders is highly important for the prevention of long-term neurological sequela by treatment.
Background: Esophageal stricture (ES) is an uncommon clinic entity in pediatrics that may be congenital or acquired in childhood. Acquired noncaustic ES is very rare, and clinical features of affected patients are unknown.Purpose: We aimed to evaluate the clinical findings, and outcomes of patients with acquired noncaustic ES to aid physicians in the early referral of patients to gastroenterologists.Methods: The medical data of patients with acquired noncaustic ES who were followed in our gastroenterology clinic between January 2009 and December 2019 were reviewed.Results: Acquired noncaustic ES was found in 12 of the 4,950 patients (0.24%) who underwent endoscopy during the study period. The main symptoms were dysphagia (58.3%), vomiting (33.3%), and chronic anemia (8.3%). Chronic malnutrition and underweight were found in 66.6% of the patients. The most common etiological factors were radiotherapy, peptic reflux, and achalasia (16.6%, each), while chemotherapy, squamous-cell carcinoma (SC) of the esophagus, eosinophilic esophagitis (EoE), esophageal web, epidermolysis bullosa, and esophageal diverticulum (8.2%, each) were the other etiological factors. Patients with EoE underwent endoscopic bougie dilation in addition to steroid use and elimination diet. Patients with epidermolysis bullosa and esophageal web underwent bougie dilation. Patients with peptic reflux-related ES were initially put on antireflux therapy, but during follow-up, one patient required esophageal replacement with colonic interposition. Patients with radiotherapy-related ES recovered with medical therapy. The patient with initially underwent surgical gastrostomy and tumoral mass excision. The patient then received chemotherapy and radiotherapy and underwent jejunal interposition. Patients with achalasia underwent surgical esophagomyotomy.Conclusion: The presence of solid dysphagia, malnutrition, and an associated disease may alert physicians to the presence of ES.
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