Inflammatory bowel disease in Turkish children.

BACKGROUND: This study was undertaken to evaluate demographics, clinical manifestations, laboratory findings and outcomes of children with inflammatory bowel disease (IBD) in Turkey. METHODS: We analyzed the medical records of 127 children diagnosed with IBD (under 18 years old) between January 2004 and January 2012 in 8 pediatric gastroenterology centers. RESULTS: Of the 127 patients, 90 (70.9%) suffered from ulcerative colitis (UC), 29 (22.8%) from Crohn's disease (CD), and 8 (6.3%) from IBD unclassified. The mean age of the 127 patients was 11.6 ± 4.1 years, and 11.8% of the patients were below 5 years old. Of the patients, 49.6% were male, and males were more predominant in patients with CD than in those with UC (72.4% vs. 42.2%, P = 0.008; a male/female ratio of 2.62 in CD, P = 0.0016). Approximately one fifth of the patients had extra-intestinal manifestations and 13.3% of the patients had associated diseases. Extraintestinal manifestations and associated diseases were more common in early onset disease [P = 0.017, odds ratio (OR) = 4.02; P = 0.03, OR = 4.1]. Of the patients, 15% had normal laboratory parameters including anemia, high platelet count, hypoalbuminemia, hypoferritinemia, and high sedimentation rate. Area under receiver operation characteristics was used to predict pancolitis in patients with UC. The values of C-reactive protein, sedimentation rate and pediatric ulcerative colitis activity were 0.61 (P = 0.06), 0.66 (P = 0.01) and 0.76 (P = 0.0001), respectively. Four (4.4%) patients with UC underwent colectomy, and finally two (1.5%, 95% confidence interval: 0-3.7%) patients died from primary disease or complications. CONCLUSIONS: IBD is an increasing clinical entity in Turkey. Features of IBD are similar to those in other populations, but prospective multicenter studies are needed to analyze the true incidence of IBD in Turkish children.

Gluconeogenesis defect presenting with resistant hyperglycemia and acidosis mimicking diabetic ketoacidosis.

Fructose-1,6-diphosphatase (FDPase) enzyme deficiency is a rare inherited metabolic disease. Affected patients usually present with metabolic crisis including hypoglycemia, acidosis, ketonuria, and hyperuricemia. A previously healthy 8-month-old male infant presented with fever, vomiting, and hypoactivity. He had tachycardia, tachypnea, and a tendency to sleep. The patient had signs of severe dehydration and shock. Laboratory findings revealed significant lactic acidosis, hyperuricemia, hyperglycemia, elevated liver enzyme level, and hyperlipidemia. The urine analysis had evidence of glycosuria and ketonuria. Hyperuricemia, lactic acidemia, and hyperglycemia persisted despite insulin infusion, adequate hydration, and perfusion. Consequently, peritoneal dialysis was started. About 12 hours after dialysis, his metabolic derangements were normalized, and clinical status was improved dramatically. His metabolic disease workup was compatible with FDPase deficiency. Here, we described a metabolic attack of FDPase deficiency presented with hyperglycemia mimicking diabetic ketoacidosis.

Severe myopathy caused by the new pandemic influenza A (H1N1) in a child.

Myopathy is a rare complication of influenza infections. Here, we report on an eight-year-old girl with severe myopathy due to new pandemic influenza A (H1N1). She presented with severe myopathy following generalized tonic-clonic seizure and recovered completely within a few days.

Acute pancreatitis as a presenting feature of Henoch-Schönlein purpura.

Henoch-Schönlein purpura (HSP) is the most common small vessel vasculitis of childhood. It is characterized by nonthrombocytopenic palpable purpura, abdominal pain, arthritis, and glomerulonephritis. Although HSP is typically known to be self-limited, serious complications can occur. Acute pancreatitis rarely presents as a complication of HSP. It is even rarer as an initial presenting feature of HSP, before the occurrence of characteristic palpable purpura. Herein, we report a 12-year-old girl with HSP who atypically presented with acute pancreatitis.

Plummer-Vinson syndrome in a 15-year-old boy.

Plummer-Vinson syndrome presents as a classical triad of dysphagia, iron deficiency anemia and upper esophageal web(s). The syndrome usually occurs in adults, and is rare in childhood. We report a case of this syndrome occurring in a 15-year-old boy. He presented with dysphagia and anemia. Radiological examination showed the presence of webs at the cervical esophagus. The boy was treated with endoscopic balloon dilation and iron supplementation and remains in good general condition six months after the treatment.

Celiac disease in 87 children with typical and atypical symptoms in Black Sea region of Turkey.

BACKGROUND: Celiac disease presents with a spectrum of clinical disorders. The variety of clinical presentations largely depends on age and extraintestinal findings. This study aimed to determine typical and atypical cases according to presenting symptoms and to evaluate their biochemical and pathological parameters. METHODS: Eighty-seven patients with celiac disease in our unit between 2000 and 2007 were reviewed. Their diagnosis was made by serological and histological examination. The patients were divided into two groups according to their typical or atypical symptoms. RESULTS: The mean age of the patients at diagnosis was 8.2 years (range, 1-18 years), but patients presenting with typical symptoms were younger than those presenting with atypical symptoms. The patients in the two groups did not differ significantly in sex, weight and height Z scores except age. Diarrhea (96.3%), abdominal distention (65.4%) and failure to thrive (60%) were the most common clinical presentations in the typical group, and short stature (62.5%) and anemia (31.2%) were the most common in the atypical group. Total/subtotal villous atrophy was significantly higher in the typical group than in the atypical group. CONCLUSIONS: Many children with celiac disease show an atypical form. The understanding of presentations of celiac disease may prevent delayed diagnosis. Celiac disease should be specially investigated in patients with recurrent iron deficiency anemia, short stature and autoimmune disorders.

Tuberculous peritonitis in children: report of nine patients and review of the literature.

AIM: To present our experience with tuberculous peritonitis treated in our hospital from 2002-2007. METHODS: We reviewed the medical records of 9 children with tuberculous peritonitis. RESULTS: Nine patients (5 boys, 4 girls) of mean age 14.2 years were diagnosed with peritoneal tuberculosis. All patients presented with abdominal distention. Abdominal pain was seen in 55.5% and fever in 44.4% of the patients. Four cases had coexisting pleural effusion and two had pulmonary tuberculosis with parenchymal consolidation. Ultrasonography found ascites with septation in 7 patients. Two patients had only ascites without septation. Ascitic fluid analysis of 8 patients yielded serum-ascite albumin gradients of less than 1.1 gr/dL. Laparoscopy and laparotomy showed that whitish tuberculi were the most common appearance. Adhesions were also seen in three cases. The diagnosis of peritoneal tuberculosis was confirmed histo-pathologically in 7 patients and microbiologically in two. Two patients had been diagnosed by ascitic fluid diagnostic features and a positive response to antituberculous treatment. All patients completed the antituberculous therapy without any complications. CONCLUSION: Tuberculous peritonitis has to be clinically suspected in all patients with slowly progressive abdominal distension, particularly when it is accompanied by fever and pain. Laparoscopy and peritoneal biopsy are still the most reliable, quick and safe methods for the diagnosis of tuberculous peritonitis.