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N Koçak

Publications and source records attributed to N Koçak.

At least 37 records · Page 2Linked to original sources
PubMed2000

Glycogen storage disease type Ia: frequency and clinical course in Turkish children.

The aim of this study was to determine the relative frequency of type Ia in glycogen storage disease (GSD) with prominent liver involvement and to determine its clinical and laboratory findings and prognosis in Turkish children. From 1980 to 1998, 45 out of 100 GSD patients (27 male) with liver involvement had been diagnosed for type Ia. The files were retrospectively evaluated and clinical and laboratory features were documented. In addition to routine laboratory evaluations, urine albumin, calcium excretions, and plasma biotinidase activity were measured. Breast-feeding was continued in all infants. After 6 months of age, uncooked cornstarch was administered to the patients. The relative frequency of type Ia in GSD with liver involvement was 45%. The diagnosis was made in 71% of patients before 2 years of age (median 1 year). Main complaint was abdominal protruding (57.8%), and main physical finding was hepatomegaly (100%). Forty percent of the patients had growth retardation at diagnosis. Among laboratory parameters, hypertriglyceridemia (97.8%) and hypertransaminasemia (95.6%) were the most frequent findings following plasma biotinidase activity, which was elevated in all patients. Microalbuminuria was determined in 52.8% of the patients and hypercalciuria in 23.8%. Histopathological findings of the liver included fibrosis (75.6%), steatosis (37.8%), mosaicism (24.4%) and nuclear hyperglycogenation (15.6%). During follow-up period, the ratio of patients with growth retardation did not change. Transaminases were decreased in 48.7% of the patients. Although triglyceride and cholesterol levels decreased in the majority of the patients, they did not normalise. The prevalence of type Ia in GSD with prominent liver involvement was found higher than the other reports. Microalbuminuria was also higher than the previous reports.

Adolescent↗
PubMed2000

Short-term effect of captopril on microalbuminuria in children with glycogen storage disease type Ia.

Early signs of renal dysfunction in glycogen storage disease type Ia (GSD Ia) are glomerular hyperfiltration and proteinuria. In a non-randomized study, the effect of captopril on the improvement of proteinuria in GSD Ia patients with microalbuminuria was investigated. A positive effect has been shown for the insulin-dependent diabetes mellitus patients. Microalbuminuria was defined as albumin/creatinine ratio (mg/mmol) more than 2.5 in spot urine. Nineteen (52.7%) out of 36 patients had microalbuminuria, and 8 patients received captopril at a dose of 1 mg/kg per day. Microalbuminuria was evaluated periodically during the follow-up period. Of the captopril-treated patients, one was lost to follow-up. In the remaining 7 patients, urinary albumin excretion normalized in 3 patients (42.9%) and decreased at least by 50% in another 3 patients (42.8%) after 6 months of treatment. One patient, who was the oldest, did not have any benefit. In untreated patients, only two patients had a decrease in microalbuminuria of more than 50%. Patients with microalbuminuria had significantly higher blood lactate (p < 0.05) and plasma triglyceride (p < 0.01) concentrations and significantly lower blood bicarbonate concentration (p < 0.05) than those patients without it. Additionally, the patients with microalbuminuria had been diagnosed earlier than those without microalbuminuria (p < 0.05). Patients with microalbuminuria have more severe clinical and laboratory findings than those without microalbuminuria. Captopril at a dose of 1 mg/kg per day seems to be effective in at least 50% of GSD Ia patients with microalbuminuria.

Adolescent↗
PubMed2000

Celiac disease in Turkish children: presentation of 104 cases.

BACKGROUND: Celiac disease is characterized by life-long gluten intolerance. Clinical features of patients with celiac disease are variable. In the present study, clinical, laboratory and histologic features of 104 patients with celiac disease were evaluated. METHODS: Intestinal biopsy and serum antigliadin and anti-endomysium antibodies were used for the diagnosis of patients. Mucosal lesions were classified according to the criteria of Marsh. Antigliadin antibodies were measured with a commercial enzyme-linked immunosorbent assay. Anti-endomysium antibodies were analyzed by indirect immunofluorescence with the use of a section of monkey esophagus. Routine hematological and biochemical analyses and measurement of immunoglobulin levels were undertaken. RESULTS: The mean (+/- SD) patient age was 5.9 +/- 4.1 years (range 10 months-16 years) and the most common symptom was diarrhea (81.7%) followed by abdominal distention, weight loss, anorexia and failure to thrive. Abdominal distention (60.6%), short stature (45.2%) and iron-deficiency anemia were the most common findings. High serum alanine aminotransferase levels were found in 38.3% of patients and these levels became normal after adoption of a gluten-free diet in all but two patients with cirrhosis. Immunoglobulin A, IgG antigliadin antibodies and IgA anti-endomysium antibodies were found in 76, 94 and 90% of patients, respectively. Biopsy of the small intestine revealed that 95, two and seven patients had type 3, type 2, and type 1 lesions, respectively, according to the Marsh classification. There was no statistically significant difference between patients and control groups with regard to antinuclear antibody, antismooth muscle antibody, anti-DNA and anticardiolipin antibodies (IgG and IgM). CONCLUSIONS: Although classical celiac disease was seen in most patients in the present study, clinical variability of the condition should be kept in mind. In particular, patients with uncommon findings, such as short stature, cryptogenic liver disease and iron-deficiency anemia, should also be screened for celiac disease.

Adolescent↗
PubMed2000

Comparison of standard and high dosage recombinant interferon alpha 2b for treatment of children with chronic hepatitis B infection.

BACKGROUND: Interferon is currently the most useful therapeutic agent for chronic viral hepatitis. The aim of this study was to compare the efficacy of standard and high dosages of interferon in children with chronic hepatitis B virus (HBV) infection. METHODS: Thirty children with chronic hepatitis B infection were randomly assigned to receive 5 million units/m2 body surface area (Group I) or 10 million units/m2 body surface area (Group II) recombinant interferon alpha 2b three times weekly for 6 months. Patients were followed for at least 6 months (range, 6 to 18; median, 9 months) after the end of therapy, by physical and serologic examination every 3 months. RESULTS: Clearance of HBV DNA occurred in 4 (27%) patients from Group I and 9 (60%) patients from Group II at the end of therapy. Hepatitis B e antigen (HbeAg) clearance was 7% (1 patient) and 53% (8 patients) in the two groups, respectively (P < 0.05). HBV DNA was undetectable in 40 and 60% of the children at the 12th month of randomization in Groups I and II, respectively. HBeAg/antibody to HBeAg seroconversion was found in 33% (5 patients) who received standard dosage and 60% (9 patients) in the high dosage group. Sustained complete response (normal alanine aminotransferase, negative HBeAg and HBV DNA at 12th month) was obtained in 5 and 9 patients respectively from groups I and II (P > 0.05). Only mean baseline serum alanine amino-transferase concentrations were predictive of response to interferon. CONCLUSIONS: A 6-month course of interferon alpha 2b in children with chronic HBV disease was well-tolerated by most patients. Sustained suppression of HBV was obtained in 60% of patients with high dosage interferon and in 33% of the patients receiving standard dosage. Although these results were not statistically significant, studies with more patients are needed to ascertain whether high dosage improves the response rate.

Adolescent↗