PubMed Health⌕ Search

Biomedical subjects

N Koçak

Publications and source records attributed to N Koçak.

At least 55 records · Page 3Linked to original sources
PubMed1999

Ursodeoxycholic acid treatment in children with Byler disease.

BACKGROUND: There have been a few reports of patients with Byler disease and the best medical treatment is not known. The aim of the present study is to show the effect of ursodeoxycholic acid (UDCA) on clinical, laboratory and histologic findings in children with Byler disease. METHODS: Nine children aged between 1.5 and 9 years with Byler disease were administered UDCA orally at doses of 15-20 mg/kg per day. They were followed for at least 12 months. Clinical, laboratory and histologic outcomes were evaluated after 12 months of treatment. RESULTS: Seven children presented in the first 6 months of life with itching and/or jaundice. Gamma-glutamyl transpeptidase and cholesterol levels were normal in all patients, despite severe cholestasis. With UDCA therapy, pruritus disappeared/diminished in four (44.4%) patients. The mean serum concentrations of alanine aminotransferase, aspartate aminotransferase (AST), total and conjugated bilirubin decreased, although it was significant only for AST (P = 0.01). Before treatment, all biopsy materials showed cellular/canalicular cholestasis and fibrosis. After UDCA therapy cholestasis was ameliorated. Two patients died during follow up. CONCLUSIONS: The results suggest that administration of UDCA leads to clinical and biochemical improvement in children with Byler disease. The UDCA ameliorates symptoms partially, improves the life quality of patients and may be given for as long as the disease continues.

Child↗
PubMed1999

Retreatment with higher dose interferon alpha in children with chronic hepatitis B infection.

BACKGROUND: More than 50% of children with chronic hepatitis B infection do not respond to interferon-alpha (IFN-alpha) treatment and are prone to have progressive liver disease. The best treatment modality is unknown in these children. The aim of this study was to evaluate the possible benefit of a second higher dose IFN-alpha therapy for children with chronic hepatitis B diseases who failed previous therapy. METHODS: Twenty-four children with chronic hepatitis B infection who had not responded to previous IFN-alpha treatment were enrolled into the study. All were hepatitis B virus DNA- and hepatitis B e antigen-positive for >6 months after initial treatment. They received 10 megaunits (MU)/m2 of IFN-alpha 2a three times a week for 24 weeks. Liver function tests, hepatitis B virus markers and hepatitis B virus DNA were determined regularly during treatment and follow-up. A complete response was defined as clearance of both hepatitis B virus DNA and hepatitis B e antigen (HBeAg). RESULTS: At the end of therapy 8 (33.3%) patients cleared hepatitis B virus DNA and seroconverted to anti-HBeAg. Patients were followed for an average period of 12.2 +/- 4.7 months after retreatment. During follow-up an additional 4 patients cleared hepatitis B virus DNA and seroconverted to anti-HBe, whereas one seroconverted patient became HBeAg-positive again. Thus 11 patients (45.8%) had complete response at the end of the follow-up period. Alanine aminotransferase normalized in 11 responder patients and in 5 nonresponders. Positive predictive factors were low baseline titers of hepatitis B virus DNA and elevated transaminase values (> 100 IU/l). CONCLUSIONS: IFN-alpha retreatment with a higher dose may be an alternative modality for treatment of children with chronic hepatitis B infections who failed previous IFN-alpha, especially in those with favorable predictive factors.

Adolescent↗
PubMed1998

Pseudotumor formation in tibia in Gaucher's disease.

A very rare case of type 1 Gaucher's disease with pseudotumor formation in the right tibia is presented. In addition to the characteristic radiographic finding of distal femoral flaring (Erlenmeyer flask deformity), a lobulated osteolytic area with surrounding sclerosis was seen in the proximal metaphysis of the right tibia. Adjacent to this pseudotumor appearance was an old pathologic fracture and no significant soft tissue swelling was evident, both of these features being reported for the first time in association with Gaucher's disease.

Adolescent↗
PubMed1997

Nonsyndromic paucity of interlobular bile ducts: clinical and laboratory findings of 10 cases.

BACKGROUND: Reports concerning nonsyndromic paucity of the interlobular bile ducts are not common. METHODS: The clinical, biochemical, and histological features of ten such children were described. RESULTS: All presented with jaundice, starting in the first month in seven and in the fourth, seventeenth, and thirtieth month in the others. Alcoholic stools were present intermittently in seven and persistently in three patients. Pruritus was a prominent symptom in five. Liver function tests were abnormal in all but one. Liver biopsies were performed at ages of 20 days to 3 years (median 5 months). In addition to a paucity of interlobular bile ducts, histology revealed intracellular cholestasis in all, portal fibrosis in four, and regenerative nodules in two patients. Complications of fat-soluble vitamin deficiency occurred in seven. Therapy consisted of supplementation of those vitamins and administration of cholestyramine, phenobarbital, prednisolone, or ursodeoxycholic acid. While one child had a successful orthotopic liver transplantation, three died. Consanguinity rate was 80% among the parents, and five of the patients had siblings with similar symptoms. CONCLUSIONS: Prognosis of these patients is variable. Differentiation from other forms of cholestasis is important especially to avoid surgery.

Bile Ducts↗
PubMed1997

Extrahepatic portal venous obstruction in childhood: etiology, clinical and laboratory findings and prognosis of 34 patients.

Extrahepatic obstruction of the portal vein is a well known cause of portal hypertension in childhood, that causes severe morbidity. We evaluated 34 children (24 boys, 10 girls, age 4.5 months to 12 years, mean 5.5 +/- 3.8 years) with this diagnosis, to define the clinical picture, laboratory changes, diagnostic tools and therapeutic modalities. Gastrointestinal bleeding was the commonest mode of presentation (64.7%), with the second being splenomegaly. The cause of the obstruction could be determined in 38.2% (13/34) of the subjects. At the beginning of the study the main diagnostic procedure was splenoportography although in more recent years pulsed duplex Doppler ultrasonography has been used. The follow up period was median of 5 years (range 1-11 years). The mean number of bleeding episodes was 4.7 +/- 5.9 (range 1-26), while nine patients never bled. There was no mortality. Ten patients underwent surgery, while sclerotherapy was performed on 10. Twenty-one patients received beta-blocker drugs. No difference was found among these therapeutic modalities. It is well established that the major risk for children with extrahepatic portal vein obstruction is gastrointestinal bleeding which is tolerated quite well. Surgery should be indicated only in children where bleeding cannot be controlled by medical means including sclerotherapy.

Child↗
PubMed1992

Gaucher's disease with mitral and aortic involvement: echocardiographic findings.

Cardiac involvement in Gaucher's disease has been reported in only a few patients, mostly adults with pericardial changes. We describe findings in two siblings with Gaucher's disease, aged 15 and 9 years respectively, in whom mitral and aortic valve lesions were evaluated by auscultation and echocardiography. In both cases the mitral and aortic valves were thick and restricted in motion. Continuous Doppler echocardiography revealed significant mitral regurgitation and mitral stenosis. At the aortic valve level there was a systolic pressure gradient. Echocardiographic investigation of patients with suspected cardiac involvement with Gaucher's disease is recommended.

Adolescent↗