Hereditary tyrosinemia type 1 in Turkey: twenty year single-center experience.

Background: Hereditary tyrosinemia type 1(HT1) is a chronic disorder leading to severe hepatic, renal and peripheral nerve damage if left untreated. Despite nitisinone treatment HT1 still carries the risks of hepatocellular carcinoma (HCC) and neuropsychological outcome.

Methods: A retrospective single center study was carried out based on the phenotype, therapy and outcome in 38 Turkish patients with HT1 diagnosed during the last 20 years.

The effect of low-carbohydrate diet on left ventricular diastolic function in obese children.

Background: This study was conducted to evaluate left ventricle (LV) functions using conventional and tissue Doppler imaging in childhood obesity and to identify the effects of diet on LV diastolic functions.

Methods: Conventional and tissue Doppler echocardiographic measurements were compared in 34 obese children and 24 age- and gender-matched lean controls. Fasting plasma glucose, insulin and homeostatic model assessment of insulin resistance levels were also obtained.

Right ventricular subclinical diastolic dysfunction in obese children: the effect of weight reduction with a low-carbohydrate diet.

This study aimed primarily to identify whether childhood obesity leads to right ventricular (RV) subclinical diastolic dysfunction or not. It also aimed to examine the effect of weight reduction on right ventricular function. Standard and tissue Doppler echocardiography was performed for 28 overweight (body mass index [BMI], 25-30 kg/m(2)) and 34 obese (BMI, > or = 30 kg/m(2)) children and for 29 age- and sex-matched lean referents (BMI, 17-25 kg/m(2)).

Nutritional status of patients hospitalized in pediatric clinic.

Background/aims: The aim of this study was to evaluate the nutritional status of children at the moment of their hospitalization and to investigate whether there is a relation between the diagnosis and nutritional status.

Methods: Patients hospitalized in the Children's Clinic of Cerrahpasa Medical Faculty (excluding emergency, newborn intensive care and newborn special care units) between December 2003 and August 2004 were included in the study. All patients' height, weight, weight for height head circumference, arm circumference, triceps skin-fold thickness, and Z results of the height and weight were measured and, in accordance with the anthropometric measurements at the moment of hospitalization, each patient's nutritional status was evaluated.