Yenidoğan ve süt çocukluğunda kolestaz

During the newborn and infancy period, it is an important to demonstrate the condition which causes cholestatic liver diseases. If direct bilirubin level is more than 20% of total bilirubin, it is defined as cholestasis. Especially early diagnosis of diseases including biliary atresia, tyrosinaemia, galactosaemia is crucial for prevention of permanent damage in the future and for benefit from early treatment. Therefore, total, direct and indirect bilirubin levels should be measured in all newborns with jaundice lasting more than two weeks. If 20% of total bilirubin is direct bilirubin, liver-related disorders should be questioned. In this review, we aimed to show which clinical and laboratory features should be considered to demonstrate the cause of cholestatic diseases. (Turk Arch Ped 2012; 47: 1-7

Role of RDW in Prediction of Burn after Caustic Substance Ingestion

A quantifiable, quick, inexpensive and reproducible predictor is needed to decide if caustic substance ingestion results in burn regardless of the symptoms. A multicenter cohort study was conducted to investigate the predictive value of red cell distribution width (RDW) in detecting the esophageal burns. The data of 174 patients were retrospectively analyzed. Eleven patients were excluded due to inability to define the substance ingested. Complete blood count (CBC) was taken at admission, and an esophagogastroduodenoscopy was performed within the first 12–24 h in all patients, regardless of their symptoms. The age and gender of the patients, the types of substances ingested, the parameters in the CBC and the severity of the esophageal injury were correlated. Esophageal burns were diagnosed in 38 of 163 patients (23.3%). The risk of esophageal burn with RDW values below 12.20 was significantly lower. Multivariate analysis showed that RDW was the most significant predictor of esophageal burn (p = 0.000, odds ratio (OR) 7.74 (95% confidence interval (CI), 3.02–19.9)). Receiver operating characteristic (ROC) curve analysis demonstrated 84.2% sensitivity at a cut-off value of 12.20 for RDW. The results showed that CBC parameters could avoid unnecessary esophagogastroduodenoscopy. The RDW values regardless of the symptomatology is a good predictor of esophageal burns, and an RDW value over 12.20 shows the increased risk of esophageal burn

Importance of the Human Leukocyte Antigen Tissue Type in Detecting Celiac Disease in Two Families

Celiac disease (CD) is strongly related to human leukocyte antigens (HLA) genes. The incidence of CD is very low in patients without the HLA-DQ2.5 and HLA-DQ8 leukocyte antigens. This study presents two families with CD and shows that the disease might be familial. The determination of HLA tissue groups is important and family screening tests may lead to early diagnose of the disease. In both of the families, the patients' siblings and fathers were asymptomatic, but diagnoses were made after family screening tests. HLA tissue typing was performed in all family members. The patients, siblings and fathers carried the HLADR3-DQA1*0501-DQB1*0201 tissue groups in both two families while this tissue group was not present in the mothers. As CD is strongly related to certain HLA groups and has a genetic predisposition, all members of a family must be screened when a case of CD is diagnosed in any family member. On the other hand, the determination of the HLA tissue type in patients with both symptoms and very positive serologic tests is important in terms of suspicion of the disease

Long-Term Follow-Up of Children with Inflammatory Bowel Disease: Evaluation of 53 Cases

Introduction: In this study it was aimed to determine the long-term demographic, clinical and laboratory characteristics, together with the responses to therapy, in children diagnosed with inflammatory bowel disease (IBD)

The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients

Background Cystic fibrosis (CF) causes malabsorption of nutrients that exacerbate pulmonary problems. Nutritional interventions can improve pulmonary functions. We aimed to evaluate the effects of nutritional intervention in CF patients with malnutrition, and to determine if there is a correlation between nutritional status and pulmonary functions

An easy method for diagnosing macro-aspartate aminotransferase: A case series

Macro-aspartate transaminase (macro-AST) must be considered when the aspartate transaminase (AST) level is chronically high without any liver, cardiac, or muscle disease. Many specialized laboratory techniques have been recommended for diagnosing macro-AST, including the polyethylene glycol immune precipitate technique, which is simple. This study presents a considerably easier method based on the studies of Davidson and Watson and Castiella et al. Our method is based on the decrease in the plasma AST level after storage of the macroenzyme at 2-8 degrees C for 5 days, and has the advantages of low cost, reliability, and practicality at any health center. In our eight cases of macro-AST, the AST activity at day 6 had decreased by more than 50% from day 1. This method is practical for primary healthcare facilities because of its easy application and accurate results, and obviated the need for unnecessary tests after diagnosis

CLINICAL EVALUATION OF CHILDRENS WITH SUBACUTE SCLEROSING PANENCEPHALITIS

Objective: The purpose of this study was to investigate retrospectively the clinical, electrophysiological and neuro radiological features of patients with subacute sclerosing panencephalitis (SSPE)