Liver transplantation from a live donor to a patient with maple syrup urine disease: Two case reports.

Liver transplantation were reported in patients with classic maple syrup urine disease in the literature. Branched chain alpha keto acid dehydrogenase activity can be improved in patients after transplant, and a protein-restricted diet is usually not needed. The first patient was a boy aged 2,5 years who presented with frequent ketosis attacks and epileptic seizures, and the second patient was an 11-month-old boy who also presented with frequent ketosis episodes, both despite adherence to diet therapy. Both patients received liver transplantations from live donors. A low protein diet was no longer required and no decline in cognitive functions was observed in either patient in the follow-up. We wanted to present these cases to show that despite a normal diet, plasma levels of branched- chain amino acids remained normal without any decline in cognitive function after liver transplantation in patients with classic maple syrup urine disease patients.

Evaluation of T-wave alternans in pediatric patients with chronic renal failure.

INTRODUCTION: Microvolt T-wave alternans (TWA) is known to be useful in prediction of ischemia and sudden death in high-risk populations and there are no studies in children with chronic renal failure (CRF). Cardiac problems seem to be responsible for an important part of death in children and young adults with CRF. The aim of this study is to evaluate Holter microvolts TWA measurements in children with CRF comparing to the control group. METHODS: This prospective study included 40 patients with CRF and 48 healthy controls. The history, echocardiography and microvolt TWA values based on 24-hour ECG recordings of the patients were evaluated. Analysis of microvolt TWA was considered on the basis of three leads (V5, V1 and AVF). RESULTS: Compared with the controls, the mean systolic and diastolic blood pressure values and average heart rates were significantly higher in the children with CRF (p = 0.001 and p = 0.026, respectively). Also, the values of left ventricular internal dimensions at end diastole and end-diastolic volume were significantly higher in CRF group (p = 0.01 and p = 0.049, respectively) and couplet ventricular extrasystole was detected in 2 patients with CRF. Consequently, all TWA values in three leads were increased in CRF group than the control group but the only increase in V5 lead was statistically significant (p = 0.028). CONCLUSIONS: This study has demonstrated that microvolt TWA values increased in pediatric patients with CRF. TWA might be used for early risk assessment in pediatric patients with CRF in the future.

Evaluation of renal functions in pediatric liver transplantation.

AKI is an important complication after LT. As our LT series contains a quite high number of children with ALF unlike published studies, we aimed to determine pre-LT and long-term renal functions in children both with ALF and with CLD. Demographic and disease-related data of 134 transplanted children were evaluated retrospectively. Pre-LT and follow-up GFR and pediatric RIFLE scores were determined. Mean pre-LT GFR was not dependent on the disease presentation or severity of chronic disease. While there was an initial decline until first week of post-LT in CLD children, an increase was observed in ALF. Neither mean GFR nor the pRIFLE on follow-up was different with respect to the type of LT or disease presentation. Mean GFR at first and sixth months were lower in children on cyclosporine compared to tacrolimus (p = 0.001 and p = 0.002, respectively). In conclusion, GFR-time curve was different in children with or without ALF. Type of LT, and severity of the CLD were not risk factors for CKD in any time, but younger age at LT, CLD, and cyclosporine usage were at sixth months of follow-up.

Management of patients with status epilepticus treated at a pediatric intensive care unit in Turkey.

We investigated the etiology, treatment, and prognosis of patients treated for status epilepticus at a pediatric intensive care unit. Medical records of 89 patients admitted to a pediatric intensive care unit with status epilepticus were reviewed retrospectively. Patients ranged in age from 2 months to 18 years (mean age ± S.D., 4.7 ± 3.8 years). Seizure etiologies comprised remote symptomatic in 47 (52.7%), febrile in 15 (16.9%), acute symptomatic in 12 (13.5%), and unknown in 15 (16.9%). Seizure durations ranged from 30-60 minutes in 58 patients, whereas 31 manifested refractory seizures longer than 60 minutes. Seizure control was achieved within 30 minutes in 55 patients, from 30-60 minutes in 19, and after 60 minutes in 15. Rectal diazepam was administered to 38 (42.7%) patients before admission to the hospital. Length of intensive care unit stay increased with increasing seizure duration (P < 0.05). The total mortality rate was 3.4%. This lower mortality rate may be considered evidence of the effectiveness and reliability of the status epilepticus treatment protocol in our pediatric intensive care unit. Prehospital rectal diazepam administration and the treatment of brain edema in the intensive care unit may be useful in the management of patients with status epilepticus.