Neurological manifestations of atypical celiac disease in childhood.

Various typical and atypical neurological manifestations can be seen as the initial symptoms of celiac disease (CD). We suggest that gluten toxicity is the most suspicious triggering risk factor for probable pathophysiological pathways of neurological involvement in atypical CD. The medical charts of 117 patients diagnosed with atypical CD were retrieved from a tertiary center in Ankara, Turkey. Eight patients reported as having neurologic manifestations as initiating symptoms were evaluated in detail. The initial neurological manifestations of CD in our study included atypical absence, which was reported first in this study, generalized tonic-clonic seizures, complex partial seizures, severe axial hypotonia and down phenotype, multifocal leukoencephalopathy, mild optic neuritis, attention deficit hyperactivity disorder, and short duration headaches. Seizures mostly emphasizing atypical absence could be the initial presentation manifestation of CD, first described in this literature. Gluten toxicity could be one of the most powerful triggering factors for developing epilepsy in CD. Learning disorders such as attention deficit hyperactivity disorder, short duration headaches, mild optic neuritis, encephalopathy, and DS could also be the initial neurological manifestations of atypical CD. A gluten-restricted diet may improve neurological complaints, epileptic discharges, and neuropsychiatric symptoms. All we found may be a small part of the full range of neurological disorders of unknown origin related to CD. Clinical suspicion should be the rule for accurate diagnosis of the disease.

Abnormal circadian blood pressure regulation in liver transplanted children.

HT is a frequent cardiovascular risk factor in liver transplant recipients. However, there are only few studies in the literature regarding the risk of HT in liver transplanted children. The aim of this study was to assess the 24 h BP profiles of liver transplanted patients and to compare the results with healthy children. ABPM was performed on 20 liver transplanted patients and 27 healthy children aged 7.1 ± 4.8 and 8.5 ± 2.9 yr, respectively. HT was defined as SDS > 1.64 (i.e., >95th percentile) adjusted for gender and height. The mean duration of post-transplant follow-up was 32 ± 19 months. Six (30%) patients were found to be hypertensive. The physiological nocturnal BP fall was attenuated significantly in the study group for diastolic BP (11.5 ± 6.1 mmHg vs. 17.7 ± 7.1 mmHg, p = 0.006). Specifically, the number of patients with high nighttime systolic and diastolic BP SDS (p = 0.02 and p = 0.004, respectively) as well as elevated nighttime systolic (p = 0.03) and diastolic (p = 0.003) BPLs was found to be significantly higher than those in the controls. Alteration of the "normal" circadian rhythm is very frequent in liver transplant recipients. Thus, it is recommended to perform ABPM on all liver transplanted children not to underdiagnose HT.

Homocysteine, fibrinogen and anti-ox-LDL antibody levels as markers of atherosclerosis in prepubertal obese children.

BACKGROUND: Homocysteine, fibrinogen and antibodies to oxidised LDL were shown to be important markers of atherosclerosis in adults. AIM: To investigate the levels of these three risk factors in prepubertal obese children. METHODS: Fasting homocysteine, fibrinogen and antibodies to oxidised LDL, plasma lipids, insulin, HbA1c and blood glucose levels were investigated in 30 prepubertal obese and 28 control children 6-9 years old. Investigations in the obese group were repeated after an oral glucose tolerance test. RESULTS: Fasting fibrinogen levels of the obese children were found to be significantly higher than those in the controls. Anti-ox-LDL antibody levels increased significantly after an oral glucose tolerance test. CONCLUSION: Fasting fibrinogen and postload ox-LDL levels which could act as important markers of coronary heart disease in later life could also be important risk factors in prepubertal obese children.

Endothelial activation and inflammation in prepubertal obese Turkish children.

To investigate the degree of endothelial activation and inflammation in prepubertal obese children and to determine the relationship between the markers of endothelial activation, inflammation, and cardiovascular risk factors. In 30 obese and 28 healthy prepubertal children, soluble intercellular adhesion molecule-1 and endothelial leukocyte adhesion molecule-1 (sE-selectin) as markers of endothelial activation and soluble vascular cell adhesion molecule-1 (sVCAM-1) and C-reactive protein (CRP) as markers of endothelial inflammation in addition to cardiovascular risk factors including blood lipids, glucose, insulin, hemoglobin A1c, and systolic and diastolic blood pressure were investigated and compared. The tests were repeated after an oral glucose tolerance test in the obese group. Fasting CRP levels were found to be significantly higher in obese children. Vascular cell adhesion molecule-1 levels were found to be significantly increased in obese children after oral glucose tolerance test. Fasting CRP was positively correlated with body mass index (BMI) and low-density lipoprotein, whereas sE-selectin was positively correlated with total cholesterol. In the obese group, postload levels of soluble sE-selectin was positively correlated with low-density lipoprotein; sVCAM-1 was positively correlated with insulin and homeostasis model assessment values. Postload soluble intercellular adhesion molecule-1, sVCAM-1, and soluble sE-selectin levels were also positively correlated with each other. In the fasting state, BMI was the significant independent risk factor for CRP, and total cholesterol was the significant risk factor for soluble sE-selectin. Insulin resistance was the significant independent risk factor for postload sVCAM-1, and postload low-density lipoprotein stood as the significant independent risk factor for postload soluble sE-selectin. Endothelial inflammation is present in obese prepubertal children and is mainly associated with insulin resistance and lipid levels as well as BMI.

Clinical approach to graft hepatic artery thrombosis following living related liver transplantation.

Hepatic artery thrombosis (HAT) has an occurrence rate of 1.7-26% following living donor liver transplantation (LDLT) and is one of the most common reasons for graft loss and mortality in this population. There is a higher incidence of HAT in pediatric recipients. The aim of this case report is to discuss clinical approaches for the treatment of HAT occurring in the early post-operative period after LDLT. An 11-month-old, 7.8-kg female with cirrhosis secondary to biliary atresia underwent LDLT at Gazi University Hospital in Ankara. The graft was a left lateral segment from her father with a left hepatic artery (HA) of 2 mm diameter and a graft weight/recipient body weight ratio of 2.0%. After an uneventful early post-operative period, HAT was diagnosed by Doppler ultrasonography (USG) on the fifth post-operative day. Following angiographic evaluation, immediate exploration and reanastomosis was performed using an operation microscope. Post-operatively, the HA was patented by Doppler USG and graft function returned to normal. Now, 42 months later, the patient continues to do well with normal graft function, using a regimen of tacrolimus monotherapy for immunosuppression. In countries which have very limited resources for urgent re-transplantation, given their serious donor shortage, graft salvage may be the only option for patient survival when HAT occurs. In these circumstances, early diagnosis and immediate revascularization may be the only method for graft salvage. A daily routine of Doppler USG examination in the early post-operative period may provide a method for the early diagnosis of HAT, before liver enzymes are elevated and hepatic necrosis has begun.