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Hypernatremic dehydration is an important problem that should be managed properly to avoid adverse outcomes.

It is well known that patients with solitary kidneys (SKs) are at risk of hypertension (HT), proteinuria, and glomerulosclerosis due to their low number of nephrons. 1-4 They tend to have higher levels of HT-associated end-organ damage markers, such as left ventricular mass index (LVMI) and microalbuminuria. 5 Congenital SKs (CSKs) or acquired SKs (ASKs) have different effects on glomerular filtration rate (GFR) and blood pressure (BP).

Proinflammatory and anti-inflammatory cytokines have an important role in the pathogenesis of febrile seizures (FS). Adipocytokines like interleukin 6 (IL-6), leptin, and adiponectin released from adipose tissue play a role in inflammation. This study aimed to assess the probable role of adipose tissue in children with FS. We measured serum IL-6, leptin, and adiponectin levels and evaluated clinical and laboratory findings in children with FS ( n = 32) and compared the results with the values of children of the same age with febrile illness without seizures (febrile control, FC; n = 26) and healthy control group (HC; n = 29). The serum levels of white blood cells, C-reactive protein, IL-6, leptin, and adiponectin were found to be significantly higher, while serum hemoglobin (Hb) levels were found to be significantly lower in FS and FC groups than in the HC group ( p < 0.001). When we compared the FS with the FC group, the serum Hb levels were significantly lower in the FS group than those in the FC group ( p = 0.001). There was no significant difference between the FS and FC group with regard to the serum levels of these adipocytokines ( p > 0.05). Our data showed that elevated levels of these adipocytokines as acute phase reactants in FS and FC groups did not contribute to the development of FS.

Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal alpha-galactosidase A (AGALA) activity. FD and familial Mediterranean fever (FMF) have typical clinical similarities, and both diseases may progress to end-stage renal diseases. In this study, we aimed to determine the prevalence of FD in patients with FMF from Central Anatolia of Turkey. The study group consisted of 177 FMF patients, followed up by the Adult and Pediatric Nephrology Clinic of Cumhuriyet University Hospital. Screening for AGALA activity was performed by the dry blood spot method. Mutation analysis for GLA gene was carried out for patients having an AGALA enzyme activity value lower than the normal reference value. Low AGALA activity was detected in 23 (13 %) patients. Heterozygous GLA gene mutation c.[937G>T] p.[D313Y] was detected in one female patient (0.56 %). The patient was a 53-year-old female with proteinuria and who had undergone left nephrectomy; her glomerular filtration rate (GFR) by scintigraphy was found to be 70 ml/min. She had M694V mutation and no clinical manifestation of FD. In our study, the prevalence rate of FD was found as 0.56 % in FMF patients. The similarities between the symptoms of FMF and FD might lead to a diagnostic dilemma in physicians at countries where FMF is observed frequently. Although the prevalence of FD is rare, physicians should keep in mind that FD has an ambiguous symptomology pattern of FMF.

AIMTo investigate different etiologies and management of the rhabdomyolysis in children.METHODSEight pediatric rhabdomyolysis cases who applied to the Dokuz Eylul University Faculty of Medicine Department of Pediatric Nephrology with different etiologies between January 2004 and January 2012 were evaluated in terms of age, gender, admission symptoms, physical examination findings, factors provoking rhabdomyolysis, number of rhabdomyolysis attacks, laboratory results, family history and the final diagnosis received after the treatment.RESULTSAverage diagnosis ages of eight cases were 129 (24-192) ± 75.5 mo and five of them were girls. All of them had applied with the complaint of muscle pain, calf pain, and dark color urination. Infection (pneumonia) and excessive physical activity were the most important provocative factors and excessive licorice consumption was observed in one case. In 5 cases, acute kidney injury was determined and two cases needed hemodialysis. As a result of the further examinations; the cases had received diagnoses of rhabdomyolysis associated with mycoplasma pneumoniae, sepsis associated rhabdomyolysis, licorice-induced hypokalemic rhabdomyolysis, carnitine palmitoyltransferase II deficiency, very long-chain acyl-CoA dehydrogenase deficiency, congenital muscular dystrophy and idiopathic paroxysmal rhabdomyolysis (Meyer-Betz syndrome).CONCLUSIONIt is important to distinguish the sporadic and recurrent rhabdomyolysis cases from each other. Recurrent rhabdomyolysis cases should follow up more regardful and attentive.

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