In this study we evaluated the indications, complications, and the spectrum of histopathological results of percutaneous renal needle biopsy (PRNB) performed in our clinic. Between June 1990 and December 2006, 679 PRNBs were performed on native kidneys of 614 children (304 boys, 310 girls) with a mean age of 10.4 years. Most frequent indications for PRNB were nephrotic syndrome (47%), hematuria, and/or proteinuria (15.9%), acute renal failure (14.6%) and complex renal manifestations (18.9%). The overall complication rate was 15.2%. The most common complications were perirenal hematoma (12.4%) and macroscopic hematuria (2.6%). The most frequent histopathological group of diseases were glomerulopathies; these were diagnosed in 376 patients (61.2%) and included membranoproliferative glomerulonephritis (11.1%), mesangial proliferation (10.7%), diffuse proliferative glomerulonephritis (7.7%), and focal segmental glomerulosclerosis (7.3%) as the most frequent. The second most frequent group of histopathology was manifestations secondary to systemic diseases; these were shown in 195 patients (31.8%). Amyloidosis (11.4%) and Henoch-Schönlein nephritis (9.9%) made the majority of this group. In conclusion, our study demonstrated that PRNB is a safe procedure with usually transient complications showing the most frequent renal diseases that cause diagnostic and therapeutic difficulties for pediatric nephrologists.

In this study, the authors aimed to evaluate buccal midazolam as a practical and safe alternative medication for children who suffer from seizures in the emergency setting and in home practice or anywhere. The effects and side effects of buccal midazolam and rectal diazepam were compared in the treatment of acute convulsions in 43 children, ranging in age from 2 months to 12 years who were seen at the emergency service of the children hospital. Midazolam was given on the even days of the month and diazepam was given on the odd days. In the midazolam group, the seizures of 18/23 (78%) patients terminated in 10 minutes; however 5/23 (22%) patients did not respond. In the diazepam group 17/20 (85%) patients responded in 10 minutes, but 3/20 (15%) did not respond. Midazolam was found to be as effective as diazepam and the difference was not statistically significant (p<0.05). Response periods of the 2 drugs showed no significant difference (p>0.05). The need for a second drug for seizures that did not stop with the first drug was equal, and the difference was not statistically significant (p>0.05). They did not observe any serious complications. In conclusion, buccal midazolam is safe and as effective as rectal diazepam for the treatment of seizures.

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Background/Aims: Evaluation of the risk factors, and phenotype-genotype correlation of familial Mediterranean fever (FMF) gene (MEFV) and serum amyloid A1 (SAA1) gene polymorphisms in renal amyloidosis. Methods: We investigated MEFV and SAA1 genotypes (α, β, and γ isoforms) in 50 FMF patients and 50 healthy children. Tel-Hashomer criteria were used for the diagnosis and severity scoring of FMF. Results: The most common MEFV mutation and SAA1 genotype were M694V/M694V (n = 26/50) and SAA1 α/α (n = 26/50), respectively. Positive family history for amyloidosis was significantly higher (p < 0.001) with more severe clinical course (p = 0.006) in the amyloidosis group than the non-amyloid group. In M694V/M694V mutation, erysipelas-like skin erythema (p = 0.029), arthritis (p = 0.004), arthralgia (p < 0.001) were significantly more frequent with higher severity scores (p = 0.008) than the patients with other mutations. Comparison of the SAA1 α/α genotype with other genotypes revealed more frequent arthritis (p = 0.003) in the SAA1 α/α genotype. In amyloidosis group patients having both M694V/M694V and SAA1 α/α genotypes were the largest subgroup (n = 14, p < 0.001). Logistic regression analysis for amyloidosis corrected risk revealed a 1.2 times increase in M694V/M694V, a 2.4 times increase in SAA1 α/α genotypes and a 2.5 times increase when both are together. Conclusion: Positive family history for amyloidosis and presence of SAA1 α/α genotype in M694V/M694V mutation may predispose to amyloidosis by increasing the clinical severity. Therefore, in such children early colchicine treatment might be recommended even if they are asymptomatic.

Kawasaki disease is the most common vasculitis of childhood. In its classical form, at least four of five diagnostic criteria including cervical lymphadenopathy (1.5 cm or more), nonsuppurative conjunctivitis, intraoral mucosal changes, edema in hands and feet, and maculopapular rash are required with prolonged fever over 5 days. Atypical cases which are different from the classical type or incomplete cases which does not include all the diagnostic criteria can be seen. The typical Kawasaki disease is a self-limiting disease with fever lasting for an average of two weeks. In such patients who have not been diagnosed and whose treatment has been delayed, coronary artery aneurysm, myocardial depression, arrhythmia, and vascular complications may increase morbidity and mortality. We would like to present a rare case of an atypical patient with gallbladder hydrops and acute cholestatic hepatitis.