Kenan Bek

The effect of passive smoking on pulmonary function during childhood.

Passive smoking, especially of maternal origin, is known to influence adversely the development of childrens pulmonary function. In this study, the effect of parental smoking on the pulmonary function of 360 primary school children aged 9–13 (mean 10.8±0.7) years was investigated. Information on parental smoking history was collected using a questionnaire, and spirometric measurements were performed on the children. All spirometric indices were lower in children who had been passively exposed to parental tobacco smoke than those not exposed. The percentage of households in which at least one parent smoked was 81.5%. This figure was significantly lower for mothers (27.5%) than for fathers (79%). Paternal smoking was associated with reduced levels of forced expiratory flow between 25–75% of vital capacity, peak expiratory flow, and flow rates after 50% and 75% of vital capacity expired (p<0.05). Maternal smoking did not have statistically significant adverse effects on childrens pulmonary function. This result might be due to the low occurrence of either pre- or post-natal smoking among mothers and confirms that, in our population, the main target group for anti-tobacco campaigns should be fathers.

Carbamazepine poisoning managed with haemodialysis and haemoperfusion in three adolescents

Carbamazepine is a widely used antiepileptic agent. Accidental or suicidal overdose in children is not uncommon. Acute toxicity is associated with seizures, coma, arrhythmias and death in severe cases. Here we report three adolescents with carbamzepine overdose, two managed with standard low‐flux haemodialysis and one with charcoal haemoperfusion. Our report emphasizes that haemodialysis might be a cheaper and easier alternative for carbamazepine overdose in milder cases, with fewer side‐effects than haemoperfusion.

Genetic risk factors of amyloidogenesis in familial mediterranean fever

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.

Cerebral vasculitis in a child with Henoch-Schönlein purpura and familial Mediterranean fever.

In this case report, a 10-year-old girl with Henoch–Schönlein purpura (HSP) with severe central nervous system involvement and also having familial Mediterranean fever (FMF) is presented.

Charcoal haemoperfusion in Amitriptyline poisoning: Experience in 20 children

Aim:  Tricyclic antidepressant (TCA) toxicity is common among children and adults due to widespread use. Amitriptyline (AT) is one of the most commonly prescribed TCAs. Current guidelines do not recommend charcoal haemoperfusion (HP) for AT overdose due to high protein binding and large volume of distribution. However evidence regarding the efficacy of charcoal HP in addition to supportive measures is accumulating in the published reports.

Physical function, muscle strength and muscle mass in children on peritoneal dialysis.

The aim of this study was to examine the physical function and muscle strength of children on peritoneal dialysis (PD) and to assess whether the muscle structure alterations influence physical function and muscle strength in these children. Twenty-two children on PD and 16 healthy children were enrolled into the study. A 6-min walk distance and gait speed tests were used to evaluate physical performance. Quadriceps muscle strength (QMS) was measured with a hand-held dynamometer. Magnetic resonance imaging was used to determine the cross-sectional area (CSA) and T2 signal intensity of the quadriceps muscle. Significant differences in the performance of these functional tests were found between PD patients and controls. Quadriceps muscle strength was significantly lower in PD patients than in controls. The CSA corrected for the body mass index (CSA/BMI) was not different between groups, whereas T2 signal intensity was significantly higher in PD patients than in the controls. Physical functioning tests and QMS had a close relationship with muscle CSA/BMI and with T2 signal intensity. In conclusion, along with the other previously documented mechanisms, increased fat in muscles may contribute to the decreased physical functioning and muscle strength in PD patients.

Central Pontine and Extrapontine Myelinolysis Owing to Disequilibrium Syndrome

Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient had depressed consciousness, agitation, tremor, stupor and hyperactive deep tendon reflexes toward the end of the second peritoneal dialysis. A brain computed tomographic (CT) scan showed hypodense lesions in pontine and extrapontine locations without radiocontrast medium enhancement After 2 days, the patient had only minimal memory deficits. A control brain CT scan 1 week later showed a decrease of the lesions in central pontine and extrapontine locations. Central pontine and extrapontine myelinolysis should be suspected and investigated in the acute neurologic disorders of dialysis patients.Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient had depressed consciousness, agitation, tremor, stupor, and hyperactive deep tendon reflexes toward the end of the second peritoneal dialysis. A brain computed tomographic (CT) scan showed hypodense lesions in pontine and extrapontine locations without radiocontrast medium enhancement. After 2 days, the patient had only minimal memory deficits. A control brain CT scan 1 week later showed a decrease of the lesions in central pontine and extrapontine locations. Central pontine and extrapontine myelinolysis should be suspected and investigated in the acute neurologic disorders of dialysis patients. (J Child Neurol 2003;18:292—296).

Cryptosporidiosis: A rare and severe infection in a pediatric renal transplant recipient

Acikgoz Y, Ozkaya O, Bek K, Genc G, Sensoy SG, Hokelek M. Cryptosporidiosis: A rare and severe infection in a pediatric renal transplant recipient.

Long-Term Efficacy and Safety of Quadruple Therapy in Childhood Diffuse Proliferative Lupus Nephritis

In this study, we evaluated the frequency, clinical presentation, treatment protocols, prognostic factors, and outcome in children with diffuse proliferative lupus nephritis (DPLN). Between June 1990 and December 2004, 46 patients were diagnosed to have systemic lupus erythematosus (SLE), and 26 of them (56.5%) were found to have DPLN. Renal manifestations were present in 25 patients, and the majority of them presented with severe renal findings, such as nephrotic syndrome and renal failure. All patients were given a quadruple therapy protocol including 6–12 monthly courses of methyl prednisolone pulse therapy combined with oral prednisolone, oral cyclophosphamide, azathioprine, and dipyridamole. Nineteen of these patients were regularly followed up with a mean follow-up period of 5.9 years. Complete remission was achieved in 15 of 19 patients, and chronic renal failure developed in four patients. Renal survival rate was calculated to be 78.9% at the end of 5, 10, and 14 years. Although nephrotic range proteinuria, hypoalbuminemia, renal failure, and activity index above 12/24 at presentation seemed to be associated with poor prognosis, no significant difference could be found. Hypertension and chronicity index greater than 6/12 were found to be bad prognostic predictors. We concluded that satisfactory results were achieved with our quadruple therapy protocol; thus, more aggressive and expensive therapies can be avoided and preserved for more serious and persistent diseases.

Low hair selenium and plasma glutathione peroxidase in children with chronic renal failure.

Selenium (Se) is a trace element that incorporates into the selenoenzyme glutathione peroxidase (GSH-Px). There are conflicting results regarding the Se levels and activity of GSH-Px in adult uremic patients. The aim of this study was to determine (1) the hair Se status, (2) the possible relation between the hair Se status and the antioxidant enzyme, GSH-Px, and (3) the influence of different treatment procedures on hair Se status and GSH-Px activity in children with CRI, those treated conservatively and those on HD and on CAPD. Ninety-three patients, including 32 patients with CRI, treated conservatively, 42 PD patients, 19 HD patients and 34 healthy children were enrolled in the study. The hair Se level was measured by the atomic absorption spectrophotometer method. Plasma GSH-Px activity was determined using a Randox test combination (RANSEL). Hair Se levels were significantly lower in the CRI, CAPD, and HD groups when compared to the control group (P=0.001, P=0.001, and P=0.001, respectively). Plasma GSH-Px activity was significantly lower in the CRI, CAPD, and HD groups when compared to the control group (P=0.001, P=0.001, and P=0.001, respectively). Plasma GSH-Px activity correlated with the GFR in patients with CRI and the control group (P=0.000; r2=0.60). There was no correlation between plasma GSH-Px and hair Se levels in the patient and control groups. These results revealed a decreased hair Se level and impaired antioxidative capacity in children with CRI on CAPD and HD. The lack of any relation between plasma GSH-Px and hair Se suggests that plasma GSH-Px is not a good marker of Se stores.