Meral Torun Bayram

Clinical and radiological course of simple renal cysts in children.

OBJECTIVE To evaluate the clinical and, particularly, radiological course of simple renal cysts in children. MATERIALS AND METHODS Children with simple renal cysts were retrospectively evaluated, especially for change in the cyst diameter during follow-up and complications. In addition, the rate of increase in cyst size per year was calculated, and those characteristics of the cyst were analyzed to predict aggressiveness. RESULTS Simple renal cysts were detected in 45 (21 male) patients. Mean ages at diagnosis and follow-up period were 7.4 ± 4.9 and 2.9 ± 1.8 years, respectively. Forty-one of the 45 patients were followed up for longer than 1 year. Diameter of the cyst increased in 20 (49%), decreased in 4 (10%), unchanged in 13 (31%), and disappeared in 4 (10%) of patients. The average size increase and average rate of enlargement in simple cysts were 0.3 mm and 1.0% per year, respectively. Furthermore, in 19 (95%) patients, the cyst size increased in the first 2 years. Among baseline parameters, only initial cyst size was an independent predictor of annual growth rates (beta = 0.628; P <.001). The relationship between the initial cyst size and annual growth rates was determined as positive (r = 0.459, P = .003). Two patients with large cysts developed severe complication in the first 6 months. One of these underwent nephrectomy because of rapid increase in cyst diameter (170 mm), renal artery/vein compression, and massive hematuria. In the other patient with severe loin pain regarding simple cyst (73 mm), percutaneous aspiration was performed. CONCLUSION Simple renal cysts in childhood tend to slowly increase in size. However, regular radiological follow-up might be important, especially in children with large size of cyst at diagnosis, because of more rapid increase in cyst size.

Place of Ultrasonography in Predicting Vesicoureteral Reflux in Patients With Mild Renal Scarring

OBJECTIVE To evaluate the role of renal ultrasonography (USG) in predicting vesicoureteral reflux (VUR) in children with mild renal scarring determined by dimercaptosuccinic acid scintigraphy performed after attack of urinary tract infections (UTI). METHODS Dimercaptosuccinic acid scintigraphy, voiding cystourethrography (VCUG), and renal USG findings were evaluated retrospectively in children with UTI. Each kidney was evaluated as a separate renal unit (RU). RUs with severe scarring were excluded from the study. RUs having mild scarring with and without abnormal USG findings (group 1 and group 2, respectively) were compared in terms of the presence of VUR. RESULTS There were a total of 228 patients (70 men, mean age 47.06 ± 44.14 months) and 456 RUs. Of the 185 RUs with mild scarring, 55 had abnormal USG findings (group 1), whereas 130 had normal USG findings (group 2). The rates of VUR and severe VUR (≥grade 4) were higher in group 1 compared with group 2 (69% vs 43%, P = .001 and 35% vs 7% respectively, P <.001). The sensitivity, specificity, positive predictive value, negative predictive value, and odds ratio of USG findings in predicting VUR in RU with mild scarring were 68%, 80%, 38%, 93%, and 8.2, respectively. CONCLUSION Normal renal USG findings exclude a diagnosis of high-grade VUR to a large extend in children with UTI and mild renal scarring. Refraining from invasive VCUG might be a reasonable approach in these patients provided that no other predisposing factors for UTI and/or renal scarring present.

Cefixime-induced oculogyric crisis.

Oculogyric crisis is a neurologic adverse event characterized by bilateral dystonic, usually upward, conjugate eye deviations. Cefixime is a third-generation cephalosporin and is widely used in clinical practice in childhood. Confusion, encephalopathy, coma, myoclonus, nonconvulsive status epilepticus, and seizures have been described with the use of cephalosporins. We presented a cefixime-induced oculogyric crisis in a 7-year-old boy during the treatment of urinary tract infection, and this is the first case of cefixime-induced oculogyric crisis whose ocular symptoms gradually disappeared within 48 hours after the drug was discontinued.

Carnitine palmitoyl transferase II deficiency in an adolescent presenting with rhabdomyolysis and acute renal failure.

The most common cause of recurrent rhabdomyolysis in childhood is inherited metabolic disorders. Carnitine palmitoyl transferase II (CPT II) deficiency is a lipidosis and is a common cause of inherited recurrent myoglobinuria. The disease is inherited in autosomal recessive trait, and the clinical phenotype ranges from a severe and multisystemic infantile form to a milder muscle form, which is characterized with rhabdomyolysis and myoglobinuria. Exercise, infection, fasting, and cold are the most important triggering factors of rhabdomyolysis in CPT II deficiency. The severity of attacks is highly variable and some of these attacks may be complicated by acute renal failure. We report a case of a 13-year-old girl with recurrent rhabdomyolysis due to CPT II deficiency whose last attack was complicated by acute renal failure.

Bioelectric Impedance Analysis in the Diagnosis of Vesicoureteral Reflux

Background: Vesicoureteral reflux (VUR) is a common abnormality of the urinary tract in childhood. Objectives: As urine enters the ureters and renal pelvis during voiding in vesicoureteral reflux (VUR), we hypothesized that change in body water composition before and after voiding may be less different in children with VUR. Patients and Methods: Patients were grouped as those with VUR (Group 1) and without VUR (Group 2). Bioelectric impedance analysis was performed before and after voiding, and third space fluid (TSF) (L), percent of total body fluid (TBF%), extracellular fluid (ECF%), and intracellular fluid (ICF%) were recorded. After change of TSF, TBF, ECF, ICF (ΔTSF, ΔTBF%, ΔECF%, ΔICF%), urine volume (mL), and urine volume/body weight (mL/kg) were calculated. Groups 1 and 2 were compared for these parameters. In addition, pre- and post-voiding body fluid values were compared in each group. Results: TBF%, ECF%, ICF%, and TSF in both pre- and post-voiding states and ΔTBF%, ΔECF%, ΔICF%, and ΔTSF after voiding were not different between groups. However, while post-voiding TBF%, ECF% was significantly decreased in Group 1 (64.5 ± 8.1 vs 63.7 ± 7.2, P = 0.013 for TBF%), there was not post-voiding change in TSF in the same group. On the other hand, there was also a significant TSF decrease in Group 2. Conclusions: Bladder and ureter can be considered as the third space. Thus, we think that BIA has been useful in discriminating children with VUR as there was no decreased in patients with VUR, although there was decreased TSF in patients without VUR. However, further studies are needed to increase the accuracy of this hypothesis.

Serum concentration of brain-derived neurotrophic factor in epileptic children.

There are interesting evidences for the involvement of the neurotrophin brain-derived neurotrophic factor in the mechanism of epileptogenesis. In this study we measured the serum concentrations of brain-derived neurotrophic factor in children with epilepsy and age-matched healthy controls. There was no statistically significant difference between the mean serum concentrations of brain-derived neurotrophic factor in epileptic children (13.8 ± 1.1 ng/mL) and age-matched healthy controls (13.9 ± 0.1 ng/mL). The mean concentration of serum brain-derived neurotrophic factor was not different between generalized epilepsy and partial epilepsy. We also did not find significant difference regarding the associated clinical features of epilepsy (the presence of history of febrile convulsion, cerebral palsy). However, epileptic children with mental retardation showed lower levels of brain-derived neurotrophic factor (P = 0.015). Here we studied serum brain-derived neurotrophic factor concentration in a group of children without an active epileptogenesis based on the clinical and electroencephalographic state. Brain-derived neurotrophic factor concentrations should be investigated in children with active epileptogenesis who had refractory epilepsy and epileptic encephalopathy.

Hydronephrosis associated with ureteral wall thickness: Answer

Ureteroscopy with bilateral ureteral wall biopsy was performed. Pathologic examination of ureteral tissue samples showed infiltration of atypical giant lymphoid cells with irregular nuclear contours (Fig. 1). Immunohistochemistry demonstrated that these cells were CD20-positive and their Ki67 indices were higher than 80 %. A diagnosis of diffuse giant B-cell lymphoma was confirmed in the light of all these findings. No atypical cells were found in bone marrow aspiration performed to differentiate primary ureteral lymphoma from secondary involvement. Abdominal computed tomography (CT) imaging performed to find associated lymphadenopathies showed no lymph node involvement, but disclosed left ureteral wall thickening in addition to right ureteral wall thickening (Fig. 2). No extra-ureteral involvement was demonstrated in positron emission tomography (PET) leading to the diagnosis of primary grade 3, non-Hodgkin lymphoma of the ureters. The patient has been given the initial course of systemic chemotherapy.

Myalgia as a symptom of familial Mediterranean fever in children.

We read the article entitled Familial Mediterranean fever gene mutation frequencies and genotype–phenotype correlations in the Aegean region of Turkey prepared by Ozalkaya et al. [1]. The results will be more rational while describing the characteristics of an inherited disease in a certain geographic region, if the data obtained from all or near all of the patients in that region is given. As being another center in the Aegean Region, serving children with familial Mediterranean fever (FMF), we compared our results with those of the above study and aimed to evaluate their consistency. There were 172 patients (M/F: 89/83) being followed up in our center with the diagnosis of FMF based on Tel Hashomer criteria. The mean ages at the onset of symptoms and at the time of diagnosis were months (1–292) and 115 § 63 months (2–612), respectively. Clinical features of the patients are shown in Table 1. MEFV gene analysis including 12 most frequent mutations seen in Turkish people was performed in 84 patients. The results are shown in Table 2. Most frequent mutation was M694V that was present in 60 (71.4%) patients. Three cases with amyloidosis had MEVF gene analysis; two cases were homozygous and one case was heterozygous for M694V mutation. There were 6 patients with none of the twelve mutations screened. The data of our cases from the same geographic region including the mean age of the patients at the diagnosis and at onset of the symptom, frequency of symptoms except for myalgia and genotypes of the patients were found similar to those described in the study of Ozalkaya et al. Although from the same region, myalgia was signiWcantly more frequent among our patients than those in the study of Ozalkaya et al. They categorized their patients due to reliability of diagnosis as deWnitive, probable and suspicious FMF groups. Myalgia frequency was reported to be 9, 7.8 and 2.4% in these subgroups, respectively, the diVerence among the groups being not statistically signiWcant. The rate of myalgia in their group was 6.1% in total, while 20.3% of our patients had myalgia [2–4]. Those patients with protracted febrile myalgia presented to our clinic mostly during the spring months, and their ASO levels were high [2, 3]. Most cases with myalgia were homozygous or compound heterozygous for M694V mutation, while only one case was homozygous for E148Q [2, 5]. One of the patients with myalgia had also amyloidosis and osteopoikilosis and he was transplanted for end stage renal disease [6].

Rhabdomyolysis with different etiologies in childhood

AIM To investigate different etiologies and management of the rhabdomyolysis in children. METHODS Eight 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. RESULTS Average 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). CONCLUSION It is important to distinguish the sporadic and recurrent rhabdomyolysis cases from each other. Recurrent rhabdomyolysis cases should follow up more regardful and attentive.

Risk factors for subclinical inflammation in children with familial mediterranean fever

Familial Mediterranean fever (FMF) is the most common autosomal-recessive inherited inflammatory disease characterized by attacks of painful inflammation. Some patients with FMF have subclinical inflammation persisting between the attacks. Persistent inflammation may lead to amyloidosis and other complications of chronic inflammation.