Demet Alaygut

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.

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.

A rare cause of neonatal hypertension: congenital mesoblastic nephroma

Soyaltın E, Alaygut D, Alparslan C, Özdemir T, Arslansoyu-Çamlar S, Mutlubaş F, Kasap-Demir B, Yavaşcan Ö. A rare cause of neonatal hypertension: Congenital mesoblastic nephroma. Turk J Pediatr 2018; 60: 198-200. A rare cause of neonatal hypertension: Congenital Mesoblastic Nephroma (CMN) is a rare renal tumor in childhood and has been reported with palpable abdominal mass, hypertension, hematuria, polyuria and hypercalcemia. Histopathologically it has been classified into two histological types: classic and cellular. We present a 32-week gestation infant and his histopathology reports of cellular CMN presented with refractory hypertension.

A novel homozygous w99g mutation in cldn-16 gene causing familial hypomagnesemic hypercalciuric nephrocalcinosis in turkish siblings

Alparslan C, Öncel EP, Akbay S, Alaygut D, Mutlubaş F, Tatlı M, Konrad M, Yavaşcan Ö, Kasap-Demir B. A novel homozygous W99G mutation in CLDN-16 gene causing familial hypomagnesemic hypercalciuric nephrocalcinosis in Turkish siblings. Turk J Pediatr 2018; 60: 76-80. Familial hypomagnesemic hypercalciuric nephrocalcinosis (FHHNC) (OMIM: 248250) is characterized by hypomagnesemia, hypercalciuria and nephrocalcinosis. FHHNC inevitably progresses to end-stage renal disease in decades. Mutations in CLDN-16 and CLDN-19 genes are associated with disrupted magnesium handling in the thick ascending limp of Henle`s loop. Patients with mutations in these genes share similar clinical features, and those with CLDN-19 gene mutations have ocular findings in addition. A 2-month-old boy, was admitted to our clinic with the complaints of upper respiratory tract infection. He was the first-born child of consanguineous parents. Laboratory findings revealed hypocalcemia and hypomagnesemia. Bilateral medullary nephrocalcinosis was detected on abdominal ultrasound. His ophthalmologic examination was unremarkable. With hypomagnesemia, hypercalciuria and nephrocalcinosis, the patient was considered to have FHHNC. Oral magnessium supplementation was initiated. Four years of follow-up has been completed uneventfully. When 6-days-old the brother of the case above was admitted with seizure. The patient was resistant to calcium and anticonvulsant drugs and the seizure activity could only be controlled after magnesium infusion. Biochemistry profile revealed hypocalcemia and hypomagnesemia. Urinary calcium extraction was 11 mg/kg/day. Medullary nephrocalcinosis was reported on renal ultrasound. His eye examination, echocardiography, transfontanel ultrasound and electroencephalography were normal. Due to the triad of hypomagnesemia, hypercalciuria and nephrocalcinosis, and the medical history of his elder brother, he was diagnosed with FHHNC. After correction of the electrolyte abnormalities, he was discharged from hospital and is currently being followed-up without any problem. In this manuscript, we shared our experience about a novel homozygous mutation (W99C) in CLDN-16 gene causing FHHNC in a couple of Turkish siblings.

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.

Can a hand radiograph indicate a special diagnosis in a child with chronic kidney disease? Questions

A 5-year-old girl was admitted to the emergency department with diffuse edema on the eyelids and lower extremities and decreased urinary output. On physical examination, bibasilar crackles and distended abdomen with mild ascites were evident. Her height (100 cm) was below the 3rd percentile, weight (16 kg) was between the 10th and 25th percentiles, and blood pressure was 125/80 mmHg (>95p/95p). Her medical history was unremarkable, except for night blindness, which developed in the early childhood. There was no history of kidney disease in her family and her parents were nonconsanguineous. Laboratory tests at admission revealed a normal complete blood count. Dipstick urinalysis revealed a pH of 6, specific gravity of 1,015, 3+ red blood cells (+3), and trace proteinuria. Other laboratory findings were as follows: blood pH 7.30, pCO2 30mmHg, HCO3 19.8 mmol/L, urea 164 mg/dL, serum creatinine 6.3 mg/dL, albumin 4.1 g/dL, sodium 131 mmol/L, potassium 3.6 mmol/L, uric acid 8.3 mg/dL, magnesium 1.3 mg/dL, calcium 8.1 mg/dL, phosphorus 5.8 mg/dL, alkaline phosphatase 111 U/L, and parathyroid hormone 228.5 pg/ mL (normal 15–65 pg/mL). Tubular reabsorption of phosphate was 63.41% (normal >85%), fractional sodium excretion was 4.1% (normal <1%), and fractional potassium excretion was 22.8% (normal <15%). Urinary protein/creatinine ratio was 2.2. The GFR calculated using the Schwartz formula was 8.59mL/min/1.73 m, and creatinine clearance calculated upon 24-h collected urine was 14.6 mL/min/1.73 m. The ultrasound showed bilateral small kidneys (length, left kidney

Çocuklarda idrar yolu enfeksiyonu sonrası gelişen renal skar ile BCG skarı boyutu arasında bir ilişki var mıdır

J Clin Exp Invest www.jceionline.org Vol 4, No 1, March 2013 1 Dokuz Eylül University, Faculty of Medicine, Department of Pediatrics, Divison of Nephrology, Izmir, Turkey 2 Dokuz Eylül University, Faculty of Medicine, and Department of Nuclear Medicine, Izmir, Turkey Correspondence: Demet Alaygut, Dokuz Eylül University Faculty of Medicine Department of Pediatrics, İzmir, Turkey Email: demetalaygut@yahoo.com Received: 21.11.2012, Accepted: 21.02.2013 Copyright

Two Adolescent Girls With Keratinizing Squamous Metaplasia of the Bladder

Squamous metaplasia is a proliferative lesion characterized by the replacement of the transitional epithelium with stratified squamous cells. In the urinary system, it is mostly seen in the bladder. It can be nonkeratinized or keratinized. We report the cases of 2 adolescent girls with keratinizing metaplasia, 1 of whom presented with difficulty with indwelling catheterization and 1 with final terminal hematuria. The predisposing factors were recurrent urinary tract infection and additional catheterization in 1 of the patients. The diagnosis was confirmed by histologic examination in both patients. We report on these cases to draw attention to this rare entity in children.

Asymptomatic proteinuria in a child with recurrent urinary tract infections—questions

An 11-year-old girl with recurrent lower urinary tract infections was referred for asymptomatic proteinuria. She was reported to have been given antibacterial prophylaxis with trimethoprim–sulfamethoxazole for 1 year, which had been discontinued 6 months prior to presentation. The physical examination was completely normal. Her parents were first-degree relatives, and her 22-year-old brother had end stage renal disease. The results of previous imaging investigations, including renal ultrasonography, voiding cystourethrography, and Tc-dimercaptosuccinic acid (DMSA) scintigraphy, were normal. Urinalysis showed 3+ proteinuria and normal sediment. Urine culture was sterile. Daily protein excretion was in the non-nephrotic range (25 mg/m/h). Laboratory test results, including a complete blood count, creatinine, albumin, liver enzymes, and complement levels, were all normal. Given the family history, renal biopsy was