Neurofibromas of the bladder in a child with neurofibromatosis type 1 causing chronic renal disease.

Neurofibromatosis type 1 (NF1) is an autosomal-dominant inherited disorder and its prominent feature is the neurofibroma and renal involvement includes renal artery stenosis and renal artery aneurysms causing renovascular hypertension. The genitourinary tract may be rarely involved, leading to urinary symptoms of obstruction and hydronephrosis. Herein, we report a 12-year-old boy with chronic renal failure associated with neurofibromas of the bladder, which leads to urinary obstruction.

Pediatric urolithiasis: an 8-year experience of single centre.

OBJECTIVE: The objective was to investigate the clinical features and metabolic and anatomic risk factors for kidney stone formation in our patient group. METHODS: Between 1998 and 2005, 179 children (94 girls, 85 boys) followed in our department because of urolithiasis were enrolled to participate in our study. Clinical presentation, urinary tract infection, stone localisation, positive family history, stone composition, presence of anatomic abnormalities and urinary metabolic risk factors, and treatment modality were evaluated retrospectively. RESULTS: The mean age at diagnosis of stone disease was 4.5 years (range 0.25-15.3 years). The mean follow-up duration was 8 months (range 1-98). The major clinical presentations of our patients were abdominal pain and/or flank pain in 100 children (55.9%) and macroscopic hematuria in 25 (14%). Urinary tract infection was detected in 20% of patients on admission. Forty-three children (24%) had a urinary tract abnormality and ureteropelvic junction obstruction was the most common abnormality. A family history of stone disease was recorded in 98 patients (54.7%). Stones were located within the renal parenchyma in 90 patients. Hypercalciuria and hyperuricosuria were detected in 42.3 and 54.8% respectively. Stone analysis was performed in 63 children and calcium oxalate was a major mineral. Surgical treatment was performed in 49 children and extracorporeal shock wave lithotripsy (ESWL) in 41 children. CONCLUSION: We think that urolithiasis remains a serious problem in children in our country. Family history of urolithiasis, urologic abnormalities (especially under the age of 5 years), metabolic disorders and urinary tract infections tend to indicate childhood urolithiasis.

Effectiveness of lycopene on experimental testicular torsion.

AIM: We aimed to demonstrate the long term effectiveness of lycopene, a precursor of vitamin A, on the testes for ischemia-reperfusion injury. MATERIALS AND METHODS: Seventy male Wistar albino rats were used for this experiment. The rats were divided into seven groups. Group 1 served as the control group; group 2 was sham-operated; group 3 received 20mg/kg/day lycopene (intraperitoneally); in group 4, the right testes of rats were kept torted for 2hours and then were detorted and the animals lived for three days; in group 5, the right testes of rats were kept torted for 2hours and then were detorted and the animals lived for ten days; in group 6, the right testes of the rats were kept torted for 2hours and then detorted and the animals received 20mg/kg/day lycopene (intraperitoneally) for three days; in group 7, the right testes of the rats were kept torted for 2hours and then were detorted and the animals received 20mg/kg/day lycopene (intraperitoneally) for ten days. Lycopene was used intraperitoneally. Some of the testes tissues were used for biochemical analyses and the other tissues were used for histological procedures. The Johnsen's score was used for seminiferous tubule deterioration. The TUNEL method was utilized to show apoptosis of testicular tissue. Testosterone levels were measured from blood samples and SOD, MDA, TNF-α, IL-1ß and IL-6 measurements were recorded from tissue samples. The results were analyzed statistically. RESULTS: In groups 1, 2 and 3 there was normal testicular structure. Rats in groups 4 and 5 had damaged testicular tissues. In groups 6 and 7, in which we used lycopene, the testes were not better than those in groups 4 and 5. The MSTD and JTBS values were better in group 6, but not in group 7 among the torsion groups. As a result, MDA, SOD, TNF-α and IL-1ß were increased and serum testosterone and IL-6 levels were decreased in groups 4 and 5 compared to group 1. There was no improvement in the groups treated with lycopene for therapeutic purposes. CONCLUSION: It was shown that lycopene, as an antioxidant agent, is not effective for testicular torsion in the long term. This study can be considered as a preliminary study showing the need for further researches using different antioxidant agents to determine their long term effects in ischemia-reperfusion injuries in an appropriate experimental design.

Decreased Brain and Placental Perfusion in Omphalopagus Conjoined Twins on Fetal MRI.

The aim of this study is to evaluate perfusional changes in brain and placenta of omphalopagus conjoined twins and to compare them with singleton fetuses by using diffusion weighted imaging and apparent diffusion coefficient. Fetal MRIs of 28-week-old omphalopagus conjoined twins with a shared liver with two separate gallbladders and portal and hepatic venous systems and three singleton fetuses with unilateral borderline ventriculomegaly at the same gestational week as control group were enrolled retrospectively. There was a significant decrease in ADC values of brain regions (p = 0.018) and placenta (p = 0.005) of conjoined twins compared to the control group. The decreased ADC values in placenta and brain regions in conjoined twins might be due to decreased placental perfusion compared to singleton pregnancy. Our results would be a keystone for future studies which will compare larger group of monochorionic multiple pregnancies with singleton pregnancies.

The effectiveness of pneumoreduction for intussusception.

INTRODUCTION: To detect the effectiveness of pneumoreduction (PR) in intussusception, which is one the most common reasons of abdominal pain in infancy and childhood. METHODS: The records of patients treated in our clinic for intussusception between January 2005 and June 2012 were reviewed retrospectively. There were 150 patients aged between 2 months - 12 years of age; 48% (72) were girls, 52% (78) were boys. RESULTS: The most common complaint and clinical findings were abdominal pain (94,6%), vomiting (82.6%), rectal bleeding (81.3%), and discomfort (70.9%). An abdominal mass was observed in 73.3% of patients. PR was successfully performed in 86% of patients. It was carried out once in 86% of these patients and twice in 8.1%. PR was unsuccessful in six patients and they underwent surgery. Manual reduction (31 or 67.4% of operated patients) and resection - anastomosis (15 or 32.6 % of patients) were performed by surgery. Perforation occured in two patients (1.3%) during manual reduction. CONCLUSION: PR is an effective method in the treatment of intussusception with a high success ratio and a low complication ratio. It was possible to perform the procedure especially in patients who came in the early stage of the condition.

A pediatric case of Zenker diverticulum: imaging findings.

Zenker diverticulum is known as an adult disease which occurs as result of outpouching of pharyngeal mucosa through a weak zone in the posterior wall of the pharynx. It is a very rare disorder in childhood, and only a few pediatric cases of Zenker diverticulum have been reported. Here, we report a 10-year-old boy with Zenker diverticulum who presented with aspiration pneumonia, dysphagia, regurgitation, and halitosis. The radiologic evaluation of the patient included chest radiography, ultrasonography, esophagography, and chest computed tomography which clearly demonstrated the pathological findings and confirmed the diagnosis.

Distal vaginal atresia resulting in obstructive uropathy accompanied by acute renal failure.

Children with hydrometrocolpos due to distal vaginal atresia may present with severe obstructive uropathy. Here we report a 27-day-old infant with a hydrometrocolpos causing life-threatening renal failure. Percutaneous drainage of the hydrometrocolpos resulted in dramatically improved clinical and laboratory findings in the patient.

Effects of prenatal flutamide on testicular development, androgen production and fertility in rats.

INTRODUCTION: To investigate the effects of blocking prenatal androgen with the anti-androgen flutamide on testicular weight (TW), seminiferous tubular diameter (STD), testicular biopsy score (TBS), testicular testosterone (TT), and fertility. MATERIALS AND METHODS: Ten pregnant Wistar albino rats were injected with flutamide (100 mg/kg) on gestational days 16 and 19. Rats injected with the solvent were used as controls. Male pups from these dams were evaluated for testicular descent on postnatal day 22. The ability of each male to produce offspring was tested when the animals were aged 130 days. The rats were killed when 180 days old, and the testes were removed. RESULTS: Sixteen of the 24 male rats (66.6%) exhibited cryptorchidism (6 unilateral, 10 bilateral) in the flutamide-treated group. Three of the rats with normally descended testicles were fertile, but none of the cryptorchid rats was fertile in flutamide-treated group. The average TW, mean STD, TBS and TT levels of the flutamide-treated rats was significantly lower than in the solvent-injected rats. However, there was no significant difference in these values between descended and undescended testes in flutamide-treated rats. CONCLUSION: Blocking of prenatal androgen with flutamide interferes with testicular development by inhibiting testicular descent, and also effects testicular morphology and function in both the descended and undescended testes of rats.

Congenital scaphoid megalourethra: report of two cases.

Congenital megalourethra is a rare congenital malformation of the penile urethra. It is defined as the diffuse dilatation of the anterior urethra due to the absence of development of the erectile tissue of the penis. Since the initial description, nearly 80 cases with megalourethra have been reported in English literature. Congenital megalourethra has been classified into scaphoid and fusiform types and is usually associated with additional urinary tract and other system anomalies, irrespective of its type and severity. We report two rare cases of scaphoid megalourethra without any associated anomaly. A 13-year-old boy and an 8-month-old boy were admitted to the Department of Pediatric Surgery with complaints of penile swelling and dysuria since birth. Physical examination and retrograde cystourethrogram confirmed the diagnosis of congenital scaphoid megalourethra. Both patients underwent a reduction urethroplasty. They did well postoperatively and voided with a normal stream without any abnormality of the penile shaft.

Serum inhibin B levels reflect contralateral testicular damage following unilateral testicular trauma.

INTRODUCTION: The aim of this study was to evaluate contralateral testicular damage (CTD) following unilateral blunt testicular trauma (BTT) and testicular capsule laceration (TCL) by the serum inhibin B level which is an accepted marker of spermatogenesis. METHODS: Fifty peripubertal male Wistar albino rats were divided into 5 groups each containing 10 rats. Group 1 was the control group. Group 2 was the BTT group in which the right testicle was placed on a firm surface and a metal rod weighing 215 g was dropped onto the testicle from a height of 5.5 cm. Group 3 was the TCL group in which right testicular tunica albuginea was lacerated using the needle of 4/0 silk suture. Group 4 had right orchiectomy initially. Group 5 was the sham group. In all groups, 3-ml blood samples were taken and bilateral orchiectomies were performed 6 weeks after initial manipulations. RESULTS: Groups 2 and 3 had decreased inhibin B levels (p < 0.001), although the orchiectomy group had normal levels. Histological analyses showed lower Johnsen scores for both trauma groups in the ipsilateral and contralateral testes (p < 0.05). CONCLUSIONS: Serum inhibin B levels decrease following unilateral testicular trauma reflecting CTD.