Feminizing Adrenocortical Tumors as a Rare Etiology of Isosexual/Contrasexual Pseudopuberty

Objective: Estrogen-secreting adrenocortical tumors (ACTs) are quite rare with feminizing adrenocortical tumors (FATs) accounting for 0.37-2% of all ACTs. The aim was to evaluate clinical and hormonal characteristics of FATS as well as treatment options and follow-up in the pediatric age group. Methods: Medical records of children with ACTs presenting to a single center in the last two decades were reviewed. Literature review within Pubmed revealed 34 pediatric patients (22 boys) with FAT among 192 articles. Results: Among the 25 children presenting with ACTs in the last two decades, two new pediatric cases of FAT were identified, one benign and the other malignant, in two genders with different clinical presentations. Literature review showed that FATs are extremely rare tumors that are most commonly seen in men and boys presenting with gynecomastia. FATs are more common in children ≤8 years of age, with a median age at diagnosis of six years. While boys present with contrasexual pseudopuberty signs, girls present with isosexual pseudopuberty. A high estrogen level strongly supports diagnosis, while elevations in other adrenal hormones may be seen. FATs are usually malignant in adults and prognosis is generally very poor. However, in children approximately half are benign although assessment of malignant potential depends on clinical behavior of the tumor. FATs are very unpredictable so even after surgery long-term follow-up is required. FATs presenting in childhood may have a better prognosis than adult presentation tumors as most FATs in children are followed without recurrence of tumor. Conclusion: FATs are more common in children ≤8 years of age, with a median age at diagnosis of six years. FATs in childhood may have a better prognosis than in adult males.

The Early Histological Effects of Intravesical Instillation of Platelet-Rich Plasma in Cystitis Models.

PURPOSE: To evaluate the early histological effects of the intravesical instillation of platelet-rich plasma (PRP) in rabbit models of interstitial and hemorrhagic cystitis. METHODS: Thirty-six rabbits were classified into 6 groups: saline (S), S+PRP, hydrochloric acid (HCl), HCl+PRP, cyclophosphamide (CyP), and CyP+PRP. At 48 hours after induction, PRP was prepared and intravesically administered to the S+PRP, HCl+PRP, and CyP+PRP groups. Bladder sections were stained with toluidine blue for mast cell counting and with hematoxylin and eosin for histopathology and mitotic index determination. The proliferation index was determined by proliferating cell nuclear antigen (PCNA) immunolabeling. The nonparametric Mann-Whitney U-test was used for statistical analysis. RESULTS: No abnormalities were observed in the S group, whereas increased interstitial edema and increased average mitotic and proliferation indices were observed in the S+PRP group (P=0.023, P=0.004, and P=0.009, respectively). Intense epithelial loss, hemorrhage, and leukocyte infiltration were detected in the HCl and HCl+PRP groups, whereas a significantly increased average mitotic index was observed in the HCl+PRP group (P=0.002). When compared with its CyP counterpart, a significant reduction in hemorrhage and an increase in leukocyte infiltration and mitotic index were observed in the CyP+PRP group (P=0.006, P=0.038, and P=0.002, respectively). In addition, PCNA staining revealed a significantly increased proliferation index in the HCl+PRP and CyP+PRP groups (P=0.032 and P=0.015, respectively). CONCLUSIONS: The intravesical instillation of PRP increased the mitotic index in the saline and cyclophosphamide groups while decreasing macroscopic bleeding.

Ureteritis cystica: A rare entity in children.

Ureteritis cystica (UC) is a benign condition. Although it can often be diagnosed with imaging techniques, we report a case of a child for whom we planned nephrectomy and ureteral augmentation cystoplasty, but abandoned the cystoplasty due to extensive UC in the ureter.

Association of urokinase gene 3'-UTR T/C polymorphism with calcium oxalate urolithiasis in children.

INTRODUCTION AND OBJECTIVES: Urokinase is synthesized by various cells such as kidney, pneumocytes, and phagocytes. It cleaves plasminogen to plasmin and hence stimulates fibrinolysis. Urokinase breaks down the matrix protein within the stone and thus prevents stone formation and growth. Urokinase concentrations are lower and urokinase gene 3'-UTR T/C polymorphism is higher in patients with recurrent stones. Our aim was to investigate the role of urokinase gene 3'-UTR T/C polymorphism in childhood recurrent stone disease. MATERIAL AND METHODS: A control group of 40 healthy children having no history of stone formation (group 1) (mean age 10.5+/-4.2 years), 40 children (mean age 10.5+/-4.33 years) who had calcium oxalate stones for the first time (group 2), and 40 patients (mean age 11.2+/-3.8 years) with recurrent calcium oxalate stone disease (group 3) were included in the study. The groups were compared with respect to age, gender and urokinase gene 3'-UTR T/C polymorphism. Polymerase chain reaction-based restriction analysis was used to identify C/T polymorphism of the urokinase gene. RESULTS: No significant difference was observed between the three groups with respect to age and gender, while urokinase gene 3'-UTR T/C gene polymorphism was observed in four patients (10%) from group 3. In groups 1 and 2 there was no patient with T/C polymorphism. CONCLUSIONS: Urokinase 3'-UTR T/C gene polymorphism seems to appear more commonly in children with recurrent calcium oxalate stone disease than in healthy children and in those with stones for the first time. These results suggest that the urokinase gene might play a role in childhood recurrent calcium oxalate stone disease.

Management of pediatric stone disease.

Pediatric and adult stone disease differs in both presentation and treatment. Children can present with a wide range of symptoms varying from flank pain and hematuria to nonspecific symptoms such as irritability and nausea. Although ultrasonography and plain radiographs can play a role in diagnosis and follow-up, the standard of care for a child who presents to the emergency department with a history suggestive of a stone is noncontrast spiral CT. Because there is a high yield in identifying predisposing factors in children with urolithiasis and high recurrence rates, metabolic evaluation of every child with a urinary stone should be undertaken and medical treatment should be given if necessary. With recent advances in technology, stone management has changed from an open surgical approach to less invasive procedures such as extracorporeal shock-wave lithotripsy and endoscopic techniques. Herein, we present a review of the recent literature and offer our own preferences to approaches for treatment.

Antibiotic prophylaxis in percutaneous nephrolithotomy: prospective study in 81 patients.

PURPOSE: To compare single-dose and short-course antibiotic prophylaxis protocols in percutaneous nephrolithotomy. PATIENTS AND METHODS: Eighty-one patients with sterile urine preoperatively who underwent percutaneous nephrolithotomy were divided into two groups. The first group (N = 43) received a single intravenous dose of antibiotic (200 mg of ofloxacin) during anesthetic induction, and the second group (N = 38) received treatment doses of antibiotic (400 mg of ofloxacin per day) until the nephrostomy catheter was removed. The two groups were identical according to demographic and treatment characteristics. For each patient, microbiologic evaluation of extracted stones and urine samples was done. If patients developed fever in the postoperative period, blood and urine cultures were taken. Factors that might have affected the development of postoperative fever and infection were analyzed. RESULTS: Nine patients in the first group had postoperative fever. Three had bacteriuria, and one had bacteremia. In the second group, eight patients had fever. One had bacteriuria and bacteremia. Nineteen patients (eleven in the first group, eight in the second) had positive stone cultures. No statistical difference was observed between the two groups in terms of bacteriuria, bacteremia, positive stone cultures, or postoperative fever. The febrile patients had longer operations with the use of more irrigation fluid and longer postoperative hospital stays. CONCLUSIONS: In patients whose preoperative urine cultures are sterile, short-term prophylaxis has no advantage over single-dose prophylaxis as a means of preventing infection. The duration of surgery and the amount of irrigation fluid are significant risk factors for postoperative fever.