The authors present a boy with a 46, XX/46, XY karyotype in whom true hermaphroditism was diagnosed, stressing the importance of establishing the cause of the condition and further management prior to discharging the neonate. The ultimate sex must allow the patient to best fulfill his/her psychosocial role in society.
Interstitial cells of Cajal (ICCs) play an important role in the regulation of gut motility as they are responsible for the slow wave activity of smooth muscle. There is strong evidence that several subpopulations of ICC are present in the wall of the urinary tract. This review presents the currently available literature on the localization and proposed functions of interstitial cells of Cajal (ICC) in the urinary tract.
Varicocele is caused by enlargement of internal testicular veins, including the pampiniform veno- us plexus. It is assumed that it occurs in 8% to 16% of the male population and predisposes to male infertility. The aim of our study was to assess laparoscopic treatment of the varicocele in children using the Palomo method.In the years 2002-2012, our clinic treated 114 boys with the varicocele on the left side. In all patients the procedure involved ligation of veins, the testicular artery and lymphatic vessels with the Roeder knot. The average time of the procedure was 29 min.Varicocele recurrence was found in 2 out of 94 patients reporting for the follow-up visit, and this corresponds to 2.1% of the studied group. The postoperative hydrocele occurred in 20 patients, of which 5 (5.3% of the studied group) required surgical treatment with the Winkelmann technique, while in 15 (15.9% of the studied group) disappeared spontaneously during the average follow-up period of 1 year. No other intra- or postoperative complications occurred in any patient. Also no case of testicular atrophy was diagnosed.The results obtained from our laparoscopy patients using the Palomo method in treatment of varicocele can be considered satisfactory.
A 5-year-old girl presented typical features of isosexual precocity with breast and pubic hair development (Tanner stage 3) and menarche, following a few months history of hirsutism of the back and thighs. Stimulation testing revealed GnRH-independent precocious puberty, secondary hypothyroidism, hyperprolactinemia and mild testosteronemia. The ovaries in ultrasound examination were prepubertal. Tumor markers beta-HCG and AFP were markedly elevated and a suprasellar germ cell tumor 25x15 mm in size was visualized by MRI. Combined chemotherapy followed by radiotherapy resulted in normalization of pubertal features along with estrogen and marker levels. These data indicate a possible development of precocious puberty in girls induced by choriogonadotropin (hCG) released by a supracellar germ cell tumor. Based on our observations and the literature on the subject the authors believe that the autonomous presence of aromatase inside the tumor may be responsible for precocious puberty in girls with hCG-releasing germ cell tumors, regardless of their location. In such cases the rare character of isosexual precocious puberty in girls may result from a unique combination of high hCG-releasing activity and high aromatase activity. In girls with precocious puberty, when discrete androgenization signs develop, the authors postulate to extend the diagnostic management so that hCG-dependent precocious puberty could be ruled out.
A 5 year-old girl presented with typical features of isosexual precocity with breast and pubic hair development (Tanner stage 3) and menarche, following a few months history of hirsutism of the back and thighs. Stimulation testing revealed GnRH-independent precocious puberty, tertiary hypothyroidism, hyperprolactinemia and mild testosteronemia. The ovaries in ultrasound examination were prepubertal. Tumor markers beta-hCG and AFP were markedly elevated and a 2.5 x 1.5 cm suprasellar germ cell tumor (GCT) was visualized by MRI. Combined chemotherapy followed by radiotherapy resulted in normalization of pubertal features along with estrogen and marker levels. Our observations support the possibility of hCG-dependent precocious puberty (PP) in girls caused by suprasellar hCG-secreting tumor. We emphasize the need of diagnostic management of hCG-dependent PP not only in boys, but also in girls, especially when they present even slight features of androgenization. We hypothesize that the rarity of isosexual PP in girls with hCG-secreting suprasellar GCT results not only from the lower occurrence of these tumors in girls than in boys, but above all from a rare simultaneous concomitant incidence of both high tumor aromatase activity and hCG secreting potency.
At least 25% of the clinical problems seen in pediatric urology are the result of neurologic lesions that affect lower urinary tract function. The advent of clean intermittent catheterization and refinements in techniques of urodynamic studies in children dramatically changed the way this pediatric population was traditionally managed. Along with this change came a greater understanding of the pathophysiology of the many diseases that primarily affect children. The applicability of urodynamic testing has expanded to the point where most pediatric urologic centers now believe that functional assessment of the lower urinary tract is an essential element in the evaluation process and is as important as x-ray visualization in characterising and managing these abnormal conditions. The natural outcome of early functional investigation has been the advocacy of proactive or early aggressive management of children who are now considered at risk of urinary tract deterioration based on specific hostile urodynamic parameters. This paper first defines the testing process as it applies to children and second, conservative and surgery methods to prevent upper urinary tract deterioration and to correct continence.
