Accuracy of Endocrine Tests for Detecting Hypogonadotropic Hypogonadism in Girls.

OBJECTIVE: To assess the accuracy of inhibin B and the gonadotropin releasing hormone agonist test for the diagnosis of hypogonadotropic hypogonadism (HH). STUDY DESIGN: We performed a retrospective analysis of data collected 2009-2014 using a strict clinical protocol. All prepubertal nonunderweight girls, aged 13-17.5 years with Tanner breast stage B1/B2 and low estradiol levels, were tested and re-examined at 6-month intervals (n = 21). Constitutional delay of growth and puberty was defined by spontaneous menarche; HH was identified by association with specific causes of HH or no spontaneous progress of puberty during follow-up. Inhibin B was measured using enzyme-linked immunosorbent assay, and follicle-stimulating hormone and luteinizing hormone (basal and stimulated by triptorelin) were measured using a chemiluminescence immunoassay. RESULTS: The cohort comprised 12 girls with constitutional delay of growth and puberty and 9 girls with HH. The causes of HH included hypopituitarism (n = 3), Prader-Willi syndrome, chromosomal aberration, intellectual disability syndrome with ataxia, and idiopathic causes (n = 2). Each measurement, basal inhibin B <20 pg/mL or stimulated follicle-stimulating hormone (4 hours) <11 IU/L, demonstrated a sensitivity and a specificity of 100% for the detection of HH. Stimulated luteinizing hormone (4 hours) <9 IU/L showed 100% sensitivity but only 83% specificity. CONCLUSIONS: Inhibin B seems to be the ideal measurement for detecting HH in girls. The gonadotropin releasing hormone agonist test is an alternative diagnostic modality, although this approach is more invasive and laborious.

Renal ultrasound volume in children with primary vesicoureteral reflux allows functional assessment.

OBJECTIVE: Renal scintigraphy represents the current diagnostic standard to assess split kidney function. We tested the hypothesis that the relative renal volume assessed by ultrasound provides an equally reliable but less invasive tool for assessment of kidney function as compared to renal scintigraphy in patients with primary vesicoureteral reflux. METHODS: Renal ultrasound and renal scintigraphy were performed in 85 patients (median age 4.5 years, range 0.25-7.7) and repeated in 74 patients after 2-13 months (mean 7) of the primary investigation. Renal size was measured by ultrasound, and relative renal volume was calculated for each kidney by using the formula of a prolate ellipsoid. Renal function was estimated for each side (split renal function) by scintigraphy with (99m)Tc MAG3. RESULTS: The mean difference between relative renal volume measured by ultrasound and split renal function determined by renal scintigraphy was 2.8% (standard deviation ± 4.1%; 95% confidence interval 10.8/-5.2%). There was a statistically significant correlation between relative renal volume estimated by ultrasound and split renal function estimated by renal scintigraphy at first examination (r = 0.98; p < 0.001) and at follow-up (r = 0.91; p < 0.001). CONCLUSION: We conclude that ultrasound measurement of relative renal volume is capable of assessing split renal function in children with primary vesicoureteral reflux and, thus, should be considered instead of the more invasive MAG3 scintigraphy.

Percutaneous revascularization of reoccluded meso-Rex shunts in extrahepatic portal vein obstruction.

Extrahepatic portal vein (PV) obstruction (EHPVO) is a rare disorder in early childhood with unknown incidence and mostly unknown etiology. In children with EHPVO, the hepatopedal flow of the mesenteric venous blood is hindered by an obstruction of the PV resulting in prehepatic portal hypertension. The curative treatment with a meso-Rex shunt connects the superior mesenteric vein to the left PV by a venous autograft. Despite good primary patency, reocclusion of a meso-Rex bypass and its treatment can be challenging. We present 2 patients with EHPVO with subtotal functional occlusion of a meso-Rex shunt treated by percutaneous interventions with short- and mid-term follow-up.

Functional outcome after laparoscopic dismembered pyeloplasty in children.

OBJECTIVE: Laparoscopic or retroperitoneoscopic pyeloplasty for ureteropelvic junction obstruction in children has become a routine procedure. The aim of this study was to evaluate functional outcome for patients who had undergone a laparoscopic dismembered pyeloplasty. PATIENTS AND METHODS: Seventy children underwent a laparoscopic dismembered pyeloplasty. Median follow up was 24 months (1-48). We reviewed differential renal function (DRF) and tracer clearance with diuretic renography before as well as 3 and 12 months after operation. Ultrasound was used to determine the grade of hydronephrosis pre- and postoperatively. RESULTS: Median age at operation was 20 months (1-178). Median operating time was 140 min (95-220). Mean DRF could be preserved with no significant difference (P>0.05). All patients showed a significant improvement in tracer clearance on diuretic renography postoperatively (P<0.0001). Ultrasound examinations postoperatively showed a diminished grade of hydronephrosis without significance (P=0.657). CONCLUSION: In terms of preservation of DRF the laparoscopic approach is as effective as open surgery. Tracer clearance is significantly improving. Operating times for laparoscopic pyeloplasty are competitive. We conclude that laparoscopic pyeloplasty requires extensive experience in laparoscopic pediatric urology but might replace the open surgical procedure as gold standard in the operative treatment of ureteropelvic junction obstruction.