SUMMARY BackgroundBile acid diarrhoea is a common, under-diagnosed cause of chronic watery diarrhoea, responding to specific treatment with bile acid sequestrants. We previously showed patients with bile acid diarrhoea have lower median levels compared with healthy controls, of the ileal hormone fibroblast growth factor 19 (FGF19), which regulates bile acid synthesis.
Widespread antenatal screening has resulted in increased detection of anomalies of the kidneys and urinary tract. The present guidelines update the recommendations published in 2000. Antenatal hydronephrosis (ANH) is transient and resolves by the third trimester in almost one-half cases. The presence of oligohydramnios and additional renal or extrarenal anomalies suggests significant pathology. All patients with ANH should undergo postnatal ultrasonography; the intensity of subsequent evaluation depends on anteroposterior diameter (APD) of the renal pelvis and/or Society for Fetal Urology (SFU) grading. Patients with postnatal APD exceeding 10 mm and/or SFU grade 3-4 should be screened for upper or lower urinary tract obstruction and vesicoureteric reflux. Infants with vesicoureteric reflux should receive antibiotic prophylaxis through the first year of life, and their parents counseled regarding the risk of urinary tract infections. The management of patients with pelviureteric junction or vesicoureteric junction obstruction depends on clinical features and results of sequential ultrasonography and radionuclide renography. Surgery is considered in patients with increasing renal pelvic APD and/or an obstructed renogram with differential renal function <35-40% or its subsequent decline. Further studies are necessary to clarify the role of prenatal intervention, frequency of follow up investigations and indications for surgery in these patients.
Gastric teratoma (GT) comprises less than 1% of all teratomas in children. Though GT in the presence of immature neuroepithelial elements is regarded as malignant, the prognosis is excellent after complete excision of the tumor. Because of its rarity the world literature lacks a large study. Clinical experience with ten cases of GT is presented and discussed. Only one patient was female; the mean age at presentation was 3.2 months. Two cases were immature grade III GT; one of these had infiltrated the left lobe of the liver and the transverse colon while the other had metastasized to the regional lymph nodes and omentum. All the patients underwent complete excision. There were no deaths, and after a mean follow-up period of 4.2 years, all the patients had no recurrence and were healthy. Both the mature and immature types of GT have an excellent prognosis after complete excision of the tumor. Even when the immature type infiltrates surrounding structures, complete excision offers recurrence-free survival without requiring chemo- or radiotherapy.
The functional outcome and factors influencing improvement after pyeloplasty for ureteropelvic junction (UPJ) obstruction are still debated. This retrospective study was aimed at evaluating the factors associated with functional improvement in symptomatic unilateral hydronephrosis (HDN). Patients (n = 68) who underwent successful pyeloplasty for unilateral symptomatic UPJ obstruction without any other associated urological abnormality were included. Preoperative evaluation included a diuretic renogram (DR) to confirm obstruction and assess the split renal function (SRF). A follow-up DR was obtained 3 months and 1, 2, and 5 years postoperatively. An absolute increase in the differential function of the operated kidney by over 5% was considered significant; such kidneys were classified as improved (group A) and the others as unimproved (group B). The difference between the preoperative and 3-month SRF was highly significant (P < 0.001). Significant (P < 0.01) improvement in SRF continued until 1 year after pyeloplasty. Patients who presented with a mass had significantly greater improvement (P < 0.05) than those who presented with other clinical features. In group A, a significantly higher number of patients presented with a mass. It was also evident that patients aged less than 1 year showed significantly greater (P < 0.01) improvement in SRF than older patients. Thus, in unilateral symptomatic HDN with impaired function, patients who present with a mass or those in whom pyeloplasty was performed before 1 year of age showed significantly greater improvement.
In order to determine that apoptosis is responsible for large-scale germ cell elimination, we analyzed cells from cryptorchid testes both in histological sections and among those isolated in vitro. Apoptotic testicular cells during 3 to 7 days were only 8 to 30%, reaching a maximum of 80% by the end of 15 days of cryptorchidism. A similar trend was also observed with the number of dead cells. The process of large-scale germ cell removal in the initial stages was facilitated by the formation of multinucleated giant cells, which stained negative for apoptosis. Increase in oxidative stress and decrease in intratesticular testosterone was also observed. The above findings indicate that large-scale germ cell removal, at least during initial stages of cryptorchidism is not solely as a result of apoptosis. Declined intra testicular testosterone, elevated temperature and high oxidative stress following cryptorchidism probably affect cell viability and trigger a fast pace cell removal through giant cell formation.
The effect of cyclosporine (CsA) on fertility has assumed greater importance with the increasing numbers of pediatric transplantations being performed all over the world. Conflicting reports on the effects of CsA on sex hormones are available. This experimental animal study was designed to examine the effect of CsA on testicular weight, sperm counts, seminiferous tubular diameter (STD), testicular morphology, DNA flowcytometry, sex hormone levels, and fertility in male rats. Those rats who received CsA (20 mg/kg per day) showed significant reductions in testicular weight (P < 0.05), sperm count (P < 0.01), Johnsen score (P < 0.05), STD (P < 0.01), serum testosterone levels (P < 0.05), haploid cell population (P < 0. 001) in the testis, and fertility (P < 0.001) compared to those receiving CsA 10 mg/kg per day and control rats. These findings will have an important bearing for children receiving cyclosporine for long periods to guide the physician in optimally adjusting long-term treatment.
Congenital anterior urethral diverticulum (CAUD) is an uncommon condition in children usually presenting as a fluctuant ventral penile swelling. Retrospective data of nine patients with CAUD were analyzed. Patients presented with penile swelling (n = 7), recurrent urinary tract infection (UTI) (n = 5), and poor urinary stream (n = 2). One patient who had gross pyuria was treated with initial marsupialization of the diverticulum and later underwent a definitive surgical procedure. One had deranged renal function, grade IV vesicourethral reflux (VUR), and UTI and was treated with initial urinary diversion followed later by excision of the diverticulum and urethral reconstruction. Of the seven patients who underwent primary repair of the diverticulum, all except one had a normal urethrogram on follow-up. One patient developed a stricture of the urethra that was treated with dilatations and is presently asymptomatic. In the two patients who had bilateral VUR, one grade III and the other grade IV, preoperatively, the reflux subsided and did not require antireflux surgery. Only one patient is on low-dose urinary antibiotic prophylaxis and presently has grade II reflux. Primary excision and repair is the preferred mode of treatment for CAUD. CAUD producing obstructive uropathy and VUR can be managed conservatively. In the presence of gross pyuria, marsupialization of the diverticulum followed by definitive surgery at a later date is safe and recommended.
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