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Context: Higher fibroblast growth factor-23 (FGF23) concentrations are associated with heart failure and mortality in diverse populations, but the strengths of associations differ markedly depending up on which assay is used.Objective: We sought to evaluate whether iron deficiency, inflammation, or kidney function account for differences in the strengths of associations between these 2 FGF23 assays with clinical outcomes.Design: Case cohort study from the Cardiovascular Health Study.Setting: A total of 844 community-dwelling individuals aged 65 years or older with and without chronic kidney disease were followed for 10 years.Outcomes: Outcomes included death, incident heart failure (HF), and incident myocardial infarction (MI). Exposure was baseline intact and C-terminal FGF23. Using modified Cox models, adjusting sequentially we tested whether observed associations of each assay with outcomes were attenuated by iron status, inflammation, kidney function, or their combinations.Results: FGF23 measured by either assay was associated with mortality in unadjusted analysis (intact FGF23 hazard ratio [HR] per 2-fold higher 1.45; 95% CI, 1.25-1.68; C-terminal FGF23 HR 1.38; 95% CI, 1.26-1.50). Adjustment for kidney function completely attenuated associations of intact FGF23 with mortality (HR 1.00; 95% CI, 0.85-1.17), but had much less influence on the association of C-terminal FGF23, for which results remained significant after adjustment (HR 1.15; 95% CI, 1.04-1.28). Attenuation was much less with adjustment for iron status or inflammation. Results were similar for the HF end point. Neither C-terminal or intact FGF23 was associated with MI risk.Conclusions: The relationship of FGF23 with clinical end points is markedly different depending on the type of FGF23 assay used. The associations of biologically active FGF23 with clinical end points may be confounded by kidney disease, and thus much weaker than previously thought. [ABSTRACT FROM AUTHOR]

Background: Randomized clinical trials have failed to show benefit from increasing intensity of renal replacement therapy (RRT) for acute kidney injury, but continue to be frequently used. In addition, intensive RRT is associated with an increase in adverse events potentially secondary to small solute losses, such as phosphate. We hypothesized that, compared with less-intensive RRT, intensive RRT would lead to longer duration of mechanical ventilation.Research Question: Does more-intensive renal replacement therapy in critically ill patients with acute kidney injury increase time to extubation from mechanical ventilation when compared with less-intensive therapy?Study Design and Methods: The Acute Renal Failure Trial Network study was a randomized multicenter trial of more-intensive (hemodialysis or sustained low-efficiency dialysis six times per week or continuous venovenous hemodiafiltration at 35 mL/kg per hour) vs less-intensive (hemodialysis or sustained low-efficiency dialysis three times per week or continuous venovenous hemodiafiltration at 20 mL/kg per hour) RRT in critically ill patients with acute kidney injury. Of 1124 patients, 907 who were supported by mechanical ventilation on study initiation were included in this Cox-proportional hazards analysis. The primary outcome was the time to first successful extubation off mechanical ventilation.Results: Patients who were assigned randomly to more-intensive RRT had a 33.3% lower hazard rate of successful extubation (hazard ratio, 0.67; 95% CI, 0.52-0.88; P < .001) when compared with patients who were assigned to less-intensive RRT. Patients who were assigned to more-intensive RRT had, on average, 2.07 ventilator-free days, compared with 3.08 days in those who were assigned to less-intensive RRT (P < .001) over 14 days from start of the study.Interpretation: Critically ill mechanically ventilated patients who were assigned randomly to more-intensive RRT had longer duration of mechanical ventilation compared with those who were assigned to less-intensive RRT. The reasons for this, such as excessive phosphate loss from more-intensive RRT, deserve further study to optimize the safety and effectiveness of CRRT delivery. This was a post hoc analysis of the Acute Renal Failure Trial Network study; clinical trial registration of the original trial is NCT00076219. [ABSTRACT FROM AUTHOR]

Purpose: We assessed the surgical outcome of cloacal malformation (CM) with emphasis on need and timing of vaginal replacement.Methods: An ambispective study of CM was carried out including prospective cases from April 2014 to December 2017 and retrospective cases that came for routine follow-up. Early vaginal replacement was defined as that done at time of bowel pull through. Surgical procedures and associated complications were noted. The current state of urinary continence, faecal continence and renal functions was assessed.Results: 18 patients with CM were studied with median age at presentation of 5 days (1 day-4 years). 18;3;2 babies underwent colostomy; vaginostomy; vesicostomy. All patients underwent posterior sagittal anorectovaginourethroplasty (PSARVUP)/ Pull through at a median age of 13 (4-46) months. Ten patients had long common channel length (> 3 cm). Six patients underwent early vaginal replacement at a median age of 14 (7-25) months with ileum; sigmoid colon; vaginal switch; hemirectum in 2;2;1;1. Three with long common channel who underwent only PSARVUP had inadequate introitus at puberty. Complications included anal mucosal prolapse, urethrovaginal fistula, perineal wound dehiscence, pyometrocolpos, bladder injury and pelvic abscess. Persistent vesicoureteric reflux remained in 8. 5;2 patients had urinary; faecal incontinence. 2 patients of uterus didelphys are having menorrhagia. One patient succumbed to sepsis at 7 months age. Renal functions remained normal in 16. One patient is undergoing dialysis.Conclusion: Early vaginal replacement in CM is feasible. Patients with inadequate introitus may suffer from menorrhagia. A regular follow-up is mandatory. [ABSTRACT FROM AUTHOR]

Introduction: Neck and thoracic trauma in children pose unforeseen challenges requiring variable management strategies. Here, we describe some unusual cases. Patients and Methods: Pediatric cases of unusual neck and thoracic trauma prospectively managed from April 2012 to March 2014 at a Level 1 trauma center were studied for management strategies, outcome, and follow-up. Results: Six children with a median age of 5.5 (range 2-10) years were managed. Mechanism of injury was road traffic accident, fall from height and other accidental injury in 2, 3 and 1 patient respectively. The presentation was respiratory distress and quadriplegia, exposed heart, penetrating injury in neck, dysphagia and dyspnea, and swelling over the chest wall in 1, 1, 1, 2 and 1 cases respectively. Injuries included lung laceration, open chest wall, vascular injury of the neck, tracheoesophageal fistula (2), and chest wall posttraumatic pyomyositis. One patient had a flare of miliary tuberculosis. Immediate management included chest wall repair; neck exploration and repair, esophagostomy, gastroesophageal stapling, and feeding jejunostomy (followed by gastric pull-up 8 months later). Chest tube insertion and total parenteral nutrition was required in one each. 2 and 4 patients required tracheostomy and mechanical ventilation. The patient with gastric pull-up developed a stricture of the esophagogastric anastomosis that was revised at 26-month follow-up. At follow-up of 40-61 months, five patients are well. One patient with penetrating neck injury suffered from blindness due to massive hemorrhage from the vascular injury in the neck and brain ischemia with only peripheral vision recovery. Conclusion: Successful management of neck and chest wall trauma requires timely appropriate decisions with a team effort. [ABSTRACT FROM AUTHOR]

BACKGROUND: Mature oocytes are prerequisite for achieving the process of in vitro fertilization. Human chorionic gonadotropin (hCG) is the standard trigger used for stimulating ovulation but is associated with ovarian hyperstimulation syndrome (OHSS). Gonadotropin-releasing hormone agonist trigger achieves oocyte maturation and lowers the incidence of OHSS, but it has limitations of higher pregnancy loss rate and miscarriage rates. Coadministration of both hormones is found to improve the pregnancy rates and the number of mature oocytes retrieved. We aimed to assess if the dual trigger is better than the conventional hCG in triggering oocyte maturation. METHODOLOGY: The study included 76 female patients aged 24-43 years who were randomly divided into two groups with 38 patients in each arm. The study included patients with antimullerian hormone (AMH) <4 ng/ml, antral follicle counts (AFCs)/ovary <12. The study excluded high responders-AMH >4 ng/ml and AFC/ovary >12 to avoid OHSS risk with hCG trigger. RESULTS: The study showed statistically insignificant differences between dual group versus hCG group in terms of the number of oocytes retrieved (10.0 ± 5.6 vs. 8.7 ± 5.0; P = 0.2816), the number of mature oocytes recovered (8.4 ± 5.0 vs. 7.2 ± 4.0; P = 0.2588), fertilization rate (5.9 ± 4.2 vs. 5.6 ± 3.3; P = 0.7390), and the number of usable embryos on day 3 (4.0 ± 3.0 vs. 4.0 ± 2.4; P = 0.8991). CONCLUSION: The dual trigger is equivalent to hCG in triggering oocyte maturation. [ABSTRACT FROM AUTHOR]

Purpose: To assess the postoperative outcome in children undergoing pyeloplasty, using a single transanastomotic indigenized stent.Methods: 329 pyeloplasties were performed by small miniflank incision (3-5 cm) with lateral position for unilateral cases and prone position for bilateral cases during 1993-2009. The procedure involved decompression of the hydronephrotic sac, single layer anastomosis at the PUJ using 5/6-0 suture (reduction of the pelvis if required). An indigenized # 6, Teflon transanastomotic stent (TAS) with multiple holes (modified recently to a self-retaining loop in pelvis) was placed and taken out through the renal pelvis and the main skin incision. The stent served both as a nephrostomy and a stent. The kidney was not mobilized during the procedure. Postoperative results were assessed for early complications.Results: The mean age of the patients was 3.4 years (1 month-14 years). Bilateral simultaneous pyeloplasties were done in 16 cases with a mean age of 5.6 months (2-12). The stent was removed after 4-5 days in most cases after confirming distal flow on clamping. The post-operative complications included slippage of stent (11), blockage/nondrainage (7), difficult retrieval (4), urine leak (4), urinoma (3) and post-operative infection (1). DTPA scan at 3 months follow-up depicted improved drainage in 271 units, preserved renal function in 58 units and improved renal function more than 5% in 246 units.Conclusion: Pyeloplasty using a single Teflon TAS is helpful not only in achieving effective drainage in children undergoing pyeloplasty, but also avoids the complications usually encountered with the use of nephrostomies, or double J stents. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate early postoperative results in a case controlled study following clinical use of stem cells in extrahepatic biliary atresia (EHBA).Methods: From July 2005 to March 2008, 30 cases of suspected EHBA were divided in two groups in an intervention study. Group A received autologous mononuclear bone marrow stem cells at the time of Kasai or after Kasai. In Group B, only Kasai was performed. Liver function tests on postoperative day 7 were compared. Serum bilirubin, clinical status, hepatic scintigraphy and survival at 6 months and 1 year were compared.Results: Mean age was 136 (74-275) days in Group A and 99.7 (56-172) days in Group B. Preoperative values of serum bilirubin (SB), aspartate aminotransferase (AST), alanine aminotransferase and alkaline phosphatase (ALP) were comparable between the groups though there was significant difference in postoperative SB, AST and ALP (p = 0.014, 0.0041, 0.0005), with and without the use of stem cells. The median post stem cell SB was 6.9 (0.5-11.6) mg/dl in Group A versus 10.1 (5.6-26.3) mg/dl in Group B. Median SB at 6 months follow-up was 0.6 (0.5-5.4) mg/dl in Group A versus 7.6 (0.8-9.2) mg/dl in Group B (p = 0.028). There was a significant difference in episodes of cholangitis at 6 months postoperatively between the two groups (p = 0.024). Hepatic scans done at <3 months; >3 months follow-up showed prompt excretion in 80% (4/5); 85.7% (6/7) in Group A versus 20% (1/5); 50% (1/2) in Group B. Survival at 6; 12 months' follow-up was 45.5%; 27.3% in Group A versus 33.3%; 6.7% in Group B. Median postoperative survival was 181 (139,223) days in Group A versus 123 (65,181) days in Group B.Conclusion: Significant biochemical and scintigraphic improvement was noted following stem cell therapy in biliary atresia, probably attributable to anti-inflammatory action of stem cells. [ABSTRACT FROM AUTHOR]

Objectives: To study the patients with hypospadias for urinary tract anomalies, bladder functions and the anatomical changes in the urethra, bladder and ureters.Patients and Methods: 65 cases of untreated hypospadias (14 anterior, 46 mid penile, 5 posterior penile) presenting between 1 and 14 years age were evaluated with ultrasonography, urodynamic study, uroflowmetry and intraoperative cystourethroscopy.Results: Ultrasound showed abnormalities in 12/65 (18.46%) with mild unilateral hydronephrosis in 2, bilateral hydroureteronephrosis in 2, small dysplastic kidney in 1, single kidney in 1, epididymal cyst in 1 and cystitis in 5. Urodynamic study performed in 60/65 (narrow meatus in 5) showed median maximum voiding pressure 32 (8-75) cmH(2)O. The median average and maximum urinary flow rates were 7 (1-19) and 11 (5-28) ml/s, respectively. Normal capacity with normal bladder pressure was seen in 34 children (56.6%). 13 children (21.6%) had large capacity bladder with normal pressure. Two children showed large capacity bladder with high pressure, three showed a large capacity with low pressure and small capacity with high pressure was seen in eight children (13.3%). Detrusor over activity was seen in 28 (46%) patients, 11 of these had meatal stenosis. Cystourethroscopy done in 60 revealed 34 abnormalities in 18 (30%) patients (single ureteric orifice in 1, dilatation of unilateral ureteric unilateral in 3 and bilateral in 2, ectopic ureteric orifice in 2, trabeculated bladder in 8, bladder diverticula in 2, prominent vertical ridges in posterior urethra in 2, annular posterior urethral valve in 1, posterior urethral diverticulum in 1, enlarged prostatic utriculus in 4, mucosal tag at verumontanum in 1, prominent cristae in 3, hypertrophied verumontanum in 3 and a small verumontanum in 1). With the hypospadias repair, VUR (Deflux) and bladder abnormalities were also treated.Conclusion: Preoperative ultrasonography, urodynamic study and cystourethroscopy detect associated abnormalities in children with hypospadias, albeit asymptomatic, irrespective of location of meatus. [ABSTRACT FROM AUTHOR]

Purpose: The varied management and counseling in disorders of sexual differentiation (DSD) depends a lot on the socioeconomic structure. A follow-up study was designed to evaluate the outcome in terms of patient satisfaction with strong socio-cultural issues.Method: Of the 1,134 DSD patients being followed up in pediatric intersex clinic, 60 adolescents and adults assigned male sex in childhood were called for follow-up. They were interviewed for psychosocial and family adjustments including level of acceptance of gender, social relationships and future expectations.Results: The ages ranged from 15 to 25 years (mean, 19.3 ± 3.7 years). The disorders were male pseudo hermaphrodite (MPH)-43, mixed gonadal dysgenesis (MGD)-3, true hermaphrodite (TH)-7 and congenital adrenal hyperplasia (CAH)-7. Of all patients, 85% (51/60) felt satisfied with their gender assignment; 76.9% (46/60) did not feel comfortable with the opposite sex. Penile erections; ejaculation and masculine voice were present in 53, 44 and 47 patients. Facial hair was normal; sparse and absent in 16, 26 and 18 patients, respectively. Stretched penile length was 2.5-9 cm (median, 5.5 cm) and 16/60 patients were satisfied with their penile length; 28 patients required redo surgeries for scrotum diverticulum (1), proximal penile diverticulum (1), stricture urethra (2), hair in the urethra (3), vaginal pouch dilatation (1), orchiopexy (2), residual chordee correction (3), distal urethroplasty (4), urethral fistula repair (21), mastectomy (6) and testicular prosthesis (4). Family support was available to all 85% (51/60) of the patients who had good family relationships. However, only 15% (9/60) felt that they fitted into society. Peer relationships were considered 'good' by 43/60 and poor by 17/60. Two patients had got married and 44.8% (26/58) patients would consider marriage in future. Most patients (42/60) were worried about the smaller size of the phallus and lack of adequate semen, leading to apprehension before marriage. As much as 15 patients had jobs, 15 attended school, 3 attended colleges and 17 illiterate patients were dependent on their families.Conclusions: Despite moral, social and economic support provided by the parents, children with DSD continue to have apprehensions in social adjustments. [ABSTRACT FROM AUTHOR]

Aim: To perform a definitive procedure in pure esophageal atresia by gastric pull-up in the newborn.Patients/methods: A primary gastric pull-up was performed in six newborns with pure esophageal atresia that presented between 1998 and 2009. The cervical esophagus was mobilized through the neck, the stomach was mobilized through laparotomy, the left gastric artery was ligated, and Pyloromyotomy was done. The stomach was brought into the neck via the trans hiatal route. A single-layer esophageo-gastric anastomosis was done in the neck in all.Results: The mean birth weight was 2.1 kg (range 1.9-2.7) and the age at surgery varied from 3 to 7 days (mean 4.5 days). The mean operative time was 146 min. All six neonates received postoperative elective ventilation for a period of 2-7 days (mean 5.3). Epidural morphine was given for postoperative pain relief. Four received TPN for 5-13 days. Three had minor leaks from the neck wound that healed spontaneously. Mean hospital stay was 14.6 with a range 13-20 days. There was no mortality.Conclusion: It is feasible to perform the gastric pull-up for long gap esophageal atresia in the newborn period, as a definitive procedure with no added risks to life in experienced hands. [ABSTRACT FROM AUTHOR]