Clinical practice recommendations for primary hyperoxaluria: an expert consensus statement from ERKNet and OxalEurope.

Primary hyperoxaluria (PH) is an inherited disorder that results from the overproduction of endogenous oxalate, leading to recurrent kidney stones, nephrocalcinosis and eventually kidney failure; the subsequent storage of oxalate can cause life-threatening systemic disease. Diagnosis of PH is often delayed or missed owing to its rarity, variable clinical expression and other diagnostic challenges. Management of patients with PH and kidney failure is also extremely challenging. However, in the past few years, several new developments, including new outcome data from patients with infantile oxalosis, from transplanted patients with type 1 PH (PH1) and from patients with the rarer PH types 2 and 3, have emerged. In addition, two promising therapies based on RNA interference have been introduced. These developments warrant an update of existing guidelines on PH, based on new evidence and on a broad consensus. In response to this need, a consensus development core group, comprising (paediatric) nephrologists, (paediatric) urologists, biochemists and geneticists from OxalEurope and the European Rare Kidney Disease Reference Network (ERKNet), formulated and graded statements relating to the management of PH on the basis of existing evidence. Consensus was reached following review of the recommendations by representatives of OxalEurope, ESPN, ERKNet and ERA, resulting in 48 practical statements relating to the diagnosis and management of PH, including consideration of conventional therapy (conservative therapy, dialysis and transplantation), new therapies and recommendations for patient follow-up.

Monitoring of micturition and bladder volumes can replace routine indwelling urinary catheters in children receiving intravenous opioids: a prospective cohort study.

In this prospective observational study, the incidence, risk factors and the time to event of urinary retention in children receiving intravenous opioids were evaluated. Urinary retention was confirmed by ultrasound following the inability to void for 8 h or earlier in patients experiencing discomfort. In total, 207 opioid episodes were evaluated, of which 199 (96.1%) concerned morphine, in 187 children admitted to the pediatric ward or pediatric intensive care unit. The median age was 7.6 years (IQR 0.9-13.8), and 123 (59.4%) were male. The incidence of urinary retention was 31/207 (15.0%) opioid episodes, in which 14/32 (43.8%) patients received continuous sedation for mechanical ventilation and 17/175 (9.7%) received no sedation. Multivariable logistic regression analysis showed a significant association with continuous sedation (OR 6.8, 95% CI 2.7-17.4, p 0.001) and highest daily fluid intake (OR 0.8 per 10% deviation of normal intake, 95% CI 0.7-0.9, p 0.01). Opioid dosage, age and gender were not significantly associated. Most events (28/31, 90.3%) occurred within 24 h.Conclusion: The incidence of urinary retention in children receiving intravenous opioids is low, indicating that placement of urinary catheters is not routinely necessary in these patients. However, micturition and bladder volumes must be monitored, especially in sedated children and during the first 24 h of opioid administration. What is Known: • Great variation exists in the routine placement of urinary catheters in children receiving IV opioids. What is New: • Confirmed by ultrasound, the incidence of urinary retention in children receiving intravenous opioids in this study was 15%, indicating that placement of urinary catheters is not routinely necessary in these patients. • Children receiving continuous sedation for invasive mechanical ventilation showed a sevenfold greater risk of developing urinary retention than non-sedated patients.

Open and Laparoscopic Colposuspension in Girls with Refractory Urinary Incontinence.

INTRODUCTION: Lower urinary tract symptoms (LUTS) are very common in children. Standard treatments consist of urotherapy, antibiotic prophylaxis, anti-muscarinics, physical therapy, and the treatment of coexisting constipation. A small group of girls also present with stress incontinence or with stress-induced urge incontinence. In cases of persistent LUTS due to congenital bladder neck insufficiency (BNI), surgical treatment might be considered. The aim of this paper is to assess the results of open and laparoscopic colposuspension in children with refractory urinary incontinence (UI). MATERIALS AND METHODS: The results of 18 open and 18 laparoscopic consecutive colposuspensions were analyzed. All patients had UI and failed conservative treatment. BNI was proven by repeated perineal ultrasound and video-urodynamic study. The laparoscopic procedure was performed preperitoneally and the open procedure was via a transverse lower abdominal incision. The same postoperative protocol was used in both groups. RESULTS: The mean operation time was 65 min for the open and 90 min for the lap procedure (p < 0.05). Full success was achieved in 7/18 in the open and in 8/18 in the lap group and partial response was seen in 3/18 and in 5/18, respectively (p = 0.64). No intraoperative complications occurred in this cohort. CONCLUSION: Open and laparoscopic colposuspension can be used to treat refractory UI in children with BNI when non-invasive methods fail.

A comparative study between continent diversion and bladder neck closure versus continent diversion and bladder neck reconstruction in children.

OBJECTIVE: To assess the long-term outcome of continent diversion in children with structural or neurogenic cause of incontinence, with special interest in differences between closed and open bladder neck procedures. PATIENTS AND METHODS: A cohort of 63 children with intractable incontinence treated with continent diversion between January 1998 and January 2008 were reviewed for underlying disease, type of surgery, complications and outcome. RESULTS: Forty patients had a continent diversion with open bladder neck (group 1) and 23 patients had their bladder neck closed (group 2: 11 primarily closed; 12 secondarily closed). There was no difference between the two groups in terms of patient characteristics, surgical re-interventions and stone formation. The continence rate however was significantly better in group 2 (95.6% vs 77.5%). CONCLUSION: Bladder neck closure with continent diversion as primary or salvage procedure in children with intractable incontinence does not result in extra morbidity and has a high success rate. Thorough urodynamic evaluation of bladder function is the key to success in therapy planning for these children, to minimize the need for re-intervention.