Epispadias in boys with an intact prepuce

ObjectiveTo present an overview of the clinical presentation and pathological anatomy, and the results of surgical correction of 7 cases of epispadias with intact prepuce; a rare condition that has only occasionally been reported in literature.Patients and methodsA retrospective search was performed in the surgical and diagnoses database between 1991 and 2011. Seven cases of epispadias with intact prepuce were identified. Five presented as a webbed and buried penis, 1 as phimosis and 1 with suspicion for congenital anomaly of the genitalia.ResultsIn 3 of 7 cases, epispadias was suspected or diagnosed at first presentation and could be surgically corrected in the first intervention. In the other 4 cases, epispadias was discovered during surgery, requiring an additional intervention to perform epispadias repair in 3 cases. One boy was diagnosed with glandular, 3 with coronal, 1 with shaft and 2 with penopubic epispadias. Epispadias repair was successful with regard to cosmesis and erectile function. Five patients developed normal continence after surgery, 1 after intensive urotherapy. An under average penile length was the main reported problem during follow-up.ConclusionIn the diagnostic process for a concealed penis, the possibility of epispadias should be considered. If epispadias is suspected or confirmed, epispadias repair can occur in the first intervention, reducing the number of additional interventions. Epispadias with intact prepuce appears to have a better prognosis concerning urinary continence compared to classical epispadias.     

Management of obstructive calcular anuria with acute renal failure in children less than 4 years in age: A protocol for initial urinary drainage in relation to planned definitive stone management

ObjectivesTo describe and evaluate our protocol for management of children ≤4years old with obstructive calcular anuria (OCA) and acute renal failure (ARF) to improve selection of initial urinary drainage (ID) method and to facilitate subsequent definitive stone management (DSM) as studies discussing this special group of patients are still few.Patients and methodsPatients with a contraindication to any method of ID were excluded. Decision (percutaneous nephrostomy (PCN) or double J (JJ) stent) was based on degree of hydronephrosis and planned DSM. We used 4.8–5Fr JJ or 6–8Fr PCN under general anesthesia and fluoroscopic guidance. According to our protocol, JJ is inserted for hydronephrosis ≤ grade 1. When the hydronephrosis is >grade 1, patients with radiolucent stones were treated by JJ whatever the site of the stone. When the stones were radiopaque, PCN was reserved for stones in a solitary functioning kidney and bilateral ureteric stones prepared for subsequent bilateral ureterolithotomy (or stone prepared for ureterolithotomy in a solitary kidney). After normalization of renal functions, DSM was staged attacking only one side before discharge. Both sides were cleared at the same session in cases with bilateral ureterolithotomy. Renal or ureteric stones suitable for SWL in a solitary kidney were treated with percutaneous nephrolithotripsy (PNL) or ureteroscopy. This was followed also in patients with bilateral stones suitable for SWL by clearing one side using ureteroscopy or PNL before discharge. Open surgery (OS) was reserved for cases with failed ureteroscopy or PNL, for ureteric stones >2.5 cm in size or very large volume complex renal stones. Stone free rate (SFR) was evaluated by CT. Our protocol was evaluated as regard recovery of renal functions, complications, and number of interventions to clear stones.ResultsThis study included 62 boys and 22 girls presented with anuria for 1–4 days. JJ and PCN were inserted in 105 and 30 ureterorenal units (URU), respectively. Creatinine returns normal within 72 h. JJ insertion formed a part of DSM in 78/159 (49%) URU (stones prepared for extracorporeal shockwave lithotripsy or oral chemolytic dissolution therapy). PCN was the ideal tract for subsequent PNL in 11/159 (6.9%) URU. Accordingly, ID participated by 55.97% in DSM. Both operative and imaging times were slightly longer with PCN than JJ. There was no statistically significant difference in the insertion success or mean period to return to normal chemistry. Complications of both methods were mild and without any significant difference. Endourologic procedures constituted the majority of our interventions. Open surgical and endoscopic interventions for clearance of stones (including ID, treatment conversion and 2ry procedures) were done once for 25 patients, twice for 43 patients while it was needed three times for 16 patients. Total number of interventions was 149 procedures. SFR was 94%.ConclusionOur protocol ensures adequate ID with minimal complications when using our selection criteria in children ≤4 years in age with OCA and ARF. It also minimizes number of subsequent procedures to clear stones. Complications and success in insertion and drainage were equivalent in PCN and JJ groups.     

Endocrine effects of valproic acid therapy in girls with epilepsy: A prospective study

Background/aimIt is controversial whether the endocrine dysfunction in epilepsy patients is caused by the epilepsy itself, the antiepileptic therapy, or both. We prospectively evaluated the long-term impact of valproic acid monotherapy compared to other anti-epileptic drugs on anthropometric, metabolic, hormonal, and ultrasonographic parameters in girls with epilepsy.MethodsFifty-seven female patients with epilepsy who had started therapy at mean age of 11.5 ± 3.3 years, 42 with valproic acid (mean dose 13.1 ± 7.0 mg/kg/day and 15 with other anti-epileptic agents were followed for a mean of 3.2 years (range 1.0–8.5 years) in our center. Clinical, hormonal and transabdominal pelvic ultrasound data were collected at 3 time points: before and 6–12 months after onset of anti-epileptic drug treatment; and at the last visit while patients were still taking anti-epileptic drugs.ResultsThere were no significant between-group differences regarding changes in height, body mass index standard deviation score, levels of glucose and insulin, or lipid and endocrine profile from first to last visits. Mean thyroid-stimulating hormone level increased significantly between first and last visit only in the valproic acid group (p < 0.001), with no significant difference in free T4 level over time or between groups. The rate of clinical polycystic ovary syndrome for the valproic acid group (11%) was comparable to that reported in healthy controls (5–10%).ConclusionsAdministration of valproic acid had no adverse effect on body weight, metabolic status or endocrine function over an average follow-up of 3.2 years. Valproic acid appears to be safe for use in girls with epilepsy.     

Continuous antibiotic prophylaxis reduces the risk of febrile UTI in children with asymptomatic antenatal hydronephrosis with either ureteral dilation,high-grade vesicoureteral reflux,or ureterovesical junction obstruction

BackgroundThe efficacy and utility of continuous antibiotic prophylaxis (CAP) in children with congenital antenatal hydronephrosis (ANH) is uncertain. The literature has both supportive and contradictory evidence. The growing trend not to place children with ANH on CAP has created varied clinical practice based on anecdotal individual case characteristics. Our goal was to compare individual infant characteristics between those children who were maintained on CAP to those that were not to try to determine predisposing risk factors to febrile.MethodsAll electronic medical records (EMRs) of children referred to our institution for congenital ANH over a period from 2001 to 2011 were examined. We excluded those referred for urinary tract infection (UTI) who had a history of congenital ANH. We also excluded those with incomplete records, or follow-up less than 2 years. Children were divided into two groups: those maintained on CAP (YCAP) and those not maintained on CAP (NCAP). Our primary endpoint was febrile UTI. Follow-up was at least 24 months. Demographic, perinatal and postnatal clinical data were recorded. Statistical analysis was performed using STATA Version 11.1.ResultsOf the 405 children fitting inclusion criteria, 278 (68.6%) children were maintained on CAP and 127 (31.4%) were not on CAP. The incidence of prematurity, oligohydramnios, perinatal respiratory complications, use of perinatal antibiotics, circumcision status, renal anomalies, associated medical diagnoses, and low birth weight did not differ between the two groups. Overall the incidence of febrile UTI during the follow-up period was 22.2%. The incidence of febrile UTI between the YCAP and NCAP groups was significant (YCAP = 7.9% and NCAP 18.7%, p = 0.021). Multivariate logistic regression using CAP as the dichotomous dependent variable revealed that ureteral dilation, high-grade vesicoureteral reflux (VUR), and ureterovesical junction (UVJ) obstruction were independent risk factors for febrile UTI. More specifically, children with ureteral dilation >11 mm NOT maintained on CAP had a 5.54 (OR = 5.54; CI = 3.15–7.42, p = 0.001) fold increased risk of febrile UTI compared to those maintained on CAP.ConclusionsThe presence of ureteral dilation, high grade VUR, and UVJ obstruction were independent risk factors for development of UTI in children with congenital ANH. Therefore CAP may have a significant role in reducing the risk of febrile UTI in children with ANH with those identifiable risk factors, but otherwise seems unnecessary.     

Development of upper tract stones in patients with congenital neurogenic bladder

ObjectivePatients with neurogenic bladder are at increased risk of developing upper tract stones. We hypothesized that patients with lower urinary tract stone disease are at greater risk of developing upper tract stones.MethodsWe performed a 10-year retrospective case–control study of patients with neurogenic bladder to determine the association between bladder and upper tract stones. Independent risk factors for upper tract stones were assessed. Cases and controls were matched 1:1. Univariable analysis was performed by Fisher's exact test and the Mann–Whitney U test. Multivariable logistic regression was performed.Results52 cases and controls were identified. Cases were significantly more likely to be non-ambulatory, have bowel–urinary tract interposition, thoracic level dysraphism, and history of bladder stones. On multivariable analysis, independent predictors of stone formation were male sex (OR 2.82; p = 0.02), dysraphism involving the thoracic spine (OR 3.37; p = 0.014) bowel–urinary tract interposition (OR 2.611; p = 0.038), and a history of bladder stones (OR 3.57; p = 0.015).ConclusionPatients with neurogenic bladder are at increased risk for upper tract stones. The presence of bladder stones may herald the development of upper tract stones. The predictors of stone disease identified should guide prospective studies to better understand the natural history of upper tract stone development in this population.     

Impact of laparoscopy for diagnosis and treatment in patients with disorders of sex development

ObjectiveTo review laparoscopy in patients with disorders of sex development (DSD) in order to clarify its usefulness in diagnosis, devising subsequent therapeutic strategies and managing patients with various conditions.Patients and methodsBetween April 1992 and December 2012, 29 laparoscopic surgeries were performed in 25 DSD patients. Among them, ten were diagnostic laparoscopy including gonadal biopsy, and 19 were therapeutic laparoscopy. Surgical procedures and complications were evaluated.ResultsFor diagnostic laparoscopy, laparoscopic gonadal biopsy was performed in three patients. Inspection, with or without open gonadal biopsy, was performed on four out of seven patients with 46XY DSD or mixed gonadal dysgenesis (MGD). Additional surgery was planned and performed based on diagnostic laparoscopic findings in six out of seven patients. In the three patients with ovotesticular DSD, the gonadal pathology was diagnosed as: testis/ovary in one, testis/ovotestis in one and ovary/ovotestis in one – this was from the laparoscopic inspection and/or gonadal biopsy. However, the final diagnoses were bilateral ovotestis in two patients and ovary/ovotestis in one patient.For therapeutic laparoscopy, surgical procedures were: gonadectomy in 17 patients (bilateral in 13, unilateral in three, partial in two); hysterectomy in two patients; orchiopexy in one; and sigmoid vaginoplasty in one patient (included multiple procedures). There were no severe perioperative complications. In the four patients with a history of diagnostic laparoscopy, no severe intra-abdominal adhesions that would disturb therapeutic laparoscopic surgery were observed.ConclusionWhile diagnostic laparoscopy was helpful in devising a therapeutic surgical strategy in most of the patients with DSD who were suspected as having complex gonadal status or Müllerian duct derivatives, attention must be paid to precisely diagnosing the gonadal status in ovotesticular DSD. On the other hand, therapeutic laparoscopic surgeries were valuable procedures in treating DSD patients, even with a history of previous diagnostic laparoscopy.     

Apnea in the term infant

Whereas apnea of prematurity has been well defined and its pathophysiology extensively studied, apnea in the term infant remains a greater challenge. Unfortunately, clear diagnostic criteria are lacking and pathogenesis and management vary widely. In this review we have arbitrarily organized the discussion chronologically into earlier and later postnatal periods. In the first days of life, presumed apnea may reflect physiologic events such as positional or feeding etiologies, or may be a manifestation of serious pathophysiology, such as a seizure disorder. Beyond the neonatal period, presumed apnea may be characterized as a BRUE event (brief resolved unexplained event; formerly referred to as ALTE: apparent life-threatening event) and most frequently a precipitating event cannot be identified. Medical providers are left with somewhat of a dilemma regarding the need to hospitalize and/or work up such patients.     

Cumulative incidence of outcomes and urologic procedures after augmentation cystoplasty

ObjectiveAugmentation cystoplasty (AC) is a major surgery that can be associated with long-term morbidity. This study aimed to describe the cumulative incidence of outcomes and urologic procedures in a large cohort of children who underwent AC, identify significant sources of morbidity, and to evaluate baseline factors associated with outcomes of interest.MethodsChildren ≤18 years who underwent AC in the Pediatric Health Information System from 1999 to 2010 were included. All follow-up encounters up to June 2012 were included. Cumulative incidences for 15 outcomes and urologic procedures were calculated using non-informative censoring. Sensitivity analyses were performed to determine effect of censoring assumptions and including hospitals without complete datasets. As an exploratory analysis, baseline patient factors were evaluated for associations with outcomes and urologic procedures of interest using multivariable Cox proportional hazards models adjusted for clustering by hospital.Results2831 AC patients were identified. Based on cumulative incidence calculations and sensitivity analyses; the cumulative incidence ranges of outcomes and procedures at 1, 3, 5, and 10 years were calculated. Examples of 10-year cumulative incidence ranges are given for the following outcomes and procedures: bladder rupture (2.9–6.4%), small bowel obstruction (5.2–10.3%), bladder stones (13.3–36.0%), pyelonephritis (16.1–37.1%), cystolithopaxy (13.3–35.1%), and reaugmentation (5.2–13.4%). The development of chronic kidney disease was strongly associated with a diagnosis of lower urinary tract obstruction (HR 13.7; 95% CI 9.4–19.9). Bladder neck surgery and stoma creation at time of AC were associated with an increased hazard of bladder rupture (HR 1.9; 95% CI 1.1–3.3) and bladder stones (HR 1.4; 95% CI 1.1–1.8) respectively.ConclusionsOutcomes of interest and urologic procedures after AC are common. Results from this large cohort can be used to counsel patients and families about expectations after AC. Pyelonephritis, chronic kidney disease, further reconstructive surgery, and calculus disease appear to cause significant morbidity. Collaborative efforts are needed to further reduce morbidity in this patient population.     

Alternating hemiplegia of childhood in Denmark: Clinical manifestations and ATP1A3 mutation status

Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder characterized by early-onset recurrent distinctive hemiplegic episodes commonly accompanied by other paroxysmal features and developmental impairment. De novo mutations in ATP1A3 were recently identified as a genetic cause of AHC. To describe the entire Danish cohort of paediatric AHC patients we approached neuropaediatricians nationwide. All currently acknowledged Danish patients ≤16 years with AHC were genetically tested and seen by the same child neurologist (PU). Ten patients; seven girls and three boys were identified. Mean present age was 10.0 years (range 1–16). Mean age at presentation was 7.4 months (range 1–18 months). Sequencing of ATP1A3 in all ten patients revealed a pathogenic mutation in seven. Two females with moderate psychomotor impairment were heterozygous for the known p.G947R mutation, whereas one severely retarded boy was heterozygous for the common p.E815K mutation. The prevalent p.D801N mutation was identified in two moderate to severely retarded children. Interestingly, in a set of monochorionic male twins a novel p.D801E mutation was identified, underscoring that the asparagine at position 801 is a mutation hotspot. Three girls aged 5–13 years did not reveal any ATP1A3 mutations. They were rather mildly clinically affected and displayed a normal or near-normal psychomotor development. This is the first study of AHC in the Danish paediatric population. The patients harboured a wide range of psychomotor difficulties. Patients with no mutation detected tended to be less severely affected. Prevalence was approximately 1 per 100,000 children.     

Pediatric laparo-endoscopic single site partial nephrectomy: Feasibility in infants and small children for upper urinary tract duplication anomalies

ObjectiveTo assess the feasibility and outcomes of laparo-endoscopic single site (LESS) partial nephrectomy (PN) in infants and small children for upper urinary tract duplication anomalies.Materials and methodsThe medical records of all patients undergoing LESS PN at a single pediatric institution were retrospectively reviewed for patient demographics, perioperative details, and outcomes. A cystoscopy was initially performed to place an externalized catheter into the ureter of the ipsilateral normal renal moiety. An Olympus TriPort, an Olympus Endoeye flexible tip laparoscope, standard 3- or 5-mm instrumentation, and a LigaSure Blunt were utilized.ResultsFour children (two boys, two girls) underwent LESS PN. Three patients underwent upper pole PN and one underwent lower pole PN. All procedures were performed for poorly functioning obstructed renal moieties (one ureterocele, one ureteropelvic junction obstruction and vesicoureteral reflux, and two ectopic ureters). Median age was 6.2 months (range 2.5–16.4 months). Median weight was 7.7 kg (range 6.1–12.6 kg). Median operative time was 126 min (range 97–180 min). No patient received inpatient postoperative narcotics. Median follow-up was 9.9 months (range 6.2–19.1 months). No postoperative complications were noted. Postoperative renal ultrasound demonstrated successful resection in all patients.ConclusionsLESS PN is technically feasible, safe, and effective for upper urinary tract duplication anomalies in infants and small children.     

Single incisional approach for reconstruction of hypospadias and concomitant inguinal hernia

ObjectiveTo demonstrate the feasibility, cosmetic outcome and therapeutic values of our single incisional approach in patients with both hypospadias and inguinal hernia (IH) in comparison with standard multiple incisional techniques.Materials and methodsSixty hypospadias-IH repairs were performed from February 2005 to February 2012. These patients were divided into two groups according to their age and hypospadias location. They were then separated randomly into multiple incision (MIG) and single incision (SIG) groups. Early and late complications were taken into consideration. Postoperative pain, need for analgesics, operative time, hospital stay and cosmetic results were recorded for further evaluation.ResultsPatients were followed up at 6-month intervals for up to 2 years postoperatively. Early and late complication rates were approximately the same in the two groups. 73.3% of patients in MIG and 96.6% in SIG attained an excellent cosmetic result according to two external surgeons. There was no case with poor cosmetic outcome in either group. More analgesic consumption was demanded in MIG patients.ConclusionThis method of surgery is reproducible with better cosmetic outcome and a slightly shorter hospital stay. It could be a viable option in the management of children with hypospadias and concomitant IH. Negligible postoperative pain and short operative time are the other advantages.     

Confirmation of chromosomal microarray as a first-tier clinical diagnostic test for individuals with developmental delay,intellectual disability,autism spectrum disorders and dysmorphic features

Background and objectivesSubmicroscopic chromosomal rearrangements are the most common identifiable causes of intellectual disability and autism spectrum disorders associated with dysmorphic features. Chromosomal microarray (CMA) can detect copy number variants <1 Mb and identifies size and presence of known genes. The aim of this study was to demonstrate the usefulness of CMA, as a first-tier tool in detecting the etiology of unexplained intellectual disability/autism spectrum disorders (ID/ASDs) associated with dysmorphic features in a large cohort of pediatric patients.Patients and methodsWe studied 349 individuals; 223 males, 126 females, aged 5 months-19 years. Blood samples were analyzed with CMA at a resolution ranging from 1 Mb to 40 Kb. The imbalance was confirmed by FISH or qPCR. We considered copy number variants (CNVs) causative if the variant was responsible for a known syndrome, encompassed gene/s of known function, occurred de novo or, if inherited, the parent was variably affected, and/or the involved gene/s had been reported in association with ID/ASDs in dedicated databases.Results91 CNVs were detected in 77 (22.06%) patients: 5 (6.49%) of those presenting with borderline cognitive impairment, 54 (70.13%) with a variable degree of DD/ID, and 18/77 (23.38%) with ID of variable degree and ASDs. 16/77 (20.8%) patients had two different rearrangements. Deletions exceeded duplications (58 versus 33); 45.05% (41/91) of the detected CNVs were de novo, 45.05% (41/91) inherited, and 9.9% (9/91) unknown. The CNVs caused the phenotype in 57/77 (74%) patients; 12/57 (21.05%) had ASDs/ID, and 45/57 (78.95%) had DD/ID.ConclusionsOur study provides further evidence of the high diagnostic yield of CMA for genetic testing in children with unexplained ID/ASDs who had dysmorphic features. We confirm the value of CMA as the first-tier tool in the assessment of those conditions in the pediatric setting.     

Lambert–Eaton myasthenic syndrome in a 13-year-old girl with Xp11.22-p11.23 duplication

Lambert–Eaton myasthenic syndrome (LEMS) is an autoimmune disease of the presynaptic neuromuscular junction, typically occurring in adults as a paraneoplastic syndrome. Only rare cases have been reported in childhood. In most childhood cases, malignancies have not been detected but a propensity to autoimmune disease was noticed. Nevertheless, little is known about genetic factors that may contribute to the susceptibility of an individual to develop LEMS.We report on a 13-year-old girl, known with the Xp11.22-p11.23 duplication syndrome, who presented with severe non-paraneoplastic LEMS. The potential role of this microduplication syndrome in the development of LEMS is explored. Previous literature review of twelve Xp11.2 duplication syndrome patients showed that three of them suffered from various autoimmune diseases. The common duplicated region in those three patients and the presented case comprises 12 disease-associated genes including the FOXP3 (Forkhead Box P3) and WAS (Wiskott–Aldrich syndrome) gene, both implicated in immune function. However, it is unclear whether increased gene dosage of one or both of these genes can cause susceptibility to autoimmune diseases.In conclusion, the presented case emphasizes that autoimmune disease is a recurrent feature of the Xp11.2 duplication syndrome, which should be considered in the follow-up of these patients. The exact mechanism underlying this autoimmune propensity remains to be elucidated.     

Genetic or pharmacologic disruption of the calcineurin-nuclear factor of activated T-cells axis prevents social stress-induced voiding dysfunction in a murine model

ObjectiveSocial stress can suppress the voiding reflex, with resultant diminished voiding frequency and increased volumes. The calcineurin-NFAT (nuclear factor of activated T cells) pathway is important in memory development. It was hypothesized that interruption of the calcineurin-NFAT pathway might prevent social stress-induced voiding dysfunction.MethodsMice were subjected to social stress in an established resident-intruder model for 1 h, followed by 23 h of barrier separation. NFATc3, NFATc4 knockout (KO) and wild-type (WT) mice were studied. At two weeks, voiding patterns were collected; this was followed by sacrifice. Corticotropin-releasing factor (CRF) mRNA expression in Barrington's nucleus (BN) was determined by in-situ hybridization.ResultsSocial stress decreased voiding frequency and increased voided volumes in WT strains. At baseline, NFATc3 KO mice showed decreased voids and increased volumes, while the NFATc4 KO mice resisted social stress. However, CRF mRNA increased in WT mice following social stress and was increased at baseline in NFATc3 KO mice. It was found that CRF mRNA did not increase following social stress in NFATc4 KO mice. The administration of CsA to WT mice normalized voiding patterns following social stress, albeit with no effect on CRF mRNA in BN.ConclusionDisrupting the calcineurin-NFAT axis by either genetic or pharmacologic approaches confers resistance to the development of social stress-induced voiding and dysfunction.     

Validity of US norms for the Bayley Scales of Infant Development-III in Malawian children

ObjectiveMost psychometric tests originate from Europe and North America and have not been validated in other populations. We assessed the validity of United States (US)-based norms for the Bayley Scales of Infant and Toddler Development-III (BSID-III), a neurodevelopmental tool developed for and commonly used in the US, in Malawian children.MethodsWe constructed BSID-III norms for cognitive, fine motor (FM), gross motor (GM), expressive communication (EC) and receptive communication (RC) subtests using 5173 tests scores in 167 healthy Malawian children. Norms were generated using Generalized Additive Models for location, scale and shape, with age modeled continuously. Standard z-scores were used to classify neurodevelopmental delay. Weighted kappa statistics were used to compare the classification of neurological development using US-based and Malawian norms.ResultsFor all subtests, the mean raw scores in Malawian children were higher than the US normative scores at younger ages (approximately <6 months) after which the mean curves crossed and the US normative mean exceeded that of the Malawian sample and the age at which the curves crossed differed by subtest. Weighted kappa statistics for agreement between US and Malawian norms were 0.45 for cognitive, 0.48 for FM, 0.57 for GM, 0.50 for EC, and 0.44 for RC.ConclusionWe demonstrate that population reference curves for the BSID-III differ depending on the origin of the population. Reliance on US norm-based standardized scores resulted in misclassification of the neurological development of Malawian children, with the greatest potential for bias in the measurement of cognitive and language skills.     

Scintigraphy evaluation of the types of functional constipation in children with bowel bladder dysfunction

PurposeTo evaluate the types of constipation according to colonic transit time in chronically constipated children with dysfunctional voiding (bowel bladder dysfunction, BBD group) and to compare the results with transit type in children with chronic functional constipation without urinary symptoms (constipation group) and children with normal bowel habits, but with lower urinary tract symptoms (control group).Patients and methodsOne-hundred and one children were included and their medical histories were obtained. The BBD group kept a voiding diary, and underwent urinalyses and urine culture, ultrasound examination of bladder and kidneys and uroflowmetry with pelvic floor electromyography. Radionuclear transit scintigraphy was performed in all children according to a standardized protocol. Patients were categorized as having either slow-transit (ST), functional fecal retention (FFR) or normal transit.ResultsFFR was diagnosed in 31 out of 38 children with BBD, and 34 out of 43 children in the constipation group. ST was found in seven children with BBD, compared with nine children in the constipation group. The control group children demonstrated normal colonic transit. Urgency, daily urinary incontinence and nocturnal enuresis were noted only in children with FFR. Both children with ST constipation and FFR complained of difficulties during voiding, voiding postponement and urinary tract infections.ConclusionsFFR is the most common form of constipation in children with dysfunctional voiding. However, some children might suffer from ST constipation. Differentiation between these two types of constipation is clinically significant because they require different treatment. Future studies with larger numbers of patients are needed to confirm the noted differences in urological symptoms in these two groups of constipated children..     

Safe use of alteplase in a 10 months old infant with cardio-embolic stroke

The knowledge about safety and efficacy of thrombolysis in paediatric stroke is limited, especially for very young children. We present an infant with cardioembolic stroke treated with alteplase. He had hypoplastic left heart syndrome since birth. He underwent Norwood operation, followed by bidirectional cavopulmonary anastomosis at 3 months. On aspirin therapy he was well until heart failure developed at the age of 9 months with 2 thrombi in the right ventricle. During the course of enoxaparin therapy sudden acute left-sided haemiplegia occurred. The emergency brain CT scan was normal. Informed consent was obtained from parents after explaining the alteplase treatment protocol and possible complications. Alteplase was administered i.v. according to standard adult stroke regimen. A control CT scan obtained 24 h later was negative for intracranial haemorrhage but the hypodense area in insula, internal capsule and subcortical area of the right parietal region were indicative of ischaemic stroke. Anticoagulation therapy was continued. He recovered hand functions after 5 days and full repertoire of movements on his left side 3 weeks later. A neurological examination performed 2 months after indicated mild residual haemiparesis and a modified Rankin scale score of 1. Three months later, the patient died of progressive heart failure. An international multicentre prospective trial is ongoing to investigate the safety and appropriate dose of alteplase for paediatric ages 2–17 years. The aim of this paper is to report safe use of alteplase even in a very young child.     

Child and adolescent obesity

Childhood obesity has reached epidemic proportions worldwide. With adult obesity-related co-morbidities now appearing in obese children, there has been a big push to educate the population and establish effective weight-losing initiatives. However, the limited success of non-operative methods has led to the introduction of bariatric surgery in the under 18-year olds. Laparoscopic Roux-en-Y gastric bypass is the most commonly performed operation, followed by the adjustable gastric band and sleeve gastrectomy. This review seeks to present the current position of bariatric surgery in the treatment of obese adolescents, and reviews the available evidence on selection and outcome, as well as the inherent uncertainties, particularly within the UK.