Combined serum creatinine and cystatin C Schwartz formula predicts kidney function better than the combined CKD-EPI formula in children.

BACKGROUND: The combined serum creatinine (SCreat) and cystatin C (CysC) CKD-EPI formula constitutes a new advance for glomerular filtration rate (GFR) estimation in adults. Using inulin clearances (iGFRs), the revised SCreat and the combined Schwartz formulas, this study aims to evaluate the applicability of the combined CKD-EPI formula in children. METHOD: 201 iGFRs for 201 children were analyzed and divided by chronic kidney disease (CKD) stages (iGFRs ≥90 ml/min/1.73 m(2), 90 > iGFRs > 60, and iGFRs ≤59), and by age groups (<10, 10-15, and >15 years). Medians with 95% confidence intervals of bias, precision, and accuracies within 30% of the iGFRs, for all three formulas, were compared using the Wilcoxon signed-rank test. RESULTS: For the entire cohort and for all CKD and age groups, medians of bias for the CKD-EPI formula were significantly higher (p < 0.001) and precision was significantly lower than the solely SCreat and the combined SCreat and CysC Schwartz formulas. We also found that using the CKD-EPI formula, bias decreased and accuracy increased while the child age group increased, with a better formula performance above 15 years of age. However, the CKD-EPI formula accuracy is 58% compared to 93 and 92% for the SCreat and combined Schwartz formulas in this adolescent group. CONCLUSIONS: The performance of the combined CKD-EPI formula improves in adolescence compared with younger ages. Nevertheless, the CKD-EPI formula performs more poorly than the SCreat and the combined Schwartz formula in pediatric population.

Preoperative assessment of neurovesical function in children with anorectal malformation: association with vertebral and spinal malformations.

PURPOSE: We preoperatively assessed neurovesical function and spinal cord function in children with anorectal malformations. In cases of neurovesical dysfunction we looked for an association with vertebral malformation or myelodysplasia. MATERIALS AND METHODS: We prospectively evaluated 80 children with anorectal malformations via preoperative urodynamics and magnetic resonance imaging of the spine. Bladder compliance and volume, detrusor activity and vesicosphincteric synergy during voiding allowed urodynamic evaluation. Results were reported according to Wingspread and Krickenbeck classifications of anorectal malformations. RESULTS: Urodynamic findings were pathological in 14 children (18%). Pathological evaluations did not seem related to type of fistula or level of anorectal malformation. Vertebral anomalies were seen in 34 patients (43%) and myelodysplasia in 16 (20%). Neither vertebral anomaly nor myelodysplasia seemed associated with type of fistula or severity of anorectal malformation. Of 14 children with pathological urodynamics no vertebral anomaly or myelodysplasia was found in 7. Of 66 children with normal urodynamics 40 presented with vertebral or spinal malformation. CONCLUSIONS: Lower urinary tract dysfunction is common in patients with anorectal malformations. Normal spine or spinal cord does not exclude neurovesical dysfunction. Myelodysplasia or vertebral anomaly does not determine lower urinary tract dysfunction. Thus, we recommend preoperative urodynamic assessment of the bladder and magnetic resonance imaging of the spine in children with anorectal malformations.

[Pediatrics]. / Pédiatrie.

This article summarizes the medical progress achieved in 2 frequent and 2 rare pathologies: 1. Cryptorchidism should be operated around 12 months of age and hormonal treatment abandoned in order to maintain fertility and avoid development of testicular tumors. 2. For the treatment of streptococcal pharyngitis oral cephalosporins for 4 to 5 days are equivalent to a Penicillin treatment of 10 days. 3. Thanks to carvedilol (a beta-blocker agent), levosimendan (a calcium sensibiliser) and nesiritide (an analog to the natriuretic peptide) a new hormonal approach to cardiac failure is possible. 4. Corticosteroids allow to improve quality of live and life expectancy in Duchenne muscular dystrophy, provided treatment starts early and a multidisciplinary approach is assured.

[Antenatally detected hydronephrosis: practical approach for the pediatrician]. / Dilatation pyélocalicielle chez le nouveau-né: attitude pratique pour le pédiatre.

Approximately 1% of the fetuses present some dilatation of their urinary tract in utero. More than 50% of these antenatally detected hydronephrosis will disappear spontaneously after birth. The other 50% comprises ureteropelvic junction obstruction, vesico-ureteral reflux and primary megaureters. Postnatal radiological evaluation (renal ultrasonography and VCUG) is performed in every infant with a significantly dilated renal pelvis (> 8 mm between 20 and 30 weeks or > 10 mm after 30 weeks in utero). Renal nuclear scan should be done in every child with significant/worsening post-natal hydronephrosis. Antibioprophylaxis will be started from birth to prevent urinary tract infection. Medical or surgical approach will be chosen in the light of the uroradiological exam results and the clinical progress.

Plasticity of the enteric nervous system in patients with intestinal neuronal dysplasia associated with Hirschsprung's disease: a report of three patients.

Intestinal neuronal dysplasia is a controversial form of dysganglionosis that has been described both as an isolated disorder and in association with Hirschsprung's disease. It has been blamed for the bad outcome of bowel function in patients operated on for Hirschsprung's disease. According to various authors, intestinal neuronal dysplasia could be a primary disorder or secondary to other diseases of the bowel. The aim of this study was to assess the plasticity of the enteric nervous system in patients with Hirschsprung's disease-associated intestinal neuronal dysplasia and its ability to evolve spontaneously to normal innervation patterns. Since we prospectively introduced the assessment of the enteric nervous system of the ganglionated bowel in patients operated on for Hirschsprung's disease, 31 patients have been operated on for Hirschsprung's disease in our institution between 1995 and 2002. Among these patients, nine suffered postoperatively from severe constipation and five from bouts of entocolitis. IND was found in eight of them. We studied the evolution of the innervation in three of these patients by repeated serial full-thickness biopsies of the colon. All three patients underwent a colostomy before or after the pull-through procedure. Histopathological assessment of the enteric nervous system was made with conventional acetylcholinesterase histochemistry, rapid acetylcholinesterase histochemistry and immunohistochemistry for the Protein Gene Product 9.5 and the antigen CD56. This evolution was compared with the clinical outcome of bowel function. In our three patients with Hirschsprung's disease-associated intestinal neuronal dysplasia, this form of dysganglionosis evolved to normal innervation patterns within a period ranging from 9 to 18 months. This evolution was accompanied by an improvement of bowel function in all. We conclude that Hirschsprung's disease-associated intestinal neuronal dysplasia can evolve to a normal innervation, at least under certain circumstances such as a colostomy. Specific histopathological techniques are required to assess accurately the enteric nervous system.

Interstitial cells of Cajal are normally distributed in both ganglionated and aganglionic bowel in Hirschsprung's disease.

Surgery for Hirschsprung's disease is often complicated by post-operative bowel motility disorders. The impact of intestinal neural histology on the surgical outcome has been previously studied, but no information is available concerning the influence of the distribution of interstitial cells of Cajal (ICC) on these complications. These cells are considered to be pacemakers in the gastrointestinal tract. The aim of this study was to assess the distribution of ICC in the proximal segment of resected bowel in Hirschsprung's disease and confront these results with the clinical outcome. Using immunohistochemistry for light microscopy, we compared the pattern of distribution of ICC in the proximal segment of resected bowel in Hirschsprung's disease with that in normal colon. We correlated these results with the corresponding neural intestinal histology determined by CD56 and the protein gene product 9.5 immunohistochemistry. The distribution of ICC in the proximal segment of resected bowel is identical to that of normal colon, regardless of normal or abnormal colon innervation. ICC distribution does not seem to contribute to post-operative bowel motility disorders in patients operated for Hirschsprung's disease

Prenatal diagnosis of urinary malformations: results in a series of 93 consecutive cases.

OBJECTIVE: To evaluate the pertinence of prenatal diagnosis in cases of congenital uropathy. STUDY DESIGN: Retrospective evaluation over a period of 6.5 years. METHOD: 93 cases were involved in the comparison of prenatal ultrasonographic diagnosis with neonatal findings, autopsy results, and follow-up data. RESULTS: 33 fetuses had renal parenchymal lesions, 44 had excretory system lesions, and 6 had bladder and/or urethral lesions. Seventy-three pregnancies lead to live births. Eighteen terminations of pregnancy were performed on the parents' request for extremely severe malformations. Two intrauterine deaths were observed, and two infants died in the postnatal period. Prenatal diagnosis was obtained at an average of 27 weeks gestation. Diagnostic concordance was excellent in 82% and partial in 12% of cases with renal parenchymal lesions; the false-positive rate was 6%. For excretory system lesions, concordance was excellent in 87% and partial in 7.4% of cases, with a false-positive rate of 5.6%. Finally, concordance was excellent in 100% of cases of bladder and/or urethral lesions. The overall rate of total concordance was 86%. Partial concordance cases consisted of malformations different from those previously diagnosed, but prenatal diagnosis nevertheless lead to further investigations in the neonatal period and to proper management. The false-positive diagnoses (5.4%) never lead to termination of pregnancy. CONCLUSION: Prenatal diagnosis of congenital uropathy is effective. A third-trimester ultrasonographic examination is necessary to ensure proper neonatal management, considering that the majority of cases are diagnosed at this gestational age.

Assessment of the colon innervation with serial biopsies above the aganglionic zone before the pull-through procedure in Hirschsprung's disease.

Different types of colonic dysganglionosis, and in particular intestinal neuronal dysplasia (IND) have been blamed for certain postoperative complications after surgery for Hirschsprung's disease (HD). We prospectively assessed colon innervation above the aganglionic zone (AZ) before proceeding to pull-through (PT) in order to rule-out IND as a cause of those complications. We first used a two-stage procedure (TSP): Full-thickness biopsies were harvested above the AZ and a colostomy was established during a first stage. Biopsies were assessed postoperatively with conventional acetyl-cholinesterase (AChE) histochemistry and immunohistochemistry for protein gene product 9.5 (PGP 9.5) and antigen CD56 (CD56). Biopsies were repeated after 6 months if IND was found. When the innervation was normal, the PT was performed during a second stage. Since having refined a rapid AChE reaction, we now use a single-stage procedure (SSP). Biopsies are harvested above the AZ and assessed intraoperatively with rapid AChE staining, proceeding to PT during the same stage when the innervation is normal. Four patients underwent the TSP; 3 had normal innervation above the AZ and subsequently underwent PT. In 1 patient serial biopsies revealed IND-like dysganglionosis; 9 months later, the innervation was normal in repeat biopsies and PT was undertaken. Eleven patients underwent the SSP. Biopsies were normal in 9 but showed unclassifiable forms of dysganglionosis in 2. As these changes were not typical for IND, all patients underwent PT in the same stage. Both patients had a poor outcome of bowel function that required a colostomy in 1 and daily saline irrigations in the other. IND was found in repeat biopsies made during the colostomy in the 1st patient and markedly hypertrophied nerves in the submucosa as well as ectopic nerve cells in the lamina propria in the proximal border of the pulled-through colon in the other. All 13 other patients have normal bowel function. The assessment of colon innervation above the AZ before proceeding to PT allows safer surgical treatment of HD. Intraoperative AChE staining is reliable, but due to the size and number of the biopsies, IND might be overlooked. Non classifiable dysganglionosis should thus be taken into account in the diagnosis and follow-up of the patients, as it may be clinically significant.

Posterior hypospadias: the French operation.

We describe a new technique for complete repair of severe posterior hypospadias in one stage. The operative technique includes some innovative points penile straightening is realized by urethral mobilization to excise the chordee, and then urethroplasty according to Duplay procedure and glanduloplasty are performed. We report our results in 34 patients. Preservation of urethral plate in hypospadias repair is a principle with significant implications to an extended variety of hypospadias.

[Treatment of pediatric fractures by intramedullary stable elastic pinning]. / Traitement des fractures de l'enfant par enclouage centro-médullaire élastique stable (ECMES).

During the past decade several new techniques for the treatment of children's fractures respecting the specificity of the growing bone have been described. The goal of all these techniques was to mechanically stabilise the fracture however to preserve a certain instability of the fracture gap itself inducing early callus formation and subsequent consolidation. The dynamic external fixation as well as the elastic stable intramedullary pinning have become accepted means in the treatment of long bone fractures in the paediatric age group. We report our experience of the last seven years with the intramedullary pinning of 105 fractures. Eighty-four were fractures of the femur, 9 of the humerus, 8 of the forearm, and a further 4 of the tibial shaft. The intramedullary elastic pinning represents a simple technique which supports or even enhances the natural process of fracture healing of the growing bone. The method is not very invasive, is cost effective, and allows short hospitalisation. Early physical activity is guaranteed due to early consolidation of the fracture. Complications are rare and the final orthopedic and cosmetic outcome is excellent.

Pitfalls in aminoacid and organic acid analysis: 3-hydroxypropionic aciduria.

Pitfalls in organic acid analysis can originate from inadequate methodology, analytical interferences, in vivo interactions and from pre-analytical conditions which often are unknown to the specialized analytical laboratory. Among the latter, ingested food and additives, metabolites of food processing or medications have to be considered. Bacterial metabolites from the gastrointestinal or urogenital system or formed after sample collection can lead to pitfalls as well. An example of such a patient whose urinary metabolites mimic at first glance inherited propionic aciduria is described.

Pre- and postoperative urodynamic and anorectal manometric findings in children operated upon for a primary tethered cord.

The aim of the study was to assess the sensitivity of both urodynamics and anorectal manometry in detecting neurological signs in children with a primary tethered cord. Between 1989 and 1991, 9 children were operated upon for untethering of primary tethered cord in Lausanne. Both manometric studies were performed by the same team preoperatively and 6 months after surgery. Gaeltec catheters with micro-tip transducers were used with a standardized method. Preoperative manometric findings were pathological in 4 children without clinical signs of bladder dysfunction and in 3 without clinical fecal disorders. Postoperatively, bladder dysfunction regressed in one child and fecal disorders in another. Manometric findings remained nevertheless pathological. The authors held both urodynamics and anorectal manometry as mandatory for preoperative evaluation as well as for the postoperative monitoring of children with primary tethered cord.

[Urethral stenosis in children: treatment using urethroplasty with a vesical or oral mucosal graft]. / Sténoses urétrales chez l'enfant: traitement par urétroplastie avec greffon de muqueuse vésicale ou buccale.

Urethral strictures are rare in children and correspond to either congenital membranes, post-traumatic (endoscopy). The precise diagnosis is obtained by voiding cystometrography at the end of urography or by retrograde opacification. Congenital membranes should be treated by dilatation and limited strictures should be treated by urethrotomy. The other cases warrant urethroplasty with a free vesical or oral mucosal flap. Good results are obtained in about 90% of cases.

Ureterosigmoidostomy: a long-term follow-up of 15 patients with urinary diversion.

We reviewed 15 patients who underwent 16 ureterosigmoidostomies from 1975 to 1989. The underlying disease was bladder exstrophy in 13 patients. Urinary diversions have been performed according to Mathisen in 15 patients, one reoperation has been performed according to Coffey. Standard post-operative controls consist of physical examinations, capillary blood gas samples, intravenous pyelograms, inulin clearance measurements, 123I-hippuran scintigraphies and colonoscopies. Results concerning continence are judged very satisfactory in 80% of patients. 34% of patients presented with one or more episodes of unilateral or bilateral pyelonephritis. Intravenous pyelograms showed improved or unchanged urinary tract dilatation in 45% of kidneys and moderate and severe dilatation in 55%. Inulin clearance remained in normal range in 4 out of 6 patients followed. Renal function assessed by means of renal scintigraphy remained unchanged in 61% of kidneys, slightly decreased in 22% and severely in 17%. All patients are kept on a sodium-citrate therapy. Only one patient shows growth disturbances. Out of 9 patients followed with colonoscopy, none showed signs of malignancy.

[Surgical therapeutic attitude towards complicated stress ulcers in children]. / Attitude thérapeutique chirurgicale face à l'ulcère de stress compliqué de l'enfant.

Perforation or uncontrollable hemorrhage following a stress ulcer rarely occurs in high risk children if strict prophylactic measures are adopted. However, we have observed and operated 3 cases over a period of 2 years. Despite the diverse etiologies and contrary to some authors, we have always chosen the simplest and least mutilating operation in order to preserve the physiologic function of the digestive tract. The aim of this article is to compare our therapeutic attitude with that of the literature.