Metanephric adenoma diagnosed on biopsy in an infant: a case report.

BACKGROUND: Metanephric adenoma is a rare benign renal tumor of the kidney, uncommonly observed in children. It is often misdiagnosed preoperatively as a malignant neoplasm, leading to an unnecessary nephrectomy. The challenge is to make the right diagnosis preoperatively and therefore manage it with conservative surgery. We report a case of a child with metanephric adenoma who underwent nephron-sparing surgery. CASE PRESENTATION: A renal tumor was discovered fortuitously in an 18-month-old Caucasian girl with several congenital malformations. Investigations showed a 28 × 27 × 27 mm left renal mass centrally located, well defined, nonvascularized, with no calcifications and which compressed the adjacent renal tissue. Furthermore, there were no signs of metastasis. The decision of a multidisciplinary meeting was to perform a computed tomography (CT)-scan-guided biopsy. Histologic examination concluded it was a metanephric adenoma. We performed a left open partial nephrectomy via a flank retroperitoneal incision. The final histopathological examination confirmed the diagnosis. The postoperative course was uneventful. CONCLUSION: Preoperative diagnosis of metanephric adenoma is challenging. Because of the high probability of unnecessary radical nephrectomy, preoperative biopsy can be safe and determining to guide a more conservative approach so nephron-sparing surgery can be performed.

Schistosoma mansoni PCR+ -infected individuals in the Sudan present elevated systemic levels of chemokines when compared to uninfected and egg+ cohorts.

Infections with Schistosoma mansoni remain a major health problem in the Sudan where endemic communities, such as those in Kassala and Khartoum states, continue to face severe social-economic difficulties. Our previous immunoepidemiological findings revealed different immune [cytokine and S. mansoni egg (SEA) antibody] profiles in individuals with active infections (eggs in stool n = 110), individuals positive for S. mansoni via polymerase chain reaction (PCR) using sera (SmPCR+ n = 63) and those uninfected (Sm uninf). As antibody responses to eggs and worms are known to change during infection, we have expanded the profiling further by determining levels of adult worm (SWA) antibodies and nine chemokines in the serum of each individual in the three different cohorts. With the exception of C-C motif chemokine ligand (CCL)2, all measured chemokines were significantly higher in SmPCR+ individuals when compared to the egg+ group and in addition they also presented elevated levels of SWA-specific immunoglobulin (Ig)G2. Multivariable regression analysis further revealed that infection per se was strongly linked to SWA-specific IgG3 levels and CCL5 was strongly associated with a SmPCR+ diagnostic state. In the absence of PCR diagnostics that recognize juvenile worms or schistosomulae motives, identifying schistosome-specific traits should provide better insights into current prevalence rates in endemic communities and, in doing so, take into consideration PCR+ non-egg+ individuals in current treatment programmes.

Biomarkers and Pharmacogenomics in Kidney Transplantation.

This review is focused on present and future biomarkers, along with pharmacogenomics used in clinical practice for kidney transplantation. It aims to highlight biomarkers that could potentially be used to improve kidney transplant early and long-term graft survival, but also potentially patient co-morbidity. Future directions for improving outcomes are discussed, which include immune tolerance and personalising immunosuppression regimens.

Subsarcolemmal and interfibrillar mitochondria display distinct superoxide production profiles.

Cardiac subsarcolemmal mitochondria (SSM) and interfibrillar mitochondria (IFM) subpopulations display distinct biochemical, morphological, and functional characteristics. Moreover, they appear to be differently influenced during cardiac pathologies or toxic injuries. Although mitochondrial reactive oxygen species seem to play a critical role in cardiac function and diseases, limited information exists about the superoxide production characteristics of these mitochondrial subpopulations. In this work, using direct measurement of superoxide by electron paramagnetic resonance, we showed that differences in superoxide production profiles were present between cardiac IFM and SSM, in terms of intensity and major sites of superoxide generation. In SSM incubated with glutamate plus malate as substrates, the total observed superoxide levels were significantly higher than those observed with IFM, with an important contribution of the NADH-oxidizing site of complex I (site If) and the quinol-oxidizing site of complex III (site IIIQ0). In both IFM and SSM, succinate leads to similar rates of total superoxide levels with a substantial role for contribution of reverse electron transfer. Finally, using two spin probes with different membrane permeabilities, our data on complex III showed direct intra- and extra-mitochondrial superoxide release whereas complex I- and II-dependent superoxide were exclusively released inside the mitochondria, confirming previous studies. Feasibility of this approach to measure intra- and extra-mitochondrial superoxide levels and to characterize distinct superoxide production profiles of cardiac IFM and SSM has been demonstrated.

[Gastric volvulus in children: five case reports]. / À propos de 5 cas de volvulus gastrique chez l'enfant.

UNLABELLED: Gastric volvulus is an abnormal rotation of all or a part of the stomach around one of its axes. It is a rare cause of intestinal obstruction in children. This anomaly can be primary, due to abnormalities of the gastric ligaments, or secondary to other congenital malformations. We report on the cases of five children treated between January 1994 and December 2011, four boys and one girl, with a medium age of 7 months. Diagnosis was based on clinical features, particularly in the upper gastrointestinal Rx contrast study, which confirmed the diagnosis. Four out of the five children underwent laparoscopic surgery with fixation of the stomach. A diaphragmatic hernia was associated in one case. Antireflux surgery was performed in three cases, and a diaphragmatic defect was closed in one case. The follow-up was uneventful after a medium period of 7 years. CONCLUSION: good knowledge of this anomaly is the guarantee of early diagnosis and optimum treatment to ameliorate the prognosis.

[Cervical teratoma in a child]. / Tératome cervical chez l'enfant.

Teratomas are unusual tumors derived from all 3 germs cells layers: endoderm, mesoderm, and ectoderm, with varying proportions. The cervical area is exceptionally affected. We report 4 cases of cervical teratoma. The clinically and radiologically suggested diagnosis was confirmed by histology. We describe herein the main clinical, radiological, and histological aspects and outcomes of this disease. Despite its most often benign histologic nature, cervical teratoma may threaten newborn infants' life due to airway compression. A multidisciplinary approach to the disease starting at delivery is required to improve the prognosis.

Prevalence and associated risk factors of male erectile dysfunction among patients on hemodialysis and kidney transplant recipients: a cross-sectional survey from Sudan.

Male erectile dysfunction (ED) is an important issue worldwide occurring in 5-69% of men in community-based studies. It is more common in patients with chronic kidney disease (CKD) and those on peritoneal as well as hemodialysis (HD), occurring in more than 80% of patients. In Sudan, there is no previous report on ED among patients with CKD. A cross-sectional study was done to determine the prevalence of ED and its associated risk factors among Sudanese CKD patients on HD and those who underwent renal transplant. This was conducted in Khartoum, Sudan from October 2005 to July 2006 including all married men who were on maintenance HD for more than three months and all married men who had received renal transplantation at least three months earlier. Single, divorced/separated men, those whose wives were living away, those who were bed-bound and those with cognitive impairment were also excluded. After obtaining consent for participation, demographic and clinical data were collected by using anonymous questionnaires and the Arabic version of International Index of Erectile Function (IIEF; the Egyptian version). Patients who did not participate in full and proper manner were considered as "non-responders." A total of 146 patients, 106 HD patients, and 40 renal transplant recipients completed the IIEF questionnaire. Non-responders constituted 43.7% and 54.5% of HD and transplant recipient patients, respectively. Blood samples were taken after completion of the IIEF questionnaire to determine the required investigations. ED prevalence was high among our study patients, 83% among the HD patients and 67.5% among the renal transplant recipients. Univariate analysis showed that there was a trend, although non-significant, of older age being associated with ED in both groups. Similar association was seen in those who were under-dialyzed in the HD group and DM in the transplant recipient group. Previous history of ED was significantly associated with current presence of ED in both groups. More studies with larger sample size are needed to clarify the results of this study.

[Isolated congenital tracheoesophageal fistula]. / Fistules oesotrachéales congénitales isolées.

INTRODUCTION: Isolated tracheoesophageal fistula without esophageal atresia is a rare congenital malformation. Its etiology is obscure. Diagnosis is difficult but must be made early. PURPOSE: To study the clinical, radiological, and evolutionary sights of this malformation. PATIENTS AND METHODS: We report 4 cases of tracheoesophageal fistula, collected in the department of pediatric surgery of Monastir Hospital and in the neonatology unit of Sousse Hospital during the period between January 2001 and December 2010. RESULTS: The clinical picture consisted in a coughing bout and cyanosis after each feeding. Thoracic and abdominal imaging showed aspiration pneumonia, atelectasis, and gas within the colon. Gastrointestinal opacification demonstrated the fistula in 2 cases. Tracheoscopy visualized the tracheoesophageal fistula in the other 2 cases. Treatment was surgical and consisted in the section-ligation of the tracheoesophageal fistula with pleural interposition in all cases. The course was simple in two cases with a 3-year and 3.5-year follow up, respectively, but the infants died in the other 2 cases. CONCLUSION: Although a rare malformation, tracheoesophageal fistula should be suggested as a diagnosis when respiratory symptoms occur during feeding starting during the neonatal period.

[Esophageal strictures in children with epidermolysis bullosa]. / Sténoses œsophagiennes de l'épidermolyse bulleuse.

UNLABELLED: Esophageal stricture is a rare but often severe complication of recessive dystrophic epidermolysis bullosa in children. The purpose of the study was to review this digestive complication with emphasis on diagnostic modalities and therapeutic management. PATIENTS AND METHODS: This was a retrospective study of two pediatric cases of esophageal stenosis that occurred during generalized recessive dystrophic epidermolysis bullosa of the Hallopeau-Siemens type. RESULTS: The 2 patients were aged 8 years 8 months and 11 years 5 months, respectively. Dysphagia was of early onset, before the age of 10 years in both cases. Esophageal opacification led to the diagnosis of esophageal stenosis located in the upper 1/3 of the esophagus in 1 case and at the junction between the middle and the lower 1/3 of the esophagus in the other case. None of the 2 patients received medical treatment, and pneumatic dilation was the treatment method that was advocated. Esophageal endoscopy showed the stenosis and helped guide the positioning of the balloon catheter. These patients underwent 2 and 3 sessions of dilation, respectively, at intervals of 2 months and 1 year. Balloon dilation has allowed the patients to have a more comfortable life with decreased dysphagia and a substantial improvement in nutritional status. However, this improvement was transient (1 patient had symptomatic recurrence of stenosis after 3 years), which shows that monitoring of the patients and the resumption of dilatation sessions may be necessary. CONCLUSION: Esophageal strictures in dystrophic epidermolysis bullosa of the Hallopeau-Simens type are severe and difficult to support. Pneumatic dilatation is the treatment of choice for the fragile esophagus. It gives satisfactory results and can be repeated without significant risk.

[A comparative study of a disposable non-prismatic cone versus the biprismatic cone over 100 IOP measurements]. / Étude comparative prospective: cône aprismatique jetable/cône biprismatique, à propos de 100 mesures de la PIO.

INTRODUCTION: In 2009, we developed a non-prismatic cone in accordance with Goldmann tonometer standards. We undertook a prospective comparative clinical study of the non-prismatic cone versus the traditional biprismatic cone. MATERIALS AND METHODS: The disposable non-prismatic Plexiglas cone contains, on the inner side of its applanating end, a raised circular pattern 3.06 mm in diameter. Patient recruitment was performed in the setting of diurnal intraocular pressure (IOP) measurement for all comers including medically treated glaucoma patients. Two operators clinically tested the non-prismatic cone, with each operator measuring his or her own series of 50 eyes, for a total of 100 IOP measurements. RESULTS: For the first operator, the mean IOP measurement with the non-prismatic cone was 17.92 mmHg, range 13 to 26 mmHg; the mean IOP measurement with the biprismatic cone was 17.40 mmHg; the difference between mean IOP measurements was 0.52 mmHg, with a correlation coefficient of 0.98. For the second operator, with a series ranging from 10 to 19 mmHg, similar results were observed. DISCUSSION: Disposable cones, including the non-prismatic cone, adapted for the Goldmann tonometer are superior to other strategies for infection prophylaxis, such as chemical disinfection or condoms. CONCLUSION: This study demonstrates the reliability of the disposable non-prismatic cone adapted for the Goldmann tonometer. Other features unique to the non-prismatic cone are evoked at the end of this paper.

Titanium nitride oxide coating on rough titanium stimulates the proliferation of human primary osteoblasts.

OBJECTIVES: Titanium is widely used in contemporary endosseous implantology and there is considerable thrust to further promote osseointegration by implant surface modifications. The aim of this study was to evaluate the effect of a titanium-nitride-oxide (TiNOx) coating on commercially pure microroughened titanium by assessing the proliferation and differentiation of human primary osteoblasts. MATERIALS AND METHODS: Cell proliferation, gene expression, alkaline phosphatase activity, osteoprotegerin and osteocalcin secretion were analyzed for a time course of 3 weeks, with or without additional stimulation by 1.25(OH)(2) vitamin D(3) 100 nM. RESULTS: A 1.5-fold increase in the proliferation rate of cells grown on TiNOx-coated titanium as compared with uncoated surfaces was observed. SEM views indicated that the cells' normal morphology with their numerous extensions was maintained. The differentiation process on the TiNOx surface was only affected to a minor degree and translated into a slight delay in osteoblast maturation when compared to uncoated titanium. CONCLUSION: Pending confirmation of these results in vivo, TiNOx coatings could potentially accelerate and enhance osseointegration.

[Epispadiac urethral duplication in boys]. / L'urètre surnuméraire épispade chez le garçon.

Urethral duplication, also called supernumerary urethra, is a rare malformation essentially affecting the boys. It is defined by juxtaposition of two or more muscular channels with urinary tract mucosal lining. Various features may be observed depending on the site of the extra urethra and its incomplete or complete nature. In this study, the authors examined a particular aspect of this malformation, the epispadiac form.

[Genetic effects of MC4R and POU1F1 gene on the growth performance in Jinghai yellow chicken].

Melanocortin-4 receptor (MC4R) gene and pituitary specific transcription factor 1 (POU1F1) gene were studied as candidate genes on the Jinghai yellow chicken in this study. Polymorphisms of MC4R and POU1F1 gene had been analyzed by PCR-SSCP and DNA sequencing method. The relationship between two candidate genes and the growth performance of Jinghai yellow chicken had also been analyzed by GLM model. The frequency of A/B alleles for MC4R gene in the population was 0.929 and 0.071, and that of C/D alleles for POU1F1 gene was 0.500 and 0.500. The results showed that AA genotype of MC4R gene had higher bodyweight at 4, 8, 12 weeks compared to AB and BB genotype (P<0.05); CD genotype of POU1F1 gene had significantly higher bodyweight at all weeks compared to CC and DD genotype (P<0.01). So, it could be preliminarily deduced that MC4R and POU1F1 gene were probably the major gene on growth performance or a QTL linked gene which associated with growth performance in chicken.

Post-surgical follow-up (by ELISA and immunoblotting) of cured versus non-cured cystic echinococcosis in young patients.

The study was designed to determine comparatively the prognostic value of immunoblotting and ELISA in the serological follow-up of young cystic echinococcosis (CE) patients exhibiting either a cured or a progredient (non-cured) course of disease after treatment. A total of 54 patients (mean age 9 years, range from 3 to 15 years) with surgically, radiologically and/or histologically proven CE were studied for a period up to 60 months after surgery. Additionally, some of the patients underwent chemotherapy. Based on the clinical course and outcome, as well as on imaging findings, patients were clustered into 2 groups of either cured (CCE), or non-cured (NCCE) CE patients. ELISA showed a high rate of seropositivity 4 to 5 years post-surgery for both CCE (57.1%) and NCCE (100%) patients, the difference found between the two groups was statistically not significant. Immunoblotting based upon recognition of AgB subcomponents (8 and 16 kDa bands) showed a decrease of respective antibody reactivities after 4 years post-surgery. Only sera from 14.3% of CCE patients recognized the subcomponents of AgB after 4 years, while none (0%) of these sera was still reactive at 5 years post-surgery. At variance, immunoblotting remained positive for AgB subcomponents in 100% of the NCCE cases as tested between 4 and 5 years after surgical treatment. Immunoblotting therefore proved to be a useful approach for monitoring post-surgical follow-ups of human CCE and NCCE in young patients when based upon the recognition of AgB subcomponents.