Nutritional intervention with TGF-beta enriched food for special medical purposes (TGF-FSMP) is associated with a reduction of malnutrition, acute GVHD, pneumonia and may improve overall survival in patients undergoing allogeneic hematopoietic stem transplantation.

Malnutrition in allogeneic stem cell transplant (allo-SCT) is associated with poor outcomes. Supplementation with Foods for Special Medical Purposes may be a valid alternative to enteral nutrition or total parental nutrition to reduce malnutrition in allo-SCT. In this study, 133 patients consecutively allo-transplanted were assessed for nutritional status by Patient- Generated Subjective Global Assessment (PG-SGA) and supplemented with TGF-beta enriched Food for Special Medical Purposes (TGF-FSMP). PG-SGA, gold standard for nutritional assessment in oncologic patients, was assessed at admission and on day 0, +7, +14, +21, and + 28 from transplant and categorized as follows: A = good nutritional status; B = moderate malnutrition; C = severe malnutrition. TGF-FSMP (Modulen-IBD) is currently used in Inflammatory Bowel Diseases (IBD) as primary nutritional support and in this study the dose was calculated according to BMI and total daily energy expenditure (TDEE). The patients assuming ≥50% of the prescribed TGF-FSMP dose were classified in Group A; the patients who received < 50% were included in Group B per protocol. The primary endpoint of the study was the assessment of the malnourished patients in Group A and B at day+28 after transplantation, according to the criteria of PG-SGA C categorization. At day +28 after transplant: i) patients in Group A were significantly less severely malnourished than patients in the Group B (21/76,28% vs 42/53, 79% respectively, OR 2.86 - CI 1.94-4.23 -, p = 0.000); ii) the incidence of severe (MAGIC II-IV) aGVHD and of any grade gastrointestinal (GI) aGVHD was higher in Group B than in Group A, (43% vs 21% p = 0.003) and (34.5% vs 9.2% p = 0.001); iii) Pneumonia was more frequent in the malnourished patients of Group B than in well/moderate nourished patients of Group A (52.7% vs 27.6% p = 0.002). In group A parenteral nutrition was avoided more frequently than in group B (67.5% vs 33.3% p = 0.000) and a median hospital stay of 27 days in comparison to 32 was reported (p = 0.006). The estimated median overall survival (OS) of the population was 33 months in Group A and 25.1 months in group B (p = 0.03). By multivariate and ANN analysis, TGF-FSMP TR < 50% assumption was significantly correlated with malnutrition, severe and GI aGVHD, pneumonia and reduced OS.

Image quality of spectral brain computed tomography angiography using halved dose of iodine contrast medium.

PURPOSE: Reduction in iodinated contrast medium (CM) dose is highly motivated. Our aim was to evaluate if a 50% reduction of CM, while preserving image quality, is possible in brain CT angiography (CTA) using virtual monoenergetic images (VMI) on spectral CT. As a secondary aim, we evaluated if VMI can salvage examinations with suboptimal CM timing. METHODS: Consecutive patients older than 18 years without intracranial stenosis/occlusion were included. Three imaging protocols were used: group 1, full CM dose; group 2, 50% CM dose suboptimal timing; and group 3, 50% CM dose optimized timing. Attenuation, noise, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) were measured in the internal carotid artery, M2 segment of the middle cerebral artery, and white matter for conventional images (CI) and VMI (40-200 keV). Qualitative image quality for CI and VMI (50 and 60 keV) was rated by 4 experienced reviewers. RESULTS: Qualitatively and quantitatively, VMI (40-60 keV) improved image quality within each group. Significantly higher attenuation and CNR was found for group 3 VMI 40-50 keV, with unchanged SNR, compared to group 1 CI. Group 3 VMI 50 keV also received significantly higher rating scores than group 1 CI. Group 2 VMI (40-50 keV) had significantly higher CNR compared to group 3 CI, but the subjective image quality was similar. CONCLUSION: VMI of 50 keV with 50% CM dose increases qualitative and quantitative image quality over CI with full CM dose. Using VMI reduces non-diagnostic examinations and may salvage CTA examinations deemed non-diagnostic due to suboptimal timing.

Malnutrition Prevention after Allogeneic Hematopoietic Stem Cell Transplantation (alloHSCT): A Prospective Explorative Interventional Study with an Oral Polymeric Formulation Enriched with Transforming Growth Factor Beta 2 (TGF-ß2).

Malnutrition is common after allogeneic Hematopoietic Stem Cell Transplantation (alloHSCT), and interventions directed to correct nutritional status are warranted to improve transplant outcomes. In this prospective study, an oral polymeric formulation enriched with TGF-ß2 (TE-OPF) was explored to correct malnutrition according to Patient-Generated Subjective Global Assessment (PG-SGA). TE-OPF was proposed to 51 consecutive patients who received transplants at our institution for hematological malignancies, and sufficient dose intake was established per protocol as at least 50% of the prescribed dose of TE-OPF: group A received adequate nutritional support; group B, inadequate. The study met the primary outcomes in terms of safety (no adverse events reported during TE-OPF intake except for its disgusting taste) and malnutrition (PG-SGA C 28 days after transplant): severely malnourished patients (PG-SGA C) accounted for 13% in group A and 88.9% in group B (p = 0.000). At the end of the study, after a median follow-up of 416 days, the estimated median Overall Survival (OS) was 734 days for well or moderately nourished patients (PG-SGA A/B) in comparison to 424 for malnourished patients (p = 0.03). Inadequate TE-OPF intake was associated with an increase in acute gastrointestinal Graft Versus Host Disease (GVHD) cumulative incidence (38% vs. 0% p = 0.006). A higher incidence of pneumonia was reported in group B (p = 0.006). IGF-1 levels at 14 and 28 days after transplant were significantly higher in group A and were associated with a lower incidence of acute GVHD (aGVHD). Higher subsets of B, T, and NK cells were found in group A, and a higher number of CD16+ NK cells was associated with a lower incidence of acute GVHD (p = 0.005) and increased survival at the end of the study (p = 0.023). Artificial neural network analysis suggested that inadequate TE-OPF intake, pneumonia, and sepsis significantly affected malnutrition 28 days after alloHSCT and survival 365 days after alloHSCT (normalized importance 100%, 82%, and 68%, respectively). In this exploratory and preliminary study, the use of TE-OPF appeared to reduce the incidence of malnutrition after alloHSCT, but larger and controlled studies are required.

Structural, energetic and kinetic database of catalytic reactions: Benzyl alcohol to benzaldehyde oxidation on MnO x clusters.

Data here reported are connected with the research article "Benzyl Alcohol to Benzaldehyde Oxidation on MnO x Clusters: Unraveling Atomistic Features" Gueci et al. [1]. This work described and discussed structural and energetic results, calculated by Density Functional Theory (DFT). In order to get kinetic information, DFT results were refined by an original approach, which will be shortly described in the following article. The crossed analysis of experimental and computational energetic and kinetic data allowed to (i) reconstruct the complicated lattice that connects the primary and secondary mechanisms of the reaction and (ii) identify alternative process pathways capable of by-passing parasitic mechanisms, decreasing selectivity. On the other hand, the data here presented show what is the basic information necessary to obtain the modeling of a complex process of heterogeneous catalysis. Moreover, they can be used either to verify the validity of the discussion outlined in the original article Gueci et al. [1] or as a starting point to computationally explore alternative routes and the related kinetics of the same oxidation processes, in the aim to further optimize the corresponding experimental approach.

A Systematic Review of the Literature and Perspectives on the Role of Biomarkers in the Management of Malnutrition After Allogeneic Hematopoietic Stem Cell Transplantation.

Malnutrition is a common problem after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and could impair immune function. Immune dysfunction after allo-HSCT may be linked with infections, GVHD, and relapse and negatively affect the outcome. Aim of this review was to identify malnutrition biomarkers, potentially useful for immune-system monitoring, in the setting of allo-HSCT. After a systematic search, no satisfying biomarker was found, except for citrulline. Citrulline could be useful in monitoring gastrointestinal function after allo-HSCT and its role in the complex relationship with immune-system function ought to be better explored. A multi-omics approach, including biomarkers and PRO (patient reported outcomes) is, in our opinion, the optimal way to study the relationship between malnutrition and transplant outcomes.

Human Epidermal Growth Factor Receptor 2 Status in Gastric Carcinomas with Distinctive Prevalent Cribriform Component.

OBJECTIVES: A cribriform architectural pattern has been reported in 9% of one unselected consecutively collected series of gastric carcinomas (GC) with unfavourable prognostic outcome. Taking into consideration the biological relevance of the human epidermal growth factor receptor 2 (HER2) status, we have analyzed a cohort of GC with a cribriform component more than 40% (CGC) to evaluate the HER2 amplification rate as a potential target for therapy with trastuzumab. RESULTS: HER2 overexpression was encountered in 21 of 100 (21%) GC; a progressive increase in HER2 amplification was appreciated moving from non-CGC (20.6%) towards CGC cases (21.6%), although this difference does not reach a statistical significance. Nevertheless, either in univariate or in multivariate analyses, stage and HER2 status showed a significant p value (<0.001) in CGC patients. CONCLUSIONS: Our data confirmed a worse prognosis in all CGC patients with HER2 amplification, resulting in a shorter survival time. We invite all pathologists in their daily practice to specify the occurrence of cribriform neoplastic component in GC, either in surgical or in bioptical samples, taking into practical assessment the high HER2 overexpression rate in order to correctly treat these patients with worse behavior.

Detection of pleural plaques in workers exposed to inhalation of natural fluoro-edenite fibres.

Fluoro-edenite is a natural mineral species initially isolated in Biancavilla, Sicily. The fibres are similar in size and morphology to certain amphibolic asbestos fibres, the inhalation of which may cause chronic inflammation and cancer. Occupational asbestos exposure is known to be associated with pleural and lung diseases, including pleural plaques. The aim of this study was to report the pleural and lung parenchymal lesions detected by high-resolution computed tomography (HRCT) in a group of construction workers exposed to fluoro-edenite. Information regarding life habits and occupational history was collected from 43 workers enrolled into the study. The participants underwent physical examination, blood analysis, search for uncoated fibres and ferruginous bodies in the sputum, pulmonary function tests, including diffusion capacity for carbon monoxide (TLCO), and HRCT chest imaging. A general descriptive outcome analysis was also conducted; a prevalence ratio (PR) with 95% confidence interval and a two-tailed test P-value were calculated for pleural plaques using log-binomial regression, measuring plaque size and thickness, and cumulative exposure index (CEI). The mean values of the functional respiratory tests were within the normal range for all participants. A restrictive ventilatory defect was identified in two (5%) subjects and an obstructive ventilatory defect in three (7%) subjects. TLCO was reduced in two additional participants. Fibres were detected in 19 (44%) of subjects. Pleural involvement was documented in 39 (91%) workers, of whom 31 (72%) had bilateral plaques. Calcifications were detected in 25 (58%) of these participants. PR indicated a progressive increase in the risk of developing pleural lesions with rising CEI, i.e. length of exposure. The present findings demonstrate for the first time the presence of pleural plaques in the lungs of subjects exposed to fluoro-edenite fibres, and not to asbestos, through residing in Biancavilla and through their occupation.

The use of microwaves ablation in the treatment of epiphyseal osteoid osteomas.

OBJECTIVE: This study was designed to demonstrate the feasibility and the reliability of microwave ablation (MWA) of epiphyseal osteoid osteomas (OO). MATERIALS AND METHODS: From February to November 2012, 7 patients (4 males and 3 females; age range 16-30 years) with epiphyseal OOs were treated with MWA. The treatment was performed with 16 G antennas with a power of 20 W for 2 min. The OOs were approached by using coaxial needles inserted with hammer or with automatic drill. All patients underwent spinal anaesthesia, with posttreatment 6-8 h observation before discharging. We treated epiphyseal OOs placed away from nervous and vascular nontarget structures, located in: femoral head (n = 2), femoral lesser trochanter (n = 2), femoral neck (n = 2), and proximal tibial epiphysis (n = 1). CT was used to visualize the nidus and to insert the needle for thermal ablation and for postprocedure control. Technical success was considered the positioning of the antenna in the nidus, while the efficacy of treatment was clinically evaluated as the complete remission of pain after the procedure by using the visual analogue score (VAS). Follow-up was performed by using VAS score 1 day, 1 week, and 1, 3, and 6 months after the procedure, whereas MRI examination was performed immediately after the procedure, at 1 month, and in any case of recurrence. Complications were also recorded. RESULTS: All patients experienced resolution of the symptomatology (VAS = 0) in ~1 week until the last follow-up, with residual VAS < 2 points occurring only from 1 to 7 days after the procedure. No intraprocedural complication was noted, whereas one patient had back pain for 2 months after the procedure, likely due to spinal analgesic injection. CONCLUSIONS: In our experience, MWA can be safely performed with excellent results without complications in selected cases of epiphyseal OOs; however, the clinical significance of this report is limited because there were only few patients included in this study. Thus, these data must be confirmed by further and larger studies.

Surgical stress after open and transumbilical laparoscopic-assisted appendectomy in children.

INTRODUCTION: The transumbilical laparoscopic-assisted appendectomy (TULAA) effects on the surgical stress response in children have not been studied. Our aim is to investigate the stress response in TULAA. METHODS: A total of 35 children underwent the appendectomy by open approach (OA) or TULAA approach. Interleukins (ILs)-6, -18, and -10 were measured before (T0), at the beginning (T1a), and at the end of surgery (T1b) and 24 hours after (T2). RESULTS: An increase in IL-6 levels at T1b and T2 and in IL-18 at T2 was observed after OA. A significant increase of both IL-6 and IL-18 were observed at T2 but values were less compared with OA (11.6 ± 4.4 vs. 31.9 ± 8.9 pg/mL for IL-6, p = 0.0006; 145.6 vs. 174.9 pg/mL for IL-18, p = NS). CONCLUSIONS: A significant reduction in the postoperative cytokines in TULAA group suggests that this approach causes less surgical trauma in children.

Thoracoscopy versus thoracotomy for esophageal atresia and tracheoesophageal fistula repair: review of the literature and meta-analysis.

INTRODUCTION: The thoracoscopic approach to esophageal atresia (EA) with tracheoesophageal fistula (TOF) represents a challenging procedure whose real benefits remains unclear. Our purpose is to identify, through a meta-analysis, clinical evidence of the reliability of the thoracoscopic repair (TR) for EA/TOF compared with the open repair. MATERIALS AND METHODS: Defined PubMed search, with analysis of intraoperative and postoperative complications after open or thoracoscopic primary anastomosis for EA/TOF. RESULTS: Five articles met the criteria of meta-analysis, being comparative studies between TR and conventional open repair (COR), although they were retrospective. One article was excluded because it was available only in Japanese. We observed a slight prevalence, statistically insignificant, of the intraoperative and postoperative complication rate for TR: odds ratio (OR) 1.29. Excluding the conversion rate, the meta-analysis between the complication rate for TR and COR did not show a significant difference (OR 0.64). Anastomosis's leaks and strictures considered together did not show a significant difference between the two techniques, p = not significant and OR of 0.56. Similar results were observed analyzing the single outcome of leaks and strictures; the meta-analysis did not show any significant differences with an OR, respectively, of 1.05 and 0.43. CONCLUSIONS: The effectiveness of the endoscopic technique for EA/TOF repair is indicated with outcomes not different from open surgery. A randomized controlled trial is needed in this field to indicate which procedure is superior, open or TR.

Morphological study of the residual ovarian tissue removed by laparoscopy or laparotomy in adolescents with benign ovarian cysts.

BACKGROUND/PURPOSE: The stripping of benign ovarian cysts has been reported to be preferable to cyst wall ablation. The procedure can be performed via either an open or laparoscopic approach. The aim of our study was to evaluate the efficacy of the technique of stripping performed by laparoscopy and laparotomy in the treatment of benign ovarian masses in the pediatric age group with respect to healthy ovarian tissue. METHODS: This retrospective study evaluated all the pediatric or adolescent patients admitted during a 5-year period (2006-2010) for a suspected benign ovarian cyst treated with a laparoscopic technique or traditional open surgery to remove the cyst. The morphological characteristics of the tissue were graded on a semiquantitative scale from 0 to 4. RESULTS: Thirty patients were treated and divided in 2 groups: group A laparoscopy (18 patients) and group B open (12 patients). The median age was 9.1 years. No ovarian tissue could be observed in 26 cases (86.7%). In the 4 remaining (2 laparoscopic and 2 open) cases (13.3%), ovarian tissue was found, but never more than 1 mm in thickness. This finding was always in endometriotic cysts. CONCLUSIONS: We demonstrated that, in the pediatric population, no ovarian tissue is removed together with the cyst if this is nonendometriotic. The type of surgical procedure does not influence the removal of ovarian tissue. Laparoscopic stripping of ovarian cysts is the preferable surgical procedure in patients with prospective potential fertility because of their young age.

Long-term follow-up of neonatally diagnosed primary megaureter: rate and predictors of spontaneous resolution.

OBJECTIVE: Primary megaureter (PM) represents 6-10% of all antenatal displaced urinary malformations. Spontaneous resolution of PM is a well-known event. This long-term follow-up study evaluated the incidence and rate of resolution of PM. Some predictive factors were revised, based on morphological classification and scintigraphic pattern. MATERIAL AND METHODS: Sixty neonates with PM were followed. The diagnosis was confirmed by ultrasound examination and (99m)Tc-DTPA diuretic renal scan. All the observed patients underwent antibiotic prophylaxis. All conservatively treated children were followed from 6 months to 15 years. Follow-up consisted of monthly urine cultures, renal ultrasound and DTPA diuretic renography. Hydroureteronephrosis was considered to have resolved when a retrovesical cross-sectional diameter of ureter less than 6 mm was found. RESULTS: In total, 72 PM were identified in this series. At the end of the follow-up period, 38 PM (52.8%) had resolved, in 18 PM (25%) ureteral dilatation persisted and 16 PM (22.2%) required a surgical procedure. The median age at resolution was significantly affected by presenting hydronephrosis grade and cross-sectional diameter at diagnosis, but not by gender. The (99m)Tc-DTPA renogram results showed no functional impairment in resolved and persisting cases, even after long-term observation. CONCLUSIONS: The data show that 22% of neonatal PM require surgical treatment. Poor drainage on (99m)Tc-DTPA scan, grade IV-V hydronephrosis and ureteric diameter more than 15.0 mm were statistically significant and independent predictive factors for surgery. The time to spontaneous resolution in neonatally diagnosed PM may exceed 3.6 years, after which recovery is rare.

Polydeoxyribonucleotide administration improves the intra-testicular vascularization in rat experimental varicocele.

OBJECTIVE: To study the effect of PDRN on angiogenesis in a model of varicocele in rats. DESIGN: After the creation of experimental varicocele, rats were randomized to one of the four treatments: vehicle, PDRN, DMPX, and PDRN plus DMPX. Twenty-one days after randomization, all animals were euthanized and the left testis was harvested. SETTING: Academic hospital. ANIMAL(S): Male Sprague-Dawley rats were used. INTERVENTION(S): A clamp was passed behind the left renal vein distally to the spermatic vein insertion. A silk ligature was placed around the left renal vein at this site and was tied over the top of a probe. The latter was then withdrawn and the vein was allowed to expand. In shams, a suture was placed but it was not tied. MAIN OUTCOME MEASURE(S): To assess testicular microvascular density using CD34 immunostaining. RESULT(S): Microvascular density in the varicocele plus PDRN group was significantly higher than in other groups. CONCLUSION(S): PDRN could represent a novel therapeutic strategy for varicocele treatment in subfertile patients, improving the innate pathophysiologic mechanism of neoangiogenesis, through compensatory oxygen and metabolite supply to tubular and extratubular testicular compartments.

Ileocystoplasty in rats: metabolic, renal and enteropatch changes in a mid- and long-term follow-up.

INTRODUCTION: Bladder augmentation predisposes humans to many metabolic, renal and enteropatch changes. Our aim was to evaluate in a rat model of ileocystoplasty mid- and long-term urinary, metabolic, renal and graft changes. MATERIALS AND METHODS: We performed an ileocystoplasty and a sham operation in 30 rats. Seven augmented rats and 3 sham-operated animals were euthanized after 1, 3 and 6 months. We performed urinalysis, urine culture and blood sampling for serum electrolytes and urea. Histopathological changes of the ileal patch and kidneys were also evaluated. RESULTS: The urine cultures were positive in 14.3, 57 and 71%, respectively, 1, 3 and 6 months after surgery. Urinary pH, serum chloride and urea of the augmented group were significantly higher. Bladder calculi were formed in 23.8% of ileocystoplasty. Histopathological examination showed urothelialization of the graft with hyperplastic/metaplastic changes. The kidneys showed glomerular depletion and a marked distal tubular ectasia. CONCLUSIONS: Our data showed that, in a mid- and long-term follow-up, rat bladders subjected to ileocystoplasty displayed hyperchloremic metabolic acidosis, electrolyte imbalance, enhanced serum blood urea levels and glomerular/tubular changes. Hyperplastic and/or metaplastic changes at the junctional zone were observed. Our experimental results suggest that frequent monitoring of renal function and surveillance of humans who have undergone ileocystoplasty are recommended.

Altered cytoskeletal structure of smooth muscle cells in ureteropelvic junction obstruction.

PURPOSE: Ureteropelvic junction obstruction is one of the most common causes of hydronephrosis in children. A malfunction of smooth muscle cells is believed to be the underlying mechanism causing obstruction. We investigated the expression of some integrins, talin and ß-dystroglycan, considered the main compound of smooth muscle cell cytoskeleton, and active caspase 3 at the level of the ureteropelvic junction obstruction. MATERIALS AND METHODS: Specimens were obtained at pyeloplasty in 12 children with ureteropelvic junction obstruction. Six control specimens were obtained during organ explantation. Specimens were divided into renal pelvis, ureteropelvic junction and ureter below the obstruction. Western blot analysis of active caspase 3, and immunofluorescence and polymerase chain reaction analysis were performed for α7A, ß1A, α7B and ß1D integrins, talin and ß-dystroglycan. RESULTS: Talin and ß-dystroglycan were slightly impaired in ureteropelvic junction obstruction, while α7B and ß1D integrins were severely reduced, and α7A, ß1A and active caspase 3 were significantly enhanced compared to controls. CONCLUSIONS: We demonstrated activation of apoptosis and a critical alteration of cytoskeleton that might explain the altered function and the increased apoptosis in smooth muscle cells in ureteropelvic junction obstruction. The delayed rearrangement of the cytoskeleton of smooth muscle cells in ureteropelvic junction obstruction might be linked to a postnatal splicing from α7A and ß1A to α7B and ß1D integrins, respectively. This relationship could explain the common clinical scenario of spontaneous improvement of hydronephrosis in children with suspected ureteropelvic junction obstruction.

Activation of adenosine A2A receptors by polydeoxyribonucleotide increases vascular endothelial growth factor and protects against testicular damage induced by experimental varicocele in rats.

In rat experimental varicocele, polydeoxyribonucleotide (PDRN) induces vascular endothelial growth factor (VEGF) production, thereby enhancing testicular function. This may point to a new therapeutic approach in human varicocele.

Laparoscopic vs open varicocelectomy in children and adolescents: review of the recent literature and meta-analysis.

PURPOSE: Laparoscopic varicocelectomy has gained popularity in recent years. The aim of this study was to identify clinical evidence about the reliability of this technique in the recent literature. METHODS: We performed a Medline search for articles published during the last 10 years, using the key words "varicocele," "treatment," and "adolescent." As limits, we used last 10 years, adolescent, clinical trials, randomized controlled trials, meta-analysis, and multicenter retrospective and prospective studies. The results were investigated in recurrence and hydrocele formation. RESULTS: We pooled 37 studies, but 26 of these were excluded because they were neither relevant nor concerned an adolescent population. Meta-analysis showed that there was no statistical difference between laparoscopic surgery and open surgery in recurrence rate and postoperative hydrocele rate. In the laparoscopic group, the incidence of recurrence was higher in the patients undergoing artery ligation compared to patients undergoing artery and venous ligation. Furthermore, a lower rate of postoperative hydrocele was recorded in patients undergoing dye injections before laparoscopic ligation. CONCLUSIONS: Meta-analysis and literature analysis showed that the results after laparoscopic varicocelectomy are comparable to other surgical procedures. The laparoscopic approach has the advantage to treat simultaneously bilateral varicocele.

Outcome and management of isolated severe renal pelvis dilatation detected at postnatal screening.

The aim of this study was to evaluate the incidence and outcome of isolated severe renal pelvis dilatation (RPD; APD>15or=3 and 16 with ureteropelvic junction obstruction (UPJO). Incidence of UTI was significantly higher (p<0.001) in infants of the study group than in infants of the control group (13.9 vs 2.5%). Our data suggest that isolated severe RPD may be a self-limiting condition and that antibiotic prophylaxis (AP) for the prevention of UTI should not be performed. Considering RDP resolution and the incidence of UTI during follow-up, investigations for uropathy in infants with isolated, severe RPD are justified in persistent cases, or when UTI occurs during follow-up. Careful clinical monitoring for signs of UTI and treatment of each episode of UTI may be sufficient and safe.

Molecular events involved in the morphogenesis of multicystic dysplastic kidney.

INTRODUCTION: Wnt-1 is capable of inducing metanephric mesenchyme to undergo tubulogenesis. A relationship between the degree of cystogenesis and reduced E-cadherin (E-cad) expression was described. Syndecan-1 (Sdc-1) has a critical role in kidney development. MATERIALS AND METHODS: Ten multicystic dysplastic kidneys (MCDKs) were stained with hematoxylin and eosin and immunohistochemistry was performed using Wnt-1, E-cad and Sdc-1 antibodies. Eight unaffected kidneys were used as controls. RESULTS: Strong Wnt-1 immunostaining occurred inside cystic/tubular epithelial cells and in blastematous foci. An immunoreaction was observed in glomerular epithelial cells. In controls, just weak cytoplasmic Wnt-1 positivity was seen in tubular epithelial cells. E-cad reaction was negative in MCDKs while strong immunostaining was common in tubular cells of controls. A strong Sdc-1 immunoreaction depicted cystic, tubular and glomerular epithelial cells in MCDKs while Sdc-1 expression documented weak positivity in tubular epithelium alone. CONCLUSIONS: Our data are in accordance with an involvement of Wnt-1 in normal nephrogenesis and with its role in altered epithelial differentiation of metanephric mesenchyme in MCDKs. Wnt-1 signal may function to suppress E-cad expression, a predisposing event for cystogenesis. High expression of Sdc-1 in tubular/cystic epithelial cells of MCDKs might alter the normal transition of stages of the developmental process and modify the anion charge of the glomerular barrier.

Abnormal distribution of sarcoglycan subcomplex in colonic smooth muscle cells of aganglionic bowel.

Hirschsprung's disease (HD) is a development disorder of the enteric nervous system in which the altered innervation explains the inability of the aganglionic segment to relax. Impairment of cytoskeleton in SMC of aganglionic bowel has been shown. Sarcoglycan subcomplex (SG) may support the development and maintenance of muscle cells. We examined the SG subunit expression in colonic aganglionic and ganglionic specimens obtained from patients with HD. Full-thickness bowel specimens were obtained from six patients with HD. Six normal colon specimens were used as controls. Immunofluorescent analysis and reverse transcriptase polymerase chain reaction evaluation were performed for alpha-, beta-, gamma-, delta- and epsilon-SG. In control colon, the indirect immunofluorescence showed a strong staining pattern of beta- gamma- delta- and epsilon-SG while a weak positivity of alpha-SG was recorded. In aganglionic bowel, immunofluorescence intensity values documented a significant lack of epsilon-SG while an enhanced alpha-SG, coupled to a loss of epsilon-SG, was recorded in ganglionic bowel in HD-affected patients. Our observations underscore the assumption that non-neuronal elements of the colon might play a key role in the pathogenesis of HD and loss of epsilon-SG might critically alter the cytoskeleton in the aganglionic bowel segment. Up-regulation of alpha-SG is probably an acquired phenomenon to reinforce the sarcolemma and to perform a forceful contraction in dilated ganglionic HD-affected colon, related to chronic pseudo-obstruction, contributing to the intestinal dysmotility that persists in 20% of patients after resection of the aganglionic bowel.