Herlyn–Werner–Wunderlich syndrome: An “early” onset case report and review of Literature

AbstractHerlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital mullerian anomaly consisting of uterus didelphys, hemivaginal septum, and unilateral renal agenesis [1,2]. Most authors reported cases of Herlyn–Werner–Wunderlich syndrome with prepuberal or postpuberal onset with cyclical abdominal pain and a vaginal mass (3–8). Only six cases are reported in Literature with early onset of this syndrome under 5 years (9–14). Our case is about 3 years old girl, with all the features of this syndrome who came to our attention for lower abdominal mass. The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment. The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis. It is common, in fact, an error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. For all these reasons, our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cystoscopy and vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis

The flow of plasma in the solar terrestrial environment

The overall goal of our NASA Theory Program was to study the coupling, time delays, and feedback mechanisms between the various regions of the solar-terrestrial system in a self-consistent, quantitative manner. To accomplish this goal, it will eventually be necessary to have time-dependent macroscopic models of the different regions of the solar-terrestrial system and we are continually working toward this goal. However, with the funding from this NASA program, we concentrated on the near-earth plasma environment, including the ionosphere, the plasmasphere, and the polar wind. In this area, we developed unique global models that allowed us to study the coupling between the different regions. These results are highlighted in the next section. Another important aspect of our NASA Theory Program concerned the effect that localized 'structure' had on the macroscopic flow in the ionosphere, plasmasphere, thermosphere, and polar wind. The localized structure can be created by structured magnetospheric inputs (i.e., structured plasma convection, particle precipitation or Birkland current patterns) or time variations in these input due to storms and substorms. Also, some of the plasma flows that we predicted with our macroscopic models could be unstable, and another one of our goals was to examine the stability of our predicted flows. Because time-dependent, three-dimensional numerical models of the solar-terrestrial environment generally require extensive computer resources, they are usually based on relatively simple mathematical formulations (i.e., simple MHD or hydrodynamic formulations). Therefore, another goal of our NASA Theory Program was to study the conditions under which various mathematical formulations can be applied to specific solar-terrestrial regions. This could involve a detailed comparison of kinetic, semi-kinetic, and hydrodynamic predictions for a given polar wind scenario or it could involve the comparison of a small-scale particle-in-cell (PIC) simulation of a plasma expansion event with a similar macroscopic expansion event. The different mathematical formulations have different strengths and weaknesses and a careful comparison of model predictions for similar geophysical situations provides insight into when the various models can be used with confidence

Spin and charge orderings in the atomic limit of the U-V-J model

In this paper we study a generalization of the 1D Hubbard model by considering density-density and Ising-type spin-spin nearest neighbor (NN) interactions, parameterized by $V$ and $J$, respectively. We present the T=0 phase diagram for both ferro ($J>0$) and anti-ferro ($J<0$) coupling obtained in the narrow-band limit by means of an extension to zero-temperature of the transfer-matrix method. Based on the values of the Hamiltonian parameters, we identify a number of phases that involve orderings of the double occupancy, NN density and spin correlations, being these latter very fragile.Comment: 4 pages, 2 figures, SCES2011 Proceeding

Using Online Role-playing Games for Entrepreneurship Training

This edited collection of chapters explores the application, potential and challenges of game-based learning and gamification across multiple disciplines and sectors, including psychology, education, business, history, languages and the ..

The High Arctic in Extreme Winters: Vortex, Temperature, and MLS and ACE-FTS Trace Gas Evolution

The first three Canadian Arctic Atmospheric Chemistry Experiment (ACE) Validation Campaigns at Eureka (80° N, 86° W) were during two extremes of Arctic winter variability: Stratospheric sudden warmings (SSWs) in 2004 and 2006 were among the strongest, most prolonged on record; 2005 was a record cold winter. New satellite measurements from ACE-Fourier Transform Spectrometer (ACE-FTS), Sounding of the Atmosphere using Broadband Emission Radiometry, and Aura Microwave Limb Sounder (MLS), with meteorological analyses and Eureka lidar and radiosonde temperatures, are used to detail the meteorology in these winters, to demonstrate its influence on transport and chemistry, and to provide a context for interpretation of campaign observations. During the 2004 and 2006 SSWs, the vortex broke down throughout the stratosphere, reformed quickly in the upper stratosphere, and remained weak in the middle and lower stratosphere. The stratopause reformed at very high altitude, above where it could be accurately represented in the meteorological analyses. The 2004 and 2006 Eureka campaigns were during the recovery from the SSWs, with the redeveloping vortex over Eureka. 2005 was the coldest winter on record in the lower stratosphere, but with an early final warming in mid-March. The vortex was over Eureka at the start of the 2005 campaign, but moved away as it broke up. Disparate temperature profile structure and vortex evolution resulted in much lower (higher) temperatures in the upper (lower) stratosphere in 2004 and 2006 than in 2005. Satellite temperatures agree well with Eureka radiosondes, and with lidar data up to 50–60 km. Consistent with a strong, cold upper stratospheric vortex and enhanced radiative cooling after the SSWs, MLS and ACE-FTS trace gas measurements show strongly enhanced descent in the upper stratospheric vortex during the 2004 and 2006 Eureka campaigns compared to that in 2005

Linear modeling of possible mechanisms for parkinson tremor generation

The power of Parkinson tremor is expressed in terms of possibly changed frequency response functions between relevant variables in the neuromuscular system. The derivation starts out from a linear loopless equivalent model of mechanisms for general tremor generation. Hypothetical changes in this model from the substrate of the disease are indicated, and possible ones are inferred from literature about experiments on patients. The result indicates that in these patients tremor appears to have been generated in loops, which did not include the brain area which in surgery usually is inactivated. For some patients in the literature, these loops could involve muscle length receptors, the static sensitivity of which may have been enlarged by pathological brain activity

Small intestine contrast ultrasonography vs computed tomography enteroclysis for assessing ileal Crohn's disease

Aim: To compare computed tomography enteroclysis (CTE) vs small intestine contrast ultrasonography (SICUS) for assessing small bowel lesions in Crohn's disease (CD), when using surgical pathology as gold standard. Methods: From January 2007 to July 2008, 15 eligible patients undergoing elective resection of the distal ileum and coecum (or right colon) were prospectively enrolled. All patients were under follow-up. The study population included 6 males and 9 females, with a median age of 44 years (range: 18-80 years). Inclusion criteria: (1) certain diagnosis of small bowel requiring elective ileo-colonic resection; (2) age between 18-80 years; (3) elective surgery in our Surgical Unit; and (4) written informed consent. SICUS and CTE were performed ≤ 3 mo before surgery, followed by surgical pathology. The following small bowel lesions were blindly reported by one sonologist, radiologist, surgeon and histolopathologist: disease site, extent, strictures, abscesses, fistulae, small bowel dilation. Comparison between findings at SICUS, CTE, surgical specimens and histological examination was made by assessing the specificity, sensitivity and accuracy of each technique, when using surgical findings as gold standard. Results: Among the 15 patients enrolled, CTE was not feasible in 2 patients, due to urgent surgery in one patients and to low compliance in the second patient, refusing to perform CTE due to the discomfort related to the naso-jejunal tube. The analysis for comparing CTE vs SICUS findings was therefore performed in 13 out of the 15 CD patients enrolled. Differently from CTE, SICUS was feasible in all the 15 patients enrolled. No complications were observed when using SICUS or CTE. Surgical pathology findings in the tested population included: small bowel stricture in 13 patients, small bowel dilation above ileal stricture in 10 patients, abdominal abscesses in 2 patients, enteric fistulae in 5 patients, lymphnodes enlargement (&gt; 1 cm) in 7 patients and mesenteric enlargement in 9 patients. In order to compare findings by using SICUS, CTE, histology and surgery, characteristics of the small bowel lesions observed in CD each patient were blindly reported in the same form by one gastroenterologistsonologist, radiologist, surgeon and anatomopathologist. At surgery, lesions related to CD were detected in the distal ileum in all 13 patients, also visualized by both SICUS and CTE in all 13 patients. Ileal lesions &gt; 10 cm length were detected at surgery in all the 13 CD patients, confirmed by SICUS and CTE in the same 12 out of the 13 patients. When using surgical findings as a gold standard, SICUS and CTE showed the exactly same sensitivity, specificity and accuracy for detecting the presence of small bowel fistulae (accuracy 77% for both) and abscesses (accuracy 85% for both). In the tested CD population, SICUS and CTE were also quite comparable in terms of accuracy for detecting the presence of small bowel strictures (92% vs 100%), small bowel fistulae (77% for both) and small bowel dilation (85% vs 82%). CONCLUSION: In our study population, CTE and the non-invasive and radiation-free SICUS showed a comparable high accuracy for assessing small bowel lesions in CD

A CASE OF APPENDIX CARCINOID IN A PEDIATRIC PATIENT. A SUBTILE CLINICAL PRESENTATION

The appendix carcinoid, although rare (with an incidence of 0,08%), is the more frequent gastrointestinal tumor in children and teen. In most cases these tumors cannot be diagnosed or they can be incidentally found during a surgical exploration for acute abdomen. Only in few cases there are symptoms of a carcinoid's syndrome (flushing, diarrhea and wheezing). In this work we present the manegement of a patient with an appendix carcinoid revealed during an appendicectomy performed for a peritonitis caused by a perforated appendicitis, underlining the importance of diagnosis and long term follow-up

choledochal cyst our experience in a single case by laparoscopic approach

Introduction Choledochal cysts are disproportionate dilatations of the biliary system for the presence of a congenital malformation, the persistence of the common biliary pancreatic channel. Complete excision of the cyst is the best treatment strategy to avoid long-term complications especially malignant transformation, recurrent cholangitis and gallstones. We present a clinical case treated at our center with minimally invasive surgery. Case Report A female patient was admitted to our center at the age of 3 years, with right hypocondrial pain, followed by jaundice, vomiting and recurrent fever. No abdominal mass present. Abdominal ultrasound was performed and also Magnetic resonance cholangiopancreatography. According to the Todani classification modified by Alonso-Lej classification we identified a type I with three gallonstones. She underwent laparoscopic cyst excision and hepatico-jejunostomy Roux-en-Y with perianastomotic drainage positioned. No early and late postoperative complications after 1 year of follow-up. Discussion and Conclusions Choledochal cysts can present at different ages with variable symptoms. Common presentations include abdominal pain, jaundice, and right upper quadrant mass and are most common seen in pediatric patients. Associated congenital anomalies of biliary tract may be present. Most cases of choledochal cyst disease have type I and IV-A cysts. If left untreated, choledochal cysts have an increased risk of malignant transformation. Early surgical excision and restoration of biliary tract continuity is mandatory, whatever the symptom severity to avoid long term complications whenever possible. Currently the gold standard treatment is the mini invasive surgery, in fact the advantages of this technique is the intraoperative visualization of deeper structures, decreased postoperative pain, shorter hospital stay, improved cosmetic result and decreased postoperative ileus. However, these cases remain reserved for highly specialized surgeons with a thorough understanding of hepatobiliary anatomy and minimally invasive techniques. Finally, limited case series of robotic pediatric choledochal cysts resection and reconstruction have been reported with acceptable outcomes, although more studies are needed before widespread acceptance and implementation of this technique in pediatric age

partial congenital bowel obstruction by duodenal atresia windsocks type case report

Introduction Duodenal atresia Windsocks type is a rare condition of congenital bowel obstruction. Thanks to recent technological advancements of prenatal diagnosis it is possible to make a diagnosis of duodenal atresia with high degree of certainty through the radiological sign of "double bubble", but up to date it is not yet possible to identify the type of duodenal atresia. We report the case of a patient with prenatal diagnosis of "double bubble". The patient had no other concomitant malformations. Case Report The patient came to our attention after prenatal ultrasound that showed a picture of double bubble. At the 27th week of gestation we performed fetal MRI that confirmed the US pattern of double bubble but it did not identify with certainty the type of duodenal atresia. At birth the patient underwent GI rx examination that showed a picture of partial duodenal obstruction compatible with the Windsocks type. On the following day, we performed endoscopy which showed the presence of duodenal membrane, so the patient underwent surgical treatment with a longitudinal duodenal incision in order to treat the wind-sock membrane. After one month a further Upper-GI rx examination showed a regular transit of the contrast. Four months after the first operation the patient underwent new surgical treatment for bowel obstruction by adhesions. The operation was successful and the patient had a complete recover.Conclusions Patients with prenatal diagnosis of "double bubble" require a multidisciplinary approach for proper clinical management. Unfortunately it is not currently possible to identify with certainty by prenatal ultrasound the type of duodenal atresia, but in case of incomplete bowel occlusion , the possibility of an atresia Windsocks type should always be considered, especially for setting the right surgical approach