Not just an upset stomach: gastric perforation in a patient with anorexia nervosa.

Gastric perforation in patients with anorexia nervosa is a rare entity associated with high morbidity and mortality [Norris in Int J Eat Disord 49:216-237, 2016]. In cases reported in the literature, the perforation was often preceded by a binge episode, and the subsequent clinical presentation was rapid and acutely deteriorating with a fatality rate as high as 80% [Norris in Int J Eat Disord 49:216-237, 2016, Pitre in J Med Case Rep 15:61, 2021]. We present a case of gastric perforation in the context of restrictive anorexia nervosa unique both for the absence of a premorbid binge episode as well as delayed clinical manifestations of medical distress, leading to abdominal compartment syndrome.Level IV Evidence obtained from multiple time series analysis such as case studies.

Umbilical hernia causing ventriculoperitoneal shunt dysfunction: illustrative cases.

BACKGROUND: When ventriculoperitoneal (VP) shunts and umbilical hernias coexist in the same patient, unique complications can occur. Typically, these are readily identified problems such as cerebrospinal fluid (CSF) fistulas or entrapment of the peritoneal catheter in the hernia. The authors present cases of two children whose VP shunt dysfunction resolved after repair of their umbilical hernias. OBSERVATIONS: The authors present two cases of infant patients with shunted hydrocephalus and umbilical hernias. In both cases, the patients presented with distal shunt malfunctions not due to infection. Their shunt function improved once the umbilical hernia was repaired by pediatric surgery. Neither has required shunt revision since umbilical hernia repair. LESSONS: Although there are case reports of VP shunts eroding through the umbilicus, developing CSF fistulas, or becoming trapped inside umbilical hernias, there is no case of VP shunt dysfunction caused by just the presence of an umbilical hernia. The authors suspect that the catheter may intermittently enter and exit the hernia. This may cause intermittent obstruction of the distal catheter, or inflammation in the hernia may occur that limits CSF absorption.

Capsaicin Causes Vasorelaxation of Rat Aorta through Blocking of L-type Ca2+ Channels and Activation of CB1 Receptors.

The aim of this work was to determine whether Capsaicin may exert a vascular regulation through the activation of CB1 and/or CB2 receptors causing vasorelaxation in the rat aorta. Our results show the location of TRPV1 mainly in the endothelial and smooth muscle cells membrane. Nevertheless, Capsaicin caused vasorelaxation of this artery through a mechanism independent of TRPV1, since the specific antagonists Capsazepine and SB-366791 did not block the effect of Capsaicin. Because the significant expression of CB1 and CB2 receptors has been previously reported in the rat aorta, we used antagonists for these two receptors prior to the addition of Capsaicin. In these experiments, we found that the inhibition of CB1 using AM281, decreases the vasorelaxant effect caused by Capsaicin. On the other hand, the vasorelaxant effect is not altered in the presence of the CB2 receptor antagonist AM630. Furthermore, a partial decrease of the effect of Capsaicin was also seen when L-type calcium channels are blocked. A complete block of Capsaicin-induced vasorelaxation was achieved using a combination of Verapamil and AM281. In accordance to our results, Capsaicin-induced vasorelaxation of the rat aorta is neither dependent of TRPV1 or CB2 receptors, but rather it is strongly suggested that a tandem mechanism between inactivation of L-type calcium channels and the direct activation of CB1 receptors is involved. These findings are supported by CB1 docking simulation which predicted a binding site on CB1 receptors for Capsaicin.

Encephalocraniocutaneous Lipomatosis.

A 5-year-old boy presented with worsening headaches for 3 months. On examination, he was found to have a hairless fatty tissue nevus of the scalp (nevus psiloliparus), subcutaneous soft tissue masses on the right side of his face, neck, mandible and right buttock and epibulbar dermoid of the right eye (choristoma) (). Magnetic resonance imaging revealed a large suprasellar mass, which was debulked and found to be a pilocytic astrocytoma. Testing was not performed for the BRAF/KIAA1549 fusion or BRAFV600E mutation. Seven years later, he was started on adjuvant chemotherapy for gradual tumor progression. Over the ensuing 3 years, he had further disease progression despite treatment with 3 frontline chemotherapy regimens: vinblastine, carboplatin/vincristine, and irinotecan/bevacizumab. Targeted sequencing of tissue from the right gluteal mass, revealed a mosaic activating FGFR1 c.1966A>G (p.Lys656Glu) mutation, absent in normal left gluteal tissue, confirming the diagnosis of encephalocraniocutaneous lipomatosis (ECCL), belonging to the family of RASopathies (including neurofibromatosis type I, Noonan syndrome, Costello syndrome), with constitutive activation of the mitogen-activated protein kinase (MAPK) pathway, and an increased risk of developing neoplasms. He was started on trametinib, a MEK inhibitor, off-label, targeting the MAPK pathway downstream from FGFR1, with stable tumor size at last follow-up, after 6 months on therapy.

ACPA and JWH-133 modulate the vascular tone of superior mesenteric arteries through cannabinoid receptors, BKCa channels, and nitric oxide dependent mechanisms.

BACKGROUND: Some cannabinoids, a family of compounds derived from Cannabis sativa (marijuana), have previously shown vasodilator effects in several studies, a feature that makes them suitable for the generation of a potential treatment for hypertension. The mechanism underlying this vasodilator effect in arteries is still controversial. In this report, we explored how the synthetic cannabinoids ACPA (CB1-selective agonist) and JWH-133 (CB2-selective agonist) regulate the vascular tone of rat superior mesenteric arteries. METHODS: To screen the expression of CB1 (Cannabinoid receptor 1) and CB2 (Cannabinoid receptor 2) receptors in arterial rings or isolated smooth muscle cells obtained from the artery, immunocytochemistry, immunohistochemistry, and confocal microscopy were performed. In addition, the effects on vascular tone induced by the two cannabinoids were tested in isometric tension experiments in rings obtained from superior mesenteric arteries. The participation of voltage and calcium-activated potassium channel of big conductance (BKCa) and the role of nitric oxide (NO) release on the vascular effects induced by ACPA and JWH-133 were tested. RESULTS: CB1 and CB2 receptors were highly expressed in the rat superior mesenteric artery, in both smooth muscle and endothelium. The vasodilation effect shown by ACPA was endothelium-dependent through a mechanism involving CB1 receptors, BKCa channel activation, and NO release; meanwhile, the vasodilator effect of JWH-133 was induced by the activation of CB2 receptors located in smooth muscle and by a CB2 receptor-independent mechanism inducing NO release. CONCLUSIONS: CB1 and CB2 receptor activation in superior mesenteric artery causes vasorelaxation by mechanisms involving BKCa channels and NO release.

Inactivation of GABAA receptor is related to heat shock stress response in organism model Caenorhabditis elegans.

The mechanisms underlying oxidative stress (OS) resistance are not completely clear. Caenorhabditis elegans (C. elegans) is a good organism model to study OS because it displays stress responses similar to those in mammals. Among these mechanisms, the insulin/IGF-1 signaling (IIS) pathway is thought to affect GABAergic neurotransmission. The aim of this study was to determine the influence of heat shock stress (HS) on GABAergic activity in C. elegans. For this purpose, we tested the effect of exposure to picrotoxin (PTX), gamma-aminobutyric acid (GABA), hydrogen peroxide, and HS on the occurrence of a shrinking response (SR) after nose touch stimulus in N2 (WT) worms. Moreover, the effect of HS on the expression of UNC-49 (GABAA receptor ortholog) in the EG1653 strain and the effect of GABA and PTX exposure on HSP-16.2 expression in the TJ375 strain were analyzed. PTX 1 mM- or H2O2 0.7 mM-exposed worms displayed a SR in about 80 % of trials. GABA exposure did not cause a SR. HS prompted the occurrence of a SR as did PTX 1 mM or H2O2 0.7 mM exposure. In addition, HS increased UNC-49 expression, and PTX augmented HSP-16.2 expression. Thus, the results of the present study suggest that oxidative stress, through either H2O2 exposure or application of heat shock, inactivates the GABAergic system, which subsequently would affect the oxidative stress response, perhaps by enhancing the activity of transcription factors DAF-16 and HSF-1, both regulated by the IIS pathway and related to hsp-16.2 expression.

The effects of the addition of a pediatric surgery fellow on the operative experience of the general surgery resident.

Adding fellows to surgical departments with residency programs can affect resident education. Our specific aim was to evaluate the effect of adding a pediatric surgery (PS) fellow on the number of index PS cases logged by the general surgery (GS) residents. At a single institution with both PS and GS programs, we examined the number of logged cases for the fellows and residents over 10 years [5 years before (Time 1) and 5 years after (Time 2) the addition of a PS fellow]. Additionally, the procedure related relative value units (RVUs) recorded by the faculty were evaluated. The fellows averaged 752 and 703 cases during Times 1 and 2, respectively, decreasing by 49 (P = 0.2303). The residents averaged 172 and 161 cases annually during Time 1 and Time 2, respectively, decreasing by 11 (P = 0.7340). The total number of procedure related RVUs was 4627 and 6000 during Times 1 and 2, respectively. The number of cases logged by the PS fellows and GS residents decreased after the addition of a PS fellow; however, the decrease was not significant. Programs can reasonably add an additional PS fellow, but care should be taken especially in programs that are otherwise static in size.

Pancreaticoduodenectomy for choledochal cyst.

BACKGROUND/AIMS: Choledochal cyst (CDC) is a rare congenital cystic dilatation of the biliary system that can affect children and adults. Standard treatment for CDC includes surgical excision of the cystic bile duct with restoration of bilioenteric continuity by Roux-en-Y hepaticojejunostomy. In rare situations, however, surgical excision of a CDC may require pancreaticoduodenectomy (PD). The goal of this study was to identify instances where a PD may be deemed necessary. METHODOLOGY: We retrospectively reviewed the medical records of all patients treated for CDC at our institution from January 1988 through December 2011. From this cohort, patients who underwent PD were reviewed in detail. Data on preoperative parameters, operative details, cyst type, pathologic diagnosis, and postoperative course were collected. RESULTS: During the 24-year period, 59 patients with CDC were identified and four (7%) underwent formal PD. Final pathology revealed that none of the resected specimens harbored malignancy and all patients are well at follow-up. CONCLUSION: While the standard surgical care for CDC remains complete cyst excision with Roux-en-Y hepaticojejunostomy, there may be particular instances where PD appears more appropriate. While the need for PD in cases of CDC is rare, it is important to consider this possibility when contemplating surgery for CDC.

[Family dysfunction and malnutrition in the elderly]. / Disfunción familiar y desnutrición en el anciano.

BACKGROUND: Malnutrition is highly prevalent in the elderly. It is related to biological, functional and psychosocial aging factors. The objective was to investigate the association between family dysfunction (FD) and malnutrition in the elderly. METHODS: Cross sectional study. Through family Apgar Short Nutritional Examination (MNA) were documented FD (Apgar = 7) , and poor nutritional status (= 22 in the MNA) in a sample of 103 elderly over 60 years, excluding those with special diet or any professional prescription disease determinant of weight changes. We calculated odds ratio (OR) with a 95 % confidence interval (CI). The variables were compared using chi-square. RESULTS: There were no differences between groups of family functionality regarding sex, occupation, education, marital status, socioeconomic status and life cycle. The proportion of people with MNA = 22 was 79.4 % (27 of 34) in the FD group vs. 10.1 % (7 of 69) in the group without FD, OR = 11.8 (95 % CI 3.97-36.5), p = 0.001. CONCLUSIONS: The FD is associated with malnutrition in the elderly through MNA, but it is not limited only to malnutrition, it is also associated with diseases such as diabetes, pneumonia and hypertension.

Introducción: la desnutrición tiene una alta prevalencia en el anciano. Se relaciona con factores biológicos, funcionales y psicosociales del envejecimiento. El objetivo es investigar la asociación entre la disfunción familiar (DF) y la desnutrición en el anciano. Métodos: estudio transversal analítico. Mediante Apgar familiar y examen nutrimental abreviado (MNA) se documentaron DF (Apgar = 7) y estado nutricional inadecuado (= 22 en el MNA) en una muestra de 103 ancianos mayores de 60 años, de los cuales quedaron excluidos aquellos con alimentación especial por prescripción profesional o cualquier enfermedad condicionante de cambios ponderales. Se calculó razón de momios (RM) con un intervalo de confianza (IC) de 95 %. Las variables se compararon mediante2. Resultados: no hubo diferencias entre los grupos de funcionalidad familiar respecto a sexo, ocupación, escolaridad, estado civil, estrato socioeconómico y ciclo vital. La proporción de ancianos con MNA = 22 fue de 79.4 % (27 de 34) en el grupo con DF frente a 10.1 % (7 de 69) en el grupo sin DF, RM = 11.8 (IC 95 % 3.97-36.5), p = 0.001. Conclusión: la DF se asocia a estado nutricional inadecuado en el anciano mediante MNA; sin embargo, no es exclusiva de la desnutrición, también se asocia a enfermedades como diabetes, neumonía e hipertensión.

Gastric tube-pericardial fistula: a remote complication of esophageal replacement for long gap esophageal atresia.

Several short and long-term complications of esophageal replacement have been described in the literature. We report the case of a gastric tube-pericardial fistula occurring 20 years after initial repair of long gap esophageal atresia with a reversed gastric tube.

Multivariate model for predicting recurrence in congenital diaphragmatic hernia.

PURPOSE: Risk factors that predispose children with congenital diaphragmatic hernia (CDH) to recurrence remain poorly defined. We report a large series of recurrent CDH and ask whether prenatal patient factors or postnatal treatment variables better predict recurrence. METHODS: Two hundred thirty-eight neonates with unilateral CDH underwent repair from 1990 to 2006. Data were assessed by chi(2) and Mann-Whitney U tests. Multivariate regression identified independent predictors of recurrence. Statistical significance was set at P < .05. RESULTS: We identified 24 recurrences (10%). Median time from repair to recurrence diagnosis was 4.9 months. Patients with recurrence were older (P = .02) and more often required abdominal wall patches at initial repair (P = .01) compared to nonrecurrence patients. Postoperative length of stay (LOS) after initial repair (P < .01) and morbidity (P = .01) were greater in recurrence patients. Use of diaphragm patch at initial repair was greater in patients with recurrence but only approached statistical significance (P = .05). Only 2 variables independently predicted recurrence by multivariate regression as follows: abdominal (not diaphragm) wall patch during initial repair (odds ratio [OR] 3.50; P = .04) and postoperative LOS (OR, 1.012; P = .01). CONCLUSION: Neonates at risk for CDH recurrence are better identified by postnatal treatment variables than by prenatal patient factors. Although age at repair and diaphragm patch use are greater in recurrence patients, the only factors to independently predict recurrence were postoperative LOS and abdominal wall patch use. These data can help optimize follow-up regimens.

A practical algorithm for accurate diagnosis and treatment of perinatally identified biliary ductal dilation: three cases that underscore the importance of an individualised approach.

Choledochal cysts (CDCs) identified during the prenatal or immediate postnatal period are unusual with a majority being identified in older children and adults. Although authors advocate surgical resection of these presumed CDCs with reconstruction, there has been no agreement on the optimal timing of such an intervention when the initial diagnosis of CDC is made prenatally or during the newborn period. We present the cases of three children with biliary ductal dilation who were presumed to have CDC by ultrasound and/or magnetic resonance imaging during the perinatal period and who ultimately required different approaches to treatment. The eventual findings in these cases support a period of close postnatal observation followed by carefully planned individualised therapy.

Percutaneous distal perfusion of the lower extremity after femoral cannulation for venoarterial extracorporeal membrane oxygenation in a small child.

Femoral cannulation in pediatric patients requiring extracorporeal membrane oxygenation (ECMO) is commonly associated with distal limb ischemia. Authors have previously reported successful lower limb perfusion using various open techniques to cannulate a distal lower extremity artery at the time of initial ECMO cannulation. These procedures include open femoral artery antegrade cannulation and distal posterior tibial artery retrograde cannulation in older children and adults. Such approaches require ample vessel diameters to accommodate an arteriotomy and catheter insertion and, therefore, are of limited use in smaller children. We hypothesized that after femoral artery cannulation for ECMO, a percutaneous technique of distal limb perfusion might offer unique advantages when treating lower extremity ischemia in small pediatric patients. We report a technique for percutaneous antegrade cannulation in a 4-year-old patient shortly after her primary cannulation for venoarterial ECMO via the femoral artery.

Button-loop feeding jejunostomy.

INTRODUCTION: Post-pyloric feeding via a surgical jejunostomy allows for enteral nutrition in patients that cannot receive oral or gastric feeding. Regardless of the technique used to create a jejunostomy, complications such as tube dislodgement, jejunostomy closure, or bowel obstruction can occur. SURGICAL TECHNIQUE: We present a simple and efficient jejunostomy technique that does not require a sewn anastomosis and employs an easily exchangeable feeding button.

Preclinical evaluation of the therapeutic effect of adenoviral vectors in human papillomavirus-dependent neoplasias.

Gene therapy with adenoviral vectors can eliminate neoplasic cells through selective replication and/or through pro-apoptotic, immunogenic or suicide gene expression. However, an adenoviral vector may provide anti-cancerous effects even in the absence of replication or therapeutic gene expression. The present study evaluates the therapeutic effects caused by the administration of an adenoviral vector, alone, in HPV-dependent neoplasias (HPV-N). In vivo trials were carried out in two HPV-N mouse models. One model was immunocompetent and the other was immunodeficient. In both models, the effect of intratumoral administration of saline solution (PBS) was compared with administration of an adenoviral vector that had no replicative capacity or therapeutic gene (Ad-BGal). In the immunocompetent mice, Ad-BGal adenoviral vector administration significantly reduced tumor growth, compared with PBS. No differences were observed in the immunodeficient mice. In conclusion, the present study lends support to the use of adenoviral vectors in HPV-N treatment since they are capable of generating an antitumoral effect in immunocompetent individuals, even in the absence of a therapeutic gene or viral vector replication.

Blink reflex, H-reflex and nerve-conduction alterations in leprosy patients.

Peripheral nerve lesions are the most important cause of disability in leprosy patients. Electrophysiological studies are used in the diagnosis and prognosis of neuropathy. Nerve conduction is the most frequently used electrophysiological test method to detect neuropathy, although it evaluates only a part of the peripheral nervous system. Blink reflex and H-reflex are electrophysiological tests which evaluate facial and trigeminal nerve function. This study determined the frequencies of blink reflex, H-reflex and motor and sensory nerve conduction alterations in twenty five heterogeneous, clinic patients with lepromatous leprosy and a control group of 20 healthy subjects. Study results showed a decrease in motor and sensory nerve conduction in 40% and 30%, respectively. In blink reflex (BR), right R1 was altered in latency in 20% of patients, left R1 in 20%, right ipsilateral R2 in 16%, left ipsilateral R2 in 20%, and right and left contralateral R2 were altered in 32% of patients. There was an absence of H-reflex in 16% (n = 4) and prolonged latency in 4% (n = 1).

UVB photoprotection with antioxidants: effects of oral therapy with d-alpha-tocopherol and ascorbic acid on the minimal erythema dose.

Ultraviolet radiation absorption is responsible for the production of free radicals in damaged cells. This side effect may be neutralized using antioxidant substances. It has been reported that ascorbic acid and d-alpha-tocopherol scavenge reactive oxygen species. In a single-blind controlled clinical trial we studied 45 healthy volunteers divided into three groups. Group 1 received d-alpha-tocopherol 1,200 I.U. daily; Group 2 ascorbic acid 2 g daily and Group 3 ascorbic acid 2 g plus d-alpha-tocopherol 1,200 I.U. daily. Treatment was sustained for one week. Before and after treatment, the minimal erythema dose was determined in all participants. The results show that the median minimal erythema dose increased from 60 to 65 mJ/cm2 in Group 1 and from 50 to 70 mJ/cm2 in Group 3. No modifications were observed in Group 2. We conclude that d-alpha-tocopherol prescribed in combination with ascorbic acid produces the best photoprotective effect.