Chagasic megacolon and large bowel neoplasms: case series and literature review

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Maxwel Capsy Boga Ribeiro

2011-12-01

Full Text Available There is a clear association between chagasic megaesophagus and the esophageal cancer. On the other hand, the association between chagasic megacolon and intestinal neoplasm is uncommon. There are only a few cases described in literature. We selected two cases of colorectal adenocarcinoma associated with adenoma from 2000 to 2011, which are added to the four patients already described by this group. The mean age of the patients, was 68.5 years. Both had been submitted to surgical resection of the neoplasm. Survival rates ranged and were directly related to tumor staging at the time of diagnosis. In this context, we report our case series and reviwed the corresponding literature, especially the clinical and epidemiological aspects of this rare association.Há uma clara associação entre megaesôfago por doença de Chagas e o câncer esofágico. Ao contrário, tal relação, entre megacólon chagásico e neoplasias do intestino grosso é, reconhecidamente, incomum. Existem poucos casos relatados na literatura. Destacamos, entre 2000 e 2011, dois casos, sendo ambos adenocarcinomas colorretais e associados a adenomas, que se somam aos outros quatro já descritos por este grupo. A média de idade dos pacientes, foi de 68,5 anos. Todos foram submetidos à ressecção cirúrgica da neoplasia. A sobrevida foi variável e diretamente relacionada ao estádio do tumor no momento do diagnóstico. Dentro desse contexto, relatamos essa série de casos e revisamos a literatura correlata, com relação aos aspectos clínicos e epidemiológicos dessa rara associação.

Megacólon tóxico fatal por cytomegalovirus em paciente com retocolite ulcerativa idiopática: relato de caso e revisão de literatura Fatal toxic megacolon due to cytomegalovirosis in a patient with ulcerative colitis: case report and review

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Sérgio Ossamu IOSHII

2002-04-01

Full Text Available Racional - O megacólon tóxico é complicação grave e pouco freqüente na retocolite ulcerativa idiopática. Ocorre, geralmente, em pacientes cuja doença ativa é resistente ao tratamento clínico e, em algumas situações, o fator desencadeante é desconhecido. A infecção pelo cytomelogalovirus em seres humanos em geral é evento subclínico; entretanto, em pacientes imunocomprometidos a primoinfecção ou mesmo a reativação de infecção latente pode ter amplas repercussões clínicas, uma das quais o desencadeamento de megacólon tóxico. Objetivo - Descrever um caso de megacólon tóxico fatal por cytomegalovirose em paciente com retocolite ulcerativa idiopática. Paciente - Masculino, 38 anos, com clínica de diarréia e perda ponderal cuja investigação mostrou tratar-se de retocolite ulcerativa idiopática e para a qual foi instituída terapêutica imunossupressora vigorosa. Resultados - Evoluiu com quadro clínico de megacólon tóxico, sendo submetido a colectomia total. Complicações clínicas culminaram no óbito pós-operatório e o estudo anatomopatológico do cólon revelou doença ativa, associada a ulcerações confluentes na base das quais foram observadas células com alterações características da cytomegalovirose. Conclusão - A cytomegalovirose deve ser considerada como um dos agentes causadores de megacólon tóxico em retocolite ulcerativa.Background - The toxic megacolon is a rare and severe complication of ulcerative colitis. In general it complicates patients with active colitis that are resistant to clinical treatment and, in some cases, the developing factor is unknown. Cytomegalovirus infection in humans in general is a subclinical condition. However, in patients with immunodeficiency the primary infection or the reactivation of latent infection could have enormous clinical effects. One of these effects is the toxic megacolon. Aim - To report a case of fatal toxic megacólon due to cytomegalovirosis in a

Neoplasia no sítio da colostomia de paciente com megacólon chagásico: relato de caso Neoplasia at the site of the colostomy of patient with chagasic megacolon: case report

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Thiago Agostini Braga

2011-06-01

Full Text Available A neoplasia no sítio da colostomia associada ao megacólon chagásico é uma entidade rara. Os autores relatam caso de um paciente com lesão avançada, o qual foi submetido a tratamento cirúrgico e discutem aspectos relacionados a esta afecção.Neoplasia at the site of the colostomy associated with chagasic megacolon is a rare clinical event. Here, the authors report the case of a patient with advanced lesion, who had to undergo surgical treatment, and discuss aspects related to this disease.

Morphometric study of the fibrosis and mast cell count in the circular colon musculature of chronic Chagas patients with and without megacolon Estudo morfométrico da fibrose e do número de mastócitos na muscular circular do cólon de chagásicos crônicos com e sem megacólon

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Simone Wanderley Pinheiro

2003-07-01

Full Text Available A morphometric study of the circular colon musculature was performed, in which the mast cell count was determined and the connective fibrous tissue in this layer was measured. The objective was to gain better understanding of Chagas megacolon morphology and contribute towards the knowledge of fibrosis pathogenesis in Chagas megas. An evaluation was made of 15 distal sigmoid rings from Chagas patients with megacolon (MCC, 15 without megacolon (CSMC and 15 non-Chagas patients (NC. The rings were fixed in formol, embedded in paraffin, and 7mm thick sections were cut and stained using Azan-Heidenhain and Giemsa. The mast cell count and fibrosis were greater in the MCC group than in the CSMC and NC groups (p Com os objetivos de conhecer melhor a morfologia do megacólon chagásico e contribuir para o conhecimento da patogênese da fibrose dos megas, realizou-se estudo morfométrico na muscular circular do cólon, contando-se o número de mastócitos e medindo o conjuntivo fibroso nessa camada. Foram avaliados anéis do sigmóide distal de 15 chagásicos com megacólon (MCC, 15 sem megacólon (CSMC e 15 não chagásicos (NC. Os anéis foram fixados em formol, incluídos em parafina, cortados com 7mm de espessura e corados por Azan-Heidenhain e Giemsa. O número de mastócitos e a fibrose foram maiores no grupo com MCC em relação ao CSMC e NC (p < 0,05; teste de Kruskal-Wallis; não houve diferença significante entre os dois últimos grupos. Diante destes achados, é possível, que haja relação entre mastocitose e fibrose no megacólon chagásico, como já se demonstrou em outras doenças.

Depopulation of interstitial cells of cajal in chagasic megacolon: towards tailored surgery? Depleção de células intersticiais de Cajal no megacólon chagásico: a caminho do tratamento cirúrgico individualizado?

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Sergio Eduardo Alonso Araujo

2010-06-01

Full Text Available BACKGROUND: The mechanism of constipation in patients with Chagasic megacolon remains partially explained. In these patients, it was recently demonstrated a reduction in the population of interstitial cells of Cajal. AIM: To evaluate density of Cajal cells in the surgically resected colon of Chagasic patients in comparison to control patients, and to verify possible association between preoperative and postoperative bowel function of Chagasic patients and colonic cell count. METHOD: Sixteen patients with Chagasic megacolon were operated on. Clinical pre- and post-operative evaluation using the Cleveland Clinic Constipation Score was undertaken. Resected colons were examined. Cajal cells were identified by immunohistochemistry using anti-CD117 antibody. The mean cell number was compared to resected colons from 16 patients with non-obstructive sigmoid cancer. Association between pre-and post-operative constipation scores and cell count for megacolon patients was evaluated using the Pearson coefficient correlation test (r. RESULTS: A reduced number of Cajal cells [cells per field: 2.84 (0-6.6 vs. 9.68 (4.3-13 - pRACIONAL: A fisiopatologia da constipação intestinal nos pacientes portadores de megacólon chagásico permanece parcialmente esclarecida. Recentemente demontrou-se que nesses pacientes, o contingente de células intersticiais de Cajal está reduzido assim como ocorre em outros distúrbios funcionais gastrointestinais. OBJETIVO: Avaliar a densidade de células intersticiais de Cajal no intestino ressecado de pacientes submetidos a tratamento cirúrgico eletivo de megacólon chagásico em comparação com a observada no cólon de pacientes controles, e verificar possível associação entre o grau de constipação intestinal de pacientes com megacólon chagásico no pré e no pós-operatório e o grau de despopulação de células de Cajal. MÉTODO: Dezesseis pacientes com megacólon chagásico foram operados. A avaliação da fun

Surgical treatment of Chagas megacolon. Critical analysis of outcome in operative methods Tratamento cirúrgico do megacólon chagásico análise crítica dos resultados dos métodos operatórios

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Ricardo Luiz Santos Garcia

2008-01-01

Full Text Available PURPOSE: Surgical treatment of chagasic megacolon has suffered innumerable transformations over the years. Poor knowledge of the disease physiopathology is one of the reasons. METHODS: From January 1977 to December 2003, 430 patients were submitted to surgical treatment for chagasic megacolon. Of these procedures, 351 were elective and 79 emergency operations carried out at the University Hospital of Ribeirão Preto. Four elective operations, most frequently used, should be singled out: anterior rectosigmoidectomy (52.71%, left hemicolectomy (18.23%, Duhamel-Haddad operation(15.95%, and total colectomy (5.98%. From the 79 exploratory laparotomies performed on an emergency basis, 53 (67.09% required intestinal resection. From the 430 patients operated upon, 268 (62.33% progressed without recurrence of intestinal constipation, and 71 (15.51% had a recurrence. RESULTS AND DISCUSSION: Based on the data collected, left hemicolectomy had the highest constipation recurrence rate compared to other operating procedures; anterior retosigmoidectomy had less complication episodes and a larger recurrence of intestinal constipation in comparison to the Duhamel-Haddad operation. Emergency operations, mainly for the treatment of volvulus and fecaloma, presented high morbidity and mortality and required extensive intestinal resections, stomas and reoperations.INTRODUÇÃO: O tratamento cirúrgico do megacólon chagásico tem passado por sucessivas modificações ao longo do tempo. A multiplicidade das operações é explicada pelo conhecimento ainda incompleto da fisiopatologia da doença, MÉTODOS: No período de janeiro de 1977 a dezembro de 2003, 430 pacientes chagásicos foram submetidos a tratamento cirúrgico para o megacólon no Hospital das Clínicas de Ribeirão Preto. Foram realizadas 351 operações eletivas e 79 de urgência. Quatro tipos de operações realizadas em caráter eletivo mereceram destaque por terem sido as mais utilizadas

Lineage Analysis of Circulating Trypanosoma cruzi Parasites and Their Association with Clinical Forms of Chagas Disease in Bolivia

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del Puerto, Ramona; Nishizawa, Juan Eiki; Kikuchi, Mihoko; Iihoshi, Naomi; Roca, Yelin; Avilas, Cinthia; Gianella, Alberto; Lora, Javier; Gutierrez Velarde, Freddy Udalrico; Renjel, Luis Alberto; Miura, Sachio; Higo, Hiroo; Komiya, Norihiro; Maemura, Koji; Hirayama, Kenji

2010-01-01

Background The causative agent of Chagas disease, Trypanosoma cruzi, is divided into 6 Discrete Typing Units (DTU): Tc I, IIa, IIb, IIc, IId and IIe. In order to assess the relative pathogenicities of different DTUs, blood samples from three different clinical groups of chronic Chagas disease patients (indeterminate, cardiac, megacolon) from Bolivia were analyzed for their circulating parasites lineages using minicircle kinetoplast DNA polymorphism. Methods and Findings Between 2000 and 2007, patients sent to the Centro Nacional de Enfermedades Tropicales for diagnosis of Chagas from clinics and hospitals in Santa Cruz, Bolivia, were assessed by serology, cardiology and gastro-intestinal examinations. Additionally, patients who underwent colonectomies due to Chagasic magacolon at the Hospital Universitario Japonés were also included. A total of 306 chronic Chagas patients were defined by their clinical types (81 with cardiopathy, 150 without cardiopathy, 100 with megacolon, 144 without megacolon, 164 with cardiopathy or megacolon, 73 indeterminate and 17 cases with both cardiopathy and megacolon). DNA was extracted from 10 ml of peripheral venous blood for PCR analysis. The kinetoplast minicircle DNA (kDNA) was amplified from 196 out of 306 samples (64.1%), of which 104 (53.3%) were Tc IId, 4 (2.0%) Tc I, 7 (3.6%) Tc IIb, 1 (0.5%) Tc IIe, 26 (13.3%) Tc I/IId, 1 (0.5%) Tc I/IIb/IId, 2 (1.0%) Tc IIb/d and 51 (25.9%) were unidentified. Of the 133 Tc IId samples, three different kDNA hypervariable region patterns were detected; Mn (49.6%), TPK like (48.9%) and Bug-like (1.5%). There was no significant association between Tc types and clinical manifestations of disease. Conclusions None of the identified lineages or sublineages was significantly associated with any particular clinical manifestations in the chronic Chagas patients in Bolivia. PMID:20502516

Protective human leucocyte antigen haplotype, HLA-DRB1*01-B*14, against chronic Chagas disease in Bolivia.

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Florencia del Puerto

Full Text Available BACKGROUND: Chagas disease, caused by the flagellate parasite Trypanosoma cruzi affects 8-10 million people in Latin America. The mechanisms that underlie the development of complications of chronic Chagas disease, characterized primarily by pathology of the heart and digestive system, are not currently understood. To identify possible host genetic factors that may influence the clinical course of Chagas disease, Human Leucocyte Antigen (HLA regional gene polymorphism was analyzed in patients presenting with differing clinical symptoms. METHODOLOGY: Two hundred and twenty nine chronic Chagas disease patients in Santa Cruz, Bolivia, were examined by serological tests, electrocardiogram (ECG, and Barium enema colon X-ray. 31.4% of the examinees showed ECG alterations, 15.7% megacolon and 58.1% showed neither of them. A further 62 seropositive megacolon patients who had undergone colonectomy due to acute abdomen were recruited. We analyzed their HLA genetic polymorphisms (HLA-A, HLA-B, MICA, MICB, DRB1 and TNF-alpha promoter region mainly through Sequence based and LABType SSO typing test using LUMINEX Technology. PRINCIPAL FINDINGS: The frequencies of HLA-DRB1*01 and HLA-B*14:02 were significantly lower in patients suffering from megacolon as well as in those with ECG alteration and/or megacolon compared with a group of patients with indeterminate symptoms. The DRB1*0102, B*1402 and MICA*011 alleles were in strong Linkage Disequilibrium (LD, and the HLA-DRB1*01-B*14-MICA*011 haplotype was associated with resistance against chronic Chagas disease. CONCLUSIONS: This is the first report of HLA haplotype association with resistance to chronic Chagas disease.

Prevalência de megas em necrópsias realizadas no triângulo mineiro no período de 1954 a 1988

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Edison Reis Lopes

1989-12-01

Full Text Available Dentre 1708 necrópsias de chagásicos crônicos, de um total de 4690, diagnosticamos 273 megas. Destes o mais freqüente foi o megacólon, seguido pelo megaesôfago, ocupando a associação megacólon e megaesôfago o terceiro lugar. Discutem-se e comparam-se os achados com outros de ordem clinico-epidemiológica e anatomopatológica. Confirmando dados da literatura, nossos achados atuais mostram, que a exemplo do que sucede na cardiopatia chagásica, o megacólon e o megaesôfago predominam no sexo masculino, discutindo-se os fatores que poderiam explicar a razão desse fato.One thousand seven hundred and eight chronic chagasic post-mortem examinations studied from a total of4690 autopsies perfomed at our Institution. Two hundred and seventy-three chagasic had megas. Megacolon was the most frequent, followed by megaesophagus. Megacolon associated with megaesophagus was, the third mostcommom finding. Our data are discussed and compared with the literature. Megacolon and megaesophagus were more prevalent in man, as shown by other workers. Higher parasitemia perhaps could explain this finding.

Surgery in adult Hirschsprung's disease

International Nuclear Information System (INIS)

Lombana, Luis Jorge; Dominguez, Luis Carlos

2007-01-01

Hirschsprung's disease of the adult is an infrequent entity of unknown incidence. Surgery is the treatment of choice, but many available options include techniques applied in newborns and children or those used in other pathologies such as idiopathic megacolon of the adult or Chagasic megacolon. Due to the nature of the disease, the alternatives more frequently used are the anorectal miectomy and the Duhamel's procedure. We decided to present a case of short segment Hirschsprun's disease and another of ultra - short segment in young adults and discuss treatment options, due to the rare number of reports, patients and surgical experiences reported in the literature

Disease: H00247 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available racterized by parathyroid adenoma, gastrinoma, and pituitary adenoma. Gastrinomas are the most common type, leading... ganglion dysfunction, leading to megacolon. MEN4, also referred to as MENX, appe... in association with marfanoid habitus, mucosal neuromas, medullated corneal fibers and intestinal autonomic

Research on Trypanosoma cruzi and Analysis of Inflammatory Infiltrate in Esophagus and Colon from Chronic Chagasic Patients with and without Mega

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Eliângela de Castro Côbo

2012-01-01

Full Text Available To compare parasitism and inflammatory process in esophagus and colon from chronic chagasic patients, immunohistochemistry was carried out to research for T. cruzi and to evaluate the inflammatory infiltrate in the muscular and myenteric plexus in 39 esophagi (20 with and 19 without megaesophagus and 50 colons (25 with and 25 without megacolon. The frequency of T. cruzi in megaesophagus was 20%, and in megacolon it was 4%. No amastigotes were found in organs without mega; considering the total of esophagi (with and without mega, the frequency of T. cruzi would be 10% and 2% in the colon. Myositis and ganglionitis were more frequent and intense in organs with mega compared to those without mega, and in esophagus compared to colon. Qualitatively, inflammatory infiltration in esophagus and colon, with or without mega, was similar, consisting predominantly of T lymphocytes (CD3+, scarce macrophages (CD68+, and rare B lymphocytes (CD20+.

Research on Trypanosoma cruzi and Analysis of Inflammatory Infiltrate in Esophagus and Colon from Chronic Chagasic Patients with and without Mega

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Côbo, Eliângela de Castro; Silveira, Thales Parenti; Micheletti, Adilha Misson; Crema, Eduardo; Adad, Sheila Jorge

2012-01-01

To compare parasitism and inflammatory process in esophagus and colon from chronic chagasic patients, immunohistochemistry was carried out to research for T. cruzi and to evaluate the inflammatory infiltrate in the muscular and myenteric plexus in 39 esophagi (20 with and 19 without megaesophagus) and 50 colons (25 with and 25 without megacolon). The frequency of T. cruzi in megaesophagus was 20%, and in megacolon it was 4%. No amastigotes were found in organs without mega; considering the total of esophagi (with and without mega), the frequency of T. cruzi would be 10% and 2% in the colon. Myositis and ganglionitis were more frequent and intense in organs with mega compared to those without mega, and in esophagus compared to colon. Qualitatively, inflammatory infiltration in esophagus and colon, with or without mega, was similar, consisting predominantly of T lymphocytes (CD3+), scarce macrophages (CD68+), and rare B lymphocytes (CD20+). PMID:22131997

A retrospective study of histopathological findings in 894 cases of megacolon: what is the relationship between megacolon and colonic cancer? Um estudo retrospectivo dos achados histopatológicos em 894 casos de megacólon: qual é a relação entre megacólon e o câncer de cólon?

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Sérgio Britto Garcia

2003-04-01

Full Text Available Patients with megaesophagus (ME have increased prevalence of cancer of the esophagus. In contrast, a higher incidence of colorectal cancer is not observed in patients with megacolon (MC. MC is very common in some regions of Brazil, where it is mainly associated with Chagas disease. We reviewed the pathology records of surgical specimens of all patients submitted for surgical resection of MC in the Hospital das Clínicas of the Faculty of Medicine of Ribeirão Preto (HC-FMRP, from the University of São Paulo. We found that 894 patients were operated from 1952 until 2001 for MC resection. Mucosal ulcers, hyperplasia and chronic inflammation were frequently found, while polyps were uncommon. No patients with MC presented any type of colonic neoplasm. This observation reinforces the hypothesis that MC has a negative association with cancer of the colon. This seems to contradict the traditional concept of carcinogenesis in the colon, since patients with MC presents important chronic constipation that is thought to cause an increase in risk for colon cancer. MC is also associated with other risk factors for cancer of colon, such as hyperplasia, mucosal ulcers and chronic inflammation. In ME these factors lead to a remarkable increase in cancer risk. The study of mucosal cell proliferation in MC may provide new insights and useful information about the role of constipation in colonic carcinogenesis.Pacientes com megaesôfago (ME possuem incidência aumentada de câncer de esôfago. Em contraste, há poucos relatos na literatura de associação entre megacólon (MC e câncer de cólon. O MC é muito comum em algumas regiões do Brasil, e na maioria das vezes, está associado à Doença de Chagas. Nós reavaliamos os arquivos de patologia de peças cirúrgicas de todos os pacientes submetidos à ressecção de MC no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto (HC-FMRP, da Universidade de São Paulo. Encontramos o número de 894

Case Report: Congenital aganglionic megacolon in Nigerian adults ...

African Journals Online (AJOL)

Congenital aganglionic mega colon (Hirschsprung's disease) is a motor disorder in the gut, due to a defect in the craniocaudal migration of the neuroblast originating from the neural crest that occurs during the first twelve weeks of gestation, causing a functional intestinal obstruction, with its attendant complications, ...

Pattern of aganglionic megacolon in Calabar, Nigeria | Archibong ...

African Journals Online (AJOL)

Surgical resection of .the aganglionic segment and anastomosis is the treatment of choice and this procedure decreases mortality and morbidity. Palliative transverse colostomy was considered essential as it protects the anastomosis. Early diagnosis of this disease in our environment can only be achieved by means of a ...

Knowledge of ancient Hindu surgeons on Hirschsprung disease: evidence from Sushruta Samhita of circa 1200-600 BC.

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Raveenthiran, Venkatachalam

2011-11-01

Congenital megacolon is eponymously named after Harold Hirschsprung, who accurately described the clinical features in 1886. Recent research revealed that this condition is perhaps well known for centuries before him. This article is intended to examine if ancient Hindu surgeons knew about congenital megacolon. Sushruta Samhita is an ancient tome of Ayurvedic surgery compiled by Sushruta (circa 1200-600 bc). Passages of interest were identified by browsing the authentic English translation of the compendium. Accuracy of translation was verified by comparing to the original Sanskrit verses with the help of a Sanskrit scholar. A condition called Baddha Gudodaram, described in the Samhita, closely resembles Hirschsprung disease. There are indications that ancient Indians even deciphered the etiology as defective vayu alias vata (nerves). Although the ailment was considered incurable, a palliative operation has been discussed. Descriptive details of the operation match with that of sigmoid colostomy. Evidence from Sushruta Samhita indicates that Hindu surgeons of prehistoric India probably had considerable knowledge about Hirschsprung disease. Further research, corroborating other sources of evidence, is required to confirm this claim. Copyright © 2011 Elsevier Inc. All rights reserved.

Revista de revistas: Ileo paralítico en el hipotiroidismo severo

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Facultad de Medicina Revista

1946-09-01

Full Text Available EI cuadra clasico del hipotiroidismo incluye constipacion severa. frecuentemente asociada con meteorismo, y el abombamiento abdominal por distension flatulent a del intestino. cuando es marcado, debe hacer pensar en esta posibilidad dlagnostica; en el presente articulo el autor da a conacer casos de megacolon con ileo paralitico, desarrollados en el curso de hipotiroidismo severo, que es fundamental reconocer puesto que el acto quirúrgico suele ser desastroso.

Enteropatia chagásica

OpenAIRE

Meneghelli, Ulysses G.

2004-01-01

Involvement of the hollow organs of the digestive apparatus can occur in patients in the chronic phase of Chagas' disease. The basic mechanism is destruction of neurons of the enteric nervous system. Whereas megaesophagus and megacolon are the most notable and most extensively studied expressions of the digestive form of Chagas' disease, involvement of the small intestine (Chagasic enteropathy) is less frequent and less known than involvement of the two above mentioned entities. Chagasic ente...

Enfermedad de Hirschsprung

OpenAIRE

K A Santos-Jasso

2017-01-01

Definición
La enfermedad de Hirschsprung, megacolon congénito o agangliónico, o Aganglionosis Intestinal Congénita, es una anomalía caracterizada por la ausencia congénita de células ganglionares del sistema parasimpático de los piejos intermientéricos de Auerbach, situado entre las capas musculares, circular y longitudinal, y el submucoso de Meissner; con conducción inefectiva del peristaltismo, ocasionando obstrucción funcional (24, 37, 65, 8...

Probiotics and Antibiotic-Associated Diarrhea and Clostridium difficile Infection

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Surawicz, Christina M.

Diarrhea is a common side effect of antibiotics. Antibiotics can cause diarrhea in 5-25% of individuals who take them but its occurrence is unpredictable. Diarrhea due to antibiotics is called antibiotic-associated diarrhea (AAD). Diarrhea may be mild and resolve when antibiotics are discontinued, or it may be more severe. The most severe form of AAD is caused by overgrowth of Clostridium difficile which can cause severe diarrhea, colitis, pseudomembranous colitis, or even fatal toxic megacolon. Rates of diarrhea vary with the specific antibiotic as well as with the individual susceptibility.

SYMPTOMS AND DIAGNOSIS OF HIRSCHSPRUNG’S DISEASE

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Putu Ayu Ines Lassiyani Surya

2014-02-01

Full Text Available Hirschsprung disease is a disease that attacks the human digestive system, mainly in the largeintestine (colon. In this disease, found enlargement of the colon (megacolon, due to the absenceof ganglion cells in the distal intestine. Hirschsprung disease often affects neonates and evenchildren, are often characterized by delays in spending the first meconium, bilious vomiting,abdominal distension. Methods diagnois do for Hirschsprung's disease was confirmed by biopsy,barium enema or contrast enema, and anorectal manometry. Early diagnosis is crucial to conductrapid and appropriate treatment and to prevent complications.

Cytomegalovirus-induced colonic stricture presenting as acute intestinal obstruction in an immunocompetent adult.

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Dinesh, B V; Selvaraju, Karthikeyan; Kumar, Sampath; Thota, Sumath

2013-09-10

Cytomegalovirus (CMV) infection causes significant morbidty and mortality in immunopromised patients. Though it is usually silent in immunocompetent adults, rarely it can cause serious life-threatening complications. Gastrointestinal tract is one of the commonly involved organs, where it produces a spectrum of clinical manifestation ranging from mild non-specific abdominal pain and diarrhoea to severe infection with toxic megacolon and death. We present a 65-year-old immunocompetent male patient admitted with acute colonic obstruction secondary to CMV-induced colonic stricture, highlighting the importance of considering it as a differential diagnosis for colonic obstruction and reviewing its management.

LACTULOSE IN PREOPERATIVE PREPARATION OF CHILDREN WITH DECOMPENSATED TYPE OF CHRONIC COLON STASIS

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I.V. Kirgizov

2009-01-01

Full Text Available This article describes the results of estimation of effectiveness of lactulose (Dupfalac in preoperative preparation of children with decompensated type of chronic colon stasis. Proved, that administration of this medication normalizes such indices of homeostasis as acid-base balance of blood and microbiocenose of colon by 7days. Use of lactulose decreases patient complaints on nausea, vomiting, abdominal pain, weakness in 1.6 times rarely then in group of children using other laxative medications in preoperative preparation.Key words: megacolon syndrome, lactulose, dysbacteriosis, blood acid-base balance.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2009;8(1:68-71

[Dolichomegacolon of the Andes and intestinal volvulus due to altitude].

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Frisancho, Oscar

2008-01-01

Sigmoid volvulus is a frequent cause of emergencies in hospitals in the Andean area, representing more than 50% of all intestinal obstructions. Andean dolichomegacolon (DCMA) and retractile mesocolonitis are the main contributing factors for volvulus. The mesocolonitis nears the proximal and distal segment of the sigmoid handle, favoring its torsion. Copious intake of fermentable food is the precipitating factor for volvulus. The majority of patients are seen during sowing and harvest periods, in which the consumption of this type of food increases. Andean people who live at an altitude of 3,000 m have a larger and thicker colon than coastal residents. We call this acquired characteristic the Andean dolichomegacolon (DCMA). A fiber-rich diet may inhibit the histological phenomenon known as elastogenesis, developing--over the years--the megacolon. Another important factor may be the lower atmospheric pressure in the altitude, and according to Boyle and Mariotte's physical law, the expansion of intraluminal gas may have an influence on intestinal enlargement. DCMA has many special anatomic, clinical, radiological, histological and serological features which make it different from the . chagasic megacolon. Mild emergency procedures may be performed to treat the sigmoid volvulus, such as endoscopic disvolvulation. Changing the colon rotation is helpful in diminishing abdominal pressure and restore complete blood circulation. An emergency surgery treatment must take the patient's general condition and the colon handle condition during surgery as a guiding point. High rates of mortality are found in relation to elderly patients, disease evolution time and stage of intestinal ischemia. Other new therapeutic procedures such as percutaneous sigmoidpexy, laparoscopic sigmoidectomy and mesosigmoplasty are under review, and have precise indications. Wider series are needed to evaluate them better.

Enfermedad de Hirschsprung y apendicitis. Una asociación rara

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C Baeza-Herrera

2016-05-01

Full Text Available La enfermedad de Hirschsprung asociada con apendicitis es una condición poco usual; suman 15 los reportados en la literatura, ya incluido este caso. Se informa el caso de un lactante de 12 meses de edad que ingresó con fiebre, anorexia, distensión abdominal, vómito, evacuaciones hemáticas y antecedente de estreñimiento. Se realizó laparotomía exploradora que reportó apendicitis y megacolon congénito o enfermedad de Hirschsprung. El reporte histopatológico reveló periapendicitis, huevecillos de áscaris lumbricoides, con ausencia de células ganglionares.

MELAS syndrome presenting as an acute surgical abdomen.

Science.gov (United States)

Dindyal, S; Mistry, K; Angamuthu, N; Smith, G; Hilton, D; Arumugam, P; Mathew, J

2014-01-01

MELAS (mitochondrial cytopathy, encephalomyopathy, lactic acidosis and stroke-like episodes) is a syndrome in which signs and symptoms of gastrointestinal disease are uncommon if not rare. We describe the case of a young woman who presented as an acute surgical emergency, diagnosed as toxic megacolon necessitating an emergency total colectomy. MELAS syndrome was suspected postoperatively owing to persistent lactic acidosis and neurological symptoms. The diagnosis was later confirmed with histological and genetic studies. This case highlights the difficulties in diagnosing MELAS because of its unpredictable presentation and clinical course. We therefore recommend a high index of suspicion in cases of an acute surgical abdomen with additional neurological features or raised lactate.

Radiological evaluation of intestinal obstruction in neonate and infant

Energy Technology Data Exchange (ETDEWEB)

Jeon, D. S.; Lim, K. Y.; Kim, S. J.; Kim, J. D.; Rhee, H. S. [Presbyterian Medical Center, Jeonju (Korea, Republic of)

1980-12-15

281 cases of neonatal and infantile intestinal obstruction confirmed by clinical and surgical procedure from 1975 till 1979 were reviewed radiologically. The result was as follows; 1. Intussusception was the most common cause of intestinal obstruction under one year of age (173/281: 61.56%), and other causes of descending order were infantile hypertrophic pyloric stenosis (20: 7.11%), congenital megacolon (19: 6.76%), anorectal malformation (17: 6.05%), meconium plug syndrome (17: 6.05%), hernia (12: 4.27%), band adhesion (8: 2.85%), rotation anomaly (5: 1.78%), small bowel atresia (3: 1.07%), Meckel's diverticulum (3: 1.07%), duodenal atresia (2: 0.71%), meconium ileus (1: 0.36%) and annular pancreas (1: 0.36%). Congenital type of intestinal obstruction (we classified intussusception acquired and others congenital) occupied 38.44%. 2. The ratio of male to female was 3: 1, congenital type 4.69 : 1 and acquired 2.39 : 1. 3. Vomiting, bloody stool, fever and abdominal distention were the most 4 symptoms. 4. The frequency of typical mechanical ileus pattern on plain abdomen films was 226 cases(80.43%) paralytic ileus or normal finding was 52 cases (18.51%) and pneumoperitoneum with ileus sign was 3 cases (1.06%). 5. Barium meal or enema was performed in 228 cases and narrowing or obstructed site was found in 213 cases of them (93.42%); intussusception 100% (173/173), infantile hypertrophic pyloric stenosis 93.33% (14/15) and congenital megacolon 82.35% (14/17). 6. Only 10 cases had associated disease such as Mongolism, thoracic kyphosis and scoliosis, microcolon, prematurity, ileocolic fistula, undescended testicle and hydrocele. 7. Meconium plug syndrome, duodenal atresia, small bowel atresia, mecondium ileus and annular pancreas were developed early after birth with average onset age of 3.6 days.

Emergency colectomy for fulminant Clostridium difficile colitis: Striking the right balance.

Science.gov (United States)

Osman, Khalid A; Ahmed, Mohamed H; Hamad, Mahir A; Mathur, Dilip

2011-10-01

The number of reported cases of Clostridium difficile (CD) infections has increased markedly worldwide. CD causes a spectrum of clinical syndromes, ranging from mild diarrhea to a very severe illness in the form of pseudomembranous colitis (PMC), toxic megacolon, leading to colonic perforation, peritonitis, and even death. In today's practice, toxic megacolon is more often caused by pseudomembranous colitis than ulcerative colitis. There is urgent need to establish clear guidelines about how and when to refer patients with fulminant CD colitis to surgeons. Furthermore, there is no strict protocol for the timing of surgical intervention. The aim of this review is to review the available evidence about the criteria for referral to surgeons and timing for surgery. Medline search was carried out for articles published on fulminant CD colitis with emergency colectomy from 1966 to 2010. There were no prospective randomized trails. All retrospective cohort and case control studies were included. We excluded case reports, letters, and studies with less than five patients. Our search showed that patients with confirmed or suspected CD who failed to respond to maximum medical therapy and develop three of the following should be referral for surgical assessment: abdominal pain, abdominal distension, localized tenderness, pyrexia >38°C, and tachycardia >100 beats per minute. In addition to the above, if the patient is above 65 years old and develops four of the following, they should be considered for an emergency colectomy: WBC >16 × 10⁹/l, lactate >2.2 mmol/l, albumin cases. In the absence of published prospective multicenter trial, we suggest that our criteria may enhance early diagnosis and consideration of early referral for surgery. Ultimately, this may reduce the significant morbidity and mortality associated with FCDC.

Radiological evaluation of intestinal obstruction in neonate and infant

International Nuclear Information System (INIS)

Jeon, D. S.; Lim, K. Y.; Kim, S. J.; Kim, J. D.; Rhee, H. S.

1980-01-01

281 cases of neonatal and infantile intestinal obstruction confirmed by clinical and surgical procedure from 1975 till 1979 were reviewed radiologically. The result was as follows; 1. Intussusception was the most common cause of intestinal obstruction under one year of age (173/281: 61.56%), and other causes of descending order were infantile hypertrophic pyloric stenosis (20: 7.11%), congenital megacolon (19: 6.76%), anorectal malformation (17: 6.05%), meconium plug syndrome (17: 6.05%), hernia (12: 4.27%), band adhesion (8: 2.85%), rotation anomaly (5: 1.78%), small bowel atresia (3: 1.07%), Meckel's diverticulum (3: 1.07%), duodenal atresia (2: 0.71%), meconium ileus (1: 0.36%) and annular pancreas (1: 0.36%). Congenital type of intestinal obstruction (we classified intussusception acquired and others congenital) occupied 38.44%. 2. The ratio of male to female was 3: 1, congenital type 4.69 : 1 and acquired 2.39 : 1. 3. Vomiting, bloody stool, fever and abdominal distention were the most 4 symptoms. 4. The frequency of typical mechanical ileus pattern on plain abdomen films was 226 cases(80.43%) paralytic ileus or normal finding was 52 cases (18.51%) and pneumoperitoneum with ileus sign was 3 cases (1.06%). 5. Barium meal or enema was performed in 228 cases and narrowing or obstructed site was found in 213 cases of them (93.42%); intussusception 100% (173/173), infantile hypertrophic pyloric stenosis 93.33% (14/15) and congenital megacolon 82.35% (14/17). 6. Only 10 cases had associated disease such as Mongolism, thoracic kyphosis and scoliosis, microcolon, prematurity, ileocolic fistula, undescended testicle and hydrocele. 7. Meconium plug syndrome, duodenal atresia, small bowel atresia, mecondium ileus and annular pancreas were developed early after birth with average onset age of 3.6 days

Imaging findings in 11 cats with feline dysautonomia.

Science.gov (United States)

Novellas, Rosa; Simpson, Kerry E; Gunn-Moore, Danièlle A; Hammond, Gawain J C

2010-08-01

Dysautonomia is caused by degeneration of the autonomic ganglia. Failure of the autonomic system affecting the gastrointestinal and urinary tracts can cause oesophageal distension and/or dysfunction, gastric and bowel distension and hypomotility, and urinary bladder distension. The aim of this retrospective study was to describe diagnostic imaging findings in cats with dysautonomia. Common findings were megaoesophagus and/or oesophageal dysfunction, gastric distension and signs of intestinal ileus. Associated aspiration pneumonia and megacolon appeared less commonly. Although diagnostic imaging findings are not specific for this disease, if findings in multiple systems are detected, along with consistent clinical signs and neurological deficits, dysautonomia should be considered among the differential diagnosis. Copyright 2010 ISFM and AAFP. Published by Elsevier Ltd. All rights reserved.

Characterization of T cell clones from chagasic patients: predominance of CD8 surface phenotype in clones from patients with pathology

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Washington R. Cuna

1995-08-01

Full Text Available Human Chagas' disease, caused by the protozoan Trypanosoma cruzi, is associated with pathological processes whose mechanisms are not known. To address this question, T cell lines were developed from chronic chagasic patients peripheral blood mononuclear cells (PBMC and cloned. These T cell clones (TCC were analyzed phenotypically with monoclonal antibodies by the use of a fluorescence microscope. The surface phenotype of the TCC from the asymptomatic patient were predominantly CD4 positive (86%. On the contrary, the surface phenotype CD8 was predominant in the TCC from the patients suffering from cardiomegaly with right bundle branch block (83%, bradycardia with megacolon (75 % and bradycardia (75%. Future studies will be developed in order to identify the antigens eliciting these T cell subpopulations.

INTUSUSCEPCIÓN CECOCÓLICA E INVAGINACIÓN DEL CIEGO EN UN CANINO: REPORTE DE CASO

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J.F. Ortiz

2011-01-01

Full Text Available Se expone el caso de un perro mestizo de Fox Terrier que presentaba diarrea sanguino-lenta que no mejoró al tratamiento inicial; se detectó a la palpación una masa a nivel del colon, que se confirmó con un estudio radiográfico de tránsito intestinal; también, se diagnosticó un megacolon. Se decidió realizar una laparotomía exploratoria donde se encontró una intususcepción cecocólica y la invaginación del ciego, el procedimiento quirúrgico realizado consistió en hacer tiflectomia y enteroanastomosis término-terminal; luego de este procedimiento, el paciente se recuperó completamente del cuadro intestinal que presentaba.

Experimental studies on Trypanosoma (Schizotrypanum cruzi strains isolated from man, from animals and from triatomine bugs in Brazil

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W. B. Petana

1974-12-01

Full Text Available A study on nine strains of T. cruzi isolated from man, from animais and from triatomine bugs in Brazil are described. The parasites were slightly viscerotropic in white mice in six of the strains, highly viscerotropic and cardiotropic in two strains, and asymptomatic on one strain. Mechanical and cyclical passage from infected to healthy mice, and treatment of the infected mice with immunosuppressant drug, did not increase the blood parasitaemia or strain virulence. The results of biometric studies on the blood trypanosomes from each strain are also described. The various aspects on the importance of T. cruzi strain Identification are emphasized and discussed.Nove cepas de T. cruzi, 5 isoladas do homem, 2 isoladas do cão e 2 outras isoladas de Triatoma infestans domiciliados, foram estudadas do ponto de vista morfólógico e de sua virulência e patogenicidade para o camundongo, em um período de observação de 8 a 12 meses. As cinco cepas isoladas do homem mostraram baixa parasitemia quando inoculadas no camundongo e provocaram nesse animal apenas redução dos glânglios mioentéricos do colon e discreto aumento desse órgão. Das duas cepas isoladas do cão uma provocou megacolon e cardiomegalia no camundongo e a outra comportau-se de forma semelhante às cepas isoladas do homem. Das cepas isoladas do T. infestans uma foi absolutamente avirulenta para o camundongo e a outra provocou redução dos gânglios do colon e do miocárdio, cardiomegalia e megacolon. Admitem os autores que a passagem "cíclica" de uma cepa de um reservatório para outro possa modificar o seu comportamento, servindo o homem como "reservatório estabilizador" da baixa virulência e patogenicidade das cepas, pelo menos daquelas isoladas de casos crônicos

Nuclear angiocardiographic evaluation of effect of the isosorbide dinitrate in chronic Chagas' patients

International Nuclear Information System (INIS)

Marin Neto, J.A.; Souza, A.C.S.; Maciel, B. C.; Gallo Junior, L.; Iazigi, N.

1988-01-01

The cardiovascular effects of a systemic vasodilator therapy (isosorbide dinitrate - ID) were studied in 13 normal individuals and in 40 chronic Chagas' patients, grouped as follows: 13 in the indeterminate phase of the disease; 14 with the chronic cardiac form and 13 presenting only clinical and laboratory findings of megaesophagus and/or megacolon (digestive form). The LV pump function was studied by means of radionuclide angiography in baseline condition and after 5-10 min of sublingual administration of 5mg of ID.Ejection fraction (EF), peak velocity of LV emptying (Vmax), heart rate (HR) and blood pressure BP) were determined under each condition. All groups showed comparable mean HR and BP values. Only the cardiac group exhibited lower values of EF and Vmax, as compared to normal controls. The vasodilator drug elicited significant comparable (p [pt

Assessment and epidemiology of Chagas' disease in patients treated in Araguaina - Tocantins; Avaliacao e epidemiologia da cardiopatia chagasica em pacientes atendidos em Araguaina - Tocantins

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Correa, Valeria Rita

2010-07-01

Chagas disease (AD) was described by Carlos Chagas in 1909. It is caused by a parasite T. cruzi, transmitted by bugs, by blood transfusion, vertical and orally. The DC has two phases: acute and chronic. The evolution to the cardiac form occurs in about 30% of chronic cases and is the largest cause of mortality in chronic Chagas disease. The aim of this study was to Chagas' disease in patients of Tocantins, compared with other heart patients and asymptomatic from the standpoint of non-invasive exams using radiant energies such as echocardiography and ECG and RX. The descriptive study included 80 patients, 20 chronic form of Chagas disease, 20 indeterminate, 20 with other heart diseases, and 20 controls. There was a prevalence of 9.5% of chagasic patients treated in outpatient cardiology at Araguaina Tocantins, and 7.3% in chronic and 2.21% in the indeterminate. Of the chronic patients in the study 50% had mega esophagus and megacolon 4 (20%). Most patients had no family history of AD, nor was a smoker or drinker. Major electrocardiographic abnormalities found refer to driving. The evaluation of ICT, the chronic chagasic showed that increased by 40% of patients, 40% had esophageal changes and 20% of patients had megacolon s. The echocardiogram was abnormal in 42%). 27% of patients had EF below 55% changed. Changes in segmental contractility and Asynchrony septum were found in 80% of chronic Chagas disease. In 80% of the patients was observed diastolic dysfunction. The valvular changes occurred in 75%. Electrocardiographic abnormalities occurred in 80% of patients with CCC, while the other heart had ECG changes. Arterial hypertension had an incidence of 45% in patients with CCC and 40% in FCI. The systolic and diastolic ventricular dysfunction was more prevalent in groups that had an abnormal ECG and arrhythmia. Observed that the group of chagasic decreased ejection fraction is correlated to a higher incidence of arrhythmias besides diastolic dysfunction and

O perfil dos portadores de doença de Chagas, com ênfase na forma digestiva, em hospital terciário de Ribeirão Preto, SP Features of Chagas' disease patients with emphasis on digestive form, in a tertiary hospital of Ribeirão Preto, SP

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Mayra Mayumi Kamiji

2005-08-01

Full Text Available Para caracterizar o perfil clínico e demográfico dos portadores da forma digestiva da doença de Chagas atualmente atendidos no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto, foram revistos 377 prontuários de pacientes com resultado positivo para reação sorológica para a doença de Chagas atendidos entre janeiro de 2002 a março de 2003. A idade mediana dos pacientes era de 67 anos e 210 (56% eram mulheres. Megaesôfago e/ou megacólon chagásicos estavam presentes em 135 pacientes, dos quais, 59% apresentavam cardiopatia. Para 49% dos pacientes com doença digestiva, havia prescrição de pelo menos dois medicamentos para tratamento de doença cardiovascular. Em 66 pacientes, foram detectadas comorbidades crônicas. A população de portadores da forma digestiva da doença de Chagas do HCFMRP é majoritariamente geriátrica e apresenta freqüência elevada de doenças cardiovasculares, o que sugere risco elevado das modalidades de tratamento cirúrgico do megaesôfago e megacólon.In order to characterize the demographic and clinical profile of patients with digestive manifestations of Chagas' disease, the medical records were reviewed of patients (n = 377 currently attended at Hospital das Clinicas da Faculdade de Medicina de Ribeirão Preto with positive serologic reaction for Chagas' disease and admitted from January 2002 to March 2003. Median age was 67 years and 210 (56% were women. Megaesophagus and/or megacolon were present in 135 patients, 59% of these had cardiopathy. For 49% of patients with digestive disease, at least two medical prescriptions of medicines for the treatment of cardiovascular diseases were found. In 66 patients, chronic comorbidities were detected. The population with digestive manifestation of Chagas' disease referred to HCFMRP is mostly geriatric, with an elevated frequency of cardiopathy, which may indicate a high risk for surgical approach to the treatment of chagasic megaesophagus and

Assessment and epidemiology of Chagas' disease in patients treated in Araguaina - Tocantins

International Nuclear Information System (INIS)

Correa, Valeria Rita

2010-01-01

Chagas disease (AD) was described by Carlos Chagas in 1909. It is caused by a parasite T. cruzi, transmitted by bugs, by blood transfusion, vertical and orally. The DC has two phases: acute and chronic. The evolution to the cardiac form occurs in about 30% of chronic cases and is the largest cause of mortality in chronic Chagas disease. The aim of this study was to Chagas' disease in patients of Tocantins, compared with other heart patients and asymptomatic from the standpoint of non-invasive exams using radiant energies such as echocardiography and ECG and RX. The descriptive study included 80 patients, 20 chronic form of Chagas disease, 20 indeterminate, 20 with other heart diseases, and 20 controls. There was a prevalence of 9.5% of chagasic patients treated in outpatient cardiology at Araguaina Tocantins, and 7.3% in chronic and 2.21% in the indeterminate. Of the chronic patients in the study 50% had mega esophagus and megacolon 4 (20%). Most patients had no family history of AD, nor was a smoker or drinker. Major electrocardiographic abnormalities found refer to driving. The evaluation of ICT, the chronic chagasic showed that increased by 40% of patients, 40% had esophageal changes and 20% of patients had megacolon s. The echocardiogram was abnormal in 42%). 27% of patients had EF below 55% changed. Changes in segmental contractility and Asynchrony septum were found in 80% of chronic Chagas disease. In 80% of the patients was observed diastolic dysfunction. The valvular changes occurred in 75%. Electrocardiographic abnormalities occurred in 80% of patients with CCC, while the other heart had ECG changes. Arterial hypertension had an incidence of 45% in patients with CCC and 40% in FCI. The systolic and diastolic ventricular dysfunction was more prevalent in groups that had an abnormal ECG and arrhythmia. Observed that the group of chagasic decreased ejection fraction is correlated to a higher incidence of arrhythmias besides diastolic dysfunction and related

Slow-transit Constipation.

Science.gov (United States)

Bharucha, Adil E.; Philips, Sidney F.

2001-08-01

Idiopathic slow-transit constipation is a clinical syndrome predominantly affecting women, characterized by intractable constipation and delayed colonic transit. This syndrome is attributed to disordered colonic motor function. The disorder spans a spectrum of variable severity, ranging from patients who have relatively mild delays in transit but are otherwise indistinguishable from irritable bowel syndrome to patients with colonic inertia or chronic megacolon. The diagnosis is made after excluding colonic obstruction, metabolic disorders (hypothyroidism, hypercalcemia), drug-induced constipation, and pelvic floor dysfunction (as discussed by Wald ). Most patients are treated with one or more pharmacologic agents, including dietary fiber supplementation, saline laxatives (milk of magnesia), osmotic agents (lactulose, sorbitol, and polyethylene glycol 3350), and stimulant laxatives (bisacodyl and glycerol). A subtotal colectomy is effective and occasionally is indicated for patients with medically refractory, severe slow-transit constipation, provided pelvic floor dysfunction has been excluded or treated.

Chronic intestinal pseudoobstruction syndrome

Energy Technology Data Exchange (ETDEWEB)

Yeon, Kyung Mo; Seo, Jeong Kee; Lee, Yong Seok [Seoul National University Children' s Hospital, Seoul (Korea, Republic of)

1992-03-15

Chronic intestinal pseudoobstruction syndrome is a rare clinical condition in which impaired intestinal peristalsis causes recurrent symptoms of bowel obstruction in the absence of a mechanical occlusion. This syndrome may involve variable segments of small or large bowel, and may be associated with urinary bladder retention. This study included 6 children(3 boys and 3 girls) of chronic intestinal obstruction. Four were symptomatic at birth and two were of the ages of one month and one year. All had abdominal distension and deflection difficulty. Five had urinary bladder distension. Despite parenteral nutrition and surgical intervention(ileostomy or colostomy), bowel obstruction persisted and four patients expired from sepses within one year. All had gaseous distension of small and large bowel on abdominal films. In small bowel series, consistent findings were variable degree of dilatation, decreased peristalsis(prolonged transit time) and microcolon or microrectum. This disease entity must be differentiated from congenital megacolon, ileal atresia and megacystis syndrome.

Cecal Perforation Associated with Clostridium difficile Infection: A Case Report.

Science.gov (United States)

Luthe, Sarah Kyuragi; Sato, Ryota

2017-04-01

Various complications are reported with Clostridium difficile infection (CDI), including fulminant CDI. Fulminant CDI is an underappreciated life-threatening condition associated with complications such as toxic megacolon and bowel perforation. A 79-year-old woman presented to the Emergency Department with altered mental status. She was admitted and conservatively treated for a left thalamic hemorrhage. While hospitalized, she developed watery diarrhea due to Clostridium difficile. Although metronidazole was initiated, she developed altered mental status and septic shock. Abdominal x-ray study and computed tomography revealed a significantly dilatated colon and a massive pneumoperitoneum. She underwent subtotal colectomy with a 14-day course of intravenous meropenem. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case suggests that we must be aware of the complications that CDI may present and adequately consider surgical management because early diagnosis and surgical treatment is critical to reduce the mortality of fulminant CDI. Copyright © 2016 Elsevier Inc. All rights reserved.

Discovery of LFF571: An Investigational Agent for Clostridium difficile Infection

Energy Technology Data Exchange (ETDEWEB)

LaMarche, Matthew J.; Leeds, Jennifer A.; Amaral, Adam; Brewer, Jason T.; Bushell, Simon M.; Deng, Gejing; Dewhurst, Janetta M.; Ding, Jian; Dzink-Fox, JoAnne; Gamber, Gabriel; Jain, Akash; Lee, Kwangho; Lee, Lac; Lister, Troy; McKenney, David; Mullin, Steve; Osborne, Colin; Palestrant, Deborah; Patane, Michael A.; Rann, Elin M.; Sachdeva, Meena; Shao, Jian; Tiamfook, Stacey; Trzasko, Anna; Whitehead, Lewis; Yifru, Aregahegn; Yu, Donghui; Yan, Wanlin; Zhu, Qingming (Novartis)

2012-11-09

Clostridium difficile (C. difficile) is a Gram positive, anaerobic bacterium that infects the lumen of the large intestine and produces toxins. This results in a range of syndromes from mild diarrhea to severe toxic megacolon and death. Alarmingly, the prevalence and severity of C. difficile infection are increasing; thus, associated morbidity and mortality rates are rising. 4-Aminothiazolyl analogues of the antibiotic natural product GE2270 A (1) were designed, synthesized, and optimized for the treatment of C. difficile infection. The medicinal chemistry effort focused on enhancing aqueous solubility relative to that of the natural product and previous development candidates (2, 3) and improving antibacterial activity. Structure-activity relationships, cocrystallographic interactions, pharmacokinetics, and efficacy in animal models of infection were characterized. These studies identified a series of dicarboxylic acid derivatives, which enhanced solubility/efficacy profile by several orders of magnitude compared to previously studied compounds and led to the selection of LFF571 (4) as an investigational new drug for treating C. difficile infection.

Transverse colon volvulus in neurologicaly imparied patient as an emergency surgical condition: A case report.

Science.gov (United States)

Miličković, Maja; Savić, Đorđe; Stanković, Nikola; Vukadin, Miroslav; Božić, Dejana

2017-01-01

Transverse colon volvulus is an uncommon cause of bowel obstruction in general. Predisposing factors are mental retardation, dysmotility disorders, chronic constipation and congenital megacolon. We presented transverse colon volvulus in a 16-year-old boy with cerebral palsy. Chronic constipation in neurologicaly impaired patient was a risk factor predisposing to volvulus. The patient was admitted to the hospital with enormous abdominal distension and acute respiratory insufficiency. A boy was emergently taken to the operating room for exploratory laparotomy. During the surgery, a 360º clockwise volvulus of the transverse colon was found. After reduction of volvulus, an enormous transverse colon was resected and colostomy was formed. In the postoperative period, despite the good functioning of stoma and intraabdominal normotension, numerous and long lasting respiratory problems developed. The patient was discharged from our institution after 8 months. Though very rare in pediatric group, the possibility of a transverse colon volvulus must be considered in the differential diagnosis of acute large bowel obstruction.

Placement of a Port Catheter Through Collateral Veins in a Patient with Central Venous Occlusion

International Nuclear Information System (INIS)

Teichgraeber, Ulf Karl-Martin; Streitparth, Florian; Gebauer, Bernhard; Benter, Thomas

2010-01-01

Long-term utilization of central venous catheters (CVCs) for parenteral nutrition has a high incidence of central venous complications including infections, occlusions, and stenosis. We report the case of a 31-year-old woman presenting with a malabsorption caused by short gut syndrome due to congenital aganglionic megacolon. The patient developed a chronic occlusion of all central neck and femoral veins due to long-term use of multiple CVCs over more than 20 years. In patients with central venous occlusion and venous transformation, the implantation of a totally implanted port system by accessing collateral veins is an option to continue long-term parenteral nutrition when required. A 0.014-in. Whisper guidewire (Terumo, Tokyo) with high flexibility and steerability was chosen to maneuver and pass through the collateral veins. We suggest this approach to avoid unfavorable translumbar or transhepatic central venous access and to conserve the anatomically limited number of percutaneous access sites.

[Congenital megacolon in neonates and infants: impact of early, one-stage repair on morbidity and surgical complications].

Science.gov (United States)

Calisti, A; Molle, P H; Vallasciani, S

2004-01-01

Hirschsprung's disease (HD) is a major cause of congenital bowel obstruction in children. Classical management includes stoma creation, a pull-through procedure, and restoration of bowel continuity. Colostomy may be also pulled through directly at second operation. Since the first report of a single stage (SS) primary pull-through without colostomy for HD, in 1980, several patients have been treated according to this procedure, even in neonatal period. All of them had a favourable outcome with better compliance of families and less hospital stay. The Authors reviewed the clinical records of 22 patients selected among 36 treated for HD between 1992 and 2000; in all, disease extended to rectum-sigma-descendent; all cases of "total aganglionosis" or "ultra short" cases were excluded. Fifteen patients underwent SS repair: 11 neonates were operated at mean age of 69 days (range 14-172). In four infants diagnosis was made later (mean age 15 +/- 9 months) and SS repair was performed 8 days after (range 4-14). Seven patients underwent multiple stage (MS) repairs for clinical reasons: pull-through of the previous colostomy was performed at mean age of 171 days (range 47-330). A Duhamel procedure with an Endo-GIA linear stapler was performed in all No differences in gestational age or birth weight were found in both groups. Complications were observed only in the MS group (2 related to colostomy, 1 to postoperative adhesions). The Authors conclude that primary SS treatment of HD is safe and effective even in newborn patient, reduce familiar stress and hospital stay. The lower morbidity and complication rates could be explained by the limited bowel manipulation. Accurate histological-hystochemical diagnosis and meticulous preoperative bowel management should be part of the management.

Radiation-induced rectal complications are not influenced by age: a dose fractionation study in the rat.

Science.gov (United States)

van den Aardweg, Gerard J M J; Olofsen-van Acht, Manouk J J; van Hooije, Christel M C; Levendag, Peter C

2003-05-01

Radiation-induced complications of the rectum are an important dose-limiting factor in radiotherapy of pelvic malignancies. In general, animal studies demonstrated no differences in acute and late normal tissue toxicity with age, but little is known about rectal complications in relation to age. For this purpose, an extensive histological and dose fractionation study was carried out on the rectum of young (12 weeks) and older (77-80 weeks) rats. In this paper, the results of dose fractionation are presented in relation to age at the time of irradiation. Young and older animals were irradiated with single and fractionated doses. After irradiation, rectal complications could lead to occlusion and stenosis, eventually resulting in the clinical symptoms of a megacolon and a possible fistula. For each dose group, cumulative survival rates were obtained with Kaplan-Meier analysis, from which dose-effect curves and the associated LD(50) values for a megacolon/fistula were calculated. The majority of responders died between 8 and 24 weeks after irradiation, irrespective of age. For both age groups, only the fractionation data showed a reduction in the mean latency with increasing dose. In the older age group, 39% of the responders developed a fistula compared to 26% for the younger animals. The LD(50) values increased from around 30 Gy after single doses to nearly 65 Gy after 10 fractions. The increases in LD(50) values with the number of fractions were independent of the age of the rats. For each of the dose fractionation schedules, log-rank testing indicated no significant differences in cumulative survival rates between younger and older animals (P > 0.10). The high alpha/beta ratios obtained for both the young and older animals strongly suggested that the late rectal complications were a consequence of early epithelial injury. Associated histological findings indicated that blood vessel damage, which was already evident at a high incidence at 4 weeks after irradiation

Transverse colon volvulus in neurologicaly imparied patient as an emergency surgical condition: A case report

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Miličković Maja

2017-01-01

Full Text Available Introduction. Transverse colon volvulus is an uncommon cause of bowel obstruction in general. Predisposing factors are mental retardation, dysmotility disorders, chronic constipation and congenital megacolon. Case report. We presented transverse colon volvulus in a 16-year-old boy with cerebral palsy. Chronic constipation in neurologicaly impaired patient was a risk factor predisposing to volvulus. The patient was admitted to the hospital with enormous abdominal distension and acute respiratory insufficiency. A boy was emergently taken to the operating room for exploratory laparotomy. During the surgery, a 360º clockwise volvulus of the transverse colon was found. After reduction of volvulus, an enormous transverse colon was resected and colostomy was formed. In the postoperative period, despite the good functioning of stoma and intraabdominal normotension, numerous and long lasting respiratory problems developed. The patient was discharged from our institution after 8 months. Conclusion. Though very rare in pediatric group, the possibility of a transverse colon volvulus must be considered in the differential diagnosis of acute large bowel obstruction.

Evaluation and treatment of constipation in infants and children.

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Biggs, Wendy S; Dery, William H

2006-02-01

Constipation in children usually is functional and the result of stool retention. However, family physicians must be alert for red flags that may indicate the presence of an uncommon but serious organic cause of constipation, such as Hirschsprung's disease (congenital aganglionic megacolon), pseudo-obstruction, spinal cord abnormality, hypothyroidism, diabetes insipidus, cystic fibrosis, gluten enteropathy, or congenital anorectal malformation. Treatment of functional constipation involves disimpaction using oral or rectal medication. Polyethylene glycol is effective and well tolerated, but a number of alternatives are available. After disimpaction, a maintenance program may be required for months to years because relapse of functional constipation is common. Maintenance medications include mineral oil, lactulose, milk of magnesia, polyethylene glycol powder, and sorbitol. Education of the family and, when possible, the child is instrumental in improving functional constipation. Behavioral education improves response to treatment; biofeedback training does not. Because cow's milk may promote constipation in some children, a trial of withholding milk may be considered. Adding fiber to the diet may improve constipation. Despite treatment, only 50 to 70 percent of children with functional constipation demonstrate long-term improvement.

[Saccharomyces boulardii in the treatment and prevention of antibiotic-associated diarrhea].

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Micklefield, G

2014-04-17

Antibiotic-associated diarrhea (AAD) is the most frequent side effect of antibiotic therapy. Clinical signs and symptoms comprise mild and self-limiting courses of diarrhea as well as life threatening courses like pseudomembranous colitis or toxic megacolon. Therapy is symptomatic, antidiarrheal drugs like Saccharomyces boulardii are the therapy of choice. Available studies on S. boulardii in the prevention of AAD are presented as a review. In 14 out of 17 studies including 4,627 patients the administration of S. boulardii achieved a protective effect between 43.7% and 87.3%. A meta-analysis (5 studies, 1,076 patients) showed a significant reduction of the risk to develop an AAD from 17.2% to 6.7%,in a furthermeta-analysis (4 studies on eradication of H. pylori, 1,215 patients) the significant reductionwasfrom 12.2% to 5.6%. There is very good evidence for the yeast S. boulardii to be effective in the prevention of AAD especially in hospitalized adults. The simultaneous administration of S. boulardii to antibiotics resulted in a significant reduction to develop AAD by more than half.

Long-Term Natural History and Complications of Collagenous Colitis

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Hugh J Freeman

2012-01-01

Full Text Available Microscopic forms of colitis have been described, including collagenous colitis, a possibly heterogeneous disorder. Collagenous colitis most often appears to have an entirely benign clinical course that usually responds to limited treatment. Sometimes significant extracolonic disorders, especially arthritis, spondylitis, thyroiditis and skin disorders, such as pyoderma gangrenosum, dominate the clinical course and influence the treatment strategy. However, rare fatalities have been reported and several complications, some severe, have been attributed directly to the colitis. Toxic colitis and toxic megacolon may develop. Concomitant gastric and small intestinal inflammatory disorders have been described including celiac disease and more extensive collagenous inflammatory disease. Colonic ulceration has been associated with the use of nonsteroidal anti-inflammatory drugs, while other forms of inflammatory bowel disease, including ulcerative colitis and Crohn disease, may evolve directly from collagenous colitis. Submucosal ‘dissection’, colonic fractures, or mucosal tears and perforation, possibly from air insufflation during colonoscopy, have been reported. Similar changes may result from increased intraluminal pressures that may occur during radiological imaging of the colon. Neoplastic disorders of the colon may also occur during the course of collagenous colitis, including colon carcinoma and neuroendocrine tumours (ie, carcinoids. Finally, lymphoproliferative disease has been reported.

SEVERE CLOSTRIDIUM DIFFICILE INFECTIONS. A SYSTEMATIC LITERATURE -review-

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Adriana Elena NICA

2016-06-01

Full Text Available Clostridium difficile is a bacterium that has been brought to the attention of the medical community recently, as the number of infections related to it has increased dramatically. This is happening mainly because of the excessive and defective use of antibiotic therapy. The pathology of a Clostridium Difficile infection is very complex, as it ranges from easy symptoms like abdominal pain and diarrhea to severe complications, like toxic megacolon. The management of these infections has become even more difficult, as they are not appearing only in the hospital environment anymore, but also outside of it. The bacterium spreads through poor hands hygiene. Also, we don’t have a clear strategy for overcoming an infection like this, so it gets even more difficult as most of the times the doctors need to rely only on their experience and knowledge to find ways of battling it. We would like to underline the research opportunities that are available in this domain as very few things are known about Clostridium difficile and also the crucial importance of research, as these infections are common and dangerous not only for patients, but for the medical staff and their families too.

A novel mutation in the endothelin B receptor gene in a moroccan family with shah-waardenburg syndrome.

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Doubaj, Yassamine; Pingault, Véronique; Elalaoui, Siham C; Ratbi, Ilham; Azouz, Mohamed; Zerhouni, Hicham; Ettayebi, Fouad; Sefiani, Abdelaziz

2015-02-01

Waardenburg syndrome (WS) is a neurocristopathy disorder combining sensorineural deafness and pigmentary abnormalities. The presence of additional signs defines the 4 subtypes. WS type IV, also called Shah-Waardenburg syndrome (SWS), is characterized by the association with congenital aganglionic megacolon (Hirschsprung disease). To date, 3 causative genes have been related to this congenital disorder. Mutations in the EDNRB and EDN3 genes are responsible for the autosomal recessive form of SWS, whereas SOX10 mutations are inherited in an autosomal dominant manner. We report here the case of a 3-month-old Morrocan girl with WS type IV, born to consanguineous parents. The patient had 3 cousins who died in infancy with the same symptoms. Molecular analysis by Sanger sequencing revealed the presence of a novel homozygous missense mutation c.1133A>G (p.Asn378Ser) in the EDNRB gene. The proband's parents as well as the parents of the deceased cousins are heterozygous carriers of this likely pathogenic mutation. This molecular diagnosis allows us to provide genetic counseling to the family and eventually propose prenatal diagnosis to prevent recurrence of the disease in subsequent pregnancies.

Chronic phase of Chagas disease: why should it be treated? A comprehensive review

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José Rodrigues Coura

2011-09-01

Full Text Available The pathogenesis and evolutive pattern of Chagas disease suggests that the chronic phase should be more widely treated in order to (i eliminate Trypanosoma cruzi and prevent new inflammatory foci and the extension of tissue lesions, (ii promote tissue regeneration to prevent fibrosis, (iii reverse existing fibrosis, (iv prevent cardiomyopathy, megaoesophagus and megacolon and (v reduce or eliminate cardiac block and arrhythmia. All cases of the indeterminate chronic form of Chagas disease without contraindications due to other concomitant diseases or pregnancy should be treated and not only cases involving children or recently infected cases. Patients with chronic Chagas cardiomyopathy grade II of the New York Heart Association classification should be treated with specific chemotherapy and grade III can be treated according to medical-patient decisions. We are proposing the following new strategies for chemotherapeutic treatment of the chronic phase of Chagas disease: (i repeated short-term treatments for 30 consecutive days and interval of 30-60 days for six months to one year and (ii combinations of drugs with different mechanisms of action, such as benznidazole + nifurtimox, benznidazole or nifurtimox + allopurinol or triazole antifungal agents, inhibition of sterol synthesis.

Clostridium difficile infection: epidemiology, diagnosis and understanding transmission.

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Martin, Jessica S H; Monaghan, Tanya M; Wilcox, Mark H

2016-04-01

Clostridium difficile infection (CDI) continues to affect patients in hospitals and communities worldwide. The spectrum of clinical disease ranges from mild diarrhoea to toxic megacolon, colonic perforation and death. However, this bacterium might also be carried asymptomatically in the gut, potentially leading to 'silent' onward transmission. Modern technologies, such as whole-genome sequencing and multi-locus variable-number tandem-repeat analysis, are helping to track C. difficile transmission across health-care facilities, countries and continents, offering the potential to illuminate previously under-recognized sources of infection. These typing strategies have also demonstrated heterogeneity in terms of CDI incidence and strain types reflecting different stages of epidemic spread. However, comparison of CDI epidemiology, particularly between countries, is challenging due to wide-ranging approaches to sampling and testing. Diagnostic strategies for C. difficile are complicated both by the wide range of bacterial targets and tests available and the need to differentiate between toxin-producing and non-toxigenic strains. Multistep diagnostic algorithms have been recommended to improve sensitivity and specificity. In this Review, we describe the latest advances in the understanding of C. difficile epidemiology, transmission and diagnosis, and discuss the effect of these developments on the clinical management of CDI.

Mast cells in the colon of Trypanosoma cruzi-infected patients: are they involved in the recruitment, survival and/or activation of eosinophils?

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Martins, Patrícia Rocha; Nascimento, Rodolfo Duarte; Lopes, Júlia Guimarães; Santos, Mônica Morais; de Oliveira, Cleida Aparecida; de Oliveira, Enio Chaves; Martinelli, Patrícia Massara; d'Ávila Reis, Débora

2015-05-01

Megacolon is frequently observed in patients who develop the digestive form of Chagas disease. It is characterized by dilation of the rectum-sigmoid portion and thickening of the colon wall. Microscopically, the affected organ presents denervation, which has been considered as consequence of an inflammatory process that begins at the acute phase and persists in the chronic phase of infection. Inflammatory infiltrates are composed of lymphocytes, macrophages, natural killer cells, mast cells, and eosinophils. In this study, we hypothesized that mast cells producing tryptase could influence the migration and the activation of eosinophils at the site, thereby contributing to the immunopathology of the chronic phase. We seek evidence of interactions between mast cells and eosinophils through (1) evaluation of eosinophils, regarding the expression of PAR2, a tryptase receptor; (2) correlation analysis between densities of mast cells and eosinophils; and (3) ultrastructural studies. The electron microscopy studies revealed signs of activation of mast cells and eosinophils, as well as physical interaction between these cells. Immunohistochemistry and correlation analyses point to the participation of tryptase immunoreactive mast cells in the migration and/or survival of eosinophils at the affected organ.

Chronic gastritis and Helicobacter pylori in digestive form of Chagas' disease Gastrite crônica e Helicobacter Pylori na forma digestiva da doença de Chagas

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A. J. A. Barbosa

1993-04-01

Full Text Available Patients with the digestive form of Chagas'disease frequently present chronic gastritis. As the microorganism Helicobacter pylori is now accepted as the most common cause of human chronic gastritis, the present work was undertaken to verify a possible relationship between the presence of this bacterium and inflammatory changes of antral mucosa in chagasic patients. Seventeen chagasics, with megaesophagus and or megacolon were studied. Fragments from two different regions of antral mucosa were obtained by endoscopy, fixed in 4% neutral formaldehyde and embedded in paraffin. The sections were stained by haematoxylin and eosin for histology analysis, and by carbolfuchsin for H. pylori identification. H. pylori was found in 16 (94.1% chagasic patients, all of them presenting chronic gastritis. Superficial gastritis was seen in 9 (52.9% while atrophic gastritis was present in 8 (47.1% patients. H. pylori was present on gastric mucosa of 8 (100% patients with atrophic gastritis and of 8 (88.8% patients with superficial gastritis. We concluded that the microorganism H. pylori should be considered a possible factor connected with the etiopathogenesis of chronic superficial and atrophic gastritis frequently observed in patients with the digestive form of Chagas' disease.Pacientes com a forma digestiva da doença de Chagas frequentemente apresentam gastrite crônica. Tendo em vista que o microrganismo Helicobacter pylori é hoje considerado a causa mais comum de gastrite crônica no homem, propôs-se a realização deste trabalho para se verificar a possibilidade de esta bactéria estar também associada com as alterações inflamatórias da mucosa gástrica em pacientes com a forma digestiva da doença de Chagas. Fragmentos de duas regiões diferentes da mucosa antral foram obtidos endoscopicamente de 17 pacientes chagásicos com megaesôfago e ou megacolon. Os fragmentos foram processados rotineiramente para inclusão em parafina e cortes de 5 |im de

E. coli Meningitis Presenting in a Patient with Disseminated Strongyloides stercoralis.

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Gomez, Juliana B; Maque, Yvan; Moquillaza, Manuel A; Anicama, William E

2013-01-01

Introduction. Spontaneous Escherichia coli meningitis is an infrequent condition in adults and is associated with some predisposing factors, including severe Strongyloides stercoralis (SS) infections. Case Presentation. A 43-year-old Hispanic man, with history of travelling to the jungle regions of Peru and Brazil two decades ago, and who received prednisone due to Bell's palsy for three weeks before admission, presented to the Emergency Department with diarrhea, fever, and hematochezia. A week after admission he developed drowsiness, meningeal signs, abdominal distension, and constipation. A cerebrospinal fluid culture showed extended spectrum β -lactamase producing E. coli. A colonoscopy was performed and showed pancolitis. Three days after the procedure the patient became unstable and developed peritoneal signs. He underwent a laparotomy, which ended up in a total colectomy and partial proctectomy due to toxic megacolon. Three days later the patient died in the intensive care unit due to septic shock. Autopsy was performed and microscopic examination revealed the presence of multiple Strongyloides larvae throughout the body. Conclusion. Strongyloides stercoralis infection should be excluded in adults with spontaneous E. coli meningitis, especially, if gastrointestinal symptoms and history of travelling to an endemic area are present. Even with a proper diagnosis and management, disseminated strongyloidiasis has a poor prognosis.

E. coli Meningitis Presenting in a Patient with Disseminated Strongyloides stercoralis

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Juliana B. Gomez

2013-01-01

Full Text Available Introduction. Spontaneous Escherichia coli meningitis is an infrequent condition in adults and is associated with some predisposing factors, including severe Strongyloides stercoralis (SS infections. Case Presentation. A 43-year-old Hispanic man, with history of travelling to the jungle regions of Peru and Brazil two decades ago, and who received prednisone due to Bell’s palsy for three weeks before admission, presented to the Emergency Department with diarrhea, fever, and hematochezia. A week after admission he developed drowsiness, meningeal signs, abdominal distension, and constipation. A cerebrospinal fluid culture showed extended spectrum β-lactamase producing E. coli. A colonoscopy was performed and showed pancolitis. Three days after the procedure the patient became unstable and developed peritoneal signs. He underwent a laparotomy, which ended up in a total colectomy and partial proctectomy due to toxic megacolon. Three days later the patient died in the intensive care unit due to septic shock. Autopsy was performed and microscopic examination revealed the presence of multiple Strongyloides larvae throughout the body. Conclusion. Strongyloides stercoralis infection should be excluded in adults with spontaneous E. coli meningitis, especially, if gastrointestinal symptoms and history of travelling to an endemic area are present. Even with a proper diagnosis and management, disseminated strongyloidiasis has a poor prognosis.

Myenteric plexus is differentially affected by infection with distinct Trypanosoma cruzi strains in Beagle dogs

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Nívia Carolina Nogueira-Paiva

2014-02-01

Full Text Available Chagasic megaoesophagus and megacolon are characterised by motor abnormalities related to enteric nervous system lesions and their development seems to be related to geographic distribution of distinct Trypanosoma cruzi subpopulations. Beagle dogs were infected with Y or Berenice-78 (Be-78 T. cruzi strains and necropsied during the acute or chronic phase of experimental disease for post mortem histopathological evaluation of the oesophagus and colon. Both strains infected the oesophagus and colon and caused an inflammatory response during the acute phase. In the chronic phase, inflammatory process was observed exclusively in the Be-78 infected animals, possibly due to a parasitism persistent only in this group. Myenteric denervation occurred during the acute phase of infection for both strains, but persisted chronically only in Be-78 infected animals. Glial cell involvement occurred earlier in animals infected with the Y strain, while animals infected with the Be-78 strain showed reduced glial fibrillary acidic protein immunoreactive area of enteric glial cells in the chronic phase. These results suggest that although both strains cause lesions in the digestive tract, the Y strain is associated with early control of the lesion, while the Be-78 strain results in progressive gut lesions in this model.

Radiological findings of congenital urethral valves

International Nuclear Information System (INIS)

Yeon, Kyung Mo; Kook, Shin Ho

1990-01-01

Congenital urethral valve is the common cause of hydronephrosis in newborn infants and the most common cause of bladder outlet obstruction in male children. We reviewed and analysed radiological findings and associated anomalies of 16 cases of congenital urethral valve which were examined during the period from January 1985 to December 1989. The most frequent age was under one year old (56%). The main symptoms were urinary dribbing (37.5%), weak stream (25%) and urinary frequency and incontinence (25%). Anterior urethral valve (AUV) was 5 cases (31%) and posterior urethral valve (PUV) was 11 cases(69%), in which 10 cases were Type I and one case was Type III. Bladder wall thickening was seen in all cases and its severity was partly correlated with the degree of vesicoureteral reflux (VUR). VUR was observed in 12 cases (75%), and relatively severe in older age group. The degree of VUR was milder in AUV than PUV. Hydronephrosis was more severe in PUV than in anterior one, and its degree was correlated with the severity of VUR. Associated anomalies were ectopic urethral opening (2 cases), PDA (1 case), congenital megacolon (1 case) and patent urachus (1 case) in PUV. So early diagnosis and treatment of congenital urethral valve is essential to the prevention of renal damage

Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report [v1; ref status: indexed, http://f1000r.es/24q

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Dino Mijatovic

2014-03-01

Full Text Available Introduction: Hemolytic-uremic syndrome (HUS is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS, toxic megacolon with ileus, pancreatitis, central nervous system (CNS disorders and multiple organ failure (MOF. Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. In the next week of, what initially appeared as typical HUS, she developed MOF, including ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.

Metallothionein-1 and nitric oxide expression are inversely correlated in a murine model of Chagas disease

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Martha Elba Gonzalez-Mejia

2014-04-01

Full Text Available Chagas disease, caused by Trypanosoma cruzi, represents an endemic among Latin America countries. The participation of free radicals, especially nitric oxide (NO, has been demonstrated in the pathophysiology of seropositive individuals with T. cruzi. In Chagas disease, increased NO contributes to the development of cardiomyopathy and megacolon. Metallothioneins (MTs are efficient free radicals scavengers of NO in vitro and in vivo. Here, we developed a murine model of the chronic phase of Chagas disease using endemic T. cruzi RyCH1 in BALB/c mice, which were divided into four groups: infected non-treated (Inf, infected N-monomethyl-L-arginine treated (Inf L-NAME, non-infected L-NAME treated and non-infected vehicle-treated. We determined blood parasitaemia and NO levels, the extent of parasite nests in tissues and liver MT-I expression levels. It was observed that NO levels were increasing in Inf mice in a time-dependent manner. Inf L-NAME mice had fewer T. cruzi nests in cardiac and skeletal muscle with decreased blood NO levels at day 135 post infection. This affect was negatively correlated with an increase of MT-I expression (r = -0.8462, p < 0.0001. In conclusion, we determined that in Chagas disease, an unknown inhibitory mechanism reduces MT-I expression, allowing augmented NO levels.

Pleiotropic effects of coat colour-associated mutations in humans, mice and other mammals.

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Reissmann, Monika; Ludwig, Arne

2013-01-01

The characterisation of the pleiotropic effects of coat colour-associated mutations in mammals illustrates that sensory organs and nerves are particularly affected by disorders because of the shared origin of melanocytes and neurocytes in the neural crest; e.g. the eye-colour is a valuable indicator of disorders in pigment production and eye dysfunctions. Disorders related to coat colour-associated alleles also occur in the skin (melanoma), reproductive tract and immune system. Additionally, the coat colour phenotype of an individual influences its general behaviour and fitness. Mutations in the same genes often produce similar coat colours and pleiotropic effects in different species (e.g., KIT [reproductive disorders, lethality], EDNRB [megacolon] and LYST [CHS]). Whereas similar disorders and similar-looking coat colour phenotypes sometimes have a different genetic background (e.g., deafness [EDN3/EDNRB, MITF, PAX and SNAI2] and visual diseases [OCA2, RAB38, SLC24A5, SLC45A2, TRPM1 and TYR]). The human predilection for fancy phenotypes that ignore disorders and genetic defects is a major driving force for the increase of pleiotropic effects in domestic species and laboratory subjects since domestication has commenced approximately 18,000 years ago. Copyright © 2013 Elsevier Ltd. All rights reserved.

Diagnosis of Clostridium difficile Infection: an Ongoing Conundrum for Clinicians and for Clinical Laboratories

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Carroll, Karen C.

2013-01-01

SUMMARY Clostridium difficile is a formidable nosocomial and community-acquired pathogen, causing clinical presentations ranging from asymptomatic colonization to self-limiting diarrhea to toxic megacolon and fulminant colitis. Since the early 2000s, the incidence of C. difficile disease has increased dramatically, and this is thought to be due to the emergence of new strain types. For many years, the mainstay of C. difficile disease diagnosis was enzyme immunoassays for detection of the C. difficile toxin(s), although it is now generally accepted that these assays lack sensitivity. A number of molecular assays are commercially available for the detection of C. difficile. This review covers the history and biology of C. difficile and provides an in-depth discussion of the laboratory methods used for the diagnosis of C. difficile infection (CDI). In addition, strain typing methods for C. difficile and the evolving epidemiology of colonization and infection with this organism are discussed. Finally, considerations for diagnosing C. difficile disease in special patient populations, such as children, oncology patients, transplant patients, and patients with inflammatory bowel disease, are described. As detection of C. difficile in clinical specimens does not always equate with disease, the diagnosis of C. difficile infection continues to be a challenge for both laboratories and clinicians. PMID:23824374

Tacrolimus Therapy for Three Patients with Elderly-Onset Ulcerative Colitis: Report of Three Cases

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Rumiko Kobayashi

2016-07-01

Full Text Available In recent years, cases of elderly-onset ulcerative colitis (UC have been increasing in number and are currently reported to account for 10–15% of all cases of UC. Although the treatment of UC is essentially similar between older and younger patients, evidence of the therapeutic efficacy of tacrolimus in elderly-onset UC patients is still limited. Herein, we report our attempt to induce remission using tacrolimus in three patients with elderly-onset UC. A 75-year-old Japanese woman, a 71-year-old Japanese man and a 76-year-old Japanese woman with severe elderly-onset UC of the pancolitis type were treated with tacrolimus. Although all three patients showed response to the drug, the eventual outcome was poor in the first patient, who developed toxic megacolon, underwent surgery, and suffered from recurrent infections and hemorrhage after the surgery. However, clinical remission was successfully achieved in the second and third patient. Tacrolimus shows some indication of effectiveness in the treatment of elderly-onset UC. However, in elderly-onset UC patients, it is necessary to keep in mind the higher risk of adverse effects of medical therapy and postoperative complications because of the high comorbidity rates. Moreover, in situations where surgery needs to be considered, it is important to ensure appropriate timing of the surgery.

The roles of host and pathogen factors and the innate immune response in the pathogenesis of Clostridium difficile infection

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Sun, Xingmin; Hirota, Simon A.

2014-01-01

Clostridium difficile (C. difficile) is the most common cause of nosocomial antibiotic-associated diarrhea and the etiologic agent of pseudomembranous colitis. The clinical manifestation of Clostridium difficile infection (CDI) is highly variable, from asymptomatic carriage, to mild self-limiting diarrhea, to the more severe pseudomembranous colitis. Furthermore, in extreme cases, colonic inflammation and tissue damage can lead to toxic megacolon, a condition requiring surgical intervention. C. difficile expresses two key virulence factors; the exotoxins, toxin A (TcdA) and toxin B (TcdB), which are glucosyltransferases that target host-cell monomeric GTPases. In addition, some hypervirulent strains produce a third toxin, binary toxin or C. difficile transferase (CDT), which may contribute to the pathogenesis of CDI. More recently, other factors such as surface layer proteins (SLPs) and flagellin have also been linked to the inflammatory responses observed in CDI. Although the adaptive immune response can influence the severity of CDI, the innate immune responses to C. difficile and its toxins play crucial roles in CDI onset, progression, and overall prognosis. Despite this, the innate immune responses in CDI have drawn relatively little attention from clinical researchers. Targeting these responses may prove useful clinically as adjuvant therapies, especially in refractory and/or recurrent CDI. This review will focus on recent advances in our understanding of how C. difficile and its toxins modulate innate immune responses that contribute to CDI pathogenesis. PMID:25242213

Importancia de la Manometría Anorrectalen la Infancia

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Daisy Naranjo Hernández

1999-09-01

Full Text Available RESUMEN Al registrar cambios de presión en órganos musculares huecos, el estudio manométrico anorrectal ha permitido llegar a conclusiones acerca del mecanismo de continencia anorrectal, la función esfinteriana y la sensación rectal. Se utilizó el método de catéteres perfundidos y balón intrarrectal para el estímulo por distensión. Se estudiaron 340 pacientes, 214 masculinos y 126 femeninos, con una edad promedio de 6,5 años. Se formaron 3 grupos: I. Constipación crónica, II. Crónica más encopresis y III. Incontinencia anal. Entre las variables manométricas que se estudiaron: presencia o no del reflejo rectoanal inhibitorio inducido (RRII, presión de reposo, longitud, tanto por ciento de relajación del mismo y volumen crítico capaz de desencadenar respuesta esfinteriana. Se realizó estudio de colon por enema y se halló mayor correlación entre megacolon radiográfico y el mayor porcentaje de ausencia manométrica del RRAI. El grupo II mostró mayor valor de presión basal del EAI con un menor tanto por ciento de relajación y un mayor número de casos con ausencia del RRAI. En el grupo III se obtuvo el valor más bajo de presión basal del EAI mayor por ciento de relajación y mayor número de casos con ausencia del EAI. Se demuestra la importancia del estudio en el manejo integral del niño con afecciones anorrectales, el que fue indispensable en el diagnóstico de la enfermedad de Hirschprung. Su empleo se recomienda en todos los servicios de Gastroenterología Pediátrica.On registering pressure changes in the muscular hollow organs, the anorectal manometric study has allowed to reach conclusions about the mechanism of anorectal continence, the sphincteral function and the anorectal sensation. The method of perfused catheters and intrarectal balloon was used to attain the stimulus by distention. 340 patients, 214 males and 126 females with an average age of 6.5 years old, were studied. Patients were divided into 3 groups

Molecular epidemiology and antimicrobial susceptibility of Clostridium difficile isolated from the Chinese People’s Liberation Army General Hospital in China

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Rui Wang

2018-02-01

Full Text Available Clostridium difficile is a Gram-positive, spore-forming obligate anaerobe responsible for antibiotic-associated diarrhoea. Its virulence is associated with the production of endotoxins A and B and endospores, which can cause symptoms, such as diarrhoea, toxic megacolon, and pseudomembranous colitis. Given the increasing elderly population and the well-recognized problem of over-prescribing of broad-spectrum antibiotics, it is critical to have an understanding of molecular epidemiology and antimicrobial susceptibility in China. This study analyzed the toxin types and multilocus sequence typing (MLST results of 74 clinical isolates of C. difficile after the glutamate dehydrogenase (GDH screening test and anaerobic culture. The minimum inhibitory concentrations (MICs of four different antibiotics were determined for all of the isolates, and the bacterial resistance mechanisms were investigated. Sixty-five strains (75% were toxigenic, including 54 tcdA-positive, tcdB-positive, and cdtA/cdtB-negative strains (A+B+CDT− and nine A−B+CDT− strains. Eleven strains (14.9% were non-toxigenic. All clinical isolates were classified into 26 MLST genotypes, with the predominant type being ST-54 (18.9%. All isolates were susceptible to vancomycin. The tetracycline, clindamycin, and levofloxacin resistance rates were 1.4%, 36.5%, and 20.3%, respectively. The expression of tet(M, erm(B, and mutations of gyrA and/or gyrB were observed in the tetracycline-, clindamycin-, and levofloxacin-resistant isolates, respectively.

Internal obturator muscle transposition for treatment of perineal hernia in dogs: 34 cases (1998-2012).

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Shaughnessy, Magen; Monnet, Eric

2015-02-01

To evaluate the outcome of dogs with perineal hernia treated with transposition of the internal obturator muscle. Retrospective case series. 34 dogs. Medical records of dogs with perineal hernia surgically treated from 1998 to 2012 were reviewed. Diagnostic methods and surgical techniques were recorded. Dogs were assigned preoperative and postoperative clinical sign scores. Complication and recurrence rates were evaluated over time. Risk factors were determined. Median follow-up time was 345 days (range, 22 to 1,423 days). Complications were observed in 10 dogs. Tenesmus (n = 9), dyschezia (7), fecal impaction (3), stranguria (4), hematochezia (2), urinary incontinence (2), diarrhea (1), urinary tract infection (1), and megacolon (1) occurred following surgery. Bladder retroflexion at the time of initial evaluation or surgery was not a risk factor for complication (hazard ratio, 1.72). One year after surgery, 51.2% dogs were free of complications. Three dogs developed a perineal hernia on the contralateral side between 35 and 95 days after surgery. The 1-year recurrence rate was 27.4%. Median time for recurrence was 28 days after surgery (range, 2 to 364 days). Postoperative tenesmus was a risk factor for the development of recurrence (hazard ratio, 2.29). Internal obturator muscle transposition was used for primary repair of perineal hernia in dogs. Recurrence was recorded as long as 1 year after surgery. Tenesmus was a risk factor for the development of recurrence after treatment of perineal hernia with internal obturator muscle transposition.

Disrupted SOX10 function causes spongiform neurodegeneration in gray tremor mice

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Anderson, Sarah R.; Lee, Inyoul; Ebeling, Christine; Stephenson, Dennis A.; Schweitzer, Kelsey M.; Baxter, David; Moon, Tara M.; LaPierre, Sarah; Jaques, Benjamin; Silvius, Derek; Wegner, Michael; Hood, Leroy E.; Carlson, George; Gunn, Teresa M.

2014-01-01

Mice homozygous for the gray tremor (gt) mutation have a pleiotropic phenotype that includes pigmentation defects, megacolon, whole body tremors, sporadic seizures, hypo- and dysmyelination of the CNS and PNS, vacuolation of the CNS, and early death. Vacuolation similar to that caused by prions was originally reported to be transmissible, but subsequent studies showed the inherited disease was not infectious. The gt mutation mapped to distal mouse chromosome 15, to the same region as Sox10, which encodes a transcription factor with essential roles in neural crest survival and differentiation. As dominant mutations in mouse or human SOX10 cause white spotting and intestinal aganglionosis, we screened the Sox10 coding region for mutations in gt/gt DNA. An adenosine to guanine transversion was identified in exon 2 that changes a highly conserved glutamic acid residue in the SOX10 DNA binding domain to glycine. This mutant allele was not seen in wildtype mice, including the related GT/Le strain, and failed to complement a Sox10 null allele. Gene expression analysis revealed significant down-regulation of genes involved in myelin lipid biosynthesis pathways in gt/gt brains. Knockout mice for some of these genes develop CNS vacuolation and/or myelination defects, suggesting that their down-regulation may contribute to these phenotypes in gt mutants and could underlie the neurological phenotypes associated with Peripheral demyelinating neuropathy-Central dysmyelinating leukodystrophy-Waardenburg syndrome-Hirschsprung (PCWH) disease, caused by mutations in human SOX10. PMID:25399070

NEONATAL SURGERY, A STUDY OF TWO YEARS AT NELSON MANDELA ACADEMIC HOSPITAL, MTHATHA, EASTERN CAPE.

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Delgado, A; Cejas, A; Bangasa, D

2017-09-01

Advances in diagnostic techniques and perioperative care have greatly improved the outcome of neonatal surgery. Despite this, disparity still exists in the outcome of neonatal surgery between developed and developing countries. We performed a prospective study of neonates admitted and treated due to surgical congenital diseases and other conditions in our hospital from April 2015 to April 2017. There were 19 (28,7%) females and 47 (70,3%) males in this group. It was found that 41 neonates had 7 days after birth or less by a 62% and 25 with more than 7 days by 38%. The Anorectal malformations (ARM) were the most frequent congenital anomaly in 21 patients (47%), followed by Gastroquises with 7 neonates (10,6%), Omphalocele with 8 (12%), and Oesophagus Atresia in 5 neonates (7,5%). Intestinal Malrotation with midgut volvulus, Pyloric Stenoses and Duodenal Atresia in 4 neonates (6,2 % each). Others alterations such as, Ileal Atresia, Strangulated Inguinal Hernia, Limb Gangrene, Necrotizing Enterocolitis, Sacro Coxigeal Theratoma, Megacolon Aganglionic, Colon perforation, Gastric perforation and Hydromethrocolpus accounted in 13 neonates by 19,7% from the total of patients. Ten babies died (15%). We conclude that Anorectal Malformations, Gastroquises and Omphalocele were the most frequent malformations. Considering the mortality is above the average of developed countries this could be improved by increasing the knowledge about the neonatal surgery characteristics among medical doctors and improving the necessary facilities and back up.

Clostridium difficile – From Colonization to Infection

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Schäffler, Holger; Breitrück, Anne

2018-01-01

Clostridium difficile is the most frequent cause of nosocomial antibiotic-associated diarrhea. The incidence of C. difficile infection (CDI) has been rising worldwide with subsequent increases in morbidity, mortality, and health care costs. Asymptomatic colonization with C. difficile is common and a high prevalence has been found in specific cohorts, e.g., hospitalized patients, adults in nursing homes and in infants. However, the risk of infection with C. difficile differs significantly between these cohorts. While CDI is a clear indication for therapy, colonization with C. difficile is not believed to be a direct precursor for CDI and therefore does not require treatment. Antibiotic therapy causes alterations of the intestinal microbial composition, enabling C. difficile colonization and consecutive toxin production leading to disruption of the colonic epithelial cells. Clinical symptoms of CDI range from mild diarrhea to potentially life-threatening conditions like pseudomembranous colitis or toxic megacolon. While antibiotics are still the treatment of choice for CDI, new therapies have emerged in recent years such as antibodies against C. difficile toxin B and fecal microbial transfer (FMT). This specific therapy for CDI underscores the role of the indigenous bacterial composition in the prevention of the disease in healthy individuals and its role in the pathogenesis after alteration by antibiotic treatment. In addition to the pathogenesis of CDI, this review focuses on the colonization of C. difficile in the human gut and factors promoting CDI. PMID:29692762

Ogilvie′s syndrome following posterior spinal arthrodesis for scoliosis

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Athanasios I Tsirikos

2013-01-01

Full Text Available We report Ogilvie′s syndrome following posterior spinal arthrodesis on a patient with thoracic and lumbar scoliosis associated with intraspinal anomalies. Postoperative paralytic ileus can commonly complicate scoliosis surgery. Ogilvie′s syndrome as a cause of abdominal distension and pain has not been reported following spinal deformity correction and can mimic post-surgical ileus. 12 year old female patient with double thoracic and lumbar scoliosis associated with Arnold-Chiari 1 malformation and syringomyelia. The patient underwent posterior spinal fusion from T 4 to L 3 with segmental pedicle screw instrumentation and autogenous iliac crest grafting. She developed abdominal distension and pain postoperatively and this deteriorated despite conservative management. Repeat ultrasounds and abdominal computer tomography scans ruled out mechanical obstruction. The clinical presentation and blood parameters excluded toxic megacolon and cecal volvulus. As the symptoms persisted, a laparotomy was performed on postoperative day 16, which demonstrated ragged tears of the colon and cecum. A right hemi-colectomy followed by ileocecal anastomosis was required. The pathological examination of surgical specimens excluded inflammatory bowel disease and vascular abnormalities. The patient made a good recovery following bowel surgery and at latest followup 3.2 years later she had no abdominal complaints and an excellent scoliosis correction. Ogilvie′s syndrome should be included in the differential diagnosis of postoperative ileus in patients developing prolonged unexplained abdominal distension and pain after scoliosis correction. Early diagnosis and instigation of conservative management can prevent major morbidity and mortality due to bowel ischemia and perforation.

Per rectal endoscopic myotomy for the treatment of adult Hirschsprung's disease: First human case (with video).

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Bapaye, Amol; Wagholikar, Gajanan; Jog, Sameer; Kothurkar, Aditi; Purandare, Shefali; Dubale, Nachiket; Pujari, Rajendra; Mahadik, Mahesh; Vyas, Viral; Bapaye, Jay

2016-09-01

Hirschsprung's disease (HD) is a congenital disorder characterized by the absence of intrinsic ganglion cells in submucosal and myenteric plexuses of the hindgut; and presents with constipation, intestinal obstruction and/or megacolon. HD commonly involves the rectosigmoid region (short segment HD), although shorter and longer variants of the disease are described. Standard treatment involves pull-through surgery for short segment HD or posterior anorectal myotomy in selected ultrashort segment candidates. Third space endoscopy has evolved during the past few years. Per oral endoscopic myotomy and per oral pyloromyotomy are described for treatment of achalasia cardia and refractory gastroparesis, respectively. Using the same philosophy of muscle/sphincter disruption for spastic bowel segments, per rectal endoscopic myotomy could be considered as a treatment option for short segment HD. A 24-year-old male patient presented with refractory constipation since childhood, and habituated to high-dose laxative combinations. Diagnosis was confirmed as adult short segment HD by barium enema, colonoscopic deep suction mucosal biopsies and anorectal manometry. Histopathology confirmed aganglionosis in the distal 15 cm. By implementing principles of third space endoscopy, per rectal endoscopic myotomy 20 cm in length was successfully carried out. At 24-week follow up, the patient reported significant relief of constipation and associated symptoms. Sigmoidoscopy, anorectal manometry and barium enema confirm improved rectal distensibility and reduced rectal pressures. The present case report describes the first human experience of per rectal endoscopic myotomy for successful treatment of adult short segment HD. © 2016 Japan Gastroenterological Endoscopy Society.

Interstitial cells of Cajal in chagasic megaesophagus.

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de Lima, Marcus Aurelho; Cabrine-Santos, Marlene; Tavares, Marcelo Garcia; Gerolin, Gustavo Pacheco; Lages-Silva, Eliane; Ramirez, Luis Eduardo

2008-08-01

Chagasic visceromegalies are the most important digestive manifestations of Chagas disease and are characterized by motor disorders and dilation of organs such as esophagus and colon. One of the theories raised to explain the physiopathogenesis of chagasic megas is the plexus theory. Recent studies have shown a reduction of interstitial cells of Cajal (ICCs) in the colon of chagasic patients. These cells are present throughout the gastrointestinal tract and are considered to be pacemaker cells, that is, they are responsible for coordinating peristalsis and for mediating nerve impulses. In view of the lack of studies on these cells in megaesophagus and the previous observation of a reduction of ICCs in chagasic megacolons, we compared the distribution of ICCs in the esophagus of chagasic and nonchagasic patients to contribute to a better understanding of the physiopathogenesis of this esophageal disease. Esophageal biopsy samples from 10 chagasic and 5 nonchagasic patients were used. Cells were identified with the anti-CD117 antibody. The number of ICCs was quantified in longitudinal and circular muscle layers and myenteric plexus. The results were analyzed statistically by comparison of means. An intense reduction in the number of ICCs was observed in muscle layers and in the myenteric plexus of patients with megaesophagus. We conclude that there is an intense reduction of ICCs in the esophagus of chagasic patients when compared to nonchagasic patients, a finding supporting the important role of these cells in gastrointestinal tract motility. A deficiency in these cells might be implied in the genesis of megaesophagus.

The host immune response to Clostridium difficile infection

Science.gov (United States)

2013-01-01

Clostridium difficile infection (CDI) is the most common infectious cause of healthcare-acquired diarrhoea. Outcomes of C. difficile colonization are varied, from asymptomatic carriage to fulminant colitis and death, due in part to the interplay between the pathogenic virulence factors of the bacterium and the counteractive immune responses of the host. Secreted toxins A and B are the major virulence factors of C. difficile and induce a profound inflammatory response by intoxicating intestinal epithelial cells causing proinflammatory cytokine release. Host cell necrosis, vascular permeability and neutrophil infiltration lead to an elevated white cell count, profuse diarrhoea and in severe cases, dehydration, hypoalbuminaemia and toxic megacolon. Other bacterial virulence factors, including surface layer proteins and flagella proteins, are detected by host cell surface signal molecules that trigger downstream cell-mediated immune pathways. Human studies have identified a role for serum and faecal immunoglobulin levels in protection from disease, but the recent development of a mouse model of CDI has enabled studies into the precise molecular interactions that trigger the immune response during infection. Key effector molecules have been identified that can drive towards a protective anti-inflammatory response or a damaging proinflammatory response. The limitations of current antimicrobial therapies for CDI have led to the development of both active and passive immunotherapies, none of which have, as yet been formally approved for CDI. However, recent advances in our understanding of the molecular basis of host immune protection against CDI may provide an exciting opportunity for novel therapeutic developments in the future. PMID:25165542

Deletions at the SOX10 gene locus cause Waardenburg syndrome types 2 and 4.

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Bondurand, Nadege; Dastot-Le Moal, Florence; Stanchina, Laure; Collot, Nathalie; Baral, Viviane; Marlin, Sandrine; Attie-Bitach, Tania; Giurgea, Irina; Skopinski, Laurent; Reardon, William; Toutain, Annick; Sarda, Pierre; Echaieb, Anis; Lackmy-Port-Lis, Marilyn; Touraine, Renaud; Amiel, Jeanne; Goossens, Michel; Pingault, Veronique

2007-12-01

Waardenburg syndrome (WS) is an auditory-pigmentary disorder that exhibits varying combinations of sensorineural hearing loss and abnormal pigmentation of the hair and skin. Depending on additional symptoms, WS is classified into four subtypes, WS1-WS4. Absence of additional features characterizes WS2. The association of facial dysmorphic features defines WS1 and WS3, whereas the association with Hirschsprung disease (aganglionic megacolon) characterizes WS4, also called "Waardenburg-Hirschsprung disease." Mutations within the genes MITF and SNAI2 have been identified in WS2, whereas mutations of EDN3, EDNRB, and SOX10 have been observed in patients with WS4. However, not all cases are explained at the molecular level, which raises the possibility that other genes are involved or that some mutations within the known genes are not detected by commonly used genotyping methods. We used a combination of semiquantitative fluorescent multiplex polymerase chain reaction and fluorescent in situ hybridization to search for SOX10 heterozygous deletions. We describe the first characterization of SOX10 deletions in patients presenting with WS4. We also found SOX10 deletions in WS2 cases, making SOX10 a new gene of WS2. Interestingly, neurological phenotypes reminiscent of that observed in WS4 (PCWH syndrome [peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, WS, and Hirschsprung disease]) were observed in some WS2-affected patients with SOX10 deletions. This study further characterizes the molecular complexity and the close relationship that links the different subtypes of WS.

Biologics in the management of ulcerative colitis – comparative safety and efficacy of TNF-α antagonists

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Fausel R

2015-01-01

Full Text Available Rebecca Fausel,1 Anita Afzali1,2 1Division of Gastroenterology, Department of Medicine, University of Washington, Seattle, WA, USA; 2Inflammatory Bowel Disease Program, UW Medicine – Harborview Medical Center, Seattle, WA, USA Abstract: Ulcerative colitis can cause debilitating symptoms and complications such as colonic strictures, colonic dysplasia, colorectal cancer, and toxic megacolon or perforation. Goals of treatment in ulcerative colitis include resolution of gastrointestinal symptoms, healing of colonic mucosa, and prevention of disease complications. Our treatment armamentarium has expanded dramatically over the past 10 years, and we now have multiple biologic agents approved for the treatment of moderate-severe disease, in addition to conventional therapies such as 5-aminosalicylates, thiopurines, and corticosteroids. In this review, we will provide a detailed discussion of the three tumor necrosis factor-alpha (TNF-α inhibitors currently approved for treatment of ulcerative colitis: infliximab, adalimumab, and golimumab. All three agents are effective for inducing and maintaining clinical response and remission in patients with ulcerative colitis, and they have comparable safety profiles. There are no head-to-head trials comparing their efficacy, and the choice of agent is most often based on insurance coverage, route of administration, and patient preference. Combination therapy with an immunomodulator is proven to be more effective than anti-TNF monotherapy, and patients who lose response to an anti-TNF agent should undergo dose intensification in order to regain clinical response. Despite therapeutic optimization, a significant percentage of patients will not achieve clinical remission with anti-TNF agents, and so newer therapies are on the horizon. Keywords: ulcerative colitis, inflammatory bowel disease, infliximab, adalimumab, golimumab

Inflammatory chronic disease of the colon: How to image

International Nuclear Information System (INIS)

Ambrosini, Roberta; Barchiesi, Annalisa; Di Mizio, Veronica; Di Terlizzi, Marco; Leo, Luca; Filippone, Antonella; Canalis, Luigi; Fossaceca, Rita; Carriero, Alessandro

2007-01-01

Inflammatory bowel disease, including Crohn's disease and UC, is a chronic disorder of the gastrointestinal tract. The inflammatory process in UC is confined to the mucosa and submucosa and it involves only the colon. In contrast, in Crohn's disease the inflammation process extends through the bowel wall layers and it can involve any part of gastrointestinal tract. Moreover, inflammatory bowel disease of the colon may be associated with complications, such as toxic megacolon, fulminant colitis, acute bleeding, fistulas and abscesses. Radiographic imaging studies are useful for the diagnosis of inflammatory bowel disease, and may be used to assess the extent and severity of disease, rule out complications, and monitor the response to therapy. The double-contrast barium study is a valuable technique for diagnosing inflammatory bowel disease colonic alterations, even in patients with early mucosal abnormalities. The earliest finding of UC is characterized by a fine granular appeareance of the colonic mucosa, usually involving the rectosigmoid junction. In chronic UC double-contrast enema may reveal marked colonic shortening with tubular narrowing of the bowel and loss of haustration. The earliest radiographics findings of Crohn's disease are represented by aphthous ulcers. As disease progresses, aphthous ulcers may enlarge and coalesce to form stellate or linear areas of ulceration. In advanced Crohn's disease, transmural ulceration may lead to the development of fissures, sinus tracts, fistulas, and abscesses. Cross sectional studies such as computed tomography, magnetic resonance imaging and sometimes ultrasound, are useful alternative tools not only in the assessment of bowel wall abnormalities, but also for the assessment of extraluminal alterations in patients with advanced disease

C. difficile 630Δerm Spo0A regulates sporulation, but does not contribute to toxin production, by direct high-affinity binding to target DNA.

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Katharina E Rosenbusch

Full Text Available Clostridium difficile is a Gram positive, anaerobic bacterium that can form highly resistant endospores. The bacterium is the causative agent of C. difficile infection (CDI, for which the symptoms can range from a mild diarrhea to potentially fatal pseudomembranous colitis and toxic megacolon. Endospore formation in Firmicutes, including C. difficile, is governed by the key regulator for sporulation, Spo0A. In Bacillus subtilis, this transcription factor is also directly or indirectly involved in various other cellular processes. Here, we report that C. difficile Spo0A shows a high degree of similarity to the well characterized B. subtilis protein and recognizes a similar binding sequence. We find that the laboratory strain C. difficile 630Δerm contains an 18bp-duplication near the DNA-binding domain compared to its ancestral strain 630. In vitro binding assays using purified C-terminal DNA binding domain of the C. difficile Spo0A protein demonstrate direct binding to DNA upstream of spo0A and sigH, early sporulation genes and several other putative targets. In vitro binding assays suggest that the gene encoding the major clostridial toxin TcdB may be a direct target of Spo0A, but supernatant derived from a spo0A negative strain was no less toxic towards Vero cells than that obtained from a wild type strain, in contrast to previous reports. These results identify for the first time direct (putative targets of the Spo0A protein in C. difficile and make a positive effect of Spo0A on production of the large clostridial toxins unlikely.

Syndromic Hirschsprung's disease and associated congenital heart disease: a systematic review.

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Duess, Johannes W; Puri, Prem

2015-08-01

Hirschsprung's disease (HD) occurs as an isolated phenotype in 70% of infants and is associated with additional congenital anomalies or syndromes in approximately 30% of patients. The cardiac development depends on neural crest cell proliferation and is closely related to the formation of the enteric nervous system. HD associated with congenital heart disease (CHD) has been reported in 5-8% of cases, with septation defects being the most frequently recorded abnormalities. However, the prevalence of HD associated with CHD in infants with syndromic disorders is not well documented. This systematic review was designed to determine the prevalence of CHD in syndromic HD. A systematic review of the literature using the keywords "Hirschsprung's disease", "aganglionosis", "congenital megacolon", "congenital heart disease" and "congenital heart defect" was performed. Resulting publications were reviewed for epidemiology and morbidity. Reference lists were screened for additional relevant studies. A total of fifty-two publications from 1963 to 2014 reported data on infants with HD associated with CHD. The overall reported prevalence of HD associated with CHD in infants without chromosomal disorders was 3%. In infants with syndromic disorders, the overall prevalence of HD associated with CHD ranged from 20 to 80 % (overall prevalence 51%). Septation defects were recorded in 57% (atrial septal defects in 29%, ventricular septal defects in 32%), a patent ductus arteriosus in 39%, vascular abnormalities in 16%, valvular heart defects in 4% and Tetralogy of Fallot in 7%. The prevalence of HD associated with CHD is much higher in infants with chromosomal disorders compared to infants without associated syndromes. A routine echocardiogram should be performed in all infants with syndromic HD to exclude cardiac abnormalities.

Rapid change of fecal microbiome and disappearance of Clostridium difficile in a colonized infant after transition from breast milk to cow milk.

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Davis, Manli Y; Zhang, Husen; Brannan, Lera E; Carman, Robert J; Boone, James H

2016-10-07

Clostridium difficile is the most common known cause of antibiotic-associated diarrhea. Upon the disturbance of gut microbiota by antibiotics, C. difficile establishes growth and releases toxins A and B, which cause tissue damage in the host. The symptoms of C. difficile infection disease range from mild diarrhea to pseudomembranous colitis and toxic megacolon. Interestingly, 10-50 % of infants are asymptomatic carriers of C. difficile. This longitudinal study of the C. difficile colonization in an infant revealed the dynamics of C. difficile presence in gut microbiota. Fifty fecal samples, collected weekly between 5.5 and 17 months of age from a female infant who was an asymptomatic carrier of C. difficile, were analyzed by 16S rRNA gene sequencing. Colonization switching between toxigenic and non-toxigenic C. difficile strains as well as more than 100,000-fold fluctuations of C. difficile counts were observed. C. difficile toxins were detected during the testing period in some infant stool samples, but the infant never had diarrhea. Although fecal microbiota was stable during breast feeding, a dramatic and permanent change of microbiota composition was observed within 5 days of the transition from human milk to cow milk. A rapid decline and eventual disappearance of C. difficile coincided with weaning at 12.5 months. An increase in the relative abundance of Bacteroides spp., Blautia spp., Parabacteroides spp., Coprococcus spp., Ruminococcus spp., and Oscillospira spp. and a decrease of Bifidobacterium spp., Lactobacillus spp., Escherichia spp., and Clostridium spp. were observed during weaning. The change in microbiome composition was accompanied by a gradual increase of fecal pH from 5.5 to 7. The bacterial groups that are less abundant in early infancy, and that increase in relative abundance after weaning, likely are responsible for the expulsion of C. difficile.

Supplementation with 0.1% and 2% vitamin e in diabetic rats: analysis of myenteric neurons immunostained for myosin-V and nNOS in the jejunum

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Eleandro Aparecido Tronchini

2012-12-01

Full Text Available CONTEXT: Diabetes mellitus is a disease characterized by hyperglycemia that, when allowed to progress long-term untreated, develops vascular and neurological complications, which are responsible for the development of alterations in the enteric nervous system in diabetic patients. In the gastrointestinal tract, diabetes mellitus promotes motor and sensory changes, and in the reflex function of this system, causing gastroparesis, diarrhea, constipation, megacolon, slow gastrointestinal transit, gastric stasis and dilation with decreased or increased peristaltic contractions. Several studies have shown that oxidative stress is the main responsible for the vascular and neurological complications affecting the enteric nervous system of diabetics. OBJECTIVE: The effects of 0.1% and 2% vitamin E on myosin-V- and nNOS-immunoreactive neurons in the jejunum of diabetic rats were investigated. METHODS: Thirty rats were divided into the groups: normoglycemic, normoglycemic treated with 0.1% vitamin E, normoglycemic treated with 2% vitamin E, diabetic, diabetic treated with 0.1% vitamin E, and diabetic treated with 2% vitamin E. The neuronal density and areas of neuron cell bodies were determined. RESULTS: Diabetes (diabetic group significantly reduced the number of myosin-V-immunoreactive neurons compared with the normoglycemic group. The diabetic treated with 0.1% vitamin E and diabetic treated with 2% vitamin E groups did not exhibit a greater density than the D group (P>0.05. Nitrergic density did not change with diabetes (P>0.05. The areas of myosin-V- and nNOS-immunoreactive neurons significantly increased in the normoglycemic treated with 2% vitamin E and diabetic groups compared with the normoglycemic group. CONCLUSION: Supplementation with 2% vitamin E had a neurotrophic effect only in the area of myosin-V-immunoreactive neurons compared with the diabetic group.

Clostridium difficile Diarrhea in the Elderly: Current Issues and Management Options.

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Mizusawa, Masako; Doron, Shira; Gorbach, Sherwood

2015-08-01

Clostridium difficile infection (CDI) is the most common cause of infectious diarrhea in healthcare settings. Along with antimicrobial exposure, advanced age has been shown to be a significant risk factor for the development and recurrence of, and mortality from, CDI. The substantial burden of CDI in the elderly may be related to frequent healthcare exposure, the necessity for more medications, altered intestinal microbiota, and complicated comorbidities. A diagnosis of CDI is based on evidence of toxin, or the C. difficile organism itself, in a stool sample in the presence of clinical signs and symptoms. Only symptomatic patients should be tested for CDI, and routine surveillance or repeat testing on asymptomatic patients as a test of cure is discouraged. Antibiotic discontinuation alone can improve or resolve CDI in some patients, and concomitant use of antibiotics is associated with decreased response to CDI treatment. Metronidazole, vancomycin, and fidaxomicin are the therapeutic agents currently available for CDI, with the selection of these agents being based on disease severity, history of recurrence, and cost. The recurrence rate after initial treatment is 20-30%. The first recurrence can be treated with the same therapeutic agent and, for subsequent recurrences, vancomycin in a tapered and/or pulsed regimen is recommended. Fecal microbiota transplantation has shown remarkable effectiveness for recurrent anti-refractory CDI, although caution is advised in treating immunocompromised hosts and those with toxic megacolon. C. difficile can be transmitted directly and indirectly via contact with patients or their environment; therefore, isolation precautions should be initiated at the first suspicion of CDI. C. difficile spores can survive for a long time on environmental surfaces, and the patient's room and all equipment used in the room should be disinfected. In order to manage CDI in the elderly, timely diagnosis, appropriate treatment based on severity of

Plain abdominal radiographs in patients with Crohn’s disease: Radiological findings and diagnostic value

International Nuclear Information System (INIS)

O’Regan, K.; O’Connor, O.J.; O’Neill, S.B.; Mc Laughlin, P.D.; Desmond, A.; McWilliams, S.R.; Quigley, E.M.M.; Shanahan, F.; Maher, M.M.

2012-01-01

Aim: To determine the diagnostic yield and clinical value of plain film of the abdomen (PFA) in Crohn’s disease (CD) patients and to determine whether performance of PFA yields definitive diagnostic information or whether additional imaging examinations are required. Materials and methods: One hundred and seventy-seven CD patients underwent 643 PFAs during the period September 1992 to August 2008. Two radiologists blinded to the clinical details independently evaluated individual PFAs and/or their reports for abnormal findings using the following criteria: normal, small bowel (SB) findings; colonic findings, acute CD complications, extra-colonic findings; global assessment/impression. The results of additional imaging studies performed within 5 days of PFA were recorded and findings were analysed. Results: A mean of 3.6 (range 1–22) PFAs was performed per patient during the study period. Almost 70% of films were normal (n = 449). SB abnormalities were detected in 21.8% (n = 140) PFAs; most commonly dilated loops (18.8%, n = 121) and mucosal oedema (5%, n = 32). Colonic abnormalities were present in 11.4% (n = 73); most commonly mucosal oedema (7.5%, n = 48) and dilated loops (5%, n = 32). Four cases of pneumoperitoneum were detected. There was no case of toxic megacolon. There was one case in which intra-abdominal abscess/collection was suspected and two cases of obstruction/ileus. Extracolonic findings (renal calculi, sacro-iliitis, etc.) were identified in 7.5% (n = 48). PFAs were followed by additional abdominal imaging within 5 days of PFA in 273/643 (42.5%) of cases. Conclusion: Despite the high rates of utilization of PFA in CD patients, there is a low incidence of abnormal findings (32.5%). Many of the findings are non-specific and clinically irrelevant and PFA is frequently followed by additional abdominal imaging examinations.

Overdiagnosis of Clostridium difficile Infection in the Molecular Test Era.

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Polage, Christopher R; Gyorke, Clare E; Kennedy, Michael A; Leslie, Jhansi L; Chin, David L; Wang, Susan; Nguyen, Hien H; Huang, Bin; Tang, Yi-Wei; Lee, Lenora W; Kim, Kyoungmi; Taylor, Sandra; Romano, Patrick S; Panacek, Edward A; Goodell, Parker B; Solnick, Jay V; Cohen, Stuart H

2015-11-01

Clostridium difficile is a major cause of health care-associated infection, but disagreement between diagnostic tests is an ongoing barrier to clinical decision making and public health reporting. Molecular tests are increasingly used to diagnose C difficile infection (CDI), but many molecular test-positive patients lack toxins that historically defined disease, making it unclear if they need treatment. To determine the natural history and need for treatment of patients who are toxin immunoassay negative and polymerase chain reaction (PCR) positive (Tox-/PCR+) for CDI. Prospective observational cohort study at a single academic medical center among 1416 hospitalized adults tested for C difficile toxins 72 hours or longer after admission between December 1, 2010, and October 20, 2012. The analysis was conducted in stages with revisions from April 27, 2013, to January 13, 2015. Patients undergoing C difficile testing were grouped by US Food and Drug Administration-approved toxin and PCR tests as Tox+/PCR+, Tox-/PCR+, or Tox-/PCR-. Toxin results were reported clinically. Polymerase chain reaction results were not reported. The main study outcomes were duration of diarrhea during up to 14 days of treatment, rate of CDI-related complications (ie, colectomy, megacolon, or intensive care unit care) and CDI-related death within 30 days. Twenty-one percent (293 of 1416) of hospitalized adults tested for C difficile were positive by PCR, but 44.7% (131 of 293) had toxins detected by the clinical toxin test. At baseline, Tox-/PCR+ patients had lower C difficile bacterial load and less antibiotic exposure, fecal inflammation, and diarrhea than Tox+/PCR+ patients (P difficile by either method. Exclusive reliance on molecular tests for CDI diagnosis without tests for toxins or host response is likely to result in overdiagnosis, overtreatment, and increased health care costs.

Radiological evaluation of surgical emergencies in neonate

International Nuclear Information System (INIS)

Suh, C. O.; Oh, K. K.; Park, C. Y.

1980-01-01

Most pathologic conditions requiring emergent operation in neonate are congenital anomalies and delayed diagnosis and associated anomaly are important factors which have contributed to the high mortality rate of congenital anomalies. To prevent this delay, early recognition of the danger signals, adequate roentagenologic examination and accurate diagnosis should be made. Furthermore radiologists should be aware of developing mechanism, clinical manifestations and roentgenographic findings of those neonatal emergencies. 135 cases of neonatal emergencies were analyzed at this point of view, which verified by surgery and pathologic examination at Yonsei University College of Medicine, Severance Hospital since 1968. Embryology and characteristic roentgenographic pictures of each disease were discussed. The conclusions are as follows; 1. Most cases (110/135) presented intestinal obstruction. Imperforate anus (29 cases) was most common disease which followed by infantile hypertrophic pyloric stenosis (25 cases), small bowel atresia and stenosis (18 cases), congenital megacolon (15 cases) and esophageal atresia (14 cases). 2. Clinical Type and time of occurrence of symptoms and signs were so characteristic that these were helpful for differential diagnosis. 3. In infantile hypertrophic pyloric stenosis, confirmative diagnosis could be made in plain abdominal film, when 'Caterpillar sign' was seen. 4. When small bowel obstruction was suspected in plain abdominal film, barium enema examination was more helpful than upper G-I study. When microcolon was found, lower small bowel obstruction was highly suggested. 5. Diagnosis of midgut malrotation was possible in larger cases (4/7). Upper G-I examination was more valuable than barium enema study, because duodenal obstruction due to Ladd's band was common problem in neonate. 6. In neonatal period, diagnosis of aganglionosis could be made with the finding of barium stasis on 24-48 hours delay film, even though no demonstration of

Enteric Neural Cells From Hirschsprung Disease Patients Form Ganglia in Autologous Aneuronal ColonSummary

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Benjamin N. Rollo

2016-01-01

� patient colon and can form ENS-like structures in aneuronal colonic muscle from the same patient. Keywords: Hirschsprung Disease, Aganglionosis, Cell Therapy, Megacolon, Enteric Nervous System

Morbidade da doença de Chagas: I - Estudo de casos procedentes de vários estados do Brasil, observados no Rio de Janeiro Morbidity of Chagas' disease: I - Study of cases originating from various states of Brazil, observed in Rio de Janeiro

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J. Rodrigues Coura

1983-09-01

Full Text Available Um estudo da morbidade através de exames clínicos, eletrocardiográficos, radiológicos, sorológicos, xenodiagnósticos e outros exames laboratoriais seriados, foi feito em 510 pacientes com sorologia positiva para doença de Chagas, procedentes de vários Estados do Brasil e observados no Rio de Janeiro a partir de 1960. Os pacientes foram classificados, de acordo com a forma clínica, em assintomáticos (forma indeterminada, cardíacos, portadores de "megas" ou com formas clínicas associadas. Foi observada uma prevalência de cardiopatia em 52,1% dos pacientes, de "megas" em 14,3% e de associação entre cardiopatia e "megas" em 10,7% e entre megaesôfago e megacolon em 10,9% dos casos. A forma indeterminada (assintomática foi observada em 39% dos pacientes. A proporção de casos de cardiopatia aumentou progressivamente da 1ª a 5ª décadas de vida, enquanto a dos "megas" continuou aumentando até a 7ª década. Entretanto, em número de casos o pico de ambas as formas ocorreu na 4ª década. Não houve diferenças significativas de formas clínicas com relação ao sexo, apesar de uma discreta predominância de cardiopatia no sexo masculino e de "megas" no sexo feminino. Com relação à raça, entre os pacientes classificados como brancos, pretos e mestiços, não foi possível determinar a significância entre as diversas formas clínicas, por desconhecimento da constituição do universo da procedência de cada paciente. Embora o reduzido número de casos não possa ser considerado como representativo da prevalência das fomas clínicas nas regiões de origem dos pacientes, tomando-se os quatro Estados representados com maior número de casos, verificou-se que as proporções de cardiopatia e "megas" foram respectivamente de 65,7 e 20,1% nos casos procedentes da Bahia, de 55,7 e 14,7% nos de Minas Gerais, de 50,9 e 15% nos de Pernambuco e de 23,3 e 0% nos procedentes da Paraíba. O reduzido número de casos procedentes dos demais

Correction of Hirschsprung-Associated Mutations in Human Induced Pluripotent Stem Cells Via Clustered Regularly Interspaced Short Palindromic Repeats/Cas9, Restores Neural Crest Cell Function.

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Lai, Frank Pui-Ling; Lau, Sin-Ting; Wong, John Kwong-Leong; Gui, Hongsheng; Wang, Reeson Xu; Zhou, Tingwen; Lai, Wing Hon; Tse, Hung-Fat; Tam, Paul Kwong-Hang; Garcia-Barcelo, Maria-Mercedes; Ngan, Elly Sau-Wai

2017-07-01

Hirschsprung disease is caused by failure of enteric neural crest cells (ENCCs) to fully colonize the bowel, leading to bowel obstruction and megacolon. Heterozygous mutations in the coding region of the RET gene cause a severe form of Hirschsprung disease (total colonic aganglionosis). However, 80% of HSCR patients have short-segment Hirschsprung disease (S-HSCR), which has not been associated with genetic factors. We sought to identify mutations associated with S-HSCR, and used the clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9 gene editing system to determine how mutations affect ENCC function. We created induced pluripotent stem cell (iPSC) lines from 1 patient with total colonic aganglionosis (with the G731del mutation in RET) and from 2 patients with S-HSCR (without a RET mutation), as well as RET +/- and RET -/- iPSCs. IMR90-iPSC cells were used as the control cell line. Migration and differentiation capacities of iPSC-derived ENCCs were analyzed in differentiation and migration assays. We searched for mutation(s) associated with S-HSCR by combining genetic and transcriptome data from patient blood- and iPSC-derived ENCCs, respectively. Mutations in the iPSCs were corrected using the CRISPR/Cas9 system. ENCCs derived from all iPSC lines, but not control iPSCs, had defects in migration and neuronal lineage differentiation. RET mutations were associated with differentiation and migration defects of ENCCs in vitro. Genetic and transcriptome analyses associated a mutation in the vinculin gene (VCL M209L) with S-HSCR. CRISPR/Cas9 correction of the RET G731del and VCL M209L mutations in iPSCs restored the differentiation and migration capacities of ENCCs. We identified mutations in VCL associated with S-HSCR. Correction of this mutation in iPSC using CRISPR/Cas9 editing, as well as the RET G731del mutation that causes Hirschsprung disease with total colonic aganglionosis, restored ENCC function. Our study demonstrates how human iPSCs can

Clinical, manometric and profilometric evaluation after surgery for Hirschsprung's disease: comparison between the modified Duhamel and the transanal rectosigmoidectomy techniques Avaliação clínica, manométrica e profilométrica após correção cirúrgica para doença de Hirschsprung: comparação entre as técnicas de Duhamel modificado e a retossigmoidectomia transanal modificada

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Elaine Cristina Soares Martins

2009-10-01

Full Text Available PURPOSE: To evaluate fecal continence, anorectal manometry (AM and profilometry (P, in patients operated for congenital megacolon, using either the modified Duhamel technique (MDT or the modified transanal rectosigmoidectomy (MTR technique. METHODS: 42 patients were evaluated clinically and via AM and P, for postoperative control. The resting, coughing, voluntary contraction, maintained voluntary contraction and perianal stimulation pressures were investigated. The rectosphincteric reflex was tested and the simple and enhanced pressure curves were evaluated. The three-dimensional profilometric outline was produced. Student's t, chi-squared and Fisher's exact tests were used for statistical analysis (pOBJETIVO: Avaliar a continência fecal, a manometria anorretal (MAR e a profilometria (PFM, em pacientes submetidos a tratamento cirúrgico do megacolo congênito pelas técnicas de Duhamel modificado (DM ou de retossigmoidectomia transanal modificada (RTM. MÉTODOS: 42 pacientes foram clinicamente avaliados e submetidos à MAR e PFM para controle pós-operatório. Foram pesquisadas as pressões no repouso (PR, à tosse (T, à contração voluntária (CV, à contração voluntária mantida (CVM e à estimulação perianal (EPA. O reflexo reto-esficteriano (RRE foi testado e as curvas pressóricas simples e potencializada foram avaliadas. Foi elaborado traçado tridimensional de PFM. Utilizamos os testes t de Student, Qui-Quadrado e exato de Fisher para análise estatística (p<0,05. RESULTADOS: A MAR mostrou médias de PR=53,44 mmHg para DM e 60,67 mmHg para RTM; CV média = 94,50 mmHg para o grupo DM e 95,47 mmHg para o grupo RTM. Não houve diferença estatística entre os grupos. A forma das CPS e CPP não apresentou diferença estatística, independentemente da técnica cirúrgica utilizada CONCLUSÃO: As duas técnicas operatórias foram manometricamente equivalentes. DM determinou maior incidência de constipação pós-operatória que RTM. A

Enfermedad de Hirschsprung

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Gonzalo López Escobar

1990-04-01

Full Text Available

Definición
La enfermedad de Hirschsprung, megacolon congénito o agangliónico, o Aganglionosis Intestinal Congénita, es una anomalía caracterizada por la ausencia congénita de células ganglionares del sistema parasimpático de los piejos intermientéricos de Auerbach, situado entre las capas musculares, circular y longitudinal, y el submucoso de Meissner; con conducción inefectiva del peristaltismo, ocasionando obstrucción funcional (24, 37, 65, 82, 120, 130, 134.

Historia
La descripción del megacolon congénito fue hecha por e! pediatra danés, HARALD HIRSCHSPRUNG, en 1886, en el Congreso de Pediatría de Berlín, donde describió dos casos, un niño de 7, Y otro de II meses de edad, quienes murieron, y al practicar la autopsia observó “dilatación enorme del colon”, con fecalomas en su interior y ulceraciones de la mucosa. Su informe apareció en la literatura médica un año después, en 1887.

Sin embargo, la prime¡a anotación de esta alteración se le asigna a FREDERICK RUYSCH, quien en 1691, publicó en Amsterdam, en la Revista “Observationum AnatomiciChirurgicarum Centuria”, la descripción de la autopsia de una niña de cinco años quien había sido tratada por dolor y distensión abdominal, y flatos, finalmente murió; al abrir el cadáver pudo apreciar “todas las vísceras abdominales cubiertas por el colon distendido desde el recto”, descartó lo demás por considerarlo normal. (67, 129.

En 1825, Parry hizo la primera descripción en la literatura inglesa, de un joven de 23 años quien sufría de estreñimiento. (69, 129.

En 1829, Billar, informó dos casos similares.

En 1867, Lewitt, en Ann Arbor, Michigan, informó el primer caso en la literatura americana, de un joven de 21 años, con dolores abdominales y distensión, y al practicar el tacto anal encontró gran masa que ocupaba toda la pelvis, al parecer un fecaloma. (129.

The value of high frequency ultrasound combined with X in diagnosis of digestive tract malformation in children%高频超声联合X片在儿童消化道畸形中的诊断价值

Institute of Scientific and Technical Information of China (English)

陈志勇; 周小渔; 何静波; 金科

2014-01-01

Objetive To investigate the value of high frequency ultrasound combined with X-ray exami-nation in the diagnosis of digestive tract malformation in children. Methods 60 cases of gastrointestinal mal-formation operation confirmed by ultrasonography and X-ray images of the retrospective analysis. Results 1 9 cases of application of ultrasound alone diagnosis of 60 cases,including 8 cases of hypertrophic pyloric obstruc-tion,intestinal malrotation in 5 cases,6 cases were intestinal duplication,1 1 cases of application of X-ray sin-gle diagnosis,including 4 cases of esophageal atresia,atresia of anus with rectal perineal fistula in 7 cases,the other 30 cases of application of high frequency ultrasound combined with X the diagnosis,the pyloric edema with gastroesophageal reflux in 4 cases,7 cases of annular pancreas,6 cases of congenital megacolon. Con-clusion High frequency ultrasound combined with X-ray can improve children's digestive tract malformation di-agnosis rate,the two complement each other.%目的：探讨高频超声联合X线检查在儿童消化道畸形中的诊断价值。方法对60例经手术证实的消化道畸形患儿进行超声及X线片图像的回顾性分析。结果60例患儿中，单纯经超声诊断为肥厚性幽门梗阻8例，肠旋转不良5例，肠重复畸形6例。单纯经X线检查诊断为食管闭锁4例，肛门闭锁伴直肠会阴瘘7例；经高频超声联合X线检查诊断为消化道重复畸形13例，幽门水肿伴胃食管反流4例，环形胰腺7例，先天性巨结肠6例。结论高频超声联合X线检查可提高儿童消化道畸形的诊断率，二者互为补充。

Chagasic enteropathy Enteropatia chagásica

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Ulysses G. Meneghelli

2004-06-01

Full Text Available Involvement of the hollow organs of the digestive apparatus can occur in patients in the chronic phase of Chagas' disease. The basic mechanism is destruction of neurons of the enteric nervous system. Whereas megaesophagus and megacolon are the most notable and most extensively studied expressions of the digestive form of Chagas' disease, involvement of the small intestine (Chagasic enteropathy is less frequent and less known than involvement of the two above mentioned entities. Chagasic enteropathy can be responsible for important clinical and laboratory manifestations resembling those of dyspeptic syndrome, intestinal pseudo-obstruction and bacterial overgrowth in the small intestine. Chagasic enteropathy also involves peculiar functional changes, especially those related to motor activity of the organ and to intestinal absorption of carbohydrates. In practice, the diagnosis is based on radiographic documentation of dilation of visceral segments. Treatment consists of clinical control of the above syndromes and, eventually, appropriate surgical operations.O comprometimento dos órgãos ocos do aparelho digestivo pode ocorrer nos portadores da fase crônica da doença de Chagas. O mecanismo básico é a destruição dos neurônios do sistema nervoso entérico. Conquanto o megaesôfago e o megacólon sejam as expressões mais notáveis e estudadas da forma digestiva da doença de Chagas, o envolvimento do intestino delgado (enteropatia chagásica é menos freqüente e menos conhecido do que o das duas entidades mencionadas. A enteropatia chagásica pode ser responsável por importantes manifestações clínicas e laboratoriais que se assemelham às das síndromes dispéptica, de pseudo-obstrução intestinal e de supercrescimento bacteriano no intestino delgado. A enteropatia chagásica também acarreta peculiares alterações funcionais, particularmente relacionadas à atividade motora do órgão, bem como, à absorção intestinal de

Pelvic lymphoscintigraphy: contribution to the preoperative staging of rectal cancer

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Silva José Hyppolito da

2002-01-01

Full Text Available PURPOSE: Preservation of the anal sphincter in surgery for cancer of the distal rectum in an attempt to avoid colostomy has been a main concern of colorectal surgeons. Various proposed procedures contradict oncological principles, especially with respect to pelvic lymphadenectomy. Therefore, prior knowledge of pelvic lymph node involvement is an important factor in choosing the operative technique, i.e., radical or conservative resection. Introduction of ultrasound, computerized tomography, and magnetic resonance have made preoperative study of the area possible. Nevertheless, these resources offer information of an anatomical nature only. Lymphoscintigraphy enables the morphological and functional evaluation of the pelvic area and contributes toward complementing the data obtained with the other imaging techniques. The objective of this prospective study is twofold: to standardize the lymphoscintigraphy technique and to use it to differentiate patients with rectal cancer from those with other coloproctologic diseases. CASUISTIC AND METHODS: Sixty patients with various coloproctologic diseases were studied prospectively. Ages ranged from 21 to 96 years (average, 51 and median, 55 years. Twenty-six patients were male and 34 were female. Thirty patients had carcinoma of the distal rectum as diagnosed by proctologic and anatomic-pathologic examinations, 20 patients had hemorrhoids, 5 had chagasic megacolon, 2 had diverticular disease, 2 had neoplasm of the right colon, and 1 had ulcerative colitis as diagnosed by proctologic exam and/or enema. The lymphoscintigraphy method consisted of injecting 0.25 mL of a dextran solution marked with radioactive technetium-99m into the right and left sides of the perianal region and obtaining images with a gamma camera. The results were analyzed statistically with a confidence level of 95% (P < .05 using the following statistical techniques: arithmetic and medium average, Fisher exact test, chi-square test

Factors predictive of severe Clostridium difficile infection depend on the definition used.

Science.gov (United States)

Khanafer, Nagham; Barbut, Frédéric; Eckert, Catherine; Perraud, Michel; Demont, Clarisse; Luxemburger, Christine; Vanhems, Philippe

2016-02-01

Clostridium difficile infection (CDI) produces a variety of clinical presentations ranging from mild diarrhea to severe infection with fulminant colitis, septic shock, and death. Over the past decade, the emergence of the BI/NAP1/027 strain has been linked to higher prevalence and severity of CDI. The guidelines to treat patients with CDI are currently based on severity factors identified in the literature and on expert opinion and have not been systematically evaluated. The objective of this study was to identify factors associated with severe CDI defined according to four different severity definitions (Def): the 2010 SHEA/IDSA guidelines (Def1), the 2014 ESCMID guidelines (Def2), complicated CDI at the end of diarrhea (Def3), and our hospital-specific guidelines (white blood cell (WBC) count ≥15 × 10(9)/L, serum creatinine concentration >50% above baseline, pseudomembranous colitis, megacolon, intestinal perforation, or septic shock requiring intensive care unit admission. A three-year cohort study was conducted in a university hospital in Lyon, France. All hospitalized (≥48 h) patients ≥18 years old, suffering from CDI, and agreeing to participate were included. Patients were followed-up for 60 days after CDI diagnosis. After bivariate regression analyses, factors associated with severe CDI during the course of disease were identified by a multivariate logistic regression. Statistical significance was reached with a two-sided p-value definition. Factors independently associated with severe CDI were: age ≥68 years, male gender, renal disease, and serum albumin 7,5 × 10(9)/L in patients with Def2 (n = 138, 59.2%); abdominal pain, serum albumin 10 × 10(9)/L according to Def3 (n = 27, 11.6%); age ≥68 years, renal disease, serum albumin 248 IU/L, and blood neutrophils >7,5 × 10(9)/L were associated with severe CDI in patients with Def4 (n = 113, 48.5%). Our results indicate that appropriate case definition is needed for characterizing

O megaesôfago tratado cirurgicamente: perfil epidemiológico dos pacientes operados no Hospital de Clínicas da Universidade Estadual de Campinas entre 1989 e 2005 Surgically treated megaesophagus: epidemiological profile of patients operated in the Clinical Hospital of the State University of Campinas between 1989 and 2005

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Gustavo Carvalho de Oliveira

2008-04-01

endemic regions, place of birth and place of residence of the patients with Chagas disease attended at our clinic, and to characterize the group. After detailed analysis, it was found that the mean age was 47 years and the mean duration of dysphagia was 9.47 years. It was observed that: a in 84.4% of the patients, dysphagia took hold progressively; b 306 (78.5% patients presented Chagas disease etiology; c grade 2 was prevalent in 48%; d 89.8% of the patients underwent cardiomyotomy; and e there were frequent associations with gastritis, esophagitis, megacolon, arterial hypertension and cardiopathy.

Síndrome de Chilaiditi associada a volvo de cólon sigmóide: relato de caso Chilaiditi's Syndrome with sigmoid colon volvulus: case report

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Marcelo Wilson Rocha Almeida

2006-12-01

laparotomy being highlighted sigmoid volvulus, diffuse megacolon and interposition of the transverse colon between the liver and the diaphragm. Subtotal colectomy with terminal ascending colon colostomy was proceeded. The patient evolved satisfactorily being discharged on the 9th day. DISCUSSION: The interposition of the colon between the liver and the diaphragmatic cupula (Chilaiditi syndrome associated to sigmoid colon volvulus constitutes a rare cause of obstructive acute abdomen, though the sigmoid volvulus is one of the main causes of mechanic intestinal obstruction in Brazil. Usually a clinical treatment is done, although when associated to complications the treatment is surgical.

Management of ulcerative colitis: a clinical update

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Fabio Vieira Teixeira

2015-10-01

Full Text Available The objective of this study was to evaluate the consensus of expert societies and published guidelines on the management of ulcerative colitis, and to compare with the experience of the authors, in order to standardize procedures that would help the reasoning and decision-making process of the physician. A search was performed in scientific literature, specifically in electronic databases: Medline/Pubmed, SciELO, EMBASE and Cochrane, and the following descriptors were used: ulcerative colitis, acute colitis, clinical treatment, surgery and randomized trial. It can be concluded that the goals of therapy in ulcerative colitis are clinical and endoscopic remission, deep, sustained remission without corticosteroids, prevention of hospitalizations and surgeries, and improved quality of life. The surgical indications are reserved for selected cases, ranging from medical intractability, complications (severe refractory acute colitis, toxic megacolon, perforation and hemorrhage and malignancy. Information in this review article must be submitted to evaluation and criticism of the specialist responsible for the conduct to be followed, in the face of his/her reality and the clinical status of each patient.The degree of recommendation and strength of evidence were based using the GRADE system (The Grades of Recommendation, Assessment, Development, and Evaluation described below:1. A: Experimental or observational studies of higher consistency.2. B: Experimental or observational studies of lower consistency.3. C: Case reports (non-controlled studies.4. D: Opinion without critical evaluation, based on consensus, physiological studies or animal models. Resumo: O objetivo deste trabalho foi avaliar os consensos de sociedades de especialistas e guidelines publicados sobre o manejo da retocolite ulcerativa, e confrontar com a experiência dos autores, a fim de padronizar condutas que auxiliem o raciocínio e a tomada de decisão do médico. Foi realizada busca

Adaptive Returns of Deficient Systemic Plasma Immunoglobulin G Levels as Rehabilitation Biomarker After Emergency Colectomy for Fulminant Ulcerative Colitis

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Alexander T Hawkins

2017-12-01

Full Text Available Restorative proctocolectomy (RPC with ileal pouch-anal anastomosis (IPAA is the standard surgical treatment for ulcerative colitis (UC. Emergency colectomies are performed for fulminant colitis (ie, toxic megacolon, profuse bleeding, perforation, or sepsis. The RPC and IPAA involve manipulation of the proximal ileum, which may influence the essential physiological function of gut-associated lymphoid tissues. Circulating plasma immunoglobulin G (p-IgG deficiency is observed in patients with fulminant UC. In addition, increased levels have been reported in colonic tissues of active UC compared with quiescent disease. We aimed to examine levels of p-IgG for clinical evaluation following emergency colectomies in patients with fulminant UC compared with patients with quiescent disease having elective RPC operations. In total 45 patients received an ileoanal pouch (IAP due to UC. In all, 27 patients were men and 18 were women. The mean age was 34 years (range: 18-55. Because of fulminant UC, 26 patients had emergency subtotal colectomies with terminal ileostomy (TI. During second operation, the rectum was excised, and an IAP with diverting loop ileostomy (DLI was performed. Nineteen patients had elective operations and had colectomies performed in conjunction with the pouch operation. Mucosectomy was performed in all groups. As a last procedure, the DLI was closed. Blood samples for immunoglobulin G (IgG analyses were collected from each patient before the colectomy, after the colectomy with TI (before construction of the pouch, during the period with pouches (prior to DLI closure, and at 1, 2, and 3 years and at mean 13.7 years (range: 10-20 after DLI closure. Immunoglobulin G was determined by immunonephelometric assay technique. The statistics were analyzed by analysis of variance and linear regression. Preoperatively, p-IgG was significantly lower in the patients who had emergency operations compared with the group that had elective

Idiopathic esophageal achalasia: a study of etiology and profile of the patients Acalásia idiopática do esôfago: análise da história clínica e antecedentes na etiologia e perfil dos pacientes

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Gustavo Carvalho de Oliveira

2010-03-01

Full Text Available BACKGROUND: The idiopathic esophageal achalasia is a disease of unknown etiology, characterized by esophageal aperistalsis and failure of its lower sphincter with dysphagia. Its etiology factors includes: esophageal gastric junction obstruction, degeneration of Auerbach´s plexus, virus infection, congenital origin, autoimmune affection and injury by toxic agent. The achalasia diagnosis is reached after excluding Chagas disease possibilities, which includes seronegative results for Trypanosoma cruzi, absence of megacolon and epidemiology for Chagas disease. AIM: To characterize the disease and propose hypothesis concerning its etiology and associated factors. METHODS: Review of medical records from 78 patients operated at the Hospital de Clinicas da Unicamp obstruction between 1989 and 2005 and the subsequent interview, using directed questionnaire, reaching for common data between them and emphasizing history, possible co-morbidities and associated factors. In the group of 78 records collected it was possible to contact and interview 33 patients. RESULTS: The main findings of this study were: 1 presence of a triggering relevant emotional factor before the symptoms (80% and over 30% with psychiatric and/or psychological treatment reported; 2 typical childhood infections highly prevalent (88% measles, varicella, rubella; 3 possible associations with: exposure to chemicals, especially herbicides; other diseases of the gastrointestinal tract, autoimmune diseases, genetic propensity and other changes in the nervous system highlighting the seizures. CONCLUSIONS: The idiopathic esophageal achalasia is probably an autoimmune disease, which seems to be highly related to emotional problems.RACIONAL: Acalásia idiopática do esôfago é doença de etiologia desconhecida, caracterizada por não relaxamento do esfíncter esofágico inferior provocando disfagia. As causas estudadas incluem: obstrução na junção esôfagogástrica, degeneração do plexo

Supplementation with 0.1% and 2% vitamin e in diabetic rats: analysis of myenteric neurons immunostained for myosin-V and nNOS in the jejunum Suplementação com vitamina E 0,1% e 2% em ratos diabéticos: análise de neurônios mioentéricos imunomarcados para miosina-V e nNOS no jejuno

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Eleandro Aparecido Tronchini

2012-12-01

Full Text Available CONTEXT: Diabetes mellitus is a disease characterized by hyperglycemia that, when allowed to progress long-term untreated, develops vascular and neurological complications, which are responsible for the development of alterations in the enteric nervous system in diabetic patients. In the gastrointestinal tract, diabetes mellitus promotes motor and sensory changes, and in the reflex function of this system, causing gastroparesis, diarrhea, constipation, megacolon, slow gastrointestinal transit, gastric stasis and dilation with decreased or increased peristaltic contractions. Several studies have shown that oxidative stress is the main responsible for the vascular and neurological complications affecting the enteric nervous system of diabetics. OBJECTIVE: The effects of 0.1% and 2% vitamin E on myosin-V- and nNOS-immunoreactive neurons in the jejunum of diabetic rats were investigated. METHODS: Thirty rats were divided into the groups: normoglycemic, normoglycemic treated with 0.1% vitamin E, normoglycemic treated with 2% vitamin E, diabetic, diabetic treated with 0.1% vitamin E, and diabetic treated with 2% vitamin E. The neuronal density and areas of neuron cell bodies were determined. RESULTS: Diabetes (diabetic group significantly reduced the number of myosin-V-immunoreactive neurons compared with the normoglycemic group. The diabetic treated with 0.1% vitamin E and diabetic treated with 2% vitamin E groups did not exhibit a greater density than the D group (P>0.05. Nitrergic density did not change with diabetes (P>0.05. The areas of myosin-V- and nNOS-immunoreactive neurons significantly increased in the normoglycemic treated with 2% vitamin E and diabetic groups compared with the normoglycemic group. CONCLUSION: Supplementation with 2% vitamin E had a neurotrophic effect only in the area of myosin-V-immunoreactive neurons compared with the diabetic group.CONTEXTO: O diabetes mellitus (DM é uma doença caracterizada pela hiperglicemia que a

MANAGEMENT OF ACUTE SEVERE ULCERATIVE COLITIS: A CLINICAL UPDATE.

Science.gov (United States)

Sobrado, Carlos Walter; Sobrado, Lucas Faraco

2016-01-01

Acute severe colitis is a potentially lethal medical emergency and, even today, its treatment remains a challenge for clinicians and surgeons. Intravenous corticoid therapy, which was introduced into the therapeutic arsenal in the 1950s, continues to be the first-line treatment and, for patients who are refractory to this, the rescue therapy may consist of clinical measures or emergency colectomy. To evaluate the indications for and results from drug rescue therapy (cyclosporine, infliximab and tacrolimus), and to suggest a practical guide for clinical approaches. The literature was reviewed using the Medline/PubMed, Cochrane library and SciELO databases, and additional information from institutional websites of interest, by cross-correlating the following keywords: acute severe colitis, fulminating colitis and treatment. Treatments for acute severe colitis have avoided colectomy in 60-70% of the cases, provided that they have been started early on, with multidisciplinary follow-up. Despite the adverse effects of intravenous cyclosporine, this drug has been indicated in cases of greater severity with an imminent risk of colectomy, because of its fast action, short half-life and absence of increased risk of surgical complications. Therapy using infliximab has been reserved for less severe cases and those in which immunosuppressants are being or have been used (AZA/6-MP). Indication of biological agents has recently been favored because of their ease of therapeutic use, their good short and medium-term results, the possibility of maintenance therapy and also their action as a "bridge" for immunosuppressant action (AZA/6-MP). Colectomy has been reserved for cases in which there is still no response five to seven days after rescue therapy and in cases of complications (toxic megacolon, profuse hemorrhage and perforation). Patients with a good response to rescue therapy who do not undergo emergency operations should be considered for maintenance therapy using

¿Cuándo es demasiado pronto y cuándo demasiado tarde para la cirugía en la enfermedad de Crohn? When is it too early or too late for surgery in Crohn's disease?

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J. I. Fernández-Blanco Hernáiz

2008-01-01

Full Text Available El abordaje quirúrgico de la enfermedad de Crohn (EC, entendido como último esfuerzo de tratamiento frente a modalidades en las que la terapia médica ha fracasado, supone una pérdida de perspectiva, que puede posponer el retraso en la recuperación del paciente y le retrae de una mejor calidad de su vida, cuando se considera que un 50% de los pacientes mantienen inactiva su enfermedad durante años tras procedimientos quirúrgicos seleccionados; unas tasas no alcanzadas por los tratamientos más eficaces. El riesgo de precisar algún procedimiento quirúrgico en el curso de la EC alcanza al 75% de sus portadores, más del 50% en el primer año desde el diagnóstico, y prácticamente el 100% de los pacientes en la evolución de su proceso cuando se contemplan lesiones de localización perianal. Es por eso que el gastroenterólogo debe estar entrenado en la selección de quién, cuándo y por qué debe ser operado. Retrasar la cirugía a fases de enfermedad avanzada incrementa la morbilidad y, si es cierto que las actuales terapias médicas permiten una mayor tasa de remisiones, también lo es que incrementar la duración del proceso y la edad de los pacientes contribuye a mayor riesgo quirúrgico y peor perspectiva en el tratamiento de complicaciones agudas y de manifestaciones crónicas de la EC, a menudo clínicamente desconsideradas, como la mesenteritis retráctil, los estados de hipercoagulabilidad y la aparición de fenómenos de malignización. Salvando las indicaciones absolutas de actuación quirúrgica inicial en el manejo de pacientes con EC, como sangrado intestinal masivo, desarrollo de megacolon tóxico o perforación libre; otros condicionantes para el tratamiento quirúrgico deben ser reevaluados a la luz de nuestros conocimientos actuales. La genotipación del paciente constituye un elemento clínico que contribuye a identificar riesgos específicos y facilita la selección terapéutica. Desgraciadamente, hasta que estos an

Successful in vitro expansion and Characterization of Human Enteric Neuronal cells- A step towards Cell based therapies for Hirschsprung’s disease

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Balamurugan M

2010-01-01

Full Text Available BACKGROUND: The Enteric Nervous system (ENS is a part of the Peripheral nervous system (PNS that controls the peristaltic activity of the gut wall which is essential for propulsion of food in the digestive tract. It is composed of a large number of neurons and glial cells, distributed throughout the length of the gut. These ganglion cells develop from the neural crest in the embryo. Failure of complete colonization of the gut by these enteric neural crest cells during early development of life results in absence of ganglia or neurons in a portion of the gut, usually the colon which leads to aperistaltis and severe intestinal obstruction. This is known as Hirschsprung’s disease (HSCR also known as congenital megacolon. HSCR affects 1 in 4500 newborns (1, 2. It appears either sporadically or has a familial basis and is often associated with other developmental defects. The main forms of treatment of HSCR are surgical resection of the aganglionic segment and pull through of the normal bowel. At present research is aimed at developing Cell based therapies for replacement of ganglion cells or enteric neuronal cells in the aganglionic portion of the gut thus aiming at restoring the function of the gut (1, 3, 5. In this study we have isolated, in vitro expanded and characterized the Enteric Neuronal cells derived from human gut full thickness biopsy samplesMATERIALS AND METHODS: The postnatal gut full thickness biopsy samples of size 2-4 mm were obtained using from 13 patients undergoing gut resection surgery after informed consent. The samples were washed in Phosphate Buffer saline and using forceps, the outer smooth muscle layers along with the myenteric plexus were peeled off from the underlying tissue as strips. The strips were washed in Phosphate Buffer saline (PBS and treated with 1mg/ml Collagenase/Dispase mixture in PBS for 30-45 min at 37°C. The digested cells were filtered with 70µm filter and the cell suspensions were centrifuged at 1800

Histological, biochemical and pharmacologycal characterization of the gastric muscular layer in Chagas disease Caracterização histológica, bioquímica e farmacológica da musculatura gástrica na doença de Chagas

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Wagner Carlucci

2011-01-01

Full Text Available PURPOSE: To assess in vitro the correlation between the number of neurons and the sensitivity to cholinergic drugs and acetylcholinesterase activity in chagasic patients. METHODS: A 3x1 cm strip of the muscle layer of the anterior part of the stomach, always close to the angular incisure, was removed from 10 chronic chagasic patients (6 men submitted to megaesophagus or megacolon surgery and from 10 non-chagasic patients (4 men submitted to other types of surgery (control group, aged on average 52.3 and 50.1 years, respectively, for histological and pharmacological studies. The action of cholinergic drugs was investigated in isolated preparations according to the superfusion method of Ferreira and Costa, and acetylcholinesterase activity was determined by the method of Ellman. For neuron count, the strips were cut into 8 µm sections according to the method standardized by Alcântara. RESULTS: There was a difference in number of neurons between the chagasic (5,6 and control (7,3 groups. Acetylcholinesterase activity, in moles of hydrolyzed substrate per minute per gram tissue, was reduced in chagasic patients (4,32 compared to the controls (7,30. No hypersensitivity of the gastric musculature to cholinergic drugs was detected, with a reduced maximum response to carbachol and betanechol in the chagasic group. CONCLUSIONS: The reduction of neurons in the myenteric plexus of the stomach of chronic chagasic patients can be demonstrated even in the absence of clinical chagasic gastropathy. The hypersensitivity of the gastric musculature to cholinergic drugs probably depends on intense denervation. The reduced acetylcholinesterase activity demonstrates the involvement of the cholinergic innervation in the stomach of chronic chagasic patients. There was no correlation between number of neurons, sensitivity to cholinergic drugs and acetylcholinesterase activity in the gastric musculature of chagasic and non-chagasic patients.OBJETIVO: Avaliar in vitro a

Chronic constipation - the role of clinical assessment and colorectal physiologic tests to obtain an etiologic diagnosis O papel da avaliação clínica e dos testes de fisiologia colo-reto anal no diagnóstico etiológico da constipação intestinal crônica

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Antônio Lacerda-Filho

2008-03-01

Full Text Available BACKGROUND: Diagnosis of subtypes of chronic constipation has been considered difficult to achieve even in specialized centers. Although colorectal physiologic tests have brought an important contribution, it remains unclear in which patients these tests should be indicated for. AIMS: This study aims to establish a differential diagnosis for chronic constipation cases using clinical assessment and physiologic tests and to identify clinical parameters that could predict which patients need physiologic tests. METHODS: One hundred and seventy nine patients (83% females; mean age, 45 with chronic constipation according to Rome II criteria were initially treated by dietary advice and functional reeducation and those unresponsive (110 or 61.5% were submitted to colonic transit time, defecography, anorectal manometry and electromyography, as needed. RESULTS: A differential diagnosis was achieved in 63.6% of patients tested. However, 61.5% of 179 patients with chronic constipation (69 with no need to tests and 40 with normal tests have etiologic diagnosis established only on clinical basis. Irritable bowel syndrome (32%, pelvic floor dysfunction (29% and functional constipation due to faulty diet and life style habits (22% were the main causes of chronic constipation. Alternating constipation and nausea/vomiting were symptoms significantly related to the diagnosis of irritable bowel syndrome; younger age, larger intervals between bowel movements, occurrence of fecal impaction and necessity of enema were related to the diagnosis of non-chagasic megacolon and digital assistance to evacuate and large rectocele or spastic pelvic floor on rectal exam were associated to pelvic floor dysfunction. Patients with long-standing constipation, fecal impaction, abdominal pain not eased after defecation, necessity for enemas, digital assistance and evidence of rectocele tended to be in need for physiologic tests to define the cause of chronic constipation. CONCLUSIONS