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Sample records for megacolon

  1. Radiology of ACllte Toxic Megacolon

    African Journals Online (AJOL)

    Crohn's disease and ischaemic necrosis of the colon. Very rarely it may occur during the violent toxic phase of typhoid fever, cholera and acute dysentery. Other causes of megacolon, such as idiopathic megacolon, colonic ob- struction and Hirschsprung's disease, do not fall within the definition of acute toxic megacolon.

  2. Collagenous colitis as a possible cause of toxic megacolon.

    LENUS (Irish Health Repository)

    Fitzgerald, S C

    2009-03-01

    Collagenous colitis is a microscopic colitis characterized by normal appearing colonic mucosa on endoscopy. It is regarded as a clinically benign disease which rarely results in serious complications. We report a case of toxic megacolon occurring in a patient with collagenous colitis. This is the first reported case of toxic megacolon occurring in this subset of patients.

  3. A case of fulminating amebic colitis associated with toxic megacolon

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Kyung Sik; Lee, Joong Suk; Suh, Soo Jhi [Kyung Hee University Hospital. Seoul (Korea, Republic of)

    1985-12-15

    Amebic colitis was common disease in Korea as well as in the world especially frequent in tropical area such as Africa, India and South America. Clinicopathological forms of this condition were ulcerative rectocolitis (95%), typhloappendicitis (3%), ameboma (1.5%), and fulminating colitis with toxic megacolon (0.5%). The fulminating amebic colitis with toxic megacolon was very rare and dangerous condition which was reported by Wruble on 1966. Toxic megacolon was seen in the cases of ulcerative colitis, Crohn's disease, typhoid fever, cholera, and acute bacillary dysentery. Radiological findings of fulminating amebic colitis with toxic megacolon were megacolon, multiple polypoid filling defects, thumbprinting, and cobble stone appearance, which were resemble with ulcerative colitis. The cause of toxic megacolon was not well known, but it has been speculated that this results from transmural disease with destruction of muscle and the myenteric plexus with resultant loss of muscle tone. Authors experienced a case of fulminating amebic colitis with toxic megacolon which was resemble with ulcerative colitis by radiologically at Kyung Hee University Hospital and reported it with review of the literatures.

  4. Laparoscopic Swenson pull-through procedure for congenital megacolon.

    Science.gov (United States)

    George, C; Hammes, M; Schwarz, D

    1995-11-01

    Constipation that is unresponsive to conventional remedies is the primary symptom of congenital megacolon (ie, Hirschsprung's disease). The cause of congenital megacolon is lack of ganglion cells in the bowel. The laparoscopic Swenson pull-through procedure involves removing the aganglionic segment of the colon, bringing the normally decompressed bowel through the pelvic floor, and anastomosing the bowel to the anorectal verge. Advantages of the laparoscopic approach include shorter lengths of hospital stay and fewer complications resulting from disruption of skin integrity.

  5. Idiopathic megacolon in a teenager treated by laparoscopic rectosigmoidectomy

    Directory of Open Access Journals (Sweden)

    Carlos Eduardo Oliveira Sodero

    2016-07-01

    Full Text Available Chronic constipation in children and adolescents is relatively common and a reason for consultation with pediatricians and proctologists. Most cases respond to medical treatment. Advanced cases of megacolon and megarectum can be treated surgically by Duhamel technique.This case report describes a 15-year-old patient with chronic intestinal constipation refractory to clinical treatment associated with megacolon and megarectum, which was surgically treated. Resumo: A constipação intestinal crônica em crianças e adolescentes é relativamente comum e motivo de consultas a pediatras e coloproctologistas. A maioria dos casos responde ao tratamento clínico. Casos avançados de megacolon e megarreto podem ser tratados cirurgicamente através da cirurgia de Duhamel.Este relato de caso descreve um paciente de 15 anos de idade com quadro de constipação intestinal crônica refratária ao tratamento clínico associada a megacolon e megarreto, o qual foi tratado cirurgicamente. Keywords: Megacolon, Megarectum, Duhamel, Palavras-chave: Megacolon, Megarreto, Duhamel

  6. Clostridium Difficile Infection Complicated By Toxic Megacolon In Immunocompetent Patient

    Directory of Open Access Journals (Sweden)

    Draganescu Miruna

    2017-02-01

    Full Text Available Toxic megacolon can be a form of severe clinical course of the infection with Clostridium difficile (ICD, life-threatening, requiring a particular course of treatment. Infection with Clostridium difficile in the Galati Infectious Disease Hospital presents rising number of cases, namely 172 cases in 2014, 271 cases in 2015 and 301 cases in 2016 with clinical evolutions with different severity degrees, including toxic megacolon and death. Among 744 patients with ICD in our clinic, since 1st January 2014 to 31 December 2016. The frequency of toxic megacolon (TM was 0,537%, so: 3 toxic megacolon cases with favorable evolution with treatment with vancomycin and metronidazole and just one case whose evolution was aggravated under this therapy and evolved favorably under treatment with tigecycline. The work presents this last case of ICD occurred in a 69 years old, immunocompetent man with unknown concomitant chronic diseases which undergoes surgery for bilateral inguinal hernia and receives antibiotherapy with cephalosporin IIIrd generation during surgery and after 7 days develops medium degree ICD with score Atlas 3 and receives therapy with oral vancomycin. He presents clinical aggravation during this therapy with the occurrence of colon dilatation, ascites and right pleurisy at ultrasound and therapy associated with metronidazole is decided. Clinical aggravation continues in this combined therapy with defining the clinical, colonoscopy and tomography criteria for TM and is decided surgical monitoring and replacing antibiotherapy with tigecycline. Evolution is favorable with tigecycline without surgical intervention.

  7. Congenital aganglionic megacolon in Nigerian adults: Two case ...

    African Journals Online (AJOL)

    2011-02-17

    Feb 17, 2011 ... Those that present late in life as in these two cases warrant a proper medical history and evaluation, to exclude other causes of chronic constipation, such as, idiopathic megacolon and chagas disease.[7] The late presentation of Hirschsprung's disease is very rare in advanced countries.[13,20,21] However ...

  8. Radiology of acute toxic megacolon | Werbeloff | South African ...

    African Journals Online (AJOL)

    A case is described in which the transition from acute idiopathic ulcerative colitis to acute toxic megacolon was followed radioJogically, and the features which indicate the development of this dangerous complication are identified. S. Afr. Med. J., 48, 912 (1974) ...

  9. Association of chagasic megacolon and cancer of the colon: case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Adad Sheila Jorge

    2002-01-01

    Full Text Available There are few descriptions of association between chagasic megacolon and colon cancer. We report a case of obstructive abdomen caused by adenocarcinoma of the left colon in chagasic megacolon. A review of the literature revealed 8 cases of this association and, analyzing together the series of findings of cancer in chagasic organomegalies, we found a frequency of 4.8% in megaesophagus and 0.1% in megacolon.

  10. Blowhole Colostomy for Clostridium difficile-Associated Toxic Megacolon

    Directory of Open Access Journals (Sweden)

    Jeroen Kerstens

    2016-01-01

    Full Text Available We present the case of a 58-year-old man who underwent urgent blowhole colostomy for toxic megacolon (TM secondary to Clostridium difficile infection (CDI. This infection occurred under antibiotic coverage with amoxicillin-clavulanic acid, four days after laparoscopic sigmoidectomy in our hospital. Although prospective clinical research regarding the surgical management of TM is lacking, decompressive procedures like blowhole colostomy are reported to carry a high risk of postoperative morbidity and mortality and are widely regarded as obsolete. Subtotal or total colectomy with end ileostomy is currently considered the procedure of choice. After presenting our case, we discuss the literature available on the subject to argue that the scarce evidence on the optimal surgical treatment for TM is primarily based on TM associated with inflammatory bowel diseases (IBD and that there might be a rationale for considering minimally invasive procedures like blowhole colostomy for CDI-associated TM.

  11. Toksisk megacolon sekundært til Clostridium difficile-associeret pseudomembranøs kolitis

    DEFF Research Database (Denmark)

    Rasmussen, Torsten Bloch; Friis, Mikkel Lønborg; Lehnhoff, Rudolf

    2008-01-01

    severe colonic dilation, inflammation and oedema consistent with toxic megacolon. Stool samples were positive for Clostridium difficile. Oral vancomycine treatment and colonic decompression were inefficient. Subtotal colectomy was performed after which the condition improved. Udgivelsesdato: 2008-May-5...

  12. Atypical presentation of acute idiopathic megacolon in a 14-year-old patient

    Directory of Open Access Journals (Sweden)

    B. Barakat

    2012-12-01

    Full Text Available In clinical practice the term “megacolon” is used to indicate a marked dilatation of the cecum and the sigmoid colon (>12 and 6.5 cm, respectively (1. From a clinical standpoint, a megacolon can be classified as chronic or acute depending on its clinical presentation. Chronic megacolon typically refers to a congenital disorder in which the enteric nervous system (ENS supplying the colon does not develop properly, thereby leaving the distal segments of the viscus without myenteric and submucosal ganglia (i.e. Hirschsprung’s disease (2. Other cases of non-aganglionic chronic megacolon can be secondary to variety of conditions such as Chagas’ disease and neurodegenerative diseases (e.g. Parkinson’s and Alzheimer’s diseases, leading to or associated with ENS abnormalities (3. The acute form of megacolon, also referred to as Ogilvie’s syndrome, is characterized by a predominant involvement of the cecum and right colon usually affecting elderly patients undergoing surgery (e.g. orthopedic procedures or taking medications altering gut motility (e.g. opioids or antidepressants (4. Some forms of acute megacolon, however, can be idiopathic in origin since no underlying etiology can be identified. Patients with acute idiopathic megacolon usually have a longstanding history of constipation, often accompanied by laxative abuse, and their clinical presentation is characterized by abdominal distension and severe pain with radiological evidence of stool impacted in the colon and rectum (1, 4. The case herein reported represents an unusual form of acute idiopathic megacolon characterized by massive descending and sigmoid colon distension complicated with a volvulus in a 14-year-old boy with no Hirschsprung’s disease. In addition, just to increase the peculiarity of this case report, the patient had an unremarkable clinical record, and never suffered from chronic constipation in the past.

  13. Anatomical and physiological changes in pelvic diaphragm in patients with chagasic megacolon submitted to Duhamel surgery

    OpenAIRE

    Moreira Júnior,Hélio; Moreira,José P. T.; Isaac,Raniere R.; Almeida,Arminda C. de; Moreira,Hélio; Klug,Wilmar A.

    2013-01-01

    ORIGINAL CONTRIBUTION: understand the reasons why Duhamel surgery results in clinical improvement of constipation in patients with Chagasic colopathy. BACKGROUND: Duhamel surgery is one of the most widespread techniques for the treatment of Chagasic megacolon, with low rates of recurrence of constipation. OBJECTIVE: evaluate the anatomical and physiological changes in the pelvic diaphragm of patients with chagasic colopathy, as well as changes occurring after undergoing Duhamel surgery. DESIG...

  14. Anatomical and physiological changes in pelvic diaphragm in patients with chagasic megacolon submitted to Duhamel surgery

    OpenAIRE

    Hélio Moreira Júnior; José P.T. Moreira; Raniere R. Isaac; Arminda C. de Almeida; Hélio Moreira; Wilmar A. Klug

    2013-01-01

    Original contribution: understand the reasons why Duhamel surgery results in clinical improvement of constipation in patients with Chagasic colopathy. Background: Duhamel surgery is one of the most widespread techniques for the treatment of Chagasic megacolon, with low rates of recurrence of constipation. Objective: evaluate the anatomical and physiological changes in the pelvic diaphragm of patients with chagasic colopathy, as well as changes occurring after undergoing Duhamel surgery. Desig...

  15. Fecal Transplant for Treatment of Toxic Megacolon Associated With Clostridium Difficile Colitis in a Patient With Duchenne Muscular Dystrophy.

    Science.gov (United States)

    Yu, Shipeng; Abdelkarim, Ahmed; Nawras, Ali; Hinch, Bryan Thomas; Mbaso, Chimaka; Valavoor, Shahul; Safi, Fadi; Hammersley, Jeffrey; Tang, Jianlin; Assaly, Ragheb

    2016-01-01

    Clostridium difficile (C diff) colitis infection is the most common cause of nosocomial infectious diarrhea and the prevalence is increasing worldwide. Toxic megacolon is a severe complication of C diff colitis associated with high mortality. Gastrointestinal (GI) comorbidity and impaired smooth muscle contraction are risk factors for the development of C diff-associated toxic megacolon. We present a case of fulminant C diff colitis with toxic megacolon in a patient with Duchenne muscular dystrophy (DMD) in the intensive care unit. C diff colitis was diagnosed by clinical presentation and positive C diff DNA amplification test (polymerase chain reaction). The impairment of GI tract due to DMD predisposes these patients to severe C diff infection and toxic megacolon, as observed in this case report. For the same reason, the recovery of GI function in these patients can be prolonged. While surgery was conducted for relieving the pressure from toxic megacolon, fecal microbiota transplantation through colonoscopy resulted in successful resolution of the C diff symptoms, although the recovery is prolonged due to DMD.

  16. Chagasic megaesophagus and megacolon diagnosed in childhood and probably caused by vertical transmission

    Directory of Open Access Journals (Sweden)

    DA-COSTA-PINTO Elizete Aparecida Lomazi

    2001-01-01

    Full Text Available Reports on children presenting symptoms compatible with the chronic phase of Chagas disease are sporadic. We report a case of a 7-year-old boy who had megaesophagus and megacolon, both of them a consequence of the trypanosomiasis. The etiology was established by means of laboratory and histological features. Based on epidemiological data, the authors concluded that vertical transmission was the most probable route of acquisition. This diagnosis should be considered in children presenting similar complaints, even those living away from endemic areas.

  17. Association of megacolon with a new dominant spotting gene (Dom) in the mouse.

    Science.gov (United States)

    Lane, P W; Liu, H M

    1984-01-01

    A new semidominant mutation in the mouse is described. In heterozygotes it produces white spotting and a deficiency of myenteric ganglion cells in the colon and, in homozygotes it is lethal prior to 13 days of gestation. The mutation, called dominant megacolon, symbol Dom, is located on chromosome (chr) 15, 20.6 +/- 1.6 units proximal to Ca. Hairy ears, Eh, a semidominant gene also on chr 15 is shown to have a suppressing effect on crossing over in this section of chr 15.

  18. Overo lethal white foal syndrome: equine model of aganglionic megacolon (Hirschsprung disease).

    Science.gov (United States)

    McCabe, L; Griffin, L D; Kinzer, A; Chandler, M; Beckwith, J B; McCabe, E R

    1990-07-01

    The lethal white foal syndrome (LWFS) is a congenital abnormality of overo spotted horses which is a model for human aganglionic megacolon or Hirschsprung disease. Foals with LWFS have an all white, or nearly all white, coat. They also present clinically with an intestinal obstruction that proves fatal within the first few days of life. The LWFS involves both melanocytes and intestinal ganglion cells, and appears to result from a genetic defect involving neural crest cells. This report describes pathologic studies of two recent cases of LWFS. Two different hypothetical models of inheritance of LWFS are presented and discussed.

  19. A patient who survived total colonic ulcerative colitis surinfected by cytomegalovirus complicated by toxic megacolon and disseminated intravascular coagulation.

    Science.gov (United States)

    Laurent, S; Reenaers, C; Detroz, B; Detry, O; Delvenne, Ph; Belaiche, J; Meurisse, M

    2005-01-01

    The authors report the case of a patient aged 60-year-old who survived ulcerative colitis complicated by toxic megacolon and disseminated intravascular coagulation. This patient was not known for this ulcerative colitis and was first hospitalised for a suspicion of diverticulitis. The admission symptoms were fever, abdominal pain and bloody diarrhoea. The evolution was defavorable under antibiotics and sulfasalazine. The patient was readmitted 5 days after he left hospital, and the diagnosis of UC was based on colon biopsy made during the first hospitalisation. A treatment with methylprednisolone was started and the patient worsened day by day with apparition of toxic megacolon and disseminated intravascular coagulation. Subtotal colectomy was performed for degradation of general status and coagulation factors. Pathological findings confirmed ulcerative colitis with toxic megacolon. Cytomegalovirus inclusions were demonstrated on the colonic specimen and confirmed by PCR. In this report the authors discuss the etiology of toxic megacolon and disseminated intravascular coagulation in ulcerative colitis surinfected by cytomegalovirus. Mortality of these pathologies is high necessitating rapid diagnosis of cytomegalovirus infection by sigmoid biopsy. Management requires immunosupression interruption and ganciclovir therapy, or surgery in unsuccessful medical treatment.

  20. Total and segmental colonic transit time in constipated patients with Chagas’ disease without megaesophagus or megacolon

    Directory of Open Access Journals (Sweden)

    S.L. Santos

    2000-01-01

    Full Text Available Manometric and pharmacological tests have shown that motor abnormalities may occur in the non-dilated colons of chagasic patients. In order to investigate the presence of abnormalities of colonic function in constipated patients with Chagas’ disease (ChC without megaesophagus or megacolon, studies of total and segmental colonic transit time with radiopaque markers were performed on 15 ChC patients, 27 healthy volunteers and 17 patients with idiopathic constipation (IC. The values obtained for the control group were similar to those reported in the literature (total colonic time: 34.1 ± 15.6 h; right colon: 9.9 ± 7.3 h; left colon: 10.8 ± 10 h, and rectosigmoid: 12.6 ± 9.9 h. Colonic transit time data permitted us to divide both IC and ChC patients into groups with normal transit and those with slow colonic transit. Colonic inertia was detected in 41% of IC patients and in 13% of ChC patients; left colon isolated stasis (hindgut dysfunction was detected in 12% of IC patients and 7% of ChC patients, and outlet obstruction was detected in 6% of IC patients and 7% of ChC patients. There were no significant differences in total or segmental colonic transit times between slow transit IC and slow transit ChC patients. In conclusion, an impairment of colonic motility was detected in about 30% of constipated patients with Chagas’ disease without megaesophagus or megacolon. This subgroup of patients presented no distinctive clinical feature or pattern of colonic dysmotility when compared to patients with slow transit idiopathic constipation.

  1. A patient who survived total colonic ulcerative colitis surinfected by cytomegalovirus complicated by toxic megacolon and disseminated intravascular coagulation

    OpenAIRE

    Laurent, S.; Reenaers, Catherine; Detroz, Bernard; Detry, Olivier; Delvenne, Philippe; Belaiche, Jacques; Meurisse, Michel

    2005-01-01

    The authors report the case of a patient aged 60-year-old who survived ulcerative colitis complicated by toxic megacolon and disseminated intravascular coagulation. This patient was not known for this ulcerative colitis and was first hospitalised for a suspicion of diverticulitis. The admission symptoms were fever, abdominal pain and bloody diarrhoea. The evolution was defavorable under antibiotics and sulfasalazine. The patient was readmitted 5 days after he left hospital, and the diagnosis ...

  2. Anatomical and physiological changes in pelvic diaphragm in patients with chagasic megacolon submitted to Duhamel surgery

    Directory of Open Access Journals (Sweden)

    Hélio Moreira Júnior

    2013-04-01

    Full Text Available Original contribution: understand the reasons why Duhamel surgery results in clinical improvement of constipation in patients with Chagasic colopathy. Background: Duhamel surgery is one of the most widespread techniques for the treatment of Chagasic megacolon, with low rates of recurrence of constipation. Objective: evaluate the anatomical and physiological changes in the pelvic diaphragm of patients with chagasic colopathy, as well as changes occurring after undergoing Duhamel surgery. Design: clinical data and results of cinedefecography, electromanometry and anorectal ultrasound of the anal canal were evaluated in patients with Chagasic colopathy, before and after Duhamel surgery. Location: Service of Coloproctology – Departament of Surgery, Faculdade de Medicina da Universidade Federal de Goiás. Patients: patients with positive serology for Chagas Disease, with constipation and radiological megacolon, who presented consecutively to the Chagas outpatient clinic and freely agreed to participate in this study, were prospectively included. Results: a total of 20 patients were included, with a mean age of 53.2 years, of which 16 were women. The following parameters were observed in the postoperative period: change in bowel frequency, of, on average, one evacuation every ten days to daily bowel movement; 16 patients used laxatives preoperatively and only one did, intermittently, in postoperative period. Electromanometry showed, postoperatively, a decrease in anal resting pressure (60.88 to 37.2 mmHg p < 0.001 and anal squeeze pressures (244.3 mL to 161.25 p = 0.01, whereas ultrasound showed that 75% of the patients had abnormalities of the internal anal sphincter in the posterior anal canal juxtaposed to the pulled-through colon. Postoperative rectal emptying observed in cinedefecographic tests occurred more quickly and with less effort when compared with the preoperative findings. There was a change in the anorectal angle postoperatively

  3. Viral Spread to Enteric Neurons Links Genital HSV-1 Infection to Toxic Megacolon and Lethality.

    Science.gov (United States)

    Khoury-Hanold, William; Yordy, Brian; Kong, Philip; Kong, Yong; Ge, William; Szigeti-Buck, Klara; Ralevski, Alexandra; Horvath, Tamas L; Iwasaki, Akiko

    2016-06-08

    Herpes simplex virus 1 (HSV-1), a leading cause of genital herpes, infects oral or genital mucosal epithelial cells before infecting the peripheral sensory nervous system. The spread of HSV-1 beyond the sensory nervous system and the resulting broader spectrum of disease are not well understood. Using a mouse model of genital herpes, we found that HSV-1-infection-associated lethality correlated with severe fecal and urinary retention. No inflammation or infection of the brain was evident. Instead, HSV-1 spread via the dorsal root ganglia to the autonomic ganglia of the enteric nervous system (ENS) in the colon. ENS infection led to robust viral gene transcription, pathological inflammatory responses, and neutrophil-mediated destruction of enteric neurons, ultimately resulting in permanent loss of peristalsis and the development of toxic megacolon. Laxative treatment rescued mice from lethality following genital HSV-1 infection. These results reveal an unexpected pathogenesis of HSV associated with ENS infection. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Goldberg-Shprintzen megacolon syndrome with associated sensory motor axonal neuropathy.

    Science.gov (United States)

    Dafsari, Hormos Salimi; Byrne, Susan; Lin, Jean-Pierre; Pitt, Matthew; Jongbloed, Jan Dh; Flinter, Frances; Jungbluth, Heinz

    2015-06-01

    Goldberg-Shprintzen megacolon syndrome (GOSHS) (OMIM 609460) is characterized by a combination of learning difficulties, characteristic dysmorphic features and Hirschsprung's disease. Variable clinical features include iris coloboma, congenital heart defects and central nervous system abnormalities, in particular polymicrogyria. GOSHS has been attributed to recessive mutations in KIAA1279, encoding kinesin family member (KIF)-binding protein (KBP) with a crucial role in neuronal microtubule dynamics. Here we report on a 7-year-old girl with GOSHS as a result of a homozygous deletion of exons 5 and 6 of the KIAA1279 gene. She had been referred with the suspicion of an underlying neuromuscular disorder before the genetic diagnosis was established, prompted by the findings of motor developmental delay, hypotonia, ptosis and absent reflexes. Neurophysiological studies revealed unequivocal evidence of a peripheral axonal sensory motor neuropathy. We hypothesize that an axonal sensory motor neuropathy may be part of the phenotypical spectrum of KIAA1279-related GOSHS, probably reflecting the effects of reduced KBP protein expression on peripheral neuronal function. © 2015 Wiley Periodicals, Inc.

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

    Directory of Open Access Journals (Sweden)

    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.

  6. BLOOD VESSELS IN GANGLIA IN HUMAN ESOPHAGUS MIGHT EXPLAIN THE HIGHER FREQUENCY OF MEGAESOPHAGUS COMPARED WITH MEGACOLON

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    Sheila Jorge Adad

    2014-12-01

    Full Text Available This study aimed to determine the existence of blood vessels within ganglia of the myenteric plexus of the human esophagus and colon. At necropsy, 15 stillborns, newborns and children up to two years of age, with no gastrointestinal disorders, were examined. Rings of the esophagus and colon were analyzed and then fixed in formalin and processed for paraffin. Histological sections were stained by hematoxylin-eosin, Giemsa and immunohistochemistry for the characterization of endothelial cells, using antibodies for anti-factor VIII and CD31. Blood vessels were identified within the ganglia of the myenteric plexus of the esophagus, and no blood vessels were found in any ganglia of the colon. It was concluded that the ganglia of the myenteric plexus of the esophagus are vascularized, while the ganglia of the colon are avascular. Vascularization within the esophageal ganglia could facilitate the entrance of infectious agents, as well as the development of inflammatory responses (ganglionitis and denervation, as found in Chagas disease and idiopathic achalasia. This could explain the higher frequency of megaesophagus compared with megacolon.

  7. Acquired non-Chagas megacolon associated with the use of psychiatric medication: case report and differential diagnosis with Chagas megacolon Megacólon adquirido não chagásico associado ao uso de medicação psiquiátrica: relato de caso e diagnóstico diferencial com megacólon chagásico

    Directory of Open Access Journals (Sweden)

    Sheila Jorge Adad

    2008-06-01

    Full Text Available A case of acquired megacolon in a 62-year-old man with acute abdomen due to sigmoid volvulus is reported. The case was associated with the use of psychiatric medications. The aim in this report was to emphasize the differential diagnosis with Chagas megacolon. Anatomopathological examination did not show any evidence of denervation, ganglionitis and/or myositis, and the serological test for Chagas disease was negative.Relata-se caso de megacólon adquirido, associado ao uso de medicamentos psiquiátricos, em homem de 62 anos, com abdome agudo por volvo de sigmóide, com o objetivo de destacar o diagnóstico diferencial de megacólon chagásico. O exame anátomo-patológico não evidenciou denervação, ganglionite e/ou miosite e a sorologia para doença de Chagas foi negativa.

  8. Male-biased aganglionic megacolon in the TashT mouse line due to perturbation of silencer elements in a large gene desert of chromosome 10.

    Directory of Open Access Journals (Sweden)

    Karl-F Bergeron

    2015-03-01

    Full Text Available Neural crest cells (NCC are a transient migratory cell population that generates diverse cell types such as neurons and glia of the enteric nervous system (ENS. Via an insertional mutation screen for loci affecting NCC development in mice, we identified one line-named TashT-that displays a partially penetrant aganglionic megacolon phenotype in a strong male-biased manner. Interestingly, this phenotype is highly reminiscent of human Hirschsprung's disease, a neurocristopathy with a still unexplained male sex bias. In contrast to the megacolon phenotype, colonic aganglionosis is almost fully penetrant in homozygous TashT animals. The sex bias in megacolon expressivity can be explained by the fact that the male ENS ends, on average, around a "tipping point" of minimal colonic ganglionosis while the female ENS ends, on average, just beyond it. Detailed analysis of embryonic intestines revealed that aganglionosis in homozygous TashT animals is due to slower migration of enteric NCC. The TashT insertional mutation is localized in a gene desert containing multiple highly conserved elements that exhibit repressive activity in reporter assays. RNAseq analyses and 3C assays revealed that the TashT insertion results, at least in part, in NCC-specific relief of repression of the uncharacterized gene Fam162b; an outcome independently confirmed via transient transgenesis. The transcriptional signature of enteric NCC from homozygous TashT embryos is also characterized by the deregulation of genes encoding members of the most important signaling pathways for ENS formation-Gdnf/Ret and Edn3/Ednrb-and, intriguingly, the downregulation of specific subsets of X-linked genes. In conclusion, this study not only allowed the identification of Fam162b coding and regulatory sequences as novel candidate loci for Hirschsprung's disease but also provides important new insights into its male sex bias.

  9. Enteric Neuronal Damage, Intramuscular Denervation and Smooth Muscle Phenotype Changes as Mechanisms of Chagasic Megacolon: Evidence from a Long-Term Murine Model of Trypanosoma cruzi Infection.

    Directory of Open Access Journals (Sweden)

    Camila França Campos

    Full Text Available We developed a novel murine model of long-term infection with Trypanosoma cruzi with the aim to elucidate the pathogenesis of megacolon and the associated adaptive and neuromuscular intestinal disorders. Our intent was to produce a chronic stage of the disease since the early treatment should avoid 100% mortality of untreated animals at acute phase. Treatment allowed animals to be kept infected and alive in order to develop the chronic phase of infection with low parasitism as in human disease. A group of Swiss mice was infected with the Y strain of T. cruzi. At the 11th day after infection, a sub-group was euthanized (acute-phase group and another sub-group was treated with benznidazole and euthanized 15 months after infection (chronic-phase group. Whole colon samples were harvested and used for studying the histopathology of the intestinal smooth muscle and the plasticity of the enteric nerves. In the acute phase, all animals presented inflammatory lesions associated with intense and diffuse parasitism of the muscular and submucosa layers, which were enlarged when compared with the controls. The occurrence of intense degenerative inflammatory changes and increased reticular fibers suggests inflammatory-induced necrosis of muscle cells. In the chronic phase, parasitism was insignificant; however, the architecture of Aüerbach plexuses was focally affected in the inflamed areas, and a significant decrease in the number of neurons and in the density of intramuscular nerve bundles was detected. Other changes observed included increased thickness of the colon wall, diffuse muscle cell hypertrophy, and increased collagen deposition, indicating early fibrosis in the damaged areas. Mast cell count significantly increased in the muscular layers. We propose a model for studying the long-term (15 months pathogenesis of Chagasic megacolon in mice that mimics the human disease, which persists for several years and has not been fully elucidated. We

  10. Úlceras em megacólons chagásicos operados na urgência e eletivamente Ulcerations in Chagas' megacolon operated at urgency and electively

    Directory of Open Access Journals (Sweden)

    Augusto Diogo-Filho

    2006-12-01

    megacolon is a frequent disease in our emergencie hospital, and approached in the urgency by your complications as fecal impaction, volvulus and perforations. The ulcerations in the megacolons contribute as probable sites of perforations AIM: To compare the frequencies of stercoral ulceration in Chagas' megacolon operated at urgency, by volvulus or fecal impaction, and electively, aiming at a better surgical conduct in the urgency surgery METHODS: It was analyzed 356 anatomy-pathological exams from colon resection of operated patients due to Chagas' megacolon at urgency (102 cases; 29% and electively (254 cases; 71%, from 1980 to 2000. The surgical urgency indications were attributed to volvulus (71 cases; 69,6%, fecal impaction (25 cases; 24,5%, perforated acute abdomen after rectal catheter or sigmoidoscopy (6 cases; 5,9%. The ulceration frequency was compared in both groups of resections, using chi-square RESULTS: The pathological anatomy - of surgery resection obtained at urgency surgeries, showed 26 cases of ulceration (25,5% and in electively resections were verified 21 cases of ulceration (8,25%. The difference observed was statistically significant. The comparison among the groups of volvulus; fecal impaction and volvulus with fecal impaction, separately with electively surgery group evidenced significant differences in relation to volvulus and fecal impaction CONCLUSIONS: The higher frequency of ulcerations in the megacolon operated at urgency character emphasizes the needs of immediate resection of sigmoid colon, instead of conservative conduct of simple decompression colostomy, even in exploration laparotomy which the macroscopic examination of sigmoid does not show necrotic signs. This way, should prevent the occurrence of perforation in megacolon at mediate postoperative, with serious results.

  11. 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

    Directory of Open Access Journals (Sweden)

    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.

  12. 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?

    Directory of Open Access Journals (Sweden)

    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

  13. Right colon volvulus associated to acquired megacolon

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    Francisco de Salles Collet e Silva

    Full Text Available O volvo de colo direito é uma rara causa de obstrução intestinal em nosso meio e apresenta uma mortalidade elevada. Apresentam-se dois casos de volvo de cola direito associado ao megacolo adquirido, que apresentaram boa evolução. Discute-se a etiologia, o tratamento e as complicações.

  14. 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

  15. 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?

    Directory of Open Access Journals (Sweden)

    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

  16. 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 ...

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

    African Journals Online (AJOL)

    We report two cases of Nigerian adults with longstanding, recurrent constipation, getting relieved by laxatives and herbal enemata, and then presented to our Emergency Department with a history of progressive abdominal distention, colicky pain, occasional vomiting, and weight loss. Per rectal examination revealed a ...

  18. [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.

  19. Complications in amoebiasis in the X-ray picture

    Energy Technology Data Exchange (ETDEWEB)

    Ludewig, C.; Fernandez Bouzas, A.; Guevara Rascado, M.; Vogel, H.

    1986-02-01

    Complications in amoebiasis comprise amoeboma, amoebic abscess of the liver, amoebic meningoencephalitis with formation of abscesses and granuloma, fistulisation and toxic megacolon. As in amoebic colitis, changes can be detected also for amoebic abscess of the liver which suggest this diagnosis at least in the sonogram. Target-specific puncture and treatment by drainage are possible. Fistulisations may originate from the colon and from the suppuration. Toxic megacolon is a relatively rare manifestation in amoebic colitis and differs in no way from toxic megacolon of a different origin.

  20. [Nutrition and health--enteral nutrition in intensive care patients

    NARCIS (Netherlands)

    Haren, F.M. van; Oudemans-van Straaten, H.M.; Mathus-Vliegen, E.M.H.; Tepaske, R.; Hoeven, J.G. van der

    2004-01-01

    Nutritional therapy in the intensive care unit exerts favourable effects on morbidity and mortality. Enteral nutrition is preferable to parenteral nutrition. Only perforation or total obstruction of the gastrointestinal tract, proven mesenteric ischaemia and toxic megacolon are absolute

  1. Natural history and pathophysiology of enterocolitis in the piebald lethal mouse model of Hirschsprung's disease.

    Science.gov (United States)

    Fujimoto, T

    1988-03-01

    A breeding colony of piebald lethal mice was established in order to study the natural history of congenital megacolon in a mouse model of Hirschsprung's disease and to investigate mucosal defense mechanisms and secretory functions in enterocolitis complicating congenital megacolon. In experiment 1, 214 mice were studied, 53 of which had congenital megacolon. All piebald lethal mice with congenital megacolon (S1/S1) died at 3 to 11 weeks of age. Two distinct patterns of mortality were identified. A majority of mice (64%) became acutely ill at 3 to 4 weeks of age and died, whereas the remainder died between 9 and 11 weeks of age. The former group of mice exhibited clinical and histologic evidence of severe enterocolitis while the latter group had massive abdominal distension and classical megacolon. In experiment 2, piebald mice with congenital megacolon were killed at the time of acute illness. Significant histologic and immunohistochemical differences were seen in the ganglionic colon between piebald mice with early clinical onset of acute illness and piebald mice with the classical clinical picture of congenital megacolon. In the former group of mice the number of immunocytes in the lamina propria was significantly higher than in control mice (P less than .001), immunoglobulin-producing cells were equally distributed throughout the lamina propria and IgA-containing cells were by far the most abundant cell type identified in the colon. In the latter group of mice, immunocyte responses were significantly low and the distribution of immunocytes markedly different, with the immunoglobulin-producing cells being located only at the deep layer of lamina propria.(ABSTRACT TRUNCATED AT 250 WORDS)

  2. [Hirschsprungs disease in adults two case reports and review of the literature].

    Science.gov (United States)

    Škába, R; Hoch, J; Jech, Z; Kynčl, M; Campr, V

    2018-01-01

    Hirschsprungs disease (HD) in adults is extremely rare, only three publications in Czech and Czechoslovak journals making reference to the condition after childhood. We present two cases of adult patients with HD. The first case is a 46-year-old male patient suffering from chronic constipation since childhood and diagnosed with megacolon at the age of 16; however, no further detailed diagnosis was done. At the age of 41, he developed a sigmoid perforation due to fecaloma and underwent urgent rectosigmoid resection and colostomy. 5 months later, Swensons coloanal anastomosis with diverting ileostomy was performed. Postoperative course was uneventful. He has two bowel movements a day. 7 years after the Swensons procedure, he also underwent adhesiolysis for acute bowel obstruction. His daughter was operated on due to HD at 16 days of age. The second case is a 57-year-old male patient. He suffered from chronic constipation and megacolon since 2 years of age and was diagnosed with congenital megacolon at the age of 19. However, no detailed diagnostics followed. He had a long interval between stools of up to 14 days. He underwent colonoscopy and, with a diagnosis of resistant Crohns disease, was referred to a surgical department where he was diagnosed with HD. Left hemicolectomy was first performed, followed by Swensons procedure with diverting ileostomy. All postoperative courses were uneventful. Currently he passes one or two soft stools a day. Adult HD is extremely rare. However, adult surgeons should consider it in case of refractory constipation since childhood associated with megacolon. Diagnosis is based on contrast radiography and rectal biopsy. Both Swensons and Duhamels procedures are suitable for surgical management. Left hemicolectomy with colonic rotation and coloanal anastomosis and/or proctocolectomy with J-pouch anastomosis are indicated in advanced forms of non-functional megacolon.Key words: adult Hirschsprungs disease - megacolon surgical therapy.

  3. Congenital Central Hypoventilation Syndrome And Intestinal

    African Journals Online (AJOL)

    to progressive hypercapnia and sustained hypoxaemia. Neonates with this condition experience hypoventilation or apnoea while asleep. Patients may also have congenital intestinal aganglionosis (CIA), aganglionic megacolon or Hirschsprung's disease, suggesting an aberrant phenotype arising from a defect of migration ...

  4. Author Details

    African Journals Online (AJOL)

    Bank, S. Vol 45, No 6 (1971) - Articles The clinical spectrum of amoebic colitis. Abstract PDF · Vol 47, No 4 (1973) - Articles Upper Gastro-intestinal Fibre-Optic Endoscopy Abstract PDF · Vol 47, No 5 (1973) - Articles Radiology of acute toxic megacolon. Abstract PDF. ISSN: 0256-95749. AJOL African Journals Online.

  5. Download this PDF file

    African Journals Online (AJOL)

    drugs. c. Epistemological issues – how do we know that something. (in this case, a symptom-complex which we have identified as a discrete phenomenon, IBS) exists?10 This is related to the way in ... multiple sclerosis), faecal impaction, and the rare megacolon. (Hirschsprung's .... And all sorts of other new-fanglia. 13.

  6. Degenerative leiomyopathy | Henning | SA Journal of Radiology

    African Journals Online (AJOL)

    Degenerative leiomyopathy (DL) is a distinctive form of acquired degenerative visceral myopathy of uncertain aetiology. It occurs mainly in ... Megacolon was the most common radiological feature, but pseudo-obstruction extended proximally into the small intestine in some patients with advanced disease. In the majority of ...

  7. Male and female differential reproductive rate could explain parental transmission asymmetry of mutation origin in Hirschsprung disease

    NARCIS (Netherlands)

    Jannot, Anne-Sophie; Amiel, Jeanne; Pelet, Anna; Lantieri, Francesca; Fernandez, Raquel M.; Verheij, Joke B. G. M.; Garcia-Barcelo, Merce; Arnold, Stacey; Ceccherini, Isabella; Borrego, Salud; Hofstra, Robert M. W.; Tam, Paul K. H.; Munnich, Arnold; Chakravarti, Aravinda; Clerget-Darpoux, Francoise; Lyonnet, Stanislas

    Hirschsprung disease (HSCR, aganglionic megacolon) is a complex and heterogeneous disease with an incidence of 1 in 5000 live births. Despite the multifactorial determination of HSCR in the vast majority of cases, there is a monogenic subgroup for which private rare RET coding sequence mutations

  8. PHYSIOTHERAPY MANAGEMENT OF FAECAL IMPACTION:

    African Journals Online (AJOL)

    Dr.MrsOdebode

    this condition. KEY WORDS: faecal impaction, megacolon, modified passive mobilization, abdominal distention. INTRODUCTION. Faecal impaction is a large mass of dry, hard stool that accumulates in the rectum due to unresolved constipation (Creason and Sparks, 2000). The dryness of the mass makes it very difficult to ...

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

    Directory of Open Access Journals (Sweden)

    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+.

  10. Strategies for the Care of Adults Hospitalized for Active Ulcerative Colitis

    Science.gov (United States)

    Pola, Suresh; Patel, Derek; Ramamoorthy, Sonia; McLemore, Elisabeth; Fahmy, Marianne; Rivera-Nieves, Jesus; Chang, John T; Evans, Elisabeth; Docherty, Michael; Talamini, Mark; Sandborn, William J

    2014-01-01

    Ulcerative colitis is a chronic inflammatory disease of the colon; as many as 25% of patients with this disease require hospitalization. The goals of hospitalization are to assess disease severity, exclude infection, administer rapidly acting and highly effective medication regimens, and determine response. During the hospitalization, patients should be given venous thromboembolism prophylaxis and monitored for development of toxic megacolon. Patients who do not respond to intravenous corticosteroids should be considered for rescue therapy with infliximab or cyclosporine. Patients who are refractory to medical therapies or who develop toxic megacolon should be evaluated promptly for colectomy. Patients who do respond to medical therapies should be discharged on an appropriate maintenance regimen when they meet discharge criteria. We review practical evidence-based management principles and propose a day-by-day algorithm for managing patients hospitalized for ulcerative colitis. PMID:22835577

  11. Journal of Special Operations Medicine, Volume 3, Edition 4

    Science.gov (United States)

    2003-01-01

    absence of external ears from an unspecified cause. A six-year old girl with internal hydrocephalus and a quarter section of her markedly enlarged head...Potter’s syn- drome, congenital megacolon, and hydrocephalus . As in the other museums, these stillborns are shown great respect. Even a plastic...oldest hospital and part of Mahidol University School of Medicine. Although Thai physicians are trained in English using the most modern textbooks and

  12. 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...

  13. Revista de revistas: Ileo paralítico en el hipotiroidismo severo

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. Pseudo-Hirschsprung's disease with rectal hypoganglionosis: an autopsied case of circulatory failure due to severe constipation.

    Science.gov (United States)

    Nishijima, Akio; Shiotani, Seiji; Hayakawa, Hideyuki; Nishijima, Haruo

    2015-05-01

    We report a 21-year-old female patient who suddenly died of circulatory failure due to pressure from megacolon allied with pseudo-Hirschsprung's disease. Since 3 years before her death, she had exhibited the feeling of abdominal distention, which was diagnosed as constipation. However, her constipation did not respond well to the prescribed oral administration of laxatives and enemas. She was found dead at home in the morning, lying on her back. An autopsy revealed a decreased number of ganglion cells in the rectum, suggesting hypoganglionosis. In cases of intractable chronic constipation, careful investigation of the cause of such symptoms is important. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  16. Enfermedad de Hirschsprung y apendicitis. Una asociación rara

    OpenAIRE

    C Baeza-Herrera; P López-Medina; J M Vidal-Medina; N Cruz-Viruel; L Velasco-Soria

    2016-01-01

    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 d...

  17. SYMPTOMS AND DIAGNOSIS OF HIRSCHSPRUNG’S DISEASE

    Directory of Open Access Journals (Sweden)

    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.

  18. Síndrome de chilaiditi associada a megaduodeno e megacólon transverso

    Directory of Open Access Journals (Sweden)

    Francisco Edilson Leite Pinto Júnior

    Full Text Available Chilaiditi syndrome is a rare abnormality, uncommon in children, whith an incidence increasing with the age, ranging from 0.025% to 0.28% in the general population usually affecting males. The patients are usually asymptomatics and the syndrome is incidently discover through radiologic exams. However, abdominal pain and distention, nauseas, vomits and changes in intestinal habits, and even intestinal obstruction can occur. The authors report a case of a patient with Chilaiditi's syndrome associated to megastomach, megaduodenum and megacolon.

  19. Probiotics and Antibiotic-Associated Diarrhea and Clostridium difficile Infection

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  1. 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.

  2. 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

  3. Chronic intestinal pseudoobstruction syndrome

    International Nuclear Information System (INIS)

    Yeon, Kyung Mo; Seo, Jeong Kee; Lee, Yong Seok

    1992-01-01

    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

  4. 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.

  5. 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.

  6. 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.

  7. [Ogilvie syndrome (colonic pseudo-obstruction). A case of the practice].

    Science.gov (United States)

    Kolev, N; Tonev, A; Ivanov, K; Ignatov, V

    2010-01-01

    Ogilvie syndrome, or acute colonic pseudo-obstruction (ACPO), is a clinical disorder with the signs, symptoms, and radiographic appearance of an acute large bowel obstruction with no evidence of distal colonic obstruction. The other name of this disease is an acute non-toxic megacolon. The colon may become massively dilated; if not decompressed, the patient risks perforation, peritonitis, and death. The acute state has to be discerned immediatelly and treated adequately. The conservative tactics includes naso-gastric drainage, resustitation the homeostasis and stimulation the colonic peristalsis. The aggressive behavior includes an operative treatment or colonoscopic decompression. Some authors report for considerable rising in the frequency of the cases in postoperative period in some abdominal diseases. That's why they think that Ogilvie syndrome is a postoperative disease. We present a case of the practice-- an operated man with acute colonic pseudo-obstruction (ACPO) involving acute colonic ischaemia with necrosis.

  8. Unsuspected invasive neonatal gastrointestinal mucormycosis: A clinicopathological study of six cases from a tertiary care hospital

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    Sushma Patra

    2012-01-01

    Full Text Available Aim: To analyse the clinicopathological features of neonatal mucormycosis Materials and Methods: Retrospective analysis of cases of neonatal gastrointestinal mucormycosis. Results : There were six neonates with male: female ratio of 1:1. Except one all were preterm babies. The clinical presentation was abdominal distension in the majority. All were clinically diagnosed as either NEC or toxic megacolon with perforation. Neonatal gastrointestinal mucormycosis was not suspected clinically in any. All the children were explored immediately. Biopsy revealed transmural hemorrhagic necrosis/infarction of the intestinal wall with fungal hyphae. Conclusions: The physicians should have a high index of suspicion for gastrointestinal tract mucormycosis in neonates with metabolic disturbances who present with abdominal distension and pneumoperitoneum. Early diagnosis and aggressive medical and surgical treatment may improve the outcome of neonates with this potentially lethal invasive disease.

  9. 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.

  10. [Consensus document for the detection and management of Chagas disease in primary health care in a non-endemic areas].

    Science.gov (United States)

    Roca Saumell, Carme; Soriano-Arandes, Antoni; Solsona Díaz, Lluís; Gascón Brustenga, Joaquim

    2015-05-01

    Chagas disease is caused by the protozoan Trypanosoma cruzi. Although it is commonly transmitted by an insect vector in continental Latin-America, in recent decades, due migration, has been diagnosed in other countries such Spain, the European country with a largest immigrant population of Latin American. For a long time, the patient remains asymptomatic, but some years after this stage, the symptoms can be serious (dilated cardiomyopathy, megacolon, megaesophagus). In addition, detection in pregnant women has a high priority because of the route of vertical transmission. Several specific guidelines about Chagas disease has been developed on the Banks of blood, maternal hospitals, HIV co-infection, organ transplant. But due to the detection of lack of information to primary care professionals, we consider to will be useful this document written and agreed to by family phisicians, pediatricians and specialists in International Health. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  11. Surgical treatment of inflammatory bowel diseases

    International Nuclear Information System (INIS)

    Fuerst, H.; Schildberg, F.W.

    1998-01-01

    Purpose: To summarize current knowledge on surgical therapy in patients with inflammatory bowel disease (Crohn's disease, ulcerative colitis and diverticulitis). Material and methods: To discuss surgical indications and strategies, we reviewed major peer review publications of the last 10 years, and we also analysed data from patients with Crohn's disease who were treated in our institution between 1978 and 1994. Results: With Crohn's disease (305 patients), emergency surgery should be avoided as much as possible, since morbidity (50% vs 8.8%) and mortality (11% vs 0.6%) rise significantly in comparison to elective procedures. With ulcerative colitis, operative therapy is indicated in patients with secondary malignoma, and urgent surgery is requested in cases with associated perforation, toxic megacolon or massive bleeding. With diverticulitis, the first episode should be managed conservatively. Surgery is indicated in patients with recurrent episodes or with secondary complications. (orig.) [de

  12. [Ulcerative colitis and Crohn's disease].

    Science.gov (United States)

    Pavlović-Calić, Nada

    2003-01-01

    There is an enigma of inflammatory bowel diseases, despite significant advantages during last 10 years in medicamentous and surgical treatment. Ulcerative colitis and Crohns disease are chronic with remissions and recidives. Crohns disease involves any part of digestive tube. Histological changes in ulcerative colitis are: inflammation of mucosa and submucosal tissue, crypt abscesses and ulcerations, pseudopolpys, bowel shortening and toxic megacolon in severe inflammation. In Crohns disease, transmural inflammation, "jumping lesions", deeper ulcerations, coble-stone mucosa, progressive fibrosis, granuloma with gigantic epithelial cells. ulcerative colitis: mesalazine, rectal 5-ASA and hydrocortisone enemas, surgery. Crohns disease: mesalazine and prednisolone. For terminal ilcitis, corticosteroid budesonid could be applied. Severe symptomatic disease: hospitalization, parenteral nutrition, antibiotics, prednisone, surgery in partial bowel obstruction, fistulas, abscessus, perforation.

  13. 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

  14. AVALIAÇÃO URODINÂMICA DE PACIENTES CHAGÁSICOS URODYNAMIC ASSESSEMENT IN PATIENTS WITH CHAGAS DISEASE

    Directory of Open Access Journals (Sweden)

    J. N. Rocha

    2001-01-01

    Full Text Available No presente trabalho estudou-se a fisiologia urinária de pacientes com Doença de Chagas nas suas diversas formas. Grupo A (controle com 21 indivíduos normais; Gupo B: 16 pacientes com sorologia positiva; Grupo C: com 16 pacientes com cardiopatia chagásica e Grupo D: 21 pacientes com esofagopatia e/ou megacolon chagásicos. Os resultados mostraram que não houve diferenças significantes (p > 0,05 entre os 4 grupos estudados para os parâmetros: capacidade cistométrica, pressão de micção e fluxo urinário. Entretanto, houve diferenças significantes (p In this article we have studied the pathophysiology of the lower urinary tract of patients with chronic Chagas' disease: Control Group (A with 21 normal individuals; Group (B 16 patients with positive serum for T. cruzi; Group (C 16 patients with Chagas' cardiophaty and Group (D 21 patients with Chagas' megacolon or megaesophagus. Urodynamics did not show difference (p > 0,05 among all groups as long as the following parameters are concerned: cystometric bladder capacity, voiding pressure, and urinary flow. On the contrary, a significant difference (p < 0,05 was observed between Groups A and D in the following parameters: abdominal pressure, detrussor pressure, urethral pressure and time of voiding. These findings suggest that Chagas' disease promotes changes of the lower urinary tract in patients that bears digestive dysfunction.

  15. Chagas disease: morbidity profile in an endemic area of Northeastern Brazil

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    Cléber de Mesquita Andrade

    2015-12-01

    Full Text Available Abstract: INTRODUCTION : This study evaluated the clinical forms and manifestation severities of Chagas disease among serologically reactive individuals from Western Rio Grande do Norte (Northeastern Brazil. METHODS : This cross-sectional study included 186 adults who were evaluated using electrocardiography, echocardiography, chest radiography, and contrast radiography of the esophagus and colon. A clinical-epidemiological questionnaire was also used. RESULTS : The indeterminate, cardiac, digestive, and cardiodigestive clinical forms of Chagas disease were diagnosed in 51.6% (96/186, 32.2% (60/186, 8.1% (15/186 and 8.1% (15/186 of the participants, respectively. Heart failure (functional classes I-IV was detected in 7.5% (14/186 of the participants, and 36.4% (24/66, 30.3% (20/66, 15.2% (10/66, 13.6% (9/66, and 4.5% (3/66 of the patients were at stage A, B1, B2, C, and D, respectively. Dilated cardiomyopathy and electrocardiographic changes were detected in 10.2% (19/186 and 48.1% (91/186 of the participants, respectively. Apical aneurysm was diagnosed in 10.8% (20/186 of the participants, and other changes in the segmental myocardial contractility of the left ventricle were diagnosed in 33.9% (63/186 of the participants. Megaesophagus (groups I-IV was observed in 7% (13/186 of the participants, megacolon (grades 1-3 was detected in12.9% (24/186 of the participants, and both organs were affected in 29.2% (7/24 of the megacolon cases. CONCLUSIONS : We detected various clinical forms of Chagas disease (including the digestive form. Our findings indicate that clinical symptoms alone may not be sufficient to exclude or confirm cardiac and/or digestive damage, and the number of patients with symptomatic clinical forms may be underestimated.

  16. RET haplotype, not linked to the C620R activating mutation, associated with Hirschsprung disease in a novel MEN2 family

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    Elisangela P. S. Quedas

    2012-01-01

    Full Text Available Hirschsprung disease is a congenital form of aganglionic megacolon that results from cristopathy. Hirschsprung disease usually occurs as a sporadic disease, although it may be associated with several inherited conditions, such as multiple endocrine neoplasia type 2. The rearranged during transfection (RET proto-oncogene is the major susceptibility gene for Hirschsprung disease, and germline mutations in RET have been reported in up to 50% of the inherited forms of Hirschsprung disease and in 15-20% of sporadic cases of Hirschsprung disease. The prevalence of Hirschsprung disease in multiple endocrine neoplasia type 2 cases was recently determined to be 7.5% and the cooccurrence of Hirschsprung disease and multiple endocrine neoplasia type 2 has been reported in at least 22 families so far. It was initially thought that Hirschsprung disease could be due to disturbances in apoptosis or due to a tendency of the mutated RET receptor to be retained in the Golgi apparatus. Presently, there is strong evidence favoring the hypothesis that specific inactivating haplotypes play a key role in the fetal development of congenital megacolon/Hirschsprung disease. In the present study, we report the genetic findings in a novel family with multiple endocrine neoplasia type 2: a specific RET haplotype was documented in patients with Hirschsprung disease associated with medullary thyroid carcinoma, but it was absent in patients with only medullary thyroid carcinoma. Despite the limited number of cases, the present data favor the hypothesis that specific haplotypes not linked to RET germline mutations are the genetic causes of Hirschsprung disease.

  17. 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.

  18. 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

    Directory of Open Access Journals (Sweden)

    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

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

    Energy Technology Data Exchange (ETDEWEB)

    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

  20. 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

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

    Directory of Open Access Journals (Sweden)

    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

  2. Radiological evaluation congenital gastrointestinal tract anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Young Hee; Kim, Ock [Hanil Hospital, Seoul (Korea, Republic of); Jang, Jung Wha [Seoul Nationl Hospital, Seoul (Korea, Republic of)

    1983-06-15

    With the improvements, during recent years, in the control of the infections and nutritional diseases the subject of congenital malformation becomes of increasing importance. The radiologic signs are crucial for prompt diagnosis of anomalies of alimentary tract and with early identification of resulting complication, surgical therapy is usually life-saving. 30 cases of congenital anomalies of alimentary tract in infants were reviewed in respect of age, sex, incidence and radiological findings. The results are summarized as follows; 1, The most common lesion was hypertrophic pyloric stenosis, followed by congenital megacolon and anorectal anomaly, tracheoesophageal fistula, intestinal atresia. 2. Male outnumbered female in most congenital anomalies of alimentary tract. 25 cases were under the age of 1 month. 3. Common symptoms of upper gastrointestinal tract obstruction are vomiting and abdominal distension. In the obstruction of lower gastrointestinal tract, abdominal distension and failure of meconium passage were noted. 4. Roentgenologic findings were as follows, a. Chest A-P and lateral view: In tracheoesophageal fistula, saccular dilatation of upper esophagus and displacement of trachea anterolaterally were the most common finding. b. Simple abdomen: Obstructive pattern of proximal portion of duodenum shows in 11 cases, of distal bowel shows in 16 cases. Duodenal atresia showed 'double bubble' sign, hypertrophic pyloric stenosis showed marked gastric distension, paucity of air in small bowel and increases gastric peristalsis were the most common findings. Hirschsprung's disease showed absenced of rectal gas almostly. The variable length between blind hindgut to anus was seen in anorectal anomalies. c. Esophagogram: Blind sac of upper esophagus was seen at the 4th thoracic spinal level and displacement of trachea anterolaterally. 1 case of tracheoesophageal fistula had an intact esophageal lumen. d. Upper G-I series: In hypertrophic pyloric

  3. Radiological evaluation congenital gastrointestinal tract anomalies

    International Nuclear Information System (INIS)

    Cho, Young Hee; Kim, Ock; Jang, Jung Wha

    1983-01-01

    With the improvements, during recent years, in the control of the infections and nutritional diseases the subject of congenital malformation becomes of increasing importance. The radiologic signs are crucial for prompt diagnosis of anomalies of alimentary tract and with early identification of resulting complication, surgical therapy is usually life-saving. 30 cases of congenital anomalies of alimentary tract in infants were reviewed in respect of age, sex, incidence and radiological findings. The results are summarized as follows; 1, The most common lesion was hypertrophic pyloric stenosis, followed by congenital megacolon and anorectal anomaly, tracheoesophageal fistula, intestinal atresia. 2. Male outnumbered female in most congenital anomalies of alimentary tract. 25 cases were under the age of 1 month. 3. Common symptoms of upper gastrointestinal tract obstruction are vomiting and abdominal distension. In the obstruction of lower gastrointestinal tract, abdominal distension and failure of meconium passage were noted. 4. Roentgenologic findings were as follows, a. Chest A-P and lateral view: In tracheoesophageal fistula, saccular dilatation of upper esophagus and displacement of trachea anterolaterally were the most common finding. b. Simple abdomen: Obstructive pattern of proximal portion of duodenum shows in 11 cases, of distal bowel shows in 16 cases. Duodenal atresia showed 'double bubble' sign, hypertrophic pyloric stenosis showed marked gastric distension, paucity of air in small bowel and increases gastric peristalsis were the most common findings. Hirschsprung's disease showed absenced of rectal gas almostly. The variable length between blind hindgut to anus was seen in anorectal anomalies. c. Esophagogram: Blind sac of upper esophagus was seen at the 4th thoracic spinal level and displacement of trachea anterolaterally. 1 case of tracheoesophageal fistula had an intact esophageal lumen. d. Upper G-I series: In hypertrophic pyloric stenosis, delayed

  4. 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

  5. Long-Term Natural History and Complications of Collagenous Colitis

    Directory of Open Access Journals (Sweden)

    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.

  6. Lauric Acid Is an Inhibitor ofClostridium difficileGrowthin Vitroand Reduces Inflammation in a Mouse Infection Model.

    Science.gov (United States)

    Yang, Hsiao-Ting; Chen, Jenn-Wei; Rathod, Jagat; Jiang, Yu-Zhen; Tsai, Pei-Jane; Hung, Yuan-Pin; Ko, Wen-Chien; Paredes-Sabja, Daniel; Huang, I-Hsiu

    2017-01-01

    Clostridium difficile is a Gram-positive, spore-forming anaerobic human gastrointestinal pathogen. C. difficile infection (CDI) is a major health concern worldwide, with symptoms ranging from diarrhea to pseudomembranous colitis, toxic megacolon, sepsis, and death. CDI onset and progression are mostly caused by intestinal dysbiosis and exposure to C. difficile spores. Current treatment strategies include antibiotics; however, antibiotic use is often associated with high recurrence rates and an increased risk of antibiotic resistance. Medium-chain fatty acids (MCFAs) have been revealed to inhibit the growth of multiple human bacterial pathogens. Components of coconut oil, which include lauric acid, have been revealed to inhibit C. difficile growth in vitro . In this study, we demonstrated that lauric acid exhibits potent antimicrobial activities against multiple toxigenic C. difficile isolates in vitro . The inhibitory effect of lauric acid is partly due to reactive oxygen species (ROS) generation and cell membrane damage. The administration of lauric acid considerably reduced biofilm formation and preformed biofilms in a dose-dependent manner. Importantly, in a mouse infection model, lauric acid pretreatment reduced CDI symptoms and proinflammatory cytokine production. Our combined results suggest that the naturally occurring MCFA lauric acid is a novel C. difficile inhibitor and is useful in the development of an alternative or adjunctive treatment for CDI.

  7. 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

  8. Surgical complications of hemolytic uremic syndrome: Single center experiences

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    Hooman Nakysa

    2007-01-01

    Full Text Available Purpose: To determine the prevalence, outcome and prognostic factors in children with hemolytic uremic syndrome (HUS who underwent laparotomy. Materials and Methods: The medical records of 104 patients with HUS who presented to our center between 1986 and 2003 were reviewed retrospectively. Data were analyzed using Student′s t test for comparing means, Fisher′s exact test for frequencies and Pearson′s correlation for finding the correlations. Results: 78% of cases presented with vomiting and diarrhea. Seven out of 104 needed surgical exploration. The indications of surgery were acute abdomen, severe abdominal distention and the sign of peritonitis. The findings at laparotomy were intussusceptions, perforation (colon, ileum, gangrene of entire colon, rectosigmoidal tearing, duodenal obstruction and toxic megacolon. Pathological findings were transmural infarction in two cases in which staged surgical management was performed (cecostomy, resection, later anastomosis. Four out of seven patients died because of pulmonary failure, coma and multiple organ failure ( P< 0.05 compared to those who did not need laparotomy. The patients requiring surgery were young (< 3 years, had high leukocyte count (>20000 mm 3 and low albumin level (< 3g/dl ( P< 0.05. Conclusion: Surgical complications of HUS are rare but are assorted with high mortality due to respiratory failure and multiple organ failure. Early decision of laparotomy associated with intensive care, including mechanical ventilation, adequate dialysis and ultrafiltration, are recommended.

  9. 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.

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

    Science.gov (United States)

    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.

  11. Mutation detection in autosomal dominant Hirschsprung disease: SSCP analysis of the RET proto-oncogene

    Energy Technology Data Exchange (ETDEWEB)

    Angrist, M.; Bolk, S.; Chakravarti, A. [Case Western Reserve Univ., Cleveland, OH (United States)

    1994-09-01

    Hirschsprung disease (HSCR), or congenital aganglionic megacolon, is the most common cause of congenital bowel obstruction, with an incidence of 1 in 5000. Recently, linkage of an incompletely penetrant, dominant form of HSCR to the pericentromeric region of chromosome 10 was reported, followed by identification of mutations in the RET proto-oncogene in HSCR patients. RET mutations have also been reported in both sporadic and familial forms of three neuroendrocrine tumor syndromes. Unlike the clustered RET mutations observed in these syndromes, the 18 reported HSCR mutations are distributed throughout the extracellular and tryosine kinase domains of RET. In an effort to determine the frequency of RET mutations in HSCR and correlate genotype with phenotype, we have begun to screen for mutations among 80 HSCR probands representing a wide range of phenotypes and pedigree structures. Non-isotopic single strand conformation of polymorphism (SSCP) analysis was carried out using the Pharmacia PhastSystem{trademark}. Initial screening of exons 2 through 6 detected variants in 11 patients not seen in 24 controls. One additional band shift in exon 6 has been observed in both patients and controls. Preliminary sequence analysis has revealed two putative familial mutations in exon 2: a single base pair deletion (49Pro del C 296) and a point mutation that leads to a conservative amino acid substitution (93Gly{r_arrow}Ser). These results suggest that HSCR may be associated with a range of alterations in the coding sequence of the RET extracellular domain. Additional mutations will be described.

  12. Sporadic Hirschsprung`s disease due to a novel nonsense mutation in the RET protooncogene

    Energy Technology Data Exchange (ETDEWEB)

    Carlson, K.M.; Donis-Keller, H.; Langer, J.C. [and others

    1994-09-01

    Hirschsprung`s disease (HSCR, aganglionic megacolon) is characterized by a lack of ganglion cells along variable lengths of the hindgut. This is most likely due to a failure of the progenitor cells (that are destined to become the ganglion cells of the submucosal and myenteric plexuses) to complete their distal migration in the colon. Recently, mutations in the RET protoocogene have been reported in association with HSCR. We report a novel nonsense mutation resulting in a severely truncated protein. Germline DNA from a panel of 6 HSCR patients was analyzed by SSCP for 20 exons of RET. Eight exons were also directly sequenced. We identified a novel mutation within RET exon 2. The mutation (TAC{sub 36}{yields}TAG{sub 36}), which occurs at nucleotide position 108, involves the replacement of tyrosine with a stop codon and results in a truncated 35 amino acid protein. This mutation is the most 5{prime} nonsense mutation reported thus far. Interestingly, the patient has no prior family history of HSCR and was also diagnosed with multiple developmental anomalies including dysplastic kidney. Recent gene targeting studies with mouse models have shown that RET is essential for normal renal development. However, a parallel phenotype has not been seen in other reported HSCR patients with RET mutations. The observations reported here provide evidence that RET plays a role in human renal development. Ongoing studies will determine the extent of RET involvement in sporadic cases of HSCR.

  13. Dynamic chronic rectal obstruction causing a severe colonic dilatation in a cat

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    Sofia García-Pertierra

    2017-08-01

    Full Text Available Case summary A 5-year-old male neutered domestic shorthair cat was presented to our referral centre with a 13 month history of chronic tenesmus due to malunion of the right caudal iliac body. Constipation and pelvic canal stenosis were initially addressed by the referring veterinarian with a right femoral head and neck excision and a right acetabulectomy without observable clinical improvement. At admission, abdominal radiographs revealed severe colonic distension and a narrowed pelvic canal caused by the right proximal femur. Rectal examination and colonography revealed a dynamic compression of the rectum, which worsened with femoral abduction and improved with femoral adduction. A right hindlimb amputation was performed to relieve the obstruction. The cat defaecated 2 days postoperatively and was discharged uneventfully. Neither faecal tenesmus nor dyschaezia were observed over the following 10 months. Relevance and novel information The dynamic nature of the rectal obstruction most likely prevented the development of an irreversible colonic dilatation leading to a megacolon. This is the first report describing a chronic dynamic rectal compression, which was successfully managed with a right hindlimb amputation without the need for subtotal colectomy.

  14. Atividade colinesterásica no plasma e em eritrócitos de portadores de megacolo chagásico

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    Valdemar Hial

    1983-03-01

    Full Text Available A atividade colinesterásica foi medida no plasma e em eritrócitos de 32 indivíduos. Em 16 pacientes, seguramente portadores de megacolo chagásico, os níveis de colinestera- se no plasma (1.540 ±318 Ul/L e nos eritrócitos (53,2 ±9,1 Ul/g Hb estavam significativamente diminuídos, quando comparados com os níveis enzimáticos no plasma (2.554 ± 826 Ul/L e nos eritrócitos (63,6 ±11 Ul/g Hb do grupo controle (n=16. Os resultados mostram um paralelismo entre a atividade colinesterásica sistêmica e os achados histopato lógicos.Cholinesterase activity was measured in the plasma and erythrocytes from 32 individuals. The 16patients, in whon the diagnosis of Chagasic megacolon confirmed, hada significantly lower concentration of the enzyme both in plasma (1.540 ± 318 Yl/L and erythrocytes (53.2 ± 9.1 Ul/g Hb when compared with the 16 Controls in whom, the plasma and erythrocyte cholinesterase leveis were, respectively, 2.554 ± 823and 63.6 ± 11 Ul/g Hb. The results show a correlation between the systemic cholinesterase activity and the histopathological findings.

  15. Analysis of the cellular immune response in patients with the digestive and indeterminate forms of Chagas' disease.

    Science.gov (United States)

    Ribeiro, Betânia Maria; Crema, Eduardo; Rodrigues, Virmondes

    2008-08-01

    The commitment of the digestive organs observed in patients during the chronic phase of Chagas' disease is mainly attributed to neuronal damage induced by immune and inflammatory processes elicited by the presence of Trypanosoma cruzi. Here we compare the cellular immune response in patients with the digestive and indeterminate forms of Chagas' disease on the basis of lymphocyte proliferation and cytokine production after antigen or mitogen stimulation. No significant differences between patients groups were observed on proliferative response or on tumor necrosis factor (TNF)-alpha and interleukin (IL)-10 levels, although IL-10 achieves higher levels than TNF-alpha after T. cruzi antigen stimulation. Interferon (IFN)-gamma basal production was significantly higher in the digestive form and IL-4 was significantly higher in patients with megaesophagus when compared with patients with megacolon. These results indicated that patients with the digestive form of Chagas' disease do not suffer immune suppression and that the cytokine balance favors a strong inflammatory reaction in patients with the digestive form, which may contribute to lesions of the mioenteric nervous system.

  16. Clinical forms of Trypanosoma cruzi infected individuals in the chronic phase of Chagas disease in Puebla, Mexico.

    Science.gov (United States)

    Sánchez-Guillén, María Del Carmen; López-Colombo, Aurelio; Ordóñez-Toquero, Guillermo; Gomez-Albino, Isidoro; Ramos-Jimenez, Judith; Torres-Rasgado, Enrique; Salgado-Rosas, Hilda; Romero-Díaz, Mónica; Pulido-Pérez, Patricia; Pérez-Fuentes, Ricardo

    2006-11-01

    In Mexico, despite the relatively high seroprevalence of Trypanosoma cruzi infection in humans in some areas, reported morbidity of Chagas disease is not clear. We determined clinical stage in 71 individuals seropositive to T. cruzi in the state of Puebla, Mexico, an area endemic for Chagas disease with a reported seroprevalence of 7.7%. Diagnosis of Chagas disease was made by two standardized serological tests (ELISA, IHA). Individuals were stratified according to clinical studies. All patients were submitted to EKG, barium swallow, and barium enema. Groups were identified as indeterminate form (IF) asymptomatic individuals without evidence of abnormalities (n = 34 cases); those with gastrointestinal alterations (12 patients) including symptoms of abnormal relaxation of the lower esophageal sphincter and absent peristalsis in the esophageal body, grade I megaesophagus, and/or megacolon; patients with clinical manifestations and documented changes of chronic Chagas heart disease who were subdivided as follows: mild (8 patients)--mild electrocardiographic changes of ventricular repolarization, sinus bradychardia); moderate (6 patients)--left bundle branch block, right bundle branch block associated with left anterior fascicular block); severe (8 patients)--signs of cardiomegaly, dilated cardiomyopathy); and the associated form (3 cases) that included presence of both cardiomyopathy and megaesophagus. These data highlight the importance of accurate evaluation of the prevalence and clinical course of Chagas disease in endemic and non-endemic areas of Mexico.

  17. 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.

  18. Clinical forms of Trypanosoma cruzi infected individuals in the chronic phase of Chagas disease in Puebla, Mexico

    Directory of Open Access Journals (Sweden)

    María del Carmen Sánchez-Guillén

    2006-11-01

    Full Text Available In Mexico, despite the relatively high seroprevalence of Trypanosoma cruzi infection in humans in some areas, reported morbidity of Chagas disease is not clear. We determined clinical stage in 71 individuals seropositive to T. cruzi in the state of Puebla, Mexico, an area endemic for Chagas disease with a reported seroprevalence of 7.7%. Diagnosis of Chagas disease was made by two standardized serological tests (ELISA, IHA. Individuals were stratified according to clinical studies. All patients were submitted to EKG, barium swallow, and barium enema. Groups were identified as indeterminate form (IF asymptomatic individuals without evidence of abnormalities (n = 34 cases; those with gastrointestinal alterations (12 patients including symptoms of abnormal relaxation of the lower esophageal sphincter and absent peristalsis in the esophageal body, grade I megaesophagus, and/or megacolon; patients with clinical manifestations and documented changes of chronic Chagas heart disease who were subdivided as follows: mild (8 patients - mild electrocardiographic changes of ventricular repolarization, sinus bradychardia; moderate (6 patients - left bundle branch block, right bundle branch block associated with left anterior fascicular block; severe (8 patients - signs of cardiomegaly, dilated cardiomyopathy; and the associated form (3 cases that included presence of both cardiomyopathy and megaesophagus. These data highlight the importance of accurate evaluation of the prevalence and clinical course of Chagas disease in endemic and non-endemic areas of Mexico.

  19. 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.

  20. Giant Meckel’s diverticulum in an adult

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    Tomas Contreras Rivas

    2014-10-01

    Full Text Available Meckel's diverticulum results from a partial persistence of the omphalomesenteric duct and is the most common congenital anomaly of the gastrointestinal tract, affecting about 2% of the general population. Its presentation as a giant Meckel’s diverticulum (>5 cm is rare and is associated with major complications. We report a case of a 53 year-old woman with constipation for at least ten years. A colonoscopy from eight years ago suggested megacolon. The patient consults in the last month for abdominal pain associated with anorexia. The computed tomography scan image suggested an ileal megadiverticulum. An exploratory laparotomy revealed a saccular dilatation of the distal ileum of 6 x 15.5 cm, located 20 cm away from the ileocecal valve. We resected the involved segment of distal ileum and performed a manual ileo-ascendo anastomosis. The biopsy showed a saccular dilatation of the wall, lined by small intestinal mucosa with areas of gastric metaplasia, supporting the diagnosis of giant Meckel’s diverticulum.

  1. Male and female differential reproductive rate could explain parental transmission asymmetry of mutation origin in Hirschsprung disease.

    Science.gov (United States)

    Jannot, Anne-Sophie; Amiel, Jeanne; Pelet, Anna; Lantieri, Francesca; Fernandez, Raquel M; Verheij, Joke B G M; Garcia-Barcelo, Merce; Arnold, Stacey; Ceccherini, Isabella; Borrego, Salud; Hofstra, Robert M W; Tam, Paul K H; Munnich, Arnold; Chakravarti, Aravinda; Clerget-Darpoux, Françoise; Lyonnet, Stanislas

    2012-09-01

    Hirschsprung disease (HSCR, aganglionic megacolon) is a complex and heterogeneous disease with an incidence of 1 in 5000 live births. Despite the multifactorial determination of HSCR in the vast majority of cases, there is a monogenic subgroup for which private rare RET coding sequence mutations with high penetrance are found (45% of HSCR familial cases). An asymmetrical parental origin is observed for RET coding sequence mutations with a higher maternal inheritance. A parent-of-origin effect is usually assumed. Here we show that a differential reproductive rate for males and females also leads to an asymmetrical parental origin, which was never considered as a possible explanation till now. In the case of HSCR, we show a positive association between penetrance of the mutation and parental transmission asymmetry: no parental transmission asymmetry is observed in sporadic RET CDS mutation carrier cases for which penetrance of the mutation is low, whereas a parental transmission asymmetry is observed in affected sib-pairs for which penetrance of the mutation is higher. This allows us to conclude that the explanation for this parental asymmetry is that more severe mutations have resulted in a differential reproductive rate between male and female carriers.

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

    Science.gov (United States)

    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.

  3. Evaluation and treatment of constipation in infants and children.

    Science.gov (United States)

    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.

  4. Clostridium difficile 027-associated pseudomembranous colitis after short-term treatment with cefuroxime and cephalexin in an elderly orthopedic patient: a case report

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    Søgaard Kirstine

    2012-10-01

    Full Text Available Abstract Background Clostridium difficile ribotype 027 has become increasingly prevalent in European countries. The clinical picture varies from self-limiting diarrhea to pseudomembranous colitis with toxic megacolon and ultimately death. Use of antibiotics is the principal risk factor; others include comorbidity, advanced age and hospitalization. However even with extensive knowledge of risk factors, it remains difficult to define “minimum risk,” as illustrated by the following case. Case presentation An 80-year-old Danish man in good health was hospitalized for a penetrating knee injury. He received 5 days of intravenous cefuroxime after surgical revision and was discharged with oral cephalexin. Post-discharge he suffered from abdominal discomfort and was readmitted with ileus 4 days after discharge, i.e. 10 days after initiation of antibiotic treatment. His condition deteriorated, and pseudomembranous colitis was diagnosed. Due to lack of response to vancomycin and metronidazole, a total colectomy was performed. Stool cultures were positive for CD 027. Conclusion Short-term use of cephalosporins may have induced CD 027 infection, and the patient’s age was the only identifiable risk factor for the fulminant course. Thus, even short-term prophylactic treatment with cephalosporins cannot be considered entirely safe.

  5. Hypoganglionosis in pregnancy: a case report

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    Figueiredo Ana

    2012-09-01

    Full Text Available Abstract Introduction We report a very rare case of isolated hypoganglionosis first diagnosed during early pregnancy, which should be discussed from an obstetric and a gastroenterological point of view. Case presentation A pregnant 18-year-old Caucasian woman presented at twelve weeks of gestation with lower abdominal pain, mild constipation and a large abdominal mass. Abdominal and pelvic magnetic resonance imaging demonstrated a megarectum and megasigmoid, and our patient was managed with medical therapy during her pregnancy, which occurred without major incidents. At the onset of labor, a fecaloma obstructing the pelvic outlet was detected, which required manual disimpaction. However, during the procedure a sudden continuous fetal bradycardia was detected. An emergency Cesarean section was performed but the fetus suffered hypoxic ischemic encephalopathy. One year after the delivery, our patient underwent a sigmoid resection. A histopathological analysis revealed a reduction of nerve cells in the myenteric and submucous plexus, suggesting hypoganglionosis. Conclusion Although there are some reports of pregnancies complicated by megacolon, they are too few and too old to delineate guidelines for clinical orientation. In our article, we discuss several issues regarding the management of these rare intestinal innervation disorders during pregnancy that we believe will enhance their obstetric and gastroenterological management during pregnancy.

  6. Ogilvie's syndrome following posterior spinal arthrodesis for scoliosis.

    Science.gov (United States)

    Tsirikos, Athanasios I; Sud, Alok

    2013-07-01

    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 T4 to L3 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.

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

    Science.gov (United States)

    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.

  8. Proteomic profile of circulating immune complexes in chronic Chagas disease.

    Science.gov (United States)

    Ohyama, K; Huy, N T; Yoshimi, H; Kishikawa, N; Nishizawa, J E; Roca, Y; Revollo Guzmán, R J; Velarde, F U G; Kuroda, N; Hirayama, K

    2016-10-01

    Immune complexes (ICs) are the direct and real-time products of humoral immune responses. The identification of constituent foreign or autoantigens within ICs might bring new insights into the pathology of infectious diseases. We applied immune complexome analysis of plasma to the study of Chagas disease caused by Trypanosoma cruzi. Twenty seropositive plasma samples including cardiac and/or megacolon determinate patients (n = 11) and indeterminate (n = 9) were analysed along with 10 seronegative individuals to characterize the antigens bound to circulating ICs. We identified 39 T. cruzi antigens and 114 human autoantigens specific to patients with Chagas. Among those antigens, two T. cruzi antigens (surface protease GP63, glucose-6-isomerase) and six human autoantigens (CD180 antigen, ceruloplasmin, fibrinogen beta chain, fibrinogen beta chain isoform 2 preprotein, isoform gamma-A of fibrinogen γ-chain, serum paraoxonase) were detected in more than 50% of the patients tested. Human isoform short of complement factor H-related protein 2 and trans-sialidase of T. cruzi were more frequently found in the indeterminate (5/9 for both) compared with in the determinate Chagas (0/11, P = 0·046 for human, 1/11, P = 0·0498 for T. cruzi). The immune complexome could illustrate the difference of immune status between clinical forms of chronic Chagas disease. © 2016 John Wiley & Sons Ltd.

  9. ABO, Secretor and Lewis histo-blood group systems influence the digestive form of Chagas disease.

    Science.gov (United States)

    Bernardo, Cássia Rubia; Camargo, Ana Vitória Silveira; Ronchi, Luís Sérgio; de Oliveira, Amanda Priscila; de Campos Júnior, Eumildo; Borim, Aldenis Albaneze; Brandão de Mattos, Cinara Cássia; Bestetti, Reinaldo Bulgarelli; de Mattos, Luiz Carlos

    2016-11-01

    Chagas disease, caused by Trypanosoma cruzi, can affect the heart, esophagus and colon. The reasons that some patients develop different clinical forms or remain asymptomatic are unclear. It is believed that tissue immunogenetic markers influence the tropism of T. cruzi for different organs. ABO, Secretor and Lewis histo-blood group systems express a variety of tissue carbohydrate antigens that influence the susceptibility or resistance to diseases. This study aimed to examine the association of ABO, secretor and Lewis histo-blood systems with the clinical forms of Chagas disease. We enrolled 339 consecutive adult patients with chronic Chagas disease regardless of gender (cardiomyopathy: n=154; megaesophagus: n=119; megacolon: n=66). The control group was composed by 488 healthy blood donors. IgG anti-T. cruzi antibodies were detected by ELISA. ABO and Lewis phenotypes were defined by standard hemagglutination tests. Secretor (FUT2) and Lewis (FUT3) genotypes, determined by Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP), were used to infer the correct histo-blood group antigens expressed in the gastrointestinal tract. The proportions between groups were compared using the χ2 test with Yates correction and Fisher's exact test and the Odds Ratio (OR) and 95% Confidence Interval (95% CI) were calculated. An alpha error of 5% was considered significant with p-values Chagas disease. Copyright © 2016 Elsevier B.V. All rights reserved.

  10. 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.

  11. Chagas disease in Europe: A review for the internist in the globalized world.

    Science.gov (United States)

    Antinori, Spinello; Galimberti, Laura; Bianco, Roberto; Grande, Romualdo; Galli, Massimo; Corbellino, Mario

    2017-09-01

    Chagas disease (CD) or American trypanosomiasis identified in 1909 by Carlos Chagas, has become over the last 40years a global health concern due to the huge migration flows from Latin America to Europe, United States, Canada and Japan. In Europe, most migrants from CD-endemic areas are concentrated in Spain, Italy, France, United Kingdom and Switzerland. Pooled seroprevalence studies conducted in Europe show an overall 4.2% prevalence, with the highest infection rates observed among individuals from Bolivia (18.1%). However, in most European countries the disease is neglected with absence of screening programmes and low access to diagnosis and treatment. Physicians working in Europe should also be aware of the risk of autochthonous transmission of Trypanosoma cruzi to newborns by their infected mothers and to recipients of blood or transplanted organs from infected donors. Finally, physicians should be able to recognize and treat the most frequent and serious complications of chronic Chagas disease, namely cardiomyopathy, megacolon and megaesophagus. This review aims to highlights the problem of CD in Europe by reviewing papers published by European researchers on this argument, in order to raise the awareness of internists who are bound to increasingly encounter patients with the disease in their routine daily activities. Copyright © 2017 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

  12. 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.

  13. 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.

  14. Intestinal Neuronal Dysplasia Type B in Adults: A Controversial Entity

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    Vasillis Vougas

    2014-01-01

    Full Text Available Intestinal neuronal dysplasia (IND is a well-defined entity which raises controversy among authors, described as a congenital malformation of gastrointestinal innervation and caused by dysplastic embryonal development of the enteric nervous system. It is potentially associated with mild and chronic gastrointestinal motility disturbances. IND is rarely reported in adults and especially elderly patients. The present study reports on the case of a 71-year-old man suffering from longstanding idiopathic constipation and who was misdiagnosed for more than 60 years, despite several hospital admissions and a sigmoidectomy in the meantime. On the last admission, the patient presented with megacolon, abdominal pain and X-ray finding of bowel obstruction. Due to massive large bowel dilatation, an exploratory laparotomy failed to reveal any obvious mechanical cause, and a subtotal colectomy and Hartmann's procedure was performed. Bowel continuity was performed 3 months later. Analysis of full-thickness biopsies revealed enlarged myenteric and submucosal neurons as well as an increased number of giant cells and increased acetylcholinesterase activity in the mucosa. The diagnosis of IND was established. The main diagnostic criteria, the underlining pathophysiology and the recommended therapeutic approach of this rare entity are extensively reviewed.

  15. Surgery for Severe Ulcerative Colitis during Pregnancy: Report of Two Cases

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    Motoi Uchino

    2015-03-01

    Full Text Available Refractory ulcerative colitis (UC that does not respond to medical therapy often requires surgery even during pregnancy. Although surgical cases of UC during pregnancy were reported previously, the standard surgical strategy for both colitis and pregnancy was unclear. Herein, fetal and maternal safety as well as the strategy for this unusual surgical procedure during pregnancy in patients with UC are considered. A 28-year-old woman was diagnosed with left-sided moderate UC at 12 weeks of pregnancy; toxic megacolon was suspected, and surgery was required. Although the baby's gestational age was 23 weeks and 3 days, a cesarean section was performed before the colectomy. In a next case, a 28-year-old woman had a 2-year history of left-sided UC. Her colitis flared up at 11 weeks of pregnancy. Colectomy was performed because her colitis was unresponsive to conservative therapy, and the pregnancy was continued, with a transvaginal delivery at 36 weeks. In patients with UC, the need for surgery should be determined promptly based on disease severity, whether or not the patient is pregnant. The need for surgery should not be affected by pregnancy. The pregnancy should be continued for as long as possible when there are no fetal and maternal complications. Both cesarean section and colectomy should be performed independently if necessary.

  16. 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.

  17. 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

  18. Chagas' disease: selective affinity and cytotoxicity of Trypanosoma cruzi-immune lymphocytes to parasympathetic ganglion cells

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    Maria Lúcia Teixeira

    1980-12-01

    Full Text Available The megaesophagus and megacolon endemic in South America are related , to Chagas' disease. These mega conditions are found in patients with chronic Chagas's infection, when the parasite is not demonstrable in the lesions. These are characterized by depopulation of parasympathetic ganglion cells, dilation and hypertrophy of the viscera. In the experiments described here we deminstrate a selective affinity and adherence of Trypanosoma cruzi-immune lymphocytes to myenteric, parasympathetic ganglion cells, leading to neuronolysis. None of these features are observed when non-immune lymphocytes from control rabbits are used, or when the immune lymphocytes are allowed to react with CNS neurons. This demonstration is an indication of the high degree of specificity of the destruction of parasympathetic neurons in Chagas' disease. We postulate that the T. cruzi-immune lymphocyte rejection of parasympathetic neurons, but not of CNS neurons, might be related to recognition of a cross-reacting antigenic determinant secreted only by the target neurons. In favor of this interpretation is the observation of lymphocytic infiltrates and parasympathetic ganglion cell destruction in chronic Chagas' infection in the absence of encephalitis.O megaesôfago e o megacolon endêmicos na América do Sul estão relacionados á doença de Chagas. Estas condições clínicas são encontradas em pacientes com infecção chagásica crônica, quando o parasito não é demonstrado nas lesões caracterizadas por povoamento de células neuronais parassimpáticas. Nos experimentos descritos aqui nós demonstramos uma afinidade seletiva de linfócitos imunes, sensibilizados pelo T. cruzi, para neurônios de gânglios mioentéricos. Os linfócitos imunes citotóxicos aderem nas células ganglionares, produzindo neuronólise. Isto não se observa quando linfócitos não-imunes são usados, ou quando os linfócitos imunes são colocados na presença de neurônios do sistema nervoso

  19. 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

  20. Dosage effects of cohesin regulatory factor PDS5 on mammalian development: implications for cohesinopathies.

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    Bin Zhang

    Full Text Available Cornelia de Lange syndrome (CdLS, a disorder caused by mutations in cohesion proteins, is characterized by multisystem developmental abnormalities. PDS5, a cohesion protein, is important for proper chromosome segregation in lower organisms and has two homologues in vertebrates (PDS5A and PDS5B. Pds5B mutant mice have developmental abnormalities resembling CdLS; however the role of Pds5A in mammals and the association of PDS5 proteins with CdLS are unknown. To delineate genetic interactions between Pds5A and Pds5B and explore mechanisms underlying phenotypic variability, we generated Pds5A-deficient mice. Curiously, these mice exhibit multiple abnormalities that were previously observed in Pds5B-deficient mice, including cleft palate, skeletal patterning defects, growth retardation, congenital heart defects and delayed migration of enteric neuron precursors. They also frequently display renal agenesis, an abnormality not observed in Pds5B(-/- mice. While Pds5A(-/- and Pds5B(-/- mice die at birth, embryos harboring 3 mutant Pds5 alleles die between E11.5 and E12.5 most likely of heart failure, indicating that total Pds5 gene dosage is critical for normal development. In addition, characterization of these compound homozygous-heterozygous mice revealed a severe abnormality in lens formation that does not occur in either Pds5A(-/- or Pds5B(-/- mice. We further identified a functional missense mutation (R1292Q in the PDS5B DNA-binding domain in a familial case of CdLS, in which affected individuals also develop megacolon. This study shows that PDS5A and PDS5B functions other than those involving chromosomal dynamics are important for normal development, highlights the sensitivity of key developmental processes on PDS5 signaling, and provides mechanistic insights into how PDS5 mutations may lead to CdLS.

  1. [Role of fetal urine sampling in the prenatal diagnosis of malformative uropathies].

    Science.gov (United States)

    Lopez, Christophe; Faure, Jean-Michel; Deschamps, Françoise; Boulot, Pierre; Averous, Michel

    2002-12-01

    To define the diagnostic and therapeutic value of foetal urine sampling (FUS). Between 1996 and 2001, FUS was performed in 16 patients for three indications: assessment of renal function (Group 1, n = 12), diagnosis of a cystic mass (Group 2, n = 2), prevention of dystocia (Group 3, n = 2). The anomaly was detected by ultrasound. Urine was aspirated from the two renal pelves and/or bladder, or the cystic mass in the other cases. b2-microglobulin and urinary sodium were assayed and karyotyping was performed at the same time. 20 FUS were performed In 4 cases, two samples were taken at an interval of 2 weeks. No complications were observed. Group 1: 9 pregnancies were terminated for potential renal failure (6 posterior urethral valves (PUV), 1 Prune Belly syndrome, 2 cases of recessive polycystic kidney disease (PKD). Three pregnancies were continued for 2 foetuses with normal renal function (1 PUV, 1 ureterocele) and one foetus died with severe renal failure (bilateral hydronephrosis). Group 2: one termination of pregnancy was performed in a case of hepatic cyst with portobiliary dysplasia and a giant bladder diverticulum was operated. Group 3: in both cases, evacuating aspiration allowed normal delivery of one live infant (left polycystic dysplasia), and one neonatal death from megabladder-megacolon syndrome. The value of FUS is controversial. We usually performed this examination for assessment of renal function. The results of FUS are useful for management decisions when they are in favour of potential impaired renal function. They usually confirmed the ultrasound results that already indicated a poor prognosis and they did not change the decision already based on ultrasound findings in any of our cases. For the diagnosis of cystic masses, after drainage of the mass, FUS facilitated investigation of the urinary tract and adjacent organs. When FUS was performed during labour, it reduced the size of the mass and the abdomen, allowing vaginal delivery with a better

  2. Surgical treatment of inflammatory bowel diseases; Chirurgische Behandlung entzuendlicher Darmerkrankungen

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    Fuerst, H.; Schildberg, F.W. [Chirurgische Klinik und Poliklinik, Ludwig Maximilians Univ. Muenchen, Klinikum Grosshadern (Germany)

    1998-01-01

    Purpose: To summarize current knowledge on surgical therapy in patients with inflammatory bowel disease (Crohn`s disease, ulcerative colitis and diverticulitis). Material and methods: To discuss surgical indications and strategies, we reviewed major peer review publications of the last 10 years, and we also analysed data from patients with Crohn`s disease who were treated in our institution between 1978 and 1994. Results: With Crohn`s disease (305 patients), emergency surgery should be avoided as much as possible, since morbidity (50% vs 8.8%) and mortality (11% vs 0.6%) rise significantly in comparison to elective procedures. With ulcerative colitis, operative therapy is indicated in patients with secondary malignoma, and urgent surgery is requested in cases with associated perforation, toxic megacolon or massive bleeding. With diverticulitis, the first episode should be managed conservatively. Surgery is indicated in patients with recurrent episodes or with secondary complications. (orig.) [Deutsch] In dieser Uebersichtsarbeit wird die operative Behandlung der entzuendlichen Darmerkrankungen M. Crohn, Colitis ulcerosa und Divertikulitis dargestellt. Anhand einer Literaturuebersicht wird sowohl die Operationsindikation als auch das operative Vorgehen besprochen, wobei beim M. Crohn auch Daten des eigenen Patientengutes zwischen 1978-1994 eingehen. Anhand der Daten von 305 Patienten wird deutlich, dass eine Notfalloperation bei M. Crohn vermieden werden muss, da darunter die Komplikationsrate (50% vs. 8,8%) und die Letalitaet (11% vs. 0,6%) deutlich steigen. Absolute Operationsindikationen bei Colitis ulcerosa sind die Perforation, das toxische Megakolon, Blutungen und das Auftreten eines Karzinoms. Der erste Schub einer Divertikulitis wird konservativ behandelt, bei Rezidivschueben sowie bei Kompliktionen besteht eine Operationsindikation. Wesentlich ist die rechtzeitige Operationsindikation bei M. Crohn und Divertikulitis, um Notfallsituationen zu vermeiden. Ziel

  3. Regulatory Lymphoid and Myeloid Cells Determine the Cardiac Immunopathogenesis of Trypanosoma cruzi Infection

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    Manuel Fresno

    2018-03-01

    Full Text Available Chagas disease is a multisystemic disorder caused by the protozoan parasite Trypanosoma cruzi, which affects ~8 million people in Latin America, killing 7,000 people annually. Chagas disease is one of the main causes of death in the endemic area and the leading cause of infectious myocarditis in the world. T. cruzi infection induces two phases, acute and chronic, where the infection is initially asymptomatic and the majority of patients will remain clinically indeterminate for life. However, over a period of 10–30 years, ~30% of infected individuals will develop irreversible, potentially fatal cardiac syndromes (chronic chagasic cardiomyopathy [CCC], and/or dilatation of the gastro-intestinal tract (megacolon or megaesophagus. Myocarditis is the most serious and frequent manifestation of chronic Chagas heart disease and appears in about 30% of infected individuals several years after infection occurs. Myocarditis is characterized by a mononuclear cell infiltrate that includes different types of myeloid and lymphoid cells and it can occur also in the acute phase. T. cruzi infects and replicates in macrophages and cardiomyocytes as well as in other nucleated cells. The pathogenesis of the chronic phase is thought to be dependent on an immune-inflammatory reaction to a low-grade replicative infection. It is known that cytokines produced by type 1 helper CD4+ T cells are able to control infection. However, the role that infiltrating lymphoid and myeloid cells may play in experimental and natural Chagas disease pathogenesis has not been completely elucidated, and several reports indicate that it depends on the mouse genetic background and parasite strain and/or inoculum. Here, we review the role that T cell CD4+ subsets, myeloid subclasses including myeloid-derived suppressor cells may play in the immunopathogenesis of Chagas disease with special focus on myocarditis, by comparing results obtained with different experimental animal models.

  4. Chromosomal imbalances are uncommon in chagasic megaesophagus

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    Silva Ana E

    2010-02-01

    Full Text Available Abstract Background Chagas' disease is a human tropical parasitic illness and a subset of the chronic patients develop megaesophagus or megacolon. The esophagus dilation is known as chagasic megaesophagus (CM and one of the severe late consequences of CM is the increased risk for esophageal carcinoma (ESCC. Based on the association between CM and ESCC, we investigated whether genes frequently showing unbalanced copy numbers in ESCC were altered in CM by fluorescence in situ (FISH technology. Methods A total of 50 formalin-fixed, paraffin-embedded esophageal mucosa specimens (40 from Chagas megaesophagus-CM, and 10 normal esophageal mucosa-NM were analyzed. DNA FISH probes were tested for FHIT, TP63, PIK3CA, EGFR, FGFR1, MYC, CDKN2A, YES1 and NCOA3 genes, and centromeric sequences from chromosomes 3, 7 and 9. Results No differences between superficial and basal layers of the epithelial mucosa were found, except for loss of copy number of EGFR in the esophageal basal layer of CM group. Mean copy number of CDKN2A and CEP9 and frequency of nuclei with loss of PIK3CA were significantly different in the CM group compared with normal mucosa and marginal levels of deletions in TP63, FHIT, PIK3CA, EGFR, CDKN2A, YES and gains at PIK3CA, TP63, FGFR1, MYC, CDNK2A and NCOA3 were detected in few CM cases, mainly with dilation grades III and IV. All changes occurred at very low levels. Conclusions Genomic imbalances common in esophageal carcinomas are not present in chagasic megaesophagus suggesting that these features will not be effective markers for risk assessment of ESCC in patients with chagasic megaesophagus.

  5. 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

  6. De novo multiple endocrine neoplasia type 2B with noncardiogenic pulmonary edema as the presenting symptom.

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    Sato, Haruhiro; Suzuki, Yasuhiro; Fukasawa, Maki; Yasuda, Masanori; Osamura, Robert Yoshiyuki

    2006-08-01

    Multiple endocrine neoplasia (MEN) type 2B is a rare hereditary disorder characterized by medullary thyroid carcinoma, pheochromocytoma, and neuroma. Early signs of MEN 2B are usually neuroma, gastrointestinal problems, and medullary thyroid carcinoma. Noncardiogenic pulmonary edema is rare as a presenting symptom. We report a 31-year-old male who was admitted to our hospital because of noncardiogenic pulmonary edema. He was 168 cm in height, weighed 55 kg, and had an arm span of 166 cm. No marfanoid habitus was evident, but thickened lips and tongue neuroma were present. Chronic constipation had been present since childhood, and the patient had a two-year history of untreated hypertension. Noncardiogenic pulmonary edema and toxic megacolon were noted, and abdominal computed tomography revealed bilateral adrenal tumors. Ultrasonography of the thyroid showed two mass lesions. Intubation and mechanical ventilation were performed because of severe hypoxemia. Endocrinological examinations showed high levels of serum and urinary fractionated catecholamines, serum calcitonin, serum carcinoembryonic antigen, and serum intact parathyroid hormone. It was suggested that the high level of catecholamine from pheochromocytoma had caused the pulmonary edema. RET gene analysis showed a codon 918 mutation in exon 16 resulting in an ATG (methionine) to ACG (threonine) substitution, but analysis of the patient's parents showed the wild type. Therefore, the patient was diagnosed as having de novo MEN 2B. He underwent laparoscopic bilateral adrenectomy and total thyroidectomy. However, the values of serum calcitonin and CEA did not decrease to the normal ranges. Patients with early-stage MEN 2B have distinct characteristics that can aid early detection of the disease, thus possibly allowing them to be saved.

  7. 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.

  8. 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

  9. Investigation of the Cross-talk Mechanism in Caco-2 Cells duringClostridium difficileInfection through Genetic-and-Epigenetic Interspecies Networks: Big Data Mining and Genome-Wide Identification.

    Science.gov (United States)

    Li, Cheng-Wei; Su, Ming-He; Chen, Bor-Sen

    2017-01-01

    Clostridium difficile is the leading cause of nosocomial antibiotic-associated diarrhea and the major etiologic agent of pseudomembranous colitis. In severe cases, C. difficile infection (CDI) can cause toxic megacolon, intestinal perforation, and death. The intestinal epithelium is the first tissue encountered in the adhesion and colonization of C. difficile , and serves as a physical defense barrier against infection. Despite the well-characterized cytotoxicity, few studies have investigated the genome-wide interplay between host cells and C. difficile . The aim of this study is to investigate the genetic-and-epigenetic molecular mechanisms between human colorectal epithelial Caco-2 cells and C. difficile during the early (0-60 min) and late stages (30-120 min) of infection. To investigate the cross-talk mechanisms during the progression of infection, we introduced a systems biology approach using big data mining, dynamic network modeling, a genome-wide data identification method, system order detection scheme, and principal network projection method (PNP). We focused on the construction of genome-wide genetic-and-epigenetic interspecies networks (GEINs) and subsequent extraction of host-pathogen core networks (HPNs) to investigate the progression of underlying host/pathogen genetic-and-epigenetic mechanisms from the early to late stages of CDI. Based on our results, we suggest that the cell-wall proteins CD2787 and CD0237, which both play an important role in cell adhesion and pathogen defense mechanisms, can be considered as potential drug targets. In addition, the crucial proteins employed by C. difficile for sporulation, including CD1214, CD2629, and CD2643, can also be considered as potential drug targets since spore-mediated re-infection is a critical issue.

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

    Science.gov (United States)

    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.

  11. Hirschprung's disease in different settings - a series of three cases from a tertiary referral center.

    Science.gov (United States)

    Singer, Cristina Elena; Coşoveanu, Carmen Simona; Ciobanu, Mircea Ovidiu; Stoica, George Alin; Puiu, Ileana; Gruia, Corina Lavinia; Streba, Liliana; Constantin, Cristian; Neagoe, Carmen Daniela

    2015-01-01

    Failure of neural crest cells to migrate from neural crests during intrauterine development result in partial or total aganglionosis of the colon in newborn. Hirschprung's disease (HD) represents the clinical manifestation of this pathogenic process, currently accounting for the majority of lower intestinal obstruction in the first period of life. Our aim was to present a series of three cases presenting to our tertiary care center with a range of symptoms, all benefiting from surgery and consequent pathology examination of biopsy or resection pieces. The first case was of a male newborn that presented several years ago with common symptoms for HD (abdominal distension, vomiting and the total lack of intestinal passage for feces). Coming from young healthy parents after normal labor, the newborn displayed signs of Down's disease after physical examination. After abdominal radiography, the patient underwent surgery and consecutive pathology revealed notable signs of Crohn's disease (CD): massive stasis in the serosa and submucosa, chronic inflammatory infiltrate and lack of nervous cells in both plexuses and mucosa. Immunohistochemistry revealed low intensity CD34 membrane staining for fibroblast-like ganglion cells while CD117 staining showed few nervous cells within the mucosa. The second case presented before one year of age with an infectious background, already being operated upon with colostoma. We performed corrective surgery of the colostoma and consecutive pathology showed low CD117 cytoplasmic staining and intensely positive NSE (neuron specific enolase) staining within myenteric plexuses. Finally, the third and most recent case was that of a 4-year-old boy with an early diagnosis of megacolon and no previous surgery, who we evaluated by laparoscopy with five biopsies and consecutive S100 staining revealed a small number of nervous cells within nervous plexuses. In conclusion, an early diagnosis of HD is essential for successful therapeutic measures

  12. Pathogenicity Locus, Core Genome, and Accessory Gene Contributions to Clostridium difficile Virulence

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    Brittany B. Lewis

    2017-08-01

    Full Text Available Clostridium difficile is a spore-forming anaerobic bacterium that causes colitis in patients with disrupted colonic microbiota. While some individuals are asymptomatic C. difficile carriers, symptomatic disease ranges from mild diarrhea to potentially lethal toxic megacolon. The wide disease spectrum has been attributed to the infected host’s age, underlying diseases, immune status, and microbiome composition. However, strain-specific differences in C. difficile virulence have also been implicated in determining colitis severity. Because patients infected with C. difficile are unique in terms of medical history, microbiome composition, and immune competence, determining the relative contribution of C. difficile virulence to disease severity has been challenging, and conclusions regarding the virulence of specific strains have been inconsistent. To address this, we used a mouse model to test 33 clinical C. difficile strains isolated from patients with disease severities ranging from asymptomatic carriage to severe colitis, and we determined their relative in vivo virulence in genetically identical, antibiotic-pretreated mice. We found that murine infections with C. difficile clade 2 strains (including multilocus sequence type 1/ribotype 027 were associated with higher lethality and that C. difficile strains associated with greater human disease severity caused more severe disease in mice. While toxin production was not strongly correlated with in vivo colonic pathology, the ability of C. difficile strains to grow in the presence of secondary bile acids was associated with greater disease severity. Whole-genome sequencing and identification of core and accessory genes identified a subset of accessory genes that distinguish high-virulence from lower-virulence C. difficile strains.

  13. Plain abdominal radiographs in patients with Crohn's disease: Radiological findings and diagnostic value

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    O' Regan, K.; O' Connor, O.J.; O' Neill, S.B.; Mc Laughlin, P.D. [Department of Radiology, Cork University Hospital and University College Cork, Cork (Ireland); Desmond, A. [Department of Medicine (Gastroenterology), Cork University Hospital and University College Cork, Cork (Ireland); McWilliams, S.R. [Department of Radiology, Cork University Hospital and University College Cork, Cork (Ireland); Quigley, E.M.M.; Shanahan, F. [Department of Medicine (Gastroenterology), Cork University Hospital and University College Cork, Cork (Ireland); Maher, M.M., E-mail: M.Maher@ucc.ie [Department of Radiology, Cork University Hospital and University College Cork, Cork (Ireland)

    2012-08-15

    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.

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

    Science.gov (United States)

    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.

  15. 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

  16. Radiological evaluation of surgical emergencies in neonate

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    Suh, C. O.; Oh, K. K.; Park, C. Y. [Yonsei University, Medical College, Seoul (Korea, Republic of)

    1980-12-15

    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

  17. The burden of Chagas disease: estimates and challenges.

    Science.gov (United States)

    Stanaway, Jeffrey D; Roth, Gregory

    2015-09-01

    Chagas disease, caused by infection with the protozoa Trypanosoma cruzi is transmitted most often by Triatominae insect vectors, but also through blood transfusion, organ transplant, and congenital transmission. Between 5 and 18 million people are currently infected and the infection is estimated to cause more than 10,000 deaths annually. The disease has 3 phases: acute, indeterminate, and chronic. The acute phase immediately follows infection. It is typically asymptomatic but produces fever and malaise in up to 5% of people. The indeterminate phase is asymptomatic. More than one-half of those infected will remain in this phase for life and never experience long-term sequelae. After a decade or more, 20% to 30% of people will experience chronic cardiovascular Chagas disease with sequelae including heart failure, arrhythmias, and thromboembolism. Another 15% to 20% will experience chronic digestive sequela including megaesophagus and megacolon. A complete accounting of the burden of Chagas disease requires estimating the prevalence of the infection, the prevalence of each of its sequelae among those with the infection, and the number of deaths attributable to the infection. Attempts to estimate Chagas disease prevalence are complicated by several challenges imposed by the disease's extreme spatial heterogeneity, quickly evolving temporal trends, the decades-long lag between infection and symptomatic disease, biased prevalence data, incomplete recognition of Chagas-attributable deaths, limited data on sequela, and a near total absence of data outside of endemic countries. Even though researchers have found methodological approaches to dealing with these challenges, there is a need for better data. Copyright © 2015 World Heart Federation (Geneva). Published by Elsevier B.V. All rights reserved.

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

    Science.gov (United States)

    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.

  19. [The Latin-American Consensus on Chronic Constipation].

    Science.gov (United States)

    Schmulson Wasserman, Max; Francisconi, Carlos; Olden, Kevin; Aguilar Paíz, Luis; Bustos-Fernández, Luis; Cohen, Henry; Passos, Maria Carmo; González-Martínez, Marina Alejandra; Iade, Beatriz; Iantorno, Guido; Ledesma Ginatta, Carlos; López-Colombo, Aurelio; Pérez, Cesar Louis; Madrid-Silva, Ana María; Quilici, Flavio; Quintero Samudio, Isaac; Rodríguez Varón, Alberto; Suazo, Jorge; Valenzuela, Jorge; Zolezzi, Alberto

    2008-02-01

    The Latin-American Consensus on Chronic Constipation aimed to establish guidelines to improve the identification, diagnosis and treatment of this disorder in the region. Two coordinators and an honorary coordinator established the process and the topics to be discussed, based on a systematic review of the literature published in the previous 10 years, since 1995. Seventeen members participated with the support of their local gastroenterology societies. The members reviewed the different subjects based on the levels of evidence and grades of recommendation; the topics were then discussed in a plenary session. A written report was drafted and the coordinators prepared the final declarations to be submitted to a vote by all the members in October 2006. The consensus concluded that chronic constipation has an estimated prevalence of 5-21% in the region, with a female-to-male ratio of 3:1. Among individuals with constipation, 75% use some type of medication, with more than 50% using home remedies. A diagnosis based on Rome Criteria was recommended and diagnostic testing only in persons older than 50 years or with alarm symptoms. The use of barium enema as an initial investigation was recommended only in countries with a high prevalence of idiopathic megacolon or Chagas' disease. Recommendations on treatment included an increase in dietary fiber of up to 25-30 g/day (grade C). No evidence was found to recommend measures such as exercise, increased water intake, or frequent visits to the toilet. Fiber supplements such as Psyllium received a grade B and pharmacological treatments such as tegaserod and polyethylene glycol, both grade A. There was insufficient evidence to recommend lactulose, but the consensus did not disadvise its use when necessary. Complementary investigations such as colonic transit followed by anorectal manometry and defecography were only recommended to rule out colonic inertia and/or obstructive defecation in patients not responding to treatment

  20. Current approaches to the management of new-onset ulcerative colitis

    Science.gov (United States)

    Marchioni Beery, Renée; Kane, Sunanda

    2014-01-01

    Ulcerative colitis (UC) is an idiopathic, inflammatory gastrointestinal disease of the colon. As a chronic condition, UC follows a relapsing and remitting course with medical maintenance during periods of quiescent disease and appropriate escalation of therapy during times of flare. Initial treatment strategies must not only take into account current clinical presentation (with specific regard for extent and severity of disease activity) but must also take into consideration treatment options for the long-term. The following review offers an approach to new-onset UC with a focus on early treatment strategies. An introduction to the disease entity is provided along with an approach to initial diagnosis. Stratification of patients based on clinical parameters, disease extent, and severity of illness is paramount to determining course of therapy. Frequent assessments are required to determine clinical response, and treatment intensification may be warranted if expected improvement goals are not appropriately reached. Mild-to- moderate UC can be managed with aminosalicylates, mesalamine, and topical corticosteroids with oral corticosteroids reserved for unresponsive cases. Moderate-to-severe UC generally requires oral or intravenous corticosteroids in the short-term with consideration of long-term management options such as biologic agents (as initial therapy or in transition from steroids) or thiopurines (as bridging therapy). Patients with severe or fulminant UC who are recalcitrant to medical therapy or who develop disease complications (such as toxic megacolon) should be considered for colectomy. Early surgical referral in severe or refractory UC is crucial, and colectomy may be a life-saving procedure. The authors provide a comprehensive evidence-based approach to current treatment options for new-onset UC with discussion of long-term therapeutic efficacy and safety, patient-centered perspectives including quality of life and medication compliance, and future

  1. Inflammatory chronic disease of the colon: How to image

    Energy Technology Data Exchange (ETDEWEB)

    Ambrosini, Roberta [Department of Diagnostic and Interventional Radiology, ' Maggiore della Carita' University Hospital, ' A. Avogadro' Eastern Piemonte University, Corso Mazzini 18, 28100 Novara (Italy)]. E-mail: rambrosini@sirm.org; Barchiesi, Annalisa [Department of Diagnostic and Interventional Radiology, ' Maggiore della Carita' University Hospital, ' A. Avogadro' Eastern Piemonte University, Corso Mazzini 18, 28100 Novara (Italy); Di Mizio, Veronica [Department of Radiology, ' S. Massimo' Hospital, Via Battaglione degli Alpini, 65017 Penne (PE) (Italy); Di Terlizzi, Marco [Department of Diagnostic and Interventional Radiology, ' Maggiore della Carita' University Hospital, ' A. Avogadro' Eastern Piemonte University, Corso Mazzini 18, 28100 Novara (Italy); Leo, Luca [Department of Diagnostic and Interventional Radiology, ' Maggiore della Carita' University Hospital, ' A. Avogadro' Eastern Piemonte University, Corso Mazzini 18, 28100 Novara (Italy); Filippone, Antonella [Department of Radiological Sciences and Bioimages, ' SS. Annunziata' University Hospital, ' G. d' Annunzio' University, Via dei Vestini, 66013 Chieti (Italy); Canalis, Luigi [Department of Diagnostic and Interventional Radiology, ' Maggiore della Carita' University Hospital, ' A. Avogadro' Eastern Piemonte University, Corso Mazzini 18, 28100 Novara (Italy); Fossaceca, Rita [Department of Diagnostic and Interventional Radiology, ' Maggiore della Carita' University Hospital, ' A. Avogadro' Eastern Piemonte University, Corso Mazzini 18, 28100 Novara (Italy); Carriero, Alessandro [Department of Diagnostic and Interventional Radiology, ' Maggiore della Carita' University Hospital, ' A. Avogadro' Eastern Piemonte University, Corso Mazzini 18, 28100 Novara (Italy)

    2007-03-15

    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.

  2. 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

  3. 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

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

    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

  5. Linsitinib (OSI-906) versus placebo for patients with locally advanced or metastatic adrenocortical carcinoma: a double-blind, randomised, phase 3 study.

    Science.gov (United States)

    Fassnacht, Martin; Berruti, Alfredo; Baudin, Eric; Demeure, Michael J; Gilbert, Jill; Haak, Harm; Kroiss, Matthias; Quinn, David I; Hesseltine, Elizabeth; Ronchi, Cristina L; Terzolo, Massimo; Choueiri, Toni K; Poondru, Srinivasu; Fleege, Tanya; Rorig, Ramona; Chen, Jihong; Stephens, Andrew W; Worden, Francis; Hammer, Gary D

    2015-04-01

    patients in the placebo group), nausea (two [2%] vs none), and hyperglycaemia (two [2%] vs none). No adverse events in the linsitinib group were deemed to be treatment related; one death (due to sepsis and megacolon) in the placebo group was deemed to be treatment related. Linsitinib did not increase overall survival and so cannot be recommended as treatment for this general patient population. Further studies of IGF-1R and insulin receptor inhibitors, together with genetic profiling of responders, might pave the way toward individualised and improved therapeutic options in adrenocortical carcinoma. Astellas. Copyright © 2015 Elsevier Ltd. All rights reserved.

  6. Frequency and Variables Associated with Fasting Orders in Inpatients with Ulcerative Colitis: The Audit of Diet Orders-Ulcerative Colitis (ADORE-UC) Study.

    Science.gov (United States)

    Gallinger, Zane R; Rumman, Amir; Pivovarov, Kevin; Fortinsky, Kyle J; Steinhart, A Hillary; Weizman, Adam V

    2017-10-01

    Current clinical practice guidelines suggest that patients with ulcerative colitis (UC) hospitalized because of a disease flare should be offered a normal diet, unless such a diet is not tolerated. Studies of hospitalized patients have demonstrated iatrogenic malnutrition from unjustified or inappropriate nil per os (NPO) or clear liquid diet (CLD) orders. In this study, we aim to characterize the burden of this problem in hospitalized patients with UC. We conducted a retrospective cohort study of all patients with UC admitted to the gastroenterology service or the general internal medicine service at a tertiary, academic hospital between January 2009 and December 2014, with a length of stay between 2 and 30 days. The frequency and duration of bowel rest and CLD orders was recorded, and the number of meals missed because of these orders was assessed. NPO or CLD diet orders were considered justified if the patient had intractable nausea or vomiting, pancreatitis, bowel obstruction, toxic megacolon or were awaiting endoscopy, or if alternative enteral nutrition was provided. Clinical and demographic factors associated with unjustified underfeeding were identified. A total of 187 admissions among 158 patients with UC were identified during the study period and included in the final analysis. Most admissions were to the gastroenterology service (148/187, 79.1%). The mean age at admission was 35.0 years (SD = 15), and 83/158 (52.5%) were female. The median length of stay was 8 days (interquartile range = 4-12). Registered dietician consultation was obtained in only 32 admissions (17.1%), and admission weight was recorded in only 68 (36.4%) admissions. A total of 252 NPO or CLD dietary orders were encountered in 142 admissions (75.9%). Of those, 112 orders were unjustified (44%). On average, patients with unjustified NPO or CLD orders spent 3 days on an NPO or CLD diet, which corresponded to a mean of 10 missed meals. Characteristics associated with unnecessary fasting

  7. 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

  8. 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.

  9. Implications of genetic variability of Trypanosoma cruzi for the pathogenesis of Chagas disease Implicações da variabilidade genética do Trypanosoma cruzi na patogênese da doença de Chagas

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    Fernanda da Silva Manoel-Caetano

    2007-10-01

    Full Text Available Trypanosoma cruzi, the etiological agent of Chagas disease, presents a high degree of intraspecific genetic variability, with possible implications for the clinical forms of the disease, like the development of cardiopathy, megaesophagus, and megacolon, alone or in combination. This tissue tropism involved in the pathogenesis of Chagas disease has still not been totally elucidated. Thus, the current review approaches key aspects of T. cruzi genetic diversity, the clinical forms of Chagas disease, and the infection of the host cell by the parasite and the immune response. Other aspects discussed here include the release of immunosuppressive factors by the parasite, acting in the host's immune response pathways; host cell apoptosis inhibition; the pathogenesis of chagasic megaesophagus, which can be related to host-parasite interaction; and finally the association between megaesophagus and increased risk for the development of squamous-cell esophageal carcinoma. However, despite great advances in the understanding of this disease, it is still not possible to establish the true relationship between the parasite's genetic variability and the clinical form of Chagas disease.O Trypanosoma cruzi, agente etiológico da doença de Chagas, apresenta elevado grau de variabilidade genética intra-específica, com possíveis implicações na forma clínica da doença, como o desenvolvimento de cardiopatia, do megaesôfago e do megacólon de forma isolada ou em associação. Este tropismo tecidual envolvido na patogênese da doença não está totalmente esclarecido. Assim, nesta revisão são abordados alguns aspectos referentes à diversidade genética dos parasitas isolados, às formas clínicas da doença de Chagas, ao processo de infecção do parasita na célula hospedeira e resposta imune. Outros aspectos também são enfocados, como os fatores imunossupressivos liberados pelo parasita que atuam na regulação das respostas imunes, a inibição da

  10. Trypanosoma cruzi Chagas, 1909: genetic variability of isolates from chronic chagasic patients in the Paraná state, Brazil

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    Rogério Luiz Kopp

    2005-05-01

    Full Text Available The present work had as objective to verify the genetic diversity among strains of Trypanosoma cruzi isolated in chronic chagasic patients in the Paraná state. Fifty patients with compatible clinical symptoms were selected (cardiopathy, megacolon and/or megaesophagus and that presented positive serological reaction to T. cruzi. Six strains of the protozoan were isolated in hemoculture and identified by electrophoresis in starch thick gel with aid of 14 isoenzymes (6PGD; G6PD; ME¹; ME²; ICD; PGM; GPI; GOT¹; GOT²; NP¹; NP²; DIA; MPI and FH. The statistical analysis were accomplished by the softwares NTSYs and UPGMA. Eighty-four electromorphs were individualized and four isoenzymes (ICD, GPI, 6PGD, e PGM showed compatibility for heterozygosis. The phenetic analysis evidenced the hypothesis of constant evolution (genetic distances = 0.0536 to 0.1429, Hardy-Weinberg = 1.0000 to 0.0769 and differentiation for exact tests = 1.0000 to 0.0658. A great intra-specific genetic diversity in T. cruzi was verified in the isolates obtained in humans and it indicates that the clonet II is associated to the domestic cycle of transmission.O presente trabalho teve como objetivo verificar a diversidade genética entre cepas de Trypanosoma cruzi circulantes em pacientes chagásicos crônicos no estado do Paraná. Foram selecionados 50 pacientes com clínica compatível (cardiopatia, megacolo e/ou megaesôfago e que apresentaram reação sorológica positiva para T. cruzi. Foram isoladas seis cepas do protozoário em hemocultura e identificadas por eletroforese em gel espesso de amido com auxílio de 14 isoenzimas (6PGD; G6PD; ME¹; ME²; ICD; PGM; GPI; GOT¹; GOT²; NP¹; NP²; DIA; MPI e FH. A análise estatística foi realizada pelos programas NTSYs e TFPGA. Oitenta e quatro eletromorfos foram individualizados sendo que quatro isoenzimas (ICD, GPI, 6PGD, e PGM apresentaram compatibilidade para heterozigose. A análise fenética evidenciou a hipótese de uma

  11. 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.

  12. 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

  13. Sindrome de currarino associada ao Tailgut Cyst: ressecção abdômino-sacral The Tailgut Cyst is na embryological remnant of the retrorectal (Presacral space that can be a part of the currarino syndrome

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    Cristiane Koizimi Martos Fernandes

    2007-12-01

    Full Text Available O Tailgut Cyst é um remanescente embriológico do intestino posterior, que pode fazer parte da Síndrome de Currarino. Relato do caso: Paciente feminino, 43 anos com lombalgia, retenção urinária e massa retrorretal. História pregressa de ânus ectópico, constipação associada a megacólon congênito. Exames de imagem evidenciando cisto retrorretal comprimindo o sacro, rechaçando as estruturas pélvicas anteriormente e deformidade sacral congênita. Realizada ressecção em bloco da lesão retrorretal, reto, útero e sacro ao nível de S2. Anátomo-patológico evidenciando tailgut cyst. Caracterizamos a paciente como portadora de síndrome de Currarino. Discusão: A síndrome de Currarino é caracterizada pela malformação anorretal, defeito sacral e massa retrorretal. Tem relação com a mutação do gene HLXB9. O tailgut cyst é um remanescente embriológico do intestino posterior, mais comum em mulher adulto jovem (75-90%. O tailgut cyst relacionado à Síndrome de Currarino é uma lesão incomum. A maioria dos pacientes é assintomática, mas podem apresentar sintomas anorretais, perineais e sacrais. Exames de imagem são úteis para a definição diagnóstica e programação cirúrgica. A excisão cirúrgica completa é recomendada devido ao risco de recorrência, degeneração maligna e infecção crônica, mesmo em pacientes assintomáticos. O prognóstico é incerto.The Tailgut Cyst is an embryological remnant of the hindgut that can be a part of the Currarino Syndrome. Report of a case: A 43-year-old woman with lombalgy, urine retain and presacral mass. Preceding history of ectopic anus, constipation associated with congenital megacolon. Imaging exams indicate retrorectal cyst that compresses the sacral region, receding the pelvic structure forward and congenital sacral deformity. A block resection of the retrorectal, rectal, uterus and sacrum lesion was carried out in a S2 level. Anatomo-pathologic indicate tailgut cyst. The

  14. 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

  15. Proctocolectomy and ileal J-pouch anal anastomosis on the surgical treatment of familial adenomatous polyposis and ulcerative colitis: analysis of 49 cases

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    Bruno Amaral Medeiros

    2012-09-01

    Full Text Available OBJECTIVE: To evaluate the results of ileal J-pouch anal anastomosis in ulcerative colitis and familial adenomatous polyposis. METHOD: Retrospective analysis of medical records of 49 patients submitted to ileal J-pouch anal anastomosis. RESULTS: Ulcerative colitis was diagnosed in 65% and familial adenomatous polyposis in 34%. Mean age was 39.5 years. 43% were male. Among familial adenomatous polyposis, 61% were diagnosed with colorectal cancer. Thirty-one percent of patients with ulcerative colitis was submitted to a previous surgical approach and 21% of these had toxic megacolon. Average hospital stay was 10 days. Post-operative complications occurred in 50% of patients with ulcerative colitis and 29.4% with familial adenomatous polyposis. Intestinal diversion was performed in 100% of ulcerative colitis and 88% of familial adenomatous polyposis. Pouchitis occurred in eight cases (seven ulcerative colitis and one FAP, requiring excision of the pouch in three ulcerative colitis. Mortality rate was 7.6%: two cases of carcinoma on the pouch and two post-operative complications. Late post-operative complications occurred in 22.4%: six familial adenomatous polyposis and five ulcerative colitis. Two patients had erectile dysfunction, and one retrograde ejaculation. One patient with severe perineal dermatitis was submitted to excision of the pouch. Incontinence occurred in four patients and two reported soil. Mean bowel movement was five times a day. CONCLUSION: Ileal J-pouch anal anastomosis is a safe surgery with acceptable morbidity and good functional results, if well indicated and performed in referral centers.OBJETIVO: Avaliar resultados da anastomose íleo-anal com bolsa ileal em J na colite ulcerativa e na polipose adenomatosa familiar. MÉTODO: Análise retrospectiva dos prontuários de 49 pacientes submetidos a anastomose íleo-anal com bolsa ileal em J. RESULTADOS: 65% de colite ulcerativa e 34% de polipose adenomatosa familiar. Idade m

  16. 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

  17. 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.

  18. ¿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

  19. 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

  20. 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

  1. MANAGEMENT OF ACUTE SEVERE ULCERATIVE COLITIS: A CLINICAL UPDATE.

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    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

  2. 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

  3. Resultados da cirurgia de reservatórios ileais em pacientes com doença de Crohn Long-term outcomes of ileal pouch after secondary diagnosis of Crohn's disease

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    Maria de Lourdes Setsuko Ayrizono

    2008-09-01

    intróito vaginal, corrigida com cirurgia de avanço mucoso. Houve também progressão da doença para o íleo proximal em três doentes. Nos outros três, a recidiva da doença foi no reservatório ileal, com boa resposta ao tratamento clínico. Atualmente, com seguimento médio de 76,5 meses, todos os doentes estão assintomáticos, sendo que quatro estão com derivação e sete com reservatórios funcionantes, estes apresentando 6-10 evacuações ao dia e continência praticamente normal. CONCLUSÃO: Recidiva da doença de Crohn após retocolectomia e reservatório ileal é relativamente freqüente, com ocorrência de complicações como abscessos, fístulas e estenoses. Entretanto, reservatórios ileais com doença de Crohn e mantidos, podem ser compatíveis com função satisfatória.BACKGROUND: Total rectocolectomy and ileal pouch-anal anastomosis is the choice surgical procedure for patients with ulcerative colitis. In cases of Crohn's disease post-operative diagnosis, it can be followed by pouch failure. AIM: To evaluate ileal pouch-anal anastomosis long-term outcome in patients with Crohn's disease. METHODS: Between February 1983 and March 2007, 151 patients were submitted to ileal pouch-anal anastomosis by Campinas State University Colorectal Unit, Campinas, SP, Brazil, 76 had pre-operative ulcerative colitis diagnosis and 11 had post-operative Crohn's disease diagnosis. Crohn's disease diagnosis was made by histopathological biopsies in nine cases, being one in surgical specimen, two cases in rectal stump, small bowel in two cases, ileal pouch in three and in perianal abscess in one of them. The median age was 30.6 years and eight (72.7% were female. RESULTS: All patients had previous ulcerative colitis diagnosis and in five cases emergency colectomy was done by toxic megacolon. The mean time until of Crohn's disease diagnosis was 30.6 (6-80 months after ileal pouch-anal anastomosis. Ileostomy closure was possible in 10 cases except in one that had ileal

  4. 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

  5. Pelvic lymphoscintigraphy: contribution to the preoperative staging of rectal cancer Linfocintilografia pélvica: contribuição ao estadiamento pré-operatório do câncer retal

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    José Hyppolito da Silva

    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 PROPÓSITO: A preservação do aparelho esfincteriano na cirurgia do câncer do reto distal tem sido objeto de preocupa