Genetic background-dependent abnormalities of the enteric nervous system and intestinal function in Kif26a-deficient mice.

The Kif26a protein-coding gene has been identified as a negative regulator of the GDNF-Ret signaling pathway in enteric neurons. The aim of this study was to investigate the influence of genetic background on the phenotype of Kif26a-deficient (KO, -/-) mice. KO mice with both C57BL/6 and BALB/c genetic backgrounds were established. Survival rates and megacolon development were compared between these two strains of KO mice. Functional bowel assessments and enteric neuron histopathology were performed in the deficient mice. KO mice with the BALB/c genetic background survived more than 400 days without evidence of megacolon, while all C57BL/6 KO mice developed megacolon and died within 30 days. Local enteric neuron hyperplasia in the colon and functional bowel abnormalities were observed in BALB/c KO mice. These results indicated that megacolon and enteric neuron hyperplasia in KO mice are influenced by the genetic background. BALB/c KO mice may represent a viable model for functional gastrointestinal diseases such as chronic constipation, facilitating studies on the underlying mechanisms and providing a foundation for the development of treatments.

[Differential indications for ileoanal pouch anastomosis : Ulcerative colitis, familial adenomatous polyposis, synchronous colorectal cancer - Crohn's disease, constipation]. / Differenzialindikation zur ileoanalen Pouchanlage : Colitis ulcerosa, FAP, multilokuläres kolorektales Karzinom ­ M. Crohn, Obstipation.

Ileoanal pouch anastomosis is the procedure of choice for patients with drug refractory ulcerative colitis, indeterminate colitis and familial adenomatous polyposis (FAP). In selected patient groups this procedure is a treatment option for patients with Crohn's disease, hereditary nonpolyposis colorectal cancer (HNPCC), synchronous colorectal cancer and for severe colorectal constipation refractory to conservative drug treatment. The pouch procedure provides the opportunity to avoid a permanent ileostomy. The majority of surgeons prefer the ileal J­pouch as the construction is the easiest to perform and complications and dysfunction rates are low. Due to functional reasons most pouch surgeons favor a circular stapled ileoanal pouch anastomosis. The more radical proctocolectomy can produce sensory defects in the anal canal with subsequent soiling and incontinence. Studies have shown that even after proctocolectomy residual rectal mucosa was found in the anal canal. Therefore, the functionally important anorectal transitional zone should be preserved if possible. Ulcerative colitis can be "healed" with proctocolectomy; however, pouchitis can still occur in one third of the patients. Patients must be informed about the risk of pouchitis and a multidisciplinary monitoring and treatment strategy must be available. In Crohn's disease the ileoanal pouch survival rate of 80% in the long-term follow-up is surprisingly good despite an increased postoperative complication rate. The anal pouch anastomosis is the standard operation in patients with drug refractory ulcerative colitis, indeterminate colitis and FAP. Synchronous colorectal cancer, HNPCC and severe therapy refractive constipation represent rare indications for proctocolectomy where decisions must be made on an individual basis.

Comparison of biofragmentable anastomosis ring and sutured anastomoses for subtotal colectomy in cats with idiopathic megacolon.

OBJECTIVE: To report use of a biofragmentable anastomosis ring (BAR) device in cats with idiopathic megacolon (FIM) and compare outcome after subtotal colectomy with sutured colocolic anastomosis. STUDY DESIGN: Retrospective study. ANIMALS: Nineteen cats with megacolon. METHODS: Medical records (January 1990-January 2004) of cats treated surgically for idiopathic megacolon with sutured (SUT) or BAR anastomosis were retrieved and reviewed. Operative, short- and long-term complications, and survival times were recorded and Kaplan-Meier survival analysis used to assess outcome. RESULTS: There were 11 SUT and 8 BAR cats. One BAR cat had anastomotic dehiscence 36 hours after surgery. Mild serosal tearing during BAR insertion in 6 cats was corrected by suture reinforcement. One SUT cat developed anastomotic stricture at 32 days. Short-term complication rates at 3 and 7 days were 18% and 45% in the SUT group and 25% and 87.5% in the BAR group, respectively (P=.058). Two SUT cats had persistent loose stool consistency and were euthanatized 254 and 1661 days after surgery. One BAR cat had recurrence of constipation which was managed medically. Long-term complication rates were not significantly different between SUT and BAR (P=.61). The 1 and 4-year survival rates were 90% for SUT and 100% for BAR (P=.29). CONCLUSIONS: No difference was detected for short and long-term complication rates and survival times between SUT and BAR groups. CLINICAL RELEVANCE: The BAR device can be used for colocolic anastomosis in cats with idiopathic megacolon. Serosal tearing during BAR insertion was a common intraoperative complication. Regardless of anastomotic technique, survival outcome after colonic resection is excellent for cats with FIM.

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

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 megacolon.

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

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 por cento) eram mulheres. Megaesôfago e/ou megacólon chagásicos estavam presentes em 135 pacientes, dos quais, 59 por cento apresentavam cardiopatia. Para 49 por cento 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.

Operación de Duhamel con doble sutura mecánica: técnica y resultados iniciales / Modified Duhamel operation with double stapler

Antecedentes: La operación de Duhamel es la que mejor responde a la fisiopatología del megacolon modificando la incoordinación motora. Objetivo: Evitar un segundo tiempo quirúrgico, disminuir el tiempo de internación, brindar mayor confort postoperatorio, evitar la colostomía perineal y sus complicaciones. Diseño: Prospectivo, comparativo. Población: Pacientes con megacolon, excluído el megacolon total. La cirugía se indicó por fecalomas a repetición y constipación rebelde al tratamiento. Método: Grupo I: Duhamel con doble sutura mecánica, 14 casos. Resultados: No hubo mortalidad. Morbilidad grupo I: 2 casos íleo prolongado (14.2 por ciento); grupo II: 1 caso (8.33 por ciento) retención urinaria y 3 casos (25 por ciento) estenosis de la anastomosis colorrectal. No hay diferencias significativas. Promedio de internación grupo I: 7 días (6-10), grupo II: 12.6 días (11-8), diferencia altamente significativa (p<0.0001). Conclusiones: La técnica de Duhamel es la preferida para solucionar el megacolon, la modificación con doble sutura mecánica mejora dichos resultados, no presenta segundo tiempo quirúrgico, disminuye el tiempo de internación, evita la colostomía perineal, sus complicaciones y su disconfort, disminuye la morbilidad, no presenta estenosis de la anastomosis.

Operación de Duhamel con doble sutura mecánica: técnica y resultados iniciales / Modified Duhamel operation with double stapler

Antecedentes: La operación de Duhamel es la que mejor responde a la fisiopatología del megacolon modificando la incoordinación motora. Objetivo: Evitar un segundo tiempo quirúrgico, disminuir el tiempo de internación, brindar mayor confort postoperatorio, evitar la colostomía perineal y sus complicaciones. Diseño: Prospectivo, comparativo. Población: Pacientes con megacolon, excluído el megacolon total. La cirugía se indicó por fecalomas a repetición y constipación rebelde al tratamiento. Método: Grupo I: Duhamel con doble sutura mecánica, 14 casos. Resultados: No hubo mortalidad. Morbilidad grupo I: 2 casos íleo prolongado (14.2 por ciento); grupo II: 1 caso (8.33 por ciento) retención urinaria y 3 casos (25 por ciento) estenosis de la anastomosis colorrectal. No hay diferencias significativas. Promedio de internación grupo I: 7 días (6-10), grupo II: 12.6 días (11-8), diferencia altamente significativa (p<0.0001). Conclusiones: La técnica de Duhamel es la preferida para solucionar el megacolon, la modificación con doble sutura mecánica mejora dichos resultados, no presenta segundo tiempo quirúrgico, disminuye el tiempo de internación, evita la colostomía perineal, sus complicaciones y su disconfort, disminuye la morbilidad, no presenta estenosis de la anastomosis. (AU)

[Basic causes of death in elderly patients with Chagas' disease]. / Causas básicas de morte em chagásicos idosos.

The main causes of death of 100 elderly Chagas' patients (group A), of 100 elderly non-Chagas' disease (group B) and of 100 non-elderly Chagas' disease patients (group C) were surveyed clinically and by postmortem data. Compared to B, the A group showed significantly more deaths due to chronic Chagas' heart disease and digestive "megas", and less frequently to malignant neoplasm. Deaths due to pulmonary emphysema and malignant neoplasms were significantly more common and deaths due to chronic Chagas' heart disease were significantly less common in the A group when compared to the C group. Chronic Chagas' heart disease caused 26 unexpected sudden deaths in the C group (the younger patients) and none in the A group (the eldest patients).

Causas básicas de morte em chagásicos idosos / Basic causes of death in elderly chagasic patients

Determinaram-se, ocm bases em dados clínicos e necroscópicos, as causas básicas de morte de 100 chagásicos idosos (grupo A), 100 idosos näo chagásicos (grupo B) e 100 chagásicos näo idosos (grupo C). Em relaçäo a B, o grupo A teve um número significativamente maior de mortes por cardiopatica hagásica crôn e "mega" digestivos e menor de neoplasias malignas. Quando comparado a C, o grupo A teve, em grua significativo, mortalidade maior por enfisema pulmonar e neoplasias malignas e menor por cardiopatia chagásica crônica. Esta causou 26 óbitos súbitos inesperados entre os näo idosos, mas nenhum entre os idosos

Megacolon primario adquirido del adulto: tratamiento quirúrgico / Surgical treatment of the adquired adult primary megacolon

Los autores presentan 56 pacientes operados por megacolon entre 1955 y 1985. La anatomía patológica y la epiopatogenia de la afección, es detallada. Las técnicas quirúrgicas empleadas son mencionadas y la operación de Black es seleccionada como la mejor. Esta técnica quirúrgica es descripta minuciosamente. Las complicaciones postoperatorias y la mortalidad son mencionadas.

Megacolon primario adquirido del adulto: tratamiento quirúrgico / Surgical treatment of the adquired adult primary megacolon

Los autores presentan 56 pacientes operados por megacolon entre 1955 y 1985. La anatomía patológica y la epiopatogenia de la afección, es detallada. Las técnicas quirúrgicas empleadas son mencionadas y la operación de Black es seleccionada como la mejor. Esta técnica quirúrgica es descripta minuciosamente. Las complicaciones postoperatorias y la mortalidad son mencionadas.(AU)

Hirschprung's disease: long-term follow-up of 65 cases.

The author reviews 65 cases of Hirschsprung's disease seen and treated in Saskatoon between 1951 and 1981. The annual incidence of this disease is 1/4000 live births. Overall mortality was 25% and related to two factors: (a) delay in diagnosis and surgical treatment with a high incidence of enterocolitis in the earlier years of the series; (b) a mortality of 83% in patients with small bowel aganglionosis. Thirty-five resections were done for colonic aganglionosis; there was one death that occurred 20 years ago, indicating that surgery is safe in this disease, but delay in treatment is not. Of six patients with small bowel aganglionosis, only three had resection and only one of these survived. The two patients who died had very high small bowel aganglionosis, probably incompatible with survival. The Rehbein type of procedure proved useful in high colonic aganglionosis where, by necessity, anastomosis was done at up to 7 cm above the white line of the pelvis, with excellent end results. There was a 35% incidence of Hirschsprung's disease in females; 37% had high aganglionosis (above the sigmoid). Low (white line) anastomosis was done in three patients available for 12-year follow-up. All have some degree of incontinence.

Modified endorectal procedure for management of long-segment aganglionosis.

During the past five years, 65 patients with Hirschsprung's disease have been treated at our institution. Ten of these patients had long-segment aganglionosis, five with total colonic involvement, four with aganglionosis to the midtransverse colon, and one child with Hirschsprung's disease extending to the distal jejunum. There are six females and four males in the series, which is a reversal of the normal sex ratio for aganglionosis. An extensive literature review of long-segment Hirschsprung's disease has revealed a total of 489 reported cases. The mean frequency of long-segment aganglionosis in the literature is 12% of all cases of aganglionosis. Our series is comparable, with an incidence of 15%. The overall mortality rate in the literature of 54% indicates the poor therapeutic results obtained with this disease in the past. All ten of our patients have been definitively treated with a modified endorectal pull-through procedure. The mortality rate with this procedure has been zero. There has been one major complication (incorrect pathologic diagnosis of the level of aganglionosis) and four minor complications. The average follow-up period is 42.6 months. The functional results have been excellent with an average of three to four soft bowel movements per day. All of the children over 3 years of age are completely toilet trained. The remaining two patients are too young to be toilet trained, but are continent in the sense that they do not soil between bowel movements. Awareness of this entity of long-segment aganglionosis should lead to earlier diagnosis, appropriate operative therapy, and lower mortality rates.

[Late postoperative complications in Hirschsprung's disease (author's transl)]. / Spätkomplikationen in der Megakolonchirurgie.

In more than 5,500 patients operated upon for Hirschsprung's disease which were obtained from the literature, the late complications following the 4 principal operative procedures of Swenson, Duhamel, Rehbein and Soave were compared. After Duhamel's operation, there was a lower incidence of postoperative constipation: 6.7%, compared with an average rate of 9.3%. Rehbein's resection showed the best results in relation to incontinence with a complication rate of 2.1% while the average was 9.8%, also in enterocolitis and diarrhoea (2.4%, average 7.8%) and enuresis (5.1% compared with 9.7%). We found the lowest incidence of late ileus (2.2% to 4.4%) and mortality with the Rehbein method. Judging by the frequency of late complications, the technique of Rehbein's anterior resection seems to be the most commendable.

The Turnbull-Cutait pullthrough procedure for certain cancers of the rectum and Hirschsprung disease.

The technique of the Turnbull-Cutait pullthrough procedure as performed at the Cleveland Clinic for carcinoma of the rectum and other conditions is described. The results in 127 patients are reported. Eighty-four patients were operated upon for cancer of the rectum and 47 for miscellaneous benign conditions. The average distance of tumors from the anal margin was 7.6 cm. The average margin of resection was 4.1 cm. The overall operative mortality was 1.2% in the cancer group. Ischemic necrosis occurred in 1.2% of the cancer patients, and minor pelvic sepsis occurred in 7.1%. The five-year survival in Dukes' A, B and C carcinoma of the rectum was 100%, 57% and 53%, respectively. The incidence of pelvic recurrence of the tumor at 6% was within acceptable limits. The quality of bowel function following the pullthrough procedure is discussed. It is concluded that this pullthrough procedure has a significant role in the management of carcinoma of the rectum and other conditions.

Congenital defects of the gastrointestinal tract and abdominal wall. A three-year review.

One hundred forty-four infants with anomalies of the gastrointestinal tract and abdominal wall were treated at Children's Memorial Hospital between July 1970 and July 1973. The overall mortality, including unoperated infants and those dying from other causes up to six months later, was 17%. Associated defects, particularly of the heart and CNS, were the most common causes for death. Many infants were premature; however, this factor alone did not contribute to mortality. Differences in surgical technique did not affect mortality in patients with esophageal atresia, but one baby with total aganglionosis of his colon and two with gastroschisis may have survived with different modes of therapy. Although previous advances in pediatric surgery contributed to the overall good results in these infants, parenteral alimentation emerged as the single most important factor in improved survival during this study period.