Adulthood hirschprung's disease: a report of 4 cases in Ile-Ife. Nigeria.

Background: Hirschsprung's disease in adulthood is very rare and is often misdiagnosed. We present four cases of adulthood Hirschsprung's disease seen in the last two decades to illustrate challenges accompanying its diagnosis and management.

Method: This descriptive case series included cases of histologically proven Hirschsprung's seen in adulthood at the Obafemi Awolowo University Teaching Hospitals Complex in the last two decades (1991-2011).

MELAS syndrome presenting as an acute surgical abdomen.

MELAS (mitochondrial cytopathy, encephalomyopathy, lactic acidosis and stroke-like episodes) is a syndrome in which signs and symptoms of gastrointestinal disease are uncommon if not rare. We describe the case of a young woman who presented as an acute surgical emergency, diagnosed as toxic megacolon necessitating an emergency total colectomy. MELAS syndrome was suspected postoperatively owing to persistent lactic acidosis and neurological symptoms.

[The endotoxine aggression in the pathogenesis of postoperative complications in children with Hirsprung disease].

Signs of disseminated intravascular clotting (DIC), which were considered the laboratory sign of the subcompensated endotoxine aggression, were detected in 20 children with the Hirsprung disease, aged 3 months - 11 years. Under the influence of the operation stress the subcompensated DIC transformed to the acute decompensated reaction, which led to early complications, such as anastomosistis with tendency to the insufficiency or stenosis, enterocolitis, etc. The study revealed not only the straight connection between the postoperative complications and endotoxine aggression, but the necessity of preoperative preparation, directed on the decrease of the intestinal endotoxin level and normalization of antiendotoxin immunity.

Surgical Management of Clostridium difficile Colitis.

Clostridium difficile infection (CDI) will progress to fulminant disease in 3 to 5% of cases. With the emergence of hypervirulent, multidrug-resistant strains, the incidence and severity of disease are continuing to rise. Prompt identification, early resuscitation, and treatment are critical in preventing morbidity and mortality in this increasingly common condition.

Damage control surgery for abdominal emergencies.

Background: Damage control surgery is a management sequence initiated to reduce the risk of death in severely injured patients presenting with physiological derangement. Damage control principles have emerged as an approach in non-trauma abdominal emergencies in order to reduce mortality compared with primary definitive surgery.

Methods: A PubMed/MEDLINE literature review was conducted of data available over the past decade (up to August 2013) to gain information on current understanding of damage control surgery for abdominal surgical emergencies.

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

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.

Toxic Megacolon and Acute Ischemia of the Colon due to Sigmoid Stenosis Related to Diverticulitis.

We present a rare case of toxic megacolon accompanied by necrosis of the colon due to chronic dilation caused by stenosis of the sigmoid colon as a complication of diverticulitis. The patient presented at the emergency department with diffuse abdominal pain, fever (38.8°C) and tachycardia (120 beats/min).

Spinal anaesthesia in a patient with post-spine surgery dural ectasia.

Dural sac ectasia is a very infrequent anatomical abnormality, usually caused by connective tissue diseases, as Marfan syndrome. Very few cases have been described being a consequence of a previous spine surgical procedure. We describe the case of an elderly patient who should be operated on twice due to sub-occlusive colon disease.

Knockdown of human TCF4 affects multiple signaling pathways involved in cell survival, epithelial to mesenchymal transition and neuronal differentiation.

Haploinsufficiency of TCF4 causes Pitt-Hopkins syndrome (PTHS): a severe form of mental retardation with phenotypic similarities to Angelman, Mowat-Wilson and Rett syndromes. Genome-wide association studies have also found that common variants in TCF4 are associated with an increased risk of schizophrenia. Although TCF4 is transcription factor, little is known about TCF4-regulated processes in the brain.

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

Cytomegalovirus (CMV) infection causes significant morbidty and mortality in immunopromised patients. Though it is usually silent in immunocompetent adults, rarely it can cause serious life-threatening complications. Gastrointestinal tract is one of the commonly involved organs, where it produces a spectrum of clinical manifestation ranging from mild non-specific abdominal pain and diarrhoea to severe infection with toxic megacolon and death.

Ogilvie's syndrome following posterior spinal arthrodesis for scoliosis.

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.

Adult Hirschsprung's disease: report of four cases.

Adult Hirschsprung's disease (HD) is a rare motor disorder of the gut that is frequently misdiagnosed as refractory constipation. The primary pathogenic defect in adult HD is identical to that seen in infancy or childhood, and is characterized by the total absence of intramural ganglion cells of the submucosal (Meissner) and myenteric (Auerbach) neural plexuses in the affected segment of the bowel. Ninety-four percent of HD cases are diagnosed before the patient reaches 5 years of age, however, on rare occasion, mild cases of HD may go undiagnosed until he or she reaches adulthood.

An update on common gastrointestinal emergencies.

The evaluation of the child with acute abdominal pain often poses as a diagnostic challenge due to the wide range of diagnoses. Surgical emergencies need to be rapidly identified and managed appropriately to minimize morbidity and mortality. Presenting symptoms, clinical examination, and laboratory findings can guide selection of diagnostic imaging.

Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention.

Hirschsprung-associated enterocolitis (HAEC) is a common and sometimes life-threatening complication of Hirschsprung disease (HD). Presenting either before or after definitive surgery for HD, HAEC may manifest clinically as abdominal distension and explosive diarrhea, along with emesis, fever, lethargy, and even shock. The pathogenesis of HAEC, the subject of ongoing research, likely involves a complex interplay between a dysfunctional enteric nervous system, abnormal mucin production, insufficient immunoglobulin secretion, and unbalanced intestinal microflora.

Smad-interacting protein 1 affects acute and tonic, but not chronic pain.

Background: Smad-interacting protein 1 (also named Zeb2 and Zfhx1b) is a transcription factor that plays an important role in neuronal development and, when mutated, causes Mowat-Wilson syndrome (MWS). A corresponding mouse model carrying a heterozygous Zeb2 deletion was comprehensively analysed in the German Mouse Clinic. The most prominent phenotype was the reduced pain sensitivity.

Contemporary outcomes for ulcerative colitis inpatients admitted to pediatric hospitals in the United Kingdom.

Background: Pediatric ulcerative colitis (UC) care is variable with a lack of appropriate guidelines to guide practice until recently.

Methods: UC inpatients <17 years old admitted to 23 U.K.

Clinical features and management of pouchitis in Japanese ulcerative colitis patients.

Purpose: Pouchitis frequently occurs after restorative proctocolectomy for ulcerative colitis. This study evaluated the incidence and treatment of pouchitis in Japanese ulcerative colitis patients.

Method: This study reviewed the clinical data from 772 patients with pouch surgery between January 2000 and December 2010.

Ischemic Colitis.

Ischemic colitis occurs as a result of the decrease of arterial blood supply at the level of the colon and may have consequences which vary from completely reversible lesions to ischemic necrosis. It is a condition that affects both sexes, predominantly people over the age of 50, having multiple etiologies in which the main role is played by atherosclerosis. The symptomatology varies from mild symptoms, which are reversible, to severe ones, with a vital risk.

A novel long-range enhancer regulates postnatal expression of Zeb2: implications for Mowat-Wilson syndrome phenotypes.

The zinc-finger, E-box-binding homeobox-2 (Zeb2) gene encodes a SMAD-interacting transcription factor that has diverse roles in development and disease. Mutations at the hZeb2 locus cause Mowat-Wilson syndrome (MWS), a genetic disorder that is associated with mental retardation and other, case- and sex-dependent clinical features. Recent studies have detailed microRNA-mediated control of Zeb2, but little is known about the genomic context of this gene or of enhancer sequences that may direct its diverse functions.

Role of surgery in severe ulcerative colitis in the era of medical rescue therapy.

Despite the growing use of medical salvage therapy, colectomy has remained a cornerstone in managing acute severe ulcerative colitis (ASC) both in children and in adults. Colectomy should be regarded as a life saving procedure in ASC, and must be seriously considered in any steroid-refractory patient. However, colectomy is not a cure for the disease but rather the substitution of a large problem with smaller problems, including fecal incontinence, pouchitis, irritable pouch syndrome, cuffitis, anastomotic ulcer and stenosis, missed or de-novo Crohn's disease and, in young females, reduced fecundity.

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

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.