1/22. Biofeedback avoids surgery in patients with slow-transit constipation: report of four cases.Biofeedback is established treatment for intractable constipation in patients with an element of pelvic floor dysfunction. In those with intractable slow-transit constipation and normal pelvic floor function, colectomy is usually recommended. We report four patients with isolated slow-transit constipation who benefited from biofeedback and avoided surgery. All four patients were extensively investigated for pelvic floor dysfunction before undergoing a standard biofeedback course of four outpatient sessions. All improved in terms of bowel frequency, laxative use, bloating, straining, and lifestyle. Improvement has been maintained for a median of nine (range, 5-12) months without the requirement for further treatment. Biofeedback represents a safe and inexpensive treatment for these patients and may avoid surgery in a significant proportion.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/22. Non-hepatic hyperammonaemia: an important, potentially reversible cause of encephalopathy.The clinical syndrome of encephalopathy is most often encountered in the context of decompensated liver disease and the diagnosis is usually clear cut. Non-hepatic causes of encephalopathy are rarer and tend to present to a wide range of medical specialties with variable and episodic symptoms. Delay can result in the development of potentially life threatening complications, such as seizures and coma. Early recognition is vital. A history of similar episodes or clinical risk factors and early assessment of blood ammonia levels help establish the diagnosis. In addition to adequate supportive care, investigation of the underlying cause of the hyperammonaemia is essential and its reversal, where possible, will often result in complete recovery. Detection of an unborn error of metabolism should lead to the initiation of appropriate maintenance therapy and genetic counselling.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/22. Disseminated intestinal hypoganglionosis treated by colectomy and tapering of the small intestine. A case report.A girl suffering from chronic constipation and abdominal distension from her first year of life underwent internal anal sphincter myectomies at 5 and 7 years of age without resolution of her symptoms. At the age of 8, an ileostomy was performed because of excessive colonic dilation and hypomotility. Biopsies from the colon and distal ileum showed intestinal neuronal dysplasia TYPE B (INDB) with hypoganglionic areas. colectomy and ileorectal anastomosis were done at the age of 10. Three years later, however, an ileostomy was re-established because of recurrent episodes of pseudo-obstruction. In the hope of improving intestinal motility, the dilated small intestine was tapered over its entire length of 3.6 meters. Histological findings still demonstrated oligoneuronal hypoganglionosis and INDB all along the resected strip of bowel wall. After 6 months, the stoma was closed. At the age of 15 years, tapering of the distal 80 cm of the ileum was repeated in combination with cholecystectomy for cholecystolithiasis. Intestinal transit time decreased from 55 hours before the first to 18 hours after the second tapering procedure. Now, 7 years after the last operation, the patient passes 3 - 4 soft stools daily, is physically active, on a normal diet and not on any regular medication.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/22. Radiographic, computed tomographic and histopathologic appearance of a presumed spinal chordoma in a dog.A 4-year-old Labrador Retriever presented for urinary incontinence and constipation of 2 weeks duration. There was a tender abdomen, lumbar pain and conscious proprioceptive deficits in both pelvic limbs. Depressed pelvic limb reflexes were present consistent with a lower motor neuron lesion. In radiographs of the lumbar spine there was narrowing of the intervertebral disc space at L5-L6 with irregular, multifocal areas of mineralized opacities dorsal to the intervertebral disc space, presumably within the vertebral canal. On computed tomography, an intramedullary, partially mineralized mass was identified in the spinal cord at the level of caudal L5 through cranial L6. At necropsy there was a four-centimeter enlarged, irregular segment of spinal cord at the level of L5-L6. When sectioned, the spinal cord bad a mineralized texture. Histologically there were variable sized cells that were stellate in appearance with vacuolated cytoplasm (physaliferous cells) and mucinous background consistent with a chordoma. chordoma is a rare, skeletal neoplasm that originates from mesoderm-derived notochord and has been reported in humans and animals. Extraskeletal development of a chordoma within the spinal cord is a rare manifestation of this neoplasm. However, based on other reports in dogs, solitary extraskeletal locations of chordomas may be the typical expression of this neoplasm in the dog. Differentiation of similar histologically appearing tumors, such as a parachordoma or myxoid chondrosarcoma, will require immunohistochemical characterization of these tumors in veterinary patients.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/22. Teenage constipation: a case study.constipation is a problem of significant magnitude. It can have a devastating impact on a patient's personal life. There are many causes of constipation. Among them are dietary factors such as decreased fiber and low fluid intake, decreased activity, lack of privacy for defecation, pharmacologic agents, physiologic problems such as bowel obstruction or metabolic disorders, and psychosocial distress. A young teenage boy is followed through a series of emergency room visits, office visits, and a hospitalization related to his experiences with constipation. A bowel program was identified and instituted with successful outcomes.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/22. Gastric trichobezoar: an important cause of abdominal pain presenting to the pediatric emergency department.abdominal pain is a common presenting complaint of children seen in urgent care settings. It is the manifestation of a wide variety of disease processes ranging from benign to immediately life-threatening. Gastric bezoars are among the etiologies of chronic childhood abdominal pain that, when undiagnosed, may result acutely in serious complications, including gastric ulceration, bleeding and perforation, intussusception, and small bowel obstruction. To reinforce the importance of including this entity in the differential diagnosis of abdominal pain, we present the case of a 10-year-old girl with a history of chronic epigastric complaints who was ultimately presented with acute small bowel obstruction following fragmentation and distal migration of her gastric trichobezoar. Finally, we review and briefly summarize the current literature regarding the etiology, diagnosis, and management of this disorder in children.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/22. methemoglobinemia caused by the ingestion of courgette soup given in order to resolve constipation in two formula-fed infants.methemoglobinemia is not a rare condition arising from the exposure to hemoglobin-oxidizing agents such as nitrates-nitrites present in well water or vegetables. Infants < 3 months of age are more susceptible than adults because of lower amounts of a key enzyme, NADH-cytochrome b5 reductase, which converts methemoglobin back to hemoglobin. We report 2 infants, aged respectively 2 and 1 months, suffering from methemoglobinemia, fed with a formula that was reconstituted with a high concentration of courgette soup to resolve constipation. They developed a severe cyanosis with methemoglobinemia (respectively 30.4 and 27%) and were hospitalized and treated with methylene blue at 1%. After 12 h the syndrome was completely resolved. Home-prepared infant foods containing vegetables are potential causes of methemoglobinemia. It is important not to feed infants with vegetables having a high nitrate content (e.g., courgette, spinach, beets and green beans) to resolve constipation since, particularly in the first months of life, they may cause severe methemoglobinemia.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/22. Permanent neonatal diabetes with arthrogryposis multiplex congenita and neurogenic bladder - a new syndrome?Neonatal diabetes mellitus is a rare (1/400 000 newborns) but potentially devastating condition, which may be transient or permanent; typical symptoms occur within the first 4 wk of life. The transient form is a developmental insulin production disorder that resolves postnatally. Fifty to 60% of cases can be seen as transient form. Cases that require lifelong insulin therapy can be described as permanent condition. This fraction of cases is less common than the transient form. There are no clinical features that can predict whether a neonate with diabetes mellitus but no other dysmorphology will eventually have permanent neonatal diabetes mellitus (PNDM) or transient neonatal diabetes mellitus. Some metabolic or genetic defects such as complete deficiency of glucokinase or heterozygous activating mutations of KCNJ11, encoding Kir6.2, were found in patients with PNDM. A preterm female infant with a gestational age of 36 wk was admitted to the neonatal intensive care unit in the first hours of life due to prematurity and intra-uterine growth retardation. She was diagnosed as having arthrogryposis multiplex congenita on the first day. hyperglycemia was detected on the third day of life, and she required insulin treatment. The patient is now 6 yr old with PNDM, arthrogryposis multiplex, neurogenic bladder, immune deficiency, constipation, and ichthyosis. Is this a new form of neonatal diabetes mellitus?- - - - - - - - - - ranking = 4keywords = life (Clic here for more details about this article) |
9/22. multiple endocrine neoplasia type II B with symptoms suggesting Hirschsprung's disease: a case report.A 3-year-old child was referred with a tentative diagnosis of Hirschsprung's disease because of life-long constipation and "megacolon" demonstrated radiographically. Our rectal biopsy revealed hyperganglionosis suggestive of multiple endocrine neoplasia (MEN) type II B. This, in addition to an elevated serum calcitonin level, prompted surgical removal of her thyroid, which appeared grossly normal but on sectioning, contained a medullary carcinoma in each lobe. She remains disease-free 5 years later. Gastrointestinal symptoms are a significant component of the MEN type II B syndrome, and often antedate the full phenotypic expression of the syndrome and the development of potentially lethal endocrine neoplasms. On the basis of this experience, it is recommended that MEN II B be included in the differential diagnosis of chronic constipation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/22. Hereditary internal anal sphincter myopathy causing proctalgia fugax and constipation. A newly identified condition.A newly identified myopathy of the internal anal sphincter is described. In the affected family, at least one member from each of five generations had severe proctalgia fugax; onset was usually in the third to fifth decades of life. Three members of the family have been studied in detail. Each had severe pain intermittently during the day and hourly during the night. constipation was an associated symptom, in particular difficulty with rectal evacuation. Clinically the internal anal sphincter was thickened and of decreased compliance. The maximum anal canal pressure was usually increased with marked ultraslow wave activity. Anal endosonography confirmed a grossly thickened internal anal sphincter. Two patients were treated by internal anal sphincter strip myectomy; one showed marked improvement and one was relieved of the constipation but had only slight improvement of the pain. The hypertrophied muscle in two of the patients showed unique myopathic changes, consisting of vacuolar changes with periodic acid-Schiff-positive polyglycosan bodies in the smooth muscle fibers and increased endomysial fibrosis. in vitro organ-bath studies showed insensitivity of the muscle to noradrenaline, isoprenaline, carbachol, dimethylpiperazinium, and electrical-field stimulation. Immunohistochemical studies for substance p, calcitonin gene-related peptide, galanin, neuropeptide y, and vasoactive intestinal peptide showed staining in a similar distribution to that in control tissue. A specific autosomal-dominant inherited myopathy of the internal anal sphincter that causes anal pain and constipation has been identified and characterized.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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