Due to the variability in the site of enteric duplications, a wide range of presenting symptoms can exist, which is challenging for diagnosis. In children with a diagnosis of acute abdomen, enteric duplication cysts should be considered, and these children should be further investigated for additional skeletal, urogenital, and gastrointestinal system pathologies. Surgical treatment depends on the site and type of the cyst.
BackgroundDisorders of sexual development (DSD) are congenital conditions in which the development of the chromosomal, gonadal or anatomical sex can be deemed atypical. The external genitalia should appear ‘normal’ in size and shape from birth, with no question of abnormality, and the individual must receive appropriate social-environmental feedback in the course of the sexual maturation process.MethodsWe review regional differences in the variables considered important for gender assignment in individuals with DSD. Various approaches to certain forms of DSD are analysed within their cultural context.ResultsThe decision to leave the sex of rearing undisturbed or to change it is difficult. It depends on the patient’s age and the extent to which the gender identity has been established with parental gender preference, social, cultural and religious factors. Severe forms of genetically female congenital adrenal hyperplasia, androgen insensitivity syndrome, 17β-hydroxysteroid dehydrogenase-3, 5α-reductase and cytochrome P450 oxidoreductase deficiencies are found to be the most difficult cases to diagnose and/or manage.ConclusionGender assignment in children with DSD is a subject of intense debate. Each case of DSD must be evaluated individually and on its merits and potentials. Although early admission and appropriate diagnostic facilities could provide the correct diagnosis, this is not the case in some cultures. It is seen that ‘gender panic’, social and religious concepts affect the decision-making process in gender assignment, especially in delayed cases.
OBJECTIVETo report experience of gender (re)assignment in genotypic female (46XX) patients with congenital adrenal hyperplasia (f‐CAH), a difficult and stressful experience if complicated with delayed presentation and inadvertent assignment.PATIENTS AND METHODSBetween 1983 and 2002, 70 patients with f‐CAH were counselled for gender assignment. The age at diagnosis and operation, the degree of virilization, parental consanguinity, the gender preference of the families, and the factors governing the decision‐making process were determined.RESULTSForty‐one (59%) patients presented after the neonatal period. All parents had already assumed or were advised of a gender for their children, based on the suggestive appearance of the external genitalia. Consequently, 49 patients were reared as female and 21 as ‘male’. Only nine of these ‘males’ could be reassigned as females (mean age at presentation 7.87 months, sd 10.42). Twelve children had to be reared as ‘male’ (mean age at presentation 55.8 months, sd 32.42) in compliance with the parents’ and the study group's decision, and appropriate masculinizing reconstructive surgery was undertaken. The difference in the mean age of those reassigned as female and those who remained ‘male’ was significant (P < 0.001). The parental consanguinity rate among the families was especially high in the ‘male’ patients.CONCLUSIONSIt is extremely difficult to correct the gender of patients with f‐CAH when they present at >2.5 years old. Furthermore, the delay in diagnosis and the male bias in choice of gender in our population might be a result of strong social pressures on families, influenced by cultural, traditional and economic factors.
Massive intestinal resection results in short-bowel syndrome (SBS) and is associated with an increased risk of infectious complications mainly caused by the egress of intestinal bacteria to distant organs, a process termed bacterial translocation (BT). The purpose of this experimental study in rats was to investigate in different models of SBS the impact of the type of intestinal resection on bacterial growth in the residual small bowel and on the occurrence of BT. SBS was created in 30 rats either by jejunal resection (JR), by ileal resection (IR) or by ileal resection including the ileocecal valve (IR+ICV). 10 animals underwent only a sham laparotomy (SL) and served as controls. Two weeks after the operative procedure, intestinal bacterial colonization and BT to the portal vein, vena cava, mesenteric lymph nodes, liver and spleen were determined. All resected animals showed a decreased weight gain and a significant bacterial overgrowth in the residual small bowel compared to the SL group. BT occurred after SL in 12%, after JR in 70%, after IR in 58%, and was significantly less frequent (35%) after IR+ICV, respectively. These experimental findings suggest that BT in SBS might be promoted by the intestinal bacterial overgrowth in the residual bowel, and the incidence of BT seems to be related to the presence or absence of the ileocecal valve.
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