Small bowel intussusception and brown bowel syndrome in association with severe malnutrition.

Brown bowel syndrome is a rare condition characterized by deposition of lipofuscin in the smooth muscle cells of the gastrointestinal tract. The number of reported cases is small, but all are associated with malabsorptive states. Despite these small numbers, there is considerable evidence that vitamin E deficiency is important etiologically. We report here the case of a severely malnourished [body mass index 11.7 kg/m (2): normal range 20-25 kg/m (2)] 31-yr-old black male with a longstanding history of alcohol abuse, who was on anti-tuberculosis therapy. The patient presented with an acute abdomen and was found, at operation, to have a mid-ileal intussusception. Histological examination of the resected specimen demonstrated lipofuscin accumulation consistent with brown bowel syndrome, but no tumor. Subsequent investigations revealed no significant quantities of vitamin E in the blood and pancreatic steatorrhea. However, deficiency of other fat-soluble (vitamin A and D) and water-soluble vitamins (vitamin C and thiamine) also were detected. This report supports the association of brown bowel syndrome with vitamin E deficiency but cannot exclude the compounding effects of protein calorie malnutrition, multiple vitamin deficiencies, and chronic alcohol toxicity.

Hydatid cyst in the head of the pancreas with obstructive jaundice.

We report a rare case of obstructive jaundice caused by an intrapancreatic hydatid cyst in a 17-year-old black girl. Ultrasonography and computed tomography demonstrated the obstructing cyst in the head of the pancreas. Cyst aspiration produced clear fluid with a low amylase content and no hydatid hooklets or protoscolices. Pancreaticoduodenectomy was performed for a presumed cystic neoplasm of the pancreas, but histology showed the true diagnosis. Pancreatic hydatidosis should be considered in the differential diagnosis of obstructing pancreatic cysts in the appropriate epidemiological setting.

Case management to enhance HIV risk reduction among users of injection drugs and crack cocaine.

Case management is being applied to increasingly diverse client populations. This article describes the application of a field-based, service-broker model of case management with a population of injection drug users and crack cocaine users within the context of an AIDS risk-reduction program. The major problem encountered involved client engagement. The feasibility of implementing an effective case management program with active users of injection drugs and crack is questionable so long as involvement with such drugs is cast primarily as a criminal justice problem.

Dermal and intravascular fasciitis. Unusual variants of nodular fasciitis.

Three cases with characteristic features of nodular (pseudosarcomatous) fasciitis arising in the dermis are described. This is only the second report documenting a dermal location for this condition. One of the three cases also showed features of intravascular fasciitis. A fourth case of intravascular fasciitis arising in periocular tissue is included. The differential diagnosis of dermal fasciitis includes benign fibrous histiocytoma, pyogenic granuloma, peripheral nerve tumors, cutaneous smooth muscle tumours, spindle cell carcinoma, spindle cell melanoma, dermatofibrosarcoma protuberans and atypical fibroxanthoma.

Abdominal tuberculosis: still a potentially lethal disease.

The findings in a 4-yr survey of 82 patients with abdominal tuberculosis are described and compared with those encountered in previous surveys. Fourteen cases of intestinal, 11 of mesenteric-lymphnodal, and 57 of peritoneal tuberculosis were identified. The disease occurred essentially in patients living under worsening socioeconomic conditions, and 51 of them had associated pulmonary tuberculosis. Symptoms and clinical findings were again nonspecific, but newer imaging, endoscopic, and other invasive procedures were helpful in establishing a definite diagnosis. In addition, adenosine deaminase determination showed great promise as a noninvasive diagnostic procedure in patients with tuberculous ascites. The six hospital deaths in the series highlight the hazard of potentially lethal delays in early diagnosis and treatment, even in centers with a high awareness of the disease.

Collagenous colitis--a rare cause of chronic watery diarrhoea. A case report.

Collagenous colitis is a clinicopathological syndrome occurring predominantly in middle-aged females presenting with chronic watery diarrhoea. Histologically the disorder is characterised by a mononuclear cell mucosal inflammatory infiltrate and subepithelial collagen thickening. The condition was first described in 1976 and since then approximately 100 cases have been reported in the literature. We present here the first reported case of this rare disorder in South Africa.

Lymphocytic gastritis in nonulcer dyspepsia.

The prevalence of lymphocytic gastritis, a specific form of chronic gastritis characterized by infiltration of gastric superficial epithelium with T lymphocytes, has been established in nonulcer dyspepsia. Among a population sample of 586 patients at risk for gastric carcinoma, 0.83% of patients with nonulcer dyspepsia and 1.63% of patients with chronic active gastritis showed lymphocytic gastritis. Among routine gastric biopsies from 5130 patients, only five cases met histological and immunohistochemical criteria of lymphocytic gastritis.

Complete appendicular inversion: the "inside-out" appendix. An unusual presentation of Crohn's disease. A case report and review of the literature.

Intussusception of the appendix into the caecum is an uncommon condition and the diagnosis is rarely made preoperatively. Specific anatomic requirements as well as pathological conditions that cause irritation or increased peristalsis should be present for this event to occur. A case is discussed in which the diagnosis was made preoperatively by barium enema and in which the lead point was a large inflammatory polyp due to Crohn's disease. It appeared in this case that inflation at the time of the barium enema resulted in reduction of the intussusception.

Primary oesophageal rhabdomyosarcoma.

A case is presented of a rhabdomyosarcoma of the oesophagus with a description of the cytology, light microscopy, and immunocytochemical findings and a discussion of spindle cell tumours occurring at this site. Cytologically, large bizarre shaped pleomorphic cells were seen in which desmin was demonstrated in order to confirm the diagnosis after destaining a Papanicolaou stained slide and restaining it with antibody to desmin.

Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease.

The pathology of 13 cases of immunoproliferative small intestinal disease (IPSID) associated with alpha-heavy-chain disease--one an apparent non-secretor and another with localized infiltration--is described. Four cases exhibited immunohistological light-chain monotypia. In one of these, evolution of a light-chain negative cell population was observed over a 7-year period. In the intestine, centrocyte-like cells produced lympho-epithelial lesions in 11 cases and enlarged lymphoid follicles in three. In lymph nodes, perifollicular infiltration was observed in 11 cases and abnormal follicles in six. Of three patients with high-grade lymphoma at presentation, one died untreated at 2 months, and two are alive at 34 and 91 months. Of 10 patients with low-grade disease at presentation, two died--one at 76 months, the other after transforming to high-grade lymphoma at 73 months. Eight patients with low-grade disease are alive, an average of 67 months after presentation. Four of five conservatively treated low-grade cases (including three in remission) showed evidence of monoclonality at presentation (light-chain monotypia in two and gene rearrangement in two), while two of the five exhibited DNA aneuploidy. It is concluded that IPSID with alpha-heavy-chain disease is neoplastic in all its stages and is a variant of mucosa-associated lymphoma. The role of centrocyte-like cells and the response to conservative therapy are discussed.

Common variable hypogammaglobulinemia. A case report.

We describe a patient with severe common variable hypogammaglobulinemia (CVH) whose clinical course was dominated by resistant giardiasis requiring prolonged hospitalization. The giardiasis was eventually controlled by initial metronidazole and subsequent mepacrine therapy, but side effects necessitated the withdrawal of both of these drugs. Replacement immunoglobulin treatment failed to restore normal serum immunoglobulin levels, but despite this, they appeared to be of value in reducing the liability to recurrent giardiasis. We discuss the use of immunoglobulin supplementation in this condition and the frequent association between CVH and pentagastrinfast achlorhydria.

Hereditary non-polyposis colorectal cancer in a Namaqualand kindred.

A family with hereditary non-polyposis colonic cancer affecting 16 males over three generations is described. Autosomal dominant inheritance with male predominance is demonstrated. The clinical features of this condition and methods for screening family members are discussed.

Immunoproliferative small-intestinal disease. An immunohistochemical study.

We performed a detailed histological and immunohistological study on both fresh-frozen and paraffin-embedded tissue from full-thickness jejunal biopsy specimens taken from three patients with immunoproliferative small-intestinal disease (IPSID). In all three patients, the mucosal infiltrate consisted of "centrocyte-like" (CCL) cells forming lymphoepithelial lesions and plasma cells. In one patient, the mucosal infiltrate was strikingly follicular. Immunohistochemistry showed alpha 1 heavy chain, but no light chain, in the perinuclear space and cytoplasm of the CCL cells and in the plasma cells. In two patients, the plasma cells (but not the CCL cells) also contained alpha 2 heavy chain. In the case showing a follicular pattern, the extrafollicular CCL cells and most of the cells within the mucosal follicles expressed alpha 1 heavy chain, but a minor and variable population of cells expressed polytypic IgM. The dendritic reticulum cells stained for alpha 1 (but not alpha 2) heavy chain, mu chain, and both light chains. In all cases, the CCL cells did not stain for common acute lymphoblastic leukaemia antigen (CALLA); in the follicles, CALLA negative cells displaced a residual CALLA-positive population to the periphery and merged with the CALLA negative cells outside the follicles. These findings confirm the homology between IPSID and low-grade B-cell "Western" lymphomas arising in mucosa-associated lymphoid tissue; they suggest that the follicular pattern sometimes seen in these lymphomas is caused by selective colonization of reactive follicles by CCL tumor cells.

The association of Campylobacter pylori with mucosal pathological changes in a population at risk for gastric cancer.

In the study of a group of 178 unselected patients (105 men, 73 women, mean age 44.5 years), from a population at high risk for gastric carcinoma, who presented with chronic dyspepsia, a minimum of 8 gastric and oesophageal biopsy specimens were taken during upper gastro-intestinal endoscopy, and examined histologically and histochemically for the presence of Campylobacter pylori and other pathological lesions. Gastric colonisation by C. pylori was found in 75% of men and 68.4% of women. In 90% of patients with duodenal or gastric ulcer and in 71.6% of patients with non-ulcer and non-cancer dyspepsia there was a moderate or severe degree of bacterial colonisation. Association between C. pylori colonisation and microscopic evidence of type B gastritis, gastric or duodenal ulcer, gastric cancer, oesophagitis and oesophageal glycogenic acanthosis was found.

Gastric carcinoma in young adults.

Of 720 patients with gastric carcinoma treated over a 6-year period, 37 (5%) were 35 years of age or younger. They differed from older patients in that the usual sex ratio was altered (18 men: 19 women), and in certain histologic features. Poorly differentiated or undifferentiated lesions predominated (34 patients), and the distribution of histologic types was unusual; two thirds each were of the diffuse type (Lauren classification) or signet ring type (World Health Organization classification), and over three quarters were infiltrative (Ming classification). Intestinal metaplasia was absent in the majority of patients, and gastritis was less commonly seen than in older patients. Although most patients had long histories of disease and advanced disease, the TNM stages and the proportion undergoing curative resection (8%) were similar to those seen in older patients. Except for one who has survived 5 years, all patients in this study have died.

Severe malnutrition associated with alpha-heavy chain disease: response to tetracycline and intensive nutritional support.

A 20-yr-old black male was admitted with a 5-month history of profound weight loss and diarrhea. Appetite and dietary intake had been remarkably well preserved up until the week before admission. The severity of his depletion was evidenced by a body weight of only 38% of standard, multiple electrolyte deficiencies, and reduced metabolic expenditure, protein turnover, protein synthesis, and pancreatic function. Immunological defects included diminished lymphocyte numbers, lymphocyte transformation, gamma-globulin concentration, and cell-mediated immunity. A diagnosis of alpha-heavy chain disease (alpha-HCD) was made on endoscopic duodenal biopsy and serology--lymphoma being excluded by scanning and laparotomy. Treatment consisted initially of intravenous nutrition (because of the extreme malnutrition, severe diarrhea, and malabsorption of fluid, electrolytes, carbohydrates, and fat) and oral tetracycline. Response was dramatic, with a doubling of body weight within 6 wk, and resolution of malabsorption. He was discharged on a normal diet and long-term oral tetracycline (250 mg/day), and at 1-yr follow-up, nutritional status and gut function were normal despite persistence of duodenal mucosal abnormalities and markers of alpha-HCD and bacterial overgrowth. These results suggest that the malabsorption initially identified in this patient was not due simply to the mucosal abnormalities that characterize alpha-HCD, but was more a consequence of the superimposition of nutrient maldigestion and absorption resulting from the extreme state of protein deficiency and its effects on gut and pancreatic function.

Randomized comparison of R1 and R2 gastrectomy for gastric carcinoma.

The contention that the R2 radical gastrectomy for localized and potentially curable gastric carcinoma may be superior to gastrectomy without lymphadenectomy (R1) was assessed by randomized trial. Five years after commencement 403 patients have been evaluated at surgery and only 43 (11 per cent) found eligible (S0-2, P0, H0, N0-1), 22 of whom underwent R1 and 21 R2 gastrectomy. Seven patients had final histological stages in excess of the protocol. The R2 group had a longer operating time (P less than 0.005), a greater blood transfusion requirement (P less than 0.005), a longer hospital stay (0.05 greater than P greater than 0.025) and required reoperation in four cases. There were no postoperative deaths. Four patients have died from the disease in the R1 group and five in the R2 group, there being no difference in the probability of survival at a median follow-up of 3.1 years. The small proportion of patients suitable for radical R2 surgery, the high associated morbidity and the fact that survival advantage has yet to be proven in trial suggest that this procedure should not yet be performed outside of controlled clinical trials.

Heterotopic thyroid tissue in the heart.

Heterotopic thyroid tissue, presumably due to an embryological developmental abnormality, was discovered incidentally within the right atrial wall of an elderly woman who underwent coronary arterial aortic saphenous vein bypass grafting. As far as we are aware, this is only the third patient with thyroid heterotopia of the heart to be documented.