Necrotizing bowel disease with candida peritonitis following severe neonatal hypothermia.

Peritonitis due to Candida albicans is rare in the newborn infant. Three low birthweight, extremely ill premature infants who were severely hypothermic at the time of admission developed perforation of the gastrointestinal tract. C. albicans was cultured from the peritoneal fluid in each case. In view of the rareness of C. albicans peritonitis in newborns, this cluster of infants suggests a relationship between severe neonatal hypothermia, and bowel perforation with peritonitis due to this fungus.

Campylobacter pylori in Israel: prospective study of prevalence and epidemiology.

Antral biopsy samples were taken from 147 patients undergoing gastroscopy. Campylobacter pylori was cultivated from 100 of these patients. C. pylori was isolated from 76% of the specimens showing any degree of histologic gastritis, but from only 11% of specimens with completely normal histology. A questionnaire was completed on all patients and included demographic, epidemiologic and clinical information. Sephardic origin, smoking, and a bad taste in the mouth were more prevalent in the campylobacter-positive group. Previous use of antibiotics was negatively associated with the presence of C. pylori. Histologically confirmed gastritis was highly associated with the presence of C. pylori, especially in the moderate or severe grades in which 84% of biopsy specimens were positive. C. pylori was also cultivated from 50% of patients with mild gastritis, in 88% of patients with duodenal ulcer and in 71% of patients with gastric ulcer. The presence of C. pylori in 11% of normal specimens and the absence of C. pylori in 24% of specimens with gastritis further raises the question of the exact role played by C. pylori in the etiology of gastritis.

Recurrence of oxalate deposition in a renal transplant during ciclosporin A therapy.

A 14.5-year-old boy with end-stage renal failure due to familial primary hyperoxaluria underwent cadaver donor renal transplantation. The graft function was stable for a period of 22 months, while he was on conventional immunosuppressive therapy (prednisolone and azathioprine) and additional oral pyridoxine, phosphorus, and magnesium supplementation. When ciclosporin A was introduced instead of azathioprine, the blood levels of oxalate rose, and oxalate deposition in the renal tubuli became evident. These observations suggest that ciclosporin A interferes with oxalate metabolism and, therefore, should be given with utmost caution in patients with primary hyperoxaluria.

Evolution of malignant lymphoma in agnogenic myeloid metaplasia.

Two young arab patients are described in whom malignant lymphoma developed within less than 1 year of the diagnosis of agnogenic myeloid metaplasia. Both patients showed a satisfactory response to combined chemotherapy. One of them died of hepatitis B at 10 months and the other is alive and in clinical remission 25 months after initial diagnosis. These observations demonstrate the close relation between myeloproliferative and lymphoproliferative syndromes and illustrate the diversity of malignant lymphoproliferative disorders into which agnogenic myeloid metaplasia may evolve in the course of disease. Our experience also demonstrates the ease with which some patients with an 'end stage' myeloproliferative disorder may respond to standard chemotherapy designed for the treatment of malignant lymphoma.

Intestinal mast cells in different stages of celiac disease.

A study of mast cell content of the small intestinal mucosa in children with celiac disease is presented. Twenty patients with true celiac disease were studied and compared with 7 patients with transient gluten intolerance and 20 normal control patients. In healthy children we found (mean +/- SE) 142.5 +/- 16.4 mast cells/mm2. In children with active celiac disease, only 40.1 +/- 19.5 cells were found. This difference was highly significant (P less than 0.001). On a gluten-free diet for 1.5 years, the number of mast cells was 82.2 +/- 27.2/mm2 and still remained significantly depressed (P less than 0.001). Upon gluten challenge in celiac disease, the numbers fell to 58.3 +/- 32.6/mm2, while in transient gluten intolerance the numbers of mast cells attained were 102.5 +/- 22.5/mm2, near normal values. These findings indicate that during the untreated phase of celiac disease the number of mast cells is depressed. On a gluten-free diet, the number rises but does not reach normal control levels even after prolonged remission. It is suggested that even during remission of celiac disease the mast cells continue to be damaged by unidentified toxic agents.

Amyloid arthropathy: characterization of the amyloid protein.

An 82 year-old man was referred for joint pain and numbness of his hands. Physical examination revealed limitation of movement of the PIP's, MCP's, wrists, shoulders and knees. There was marked synovial thickening of the wrists and atrophy of the thenar muscles of both hands due to arpal tunnel syndrome. The patient was operated on both hands, the median nerves were released and a synovectomy of the wrist was performed. Two months later, a synovectomy of the right shoulder was performed. Histological examination of tissues from the wrists and shoulder demonstrated large deposits of amyloid in the synovia. Amyloid fibrils were extracted, solubilized in 6M and were fractionated on a Sepharose 6B. All three proteins that were purified from the amyloid fibrils proved to be derived from VkI light chain by their amino terminal sequences. This is the first amyloid protein to be characterized from amyloid arthropathy.

Chronic cutaneous polyarteritis nodosa simulating recurrent thrombophlebitis.

A 30-year-old man who had 12 repeated hospital admissions within a period of 7 years for localized pain and swelling of the extremities is described. Excision biopsy of several subcutaneous lesions revealed histologic changes typical of polyarteritis nodosa (PN). Systemic disease was excluded because of a normal testicular biopsy, negative abdominal angiogram, and the lack of evidence for renal or other visceral involvement. Response to steroid therapy was excellent. Cutaneous PN is a distinct subset of polyarteritis with a chronic course and excellent prognosis. The present case underlines the need for a greater awareness, and for considering early biopsy of suspected lesions in patients with multiple episodes of apparent superficial thrombophlebitis.

Clinical, histological, and electron microscopic study of mast cell disease of the small bowel.

A patient with mast cell disease of the small bowel is described in whom clinical, histological, and ultrastructural studies served to delineate the characteristic features of the disease. Urticaria pigmentosa, steatorrhea, eosinophilia, absence of antireticulin antibodies, and submucosal nodularity seen on radiographic study of the duodenum were the clinical characteristics. The endoscopic appearance was that of severe exudative duodenitis. The histology of the small intestinal mucosa showed crypt cell destruction and villous atrophy. Marked infiltration of the lamina propria with mast cells, eosinophils, and neutrophils was also distinctive. The enterocytes retained their columnar epithelium, confirmed on electron microscopy. The fine structural abnormalities of the mast cells are demonstrated for the first time. Degranulated mast cells predominated within the lamina propria and none was seen among the epithelial layers. The mast cell nuclei were irregular, often binuclear, and showed loss of their normal heterochromatin pattern. In their cytoplasm only few granulated bodies were seen and even more rarely inclusions with whorls and scrolls. We conclude that the clinical, histopathological, and ultrastructural appearances in mast cell disease of the small bowel are distinctive and should be used as criteria for diagnosis. Care should be taken in the evaluation of the number of mast cells since the demonstration of these cells may be affected by various fixing and staining techniques.

Acute osteolytic lesions following pancreatitis in a dialysis patient.

A 42-year-old man undergoing maintenance hemodialysis suffered an attack of acute pancreatitis. Convential treatment resulted in quick recovery. 10 days after its onset his hands and feet became swollen, hot, red and painful. Multiple intramedullary osteolytic lesions of the metatarsals, metacarpals and phalanges, with cortical destruction and a number of fractures were found. These lesions subsided over many weeks and did not recur. We believe that such acute osteolytic lesions following pancreatitis are not 'renal osteodystrophy' as such, but should be recognized as a possible complication in renal failure patients.

Familial erythrophagocytic lymphohistiocytosis in infancy.

A 2 1/2-month-old infant suffering from pyrexia, purpura, hepatosplenomegaly, pancytopenia and hyperlipidemia is reported. Liver and spleen biopsies revealed mononuclear histiocytic infiltration with marked erythrophagocytosis. The girl died at 7 1/2 months of age. Her brother died in infancy with an analogous clinical picture. The parents were first cousins. The clinical presentation and laboratory findings are consistent with the diagnosis of familial erythrophagocytic lymphohistiocytosis.

Gastrointestinal mast cells in health, and in coeliac disease and other conditions.

Intestinal biopsies were performed in children suffering from coeliac and other diseases and were stained by the 'astra-blau' method for visualising granulated mast cells. The density of granulated mast cells (per microscopical field) in treated coeliac disease and in 3 control groups (patients suffering from milk allergy, failure to thrive, or protracted diarrhoea) was 15--18 cells per microscopical field, which contrasted with a mean of 6.8 cells in untreated coeliac disease. It is concluded that exposure to gluten in coeliac disease results in degranulation of mast cells, while exposure to milk in milk allergy has no such effect.