Castleman's disease is a rare lymphoproliferative disorder of unknown aetiology. The presentation is varied, diagnosis is difficult, and optimum management is still unknown. We report our experience with a case of Castleman's disease in a 34-year old woman who presented with pallor, hepatosplenomegaly, and a right iliac fossa mass that was 5 cm in diameter. this was initially diagnosed as a soft tissue sarcoma and preoperative tumour embolization was planned before excision. Mesenteric arteriogram revealed that the feeder arteries arose from the superior mesenteric artery and embolization was aborted for fear of causing bowel ischaemia. On laparotomy, lymphoid enlargement was found between the leaves of the jejunal mesentery. The tumour was relatively avascular and the overlying mesenteric vessels contributed to teh duplex ultrasound and computerized tomography appearance of hypervascularity. The tumour with the mesentery and the overlying segment of jejunum was excised completely. Histopathology confirmed Castleman's disease. The purpose of this report is to present this rare case that caused a diagnostic dilemma and to review the management of this disorder.
The clinical response and the histological changes in the mucosa of the small bowel in response to continued feeding with cows' milk protein were assessed over a period of 2-6 weeks in 24 infants who had shown histological changes without immediate clinical symptoms after challenge with a diet containing cows' milk protein. Twenty of the 24 infants (83%) thrived well on cows' milk protein. Jejunal biopsy specimens taken six to eight weeks after the initial biopsy showed histological improvement in all 20 infants compared with biopsy specimens taken soon after the challenge, which had shown mucosal damage. The mucosa had returned to normal in 12, was mildly abnormal in seven, and moderately abnormal in one. Corresponding improvements in the activities of mucosal enzymes were seen. In four of the 24 infants (17%) symptoms developed between three and six weeks. Histological examination of the jejunal biopsy specimens showed that mucosal damage had progressed in two, and remained the same in two; moreover, the disaccharidase activities remained depressed. The present study shows that most infants with enteropathy caused by sensitivity to cows' milk protein but without clinical symptoms develop tolerance to the protein and the mucosa returns to normal despite continued feeding with cows' milk protein.
Investigations in 30 healthy control subjects of Indian, Chinese and Malay ethnic origin, revealed that all the Chinese and about two thirds of the Indians and Malays had a normal capacity to absorb D—xylose, Vitamin A, Co58 labelled Vitamin B12 and dietary fat. About a third of the Indians and Malays were unable to absorb one or two of the four test substances used in the absorption studies. Jejunal biopsies did not differ in the 3 ethnic groups and were normal or mildly abnormal. 27 patients presenting with anorexia, a sore tongue and pallor were also investigated. Indians formed the majority of the patients. Diarrhoea occurred in only 51 % of the patients. All had a megaloblastic anaemia. Absorption studies revealed malabsorption of xylose, Vitamin A and Vitamin B12 in the majority but steatorrhoea occurred in only 26% of the patients. Jejunal biopsies were mildly abnormal in 8% and moderately or severely abnormal in 92 % of the patients. All responded to folic acid or Vitamin B12 therapy. 16 patients were restudied after 5 to 24 months therapy and the majority were found to have improved. Results of investigations and response to therapy indicate that these patients were suffering from tropical sprue. These studies indicate that tropical sprue in Singapore affects Indians mainly and can often present without diarrhoea and steatorrhoea.
Historically a calibre persistent submucosal artery was most often described in the stomach. However in later years it was also discovered in the duodenum and jejunum. It is an uncommon and important cause of massive gastrointestinal bleeding in which failure of detection and early intervention would lead to death. In this paper we report a 27-year-old man with no significant medical history who presented at the emergency unit for recurrent melaena, haematochezia and hypotension. Initial investigations failed to localize the source of bleeding. Emergency exploratory laporatomy revealed a small jejunal mucosal nodule that was actively spurting blood. Histopathological evaluation identified a calibre persistent submucosal artery.
Some infants intolerant to cow's milk protein (CMP) are often also intolerant to other food proteins including soy protein (SP). The effect of CMP and SP in infants recovering from diarrhoeal disease was studied in 22 infants who were maintained on an hypo-allergenic formula for 4-6 weeks. The infants were then challenged successively, initially with SP, followed 24 h later with CMP and then rechallenged with SP 24 h after CMP provocation. Three groups were recognized on the basis of clinical symptoms and mucosal changes following SP challenge. Group 1 comprised four infants who developed clinical and histological reactions on SP challenge. The subsequent CMP challenge, 24 h after the initial SP challenge, resulted in clinical symptoms in three of the four infants, and they developed increased mucosal injury. Rechallenge with SP in the three infants caused development of severe clinical symptoms. Group 2 comprised 12 infants who developed histological reaction but had no clinical symptoms to initial SP challenge. The subsequent CMP challenge caused further progression in mucosal pathology in 11 of the 12 infants and six also had associated clinical symptoms. Rechallenge with SP in the latter six infants resulted in development of clinical symptoms in three and tolerance to SP in three infants. Group 3 comprised six infants who tolerated SP and CMP but one of these infants developed mild histological changes to CMP. The progression of mucosal injury following SP and CMP challenge was associated with a significant decrease in mucosal disaccharidases, alkaline phosphatase levels and presence of reducing sugar in the stools. The 1 h blood xylose level continued to decrease significantly following the pre-SP, post-SP, and post-CMP challenge. It appears that the small bowel mucosa of young infants recovering from diarrhoeal disease remains sensitive not only to CMP but also to SP. The feeding of these proteins in rapid successive sequence to infants with mucosal damage might result in further progression of the mucosal injury. Thus, the exclusion for a variable period of time of antigenic food proteins like CMP and SP from the diet of young infants recovering from diarrhoea might reduce the risk of inducing mucosal sensitivity to these proteins in susceptible infants.
Eleven infants who were suspected clinically of having cows' milk protein sensitive enteropathy were fed with a protein hydrolysate formula for six to eight weeks, after which they had jejunal and rectal biopsies taken before and 24 hours after challenge with cows' milk protein. When challenged six infants (group 1) developed clinical symptoms and five did not (group 2). In group 1 the lesions developed in both the jejunal mucosa (four infants at 24 hours and one at three days), and the rectal mucosa, and the injury was associated with depletion of alkaline phosphatase activity. Infants in group 2 were normal. It seems that rectal injury that develops as a direct consequence of oral challenge with the protein in reactive infants may be used as one of the measurements to confirm the diagnosis of cows' milk protein sensitive enteropathy. Moreover, ingestion of such food proteins may injure the distal colonic mucosa without affecting the proximal small gut in some infants.
A series of 31 infants, 28 with cow's milk protein sensitive enteropathy (CMPSE) and 3 controls, was studied for severity and extent of mucosal damage of the upper small bowel in relation to the development of clinical symptoms. Following challenge with the offending cow's milk, 18 infants (Group 1) developed severe mucosal changes at both the proximal and distal small bowel mucosa and all of these infants presented with clinical symptoms. The other 10 infants (Group 2) who did not develop clinical symptoms following the challenge had less severe damage to the distal small bowel mucosa as compared to the proximal region. The histological score of both the proximal and distal postchallenge biopsies were significantly lower in Group 2 as compared to Group 1 infants. The mucosal disaccharidase and alkaline phosphatase levels were depleted in both the proximal and distal biopsies following challenge but the depletion was greater in the proximal than the distal biopsies. It is suggested that the extent and severity of mucosal damage to the proximal duodenum and jejunum have a critical bearing on the development of clinical symptoms.