Nesidioblastosis is a rare metabolic disease characterised by inappropriate insulin secretion often associated with life-threatening hypoglycaemia. While severe cases present in the newborn period, patients have been described later in infancy. Familial cases suggest an autosomal recessive trait, and recently mutations in the sulphonlurea receptor gene, possibly a regulator of insulin secretion, have been identified and associated with disease expression. We report a twin boy who developed normally until the age of six months when he was noted to regress. The boy is the older twin born to non-consanguinous parents. He presented to a hospital first at the age of 13 months with fever and generalised seizures. Low blood glucose was noted, but he recovered easily and was able to maintain euglycaemia during a 48-hour period of observation. Microcephaly and developmental delay were documented and anticonvulsant therapy was started. At 18 months, low blood glucose with high C-peptide was documented during reevaluation. Follow-ing a short trial of subcutaneous long-acting somatostatin analogue, the child was subjected to near-total pancreatectomy. The histology revealed findings consistent with nesidioblastosis. The child's condition improved but he remained significantly delayed This case emphasises the importance of recognising and treating hypoglycaemia early to avoid irreversible brain damage. It is interesting to note that the twin brother has always been well and is developmentally normal. Further studies to identify the inheritance pattern in the family would be of great interest.
We report two patients who had cerebral malaria, heavy parasitemia, hyperbilirubinemia, hypercatabolism with rapid rises of blood urea and serum creatinine and acute renal failure. There was no evidence of intravascular hemolysis. Renal biopsy was consistent with acute tubular necrosis. Both patients responded to treatment with intravenous quinine and dialysis.
Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disease primarily involving inflammation of the joints. Although the management of the disease has advanced significantly in the past three decades, there is still no cure for RA. The aim of this study was to determine the therapeutic efficacy of δ-tocotrienol, in the rat model of collagen-induced arthritis (CIA). Arthritis was induced by intradermal injection of collagen type II emulsified in complete Freund's adjuvant. CIA rats were orally treated with δ-tocotrienol (10 mg/kg) or glucosamine hydrochloride (300 mg/kg) from day 25 to 50. Efficacy was assessed based on the ability to reduce paw edema, histopathological changes, suppression of collagen-specific T-cells, and a reduction in C-reactive protein (CRP) levels. It was established that δ-tocotrienol had the most significant impact in lowering paw edema when compared to glucosamine treatment. Paw edema changes correlated well with histopathological analysis where there was a significant reversal of changes in groups treated with δ-tocotrienol. The results suggest that δ-tocotrienol is efficient in amelioration of collagen-induced arthritis. Vitamin E delta-tocotrienol may be of therapeutic value against rheumatoid arthritis.
To highlight the first reported case of necrobiotic xanthogranuloma of the parotid gland. We also review the clinical presentations and treatments for this rare condition.
Cardiac sarcoidosis is a disease of young adults. In most cases it presents with sudden death, arrhythmias, conduction disorders, heart failure or cardiomyopathy. The authors describe two cases of myocardial involvement by sarcoidosis that lead to death of the patients. Case one was a 26-year-old Indian man who was previously well and presented with sudden death. Autopsy showed nodules of sarcoid granuloma involving the heart, lungs and lymph nodes. Case two was a 47-year-old Indian lady who complained of reduced effort tolerance. Echocardiography showed that she had restrictive hypertrophic cardiomyopathy with heart failure. Seven months after initial presentation, she developed worsening of heart failure and died. Autopsy revealed involvement of the heart, lungs and liver by sarcoidosis.
This report describes a patient with acute renal failure that resulted from the ingestion of djenkol beans. Features of acute djenkolism include nausea, vomiting, bilateral loin pain, gross hematuria, and oliguria. The blood urea level was 16.2 mmol/L and the serum creatinine was 460 mumol/L. Phase contrast microscopy of the urinary sediment indicated that the hematuria was nonglomerular. Ultrasound of the kidneys showed slightly enlarged kidneys with no features of obstruction. Renal biopsy showed acute tubular necrosis similar to the single animal study reported in the literature. With conservative therapy, which included rehydration with normal saline and alkalinization of the urine with sodium bicarbonate, the acute renal failure resolved. Based on its chemistry, djenkol bean-associated acute renal failure may be analogous to acute uric acid nephropathy.
Parathyroid carcinoma is an uncommon cause of parathyroid hormone (PTH)-dependent hypercalcaemia, accounting for less than 1% of all cases of hyperparathyroidism. Parathyroid carcinoma is an indolent tumour with rather low malignant potential. Consideration of parathyroid carcinoma in the differential diagnosis of hypercalcaemic disorders is important because the morbidity and mortality are substantial and the best prognosis is associated with early recognition and surgical resection. Clinical indicators favouring parathyroid carcinoma over benign disease include markedly raised serum calcium levels, PTH and alkaline phosphatase. A palpable neck mass with both kidney and skeletal manifestations also give a high index of suspicion of parathyroid carcinoma. Histopathology alone is not sufficient to diagnose parathyroid cancer; it has to be correlated with the clinical findings. The initial and most effective treatment for parathyroid carcinoma is complete resection of the primary lesion, and repeated operations for recurrence are useful. The prognosis of parathyroid carcinomas is quite variable; 5-year survival rates vary from 40% to 86%, while the 10-year survival rate is approximately 49%. We report a case of parathyroid carcinoma occurring in a 55-year-old woman who presented with bone pain and hypercalcaemia.
A 45-year-old Malay lady who presented with intermittent abdominal pain and a left hypochondrial mass was found to have a 10 x 8 x 5 cm duodenal tumour without local invasion at laparotomy. En bloc resection of the tumour with adequate margin of clearance was done and histopathological diagnosis of low grade leiomyosarcoma was made. Fourteen months later, she returned with multiple metastases in the liver and needed palliative chemotherapy for pain relief. Duodenal leiomyosarcomata are very rare tumour. Their prognostic indicators include biological grading, tumour size and presence of metastases. Recognition of its high malignant potential calls for close surveillance calls even after apparent curative surgery.
Muscarinic acetylcholine receptor-active compounds have potential for the treatment of Alzheimer's disease. In this study, a series of natural and synthetic flavones and flavonols was assayed in vitro for their ability to inhibit radioligand binding at human cloned M1 muscarinic receptors. Several compounds were found to possess competitive binding affinity (Ki=40-110 µM), comparable to that of acetylcholine (Ki=59 µM). Despite the fact that these compounds lack a positively-charged ammonium group under physiological conditions, molecular modelling studies suggested that they bind to the orthosteric site of the receptor, mainly through non-polar interactions.
Primary peritoneal carcinoma (PPC) is a rare tumor that is histologically and immunohistochemically indistinguishable from epithelial ovarian carcinoma. The diagnosis is usually made after excluding gross ovarian involvement or the ovarian involvement is only confined to the surface. A 68-year-old lady presented with right iliac fossa pain and increasing CA125. The CT scan showed bilateral pelvic adnexal masses with peritoneal deposits within the right side of abdomen. She was initially diagnosed as carcinomatosis peritonei from the omental cake removed after exploratory surgery. She was managed as advanced ovarian tumor with peritoneal metastasis and was then administered six cycles of chemotherapy. Surgical intervention included debulking surgery consisting of total abdominal hysterectomy, bilateral salpingooophorectomy and omentectomy and also with right hemicolectomy. The histopathological findings were of primary peritoneal serous carcinoma with only minimal involvement of the serosal surface of the right ovarian capsule. No microscopic invasion into underlying ovarian cortex and stroma was observed. Multiple tumor deposits were also seen over the right paratubal and paraovarian tissue, both parametrium as well as serosal surface of the terminal ileum and periappendicular tissue. Immunohistochemically, the malignant cells were positive to CA125, focally positive to CK7 and negative to CD20 and Calretinin. PPC is one of important differential diagnosis which needs to be considered in cases of advanced ovarian tumor, although the former can only be ascertained after excluding the ovarian involvement microscopically.
Non-secretory multiple myeloma (NSMM) is a rare variant of the classic form of multiple myeloma (MM). In NSMM, no monoclonal gammopathy can be detected in serum or urine by conventional techniques, making the diagnosis more difficult. We describe a 71-year-old man who had been diagnosed and treated for granulocytic sarcoma one year prior to his recent problems of progressive low-back pain of two months duration. Skeletal X-rays showed diffuse osteolytic lesions with multiple pathological fractures but there was no monoclonal gammopathy in the serum or urine. The biopsy of the lytic lesion on the upper part of the femur showed infiltration by abnormal plasma cells. A diagnosis of NSMM was made and he was treated with chemotherapy. The early diagnostic difficulty and the challenges faced regarding the case are discussed.