Calciphylaxis – a topical overview

'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.     

Change in waist-hip ratio with weight loss and its association with change in cardiovascular risk factors.

We investigated whether weight loss decreases the waist-hip ratio (WHR) in overweight men and women, and whether changes in WHR relates to change in cardiovascular risk factors, independent of change in weight. Weight loss correlated significantly with decreases in the circumference of the waist and hips, and decreases in WHR in men and women. At comparable levels of weight loss, men had greater decreases in the waist, and smaller decreases in the hips than women, resulting in greater decreases in WHR. Cardiovascular risk factors improved significantly with weight loss. However, after controlling for weight loss, there was no evidence that change in WHR or change in circumference measures were related to change in risk factors. These data suggest that WHR is modifiable by weight loss, especially in men, but that change in WHR may not be independently related to changes in cardiovascular risk factors.     

The Sigma-trial protocol: a prospective double-blind multi-centre comparison of laparoscopic versus open elective sigmoid resection in patients with symptomatic diverticulitis

Backround  

Diverticulosis is a common disease in the western society with an incidence of 33–66%. 10–25% of these patients will develop diverticulitis. In order to prevent a high-risk acute operation it is advised to perform elective sigmoid resection after two episodes of diverticulitis in the elderly patient or after one episode in the younger (< 50 years) patient. Open sigmoid resection is still the gold standard, but laparoscopic colon resections seem to have certain advantages over open procedures. On the other hand, a double blind investigation has never been performed. The Sigma-trial is designed to evaluate the presumed advantages of laparoscopic over open sigmoid resections in patients with symptomatic diverticulitis.     

Trends in the teaching of neurology to family practice residents, 1981-1990.

A decade ago, less than one-half of the family practice (FP) residency programs surveyed provided a defined rotation in neurology. To learn how neurology is currently being taught in FP residencies, we sent questionnaires to 384 approved FP residencies in the United States, receiving responses from 241 (63%). Seventy-eight percent provide a defined rotation in neurology, usually in the form of a preceptorship with a community neurologist, and 57% systematically present the core curriculum recommended by the American Academy of Neurology and the American Academy of Family Physicians in 1987. Asked about the level of cooperation they encounter in recruiting neurologists to teach, 54% of the respondents reported "too little" or "no" cooperation from academic neurologists, while just 20% reported these responses in regard to community neurologists. Most (87%) reported that involvement in the residency program leads to increased patient referrals for the participating neurologist. Most (81%) respondents would welcome more participation by neurologists.     

Hepatocellular lymphoepithelioma-like carcinoma associated with epstein barr virus: a hitherto unrecognized entity.

OBJECTIVE: Lymphoepithelioma-like carcinoma (LELC) is an undifferentiated carcinoma with a dense lymphoid stroma. It has been reported in diverse organs and shows variable association with Epstein-Barr virus (EBV). Only a few EBV positive cases have been observed in the hepatobiliary system, all of which were considered to be cholangiocarcinomas. We report a unique case of hepatocellular LELC arising in a cirrhotic liver with EBV demonstrated in the tumor cells. METHODS AND RESULTS: A 39-year-old Hispanic female underwent an orthotopic liver transplant for end stage liver disease secondary to chronic hepatitis C. A high-grade hepatocellular carcinoma with a dense lymphocytic infiltrate was found in the explant as well as in a portal lymph node. Three months posttransplant, the patient developed numerous hepatic nodules with enlarged periaortic and portacaval lymph nodes. Biopsy of the hepatic nodules showed a recurrent hepatocellular carcinoma devoid of a dense lymphocytic infiltrate. Both the primary and recurrent tumors were positive for EBV by molecular studies. The patient eventually expired from liver failure over a 6-week period. CONCLUSION: This case represents the first report of EBV-positive hepatocellular LELC. It is particularly interesting given the precipitous clinical outcome, which was possibly related to immunosuppresive therapy.