Medical treatment of primary sclerosing cholangitis

Until 1970, primary sclerosing cholangitis (PSC) was considered to be a medical curiosity. With the development of endoscopic cholangiography, PSC is now recognized more frequently and is a common indication for liver transplantation. PSC is usually progressive, leading to cirrhosis, portal hypertension, and liver failure. The manifestations of disease may be clinically similar to those of other causes of bile duct obstruction and must be distinguished from gallstone disease, bile duct carcinoma, primary biliary cirrhosis, and secondary biliary cirrhosis due to bile duct stricture. Medical management of PSC must take into account the likelihood that destroyed bile ducts do not regenerate as hepatocytes do. Hence, PSC should be treated early in its course. The goal of therapy is to prevent further damage and destruction of bile ducts. In this article, we will present relevant data concerning the medical management of primary sclerosing cholangitis. Received for publication on March 8, 1999; accepted on April 5, 1999     

Risk factors for seizure-related motor vehicle crashes in patients with epilepsy

OBJECTIVE: We identified clinical risk factors for seizure-related motor vehicle crashes in patients with epilepsy. BACKGROUND: Current US laws permit epilepsy patients with controlled seizures to drive. These laws attempt to balance the important economic and social value of driving with the risk to public safety from seizure-related crashes. Various clinical factors are considered in these laws, particularly the seizure-free interval. Driving restrictions range from 3 to 18 months, however, and studies have not established how these various seizure-free intervals and other clinical factors influence the risk for seizure-related motor vehicle crashes. METHODS: We performed a retrospective case-control study to determine the influence of clinical risk factors associated with seizure-related motor vehicle crashes. Both "case" and "control" patients had epilepsy, drove, and were from the same clinic, but the cases differed in having had seizure-related crashes. RESULTS: Fifty patients with epilepsy who crashed during seizures and 50 matched control patients were compared. Factors that significantly decreased the odds of patients with epilepsy having motor vehicle crashes due to seizures were: long seizure-free intervals, reliable auras, few prior nonseizure-related accidents, and having had their antiepileptic drugs (AEDs) reduced or switched. For example, patients who had seizure-free intervals > or = 12 months had a 93% reduced odds for crashing compared to patients with shorter intervals. Other findings were: 25% of patients had more than one seizure-related crash and 20% had missed an AED dose just prior to their crash. The majority (54%) of patients who crashed were driving illegally, with seizure-free intervals shorter than legally permitted. CONCLUSION: Seizure-free intervals, the presence of reliable auras, AED therapy modifications, and a history of nonseizure-induced crashes should be considered when counseling patients with epilepsy on driving and when formulating driving regulatory policy. Case control studies of crashes due to seizures can help in assessing and monitoring such risks.     

Developmental and psychiatric evaluation in the preschool context

This article documents the ways in which a school-based developmental evaluation can be an effective and powerful alternative to the traditional clinic-based evaluation. The child is not taken to a strange new place but observed and evaluated in his or her naturalistic environment. The families have increased access to care, and the foundation is laid for cohesive, trusting working relationships with families and teachers. Children are evaluated in one of two models or a combination of both naturalistic observation and individual direct assessment. Tensions between the clinical and educational system exist, but the early childhood evaluator who educates himself or herself about the cultures and forces at work can build from the strengths of each system to effect positive growth for young children at risk for developmental delays or psychiatric disorders.     

Arthritis and nephritis in patients with systemic lupus erythematosus

Previous publications suggest that in patients with systemic lupus erythematosus (SLE), rheumatoid factor (RF) may be "protective" against nephritis. In our study of 662 patients with SLE, we found that persistent, rheumatoid-like arthritis showed a much stronger inverse correlation with nephritis than RF. Of 186 such patients, 59 developed clinically evident nephritis (32%) compared to 263 of the other 476 patients (55%) (p less than 10(-7). RF showed only a weak inverse relationship to nephritis (p = 0.064). We conclude that the presence of persistent rheumatoid-like arthritis in patients with SLE identifies a clinical subset of patients who are less likely to develop nephritis than those with no arthralgia, no objective arthritis or only episodic arthritis. We hypothesize that such patients represent a genetically determined subset among patients with SLE and that perhaps they are more likely to bear the HLA-DR4 allele.