Bile porphyrin analysis in the evaluation of variegate porphyria

Article Abstract:

Variegate porphyria is an inherited disorder of porphyrin metabolism. Porphyrins are a class of organic chemical compounds which are important constituents of hemoglobin and a number of critical enzymes. Patients with variegate porphyria may have skin that is especially susceptible to mechanical damage, particularly areas exposed to light. Neurologic complications are also common among these patients. The diagnosis of variegate porphyria is not easy. Even during acute attacks of the symptoms, the urinary output of abnormal porphyrin metabolic products may resemble those from other sorts of porphyria disorders, particularly acute intermittent porphyria. While the porphyrin precursors produced in the feces may be more diagnostic, the porphyrin content of the feces is affected not only by the disease process, but also by the consumption of different sorts of foods and by variations in the bacteria which colonize the digestive tract. A study was thus undertaken to determine if direct sampling of the bile might provide better diagnostic capabilities than other methods. Bile is secreted by the liver and stored in the gallbladder until it is dumped into the duodenum of the small intestine during the digestive process. A sample of bile may be obtained by anesthetizing the patient's throat, then passing a tube down through the stomach into the duodenum. When an analysis was performed on the bile obtained in this manner, the lowest porphyrin amount of any patient with variegate porphyria was found to be nine time greater than the highest amount for any normal individual tested. The analysis of fecal samples from the same patients showed much smaller differences, and four of the 10 patients tested had amounts of porphyrin within normal range when tested in this way. The results of the study suggest that the analysis of bile may be more useful in the diagnosis of variegate porphyria than fecal samples. (Consumer Summary produced by Reliance Medical Information, Inc.)

Analysis, Bile, Metabolism, Inborn errors of, Inborn errors of metabolism, Porphyria

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Evaluation of dementia

Article Abstract:

Signs and symptoms of dementia require a comprehensive work-up because many causes of dementia are reversible. Dementia is characterized by reduced intellectual and emotional abilities that interfere with independent functioning. Alzheimer's disease accounts for about 70% of cases, and blood vessel diseases cause another 10% to 20%. Diagnostic work-ups should include mental status tests, thyroid function tests and vitamin B12 measurements. Some infectious diseases can also affect mental status. CT and MRI scans can identify anatomical brain lesions. Extensive neurological testing can assist in diagnosing the more difficult cases.

Dementia, Alzheimer's disease

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Hans Scholl and the 'Weisse Rose': an aspect of medical resistance during the third reich

Article Abstract:

Hans Scholl, a son of a Nazi movement oppositionist, was an early supporter of the movement. He was even the flag carrier of the Hitler Youth. He became a doctor because of his love for the natural sciences and while pursuing the vocation, Hans realized the evils of the Nazis, prompting him to join the medical resistance. He and his friends published and distributed the 'Weisse Rose,' a series of pamphlets containing philosophical accusations and condemnations of the Nazis. Such was a courageous expression of their resistance and for that, Hans and his friends deserve recognition.

History, Biography, Personalities, National socialism, Anti-Nazi movement, Anti-Nazi movements, Scholl, Hans

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