31/36. Small bowel intussusception and brown bowel syndrome in association with severe malnutrition.Brown bowel syndrome is a rare condition characterized by deposition of lipofuscin in the smooth muscle cells of the gastrointestinal tract. The number of reported cases is small, but all are associated with malabsorptive states. Despite these small numbers, there is considerable evidence that vitamin e deficiency is important etiologically. We report here the case of a severely malnourished [body mass index 11.7 kg/m (2): normal range 20-25 kg/m (2)] 31-yr-old black male with a longstanding history of alcohol abuse, who was on anti-tuberculosis therapy. The patient presented with an acute abdomen and was found, at operation, to have a mid-ileal intussusception. Histological examination of the resected specimen demonstrated lipofuscin accumulation consistent with brown bowel syndrome, but no tumor. Subsequent investigations revealed no significant quantities of vitamin E in the blood and pancreatic steatorrhea. However, deficiency of other fat-soluble (vitamin a and D) and water-soluble vitamins (vitamin C and thiamine) also were detected. This report supports the association of brown bowel syndrome with vitamin e deficiency but cannot exclude the compounding effects of protein calorie malnutrition, multiple vitamin deficiencies, and chronic alcohol toxicity.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
32/36. Death caused by undiagnosed acute pancreatitis.A fatal case of acute pancreatitis is reported. On account of difficulty in eating caused by persistent dysphagia and heartburn, the clinical condition of a 39-year-old man who had been a heavy drinker deteriorated rapidly. He was taken to a hospital in an ambulance in an unconscious state. Based on the endoscopic examination and blood chemistry data, the diagnosis of hemorrhagic esophagitis and hepatic failure was made. Treatment including fluid infusion was unsuccessful and he died on the second hospital day. Based on a strong suspicion that the pathologic change in the esophagus may have been chemical esophagitis caused by corrosives of some type, the police ordered an administrative autopsy. The postmortem examination revealed marked necrosis in the pancreas and in the abdominal fatty tissue including the omentum and the mesentery. The necrotic areas in the pancreas were accompanied by only a slight degree of hemorrhage. The cause of death was diagnosed as acute pancreatitis. The pathologic change in the esophagus was identified as candida esophagitis. Alcohol abuse and malnutrition caused by esophagitis were both considered to be factors which lead to the acute fatal pancreatitis.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
33/36. Hypokalaemic myopathy in alcoholism.We describe the clinical and pathological features of a patient with an acute painless proximal myopathy due to hypokalaemia associated with alcoholism. There was an excellent response to treatment with potassium supplements. The importance of recognition of low potassium states in alcohol-dependent patients with muscular weakness is emphasized.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
34/36. witchcraft's syndrome: Munchausen's syndrome by proxy.A 28-year-old man presented to the dermatology Outpatient Department with a complaint of a burning sensation and soreness over his left cheek and left ear of 10 days duration. It had started suddenly one morning when he woke up from sleep. He noticed a large blister with intense redness over his left cheek, associated with a burning sensation. There was a history of similar episodes over the past year, and all were sudden in onset, involved the cheeks, and were noticed after waking up from sleep. The patient volunteered that the episodes were always associated with a drinking spree the previous night. The individual was a healthy man with a wife and two children. The patient had been dependent on alcohol for the past year, and had been consuming alcohol for many years. On examination, there was an eschar occupying almost the entire cheek, with a few scattered lesions over the left tragus and left external ear. Peripheral scarring was noted with hyperpigmentation. While the angle of the mouth was superficially involved on the left side, the oral mucosa was normal. The right cheek also showed a few areas of scarring with patches of alopecia. There were no similar lesions elsewhere on the body. The peculiar history and the morphology of the lesion, that defied any classical diagnosis description, prompted us to interrogate both the man and his wife with regard to any serious differences. After much persuasion and on assurance of secrecy, the wife admitted that her husband was an alcoholic and was neglecting his family. When her efforts to prevent his drinking failed, she resorted to this drastic measure. Each time he passed out after a drinking bout, she poured acid on his cheek, hoping that the sequelae would frighten him from drinking. The acid was readily available to her as she used it for domestic cleaning. The couple were sent for psychiatric evaluation as Munchausen's syndrome by proxy (MSBP) or witchcraft's syndrome (WS) was suspected. Detailed psychiatric evaluation, together with psychometric assessment, revealed that the patient had an alcohol dependence (axis-I diagnosis) and had a cyclothymic personality. Severe marital discord due to alcohol dependence had been present for the past 2 years. Evaluation of the patient's wife revealed that she was under significant psychologic distress. She showed major depressive symptoms with a histrionic personality. She revealed that she had resorted to using the corrosive out of frustration and anger over the behavior of the patient while he was in an inebriated state. The couple are currently undergoing psychiatric treatment.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
35/36. Severe acidosis and hyperdynamic circulation in a 39-year-old alcoholic.A case of acute fulminant cardiovascular beriberi, or "shoshin beriberi," is presented. beriberi is classified as "dry" (neurologic) or "wet" (cardiovascular) and may be mixed. "Wet" beriberi refers to a spectrum of clinical manifestations ranging from a hyperdynamic state with high output cardiac failure to fulminant failure with collapsed peripheral circulation, lactic acidosis, and shock. beriberi is probably much more common than previously recognized. beriberi is caused by thiamine deficiency and should be suspected in alcoholics who present with unexplained lactic acidosis, a hyperdynamic state, high output cardiac failure, or cardiogenic shock without electrocardiographic (EKG) evidence of myocardial necrosis. Shoshin beriberi is rapidly fatal without immediate treatment with thiamine.- - - - - - - - - - ranking = 2keywords = state (Clic here for more details about this article) |
36/36. An inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activity.uroporphyrinogen decarboxylase activity was measured in liver and erythrocytes of normal subjects and in patients with porphyria cutanea tarda and their relatives. In patients with porphyria cutanea tarda, hepatic uroporphyrinogen decarboxylase activity was significantly reduced (mean 0.43 U/mg protein; range 0.25-0.99) as compared to normal subjects (mean 1.61 U/mg protein; range 1.27-2.42). Erythrocyte uroporphyrinogen decarboxylase was also decreased in patients with porphyria cutanea tarda. The mean erythrocyte enzymatic activity in male patients was 0.23 U/mg Hb (range 0.16-0.30) and in female patients was 0.17 U/mg Hb (range 0.15-0.18) as compared with mean values in normal subjects of 0.38 U/mg Hb (range 0.33-0.45) in men and 0.26 U/mg Hb (range 0.18-0.36) in women. With the erythrocyte assay, multiple examples of decreased uroporphyrinogen decarboxylase activity were detected in members of three families of patients with porphyria cutanea tarda. In two of these families subclinical porphyria was also recognized. The inheritance pattern was consistant with an autosomal dominant trait. The difference in erythrocyte enzymatic activity between men and women was not explained but could have been due to estrogens. This possibility was supported by the observation that men under therapy with estrogens for carcinoma of the prostate had values in the normal female range. It is proposed that porphyria cutanea tarda results from the combination of an inherited defect in uroporphyrinogen decarboxylase and an acquired factor, usually siderosis associated with alcoholic liver disease.- - - - - - - - - - ranking = 1keywords = state (Clic here for more details about this article) |
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