Cutaneous gangrene in a renal dialysis patient.
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Biomedical subjects
Publications and source records attributed to B Beckwith.
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PURPOSE: To determine whether a human chorionic gonadotropin (hCG) level of 2,000 mIU/mL is a reasonable threshold for diagnosing ectopic pregnancy in the absence of ultrasound (US) findings of intrauterine pregnancy (IUP) and thus to prevent inappropriate treatment that will result in the loss of an otherwise normal pregnancy in women with early IUPs. MATERIALS AND METHODS: The authors reviewed the medical records of and US scans obtained in 676 patients in whom ectopic pregnancy was clinically suspected between January 1, 1994, and December 31, 1995. RESULTS: Five hundred forty-eight patients had evidence of a normal or abnormal IUP. Fifty-one (40%) of the 128 patients without evidence of an IUP had an hCG level of more than 2,000 mIU/mL. Of these 51 patients, 15 (29%) were treated for ectopic pregnancy; 17 (33%) were not immediately treated for ectopic pregnancy and had a normal IUP at follow-up US. CONCLUSION: An hCG level of 2,000 mIU/mL without US findings of IUP, while suggestive of an abnormal pregnancy, is not diagnostic. Per the results of recent studies, it is reasonable to closely follow up rather than treat many of these early, stable cases of ectopic pregnancy.
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Alpers disease consists of diffuse cerebral degeneration manifested as developmental delay, seizures, vomiting, and progressive neuromuscular deterioration, with liver disease and death. We report the clinical course of the liver disease, histologic progression of the hepatic lesions, and etiologic investigations in five patients (four girls, three kinships). All had grown and developed normally until seen at 6 to 36 months of age (mean 20 months), with vomiting (n = 5), progressive hypotonia (n = 3), or seizures (n = 2). All had been given anticonvulsants, including valproic acid in three. Liver disease was noted at a mean age of 35 months (range 9 to 67 months), with hepatomegaly (two patients), abnormal hepatic synthetic function (three) or transaminase values (three), and cirrhosis in one. Patients survived for a mean of 4.6 weeks (range 1 to 8 weeks) after the identification of liver disease; all died of hepatic failure. Results of evaluation for infectious and metabolic causes of liver disease and causes of degenerative neuromuscular disease were negative in all patients. Premortem liver biopsy specimens (n = 3) demonstrated an early lesion consisting of lobular disarray, microvesicular steatosis, periportal acute and chronic inflammation, and individual hepatocyte necrosis. Autopsy findings (n = 5) consisted of macrovesicular steatosis, massive hepatocyte dropout, and proliferation of bile ductular elements, with almost complete replacement of hepatocytes by proliferating bile ductular elements in two patients. Brain showed characteristic neuronal degeneration. We conclude that Alpers disease can be a cause of rapidly progressive liver failure in early childhood. Although the cause of this autosomal recessive disease is not known, it does not appear to be related to peroxisomal dysfunction.
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One-hundred-eighty-eight children up to 16 years of age were randomized in the second National Wilms' Tumor Study (NWTS) with tumors that were confined to the kidney and that had been totally excised (Group I). Most fared well whether treated for six or for 15 months with both actinomycin D (AMD) and vincristine (VCR). No postoperative radiation therapy (RT) was given. The two-year relapse-free survival (RFS) and two-year survival rates were 88 and 95%, respectively. Two-hundred-sixty-eight randomized patients with more advanced local lesions (Groups II and III) and 57 with distant metastases (Group IV) had postoperative RT and were scheduled for 15 months treatment with either AMD and VCR (Reg. C) or AMD plus VCR plus Adriamycin (Reg. D). The 77% two-year RFS rate for Reg. D was significantly different from the 63% with Reg. C. As in the first NWTS, patients with tumors of unfavorable histology (UH) had a significantly worse prognosis than those with favorable histology (FH), as did those with positive nodes. Survival rates at two years were 54% for UH vs. 90% for FH, and 54% vs. 82% for those with and without lymph node involvement.
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The National Wilms' Tumor Study, initiated in 1969, tested competing treatment strategems for patients with tumors ranging from Group (Gp) I (tumors confined to the kidney and totally removed) to Gp IV (remote metastases present at diagnosis). Three hundred and fifty-nine of 606 registered patients were randomized in the trial. Gp I patients under 2 years of age fared well whether postoperative radiation therapy (RT) was or was not added to 15 months' maintenance actinomycin D (AMD). Their prognosis was better than that for older cohorts similarly treated, in whom the difference in relapse rates between treatment groups were suggestive of an RT effect. Combined AMD and vincristine (VCR) gave better results than either agent alone in patients with more advanced tumors (Gps II and III) still confined to the abdomen, all of whom received postoperative RT as well. Preoperative VCR given Gp IV patients in addition to postoperative RT, AMD, and VCR did not improve results. The frequency of mesoblastic nephroma (1%), of bilateral tumors (5%), and of incorrect preoperative diagnosis of Wilms' tumor (5%), the toxicities of the various regimens, and other ancillary data are presented and discussed.
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We report a case of a 9-year-old boy with focal, segmental glomerulosclerosis who, following peritoneal dialysis, underwent renal transplantation and bilateral nephrectomy. The kidneys showed histological features of embryonal hyperplasia of Bowman's capsular epithelium, an uncommon lesion that is seen most often in patients with chronic renal failure who are being maintained on dialysis. In addition, a 1-cm tumor in the left kidney showed features of metanephric adenoma. Although both lesions are uncommon, they share many similarities on a morphological, immunohistochemical, and ultrastructural basis. This association has not been previously reported and may shed some light on the histogenesis of these recently described lesions.