Pathological case of the month. Osteoma cutis/pseudohypoparathyroidism.
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Diagnosis, Differential↗
Biomedical subjects
S Caldwell
Publications and source records attributed to S Caldwell.
Multicenter study of lamivudine therapy for hepatitis B after liver transplantation. Lamivudine Transplant Group.
Hepatitis B after liver transplantation is often fatal, and no proven medical therapy exists for this condition. We chose to study the potential efficacy of lamivudine therapy for patients with chronic hepatitis B after liver transplantation. Fifty-two patients with chronic hepatitis B after liver transplantation were treated in an open label, multicenter study. Each had detectable hepatitis B virus (HBV) DNA in serum and 45 (87%) had detectable serum hepatitis B e antigen before treatment. Patients were treated for 52 weeks with lamivudine (100 mg daily). The primary endpoint was undetectability of HBV DNA; secondary endpoints included normalization of serum alanine transaminase (ALT) levels, disappearance of hepatitis B e antigen, and improvement in liver histology. After treatment, 60% of patients had undetectable HBV DNA by solution hybridization assay, 14 (31%) of the initially positive patients lost hepatitis B e antigen; hepatitis B surface antigen was undetectable in 3 (6%); and serum ALT levels normalized in 71%. Blinded histological assessments showed improvement in the histological activity index (P =.007 for periportal necrosis,.001 for lobular necrosis, and.013 for portal inflammation). YMDD variants of HBV, potentially associated with drug resistance, were detected in 14 (27%) of the patients. Repeat liver biopsies in 7 patients with the mutated virus were unchanged in 2, improved in 2, and worse in 3. We conclude that lamivudine is a potentially effective therapy for hepatitis B after liver transplantation.
Adult↗
Predicting the outcome of twin block functional appliance treatment: a prospective study.
A prospective study was undertaken to investigate the relationship between various measured pre-treatment parameters and the reduction in overjet achieved when using a twin block functional appliance. Forty-three subjects were fitted with a twin block functional appliance, and a number of pre-treatment clinical and radiographic morphological features were recorded. The functional appliance wear was monitored for 6 months and any individual who did not co-operate with wear was excluded from the subsequent analysis. Multiple regression analysis with stepwise inclusion was used to relate the percentage reduction in overjet achieved by functional appliance wear to any of the pre-treatment parameters. The data from 22 individuals was included in the final analysis. The overbite and SNB angle were the most strongly related variables to percentage reduction in overjet. These were then used to construct a predictive equation for the expected percentage reduction in overjet: Percentage reduction in overjet in 6 months = 132 + 4.9x1 - 1.4x2, where x1 = overbite and x2 = SNB. The pre-treatment overbite was, in isolation, the most influential feature in predicting the percentage of overjet reduction.
Adolescent↗
Solitary renal myofibromatosis: an unusual cause of infantile hypertension.
INTRODUCTION: Renovascular disease accounts for the vast majority of cases of infantile hypertension with complications resulting from umbilical arterial catheterization predominating in the neonatal period and fibrodysplastic lesions of the renal artery predominating outside the neonatal period. We report a previously undescribed cause of renovascular hypertension: solitary renal myofibromatosis. CASE REPORT: A 9-month-old male infant was transported to the intensive care unit at Children's Hospital in Denver, Colorado, for evaluation and treatment of a dilated cardiomyopathy and severe systemic hypertension. The child was full-term with no perinatal problems. Specifically, the child never required umbilical arterial catheterization. He was well until 6 months of age when his parents noted poor weight gain. At 9 months of age, he was evaluated at the referral hospital for failure to thrive. On examination he was noted to have a blood pressure of 170/110 mm Hg, but no other abnormalities. A chest radiograph showed cardiomegaly. Laboratory studies demonstrated normal electrolytes, blood urea nitrogen, and creatinine. However, urinalysis demonstrated 4+ protein without red blood cells. An echocardiogram showed severe left ventricular dilatation with an ejection fraction of 16%. On admission the child was noted to be cachectic. His vital signs, including blood pressure, were normal for age. The physical examination was unremarkable. Serum electrolytes, blood urea nitrogen, and creatinine were normal. Echocardiographic studies suggested a dilated hypertrophic cardiomyopathy. He was started on digoxin and captopril. Subsequently, he demonstrated episodic hypertension ranging from 170/90 to 220/130 mm Hg. A repeat echocardiogram 24 hours after admission demonstrated a purely hypertrophic cardiomyopathy. Verapamil and nifedipine were added to the treatment regimen in an effort to better control the blood pressure without success. Urine and blood for catecholamines and plasma renin activity, respectively, were sent and treatment with phentolamine instituted because of a possible pheochromocytoma. A spiral abdominal computerized tomographic scan revealed a markedly abnormal right kidney with linear streaky areas of calcification around the hilum and also an area of nonenhancement in the posterior upper pole. The adrenals and the left kidney were normal. Doppler ultrasound revealed a decrease in right renal arterial flow. The urinary catecholamines were normal and surgery was scheduled after the blood pressure was brought under control by medical treatment. At surgery, tumorous tissue and thrombosis of the renal artery were found in the right upper pole. A right nephrectomy was performed. Pathologic examination of the kidney showed the presence of a diffuse spindle cell proliferation in the interstitium of the kidney. The angiogenic/angiocentric character of the proliferation was demonstrated in several large renal vessels. The lumen of most vessels was narrowed and some vessels were totally occluded with recanalization and dystrophic calcifications observed. Immunostaining of the tumor demonstrated strong desmin and vimentin positivity and minimal actin positivity in the spindle cells. Mitotic activity was not noted in the spindle cell process. These pathologic changes were consistent with a diagnosis of infantile myofibromatosis (IM). The child's preoperative plasma renin activity was 50 712 ng/dL/h (reference range, 235-3700 ng/dL/h). DISCUSSION: The causes of systemic hypertension in infancy are many although renal causes are by far the most common. Renal arterial stenosis or thrombosis accounts for 10% to 24% of cases of infantile hypertension. Renal artery thrombosis is usually a consequence of umbilical arterial catheterization, which can also lead to embolization of the renal artery. Renal artery stenosis may result from fibrodysplastic lesions (74%), abdominal aortitis (9%), a complication of renal transplantation (5%), and ren
Cardiomyopathy, Dilated↗
Pediatric bronchioloalveolar carcinoma: a favorable pediatric malignancy?
Primary pulmonary tumors are infrequent in children. Bronchioloalveolar carcinoma has been documented rarely in the pediatric population. Before this report, there have been only three cases of bronchioloalveolar carcinoma in patients less than 16 years of age. Our two cases represent two of the youngest cases (ages 6 and 15 years) reported with bronchioloalveolar carcinoma. They illustrate many of the typical findings of this disease including clinical presentation, diagnostic difficulty, and better prognosis compared with other pulmonary malignancies. This neoplasm appears to have a favorable outcome in childhood.
Adenocarcinoma, Bronchiolo-Alveolar↗
Hyperhomocysteinaemia.
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Aortic Aneurysm, Abdominal↗
A descriptive analysis of the Journal of Manipulative and Physiological Therapeutics, 1989-1996.
BACKGROUND: Two previous reports have summarized the content, institutional affiliations, academic training and funding sources for articles published in the Journal of Manipulative and Physiological Therapeutics (JMPT) from 1978-1986 and 1987-1988. OBJECTIVES: (a) to quantitatively assess the types of articles published in the JMPT from 1989-1996; (b) to identify the affiliations of contributors to the JMPT during this period; (c) to identify the academic backgrounds of contributors to the JMPT from 1989-1996; (d) to identify funding sources for scholarly works published in the JMPT during this period; (e) to identify the proportionate contributions of female authors; (f) to assess the proportion of articles contributed, i.e., foreign vs. domestic sources; and (g) to compare findings for the JMPT from 1989-1996 with similar data for 1978-1988. STUDY DESIGN: Survey of the contents of the JMPT from 1989-1996. METHODS: The contents of the 69 issues of the JMPT from 1989-1996 were reviewed by all authors. Characteristics extracted included category of the article, academic backgrounds of authors, institutional affiliations of authors, funding sources, gender of authors and nation(s) of origin of articles. RESULTS: The annual rate of published contributions to the Journal has more than doubled compared with its first 11 yr of publication, and the proportion of original data reports has grown slightly. Controlled and quasicontrolled clinical trials were 7 times more numerous (n = 28 articles) during the past 8 yr. Chiropractic colleges were the most frequently mentioned affiliation of authors, followed by private practice and nonchiropractic colleges. Collaborative articles submitted by authors at two or more chiropractic colleges grew from only 4 articles from 1978-1988 to 31 articles from 1989-1996. As in previous years, the National College of Chiropractic continued to be the most frequently mentioned academic affiliation of authors. The numbers of articles contributed by those holding scientific (e.g., PhD) and medical degrees have grown substantially. The number of articles mentioning financial support grew from 78 from 1978-1988 to 179 from 1989-1996, and 58 new funding sources were identified. The Foundation for Chiropractic Education and Research continues to be the most frequently mentioned source of funding. Of all articles published in the JMPT from 1989-1996, 21% were authored or coauthored by women. Of 1050 articles, 286 (27%) were authored or coauthored by individuals residing outside the United States of America. CONCLUSIONS: Substantial increases in scholarly activities within the chiropractic profession are suggested by the growth in scholarly products published in the discipline's most distinguished periodical. Increases in controlled outcome studies, collaboration among chiropractic institutions, contributions from nonchiropractors, contributions from nonchiropractic institutions and funding for research suggest a degree of professional maturation and growing interest in the content of the discipline.
Authorship↗
Retransplantation of patients with severe posttransplant hepatitis B in the first allograft.
BACKGROUND: The outcome of orthotopic liver transplantation (OLTX) in patients retransplanted for severe hepatitis B virus (HBV) in the first allograft has been poor due to high rates of HBV reinfection and even more aggressive disease in the second graft. Recent data suggest that hepatitis B immunoglobulin (HBIg) given after transplantation can be successful in delaying or preventing HBV reinfection in patients transplanted for chronic hepatitis B cirrhosis. We report the successful retransplantation of patients who developed recurrent or de novo hepatitis B after OLTXY. METHODS: Using similar HBIg regimens, two centers retransplanted seven patients after they developed recurrent or de novo hepatitis B in the first allograft. At retransplantation all seven patients were HBs antigen (Ag) positive; four patients were positive for HBeAg and HBV DNA by immunoblot assay, two patients were negative for HBeAg and HBV DNA, and one patient was positive for HBV DNA and negative for HBeAg. All patients were either HDV Ag or anti-HDV negative. One patient was anti-HCV positive. All patients received HBIg infusions after retransplantation to maintain serum anti-HBs levels >500 IU/L indefinitely. RESULTS: After retransplantation, six of seven patients are alive (86%): all are without evidence of HBV recurrence with serum negative for HBsAg, HBeAg, and HBV DNA by immunoblot assay. Liver biopsies are normal on routine studies with immunohistochemical stains for HBcAg and HBsAg also being negative. Mean follow-up of these six patients is 40.1 months (range 21-63 months). One patient (14%) developed HBV reinfection 7 months after his second transplant, in spite of maintaining target anti-HBs levels. He maintained stable liver function with minimal evidence of clinical hepatitis B, but died 8 months later from an unrelated stroke. CONCLUSIONS: We conclude that patients with recurrent or de novo hepatitis B after OLTX can be successfully retransplanted using aggressive immunoprophylaxis to prevent HBV reinfection. The failure of HBIg therapy in one patient underscores the need for other effective adjunctive anti-HBV modalities.
Adult↗
NADH oxidase activity from sera altered by capsaicin is widely distributed among cancer patients.
A cancer-specific form of NADH oxidase inhibited or stimulated by 1 or 100 microM capsaicin (8-methyl-N-vanillyl-6-noneamide) is present in sera from cancer patients. The capsaicin-inhibited NADH oxidase activity appears to be absent from sera of individuals free of cancer. The capsaicin-inhibited activity is present both in freshly collected sera and in sera stored frozen for varying periods of time. For the latter, an assay was carried out under renaturing conditions in the presence of NADH and reduced glutathione followed by dilute hydrogen peroxide. Inhibition was half maximal at about 1 microM capsaicin. The capsaicin-inhibited activity was found in sera over a broad spectrum of cancer patients including patients with solid cancers (e.g., breast, prostate, lung, ovarian) as well as with leukemias and lymphomas.
Capsaicin↗
Liver transplantation: incidence and management of deep venous thrombosis and pulmonary emboli.
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Adult↗
The effect of cryopreservation on the lethal mutation rate in Drosophila melanogaster.
Although cryopreservation is routinely used for the storage of a range of biological organisms, few studies have been conducted to determine whether cryopreservation increases the frequency of mutation. A procedure for the cryopreservation of Drosophila melanogaster embryos has recently been developed. Cryopreservation of D. melanogaster is of special interest to geneticists and evolutionary biologists because it would make it possible to assay control and experimental populations simultaneously during long-term studies. Before cryopreserved embryos can be used for such studies, it is first necessary to show that cryopreservation is not mutagenic. We tested for mutagenic effects or cryopreservation in D. melanogaster embryos with an X-linked, recessive lethal assay. The mutation rates of cryopreserved and control flies were not significantly different. We can be 95% certain that cryopreservation does not increase mutation by a factor greater than 2.39. This is the first quantitative estimate of the mutagenic effect of cryopreservation on the germ line of a metazoan. The results are reassuring when considering the genetic impact of cryopreservation on mammalian gametes and embryos.
Animals↗
Successful retransplantation for recurrent posttransplant hepatitis B virus infection in the primary allograft.
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Adult↗
Cysteine supplementation and reduction of total parenteral nutrition-induced hepatic lipid accumulation in the weanling rat.
Total parenteral nutrition (TPN)-induced hepatic steatosis is the most common complication of TPN administration to humans. The mechanism of TPN-induced hepatic steatosis has not been studied in young mammals. The goal of this study was to determine the mechanism of TPN-induced hepatic steatosis in the weanling rat and the effect of supplementation of TPN with choline and/or cysteine on TPN-induced hepatic steatosis. In the weanling rat, we investigated the effect of TPN administration on histologic hepatic steatosis, total hepatic lipid, hepatic acetyl-CoA-carboxylase (ACC--the rate limiting enzyme in fatty acid synthesis) specific activity, and total plasma lipids. TPN administration resulted in a threefold increase in hepatic lipid as compared with control and sham animals (TPN 138 +/- 12 mg/g liver versus control 57 +/- 1), an increase in histologic steatosis (TPN 3.7 versus control 1.3), and a decline in total plasma lipid (TPN 2.1 +/- 0.3 g/L versus control 4.1 +/- 0.3). TPN-induced hepatic steatosis in the weanling rat was not associated with an increase in ACC specific activity (TPN 2.10 +/- 0.33 nmol/min/mg protein versus control 2.85 +/- 0.23). Supplementation of the TPN with choline (15 mg/day) did not significantly lessen hepatic steatosis; however, supplementation of TPN with cysteine (2.5 mg/day) or with cysteine and choline did result in a significant lessening of hepatic lipid content and of histologic steatosis and a normalization of total plasma lipid.(ABSTRACT TRUNCATED AT 250 WORDS)
Acetyl-CoA Carboxylase↗
Clinical course of four patients receiving the experimental antiviral agent fialuridine for the treatment of chronic hepatitis B infection.
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Antiviral Agents↗
Michelin tire baby syndrome: familial constriction bands during infancy and early childhood in four generations.
We report on an infant with an unusual pattern of transitory familial constriction bands distributed symmetrically and circumferentially over the neck, forearms, and lower legs. Family history showed the occurrence of similar bands among individuals in 4 generations transmitted as an autosomal dominant trait. Neck and limb distribution in the other affected family members was also symmetrical and circumferential, with spontaneous resolution taking place during childhood. This case represents another example of the Michelin tire baby syndrome, also known as multiple benign circumferential skin creases of the limbs, and further demonstrates its autosomal dominant mode of inheritance.
Amniotic Band Syndrome↗
Miller-Dieker syndrome. Detection of a cryptic chromosome translocation using in situ hybridization in a family with multiple affected offspring.
OBJECTIVE: To describe a family in whom fluorescence in situ hybridization allowed for accurate diagnosis of Miller-Dieker syndrome in an at-risk pregnancy and determination of parental carrier status. DESIGN: Retrospective case analysis and application of a new molecular tool to evaluate the family. SETTING: Health maintenance organization. The family was followed up by the Departments of Medical Genetics, Pediatrics, and Obstetrics and Gynecology, Kaiser Permanente Medical Center, Panorama City, Calif. PARTICIPANTS: Members of a single family. INTERVENTIONS: Clinical evaluation and neuroimaging studies of the proband. Prenatal diagnosis via ultrasonography and amniocentesis. Chromosomal evaluation of the couple and their offspring. In situ hybridization studies in both parents and an affected fetus. MEASUREMENTS/MAIN RESULTS: We describe a family in whom fluorescence in situ hybridization detected a submicroscopic deletion of the Miller-Dieker syndrome critical region 17p13.3 arising from a cryptic translocation in one of the parents. The proband was determined at birth owing to the presence of multiple congenital anomalies, including low birth weight, microcephaly, agenesis of the corpus callosum, lissencephaly, cerebral atrophy, unilateral ptosis, polydactyly, and omphalocele. High-resolution chromosome-banding analysis findings were normal in the parents and proband, who died at age 4 years. There were four subsequent pregnancies: two ended in first-trimester spontaneous abortion, and in the other two, large omphaloceles were detected in fetuses at 15 and 13 weeks' gestation. Both pregnancies were terminated. Fluorescence in situ hybridization probes for 17p13.3 had become available before the most recent pregnancy and were used to study parental and fetal cells. As a result, a balanced cryptic translocation between chromosome 17 and chromosome 19 was identified in the father: 46,XY,t(17;19)(p13.3q13.33). An unbalanced form of the translocation, involving a deletion of 17p13.3, was detected with fluorescence in situ hybridization in the fetus. This finding was in accordance with a clinical diagnosis of Miller-Dieker syndrome. CONCLUSIONS: Molecular cytogenetic technology should be used in cases of suspected Miller-Dieker syndrome when high-resolution cytogenetic analysis fails to detect del(17) (p13.3). Positive findings should be followed up with parental studies. In addition, omphalocele should be included among the list of malformations that make up the Miller-Dieker syndrome.
Adult↗
Underrecognition of morbidity from stings of the red imported fire ant in the southeastern United States.
This cross-sectional study was conducted to determine the health-seeking behaviors of a group of patients stung by red imported fire ants (RIFA) and the number of nursing referrals resulting in treatment. The following two research questions were asked: (1) Is there a delay in seeking the care of a board-certified allergist for symptoms of fire ant hypersensitivity? (2) Are nurses referring patients for treatment? The entire population of board-certified allergists in Alabama, Georgia, and South Carolina (n = 98) was sampled. A questionnaire was developed for the office nurses to use to collect information from allergy patients actually receiving fire ant injections (n = 257). Each patient reported on the reaction, when it occurred as well as age, sex, and county of residence. Descriptive statistics revealed that a delay of a month or more in seeking care of an allergist was experienced by 154 (56%) of patients who completed the questionnaire. The average delay was 1.8 years. The main source of referrals was a general practitioner or emergency room personnel. Nursing and other health professionals must recognize the problem and to institute prompt referral for treatment. In areas where RIFA colonies are spreading, they have to be fully informed and trained in effective primary and secondary techniques to prevent serious reaction to the stings of these ants.
Adolescent↗
Overview of proposed revisions to the superfund hazard ranking system.
The Comprehensive Environmental Response, Compensation, and Liability Act of 1980 required the federal government to establish criteria for setting priorities among releases of hazardous substances, pollutants, and contaminants. The U.S. Environmental Protection Agency responded by developing the Hazard Ranking System (HRS), which is a scoring system used to establish the National Priorities List (NPL). The Superfund Amendments and Reauthorization Act of 1986 required EPA to amend the HRS so it will more accurately assess relative risks and take into account certain specific elements of risk. On December 23, 1988, EPA published in the Federal Register the proposed rule to revise the HRS. EPA expects to issue the final rule in 1990 after reviewing public comments. This paper describes the proposed revisions and summarizes major technical findings that support the revisions. As a result of the HRS revisions, there may be some changes in the types of sites that score high enough to be placed on the NPL. A projection of those changes is discussed.
Environmental Pollutants↗