Porphyria in childhood.

Porphyria in childhood is an uncommon problem but the recognition of these disorders is vitally important for affected children. Of the cutaneous porphyrias, erythropoietic protoporphyria, congenital erythropoietic porphyria, hepatoerythropoietic porphyria, and the hereditary form of porphyria cutanea tarda (PCT) can present in infancy or childhood. This article focuses on the porphyrias that present in infants and children along with a brief discussion of pathogenesis, cutaneous histopathology, and genetics of these metabolic disorders.

International symposium on epidermolysis bullosa. The William and Ida Friday Continuing Education Center, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, April 25-26, 1994. Abstracts.

An international symposium on inherited epidermolysis bullosa was held at the University of North Carolina at Chapel Hill on April 25-26, 1994. All areas currently of clinical and research interest pertinent to this disease were discussed, ranging from basic epidemiologic issues to the definition of molecular defects in each of the three major types of epidermolysis bullosa and the potential for gene therapy. A major focus of this meeting was the presentation of data collected by the National Epidermolysis Bullosa Registry.

Psychological factors associated with chronic dizziness in patients aged 60 and older.

Objective: To identify the prevalence and character of psychological disorders accompanying chronic dizziness in older patients.

Design: Case series of patients from a geriatric dizziness clinic, with comparison data from age- and sex-matched healthy community controls. Both cases and controls received screening psychological testing; all cases were evaluated by a clinical psychologist as part of the dizziness clinic evaluation.

Increased virulence of a human enterovirus (coxsackievirus B3) in selenium-deficient mice.

Coxsackievirus B3 (CVB3/20)-induced myocarditic lesions occurred more quickly and were more severe and virus titers in heart and liver were higher in selenium (Se)-deficient than Se-adequate mice. NK cell activity and serum neutralizing antibody titers were similar in both Se-adequate and -deficient CVB3/20-infected mice; however, lymphocyte proliferation to both mitogen and antigen was decreased in Se-deficient mice. CVB3/20 isolated from Se-deficient donor mice and inoculated into Se-adequate recipient mice induced severe myocarditis.

Effects of chronically elevated pulmonary arterial pressure and flow on right ventricular afterload.

The effects of pulsatile hemodynamics on right ventricle-pulmonary circulation interactions were studied in control lambs and in two lamb models of altered pulmonary hemodynamics induced at infancy: elevated pulmonary arterial pressure (PAP) was created by the infusion of monocrotaline pyrrole (MCTP), and elevated pulmonary arterial blood flow was obtained by the creation of an arteriovenous fistula (Shunt). High-fidelity PAP, midvessel Doppler blood velocity (PAV), and cardiac output (CO) were measured in open-chest, anesthetized lambs. PAV waveforms were normalized to match the measured CO.

Neurobehavioural performance of adults with closed-head injury, adults with attention deficit, and controls.

The symptoms experienced by people with mild closed-head injury (mCHI) and by people with attention deficit disorder (ADD) are similar in many aspects. We examined the performance of 26 adults with mCHI, 23 adults with ADD, and 25 matched controls on four functional areas: (1) simple motor response, (2) response speed and attention, (3) complex perceptual-motor performance, and (4) memory and learning. Analyses of variance and multivariate analyses of variance were used to compare the performance of the three groups.

The National Epidermolysis Bullosa Registry.

Since its inception in 1986, the NEBR has proved to be an excellent example of how a relatively small allocation of federal research funds for the development of a registry of cases of a single rare disease can have a major impact on the rapid expansion in the depth of knowledge of not only the disease itself but of a number of associated biologic principles, including keratinization and epithelial cell-extracellular matrix interactions. At present, the NEBR is generating extensive clinical, laboratory, and demographic data, both from cross-sectional and longitudinal perspectives, as well as establishing a centralized cell and tissue bank that will serve the scientific community at large as a valuable resource for future basic research on this oftentimes devastating genetic disease.

When war comes to men's lives: life-course patterns in family, work, and health.

Men generally come to military service at a time of youth. However, the Second World War expanded the period of service eligibility from age 18 to the late 30s. Each year of delay in entry promised a smaller return from military service (economic and job benefits) and a greater risk of life disruption and related costs.

Vitamin E deficiency intensifies the myocardial injury of coxsackievirus B3 infection of mice.

Feeding a vitamin E-deficient diet increases pathology in hearts of mice infected with a myocarditic coxsackievirus B3 (CVB3/20). Hearts from infected mice fed a vitamin E-deficient diet rich in highly unsaturated fat (menhaden oil) exhibited more severe pathology than hearts from infected mice fed a vitamin E-deficient diet based largely on saturated fat (lard). Furthermore, a cloned and sequenced amyocarditic coxsackievirus B3 (CVB3/0), which caused little or no pathology in the hearts of vitamin E-supplemented mice, induced extensive cardiac pathology in vitamin E-deficient mice.

Association between maternal education and infant diarrhea in different household and community environments of Cebu, Philippines.

Maternal education is one of the strongest determinants of infant survival in developing countries, however, questions remain regarding the extent to which its effects vary as a function of contextual variables. In this study, a multi-level interactive model is used to assess whether the protective effect of maternal education on the risk of infant diarrhea is modified by three aspects of the mother's familial and community environment: household assets, community economic resources and the availability of mothers' clubs. 2484 study participants were interviewed in 1984 as part of the Cebu Longitudinal Infant Health and Nutrition Study.

Management of dizziness in primary care.

Background: We sought to determine the types of dizziness problems that are commonly seen in primary care practices, and to bring to light clinical and demographic factors that predict management decisions.

Methods: We undertook a prospective cohort study with a 6-month follow-up using data gathered in nine primary care practices in two North Carolina counties. Subjects were 144 dizziness patients examined by primary care physicians.

Dyskeratosis congenita or chronic graft-versus-host disease? A diagnostic dilemma in a child eight years after bone marrow transplantation for aplastic anemia.

A 12-year-old boy had striking reticulate hyperpigmentation of the neck and upper chest, dystrophic nails, patchy alopecia, and a white streak on the buccal mucosa. He was diagnosed as having chronic graft-versus-host disease (GVHD) based on clinical findings, skin biopsy findings, and his history of a bone marrow transplantation for aplastic anemia eight years earlier. Dyskeratosis congenita (DC) was not a diagnostic consideration, although the clinical findings and history of aplastic anemia made it a compelling possibility.

Hemorrhagic complications in a rapidly growing, congenital hemangiopericytoma.

A 3-week-old infant had a massive hemangioma-like vascular neoplasm that had enlarged rapidly since being noted at birth. Less than a day after this initial evaluation the tumor underwent spontaneous ulceration and the infant had a near-fatal hemorrhage. Histologically, the tumor was a spindle cell neoplasm most consistent with the diagnosis of congenital hemangiopericytoma.

The reaction of bovine alpha-thrombin with tetranitromethane. Characterization of the modified protein.

Previous studies from several laboratories have shown that thrombin is inactivated by tetranitromethane with the formation of nitrotyrosine. The inactivation is characterized by an apparently greater loss of fibrinogen-clotting activity than activity toward synthetic ester substrates, suggesting that the residues modified by tetranitromethane are involved in the interaction of thrombin with fibrinogen. This study was designed 1) to determine the effect of solvent conditions on the rate of modification and the stoichiometry of the reaction of tetranitromethane with bovine alpha-thrombin; 2) to identify the residue(s) modified; and 3) to characterize the modified enzyme with respect to its interaction with peptide nitroanilide substrates and fibrinogen.

Escherichia coli DNA helicase I catalyzes a unidirectional and highly processive unwinding reaction.

Helicase I has been purified to greater than 95% homogeneity from an F+ strain of Escherichia coli, and characterized as a single-stranded DNA-dependent ATPase and a helicase. The duplex DNA unwinding reaction requires a region of ssDNA for enzyme binding and concomitant nucleoside 5'-triphosphate hydrolysis. All eight predominant nucleoside 5'-triphosphates can satisfy this requirement.

Studies on Ca(II) binding to gamma-carboxyglutamic acid. Use of thermal decarboxylation to probe metal ion/gamma-carboxyglutamic acid interactions.

The thermal decarboxylation of N-benzyloxycarbonyl-L-gamma-carboxyglutamic acid alpha-methyl ester [Z)-L-Gla-OMe) has been studied. In the presence of increasing amounts of calcium or magnesium ions, lyophilized powders of (Z)-L-Gla-OMe exhibit a corresponding increase in thermal stability. Both magnesium and calcium form relatively tight, thermally stable complexes with (Z)-L-Gla-OMe at high metal ion concentrations.

Wear of composite resin restorations in primary versus permanent molar teeth.

The purpose of this investigation was to compare the wear rates of posterior composite resin restorations in primary versus permanent teeth. Based on theories of composite wear in primary molars, as well as on empirical observations, we hypothesized that wear would be greater in permanent tooth restorations. In Part I of the study, quantitative wear data from four different clinical trials (three permanent tooth studies and one primary tooth study) were compared.

Differential effects of opioid and nonopioid analgesics on conditional discriminations in pigeons.

Under the fixed-consecutive-number schedule (FCN), pigeons were reinforced for responding eight or more times on one response key (work key), and then responding once on a second response key. In one component of this schedule, an external stimulus signalled the completion of the response requirement on the work key (FCN 8-SD), whereas no stimulus change was programmed under the other (FCN 8). Across a range of doses, the mu opioid agonist morphine, the kappa opioid agonist U50,488 and the opioid antagonist naloxone had no consistent effect on accuracy under either FCN schedule.

Purification of two smooth muscle glycoproteins related to integrin. Distribution in cultured chicken embryo fibroblasts.

We have purified two membrane glycoproteins from chicken gizzard smooth muscle. In the presence of reducing agents, these proteins have molecular weights on sodium dodecyl sulfate-polyacrylamide gel electrophoresis of 165,000 and 130,000, but they migrate at 165,000 and 110,000 without reduction. The two proteins can also be isolated as a complex in buffers containing physiologic salt concentrations.

Stability and predictability of quality of women's marital relationships and demoralization.

This longitudinal study was motivated by cross-sectional, clinical, and survey research on marital relationships and individual well-being. Perceptions of husbands' behavior toward themselves and self-reports of demoralization were collected from 51 low-income women during their children's kindergarten year and 3 years later. Longitudinal correlations of specific components and aggregated scores of perceptions of husbands' behavior and of demoralization revealed significant stability.

A kinetics analysis of brain-mediated 20-hydroxyecdysone stimulation of the corpora allata of Manduca sexta.

An in vitro method for investigating 20-hydroxyecdysone regulation of the corpora allata (CA) has been used to assess the kinetics of stimulation of precommitment day 3 fifth (V) larval instar Manduca sexta CA by 20-hydroxyecdysone. The synthesis of juvenile hormone (JH) I and III acids by 20-hydroxyecdysone-stimulated CA incubated as complexes with the brain-corpora cardiaca (Br-CC) increased similarly over time; the synthesis of JH III acid was greater than that of JH I acid. Maximal stimulation of the CA to synthesize both JH acids occurred when the Br-CC-CA were exposed to 20-hydroxyecdysone for 30-60 min.