A "new" skeletal dysplasia in two unrelated boys

We report on 2 unrelated boys with similar physical and radiographic findings that may represent a "new" skeletal dysplasia. Findings in common include early speech delay, short stature, frontal bossing with a depression over the metopic suture, a narrow nasal root with beaked nose, midfacial hypoplasia with relatively prominent eyes, and brachydactyly with blunt fingers. Radiographic findings include mild irregularities of the vertebral bodies, hypoplasia of the odontoid process, short phalanges with increased distal width, coning and sclerosis of several epiphyses, and overtubulation of the long bones. Although these patients share some manifestations with the floating-harbor syndrome (Robinson et al.: J Pediatr 113:703-706, 1988), their radiographic changes are distinctive and are not suggestive of a recognized skeletal dysplasia syndrome.     

Minimally invasive palliative tumor therapy guided by imaging techniques: the UCLA experience

Imaging-guided palliative therapy of recurrent and/or inaccessible head and neck tumors may soon become clinically practical since sensitive and noninvasive monitoring techniques of energy deposition in tissues are now available. Interstitial tumor therapy (ITT) is a technique whereby a source of energy (laser, radiofrequency, ultrasonic, cryoenergy, etc.) is directly applied into tumors at various depths. Recent studies have demonstrated the efficiency of ultrasound (UTZ) and magnetic resonance imaging (MRI) for real and/or "near" real time tumor and vessel identification as well as monitoring and quantifying energy-induced tissue damage. We now report our initial clinical experience with patients in which UTZ and/or MRI-guided ITT techniques were successfully applied for the treatment of recurrent, nonresectable, local, and/or metastatic head and neck carcinomas. Patients were treated on an outpatient basis either in the operating room or in an upgraded specially equipped SIGNA 1.5T MR suite. Most patients tolerated these procedures well and were successfully palliated for periods ranging from 3 months to 5 years posttreatment. The upgrades introduced in a standard MRI suite, the clinical experience, and future perspectives will be reviewed.     

The emerging role of the health information management professional in data quality and analysis of an electronic medical record system

The role of the health information management professional is changing with the advancement of the electronic medical record system (EMRS) and electronic financial database systems. The Medical ARchival System, a longitudinal EMRS developed at the University of Pittsburgh Medical Center, was used to describe the methodology that the health information management professional should use when performing data quality evaluations and data analysis of an EMRS. Specific examples of steps used to integrate databases to collect the data and generate appropriate reports and tables are also described and discussed.     

Postnatal development of vascular β-adrenoceptor-mediated responses and the increase in the adrenaline content of the adrenal gland have a parallel time course

The present study was undertaken to analyse the relationship between postnatal development of vascular ₂-adrenoceptor-mediated responses and the content of adrenaline in the adrenal gland and its concentration in plasma. Dog saphenous vein tissue from newborn, two-weeks old and adult animals were either preloaded with ³H-noradrenaline (or ³H-adrenaline) to study prejunctional -adrenoceptor-mediated effects or mounted in organ baths to determine isoprenaline-induced relaxation of preparations contracted by phenylephrine to about 65010 of the maximum. The adrenal glands and samples of blood from the same animals were taken for estimation of adrenaline and noradrenaline.At birth, there were no -adrenoceptor-mediated effects pre- or postjunctionally. At two weeks, while the results at the prejunctional level were not significantly different from those obtained in newborns, at the postjunctional level there was a relaxant response to isoprenaline, which antagonised about 35010 of the previous contraction to 1.75 mol·l^–1 phenylephrine. In adults, isoprenaline (50 nmol·l^–1) increased by 24% tritium overflow evoked by electrical stimulation of tissues preloaded with ³H-noradrenaline but not that of tissues preloaded with ³H-adrenaline. On the other hand, propranolol (1 mol·l^–1) reduced by 21% the overflow of tritium evoked by electrical stimulation of tissues preloaded with ³H-adrenaline but not that of tissues preloaded with ³H-noradrenaline; postjunctionally, the maximal response to isoprenaline antagonised 70% of the previous contraction to 1.75 mol·l^–1 phenylephrine.At birth the catecholamine content of the adrenals was relatively low (2.9 ol·g^–1) and the adrenaline/noradrenaline ratio was 0.26; two weeks later, the catecholamine content was 14.5 mol·g-1and the adrenaline/noradrenaline ratio was 0.74; in adults, the catecholamine content was 24.5 mol·g^–1 and the adrenaline/noradrenaline ratio was 2.3. In plasma, the highest concentration of adrenaline was observed at birth (11.8 nmol·l^–1); two weeks later it was 5.5 nmol·l^–1 and in adulthood it fell to 3.1 nmol·l^–1.On the basis of these results, it is concluded that some link between the postnatal increase in adrenaline adrenal content and the development of ₂-adrenoceptor-mediated pre- and postjunctional effects may exist. Additionally it is suggested that circulating adrenaline may trigger the development of ₂-adrenoceptor-mediated responses as well as some hypertensive states occurring as a consequence of an overreactivity of the sympathoadrenal system. Correspondence to: S. Guimarães at the above address     

Psychosocial and sexual aspects of pregnant adolescents at Belém-Pará

Pregnancy is a major factor that modifies psychosocial aspects of the adolescents. A study with 120 pregnant adolescents aged 12 to 18 years, without regarding gestational age, was carried out at Prenatal Risk Program in the Secretaria Estadual de Saúde, Belém-Pará, from 10/1991 to 04/1992. At the first encounter, it was made an approach of psychosocial and sexual subjects, with the following results: onset of menarche primarily between 12-13 years old (median 12.2 years old); beginning of sexual practice at 14 years old in 50.9% of the adolescents. 56.6% were pregnant at 16 years old. Most of the adolescents (98.4%) first had sexual intercourse with their boy friends and by their own wish (72.5%). In one year of loving affair, 67.5% of the adolescents have already begun sexual activities, this attitude more prevalent among the younger(79.4%). Adolescents had learned about sexuality mainly from mates (65.1%). Although 55.9% of the adolescents were aware of some contraceptive method, just 13.3% of them used one. The majority of the pregnant adolescents (96.7%) were financially supported by their families. Respectively, 30.3% and 32.1% of these girls left school before or during pregnancy. The risk factors that may contribute to an early and unintended pregnancy among adolescents, specially the younger ones, were pointed out: little information about sex and contraceptive methods, limited practice on their use and a high rate of precocious sexual activities.     

Haemophilus influenzae type b infections and its prevention by vaccines

Haemophilus influenzae type b (Hib) infections and its prevention by vaccines are reviewed. Its prevalence is underestimated, due to technical pitfalls in the laboratory and to the difficulties of diagnosing respiratory infections, chiefly pneumonia. Taking into account all infections (respiratory, meningitis and others), Hib is probably the most important pathogen, compared to S. pneumoniae and N. meningitidis. The incidence of Hib meningitis in Brazil is presented and risk factors are discussed, as well as the preventive alternatives. The different Hib vaccines are analyzed and schedules of immunization with two of them (PRP-T and HbOC) are presented. Possible eradication of Hib diseases is discussed.     

Measles seroprevalence among pediatricians in a teaching hospital]

OBJECTIVE: A measles outbreak occurred in S. Paulo state, during 1996 and 1997, resulting in 20,921 cases. Forty seven percent of the cases occurred in people between 20 and 29 years of age, and one of the control strategies of the Department of Health was the vaccination of health care workers. The prevalence of antibodies against measles among the hospital pediatricians was investigated. METHODS: One hundred and fifty samples were taken from volunteer pediatricians to test for measles antibodies using ELISA. A questionnaire about their having had measles and the vaccine was filled out. RESULTS: Of the 150 doctors, 122 (81.4%) were female and 28 (18.6%) male, of between 23 and 46 years of age (mean and median 27 years). The majority (98%-147/150) had protective levels of antibodies against measles (>100 UI/ml); 118 (80.3%-118/147) without and 29 (19.7%-29/147) with a history of measles. Only 3 pediatricians (2%-3/150), had negative serology, 2 without and 1 with a history of measles. Out of the 118 without history of measles, 79 (67%-39/118) in spite of the protective level of antibodies against measles, did not know if they, had been vaccinated. Out of the 79 vaccinated pediatricians, 64 (81%-64/79), had been vaccinated 25 years before, and still maintained protective levels of antibodies. Of the 3 doctors with negative serologies only one declared that he had been vaccinated. CONCLUSIONS: Measles seroprevalence among pediatricians of this hospital is high, especially due to preceding vaccination. On the other hand, the 2% of pediatricians with negative serology, in an epidemic situation could constitute a significant population for the acquisition and dissemination of the disease.     

Apolipoprotein E promotes the binding and uptake of β-amyloid into Chinese hamster ovary cells in an isoform-specific manner

The 4 allele of apolipoprotein E gene is a major risk factor for Alzheimer's disease. However, the mechanism by which the E4 isoform of apolipoprotein E increases the risk of Alzheimer's disease is poorly understood. To determine whether the isoform-specific effects of apolipoprotein E may be mediated via clearance of bound β-amyloid, we examined the uptake of β-amyloid 1–40 into Chinese hamster ovary cells in the presence or absence of the apolipoprotein E isoforms E2, E3 and E4. Apolipoprotein E2 and E3 treatments were associated with higher association of β-amyloid with cells as compared to treatment with E4. Heparin blocked the association of β-amyloid with cells, as did an antibody to one of the apolipoprotein E receptors (the low-density lipoprotein receptor-related protein).

Thus, the apolipoproteins E2 and E3, but not E4, may play important roles in the clearance of β-amyloid from the extracellular space via the low-density lipoprotein receptor-related protein.