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Biomedical subjects

E Young

Publications and source records attributed to E Young.

At least 19 recordsLinked to original sources
PubMed1992

Female twin with Hunter disease due to nonrandom inactivation of the X-chromosome: a consequence of twinning.

We report the occurrence of Hunter disease (mucopolysaccharidosis type II) in a karyotypically normal girl who was one of identical twins. Molecular studies showed nonrandom X-inactivation in both her fibroblasts and lymphocytes, while her normal twin showed equal usage of both X chromosomes. In view of previous reports of 7 pairs of identical female twins in which one had Duchenne muscular dystrophy, it seems that twinning may be strongly associated with nonrandom X-inactivation, and is not specific to the properties of the disease causing gene.

DNA Probes↗
PubMed1992

Administration of human recombinant IL-7 to normal and irradiated mice increases the numbers of lymphocytes and some immature cells of the myeloid lineage.

In vitro experiments performed by several investigators have demonstrated that IL-7 is a growth factor for immature B lymphocytes, thymocytes, and mature T lymphocytes. To evaluate the potential therapeutic use for human rIL-7 (rhuIL-7) as a hematopoietin, we have studied the in vivo hematopoietic effects of rhuIL-7 in mice. In these experiments, sublethally irradiated and normal mice were treated with or without rhuIL-7 for up to 26 days. Administration of rhuIL-7 significantly increased the white blood cell count in the peripheral blood and spleen in both normal and irradiated mice. Treatment with rhuIL-7 also accelerated lymphocytic recovery in irradiated mice. Precursor and mature B lymphocytes showed the greatest expansion in response to rhuIL-7 administration, with smaller increases in T lymphocytes being observed. In mice recovering from high dose irradiation, rhuIL-7 treatment resulted in preferential expansion of CD8+ T lymphocytes and more rapid normalization of the CD4/CD8 ratios. Differential analysis of peripheral blood smears demonstrated that rhuIL-7 also increased the numbers of immature granulocytes in both normal and irradiated mice. Moreover, administration of rhuIL-7 to normal, irradiated, cyclophosphamide-pretreated, or 5-fluorouracil-pretreated mice increased the number of acetylcholinesterase-positive megakaryocytes in the spleen, but not the bone marrow. Therefore, although the major in vivo effects of rhuIL-7 were on cells of the lymphocytic lineage, rhuIL-7 also increased the numbers of some immature cells of the myeloid lineage.

Animals↗
PubMed1992

Heparin binding to plasma proteins, an important mechanism for heparin resistance.

Heparin dosage requirements vary widely among patients with venous thromboembolism. In this study, we measured the proportion of anticoagulantly-active heparin which was reversibly bound and neutralized by plasma proteins (defined as reversible heparin neutralization) in the pre-treatment plasma (in vitro) and in the 6 h post-treatment plasma (ex vivo) of patients with venous thromboembolism treated with a fixed dose of heparin. Reversible heparin neutralization was assessed by comparing the heparin levels measured as anti-factor Xa activity before and after the addition of low affinity heparin which is essentially devoid of anti-factor Xa activity, in order to displace heparin bound to plasma proteins. The results indicate that reversible heparin neutralization due to binding to plasma proteins is a major determinant of the anticoagulant response to a fixed dose of standard heparin 6 h post-treatment and of the eventual heparin dose required to achieve a therapeutic anticoagulant effect on days 3-5 of heparin treatment.

Adult↗
PubMed1992

Fetal presentation of Morquio disease type A.

A fetus with mucopolysaccharidosis type IV A (Morquio type A) is described. The family had one affected child exhibiting symptoms of classical Morquio A disease, and late in the subsequent pregnancy prenatal diagnosis was requested. At 23 weeks' gestation, moderate ascites was detected by detailed ultrasound scan and keratan sulphate was found in the amniotic fluid. The pregnancy was terminated by prostaglandin induction and the diagnosis of mucopolysaccharidosis type IV A was confirmed by demonstration of a deficiency of N-acetylgalactosamine-6-sulphate (GalNac-6-S) sulphatase in cultured amniotic cells and in post-mortem fibroblast cultures. The activities of beta-galactosidase and arylsulphatase A were normal, ruling out Morquio disease type B and multiple sulphatase deficiency. These results indicate that mucopolysaccharidosis IV A (a disease that predominantly affects the skeletal system) may produce ascites in the fetus to such an extent that it can be detected by ultrasound.

Amniocentesis↗
PubMed1992

Bone marrow transplantation for Sanfilippo disease type B.

Allogeneic bone marrow transplantation was performed on twins with Sanfilippo B disease. They were the first two patients with this disorder to undergo the procedure. There was definite evidence of engraftment as shown by conversion to donor blood group antigen and tissue type, and increased leukocyte alpha-glucosaminidase activity. Nine years post transplant, neither twin is as handicapped as her untreated brothers were at the same age, although in one twin hyperactivity and behavioural problems, characteristic of the disorder, are present. Details of the twins' intellectual development and growth, their alpha-glucosaminidase activity and urinary glycosaminoglycan excretion are reported.

Bone Marrow Transplantation↗
PubMed1992

Salpingoscopy: systematic use in diagnostic laparoscopy.

OBJECTIVE: To evaluate the importance of salpingoscopy together with laparoscopy in the diagnosis of tubal pathology. DESIGN: Salpingoscopy was performed as a complementary method in patients who were subjected to diagnostic laparoscopy. The relationship between the salpingoscopy and (1) the patient's previous history of tubal disease and (2) laparoscopic diagnoses was evaluated. SETTING: Private patients referred to the Instituto de Fertilidad, Buenos Aires. PATIENTS, PARTICIPANTS: Forty-two patients undergoing a diagnostic laparoscopy during the evaluation of their fertility or as a follow-up of previous therapy. MAIN OUTCOME MEASURE(S): Salpingoscopy was performed, using a colpomicrohysteroscope. We evaluated alterations in major and minor folds and their vascularization, the presence of microadhesions, and cellular nuclei dyed with methylene blue in the tubal lumen. RESULTS: Fifty percent of the patients who had no previous history of tubal disease presented with endosalpingeal alterations, and in 37% of the normal laparoscopies the salpinx had unilateral or bilateral salpingoscopic abnormalities. CONCLUSIONS: Salpingoscopy is a useful method to evaluate oviducts, before assuming their normality, and consideration of these women for assisted reproductive technology.

Abortion, Spontaneous↗
PubMed1992

Care of the dying: an ethical and historical perspective.

OBJECTIVE: To provide a historical perspective, from ancient Greece to the middle of the 20th century, on ethical issues and principles commonly associated with medical care for the dying in Western civilization. SOURCES: Writings of noted philosophers, historians, ethicists, and physicians, as well as published legal and ethical guidelines. INFORMATION EXTRACTION: The sources used highlight the origins of various ethical principles associated with care of the dying. They also identify the opinions of prominent individuals throughout the history of medical ethics. SUMMARY: Devotion to medical beneficence, concern for the quality of life, and respect for the sanctity of life are all expressed in the earliest medical and philosophical writings of ancient Greece. With regard to care of the dying, these considerations led to a wide acceptance of avoiding or terminating treatment in hopeless cases. They also led to active debate regarding medicine's role in hastening the dying process. The rise of Christianity during the Middle Ages markedly suppressed such debate by strongly reinforcing the principle of sanctity of life. Later, the optimism of the enlightenment added the hope of prolonging life. Finally, modern advances in medical science have made that hope a reality of complex ethical dimensions. CONCLUSIONS: Ethical debates regarding appropriate care for the dying are as old as medicine itself. Although beneficent concerns have characterized the medical community in almost every period of history, tensions have repeatedly arisen as diverse religious and philosophical ideologies have produced varying standards to define such beneficence. In the Christian world, the sanctity of life was often extolled as the paramount standard. For the ancient Greeks and Romans, and again in many post-Renaissance philosophies, quality of life considerations assumed equal or greater importance. Modern life-prolonging technologies heighten the debate by allowing these two standards to dramatically conflict, particularly in the critical care setting.

Beneficence↗
PubMed1992

Deposition of aerosolized pentamidine and failure of pneumocystis prophylaxis.

OBJECTIVE: To determine if outcome of Pneumocystis carinii prophylaxis is related to total lung dose of aerosolized pentamidine. SETTING: AIDS treatment centers at a VA and University Hospital. PATIENTS: Fifty-eight HIV-infected patients receiving P carinii prophylaxis with aerosolized pentamidine using a nebulizer (CIS-US AeroTech II) were followed up over a 90-week period. Treatment consisted of 60 to 90 mg every two weeks. MEASUREMENTS: In all patients, deposition of pentamidine aerosol was measured using a radioaerosol filter technique. Factors thought to be important in deposition, nebulizer output and breathing pattern were also measured. Six months later, repeated deposition studies were performed in 20 patients. Pentamidine dose to the lung was related to occurrence of P carinii pneumonia and correlated with nebulizer function and breathing parameters. Outcome was assessed in terms of pentamidine deposition and patient characteristics, including demographic, immunologic, physiologic, and medical variables. RESULTS: Ten patients (17.2 percent) had development of P carinii pneumonia. However, pentamidine deposited in the failures (8.18 +/- 4.74 mg) was no different than deposition in protected patients (6.39 +/- 3.07 mg, p = NS). Most of the variability in deposition was accounted for by variability in nebulizer output (r = 0.919, p less than 0.001). Deposition did not significantly correlate with any of the measured breathing parameters. Serial deposition measurements were not significantly different by paired analysis. The incidence of P carinii pneumonia did not correlate with any measured patient characteristic. CONCLUSIONS: Failure of aerosolized pentamidine prophylaxis is not related to total lung dose of pentamidine. Other factors such as inadequate microscopic deposition between alveoli, pentamidine clearance, or drug resistance may be important. In HIV-infected patients, interpatient variability in aerosol deposition can be reduced by reducing variability in nebulizer output rather than control of breathing pattern.

Aerosols↗
PubMed1991

IgE antibodies to Pityrosporum ovale in atopic dermatitis.

An enzyme-linked immunosorbent assay (ELISA) was developed to assess serum IgE antibodies directed against Pityrosporum ovale in patients with atopic dermatitis (AD), atopic patients with allergic respiratory disease (ARD: rhinitis or asthma) but without eczema, and in healthy controls. IgE binding to P. ovale extract was demonstrated in 49% (35/72) of AD patients. In contrast, anti-P. ovale IgE was found in only one of 27 atopic controls without eczema; all healthy control sera (n = 17) were negative. Of 37 AD patients tested intracutaneously with P. ovale, 31 showed immediate-type reactivity, and 20 of these 31 patients had anti-P. ovale IgE detectable by ELISA, while sera from the six non-responders were all negative. Levels of anti-P. ovale IgE were highest in AD patients aged 20-30 years. No correlation was found with the severity of AD, but there was a non-significant tendency (P = 0.06) to higher levels in AD patients with concomittant respiratory allergy. Anti-P. ovale IgE was significantly correlated with total serum IgE, with specific IgE against various aeroallergens as measured by RAST, and with levels of anti-Candida albicans IgE, measured with a similar ELISA. Thus, production of IgE antibodies against P. ovale occurs very frequently in AD, and rarely in patients with atopic disease without skin involvement.

Adolescent↗
PubMed1991

Self-perceived needs of primary caregivers of home-hospice clients.

The purpose of this study was to assess the self-perceived needs of primary caregivers of terminally ill home-hospice clients. A home interview was conducted using an instrument based on the Neuman Systems Model. Data were analyzed in relation to intrapersonal stressors (within the individual), interpersonal stressors (between the individual and others), and extrapersonal stressors (between the individual and the environment). Five community nursing diagnoses (Porter, 1987) were identified: (a) Population at risk for health problems, (b) population at risk for role constriction/role fatigue, (c) population at risk for insufficient support, (d) population at potential risk for lack of information, and (e) population at potential risk for lack of service coordination. Early attention to limiting the impact of stressors on the caregiver can improve the caregiver's ability to care for the hospice client.

Adult↗
PubMed1990

Comparison of non-mydriatic retinal photography with ophthalmoscopy in 2159 patients: mobile retinal camera study.

OBJECTIVE: To determine whether non-mydriatic Polaroid retinal photography was comparable to ophthalmoscopy with mydriasis in routine clinic screening for early, treatable diabetic retinopathy. DESIGN: Prospective study of ophthalmoscopic findings according to retinal camera screening and ophthalmoscopy and outcome of referral to ophthalmologist. SETTING: Outpatient diabetic clinics of three teaching hospitals and three district general hospitals. PATIENTS: 2159 Adults selected randomly from the diabetic clinics, excluding only those registered as blind or those in wheelchairs and unable to enter the screening vehicle. MAIN OUTCOME MEASURES: Numbers of patients and eyes correctly identified by each technique as requiring referral with potentially treatable retinopathy (new vessel formation and maculopathy) and congruence in numbers of microaneurysms, haemorrhages, and exudates reported. RESULTS: Camera screening missed two cases of new vessel formation and did not identify a further 12 but indicated a need for referral. Ophthalmoscopy missed five cases of new vessel formation and indicated a need for referral in another four for other reasons. Maculopathy was reported in 147 eyes with camera screening alone and 95 eyes by ophthalmoscopy only (chi 2 = 11.2; p less than 0.001), in 66 and 29 of which respectively maculopathy was subsequently confirmed. Overall, 38 eyes received laser treatment for maculopathy after detection by camera screening compared with 17 after ophthalmoscopic detection (chi 2 = 8.0; p less than 0.01). Camera screening underestimated numbers of microaneurysms (chi 2 = 12.9; p less than 0.001) and haemorrhages (chi 2 = 7.4; p less than 0.01) and ophthalmoscopy underestimated hard exudates (chi 2 = 48.2; p less than 0.001). CONCLUSIONS: Non-mydriatic Polaroid retinal photography is at least as good as ophthalmoscopy with mydriasis in routine diabetic clinics in identifying new vessel formation and absence of retinopathy and is significantly better in detecting exudative maculopathy.

Adolescent↗
PubMed1990

Comparison of narrow-band UV-B phototherapy and PUVA photochemotherapy in the treatment of psoriasis.

The therapeutic effectiveness of a new fluorescent lamp, Philips TL-01, which emits a narrow peak around 311-312 nm, was compared with the currently used PUVA photochemotherapy consisting of oral 8-MOP followed 2 h later by UV-A from fluorescent lamps Philips TL-09. Comparisons of therapeutic efficacy were performed in 10 patients with widespread, symmetrically distributed psoriasis lesions. They received treatment with PUVA on one half of the body and with TL-01 light on the other half; both treatments were given twice a week. It is concluded that on the average phototherapy with narrow-band UV-B is an effective as PUVA; it is certainly more convenient and probably less carcinogenic.

Adult↗
PubMed1979

The assay and partial characterization of macromolecular heparin depolymerase activity in rat small intestine.

Homogenates of rat small intestine can depolymerize macromolecular rat skin heparin (RS heparin) to products similar in size to commercial heparin [Horner (1972) Proc. Natl. Acad. Sci. U.S.A. 69, 3469--3473]. This activity is attributed to an enzyme provisionally named 'macromolecular heparin depolymerase'. An assay for macromolecular heparin depolymerase activity in rat small intestine has been developed, based on the action of the enzyme on 35S-labelled macromolecular RS heparin. The depolymerized products are separated into two peaks by gel chromatography through columns of Bio-Gel A-15m. The amount of label in the second peak, expressed as a percentage of the total radioactivity, is the index of enzyme activity. The pH optimum was found to be 6.0 and the temperature optimum 45 degrees C. The enzyme was shown to be most stable in 50mM-Tris/maleate buffer containing 1 mM-EDTA. Macromolecular heparin depolymerase activity measured as a function of time and substrate concentration produced curves typical of an enzymic reaction. Evidence was obtained demonstrating that the activity did not originate from bacteria in the intestine. Macromolecular heparin depolymerase activity was increased by dilution and storage at 7 degrees C for 24 h. This suggests that homogenates of rat small intestine contain an unstable inhibitor of the enzyme.

Animals↗
PubMed1979

Immunological cross-reactivity between a glycoprotein isolated from Trichophyton mentagrophytes and human isoantigen A.

A glycoprotein isolated from the cell wall of Trichophyton mentagrophytes was assessed for its cross-reaction with human blood group isoantigens. Rabbit antiglycoprotein antibodies agglutinated human erythrocytes of blood groups A1 and A2, and precipitated Blood Group Substance A in agarose gels. Erythrocytes of blood group B were only slightly agglutinated, and O(Rho+) and O(Rho-) erythrocytes were not. Additionally, the glycoprotein was shown to specifically inhibit isoagglutination of erythrocytes of group A. Partial identity between the glycoprotein and a crude extract of the fungus was demonstrated by immunodiffusion. Analyses revealed the glycoprotein to be composed of approximately 17% protein and 80% carbohydrate. The glycoprotein was found by indirect immunofluorescence to be located in the mycelial cell wall. The possibility that cross-reacting antigens may lead to a chronic, spreading infection is discussed.

ABO Blood-Group System↗