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1.
Domest Anim Endocrinol ; 74: 106499, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-32858465

RESUMEN

Vasoactive intestinal polypeptide (VIP) is involved in gastric smooth muscle relaxation, vasodilation, and gastric secretions. It is also associated with appetite regulation, eliciting an anorexigenic response in mammals, birds, and fish; however, the molecular mechanism mediating this response is not well understood. The aim of the present study was thus to investigate hypothalamic mechanisms mediating VIP-induced satiety in 7-d old Japanese quail. In experiment 1, chicks that received intracerebroventricular (ICV) injection of VIP had reduced food intake for up to 180 min after injection and reduced water intake for 90 min. In experiment 2, VIP-treated chicks that were food restricted did not reduce water intake. In experiment 3, there was increased c-Fos immunoreactivity in the arcuate (ARC) and dorsomedial (DMN) nuclei of the hypothalamus in VIP-injected quail. In experiment 4, ICV VIP was associated with decreased neuropeptide Y mRNA in the ARC and DMN and an increase in corticotropin releasing factor mRNA in the DMN. In experiment 5, VIP-treated chicks displayed fewer feed pecks and locomotor behaviors. These results demonstrate that central VIP causes anorexigenic effects that are likely associated with reductions in orexigenic tone involving the ARC and DMN.


Asunto(s)
Núcleo Arqueado del Hipotálamo/efectos de los fármacos , Coturnix , Núcleo Hipotalámico Dorsomedial/efectos de los fármacos , Péptido Intestinal Vasoactivo/farmacología , Animales , Núcleo Arqueado del Hipotálamo/fisiología , Conducta Animal/efectos de los fármacos , Núcleo Hipotalámico Dorsomedial/fisiología , Relación Dosis-Respuesta a Droga , Ingestión de Líquidos/efectos de los fármacos , Conducta Alimentaria/efectos de los fármacos , Inmunohistoquímica/veterinaria , Proteínas Proto-Oncogénicas c-fos/genética , Proteínas Proto-Oncogénicas c-fos/metabolismo , Péptido Intestinal Vasoactivo/administración & dosificación
2.
Water Sci Technol ; 71(7): 980-5, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25860699

RESUMEN

Ozone transforms various organic compounds that absorb light within the UV and visible spectra. UV absorbance can therefore be used to detect the transformation of chemicals during ozonation. In wastewater, decolourisation can be observed after ozonation. This study investigates the correlation of the UV absorbance difference between the ozonation inlet and outlet and the removal efficiency of micropollutants in wastewater. The absorbance at 254 and 366 nm was measured at the ozonation inlet and outlet, as was the concentration of 24 representative micropollutants and the dissolved organic carbon (DOC). The results clearly showed that the relative decrease of absorbance (ΔAbs) is positively correlated with the relative removal efficiency of micropollutants. We therefore suggest that UV absorbance can be used as a feedback control parameter to achieve optimal ozone dosage in wastewater treatment plants and to gain a fast insight into the process efficiency and stability of the ozonation.


Asunto(s)
Ozono/química , Rayos Ultravioleta , Eliminación de Residuos Líquidos , Aguas Residuales/análisis , Contaminantes Químicos del Agua/química , Suiza , Contaminantes Químicos del Agua/análisis
3.
Water Sci Technol ; 60(2): 497-506, 2009.
Artículo en Inglés | MEDLINE | ID: mdl-19633393

RESUMEN

Reliable and efficient operation of membrane bioreactors (MBR) for centralized municipal wastewater treatment strongly depends on a good monitoring of the membrane permeability, a value often used to quantify the transmissibility (i.e. the inverse of the flow resistance) of the membrane. By directly or indirectly evaluating this parameter the operator normally plans on the necessity and effectivity of maintenance activities. To allow the operator monitoring the membrane performance closely, the present paper proposes a method for online permeability based on the measurements of permeate flow, transmembrane pressure and water temperature. The theoretical background of the method is discussed and a data set of 250 days of continuous pilot operation of a municipal MBR equipped with three different standard membrane modules is used for testing. The method is numerically simple enough to allow being implemented on any programmable logical controller.


Asunto(s)
Reactores Biológicos , Eliminación de Residuos Líquidos/métodos , Purificación del Agua/métodos , Diseño de Equipo , Residuos Industriales , Membranas Artificiales , Modelos Estadísticos , Permeabilidad , Presión , Eliminación de Residuos/métodos , Análisis de Regresión , Programas Informáticos , Factores de Tiempo , Contaminantes Químicos del Agua/análisis , Contaminantes Químicos del Agua/aislamiento & purificación
4.
Homeopathy ; 97(2): 83-8, 2008 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-18439969

RESUMEN

This study is based on 25 well documented reports of cases which responded well to treatment with Petroleum. Materia medica data were compared with results in contemporary clinical practice. Many patients had characteristic skin problems; children often had recurrent or chronic upper respiratory tract problems. The most prominent mental feature is a quiet, withdrawn or stubborn disposition. The mental symptoms may be difficult to recognise. Detailed documentation in daily practice can be helpful for preserving data of the effect of a medicine; confirmation of statements given in materia medica; improving understanding of homeopathic medicines and differentiating the indications for medicines.


Asunto(s)
Eccema/terapia , Homeopatía/métodos , Otitis Media/terapia , Petróleo , Infecciones del Sistema Respiratorio/terapia , Ansiedad/etiología , Ansiedad/terapia , Niño , Eccema/complicaciones , Femenino , Humanos , Masculino , Persona de Mediana Edad , Otitis Media/complicaciones , Proyectos de Investigación , Resultado del Tratamiento
5.
Int J Tuberc Lung Dis ; 9(1): 32-6, 2005 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-15675547

RESUMEN

SETTING: The Sudan National Tuberculosis Programme serves internally displaced and settled populations in Khartoum, Sudan. OBJECTIVES: To investigate whether treatment in the camps is satisfactory compared to the settled population and to World Health Organization (WHO) standards, and to map certain characteristics of the clinics that may explain differences in treatment outcome. DESIGN: A register analysis was done on sputum smear-positive and retreatment tuberculosis patients registered consecutively in the first and second quarters of 2000. The nine health facilities from which the patients were selected were investigated using a questionnaire. State tuberculosis officers were interviewed about regional treatment policies. RESULTS: Two hundred and ninety-five internally displaced and 154 settled patients from respectively five and four clinics were included. The cure and completion rates among new sputum smear-positive cases were significantly higher among displaced (65% and 9.3%) than among settled persons (43.5% and 21%). Displaced groups had better adherence to 5 and 8 month smear examinations, and the quantity of health education was higher in the camps than among the settled population. CONCLUSION: Tuberculosis treatment among the displaced population in Khartoum compares favourably with the neighbouring settled population, but both groups still face serious challenges to fulfil the WHO goals.


Asunto(s)
Antituberculosos/uso terapéutico , Accesibilidad a los Servicios de Salud , Refugiados , Tuberculosis Pulmonar/tratamiento farmacológico , Organización Mundial de la Salud , Adulto , Femenino , Encuestas de Atención de la Salud , Humanos , Masculino , Cooperación del Paciente , Educación del Paciente como Asunto , Calidad de la Atención de Salud , Esputo/microbiología , Sudán , Resultado del Tratamiento
6.
Water Sci Technol ; 49(10): 41-9, 2004.
Artículo en Inglés | MEDLINE | ID: mdl-15259936

RESUMEN

Disposal of sewage sludge is forbidden and agricultural use of stabilized sludge will be banned in 2005 in Switzerland. The sludge has to be dewatered, dried, incinerated and the ashes disposed in landfills. These processes are cost intensive and lead also to the loss of valuable phosphate resources incorporated in the sludge ash. The implementation of processes that could reduce excess sludge production and recycle phosphate is therefore recommended. Partial ozonation of the return sludge of an activated sludge system reduces significantly excess sludge production, improves settling properties of the sludge and reduces bulking and scumming. The solubilized COD will also improve denitrification if the treated sludge is recycled to the anoxic zone. But ozonation will partly inhibit and kill nitrifiers and might therefore lead to a decrease of the effective solid retention time of the nitrifier, which reduces the safety of the nitrification. This paper discusses the effect of ozonation on sludge reduction, the operation stability of nitrification, improvement of denitrification and gives also an energy and cost evaluation.


Asunto(s)
Reactores Biológicos , Nitritos/química , Ozono/química , Aguas del Alcantarillado/química , Eliminación de Residuos Líquidos/métodos , Purificación del Agua/métodos , Agricultura , Conservación de los Recursos Naturales , Control de Costos , Fosfatos/química , Suiza , Factores de Tiempo , Eliminación de Residuos Líquidos/instrumentación , Purificación del Agua/instrumentación
7.
J Bone Joint Surg Br ; 84(1): 128-36, 2002 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-11837818

RESUMEN

We revised seven alumina-blasted cementless hip prostheses (Ti-alloy stems, cp Ti threaded sockets) with low- or high-carbon Co-alloy bearings at a mean of 20.1 months after implantation because of pain and loosening. Histological examination of the retrieved periprosthetic tissues from two cases in which the implant was stable and three in which the socket was loose showed macrophages with basophilic granules containing metal and alumina wear particles and lymph-cell infiltrates. In one of the two cases of stem loosening the thickened neocapsule also contained definite lymphatic follicles and gross lymphocyte/plasma-cell infiltrates. Spectrometric determination of the concentration of elements in periprosthetic tissues from six cases was compared with that of joint capsules from five control patients undergoing primary hip surgery. In the revisions the mean concentration of implant-relevant elements was 693.85 microg/g dry tissue. In addition to Cr (15.2%), Co (4.3%), and Ti (10.3%), Al was predominant (68.1%) and all concentrations were significantly higher (p < 0.001) than those in the control tissues. The annual rates of linear wear were calculated for six implants. The mean value was 11.1 microm (heads 6.25 microm, inserts 4.82 microm). SEM/EDXA showed numerous fine scratches and deep furrows containing alumina particles in loosened sockets, and stems showed contamination with adhering or impacted alumina particles of between 2 and 50 microm in size.


Asunto(s)
Artroplastia de Reemplazo de Cadera , Anciano , Aleaciones , Cobre , Femenino , Humanos , Cápsula Articular/patología , Macrófagos/patología , Masculino , Persona de Mediana Edad , Falla de Prótesis , Reoperación
8.
J Bone Joint Surg Br ; 82(6): 901-9, 2000 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-10990321

RESUMEN

We compared wear particles from two different designs of total hip arthroplasty with polycrystalline alumina-ceramic bearings of different production periods (group 1, before ISO 6474: group 2, according to ISO 6474). The neocapsules and interfacial connective tissue membranes were retrieved after mean implantation times of 131 months and 38 months, respectively. Specimen blocks were freed from embedding media, either methylmethacrylate or paraffin and digested in concentrated nitric acid. Particles were then counted and their sizes and composition determined by SEM and energy-dispersive x-ray analysis (EDXA). The mean numbers and sizes of most alumina wear particles did not differ for both production periods, but the larger sizes of particle in group 1 point to more severe surface destruction. The increased metal wear in group 2 was apparently due to alumina-induced abrasion of the stems. In this study the concentrations of particles in the periprosthetic tissues were 2 to 22 times lower than those observed previously with polyethylene and alumina/polyethylene wear couples.


Asunto(s)
Óxido de Aluminio/efectos adversos , Artroplastia de Reemplazo de Cadera/instrumentación , Cerámica/efectos adversos , Tejido Conectivo/patología , Prótesis de Cadera/efectos adversos , Falla de Prótesis , Adulto , Anciano , Biopsia , Microanálisis por Sonda Electrónica , Análisis de Falla de Equipo , Prótesis de Cadera/provisión & distribución , Humanos , Microscopía Electrónica de Rastreo , Persona de Mediana Edad , Tamaño de la Partícula , Diseño de Prótesis , Factores de Tiempo
9.
Z Orthop Ihre Grenzgeb ; 138(1): 69-73, 2000.
Artículo en Alemán | MEDLINE | ID: mdl-10730368

RESUMEN

ISSUE: Is a one stage procedures with open synovectomy and implantation of cemented knee endoprostheses in patients with empyemas of the knee and concomitant osteoarthritis suitable to reduce morbidity and increase early mobility? METHOD: Three female patients (age [symbol: see text] 88 yrs.) with acute empyema of arthritic knee joints (Pathogen: Staph. aureus) were treated with open synovectomies and implantations of cemented TKA's in a one stage procedure at mean 3 days (1-6) after admission. After 4.6 months (3-6) we carried out the last clinical and radiographic examination and after 15 months (11-21) a telephone questionnaire. RESULTS: The mean operation time was 2.4 hrs. (2-3), the perioperative blood loss at mean 1040 cc (800-1180 cc) and patients received 4 units blood (4-5). Parenteral antibiotics were administered for at mean 21 days (18-25 d). 33 days (27-39) after surgery the patients could be discharged, all of them ambulated with a walker, the operated joints had no signs of infection and blood parameters i.e. leucocyte count, crp and ESR dropped. At the time of the latest follow up examination there were neither clinical nor radiographic any signs that infections recurred. Two patients ambulated with crutches, one still with a walker. The HSS-Score increased from preop. 54 pts. up to 80 pts. at the follow up. CONCLUSION: Due to our results, a one stage procedure with open synovectomy and implantation of cemented total knee endoprostheses seems to be a considerable alternative for the treatment of geriatric patients suffering from knee empyema and severe osteoarthritis.


Asunto(s)
Artritis Infecciosa/cirugía , Artroplastia de Reemplazo de Rodilla , Empiema/cirugía , Osteoartritis de la Rodilla/cirugía , Infecciones Estafilocócicas/cirugía , Sinovectomía , Anciano , Anciano de 80 o más Años , Cementos para Huesos , Terapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Complicaciones Posoperatorias/diagnóstico por imagen , Radiografía
10.
Orthopedics ; 23(12): 1261-6, 2000 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-11144494

RESUMEN

This study compared migration in 73 cementless total hip arthroplasties (THAs) with either alumina ceramic (n = 23) or polyethylene (n = 50) inlays; prosthesis sockets and stems were identical except for the inlays. After 7 years of follow-up, 5 sockets (4 with ceramic and 1 with polyethylene inlays) had been revised an average of 63.3 months (range: 49-81 months) after implantation, and survival analysis showed a significantly higher revision rate for sockets with ceramic inlays (89 +/- 6%) versus polyethylene inlays (98.2 +/- 1.7%) (P = .032). Migration analysis of the first three postoperative years revealed significantly higher vertical migration in sockets with ceramic inlays (P = .047), in patients aged <60 years (P = .02), and in osteoporotic type C bone (A versus C, P = .0071 and B versus C, P = .0004).


Asunto(s)
Cerámica , Migración de Cuerpo Extraño , Prótesis de Cadera , Ensayo de Materiales , Polietileno , Estudios de Seguimiento , Humanos , Diseño de Prótesis , Factores de Tiempo
11.
Arch Orthop Trauma Surg ; 119(3-4): 232-5, 1999.
Artículo en Inglés | MEDLINE | ID: mdl-10392528

RESUMEN

In 12 human cadaver tibiae, osteotomies were carried out at two levels (2 and 3 cm from the distal joint line) with three different wedges (5 degrees, 10 degrees, 15 degrees) to evaluate the influence of displacement of the osteotomy fragments on areas of cortical contact. In undisplaced osteotomies (medical cortical edges superposed) cortical contact areas formed 28% (level 2 cm) and 40.5% (level 3 cm) of the cortical circumference of the proximal fragments (NS). Wedge angles and levels of osteotomy displayed no statistical differences. In displaced osteotomies cortical contact decreased significantly (P < 0.05). Displacing the distal fragment laterally, medial cortical contact is lost, and weight-bearing leads to revarisation as cancellous bone sustains only 3 MPa, and the measured compressive stresses at the medial edge amounted to 6 MPa on average. Displacing the distal fragment medially leads to a decrease of total cortical contact, too, but at the medial edge of the osteotomy cortical contact areas are still present. As a result of the study, postoperative weight-bearing without additional plaster cast fixation is recommended only in cases with undisplaced fragments.


Asunto(s)
Osteotomía/métodos , Tibia/cirugía , Cadáver , Humanos
12.
Z Orthop Ihre Grenzgeb ; 134(2): 125-30, 1996.
Artículo en Alemán | MEDLINE | ID: mdl-8779255

RESUMEN

41 draining sinuses after THR were treated by debridement without exchanging the implants. Positive bacterial cultures were obtained in 23 patients-the ilio tibial tract was found open in 19 cases (82%). The bacterial cultures were negative in 18 patients-the ilio tibial tract was found open in 7 cases (38%). Revisions took place 21 days (9-43) after primary implantation. At the time of the follow up (0 72 months postoperatively) 7 patients had been revised (8-97) because of recurrence of infection. The results of debridements of contaminated sinuses depend on the ilio tibial tract. Revisions were successful in three out of four paces with an intact, and only in eight out of nineteen cases with an open ilio tibial tract. Besides proper bacterial investigation preoperative sinograms give useful information about the prognosis of revision. In cases with positive bacterial cultures debridements should be done very early to eradicate infection without removing the implants.


Asunto(s)
Prótesis de Cadera , Complicaciones Posoperatorias/cirugía , Infección de la Herida Quirúrgica/cirugía , Anciano , Anciano de 80 o más Años , Infecciones Bacterianas/etiología , Infecciones Bacterianas/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/etiología , Pronóstico , Falla de Prótesis , Reoperación , Infección de la Herida Quirúrgica/etiología
13.
Arch Orthop Trauma Surg ; 113(2): 57-60, 1994.
Artículo en Inglés | MEDLINE | ID: mdl-8186048

RESUMEN

The first 100 consecutive cemented Weller-type total hip arthroplasties carried out between 1976 and 1977 were reexamined. Clinical analysis of 43 implants with more than 10 years follow-up showed that results were excellent in 21 patients (49%), good in 13 (30%), fair in 7 (16%) and poor in 2 (5%). Radiographic analysis revealed 33 (87%) stable femoral and 31 (81%) stable acetabular components. Statistical survivorship analysis of all 100 implants produced a probability of reoperation of 6.2%, a probability of radiographic implant loosening of 28.2% and a probability of radiographic loosening with clinical symptoms of 7.6%. The definition of failure should be "radiographic loosening of the prosthesis with clinical symptoms" because this evaluation seems to have the best clinical relevance. Concerning the fact that the cement fixation was not performed to today's standard, the results after more than 10 years with this total hip system (Weller long stem) are sufficient.


Asunto(s)
Prótesis de Cadera , Adulto , Anciano , Anciano de 80 o más Años , Cementos para Huesos , Femenino , Humanos , Artropatías/cirugía , Masculino , Persona de Mediana Edad , Falla de Prótesis , Estudios Retrospectivos , Resultado del Tratamiento
14.
Eur J Clin Invest ; 22(6): 403-6, 1992 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-1633835

RESUMEN

Chronic Granulomatous Disease (CGD) manifests as a predisposition to infection as a result of defective function of the NADPH oxidase of phagocytic cells. Proteins identified as part of this system include two subunits of a cytochrome b (cytochrome b-245) and two cytosolic factors. The affected oxidase component was determined in 63 CGD patients from 57 families, by Western blotting of extracts of their neutrophils with antibodies to those proteins. 38 (67%) of the families were X-linked with a defect of the beta subunit of the cytochrome. 13 (23%) lacked p47-phox, 3 (5%) p67-phox, and 3 (5%) the alpha subunit of the cytochrome.


Asunto(s)
Enfermedad Granulomatosa Crónica/enzimología , NADH NADPH Oxidorreductasas/genética , Secuencia de Aminoácidos , Western Blotting , Europa (Continente) , Femenino , Enfermedad Granulomatosa Crónica/genética , Humanos , Masculino , Datos de Secuencia Molecular , NADPH Oxidasas
15.
Orthopedics ; 14(6): 661-6, 1991 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-1866347

RESUMEN

Thirty-three patients were operated for septic (n = 20) and aseptic (n = 13) loosening of their hip prostheses using Girdlestone's operation over 10 years. An average of 46.6 months later, 22 of these patients were given a follow-up examination. Clinical evaluation according to Harris showed an improvement in mean values from 25 to 53 points, which can be attributed mainly to a marked reduction in pain, as function remained poor and the patients still depended on walking aids. The infection ceased in 92% of the patients with septic prosthesis loosening.


Asunto(s)
Artroplastia/métodos , Prótesis de Cadera , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Seguimiento , Articulación de la Cadera/diagnóstico por imagen , Articulación de la Cadera/fisiología , Humanos , Masculino , Persona de Mediana Edad , Osteítis/cirugía , Falla de Prótesis , Radiografía , Rango del Movimiento Articular , Reoperación
16.
Hum Genet ; 80(1): 85-9, 1988 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-3417309

RESUMEN

The clinical, biochemical, and molecular analysis of a patient with chronic granulomatous disease (CGD), retinitis pigmentosa (RP), and McLeod phenotype and of his parents demonstrated the X-linked transmission of these three traits in this family and a deletion of the entire X-CGD gene of the patient DNA. All but one other DNA markers tested, including those in Xp21, were present. These findings strongly suggest that the McLeod locus and at least one XL RP gene are closely linked to the X-CGD locus in the Xp21 region of the human X chromosome.


Asunto(s)
Deleción Cromosómica , ADN/genética , Enfermedad Granulomatosa Crónica/genética , Retinitis Pigmentosa/genética , Cromosoma X , Preescolar , Bandeo Cromosómico , Enfermedad Granulomatosa Crónica/complicaciones , Humanos , Cariotipificación , Masculino , Fenotipo , Retinitis Pigmentosa/complicaciones , Aberraciones Cromosómicas Sexuales
17.
Z Orthop Ihre Grenzgeb ; 125(4): 375-81, 1987.
Artículo en Alemán | MEDLINE | ID: mdl-3673191

RESUMEN

The acetabular socket "Model Gersthof" is a spheric polyethylene component which can be anchored cement-free by means of three symmetrically arranged pegs at the outside. Between July 1979 and October 1986, 1316 implants of cement-free sockets were performed at our own department. The evaluation of the socket was based on the criterium of radiological instability (= aseptic loosening). After five years of observation, this design-oriented evaluation of success shows a survival of 92% +/- 2.9%.


Asunto(s)
Articulación de la Cadera/cirugía , Prótesis de Cadera , Osteoartritis/cirugía , Polietilenos , Acetábulo/patología , Adulto , Anciano , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/patología , Diseño de Prótesis , Falla de Prótesis , Reoperación
18.
Arch Orthop Trauma Surg (1978) ; 106(5): 297-300, 1987.
Artículo en Inglés | MEDLINE | ID: mdl-3632315

RESUMEN

Follow-up examinations of 67 implants of cement-free ceramic sockets show the need for an exact definition of failure, to warrant comparable evaluations of results. Statistical survival analysis offers the possibility of presenting both the incidence of failures and the dates of their occurrence. If only revision surgery with removal of the socket is considered to be a failure, our material shows the "survival quota" of the ceramic socket after 8 years to be 96.7% +/- 2.2%. If radiological signs of loosening are included in the evaluation of failures, the "survival quota" of the stable implants is reduced to 81.9% +/- 6.9%.


Asunto(s)
Cerámica , Prótesis de Cadera/normas , Acetábulo , Humanos , Falla de Prótesis
19.
J Clin Immunol ; 6(2): 136-45, 1986 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-3011845

RESUMEN

Twenty-five patients suffering from chronic granulomatous disease (CGD) and their families were investigated. Defects in the superoxide generating system were characterized at the level of the heme-containing cytochrome b and of the FAD-containing flavoprotein, both localized in the plasma membrane of granulocytes. It was confirmed that in most of the typical cases (18 of 22), the complete inability of superoxide generation was associated with the absence of detectable cytochrome b. Mothers but not fathers of such male patients were characterized by a diminished content of cytochrome b, confirming that the affected gene is localized on the X chromosome. In contrast, the granulocytes of four other typical patients (two female and two male) contained normal amounts of cytochrome b, whereas oxidative activity was absent. Since no abnormality of oxidative activity as well as of cytochrome b was found in granulocytes of the mothers and fathers of these patients, an autosomal recessive mode of inheritance of the disease is probable. The flavoprotein deficiency found in the granulocytes of four male patients was always associated with an absence of detectable cytochrome b. This could indicate a structural relationship between flavoprotein and cytochrome b (e.g., a flavocytochrome). Three further patients with mild X-linked CGD contrasted with the patients with severe or classic X-linked disease; the oxidative activity of their phagocytes was diminished but not absent, and the cytochrome b present, albeit in small amounts.


Asunto(s)
Grupo Citocromo b/sangre , Flavoproteínas/sangre , Enfermedad Granulomatosa Crónica/clasificación , Adolescente , Preescolar , Grupo Citocromo b/deficiencia , Femenino , Flavina-Adenina Dinucleótido/sangre , Flavoproteínas/deficiencia , Genes Recesivos , Ligamiento Genético , Granulocitos/metabolismo , Enfermedad Granulomatosa Crónica/sangre , Enfermedad Granulomatosa Crónica/genética , Humanos , Técnicas In Vitro , Lactante , Recién Nacido , Masculino , Nitroazul de Tetrazolio/metabolismo , Superóxidos/sangre , Cromosoma X
20.
Eur J Immunol ; 16(2): 205-8, 1986 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-3514236

RESUMEN

Membrane and intracellular processing of the LFA-1 macromolecular complex, known to be involved in cytolytic function of T lymphocytes, was investigated in a child with recurrent bacterial infections, impaired natural killer activity, T cell-mediated lymphocytolysis and absent adhesion and migration of phagocytic cells. Monoclonal antibodies to the LFA-1 alpha and beta subunits, able to precipitate the LFA-1 alpha, 180-kDa chain, the p151 chain and beta 94-kDa chain (shared by both alpha chains), were used in immunoprecipitation studies of patient and control phytohemagglutinin-blasts. Neither of the alpha chains nor the beta chain were found in precipitates obtained from 125I-surface-labeled patient cells in contrast to controls. However, the precursor of the LFA-1 alpha chain, a 170-kDa polypeptide, was identified in lysates of biosynthetically labeled patients' cells. These results suggest that the defective membrane expression of the LFA-1 complex may be secondary to the absence of the mature beta chain.


Asunto(s)
Antígenos de Superficie/biosíntesis , Infecciones Bacterianas/inmunología , Antígenos de Superficie/análisis , Antígenos de Superficie/inmunología , Infecciones Bacterianas/etiología , Femenino , Humanos , Antígeno-1 Asociado a Función de Linfocito , Metionina/metabolismo , Recurrencia , Radioisótopos de Azufre
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