RESUMEN
A 3-year-old boy presenting with convulsions and carpopedal spasm had hypomagnesemia and hypermagnesuria due to congenital magnesium-losing nephropathy. Despite chronic oral and intermittent intravenous magnesium supplementation, he remained chronically hypomagnesemic. At age 4, he developed a progressive proximal myopathy and dilated hypertrophic cardiomyopathy that ultimately contributed to his death at age 14 years. Skeletal and cardiac muscle specimens showed a mitochondrial myopathy with increased numbers of enlarged, structurally abnormal mitochondria. Muscle magnesium content was markedly decreased. Chronic oral and intermittent intravenous magnesium supplementation may be inadequate to prevent the progressive cardioskeletal myopathy associated with the chronic magnesium deficiency of congenital magnesium-losing nephropathy.
Asunto(s)
Cardiomiopatías/etiología , Deficiencia de Magnesio/complicaciones , Mitocondrias Cardíacas/ultraestructura , Mitocondrias Musculares/ultraestructura , Enfermedades Musculares/etiología , Biopsia , Cardiomiopatías/patología , Preescolar , Enfermedad Crónica , Humanos , Deficiencia de Magnesio/patología , Masculino , Microscopía Electrónica , Enfermedades Musculares/patologíaRESUMEN
Epstein-Barr virus (EBV) infection in a family resulted in a fatal disseminated heterophil negative infectious mononucleosis syndrome in a nine-year-old girl. This was followed closely by a similar disease process in her six-year-old brother which evolved over a one-year period into Stage IIIB Hodgkin's disease. Finally, three years after the index EBV case in the daughter, the mother was diagnosed with a non-Burkitt's-type undifferentiated lymphoma that proved rapidly fatal. The EBV involvement in the sister and brother was well documented serologically and virologically. The pathologic diagnosis was established and confirmed by more than one pathologist. There was no obvious evidence for either a specific or general immune defect in any of the family members tested. The progression of the six-year-old boy's EBV infection from a benign, yet disseminated disease process into a histopathologically confirmed case of Hodgkin's disease offers a strong suggestion that this virus was not behaving solely as a passenger. Especially relevant is the fact that the boy never fully recovered from his EBV infection and essentially became persistently infected with the virus as evidenced by his EBV-EA serology and virology results.
Asunto(s)
Enfermedad de Hodgkin/genética , Mononucleosis Infecciosa/genética , Linfoma/genética , Adulto , Anticuerpos Antivirales/análisis , Niño , Femenino , Herpesvirus Humano 4 , Enfermedad de Hodgkin/inmunología , Humanos , Inmunoglobulinas/análisis , Mononucleosis Infecciosa/inmunología , Hígado/patología , Ganglios Linfáticos/patología , Linfocitos/inmunología , Linfoma/inmunología , Masculino , Formación de RosetaRESUMEN
This is a case report of a patient treated at West Virginia University Medical Center that illustrates the successful use of preoperative percutaneous angiographic embolization of a pheochromocytoma in controlling a malignant hypertensive crisis.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Glándulas Suprarrenales/irrigación sanguínea , Embolización Terapéutica , Feocromocitoma/cirugía , Cuidados Preoperatorios , Adolescente , Neoplasias de las Glándulas Suprarrenales/irrigación sanguínea , Neoplasias de las Glándulas Suprarrenales/fisiopatología , Presión Sanguínea , Cateterismo , Esponja de Gelatina Absorbible/uso terapéutico , Humanos , Hipertensión Maligna/fisiopatología , Hipertensión Maligna/terapia , Masculino , Feocromocitoma/irrigación sanguínea , Feocromocitoma/fisiopatologíaRESUMEN
A patient had recurrent pyogenic infections, chronic mucocutaneous candidiasis, and repeated long-bone fractures. Her disorder was diagnosed as hyper-IgE syndrome on the basis of clinical data, elevated levels of serum IgE, increased levels of IgE specific for Staphylococcus aureus, and impaired T-lymphocyte function. Roentgenograms confirmed the diagnosis of osteogenesis imperfecta tarda. The estimated likelihood of both conditions occurring in the same person is approximately one in 10 billion. The coincident finding of bone dysplasia in a patient with impaired T-lymphocyte function suggests a common mechanism for birth defects.
Asunto(s)
Hipergammaglobulinemia/complicaciones , Inmunoglobulina E , Osteogénesis Imperfecta/complicaciones , Niño , Femenino , Humanos , Hipergammaglobulinemia/etiología , Inmunoglobulina E/análisis , Osteogénesis Imperfecta/etiología , Linfocitos T/fisiologíaRESUMEN
An influenza surveillance program developed and conducted in three districts in Israel during winter 1976--77, was based mainly on morbidity data in the general population, corroborated by sero-epidemiologic surveys on selected groups. This information was supplemented by data on mortality organized according to specific age groups. During the period under study, similar results were observed in each of the three districts surveyed. Two successive waves of influenza were recorded: an early wave due to B/Hong Kong/5/72 followed by an A/Victoria/3/75 outbreak. Both waves were of moderate extent, with the highest frequency of clinical influenza occurring in the youngest group, age 0--14 years. The age-specific mortality rates were highest and rather similar in the extreme age groups 65+ and 0. The search of A/New Jersey/8/76 antibody revealed a considerable proportion of positives with a higher titer in the older age groups. A special serologic survey among Yemenite Jews over 50 years of age, who immigrated to Israel in 1949, showed that the 1918 influenza pandemic also reached that isolated country. The surveillance program provided an early warning system as well as a rather accurate measurement of influenza impact in Israel.
Asunto(s)
Brotes de Enfermedades/epidemiología , Encuestas Epidemiológicas , Gripe Humana/epidemiología , Adolescente , Adulto , Factores de Edad , Anciano , Anticuerpos Antivirales/análisis , Niño , Preescolar , Humanos , Lactante , Gripe Humana/inmunología , Gripe Humana/mortalidad , Israel , Judíos , Persona de Mediana Edad , Orthomyxoviridae/inmunología , Yemen/etnologíaRESUMEN
The main objectives of the monitoring were: (1) to define the prevalent virus involved in influenza activity: (2) to determine the time of its occurrence; and (3) to evaluate its extent and impact. A sudden rise in the frequency of visits associated with acute respiratory conditions in the age group 0-14 to the emergency rooms of 14 hospitals throughout the country, correlated well with the start of influenza B/Hong Kong activity; this was simultaneously attested by two different laboratories. The later and sporadic occurrence of influenza A/Victoria activity did not affect the usual trend observed in the frequency of visits to emergency rooms. An age group stratified serologic follow-up pointed to a progressively increasing rate of influenza B infection up to the age group 15-24. A crude morbidity rate of 13-18% was recorded during influenza B outbreaks in several agricultural settlements. The percentage distribution of cases was about 15,21, 26 and 37 in the age groups 0-4, 5-9, 10-14 and 15+, respectively. The mortality associated with influenza B and influenza A activity did not exceed the expected rate.
Asunto(s)
Gripe Humana/epidemiología , Adolescente , Adulto , Factores de Edad , Anciano , Niño , Preescolar , Brotes de Enfermedades , Hospitales Generales , Humanos , Lactante , Virus de la Influenza A/inmunología , Virus de la Influenza A/aislamiento & purificación , Gripe Humana/microbiología , Israel , Persona de Mediana Edad , Vigilancia de la Población , Enfermedades Respiratorias/epidemiología , Enfermedades Respiratorias/mortalidad , Estaciones del AñoRESUMEN
Two inactivated influenza-virus vaccines were tested and compared in three army training units in Israel. The serological responses to the vaccines and the side-effects were assessed. The vaccines contained the influenza strains which were prevalent in 1974: A2/Port Chalmers/1/73 and B/Hong Kong/8/73. One of the vaccines also contained A2/England/42/72. Both vaccines caused a more than three-fold rise in geometric mean titers against influenza A strains, and about a twofold rise in geometric mean titers against influenza B/Hong Kong/5/73. Approximately 75%-80% of the vaccinees acquired protective hemagglutination-inhibition antibody titers against influenza A strains, while less than 30% acquired protective titers against B strains. In general, there were no significant differences between the serological responses to the two vaccines. More than 50% of the vaccinees experienced at least one systemic side-effect (50.3% with one vaccine and 61.0% with the other). The average number of side-effects per person was between 1.78 and 2.11. However, these side-effects were generally of short duration and caused minimal disability. On the whole, the two vaccines did not differ significantly with regard to the side-effects they caused.
Asunto(s)
Formación de Anticuerpos , Vacunas contra la Influenza/efectos adversos , Gripe Humana/prevención & control , Medicina Militar , Vacunación , Humanos , Vacunas contra la Influenza/administración & dosificación , Gripe Humana/inmunología , IsraelAsunto(s)
Vacuna Antisarampión/administración & dosificación , Vacuna contra la Parotiditis/administración & dosificación , Vacuna contra la Rubéola/administración & dosificación , Vacunación , Anticuerpos Antivirales/análisis , Formación de Anticuerpos , Niño , Preescolar , Ensayos Clínicos como Asunto , Fiebre/etiología , Pruebas de Inhibición de Hemaglutinación , Humanos , Lactante , Israel , Vacuna Antisarampión/efectos adversos , Virus del Sarampión/inmunología , Vacuna contra la Parotiditis/efectos adversos , Virus de la Parotiditis/inmunología , Pruebas de Neutralización , Vacuna contra la Rubéola/efectos adversos , Virus de la Rubéola/inmunología , Vacunas Atenuadas/administración & dosificaciónAsunto(s)
Infecciones por Rickettsia/inmunología , Rickettsia/inmunología , Adulto , Animales , Reacciones Antígeno-Anticuerpo , Embrión de Pollo , Pruebas de Fijación del Complemento , Reacciones Cruzadas , Perros , Femenino , Técnica del Anticuerpo Fluorescente , Cobayas , Humanos , Sueros Inmunes , Israel , Conejos , Rickettsia/aislamiento & purificación , Rickettsia rickettsii/inmunología , Fiebre Maculosa de las Montañas Rocosas/inmunologíaAsunto(s)
Enfermedades en Gemelos , Tumor de Células de Leydig/complicaciones , Neoplasias Testiculares/complicaciones , Preescolar , Humanos , Tumor de Células de Leydig/patología , Tumor de Células de Leydig/cirugía , Masculino , Pubertad Precoz/etiología , Neoplasias Testiculares/patología , Neoplasias Testiculares/cirugía , Testosterona/sangreAsunto(s)
Fiebre Q/epidemiología , Agricultura , Animales , Bovinos/inmunología , Pruebas de Fijación del Complemento , Coxiella/inmunología , Reservorios de Enfermedades , Vectores de Enfermedades , Exposición a Riesgos Ambientales , Femenino , Humanos , Humedad , Israel , Tamizaje Masivo , Fiebre Q/diagnóstico , Instituciones Académicas , Ovinos/inmunología , TemperaturaAsunto(s)
Virus de la Rubéola/inmunología , Rubéola (Sarampión Alemán)/inmunología , Vacunas Virales , Adolescente , Adulto , Factores de Edad , Animales , Formación de Anticuerpos , Niño , Patos , Femenino , Fiebre/inducido químicamente , Pruebas de Inhibición de Hemaglutinación , Humanos , Israel , Artropatías/inducido químicamente , Linfadenitis/inducido químicamente , Menstruación , Vacuna contra la Rubéola/efectos adversos , Factores de Tiempo , VacunaciónRESUMEN
Abstract A study on recrudescent typhus was conducted among 294 volunteers most of whom had contracted typhus in Eastern Europe and who immigrated to Israel after the Second World War. The main aim of the study was to determine whether persons, once infected, might subsequently suffer relapses without clinical signs which could presumably serve as sources of the microorganism in the interepidemic period and might be responsible for the initiation of new epidemics. The selection of volunteers was based on the reliability of their histories and on the absence of murine typhus in their present environment. Those under study were interviewed at regular 2-3 months' intervals during which blood samples were taken from them. Six blood samples were obtained from 262 individuals and at least 3 samples from the rest. Complement-fixation and microagglutination tests were carried out in order to establish the presence of antibodies specific for epidemic typhus. In 12% of the volunteers no demonstrable antibodies were found, while 67% considered to have such antibodies showed no significant titer fluctuations. However, 21% of the volunteers exhibited 4- to 8-fold fluctuations in titer in at least one of the tests used-a result which suggests the possibility of serological relapse.
Asunto(s)
Virus de la Rubéola , Rubéola (Sarampión Alemán)/inmunología , Vacunas Virales , Adolescente , Adulto , Factores de Edad , Animales , Anticuerpos/análisis , Formación de Anticuerpos , Temperatura Corporal , Niño , Cricetinae , Técnicas de Cultivo , Patos , Embrión de Mamíferos , Embrión no Mamífero , Eritrocitos , Exantema/etiología , Femenino , Pruebas de Inhibición de Hemaglutinación , Pruebas de Hemaglutinación , Humanos , Israel , Riñón , Linfadenitis/etiología , Menstruación , Métodos , Neuralgia/etiología , Vacuna contra la Rubéola/efectos adversos , Factores de Tiempo , Vacunación , Cultivo de VirusRESUMEN
While an earlier live measles vaccine induced a high degree of immunity it also caused clinical reactions of variable severity in some vaccinated children. A number of attenuated measles vaccine strains have been developed to avoid these problems, and this paper reports the results of small-scale trials with 3 attenuated measles vaccines and a new further-attenuated vaccine on children from 9 months to over 4 years old.All 4 vaccines produced a satisfactory serological response. The new further-attenuated vaccine (Moraten) produced less fever and fewer rashes in the vaccinated children than the other vaccines which had been extensively tested by other workers. The incidence of severe reactions and complications was low with all vaccines.