RESUMEN
The authors present the case of a child with recurrent infections since the age of 4 months, including bilateral pneumonia by Pneumocystis carinii and protracted varicella. Serum immunoglobulin values (when 10 months old), and B cell values were normal. There was persistent lymphocytic leucocytosis, near absence of CD8+ cells, and an increased CD4/CD8 ratio. The percentage of activated T cells and the expression of HLA class I were normal. Proliferation, activation and IL-2 synthesis studies in T cells showed a TCR/CD3-associated signal transduction deficit. ZAP-70 cDNA sequencing showed a mutation, and no ZAP-70 protein was detected in T cells. ZAP-70 deficiency is associated with a rare immune deficiency with absence of CD8+ T cells as well as a functional deficiency in T cells. Seven months after bone marrow transplantation the child is clinically well and immunologically recovered.
Asunto(s)
Gránulos Citoplasmáticos , Síndromes de Inmunodeficiencia/inmunología , Glicoproteínas de Membrana/deficiencia , Femenino , Proteínas Ligadas a GPI , Humanos , LactanteRESUMEN
In an attempt to discern the paradigms underlying our medical practice (more specifically paediatrics) over the last 30 years, it was found that these are rather similar to the ones prevailing at the beginning of this period--all wrong-doings were attributed to outside forces which had to be neutralized; the obsession to standardise the internal milieu; the compulsion to always do something, little attention being paid to collateral effects; the ends justifying the means and the charm of technology.
Asunto(s)
Pediatría/historia , Actitud del Personal de Salud , Infecciones Bacterianas/historia , Niño , Deshidratación/historia , Ecología , Fluidoterapia/historia , Historia del Siglo XX , Humanos , Portugal , Terapéutica/historia , Equilibrio HidroelectrolíticoRESUMEN
AIM: To determine immunocytochemically whether preterm and newborn infants with necrotising enterocolitis (NEC) show differences in numbers of lysozyme positive Paneth cells compared with normal controls, and to relate the findings to the possibility that lysozyme deficiency may facilitate the bacterial infections thought to be associated with this condition. METHODS: Tissues from 10 infants with NEC and from 11 matched controls were sectioned and stained immunocytochemically for lysozyme. Differences in the numbers of Paneth cells and degree of lysozyme positivity in the tissues were assessed. RESULTS: Tissues from NEC patients showed no, or very few, lysozyme positive Paneth cells, whereas controls showed strong positive staining. CONCLUSIONS: A deficiency or developmental defect in Paneth cells, resulting in an absence of lysozyme, may render the intestine more susceptible to bacterial infection, allowing organisms to adhere and translocate across the mucosa. Such enhancement of infection may contribute to the pathogenesis of NEC.
Asunto(s)
Enterocolitis Necrotizante/enzimología , Muramidasa/análisis , Células de Paneth/enzimología , Infecciones Bacterianas/complicaciones , Biomarcadores/análisis , Enterocolitis Necrotizante/etiología , Humanos , Inmunohistoquímica , Recién Nacido , Intestino Delgado/enzimologíaRESUMEN
UNLABELLED: Neurobrucellosis is an uncommon disease in children. The authors present two cases of brucellar meningo-encephalitis. Headache and vomiting were the main complaints and one child had also some behavioural disturbance as well as papilloedema and sixth cranial nerve palsy. The clinical diagnosis was suggested by epidemiological data in both cases. Blood and CSF cultures confirmed brucellar aetiology in one of the cases and positive serum and CSF specific antibodies in both. Clinical course was favourable after treatment with doxycycline, rifampicin and streptomycin. No relapse occurred and there were no sequelae. CONCLUSION: Neurobrucellosis should be considered in the differential diagnosis of neurobehavioural disturbance of children living in areas where brucellosis is endemic.
Asunto(s)
Brucelosis/diagnóstico , Meningoencefalitis/diagnóstico , Brucelosis/tratamiento farmacológico , Niño , Doxiciclina/uso terapéutico , Humanos , Masculino , Meningoencefalitis/tratamiento farmacológico , Rifampin/uso terapéutico , Estreptomicina/uso terapéuticoRESUMEN
The height values of 122 boys and 124 girls, 7 to 11 years old, from 19 primary schools of the central area of Portugal were evaluated in 1988. The values from boys of all ages and girls younger than 9 years were normally spread, according to american (NCHS-USA 1976) standards. Girls older than 9 years were relatively shorter which could be related to the late puberty of these country girls. These figures are about 4 cm higher than those collected from portuguese school children born in 1964.
Asunto(s)
Estatura , Factores de Edad , Niño , Femenino , Humanos , Masculino , Portugal , Factores de TiempoRESUMEN
In this study the authors analysed the evolution of blood urea nitrogen in 14 infants during treatment of hypernatremic dehydration. BUN values decreased at a low rate comparing with usual standard limits: a) a correlation between initial BUN and natremia was observed, b) in 9 out of 14 infants there was a transient elevation of BUN, between 5 to 24 hours after the beginning of rehydration, and c) renal function was normal in 8 children who were reevaluated 3 to 28 months after the episode of dehydration.
Asunto(s)
Lesión Renal Aguda/sangre , Nitrógeno de la Urea Sanguínea , Deshidratación/sangre , Hipernatremia/sangre , Lesión Renal Aguda/etiología , Deshidratación/complicaciones , Femenino , Humanos , Hipernatremia/complicaciones , Lactante , Recién Nacido , MasculinoRESUMEN
A 3-month-old child with a grossly symmetrical monomorphic papular acral-located skin eruption, a voluminous lymphadenopathy post-BCG vaccination, and hepatomegaly was seen. The diagnoses of Gianotti-Crosti's syndrome and generalized tuberculous infection have been discarded. The appearance of these lesions 48 hours after a tuberculin patch test, their tuberculoid structure, and the absence of any systemic involvement favored the diagnosis of papular tuberculids, a rarely reported complication of BCG vaccination.