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Biomedical subjects

A Silva

Publications and source records attributed to A Silva.

At least 217 records · Page 12Linked to original sources
PubMedDate not supplied

[Young-onset multiple sclerosis].

INTRODUCTION: Young onset multiple sclerosis is an infrequent situation which may present with atypical symptoms and uncertain outcome. OBJECTIVE: Our aim was to assess the clinical presentation and course in young onset multiple sclerosis, and analyze eventual data which might be helpful in establishing its prognosis. PATIENTS AND METHODS: We have retrospectively reviewed the clinical protocols of 17 patients with young onset multiple sclerosis, defined as presentation of symptoms before 21 years. Diagnosis was made according to Poser's criteria including clinical features, magnetic resonance imaging, cerebrospinal fluid findings, and evoked potentials. RESULTS: The mean age at onset was 16.9 +/- 4.4 and median time to diagnosis was four weeks. The clinical course was relapsing-remitting in 76.5% and secondary progressive in 23.5%. The mean annual exacerbation rate was 1.5 +/- 0.9 and median time to second exacerbation was 12 months. The actual Expanded Disability Status Scale score is 2.6 +/- 2 after a mean disease duration of 11.4 +/- 8.0 years. The correlation between the Expanded Disability Status Scale score and the mean disease duration was the only statistically significant result. CONCLUSIONS: These results are similar to other studies, namely, age at onset did not correlate with final neurological disability. However, we must emphasize that any primary progressive form was found in our study. We conclude that in young onset multiple sclerosis, progression is not dependent on the age of onset and does not necessarily lead to an unfavorable outcome.

Adolescent↗
PubMedDate not supplied

[Glucose-6-phosphate dehydrogenase deficiency in 2 girls].

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common erythrocyte enzymopathy, affecting over 400 million people worldwide. Portugal is a low prevalence country, but immigrants from endemic regions are common, particularly in the south of the country. In the present study, we report the laboratory findings observed in two black proband children with low G6PD enzyme activity (23 and 18%). The study also included their first-degree relatives. Both biochemical parameters (enzyme activity, electrophoretic mobility and cytochemical test) and genetic determinations (mutation and haplotype characterisation) were performed.

Base Sequence↗
PubMedDate not supplied

[Interface neurology/psychiatry: a case report of neurosyphilis].

Organic diseases can occur with different psychiatric symptoms. Neurosyphilis was considered to be a landmark in the history of organic mental syndromes. The complexity of its clinical picture decreases the boundaries between Neurology and Psychiatry and requires a multidisciplinary approach. We report a case of neurosyphilis that began with psychiatric symptoms in a twenty five years old male.

Adult↗
PubMedDate not supplied

[Celiac disease and its diagnostic evolution. Comparisons and experiences in a hospital pediatric department (1975-1992). I].

The coeliac disease (CD) or gluten-sensitive enteropathy (GSE) is a permanent intolerance to wheat gliadin and to correlated proteins inducing malabsorption and typical damages of the jejunal mucosa (total or subtotal villous atrophy = SVA) in genetically-predisposed individuals ("DQW2"). A large amount of research has been devoted to CD pathogenesis: the most recent studies, thanks to sophisticated and experimental methods, support the pathogenetic immunological theory and the one of direct cytotoxicity. The correct diagnostic procedure for CD, established in 1970 by the European Society for Pediatric Gastroenterology and Nutrition (ESPGAN), suggested three small bowel mucosal biopsies. In the last years, because of the difficulties of such a practice, the necessity of non-invasive diagnostic approaches has developed; such approaches have been verified in absorption tests (one-hour blood xylose, intestinal permeability methods) and in immunogenetic tests (antibodies antigliadin, anti-reticulin, anti-endomysium, anti 90 KD glycoprotein, anti-human jejunum, HLA I/II antigens). The specific MHC antigens establish CD's incidence in several population and in particular situations, as in first-degree relatives and in diseases associated with CD (dermatitis herpetiformis (DH), insulin dependent diabetes mellitus (IDDM) and other auto-immune syndromes). The specific serum antibodies singly used as first level screening if estimated in combination with absorption tests, reach the highest levels of specificity and sensibility in CD diagnosis. It's anyway fundamental the comparison with at least a typical CD histological feature, caused by a challenge with a sufficient gluten to be carried in dubious cases and in non high auxological risk age (ESPGAN 1989). Adolescence is a period of frequent non compliance with a gluten-free diet and of particular psychological and physical problems: the apparent "gluten insensitivity", typical of teen-agers and adults, recalls the definitions of silent CD and latent CD (iceberg like). In the first case the jejunal mucosa is abnormal and the symptomatology isn't evident. In latent CD, genetically restricted, the mucosa is normal but there are minimal markers of inappropriate immunity to gliadin (at intestinal humoral immunity level) and a possible worsening of histological lesions to the third stage under environmental stimuli. This represents a two-stage model CD. That's why CD is still under-evaluated despite recent statistics reporting an increasing incidence (late and atypical forms). Prevalence rates between 1:300 and 1:4,000 and more are quoted in literature. The necessity of a strict gluten-free diet is confirmed by the evident frequency of lymphoma and by the increased risk of malignancy in untreated CD.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMedDate not supplied

[Isolation of Shiga-toxin-producing Escherichia coli strains during a gastrointestinal outbreak at a day care center in Mar del Plata City].

From October 15 to November 8, 2003, a gastrointestinal outbreak occurred at a day care center in a Hospital in Mar del Plata City. Fourteen out of 80 (17.5%) children, mean age 23.6 +/- 13.9 months, and the mother of one of them had diarrhea. One case developed hemolytic uremic syndrome. No conclusive evidence of the origin of the outbreak was found, but the epidemic curve suggested person-to-person spread. The usual practices at the place where infant milk formula was prepared at the day care center, together with the inadequate infrastructure conditions and hygiene practices at the kitchen of the hospital, were considered risk factors. One case had Shiga toxin-producing Escherichia coli (STEC) O103:H2 infection and other STEC O26:H11. The duration of shedding for the child with O26:H11 infection was 37 days. In the other symptomatic children, the pathogen was not recovered from fecal samples collected 6 or more days after the onset of the illness. This emphasizes that the collection of early samples is necessary to recover STEC strains. In order to prevent and control enteric diseases in day care facilities the following measures are necessary: optimal hygiene standards, early case reporting, and exclusion of those who remain culture-positive.

Adult↗
PubMedDate not supplied

[Chronic diseases in childhood: an approximate measurement of their impact].

As an approach to measurement of the importance of chronic diseases in childhood, type and frequency of diagnoses in children admitted to the pediatric wards of a general metropolitan hospital at Santiago, Chile, were reviewed and recorded along hospitalization and at the time of discharge from March 1 through June 30, 1989 (n: 426). Newborns were excluded. Main problems of the study were lacks of uniform national criteria to define chronic illness and of modern technology to certify diagnoses. Ninety five hospital discharged children (23.3%) were considered to have a definitive, confirmed chronic disease (CCD), other 51 (12%) were cases of possibly chronic disease (PCD) while the remainder were thought to be carriers of acute illness. Among 146 patients taken as CCD or PCD cases, 126 (87.7%) were considered to have single organic system diseases as defined by areas of medical interest or specialty; other 3 cases (8.9%) had two affected systems and 5 children (3.4%) had three or more involved systems. More frequently affected systems (in number of cases) and their corresponding proportions of CCD were as follows: neurologic (31 cases and 58.1% CCD), oncohematologic (28 cases and 96.4% CCD) and gastrointestinal (26 cases and 26.9% CCD). More extensive studies, covering other medical care providing settings are desirable and necessary to measure the magnitude and features of chronic disease entities in chilean childhood.

Age Factors↗
PubMedDate not supplied

[Hemoglobin H disease. Presentation of a case].

Haemoglobin H (Hb H) disease, the most important clinical form of alpha-thalassaemia, shows remarkable clinical variability. Hb H si an unstable tetramer of beta-globin chains which accumulates because of the lack of adequate numbers of alpha-globin chains and precipitates in the red cells, causing their premature destruction. A case of Hb H disease in a 9-yr-old child, admitted into hospital for acute haemolysis after use of pyrazolone derived, is presented. Haematologic data with synthesis in vitro of globin chains were obtained from the parents and sister. The clinical and haematologic features of this form of haemoglobinopathy are briefly discussed in the light of recent knowledges of his genetic mechanism of transmission.

Anti-Inflammatory Agents↗
PubMedDate not supplied

[Indications and results of surgical treatment in gastroesophageal reflux and hiatal hernia].

It is well known that closure of the cardia is incomplete in about 25-30% of all infants; the GER is a direct consequence. Roughly two-thirds of these infants do not show symptoms and only one-third become symptomatic. The symptoms are mild in about 75% of the symptomatic children; no treatment or medical treatment by pediatrician is required. In the remaining 25% the symptoms are moderate or severe and the clinical treatment is necessary. About the 85% of these children are cured with conservative treatment and only 15% of this small remaining group require surgery. In the paper the diagnostic problems and indications for surgery are considered. The Authors report the results of 66 children operated on for GER without (44 children) and with (22 children) hiatus hernia. The operative technique was gastropexy according to Boerema plus retroesophageal hiatopexy in the cases of important hiatus hernia. At the follow-up 61 children (92.5%) were completely asymptomatic and three showed mild symptoms without pathological radiological findings. Clinical and radiological recurrences occurred in two patients (4.5%) with severe brain damage. Two children were reoperated on postoperatively for an ileus due to adhesion. The mortality rate has been zero. In the author's opinion, the Boerema procedure is a simple, physiologic and fast technique, associated with very few complications and no mortality rate and should be considered the elective method in the surgical treatment of GER and hiatal hernia in pediatric patients.

Child, Preschool↗
PubMedDate not supplied

[Posterior urethral valves. Type of treatment and short- and long-term evaluation of renal function].

In this paper the authors have analyzed the management and the outcome of 81 cases of posterior urethral valves which occurred during the period January 1972 - April 1985. 53 children presented very severe urethral valves (grade 4 according to Hendren), 28 mild valve type. All the children of the first group but two had a dilatation of the U.U.T. Vesico-renal reflux, usually severe, was present in 51 ureters; in another 50 ureters a dilatation without reflux was present. 47% of the children of the first group had a renal function within the limits of normality at the moment of presentation and the remaining 53% a reduced renal function. In 9 patients (8 under 50 days of age) a cutaneous vesicostomy according to Blocksom followed, at the age of 10-18 months, by transurethral valve destruction was done. In 6 infants, in the early part of the series, the valve was removed with a hook via the perineal approach. In 38 patients we performed a transurethral valve destruction with the n. 3 Bugbee electrode. 36 out of 53 children (68%) had exclusively a removal of the valvular obstruction. After the removal of the obstruction, 32 out of 37 non refluxing dilated ureters (86.5%) showed a clear improvement. In 7 out of 29 refluxing ureters a nephrectomy was carried out. In the remaining 22 ureters the reflux vanished in 17 and improved in the other 5. 17 children had other types of operations after valvular removal. 23 ureters in 13 patients were reimplanted, with 3 failures (13%). In the 53 children with very severe valve (grade 4) supravesical diversions were not carried out. In the follow-up of 51 children (from 6 month to about 14 years) the renal function was within the limits of normality in 74% (before the operation it was 47%). The best results were obtained in children diagnosed and treated in the first months of life. Cutaneous vesicostomy showed itself to be a very useful method of treatment in very young babies with severe complications. We observed a slight terminal urethral stricture, easily dilatable, in only 1 child. All the children over the age of 12-13 years were continent. In 28 children with mild valves, as well as transurethral valve destruction, an ureteric reimplantation was carried out with success in 5 children (8 ureters) and a vesical diverticulectomy in another 2.

Child↗
PubMedDate not supplied

[Seidlmayer's "cockade" purpura. Report of a case].

Among hemorrhagic disorders affecting infants and depending on a vessel increased permeability, Seidlmayer "cockade purpura" is characteristic for its typical cutaneous manifestations. Owing to the rarity of this disease, we report a recently observed case.

Diagnosis, Differential↗