Vaccine-associated paralytic poliomyelitis: a case report of domiciliary transmission.

Poliomyelitis associated with live strain vaccine is defined as the paralytic form of the acute anterior poliomyelitis related to the vaccine strain. Since these strains behave similarly to the wild-type virus, we can differentiate, epidemiologically, two types of vaccine-associated poliomyelitis: cases in which the patient was vaccinated and cases in which the patient had had contact with vaccinated individuals. We herein present the case of an unvaccinated child, with a clinical picture of an acute anterior poliomyelitis associated with the live strain vaccine, whose brother received the Sabin vaccine 20 days before the onset of the symptoms. Vaccine strain of the type 3 poliovirus was isolated in fecal culture and a presented mutation in nucleotide 472 (C-->U) in the 5' non-coding region, which is strongly related to the higher strain virulence.

[Audiological assessment and follow-up after bacterial meningitis]. / Avaliação e acompanhamento audiológico após meningite bacteriana.

Hearing loss is the more frequent sequel of bacterial meningitis in children. This study describes the audiological profile (peripheric and central) of 89 children admitted to the hospital wards with the diagnosis of bacterial meningitis. Those children attended audiological follow up, after their hospital discharge, and were submitted to audiological tests. The results showed that 85.4% among them presented normal hearing in both ears 10.1% presented bilateral neurosensorial hearing loss and 4.5% presented unilateral neurosensorial hearing loss. The results from the auditory processing skills assessment showed that 10% of those children presented auditory localization and recognition of sentences with competitive messages (Paediatric Sentences Identification-ipsilateral) disorders.

[Management of acute seizure episodes and status epilepticus in children] / Abordagem da crise convulsiva aguda e estado de mal epiléptico em crianças.

OBJECTIVE: The authors make an up-to-date review about diagnosis and treatment of the acute seizures and status epilepticus.METHODS: Bibliographic review of Medline database including articles published in the last ten years.RESULTS: Acute seizures and status epilepticus affect children of all the age groups. Acute seizures can either represent the first manifestation of an epileptic condition or be an acute symptomatic event. Status epilepticus represents a serious cerebral insult determined by various causes. The emergency, neurology and pediatric intensive care units should elaborate clinical policy for the establishment of precise diagnostic criteria and therapeutic approach. A good clinical approach should include immediate life support, monitoring and rational drug administration to end up the seizure and reduce morbidity and mortality risks.CONCLUSION: The better understanding of the pathophysiology as well as the elaboration of a rational clinical policy improved the outcome in these medical emergencies.

[Glutaric aciduria type 1: phenotypic variability. Report of 6 patients]. / Acidúria glutárica tipo 1. Variabilidade fenotípica. Estudo de seis pacientes.

We report six patients with glutaric aciduria type 1 in four families. The patients had marked clinical variability, even within families. Three of the patients studied were normal until the onset of neurologic abnormalities, that presented as an encephalitis-like illness in the first year of age. One patient had an early and important developmental delay, but never suffered an encephalopathic crisis. Two patients have intellectual preservation; one of them has a mild tremor and choreoathetosis since the first year of age, and the other had only two afebrile seizures in infancy and no other neurologic signs. Three patients are severely handicapped, with a severe dystonic-dyskinetic disorder and unable to even sit. All the six patients have macrocephaly and in all the computed tomography showed enlarged CSF spaces and sulcal separation over the frontal and temporal lobes. Urine organic acids study of all patients showed large quantities of glutaric acid.

[3-Hydroxy-3-methyl-glutaryl-CoA-lyase deficiency as coma etiology in the neonatal period: case report]. / Deficiência da 3-OH-3-metil-glutaril-CoA-liase como causa de coma no período neonatal. Relato de caso.

We report a patient that presented two episodes of coma in the neonatal period, with severe metabolic acidosis and hypoglycemia, without ketosis. The urinary organic acid analysis showed increased amounts of 3-hydroxy-3-methyl-glutaric, 3-methylglutaconic, 3-methylglutaric and 3-hydroxyisovaleric acid. The deficiency of 3-hydroxy-3-methylglutaryl-CoA lyase was diagnosed by the clinical and metabolic features. This disease shows autosomal recessive inheritance and the treatment is done by a diet with restriction of protein (mainly leucine) and lipids, high in carbohydrate content, and the avoidance of fasting and carnitine supplementation.

[Evaluation of the therapeutic efficacy of dexamethasone in meningococcal meningitis] / Avaliação da eficácia terapêutica da dexametasona na meningite meningocócica.

OBJECTIVE: To evaluate the efficacy of dexamethasone as an auxiliary therapeutic tool to the antibiotics in hospitalized children with meningococcal meningitis. METHODS: A retrospective clinical comparative study was undertaken with children from a pediatric ward affected by laboratory proved meningococcal meningitis at a university hospital. Cases of children in state of shock at admission or deceased in the first 24 hours were excluded. During the period from 1987 to 1989 33 children were treated only with antibiotics (group A), while from 1990 to 1993 other 66 children received additionally dexamethasone (12mg/m2/24h) by intravenous route during four days beginning at the admission to the hospital (group B). The two groups were evaluated at baseline through prognostic scores and analysis of their clinical and laboratorial characteristics obtained from data recorded at the admission. The parameters to evaluate dexamethasone efficacy were the comparative number of neurologic and systemic complications detected at the hospital, and the liquoric profile (leukocyte count, glucose and protein content) verified between day 9 and day 11 of hospitalization. RESULTS: The profile of the two groups (A and B) were homogeneously evaluated by the illness severity scores and their clinical and laboratorial characteristics. Nine complications were recognized in group A (27.2%) and 21 (31.8%) among those of group B, difference not significant. Likewise, there were not observed liquoric differences between the two groups related to the chimiocytologic pattern. CONCLUSIONS: No effect of dexamethasone therapy to prevent neurologic and systemic meningococcal meningitis complications was observed during hospitalization. Similarly no favorable effect in relation to the liquoric pattern verified between day 9 and day 11 of hospitalization was recognized.

[Auditory behavior monitoring after bacterial meningitis. Case report]. / Evolução do comportamento auditivo após meningite bacteriana. Relato de caso.

Bacterial meningitis is the main cause for acquired hearing loss. Nevertheless very little has been written about the development of the auditory behaviour either for improvement or for deterioration, after hospital release. The present study describes the case of a five month old boy with Haemophilus influenzae meningitis. Amongst various complications, a decrement in the auditory acuity was detected in the immediate evolution, with significant improvement later on by qualitative and quantitative tests.