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Biomedical subjects

E Fontana

Publications and source records attributed to E Fontana.

52 records · Page 3Linked to original sources
PubMed1989

Progressive myoclonic encephalopathy in X-linked hypogamma-globulinemia. Case report, review of the literature and its relationship with progressive encephalopathy in children with A.I.D.S.

A 7-year-old boy suffering from X-linked hypogammaglobulinemia and progressive myoclonic encephalopathy is reported. The onset of neurological disturbances is at four years of age with ataxic gait and myoclonic jerks. The EEG shows a progressive slowing of background activity, bilateral diffuse and repetitive, pseudoperiodic, high amplitude slow waves, myoclonic jerks polygraphically documented. The CT-scan shows generalized cerebral atrophy, white matter hypodensity--principally in the frontal regions -, multiple nodular calcifications, also in the basal ganglia. Two years after the onset of neurological signs, the boy is completely bedridden, spastic, dement and blind; the myoclonic jerks persist. Finally the relationship is discussed with both the previously reported patients with the same affection, and with similar progressive encephalopathy in children suffering from A.I.D.S.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome.

We report the fourth case of partial status epilepticus (SE) in benign epilepsy of childhood with rolandic spikes (BECRS). The child suffered long-lasting attacks involving the mouth and pharynx, clinically manifest as speech arrest, sialorrhea, and drooling. Both clinical and electroencephalogram (EEG) data were compatible with the diagnosis of BECRS. Only during SE was the clinical picture similar to that observed in the operculum or Foix-Chavany-Marie syndrome. SE remission was obtained with the usual antiepileptic drug therapy (diazepam, clobazam, valproate). EEG records showed additional patterns of continuous spike-waves during slow sleep and specific inhibition and blocking of interictal centrotemporal spikes by mouth and/or tongue voluntary movements.

Brain↗
PubMedDate not supplied

[Percutaneous endoscopic gastrostomy in children with ventriculoperitoneal shunt].

The complications of percutaneous endoscopic gastrostomy (PEG) placement or replacement or of home management of gastrostomy, must be taken in account in patients with hydrocephalus and ventriculoperitoneal shunt. In this report we describe four children with spastic quadriplegia and ventriculoperitoneal shunt who had a median follow-up of 15 months (range 4-32 months) after PEG placement. Intravenous antibiotic prophylaxis was always used during routine procedures and no shunt infection was observed. In a patient, during accidental PEG dislodgement, peritoneal infection developed that required temporary diversion of the catheter. A second dislodgement, in the same individual, determined a large amount of serous peritoneal fluid that needed to be evacuated but no shunt infection or malfunction. In nobody of our patients, the shunt, located in the upper left abdomen, interfered with gastrostomy placement. Our experience confirms that PEG is not contraindicated in patients with ventriculoperitoneal shunt, provided that the risks of catheter infection are known and prevented.

Adolescent↗
PubMedDate not supplied

[Feeding neurologically disabled children with dysphagia: the role for gastrostomy].

INTRODUCTION: Undernutrition and growth retardation are often observed in children with severe neurological disabilities. Our experience of feeding gastrostomy in children and adolescents with central nervous system (CNS) disease and dysphagia is reported. PATIENTS AND METHODS: A gastrostomy feeding tube was placed in 11 children who had severe impairment of swallowing and clinically evident food aspiration. Percutaneous endoscopic technique was preferred, unless operative placement was suggested by unfavourable anatomical conditions or concomitant abdominal surgery. Commercial formulas or natural food were used at home and the children were regularly followed-up at the outpatient clinic. RESULTS: Median age at the gastrostomy placement and median follow-up lenght were 5.9 years (range 1.8-16.7 years) and 15 months (3-66 months) respectively. Four of 11 patients had moderate (weight/height (W/H) ratio = 80%) and 3 severe (W/H ratio < 70%) malnutrition. Ten of 11 subject were exclusively gastrostomy fed. After 3 months of enteral nutrition a weight gain was observed in all patients as well as a significant increase of mean W/H ratio (81.2% vs. 87.2%, p = .002). Nutritional improvement was confirmed at follow-up, despite caloric intakes lower (< 50%) than recommended for age and weight. Micronutrients and vitamins were supplemented on the basis of calculated intakes. CONCLUSIONS: In children with severe CNS disease and dysphagia, long-term gastrostomy feeding is a safe and useful method that allows adequate nutritional and micronutrient intakes and prevents the risk of dystrophy.

Adolescent↗