Creutzfeldt-jacob disease: a comparative light-microscopic, histochemical and electron-microscopic study.

The morphological and histochemical findings in a brain biopsy of a case of Creutzfeldt-Jacob disease are compared. The histochemical changes in the cytoplasm of the neurons precede the morphological alterations found with the electron microscope. In this way the neuronal enzymatic failure can explain the clinical picture of this disease.     

Neuroradiologic findings in a young patient with characteristics of Sturge-Weber syndrome and Klippel-Trenaunay syndrome

The patient presented here has extensive hemangiomata plana, especially on the right forehead, right upper eyelid, and right leg, as well as right leg hypertrophy and macrocephaly. Cerebral magnetic resonance imaging (MRI) showed abnormalities in only one occipital lobe consisting of focal cortical atrophy, leptomeningeal enhancement, and ipsilateral choroid plexus enlargement. Mental and motor development is normal, and he has no seizures. The parents are consanguineous. Leg hypertrophy associated with ipsilateral cutaneous vascular malformations is suggestive of Klippel-Trenaunay syndrome. The patient's central nervous system abnormalities on MRI and the hemangiomata plana on the ipsilateral upper eyelid and forehead point to Sturge-Weber syndrome. We conclude that the patient has an overlap syndrome between Klippel-Trenaunay syndrome and Sturge-Weber syndrome.     

Glutamine: metabolism and application in nutrition support

Glutamine is the most abundant free amino acid in the body. It is avidly consumed by rapidly dividing cells, such as those lining the gut, because its 5-carbon skeleton can provide energy whilst the nitrogen molecules support the synthesis of nucleic acids. Patients who are maintained using conventional solutions of parenteral nutrients become depleted in glutamine, which has led to the reclassification of glutamine as a conditionally essential nutrient. Unfortunately, glutamine is unstable in solution and produces toxic byproducts on decomposition. This means that solutions of nutrients containing glutamine have a relatively short half-life, which has led to the commercialisation of stable dipeptides containing glutamine. Although it is evident that glutamine enhances nitrogen metabolism, there is a lack of consistent evidence from the initial clinical trials demonstrating that supplementation with glutamine has specific clinical advantages. The next few years will witness the performance of larger scale clinical trials and the results of these studies should define a more certain role for glutamine in routine clinical practice.     

Early prophylactic inhaled beclomethasone in infants less than 1250 g for the prevention of chronic lung disease

OBJECTIVE:

Inflammation plays an important role in the development of chronic lung disease (CLD), which has become a major cause of morbidity in surviving infants less than 1250 g at birth. The authors hypothesized that the progression of this inflammation and, therefore, the establishment of CLD would be decreased with the use of early prophylactic inhaled corticosteroids. Short, and long term respiratory and neurodevelopmental outcomes were also examined.

DESIGN:

A double-blind, randomized placebo controlled trial.

SETTING:

Level-III neonatal intensive care unit.

POPULATION STUDIED:

Sixty infants less than 1250 g at birth, diagnosed with respiratory distress syndrome and requiring ventilatory support at 72 h of age were enrolled in the study.

INTERVENTION:

Infants enrolled received either placebo or beclomethasone diproprionate by a metered dose inhaler, which was used in-line with the ventilator circuit while the infant was ventilated and then via a spacer until 28 days of age.

RESULTS:

Thirty infants were given beclomethasone and 30 were given placebo. There were two deaths in each group. Among the surviving infants, the frequency of moderate-to-severe CLD was 17% in each study group. Mean time to extubation was not different for beclomethasone compared with placebo at 16.4 and 12.5 days (P=0.12), respectively. The requirement for intravenous corticosteroids was lower in the beclomethasone-treated group (RR 0.67, 95% CI 0.43 to 1.04), although this difference was not statistically significant. The incidence of growth failure, infection and intraventricular hemmorhage did not differ between the two groups. Long term outcomes were not different with respect to the incidence of respiratory re-admissions, cerebral palsy, developmental delay, blindness or deafness.

CONCLUSIONS:

Early treatment with inhaled beclomethasone diproprionate did not reduce the incidence of CLD or decrease the duration of mechanical ventilation. The decrease in intravenous corticosteroid use was not statistically significant. Long term outcome was not affected.