Long-term follow-up of elderly patients with severe aortic stenosis treated by balloon aortic valvuloplasty. Importance of haemodynamic parameters before and after dilatation

Early and late prognosis after percutaneous balloon aortic valvuloplasty (PBAV) was assessed in 38 consecutive elderly patients (mean age, 78.5 +/- 6.1 years). Significant valve opening was achieved in 35 patients. The hospital mortality was 8% (three patients); two other patients died within the first month and three underwent aortic valve replacement. At 2 years follow-up, there were 10 additional deaths (seven cardiac deaths) and five patients had symptom recurrence managed by aortic valve replacement (3) or repeat PBAV (2). Overall, six patients underwent surgery without untoward events and six had repeat PBAV. Only two out of six patients with repeat PBAV had sustained improvement; one was referred to surgery and the remaining three died soon after the second PBAV. One- and 2-year survival were respectively 72 and 62% and percentage of survivors with persistent improvement 68 and 41%. Although aortic valve area after PBAV was associated with outcome, predictors of poor long-term prognosis were primarily related to the pre-operative haemodynamic status. Patients with pulmonary resistances greater than 400 dynes cm-1 s-5 had the poorest outcome (chi 2 = 18.4-P less than 0.0001). Overall, signs of heart failure were predictors of poor long term follow-up. These data indicate that long-term success of PBAV is mainly related to the left ventricular dysfunction noted prior to intervention.     

Obturator hernias — Report of seven cases

Summary Obturator hernias comprise a rare group of abdominal hernias. They are often diagnosed when the small bowel has become obstructed and the patients are operated on without the causes of the obstruction being known. Although many techniques for closure of the defect are available, there is no agreement at the present time as to which is the procedure of choice.     

Infantile hypopituitarism: etiological variability evidenced by MRI

In this study, magnetic resonance imaging (MRI) was performed to document potentially present morphostructural abnormalities of the hypothalamopituitary region in seven infants (age 0–21 months) who presented very early in life with clinical and biochemical evidence of hypopituitarism. Four infants had associated congenital cerebro-facial malformations.The following anatomical abnormalities were identified in variable combinations: ectopic neurohypophysis absence of the pituitary stalk, extreme elongation of the pituitary stalk, aplasia of the anterior pituitary lobe and no identification of the hypothalamopituitary complex.MRI proved to be very sensitive in the identification of structural malformations of the hypothalamopituitary region in infants with or without cerebro-facial malformations. AllhwuLll the magnetic resonance image does not appear to be a good predictor of endocrine dysfunction, it provides us more insight into the precise aetiology of this disorder and may be therefore of diagnostic, prognostic and therapeutic importance.     

Alteration of bronchomotor tone after deep inhalation. Mechanisms and pediatric data]

There is significant alteration of airway calibre after deep inhalation (DI). An increase of airway calibre has been evidenced in children in case of mild asthmatic obstruction and after a positive response to methacholine or exercise challenge. Such an increase is blunted in the presence of a moderate to severe asthmatic airway obstruction and no bronchodilatory effect of DI has been found in infants. Bronchial hyperreactivity and airway inflammation strongly influence post-DI alterations of bronchomotor tone. Airways and lung parenchyma are interdependent but both exhibit imperfect elasticity. Hysterisis characterizes imperfect elasticity of pressure-volume relationship. The mechanical theory of relative hysteresis between airway and lung parenchyma is the main mechanism implicated in the induced changes of airway calibre. DI may influence results of lung function testing in children. The technique may be of interest to increase sensibility of routine lung function testing in children.     

Axial vs sagittal T2-weighted brain MR images in the evaluation of multiple sclerosis

Axial and sagittal proton density and T2-weighted MR images (TR 2,500-3,000 ms, TE 15-22 and 85-90 ms) were performed in 50 patients with multiple sclerosis (MS) on a 1.5 T superconductive system. The number of plaques on the axial and sagittal images in the periventricular white matter, the corpus callosum, the brain stem, the cerebellum, and the basal ganglia were counted separately by two independent observers. A total of 858 lesions (mean 17.40 +/- 21.57) were seen on the axial series and 1,196 (mean 24.32 +/- 26.22) on the sagittal scans. More lesions were visualized on sagittal images in the periventricular region (mean 18.79 +/- 21.69 versus 13.34 +/- 16.45; p less than 0.001) and the corpus callosum (mean 3.00 +/- 2.72 versus 0.57 +/- 1.19; p less than 0.001). In the brain stem more lesions were visualized on the axial images (mean 1.55 +/- 2.55 versus 0.87 +/- 1.20; p less than 0.05). In the cerebellum and basal ganglia, scans in the two planes were equivalent (p greater than 0.5). In three patients lesions were seen on the sagittal series, while the axial scans were normal. Sagittal T2-weighted images appear to demonstrate significantly more MS plaques than transverse images, especially in the periventricular region and the corpus callosum. This is explained by partial volume averaging, by the orientation of some cerebral structures (e.g., corpus callosum) with regard to the section plane, and by the longer diameter of the lesions in the axial plane.     

Irreversible inhibition of rat S-adenosylmethionine decarboxylase by 5'-([(Z)-4-amino-2-butenyl]methylamino)-5'-deoxyadenosine

5'-([(Z)-4-Amino-2-butenyl]methylamino)-5'-deoxyadenosine [Z)-AbeAdo) was tested in vitro and in vivo as a potential inhibitor of S-adenosyl-L-methionine decarboxylase (AdoMetDC), a pyruvoyl-containing enzyme, purified from rat liver. In vitro (Z)-AbeAdo produces a time- and dose-dependent irreversible inhibition of the enzyme. Saturation kinetics are observed when the enzyme is preincubated with (Z)-AbeAdo in the presence of 50 microM putrescine, a known activator of AdoMetDC. Under these conditions kinetic constants were measured (Ki = 0.56 +/- 0.04 microM; tau 1/2 = 0.51 +/- 0.03 min). The inhibition is not relieved by prolonged dialysis of the inactivated enzyme. The turnover number for (Z)-AbeAdo, i.e. the number of inactivator molecules required to inactivate one enzyme molecule, is approximately 1.5. The selectivity of (Z)-AbeAdo was explored: the compound is not a substrate of adenosine deaminase, mitochondrial monoamine oxidase and diamine oxidase, but is slowly oxidized by benzylamine oxidase from rat aorta. The (E)-isomer of AbeAdo, is at least 100-fold less active than (Z)-AbeAdo as a time-dependent inhibitor of rat liver AdoMetDC. In rats, intraperitoneal administration of (Z)-AbeAdo produces a rapid, long-lasting and dose-dependent decrease of AdoMetDC activity in ventral prostate, testis and brain.     

Estimation of the bronchodilatory effect of deep inhalation after a free run in children.

The bronchomotor effects of a deep inhalation (DI) may provide relevant information about the mechanisms of exercise-induced airway obstruction in children and may be assessed by respiratory conductance (Grs) measured using the forced oscillation technique. The aims of the present study were to assess the effect of DI on Grs after exercise in relationship to the lung function response to exercise. Grs at 12 Hz using a head generator and spirometric data were measured in 62 children suspected of asthma before and 5 min after a 6-min free run. After exercise, Grs was significantly increased by DI in 38 subjects, who also showed larger Grs and forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) responses to exercise than the 24 nonresponders. Stepwise regression indicated significant correlation between the response of Grs to DI and both Grs and FEV1/FVC responses to exercise. The data are consistent with exercise-induced bronchoconstriction being reversed by deep inhalation.     

Surgical Treatment of an Early Epileptic Encephalopathy with Suppression-Bursts and Focal Cortical Dysplasia

Summary: A case of early epileptic encephalopathy (EIEE) with suppression-bursts or Ohtahara's syndrome, associated with focal cortical dysplasia is reported. Infantile spasms and brief tonic unilateral seizures began on the fifth day of life. Interictal EEG demonstrated an asymmetrical "suppression-burst" pattern with no wake or sleep cycling. Seizures were refractory to all antiepileptic drug (AED) and steroid therapy. Magnetic resonance imaging (MRI) showed right frontotemporal cortical thickening. After three weeks of an ineffective medical treatment a preoperative evaluation with single photon emission computed tomography (SPECT) and electrocorticography (ECoG) was performed to characterize epileptogenic focus. Surgical resection of the precentral area was performed at age 1 month. Neuropathologic examinations confirmed diagnosis of focal cortical dysplasia by identifying cytoarchitectural disarray and ectopic neurons located deep in subcortical white matter. During follow-up, 1-year postoperative the child had a single febrile seizure. Neurologic examination showed minor developmental delay and slight left-sided weak ness.