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The case of a mild hemophiliac who underwent a successful and uncomplicated myocardial revascularization is presented. The procedures was safely performed while the patient was receiving factor VIIIC.

The most favorable type of traumatic intracranial bleeding in childhood is the extradural hemorrage (EDH). The posterior fossa location is less frequent than the supratentorial site. In the period from January 1989 to January 1994 we treated 2,372 patients with craniocerebral trauma; 31 had extradural hematomas (1.3%); 3 of them were located in the posterior fossa (9.7%): 1 boy and 2 girls aged from 6 to 16 years. The traumatic mechanism was an occipital fall in all cases. Diagnosis was made by computed tomography scan (CT). Two of them had a rapidly deteriorating course. The three patients were operated on without mortality and there was no morbidity. The role of CT in the early detection of lesions and prompt surgical evacuation may reduce the mortality and morbidity from this lesion. The interaction between these factors is discussed.

Isolated right ventricular aneurysms are rare. Postinfarction right ventricular aneurysm associated with a ventricular septal defect is a very unusual complication. We present such a case that was successfully treated surgically.

A simple structured mathematical model coupled with a methodology of state and parameter estimation is developed for lipase production by Candida rugosa in batch fermentation. The model describes the system according to the following qualitative observations and hypothesis: Lipase production is induced by extracellular oleic acid present in the medium. The acid is transported into the cell where it is consumed, transformed, and stored. Lipase is excreted to the medium where it is distributed between the available oil-water interphase and aqueous phase. Cell growth is modulated by the intracellular substrate concentration. Model parameters have been determined and the whole model validated against experiments not used in their determination. The estimation problem consists in the estimation of three state variables (biomass, intra- and extracellular substrate) and two kinetic parameters by using only the on-line measurement provided by exhaust gas analysis. The presented estimation strategy divides the complex problem into three subproblems that can be solved by stable algorithms. The estimation of biomass (X) and the specific growth rate (mu), is achieved by a recursive prediction error algorithm using the on-line measurement of the carbon dioxide evolution rate. mu is then used to perform an estimation of intracellular substrate and the other kinetic parameter related to substrate transport (A) by an adaptive observer. Extracellular substrate is then evaluated by means of the estimated values of intracellular substrate and biomass through the material balance of the reactor. Simulation and experimental tests showed good performance of the developed estimator, which appears suitable to be used for process control and monitoring. (c) 1995 John Wiley & Sons, Inc.

The production of lipase by Candida rugosa in batch cultures was studied. The initial concentration of the carbon source employed, oleic acid, had an important effect on the final lipolytic activity levels. The maximum lipase/substrate yield and specific productivity obtained correspond to an initial oleic acid concentration of 2 g/l. At higher concentrations, up to 8 g/l oleic acid, specific productivity decreased. Lipase production was not observed below 1 g/l oleic acid. Lipase inactivation in culture broth due to surface forces and shear stress at the gas/liquid interface was not observed. There was no shear stress denaturation at stirring rates of 250, 500 and 750 rpm. No temperature inactivation was detected up to 50 degrees C. Two different lipases with a similar molecular weight of 60 kDa were purified from culture broth.

A simple method for monitoring and quantifying automatically the production by fermentation of beta-galactosidase fusion proteins, making use of the remaining activity of the beta-galactosidase part, is considered. A hybrid protein carrying the major antigenic domain of foot-and-mouth disease virus C1 joined at the N-terminus of beta-galactosidase has been expressed in Escherichia coli. The yield of the chimeric protein has been monitored by flow injection analysis (FIA) during batch fermentations at laboratory scale, and a high correlation between values of product concentration from FIA and from immunological quantizations has been obtained. Because of the possibility of employing FIA in large-scale experiments, and the high sampling frequency, versatility, and reproducibility offered by this method, we propose FIA as a general, simple, quick, flexible, and reliable instrument for both monitoring the yield of recombinant proteins produced industrially, and performing basic research at laboratory scale.

We report a proven case of CJD with prolonged clinical course and white matter involvement which consisted of severe, widespread myelin damage in the forebrain and cerebellum but with sparing of the internal capsule. Histologically, there was status spongiosus of the involved white matter with axonal loss, proliferation of hypertrophic astrocytes and scattered and perivascular foamy macrophages. White matter lesions have not been considered to be a significant feature of CJD. In the last few years, however, a few cases have been described with prominent degeneration of cerebral white matter. We believe that our findings corroborate the existence of an entity that at present may only be defined as spongiform panencephalopathy.

A posttraumatic cerebellar hematoma in a 12-year-old boy is reported. It was observed by CT scan that the hematoma is discharged incompletely into the subdural space. The surgical treatment was successful with complete recovery. It is suggested that CT scan should be performed as soon as possible in cranial trauma with cerebellar signs.

Slit-ventricle syndrome (SVS) may appear in shunted hydrocephalic children as a complication by chronic overdrainage. SVS is defined by clinical features (headache, nausea and/or vomiting, disturbance of consciousness), slow valve refilling and slit or slit-like ventricles at computerized tomography. Authors report eight cases of SVS and review physiopathological, clinical and therapeutic aspects of this syndrome. It is concluded that SVS at present time may be considered an iatrogenic complication and the placement of high-pressure valves as a prophylactic measure can be a useful system to avoid SVS. It seems necessary to make more studies in order to evaluate efficacy of antisiphon devices.

The first reported Spanish family with autosomal dominant endosteal hyperostosis is presented and two members in two different generations studied. Neurological involvement with sensorineural hearing loss, chronic intracranial hypertension, and mild corticospinal tract abnormalities were found in one case with radiological evidence of progressive bone disease at follow-up. In addition to mild hydrocephalus, CT-scan of the head documented a reduction in size of the posterior fossa and encroachment of the foramen magnum. A pattern of selective increase in the bone fraction of serum alkaline phosphatase was also recorded. This family supports the view that severe forms of endosteal hyperostosis are not confined to the autosomal recessive variant, as individuals with the autosomal dominant form may also show relentless progression to neurological involvement during adulthood.

Creutzfeldt-Jakob disease (CJD) occurring in later life has a duration of about one year. Gait disturbance, myoclonic jerks, dementia and akinetic mutism occur. Post-mortem examination of the brain shows nerve cell loss, gliosis and spongiform changes of the grey matter. Clinical and neuropathological features of a case of the panencephalopathic type of CJD, with additional extensive degeneration of the cerebral white matter, are described. The panencephalopathic type of CJD is characterized clinically by a longer duration and a biphasic course.

The authors present a case of tumor thrombus in the inferior vena cava produced by angiomyolipoma in a young woman with tuberous sclerosis. Diagnosis was made by computed tomography (CT) by measuring the attenuation values of the tumor thrombus. The values obtained were characteristic of fat, demonstrating that tumoral invasion was the cause of the thrombus. To our knowledge, this is the first description of such an association shown by CT in the literature.

A recursive estimation scheme, the Extended Kalman Filter (EKF) technique, was applied to study enzymatic deactivation in the enzymatic hydrolysis of pretreated cellulose using a model previously developed by the authors. When no deactivation model was assumed, the results showed no variation with time for all the model parameters except for the maximum rate of cellobiose-to-glucose conversion (r'(m)).The r'(m) variation occurred in two zones with a grace period. A new model of enzymatic hydrolysis of pretreated cellulose deactivation was proposed and validated showing better behavior than the old deactivation model. This approach allows one to study enzyme deactivation without additional experiments and within operational conditions.

Communication between a hydatid cyst of the liver and biliary tract results in a clinical picture of obstructive jaundice because of occupation of the extrahepatic biliary tract by intracystic material. Five cases of this complication are presented. Ultrasound criteria for diagnosis are analyzed, based on previous publications as well as our own experience.