Refractory status epilepticus due to acute hepatic porphyria in a pregnant woman: induced abortion as the sole therapeutic option?

A 22-years old, 55 kg female patient in the twelfth week of pregnancy developed neuropsychiatric syndromes and in the following status epilepticus. Raised porphyrines and porphyrine precursors were found in the patient's urine. Despite intravenous glucose infusions and appropriate medication no reduction in seizure-frequency and neuropsychiatric syndromes was observed. An abortion was induced. After the interruption and starting of haem arginate therapy, seizure activity stopped and porphyrine precursors returned to normal levels, and after 6 weeks the patient was discharged in excellent clinical condition. This report describes a status epilepticus caused by acute hepatic porphyria, triggered by pregnancy, in a 22-years old woman. To our knowledge this is the first report of induced abortion as successful treatment in acute hepatic porphyria induced status epilepticus.

Java-based graphical user interface for the MRUI quantitation package.

This article describes the Java-based version of the magnetic resonance user interface (MRUI) quantitation package. This package allows MR spectroscopists to easily perform time-domain analysis of in vivo MR spectroscopy data. We show that the Java programming language is very well suited for developing highly interactive graphical software applications such as the MRUI software. We have also established that MR quantitation algorithms, programmed in other languages, can easily be embedded into the Java-based MRUI by using the Java native interface (JNI). This new graphical user interface (GUI) has been conceived for the processing of large data sets and uses prior knowledge data-bases to make interactive quantitation algorithms more userfriendly.

Comparative study of a biochemical index for the estimation of bone demineralization and dual photon absorptiometry.

Multivariate classification methods were used to create an early detection technique for determining bone density. This biochemical index (QuiOs) is clinically useful as a potential adjunct in identifying the presence of biochemical deficiencies known to cause osteopenia and the devastating effects of osteoporosis. The test uses the following serum concentrations of a predetermined set of blood constituents: calcium, phosphorus, alkaline phosphatase (ALP), two alkaline phosphatase isoenzymes (liver and intestine), estradiol, and progesterone. Using the results of these six biochemical and hormonal tests, a correlation equation was developed that demonstrates a nonlinear relationship between QuiOs and Ward's triangle of DPA. A sensitivity of 86% and a specificity of 80% was demonstrated for this biochemical index against DPA in this clinical trial.

1H image-guided localized 31P MR spectroscopy of human brain: quantitative analysis of 31P MR spectra measured on volunteers and on intracranial tumor patients.

1H image-guided 31P MR spectra of normal human brain and of intracranial tumors have been analyzed quantitatively. Tumor types examined include prolactinoma, lymphoma, and various grade gliomas. The experimental signals were processed by means of a time-domain least-square fitting procedure, which yields the spectral parameters, as well as a prediction of the standard deviations. Significant spectral variations are observed within both populations of normal brain and of intracranial tumor 31P MR spectra. The metabolic ratios derived from the glioma 31P MR spectra and from corresponding uninfiltrated brain tissue do not differ significantly. Significant differences are, however, observed between the metabolic ratios of prolactinoma and uninfiltrated tissue 31P MR spectra. Alkaline pH values are found for the prolactinoma and the high-grade gliomas. Furthermore, spectral differences are observed between the patient's uninfiltrated tissue 31P MR spectra and those of an unmatched population of volunteers. This underscores the necessity for control measurements on the uninfiltrated tissue of the patient and for controls from a matched population of healthy individuals.

In vivo 31P NMR spectroscopy of the rat cerebral cortex during acute hepatic encephalopathy.

During the development of acute hepatic encephalopathy, induced by acute liver ischemia, changes in brain 31P NMR spectra and EEG spectra were studied over 8:45 h in eight rats. At the end of this period the brain amino acid concentrations were determined. The results were compared with the same measurements in four normal and three portacaval shunted rats. Signs of acute HE, as judged by the EEG left index, started 5 h after the induction of acute liver ischemia. No accompanying significant changes in the cortical relative phosphocreatine and ATP concentration and intracellular pH were observed. The cortical relative Pi concentration had only slightly increased at t = 8 h. The concentrations of almost all measured brain amino acids, especially glutamine had increased at t = 8:45 h. At t = 8 h, rats with very severe HE had a small, but significant decrease of brain ATP concentrations. Their brain amino acid concentrations were more disturbed than in rats with less severe HE. It is concluded that a change in the cortical cerebral energy rich phosphate concentration is not an important pathophysiological mechanism during the development of acute HE. The observed changes in brain amino acids concentrations could be either part of a multifactorial pathogenesis or could be epiphenomena.

Accurate quantification of in vivo 31P NMR signals using the variable projection method and prior knowledge.

Free induction decay signals are analyzed by fitting a model function directly in the time domain. No starting values are needed for linear model parameters, and omission of corrupted data points poses no problems. A significant gain of accuracy is achieved by imposing prior knowledge about the model parameters.

The treatment of soil infested with the human whipworm, Trichuris trichiura.

The soil fumigants methyl bromide, chloropircrin and Basamid or mixtures of Basamid and chloropicrin proved to be of no use in filling T. trichiura ova in soil. However, on untreated plots the eggs died at a rate such than only 20% of the ova remained viable after 18 months.