[Rhabdomyolysis caused by carnitine palmitoyltransferase deficiency].
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Acyltransferases↗
Biomedical subjects
M Weber
Publications and source records attributed to M Weber.
Anti-glomerular basement membrane antibody-mediated glomerulonephritis due to glue sniffing.
A 16-year-old girl developed rapidly progressive glomerulonephritis and renal failure. The disease was associated with high titres of antiglomerular basement membrane antibodies in serum, linear deposits of immunoglobulin G and diffuse epithelial crescents on renal biopsy. Past history revealed heavy smoking and deliberate sniffing of Pattex glue, a mixture of hydrocarbons which possibly may affect the structure of glomerular basement membrane. After treatment by repeated plasmapheresis and drug immunosuppression autoantibodies disappeared from serum but renal function was not influenced. Renal damage is a potential hazard for glue sniffing adolescents.
Adhesives↗
[Focal nodular hyperplasia of the liver in 930 patients].
Analysis of 930 patients with focal nodular hyperplasia of the liver (FNH), including 23 seen by the authors, showed the following results: 82.2% of patients were female, 37.8% of these had taken female steroidal hormones. There was no preference for a particular lobe of the liver. 57.7% of FNH were subcapsular, 33.7% were intrahepatic and 8.8% were pedunculated. Average size was 5.9 cm, 80.8% of the patients were asymptomatic. In 93.5% (115 out of 123) a space-occupying lesion in the liver could be demonstrated by sonography. In 13%, second or multiple tumours were missed by sonography. CT without enhancement demonstrated FNH in 90.4% (94 out of 104 patients). A rapid, marked increase in density after contrast was seen in 64 out of 68 lesions (94.1%). Colloid scintigraphy showed normal uptake in 34.9% of patients. Hepato-biliary functional scintigraphy demonstrated 32 out of 35 lesions (91.4%). In 97.3% (183 out of 188) FNH was demonstrated by angiography, in 95.4% as a highly vascular tumour.
Adolescent↗
The isolation of cross-reactive monoclonal antibodies: hybridomas to streptococcal antigens cross-reactive with mammalian basement membrane.
Based upon the assumption that post-streptococcal sequelae are the result of cross-reactive antibodies, hybridomas were prepared from the spleens of mice immunized with Group A type 12 streptococcal cell membranes (SCM) specifically to screen for such cross-reactive antibodies. One fusion produced a cell population displaying antibodies reactive to both SCM and glomerular basement membrane (GBM) antigens as demonstrated by ELISA technique. Ascites produced by this cell population also showed reactivity to lung basement membrane (LBM). Limiting dilution procedures have produced 15 monoclonal hybrids with both anti-SCM and anti-GBM activity. Confirmation of the cross-reactive and monoclonal nature of the antibody was accomplished by both direct and indirect competitive ELISA. These observations have established that unique cross-reactive antibody-secreting hybrid cells with reactivity to both SCM and basement membrane (BM) antigens can be isolated by standard cloning procedures.
Animals↗
No increased incidence of postoperative sore throat after administration of suxamethonium in endotracheal anaesthesia.
Sixty patients were divided into two groups (A and B) of 30 patients each to investigate the effect of using suxamethonium in endotracheal anaesthesia on the incidence of postoperative sore throat. The patients were anaesthetized with thiopentone, fentanyl, droperidol, N2O and pancuronium. Before endotracheal intubation with a Mallinckrodt lo-pro-tube, patients in Group A were given pancuronium, whereas patients in Group B were given suxamethonium. There was no difference in the incidence or severity of sore throat 20-30 h postoperatively between the two groups (P = 0.5). The type 2 error (beta) was low (the risk of overlooking a "true" difference in incidence of 0.20 was calculated to be 0.04). These results contradict those of a recent study, which demonstrated an increased incidence of postoperative sore throat following the use of suxamethonium in mask anaesthesia.
Adolescent↗
Isolation and partial characterization of a 56,000-dalton phosphoprotein phosphatase from the blood-brain barrier.
A 56,000-dalton protein with inherent phosphoprotein phosphatase activity was isolated from porcine brain capillaries. The enzyme is not activated by divalent metal ions but strongly inhibited by zinc ions. As phosphatase inhibitor 2 readily inhibits the enzymatic activity, the protein can be classified as a type I phosphatase. The protein is stable toward protease treatment. Limited digestion with trypsin does not convert the enzyme into an active form of lower molecular weight. The physical and enzymatical properties of the phosphatase exhibit considerable similarities to those of another 56,000-dalton phosphatase derived from rabbit reticulocytes.
Amino Acid Sequence↗
Frequency analysis of the surface electrocardiogram for recognition of acute rejection after orthotopic cardiac transplantation in man.
Recognition of acute rejection after heart transplantation has been based mainly on invasive methods until now. In this study we analyzed two well-defined surface electrocardiographic recordings by fast Fourier transform (FFT) (Blackman Harris window, 512 points) after low-noise, high-gain amplification (filter setting 0.5 to 300 Hz) each day for 4 weeks after cardiac transplantation in 27 patients. Twenty acute rejection crises requiring treatment were diagnosed by cytoimmunologic monitoring and endomyocardial biopsy. Single-beat analysis of the QRS complex by FFT revealed a progressive change of the spectral morphology (increase of the frequency content between 70 and 110 Hz) on the days of rejection in 19 of 20 patients. At that time there were no visible changes on the electrocardiogram in the time domain in most patients. At the same time, the frequency content of the ST segment decreased between 10 and 30 Hz in 16 of 20 patients. After successful treatment, the frequency spectra of the QRS complex and ST segment returned to control within 1 to 2 weeks in most patients. One false-positive result was seen in a patient with mediastinitis and large pericardial effusion. A drop in QRS amplitude (greater than 20%) occurred in 10 of 20 rejection crises and in 10 patients without rejection. Nine patients after cardiac transplantation without rejection and seven control patients after cardiac surgery (not transplantation) showed stable frequency plots from one day to the other after the first postoperative day, but with considerable changes in QRS amplitude.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Doppler echocardiographic assessment of left ventricular systolic and diastolic function in mild hypertension.
Abnormalities in left ventricular (LV) wall thickness and mass have been demonstrated in patients with mild hypertension utilizing M-mode echocardiography. In addition, studies using radionuclide angiography have demonstrated abnormalities in early diastolic LV filling in asymptomatic hypertensive patients with normal ejection fraction and cardiac output. Recently, Doppler recordings of flow velocity in the ascending aorta and through the mitral valve have been shown to provide useful information about LV function. To determine whether flow abnormalities could be detected in patients with mild hypertension, we recorded Doppler aortic and mitral valve flow velocities in 21 men with mild hypertension. Casual systolic blood pressure was 147 +/- 18 mm Hg (mean +/- SD) and diastolic blood pressure was 96 +/- 9 mm Hg. LV mass (310 +/- 75 g) was elevated (i.e., above the 95% normal prediction interval) in 8 of 19 patients who underwent M-mode echocardiography; LV ejection fraction was normal in all patients (mean, 80%). As in previous studies in normal subjects, we found in these hypertensive patients an inverse correlation between age and both aortic peak flow velocity (r = -0.51, p less than 0.05) and transmitral early diastolic peak flow velocity (r = -0.44, p less than 0.05) and a positive relationship between age and mitral valve late diastolic peak flow velocity (r = 0.73, p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Hemidesmosome deficiency of gastro-intestinal mucosa, demonstrated in a child with Herlitz syndrome and pyloric atresia.
In an 11-day-old premature girl with Epidermolysis bullosa atrophicans generalisata gravis Herlitz and pyloric atresia, hypoplasia of hemidesmosomes in the skin, the gastrointestinal mucosa and the atretic pyloric segment was found by electron microscopy. Pyloric atresia is explained by hemidesmosomal defects causing junctional blistering of the mucosa, subsequent peptic digestion and inflammatory scarring reaction.
Desmosomes↗
[The testing of long-term antirheumatics in animals].
There is no group proof of long acting antirheumatics (LAA) in laboratory animal models, and it is not to be expected without an identical rheumatoid arthritis model in animals and with regard to the heterogeneity of LAA. However, LAA are to be detected according to D-penicillamine-like, levamisol-like etc. actions, which can be disclosed in the adjuvant arthritis as well as in the B. pertussis-vaccine pleuritis in rats the latter model best by including parameters of inflammatory exudate cells. Modification of the models or of model parameters (BCG-sensibilization, PPD reaction, vasoreactivity, RNA content of exudate cells, SH groups, copper zinc) are hardly advantageous, contrarily to dosage. Other models, among them paw edemas, do not permit sufficient testing of LAA, even not the methyl-albumin mice paw edema. There is no problem of pharmacologically separating LAA actions from nonsteroidal or steroidal antiphlogistics actions.
Animals↗
[Pyoderma gangrenosum: treatment by salazosulfapyridine].
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Drug Combinations↗
[Drug-induced pellagroid erythema. A case of pellagroid erythema caused by isoniazide].
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Antineoplastic Agents↗
[Cutaneous accidents caused by self-defense sprays].
The free sale of "self-defence sprays" and the use of such weapons by the police and riot squads account for the increase observed in the frequency of cutaneous accidents. Tear gases. 1. CN, or chloracetophenone, is a compound insoluble in water and soluble in alcohol, ether and carbon sulphide. MACE is CN dissolved in methylchloroform. 2. CS, or orthochlorobenzylidene malononitrile is also insoluble in water and can be used in sprays in concentrations of 2 to 8 p. 100, propelled by such gases as freons. Clinical effects. 1. Experimental. When these gases are suspended in air they mostly act on the eyes, producing blepharospasm, conjunctivitis and photophobia; they have only minor effects on the skin. When applied directly onto the skin they produce extreme irritation with erythema and vesicles. The higher the degree of humidity, the more severe the lesions. In animals and in man CN and CS behave as potential allergens on repeated exposures. 2. Accidental. Tear gases may have two effects: they usually produce irritant dermatitis, but also sometimes a genuine eczema. In aggressors exposed to these sprays the lesions develop in two stages: first, redness and burning sensation on the face--which characteristically is only affected on one side owing to the lateral projection of the tear gas--then, on the following day there appears an oedema similar to Quincke's oedema, with swelling of the eyelids. Oozing rapidly turns to crusts, and in the absence of treatment infection is the rule. Cure requires as many as 10 to 15 days of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
Aerosols↗
[Five years after a first epileptic seizure appearing late in life].
A first epileptic seizure in adulthood leads to fear a brain tumor and other important features of late epilepsies have received little attention. We have analyzed the etiologies in a group of 392 patients consecutively seen from 1977 to 1980. Our data are in keeping with previously reported data. One hundred and eighty patients were followed for five years or more. Fifty eight had only a single seizure (only 23 were treated, and 20 stopped therapy). Seizures recurred in 122 patients (97 untreated). The characteristics of recurrence have been analyzed. Some predictive factors appear significant. Socio-professional results have been considered. By and large, the prognosis was good for 75 p. 100 of patients.
Adult↗
Frequency analysis of surface electrocardiogram and two-dimensional echocardiography for noninvasive diagnosis of rejection after heart transplantation.
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Echocardiography↗
[Computerized data storage of antibiograms obtained by various methods (automatic reading by 2 different systems or manual processing by the disk method) and retrieval of antibiograms and cytobacteriological data using microcomputers].
This software is composed of different programmes. It works on Apple IIe computers. It enables to capture all the data information concerning bacteriological tests to store them and to edit either personnel reports or global lists made out according to different criteria and to study them under both the statistical or the epidemiological aspects. The results of the antibiotic sensitivity test are notified either by an automatic reader (ABAC or API system) when carried out in a liquid environment or by an original computerized device (compass or enlarger) when manually carried out by the disc method. The informations are directly transmitted to the computer. It's possible to make up a common data file that can be exploited as a whole.
Bacteriological Techniques↗
[Treatment of post-traumatic and post-neurosurgical bacterial meningitis with ceftriaxone alone or in combination with fosfomycin].
From 1984 to 1986, 13 patients (10 adults, 3 children) with bacterial meningitis following neurosurgery or traumatism were given ceftriaxone alone 6 times at a dose of 40 mg/kg one IV injection per day, or in association 7 times with fosfomycin at a dose of 200 mg/kg/day, 3 IV perfusions every 4 h. The bacteriological diagnosis was confirmed in 9 cases (3 Staphylococcus aureus, 4 Streptococcus pneumoniae, 1 Klebsiella, 1 Peptococcus). In vitro neither synergy nor antagonism were observed between the two antimicrobial agents. The acute infections episode resolved in all patients except on who died with a negative CSF culture. One superinfection meningitis with Achromobacter was seen. CSF concentrations of ceftriaxone were assayed and found to be comparable with those reported by most authors. Tolerance was excellent for all our patients.
Adult↗