Canalicular obliteration from toxic epidermal necrolysis.
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Biomedical subjects
Publications and source records attributed to D Day.
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The Jarcho-Levin syndrome is a condition manifested by vertebral body and related rib malformations. We report on four new cases and review 57 cases from the literature. Analysis of the 61 cases suggests that there are two major subtypes (spondylocostal dysostosis and spondylothoracic dysostosis) with different survival rates, associated malformations, and inheritance patterns. Individuals with spondylothoracic dysostosis have vertebral body malformations and ribs which flare in a fanlike pattern but which are not significantly malformed. This is an autosomal recessive trait, and the patients have a higher mortality rate and greater incidence of neural tube defects. Individuals with spondylocostal dysostosis have vertebral malformations, frequent dramatic rib malformations, and short stature, but do not have a fanlike thoracic configuration. Most cases of spondylocostal dysostosis are inherited in an autosomal recessive fashion, although in a few families it is a dominant trait which is correlated with better survival. Respiratory compromise previously accounted for the high mortality in these conditions, but improvements in respiratory technology have increased survival. Appropriate classification of these similar phenotypes will improve counseling concerning recurrence risk, management, and prognosis.
In 1962 bone abnormalities were described radiographically in phenylketonuria patients. Later, observations were made on PKU infants during the neonatal period, which allowed differentiation between inherited alterations in bone development from those changes due to dietary restriction. Similar changes have been described in other aminoacidurias. Wrist radiographs and serum phenylalanine levels were obtained on 73 patients first seen between 1965 and 1990. All radiographs were taken on the day of referral, when the patient was between 6 and 57 days old. Forty-nine patients were less than 28 days old. Bone abnormalities were present in 56 of 73 (77%) of the children, as compared to 0 of 16 in a control group. The presence or absence of bone abnormality is unrelated to serum phenylalanine level, and to the age of the children at referral. This finding suggests that the cause of these mesodermal changes is not a deviation of a single amino acid in the infant, but that they are caused by an intrauterine amino acid imbalance.
Tumor necrosis factor (TNF) is a multifunctional immunoregulatory protein that is secreted by activated macrophages and is believed to have antiviral activities. We reported earlier that when mouse C3HA fibroblasts are infected with human adenoviruses, the 289R and 243R proteins encoded by region E1A render the cells susceptible to lysis by TNF, and a 14,700-molecular-weight protein (14.7K protein) encoded by region E3 protects the cells against lysis by TNF. We now report that the 19,000-molecular-weight (19K) (176R) protein encoded by the E1B transcription unit can protect human HEL-299 fibroblasts and human ME-180 cervical carcinoma cells against lysis by TNF. This was determined by infecting cells with adenovirus double mutants that lack region E3 and do or do not express the E1B-19K protein and by measuring cytolysis by using a short-term (18-h) 51Cr-release assay. Under these assay conditions, the 51Cr release was specific to TNF and was not a consequence of the cyt phenotype associated with E1B-19K protein-negative mutants. Also, by using virus double mutants that lack E3 in combination with other early regions, we found that E1A, the E1B-55K protein-encoding gene, E3, and E4 are not required to protect HEL-299 cells against TNF cytolysis. Three additional human cancer cell lines (HeLa, HCT8, and RC29) and a simian virus 40-transformed WI38 cell line (VA-13) also required E1B for protection against TNF cytolysis, indicating that the E1B-19K protein is required to protect many if not all human cell types against lysis by TNF when infected by adenovirus. The E1B-19K protein was not able to protect six different adenovirus-infected mouse cell lines against TNF lysis, even though the protein was shown to be efficiently expressed in one of the cell lines. HEL-299 or ME-180 cells infected by a mutant that lacks the E1B-19K protein but retains region E3 were not lysed by TNF, indicating that one or more of the E3 proteins can protect these cells against TNF lysis in the absence of the E1B-19K protein. Thus, the E3-14.7K but not the E1B-19K protein can protect adenovirus-infected mouse cells against TNF cytolysis, whereas the E1B-19K protein as well as one or more of the E3 proteins can protect adenovirus-infected human cells against TNF cytolysis.
Previous studies have shown that venous lactate specific activity during arterial tracer lactate infusion differs from arterial lactate specific activity during systemic venous tracer lactate infusion. We performed paired experiments on chronically catheterized rabbits to compare left ventricular (LV) infusion with femoral venous (FV) infusion of L-[U-14C]lactate. Blood was sampled from both the femoral artery (FA) and right ventricle (RV) during both modes of infusion. The mean lactate specific activity measured for each combination (infusion site, sampling site) was (FV,FA) 4,380 +/- 452, (FV,RV) 4,370 +/- 471, (LV,FA) 4,364 +/- 239, and (LV,RV) 3,325 +/- 240 (SE) dpm/mumol. Lactate turnover calculated from the specific activity in the (LV,RV) mode was significantly higher than from the other three modes (P less than 0.001). Models of lactate turnover are discussed demonstrating that the (FV,FA) and analogous modes of infusion sampling measure the turnover rate of lactate molecules that cycle through the circulation. This estimate of turnover is less than the turnover rate by the whole organism to the extent that some produced lactate is metabolized locally without entering the general circulation. The turnover calculated by the (LV,RV) mode overestimates the turnover of circulating lactate and relates to whole body lactate turnover in a complex manner.
Dextranase produced by Lipomyces starkeyi was purified 43-fold, by carboxymethyl-Sepharose chromatography followed by agarose gel-filtration chromatography. The purified enzyme showed four bands by SDS/polyacrylamide gel electrophoresis with estimated mass 74 kDa, 71 kDa, 68 kDa and 65 kDa. This preparation exhibited multiple isoelectric points between 5.6 and 6.1. All the isoelectric forms were active and catalytically similar. The dextranase contained a carbohydrate moiety (8%). The physical properties of the enzyme were pH and temperature optima of 5.0 and 55 degrees C, respectively. This dextranase was stable between pH 2.5 and 7.0 at temperatures below 40 degrees C. Lipomyces dextranase was a typical endodextranase with the final product of dextran hydrolysis being isomalto-oligosaccharides from glucose to isomaltotetrose.
The nucleotide and deduced amino acid sequence of a novel small (beta) subunit of methanol dehydrogenase of Methylobacterium extorquens AM1 (previously Pseudomonas AM1) has been determined. Work with the whole protein has shown that is has an alpha 2 beta 2 configuration.
Small-magnitude (2-3 beats per minute) heart-rate responses can show sex differences if assessed with a psychophysiological approach in which temporally fine-grained methods are used to determine topographical differences. Such differences emerged when 15 males and 37 females were shown videosegments depicting emotional scenes. Specifically, males accelerated to erotic segments (couples making love), while females accelerated to segments showing babies crying. In addition, the peak development of baby-cry-elicited accelerations occurred about 1 second before that of erotic segment-elicited accelerations. The results are consistent with a preparatory-response interpretation, but more research is needed both to investigate the generality of these sex differences in heart-rate responses, and to determine the role of experiential and psychosocial factors.
In vitro bacterial susceptibility data are often considered in both hospital formulary and antibiotic prescribing decisions. In this multicenter study, susceptibility data from 105 United States hospitals resulted in a database containing 211,142 isolates for 31 different bacteria and 38 antibiotics. To identify susceptibility patterns of bacteria to antibiotic alternatives, this computerized database was subjected to confidence interval analysis, while controlling for potential sources of random error. This paper describes the multicenter study design and provides several examples of the type of information the analysis can provide to P & Committee members and to practitioners who ultimately must make decisions regarding antibiotic use, sometimes without the benefit of complete hospital-specific historical antibiogram data. Limitations on the interpretation of aggregate in vitro susceptibility data gained from multiple institutions are also discussed.
Fifty patients were compared for the purpose of investigating the usefulness of intrauterine resuscitation with tocolysis (IURT). Terbutaline was given, as an intravenous bolus, to 31 women in labor in whom fetal distress was diagnosed and urgent delivery by cesarean section was indicated. In alternate months, a control group of 19 women with similar diagnoses was urgently delivered after standard interventions such as maternal positioning, oxygen administration, hydration, and discontinuation of oxytocin. Improvement in perinatal outcome was shown in infants after IURT. Apgar scores were less than 7 in 42% of the study group and in 71% of the control group at 1 minute (P = .04). Five-minute Apgar scores less than 7 occurred in 7% of the study group and 24% of the control group. A low venous pH was seen in 55% of the control group compared with 29% of the infants resuscitated with terbutaline. Estimated maternal blood loss and hematocrit change was not different in the two groups. Maternal blood pressure and pulse changes following IURT were modest and of doubtful significance. We conclude that intravenous terbutaline administered as a bolus injection at the time of fetal distress in labor improves infant outcome as evidenced by more vigorous Apgar scores and less acidemia without significant adverse physiologic effects on the mother.
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The case report of a newborn infant with a splenic hemangioendothelioma with the life-threatening complications of thrombocytopenia, anemia, and disseminated intravascular coagulation is presented together with a review of the literature. Removal of the tumor via splenectomy, despite the known risk of subsequent overwhelming sepsis due to encapsulated organisms in the young child, is the treatment of choice for splenic hemangiomas complicated by thrombocytopenia. The use of enhanced CT with delayed filling is a diagnostic tool in the workup of suspected hemangiomatous lesions.
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A prospective study of gastroenteritis based on a population was carried out for 12 months on over 7000 children in general practice. The incidence of gastroenteritis was highest in the first year (127.7 children affected per 1000) and second year (90.8) of life, and gastroenteritis was rare after six years of age. Children from urban areas had gastroenteritis more commonly than children from semirural areas. A potential pathogen was isolated from half of the specimens: 78% were viruses, and rotavirus was identified most often.
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