Increases in levels of procollagenase messenger RNA in cultured fibroblasts induced by human recombinant interleukin 1 beta or serum follow c-jun expression and are dependent on new protein synthesis.

The protein encoded by the protooncogene c-jun, included in the activator protein-1 (AP-1) complex, is probably the critical trans-acting factor controlling transcription of the procollagenase gene which is rate limiting for subsequent synthesis of procollagenase. Therefore, to elucidate possible mechanisms whereby IL-1 stimulates procollagenase synthesis, we measured levels of c-jun and procollagenase mRNA in human serum-starved dermal fibroblasts in response to human recombinant IL-1 beta (hrIL-1 beta). hrIL-1 beta or serum induced rapid increases in c-jun mRNA levels; mRNA levels declined rapidly after hrIL-1 beta and more slowly after exposure to serum. The increases in levels of c-jun mRNA preceded the increases in procollagenase mRNA. Whereas the increases in levels of procollagenase mRNA were blunted by cycloheximide, those of c-jun mRNA were enhanced. We interpret these results as follows: IL-1 or serum induce transcription of c-jun by mechanisms independent of new protein synthesis; c-JUN, the protein product of c-jun in the AP-1 complex, is an essential mediator of the effects of IL-1 or serum in the subsequent induction of expression of the procollagenase gene.

A perimortem protocol for suspected genetic disease.

A considerable portion of pediatric deaths represent disease with risk of recurrence in subsequent family members. Procedures to obtain samples of body fluids and tissues suitable for diagnosis of mendelian and chromosomal disorders are described. These procedures, the "perimortem protocol," are used in studying children who died of suspected but undiagnosed genetic disease.

Evaluation of gestational age based on ultrasound fetal growth measurements.

Monitoring fetal growth and assessing its predictors have important place in antenatal care management. Accurate prediction of gestational age (GA) and birth weight (BW) is clinically important. Standard growth curve chosen should be evaluated to see if it satisfies the criteria for a valid assesment. In this paper, for the purpose of contributing to develop national standards and to evaluate Hadlock's standard data pertaining to 1411 fetuses were examined. Of 1411 normally growing fetuses, one measurement for AC, BPD and FL was taken by ultrasound. GA was assessed via menstrual history which is also confirmed by ultrasonography. Several variables, AC, BPD, FL, FL/AC, BPD/FL and dependent variables (GA & BW) were modelled mathematically. Percentile values, correlation coefficients were calculated and well functioning regression equations were produced for the fetal growth evaluation. Simple correlation model re-confirmed that AC, BPD and FL were well predictors of GA. Via modelling by multivariate regression analysis (adj. R2=937), GA=4.945 (95% CI: 4.661- 5.654) + .606 AC + .105 BPD + .286 FL can be estimated. It couldn't be possible establishing an appropriate equation for prediction of BW with current data. Our study is intended to draw an attention on requirement of national standards although Hadlock's standard growth curve may evaluate fetal development accurately. Forming comprehensive cohort group is under our consideration. The equation we developed (shown in the results), might be a working contribution.

Ornithine transcarbamylase deficiency and pancreatitis.

We describe a male patient with a Y202H ornithine transcarbamylase deficiency gene mutation who had pancreatitis while taking a low-protein diet, citrulline, and sodium phenylbutyrate.

Hepatic glutamine synthetase deficiency in fatal hyperammonemia after lung transplantation.

BACKGROUND: The cause of severe acquired hyperammonemia, an uncommon but often fatal complication of organ transplantation and chemotherapy for cancer, is obscure. OBJECTIVE: To test the hypothesis that liver glutamine synthetase deficiency may explain hyperammonemia in patients who have had organ transplantation or are receiving chemotherapy. DESIGN: Case report. PATIENTS: Two patients who had fatal hyperammonemia after orthotopic lung transplantation. MEASUREMENTS: Liver tissue was analyzed to determine the activities of two urea cycle enzymes and glutamine synthetase. Western blot assays for hepatic glutamine synthetase were performed to determine whether glutamine synthetase deficiency resulted from reduced enzyme levels. RESULTS: Activities of carbamoyl phosphate synthetase I and ornithine carbamoyltransferase in the liver were normal. The activity of hepatic glutamine synthetase was markedly reduced (in patient 1, 12% of the mean value in controls; in patient 2, 28% of the mean value in controls), and a concomitant reduction in the amount of glutamine synthetase protein was observed. CONCLUSION: Hyperammonemia after transplantation was associated with hepatic glutamine synthetase deficiency in two patients, but the causal relation between these two conditions must be further studied.