Systemic lupus erythematosus in children: the complex problems of diagnosis and treatment encountered in 101 such patients at the Mayo Clinic

Between 1945 and 1970, 101 children (86 girls and 15 boys) with systemic lupus erythematosus were evaluated at the Mayo Clinic. Only 9 children were less than 9 years old at the time of diagnosis. The most frequent presenting complaint was arthralgia; fever, fatigue, and a "butterfly" malar rash also were common. Renal involvement, found in more than 76 per cent of patients, was a prognostically poor sign. The overall survival of children with renal involvement is improved by the use of adequate steroid therapy.     

Persistent acute renal failure with renal artery stenosis: cure following reconstructive arterial operation

A case of acute renal failure is reported in which renal function returned after surgical correction of renal artery stenosis to the sole functioning kidney. Acute renal failure associated with renal vascular lesions is reviewed. Although often clinically apparent vascular obstruction may be asymptomatic and should be considered in cases of prolonged acute renal failure.     

Patient compliance in glaucoma

Of a randomly selected sample of 40 patients with chronic simple glaucoma 11 were identified as having failed to comply adequately with medical advice. Noncompliers were more likely: to be men, to have had no other medical disorder but glaucoma, not to rank glaucoma as most troubling if they had another illness, to have experienced side effects from the treatment, and not to have appreciated the association between glaucoma and blindness. Detailed clinical study revealed that several interrelated psychosocial factors contributed to noncompliance.     

Modification and abolition of re-entry within the His-Purkinje system in man by diphenylhydantoin

The phenomenon of macrore-entry (Re) within the His-Purkinje system (HPS) was consistently observed in 10 of 19 patients during retrograde refractory period studies. Effects of intravenous infusion of diphenylhydantoin (DPH) on Re were studied in these 10 patients 10 minutes after completion of infusion (mean plasma level equal to 17.0 microgram/ml). Diphenylhydantoin modified determinants of Re in seven patients (group I) and abolished Re in the remaining three patients (group II). In group I, DPH shortened the critical V1 V2 from 310.0 +/- 30.5 to 292.9 +/- 25.6 msec (P less than 0.025) and critical V2 H2 intervals for Re from 201.4 +/- 18.4 to 185.0 +/- 13.8 msec (P greater than 0.05). In group II, DPH abolished Re in two of three patients by precluding attainment of critical V2 H2 intervals whereas Re was abolished in the remaining one patient despite attainment of critical V2 H2 intervals (vs control). For both groups, DPH significantly shortened functional and effective refractory periods of the HPS (P less than 0.001 and less than 0.01, respectively) without significantly affecting the effective refractory period of the ventricular muscle. Diphenylhydantoin either completely abolished or significantly shortened the retrograde gap zones in the HPS. It is concluded that diphenylhydantoin significantly shortens His-Purkinje system refractoriness, abolishing Re in the patients with higher degree of improvement in refractoriness.     

Pancreatic cancer screening. Analysis of the problem and the role of radionuclide imaging

The clinical, surgical, and pathologic findings in a five year prospective study of 192 patients referred with a high probability of pancreatic cancer are reported. We have defined the requirements of any pancreatic imaging procedure as its ability to distinguish a normal pancreas from pancreatic cancer or chronic pancreatitis and the capability of detecting tumors less than 5 cm in diameter. There was a 47 percent incidence of pancreatic disease (27 percent pancreatic cancer and 20 percent chronic pancreatitis). Prospective radionuclide imaging as routinely performed was found to be of little clinical value in this patient population; it was neither specific nor sensitive to pancreatic cancer or chronic pancreatitis. Preliminary data with longitudinal multiplane emission tomography show an improved diagnostic accuracy and the ability to detect resectable tumors, but its efficacy needs to be prospectively compared with other screening tests on a carefully defined patient population.     

Effectiveness of Zataria multiflora Boiss essential oil and grape seed extract impregnated chitosan film on ready-to-eat mortadella-type sausages during refrigerated storage

BACKGROUND: The effectiveness of chitosan films containing Zataria multiflora Boiss essential oil (ZEO) (5 and 10 g kg^?1) and grape seed extract (GSE) (10 g kg^?1) on lipid oxidation and microbial (lactic acid bacteria, aerobic mesophiles and inoculated Listeria monocytogenes) characteristics of mortadella sausage at 4 °C for 21 days was evaluated. The release of total phenolics (TPs) into sausage was also assessed. RESULTS: All films exhibited antibacterial activity against L. monocytogenes on agar culture media. Chitosan films containing ZEO were the most effective on the growth of bacteria. The growth of L. monocytogenes was significantly inhibited by ZEO‐GSE containing films especially during storage of the sausages for 6 days. Aerobic mesophiles and lactic acid bacteria were the most sensitive and resistant groups to films by 0.1–1.1 and 0.1–0.7 log cycles reduction, respectively. Sausages wrapped by 10 g kg^?1 GSE + 10 g kg^?1 ZEO films had the lowest degrees of lipid oxidation, which was 23% lower than the control. The TPs of ZEO films decreased to zero after 6 days, whereas TPs of GSE films followed a slight decrease during the storage. CONCLUSION: Antimicrobial/antioxidant chitosan film could be developed by incorporating GSE and ZEO for extending the shelf life of mortadella sausage. Copyright © 2011 Society of Chemical Industry     

Rapid activation of protein C by factor Xa and thrombin in the presence of polyanionic compounds

A recent study indicated that negatively charged substances such as heparin and dextran sulfate accelerate thrombin activation of coagulation factor XI by a template mechanism. Because the serine proteinase of the natural anticoagulant pathway, activated protein C, can bind heparin, it was reasonable to think that these compounds may also bind protein C (PC) and accelerate its activation by thrombin or other heparin binding plasma serine proteinases by a similar mechanism. To test this, PC activation by thrombin and factor Xa (fXa) was studied in the presence of these polysaccharides. With thrombin in the absence of thrombomodulin (TM), these polysaccharides markedly reduced the Km for PC and Gla-domainless PC (GDPC) activation in the presence of Ca2+. With TM containing chondroitin sulfate, heparin did not influence PC activation by thrombin, but with TM lacking chondroitin sulfate, the characteristic high-affinity PC interaction at low Ca2+ (approximately 50 to 100 micromol/L) was largely eliminated by heparin. In EDTA, heparin enhanced thrombin activation of GDPC by reducing the Km, but it inhibited PC activation by increasing the Km. PC activation in EDTA was insensitive to the presence of heparin if the exosite 2 mutant, R93,97,101A thrombin, was used for activation. These results suggest that, when the Gla-domain of PC is not fully stabilized by Ca2+, it interacts with the anion binding exosite 2 of thrombin and that heparin binding to this site prevents this interaction. Additional studies indicated that, in the presence of phospholipid vesicles, heparin and dextran sulfate dramatically accelerate PC activation by fXa by also reducing the Km. Interestingly, on phospholipids containing 40% phosphatidylethanolamine, the activation rate of near physiological PC concentrations ( approximately 80 nmol/L) by fXa in the presence of dextran sulfate was nearly comparable to that observed by the thrombin-TM complex. The biochemical and potential therapeutical ramifications of these findings are discussed.