Aortitis syndrome in children: Clinical observation of 35 cases in Japan

The results of clinical observation of 35 patients with aortitis syndrome (AS) in childhood, obtained by a nationwide survey in Japan, are reported. The male to female ratio was 1:2.5, the estimated age of onset averaged 10.2 years, and the duration from the estimated age of onset to the diagnosis averaged 15 months. In HLA examination A24, Bw52, Cw7 and DR2 were relatively common. Arterial lesions tended to extensively involve the aortic arch and its branches. Fever was the most frequently noted clinical symptom, followed by abdomen, joint and muscle pain. The physical findings in order of frequency were impaired circulation of the upper extremities, cardiac and vascular murmurs, hypertension, impaired cerebral circulation, visual disorder and impaired circulation of the pulmonary artery. The murmurs were found not only over the chest wall but also over the cervical area and abdomen. Pulselessness of the upper extremities occurred in 66% of patients. Percutaneous retrograde aortography and/or intravenous digital subtraction angiography to make the final diagnosis was employed except for three cases. There were not any specific abnormal signs in laboratory data. Steroid hormones were administered in 34 cases, and immunosuppressive agents in 8 cases. Five cases had percutaneous transluminal angioplasty to the right renal artery as an interventional treatment. The high frequency of abdominal pain is considered to be one of the characteristics of AS in childhood. The high frequency of pulselessness of the upper extremities and cardiac and vascular murmurs in this report is considered significant for the diagnosis of AS in childhood.     

Synthesis of polypeptide models of elastin

Synthesis of two copolymers, H-(φO-Pro-Gly-Gly)_n-Val-OMe and H-(φO-Pro-Gly-Gly)_n-Val-OMe, where φO is Val or Lys and φO is Val or Glu is described. Cross-linking between the two copolymers is achieved by a coupling reaction between the εe-amino groups of the lysine containing copolymer and δ-carboxyls of the glutamic acid of the other copolymer. The cross-linking reaction was performed during a temperature elicited phase separation with flow orientation of the copolymers. Coacervation of the intermediate polymers is presented, as is the scanning electron micrograph of the insoluble cross-linked product and its calcifiability as determined with electron probe microanalysis. The purity of the key intermediates and polymers is demonstrated by the usual analytical methods. Carbon-13 magnetic resonance spectra of the intermediate monomer units are included to validate the purity of their synthesis.     

An outbreak of group B streptococcus infection in a neonatal nursery and subsequent trial for prophylaxis of nosocomial transmission

An outbreak of sepsis and meningitis caused by group B streptococcus occurred in three very low birthweight infants. To prevent further nosocomial transmission, immune globulin and ampicillin sodium were administered intravenously to other very low birthweight infants. After this prophylaxis, no other infants were involved in this outbreak. Immuno- and chemoprophylaxis may be considered as procedures to prevent nosocomial infection for very low birthweight infants.     

Developmental Toxicity of Aspirin Prenatally Given to Rats: Assessment in Sic: Wistar-KY Rats

Abstract Slc:Wistar-KY rats were administered orally with 62.5, 125, 187.5 or 250 mg/kg aspirin suspended with 0.5 % CMC-Na on days 9–11 of gestation (plug += day 0). Suppression of maternal weight gain and food consumption during treatment was observed at and over 187.5 mg/kg. At term, the fetal mortality increased at and over 187.5 mg/kg and the fetal weight was lowered at and over 125 mg/kg. Among 15 live fetuses at 250 mg/kg, 4 had external malformations. In the skeletal examination (double staining), skeletal anomalies increased at and over 187.5 mg/kg. The skeletal variations such as vertebral anomalies, fused costal cartilages and increased presacral vertebrae were often encountered and delayed ossification was also found at and over 125 mg/kg. The internal anomalies tended to increase at and over 187.5 mg/kg. The live birth rate was significantly lower at 187.5 mg/kg than that in controls, and all pups, except for 3 from a dam, died before weaning. At 125 mg/kg, the pivoting locomotion on day 7 post partum was poorer as compared with controls. The physical and functional development at 62.5 mg/kg was not changed. There were no significant effects on male offspring in the open-field, rotarod, under-water T-maze and avoidance learning tests. However, in the Biel T-maze test (9–10 weeks of age), the aspirin-treated groups showed more errors and the increased elapsed time on the 1st trial day than controls. These results indicate that aspirin may induce a slight learning defect on rat offspring even at the non-teratogenic dose and the Biel T-maze test is more sensitive than any other learning tests given in this study.     

Differential regulation of protein S expression in hepatocytes and sinusoidal endothelial cells in rats with cirrhosis

Summary.  Background: Liver dysfunction caused by intrasinusoidal microthrombi is frequently observed in patients with cirrhosis after hepatectomy, but the mechanistic pathway remains unknown. Objective: In the present study, we evaluated the expression of protein S (PS) in hepatocytes and sinusoidal endothelial cells (SECs) from rats with dimethylnitrosoamine-induced cirrhosis before and after hepatectomy. Results: The plasma level of PS antigen was significantly decreased in cirrhotic rats as compared to control rats treated with vehicle. PS expression was significantly decreased in hepatocytes isolated from cirrhotic rats as compared to controls. In contrast, PS expression was significantly increased in SECs isolated from rats with cirrhosis as compared to controls. Interkeukin-6 (IL-6) upregulated the expression of PS in hepatocytes, and tumor necrosis factor- α (TNF- α ) decreased its expression in SECs from both cirrhotic and normal rats. The production of IL-6 and TNF- α by Kupffer cells and SECs was decreased in rats with cirrhosis as compared to controls. After hepatectomy, microthrombus formation was markedly enhanced in sinusoids from rats with cirrhosis, and the plasma levels of IL-6 and TNF- α were significantly increased in rats with cirrhosis as compared to controls. Furthermore, PS production in SECs was decreased, whereas that in hepatocytes was significantly increased in cirrhotic rats as compared to controls. Conclusions: These findings suggest that PS expression is differently regulated in hepatocytes and SECs of rats with cirrhosis before and after hepatectomy, that the expression of PS is regulated by locally released inflammatory cytokines, and that decreased expression of PS in SECs may cause liver microthrombus formation, which is frequently observed in patients with cirrhosis after hepatectomy.     

Whole body oxygen consumption during extracorporeal hepatic resection: Usefulness of continuous monitoring of mixed venous oxygen saturation

Whole body oxygen consumption was measured using a thermodilution fibreoptic catheter in two patients undergoing extracorporeal hepatic resection. Each patient had virtually normal liver function before the operation. Anaesthesia was induced and maintained in a standard fashion and a venovenous bypass instituted. The anhepatic periods were 302 and 157 min. Upon removal of the liver, the oxygen consumption decreased by about 40% (50 mL/min), while the mixed venous oxygen saturation increased by about 15%. Following re-implantation, the oxygen consumption recovered and increased transiently above control values, while the mixed venous oxygen saturation changed in a reciprocal way. Monitoring whole body oxygen consumption instead of hepatic oxygen consumption seemed helpful in estimating restoration of blood flow and functions in the liver after reperfusion. It was also suggested that changes in oxygen consumption as well as those in cardiac output and haemoglobin concentration could be predicted easily by continuous monitoring of mixed venous oxygen saturation during the peri-anhepatic period.