A Clinical Study for the Durability of Oxygenators on Cardiopulmonary Support

Abstract: Cardiopulmonary support (CPS) requires durability of the oxygenator. The life span of the oxygenator is affected by various clinical factors, including patient condition, perfusion condition, and equipment usage. Predictors for the durability of oxygenators were evaluated clinically in this study. Thirty-two patients, who had undergone CPS during the last 3 years in our institute were assigned to this study. Fifty oxygenators had been used (Capiox SX in 19, CB Maxima in 23, and AL-6000 in 8). Significant predictors for the durability of oxygenators were evaluated by nonparametric survival analysis and proportional hazards regression analysis. Univariate regression analysis revealed 6 significant predictors for the life span of oxygenators. These were the oxygenator type, type of centrifugal pump, acidosis with blood pH less than 7.35, base excess less than -5, blood glutamic-oxaloacetic transaminase (GOT) levels greater than 1,000 IU, and blood lactate dehydrogenase (LDH) levels greater than 3,000 IU. After multivariate analysis, there remained only 2 significant predictors. An oxygenator used with a noncoated CPS system (Capiox SX with Capiox EBS) proved to have a significantly shorter life span than one used with a heparin-coated system (CB Maxima or AL-6000 with CB BP-80) (hazards ratio, 3.588, p = 0.0065). Patient conditions, which revealed acidosis with less than -5 of base excess, significantly shortened the life of the oxygenator (hazards ratio, 3.595, p = 0.0188).     

Ileal atresia with perforation in siblings

Two siblings with ileal atresia are described; one boy presented with ileal perforation and the other with meconium peritonitis, diagnosed by antenatal ultrasonography. The site and type of atresia, complications and associated anomalies of reported cases among siblings also are discussed.     

Heterozygous abnormal fibrinogen Osaka III with the replacement of gamma arginine-275 by histidine has an apparently higher molecular weight gamma-chain variant.

Congenitally abnormal fibrinogen Osaka III with the replacement of gamma Arg-275 by His was found in a 38-year-old female with no bleeding or thrombotic tendency. Release of fibrinopeptide(s) by thrombin or reptilase was normal, but her thrombin or reptilase time in the absence of calcium was markedly prolonged and the polymerization of preformed fibrin monomer which was prepared by the treatment of fibrinogen with thrombin or reptilase was also markedly defective. Propositus' fibrinogen had normal crosslinking abilities of alpha- and gamma-chains. Analysis of fibrinogen chains on sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) in the system of Laemmli only revealed the presence of abnormal gamma-chain with an apparently higher molecular weight, the presence of which was more clearly detected with SDS-PAGE of fibrin monomer obtained by thrombin treatment. Purified fragment D1 of fibrinogen Osaka III also seemed to contain an apparently higher molecular weight fragment D1 gamma remnant on Laemmli gels, which was digested faster than the normal control by plasmin in the presence of [ethylenebis(oxyethylenenitrilo)]tetraacetic acid (EGTA).     

GLUCOCORTICOID-UNRESPONSIVE FEVER IN A PATIENT WITH WEBER-CHRISTIAN DISEASE

SUMMARY Weber-Christian disease, a disease of unknown aetiology, is characterised by relapsing febrile episodes and systemic panniculitis. Glucocorticoid therapy is often useful during acute phases of the disease. This report describes a patient in whom hyperpyrexia did not respond to high-dose glucocorticoid treatment, yet did respond to a non-steroidal anti-inflammatory drug (NSAID).     

Incidence of renal cell carcinoma in Chiba Prefecture, Japan; ten years experience]

Many reports about the increase of renal cell carcinoma patients have been published in Japan recently, however, the real fluctuations in the total number of patients in relation to the change of population have not been reported yet. Most of the patients with renal cell carcinoma in the last 10 years were examined in Chiba prefecture, which has a population of about five million and 25 active urological offices. Histologically confirmed cases were investigated by sending questionnaire letters. The items were as follows; sex, age, address, occupation, family history, past history, symptoms, examination methods that first detected the tumor, operation date, tumor diameter and clinical stage. Twenty two offices returned answers and 560 cases who lived in Chiba were found to have renal cell carcinoma from 1980 to 1989. Yearly incidence rates per 100,000 persons demonstrated a significant increase from 0.32 to 2.07. Small, asymptomatic and low stage cancers have been increasing rapidly, however, the rate of metastatic disease has not shown any decrease. The main cause of rapid increase seems to be attributed to progress in diagnostic methods and increase of early detection, but the possibility of an increase in some carcinogenic factors can not be ruled out.     

Mutations of the human thyrotropin-beta subunit glycosylation site reduce thyrotropin synthesis independent of changes in glycosylation status.

In recent studies, site-directed mutagenesis has been used to alter the tripeptide glycosylation recognition sequences of glycoprotein hormone subunits, thereby affecting their structure and function. However, it is not known whether these effects result from changes in glycosylation status, amino acid sequence, or both. We therefore studied the synthesis of wild-type and mutant recombinant human thyrotropins produced by transient transfection of a human cell line. Mutating the TSH-beta subunit glycosylation recognition sequence, Asn-Thr-Thr (codons 23-25), to either Gln-Thr-Thr or Asn-Thr-Tyr abolished subunit glycosylation, as demonstrated by the inability to incorporate 3H-carbohydrates. However, a third mutation (Asn-Thr-Ser) contained an intact glycosylation recognition sequence site, and was shown to retain glycosylation. The mutations that abolished TSH-beta subunit glycosylation resulted in greater than 90% decreases in TSH synthesis. However, the glycosylation recognition sequence mutant that retained beta subunit glycosylation exhibited a 70% decrease in TSH production. These decreases were not attributable to the intracellular accumulation of TSH or its free beta subunit. We also engineered two TSH-beta subunit mutations that did not alter the glycosylation recognition sequence. A glycine to arginine mutation adjacent to the glycosylation recognition sequence, in a region thought to be critical for heterodimer formation, abolished TSH production. In contrast, shortening the TSH-beta subunit carboxyterminus by six amino acids increased TSH synthesis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Primary malignant lymphoma of the rectum with distant metastasis to the stomach: Report of a case

Primary extranodal malignant lymphoma frequently occurs in the gastrointestinal tract; however, it is rarely encountered in the rectum. We report herein the case of an 85-year-old man who underwent abdominoperineal resection for primary malignant lymphoma of the rectum, and 1 year later, developed hematemesis caused by gastric metastasis. To our knowledge this is the first case report of such distant metastasis developing from malignant lymphoma of the rectum.     

Kimura's disease associated with minimal change nephrotic syndrome: A case report

Kimura's disease is a rare disorder that involves regional lymph nodes and the major salivary glands, which become infiltrated by eosinophils and lymphocytes. Renal lesions associated with Kimura's disease are rare. We describe the case of a 60-year-old Japanese woman who first noted a nodular mass in a salivary gland. As the nodule grew, nephrotic syndrome and heart failure developed. A biopsy of the nodule revealed Kimura's disease, and surgical excision was performed. After the operation, the heart failure and nephrotic syndrome, which were diagnosed as minimal change disease on renal biopsy, improved rapidly without steroid therapy. Four months later, the nephrotic syndrome recurred without recurrence of Kimura's disease. The patient showed marked improvement during prednisolone therapy (40 mg/d) and was in complete remission 4 weeks after the initiation of steroid therapy. This case shows that surgical excision and prednisolone therapy are useful for nephrotic syndrome associated with Kimura's disease.     

Periodic alternating nystagmus and rebound nystagmus in spinocerebellar ataxia type 6.

We report on a family with ataxia type 6 (SCA6) showing peculiar oculomotor symptoms. The proband presented with periodic alternating nystagmus (PAN), and her 2 brothers had rebound nystagmus and gaze-evoked nystagmus. They carried the identical mutation (the number of expanded CAG repeat, 24) in the CACNA1A gene. The intrafamilial variability of oculomotor symptoms may be ascribed to factors other than CAG repeat expansion size in SCA6.