Protein C has an important role in the regulatory mechanisms of coagulation and fibrinolysis. In patients with heterozygous protein C deficiency, there is an increased risk for thromboembolic disease, especially in the venous system. We describe a patient with protein C deficiency presenting with an acute myocardial infarction (AMI). Direct sequence analysis of the whole protein C gene detected a single base mutation at exon 7; 157 [Arg(CGA) to stop codon (TGA): 6182 C to T]. Thus, the patient was suspected to have a deficiency of the protein C heavy chain molecule, resulting in both a low protein C antigen and activity level. The mutation was also found in the propositus' son and was confirmed by differential termination of the primer extension (DTPE).

Base Sequence↗

PubMed1994

[A successful case of two-staged operation for Stanford type B acute dissecting aneurysm complicated with total occlusion of the distal abdominal aorta].

A successful case of two-staged operation for Stanford type B acute dissecting aneurysm complicated with total occlusion of the distal abdominal aorta was reported. A 62-year-old male patient with a long history of systemic hypertension developed acute severe chest, back and bilateral legs pain. An enhanced CT demonstrated Stanford type B dissecting aneurysm with occlusion of the left renal artery and bilateral common iliac arteries. On the day of admission, an emergent right axillo-femoral bypass operation using 8 mm ringed EPTFE graft was undertaken to rescue the ischemic legs. The patient's postoperative course was complicated with acute renal failure and paralytic ileus, which were treated with medical treatment. Four months later, the second operation was done for the localized residual dissecting aneurysm in the proximal descending thoracic aorta. The aneurysm was excised, and the entry was closed with Dacron patch using the previously placed axillo-femoral bypass as a technique for preventing distal ischemia. He was recovered uneventfully and was discharged in a good condition.

Acute Disease↗

PubMed1994

Distinct relationship between HLA-DR genes and intractability of ulcerative colitis.

OBJECTIVE: To explain the correlation between HLA class II genes and the intractability of ulcerative colitis (UC), DR genotyping and clinical analysis were performed for patients with UC. METHODS: HLA-DRB types and HLA-DR2 subtypes of 45 patients with UC in our hospital were determined using molecular genotyping in combination with sequence-specific oligonucleotide hybridization by polymerase chain reactions. The extent of involvement, clinical course, and treatment were studied to evaluate the intractability of UC. RESULTS: The gene frequency of DRB1*1502 in 18 patients with total colitis (0.444) was significantly higher than the group with left-sided colitis and proctitis (0.148; p < 0.05). In addition, DRw11 and DR2 increased significantly in the group including 18 cases with total colitis. In the 11 patients with total colectomy there were no cases with DRB1*1501, whereas 34 patients without operation included 12 genes of DRB1*1501 (p < 0.05). In addition, DRw11 increased significantly in the group with total colectomy compared to the group without operation (p < 0.05). When comparing between patients with and without intractability (defined based on the Japanese definition), DRB1*1502 and DRw11 were found more frequently in intractable UC patients (p < 0.01), whereas DRB1*1501 increased significantly in cases without intractability (p < 0.05). CONCLUSIONS: DRB1*1502 or DRw11 have a probability of being closely related to the intractability of UC. In addition, patients with UC possessing DRB1*1501 have a lower probability of their cases leading to intractable UC. Thus, we believe there are unknown genes present that encode the intractability of UC.

Adult↗

PubMed1994

[Coordination structures around metal ions in metalloproteins and their functions].

Explore the source record for details and available documents.

Animals↗

PubMed1994

[Inverted Y ureteral duplication with a ureteral stone in atretic segment: a case report].

A 46-year-old male was admitted with the chief complaint of right side abdominal pain. A retrograde urogram disclosed right hydronephrosis and an anomalous branch in the shape of an inverted Y accompanied by a calculus on the right ureter. Extirpation of the calculus and vesicoureteroneostomy were performed just above the bifurcation. The anomalous branch was blind-ending.

Anastomosis, Surgical↗

PubMed1994

Stabilization of human brain natriuretic peptide in blood samples.

Explore the source record for details and available documents.

Drug Stability↗

PubMed1994

[Surgical treatment of infective endocarditis--an analysis of the risk factors for operative mortality and long-term follow-up].

From September, 1978, through January, 1993, 67 patients (mean age: 44 years) underwent surgical treatment for infective endocarditis (IE) at Kagoshima University. Of 67 patients, 36 showed active endocarditis and 31 healed endocarditis at the time of operation. The over-all hospital mortality was 22.4% (15/67). Risk factors associated with operative mortality by univariate analysis included increased blood urea concentration (BUN), increased cardiothoratic ratio, higher New York Heart Association functional class, infection status (active/healed), aortic valve infection, prolonged aortic cross-clamp time, annular abscess, and calendar year. The patients were operated earlier in the latter part of this series (1988-1993. Multiple logistic regression analysis demonstrated three factors to be statistically significant: BUN, operative year, and aortic valve infection. Complete survival information was obtained in all 52 discharged patients with a mean follow-up time of 4.8 years. There were 5 late deaths including 3 patients with valve related complication. The actuarial survival rate at 10 years after operation excluding hospital mortality was 89%, 92% for active infection 88% for healed infection. The actuarial freedom from valve related complication was 86% at 10 years. The degree of activity of the infection is not a risk factor for operative mortality by multiple logistic regression analysis. We conclude that surgical intervention should be undertaken without delay in the patients with IE before development of hemodynamic deterioration causing renal dysfunction and hypercatabolism.

Adolescent↗

PubMed1994

[The correlation of the severity of stress urinary incontinence with static and stress urethral pressure profiles].

The urethral pressure profile (UPP) at rest and under stress was recorded by means of double lumen microtip transducer catheter in 58 females with stress urinary incontinence (SUI). The patients were divided into three groups including mild to moderate SUI patients (n = 29, group 1), severe SUI patients (n = 16, group 2) and very severe SUI patients (n = 13, group 3) according to a one-hour pad test. The functional urethral length (FUL) was divided into four equal lengths and the pressure transmission ratio (PTR) was calculated for each quartile for each female. We examined the correlation of the severity of SUI with urethral pressure parameters: maximum urethral closure pressure (MUCP) at rest and functional urethral length (FUL) at rest and PTR. SUI patients showed a negative association between the severity of SUI and MUCP, but no significant differences in the FUL were seen between the different groups. PTR was shown to be similar in order for all groups of SUI patients showing a linear decrease in the value of the PTR along the FUL. However, we found a negative association between the severity of SUI and PTR (P < 0.05) at the middle part of the FUL (Q3). These findings indicate that there are more defective distal sphincter mechanisms in severe SUI patients than in mild to moderate SUI patients. There was an overlap between the different groups in both MUCP and PTR at the middle urethra (Q3), suggesting that other parameters are related with the severity of SUI.

Adult↗

PubMed1994

[Studies on hypogeusia in hyperemesis gravidarum].

We measured changes in gustatory sensitivities and gustation threshold levels and those of some trace elements, vitamin E and lipids in the serum of 38 patients suffering from hyperemesis gravidarum, 12 normal pregnant women in the first trimester and 22 healthy non-pregnant women by means of filter discs and an electrogusto meter (EGM) and we obtained the following results. 1. Gustatory sensitivity to sweetness was found to be significantly lowered in the patients with hyperemesis gravidarum when tested with filter discs (p < 0.05). 2. Gustation threshold levels were significantly lowered in the patients with hyperemesis gravidarum and the normal pregnant women tested with the EGM (p < 0.01). 3. There was considerable heterogeusia of women in the patients with hyperemesis gravidarum (p < 0.05). 4. Zn and Mg in serum decreased (p < 0.01), but Cu increased significantly in the patients with hyperemesis gravidarum and the normal pregnant women (p < 0.01). 5. Total cholesterol and phospholipid in serum significantly decreased, but lipid peroxide in serum significantly increased in the patients with hyperemesis gravidarum (p < 0.01). These results suggested that the gustatory test was helpful in investigating hyperemesis gravidarum and that biological defense systems (scavenger systems) to free radicals were damaged in patients with hyperemesis gravidarum.

Adult↗

PubMed1993

Modification of contractile proteins by oxygen free radicals in rat heart.

This study was undertaken to investigate the effects of oxygen free radicals on myofibrillar creatine kinase activity. Isolated rat heart myofibrils were incubated with xanthine+xanthine oxidase (a superoxide anion radical-generating system) or hydrogen peroxide and assayed for creatine kinase activity. To clarify the involvement of changes in sulfhydryl groups in causing alterations in myofibrillar creatine kinase activity, 1) effects of N-ethylmaleimide (sulfhydryl groups reagent) on myofibrillar creatine kinase activity, 2) effects of oxygen free radicals on myofibrillar sulfhydryl groups content, and 3) protective effects of dithiothreitol (sulfhydryl groups-reducing agent) on the changes in myofibrillar creatine kinase activity due to oxygen free radicals were also studied. Xanthine+xanthine oxidase inhibited creatine kinase activity both in a time- and a concentration-dependent manner. Superoxide dismutase (SOD) showed a protective effect on the depression in creatine kinase activity caused by xanthine+xanthine oxidase. Hydrogen peroxide inhibited creatine kinase activity in a concentration-dependent manner; this inhibition was prevented by the addition of catalase. N-ethylmaleimide reduced creatine kinase activity in a dose-dependent manner. The content of myofibrillar sulfhydryl groups was decreased by xanthine+xanthine oxidase; this reduction was prevented by SOD. Furthermore, the depression in myofibrillar creatine kinase activity by xanthine+xanthine oxidase was protected by the addition of dithiothreitol. Oxygen free radicals may inhibit myofibrillar creatine kinase activity by modifying sulfhydryl groups in the enzyme protein. The reduction of myofibrillar creatine kinase activity may lead to a disturbance of energy utilization in the heart and may contribute to cardiac dysfunction due to oxygen free radicals.

Animals↗

PubMed1993

The outcome of patients with thyroid carcinoma and Graves' disease.

A total 847 consecutive patients with Graves' disease who underwent thyroidectomy between 1965 and 1990 were found to have a 4.3% incidence of coincident carcinoma of the thyroid. In 68.2% of these cases the tumors were less than 1 cm in diameter and were detected in only 7 patients (19.4%) prior to surgery. Dissection of the regional lymph nodes was necessary in only 11 patients. A review of the patients in 1990 revealed no carcinoma-related deaths with metastases occurring most commonly in the patients with larger tumors and in those whose cancer had been detected prior to thyroidectomy. Although the detection of potential tumors occurring with Graves' disease using preoperative echogram may be of interest, our results suggest that occult microcarcinoma occurring coincidentally with Graves' disease is not clinically significant as it did not cause any recurrence in this study. Moreover, there was no evidence to suggest that Graves' disease was associated with the increased or rapid growth of these tumors.

Adenocarcinoma↗

PubMed1993

Lumbar artery pseudoaneurysm: an unusual cause of a retroperitoneal hematoma: report of a case.

Pseudoaneurysm of the lumbar artery is a rare complication of penetrating trauma. We present herein a case thought to have been caused by a blow to the left flank without any evidence of a stab wound. In this patient, the diagnosis of a first lumbar artery pseudoaneurysm with a retroperitoneal hematoma was confirmed by computed tomography (CT) findings, after which transcatheter embolization was successfully performed.

Aneurysm↗