The kidney plays an important role in the blood pressure regulation primarily by modulating tubular sodium reabsorption. Various hormones, vasoactive peptides, autacoids and transporters or channels in renal tubules are involved in this process. Genes associated with renal tubular sodium handling are possibly related to the development of hypertension. Genes of the renin-angiotensin-aldosterone system are thought to be especially important as causal genes of hypertension. Na-K-ATPase, biochemically equal to Na pump, exists on the basolateral membrane of renal epithelial cells. It plays a central role in Na reabsorption and creates a driving force for transepithelial transport. Na-K-ATPase activity is regulated by adducin, a membrane-bound skeletal protein, as well as by several hormones such as dopamine, endogenous ouabain-like factor or cytochrome P450 metabolites. Genes of these factors involved in Na-K-ATPase regulation should be related to the development of hypertension. The endothelin system, atrial natriuretic peptide and nitric oxide regulate the tonus of blood vessels as well as renal sodium excretion. Several reports have indicated that genes of these substances are crucial in the pathogenesis of hypertension.
(Internal Medicine 38: 919-926, 1999)

抄録全体を表示

Objective Evaluation of the usefulness of the serial daterminations of serum alpha-L-fucosidase (AFU) activity for prediction of the development of hepatocellular carcinoma (HCC) was performed. Methods and Patients Serum AFU activity was determined monthly for 42 months in 73 patients with liver cirrhosis (LC). Results HCC was diagnosed in 27 patients by means of ultrasonography during this observation period. In 23 (85%) of the 27 patients, serum AFU activity was found to exceed 700 nmole/ml/h during the LC stage. HCC developed within a few years in 23 (82%) of 28 LC patients with AFU activity exceeding 700 nmole/ml/h, in contrast, it developed in only 4 (9%) of 45 LC patients with AFU activity below 700 nmole/ml/h. AFU activity was already elevated in 23 (85%) of 27 patients at least 6 months before the detection of HCC by ultrasonography. Conclusion It is conceivable that the development of HCC can be predicted by means of serial determinations of serum AFU activity in patients with LC.
(Internal Medicine 38: 927-931, 1999)

抄録全体を表示

Objective The distribution of JC virus DNA in peripheral blood was surveyed by the polymerase chain reaction using the late genes as markers. Results Six out of 52 cases of hematological diseases and one systemic lupus erythematosus case out of 17 cases were positive for JCV DNA. After separation into B and T lymphocytes by a cell sorter, JCV DNA was found in both cell types prepared from adult T cell leukemia and PML patients. Conclusion Only 1 or 2 copies of JCV genome were calculated to exist in a cell based on the time course analysis of PCR. Only B lymphocytes and glial brain cells are known to produce nuclear factors which support the growth of the virus. The result that B lymphocytes contained a copy number of JCV genome similar to T lymphocytes suggests that there is some barrier to viral growth in susceptible B lymphocytes, and that the growth of JCV is different from that of other virulent viruses.
(Internal Medicine 38: 932-937, 1999)

抄録全体を表示

Objective To evaluate efficacy of low dose prednisolone maintenance in patients with primary Sjögren's syndrome. Methods An open, prospective pilot study of prednisolone for the treatment of 20 patients with primary Sjögren's syndrome was performed. Evaluations included the amount of whole saliva measured by the Saxon test, serological abnormalities and oral symptoms. Results Initial dosage of prednisolone was 15.0±1.5 (mean±SEM) mg/day. Maintenance dosage was 7.5-5.0 mg/day. Follow-up period was 26.3±3.8 months (range 3-48). The amount of whole saliva significantly increased after 1 month of prednisolone therapy and the increase continued up to 48 months by maintaining low-dose prednisolone. A mean percent increase of whole saliva from baseline ranged from +105.2±36.2% to +245.7±82.1%. Serum IgG, anti-SS-A/Ro, anti-SS-B/La antibodies and IgM rheumatoid factor levels significantly decreased throughout the study with partial decreases of IgA and IgM levels. The improvement of subjective oral symptoms was also confirmed. Conclusion Low-dose prednisolone maintenance may have a worthwhile clinical benefit in patients with primary Sjögren's syndrome that deserves further evaluation in a controlled trial.
(Internal Medicine 38: 938-943, 1999)

抄録全体を表示

Objective Thiopurine S-methyltransferase (TPMT) is a cytosolic enzyme that preferentially catalyzes the S-methylation of aromatic and heterocyclic sulfhydryl compounds, including Azathioprine (AZA). It has been reported that the level of AZA toxicity is dependent on the TPMT genotypes in Caucasian individuals; we thus investigated this relationship in Japanese. Methods The TPMT genotype was determined using peripheral blood cell DNA obtained from 36 Japanese patients with rheumatic diseases who were treated with AZA, by polymerase chain reaction (PCR) technique. Duration of AZA administration, white blood cell counts before and after AZA administration, and side effects were investigated in each subject, and were compared between the patients with or without TPMT mutation. Results The TPMT allelotype was TPMT^*1/TPMT^*1 in 33 (91.7%) and TPMT^*1/TPMT^*3C in 3 (8.3%) individuals. All 3 patients (100%) with the mutant TPMT allele (TPMT^*3C) discontinued AZA treatment due to leucopenia while only 4 patients (12%) without mutant TPMT alleles showed leucopenia (p=0.0049, Fisher's exact test). However, leucopenia developed relatively late in patients with mutant TPMT. Conclusion The TPMT mutant allele, TPMT^*3C, also exists in Japanese individuals, and the bone marrow toxicity of AZA is likely stronger in patients with this mutant allele.
(Internal Medicine 38: 944-947, 1999)

抄録全体を表示

Objective The detection of circulating Aspergillus galactomannan antigen is a useful tool for serodiagnosis of aspergillosis. However, the latex agglutination test for the detection of galactomannan is not completely reliable due to it's low sensitivity. The sandwich ELISA was developed to achieve high sensitivity. Materials The sandwich immunocapture ELISA was evaluated by testing 56 sero-positive and 56 sero-negative samples of circulating galactomannan detected by LA test retrospectively. Results Sixty of the samples were positive for galactomannan as measured by sandwich ELISA. Fifteen samples out of 56 samples negative by LA test were positive by ELISA and 4 samples out of 56 samples positive by LA test were negative by ELISA. Among 47 serum samples positive for anti-Aspergillus antibody, 14 samples were positive by ELISA. Conclusion In conclusion, galactomannan may be detected in more samples of by the new sandwich ELISA than by LA test.
(Internal Medicine 38: 948-950, 1999)

抄録全体を表示

The incidence of invasive ductal carcinoma of the pancreas was 3.1% (6 cases) in 196 patients with definite chronic pancreatitis. Five patients (3 men and 2 women) had calcific pancreatitis and 1 patient (man) had non-calcific pancreatitis. Large pancreatic stones were recognized in 2 women. Most of the patients complained of continuous intractable abdominal pain and/or back pain together with weight loss and appetite loss. Serum CA19-9 levels and exacerbation of glucose intolerance were retrospectively noted to have been elevated in 1 patient. However, it was difficult to obtain a definitive diagnosis by imaging examinations earlier, due to the presence of chronic pancreatitis. Median survival of the 6 patients was 6.5 months from admission.
(Internal Medicine 38: 951-956, 1999)

抄録全体を表示

Primary duodenal MALT lymphoma (MALToma) is a very rare neoplasm arising from the mucosa-associated lymphoid tissue of the duodenum. We report a 55-year-old woman with MALToma located in the descending duodenum and accompanying Helicobacter pylori infection of the stomach. We performed operative resection due to involvement of the papilla of Vater and submucosal tumor infiltration. Despite wide mucosal spreading, postoperative examination revealed only a small amount of MALToma cells infiltrating into the submucosa. No invasion into the adjacent structure or metastasis to regional lymph nodes was confirmed, suggesting the disease could have been controlled by eradication of Helicobacter pylori.
(Internal Medicine 38: 957-961, 1999)

抄録全体を表示

A 55-year-old Japanese housewife, who had Osier-Weber-Rendu disease, was admitted to our hospital because of frequent epistaxis and worsening exertional dyspnea. The computed tomography and hepatic arteriography revealed large hepatic arteriovenous malformation, which was considered to be the leading cause of her high output heart failure. Two series of hepatic arterial coil embolization procedures were performed to reduce hepatic shunt flow. They temporarily improved her cardiac condition, but gradually induced progressive hepatic failure due to intrahepatic cholangitis. Hepatic dysfunction restricted her quality of life and lead to a fatal clinical course one year after the second coil embolization.
(Internal Medicine 38: 962-968, 1999)

抄録全体を表示

We report a case of adrenocorticotropic hormone (ACTH)-independent bilateral macronodular adrenocortical hyperplasia (AIMAH), which was successfully treated with niitotane. A 71-year-old man visited our hospital because of central obesity and enlarged bilateral adrenal glands. The endocrinological studies showed elevated plasma cortisol and undetectable levels of ACTH, a lack of suppression with high-dose dexamethasone and a hyper-response to exogenous ACTH. These clinical features were compatible with the diagnosis of AIMAH. In this patient, extra-adrenal multiple tumors were also detected. After treatment with mitotane, the plasma level of cortisol was decreased while that of ACTH was increased and the signs of Cushing's syndrome were resolved.
(Internal Medicine 38: 969-973, 1999)

抄録全体を表示

We report the transient spontaneous disappearance of a mucocele due to bronchial atresia. Two years before presentation, a chest radiograph showed a hyperlucent right upper lung and a mucocele near the right hilum. A chest radiograph taken 1 year later showed that the mucocele had disappeared leaving an ovoid outline of a dilated bronchus. A chest radiograph obtained 3 months before presentation showed that the mucocele was present again. Atresia of the B³_b bronchus of the right upper lobe was noted on thoracotomy. The "disappearance" of the mucocele probably was due to the clearance of mucoid material through collateral airways.
(Internal Medicine 38: 974-978, 1999)

抄録全体を表示

A 20-year-old man with squamous cell carcinoma of the lung is described. Histology of the open lung biopsy specimen revealed squamous cell carcinoma with definite keratinization. Interestingly, the tumor cells were characterized by partial expression of CD34 antigen and neuroendocrine differentiation. The diagnosis was delayed in this case because of his young age. This delayed diagnosis resulted in rapid progression and short survival time. In our review of 667 cases of lung cancers at Okayama University Hospital, only 3 (0.4%) of them were under 30 years of age and also showed advanced stage and very poor prognosis. It is important to take note of lung cancer as a differential diagnosis to detect early-stage lung cancer in young patients when they present with abnormal shadow on chest radiograph.
(Internal Medicine 38: 979-983, 1999)

抄録全体を表示

Disease activity in Japanese sarcoidosis patients is generally mild. However, the pulmonary sarcoidosis coexisting with connective tissue disease is likely to be progressive. We report here three cases of sarcoidosis coexisting with connective tissue diseases, who developed pulmonary manifestations from stage II to stage III.
(Internal Medicine 38: 984-987, 1999)

抄録全体を表示

The case of a 53-year-old man who developed cavernous sinus syndrome (CSS) four years after being diagnosed as having nonsecretory myeloma is described. He was admitted with diplopia and dull pain over the right infraorbital and zygomatic region in June 1997. The cause of CSS was the intracranial involvement of myeloma, which was diagnosed by fiberscopic biopsy. The results of endocrinologic evaluation were almost normal. The response to radiotherapy and chemotherapy was mild. CSS caused by nonsecretory myeloma is rare and its prognosis is poor. More aggressive chemotherapy with stem cell support may be indicated.
(Internal Medicine 38: 988-990, 1999)

抄録全体を表示

A 57-year-old man with renal cell carcinoma and erythrocytosis showed a high serum level of erythropoietin (EPO). High EPO signal was observed on Northern blot analysis and RT-PCR in the total RNA extracted from the renal tumor. Immunohistochemical staining also demonstrated tumor tissue with high immunostaining of EPO. Nucleotide sequences of EPO cDNA in the tumor were normal. To date, only one report has discussed the nucleotide sequences of a tumor's EPO gene; it showed mutant EPO cDNA in hepatocellular carcinoma tissue. This is the first demonstration of normal EPO cDNA in renal cell carcinoma.
(Internal Medicine 38: 991-994, 1999)

抄録全体を表示

We encountered a case of Behçet's disease complicated with central diabetes insipidus. A hypothalamopituitary dysfunction is rare in Behçet's disease; only three cases of this association have been reported in the literature. Magnetic resonance imaging of the brain showed a dilatation of the left Sylvian vein and thickening of the pituitary stalk, which suggested intracranial vasculitic processes and lymphocytic infundibuloneurohypophysitis. The possible relationship of this disease combination is discussed as a form of autoimmune disease.
(Internal Medicine 38: 995-999, 1999)

抄録全体を表示