Primary focal segmental glomerulosclerosis (FSGS) is the representative of refractory nephrotic syndrome in both adults and children. We review the clinical course and predictors of renal outcome in adult FSGS. Patients resistant to treatment frequently develop end-stage renal disease (ESRD), whereas patients achieving a remission show an excellent outcome. The renal survival rate in Japanese patients is 68.7% in 10 years and 31.4% in 20 years, indicating a better prognosis compared with the previous studies. When clinical and histological features at presentation have been evaluated by multivariate analysis, serum creatinine concentrations (>1.5 mg/dl) and the presence of tubulo-interstitial lesions (>20%) are significant positive predictors of progression to ESRD. We also discuss treatment for adult FSGS, with emphasis on intensive and prolonged therapy.
(Internal Medicine 39: 606-611, 2000)

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Objective The quantitative gated SPECT (QGS) software is able to calculate LV volumes and visualize LV wall motion and perfusion throughout the cardiac cycle using an automatic edge detection algorithm of the left ventricle. We evaluated the reliability of global and regional LV function assessment derived from QGS by comparing it with the results from left ventriculo-cineangiography (LVG).
Patients In 20 patients with left ventricular dysfunction who underwent ECG gated ^99mTc-tetrofosmin SPECT, the end-diastolic volume (EDV), end-systolic volume (ESV) and ejection fraction (LVEF) were calculated. The QGS-assessed regional wall motion was determined using the cinematic display.
Results QGS-derived EDV, ESV and LVEF correlated well with those by LVG (p<0.001 for each). There was a good correlation between wall motion score (WMS) derived from the QGS and the LVG (r=0.40, p<0.05). In some patients with extensive myocardial infarction, there was a discrepancy in the regional wall motion results between QGS and LVG.
Conclusions The ECG-gated SPECT using QGS is useful to evaluate global and regional LV functions in left ventricular dysfunction.
(Internal Medicine 39: 612-617, 2000)

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Objective IL-5 is strongly involved in the eosinophilic inflammation in bronchial asthma and atopic dermatitis. We have previously reported that IL-5 synthesis in atopic and nonatopic asthmatics is significantly enhanced compared to control subjects. T cell IL-5 synthesis is regulated through several transcriptional elements, one of which is the proximal human IL-5 promoter (-62 to -46). The present study was undertaken to delineate the transcriptional regulation through this element using nontransformed human T cells.
Methods Con A blast lymphocytes which tolerate electroporation were derived from peripheral blood lymphocytes. Luciferase reporter analysis and gel shift analysis were performed.
Results The proximal promoter element is the overlapping binding site for the constitutive binding factor, Oct-1, and the inducible one, AP-1. The transcriptional induction was ascribed to the inducible binding, while the constitutive binding was rather inhibitory. A mutant element which lost the constitutive binding but retained the inducible binding exerted 3 times more transcriptional activity compared to the wild type element. In contrast, another mutant element which lost the inducible binding and retained the constitutive binding exhibited no transcriptional induction. Gel shift analysis clarified that the inducible binding was more prominent and the constitutive binding was less in IL-5-producing T cells derived from asthma patients compared to IL-5-nonproducing cells derived from control subjects.
Conclusions The ratio of the inducible/constitutive binding to the proximal promoter element may determine the capacity of human Th cells to transcribe IL-5 gene, and its regulation may control eosinophilic inflammation.
(Internal Medicine 39: 618-625, 2000)

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A 29-year-old man was admitted to our hospital with a history of recurrent right upper quadrant abdominal pain and vomiting. These symptoms appeared intermittently for 7 years. Various examinations revealed a diagnosis of midgut malrotation. Laparotomy was performed and revealed reverse rotation of the duodenum with paraduodenal hernia and a normal rotating colon. This case suggests that recurrent abdominal complaints in an adult should arouse suspicion of midgut malrotation.
(Internal Medicine 39: 626-631, 2000)

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We report a case of fulminant myocarditis after steroid pulse therapy for acute hepatitis. Serological studies demonstrated a four-fold increase in the antibodies against human herpesvirus-6 (HHV-6) IgG, and a PCR showed the existence of HHV-6 virus DNA. HHV-6 virus DNA was also isolated from the liver and the heart. We believe that exacerbation of fulminant myocarditis was probably associated with the reactivation of HHV-6 due to the immunosuppressive state of the host.
(Internal Medicine 39: 632-636, 2000)

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The echocardiographic findings of hypertrophic cardiomyopathy (HCM) are very similar to those of cardiac amyloidosis. A 76-year-old Japanese man was admitted for treatment of early stage gastric cancer in July 1996. His electrocardiogram indicated left ventricular hypertrophy and echocardiography showed left ventricular hypertrophy with asymmetric septal hypertrophy. He was re-admitted complaining of dyspnea on effort and pretibial edema in October 1998. The amplitude of QRS complex on electrocardiogram was decreased. Echocardiogram showed left ventricular wall thickening with granular sparkling. He was diagnosed as HCM with cardiac amyloidosis.
(Internal Medicine 39: 637-640, 2000)

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We present here a rare case of hypopituitarism accompanied by growth hormone (GH) deficiency and hypogonadotropic hypogonadism, in which the patient attained normal height but was of eunuchoid appearance. A 23-year-old man who had not reached puberty was referred to Saitama Medical School for hormonal evaluation. Basal hormonal data and hormone-stimulating tests revealed impaired secretion of GH, gonadotropins and adrenocorticotropic hormone (ACTH). Serum levels of testosterone, estrone, estradiol and estriol were all below the detectable ranges. The patient's plasma ACTH responded to corticotropin releasing hormone, but not to insulin-induced hypoglycemia. Serum GH showed a minimal response to GH-releasing hormone, but was unresponsive to insulin-induced hypoglycemia. Serum luteinizing hormone and follicle stimulating hormone did not respond to luteinizing hormone-releasing hormone. The results were compatible with a diagnosis of hypothalamic hypopituitarism. Magnetic resonance images of the brain showed a small anterior pituitary, an ectopic posterior lobe and transection of the pituitary stalk. Although the patient showed signs of hypopituitarism, he finally attained normal height, possibly because of failed epiphyseal maturation. His bone mineral density was markedly reduced to 0.647 g/cm2 in the lumbar spine; this level was 61.7% of the average level of healthy young males. Our findings were compatible with a recently advocated view that estrogen is important in promoting epiphyseal fusion and in determining bone density in males as well as females.
(Internal Medicine 39: 641-645, 2000)

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We describe a 65-year-old man who presented with pulmonary hemorrhage and progressive renal insufficiency three months after resection surgery for an abdominal aortic aneurysm. Intensive treatment with corticosteroids and hemodialysis were not effective, and the patient died. Postmortem examination of the kidneys revealed widespread cholesterol clefts within the renal arterioles and a number of lamellar inclusion bodies were observed by electron microscopy. The diagnosis of Fabry's disease was made by the absence of plasma α-galactosidase A activity. This was a very rare case of subclinical Fabry's disease coexistent with cholesterol crystal embolization, mimicking pulmonary-renal syndrome.
(Internal Medicine 39: 646-649, 2000)

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A 41-year-old woman was admitted to the hospital with severe uremia, hemolytic anemia, and thrombocytopenic purpura. Emergency heniodialysis with plasmapheresis was started in view of consideration of hemolytic uremic syndrome (HUS), which resulted in improvement of renal function and platelet count. Positive antineutrophil cytoplasmic autoantibody specific for myeloperoxidase (MPO-ANCA) suggested crescentic glomerulonephritis, which was pathologically evidenced by renal biopsy. The diagnosis of MPO-ANCA associated crescentic glomerulonephritis with autoimmune hemolytic anemia (AIHA) and thrombocytopenic purpura were comfirmed. Three courses of steroid pulse therapy with heparin were successfully performed, followed by oral prednisolone and warfarin. Such a case has not been previously reported to our knowledge.
(Internal Medicine 39: 650-654, 2000)

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The occurrence of synchronous epithelial cancer of the lung and leiomyosarcoma of the small intestine is rare. We report here the case of a 62-year-old man with adenocarcinoma of the lung in clinical stage IIIB (T4NOMO). After two courses of chemotherapy (cisplatin, 80 mg/m² and mitomycin C, 8 mg/m²) the patient developed symptoms of a small bowel obstruction. Palliative surgical resection was performed and a leiomyosarcoma of the small intestine was found and defined by an immunohistological study. The resection ameliorated the patient's symptoms. The patient died of disseminated adenocarcinoma 26 months following chemotherapy.
(Internal Medicine 39: 655-658, 2000)

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We compared the significance of several serum markers to evaluate the activity of Pneumocystis carinii pneumonia (PCP) in an immunocompromized patient. We successively measured KL-6, an ammo-terminal propeptide of Type III procollagen (PIIINP), and the cytokeratin 19 fragment (CK19) in the sera of a patient with PCP. Interestingly, PIIINP, KL-6, and CK19 levels in the sera did not increase at the time of onset of PCP during a neutropenic phase. Instead, they markedly increased after the recovery of WBC counts. This case suggests that values of PIIINP, KL-6, and CK19 used for monitoring the activity of PCP might be underestimated in neutropenic patients.
(Internal Medicine 39: 659-662, 2000)

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We describe a 24-year-old pregnant woman complicated by cyclic neutropenia (CN), who was successfully treated with granulocyte-colony stimulating factor (G-CSF). Her white blood cell (WBC) and neutrophil count fluctuated from 2, 600 to 4, 600/μl, and 26 to 2, 530/μl, respectively. The peak neutrophil count gradually decreased as pregnancy advanced, resulting in the disappearance of its cyclicity. At 39 weeks of pregnancy when the neutrophil count became 84/μl, the patient was started on G-CSF and her neutrophil count increased to 1, 550/μl on the fourth day after delivery. She delivered a healthy baby without any complications at 39 weeks of pregnancy.
(Internal Medicine 39: 663-666, 2000)

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At the age of 20 years, a Japanese man with recurrent oral aphthae, genital ulcers, folliculitis, erythema nodosum, episodic arthritis and epididymitis was diagnosed as having Behçet's disease (BD) in 1966. He has had active ocular manifestations of BD since 1990. These symptoms recurred and never abated for a long period of time. A right renal cell carcinoma developed and he underwent right nephrectomy in April 1996. Treatment with interferon-alpha was started from June 1996 as supplemental chemotherapy. No active phase developed during administration of IFN-alpha. We suggest that IFN-alpha may play a role as an inununomodulatory agent in BD.
(Internal Medicine 39: 667-669, 2000)

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We report two cases of severe measles pneumonia. Patient 1, a 17-year-old boy who contracted measles in the acute phase of infectious mononucleosis caused by Epstein-Barr virus (EBV), transmitted the disease to patient 2, his father. Both patients presented severe pneumonia with bilateral diffuse micronodular shadows. Diagnoses were established in both patients by antibody titers for measles and reverse transcriptase-polymerase chain reaction (RT-PCR) of blood and throat swab. Multinucleated giant cells with intranuclear inclusion bodies were revealed in the transbronchial lung biopsy (TBLB) specimen of patient 2. Both patients recovered with pulse steroid therapy.
(Internal Medicine 39: 670-674, 2000)

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