Adult reversible cardiomyopathy with pituitary adrenal insufficiency caused by empty sella--a case report.

The authors report a first case of reversible cardiomyopathy in an adult with adrenal insufficiency. A 62-year-old man was administered to hospital because of congestive heart failure. The electrocardiogram showed prolonged QT interval and negative T wave on the right precordial leads and frequent multifocal ventricular premature contractions. On the echocardiogram, the left ventricle was revealed to be diffusely hypokinetic with remarkable enlargement. 201Thallium single photon emission computed tomography (SPECT) revealed multiple flow defects in the left ventricular wall. He also had pituitary adrenal insufficiency secondary to an empty sella, which was diagnosed by hormonal studies and magnetic resonance images of the brain. After the replacement therapy with hydrocortisone, heart failure was sufficiently resolved. Abnormal flow defects in the myocardium on SPECT were also improved. To the authors' knowledge, adrenal insufficiency with cardiomyopathy has not been previously described in adult patients.

Neurally mediated syncope induced by lung cancer--a case report.

The authors present hemodynamic and autonomic features of recurrent and episodic neurally mediated syncope in a man with lung cancer involving afferent vagus. He revealed extreme hypotension with bradycardia occurring during sitting or standing. A head-up tilt test also induced syncope. However, syncope attacks no longer occurred 2 weeks after admission. Alternatively, the paralyses of the left recurrent laryngeal nerve and the left phrenic nerve developed. It is suggested that the lung cancer involved upper rootlets of the left vagus and caused transient hypersensitivity of baroreceptor function that resulted in neurally mediated syncope.

Internal jugular vein thrombosis, Lemierre's syndrome; oropharyngeal infection with antibiotic and anticoagulation therapy--a case report.

The authors present a case of Lemierre's syndrome that is an uncommon septic thrombophlebitis of the internal jugular vein. A 31-year-old man developed pharyngeal pain one month before hospital admission when he suffered from a severe headache and painful swelling of the left side of his neck. He was diagnosed with tonsillitis. Contrast-enhanced computed tomography and magnetic resonance imaging of the neck revealed the presence of an occlusive thrombosis of the left internal jugular vein and an inflamed mesopharynx. His symptoms and the jugular vein thrombus showed remarkable improvement after administration of antibiotic and anticoagulation therapy. No pulmonary embolism or other metastatic infection were observed. It was suggested that accurate diagnosis during early treatment is essential to obtain a successful prognosis for Lemierre's syndrome.

Primary ileal plasmacytoma arising in mixed low- and high-grade B-cell lymphoma of mucosa-associated lymphoid tissue type.

We report an extremely rare case of primary ileal plasmacytoma accompanied by mixed low- and high-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type. The radiographic and macroscopic features of the tumor were characterized by two constricting lesions in the ileum. Histologic examination of the resected specimen showed that one constrictive lesion was plasmacytoma and the other MALT lymphoma was low and high grade. The plasmacytoma seemed to have differentiated from the MALT lymphoma.

Adult T-cell leukemia/lymphoma in which the pathohistological diagnosis was identical to that of Ki-1 positive anaplastic large cell lymphoma.

A 65-year-old man developed severe lumbago and a loss of appetite two months before presentation. A computerized tomograph at admission revealed soft tissue masses destroying the Th12, L4 and L5 vertebral bones. We diagnosed the lesions to be metastatic bone tumors, but the primary focus could not be determined. Just after the irradiation treatment, abnormal lymphocytes were detected in the peripheral blood cells. Under the suspicion of adult T-cell leukemia/ lymphoma (ATL), we thus performed a lymph node biopsy. The specimens were histologically composed of Ki-1 positive anaplastic large cell lymphoma (ALCL). The lymphoma cells demonstrated a biclonal integration of HTLV-1 proviral DNA. After 6 cycles of chemotherapy, the patient has demonstrated a partial and favorable remission from ATL.

Minimal-change nephrotic syndrome and acute renal failure in a patient with aged onset insulin-dependent diabetes mellitus and autoimmune thyroiditis.

A 61-year-old woman with a 2-year history of insulin-dependent diabetes mellitus (IDDM) developed nephrotic syndrome. Renal biopsy showed minimal-change nephrotic syndrome (MCNS), and no evidence of diabetic glomerulosclerosis. Although steroid therapy was initiated, plasma urea and creatinine rose and hemodialysis was required. After 4 weeks, she responded to steroids and her renal function returned to normal. MCNS, which is not associated with diabetic glomerulosclerosis, has rarely been seen in IDDM patients with nephrotic syndrome. Her human leukocyte antigen typing was A24, BW52, BW61, DR2 and DR9. This typing has been reported to be associated with both IDDM and renal disease.

Ileal varices associated with recurrent bleeding in a patient with liver cirrhosis.

We report a rare case of massive and recurrent bleeding from ileal varices in a patient with hepatitis C virus-positive liver cirrhosis. A 66-year old woman, who had undergone laparotomy and blood transfusion 36 years before (because of an extrauterine pregnancy) and endoscopic sclerotherapy for esophageal varices 1 year previously, was admitted to our hospital with loss of bright red blood per rectum. The bleeding was massive and recurrent, and frequent blood transfusions were required. Endoscopic studies failed to find the bleeding site. In the venous phase of selective superior mesenteric angiography, mesenteric varices in the lower part of the abdominal cavity were observed. Laparotomy was performed to control the repeated bleeding which had lasted for more than 1 month. Varices communicating with the right ovarian vein were found on the ileal wall and segmental resection of the ileum was performed. Histological examination demonstrated a massive varicose vein and several dilated veins in the submucosa. The patient's postoperative course was favorable, with no hemorrhagic events during a follow-up of more than 6 months after surgery. Ileal varices should be considered in the diagnosis of a patient who presents with lower gastrointestinal bleeding and portal hypertension.

Circumferential spontaneous coronary artery dissection in an elderly man: a case report.

An 82-year-old man developed acute inferior myocardial infarction. Emergent coronary angiography demonstrated thrombotic occlusion in the right coronary artery. Intracoronary thrombolytic therapy was performed with successful recanalization. However, circumferential dissection with luminal stenosis was revealed at the point of thrombus formation. Repeat angiography 6 months later showed resolution of the dissection. This is the first description of circumferential spontaneous coronary artery dissection. In addition, the present patient is older than any previous patients.

Ambulatory blood pressure monitoring in diagnosing a pheochromocytoma of the urinary bladder. A case report.

A patient is presented in whom the preoperative diagnosis of an intravesical pheochromocytoma was facilitated by noninvasive ambulatory blood pressure monitoring. A fifty-two-year-old Japanese man suffered headache and palpitation after micturition. Cystoscopy revealed an intravesical tumor. To investigate whether his symptoms were associated with an elevation of blood pressure, the authors monitored his ambulatory blood pressure automatically for twenty-four hours. The patient was also instructed to activate the recording manually upon the onset of symptoms. As a result, elevations of blood pressure were apparent following micturition. The twenty-four-hour urinary excretion of norepinephrine was elevated on the day of the blood pressure monitoring. Pheochromocytoma was suspected and was confirmed by histopathologic studies following the operation. Thus, ambulatory blood pressure monitoring may be useful in detecting the transient hypertension induced by micturition, which can provide preoperative evidence of pheochromocytoma of the urinary bladder.

[Home infusion therapy for cancer patients at a near-terminal stage--analysis of cases who died at home].

To improve the quality of life in cancer patients at a near-terminal stage, we assisted patients to stay home using a system for home infusion therapy (HIT) established at Osaka Prefectural Habikino Hospital in 1994. From April in 1995 to April in 1996, 25 patients were treated at home using the HIT system for longer than 7 days. Thirteen out of 25 patients still stayed at home even at a terminal stage and died in their residences. There was no difference either in the mean age or in the HIT duration between 13 patients who died at home and the remaining 12 patients who were readmitted to their hospitals before death. However, 47% of the patients who died at home had been told their diagnoses by doctors and realized their own prognoses, while only 17% of the patients who died in the hospital knew their diagnoses and prognoses. Seventy-seven percent of the patients who died at home were given home oxygen therapy (HOT), against 33% of the patients who died in the hospital. Furthermore, the patients who died at home were given more intensive care than the patients who died in the hospital, because the former had a larger family than the latter. In conclusion, HIT at a near-terminal stage and death at home may be more readily accepted by patients who know their diagnoses and prognoses and have a large family.

Hypercholesterolemia and the progression of the renal dysfunction in chronic renal failure patients.

The effect of hyperlipidemia on the progression of chronic renal failure was investigated in 104 chronic renal failure patients, aged 39.3 +/- 2.9 years. The follow up period was 4.1 +/- 2.9 years. The serum creatinine level was 2.1 +/- 1.1 (mean +/- SD) mg/dl at the beginning of study and increased to 8.7 +/- 4.4 mg/dl at the end of the study. The reciprocal serum creatinine concentration (1/Cr) was plotted against the observation time, and the slope was calculated. The absolute value of the slope was used as the progression rate of renal impairment. The progression rate was positively related to total cholesterol level or urinary protein score, while it was negatively related to total protein level. Without the influence of urinary protein score, the progression rate correlated with total cholesterol level. The result suggests that hypercholesterolemia may be an independent aggravating factor in the progression of renal dysfunction in chronic renal failure patients.

Evidence for an immunological pathogenesis of thrombocytopenia in chronic liver disease.

OBJECTIVES: To elucidate the role of platelet-associated IgG (PA-IgG) in the mechanism of thrombocytopenia associated with chronic liver disease. METHODS: Platelet count in blood, PA-IgG, and scintigraphic spleen/liver ratio as a marker of splenomegaly was examined in 214 individuals, including 16 controls showing nonspecific reactive change in liver biopsy and 198 patients with chronic liver disease. RESULTS: The mean blood platelet count decreased significantly according to severity of liver disease, from control to liver cirrhosis. PA-IgG levels increased significantly in relation to severity of liver disease, as did spleen/liver ratio. In chronic hepatitis or liver cirrhosis, an inverse correlation was found between platelet counts and PA-IgG levels. An inverse correlation was also observed between platelet count and spleen/liver ratio in liver cirrhosis. The splenic embolization resulted in a significant rise in platelet count and a significant fall in PA-IgG in the 14 cirrhotic patients. CONCLUSIONS: These results may give support to evidence for an immunological mechanism mediated by PA-IgG for the thrombocytopenia occurring in chronic liver disease. In the case of liver cirrhosis, this mechanism would act in addition to platelet pooling in the spleen on thrombocytopenia. PA-IgG may also have an important role in thrombocytopenia associated with chronic hepatitis, in which splenic platelet pooling is less marked.

Idiopathic sudden sensorineural hearing loss in patients undergoing long-term haemodialysis.

Idiopathic sudden sensorineural hearing loss (ISHL) has been recently recognized and is increasing in frequency in patients undergoing long-term haemodialysis. Although this is one of the annoying complications impairing quality of life in haemodialysis patients, there has been little clinical evidence about ISHL in haemodialysis patients until now. We have examined retrospectively the clinical features of 6 ISHL patients, 3 males and 3 females, with a mean age of 54 years, who underwent regular haemodialysis with a mean haemodialysis duration of 90 months from 1985 to 1989. Although a specific cause for ISHL could not be identified in this study, more precise clinical features of ISHL in patients undergoing haemodialysis have been elucidated. ISHL seemed to develop more frequently in haemodialysis patients and occurred in patients undergoing haemodialysis for a relatively long time, being independent of the high-frequency-deficit type hearing disturbances usually observed in the early course of haemodialysis therapy. Above all, diabetic haemodialysis patients were more susceptible to ISHL, occurring in 4 cases out of 6. Its prognosis was not necessarily bad, showing a recovery rate of 83%, but more effective therapy should be explored for improving the quality of life in haemodialysis patients.

[Nephrotic syndrome due to membranous glomerulopathy in hypocomplementemic urticarial vasculitis syndrome;--a case report].

We report a case of hypocomplementemic urticarial vasculitis syndrome (HUVS) with membranous glomerulopathy in a 62-year-old man who had a 2-month history of secondary iritis. He was transferred to our hospital because of uncontrollable edema and respiratory dysfunction. Physical examination revealed anasarca, pulmonary edema, hypertension and urticaria-like eruption on his arms. Urinalysis, blood chemistry and serological studies showed massive proteinuria (10.5g/day) with numerous granular casts, hypoalbuminemia (1.5g/dl), renal dysfunction (creatinine; 1.6mg/dl, BUN; 86mg/dl), hypercholesterolemia (total cholesterol; 455mg/dl), positive results for antinuclear factor, microsome test, thyroid test, lupus anticoaglant, antithyroglobulin test and rheumatoid factor, but LE cell or double-strand anti DNA antibody was negative. Serum complement levels were persistently low as CH50 of 13 U/ml and Clq of 6.0 micrograms/dl. The patient serum precipitated with normal human Clq by immunodiffusion analysis, indicating the presence of anti-Clq antibody. Renal biopsy revealed membranous glomerulopathy with prominent fine granular deposition of Clq along the glomerular basement membrane by immunofluorescent study and subepithelial dense deposit by electron microscopy. Corticosteroid treatment was ineffective for hypocomplementemia and nephrotic syndrome. Acute subendocardial infarction occurred on the 25th hospital day and he died of acute respiratory distress syndrome on the 45th hospital day. Autopsy revealed leucocytoclastic vasculitis in the alveolar wall. HUVS was confirmed by clinical symptoms, such as iritis and urticaria-like eruption, serum anti-Clq antibody, the absence of any specific autoantibody for systemic lupus erythematosus (SLE) and leucocytoclastic vasculitis in the alveolar wall.(ABSTRACT TRUNCATED AT 250 WORDS)

Clinicopathological study of poststreptococcal glomerulonephritis in the elderly.

In order to characterize the clinical features in elderly patients with poststreptococcal glomerulonephritis (PSGN), 31 patients, who were both histologically and immunologically proven to be PSGN, were divided up into 3 groups according to age; elderly patients being 55 years or older (n = 7), middle-aged patients being 40 to 54 years old (n = 7) and younger patients being 20 to 39 years old (n = 17). Renal functional impairment as indicated by serum creatinine levels of over 2.0 mg/dl, developed in 4 of the elderly patients and later completely improved at the end of the follow-up period (178.9 +/- 150.7 days). On the other hand, none of the middle-aged and younger patients revealed any renal function impairment. Hypertension was observed more frequently in elderly patients than in younger patients, and was 86% and 6% at the time of admission, respectively. In addition, 43% of elderly patients remained hypertensive at the time of discharge. There was no difference in total protein, ASO, CH50, the degree of proteinuria or proliferative and exudative features in renal histology among the three groups. None of the elderly patients with PSGN died or developed persistent renal failure. In conclusion, elderly patients with PSGN had a high incidence of renal functional impairment and hypertension compared to the younger patients on admission, however, their short-term prognosis seems to be favorable.

Alpha tocopherol improves focal glomerulosclerosis in rats with adriamycin-induced progressive renal failure.

The effect of d-alpha-tocopherol on the progression of renal dysfunction was investigated in rats injected with adriamycin (ADR), a model of progressive glomerulosclerosis associated with the nephrotic syndrome. Treatment with d-alpha-tocopherol was started 1 day before or 1 day after ADR injections (BE-TOC or AF-TOC rats). When compared to rats without d-alpha-tocopherol treatment (ADR-CON rats), the serum total cholesterol and triglyceride levels were significantly lower in the BE-TOC and AF-TOC groups. In week 16, the LDL cholesterol level and the atherogenic index were both significantly lower in BE-TOC and AF-TOC rats than in ADR-CON rats. The urinary protein, serum creatinine, blood urea nitrogen, malondialdehyde, and systolic blood pressure levels as well as the glomerulosclerosis score were high in ADR-CON rats, and reduced in BE-TOC or AF-TOC rats. There were no significant differences in body weight and serum albumin between the three groups in week 16. It is concluded that d-alpha-tocopherol can improve hyperlipidemia and ameliorate glomerulosclerosis in rats with ADR-induced progressive renal failure. Thus, d-alpha-tocopherol may have the potential for clinical application to treat focal glomerulosclerosis.

Inhibitory effects of antihypertensive drugs on mesangial cell proliferation after anti-thymocyte serum (ATS)-induced mesangiolysis in spontaneously hypertensive rats.

The effects of antihypertensive drugs on mesangial cell proliferation were studied in spontaneously hypertensive rats (SHR) with anti-thymocyte serum (ATS)-induced glomerulo-nephritis. Rats were treated with either enalapril (Group 1), nifedipine (Group 2), or reserpine + hydrochlorothiazide + hydralazine (Group 3), or were untreated (Group 4). The animals were sacrificed 2, 4 and 7 days after ATS injection and the glomerular cell number and degree of mesangial area expansion were examined. A marked, similar decrease in glomerular nuclear cell number (NC) due to severe mesangiolysis was observed in all of the groups on day 2. Thereafter, an increase in NC reflecting mesangial cell proliferation after mesangiolysis occurred in Group 4 on days 4 and 7. In Group 1 and 2, the NC was significantly smaller than that in Group 4 on days 4 and 7, indicating suppression of mesangial cell proliferation. In Group 3, however, the number of NCs did not differ from that in Group 4 on days 4 and 7, indicating a lack of such suppression by conventional antihypertensive drugs. The degree of mesangial area expansion (MS) showed the same pattern as mesangial cell proliferation. That is, the rapid increases in MS seen in Group 4 on days 4 and 7 were apparently suppressed in Groups 1 and 2, but not in Group 3. Our in vivo observations that both an angiotensin converting enzyme (ACE) inhibitor and a calcium channel blocker suppress mesangial cell proliferation and mesangial area expansion suggest that these agents have practical implications in the treatment of mesangial proliferative glomerular diseases through the suppression of excess mesangial cell proliferation.