Urinary pancreatic stone protein (PSP) levels were measured in 68 diabetic patients and 170 healthy controls to investigate the relationship between the progression of diabetic nephropathy and PSP excretion. Urinary albumin, N-acetyl-β-glucosaminidase (NAG), (α₁-microglobulin, creatinine clearance, and the blood PSP level were also determined in the diabetic patients. The urinary glucose level and glycemic control did not influence the urinary PSP level. In patients with normoalbuminuria (urinary albumin <20 mg/gCr, n=31), microalbuminuria (20-200 mg/Cr, n=19), and macroalbuminuria (>200 mg/gCr, n=18), the mean urinary PSP level was 347, 507, and 860 μg/gCr, respectively. These levels were significantly higher than the level in normal volunteers (168 μg/gCr, p<0.01). A significant positive correlation was observed between the urinary PSP level and the NAG or α₁-microglobulin levels (p<0.01). There was a stronger correlation with α₁-microglobulin. Blood PSP levels were also elevated in patients who had renal impairment with a decreased creatinine clearance. In conclusion, urinary PSP excretion was increased from the initial stage of diabetic nephropathy and this increase became more marked as nephropathy progressed. Increased PSP excretion may reflect renal tubular dysfunction.
(Internal Medicine 37: 500-503, 1998)

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There is a considerable overlap between diffuse panbronchiolitis (DPB) and bronchiolar disease associated with rheumatoid arthritis. The present study assessed how these conditions could be differentiated. The subjects included 25 DPB patients and 15 RA patients with bronchiolar disease (RA-BD). Patients with either condition had chronic cough, purulent sputum, dyspnea and coarse crackles. Most patients with either DPB or RA-BD had a history of sinusitis as well as elevated cold hemagglutin titers and decreased levels in partial pressure of oxygen (PaO₂), forced expiratory volume in one second (FEV_1.0) and V 25/Ht. On histological examination, both conditions also shared various histological patterns although panbronchiolitis lesions were more common in DPB than RA-BD (68% vs 20%) and bronchiolar obliteration appeared to occur at more proximal sites in RA-BD than DPB. However, there were important differences: long-term treatment with erythromycin had less effect in RA-BD than DPB, and the frequency of HLA B54 tended to be higher in DPB than RA-BD (50.0% vs 22.2%), suggesting that they are distinct conditions.
(Internal Medicine 37: 504-508, 1998)

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Blood-brain barrier (BBB) permeability in the demented elderly was investigated by use of the ratio of cerebrospinal fluid (CSF) albumin (Alb) to serum Alb (Q-Alb). Subjects with Alzheimer type dementia (AD), vascular dementia (VD), and controls without dementia (C) were investigated. Patients with AD were divided into mild AD (mAD) and severe AD (sAD) by the use of Hasegawa's dementia scale. The Q-Alb and the ratio of CSF α₁-antichymotrypsin (ACT) to serum ACT (Q-ACT) were evaluated. Correlations between Q-Alb and Q-ACT were compared among the groups (mAD, sAD, VD, C). Correlations between Q-Alb and major monoaminergic neurochemicals were also analyzed. It was suggested that BBB permeability was preserved in C group, while it was impaired in the patients with VD. In AD group it appeared to be rather well preserved in mAD, while it seemed to be disturbed in a graded manner according to the progression of dementia.
(Internal Medicine 37: 509-513, 1998)

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It is controversial whether long-term anticholinergic therapy (AC-T) can cause short-term memory disturbance, long-term memory disturbance or intelligence impairment. Ninety patients with Parkinson's disease were examined prospectively to clarify the existence of memory and intelligence impairment induced by long-term AC-T. Neuropsychological tests detected eight patients with at least one of four types of deficits; intelligence impairment in four patients, verbal delayed recall impairment in eight, verbal short-term memory disturbance in five, and verbal long-term memory disturbance in six. Two types of verbal memory disturbance (short-term one and long-term one) appeared with double dissociation, suggesting that there may be an independence between the two types of verbal memory system. Older patients were more prone to suffer from these reversible deficits. Moreover, all of the patients who resumed AC-T, showed recurrence of the deficits acutely. These observations should indicate the tight relation between long-term AC-T and neuropsychological deficits.
(Internal Medicine 37: 514-518, 1998)

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A 57-year-old woman was admitted with symmetrical proximal muscle weakness, liver dysfunction, abnormal muscle enzymes, and she was an antibody to hepatitis B e (anti-HBe) positive hepatitis B virus (HBV) carrier. Biopsy of her left quadriceps femoris showed myositis, so prednisolone was started at 40 mg/day. However, her hepatic function deteriorated and liver biopsy after 4 months showed acute hepatitis with partial submassive necrosis. Treatment with interferonalpha and cyclosporin A progressively reduced the transaminase and HBV-DNA levels. Early treatment with interferon-alpha plus cyclosporin A can control exacerbation of hepatitis B.
(Internal Medicine 37: 519-522, 1998)

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A 21-year-old male was clinically diagnosed with familial hypercholesterolemia (FH) by the manifestations of hypercholesterolemia, tendon xanthoma and family history of premature coronary heart disease. Low density lipoprotein receptor gene was analyzed in attempt to determine a possible point mutation. The normal sequence was partially preserved, and the patient was genetically diagnosed as a heterozygote of FH. In addition, screening for two cholesteryl ester transfer protein (CETP) gene mutations common to Japanese revealed the patient to be a heterozygote of CETP deficiency. A complication of two influential mutations for atherosclerotic ailments was genetically ascertained.
(Internal Medicine 37: 523-527, 1998)

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A 68-year-old woman was referred for evaluation of an incidentally discovered left adrenal mass. Her chief complaint was body weight loss. She showed no symptoms or signs suggestive of Cushing's syndrome. The circadian rhythm of blood pressure was also normal. Hormonal assessment revealed an abnormal diurnal variation in serum cortisol levels, suppressed baseline plasma adrenocorticotrophic hormone, and nonsuppression of serum and urinary cortisol with the dexamethasone suppression test. Adrenal scintigraphy with¹³¹I-6-beta-iodomethyl-norcholesterol showed uptake on the left adrenal and inhibition of the contralateral adrenal gland. She was diagnosed as pre-clinical Cushing's syndrome. Due to the lack of clinical symptoms and the risks of surgery, we emphasize the importance of careful assessment of the cortisol metabolism and scintigraphic scanning under dexamethasone suppression to avoid post-surgical Addisonian crisis.
(Internal Medicine 37: 528-533, 1998)

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A 52-year-old woman with a seven-year history of rheumatoid arthritis (RA) was transferred to our department with chronic renal failure to undergo hemodialysis. She had been treated with prednisolone for a long time, and had renal amyloidosis secondary to RA. During her hospitalization, a left pleural effusion developed. Pleural fluid cultured positive for Cryptococcus neoformans (CN), and the CN antigen was detected in both pleural fluid and serum. Chest computerized tomography revealed an infiltrate shadow in the left lower lung field suggestive of CN infection. This was successfully treated with anti-fungal agents. Pleural effusion is an unusual manifestation of pulmonary cryptococcosis. We should consider a diagnosis of CN infection when pleural effusion is observed in compromised patients such as those receiving a long-term corticosteroid treatment.
(Internal Medicine 37: 534-537, 1998)

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We report a rare case of a woman with inflammatory carcinoma, an unusual type of cutaneous metastasis, arising from signet-ring cell carcinoma of the stomach, who developed chylothorax as the skin lesion progressed over the chest. No thoracoabdominal lymphadenopathy which can cause obstruction of the thoracic duct was shown by computed tomography. Although a very rare condition, inflammatory carcinoma could be a cause of non-traumatic chylothorax.
(Internal Medicine 37: 538-541, 1998)

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A 26-year-old man, diagnosed with acute myelogenous leukemia had multiple inflammatory pseudotumors (IPT) in the liver. The patient presented complete remission after remission induction therapy, and then showed right upper quadrant discomfort and intermittent fever. An ultrasonography disclosed multiple hypoechoic nodules in the liver. A biopsy of the nodules showed focal liver cell necrosis with scant inflammatory cells, compatible with IPT. After several courses of chemotherapy, the nodules in the liver increased. The second liver biopsy of the nodule showed fibrosis. Multiple IPTs in the liver should be distinguished from abscess and metastatic nodules.
(Internal Medicine 37: 542-545, 1998)

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We report a rare case of pseudotumor cerebri associated with all-trans retinoic acid (ATRA) treatment of acute promyelocytic leukemia (APL). An 18-year-old male was admitted to our hospital complaining of palpitations and shortness of breath; he was found to have APL. The administration of ATRA and chemotherapy was started. After 23 days, he complained of nausea, headache and double vision. Computed tomography and magnetic resonance imaging of the head showed no intracranial abnormalities. Bilateral papilledema, a symptom of increased intracranial pressure, was noted. A diagnosis of pseudotumor cerebri was made. Symptoms were improved by administration of glycerin and the discontinuation of ATRA. After 29 days, a complete remission was achieved.
(Internal Medicine 37: 546-549, 1998)

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A patient with systemic lupus erythematosus (SLE) developed reactive hemophagocytosis. This case did not show any underlying diseases such as infection or malignancy other than SLE itself. The mechanisms inducing hemophagocytosis in SLE seem to be heterogeneous and remain to be elucidated. Although an immune complex-mediated mechanism in cases with acute lupus hemophagocytic syndrome has been proposed, we suggest the possible involvement of IL-1β as the pathogenesis of our case.
(Internal Medicine 37: 550-553, 1998)

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A 22-year-old female with Raynaud's phenomenon, swollen hands and a high titer of anti-RNP antibodies developed fever and myositis. Prednisolone (40 mg/day) was considered effective for myositis since circulating myogenic enzymes rapidly decreased. However, she suddenly developed respiratory distress with bilateral pulmonary infiltrates and bloody sputum. Under the diagnosis of alveolar hemorrhage (AH), intravenous methylprednisolone pulse therapy was given, but she died of respiratory failure. Autopsy findings demonstrated massive AH with hematoma formation, and myositis in the iliopsoas muscle. Depositions of immune complex and vasculitic lesions were not recognized in her lungs.
(Internal Medicine 37: 554-560, 1998)

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A 61-year-old man developed mononeuritis multiplex accompanied by eosinophilia in 1993. Approximately 3 years later, acute renal dysfunction, a subendocardial tumor, and a high peripheral anti-neutrophil cytoplasmic antibody titer were also detected. Renal biopsy revealed glomerular crescents and interstitial infiltration of eosinophils, so allergic granulomatosis and angiitis was diagnosed. These clinical abnormalities regressed with steroid therapy. He had no history of asthma. This was therefore considered to be an atypical form of Churg-Strauss syndrome with rapidly progressive glomerulonephritis.
(Internal Medicine 37: 561-563, 1998)

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