To examine the relationship of nonspecific bronchial responsiveness to baseline pulmonary function, and to compare the reactivity in asthma and sinobronchitis, we measured the provocative concentration of methacholine, causing a 20% fall in forced expiratory volume in one second (PC₂₀-FEV₁) in 82 asthmatic patients, 54 patients with sinobronchial syndrome and 57 normal subjects. Log PC₂₀-FEV₁ was directly correlated to baseline pulmonary function. The strongest correlation was seen between log PC₂₀-FEV₁ and baseline FEV₁ as a %of the predicted FEV₁ (%FEV₁), both in asthma (log PC₂₀-FEV₁ = 0.020x %FEV₁ - 1.988, r = 0.54, p< 0.01) and in sinobronchial syndrome (log PC₂₀-FEV₁ = 0.025 x %FEV₁ - 1.233, r = 0.64, p< 0.01). From the regression equations, the mean value of PC₂₀-FEV₁ at 100% FEV₁ was calculated as 1.02 and 18.5 mg/ml in asthmatic and sinobronchitic patients, respectively, and the value was 35.5 mg/ml in normal subjects. The PC₂₀-FEV₁ to %FEV₁ relationship may be helpful to assess individually measured PC₂₀-FEV₁ values in patients with airway obstruction.
(Internal Medicine 32: 1-5, 1993)

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Complete thyroxine-binding globulin deficiency (TBG-CD) was uncovered in a subject receiving a comprehensive health examination. The subject had an abnormally high T₃ resin uptake. A family study showed that the TBG abnormality had been inherited by X-chromosome linkage. Genetic analysis revealed single nucleotide deletion, common among Japanese with TBG-CD, from the allele specific amplification of the TBG genes of the family.
(Internal Medicine 32: 6-9, 1993)

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A 61-year-old man with liver cirrhosis showed a symmetrical increase in the signal intensity of the basal ganglia on T1-weighted magnetic resonance (MR) images, which was diminished after 3 months of treatment for hepatic encephalopathy. He recovered from encephalopathy with treatment, and liver dysfunction (hyperammonemia and abnormal blood coagulation) as well as the results of quantitative psychometric tests showed a marked improvement. The cause of these high signal intensity changes on T1-weighted images and the reason for their partial reversibility are not known, but hyperammonemia due to portal-systemic shunting might be closely related to these clinical observations.
(Internal Medicine 32: 10-14, 1993)

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A 64-year-old male complained of weight loss and slight fever. Bilateral hilar lymphadenopathy and hepato-splenomegaly were observed. Serum ACE level was high, and liver function was impaired. Laparoscopy demonstrated small white nodules on the liver surface. A definite diagnosis of sarcoidosis was made by histological study of the specimen by liver biopsy and noncaseating epithelioid cell granulomas composed of giant cells and epithelioid cells were revealed. Various pancreatic enzymes were increased on admission, suggesting pancreatic impairment due to sarcoidosis. Corticosteroid therapy improved liver and pancreatic functions and decreased splenomegaly. He has been followed up for 4 years and 5 months.
(Internal Medicine 32: 15-20, 1993)

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A 40-year-old hypothyroid female who had been treated with synthetic thyroxine was admitted to our hospital in October 1988 due to abnormal liver function tests. She had low serum free triiodothyronine (T₃; 2.3pg/ml) and high serum thyrotropin (TSH; 20.8 /μU/ml) concentrations. On the other hand, the serum free thyroxine (FT₄) level was inappropriately high, being 2.46 ng/dl. Immune precipitation of radiolabeled thyroid hormones with her serum disclosed the binding of ¹²⁵I-T₃ and ¹²⁵I-T₄ to the extent of 9.5% and 11.3%, respectively (normal ranges for ¹²⁵I-T₃ and ¹²⁵I-T₄ binding are less than 6.3% and 5.9%, respectively). ¹²⁵I-T₄ binding to the patient's serum gamma globulin was completely displaced with the addition of unlabeled T₄. Further examination disclosed that anti-T₄ antibodies in her serum belong to IgG κ class immunoglobulin.
(Internal Medicine 32: 21-25, 1993)

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A 14-year-old female with microscopic hematuria was admitted for a renal biopsy. She had a family history of renal disease without deafness. The findings of light microscopy and conventional immunofluorescence were normal. Electron microscopy showed a diffuse thinning of the glomerular basement membrane (GBM) with its mild splitting. Irregular thickening of GBM and glomerular small dense particles was not observed. Thin basement membrane syndrome was suspected from these findings. However, it was difficult to differentiate from Alport syndrome. Immunofluorescence analysis using the monoclonal antibody to the 28-kilodalton monomers of the noncollagenous domain of type IV collagen verified the diagnosis of heterozygous Alport syndrome.
(Internal Medicine 32: 26-30, 1993)

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Acute renal insufficiency developed in four idiopathic nephrotic patients with minimal change or mild proliferative glomerulonephritis. The reduction in glomerular filtration rate (CInulin) was not in proportion to the renal plasma flow (CPAH) as evidenced by a low filtration fraction. Diuretic therapy failed to reverse renal insufficiency, and renal biopsy showed no evidence of interstitial nephritis, acute tubular necrosis or interstitial edema. Corticosteroid therapy induced a recovery of renal function with a decrease in proteinuria. These observations suggest that acute renal insufficiency in the idiopathic nephrotic syndrome might be caused by impaired glomerular permeability.
(Internal Medicine 32: 31-35, 1993)

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We describe two patients who displayed unusual neurologic manifestations associated with reactivation of latent Epstein-Barr virus (EBV) infection. The patients included a 34-year-old woman who suffered from meningoencephalitis presenting with symptoms identical to those of transient global amnesia, and a 57-year-old man with jumbling phenomenon and gait ataxia. The protean neurologic manifestations of EBV infection underscore the need to suspect this infection in any acute neurologic disorders of possible infectious origin irrespective of the age of the patient.
(Internal Medicine 32: 36-38, 1993)

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A case of Cowden's disease is reported. A 38-year-old man was admitted to our hospital because of an abnormal left lung shadow on a chest radiograph. There were no symptoms, although the patient had multiple papules on the nose and pharynx mucosa. There were a few fibromas on the axillas and the inguinal regions. The patient had thyroid goiters, gastrointestinal poliposis, and a pulmonary hamartoma. The latter has not been reported previously in Cowden's disease.
(Internal Medicine 32: 39-41, 1993)

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We previously reported two siblings with decreased subcutaneous adipose tissue, muscular atrophy, joint contractures, recurrent skin eruptions, hyper-γ-globulinemia, and reduced natural killer cell activity. Some of their clinical features are similar to those of partial lipodystrophy, but they are distinct in that muscular atrophy, joint contractures and recurrent skin eruptions are not found in patients with partial lipodystrophy. Thirteen other Japanese patients with similar clinical manifestations have been reported. We propose that such cases should be considered a distinct clinical entity.
(Internal Medicine 32: 42-45, 1993)

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A 49-year-old female with a metastatic renal cell carcinoma to the thyroid is presented. The thyroglobulin (Tg) level in tumor aspirates of this patient was 37.3 μg/l, while that of 49 patients with primary thyroid nodules ranged from 2.0 × 10³ to 7.5 × 10⁸ μg/l. Ultrasonogram of the thyroid demonstrated a well-demarcated oval nodule with a cystic region, the solid part of which was hypoechoic and homogeneous. However, similar ultrasonic findings were observed in only 1.1% of 1, 054 patients with primary thyroid nodules. Determination of Tg in tumor aspirates and ultrasonography of the thyroid may contribute to the differentiation of metastatic thyroid nodules from primary thyroid nodules.
(Internal Medicine 32: 46-49, 1993)

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A 39-year-old woman presented clinical features of adult onset Still's disease. Seven years after the onset, she developed renal insufficiency and biopsy studies revealed amyloid deposits involving amyloid A protein, P component, λ chain and κ chain in the kidney and rectum. She died in 1992, primarily due to cardiac failure associated with amyloidosis, indicating that amyloidosis should be considered one of the fatal complications in adult onset Still's disease with a long history.
(Internal Medicine 32: 50-52, 1993)

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A 37-year-old man with ankylosing spondylitis (AS) and psoriasis who was successfully treated with methotrexate (MTX) is reported. In 1980, he had low back pain, limited motion in the lumbar spine, radiological findings of bilateral sacroiliitis, and HLA-B27 positivity. In January 1991, he developed psoriasis and he had difficulty in performing desk work in spite of treatment with antirheumatic drugs. In May 1991, MTX 7.5 mg/week per os was started. Joint symptoms, psoriasis, and acute phase reactants improved within 1 month after the treatment and this improvement continued for more than 6 months after the treatment. After discharge he was able to return to his job.
(Internal Medicine 32: 53-56, 1993)

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A 39-year-old man was admitted to our hospital complaining of general malaise, polyuria, disturbance of ocular movementand right cervical tumor. Blood examination revealed increased parathyroid hormone, hypercalcemia and hypophosphatemia, suggestive of hyperparathyroidism. Histology of the resected tumor revealed a benign parathyroid adenoma. Ectopic calcifications in the choroid and sclera were noted by computed tomography and further ophthalmological examination. Although ocular calcification in conjunctiva and cornea associated with hyperthyroidism is not unusual, sclerochoroidal calcification has not been reported previously in Japan. The possible cause of this unusual condition in this patient is discussed.
(Internal Medicine 32: 57-59, 1993)

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Carney's complex is composed of myxoma, spotty pigmentation and endocrine overactivity. A 27-year-old male was diagnosed to have Carney's complex on the basis of intense spotty pigmentations on his face, soles and palms, and bilateral adrenal nodular hyperplasia on computed tomography scanning (CT) and magnetic resonance imaging (MRI). Total bilateral adrenectomy was done; histological findings were compatible with primary pigmented adrenocortical disease (PPNAD). Recently, his sister and one of his brothers were suspected to have Carney's complex with PPNAD. We report the first familial case of Carney's complex with PPNAD and spotty pigmentations in Japan.
(Internal Medicine 32: 60-62, 1993)

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An unusual pheochromocytoma was incidentally discovered in a 48-year-old woman. The patient had a 3-year history of myasthenia gravis. At the time of examination in our hospital, the right adrenal tumor was incidentally discovered by ultrasonography of the abdomen. She had no history of headache, perspiration, palpitation or hypertension. Although blood catecholamine levels were within the normal limits, urinary secretion of catecholamine was elevated. Histologically, the tumor was diagnosed to be mixed ganglioneuroma/pheochromocytoma and histochemically confirmed to produce vasoactive intestinal polypeptide. Such a tumor is quite rare.
(Internal Medicine 32: 63-66, 1993)

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A 63-year-old man with symptoms of obstruction of the inferior vena cava was examined by computed tomography, ultrasound imaging and angiography. Examination revealed a tumor in the inferior vena cava, and transvenous biopsy revealed a rhabdomyosarcoma. The tumor was surgically resected and was easily separated from the surrounding tissues. Nevertheless, a local recurrence developed 43 days after the operation, and the patient's condition deteriorated rapidly. Hepatomegaly and ascites believed to represent the Budd-Chiari syndrome were noted. The patient died on the 163rd postoperative day. Autopsy revealed a tumor extending from the inferior vena cava just above the right renal vein to the right atrium and involving the lobus caudatus of the liver. Clinically, the tumor was thought to have arisen from the middle segment of the inferior vena cava. However, a diagnosis of primary hepatic rhabdomyosarcoma with extrahepatic growth could not be excluded. Only 12 cases of primary liver rhabdomyosarcoma have been reported, and none of those patients demonstrated Budd-Chiari syndrome. Our patient, diagnosed as rhabdomyosarcoma with secondary Budd-Chiari syndrome, is believed to be the first such report.
(Internal: Medicine 32: 67-71, 1993)

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A 49-year-old woman was admitted to our hospital for detailed evaluation of an abdominal mass. Ultrasonography and computed tomography revealed two tumors, one a submucosal lesion of the duodenal second portion and the other a left adrenal tumor. Angiography showed that the tumors were hypervascular. Both tumors were removed surgically and examined histologically using hematoxylin-eosin staining. The duodenal tumor (10 × 6 × 5 cm) was initially diagnosed as a schwannoma, but immunohistochemical studies showed that it was S-100 protein negative and neuron-specific enolase positive. Therefore, this tumor was identified as a stromal tumor with neural differentiation. The left adrenal tumor was a nonfunctioning adenoma.
(Internal Medicine 32: 72-76, 1993)

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A 24-year-old woman developed rheumatoid arthritis in 1986. Anti-SS-A antibody was negative, and Sjögren's syndrome was not associated with the disease. In July 1988 the patient developed pyrexia and cervical lymph node swelling. Infectious mononucleosis was suspected, and a previous contact with Epstein-Barr virus (EBV) was indicated. Pyrexia and cervical lymph node swelling occurred repeatedly and after this dry eyes and mouth also developed. In May 1990 the patient again developed pyrexia and cervical lymph node swelling, and she also had anti-SS-A antibodies (64×) and symptoms associated with Sjögren's syndrome. EBV antibody showed reactivation of EBV by the presence of viral capsid antigen-IgM antibody and a decrease in antibody titer against EBV-associated nuclear antigen. The clinical symptoms of repeated fever and cervical lymph node swelling, as well as the reactivation of EBV, suggest that EBV might have been a factor participated in the onset of Sjögren's syndrome in this patient.
(Internal Medicine 32: 77-79 1993)

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We encountered a patient with Graves' disease showing superior vena cava (SVC) syndrome. The patient was a 72-year-old woman with diffuse nontoxic goiter (diagnosed as chronic thyroiditis); she developed Graves' disease during L-T₄ administration. Radioiodine-131 therapy failed to give sufficient effect, and the intrathoracic goiter became enlarged in association with increases in thyroid stimulating antibody activities, followed by the development of SVC syndrome. The surgically excised thyroid gland was diffuse without any nodules. Microscopic findings revealed adenomatous hyperplasia. The present case, though extremely rare, seems important for the understanding of the mechanism of onset of SVC syndrome in relation to thyroid gland enlargement.
(Internal Medicine 32: 80-83, 1993)

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