Corticosteroids are effective in bringing about a clinical remission in patients with ulcerative colitis. However, in severely relapsed cases, corticosteroids are not always effective even when a high dosage is administered. In addition, the long-term use of corticosteroids often causes serious side effects. Therefore, an alternative treatment for active ulcerative colitis is necessary in order to avoid these clinical problems. In the present pilot study, the efficacy of leukocytapheresis using a centrifugal procedure was evaluated for corticosteroid-resistant, active ulcerative colitis. Fourteen patients with corticosteroid-resistant severely active ulcerative colitis were treated by leukocytapheresis. Thirteen patients (92.9%) achieved clinical remission within 4 weeks after the apheresis, and remained in remission for 8 months on average without any additional corticosteroid therapy. In the remaining patient, in whom remission was not induced, a total colectomy was performed immediately after the fourth course of leukocytapheresis. No significant side effects were noticed throughout the therapy. Both colonoscopic and histological examinations confirmed the beneficial effect of this procedure in terms of the reduction of severe inflammation of the affected colon. We found that the expression of two adhesion molecules, L-selectin and VLA4a, on the surface of peripheral leukocytes was decreased after this new therapy.
(Internal Medicine 36: 322-326, 1997)

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Although a high incidence of thrombotic accidents in Fabry's disease has been postulated, few investigations have been performed. To clarify the incidence of thrombosis in Fabry's disease, we undertook a systematic study on thrombosis in patients with Fabry's disease including hemizygous males and heterozygous females. Sixty patients with Fabry's disease (45 hemizygotes and 15 heterozygotes) from 36 Japanese families were subjected to clinical, biochemical and genetic investigations. We found that seven patients with Fabry's disease (4 hemizygous males and 3 heterozygous females) had experienced thrombotic accidents. Six of these thrombotic patients developed brain infarctions, including one man who had the complication of recurrent thrombophlebitis. The remaining woman showed central retinal artery occlusion and thrombophlebitis. We demonstrated a high incidence of thrombosis in Fabry's disease. Thrombotic accidents occurred not only in hemizygous males but also in heterozygous females. The complication of thrombotic accidents should be taken into account in patients with Fabry's disease.
(Internal Medicine 36: 327-329, 1997)

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We retrospectively analyzed 20 cases of renal infarction to identify the problems in tentatively diagnosing renal infarction. The subjects consisted of 12 outpatients and 8 inpatients whose diagnosis was confirmed by renal scintigram and/or contrast computed tomography. Renal infarction was tentatively diagnosed in only 4 of the 12 outpatients. Causes of hospitalization were cerebral emboli in 5 cases, peripheral emboli in the extremities in 2 cases and one case involved percutaneous transmitral commissurotomy. On initial urinalysis, 11 cases (55%) showed less than 2+ hematuria using dipsticks to test for occult blood. The mean lactic dehydrogenase value was as high as 2, 096 IU while the mean aspartate aminotransferase and mean alanine aminotransferase were 83.1 IU and 78.6 IU. Abdominal ultrasonography revealed abnormalities in only one of 18 cases. In conclusion, since only a moderate degree of hematuria was seen in about half the cases and it was difficult to detect renal abnormalities by ultrasonography, a tentative diagnosis of renal infarction may be difficult in some cases.
(Internal Medicine 36: 330-332, 1997)

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We studied 71 Japanese individuals, 42 patients (30 familial and 12 sporadic) suspected to have facioscapulohumeral muscular dystrophy (FSHD) and 29 family members, clinically and genetically using the chromosome 4qter DNA marker p13E-11. Early onset FSHD was detected in 7 patients, tortuosity of retinal arterioles and hearing impairment in 3 patients, progressive respiratory failure in 3 patients and limb-girdle type muscular weakness in 6 patients. Thirty-six (85.7%) FSHD patients, 3 asymptomatic family members and 1 of 35 healthy volunteers showed EcoRl digestion fragments shorter than 28kb. New mutations were detected in 25% of the patients with shorter EcoRI fragment. The age of disease onset appeared younger with successive generations in 6 parent-child pairs in FSHD families. We confirmed the existence of phenotypic and genetic diversities in Japanese patients with FSHD. It is still difficult to explain the phenotypic diversity merely by the size of the abnormal EcoRl fragment detected with the p13E-11 probe.
(Internal Medicine 36: 333-339, 1997)

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This report describes a 91-year-old patient with patent ductus arteriosus (PDA) complicated by combined valvular disease (CVD) (aortic and mitral stenosis, and aortic, mitral, pulmonic and tricuspid regurgitation). This patient seems to be the oldest living female with PDA and CVD hitherto reported in the medical literature. The patient developed several bouts of congestive heart failure which were treated medically. She not only has survived without surgical management, but is still enjoying her life at age 91. The features of PDA in the elderly are reviewed.
(Internal Medicine 36: 340-344, 1997)

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A case with diabetes mellitus associated with growth hormone (GH)-producing pituitary adenoma is described. A 56-year-old woman who had been treated for diabetes mellitus for 3 years, was admitted for the treatment of hyperglycemia. She showed a few acromegalic features and her plasma GH level was 146±16 ng/ml. After improvement of plasma glucose level by insulin injection, octreotide therapy (100 μg/8 hours) was started. Seven days after the initiation of octreotide therapy, the fasting plasma glucose level was almost normalized without insulin injection. After the octreotide treatment, urinary C-peptide excretion was significantly decreased and the plasma GH level became within normal range. In this case, octreotide appears to have improved the insulin sensitivity by reducing the plasma GH level.
(Internal Medicine 36: 345-350, 1997)

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A diabetic patient with hemoglobin (Hb) J-Meerut and low HbA_1C levels is reported. An automatic glycohemoglobin analyzer used for the determination of HbA_1C revealed an abnormal peak of the peripheral blood obtained from a Japanese female with diabetes. She showed a lower HbA_1C level (3.7%) than expected from her fasting plasma glucose (172 mg/dl). High performance liquid chromatography and isoelectric focusing indicated that her abnormal hemoglobin was Hb J-Meerut [α120(H3)AIa→GIu] and it accounted for 28.3% of the total hemoglobin. Abnormal hemoglobinemia should be considered when a major discrepancy between the levels of HbA_1C and fasting plasma glucose is observed.
(Internal Medicine 36: 351-356, 1997)

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A 62-year-old female with diffuse pulmonary amyloidosis developed abnormal radiographic findings while under observation for hyperimmunoglobulinemia over a ten-year period. Serum immunoglobulin G (IgG) was elevated (4, 620 mg/dl), and associated with monoclonal gammopathy (M protein) of the kappa type, but no evident abnormalities were apparent in bone marrow. Chest radiograph and computed tomography showed a diffuse reticulonodular shadow in the bilateral lung. Thoracoscopic lung biopsy specimen revealed depositions of amyloid in the bronchus and pulmonary vessel. We emphasize that diffuse pulmonary amyloidosis should be considered a possible diagnosis in the presence of monoclonal immunoglobulin.
(Internal Medicine 36: 357-359, 1997)

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We treated three cases of interstitial pneumonitis (IP) in 26 elderly (≥65 years old) patients with non-Hodgkin's lymphoma (NHL) who received the same chemotherapeutic protocol including granulocyte colony-stimulating factor (G-CSF) administration. Fortunately, all three patients recovered from IP spontaneously by discontinuation of G-CSF alone or with administration of corticosteroid. Because the duration and extent of neutrophilia induced by G-CSF administration was not different between the cases complicated by IP and those without IP, underlying pulmonary damage is suggested to be more involved than neutrophil count in the development of IP.
(Internal Medicine 36: 360-364, 1997)

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Two members of a family had chronic hemolytic anemia due to unstable hemoglobin. The abnormal β-chain with a molecular weight of 16 u smaller than the normal β-chain was found within 5 minutes by analysis using electrospray ionization mass spectrometry. The substitution of amino acid was also determined rapidly by this new strategy. The leucine at the 88th position of the normal β-chain was substituted by proline in the hemoglobin as in Hb Santa Ana. This is the first report of a Japanese case of Hb Santa Ana; the clinical course was similar to that in the previously reported cases.
(Internal Medicine 36: 365-370, 1997)

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A 47-year-old woman was referred to our hospital because of severe anemia and polyclonal gammopathy. She developed sicca syndrome after admission. Laboratory data revealed pancytopenia (white blood cells, 2, 800/μl; hemoglobin, 6.4 g/dl; platelets, 6.1×104/μl) and hyper γ globulinemia (5.2 g/dl), and bone marrow was hypoplastic. Histology of the salivary gland showed infiltration of lymphocytes. We report a good response to immunosuppressive therapy in a rare case of aplastic anemia complicating Sjöogren's syndrome.
(Internal Medicine 36: 371-374, 1997)

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A 29-year-old woman with aplastic anemia who complained of visual disturbances and pain in the right eyeball was diagnosed as having cytomegalovirus (CMV) retinitis based upon the characteristic retinal changes and isolation of CMV. She received treatment with ganciclovir (GC V), and the retinitis initially responded for several months. However, the patient was found to have CMV lesions in the left eye followed by neurological symptoms. The CMV isolated just before her death was approximately 20 times more resistant to GCV than that isolated previously, suggesting that the GCV-resistant CMV had developed during the long-term treatment with GCV.
(Internal Medicine 36: 375-379, 1997)

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