Objective The purpose of this study was to survey the clinical characteristics, complications, and therapeutic outcome in patients with acromegaly.
Patients and Methods The clinical features of 65 patients with acromegaly (31 males, 34 females; mean age: 50±2 yr.) who were admitted to Tokyo Women's Medical University between 1990 and 1999 were analyzed retrospectively from medical records.
Results The retrospective analysis revealed that the diagnosis of acromegaly was preceded by approximately 8.1±1.1 years of signs and symptoms of the disease. Forty-six of the 65 patients (71%) had macroadenomas, 16 (25%) had microadenomas, and the remaining three had empty sella. The rate of biochemical cure or remission was 81% for microadenoma (13/16), 64% for macroadenoma without extrasellar extension (9/14), and 13% for macroadenoma with cavernous sinus extension (2/15). Eighteen (28%) patients had impaired glucose tolerance (IGT) and 32 (49%) had diabetes mellitus (DM). After treatment for acromegaly, glucose metabolism was analyzed again in 38 patients, and it improved in 26 patients with IGT or DM. Twenty-five of 65 patients (38%) had hypertension. Of 26 patients who underwent barium enema or colonoscopy, 10 had colonic polyps and 4 had colon cancer.
Conclusion This study suggests that long-term excessive growth hormone (GH) secretion causes many complications. Therefore, awareness of the early symptoms and signs of acromegaly and long-term careful management of complications, along with therapy to reduce serum GH/insulin-like growth factor (IGF)-I levels, are important for patients with acromegaly.
(Internal Medicine 40: 987-992, 2001)

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Objective In order to elucidate whether or not genetic variations of TSC-22 (TGF [transforming growth factor]-β-stimulated clone 22), which was originally identified as a TGF-β-responsive leucine zipper protein in murine osteoblastic cells, are associated with type 2 diabetes, the genomic organization of the human TSC-22 gene was determined and the association between its polymorphisms and type 2 diabetes was examined.
Results The human TSC-22 gene spans approximately 5 kilobase pairs and is encoded in three exons. Two single nucleotide polymorphisms (SNPs) were identified in the coding region of the first exon, two other SNPs in the first intron, and one SNP in the putative promoter region. There were, however, no significant differences in the frequency of these polymorphisms between patients with type 2 diabetes and non-diabetic control subjects.
Conclusion It is unlikely that the TSC-22 gene is a locus responsible for type 2 diabetes.
(Internal Medicine 40: 993-997, 2001)

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Objective To define the clinico-epidemiological features of pulmonary histiocytosis X in Japan.
Methods A nationwide survey was carried out in 1997 using two questionnaires.
Results The first questionnaire, which attempted to determine the number of patients during 1996, revealed that the number of patients treated at hospitals with 200 or more beds during the one-year period was estimated to be 160 (95% confidence interval: 140-180). The estimated crude prevalence among those aged 16 to 70 years was calculated as 0.27 and 0.07 per 100, 000 population in males and females, respectively. The second questionnaire was concerned with the clinico-epidemiological features of the disease. Seventy-three histologically diagnosed patients were evaluated. It primarily afflicted younger adults, between the ages of 20 and 50, and showed a male predominance. Over 90% of the patients were smokers or ex-smokers and over 50% started smoking before 20 years of age, suggesting a strong association with cigarette smoking. Steroid therapy was applicable to 34% of the patients. In the patients who received steroid therapy, regression and stabilization were observed in 28% and deterioration in 36%. As for the patients for whom steroids were not required, remission occurred in 63% and progression in 10%. The ratio of remissions plus stabilization was higher in the patients who were not treated with steroids compared with those who required steroid therapy (p<0.05).
Conclusion In patients with pulmonary histiocytosis X therapeutic results obtained with steroids seemed not to be encouraging, although steroids are thought to be the most plausible treatment.
(Internal Medicine 40: 998-1003, 2001)

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A 20-year-old woman with IgA nephropathy was admitted to Jikei University Hospital for the treatment of rapid deterioration of renal function after receiving ¹³¹I-therapy against hyperthyroidism on October 23, 1999, and hemodialysis was started. On admission, she was diagnosed as having Evans' syndrome in addition to known Graves' disease. Renal biopsy revealed end-stage renal damage, then, hemodialysis was maintained. Treatment for Evans' syndrome was also started and her general condition gradually improved. The present case implied that "Graves' disease" and "Evans' syndrome" could represent some of the manifestations of an underlying immunological disorder in the patient.
(Internal Medicine 40: 1004-1010, 2001)

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A 29-year-old man, who had been treated with potassium, spironolactone and indomethacin for over 9 years, was admitted because of nausea, vomiting, diarrhea and tetany manifestation. At the age of 20, he had been diagnosed as having Bartter's syndrome according to the criteria of the Japanese Ministry of Health and Welfare. Findings on admission were hypokalemia, hypomagnesemia and hypocalciuria. Renal distal fractional reabsorption rates of sodium, chloride and calcium were markedly decreased by administration of furosemide but there was no obvious change with administration of thiazide. These findings indicate that the patient had Gitelman's syndrome rather than Bartter's syndrome.
(Internal Medicine 40:1011-1014, 2001)

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We report a 43-year-old Japanese man with hypobetalipoproteinemia likely due to apolipoprotein (apo) B-76, a new truncation of apo B, and with homozygousity for the apo E2 isoform. He had no history suggestive of fat malabsorption and no sign of neurological disorder. His fasting baseline serum low-density lipoprotein (LDL) cholesterol and apo B levels were approximately half of normal. His plasma apo E level was elevated and its phenotype showed the E2/E2 homozygote. SDS-polyacrylamide gel electrophoresis of delipidated LDL fraction revealed a new truncated apo B, designated as apo B-76 according to the centile system of nomenclature. The postprandial lipid metabolism of the patient showed an almost normal response after fat loading.
(Internal Medicine 40: 1015-1019, 2001)

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We report a patient with pulmonary adenocarcinoma complicated by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) following systemic chemotherapy with cisplatin (CDDP) and vindesine (VDS). A 66-year-old woman was diagnosed as having pulmonary adenocarcinoma with malignant pleural effusion following investigations for cough and dyspnea. After drainage of the effusion she received combination chemotherapy with CDDP and VDS. She developed SIADH 48 hours following chemotherapy. Interestingly, the use of carboplatin (CBDC A) and VDS in the subsequent treatment course was well tolerated indicating that the SIADH was most likely to have been induced by administration of CDDP.
(Internal Medicine 40: 1020-1023, 2001)

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A 48-year-old man was diagnosed with primary alveolar proteinosis (PAP). Whole lung lavage was performed for treatment, and the opacity on his chest X-ray completely disappeared. The value of cytokeratin 19 fragment (C YFRA) in the serum was high at the beginning, decreased after lung lavage, and became elevated again when the disease recurred 7 months later. As PAP is thought to be a problem of surfactant secreted from type II pneumocytes, and a cytokeratin is present in the alveolar epithelial tissue, the value of serum CYFRA might be related to the severity of PAP.
(Internal Medicine 40:1024-1027, 2001)

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Primary uterine non-Hodgkin's lymphoma (NHL) is an extremely rare disease. To accumulate more information on clinical data, we report three cases of primary uterine NHL with apparently the first demonstration of karyotypic analysis. Histological diagnosis was diffuse large B cell type in all patients. Two of them with advanced stage showed chemoresistance and a short survival. The remaining case with early stage showed an uneventful course following operation. No common chromosomal abnormality was detected. The therapeutic strategy for uterine NHL might therefore be similar to that for other types of aggressive NHL, although a larger study is needed.
(Internal Medicine 40: 1028-1031, 2001)

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Postural tachycardia syndrome is defined as the development of orthostatic symptoms without orthostatic hypotension. We report a 28-year-old female patient with postural tachycardia syndrome who exhibited palpitation, lowgrade fever and weight loss. Evaluation of autonomic nervous system functions showed that cardiovagal function was normal. Sweat response to acetylcholine was decreased. Excessive blood pressure elevation was seen in phase IV of the Valsalva maneuver. Pathophysiologic factors in this case were considered to be α adrenergic denervation and β adrenergic hyperresponsiveness. It is important that this syndrome be widely recognized and properly diagnosed.
(Internal Medicine 40: 1032-1036, 2001)

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We describe a 19-year-old woman with hemophagocytic syndrome and adult Still's disease who showed rare features of central neurological involvement, including cerebellar symptoms and the sudden onset of unconsciousness with pleocytosis in the cerebrospinal fluid during the early course of the illness. As this patient's serum showed a high level of interferon-γ and soluble interleukin 2 receptor, this might play a pathologic role in the development of central nervous system symptoms. Intensive treatment consisting of methylprednisolone pulse therapy followed by the oral administration of methylprednisolone and cyclosporine, as well as plasma exchange, was found to achieve good results.
(Internal Medicine 40: 1037-1040, 2001)

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We report here a patient with myelodysplastic syndromes (MDS), which was complicated with several autoimmune disorders and asymptomatic immunologic abnormalities. An 82-year-old woman with refractory anemia (RA) rapidly developed thrombocytopenia with the appearance of symptoms such as purpura, fatigue, anorexia, and weight loss. Furthermore, clinical examinations revealed that she also had Addison's disease, rheumatoid arthritis, and autoimmune hematological diseases such as thrombocytopenia and hemolytic anemia. However, the cytopenia and all autoimmune disorders were remarkably improved after she received steroid therapy.
(Internal Medicine 40: 1041-1044, 2001)

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Mycobacterium kansasii, an atypical Mycobacterium, may cause serious illness in humans. We describe a M. kansasii infection of the foot joint, which was diagnosed in a 46-year-old woman with systemic lupus erythematosus. The diagnosis was based on a positive culture from degenerative tissue and histological diagnosis of a synovium. We reviewed the literature regarding M. kansasii infection of the joint, bone, and periarticular structures focusing on the complication of rheumatic diseases.
(Internal Medicine 40: 1045-1049, 2001)

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The initial presentation of a patient with Wegener's granulomatosis was indistinguishable from that of Henoch-Schönlein purpura. The patient presented with skin purpura and pulmonary hemorrhage followed by purpura in the colon. The diagnosis of this patient at that time was Henoch-Schönlein purpura. With time, massive lesions in the sinus and those with cavities in the lung became apparent, and a specimen obtained from the sinus massive lesion was disclosed to be granulomatous inflammation. Retrospectively, the proteinase 3 antineutrophil cytoplasmic antibody turned out to be strongly positive in her stored serum from the time of the initial presentation.
(Internal Medicine 40: 1050-1054, 2001)

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A 29-year-old man was admitted because of fever, arthralgia and swelling of the cervical lymph nodes. A diagnosis of histiocytic necrotizing lymphadenitis (HNL) was made based on the findings of a lymph node biopsy. At first, piperacillin sodium (PIPC) was started, but a spiking fever persisted. We therefore changed the PIPC treatment to minocycline. On the following day, his clinical and laboratory findings were dramatically improved. After administering minocycline for 10 days, the HNL symptoms completely disappeared. He has been in good health for 3 years since undergoing treatment. This case strongly indicates that minocycline-sensitive microorganism(s) may be related, at least in part, to the etiology of HNL.
(Internal Medicine 40: 1055-1058, 2001)

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A 58-year-old woman with calcinosis Raynaud esophageal sclerodactyly telangiectasia (CREST) syndrome presented with slowly progressive renal dysfunction. She was normotensive with normal plasma renin activity and lacking symptoms of vasculitis. Mild proteinuria was of tubular origin, but serological tests and an absence of sicca symptoms excluded the possibility of Sjogren's syndrome. Light microscopic study of renal biopsy showed interstitial fibrosis with ectasia and degeneration of proximal tubule and lymphocyte infiltration. There were no remarkable changes in the glomeruli. Chromatographic analysis of the Chinese herbs regimen that she had been taking for several years demonstrated aristolochic acid. She was diagnosed as Chinese herbs nephropathy. Therapy with oral prednisolone was markedly effective in improving renal function and anemia. To our knowledge, this is the first report of Chinese herbs nephropathy complicating connective tissue disease. It is important to consider the possibility of Chinese herbs nephropathy when patients treated with Chinese herbs develop renal dysfunction.
(Internal Medicine 40:1059-1063, 2001)

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A 57-year-old man presented with pneumonia, respiratory distress, and myelodysplastic syndrome. A diagnosis of Legionnaires' disease due to Legionella pneumophila (L. pneumophila) was established. The patient had long been drinking tap water via a conduit from a hot spring resource, from which L. pneumophila was also isolated. Both the patient's strain and the water strain of L. pneumophila were identified as serogroup 1, and the genetic relatedness between the two strains as seen by pulsed-field gel electrophoresis was 87%. The patient was successfully treated with erythromycin, fluoroquinolone, and rifampicin. This case raises an important issue on public health represented by legionellosis in Japan.
(Internal Medicine 40:1064-1067, 2001)

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