The change of IgA system for Pseudomonas infection was examined by enzyme-linked immunosorbent assay of the system in sera and sputa of patients with chronic airway diseases. The anti-Pseudomonas total IgA antibody titers in both sera and sputa were not elevated in group I with no infection (mainly chronic bronchitis) and group II infected with bacteria other than Pseudomonas, but were elevated in group III colonized transiently with Pseudomonas [diffuse panbronchiolitis (DPB) and bronchiectasis] and group IV colonized persistently with Pseudomonas (mainly DPB). The elevation in the sera and sputa were mainly due to monomeric IgA and polymeric IgA (S-IgA), respectively, and values were significantly higher in group III than in group IV only in the sera. These results indicate that the IgA system is enhanced in advanced DPB and bronchiectasis complicated by Pseudomonas infection, and that the anti-Pseudomonas IgA antibody titer in serum is more useful than that in sputum for the diagnosis of respiratory Pseudomonas infection.
(Internal Medicine 31 : 575-582, 1992)

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This study was undertaken to determine whether the site of ST depression on 12-lead exercise electrocardiography can identify the ischemic site assessed by myocardial Tl-201 emission computed tomography in 409 patients with transient defects. ST depression in some leads was more frequent in those with inferior or lateral ischemia than in those with anterior ischemia. In 214 patients with ST depression and no ST elevation, however, the frequency of ST depression in each lead was similar between those with and without anterior ischemia. In 63 patients with ST depression, single vessel disease and no infarction, the frequency of ST depression in each lead was similar among those with anterior, inferior and lateral ischemia. Moreover, in patients with abnormal Q waves, the site of ST depression was not related to the location of ischemia. In conclusion, the site of exercise-induced ST depression could not be used to determine an ischemic region.
(Internal Medicine 31 : 583-588, 1992)

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The usefulness of ultrasonography (US) in the diagnosis of aldosterone-producing adenoma(s) (APA) was compared with computed tomography (CT) and adrenal scintigraphy in 13 patients. The initial examination with US could demonstrate four of seven right APA. Repeat examinations later in two patients whose tumors were initially not detectable did reveal a tumor in one patient. On the other hand, two left APA were delineated on the initial trial. The other seven left APA, except two small APA in one patient, could be delineated during repeat US examinations performed on other days. With CT, all APA were detected during the first or second trial except for the smallest one. The rate of localization by adrenal scintigraphy with dexamethasone pretreatment was 11 of 13 patients. CT had the highest localization rate. However, US was shown to be useful in the localization diagnosis of APA on repeat examinations with a high delineation rate comparable to CT.
(Internal Medicine 31 : 589-592, 1992)

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We examined the records of sixty-one patients (17 males and 44 females) with endobronchial tuberculosis (EBTB). Smear tests of acid-fast bacilli were positive in 42 cases ad cultures of tubercle bacilli (TB) were positive in 57. The main findings of chest roentgenogram on admission were as follows : no abnormal findings in 8, atelectasis in 30, infiltration in 25, and cavitary lesions in 6. The localization and cross-sectional extension of lesions confirmed bronchoscopically were as follows : trachea in 15, with 3 circular lesions (CLs). Right (R-) main bronchus in 19 with 11 CLs, left (L-) main bronchus in 18 with 11 of CLs, R-truncus intermedius in 14 with 6 of CLs, R-upper lolar bronchus (UB) in 17 with 12 CLs, R-middle lobar bronchus in 14 with 11 of CLs, R-lower lobar bronchus (LB) in 6 with 2 CLs, L-UB in 10 with 7 CLs and L-LB in 3 with 2 CLs. All cases were treated by combination chemotherapy with isoniazid, refampicin, streptomycin and/or ethambutol and the rate of negative conversion of TB was good, but most of circular lesions resulted in severe bronchial stenosis or complete obstruction during and after chemotherapy, and no improvement was seen in any of the atelectasis cases at the cessation of chemotherapy. We discuss the points of early diagnosis and management of EBTB.
(Internal Medicine 31 : 593-598, 1992)

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To examine the mechanism of tissue damage which causes bronchiolectasis in diffuse pan-bronchiolitis (DPB), the cellular components, elastase and its main inhibitor, alpha 1-protease inhibitor (α1-PI) were measured in bronchoalveolar and bronchial lavage fluid (BALF and BLF) from 14 DPB patients. A predominant increase in the neutrophil count was observed in DPB. Elastase activity in BALF and BLF was about 1, 000-fold higher in the DPB group than in the control group. An inhibitor study and a positive correlation between elastase activity and the neutrophil count in both lavage fluids from the DPB group indicated that the activity was mainly that of neutrophil elastase. Western blot analysis of αl-PI showed that most of the α1-PI in the lavage fluids from DPB group was degraded. These results indicated that neutrophil infiltration increases the level of elastase in the DPB lesions ; this increase seems to be closely related to tissue damage.
(Internal Medicine 31 : 599-605, 1992)

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To investigate the relationship among systolic blood pressure (SBP) during exercise (EX), left ventricular hypertrophy (LVH) and plasma norepinephrine concentration (NE) in patients with essential hypertension (HT), 20 patients with HT and 20 age- and sex-matched normal subjects were studied using treadmill testing. According to the change in SBP from rest to EX (ΔSBP), patients with HT were classified into two groups (group I, n=12, ΔSBP < 72mmHg : group II, n=8, ΔSBP ?? 72mmHg).There were no significant differences between the two groups with regard to SBP at rest, NE at rest and NE on EX. However, SBP at peak EX, ΔSBP and the incidence of LVH were greater in group II than in group I. We conclude that exaggerated SBP response to EX in patients with HT is related to LVH.
(Internal Medicine 31 : 606-610, 1992)

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A 46-year-old male patient was diagnosed as suffering from acute myocardial infarction, but his serum creatine kinase (CK) level was extremely low and no CK isozymes were detected in the serum. The total CK activities in the skeletal muscle amounted to only 2% of that of the control, Electrophoresis of the CK isozymes in the skeletal muscle showed that CK-MM was absent but the CK-BB and abnormal isozyme bands were present. There was no evidence of myocardial ischemia, although the exercise treadmill test revealed ST segment depression in the chest leads. One of the patient's sisters had an extremely low serum CK level suggesting inheritance of this abnormality. This is the first report of a case showing familial deficiency of CK.
(Internal Medicine 31 : 611-616, 1992)

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A 73-year-old female patient with myelomatous pleural effusions is described. She was admitted to our hospital with lumbago and emaciation. Laboratory findings revealed cytopenia and hypogammaglobulinemia. Immunoelectrophoresis demonstrated Bence-Jones monoclonal protein in the serum, but not in the urine. Bence-Jones myeloma was diagnosed by the bone marrow aspiration. Chest X-ray film, however, showed bilateral pleural effusions. Fluid cytology revealed numerous immature plasma cells, indicating pleural involvement. Intrapleural administration of α-interferon combined with systemic chemotherapy (oral melphalan-prednisolone with α-interferon im.) was successful in maintaining the resolution of pleural effusions. Intrapleural α-interferon administration seems to be effective in the management of myelomatous pleural effusions.
(Internal Medicine 31 : 617-621, 1992)

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Fungemia, due to Hansenula anomala, developed in an adult patient with small cell lung cancer who received anti-cancer chemotherapy and plasmapheresis for a sensori-motor neuropathy complication. Treatment with intravenous infusion of fluconazole in addition to the removal of the central venous catheter was successful in treating the fungemia. Pathogenic Hansenula anomala infections are rare, but reports of this infection have been increasing. The use of fluconazole treatment for this infection has not been reported in the literature, and this is the first case of an adult infection of Hansenula anomala in Japan.
(Internal Medicine 31 : 622-624, 1992)

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A 48-year-old woman was admitted to our hospital because of proteinuria associated with persistent hypocomplementemia. Intravenous pyelography indicated the presence of horseshoe kidney without other abnormalities. Hypocomplementemia was caused by cold activation of complement. There were some findings suggestive of chronic liver disease (positive HCV antibody, hypergammaglobulinemia, low cholinesterase, etc.). Percutaneous renal biopsy showed the features of multiple evolutional phases of membranous glomerulonephritis.
(Internal Medicine 31 : 625-628, 1992)

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A 44-year-old woman who had been therapeutically irradiated with 45 Gy to the sellar lesion for Cushing's disease has been in remission for 25 years. A large but asymptomatic brain tumor was accidentally found in the right middle fossa by a routine follow-up computed tomography. Upon surgical removal of the tumor, the histology revealed a fibroblastic meningioma. Although the incidence of meningioma following irradiation to pituitary adenoma is rare, a follow-up computed tomography should be taken to any possible late onset, and curable complication.
(Internal Medicine 31 : 629-632, 1992)

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An interesting case of hereditary angioedema in a 26-year-old female is reported, with a finding of transient effusion of fluid into the peritoneal cavity during the attacks. The patient suffered from recurrent abdominal pain for several years, but no family members had any similar symptoms. In spite of repeated hospital admissions and manyexaminations, accurate diagnosis was not made until the most recent admission. The recognition of hereditary angioedema as a cause of acute and/or recurrent abdominal pain may avoid useless invasive procedures and lead to adequate treatment in other similar cases.
(Internal Medicine 31 : 633-635, 1992)

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A 76-year-old man with membranoproliferative glomerulonephritis complicated by methyldopa-induced colitis is reported. Eight months after administration of methyldopa, mucous bloody stool was noted. A barium enema examination showed disappearance of haustra and a spastic rectosigmoid with pseudo-polyposis. Biopsy specimens obtained from the sigmoid mucosa revealed interstitial edema and small inflammatory cells. After cessation of methyldopa treatment, the sigmoid findings, blood pressure, and proteinuria were improved, suggesting that methyldopa not only induced the acute colitis but also worsened the nephrotic syndrome in this patient.
(Internal Medicine 31 : 636-640, 1992)

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A middle-aged woman with hypothyroidism, idiopathic portal hypertension and nephrotic syndrome is presented. This unusual clinical appearance could not be explained as SLE by serological examinations. Pathohistological examinations showed "Banti's liver", Hashimoto's thyroiditis and diffuse proliferative glomerulonephritis with severe tubulo-interstitial nephritis. Immunohistochemical studies revealed IgA deposits in glomeruli. Electron microscopic study disclosed peculiar lucent areas of rare faction with osmiophilic particles in tubular basement membranes. This tubulointerstitial nephritis was considered to be related to the immunological mechanism involving thyroid gland, liver and kidney disorders. This case thus had a clinically rare combination of these three.
(Internal Medicine 31 : 641-648, 1992)

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A 47-year-old woman who was diagnosed as progressive systemic sclerosis (PSS) had acute and severe interstitial pneumonia. Based on the results of her chest roentgenogram, computed tomography, transbronchial lung biopsy (TBLB) and bronchoalveolar lavage (BAL), her interstitial pneumonia was considered to be atypical of PSS. Although she was treated with corticosteroid, methylprednisolone pulse therapy and immunosuppressive drug, the effect of these drugs was insufficient as treatment for the interstitial pneumonia. Therefore, plasma exchange was attempted. After plasma exchange was carried out for three days, her symptoms improved as well as the laboratory data and chest roentgenogram without any severe side effects. We recommend plasma exchange for interstitial pneumonia of PSS as an effective treatment.
(Internal Medicine 31 : 649-654, 1992)

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A 44-year-old female was admitted with the complaint of choking. On fiberbronchoscopic examination, multiple, polypoid lesions with a smooth surface were exhibited at the cervical trachea. Histological diagnosis was B-cell malignant lymphoma, diffuse, medium sized cell type (LSG classification). No other lesions were detected in the systemic evaluation. After combined chemotherapy and regional irradiation, the tumor disappeared. The clinical and pathological characteristics of this case are similar to those of malignant lymphoma of mucosa-associated lymphoid tissue (MALT).
(Internal Medicine 31 : 655-658, 1992)

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Hemoglobin Andrew-Minneapolis, an abnormal hemoglobin with an asparagine residue substituted for a lysine at position 144 of the β-globulin chains, was identified in three members of two generations in a Japanese family. The carriers of hemoglobin Andrew-Minneapolis showed false high levels of hemoglobin A₁c, as measured by the standard cation exchange high performance liquid chromatography, and a moderate tendency for erythrocytosis. This family is the first report of this abnormal hemogloblin in Japan.
(Internal Medicine 31 : 659-661, 1992)

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A 68-year-old man had hydronephrosis due to ureteral stones for two months earlier and then increasing muscle weakness developed. A 30-year-old woman had rapidly progressive quadriparesis. In both cases, severe hypokalemia with metabolic acidosis was observed and the diagnosis of distal renal tubular acidosis was made. The former was considered to be an idiopathic incomplete form and the latter was a secondary complete form associated with Sjögren syndrome. Hypokalemic paralysis may occur as a complication of distal renal tubular acidosis.
(Internal Medicine 30 : 662-665 1992)

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A 75-year-old woman suffered from intermittent high-grade fever and hypertension without any focal sign. Serum lactic dehydrogenase (LDH) was markedly elevated. The fever was resistant to antibiotics and temporarily sensitive to prednisolone. She had heart failure and died. Postmortem examination revealed intravascular proliferation of B lymphocytes, indicative of the diagnosis of intravascular lymphomatosis. The clinical diagnosis is usually very difficult because of the absence of pathognomonic clinical manifestations.
(Internal Medicine 31 : 666-670, 1992)

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We recently had the opportunity to study a 25-year-old male with both Ehlers-Danlos syndrome (EDS) and von Recklinghausen neurofibromatosis (VRNF). We describe the clinical manifestations of the case and discuss the probable pathomechanism of the combination of the two syndromes, with a review of the literature. As recent literature suggests that both syndromes are linked to chromosome 17, we conclude that their combination is not coincidental, but genetically linked.
(Internal Medicine 31 : 671-673, 1992)

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Follicular bronchiolitis is a rare disorder, though it has been recognized as a pulmonary involvement of rheumatoid arthritis in recent years. A57-year-old woman with rheumatoid arthritis was admitted to the hospital with persistent productive cough and breathlessness on exertion. An open lung biopsy was performed to establish a definite pathologic diagnosis of her disease, and she was diagnosed as having follicular bronchiolitis on the basis of the histopathological findings. It is essential to differentiate this disease from other bronchiolar or lymphoproliferative disorders of the lung.
(Internal Medicine 31 : 674-677, 1992)

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A 36 year-old woman was admitted to the hospital with the diagnosis of nephrotic syndrome due to lupus nephritis. The patient had panperitonitis caused by Staphylococcus aureus as a complication, and an emergency laparotomy was performed. After the operation, the patient developed a massive lung alveolar hemorrhage. Methylprednisolone pulse therapy showed a marked effect on the lung hemorrhage. It is known that lung alveolar hemorrhages associated with systemic lupus erythematosus have a very high mortality ; the present case is relatively rare because of the good response to steroid pulse therapy.
(Internal Medicine 31 : 678-681, 1992)

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A 20-year-old man presented with generalized acquired anhidrosis and heat intolerance which was confirmed by a sweat test. Other clinical features included severe pain of the extremities and cutaneous angiokeratomas. On electronmicroscopy, granules specific for Fabry'sdisease were observed in the endothelial cells. Biochemical examination revealed a decreased level of serum α-galactosidase A. These findings confirmed the diagnosis of Fabry's disease.
(Internal Medicine 31 : 682-685, 1992)

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A 34-year-old man with Sweet's syndrome associated with subacute necrotizing lymphadenitis is reported. Histological examination of an erythematous, painful, cutaneous plaque revealed a dermal interstitial neutrophilic infiltrate. A biopsy specimen obtained from an inguinal lymph node showed granulomatous formation, consisting of histiocytes, with central necrosis in the paracortex and macrophages in the sinus. Although the causes of the two diseases remain obscure, this appears to be the first report of Sweet's syndrome associated with subacute necrotizing lymphadenitis.
(Internal Medicine 31 : 686-689 1992)

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We describe siblings with the non-salt-losing form of 21-hydroxylase deficiency who had hypersecretion of aldosterone and plasma renin activity (PRA). Blood pressure and serum electrolytes in both cases were normal despite the aldosterone hypersecretion. Aldosterone secretion was elevated markedly with ACTH administration and with sodium deprivation and/or volume depletion during ACTH suppression by dexamethasone. With suppression by dexamethasone, aldosterone hypersecretion was decreased with lowering of the steroids proximal to the block in the biosynthetic pathway. However, urinary sodium excretion was decreased. These results suggest that the biosynthetic pathway for aldosterone production was preserved. Furthermore, aldosterone hypersecretion and high PRA may serve to compensate for the sodium loss which results in turn from the overproduction of the sodium-losing steroids, such as progesterone and 17α-hydroxyprogesterone which are aldosterone antagonists.
(Internal Medicine 31 : 690-694, 1992)

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Diffuse panbronchiolitis (DPB), which is prevalent in Japan, is known to be rare outside Japan. Although a case of diffuse panbronchiolitis in a second generation Korean was reported in Japan in 1986, no case has been reported in Korea. Recently we found 5 cases of diffuse panbronchiolitis in Korea, two histologically proven and three clinically and radiologically suspected. All 5 cases had the typical respiratory symptoms and signs and a history of chronic sinusitis. In three clinically and radiologically suspected cases, high resolution computed tomography showed the typical findings of DPB and other diseases such as pulmonary emphysema, bronchial asthma, chronic bronchitis and bronchiectasis could be ruled out. More cases of DPB are expected to be found in Korea in the near future.
(Internal Medicine 31 : 695-701, 1992)

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A 38-year-old male with pulmonary emphysemadue to severely reduced serum alpha-1-antitrypsin (AAT) level (14.5 mg/dl) was found to have an inherited new AAT deficient variant Siiyama. Chest roentogenogram and CT scanning revealed advanced emphysema, and severe obstructive ventilatory impairment was observed. During the 4-year follow-up period, the annual rate of decline of FEV 1.0 showed approximately 10-fold greater than the normal decline in FEV 1.0 (-380 ml/yr). Treatment with tamoxifen in order to raise the serum AAT level only resulted in an insufficient increase. Augmentation therapy of human AAT should be considered in the future.
(Internal Medicine 31 : 702-707, 1992)

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A 72-year-old man developed acute renal failure (ARF) following severe hypokalemic rhabdomyolysis. The hypokalemia was due to chronic glycyrrhizin (glycyrrhizic acid) administration. Although glycyrrhizin-induced hypokalemic rhabdomyolysis has been occasionally reported, the association of this type of rhabdomyolysis with ARF has not been described. In the present case, there was a sensitivity to glycyrrhizin the preceding renal insufficiency, and dehydration which was thought to have contributed to ARF. It should be recognized that elderly patients with such predisposing factors may be susceptible to a rapid deterioration of renal function after glycyrrhizin-induced hypokalemic rhabdomyolysis.
(Internal Medicine 31 : 708-711, 1992)

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The familial occurrence of primary hyperparathyroidism in which the proband is a 55-yearold man is reported. His 58-year-old sister and 40-year-old brother had undergone partial parathyroidectomy, and histological examination revealed hyperplasia in both cases. Their father and a daughter of the proband had a history of nephrolithiasis. The three siblings showed high levels of plasma parathyroid hormone (even the two postoperative cases). All of them had a history of nephrolithisis and peptic ulcers. In the proband, image studies did not reveal any abnormality in the neck region. At present, the three cases do not exhibit any abnormalities in the pancreas or the pituitary by imaging studies and endocrine tests.
(Internal Medicine 31 : 712-715, 1992)

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