We analyzed basal energy metabolism in 20 healthy volunteers and 41 cirrhotic patients by indirect calorimetry. Subjects were then given either glucose, branched-chain amino acids (BCAA) or fatty acids as an energy substrate. Resting energy expenditure (REE), nonprotein respiratory quotient (npRQ), and oxidation rates of glucose (%CHO), protein (%PRO) and fat (%FAT) were analyzed. REE and %FAT were significantly higher and %CHO and %PRO were significantly lower in cirrhosis than in controls. These changes correlated with disease severity. Glucose and BCAA were utilized efficiently as energy substrates and reduced %FAT in cirrhosis. Energy efficacy (increased energy expenditure/energy equivalent of the supplemented nutrient) was significantly higher in BCAA (96±16%) than in glucose (41±8%) (p<0.01) and fatty acids (27±13%) (p<0.05). Patients with cirrhosis have an increased energy requirement. BCAA seems to be the preferred substrate to meet this demand, because its energy efficacy is higher than glucose or fatty acids in cirrhosis.
(Internal Medicine 37: 429-434, 1998)

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In order to explore the role of Helicobacter pylori (H. pylori) infection in hypergastrinemia in patients on dialysis, the changes in serum gastrin concentration were examined before and after eradication treatment for H. pylori. Twenty-seven patients on dialysis were treated for the eradication of H. pylori. Fasting serum gastrin concentrations were measured by a radioimmunoassay which detects gastrin 17. Ammonia and pH levels of the gastric juice were also measured. The serum gastrin concentrations were significantly decreased following eradication of H. pylori, and the mean value reached the normal range. The restoration of hypergastrinemia was associated with marked reductions of gastric juice ammonia and pH levels. In contrast, patients in whom H. pylori was not eradicated showed no changes in these parameters. In conclusion, the elevation of the fasting serum gastrin 17 concentration seen in dialysis patients appeared to be attributable to H. pylori infection in the stomach.
(Internal Medicine 37: 435-439, 1998)

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Localized edema of the larynx and pharynx leading to death from asphyxia has long been recognized as a characteristic symptom of hereditary angioneurotic edema (HANE). Long-term follow-up of younger HANE patients has revealed that transient localized acute attacks of edema affect tissues where the microcirculation maintains the blood supply. However, with aging, HANE attacks precipitate disseminated intravascular coagulation(DIC) or multiple organ failure (MOF). Substitution with a C1-inhibitor (C1-INH) has resulted in a fulminant lethal end with a rapid and profound decrease in antithrombin-III (AT-III) activity. A possible mechanism is as follows: Exogenous stimuli activate plasma proteinase systems with the generation of plasma kallikrein that activates the tissue factor pathway (TF) and liberates bradykinin (BK). In younger patients, BK enhances vascular permeability. In the elderly, activated TF is controlled by tissue factor pathway inhibitor (TFPI) and generates thrombin, which is the target enzyme of AT-III and precipitates DIC or MOF. In elderly patients, the characteristic symptom of HANE is hypercoagulation by agerelated changes in the biosynthesis of AT-III or TFPI.
(Internal Medicine 37: 440-443, 1998)

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Five parkinsonian patients with motor fluctuations and dyskinesia after long-term treatment with levodopa were treated with subcutaneous lisuride infusion (0.24-0.42 mg/day) together with oral levodopa for a mean period of 27 (range 13-36) months. AH 5 patients showed marked initial improvement in mobility. Mild psychiatric side effects were observed in three patients; however, these side effects disappeared with reduction in the dosage of lisuride to 0.06 mg per day without a significant increase in motor fluctuations. A low dose of subcutaneous lisuride infusion with oral levodopa is an effective treatment for fluctuations of motor performance in parkinsonian patients without adverse psychiatric effects.
(Internal Medicine 37: 444-448, 1998)

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We present a case of hepatosplenic sarcoidosis. A 51-year-old Japanese male, who was diagnosed to have sarcoidosis 4 years previously, was presented to our hospital because of dry cough and anorexia with weight loss. He had tender hepatosplenomegaly. A dynamic abdominal computed tomography (CT) revealed multiple small low-density areas in both liver and spleen, as well as in magnetic resonance imaging (MRI). The laparoscopic photographs showed many small whitish nodules surfacing on the liver and several tumorous nodules on the spleen. Multiple imaging modalities including dynamic CT and MRI are valuable for detecting focal hepatic and splenic lesions of sarcoidosis.
(Internal Medicine 37: 449-453, 1998)

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A 70-year-old man with diabetic triopathy was hospitalized with left lower quadrant abdominal pain and tenderness, muscle guarding and absent bowel sounds. Three hours after admission, creatine phosphokinase (CPK) was elevated and an abdominal plain film X-ray showed intestinal gas retention, indicating paralytic ileus due to inferior mesenteric artery occlusion. Urokinase (60, 000 units/day) and heparin (10, 000 units/day) were administered. Angiography showed no occlusion in the mesenteric artery. On the 16th day, the abdominal signs had disappeared and CPK was normalized. We diagnosed this case as nonocclusive colonic ischemia because of the hemorheological abnormalities due to diabetic triopathy and the hypercoagulable state.
(Internal Medicine 37: 454-456, 1998)

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We report the case of a 48-year-old woman with apical hypertrophy with massive myocardial flbrosis. She was admitted to our hospital because of general malaise. Echocardiographic examination showed asymmetrical apical hypertrophy, and an electrocardiogram showed a giant negative T wave on V₃-V₆. Right ventricular endomyocardial biopsy revealed massive myocardial fibrosis. Apical hypertrophy can lead to disorders that vary in severity, including rare massive myocardial flbrosis.
(Internal Medicine 37: 457-462, 1998)

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We report a case of T-cell-rich large B-cell lymphoma demonstrating sick sinus syndrome as a single initial symptom, followed by the retention of pericardial and pleural effusions. Intrapleural administration of interferon-α prevented reaccumulation of the pleural effusion for one and a half months, whereas systemic chemotherapy failed to control tumor growth in the skin and lymph nodes. The autopsy revealed involvement of the sinoatrial node of the heart by lymphoma cells.
(Internal Medicine 37: 463-466, 1998)

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The sex-determining region Y chromosome (SRY) triggers testis determination. We report a 46, XX true hermaphrodite who had ambiguous genitalia at birth. A laparotomy at one year of age revealed this patient to have a testis on the right side and an ovotestis on the left side. By polymerase chain reaction analysis no SRY was detected in the DNA from the leukocytes but it was found in the DNA from the ovotestis. The hidden mosaicism with the Y-bearing cells in the gonads is likely the cause of the dual gonads in this patient.
(Internal Medicine 37: 467-471, 1998)

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We describe an unusual patient with hypopituitarism who attained tall stature even without growth hormone (GH). A 37-year-old man was devoid of secondary sexual characteristics, but manifested tall stature with a eunuchoidal feature. Serum levels of GH, insulin-like growth factor-I, gonadotropins and testosterone were all below normal. GH secretion was not enhanced by any provocative stimulus. Adrenocorticotropic hormone increased after administration of corticotropin releasing hormone, but not after insulin-induced hypoglycemia. Thyrotropin increased in response to thyrotropin releasing hormone, but both free T3 and T4 did not rise. Magnetic resonance imaging disclosed a transected pituitary stalk. The present patient had hypopituitarism due to perinatal problems but had grown with the aid of non-GH growth-promoting factors, which suggests that man may be able to achieve statural growth even without GH.
(Internal Medicine 37: 472-175, 1998)

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A 56-year-old man was referred to our hospital for evaluation of episodic disturbance of consciousness. Hypoglycemic symptoms were noted and Whipple's triad was satisfied. The 75 g OGTT and the glucagon test revealed a high baseline insulin level and hyperreactivity to glucagon. A pancreatic tumor and liver metastases were found by abdominal computed tomography (CT). Based on the finding of liver biopsy, the final diagnosis was malignant insulinoma with liver metastasis. He selected conservative treatment and no hypoglycemic crisis has occurred for one year since discharge. Early diagnosis and long-term follow-up is necessary since this tumor is slow growing.
(Internal Medicine 37: 476-479, 1998)

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We report a case of multiple diffuse flbrosarcoma of bone. The patient, a 38-year-old man, was referred to our hospital with knee pain, anemia and thrombocytopenia. No solid mass was seen on radiographic examination of the knee joint, but magnetic resonance imaging showed hypointensity of the distal femur. Femoral biopsy revealed proliferation of long spindle-shaped fibrosarcoma cells, while a bone marrow biopsy of iliac bone (which appeared normal on radiographic examinations) showed replacement of hematopoietic cells by flbroblast-like spindle cells. A diagnosis of multiple diffuse fibrosarcoma of bone was therefore made. Autopsy revealed tumor invasion into multiple bones and several visceral organs and extramedullary hematopoiesis in the liver, spleen and lymph nodes. As this patient had leukoerythroblastic anemia with poikilocytosis, splenomegaly exhibiting extramedullary hematopoiesis, and apparent fibrotic change in his bone marrow, we suggest that this extremely rare disease should be considered in the differential diagnosis of myelofibrosis.
(Internal Medicine 37: 480-483, 1998)

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A 35-year-old female presented in 1989 with hepatosplenomegaly, but no conclusive diagnosis was established. From 1992, she experienced transient episodes of facial flushing and palpitations. Osteosclerotic change was detected radiologically. Colonoscopy revealed massive polypoid lesions. Mast cells were demonstrated in bone marrow smear and imprinted preparations of colon biopsy specimens by toluidine blue staining. Plasma concentrations of histamine and soluble c-kit were elevated. She was successfully treated with interferon-α and prednisolone, resulting in the disappearance of histamine-related attacks and a gradual decrease in tumor size. However, the remission was interferon dose dependent. This case was considered as systemic mastocytosis with massive polypoid colon lesions and showed the importance of maintenance therapy with interferon-α.
(Internal Medicine 37: 484-488, 1998)

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While traveling in India, a previously healthy twenty-year-old man had febrile diarrhea. The patient was prescribed medical therapy, and all symptoms were resolved. Fourteen days later, however, similar symptoms recurred. The patient was admitted to a local hospital, but was transferred to our department because of oliguria. Salmonella paratyphi A was isolated from blood and stool specimens, and Giardia lamblia was identified in his stool. The patient's condition was complicated by acute renal failure. The patient received tosufloxacin and metronidazole. Renal function recovered completely without hemodialysis. Paratyphoid fever and giardiasis were resolved. It is suggested that giardiasis exacerbated the paratyphoid fever.
(Internal Medicine 37: 489-492, 1998)

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