We report a male Japanese with corticotropin (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH) associated with multiple colon adenomas/carcinomas. The plasma cortisol level was elevated with no diurnal rhythm and was not suppressed with dexamethasone. Basal plasma ACTH was unmeasurable but subnormally increased after administration of metyrapone or corticotropin releasing hormone. Both adrenals were resected and weighed 90g; the histopathologic findings were similar to those of AIMAH as previously reported. At least 21 colon lesions which were adenomas or carcinomas, were resected endoscopically or surgically. This is the second reported case of the association of AIMAH with multiple colon polyps. An APC gene point mutation was detected in the colon cancer tissue by polymerase chain reaction (PCR)-single strand conformation polymorphism (SSCP)/direct sequencing analysis at the putative splice acceptor site consensus sequence. However, no abnormality of APC gene was detected in the adrenocortical hyperplastic tissue. The possible etiological coexistence of these two diseases is discussed.
(Internal Medicine 36: 536-542, 1997)

View full abstract

In awake human subjects input from the forebrain has significant effects on the respiratory pattern during assisted (AMV) and controlled (CMV) mechanical ventilation. The hypothesis in this animal study was that if the influence from the forebrain is eliminated, the respiration during AMV and that during CMV is controlled by the same mechanism. Fifteen decerebrated and tracheostomyzed dogs were subjected to CMV with a variety of combinations of tidal volume and frequency. The respiratory rhythm during CMV was simulated by a mathematical model composed of the central respiratory activity and inputs from pulmonary receptors. During AMV, the respiratory cycle duration was prolonged, and this was found to be the summated effect of prolonged Ttg (ventilator trigger period) and shortened Tinf (lung inflation period). When these changes in Ttg and Tinf were included, the model for CMV predicted respiratory changes during AMV. We concluded that the basic mechanism controlling AMV and CMV may be the same.
(Internal Medicine 36: 543-549, 1997)

View full abstract

We investigated the ascending somatosensory pathway for pain in 8 patients with Machado-Joseph disease (MJD) and multiple system atrophy (MSA). Pain-related somatosensory evoked potentials (pain SEPs) by CO₂ laser stimulation were examined together with conventional electrically stimulated somatosensory evoked potentials (electric SEPs). The sporadic cerebellar ataxia of patients with MSA showed a normal pattern of pain SEPs as well as electric SEPs. However, pain and electric SEPs were abnormal for the central and/or peripheral ascending pathway in MJD. These abnormalities of pain and electric SEPs in MJD were not related to the clinical severity of sensory impairment, but they indicate that MJD presents a subclinical abnormality for the ascending somatosensory pathways not only for vibratory sense but also for pain sense.
(Internal Medicine 36: 550-555, 1997)

View full abstract

A 50-year-old female patient, who had been followed for 15 years for protein-losing enteropathy, was hospitalized due to epigastric pain. Examination on admission revealed that the patient was in the sub-ileus state. On the 26th day after admission, she complained of severe abdominal pain and shortly after she went into shock. The emergency laparotomy documented intestinal perforation and a tumor. The perforated site was right at the middle of tumor. The histological and histochemical studies identified the tumor as malignant lymphoma of B lymphocyte lineage. As far as we know, this is the third case of malignant lymphoma occurring in the jejunum in a patient with protein-losing enteropathy in Japan. The possible relationship between lymphomas and protein-losing enteropathy is discussed.
(Internal Medicine 36: 556-560, 1997)

View full abstract

A 15-year-old male with hypereosinophilic syndrome was admitted to our hospital because of general malaise and abnormal liver function tests. Endoscopic retrograde cholangiopancreatography demonstrated bile duct changes consistent with primary sclerosing cholangitis. The patient's liver profile tests returned to normal after treatment with prednisolone and ursodeoxycholic acid. In this case, eosinophils might have played an important role in the pathogenesis of the bile duct lesion.
(Internal Medicine 36: 561-564, 1997)

View full abstract

We report an autopsy case of Cockayne syndrome (CS). A 40-year-old Japanese woman was admitted to our hospital for cachexia. She had displayed the striking features of CS, including dwarfism, mental retardation, neural deafness, ataxia, intracranial calcifications, and progeria since her childhood. Endocrinological examinations suggested normal pituitary function and a disorder of the hypothalamus or the cerebrum. She died of acute pneumonia at the age of 42. Autopsy findings showed typical abnormalities in the central nervous system compatible with CS; however, no atherosclerotic change was observed in the systemic arteries.
(Internal Medicine 36: 565-570, 1997)

View full abstract

A 19-year-old woman with diabetic lipemia and maturity-onset diabetes of the young (MODY) is reported. Though her insulin secretory activity was preserved, she fell into mild diabetic ketoacidosis (DKA) and showed type V hyperlipidemia. Post-heparin plasma activity of lipoprotein lipase (LPL) was decreased even 10 days after initiating insulin injection but not deficient. The abnormalities in lipid metabolism were improved by long-term insulin treatment. Though the contribution of the genetic background to the lipid abnormalities is not clear, the characteristics of MODY in this patient including insulin secretory capacity under stress conditions such as DKA might play a role in the development of diabetic lipemia.
(Internal Medicine 36: 571-574, 1997)

View full abstract

A 63-year-old insulin-dependent diabetic woman was hospitalized with itchy skin wheals at the injection sites of human insulin. After intradermal skin testing was performed, the erythema and wheal was recorded immediately. The increased titer of human insulin-specific IgE antibody indicated immediate-type allergy against human insulin. Administration of an anti-allergic drug or desensitization for human insulin every 2 hours was not effective. After continuous subcutaneous insulin infusion (CSII) therapy was performed, both the itching and wheal disappeared. The process may be a desensitization through CSII. CSII may be useful in the treatment for human insulin allergy.
(Internal Medicine 36: 575-578, 1997)

View full abstract

Postprandial atrioventricular (AV) block and hypotension occurred in an 80-year-old man with a 13-year history of diabetes mellitus. Two weeks before admission, he experienced syncope on two occasions after lunch. Master's two-step test induced second degree AV block (2:1 block). Four days after admission, syncope recurred while walking 1 hour after eating; electrocardiogram (ECG) showed complete AV block following 2:1 block. PP interval analysis suggested phase 3 block as the mechanism of AV block. Electrophysiologic study revealed 2:1 HV block. Postprandial syncope ceased after permanent pacemaker implantation. Postprandial AV block and hypotension merit close attention in diabetics.
(Internal Medicine 36: 579-581, 1997)

View full abstract

Three adult Japanese cases of Gitelman's syndrome were characterized by secondary aldosteronism, hypokalemic alkalosis, hypomagnesemia, and hypocalciuria. Two were revealed to be familial cases. A mutation in the thiazide-sensitive Na-Cl cotransporter gene, which had already been confirmed in one family (Takeuchi et al. J Clin Endocrinol Metab 81: 4496, 1996), was not detected in the other two cases. These observations may possibly support the previous report (Simon et al. Nature Genet 12: 24, 1996) that Gitelman's syndrome is caused by a variety of mutations in the thiazide-sensitive Na-Cl cotransporter.
(Internal Medicine 36: 582-585, 1997)

View full abstract

A 76-year-old woman presented with spiking fever and right back pain. Ultrasonography (US) revealed that her right kidney was enlarged. Computed tomography (CT) showed the parenchyma was replaced by non-enhancing masses but the overall kidney shape was maintained. These findings were compatible with those of xanthogranulomatous pyelonephritis (XP). The nonenhancing masses on CT coincided with the multiple butter yellow nodules of the resected kidney and microscopically these lesions were proved to be abscesses with xanthoma cells. In this case, the US and CT findings reflected the pathological feature of XP and thus these techniques are thought to be useful for the diagnosis of XP.
(Internal Medicine 36: 586-590, 1997)

View full abstract

A 55-year-old male who had a remote history of occupational asbestos exposure consulted us because of chest pain. Chest X-ray revealed diffuse pleural thickening and pleural effusion on the right. A diagnosis of malignant mesothelioma, biphasic type was made by needle pleural biopsy. Fourteen months later, the patient died of brain metastasis. At autopsy, malignant mesothelioma of the pleura with metastasis to the brain and bilateral adrenal glands was observed. Brain metastases proven by autopsy are rare in cases of malignant mesothelioma. The ferruginous body count in the lung tissue was 16 per gram of wet weight.
(Internal Medicine 36: 591-594, 1997)

View full abstract

A 66-year-old woman suffering from fever and thrombophlebitis was referred to our hospital. A peripheral blood examination revealed hyperleukocytosis with 96% blast cells and thrombocytopenia. The patient was diagnosed as having acute myeloid leukemia (AML) accompanied by disseminated intravascular coagulation (DIC). A marked decrease in protein C (PC) antigen and activity were observed. In this case, PC levels were lower than those observed in AML with DIC. Induction therapy for leukemia and treatment of DIC were started on the first day of hospitalization. The patient achieved complete remission, with PC antigen and activity levels normalized.
(Internal Medicine 36: 595-597, 1997)

View full abstract