A 76-year-old man with amyotrophic lateral sclerosis was admitted to our hospital because of progressive exertional dyspnea (PaCO2 = 68.5 torr, PaO2 = 62.5 torr). He was put on mechanical ventilation, and thereafter slee...A 76-year-old man with amyotrophic lateral sclerosis was admitted to our hospital because of progressive exertional dyspnea (PaCO2 = 68.5 torr, PaO2 = 62.5 torr). He was put on mechanical ventilation, and thereafter sleep apnea of a central type was recognized. After improvement of general conditions, a portable ventilator "Pneu-PAC" was used for the self-management of nocturnal respiratory insufficiency. A portable ventilator might be of clinical benefit for the management of a patient with neuromuscular disorder whose activities of daily living are still functional.
A 56-year-old man was admitted with hemiparesis and shortness of breath. He was positive to human immunodeficiency virus (HIV) antibody and was diagnosed as acquired immunodeficiency syndrome (AIDS) with Kaposi's sarcoma...A 56-year-old man was admitted with hemiparesis and shortness of breath. He was positive to human immunodeficiency virus (HIV) antibody and was diagnosed as acquired immunodeficiency syndrome (AIDS) with Kaposi's sarcoma and pneumocystis carinii pneumonia. He developed chronic photosensitivity and vitiligo preceding the onset of the AIDS-related complex (ARC). Association of the two skin lesions with HIV infection is very rare. Although the role of HIV infection in these skin lesions is not significant, the immunological responses in the early course of HIV infection may have contributed to the development of both of these skin lesions.
A 16-year-old boy had incomplete Behçet's disease with common carotid artery aneurysm and an ulcer in the transverse colon. To our knowledge, this complication in the carotid artery has been previously reported in only 6...A 16-year-old boy had incomplete Behçet's disease with common carotid artery aneurysm and an ulcer in the transverse colon. To our knowledge, this complication in the carotid artery has been previously reported in only 6 cases. Arterial repair and high-dose corticosteroid therapy (pulse therapy) were successful.
A 53-year-old woman was admitted to our hospital due to high fever, arthralgia and skin rash. Main laboratory data included the following: WBC 17,100/mm, GOT 58 U, GPT 47 U, LDH 1,510 U, ferritin 19,000 ng/ml, adenosine...A 53-year-old woman was admitted to our hospital due to high fever, arthralgia and skin rash. Main laboratory data included the following: WBC 17,100/mm, GOT 58 U, GPT 47 U, LDH 1,510 U, ferritin 19,000 ng/ml, adenosine deaminase 79.1 U/l. She was diagnosed as having adult-onset Still's disease. Aspirin (3.0 g/day) and prednisolone (40 mg/day) were administered. All the symptoms and laboratory data improved rapidly. Adenosine deaminase, ferritin, and LDH are considered to originate mainly from the liver. Liver injury in this disease may be a primary lesion, and various serum markers may be associated with the liver abnormalities.
A case of crescentic glomerulonephritis associated with renal amyloidosis is reported. A 62-year-old woman with a 6-yr history of rheumatoid arthritis developed rapidly progressive glomerulonephritis associated with rena...A case of crescentic glomerulonephritis associated with renal amyloidosis is reported. A 62-year-old woman with a 6-yr history of rheumatoid arthritis developed rapidly progressive glomerulonephritis associated with renal amyloidosis and crescent formation. Amyloid protein was positively stained with anti-AA antibody and its fibrils were demonstrated by electron microscopy. In connection with epithelial cell proliferation in the capsular space, destruction of the capillary basement membrane was observed at the sites where amyloid fibrils were accumulated, whereas glomerular deposition of immunoglobulin and complement was not detected. It is conceivable that the destruction of the glomerular tufts induced by amyloid deposition may be responsible for the crescent formation.
A 77-year-old man complained of headache, dizziness and tactile hallucination. Based on those clinical signs and the findings of computed tomography scanning and magnetic resonance imaging, he was diagnosed as having pit...A 77-year-old man complained of headache, dizziness and tactile hallucination. Based on those clinical signs and the findings of computed tomography scanning and magnetic resonance imaging, he was diagnosed as having pituitary adenoma. Clinical signs and symptoms of Cushing's disease had not been apparent because of the occurrence of the disease at an old age. An increase in serum cortisol and adrenocorticotropic hormone indicated the presence of Cushing's disease. Physical findings obtained thereafter were also compatible with the disease. While the patient was being prepared for surgery, pituitary apoplexy and intraventricular hemorrhage occurred. Massive ascites appeared as a result of tuberculous peritonitis. In spite of treatment for these complications, his general condition progressively deteriorated and he died 39 days after the intraventricular hemorrhage. This case presents the difficulty in the treatment of masked Cushing's disease in the elderly population.
We report a case of minimal thyroid ophthalmopathy treated with intravenous methylprednisolone, in which precise identification of the involved muscle was possible with the use of surface coil magnetic resonance (MR) ima...We report a case of minimal thyroid ophthalmopathy treated with intravenous methylprednisolone, in which precise identification of the involved muscle was possible with the use of surface coil magnetic resonance (MR) imaging. Intravenous methylprednisolone was more effective than the oral prednisolone as judged by orbital MR imaging. MR imaging is one of the useful tools in the diagnosis and assessment of treatment in this field.
We previously demonstrated that neutrophils biosynthesize the Iinoleate epoxide, 9,10-epoxy-12-octadecenoate, from linoleate and hydroxyl radical. This epoxide is highly cytotoxic, and has been termed leukotoxin. We dete...We previously demonstrated that neutrophils biosynthesize the Iinoleate epoxide, 9,10-epoxy-12-octadecenoate, from linoleate and hydroxyl radical. This epoxide is highly cytotoxic, and has been termed leukotoxin. We detected leukotoxin in plasma from two patients with infectious endocarditis and circulatory shock. Maximal leukotoxin levels were 580 nmol/ml and 880 nmol/ml, respectively. The leukotoxin levels were affected by hemodialysis or hemofiltration. Disseminated intravascular coagulation was confirmed by blood coagulation studies in these two patients. Leukocytosis was also observed in these patients. In contrast, leukotoxin was not detected in plasma of normal volunteers. Accordingly, leukotoxin synthesized by recruited neutrophils might be a contributory factor in circulatory shock.
A 35-year-old man presented with acute onset of bilateral lower extremity weakness after ingesting a large amount of carbohydrates. Laboratory investigation revealed severe hypokalemia (1.9 mEq/l) and hyperthyroidism. Th...A 35-year-old man presented with acute onset of bilateral lower extremity weakness after ingesting a large amount of carbohydrates. Laboratory investigation revealed severe hypokalemia (1.9 mEq/l) and hyperthyroidism. The patient also exhibited primary aldosteronism due to a left adrenal adenoma. As a diagnostic tool, paralysis with hypokalemia (2.8 mEq/l) was induced with a glucose infusion. After treatment with methimazole, there were no further episodes of paralysis and subsequent induction of paralysis with glucose was impossible, though primary aldosteronism persisted. These findings indicate that hyperthyroidism played a major role in the development of periodic paralysis, while primary aldosteronism apparently increased the patient's vulnerability to paralytic attacks.
Myocardial metastasis from neoplastic disease is often clinically unapparent, and very difficult to diagnose. Of 151 consecutive autopsies of lung cancer patients, cardiac metastases were found in 67 patients (44.4%). My...Myocardial metastasis from neoplastic disease is often clinically unapparent, and very difficult to diagnose. Of 151 consecutive autopsies of lung cancer patients, cardiac metastases were found in 67 patients (44.4%). Myocardial metastasis was found in only 8 patients (11.9%). ECG of patients with myocardial metastasis revealed ST-T wave changes and various types of arrhythmia. ST-T wave changes were observed in 4 with myocardial metastasis, and in 6 without myocardial metastasis (pericardial metastasis alone). ST-T wave changes is not a specific finding of myocardial metastasis. Two very rare cases with myocardial metastasis showing progressive ST segment elevation with a QS pattern are presented. The appearance of ST segment elevation with a QS pattern in clinically stable lung cancer patients without cardiac symptoms suggestive of myocardial injury indicates the possibility of myocardial metastasis. Myocardial metastasis is often elusive, thus careful observation of ECG changes is of primary importance for the antemortem diagnosis.
We demonstrated TRH-induced release of 7B2 (a neuroendocrine polypeptide) in vivo and in vitro (somatotroph adenoma cells) in a patient with acromegaly. The mean basal plasma 7B2 and growth hormone (GH) levels before ope...We demonstrated TRH-induced release of 7B2 (a neuroendocrine polypeptide) in vivo and in vitro (somatotroph adenoma cells) in a patient with acromegaly. The mean basal plasma 7B2 and growth hormone (GH) levels before operation were 142.8 +/- 3.2 ng/l and 52.4 +/- 1.6 micrograms/l (mean +/- SEM), respectively and these levels significantly rose after an i.v. administration of 500 micrograms of thyrotropin releasing hormone (TRH). After the transsphenoidal adenomectomy, the basal level of plasma GH was restored to the normal level and that of plasma 7B2 was slightly decreased. In addition, TRH-induced response of plasma 7B2 and GH disappeared post-operatively. In a primary culture of somatotroph adenoma cells obtained at surgery, TRH significantly induced secretions of both 7B2 and GH. Immunohistochemical studies showed the positive 7B2 and GH immunoreactivities in somatotroph adenoma cells. These findings strongly suggest that the somatotroph adenoma cells in this case produced and released 7B2 concomitant with GH.
To obtain an overall view of the QRS loop on vectorcardiograms (VCG) of hypertrophic cardiomyopathy (HCM), the coordinate axis was transformed using the resolver method. The morphological features and planarity of the lo...To obtain an overall view of the QRS loop on vectorcardiograms (VCG) of hypertrophic cardiomyopathy (HCM), the coordinate axis was transformed using the resolver method. The morphological features and planarity of the loop were compared with hypertrophic patterns and hypertensive heart disease (HHD). The subjects in the present study included 30 normal individuals, 40 patients with HCM and 30 with HHD. The HHD group was selected from patients showing left ventricular hypertrophy on VCG similar to that of HCM patients. The HCM group showed significantly greater values than the HHD group in the thickness/length ratio, which represents the planarity of the spatial QRS loop. The above finding suggests that the HCM group had greater deformation in the QRS loop than the HHD group. This may provide a useful indicator for the differential diagnosis of the two diseases.
We performed 93 sclerotherapy sessions on liver cirrhosis patients with recurrent variceal bleedings. In each session, hypertonic glucose, thrombin and 1% polidocanol were consecutively injected into the varices, and cha...We performed 93 sclerotherapy sessions on liver cirrhosis patients with recurrent variceal bleedings. In each session, hypertonic glucose, thrombin and 1% polidocanol were consecutively injected into the varices, and changes in the hemostatic system were examined in relation to the symptoms observed during the treatment. Patients underwent sclerotherapy with no complaints in 62 (67%) sessions, and complained of slight symptoms of general fatigue and headache in 19 (20%). In the other 12 (13%) sessions, the procedure was discontinued due to marked manifestations of these symptoms. All symptoms were temporary and disappeared completely after the procedure. These temporary symptoms were closely related to changes in coagulation tests similar to those of disseminated intravascular coagulation, which were observed just after the treatment. Possible activation of the renal kallikrein-kinin system following injection sclerotherapy was also demonstrated.
Familial primary pulmonary hypertension was found in two siblings (sister and brother). The woman noted exertional dyspnea at the age of 28 yr. The younger brother noticed exertional dyspnea, cough with bloody sputum and...Familial primary pulmonary hypertension was found in two siblings (sister and brother). The woman noted exertional dyspnea at the age of 28 yr. The younger brother noticed exertional dyspnea, cough with bloody sputum and pretibial edema at the age of 38 yr. We diagnosed them as primary pulmonary hypertension as based on hemodynamic and histopathological findings. Regardless of the treatment, both patients died of right-sided heart failure with a short time course. We examined their family members, but there were no other members with primary pulmonary hypertension.
A 20-year-old woman was diagnosed as hypodipsic hypernatremia syndrome in association with a variety of hypothalamic syndromes. Computed brain tomography disclosed a space occupying lesion over the region of the hypothal...A 20-year-old woman was diagnosed as hypodipsic hypernatremia syndrome in association with a variety of hypothalamic syndromes. Computed brain tomography disclosed a space occupying lesion over the region of the hypothalamus, lateral ventricle and paraventricles. Evaluation revealed defective osmoregulation of thirst and AVP release and hypothalamic syndrome. She showed no desire to drink at a plasma osmolality of above 320 mOsm/kg. Dissociation in the plasma vasopressin response to osmotic change and hemodynamic change was demonstrated in this patient. Treatment with a vasopressin analogue, desamino-D-arginine vasopressin and forced intake of water restored plasma osmolality and serum sodium levels to normal. In this case, selective osmoregulating dysfunction was presumably associated with pathologic conditions in or around the hypothalamus.
The neurophysiological complications in ACTH deficiency have not been well documented. In this paper, we present a patient with isolated ACTH deficiency who developed various neurological signs. The neurophysiological ab...The neurophysiological complications in ACTH deficiency have not been well documented. In this paper, we present a patient with isolated ACTH deficiency who developed various neurological signs. The neurophysiological abnormalities, including slow wave activity on electroencephalogram, delayed conduction velocity of the peripheral nerves and low amplitude of muscle action potentials, were improved by replacement of glucocorticoid. These findings suggested that glucocorticoid is directly involved in the function of the peripheral and central nervous systems.
A 67-year-old woman was admitted for nephrotic syndrome. In spite of the lack of lymphadenopathy, hepatosplenomegaly and serum hyperviscosity, remarkable monoclonal IgM-proteinemia was demonstrated. Amyloid kidney was sh...A 67-year-old woman was admitted for nephrotic syndrome. In spite of the lack of lymphadenopathy, hepatosplenomegaly and serum hyperviscosity, remarkable monoclonal IgM-proteinemia was demonstrated. Amyloid kidney was shown by renal biopsy. However, in the bone marrow and other organs, neither proliferation nor invasion of monoclonal immunoglobulin-producing cells was revealed by immunohistological investigations of the specimens biopsied or examined at autopsy, excluding Waldenström's macroglobulinemia. Immunosuppressive chemotherapy with cyclophosphamide, vincristine and prednisolone was effective in reducing serum IgM, but could not slow the progression of renal failure. This case suggested the association of generalized amyloidosis with excessive IgM-proteinemia caused by a non-malignant mechanism.
A 43-year-old male with non-Hodgkin's lymphoma accompanied by autoimmune hemolytic anemia (AIHA). His blood group was group B, CcDEe. Alloantibody was absent in his serum, but autoantibody, a combination of IgG and C3d t...A 43-year-old male with non-Hodgkin's lymphoma accompanied by autoimmune hemolytic anemia (AIHA). His blood group was group B, CcDEe. Alloantibody was absent in his serum, but autoantibody, a combination of IgG and C3d type which showed anti-e specificity, was present. Although transient improvement of AIHA was achieved by treatment with 60 mg/day of prednisolone, subsequent worsening of the anemia resulted in the patient's death. Identification of blood group-specific autoantibodies may be beneficial in such a case for blood transfusion.
The kappa/lambda (kappa/lambda) ratios of IgG, IgA and IgM in the cerebrospinal fluid (CSF) and in the sera of 10 patients with Guillain-Barré syndrome were analyzed. The kappa/lambda ratios of IgG, IgA and IgM in the CS...The kappa/lambda (kappa/lambda) ratios of IgG, IgA and IgM in the cerebrospinal fluid (CSF) and in the sera of 10 patients with Guillain-Barré syndrome were analyzed. The kappa/lambda ratios of IgG, IgA and IgM in the CSF and in the serum were not significantly different between the acute and the late stages of Guillain-Barré syndrome nor between Guillain-Barré syndrome and the normal controls. In the CSF, however, the concentration of albumin, IgG, IgA and IgM in the acute phases of Guillain-Barré syndrome were significantly higher than in the normal controls. These results suggest that in Guillain-Barré syndrome, the increase of immunoglobulins may not be due to intrathecal synthesis and therefore there are no significant changes in the kappa/lambda ratios in the CSF.
Adenosine deaminase (ADA) activity and free interleukin (IL)-2 receptor levels were assayed in serum samples from patients with mycoplasma and bacterial pneumonia to evaluate the usefulness of these parameters in disting...Adenosine deaminase (ADA) activity and free interleukin (IL)-2 receptor levels were assayed in serum samples from patients with mycoplasma and bacterial pneumonia to evaluate the usefulness of these parameters in distinguishing between these diseases at an early stage. Serum ADA and free IL-2 receptor levels in patients with mycoplasma pneumonia (32.4 +/- 9.2 U/l, 960 +/- 204 U/ml) were significantly higher than those in patients with bacterial pneumonia (12.5 +/- 3.3 U/l, 425 +/- 86 U/ml) and in healthy controls (14.0 +/- 3.4 U/l, 286 +/- 49 U/ml) (p less than 0.001). Of the 20 mycoplasma pneumonia cases, 19 showed increased levels of ADA over 20.8 U/l; in 17 of the 19, the increase of ADA was seen before the elevation of the specific antibody to Mycoplasma pneumoniae. In contrast, serum ADA levels in all 20 cases of bacterial pneumonia were lower than 20.8 U/l. There results indicate that assays for serum ADA and free IL-2 receptor levels are useful in distinguishing between bacterial and mycoplasma pneumonia at an early stage.