Altered cerebellum development and dopamine distribution in a rat genetic model with congenital hypothyroidism.

Thyroid hormones play crucial roles in the development and functional maintenance of the central nervous system. Despite extensive studies of the neural function of thyroid hormones, little is known about the effects of hypothyroidism on behavioural traits and the mechanisms underlying such effects. In the present study, we report an investigation of congenitally hypothyroid mutant rdw rats, revealing a novel function of thyroid hormones in the central nervous system. The rdw rats were subjected to behavioural analyses such as the rotarod test, open field test and circadian activity measurement. To determine the cause of behavioural disorders, cerebellar morphogenesis was examined by immunohistochemical analysis, and the axonal transport of dopamine in the nigrostriatal pathway was analysed by high-performance liquid chromatography and western blotting. The effects of thyroxine administration to the rdw rats were examined by behavioural analysis. The rdw rats showed severe impairment of motor coordination and balance. This could be explained by the fact that the rats showed severe retardation of cerebellar morphogenesis, which correlates with the small somata and poor dendritic arborisation of Purkinje cells and retarded migration of granule cells particularly during the first two postnatal weeks. Moreover, the rdw rats showed hypoactivity, characterised by decreased circadian locomotor activity. After weaning, thyroxine administration improved the dwarfism in rdw rats but had no effect on cerebellar function. In addition, the rdw rats showed anxiety and depression intrinsically to novel surroundings. Interestingly, the rdw rats showed high levels of dopamine in the substantia nigra and low levels in the striatum, an important centre for the coordination of behaviour. Furthermore, low levels of tubulin in the striatum were detected, indicating the aberrant axonal transport of dopamine in the nigrostriatal pathway as a result of the reduced delivery of microtubules. These findings indicate an important function of thyroid hormones in cerebellar formation and in the regulation of axonal transport of dopamine. Moreover, rdw rats will be useful for studies of brain function and behavioural disorders in congenital hypothyroidism.

[Aortic valve repair with minimal access "J" sternotomy].

Recently many cardiovascular operations have been performed with minimally invasive cardiac surgery (MICS). It is reported that MICS is superior to standard full sternotomy in regard to cosmetic and post-operative recovery. In our institution, aortic valve repair operations have been performed to aortic valve insufficiency. This time, we performed aortic valve repair by minimal access "J" sternotomy. A 63-year-old male with degenerative aortic regurgitation underwent aortic valve repair by MICS. He had experienced dyspnea on exertion 7 days before admission. Preoperative transthoracic echocardiogram showed the grade of aortic regurgitation (AR) was severe. Surgery was successful and the grade of AR became trivial. This combined procedure have merits for patients because of freedom from anticoagulation and small incision. Such combined surgery may be indicated in the treatment in a subset of patients with degenerative AR.

[Giant coronary artery aneurysm in diagonal artery; report of a case].

We report a giant coronary artery aneurysm occurred in the diagonal artery. A 44-year-old woman was referred to our institution for further examination of chest X-ray abnormality. Computed tomography revealed a 6 cm intracardiac mass adjacent to pulmonary artery. Cardiac catheterization revealed a giant coronary artery aneurysm with the large thrombus in the diagonal artery. A giant aneurysm 6 cm in diameter was exposed through a median sternotomy. Under beating heart with cardiopulmonary bypass, the aneurysm was opened and organized thrombus was removed. The influx and efflux of the aneurysm were identified and ligated. Under arrested heart with cardioplegia, the diagonal artery was bypassed with the left internal thoracic artery. Finally the aneurysm was obliterated with the running suture. The patient discharged at the 17th postoperative day without any complications. Histologic evaluation of the resected aneurysm revealed atherosclerotic change, destruction of vascular layers and infiltration of inflammatory cells. These findings suggested previous history of coronary arteritis. The coronary aneurysm in this case might be resulted from Kawasaki disease.

Cleavage of S-S bond by nitric oxide (NO) in the presence of oxygen: a disproportionation reaction of two disulfides.

Disulfide bond was cleaved by a catalytic amount of nitric oxide in the presence of oxygen, which was confirmed by experiments employing two symmetrical disulfides. The reaction resulted in the formation of unsymmetrical disulfides in nearly 50% yields. The steric hindrance of alkyl disulfide slowed the reaction rate, and an electron-donating group on the aryl disulfide promoted the reaction. The substituent and S-nitrosothiol effects suggested that the reaction was initialized with an oxidative process by NO+.

Radical scavenging by N-aminoazaaromatics.

N-Aminoazaaromatics were found to react with nitric oxide in the presence of oxygen to afford deaminated products in high yields. The reaction proceeded almost instantaneously in various solvents including water, and one to two equivalent of NO was consumed depending upon the amount of oxygen coexisted, and 1 equivalent of N2O was released in the reaction. In addition, N-aminoazoles were deaminated by potassium superoxide to give parent azoles in good yields. Two equivalents of superoxide was consumed, and about half equivalents of both nitrite and nitrate ion were released. The results demonstrated that N-aminoazoles have ability to protect the biological system against the oxidation promoted by radicals such as nitrogen oxides and superoxide.

Disproportionation reaction of disulfides promoted by nitric oxide (NO) in the presence of oxygen.

Two disulfides brought about disproportionation reaction to afford an unsymmetrical disulfide in 50% yield with a catalytic amount of nitric oxide in the presence of oxygen. The reaction proceeded faster when alkyl disulfides were employed for the reaction, and the substituent effects suggested that the reaction commenced with an oxidative process.

Relapsing polychondritis diagnosed by Tc-99m MDP bone scintigraphy.

PURPOSE: Relapsing polychondritis is a generalized recurring disease of cartilage that involves joints, trachea, bronchi, laryngeal cartilages, costal cartilages, and cartilages of the ear and nose. It is associated with autoimmune diseases, including Hashimoto disease in some cases. METHODS: The authors evaluated a 29-year-old man with relapsing polychondritis who had symptoms and signs of a common cold for 2 months and anterior chest pain near the sternum for 1 month. RESULTS: After the diagnosis, the authors found that the patient had a history of thyroid therapy for hyperthyroidism 15 years before. Tc-99m MDP bone scintigraphy performed to evaluate anterior chest pain showed diffusely increased accumulation of radioactivity in all costocartilages and sternoclavicular joints. Based on that information, relapsing polychondritis was diagnosed. Ga-67 citrate scintigraphy was preformed to determine the optimum biopsy site of the cartilage. The diagnosis was histologically supported by the results of the open biopsy. CONCLUSIONS: In this case, Tc-99m MDP bone scintigraphy was useful for diagnosing relapsing polychondritis, and Ga-67 citrate scintigraphy was helpful in determining the biopsy site.

Rhabdomyolysis after infection and taking a cold medicine in a patient who was susceptible to malignant hyperthermia.

A case of rhabdomyolysis after a possible viral infection and the use of a cold medication is reported. A 41-year-old man who presented with dysarthria, dysphagia, progressive weakness of his muscles and a high grade fever was admitted. He suffered from massive rhabdomyolysis, acute renal failure, and bronchopneumonia. Hemodialysis, antibiotics, and hydration therapy were effective in the treatment of his illness. Although the cause of the rhabdomyolysis was not completely clear, he was subsequently shown to be susceptible to malignant hyperthermia (MH) based on the results of a caffeine-halothane contracture test. When a mild recurrence occurred during a follow-up muscle biopsy, intravenous dantrolene sodium was administered and he improved immediately. This case suggests that MH should be considered in patients with rhabdomyolysis when the cause is unclear. The caffeine-halothane contracture test may also be helpful in the diagnosis.

Intraoperative spasm of coronary and peripheral artery--a case occurring after tourniquet deflation during sevoflurane anesthesia.

A 68-yr-old man with a 9-yr history of hypertension presented for hemiglossectomy, segmental resection of the mandible, and the radial forearm free flap grafting. Intraoperatively, facial artery spasm was observed during microvascular suturing of the radial artery to the facial artery. Simultaneously, systolic blood pressure decreased from 100 to 80 torr and the ST segment elevated to 15 mm from the base line. The possible mechanisms responsible for vasospasm in coronary as well as in peripheral arteries under sevoflurane anesthesia are discussed.

Simultaneous determination of cysteine sulfinic acid and hypotaurine in rat tissues by column-switching high-performance liquid chromatography with electrochemical detection.

Cysteine sulfinic acid (CSA) and hypotaurine (HT) were determined by electrochemical detection with a glassy carbon electrode at 0.95 V vs. Ag/AgCl. The separation of CSA and HT was accomplished by coupled-column liquid chromatography, consisting of an anion-exchange column and a cation-exchange column. For the determination of CSA and HT in rat tissues, a column-switching system was introduced to remove interferences from late-eluting endogenous substances. The limits of determination were 0.05 microM for both sulfinic acids. The average precisions (C.V.) over the concentration range of 0.05 to 5 microM were 4.3% for CSA and 4.2% for HT.

[Influence of KU-1257 on the recurrence and relapse of acetic acid-induced chronic gastric ulcers in rats].

The influence of KU-1257 on the recurrence and relapse of acetic acid ulcers in rats was investigated grossly and histologically in comparison with that of cimetidine. The ulcer was induced by topical application of glacial acetic acid at the junction of the corpus and antrum on the anterior wall of the stomach. The drug was administered from the 5th to the 153rd day after the ulcer induction and then discontinued to the 238th day. The healing rates of the control groups (control) rose until the 119th day after the ulcer induction, followed by ups and downs. The quality of healing in the regenerated mucosa and the granulation tissue of the healed ulcer was poor, resulting in the recurrence and relapse of ulcers. The recurrence and relapse of ulcers also occurred in the cimetidine groups (CIM). On the other hand, the KU-1257 groups (KU-1257) showed much lower recurrence and relapse rates of ulcers than the control and CIM groups. Moreover, KU-1257, unlike CIM, improved the quality of ulcer healing throughout the period of its administration and even after it was discontinued. These results suggest that KU-1257 improves the quality of ulcer healing, and this may contribute to the low recurrence and relapse rates of ulcers.

Adult T-cell leukemia involving the central nervous system after remission of adult respiratory distress syndrome.

We report a rare case of adult T-cell leukemia (ATL) in which the patient had an acute type of ATL involving the central nervous system (CNS) after remission of adult respiratory distress syndrome (ARDS) due to human T lymphotropic virus type 1 associated bronchopneumopathy. A 62-year-old woman was admitted to the hospital because of ARDS. Pulse therapy with methylprednisolone improved ARDS, but she fell into a coma due to ATL and CNS invasion 5 months after recovery. Although chemotherapy decreased the fraction of abnormal lymphocytes, her consciousness level did not improve and she died.

Insulin-dependent diabetes mellitus associated with Graves' disease and idiopathic hypoparathyroidism.

We report a case of 26-year-old woman with Graves' disease and idiopathic hypoparathyroidism diagnosed at 11 years of age, who subsequently developed insulin-dependent diabetes mellitus (IDDM) at 17 years of age. Treatment with antithyroid agents had failed to control her Graves' disease and her IDDM was unmanageable despite insulin therapy. Surgical intervention was carried out, resulting in an improvement of both her hyperthyroidism and IDDM. This case is the first report of polyglandular autoimmune (PGA) syndrome, presenting the association of IDDM, Graves' disease and idiopathic hypoparathyroidism.

Cytomegalovirus mononucleosis with mixed cryoglobulinemia presenting transient pseudothrombocytopenia.

A 61-year-old man was admitted to our hospital because of fever and general malaise. We diagnosed his condition as cytomegalovirus (CMV) mononucleosis by hematological testing. His platelet count was decreased in spite of collection with several anticoagulants at room temperature (20 degrees C); no decrease was observed at 37 degrees C. Mixed cryoglobulinemia, monoclonal IgM and polyclonal IgG, which acted as anti-platelet antibodies in cold condition, were found in the patient's serum. With the resolution of CMV infection, the cryoglobulins disappeared and the platelet count returned to normal without platelet agglutination. This is the first report of transient pseudothrombocytopenia in CMV mononucleosis with mixed cryoglobulinemia.

Cytomegalic inclusion disease presenting acute intrahepatic cholestasis.

An 83-year-old man suffering from pulmonary emphysema was admitted to our hospital because of jaundice. He was diagnosed as acute intrahepatic cholestasis but the etiology could not be determined during the treatment period. In spite of treatment, the jaundice worsened progressively without any elevation in serum transaminase, and he died of respiratory failure 58 days later. An autopsy revealed a generalized cytomegalic inclusion disease, predominantly in the biliary tracts, liver and lungs. This is a rare case of cytomegalic inclusion disease presenting acute intrahepatic cholestasis without any elevation of transaminase during the clinical course.

[Reduction in leukocyte elastase levels in patients with sepsis by administration of urinastatin].

Leukocyte elastase (LE) plays a role in the development of shock. Therefore, we examined the effects of urinastatin, a protease inhibitor, on LE in 7 patients with sepsis. LE levels before the administration of urinastatin were high in all patients, especially those in shock. Administration of urinastatin markedly decreased LE levels, with the decrease being more remarkable in patients showing high LE levels before treatment. These results suggest that urinastatin not only reduces LE levels but also suppresses factors that are involved in the development of septic shock.