Multicenter investigation of lifestyle-related diseases and visceral disorders in thalidomide embryopathy at around 50 years of age.

BACKGROUND: In utero exposure to thalidomide causes a wide range of birth defects, including phocomelia, hearing loss and visceral disorders, known as thalidomide embryopathy (TE). Fifty years after the first report of TE, we conducted the first cross-sectional multicenter study to investigate the development of lifestyle-related diseases and identify risk factors for visceral disorders in subjects with TE. METHODS: Seventy-six cases with TE (31 men, 45 women) underwent medical examinations between 2011 and 2014 to determine the types of TE-related anomalies (limbs, auditory organs, or visceral organs) and lifestyle-related diseases present. Logistic multiple regression analyses, adjusted for gender and age, were conducted between TE and lifestyle-related diseases and to evaluate association between block vertebra and gallbladder aplasia. RESULTS: Fatty liver (FL), nonalcoholic FL disease and dyslipidemia were detected in 52.6%, 35.0%, and 23.7% of subjects, respectively, with higher incidences among men. Dyslipidemia was detected in 40.0% of subjects with FL and was significantly associated with FL (odds ratio = 8.86; p = 0.008). Block vertebrae were detected in 44.4% of subjects with gallbladder aplasia, and this association was significant (odds ratio = 9.96; p = 0.006). CONCLUSION: Subjects with TE have also a risk for lifestyle-related disease as well as the general Japanese population. In addition, cervical spine radiography and magnetic resonance imaging are recommended to assess block vertebrae in subjects with TE with gallbladder aplasia who develop shoulder pain.

Measurement of blood pressure in a thalidomide-impaired patient who required ovarian cystectomy: A case report.

INTRODUCTION: Thalidomide was available for use over-the-counter between 1958 and 1962, and more than 300 thalidomide-impaired people have been confirmed in Japan. Currently, thalidomide-impaired people are nearing the age of 50 years and sometimes require medical treatment or surgery. However, a sphygmomanometer cannot be used to measure the blood pressure in some thalidomide-impaired people because of upper-limb shortening or hypoplastic defects. We encountered a patient with thalidomide-related upper limb defects who required abdominal ovarian cystectomy. PRESENTATION OF CASE: The patient was a 49-year-old woman (146.5cm, 35.9kg) with thalidomide-related upper-limb defects, but no dysplasia of the lower limbs, who underwent abdominal ovarian cystectomy. During the surgery, the patient's arterial blood pressure was monitored in her lower limbs by both non-invasive and invasive methods, and almost the same variations of the blood pressure between the invasive and non-invasive measurements were observed. DISCUSSION: Usually, blood pressure measurements are performed in a non-invasive manner in the upper limbs, however, such measurement could not be performed in the present case. There are few reports of measurement of the blood pressure or surgery under anaesthesia in thalidomide-impaired patients, and we report here that it was useful to measure the blood pressure in the lower limbs in the current patient. Invasive arterial pressure measurements showed almost the same changes as the non-invasive pressure measurements, although the systolic blood pressure was 10-20mmHg lower than the noninvasively measured systolic blood pressure. CONCLUSION: Non-invasive blood pressure measurements in the lower limbs might be useful in thalidomide-impaired patients requiring blood pressure monitoring, but further studies are required to validate this method.

Psychological and mental health problems in patients with thalidomide embryopathy in Japan.

AIM: The aim of the study was to examine the presence of psychological and mental health problems in patients with thalidomide embryopathy in Japan in order to develop and build future support systems. METHODS: The present study examined the presence/absence of electroencephalographic abnormalities, intellectual/cognitive functions, and mental health problems in 22 participants (nine men, 13 women) with thalidomide embryopathy. Participants completed the electroencephalograph instrument. Participants were also assessed using the Wechsler Adult Intelligence Scale-III; the Autism-Spectrum Quotient; the General Health Questionnaire-28, and the Mini-International Neuropsychiatric Interview. RESULTS: The results suggest the following: (i) electroencephalographic abnormality observed in several thalidomide embryopathy participants is unlikely to be the direct result of thalidomide; (ii) the cognitive functions of working memory and processing speed are lower in thalidomide embryopathy patients than in healthy individuals; and (iii) 40.9% of the thalidomide embryopathy participants have possible mental disorders, with more mental problems observed than in healthy individuals. CONCLUSIONS: Deterioration of mental health in patients with thalidomide embryopathy is indicated. Anxiety, insomnia, and physical symptoms were especially remarkable and may have resulted in restriction of social activities. Therefore, careful examination and active support of patients' psychological and mental problems is essential.

Value of carotid artery tenderness for the early diagnosis of Takayasu arteritis.

The early diagnosis and treatment of Takayasu arteritis (TA) is crucial to prevent the devastating complications of vascular insufficiency. This report describes a patient with a persistent fever in whom carotid artery tenderness led to a diagnosis of TA. This case suggests that carotid artery tenderness is a useful physical finding that warrants 2-deoxy-2-[Fluorine-18] fluoro-D-glucose positron emission tomography/computed tomography to confirm a diagnosis of early TA.

A case of yellow nail syndrome with dramatically improved nail discoloration by oral clarithromycin.

An 80-year-old woman was admitted to our hospital with pneumonia and exacerbation of sinobronchial syndrome (SBS). She presented with yellow discoloration of the nail beds of all fingers and toes, and her nails were recognized as growing slowly. Chest X-ray revealed bronchiectasis in the bilateral lower lobe and bilateral pleural effusion. We diagnosed her as having yellow nail syndrome (YNS), based on the triad of yellow nails, lymphedema, and lung disease. After treatment with antibiotics [ampicillin/sulbactam and clarithromycin (CAM)] for pneumonia and SBS, her general condition improved, and the yellow nails disappeared in some fingers. When she was previously treated with 200 mg CAM for SBS, her yellow nails had not shown improvement. This time, her yellow nails improved after treatment with 400 mg CAM. The literature reports vitamin E, zinc, and topical corticosteroid plus active vitamin D3 to be effective in the treatment of yellow nails. Two studies have reported treatment for YNS using CAM, though they found a lack of efficacy. Thus, the present case is the first to report improved yellow nails using CAM alone. We conclude that not only SBS and lung disease but also YNS were improved by treatment with 400 mg CAM.

[A survey of perioperative asthmatic attack among patients with bronchial asthma underwent general anesthesia].

BACKGROUND: We investigated the risk factor of perioperative asthmatic attack and effectiveness of preventing treatment for asthmatic attack before operation. METHODS: We performed retrospective chart review of one hundred eleven patients with asthma underwent general anesthesia and surgical intervention from January 2006 to October 2007 in our hospital. RESULTS: The rate of perioperative asthmatic attack were as follows; 10.2% (5 in 49 cases) in no pretreatment group, 7.5% (3 in 40 cases) in any pretreatments except for systemic steroid, and 4.5% (1 in 22 cases) in systemic steroid pretreatment group. Neither preoperative asthma severity nor duration from the last attack had significant relevancy to perioperative attack rate. The otolaryngological surgery, especially those have nasal polyp and oral surgery had high perioperative asthma attack rate, although there was no significant difference. CONCLUSION: We recommend the systemic steroid pretreatment for asthmatic patients, especially when they have known risk factor such as administration of the systemic steroid within 6 months, or possibly new risk factor such as nasal polyp, otolaryngological and oral surgery.

[Case of repeated hemoptysis successfully treated with bronchial artery embolization with N-butyl cyanoacrylate].

We report a case of repeated hemoptysis successfully treated with bronchial artery embolization (BAE) with N-butyl cyanoacrylate (NBCA). A 75-year-old woman with non-tuberculous mycobacteriosis and pulmonary aspergillosis was admitted with recurrent hemoptysis despite repeated BAE. Considering the ineffectiveness of BAE with Spongel or polyvinyl alcohol, BAE with NBCA was selected. Immediate cessation of hemoptysis was obtained and it has not been seen for 2 years. Although NBCA is the most widely used liquid embolic material to treat brain aneurysm, arteriovenous malformations or gastric varices, there are only a few cases are reported in the treatment of hemoptysis. It seems to be a possible useful treatment for patients with repeated hemoptysis.

[Clinical analysis on tuberculosis cases among foreigners in our hospital].

BACKGROUND: Although the incidence rate in Japan has been decreasing since the declaration of tuberculosis emergency in 1999, the reported tuberculosis cases among foreigners have been increasing year by year (from 5.1% in 2000 to 6% in 2003). As the number of foreign residents in Japan has been increasing every year, tuberculosis cases among them are also expected to increase. PURPOSE: The aim of this study is to investigate and clarify clinical features of recent tuberculosis patients among foreigners. OBJECT: Fifty-two cases were analyzed, who were admitted to our hospital because of active tuberculosis from January 2004 to April 2007. RESULTS: Among total 52 cases, male was 29, female 23, and the mean age (SD) of the patients was 31.8 (+/- 8.8) years old. Their mother countries were China, Republic of Korea and so on. The cavitary lesions were found on chest X-ray in 54%, the drug resistant rate was 8.2%, and the treatment completion rate was 92%. DISCUSSION & CONCLUSION: Comparing with reports in the past, almost parameters about tuberculosis control have improved, for example the drug resistant rate was decreased and the treatment completion rate was increased. The promotion of DOTS strategy in Japan might be attributed to the improvement of these parameters. Because more immigrants from the developing countries are expected in near future, not only strengthening current DOTS strategy but also new countermeasures such as QFT-2G and Electronic-Nose Technology should be introduced into tuberculosis control of foreigners living in Japan to decrease tuberculosis incidence and improve treatment outcome by early detection and adherence to treatment.

[Case of minocycline-induced pneumonitis with bilateral pleural effusion].

A 51-year-old man was admitted to our hospital with fever, dry cough and dyspnea. He had taken minocycline for 11 days because of urinary tract infection. Chest X-ray on admission showed diffuse reticular shadows in bilateral lung fields with bilateral pleural effusion. Cessation of minocycline led to spontaneous improvement of symptoms and radiographic findings. The lymphocyte stimulation test for minocycline with peripheral blood and pleural effusion were negative. After provocation test with minocycline, he developed fever and dry cough and bilateral ground glass opacity appeared on his chest X-ray. He was diagnosed as minocycline-induced pneumonitis and recovered rapidly following corticosteroid therapy.

Amiodarone pulmonary toxicity: a patient with three recurrences of pulmonary toxicity and consideration of the probable risk for relapse.

A 44-year-old man was treated with amiodarone for dilated cardiomyopathy. After 53 months, he developed amiodarone-induced interstitial pneumonia. Amiodarone treatment was terminated, and the patient was given corticosteroids. These treatments were effective. However, pneumonitis recurred whenever prednisolone was reduced to less than 5 mg per day. Considering the patient's background characteristics, we considered his body mass index (BMI, kg/m(2)) and found his to be high. When four additional patients with amiodarone pulmonary toxicity were reviewed at our institute, a correlation between BMI and the duration of shadow disappearance was found (R(2)=0.8695). Because amiodarone is lipophilic, the patient's high BMI might have influenced the repeated appearance of pulmonary toxicity.

[Case of cavitary coccidioidomycosis with fungus balls in the apices of both lungs].

Pulmonary cavitary coccidioidomycosis with a fungus ball was observed in a immunocompetent case. A 32-year-old Japanese man visited Arizona to play golf. After 1 month he consulted a local hospital complaining of a prolonged cough and hematopysis. The laboratory examination revealed eosinophillia and chest radiograph showed 2 cavitary lesions, surrounded by small nodules in the apices of both lungs. Pulmonary tuberculosis was suspected and treated with 4 antituberculosis drugs for 3 months. However, the cavities enlarged and he was admitted to our hospital for further examination and treatment. Transbronchial lung biopsy was performed and serologically, bacteriologically and histologically a diagnosis of chronic coccidioidmycosis was made. It is very rare for fungus ball formation and coexistence of spherules and hyphae of Coccidioides immitis to be seen. Fluconazole was temporarily effective, causing cavities to shrink and eosinophilia to decrease, however Amphotericin B needed to be used later. Eosinophilia was closely related to the severity of the disease gravity.

[Effects of new discharge criteria incorporating DOTS on treatment outcome of patients with smear-positive tuberculosis].

BACKGROUND: Some problems remain in the treatment of tuberculosis (TB) in Japan, with a higher prevalence of TB, low percentages of completed therapy and cases given DOTS, and longer admission period compared to the United States. We defined our own new criteria for discharge as sputum smear negativity instead of culture negativity, modified according to CDC criteria with shortened admission periods. However, the effects on treatment outcome have not been evaluated. OBJECTIVES: The aim of this study was to ensure the effectiveness of the new criteria, including DOTS undertaken after discharge. PATIENTS/METHODS: Group I comprised 459 cases hospitalized between January 2000 and December 2002 that were discharged under the old criteria, while Group II comprised 259 cases hospitalized between January 2003 and April 2004 that were discharged under the new criteria. We tried to undertake DOTS in cooperation with local health centers. The main outcome measures were admission period, treatment completion and relapse rates at 1 year after the completion of treatment. RESULTS: The new criteria enabled median admission period to be shortened from 84 days to 69 days, although patients in Group II were older and displayed more severe tuberculosis lesions compared to Group I. DOTS coverage rate increased significantly from 5.9% to 40.5%, and treatment completion rate, percentage of lost cases and relapse rate for completed cases at 1 year changed from 83.0% to 86.6%, 6.3% to 3.9%, and 2.5% to 2.5%, respectively. No significant differences in these 3 rates were noted between Groups I and II. CONCLUSION: The new criteria incorporating DOTS enabled shortened admission period without any adverse effect on treatment outcomes.

[Impact of the change in criteria for hospital discharge of patients with tuberculosis based on the duration of hospitalization].

OBJECTIVES: The criteria for discharge from TB ward was changed from sputum culture negative to smear negative since January 2003 and the duration of hospitalization and the applicability of each criteria were analyzed. METHODS: Cases hospitalized from Jan. to Dec. 2003 (Group I) were compared with those hospitalized from Jan. 2000 to Dec. 2002 (Group II). In group I, we investigated which criteria was applied for each case. RESULTS: Shifting criteria from culture negative to smear negative, the median duration of hospitalization was shortened from 83 to 70 days, in particular in 20s, 30s and 70s. No effect however were found in patients with initially high grade excreter of AFB or extensive cavitary lesions. Although 44% of patients were discharged according to smear conversion to negative, this criteria could not be applied in 17% due to some reasons. CONCLUSIONS: Conversion to culture negative has been conventionally used for discharge criteria in Japan considering not only contagiousness of the disease but to maintain patients adherence to treatment. After the introduction of new criteria to shorten the hospitalization periods, Japanese style DOTS after discharge would be more important to maintain patients adherence to treatment. We will continue to evaluate the treatment completion rate.

[Usefulness of directly observed therapy (DOT) during hospitalization as DOTS in Japanese style].

Many patients with smear positive tuberculosis were hospitalized during the initial phase of chemotherapy but DOT was not applied to patients with tuberculosis in Japan. We tried randomized clinical study to evaluate the usefulness of DOT during admission. 135 culture positive TB patients were administered by DOT and 124 culture positive TB patients were self-administered during admission. There was no significant difference between 2 groups in the clinical background factors, treatment and the incidence of adverse reactions. Treatment completion rate was 94.1% in DOT group and 87.9% in non-DOT group. Default rate was significantly lower in DOT group (4.4%) than in non-DOT group (11.3%). We concluded that "DOT during admission" was useful to improve the outcome of chemotherapy for tuberculosis and it is preferable to apply DOT throughout treatment course for patients with tuberculosis in Japan.

[A case of lymphomatoid granulomatosis with multiple thin-walled cavities].

We report a case of rapidly progressive lymphomatoid granulomatosis. A 48-year-old man was admitted because of hemoptysis and high fever. A chest radiograph and chest computed tomograms revealed multiple nodules, thin-walled cavities in the lungs, some containing ball-like masses. After admission, clinical studies, including percutaneous and transbronchial biopsies failed to provide sufficient evidence for proper diagnosis. Fluoro-deoxyglucose positron emission tomography (FDG-PET) showed strong accumulations of abnormal shadows in the lesions. A VATS biopsy was performed to make possible an exact diagnosis. The histological findings showed angiocentric lesions with infiltration of polymorphous cells, and were compatible with lymphomatoid granulomatosis (LYG). The respiratory failure progressed as the reticular shadows in the chest radiograph increased, so treatment with methylprednisolone pulse therapy (1,000 mg/day for 3 days) was started, and followed with prednisolone therapy (60 mg/day for 3 weeks). Initially, the symptoms improved, but gradually deteriorated, and the pulmonary nodules became enlarged. The patient finally died of progressive respiratory failure in addition to uncontrollable empyema with an MRSA infection. We attributed the cavity formation and ball-like masses in the cavities to the occlusion of small pulmonary arteries and tissue necrosis along the drainage bronchus.