No abstract available.
Gaucher Disease*

No abstract available.
Child* ; Fever* ; Humans ; Parents*

The pleomorphic adenoma is the most common neoplasm involving both the major and minor salivary glands. It is a benign, slowgrowing tumor, but local recurrences can occur. The pleomorphic adenoma gene 1 (PLAG1), which is a novel zinc finger gene, is frequently activated by reciprocal chromosomal translocations involving 8q12 in a subset of salivary gland pleomorphic adenomas. This experimental study was preformed to observe the translocation patterns between PLAG1 gene and the three translocation partner genes. We also have analyzed the presence of PLAG1 transcripts by RT-PCR. CTNNB1/PLAG1 gene fusion was observed in three of nine pleomorphic adnomas. However, LIFR/PLAG1 and SII/PLAG1 gene fusions were not detectable. All of three gene fusions was not detectable in one Warthin's tumor and three inflammatory salivary gland tissues. PLAG1 transcripts were expressed in all inflammatory salivary gland tissues and tumors except for three pleomorphic adenomas. Of particular one pleomorphic adenoma showing CTNNB1/P AG1 gene fusion did not express PLAG1 transcipt. Our data indicate that gene fusion involving PLAG1 is a frequent event in pleomorphic adenoma, but correlation between gene fusion involving PLAG1 and PLAG1 transcription is not definite.
Adenoma, Pleomorphic* ; Gene Fusion ; Recurrence ; Salivary Glands* ; Salivary Glands, Minor ; Translocation, Genetic ; Zinc Fingers

Sarcoidosis is a chronic granulomatous disease of unknown cause characterized by widespread occurence of epithelioic cell. The authors experienced a case of sarcoidosis in 34-year-old female who has lacrimal gland enlargement, severe subcutaneous nodules and bilateral granulomatous uveitis. Histopathologic examination of the subcutaneous nodule of lid revealed sarcoidosis. In the course of corticosteroid therapy, subcutaneous nodules and enlarged lacrimal gland subsided gradually.
Adult ; Female ; Granulomatous Disease, Chronic ; Humans ; Lacrimal Apparatus ; Sarcoidosis* ; Uveitis*

PURPOSE: This study was designed to provide the basic data for the development of the urinary incontinence nursing management program by identifying voiding patterns and pressure ulcer prevalence of residents in long term care facilities. MATERIALS AND METHODS: The study was performed from January to February, 2003. The subjects consisted of 92 residents in long term care facilities around S city. Researcher visited the facilities and assessed their voiding patterns, voiding management, and skin. To investigate the voiding patterns of the residents, care givers in the facilities were educated how to fill up voiding diaries and the diaries were made for two days. The data were analyzed by t-test and x2-test with using SAS program. RESULTS: The mean score for the activities of daily living of the subjects was 17.7+/-10.3 indicating medium. Mean score for mental status was 2.5+/-3.5 indicating very low. Among the subjects, self-voiding group was 40.8%, the group with self-voiding at daytime and diapers at night 10.9%, the group managed with diapers all the time 41.1%. In UI status of self-voiding group, urge urinary incontinence 22.8%, mixed urinary incontinence 13.6%, stress urinary incontinence 6.8%, and 56.8% had no urinary incontinence. About 9.8% of the subjects suffered from pressure ulcers and 15% of the group managed with diapers all the time had skin problems at perineal area. Between the group with pressure ulcer and the group without pressure ulcer, there were significant differences by length of residence, activities of daily living, mental status, and Braden scale. CONCLUSION: The above findings indicated that the care programs for the group managed with diaper change and the group with skin problems were required to improve, and the voiding management focusing on rehabilitation rather than simple diaper change should be encouraged.
Activities of Daily Living ; Caregivers ; Humans ; Long-Term Care* ; Nursing ; Pressure Ulcer* ; Prevalence ; Rehabilitation ; Skin ; Urinary Incontinence

Acute respiratory failure is an important risk factor for mortality in patients with acute pesticide poisoning. Therefore, it is necessary to investigate the risk factors to predict respiratory failure in these patients. This study retrospectively investigated the clinical features of respiratory failure among patients with acute pesticide poisoning requiring mechanical ventilation. This study included patients who were admitted with intentional poisoning by pesticide ingestion from January 2017 to December 2019. Paraquat intoxication was excluded. Among 469 patients with acute pesticide poisoning, 398 patients were enrolled in this study. The respiratory failure rate was 30.4%. The rate of respiratory failure according to the type of pesticide was carbamate (75.0%), organophosphate (52.6%), glufosinate (52.1%), glyphosate (23%), pyrethroid (8.9%), and others (17%). The mortality was 25.6% in the respiratory failure group. The risk factors for respiratory failure were old age, low body mass index, and ingestion of more than 300 mL. In conclusion, respiratory failure is a risk factor for mortality in pesticide poisoning. Old age, low body mass index, and ingestion of more than 300 mL are the risk factors for predicting respiratory failure.

Acute respiratory failure is an important risk factor for mortality in patients with acute pesticide poisoning. Therefore, it is necessary to investigate the risk factors to predict respiratory failure in these patients. This study retrospectively investigated the clinical features of respiratory failure among patients with acute pesticide poisoning requiring mechanical ventilation. This study included patients who were admitted with intentional poisoning by pesticide ingestion from January 2017 to December 2019. Paraquat intoxication was excluded. Among 469 patients with acute pesticide poisoning, 398 patients were enrolled in this study. The respiratory failure rate was 30.4%. The rate of respiratory failure according to the type of pesticide was carbamate (75.0%), organophosphate (52.6%), glufosinate (52.1%), glyphosate (23%), pyrethroid (8.9%), and others (17%). The mortality was 25.6% in the respiratory failure group. The risk factors for respiratory failure were old age, low body mass index, and ingestion of more than 300 mL. In conclusion, respiratory failure is a risk factor for mortality in pesticide poisoning. Old age, low body mass index, and ingestion of more than 300 mL are the risk factors for predicting respiratory failure.

Sjogren syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in dry mouth and eyes. Approximately one-third of patients present with systemic manifestations, but respiratory muscle involvements have been rarely reported. We report a case of acute respiratory failure complicated by primary Sjogren syndrome. Muscle biopsy revealed perivascular lymphocytic infiltrations. Corticosteroid therapy improved respiratory muscle weakness. Sjogren syndrome should be considered as one of the underlying diseases causing acute respiratory failure.
Autoimmune Diseases ; Biopsy ; Exocrine Glands ; Humans ; Mouth ; Muscular Diseases ; Respiratory Insufficiency ; Respiratory Muscles* ; Respiratory Paralysis ; Sjogren's Syndrome*

An increased pericoronary fat attenuation index (FAI) on computed tomography angiography (CTA) is associated with increased all-cause and cardiac mortality in the general population. However, the ability of pericoronary FAI to predict long-term outcomes in chronic kidney disease (CKD) patients is unknown. Methods: In this single-center retrospective longitudinal cohort study, we assessed the utility of CTA-based pericoronary FAI measurement to predict mortality of CKD patients, including those with end-stage renal disease (ESRD). Mapping and analysis of pericoronary FAI involved three major proximal coronary arteries. The prognostic value of pericoronary FAI for long-term mortality was assessed with multivariable Cox regression models. Results: Among 268 CKD participants who underwent coronary CTA, 209 participants with left anterior descending artery (LAD) FAI measurements were included. The pericoronary FAI measured at the LAD was not significantly associated with adjusted risk of allcause mortality (hazard ratio [HR], 2.08; 95% confidence interval [CI], 0.94–3.51) in any CKD group. However, ESRD patients with elevated pericoronary FAI values had a greater adjusted risk of all-cause mortality compared with the low-FAI group (HR, 2.26; 95% CI, 1.11–4.61). Conclusion: The pericoronary FAI measured at the LAD predicted long-term mortality in patients with ESRD, which could provide an opportunity for early primary intervention in ESRD patients.

Primary aldosteronism is characterized by hypokalemic metabolic alkalosis, low plasma renin activity, elevated plasma aldosterone level and can be suspected in the patients with hypertension and unexplained hypokalemia. Small adrenal cortical adenomas are responsible for this syndrome in most cases. The incidence of thyrotoxic periodic paralysis ranges from 1.9 to 6.2 % in Japan. Thyrotoxic periodic paralysis usually subsides following treatment of hyperthyroidism and has good prognosis. A 56 year-old man presented with hyperthyroidism, hypertension and recurrent hypokalemia. During the treatment of hyperthyroidism, he repeatedly experienced weakness of both lower extremities. Hormonal evaluation was performed and he was found to have a 2*2*1.5 cm sized right adrenal tumor by abdominal computerized topography(CT). After right adrenalectomy, hypokalemic periodic paralysis was improved. Both thyroid and adrenal function should be comprehensively investigated in periodic paralysis. In conclusion, physicians must be aware of the possibility of primary aldosteronism in hyperthyroid patients with hypokalemic periodic paralysis. We report a case of aldosteronoma complicated with hyperthyroidism and literatures are reviewed.
Adrenalectomy ; Adrenocortical Adenoma ; Aldosterone ; Alkalosis ; Humans ; Hyperaldosteronism ; Hypertension ; Hyperthyroidism* ; Hypokalemia ; Hypokalemic Periodic Paralysis ; Incidence ; Japan ; Lower Extremity ; Middle Aged ; Paralysis ; Plasma ; Prognosis ; Renin ; Thyroid Gland