Sjogren's syndrome is a hererogenous autoimmune disease characterized by progressive destruction of the exocrine glands and accompanied by a variety of autoimmune phenomena. Sjogren's syndrome patients can develop symptoms of ocular and oral dryness as well as extraglandular complications including central and peripheral nervous system disease. Sometimes neuropathy precedes the diagnosis of Sjogren's syndrome. Adiets syndrome is characterized by tonic pupil and the absence of tendon reflex. Sweating abnormality and chronic peripheral polyneuropathy can also be present. We report a case of primary Sjogren's syndrome preceded by Adie's syndrome with peripheral neuropathy. A 26-year-old woman was admitted for photophobia and paresthesia. On examination, her pupils were anisocoric and did not react to light but constricted promptly to pilocarpin. Sensation decreased on her left side of body and deep' tendon reflexes were absent. Biopsy of minor salivary gland demonstrated infiltration by lymphocyte consistent with Sjogren's syndrome, but Schirmer test was negative. So she was diagnosed as Adie's syndrome with peripheral neuropathy. Five month later she complained of dry eye and dizziness. Rose bengal staining was positive. Sjogren's syndrome was diagnosed and she was discharged with local therapy for the sicca symptoms.
Adie Syndrome* ; Adult ; Autoimmune Diseases ; Biopsy ; Diagnosis ; Dizziness ; Exocrine Glands ; Female ; Humans ; Lymphocytes ; Paresthesia ; Peripheral Nervous System Diseases ; Photophobia ; Polyneuropathies ; Pupil ; Reflex, Stretch ; Rose Bengal ; Salivary Glands, Minor ; Sensation ; Sjogren's Syndrome* ; Sweat ; Sweating ; Tonic Pupil

The aim of the present study was to investigate the pathophysiological etiology of osteoarthritis that is mediated by the apoptosis of chondrocytes exposed to 25-hydroxycholesterol (25-HC), an oxysterol synthesized by the expression of cholesterol-25-hydroxylase (CH25H) under inflammatory conditions. Interleukin-1β induced the apoptosis of chondrocytes in a dose- dependent manner. Furthermore, the production of 25-HC increased in the chondrocytes treated with interleukin-1β through the expression of CH25H. 25-HC decreased the viability of chondrocytes. Chondrocytes with condensed nucleus and apoptotic populations increased by 25- HC. Moreover, the activity and expression of caspase-3 were increased by the death ligand-mediated extrinsic and mitochondria-dependent intrinsic apoptotic pathways in the chondrocytes treated with 25-HC. Finally, 25-HC induced not only caspasedependent apoptosis, but also induced proteoglycan loss in articular cartilage ex vivo cultured rat knee joints. These data indicate that 25-HC may act as a metabolic pathophysiological factor in osteoarthritis that is mediated by progressive chondrocyte death in the articular cartilage with inflammatory condition.

The aim of the present study was to investigate the pathophysiological etiology of osteoarthritis that is mediated by the apoptosis of chondrocytes exposed to 25-hydroxycholesterol (25-HC), an oxysterol synthesized by the expression of cholesterol-25-hydroxylase (CH25H) under inflammatory conditions. Interleukin-1β induced the apoptosis of chondrocytes in a dose- dependent manner. Furthermore, the production of 25-HC increased in the chondrocytes treated with interleukin-1β through the expression of CH25H. 25-HC decreased the viability of chondrocytes. Chondrocytes with condensed nucleus and apoptotic populations increased by 25- HC. Moreover, the activity and expression of caspase-3 were increased by the death ligand-mediated extrinsic and mitochondria-dependent intrinsic apoptotic pathways in the chondrocytes treated with 25-HC. Finally, 25-HC induced not only caspasedependent apoptosis, but also induced proteoglycan loss in articular cartilage ex vivo cultured rat knee joints. These data indicate that 25-HC may act as a metabolic pathophysiological factor in osteoarthritis that is mediated by progressive chondrocyte death in the articular cartilage with inflammatory condition.

PURPOSE: In Korea, pregnancy termination is frequently reported among women who took medications for an acute or chronic disease during pregnancy, for fear of teratogenic risk. We have previously shown that a service providing evidence-based information is helpful for women who week counseling to make a rational decision regarding their pregnancies. This study aimed to evaluate whether termination of pregnancy based on such perceptions, is justified using the 'DRug Exposure and risk Assessment in Moms' (DREAM) registry. METHODS: The study included 5,032 consenting pregnant women from the clinic and call center at the Korean Motherisk Program, from November 1999 to October 2008. The DREAM registry recorded the pregnancy outcomes (preterm birth, low birth weight, intrauterine fetal death, and congenital anomaly) of 3,328 women. RESULTS: Among women exposed to medications, time of exposure ranged from 3.5-4.6 weeks of gestation. There were 1,308 different drugs prescribed to these women. The drug most frequently prescribed was acetaminophen followed by chlorpheniramine maleate, and pseudoephedrine. There were 4.7% (n=156/3,328) women who underwent a voluntary abortion for fear of birth defects. We compared frequency of birth defects between exposed women and unexposed pregnant women in our institution during gestation. The frequency of major congenital malformations was 2.5% (n=74/2,977) in exposed group and 2.9% (n=75/2,573) in unexposed group (P=0.32). There was no statistically significant difference between exposed and control group in the rate of preterm births, intrauterine fetal death and low-birth weight babies. CONCLUSION: We did not observe increased risk of congenital malformations and adverse pregnancy outcomes in a population of pregnant women exposed to a variety of medications. Therefore these medications are not considered teratogen.
Acetaminophen ; Chlorpheniramine ; Chronic Disease ; Congenital Abnormalities ; Counseling ; Female ; Fetal Death ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Korea ; Maleates ; Parturition ; Pregnancy ; Pregnancy Outcome ; Pregnant Women ; Premature Birth ; Pseudoephedrine ; Risk Assessment