The development of cutaneous sarcoidosis as a paradoxical adverse event of tumor necrosis factor alpha (TNF-α) blockers has been reported in the literature; however, an erythrodermic form of cutaneous sarcoidosis during anti-TNF-α therapy has not yet been reported. Herein, we report the first case of an erythrodermic form of cutaneous sarcoidosis during anti-TNF-α therapy and review previous studies of cutaneous sarcoidosis. A 6-year-old Korean girl who had been suffering from juvenile rheumatoid arthritis presented with generalized erythematous skin eruption involving more than about 90% of her body surface area. After 14 months of etanercept treatment, the new erythematous skin eruption had developed and progressed into generalized erythroderma. Exclusion of suspected co-medication had been performed based on medication history. She had no other systemic symptoms, and ophthalmologic and neurologic examinations were normal. Histopathologic findings of the skin lesion revealed diffuse non-caseating granulomatous infiltrates composed of epithelioid histiocytes with sparse lymphocytes involving the entire dermis. Periodic-acid-Schiff and acid-fast stains were negative, and acid-fast bacilli was not detected by polymerase chain reaction of the skin biopsy. Based on clinicopathologic findings, she was diagnosed with etanercept-induced sarcoidal granuloma. After discontinuation of the suspected agent, the lesions spontaneously disappeared.
Arthritis, Juvenile* ; Biopsy ; Body Surface Area ; Child ; Coloring Agents ; Dermatitis, Exfoliative ; Dermis ; Etanercept ; Female ; Granuloma ; Histiocytes ; Humans ; Lymphocytes ; Neurologic Examination ; Polymerase Chain Reaction ; Sarcoidosis* ; Skin ; Tumor Necrosis Factor-alpha*

The development of cutaneous sarcoidosis as a paradoxical adverse event of tumor necrosis factor alpha (TNF-α) blockers has been reported in the literature; however, an erythrodermic form of cutaneous sarcoidosis during anti-TNF-α therapy has not yet been reported. Herein, we report the first case of an erythrodermic form of cutaneous sarcoidosis during anti-TNF-α therapy and review previous studies of cutaneous sarcoidosis. A 6-year-old Korean girl who had been suffering from juvenile rheumatoid arthritis presented with generalized erythematous skin eruption involving more than about 90% of her body surface area. After 14 months of etanercept treatment, the new erythematous skin eruption had developed and progressed into generalized erythroderma. Exclusion of suspected co-medication had been performed based on medication history. She had no other systemic symptoms, and ophthalmologic and neurologic examinations were normal. Histopathologic findings of the skin lesion revealed diffuse non-caseating granulomatous infiltrates composed of epithelioid histiocytes with sparse lymphocytes involving the entire dermis. Periodic-acid-Schiff and acid-fast stains were negative, and acid-fast bacilli was not detected by polymerase chain reaction of the skin biopsy. Based on clinicopathologic findings, she was diagnosed with etanercept-induced sarcoidal granuloma. After discontinuation of the suspected agent, the lesions spontaneously disappeared.
Arthritis, Juvenile* ; Biopsy ; Body Surface Area ; Child ; Coloring Agents ; Dermatitis, Exfoliative ; Dermis ; Etanercept ; Female ; Granuloma ; Histiocytes ; Humans ; Lymphocytes ; Neurologic Examination ; Polymerase Chain Reaction ; Sarcoidosis* ; Skin ; Tumor Necrosis Factor-alpha*

PURPOSE: To compare the effectiveness of device closure and medical therapy in prevention of recurrent embolic event in the Korean population with cryptogenic stroke and patent foramen ovale (PFO). MATERIALS AND METHODS: Consecutive 164 patients (men: 126 patients, mean age: 48.1 years, closure group: 72 patients, medical group: 92 patients) were enrolled. The primary end point was a composite of death, stroke, transient ischemic attack (TIA), or peripheral embolism. RESULTS: Baseline characteristics were similar in the two groups, except age, which was higher in the medical group (45.3±9.8 vs. 50.2±6.1, p<0.0001), and risk of paradoxical embolism score, which was higher in the closure group (6.2±1.6 vs. 5.7±1.3, p=0.026). On echocardiography, large right-to-left shunt (81.9% vs. 63.0%, p=0.009) and shunt at rest/septal hypermobility (61.1% vs. 23.9%, p<0.0001) were more common in the closure group. The device was successfully implanted in 71 (98.6%) patients. The primary end point occurred in 2 patients (2 TIA, 2.8%) in the closure group and in 2 (1 death, 1 stroke, 2.2%) in the medical group. Event-free survival rate did not differ between the two groups. CONCLUSION: Compared to medical therapy, device closure of PFO in patients with cryptogenic stroke did not show difference in reduction of recurrent embolic events in the real world's setting. However, considering high risk of echocardiographic findings in the closure group, further investigation of the role of PFO closure in the Asian population is needed.
Adult ; Aged ; Aged, 80 and over ; Cardiac Catheterization/adverse effects ; Disease-Free Survival ; Embolism/etiology/*prevention & control ; Female ; Fibrinolytic Agents/adverse effects/*therapeutic use ; Foramen Ovale, Patent/complications/*drug therapy/mortality/*surgery ; Humans ; Ischemic Attack, Transient/*drug therapy/mortality/*surgery ; Male ; Middle Aged ; Republic of Korea/epidemiology ; Risk ; Secondary Prevention/methods ; *Septal Occluder Device/adverse effects ; Stroke/etiology/prevention & control ; Treatment Outcome

Striatal-enriched protein tyrosine phosphatase (STEP) is abundantly expressed in the striatum, which strongly expresses dopamine and opioid receptors and mediates the effects of many drugs of abuse. However, little is known about the role of STEP in opioid receptor function. In the present study, we generated STEP-targeted mice carrying a nonsense mutation (C230X) in the kinase interaction domain of STEP by screening the N-ethyl-N-nitrosourea (ENU)-driven mutant mouse genomic DNA library and subsequent in vitro fertilization. It was confirmed that the C230X nonsense mutation completely abolished functional STEP protein expression in the brain. STEP(C230X−/−) mice showed attenuated acute morphine-induced psychomotor activity and withdrawal symptoms, whereas morphine-induced analgesia, tolerance and reward behaviors were unaffected. STEP(C230X−/−) mice displayed reduced hyperlocomotion in response to intrastriatal injection of the μ-opioid receptor agonist DAMGO, but the behavioral responses to δ- and κ-opioid receptor agonists remained intact. These results suggest that STEP has a key role in the regulation of psychomotor action and physical dependency to morphine. These data suggest that STEP inhibition may be a critical target for the treatment of withdrawal symptoms associated with morphine.
Analgesia* ; Animals ; Brain ; Codon, Nonsense ; Dopamine ; Enkephalin, Ala(2)-MePhe(4)-Gly(5)- ; Ethylnitrosourea ; Fertilization in Vitro ; Gene Library ; Mass Screening ; Mice ; Morphine* ; Phosphotransferases ; Protein Tyrosine Phosphatases ; Receptors, Opioid ; Reward* ; Street Drugs ; Substance Withdrawal Syndrome*

Adrenocortical carcinoma (ACC) in pediatric and adolescent patients is rare, and it is associated with various clinical symptoms. We introduce the case of an 8-year-old boy with ACC who presented with peripheral precocious puberty at his first visit. He displayed penis enlargement with pubic hair and facial acne. His serum adrenal androgen levels were elevated, and abdominal computed tomography revealed a right suprarenal mass. After complete surgical resection, the histological diagnosis was ACC. Two months after surgical removal of the mass, he subsequently developed central precocious puberty. He was treated with a gonadotropin-releasing hormone agonist to delay further pubertal progression. In patients with functioning ACC and surgical removal, clinical follow-up and hormonal marker examination for the secondary effects of excessive hormone secretion may be a useful option at least every 2 or 3 months after surgery.
Acne Vulgaris ; Adolescent ; Adrenocortical Carcinoma* ; Child ; Diagnosis ; Follow-Up Studies ; Gonadotropin-Releasing Hormone ; Hair ; Humans ; Male ; Penis ; Puberty, Precocious* ; Virilism

OBJECTIVE: To determine the in vivo efficiency of monopolar radiofrequency ablation (RFA) using a dual-switching (DS) system and a separable clustered (SC) electrode to create coagulation in swine liver. MATERIALS AND METHODS: Thirty-three ablation zones were created in nine pigs using a DS system and an SC electrode in the switching monopolar mode. The pigs were divided into two groups for two experiments: 1) preliminary experiments (n = 3) to identify the optimal inter-electrode distances (IEDs) for dual-switching monopolar (DSM)-RFA, and 2) main experiments (n = 6) to compare the in vivo efficiency of DSM-RFA with that of a single-switching monopolar (SSM)-RFA. RF energy was alternatively applied to one of the three electrodes (SSM-RFA) or concurrently applied to a pair of electrodes (DSM-RFA) for 12 minutes in in vivo porcine livers. The delivered RFA energy and the shapes and dimensions of the coagulation areas were compared between the two groups. RESULTS: No pig died during RFA. The ideal IEDs for creating round or oval coagulation area using the DSM-RFA were 2.0 and 2.5 cm. DSM-RFA allowed more efficient RF energy delivery than SSM-RFA at the given time (23.0 +/- 4.0 kcal vs. 16.92 +/- 2.0 kcal, respectively; p = 0.0005). DSM-RFA created a significantly larger coagulation volume than SSM-RFA (40.4 +/- 16.4 cm3 vs. 20.8 +/- 10.7 cm3; p < 0.001). Both groups showed similar circularity of the ablation zones (p = 0.29). CONCLUSION: Dual-switching monopolar-radiofrequency ablation using an SC electrode is feasible and can create larger ablation zones than SSM-RFA as it allows more RF energy delivery at a given time.
Animals ; Catheter Ablation/*instrumentation/*methods ; *Electrodes ; Feasibility Studies ; Liver/*surgery ; Male ; Sus scrofa ; Time Factors

BACKGROUND: With advances in immunosuppression, graft and patient survival rates have increased significantly, but acute cellular rejection remains an important problem following liver transplantation (LT), and late acute rejection (LAR) occurs in a small percentage of recipients. Some risk factors for LAR have been identified, yet the cause of LAR has not been completely investigated. The efficacy of mycophenolate mofetil (MMF) administered in combination with calcineurin inhibitor (CNI) for reduction of LAR has been demonstrated. METHODS: Between January 2006 and August 2007, adult LT recipients (n=309) were enrolled in this study. Biopsy-proven acute rejection that occurred >6 months after LT was defined as LAR. The immunosuppression regimens, CNI or CNI plus MMF, were used continuously for at least 6 months after LT. The mean follow-up period was 34.8 months (range, 25~46 months). RESULTS: LAR occurred in 17 cases (5.5%). The incidence of LAR in the CNI (n=138) or CNI plus MMF groups (n=171) was 8.6% (n=12) and 2.9% (n=5), respectively (P=0.015). Multivariate Cox regression confirmed that CNI plus MMF versus CNI therapy is associated with a decreased risk of LAR (relative risk, 0.33; P=0.04). CONCLUSIONS: The incidence of LAR in the CNI plus MMF group was significantly lower than the CNI group. Thus, continuous use of CNI plus MMF may represent a better immunosuppression regimen to decrease the rate of LAR in LT recipients.
Adult ; Calcineurin ; Follow-Up Studies ; Humans ; Immunosuppression ; Incidence ; Liver ; Liver Transplantation ; Mycophenolic Acid ; Rejection (Psychology) ; Risk Factors ; Survival Rate ; Transplants

PURPOSE: We investigated the causative organism and its antibiotic susceptibility of community acquired urinary tract infection (UTI) in children at a secondary hospital to test the adequacy of the current guidelines. METHODS: Children diagnosed with UTI at the Department of Pediatrics, Kwandong University Myongji Hospital by pyuria and bacterial growth of greater than 1.0x10(5) CFU/mL on clean catch midstream urine from January 2005 to December 2008 were studied retrospectively. The epidemiologic data, causative organism, and the antibiotic susceptibility were analyzed. RESULTS: Sixty two children were diagnosed with sixty four cases of UTI's. Two bacteria were isolated in one case and thus data on 65 urine cultures were analyzed. The male:female ratio was 1.6:1 and 78.1% were less than 12 months of age. Escherichia coli was the predominant cause consisting of 53 cases (82.8%) of the cases. K. pneumoniae (5), Enterobacter (4), Enterococcus (1), beta-streptococcus (1), Diphtheroides (1) were isolated. The antibiotic resistance of E. coli were as follows; ampicillin 69.8%, cefotaxime 1.9%, gentamicin 15.1%, amikacin 0.0%, levofloxacin 1.9%, and trimethoprim/sulfamethoxazole 26.4%. Only one case of the E. coli was extended spectrum beta-lactamase (ESBL) positive. CONCLUSION: Compared to prior reports from other tertiary hospitals in Korea, E. coli was the predominant cause in childhood UTI and the rate of ESBL positivity was low. The antibiotic resistance was also different compared to prior reports. We conclude that a difference in the cause and antibiotic resistance of childhood UTI exists between centers and this should be taken into consideration when prescribing antibiotics for childhood UTIs.
Amikacin ; Ampicillin ; Anti-Bacterial Agents ; Bacteria ; beta-Lactamases ; Cefotaxime ; Child ; Drug Resistance, Microbial ; Enterobacter ; Enterococcus ; Escherichia coli ; Gentamicins ; Humans ; Korea ; Ofloxacin ; Pediatrics ; Pneumonia ; Pyuria ; Retrospective Studies ; Tertiary Care Centers ; Urinary Tract ; Urinary Tract Infections

Glycogen storage disease(GSD) type Ia is an autosomal recessive disease, caused by the absence or deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestinal mucosa. Glucose-6-phosphatase is an essential enzyme necessary for gluconeogenesis and glycogenolysis. GSD type Ia is characterized by hypoglycemia, lactic acidosis, hepatomegaly, seizures, doll-like faces with fat cheeks, thin extremities, short stature, protuberant abdomen, easy bruising and epistaxis, delayed puberty, early gout, pancreatitis, kidney stone, and other metabolic derangements such as hyperlipidemia. The most important complications of GSD-Ia are focal segmental glomerulosclerosis and hepatic adenomas. Various mutations have been reported. The most common mutation sites are g727t, G122D, and T255I and also P178A and Y128X muations have been reported. We experienced a female patient showing typical clinical characteristics, laboratory findings such as hypoglycemia, hyperuricemia, and hyperlipidemia, and g727t mutation confirmed by DNA analysis. We present this case with a brief review of related articles
Abdomen ; Acidosis, Lactic ; Adenoma ; Cheek ; DNA ; Epistaxis ; Extremities ; Female ; Glomerulosclerosis, Focal Segmental ; Gluconeogenesis ; Glucose-6-Phosphatase ; Glycogen ; Glycogen Storage Disease ; Glycogen Storage Disease Type I ; Glycogenolysis ; Gout ; Hepatomegaly ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Kidney Calculi ; Liver ; Pancreatitis ; Puberty, Delayed ; Seizures

BACKGROUND: Severe graft dysfunction has been occasionally encountered following adult living donor liver transplantation (LDLT). This study intended to assess the effectiveness of plasmapheresis (PP) as a liver supportive measure in LDLT recipients showing severe graft dysfunction. METHODS: During 1 year of 2007, 276 adult LDLTs were performed in our institution. Of them 27 underwent PP therapy as a liver support. RESULTS: Seventeen underwent PP during the first month following LDLT and another 10 underwent PP after that period. The underlying causes of such liver support were acute and chronic rejections, ischemic damage, viral hepatitis recurrence and unknown causes. A total of 329 sessions of PP were performed for these 27 patients, indicating 12.2+/-9.9 times per patient for 28.1+/-32.2 days. Concurrent hemodiafiltration was done in 66.7%. Serum total bilirubin level was significantly reduced following PP therapy: 23.2+/-6.5 mg/dL before PP and 14.4+/-5.6 mg/dL at 1 week after completion of PP (P<0.001). Other biochemical parameters did not significantly affected by PP. Overall 1-year patient survival rate was 63.0%. Six-month graft survival rate after completion of PP was 82.6% in 17 patients undergoing PP during the first posttransplant month and 30% in 10 patients undergoing PP after 1 month (P= 0.013). CONCLUSIONS: The results of this study implicate that PP has a beneficial effect on the recovery of liver graft function, especially during the early posttransplant period. We suggest to perform active application of PP therapy for liver recipients showing severe graft dysfunction of total bilirubin greater than 15~20 mg/dL.
Adult ; Bilirubin ; Graft Survival ; Hemodiafiltration ; Hepatitis ; Humans ; Liver ; Liver Transplantation ; Living Donors ; Plasmapheresis ; Recurrence ; Rejection (Psychology) ; Survival Rate ; Transplants