Camptodactyly, Arthropathy, Coxa vara, Pericarditis (CACP) Syndrome: A Case Report

The camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is characterized by congenital or early-onset camptodactyly, childhood-onset noninflammatory arthropathy associated with synovial hyperplasia. Some patients have pro-gressive coxa vara deformity and/or noninflammatory pericardial effusion. CACP is inherited as an autosomal recessive mode and the disease gene is assigned to a 1.9-cM interval on human chromosome 1q25-31. We describe a 10-yr-old boy who has typical features of CACP without familial association.

The efficacy and safety of etanercept in patients with active rheumatoid arthritis receiving methotrexate / 대한내과학회지

BACKGROUND: This study was performed to investigate the efficacy and safety of etanercept in active rheumatoid arthritis patients with stable dose of methotrexate in Korean. METHODS: In a 12 week, single arm, open trial, we assigned 76 patients with active rheumatoid arthritis who had an inadequate response to disease-modifying antirheumatic drugs. Patients received twice-weekly subcutaneous injections of etanercept 25 mg while continuing to receive methotrexate at a stable dose of 7.5~25 mg per week. The clinical response was defined as the percent improvement in disease activity according to the criteria of the American College of Rheumatology (ACR) at 12 weeks. RESULTS: Etanercept led to significant improvements in disease activity and was safe and well tolerated. At 12 week, 84.4% of the patients receiving 25 mg of etanercept achieved a 20% ACR response, and 53.1% of those receiving etanercept achieved a 50% ACR response. The most common adverse event was injection-site reaction. Other adverse events were upper respiratory infection, nausea, and facial edema, but there were no serious adverse events associated with etanercept. CONCLUSION: In active rheumatoid arthritis patients, etanercept was safe, well tolerated, and provided rapid clinical improvements.

Acute Monoarthritis Accomanying with Herpes Zoster Skin Eruption / 대한류마티스학회지

No abstract available.

Results of One level Anterior Cervical Discectomy and Fusion in the Degenerative Cervical Spinal Disorder: Comparison of 3 Arthrodesis Techniques / 대한정형외과학회잡지

PURPOSE: This study was designed to compare the outcome of anterior cervical discectomy and fusion (ACDF) with autogenous bone graft, ACDF with plating, and ACDF with carbon cage and plating for the surgical treatment of single-level degenerative cervical spine disorder. MATERIALS AND METHODS: We analyzed 60 cases who received single level ACDF for the treatment of degenerative cervical disorder with at least one year follow-up. Group A consisted of 21 patients treated by ACDF and autogenous bone graft. Group B consisted of 20 patients treated by ACDF and plate fixation, and group C consisted of 19 patients treated by Carbon cage and plate fixation. We compared 3 groups in terms of fusion status, change in Cobb angle and disc height, and clinical results using Odom method. RESULTS: The change of Cobb angle was -3.8 degreesin group A, -0.62 degreesin group B and 0.6 degreesin group C (p=0.042). The decreased disc height was 2.8 mm in group A, 1.4 mm in group B and 0.4 mm in group C respectively (p=0.001). In group A, the clinical results were excellent in 3 cases, good in 16 cases and fair in 2 cases. In group B, 4 cases were excellent, 13 good and 3 fair. And in group C, 5 cases were excellent and 14 good. CONCLUSION: ACDF with carbon cage and plate fixation proved an excellent surgical alternative that provided initial stability, maintained disc height and lordotic angle, and was accompanied by low donor site morbidity and reduced operation time for the treatment of single level degenerative cervical spinal disorder.

Predictable Factors for Paradoxical Reactions in Conscious Sedation with Midazolam During ERCP / 대한소화기내시경학회지

BACKGROUND/AIMS: Paradoxical reaction after midazolam administration is relatively uncommon and can obstruct the performance of ERCP. But it can not be predicted before drug administration. We investigate the difference in occurrence of paradoxical reaction according to personal characteristics and clinical status of patients. METHODS: During 155 ERCP procedures, we injected midazolam and meperidine intravenously for conscious sedation until deep sleep occurred. Among 155 patients, 108 patients did not showed paradoxical reaction (group I) and 47 patients (30.3%) showed gross behavioral disturbance and/or agitation (group II). Paradoxical agitation was seen in 9 (7.1%) procedures. RESULTS: Type A-like personality (p=0.002), sleep-talking habit (p=0.026) and presence of pain at the beginning of ERCP (p=0.036) and during ERCP (p=0.021) were seen more frequently in group II. Duration of ERCP was longer (p=0.034) and dosage of midazolam was larger (p=0.009) in group II. In multivariate analysis, having sleep-talking (OR, 5.5), type A-like personality (OR 3.9) and dosage of midazolam (OR 1.3) were risk factors of paradoxical reaction. CONCLUSIONS: Paradoxical agitation after midazolam administration was uncommon and can be managed with flumazenil. Paradoxical reaction can be predicted more often in patients with type A-like personality, sleep-talking habit, complaining pain before ERCP, and in patients injected large dosage of midazolam.

Risk Factors for Early Rebleeding after Initial Endoscopic Hemostasis in Patients with Bleeding Peptic Ulcers / 대한소화기내시경학회지

BACKGROUND/AIMS: Rebleeding rate after initial endoscopic hemostasis in patients with ulcer hemorrhage has been reported in 20-30%. Identification of patients who are at high risk for rebleeding would be expected to improve the outcome of endoscopic hemostasis. The purpose of this study was to evaluate risk factors for early rebleeding after initial hemostasis in the view of clinical and endoscopic characteristics. METHODS: We reviewed 99 patients who presented with bleeding peptic ulcers and were treated with endoscopic hemostasis including hypertonic saline injection, electrocautery and clipping. We compared the clinical variables (age, pulse rate, hemoglobin), endoscopic characteristics of ulcer (size, number, and location of ulcer, clots on the base, bleeding stigmata, size and color of exposed vessel) between the patients who bled early (n=22) and who didn't bleed (n=77) within 5 days. RESULTS: The statistically significant correlates with early rebleeding after hemostasis were number of comorbid illness (> or =2) (p=0.031), volume of transfusion (> or5 units) (p=0.001), size of ulcer (>1 cm) (p=0.038), multiple ulcers (p=0.020), presence of blood clots on ulcer base (p=0.012), stigmata (active bleeding and visible vessels) (p=0.010), size of exposed vessel (>1 mm) (p<0.0001). In multivariate analysis, volume of transfusion (odds ratio[OR] 14.4), size of ulcer (OR 11.7), multiple ulcers (OR 5.5) and size of exposed vessel (OR 13.2) were significant risk factors. CONCLUSIONS: The risk factors for early rebleeding after hemostasis in bleeding peptic ulcer can be predicted by clinical variables and endoscopic findings. Early identification of risk factors such as transfusion over 5 units, large-sized ulcer, multiple ulcers, bleeding stigmata and size of exposed vessel over 1 mm can predict the prognosis of peptic ulcer bleeding.

A Case of Gastroduodenal Crohn's Disease Mistaken for a Gastric Malignancy / 대한소화기내시경학회지

Gastroduodenal Crohn's Disease (CD), which is uncommon in adults, involves the gastric antrum and proximal duodenum in most cases, and results in epigastric pain, weight loss, and gastric outlet obstruction, raising initial suspicion that it may be peptic ulcer disease or a malignancy. On upper endoscopy, aphthous ulcers are typical in the early stages but linear or stellate ulcers with a cobblestone appearance and luminal narrowing can be found in advanced diseases. Multiple mucosal biopsies are helpful for diagnosis despite the fact that granulomas are seen in less than one third of all cases. Gastroduodenal CD requires oral sulfasalazine therapy or systemic corticosteroid therapy in advanced cases. A case of gastroduodenal CD in a 37-year-old man admitted with epigastric pain, anorexia, vomiting and weight loss is herein reported. Malignancy was suspected as a result of the patient's clinical manifestations and endoscopic findings. An endoscopic biopsy revealed an active inflammation in the mucosa and lamina propria. The patient's condition improved with oral sulfasalazine and prednisolone therapy.

A Case of Primary Antiphospholipid Syndrome Presented with Membranoproliferative Glomerulonephritis pattern / 대한신장학회잡지

Though the renal abnormality is usually accompanied in the patient with antiphospholipid syndrome, it is not frequent that the symptoms of antiphospholipid syndrome are confined to kidney. We experienced a case of 40-year-old woman suffered from intermittently developed nephrotic range proteinuria combined with generalized edema during last 10 years. She had no past history of thrombosis, spontaneous abortion, or symptoms related to SLE. Laboratory findings revealed that she had nephrotic syndrome. Her antinuclear antibody was negative, but her serum antiphospholipid IgG antibody level was 32 GPL IU. In the renal biopsy specimen, half of the glomeruli showed global sclerosis and remaining glomeruli showed chronic thrombotic microangiopathy and membranoproliferative glomerulonephritis without immune deposit. Combined therapy with steroid and aspirin improve the amount of proteinuria, and she is free from edema without deterioration of renal function during follow up. Though this case lacked the typical symptoms of antiphospholipid syndrome, her glomerulonephritis presented with membranoproliferative pattern may be associated with it. We report this case with a review of literatures.

Heparin Therapy for Disseminated Intravascular Coagulation in Childhood

No abstract available.

A Study of Liver Span in Normal Newborn Infant

No abstract available.

A case of Incontinentia Pigmenti

No abstract available.

A Case of Alveolar Rhabdomyosarcoma Originating from the Chest Wall / 영남의대학술지

Rhabdomyosarcoma originating from the chest wall is a rare malignant tumor in children and was considered to be guarded in prognosis previously. However, recent advances in multidisciplinary treatment of rhabdomyosarcoma in children have improved the disease free survival rate. Authors report a case of alveolar rhabdomyosarcoma, stage III, originating from the chest wall who is surviving free of disease for 15 months with aggressive chemotherapy and radiotherapy.