Thyroid acropachy is a rare complication of Graves' disease, manifested by clubbing of the terminal phalanges, periosteal new bone formation and overlying soft tissue swelling, It may occur when the patient is hypothyroid, euthyroid or hyperthyroid. In most cases, it is a part of the syndrome, including exophthalmos and/or pretibial myxedema. The authors have experienced one case of thyroid acropachy and report with a review of the literature review. The patient a 56-year-old female with a characteristic feathery new bone formation on the medial side of the shaft of the left first metatarsal bone and overlying soft tissue swelling. However, there was no pretibial myxedema and clubbing of fingers. She was hypothyroid and treated with systemic corticosteroid for mild pain and persistent swelling. The treatment had temporarily improved the patient's condition.
Exophthalmos ; Female ; Fingers ; Graves Disease ; Humans ; Metatarsal Bones ; Middle Aged ; Myxedema ; Osteogenesis ; Thyroid Gland*

Primary systemic amyloidosis is a progressive disease that is frequently fatal. Nephrotic syndrome is present in almost one-third, congestive heart failure in one-quarter, and peripheral neuropathy in one-sixth of patients at the time of diagnosis. If heart or renal failure are presented, survival rate is poor. We experienced a case of a 66 year-old female patient who had complained lower leg edema and paresthesia of extremities for about 5 months. The laboratory findings were consistent with nephrotic syndrome, but the lower leg edema was non-pitting and the cause of paresthesia was unknown. We performed kidney and nerve biopsy and confirmed a case of primary systemic amyloidosis. In this case, presence of postural hypotension, probable cardiac involvement and relatively long spikes along the outside of the glomerular capillary loops on methenamine silver stain is suggestive of poor prognosis. We can predict chronic renal failure and congestive heart failure in the course of this case. We report a case of primary systemic amyloidosis predominantly presenting nephrotic syndrome and peripheral neuropathy with review of related literatures.
Aged ; Amyloidosis* ; Biopsy ; Capillaries ; Diagnosis ; Edema ; Extremities ; Female ; Heart ; Heart Failure ; Humans ; Hypotension, Orthostatic ; Kidney ; Kidney Failure, Chronic ; Leg ; Methenamine ; Nephrotic Syndrome* ; Paresthesia ; Peripheral Nervous System Diseases* ; Prognosis ; Renal Insufficiency ; Survival Rate

PURPOSE: Angiogenesis is involved in the pathogenesis of chronic rhinosinusitis with nasal polyps. We aimed to investigate the effects of prostaglandin E2 (PGE2) on vascular endothelial growth factor (VEGF) production, the role of E-prostanoid (EP) 4 receptors, and the signal transduction pathway mediating VEGF production in nasal polyp-derived fibroblasts (NPDFs). METHODS: Eight primary NPDF cultures were established from nasal polyps, which were incubated with or without PGE2. Reverse transcription-polymerase chain reaction amplification of EP receptors (EP1, EP2, EP3, and EP4) and immunofluorescence staining for VEGF production were performed. VEGF production via the cyclic adenosine monophosphate (cAMP)-dependent protein kinase A (PKA) and phosphatidylinositol 3-kinase (PI3K) pathways was evaluated by enzyme-linked immunosorbent assay. RESULTS: All EP receptors were expressed in NPDFs. PGE2 significantly increased VEGF production concentration- and time dependently, and VEGF production was regulated by an EP4 receptor. Activation of intracellular cAMP regulated VEGF production. VEGF production was decreased by PKA and PI3K inhibitors via intracellular cAMP. CONCLUSIONS: PGE2 stimulates VEGF production via the EP4 receptor in NPDFs. These results indicate that PGE2-induced VEGF production is mediated, at least partially, through cAMP-dependent signaling pathways. Therapies targeting the EP4 receptor may be effective in inhibiting the development of nasal polyps.
Adenosine Monophosphate ; Cyclic AMP-Dependent Protein Kinases ; Dinoprostone ; Fibroblasts ; Fluorescent Antibody Technique ; Nasal Polyps ; Negotiating ; Phosphatidylinositol 3-Kinase ; Signal Transduction ; Vascular Endothelial Growth Factor A

PURPOSE: To evaluate the utility of quantification of coronary artery calcification using spiral CT. MATERIALS AND METHODS: Spiral CT scans of the heart were obtained in 25 patients with coronary artery disease diagnosed by coronary angiography and in six controls without coronary artery disease. Spiral CT was performedwith 3 mm collimation at 3 mm/sec table speed and the obtained volume data of the heart was reconstructed at 2 mm intervals. Total calcium scores of the 30 contiguous slices of the proximal coronary artery were calculated basedon the areas and peak density. Two groups were compared for total scores and sensitivity, and specificity and positive predictive values were calculated. RESULTS: The number of subjects with coronary calcification(totalcalcium score>0) detected by spiral CT were 20(80%) of 25 with coronary artery disease and 2(33%) of 6 without coronary artery disease. The sensitivity, specificity, and positive predictive values were 80%, 67% and 91% respectively. Sensitivity was 64%, specificity was 80% in patients aged < or =60. Sensitivity was 76% and specificity was 83%(total calcium score 10). CONCLUSION: Quantification of coronary artery calcification using spiral CT haslow specificity in the older group and low sensitivity in the younger group ; the procedure is therefore may notbe useful as a non invase screening test to predict the prescence of coronary artery disease. In the younger group, however, a cardiac workup is strongly indicated if calcification is present.
Arteries ; Calcium ; Coronary Angiography ; Coronary Artery Disease ; Coronary Vessels* ; Heart ; Humans ; Mass Screening ; Sensitivity and Specificity ; Tomography, Spiral Computed*

Complement component 3 glomerulonephritis (C3GN) is a rare kidney disease characterized by complement dysregulation that results in prominent complement component 3 (C3) deposition in the kidneys. The clinical course of C3GN varies from mild hematuria to progressive chronic kidney disease. In most patients, C3GN is driven by acquired factors, namely, autoantibodies that target C3 or C5 convertases. Genetic variations in complement-related genes are less frequent. We report the case of a 9-yearold Korean boy who presented with microscopic hematuria and a persistently low C3 level and had biopsy findings of C3GN, with the presence of a C3 gene mutation: a frameshift mutation associated with C3 deficiency. However, the patient did not exhibit any other symptoms of complement deficiency. Direct DNA sequencing of his family members revealed the same genetic mutation in his father and older brother. This case report is significant because there are very few such reports worldwide concerning gene mutations related to C3 deficiency to be discovered in patients with C3GN. Explaining C3GN pathogenesis is challenging; therefore, additional research is required in the future.

The standard treatment for choledocholithiasis is to remove the stones by performing endoscopic retrograde cholangiopancreatography (ERCP). With various techniques such as basket extraction, balloon catheter extraction, mechanical lithotripsy, followed by endoscopic papillary sphincterotomy or endoscopic papillary balloon dilatation, the success rate of stone extraction is known to be over 90%. However, stone extraction can fail in cases with large common bile duct stones or biliary stricture. We report a case of impacted choledocholithiasis after conventional ERCP and mechanical lithotripsy treated with extracorporeal shock wave lithotripsy.

Complement component 3 glomerulonephritis (C3GN) is a rare kidney disease characterized by complement dysregulation that results in prominent complement component 3 (C3) deposition in the kidneys. The clinical course of C3GN varies from mild hematuria to progressive chronic kidney disease. In most patients, C3GN is driven by acquired factors, namely, autoantibodies that target C3 or C5 convertases. Genetic variations in complement-related genes are less frequent. We report the case of a 9-yearold Korean boy who presented with microscopic hematuria and a persistently low C3 level and had biopsy findings of C3GN, with the presence of a C3 gene mutation: a frameshift mutation associated with C3 deficiency. However, the patient did not exhibit any other symptoms of complement deficiency. Direct DNA sequencing of his family members revealed the same genetic mutation in his father and older brother. This case report is significant because there are very few such reports worldwide concerning gene mutations related to C3 deficiency to be discovered in patients with C3GN. Explaining C3GN pathogenesis is challenging; therefore, additional research is required in the future.

Complement component 3 glomerulonephritis (C3GN) is a rare kidney disease characterized by complement dysregulation that results in prominent complement component 3 (C3) deposition in the kidneys. The clinical course of C3GN varies from mild hematuria to progressive chronic kidney disease. In most patients, C3GN is driven by acquired factors, namely, autoantibodies that target C3 or C5 convertases. Genetic variations in complement-related genes are less frequent. We report the case of a 9-yearold Korean boy who presented with microscopic hematuria and a persistently low C3 level and had biopsy findings of C3GN, with the presence of a C3 gene mutation: a frameshift mutation associated with C3 deficiency. However, the patient did not exhibit any other symptoms of complement deficiency. Direct DNA sequencing of his family members revealed the same genetic mutation in his father and older brother. This case report is significant because there are very few such reports worldwide concerning gene mutations related to C3 deficiency to be discovered in patients with C3GN. Explaining C3GN pathogenesis is challenging; therefore, additional research is required in the future.

Complement component 3 glomerulonephritis (C3GN) is a rare kidney disease characterized by complement dysregulation that results in prominent complement component 3 (C3) deposition in the kidneys. The clinical course of C3GN varies from mild hematuria to progressive chronic kidney disease. In most patients, C3GN is driven by acquired factors, namely, autoantibodies that target C3 or C5 convertases. Genetic variations in complement-related genes are less frequent. We report the case of a 9-yearold Korean boy who presented with microscopic hematuria and a persistently low C3 level and had biopsy findings of C3GN, with the presence of a C3 gene mutation: a frameshift mutation associated with C3 deficiency. However, the patient did not exhibit any other symptoms of complement deficiency. Direct DNA sequencing of his family members revealed the same genetic mutation in his father and older brother. This case report is significant because there are very few such reports worldwide concerning gene mutations related to C3 deficiency to be discovered in patients with C3GN. Explaining C3GN pathogenesis is challenging; therefore, additional research is required in the future.

Genital HPV infection is the most common sexually transmitted infection, but the majority of infections are self-limited. However, persistent infection with high-risk types can cause cervical cancer in women, which is the most common female genital cancer in Korea. In addition, HPV infection is the cause of genital warts and is associated with other anogenital cancers. The HPV vaccine is composed of the HPV L1 protein, the major capsid protein of HPV. Expression of the L1 protein in yeast using recombinant DNA technology produces noninfectious virus-like particles (VLP) that resemble HPV virions. The quadrivalent HPV vaccine is a mixture of four HPV type-specific VLPs prepared from the L1 proteins of HPV 6, 11, 16, and 18 combined with an aluminum adjuvant. Clinical trials indicate that the vaccine has high efficacy in preventing persistent HPV infection, cervical cancer precursor lesions, vaginal and vulvar cancer precursor lesions, and genital warts caused by HPV types 6, 11, 16, or 18 among females who have not already been infected with the respective HPV type. The recommended age for primary vaccination of Korean females is 15-17 years, considering sexual debut and duration of protection of the vaccine. Vaccine can be administered as young as age 9 years. Catch-up vaccination is recommended for females aged 18-26 years who have not been previously vaccinated. Vaccination is not a substitute for routine cervical cancer screening, and vaccinated females should have cervical cancer screening as recommended.
Aluminum ; Capsid Proteins ; Colposcopy* ; Condylomata Acuminata ; DNA, Recombinant ; Female ; Human papillomavirus 6 ; Humans* ; Korea ; Mass Screening ; Uterine Cervical Neoplasms ; Vaccination ; Virion ; Vulvar Neoplasms ; Yeasts