No abstract available.
Adolescent ; Adrenergic beta-Antagonists/therapeutic use ; Calcium Channel Blockers/therapeutic use ; Cardiomyopathy, Hypertrophic, Familial/complications/genetics/*pathology/physiopathology/therapy ; Electric Countershock ; Electrocardiography ; Female ; Genetic Predisposition to Disease ; Heart Failure/etiology/therapy ; Heart Septum/drug effects/*pathology/physiopathology/ultrasonography ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pedigree ; Phenotype ; Tachycardia, Ventricular/etiology/therapy ; Treatment Outcome ; Ventricular Outflow Obstruction/etiology

BACKGROUND/AIMS: Anti-C-reactive protein (CRP) antibody has been introduced as a potential biologic marker in Systemic lupus erythematosus (SLE). The aim of study is to evaluate the level of anti-CRP antibody in patients with SLE. METHODS: This study investigated the relationship between levels of anti-CRP antibodies and disease activity markers, such as complement, anti-double-stranded DNA antibody, and SLE disease activity index in 34 patients with SLE. RESULTS: The serum anti-CRP antibody levels of the patients with SLE were significantly higher than those of the healthy controls (11.3 ± 5.6 µg/mL vs. 9.1 ± 2.8 µg/mL). The percentages of the positive anti-CRP antibody were 52.9% in SLE and 27.8% in controls. Disease duration of SLE showed significant correlation with the anti-CRP antibody (r = 0.234, p = 0.026). However no significant relationship was observed between the levels of anti-CRP antibodies and disease activity markers. CONCLUSIONS: These data show that the anti-CRP antibody levels of the patients with SLE were significantly higher than those of healthy controls. We observed that the presence of the anti-CRP anti-CRP antibody was not associated with disease activity of SLE.
Antibodies ; Biomarkers ; Complement System Proteins ; DNA ; Humans ; Lupus Erythematosus, Systemic*

Craniodiaphyseal dysplasia is a rare genetic disorder of bone due to modelling errors of long bones and skull bones. Facial and cranial thickening and distortion are particularly striking in this form. The genetic understanding of this disorder is unsatisfactory. We present one case of Craniodiaphyseal dysplasia involving facial bone, skull that causes nasal obstruction. This 3-year old female who was diagnosed of craniodiaphyseal dysplasia presented abnormal facial figuring, which is excessively thickened cortical bone resulting in very large craniofacial bony structure. The patient sufferred from respiratory difficulty due to complete obstruction of choana. We performed surgical recanalization of choanal atresia by transpalatal approach and gained good postoperative result.
Child, Preschool ; Choanal Atresia ; Constriction, Pathologic* ; Facial Bones ; Female ; Humans ; Nasal Obstruction ; Skull ; Strikes, Employee

PURPOSE: Malignant degeneration of fibrous dysplasia is an uncommon recognized complication of this disease. Especially, degeneration of fibrous dysplasia to malignant fibrous histiocytoma(MFH) in facial bone is rare and the publications had been limited. The purpose of this report is to share our experience. METHODS: A 46-year-old patient with facial fibrous dysplasia visited our clinic for recent facial tingling and swelling. Malignant degeneration of fibrous dysplasia was suspected. RESULTS: Total excision of the mass and adjacent facial bone was performed. Defect was immediately reconstructed with bone graft and bone cement. At a month follow up, metastasis was detected at ipsilateral parotid gland. Superficial parotidectomy and neck dissection was performed. The patient is currently taking chemotherapy. CONCLUSION: Because of the uncommon presentation of this entity, clinical course of treatment was dependent on other histological types of malignant degeneration. We report this case to share our experience.
Drug Therapy ; Facial Bones* ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous* ; Humans ; Middle Aged ; Neck Dissection ; Neoplasm Metastasis ; Parotid Gland ; Transplants

Breast reconstruction using TRAM flap after mastectomy has been widely adopted and regarded as a gold standard method. In patients with the abdominal midline scar, insufficient blood circulation to regions contralateral to the pedicle disables inclusion of them in the TRAM flap design. So modifications including Double- pedicled TRAM, Super-charged, Turbo-charged, Bipedicled free TRAM et al. have been attempted to resolve the problem of circulatory interruption by midline scar. The authors designed a new bipedicled free TRAM flap to avoid limitations like epigastric buldging, significant abdominal weakness and complexity of flap insetting. The two donor arteries(right and left deep inferior epigastric arteries) are anastomosed to the proximal and distal ends of divided internal mammary arteries using the reverse flow. Vein anastomoses were performed in the same manner through the divided venae comitantes. Two patients having infraumbilical midline scar underwent breast reconstruction using author`s new flap. Flaps survived without fat necrosis or partial flap loss in both patients and their results in aesthetic aspects were very satisfactory. Compared to previous method for breast reconstruction of patients having infraumbilical midline scar, author's new method is useful and effective in terms of the aesthetic and technical aspect.
Blood Circulation ; Breast* ; Cicatrix* ; Fat Necrosis ; Female ; Humans ; Mammaplasty* ; Mammary Arteries ; Mastectomy ; Tissue Donors ; Veins

Critical ill patients with pandemic 2009 H1N1 influenza A are associated with mortality, including cardiovascular, respiratory and renal dysfunction. Understanding of risk factor and clinical manifestation that suggest a higher mortality can recognize high risk patients earlier. There are many reports for severe acute respiratory distress syndrome, multiple organ failure and renal failure with pandemic 2009 H1N1 influenza A. But cardiovascular disease with pandemic 2009 H1N1 influenza A remains unknown. This is the report of pandemic 2009 H1N1 influenza A association with apical balloning syndrome.
Cardiovascular Diseases ; Humans ; Influenza, Human ; Multiple Organ Failure ; Pandemics ; Renal Insufficiency ; Respiratory Distress Syndrome, Adult ; Risk Factors ; Takotsubo Cardiomyopathy

Ullrich-Noonan syndrome was first recognized as a unique entity in 1963 when Noonan and Ehmke described a series of patients with unusual faces and multiple malformations. These patients proved to have phenotypes of Turner syndrome with normal karyotyes, which is important feature that distinguishes Noonan from Turner syndrome. Typical features are triangular shaped face, hypertelorism, down slanting eyes, ptosis, strabismus, amblyopia, refractive errors, low set ears with thickened helices, high nasal bridge and webbed neck. A 5-year old female was admitted to our center for a surgical intervention of the known webbed neck deformity, ipsilateral upper eyelid ptosis and bilateral epicanthal fold. She had normal karyotye(46, XX) without further abnormality on physical examination. We corrected the webbed neck deformity, ptosis and epicanthal fold simultaneously with satisfactory result.
Amblyopia ; Blepharoptosis ; Child, Preschool ; Congenital Abnormalities ; Ear ; Female ; Humans ; Hypertelorism ; Neck ; Noonan Syndrome* ; Phenotype ; Physical Examination ; Pterygium* ; Refractive Errors ; Strabismus ; Turner Syndrome

Radial artery perforation is one of the major complications of transradial percutaneous coronary intervention (PCI). Previous reports have suggested that sealing the perforation with a smaller guiding catheter may be possible. In one such study, the perforated segment was sealed with a 0.014- or 0.021-inch guidewire, and PCI was successfully completed. In this study, we describe a radial artery perforation that occurred after diagnostic coronary angiography and during insertion of a 6 French (FR) guiding catheter. PCI and the perforation were successfully managed through the use of a 5 Fr guiding catheter and a 0.035-inch guidewire.
Catheters ; Coronary Angiography ; Percutaneous Coronary Intervention* ; Radial Artery*

Benign symmetrical lipomatosis, also known as Madelung's disease, is a rare condition characterized by massive fatty deposits arranged symmetrically around the neck, shoulders, and arms. Patients usually complain of their cosmetic appearance, but treatment can be considered for decreased neck motion and/or aerodigestive problems. Surgical lipectomy and liposuction are treatment of choice, but recurrence is common. Because the distribution of the lesion tends to be wide and diffuse, single operation is not sufficient for improving contour and functional problems, especially in severely affected case. We experienced a progressed patient who suffered from limitation of neck motion, dyspnea including sleep apnea due to massive deposits on whole neck. We performed 2 staged operations for the purpose of removing as much lipomatous tissue as possible. After 2 staged excisions, patient's appearance and aerodigestive problems are much improved and recurrence is not observed.
Arm ; Dyspnea ; Humans ; Lipectomy ; Lipomatosis ; Lipomatosis, Multiple Symmetrical* ; Neck ; Recurrence ; Shoulder ; Sleep Apnea Syndromes

OBJECTIVE: Contrast-induced nephropathy (CIN) frequently occurs after percutaneous intervention. Objective of this study was to investigate the usefulness of serum cystatin C, neutrophil gelatinase-associated lipocalcin (NGAL), urinary kidney injury molecule-1 (KIM-1), and interleukin-18 (IL-18) as early predictors for CIN after percutaneous coronary intervention (PCI). METHODS: In 53 patients who underwent PCI were enrolled. Serum creatinine and cystatin C level were measured immediately before, and 24 hours and 48 hours after catheterization. Serum NGAL, urinary KIM-1, and IL-18 were measured immediately before, and 4 hours, 24 hours, and 48 hours after catheterization. CIN was defined as a rise in creatinine 0.5 mg/dL or 25% above baseline. RESULTS: CIN occurred in four patients (7.5%). Serum cystatin C levels were higher at 24 hours and 48 hours in CIN patients than in those without CIN (P<0.05). Serum NGAL levels were higher at 48 hours in CIN patients than in those without CIN. Urinary KIM-1 levels were higher at 48 hours in CIN patients than in those without CIN. There were no significant markers of CIN on multi-variate analysis. CONCLUSION: In this study, the occurrence of CIN after PCI was 7.5%. Although there were some time-course changes in serum cystatin C and urinary KIM-1 after PCI, there was no significant predictor for CIN after PCI.
Catheterization ; Catheters ; Contrast Media ; Creatinine ; Cystatin C ; Humans ; Interleukin-18 ; Kidney ; Neutrophils ; Percutaneous Coronary Intervention*