The majority of cardiac myxoma occur sporadically as isolated lesions in the left atrium of middle-aged women. However, a familial form and a syndrome form of this lesion have also been identified. The syndrome myxoma can present itself with pigmented skin lesions and peripheral or endocrine neoplasms. The familial and syndrome forms of cardiac myxomas can usually be distinguished from the sporadic form by their occurrences at younger ages, their unusual locations, the multicentricity of the lesions, and the presence of rare pathological conditions. In addition, a higher rate of recurrent lesions is usually associated with the familial and syndrome forms of this disease. We present a case of complex cardiac myxoma with pigmented skin lesions and breast myxoma and report it with a review of literature.
Breast ; Female ; Heart Atria ; Humans ; Myxoma* ; Nipples* ; Skin

PURPOSE: We analyzed pelvic ultrasonography (USG) findings in girls with central precocious puberty (CPP) and assessed the role of uterine and ovarian measurements in discriminating between CPP and other pubertal conditions. METHODS: Seventy-four girls (chronological age 7.8 +/- 0.5 years, bone age 9.9 +/- 0.8 years) with precocious pubertal signs were enrolled. Measurements of uterine and ovarian parameters by pelvic USG included antero-posterior diameters of the uterine fundus and cervix, diameter of each ovary, number of follicles, and maximal diameter of the largest follicle. The pelvic USG parameters were compared between girls with CPP (n = 49) and girls with atypical premature thelarche (PT) (n = 25). RESULTS: Antero-posterior diameter of uterine fundus (1.05 +/- 0.34 vs. 0.74 +/- 0.78 cm, P = 0.001), maximal ovarian diameter (2.13 +/- 0.48 vs. 1.84 +/- 0.74 cm, P = 0.048) and mean ovarian area (2.31 +/- 0.79 vs. 1.69 +/- 0.71 cm, P = 0.002) were significantly greater in girls with CPP than in girls with atypical PT. For the diagnosis of CPP, the sensitivity and specificity of A-P diameter of uterine fundus (> 0.9 cm) was 65.3% and 84.0%, the sensitivity and specificity of maximal ovarian diameter (> 2.0 cm) was 55.1% and 76%, and the sensitivity and specificity of mean ovarian area (> 2.0 cm2) was 62.9% and 80.0%. CONCLUSION: Girls with CPP had significantly higher dimensions of the uterus and ovary measurements compared to girls with atypical PT, but sensitivity and specificity were not high enough to differentiate CPP from atypical PT. Pelvic USG may help the diagnosis of CPP in girls.
Sensitivity and Specificity

The Ael subgroup expresses the least amount of A antigens and could only be detected by performing the adsorption-elution test. The frequency of the Ael subgroup is about 0.001% in Koreans, and the Ael02 allele, which originates from A102, is the most frequently identified allele in the Korean population. We report a Korean family with the Ael03 allele identified by molecular genetic analysis. To the best of our knowledge, this is the first such report in Korea to date.
ABO Blood-Group System/*genetics ; Alleles ; Base Sequence ; DNA Mutational Analysis ; Exons ; Frameshift Mutation ; Humans ; Male ; Middle Aged ; Pedigree ; Phenotype ; Polymerase Chain Reaction ; Republic of Korea

An 18-year-old female was admitted because of dyspnea at rest. A chest computed tomography (CT) scan and fiberoptic bronchoscopy demonstrated a polypoid tumor in the left main bronchus, 0.5cm distal from the carina. Surgical resection of the tumor was performed, along with. A pathological evaluation and the immunohistochemical findings led to the diagnosis of a glomus tumor, which originated from the bronchus, an area where this type of tumor has rarely been reported.
Adolescent ; Bronchi ; Bronchoscopy ; Diagnosis ; Dyspnea ; Female ; Glomus Tumor* ; Humans ; Thorax

PURPOSE: Almost 80% of primary lung cancers are non-small cell lung cancer (NSCLC), and their prognosis is very poor since only one-fourth of patients with NSCLC present with a resectable disease at the time of diagnosis. During the last 10 years, the role of chemotherapy for NSCLC has been expanding as an adjunctive to radiation and surgery, as well as to palliative therapy for stage IV NSCLC. This study is a retrospective analysis of two chemotherapeutic regimens for the treatment of advanced NSCLC. MATERIALS AND METHODS: Between January 1999 and December 2001, 109 patients with histologically proven NSCLC (> or = stage IIIA), who received either the DP (Docetaxel 75 mg/m2 +Cisplatin 75 mg/m2, n=63, 57.8%) or the TC (Paclitaxel 175 mg/m2+ Carboplatin 5* AUC mg, n=46, 42.2%) combination chemotherapies were included. RESULTS: The patients ages ranged from 46 to 77 years, and the patients in the DP group (56.3+/-8.6 years) were younger than those in the TC group (62.1+/-8.8 years) (p<0.05). Seventy (DP: 39 and TC: 31) of 109 patients were eligible for their response to the combination chemotherapies. There were 2 complete responses (CR) (5.1%) and 19 partial responses (PR) (46.2%) documented in the DP group (response rate, RR: 51.3%), and 11 PR (35.5%) in the TC group (RR: 35.5%). The survival was longer in the DP group compared to the TC group (median survival 19.5 months vs. 17.1 months, p< 0.05). Grade 4 neutropenia occurred in 30 patients (47.6%) treated with the DP regimen and in 10 (21.7%) treated with the TC regimen (p<0.05). Grade 3~4 nausea and vomiting occurred in 11 patients (17.5%) in the DP group and 4 (8.7%) in the TC group (p>0.05). Grade 3~4 peripheral neuropathy occurred in 5 patients (7.9%) in the DP group and in 8 (17.4%) in the TC group (p>0.05). CONCLUSION: The combination chemotherapies of docetaxel plus cisplatin and paclitaxel plus carboplatin are active against advanced stage NSCLC, with acceptable toxicities. As there are differences in the baseline characteristics between the two groups, no differences in survivals or response rates could be concluded.
Area Under Curve ; Carboplatin* ; Carcinoma, Non-Small-Cell Lung* ; Cisplatin* ; Diagnosis ; Drug Therapy ; Drug Therapy, Combination ; Humans ; Lung Neoplasms ; Nausea ; Neutropenia ; Paclitaxel* ; Palliative Care ; Peripheral Nervous System Diseases ; Prognosis ; Retrospective Studies ; Vomiting

Aggressive fibromatosis is best difined as a group of non-encapsulated, non-metastasizing fibrous tumors that have tendency for local recurrence. This tumor is chariaterized histologically by fibroblastic proliferation and clinically by the potential to infiltrate and to recur after surgical excision, but not metastasize. The most common anatomic location have been the abdominal muscle and the extrimities. The incidence of the head and neck is mentioned as from 9.5% to 50% of all desmoid tumors. Within this area, 40%-to 80% of the tumors are located in the neck, but rare in oral cavity. Histologically the tumor is composed of proliferative fibroblasts with a collagenous component. The degree of cellularity varies from area to area and from tumor to tumor. The cells are usually spindle-shaped and the nuclei present only slightly polymorphism, but mitosis are rare and never atypical. Oral fibromatosis has been described as fibrosarcoma, pseusarcoma, pseudosarcomatous fibrosarcoma, metastasizing fibromatosis, juvenile aggressive fibromatosis, and aggressive fibromatosis, but Wilkin and Waldron suggested that the aggressive fibromatosis was a more appropriate term, reflecting the invasive characteristics of the tumor. The treatment of aggressive fibromatosis is traditionally surgical resection with an area of tumor free tissue. But when the complete surgical excision is not possible, radiation and chemotherapy also can be used. Clinically the tumor reported to be not painful in most cases, but capable of rapid growth. Careful clinical and histological examinations are essential for correct diagnosis. We report a case of aggressive fibromatosis. Occurring of the maxilla in 10 year female patient.
Abdominal Muscles ; Collagen ; Diagnosis ; Drug Therapy ; Female ; Fibroblasts ; Fibroma ; Fibromatosis, Aggressive* ; Fibrosarcoma ; Head ; Humans ; Incidence ; Maxilla* ; Mitosis ; Mouth ; Neck ; Recurrence

Ectropion is a drooping or eversion of the lower eyelid as a result of lower lid laxity, canthal tendon laxity, or cicatrix formation from scarring. Ectropion can also be congenital or develop with aging. This complication can arise from primary trauma to the lower lid apparatus or as a result of unfavorable healing and scar contracture. To prevent development of ectropion, a careful attention in case of operation for orbital frature repair or blepharoplasty is necessary. Especially, excessive dissection or retraction of skin and orbicularis muscle should be avoided, and formation of edema and hematoma should be prevented by complete hemostasis. It might be helpful to massage on the eyelid frequently. In spite of endeavors mentioned above, lareral canthoplasty or skin graft must be performed when postoperative ectropion is occurred. With literature review the authors present a case report that good clinical result is achieved by lateral tarsal strip procedure on cicatricial ectropion caused by a complication of reconstruction of blow-out fracture.
Aging ; Blepharoplasty ; Cicatrix ; Contracture ; Ectropion* ; Edema ; Eyelids ; Hematoma ; Hemostasis ; Massage ; Orbit ; Orbital Fractures* ; Skin ; Tendons ; Transplants

Heat stroke occurs when body thermal regulation is upset and unable to dissipate adequate amounts of heat with rise in body temperature. Thus heat stroke may result in rhabdomyolysis and multiorgan failure. We observed two childhood cases of heat stroke with rhabdomyolysis and multiorgan failure. One case was caused by accidentally being left in a car trunk for 3 hours on a sunny afternoon in late spring and the other case occurred climbing a mountain on a hot summer day during a school trip for improving individual self-control. The first case, a boy almost 5 years old, displayed thetypical clinical features of heat stroke including hyperpyrexia, CNS disturbance, hot dry skin, acute liver failure, rhabdomyolysis, oligo- anuric acute renal failure, and disseminated intravascular coagulopathy. He was treated with general supportive care and daily hemodialysis. Despite the aggressive management, he died of shock on the sixteenth day of admission day. The second case, a 14-year-old middle school boy, displayed similiar features of the first case but showed milder symptoms. He was also treated with general supportive care and daily hemodialysis for 10 days. He recovered completely and was discharged in good condition. The public including medical and paramedical personnels should be warned of the dangers of childhood heat stroke, which is potentially lethal, but preventable.
Acute Kidney Injury ; Adolescent ; Allied Health Personnel ; Body Temperature ; Child ; Child, Preschool ; Heat Stroke* ; Hot Temperature* ; Humans ; Liver Failure, Acute ; Male ; Renal Dialysis ; Rhabdomyolysis* ; Shock ; Skin

BACKGROUND/AIMS: Available data about reflux patterns and symptom determinants in the gastroesophageal reflux disease (GERD) subtypes off proton pump inhibitor (PPI) therapy are lacking. We aimed to evaluate reflux patterns and determinants of symptom perception in patients with GERD off PPI therapy by impedance-pH monitoring. METHODS: We retrospectively reviewed the impedance-pH data in patients diagnosed as GERD based on results of impedance-pH monitoring, endoscopy and/or typical symptoms. The characteristics of acid and weakly acidic reflux were evaluated. Symptomatic and asymptomatic reflux were compared according to GERD subtypes and individual symptoms. RESULTS: Forty-two patients (22 males, mean age 46 years) were diagnosed as GERD (17 erosive reflux disease, 9 pH(+) non-erosive reflux disease [NERD], 9 hypersensitive esophagus and 7 symptomatic NERD). A total of 1,725 reflux episodes were detected (855 acid [50%], 857 weakly acidic [50%] and 13 weakly alkaline reflux [< 1%]). Acid reflux was more frequently symptomatic and bolus clearance was longer compared with weakly acidic reflux. In terms of globus, weakly acidic reflux was more symptomatic. Symptomatic reflux was more frequently acid and mixed reflux; these associations were more pronounced in erosive reflux disease and symptomatic NERD. The perception of regurgitation was related to acid reflux, while that of globus was more related to weakly acidic reflux. CONCLUSIONS: In patients not taking PPI, acid reflux was more frequently symptomatic and had longer bolus clearance. Symptomatic reflux was more frequently acid and mixed type; however, weakly acidic reflux was associated more with globus. These data suggest a role for impedance-pH data in the evaluation of globus.
Electric Impedance ; Endoscopy ; Esophageal pH Monitoring ; Esophagus ; Gastroesophageal Reflux ; Humans ; Male ; Proton Pumps ; Protons ; Retrospective Studies

PURPOSE: To investigate the clinical features and surgical outcomes of rhegmatogenous retinal detachment (RRD) requiring surgery according to age. METHODS: Medical records of patients who underwent surgery for primary RRD between January 2008 and March 2016 were reviewed retrospectively. Patients were classified into two groups according to age at diagnosis: the under-40 group and the over-40 group. The two groups were compared in terms of demographic features, ocular manifestation, operating methods, primary anatomical success rate, and visual outcome. RESULTS: One hundred and forty-four eyes from 144 patients were included. Mean subject age was 48.6 ± 16.9 years old. The under-40 group involved 42 eyes from 42 patients, and the over-40 group included 102 eyes from 102 patients. Symptom duration was shorter in the under-40 group compared to the over-40 group (7.6 ± 10.7 days vs. 14.5 ± 24.4 days; p = 0.029). Proliferative vitreoretinopathy (PVR) occurred more frequently in the under-40 group (40.0% vs. 17.4%, p = 0.007) than in the over-40 group. The anatomical success rate of primary surgery was significantly different between the two groups; 78.6% in the under-40 group and 91.2% in the over-40 group (p = 0.038). Preoperative PVR increased the rate of anatomical failure (40.0% vs. 6.2%, p < 0.001). The visual outcomes were not significantly different between the two groups. CONCLUSIONS: RRD is combined with PVR more frequently in young patients than in old patients, which increases the failure rate of primary re-attachment surgery.
Diagnosis ; Humans ; Medical Records ; Retinal Detachment* ; Retinaldehyde* ; Retrospective Studies ; Scleral Buckling ; Vitrectomy ; Vitreoretinopathy, Proliferative