A 21-year-old woman, who had a one-year history of pancytopenia with histiocytic necrotizing lymphadenitis and hepatosplenomegaly, presented with a 5 day history of tense bullae, which were localized on the face. These clusters of tense bullae occurred on clinically normal skin, she did not have other skin lesions. A diagnosis of bullous systemic lupus erythematosus (BSLE) was established based on clinical, laboratory, histological, and immunological findings. The bullae showed good responses to dapsone (100mg, daily) and resolved within 10 days with-out scaring. Bullous lesions of SLE may be the first cutaneous manifestation in some patients with SLE and should be considered in the differential diagnosis of the other subepidermal bullous disorders.
Blister ; Dapsone ; Diagnosis ; Diagnosis, Differential ; Female ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Lupus Erythematosus, Systemic ; Pancytopenia ; Skin ; Transcutaneous Electric Nerve Stimulation* ; Young Adult

Bipotent progenitors for T and natural killer (NK) lymphocytes are thought to exist among early precursor thymocytes or liver lymphocytes. The identification of such a progenitor population or mature NK cells in such organs remains undefined. Here we report the identification of a novel receptor of NK cells, p58 (HLA class I-specific inhibitory receptors), in fetal thymocytes and fetal liver lymphocytes. Our finding suggests the NK cells mature in the developmental stage during feta1 ontogeny. Flow cytometric analysis revealed p58 positive cells in thymocytes or in fetal liver lymphocytes and reverse transcription PCR also showed amplification of p58 RNA. The result of single stranded conformational polymorphism (SSCP) showed it discriminates one or two base pair differences of the p58 gene. Although the question still remains as to whether the expression of p58 is due to the NK cells or natural T cells, it is clear the p58 is expressed in fetal thymocytes or liver lymphocytes. And SSCP analysis using appropriate sets of primers used in this study, is helpful to study the diversity of p58.
Base Pairing ; Killer Cells, Natural ; Liver* ; Lymphocytes* ; Polymerase Chain Reaction ; Polymorphism, Single-Stranded Conformational ; Reverse Transcription ; RNA ; T-Lymphocytes ; Thymocytes*

BACKGROUND: Due to its anatomical location and vague symptom, early diagnosis of traumatic pacreatic injury is difficult. Furthermore it is very difficult to carry out appropriate management for the injury, so morbidity and mortality rates of traumatic pancreatic injuries remain high. Patients and METHODS: We reviewed medical records of 35 cases of patients who had been diagnosed as pancreatic injury and operated at Department of Surgery, Busan National University College of Medicine between January 1995 and December 2000. RESULT: 1) The male to female ratio was 2.5:1 and the highest incidence occurred in the 4th decade(31.3%). 2) Thirty two cases(91.4%) were injured by blunt trauma and 3 cases(8.6%) were injured by penetrating trauma. 3) The most common clinical manifestation was abdominal pain. 4) Twenty two patients(62.9%) were operated within 24hours after injury, and the mean time interval between injury and operation was 26.4 hours. 5) The serum amylase levels higher than 150 somogi unit were recorded in 20 cases(57.1%) of the patients. 6) Twenty seven patients(77.1%) had another associated injury with an average of 2 associated intraabdominal injuries. The most frequently injured intraabdominal organ was mesentery. 7) Complications occurred in 20 of 35 patients(57.1%) and the most common complication was pancreas-related complications, such as fistula, pseudocyst, abscess, pancreatitis. 8) Six patients(17.1%) were died due to hypovolemic shock, sepsis, respiratory failure and renal failure. CONCLUSION: Early diagnosis and appropriate operative methods may reduce morbidity and mortality from traumatic pancreatic injury
Abdominal Pain ; Abscess ; Amylases ; Busan ; Early Diagnosis ; Female ; Fistula ; Humans ; Incidence ; Male ; Medical Records ; Mesentery ; Mortality ; Pancreatitis ; Renal Insufficiency ; Respiratory Insufficiency ; Sepsis ; Shock

PURPOSE: We analyzed the oncological outcome and prognostic factor of the chondrosarcoma arising from benign bone tumor due to malignant transformation. MATERIALS AND METHODS: From April 1986 to April 2009, 18 cases were considered eligible. We analyzed retrospectively the patient's characteristics and prognostic factors that affect to the local recurrence and distant metastasis. RESULTS: As classified by primary benign bone tumor, 4 cases were solitary osteochondroma, 11 cases were multiple osteochondromatosis and 3 cases were multiple enchondromatosis. The mean follow-up period was 85 months. The 5-year disease free survival rate of 18 patients was 85.9%. Their overall MSTS score was 25.2 (84%). There were local recurrence in 3 cases and no distant metastasis. We found that tumor location and surgical margin affected to the prognosis significantly. CONCLUSION: In secondary chondrosarcoma patients, the prognosis was good relatively and tumor location and surgical margin are important prognosis factor.
Chondrosarcoma ; Disease-Free Survival ; Enchondromatosis ; Follow-Up Studies ; Humans ; Neoplasm Metastasis ; Osteochondroma ; Osteochondromatosis ; Prognosis ; Recurrence ; Retrospective Studies

Fetal thymus may be the organ for NK cell maturation, but the in vivo evidences are few, Here, by analyzing NK cell receptor, we present that NK cells develop in fetal thymus and fetal liver and that NK cell receptor appears earlier than the expression CD16 or CD56. Moreover, the finding that the repertoire of NK cell receptor is different between fetal thymus and fetal liver lymphocytes suggests that the environmental factors may influence the NK cell receptor repertoire during NK cell maturation.
Killer Cells, Natural* ; Liver* ; Lymphocytes* ; Thymocytes* ; Thymus Gland

Bilateral tubal pregnancy is the least common type of all extrauterine pregnancies, 1:725-1:1580 of pregnancies are bilateral. Simultaneous tubal pregnancies have been reported in natural cycles, recently, after ovulation induction, assisted reproductive techniques. The high incidence of ectopic pregnancy assisted with ovulation induction continues to be a problem. Bilateral tubal pregnancy associated with the ovulation induction has been reported few cases of 3.0% to 6.0% in frequency. This paper describes a case of simultaneous bilateral tubal pregnancy after ovulation induction and intrauterine insemination in a 27 year old patient. The diagnosis was confirmed by laparoscopy performed 30 days after intrauterine insemination, which revealed bilateral tubal pregnancy and mild hyperstimulated ovaries. Bilateral salpingectomy was performed. With a review of the literature on this topic, diagnostic aspect and incidence and treatment options are discussed.
Adult ; Diagnosis ; Female ; Humans ; Incidence ; Insemination* ; Laparoscopy ; Ovary ; Ovulation Induction* ; Ovulation* ; Pregnancy ; Pregnancy, Ectopic ; Pregnancy, Tubal* ; Reproductive Techniques, Assisted ; Salpingectomy

Reconstructions of extensive composite scalp and cranial defects are challenging due to high incidence of postoperative infection and reconstruction failure. In such cases, cranial reconstruction and vascularized soft tissue coverage are required. However, optimal reconstruction timing and material for cranioplasty are not yet determined. Herein, we present a large skull defect with a chronically infected wound that was not improved by repeated debridement and antibiotic treatment for 3 months. It was successfully treated with anterolateral thigh (ALT) free flap transfer for wound salvage and delayed cranioplasty with a patient-specific polyetheretherketone implant. To reduce infection risk, we performed the cranioplasty 1 year after the infection had resolved. In the meantime, depression of ALT flap at the skull defect site was observed, and the midline shift to the contralateral side was reported in a brain computed tomography (CT) scan, but no evidence of neurologic deterioration was found. After the surgery, sufficient cerebral expansion without noticeable dead-space was confirmed in a follow-up CT scan, and there was no complication over the 1-year follow-up period.

There are many variant forms in Turner's syndrome which is characterized by female phenotype, short height, primary amenorrhea, infertility, abnormal secondary sexual development, and mental retardation. Among these 45,XO/46,XX/47,XXX form is known as rare variant of Turner's syndrome. 45,XO/46,XX/47,XXX mosaicism is characterized by normal height, possibility of normal sexual development, menstrual/pregnancy history and normal intelligence. We report a cse of 45,XO/46,XX/47,XXX Turner's syndrome associated with primary amenorrhea in 17-year old Korean female.
Adolescent ; Amenorrhea* ; Female ; Humans ; Infertility ; Intellectual Disability ; Intelligence ; Mosaicism* ; Phenotype ; Sexual Development ; Turner Syndrome

PURPOSE: We analyzed the oncologic outcome of the malignant transformed benign giant cell tumor of bone. MATERIALS AND METHODS: Between January 2000 and February 2012, 5 cases were referred with suspicious malignant transformation of benign giant cell tumor. No patients underwent radiation therapy. RESULTS: After referral, all patients received the wide excision of the tumor and its' pathologic diagnosis were osteosarcoma. As classified by the location of tumor lesion, 3 cases were located in the distal femur, 1 case was in the distal radius and 1 case was in the proximal femur. The average latent period between diagnosis of benign giant cell tumor and diagnosis of secondary malignant giant cell tumor was 49.2 months. (range, 24-126 months) The mean follow-up period was 21.6 months. There were subsequent local recurrence in 2 cases and 3 patients developed distant metastasis. All patients with lung metastasis were dead. CONCLUSION: Malignant transformation of benign giant cell tumor of bone can be occurred within 5 years. Therefore, when benign giant cell tumor suspicious malignant transformation, it is necessary to do more aggressive treatment.
Femur ; Follow-Up Studies ; Giant Cell Tumor of Bone ; Giant Cell Tumors ; Giant Cells ; Humans ; Lung ; Neoplasm Metastasis ; Osteosarcoma ; Radius ; Recurrence ; Referral and Consultation

Eclampsia is defined as the occurrence of convulsions, not caused by any coincidental neurologic disease such as epilepsy, in a woman whose condition also meets the criteria for preeclampsia. Intracerebral hemorrhage with eclampsia is rare but maternal mortality is 30-40%. Fetal outcome parallels that of the mother and reflects the maternal condition as well as gestational age at delivery. With noninvasive imaging technique of CT, the early diagnosis and proper management of intracerebral hemorrhage in a patient can be achieved. The syndrome of Hemolysis, Elevated Liver enzyme, Low Platelets (HELLP) is considered to be complication of severe preeclampsia-eclampsia. We report a case of intracerebral hemorrhage and HELLP syndrome in a patient with eclampsia.
Cerebral Hemorrhage* ; Early Diagnosis ; Eclampsia* ; Epilepsy ; Female ; Gestational Age ; HELLP Syndrome* ; Hemolysis ; Humans ; Liver ; Maternal Mortality ; Mothers ; Pre-Eclampsia ; Pregnancy ; Seizures