The congenital long-QT syndrome (LQTS) is characterized by recurrent syncope, prolonged QT intervals, QT interval lability, polymorphic ventricular tachycardia, and sudden death. We report a case of congenital long QT syndrome in a 28-day-old male infant who presented with syncope, bradycardia with 2: 1 pseudo-atrioventricular block and a markedly prolonged QT inteval. One episode occured after crying and degenerated into ventricular fibrillation and terminated after cardioversion. A VVI type cardiac pacemaker was implanted. Subsequently, the infant's heart rate was over 110/min and 2: 1 AV block and any other arrhythmia were absent. The infant recovered from the accompanied pneumonia and sepsis and was discharged 47 days after adrnission. However, 13 days after discharge, the infant returned to our hospital ER with syncope. Ventricular fibrillation ceased after cardioversion. Despite medication with propranolol, ventricular tachycardia persisted. The infant expired the day after he was discharged against medical advice.
Arrhythmias, Cardiac ; Atrioventricular Block* ; Bradycardia ; Crying ; Death, Sudden ; Electric Countershock ; Heart Rate ; Hospitals ; Humans ; Infant ; Long QT Syndrome ; Male ; Pneumonia ; Propranolol ; Sepsis ; Syncope ; Tachycardia, Ventricular ; Ventricular Fibrillation

The Sertoli-Leydig cell tumor is a rare sex cord stromal tumor of the ovary. It is the most common type of all virillizing ovarian tumors, accounting for less than 0.5% of all primary ovarian neoplasm. Recently, we experienced two cases of intermediately differentiated Sertoli-Leydig cell tumor with amenorrhea and so we present it with brief review of literature.
Amenorrhea ; Female ; Ovarian Neoplasms ; Ovary* ; Sertoli-Leydig Cell Tumor* ; Sex Cord-Gonadal Stromal Tumors

The Sertoli-Leydig cell tumor is a rare sex cord stromal tumor of the ovary. It is the most common type of all virillizing ovarian tumors, accounting for less than 0.5% of all primary ovarian neoplasm. Recently, we experienced two cases of intermediately differentiated Sertoli-Leydig cell tumor with amenorrhea and so we present it with brief review of literature.
Amenorrhea ; Female ; Ovarian Neoplasms ; Ovary* ; Sertoli-Leydig Cell Tumor* ; Sex Cord-Gonadal Stromal Tumors

The amniotic band syndrome is rare congenital deformity, presumably due to rupture of amniotic sac during the early pregnancy and appears to cause fetal injury through deformation, malformation, or disruption. This syndrome is given many names yet follows a clearly defined clinical pattern. The diagnosis was based on sonographic visualization of either amniotic bands or bands associated with fetal deformation or deformities in nonembryologic distribution. Recently, we experienced a case of amniotic band syndrome, in which partial absence of cranial bone, asymmetric facial dysmorphism and amputation of right lower leg were detected. At our best knowledge, this is one of few case describing prenatal ultrasound diagnosis of amniotic band syndrome in Korea. So, we present this case with a brief review of the literatures.
Amniotic Band Syndrome* ; Amputation* ; Congenital Abnormalities* ; Diagnosis ; Infant, Newborn ; Korea ; Leg* ; Pregnancy ; Rupture ; Ultrasonography

No abstract available.

Retroperitoneal foregut duplication cyst is an extremely rare congenital malformation. Pathologically, this lesion contains both gastric mucosa and respiratory type mucosa; radiologically, it is often challenging to differentiate it from the other cystic neoplasms that present a similar appearance. We report here on a case of retroperitoneal foregut duplication cyst that was lined by both gastric and pseudostratified ciliated columnar epithelium, and it was also accompanied by a pancreatic pseudocyst. Initially, it presented with peripancreatic and intrapancreatic cystic masses in an asymptomatic 30-year-old man, and this man has since undergone surgical resection.
Adult ; Epithelium ; Gastric Mucosa ; Humans ; Mucous Membrane ; Pancreatic Pseudocyst

Endometrial adenocarcinoma in a double uterus has rarely been reported.We had a very rare case of double uterus with endometrial adenocarcinoma involving onehemiuterus and endometrial hyperplasia involving another hemiuterus. By report this case, wewant to share our experiance. The abnomal anatomy of the uterine cavities could have madeadequate biopsy difficult and endometrial cancer is clinically suspected but histology fails toconfirm the diagnosis.
Adenocarcinoma* ; Biopsy ; Diagnosis ; Endometrial Hyperplasia ; Endometrial Neoplasms ; Female ; Uterus*

Sodium nitroprusside (SNP) is an anti-hypertensive drug, commonly used to decrease the systemic vascular resistance and lower the blood pressure. When the amount of cyanide generated by the SNP exceeds the metabolic capacity for detoxification, cyanide toxicity occurs. Under general anesthesia and cardiopulmonary bypass (CPB), it may be difficult to detect the development of cyanide toxicity. In cardiac surgical patients, hemolysis, hypothermia and decreased organ perfusion, which emphasize the risk of cyanide toxicity, may develop as a consequence of CPB. In particular, hemolysis during CPB may cause an unexpected overproduction of cyanide due to free hemoglobin release. We experienced a patient who demonstrated SNP tachyphylaxis and cyanide toxicity during CPB, even though the total amount of SNP administered was much lower than the recommended dose. We therefore report this case with a review of the relevant literature.
Anesthesia, General ; Blood Pressure ; Cardiopulmonary Bypass* ; Cyanides ; Hemolysis ; Humans ; Hypothermia ; Nitroprusside* ; Perfusion ; Tachyphylaxis ; Vascular Resistance

PURPOSE: Serum level of carcinoembryonic antigen (CEA) is clinically one of the prognostic factors in the follow-up evaluation of the colorectal cancer (CRC) patient. It has been recently suggested that the bile CEA level is also useful in early detection of the liver metastasis of colrectal cancer. If the bile CEA is also correlated with the cancer progression or prognosis like as serum CEA, it will be another useful clinical parameter in the evaluation and treatment of CRC patients. Therefore this study is aimed to reveal the correlation of the bile CEA with the progression of tumor and to estimate the possibility of bile CEA as a useful clinical parameter. METHODS: Preoperative serum levels of CEA were measured in 58 patients of CRC who were operated in Pundang CHA hospital. The levels of bile CEA were also checked with the aspiration of bile in gall bladder at laparotomy. The positive value of CEA was settled as more than 5ng/ml. RESULTS: The 58 patients were classified into 29 cases of Dukes'AB group, 23 cases of Dukes'C group & 6 cases of Dukes'D group. The positive rates of serum CEA were 24.1% in AB group, 30.4% in C group & 66.7% in D group, and those of bile CEA were 44.8%, 56.5% & 83.3% individually. When group C was also divided into N1(13 cases) & N2 (10 cases) groups according to the number of the metastatic lymph nodes, serum & bile CEA positive rates were 15.4% & 46.1% in N1 group, and 50% & 70% in N2 group. Both of serum & bile CEA levels were all positive and markedly elevated in 4 hepatic metastasis cases of group D. CONCLUSIONS: Positive rate of bile CEA was increased according to the progression of tumor stage. Marked elevation of bile CEA was especially noted in liver metastatic cases. Therefore bile CEA can be considered as a clinical parameter in evaluation of cancer progression & prognosis like as serum CEA, and also as a useful indicator of hepatic metastasis.
Bile* ; Carcinoembryonic Antigen* ; Colorectal Neoplasms* ; Follow-Up Studies ; Humans ; Laparotomy ; Liver ; Lymph Nodes ; Neoplasm Metastasis ; Prognosis ; Urinary Bladder

Colonic palyps are one of the most risky factors for colon cancer. The pathology of the specimen obtained by forceps biopsy does not represent the whole specimen of the polyp obtained by polypectomy or surgery in some cases. To evaluate these pathologic differences. we analysed the 39 patients with colonic polyps who underwent forceps biopsy and polypectomy. (continue...)
Biopsy* ; Colon* ; Colonic Neoplasms ; Colonic Polyps* ; Humans ; Pathology ; Polyps ; Surgical Instruments*