No abstract available.
Bilirubin* ; Fetal Blood* ; Hyperbilirubinemia, Neonatal*

No abstract available.
Primary Myelofibrosis*

Holt-Oram Syndrome, well described by Holt and Oram in 1960, is an autosomal dominant disorder consisting of upper extremity and cardiovascular anomalies with variable expression. A typical case of this syndrome was experienced by authors recently and is to be presented with review of literatures.
Upper Extremity

No abstract available.
Echocardiography* ; Endocarditis*

We experienced a case of transient complete A-V block, which developed probably due to acute myocarditis of viral etiology. The patient was 13 year old boy, and was admitted to our ward with chief complaints of intermittent abdominal pain and chest discomfort. The auscultation of the heart revealed, the 1st heart sounds varied in intensity, and the E.K.G. findings, checked then, showed complete A-V block with atrial rate of 110/min and ventricular rate of 73/min. We recommended absolute bed rest and oral prednisolove(50mg/day). He discharged on 10th hospital day in good condition with normalized E.K.G. finding.
Abdominal Pain ; Adolescent ; Auscultation ; Bed Rest ; Heart ; Heart Sounds ; Humans ; Male ; Myocarditis ; Thorax

The authors presented a 17 months old female with malignant osteopetrosis, who was admitted to the Pediatric Department of Kyung Hee University Hospital on May, 1981 because of epistaxis and growth retardation. Diagnosis was made by typical clinical manifestations, hematologic and radiologic findings, such as frontal bossing, opened anterior fontanel, exophalmoses, strabismus, optic atropy, marked hepatosplenomegaly, severe anemia, thrombocytopenia, reticulocytosis and typical mask sign and sun burst sign at the skull X-ray, wide dense ribs with fracture at the vertebrae, clubbings at the distal parts of the femurs and radii and at the proximal parts of the tibias and humerii transverse bands at the metaphyses, and finally increased density of the entire skeleton on the bone scanning and marked decrease uptake of 99mTc on the bone marrow scanning. Prednisolone was administrated orally, resulted in gradual improvement of hematologic abnormalities to the normal range, associated wited with increased cellularity of the bone marrow and increased uptake of 99mTc and decreased spleen size on the bone marrow scanning after one month of therapy.
Anemia ; Bone Marrow ; Cranial Fontanelles ; Diagnosis ; Epistaxis ; Female ; Femur ; Humans ; Infant ; Masks ; Osteopetrosis* ; Prednisolone ; Reference Values ; Reticulocytosis ; Ribs ; Skeleton ; Skull ; Solar System ; Spine ; Spleen ; Strabismus ; Thrombocytopenia ; Tibia

The authors presented a 17 months old female with malignant osteopetrosis, who was admitted to the Pediatric Department of Kyung Hee University Hospital on May, 1981 because of epistaxis and growth retardation. Diagnosis was made by typical clinical manifestations, hematologic and radiologic findings, such as frontal bossing, opened anterior fontanel, exophalmoses, strabismus, optic atropy, marked hepatosplenomegaly, severe anemia, thrombocytopenia, reticulocytosis and typical mask sign and sun burst sign at the skull X-ray, wide dense ribs with fracture at the vertebrae, clubbings at the distal parts of the femurs and radii and at the proximal parts of the tibias and humerii transverse bands at the metaphyses, and finally increased density of the entire skeleton on the bone scanning and marked decrease uptake of 99mTc on the bone marrow scanning. Prednisolone was administrated orally, resulted in gradual improvement of hematologic abnormalities to the normal range, associated wited with increased cellularity of the bone marrow and increased uptake of 99mTc and decreased spleen size on the bone marrow scanning after one month of therapy.
Anemia ; Bone Marrow ; Cranial Fontanelles ; Diagnosis ; Epistaxis ; Female ; Femur ; Humans ; Infant ; Masks ; Osteopetrosis* ; Prednisolone ; Reference Values ; Reticulocytosis ; Ribs ; Skeleton ; Skull ; Solar System ; Spine ; Spleen ; Strabismus ; Thrombocytopenia ; Tibia

Tracheoesophageal fistula is a rare disease of abnormal communication between esopha- gus and respiratory system. The common causes are the acquired origins in adult such as trauma, infection of the adjacent organs, malignant tumor, and foreign body. Among the traumatic origins, chemical drug, the procedure of the dilatation on the stenotic area, blunt trauma(fall, collisions), penetrating trauma(bullet, knife), and pressure injury are much more common than others. Recently, trauma and foreign body in the esophagus and bronchus are becoming the main cause of the tracheoesophageal fistula, however the frequency of the development of tracheoesophageal fistula caused by the infectious diseases is getting decreased. Fibrinogen-thrombin glue stimulates the healing process of the wound and the ulcer. We treated a 52-year-old male patient with nan-inalignant tracheoesophageal fistula, who had symtoms of pharyngolaryngeal and chest discomfort concomitant with a paroxysmal cough on swallowing food which were caused by fish bone. The diagnosis of tracheoesophageal fistula was made by the esophagogram, chest CT, and esop aecopy. By using the therapeutic endoscopy with an injection of the fibrinogen-glue, the tracheoesopeal fistula was obliterated completely with dramatic symptomatic improvement. Here we conqluded that this method would be the one of the best methods for the treatment of tracheoesophageal fistula.
Adhesives* ; Adult ; Bronchi ; Communicable Diseases ; Cough ; Deglutition ; Diagnosis ; Dilatation ; Endoscopy ; Esophagus* ; Fibrinogen* ; Fistula ; Foreign Bodies ; Humans ; Intestines* ; Male ; Middle Aged ; Rare Diseases ; Respiratory System ; Stomach* ; Thorax ; Thrombin* ; Tomography, X-Ray Computed ; Tracheoesophageal Fistula* ; Ulcer ; Wounds and Injuries

BACKGROUND: The purpose of this study was to report outcomes of congenital hallux varus deformity after surgical treatment. METHODS: We evaluated ten feet of eight patients with a congenital hallux varus deformity, including four feet combined with a longitudinal epiphyseal bracket (LEB). There were seven male patients and one female patient with a mean age of 33 months (range, 7 to 103 months) at the time of surgery. Two patients were bilaterally involved. The mean duration of follow-up was 5.9 years (range, 2.3 to 13.8 years). Clinical outcomes were assessed according to the criteria of Phelps and Grogan. Surgical procedures included the Farmer procedure, the McElvenny procedure or an osteotomy at the first metatarsal or proximal phalanx. RESULTS: The clinical results were excellent in two feet, good in six and poor in two feet. The LEB was associated with hallux varus in four feet and were treated by osteotomy alone or in conjunction with soft tissue procedure. CONCLUSIONS: Congenital hallux varus was successfully corrected by surgery with overall favorable outcome. Preoperatively, a LEB should be considered as a possible cause of the deformity in order to prevent recurrent or residual varus after surgery.
Child ; Child, Preschool ; Female ; Foot Deformities, Congenital/radiography/*surgery ; Hallux Varus/radiography/*surgery ; Humans ; Infant ; Male ; Osteotomy

BACKGROUND/AIMS: Hilar cholangiocarcinomas have an extremely poor prognosis. Although endoscopic bilateral metal stenting with the "stent in stent" technique using a Y stent is currently employed to treat patients with an unresectable hilar cholangiocarcinoma, this method has limited application in cases of tight strictures. Furthermore, insertion of stents into the Y stent side (first stent side) is problematic in cases of tumor recurrence. We evaluated the clinical efficacy of the use of endoscopic bilateral metal stenting with the Zilver stent. METHODS: Seven patients with an unresectable Bismuth type III or IV hilar cholangiocarcinoma were included in this study. For endoscopic bilateral metal stenting, we first inserted a Y stent with a central wide-open mesh. The Zilver stent was placed into the contralateral hepatic duct through the central portion of the Y stent. RESULTS: Both technical and functional success was achieved in seven patients (100%). The early complication rate was 0%, and late complications due to tumor recurrence occurred in 2 out of 7 (28.6%) patients. These patients were managed by the placement of additional stents or with the use of percutaneous transhepatic biliary drainage. CONCLUSIONS: We suggest that a combination technique using Y and Zilver stents improves bilateral stenting for patients with advanced hilar cholangiocarcinoma and facilitates stent reinsertion in cases of tumor recurrence.
Bismuth ; Cholangiocarcinoma ; Constriction, Pathologic ; Hepatic Duct, Common ; Humans ; Prognosis ; Recurrence ; Stents