No abstract available.
Leukemia, Basophilic, Acute*

This study is based on 155 patients of congenital me colon. For the diagnosis, 93 cases were histologically proven and the remaining 63 cases were diagnosed on clinical basis including barium enema or surgical gross findings. On histologic examination, 80 cases(86%) showed typical features of absence of ganglion cell in the myenteric plexus and the 13 cases(14%) had atypical features which were segmental absence Of ganglion cell in one case. There we 127 males(82%) and 28 females(18%). The age at diagnosis was younger than 30 days in 87 cases(56%), I month to 1 year in 39 cases(25%) and older than I year in 29 cases(18%). The levels of aganglionosis were variable: short segment (rectosigmoid) in 134 cases(86%), intermediate segment (more proximal colon) in 14 cases(100%). and 6 cases(4%) had total aganglionosis. Common clinical presentations were abdominal distention, delayed meconium passage or bilious vomiting in neonate, and chronic constipation in infancy or childhood. Following initial colostomy or ileostomy, a definitive procedure was performed in 151 cases(Duhamel type in 150 cases; Soave type in 2 cases; Swenson type in 3 cases). Frequently associated problems after definitive procedure were persistent constipation(ll%) due to septum formation, fecaloma, remnant aganglionic segment and rectal stenosis. Overall mortality rate was 4%, and increased mortality was associated with enterocolitis(14%) which was the most frequent cause of death. The follow-up study longer than 3 months was available in 138 patients who underwent a definitive procedure(mean 2 year 11 months). Seventy-three cases(53%) had normal bowel function, 38cases(27.5%) had occasionally used enema or stool softners, and 27 cases(19.5%) had severe constipation or soiling. The bowel habit improved with time, and were considered normal in 60% of patients after follow-up more than 3 years. The results of definitive procedures for congenital megacolon including Duhamel operation was satisfactory, and long-term follow-up appeared an important and critical component of patients'care.
Child ; Male ; Female ; Infant, Newborn ; Humans ; Mortality ; Follow-Up Studies

Chronic idiopathic intestinal pseudo-obstruction syndrome is a clinical condition induced by an impaired function of intestinal motility. Although its clinical symptoms are those of intestinal obstruction, mechanical obstruction of the intestine cannot be found by vigorous studies, even by operative exploration. We have experienced nine cases of chronic idiopathic intestinal pseudo-obstruction syndrome. It will help in diagnosis and treatment of the pseudo-obstruction to present the patients with these syndromes. In all cases severely delayed intestinal transit times were noted in radiological gastrointestinal studies without evidence of mechanical obstruction. The most frequent tentative diagnoses were congenital megacolons. But all of the patients showed the presence of ganglion cells in the intestinal wall. Histological abnormalities of the muscle layer were found in three patients. We could not resolve the symptoms by the surgical decompression of the intestine (such as colostomy, ileostomy etc.). But the symptoms were improved after total colectomy in a patient whose main affected site was confined to the colon. The megacystis was the most frequent combined anomaly. All patients had been improved with NPO and total parenteral nutrition therapies were inevitable. The drugs which facilitate the intestinal activities such as cisapride could not resolve the symptoms of obstruction. Three patients were died with the complications of total parenteral nutrition, and four patients improved clinically. Early diagnosis and proper management will reduce the mortality and morbidity.
Child ; Cisapride ; Colectomy ; Colon ; Colostomy ; Decompression, Surgical ; Diagnosis ; Early Diagnosis ; Ganglion Cysts ; Gastrointestinal Motility ; Hirschsprung Disease ; Humans ; Ileostomy ; Intestinal Obstruction ; Intestinal Pseudo-Obstruction* ; Intestines ; Mortality ; Parenteral Nutrition, Total

To investigate the effect of additional factor XIII on the rate of cross-linked fibrin formation in fibrin glue, we prepared concentrated fibrinogen as a source of fibrin glue using thaw -syphon method from fresh frozen plasma, purified human factor XIII from plasma, and performed biochemical analysis of the fibrin glue formed with or without additional factor XIII. It was concluded that additional factor XIII promoted rapid formation of cross-linked fibrin (gamma-gamma dimer and a-polymer) which would be essential to clinically effective fibrin glue.
Factor XIII* ; Fibrin Tissue Adhesive* ; Fibrin* ; Fibrinogen ; Humans ; Plasma

No abstract available.
Dysautonomia, Familial*

Intractable ulcerating enterocolitis of infancy is uncommon, inhereditary disease characterized by ulcerating stomatitis, severe perianal disease, affecting the whole gastrointestinal tract, mainly colon with flask shaped large ulcer. It was first described by Sanderson et al in 5 cases of infant with intractable diarrhea having above clinical manifestation. It should be differentiated with Crohn's disease and Behcet's disease. We experienced a case of intractable ulcerating enterocolitis in an infant. A 17 month old patient was admitted because of intractable diarrhea since 2 months of age. Radilogical and endoscopic examination revealed chronic ulcerative inflammation with pseudopolyps involving ileum and entire colon. Ileocolectomy was performed because of its unresponsiveness to medical theraphy. The histology of resected specimen showed large flask shaped ulcer with underlying edge in the colon, terminal ileum. No evidence of granuloma suggesting Crohn's disease or vasculitis suggesting Behcet's colitis were noted. We report this case as an example of Intractable ulcerating enterocolitis (Sanderson et al).
Colitis ; Colon ; Crohn Disease ; Diarrhea ; Enterocolitis ; Gastrointestinal Tract ; Granuloma ; Humans ; Ileum ; Infant* ; Inflammation ; Stomatitis ; Ulcer* ; Vasculitis

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Infant ; Male ; Female ; Humans

Removal of excess cement is important to prevent biological complication in cementation of implant restoration with subgingival margin. It can be difficult to completely remove excess cement. Several techniques have been introduced to minimize excess cement using abutment replica. In this case report, a simple method for making abutment replica with hot melt adhesive material in dental office was described. This technique is simple and effective because it can be used for pre-fabricated or custom abutment without additional laboratory procedure. In addition, it can minimize excess cement after cementation of implant restoration.
Adhesives* ; Cementation ; Dental Offices ; Methods

A 62-year-old man was transferred to our emergency room because of acute encephalopathy. He was a nondrinker with history of a gastrectomy nineteen years ago. Two weeks before his visit, he had experienced transient poor oral intake due to gastroesophageal reflux (GER). His brain MRI showed definite lesions compatible with Wernicke Encephalopathy (WE). A trivial event like GER can complicate the marginal equilibrium of thiamine and, after a long latent interval following a gastrectomy, may induce delayed-onset WE.
Brain ; Emergencies ; Gastrectomy ; Gastroesophageal Reflux ; Humans ; Middle Aged ; Thiamine ; Wernicke Encephalopathy