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

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

The localizations and morphological characteristics of gonadotropes in the adenohypophy-sis of Korean native goat were investigated with double immunohistochemistry. The gonadotropes were present in the pars distalis and pars tuberalis, but not in the pars intermedta. Gonadotropes occupied about 49.0% of the cells in the pars distalis in females, and about 40.8% in males. Three types of gonadotropes ; FSH immunoreactive cells[FSH cells], LH immunoreac-tive cells[LH cells], and FSH and LH immunoreactive cells[FSH/LH cell], were identified according to their immunoreactivities for FSH and LH antisera. The possessional perce-ntages of FSH cells, LH cells and FSH/LH cells were 1.1%, 40.6%, 58.3% in females and 1.8%, 30.0%, 68.8% in males, respectively. FSH/LH cells were large and oval or round in shape. These cells were distributed throughout the pars distalis, but were more abundant on the dorsal part adjacent to the hypophyseal cavity and along the lateral and ventral peripheral regions. LH cells were smaller than other gonadotropes and were observed throughout the pars distalis, but predominant in the central region. FSH cells were large and oval in shape. These cells were intercalated between FSH/LH cells.
Female ; Goats* ; Gonadotrophs ; Humans ; Immune Sera ; Immunohistochemistry ; Male

PURPOSE: Pemphigus vulgaris, a rare autoimmune blistering disease of the skin and mucous membranes remains a challenging disease to treat. Management is focused on immunotherapy against autoimmune antibodies that target keratinocyte cell adhesion molecules, and antibiotics preventing secondary infections. There is no established dressing protocol and skin is usually manipulated the least amount possible in order to minimize irritation. The authors suggest that early initiation of aggressive bathing and debridement of skin lesions, with nutritional support, is essential in accelerating resolution. METHODS: A 40 year-old male previously diagnosed with pemphigus vulgaris was admitted due to exacerbation of mucocutaneous lesions involving the epidermis and mucosa of the whole body. Steroids, immunosuppressants, intravenous immunoglobulin and antibiotics were administrated, but infection and de-epithelialization progressed, while his general condition deteriorated with a weight loss of over 20 kilograms. The plastic surgery department intervened with daily bathing, debridement of unhealthy debris and non-traumatizing coverage of growing epithelium. Total parenteral nutrition and mobilization with rehabilitation therapy was initiated as early as possible. RESULTS: After bathing, healthy epithelium gradually covered the patient's entire body, while his general condition improved with a corresponding weight gain of 14 kgs. CONCLUSION: Treatment of pemphigus vulgaris focuses on immunotherapy and infection control. However, an equal amount of attention should be laid on early intervention with daily dressings including bathing and irrigation, nutritional support, and exercise as this accelerates resolution of existing infections, promotes healthy epithelialization and leads to faster recovery.
Anti-Bacterial Agents ; Antibodies ; Bandages ; Baths ; Blister ; Cell Adhesion Molecules ; Coinfection ; Debridement ; Early Intervention (Education) ; Epidermis ; Epithelium ; Humans ; Immunoglobulins ; Immunosuppressive Agents ; Immunotherapy ; Infection Control ; Keratinocytes ; Male ; Mucous Membrane ; Nutritional Support ; Parenteral Nutrition, Total ; Pemphigus ; Skin ; Steroids ; Surgery, Plastic ; Weight Gain ; Weight Loss

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

No abstract available.
Dysautonomia, Familial*

PURPOSE: To evaluate the effect of intravitreal expansile gas (C3F8) with anti-VEGF injection for the treatment of large submacular hemorrhage (SMH) secondary to age-related macular degeneration (ARMD). METHODS: In this report, 18 eyes of 18 patients with large SMH secondary to ARMD were treated with a simultaneous injection of 0.3 cc C3F8 and 0.05 ml anti-VEGF intravitrealy. RESULTS: The mean age was 64.89 +/- 5.68 years and the mean size of SMH was 4.44 +/- 1.25 disc diameters (DD). The minimum follow-up period was 12 months (range: 12-17 months). Mean preoperative best corrected visual acuity (BCVA) was 1.72 +/- 0.56 log MAR which improved significantly to 1.01 +/- 0.68 log MAR at 12 months (p = 0.002). SMH displacement occurred in all eyes. BCVA improved 2 or more lines in 11 eyes (61.1%) and deteriorated in 1 eye (5.6%). CONCLUSIONS: In this report, intravitreal injection of an expansible gas (C3F8) with anti-VEGF produced successful results in anatomical displacement of SMH and early visual improvement.
Displacement (Psychology) ; Eye ; Follow-Up Studies ; Hemorrhage ; Humans ; Intravitreal Injections ; Macular Degeneration ; Visual Acuity

Ischemic enteritis is caused by embolism or thrombosis of superior mesenteric artery and nonocclusive ischemia. Mesenteric venous thrombosis, drugs, and vasculitis are less frequent etiologic factors. In children, occlusion of microcirculation by fibrin thrombi initiated by endotoxemia may be an etiology. Severe abdominal pain, vomiting, and diarrhea with evidence of gross or microscopic bleeding are common presenting symptoms. Angiography may be diagnositic and permit therapeutic intervention. Revascularization with resection of necrotic bowel is the treatment of choice. We experienced a case of ischemic enteritis that was presented with projectile vomiting and diarrhea. Diagnosis was confirmed histologically. Radiological findings suggested multiple adhesive ileus. Laparatomy was followed by resection of the necrotic bowel.
Abdominal Pain ; Adhesives ; Angiography ; Child ; Diagnosis ; Diarrhea ; Embolism ; Endotoxemia ; Enteritis* ; Fibrin ; Hemorrhage ; Humans ; Ileus ; Ischemia ; Mesenteric Artery, Superior ; Microcirculation ; Thrombosis ; Vasculitis ; Venous Thrombosis ; Vomiting

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