No abstract available.
Child* ; Humans ; Pneumonia*

No abstract available.
Ischemia* ; Technetium Tc 99m Exametazime* ; Tomography, Emission-Computed, Single-Photon*

Kinky hair syndrome is a sex-linked recessively inherited copper metabolic disorder with severeneurodegenerative change and infant death. In 1962, Menkes and associates described five boys of a relatedpedigree with severe psychomotor retadation, seizures and widespread cerebral and cerebellar degeneration. In1969, Wesenberg and associated specified the radiological characterization of the syndrom. Symmetrical metaphsealspurring and diaphyseal periosteal reaction fo the long bones, anterior rib flaring, a malformed cerebral arterialsystem and subdural effusion. In 1972, Danks and associates found the disease to be associated with a defect ofcopper metabolism, confirmed by studies with labelled Cu. Authors experienced a case with characteristic clinicalpicture, and report cebral and abdominal arteriographic changes and plain radiographic findings with brain CT, DSAand post-mortem angiography.
Angiography ; Brain ; Copper ; Infant Death ; Menkes Kinky Hair Syndrome ; Metabolism ; Ribs ; Seizures ; Subdural Effusion

63 cases of meningitis in children were reviewed to study correlation between brain CT findings and clinical course. We divided 63 cases into 3 groups according to clinical course, that is , Group I :Healed without significant sequelae. Group II: Discharged with sequelae such as neurologic deficit or complicated clinical course. Group III : Expired or considered to be expired after hopeless discharge. The CT finding were retrospectively analyzed and compared with each clinical group. We drawed several conclusions as follows; 1. The wrost prognostic CT finding is dirty basal cisternal enhancement. (Group I only 5%, Group II 50%, Group III 45%).2. Focal brain parenchymal lesion, especially multiple, such as granuloma and infarct shows unfavorable clinical outcome, that is, high rate of Group III and evident neurologic deficit, in contrast to only 7% of Group I. 3. In 7 cases of which CT finding is only hydrocephalus, the prognosis is rather favorable, that is, 57% were Group I, 43% were improved after V-P shunt (Group II) and no Group III. But hydrocephalus with dirty disternal enhancement results in grave prognosis, that is, Group I only 8%, Grouop II 54%, Group III 38%, With regard to overall hydrocephalus, predilectron for good or bad prognosis can't be mentioned. 4. No prognostic difference were noted between presence and absence of periventricular low desnity in hydrocephalus. 5. CSF pressure of hydrocephalus ismostly high (over 20cm H20). but normal pressure hydrocephalus were noted in 24%. CSF pressure of normal ventriclesize is mostly under 18cm H2O) but high pressure were noted in 18% of the nomral sized venticle (most of them shows intracranial space occupying lesion such as granuloma, acute infarct, subdural effusion, etc). 6. Most of diffuse braine swelling, diffuse brain atrophy and subdural effusion result in Group I, that is, favorable clinical outcome. 7. Normal CT findings ar found in 29%, of which 61% belong to Group I and 31% to Group II.
Atrophy ; Brain ; Child ; Granuloma ; Humans ; Hydrocephalus ; Hydrocephalus, Normal Pressure ; Meningitis ; Neurologic Manifestations ; Prognosis ; Retrospective Studies ; Subdural Effusion

Congenital obstuction of pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veinsor total pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which accompanied other cardiac anomalies. Right pulmonary veins were involved in all seven cases including one bilateral case. Pulmonary veins were occluded totally in five and partially in three lungs. Pumonary catheterization and angiography were done for diagnosis. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural thickening, which were quite specific, whereas pulmonry venous dilatation was dominant findings in partial obstruction cases. Pulmonary perfusion scans (n=3) showed total perfusion defects in the cases of total occlusion of veins. MR imaging (n=2) demonstrated total occlusion of pulmonary veins in the venoatrial junction in two, and membranous focal obstruction in one lung. Two patients had pneumonectomy and histological confirmation, Although catheterization and angiography are essential for the diagnosis, MR imaging is thought to be useful for the diagnosis of pulmonary vein obstruction.
Angiography ; Catheterization ; Catheters ; Constriction, Pathologic ; Diagnosis ; Dilatation ; Drainage ; Estrogens, Conjugated (USP) ; Humans ; Hypertension ; Lung ; Magnetic Resonance Imaging ; Perfusion ; Pneumonectomy ; Pulmonary Edema ; Pulmonary Veins* ; Radiography, Thoracic ; Veins

Isolated absence of the ductus arteriosus is extremely rare condition although the ductus arteriosus may be hypoplastic or aplastic in association with other aortic arch anomalies. Authors described a case of isolated agenesis of the ductus arteriosus documented by postmortem examination of a newborn infant who died of pneumonia following operation for a large omphalocele. The heart showed ventricular septal defect. However, no other cardiovascular anomalies were associated in this case. There were three vessels that were taking off from the aorta consisted of the right brachiocephalic artery, left common carotid-artery and left subclavian artery. The anteriorly located pulmonary artery was divided into the right and left pulmonary arteries. There was no connection of vessel between the pulmonary artery and the aorta.
Abnormalities, Multiple/embryology/pathology ; Ductus Arteriosus/*abnormalities ; Heart Defects, Congenital/embryology/pathology ; Hernia, Umbilical/complications ; Humans ; Infant, Newborn ; Male

This paper reviews our five years' clinical experience (1987 to 1991) of 22 patients with inflammatory bowel disease (IBD). There were 12 patients with Crohn's disease and 10 patients with ulcerative colitis. The mean age at diagnosis was 8.7 years (2 to 14 years). Clinical impressions before referral were chronic diarrhea in 11, irritable bowel syndrome in 5, colon polyp in 4, lymphoma in 3, intestinal tuberculosis in 2, amoebic colitis in 2, ulcerative colitis in 2 children and other diseases. The mean interval from the onset of symptoms to the diagnosis of IBD was 18 months. Diagnosis of Crohn's disease was delayed for more than 13 months in 8 (67%), whereas that of ulcerative colitis was delayed for more than 13 months in 4 (40%). Diarrhea (50%), abdominal pain (36%) and rectal bleeding (36%) were the three most frequent presenting complaints of IBD. Moderately severe abdominal pain was a more common chief complaint in Crohn's disease (58%) than in ulcerative colitis (10%). Hematochezia (90% vs 17%) and moderately severe diarrhea (90% vs 75%) were more common gastrointestinal manifestations in ulcerative colitis than in Crohn's disease. The associated extraintestinal manifestations were oral ulcer in 7, arthralgia in 11 and arthritis in 4, skin lesions in 2, eye lesions in 2 and growth failure in 9 patients. Of 12 children with Crohn's disease, granuloma was found in 5, aphthous ulcerations in 8, cobble stone appearance in 8, skip area or asymmetric lesions in 6, transmural involvement in 7, and perianal fistula in 3. Among 10 children with ulcerative Colitis, there were crypt abscess in 8, granularity or friability in 10 and rectosigmoid ulcerations with purulent exudate in 8 children. The main sites of involvement in children with Crohn's disease were both the small and large bowels in 7 (58%), small bowel only in 2 (16%), and colon only in 3 (25%). Terminal ileum involvement was seen in 75% of Crohn's disease cases. The main sites of involvement in children with ulcerative colitis were total colon in 4 (40%), up to the splenic flexure in 2 (20%), rectosigmoid in 3 (30%) and rectum only in one (10%). Medical treatment including sulfasalazine, and systemic or topical steroid was administered initially in most patients. Seven of 12 patients with Crohn's disease and 2 of 10 patients with ulcerative colitis were operated on.(ABSTRACT TRUNCATED AT 400 WORDS)
Adolescent ; Child ; Child, Preschool ; Colonoscopy ; Female ; Follow-Up Studies ; Humans ; Incidence ; *Inflammatory Bowel Diseases/epidemiology/pathology/physiopathology/therapy ; Korea/epidemiology ; Male ; Recurrence

A case of rather typical Crohn's disease in a 10 year old girl is described. She had suffered from intractable abdominal pain, diarrhea and fever for 1 year. Eventual right hemicolectomy revealed diffuse involvement of terminal ileum, cecum and ascending colon by confluent ulcerations and transmural inflammation. Histologically there were numerous well developed non-caseating granulomas scattered transmurally and in regional lymph nodes. Deep penetrating ulcerations were characteristic. Acid fast staining failed to demonstrate any organism. The rarity of Crohn's disease in Korea and this occurrence in pediatric age prompted this report.
Child ; Colitis/pathology ; Crohn Disease/diagnosis/*pathology ; Diagnosis, Differential ; Female ; Granuloma/pathology ; Humans ; Ileitis/pathology ; Intestines/pathology

No abstract available.
Protein-Losing Enteropathies