PURPOSE: To evaluate types of gray matter heterotopias, associated brain anomalies, and its correlation with the patterns of seizure. MATERIALS AND METHODS: We evaluated retrospectively 19 patients(male :female=10:9, mean age 21 years) with gray matter heterotopias on brain MRI. Using 1.0T superconducting MR unit, spin echo TI-, proton-density- and T2-weighted images in axial, coronal and sagittal planes were obtained. RESULTS: Types of gray matter heterotopias were single subependymal in four patients, multiple subependymal in one, focal subcortical in eight, diffuse subcortical in two, mixed multiple subependymal and focal subcortical in four. Associated anomalies were seen in 11 patients:other neuronal migration anomalies in eight patients, corpus callosum agenesis in two, and combined holoprosencephaly and Dandy-Walker malformation in one. Fifteen patients had seizure. The patterns of seizure were not correlated with the types of heterotopias. CONCLUSION: In addition to subependymal, focal subcortical, and diffuse subcortical types, gray matter heterotopias included mixed variant of of multiple subependymal and subcortical type. Schizencephaly was the most common form of accompanying anomalies, and patterns of seizure were not correlated with types of gray matter heterotopias, even though main clinical menifestation was seizure.
Agenesis of Corpus Callosum ; Brain ; Dandy-Walker Syndrome ; Holoprosencephaly ; Humans ; Magnetic Resonance Imaging ; Malformations of Cortical Development ; Neurons ; Retrospective Studies ; Seizures

No abstract available.
Intuition* ; Nursing* ; Philosophy*

Rickets is a syndrome, characterized pathophysiologically by a failure of normal mineralization of bone and epiphyseal cartilage and clinically by skeletal deformity in growing children. The five principal causes of rickets are vitamin D deficiency, absorptive defects, renal tubular insufficiency, chronic renal insufficiency and hypophosphatasia. In addition to these causes of rickets a defective reabsorption mechanism of the proximal renal tubule is currently believed to be a factor in the development of hypophosphatemic vitamine D resistant rickets. Simple type of hypophosphatemic vitamine D resistant rickets is the most common of all the cases of rickets due to tubular insufficiency. It has a familiar incidence and is a sex-linked dominant disease. There is hypophos-phatemia and an elevated alkaline phosphatase but usually no glomerular disease or uremia. All the clinical manifestations are those of advanced rickets of the ordinary type and usually begin early. We experienced four cases of hypophosphatemic vitamine D resistant rickets which developed in a family.
Alkaline Phosphatase ; Child ; Congenital Abnormalities ; Growth Plate ; Humans ; Hypophosphatasia ; Incidence ; Kidney Tubules, Proximal ; Miners ; Renal Insufficiency, Chronic ; Rickets ; Uremia ; Vitamin D Deficiency ; Vitamins

Femoral fractures are difficult to treat in the child with head or multiple injuries. Traction may fail due to spasticity and restlessness. Poor results after closed reduction have led some authors to advocate open reduction and internal fixation which facilitates overall care. Anatomical reduction with internal fixation leads to limb overgrowth. We report our experience in anatomical open reduction of femoral fracture which had head or multiple injuries or difficult to maintain traction in children at the Kang-Nam St. Mary's Hospital. The results are as follows : 1. Among the 28 cases, there were 20 cases in male and 8 cases in female. Average age was 9.4 years. 2. Plate fixation was used in 27 cases and Kuntscher Kuentscher nail was used in one case. 3. Average union period was 11.5 weeks. There was one delayed union which was completely united in 20 weeks. 4. Average overgrowth of femur was 5.2mm. The overgrowth was related with age and handedness but it was not related with fracture site. For children who have head or multiple injuries or difficult to maintain the skin traction, open reduction with plate fixation appears the most satisfactory treatment. The overgrowth of the femur after open reduction was not significant problems clinically.
Child ; Extremities ; Female ; Femoral Fractures ; Femur ; Functional Laterality ; Head ; Humans ; Male ; Multiple Trauma ; Muscle Spasticity ; Psychomotor Agitation ; Skin ; Traction

We have experienced 11 cases of large bony defect that were treated using microsurgery since 1981. Free vascularized bone transplantation was performed in 8 of them, vascularized bone transposition in 2 cases, and free vascularized joint transplantation in the remaining 1 case. The causes of the large bony defect were primary bone tumor (4 cases), congenital pseudoarthrosis (3 cases), open comminuted fracture (2 cases), sequela of osteomyelitis (1 case), and post-traumatic ankylosis of PIP joint of second finger (1 case). As a donor, fibula was used in 8 cases, iliac crest in 1 case, rib in 1 case and in the remaining 1 case, the second M-P joint of foot was transplanted. In 9 of 11 cases, successful result was obtained and 2 cases were failed because of vascular damage following tibial lengthening in one case and infection on the grafted area in the other one. From the above data and review of articles, following conclusions were obtained. 1. Fibula is thought to be the most appropriate donor for the large bony defect in the extremity, especially in lower extremity, but the donor site must be determined according to the anatomical and physilogical condition of the patient. 2. After mechanical lengthening of the extremity, it is recommened to perform the microvascular surgery after sufficient time for the recovery of vascular damage. But further studies are required for the identification of the change in the vascular tissue following stretching and its recovery time. 3. Progression of the ossification in the epiphysis of transplanted iliac crest was observed and this finding proposed us the idea that the epiphyseal plate injury or leg length discrepancy will be able to be treated with free vascularized epiphyseal plate transplantation and the reconstruction of the destroyed joint of growing children will be possible using free vascularized joint transplantation. 4. As the technique become more popular, the free vascularized bone transplantation is being used for the reconstruction of the extremity more frequently, but it seems to be wise to restrict its indication to cases which are impossible to be treated with more simple methods such as vascularized bone transposition or pedicled bone graft.
Ankylosis ; Bone Transplantation ; Child ; Epiphyses ; Extremities ; Fibula ; Fingers ; Foot ; Fractures, Comminuted ; Growth Plate ; Humans ; Joints ; Leg ; Lower Extremity ; Microsurgery ; Osteomyelitis ; Pseudarthrosis ; Ribs ; Tissue Donors ; Transplants

Elucidation of the mechanism of fracture seems to be important to prevent to it and determin the position on immobilization most conducive to union. The scaphoid is more susceptable to injury than any of other carpal bones due to its position in bridging the proximal and distal raws of carpal bones and the mechnism of fracture has been a subject of considerable controversy. Reviewing 20 cases of scaphoid nonunion, we incidentally had found that 4 patients had the history of striking a punch bag with the heads of the second and third metacarpals (Punching injury), and a review of the literatures failed to find any reports of such an injury as a mechanism of fracture. On a scale, the wrist was subjected to progressive loading via the heads of the second and third metacarpals and lateral roentgenograms of the wrist were obtained at Okg, 20kg and 35kg and we measured the carpal angles. With progressive loading there is a volar flexion of proximal carpal raw and the volar flexion of scaphoid is smaller than that of lunate. We believed that the violent impact applied at the second and third metacarpal heads forced the scaphoid into the position of extreme dorsiflexion as compared with lunate, so compression force is exerted on the dorsal side of scaphoid and tension on volar side leading the fracture of scaphoid. We also recognized that the fracture of the scaphoid could be occurred by the compression and shearing force of capitate and the bending force of volar radiocapitate ligament.
Carpal Bones ; Head ; Humans ; Immobilization ; Ligaments ; Metacarpal Bones ; Strikes, Employee ; Wrist

The majority of cystic lesions of the pancreas are psudocysts and a small fraction neoplastic. Failure to recognize the true nature of neoplastic cyst will lead to an incorrct treatment strategy. Ultrasonography, computerized tomography and angiography were used to distingish these lesions, but diagnostic value of ERCP is in controversy. To evaluate the diagnostic value of ERCP in cystic lesions of the pancreas, we analysed 33 cases of pancreatic cystic lesions (pseudocyst 18 cases, retention cyst 3 cases and cystic, neoplasm l2 cases) between Apr. 1985 and June 1993. In 18 cases of pseudocysts, ERP findings were communication with cyst in 8 cases (44.4%), chronic pancreatitis in 8 cases (44.4%), obstruction in 4 cases (22.2%) and displscement of pancreatic duct in 2 cases (11.1%), and ERC findings, which were perfomed in 8 cases, showed cholangitis in 3 cases (37.5%), CBD stone in 2 cases (25%), mass effect in 1 case (12.5%) and normal in 2 cases (25%). There was no communication with the cyst and pancreatic duct, except two mucinous ductal ectasia, in 12 cases of cystic neoplasms, and the other findings were displacement of pancreatic duct in 4 cases (33.3%), obstruction in 2 cases (16.7%) and normal in 4 cases (33.3%). ERC findings of cystic neoplasm were almost normal (85.7%) except 1 case of cholangitis. In conclusion, ERCP findings of pseudocysts were communication with pancreatic duct, chronic pancreatitis and biliary tract abnormality. In contrast, ERCP findings of cystic neoplasms were displacement or obstruction of pancreatic duct without communication and chronic pancreatitis, and biliary tract abnormality were rare.
Angiography ; Biliary Tract ; Cholangiopancreatography, Endoscopic Retrograde* ; Cholangitis ; Dilatation, Pathologic ; Mucins ; Pancreas ; Pancreatic Cyst* ; Pancreatic Ducts ; Pancreatitis, Chronic ; Ultrasonography

Bone marrow transplantation has become an accepted treatment for malignancy(particulary leukemia and lymphoma), aplastic anemia, and certain inborn errors of metabolism. In addition to the problem of severe, prolonged myelosuppression, bone marrow transplantation is associated with several unusual complications. Among the complications such as GVHD, graft rejection, interstitial pneumonia and veno-occlusive disease, involvement of the gastrointestinal tract by GVHD is associated with high graft failure and mortality. Intestinal GVHD is usually manifest clinically as voluminous secretory diarrhea accompanied by abdominal cramping, ileus, nutritional depletion, and, at times, hemorrhage. We experienced a case of severe intestinal GVHD after allogeneic marrow transplantation for treatment of severe aplastic anemia. He received bone marrow from his elder sister, HLA-matched multiparous woman and suffered from large amount of watery diarrhea with skin rash 34 days after transplantation. 1n spite of prednisolone therapy the symptom was progressed. After sigmoidoscopic mucosal biopsy, intestinal GVHD was confirmed and we tried methylprednisolone pulse therapy. Skin lesion was improved but the amount of diarrhea was increased with intermittent abdominal cramping. We tried ALG(anti-lymphocyte globulin) and conservative management but the patient did not respond the therapy. He succumbed to pneumonia and acute respiratory insufficiency complicated with GVHD, 70days after transplantation.
Anemia, Aplastic* ; Biopsy ; Bone Marrow Transplantation* ; Bone Marrow* ; Colic ; Diarrhea ; Exanthema ; Female ; Gastrointestinal Tract ; Graft Rejection ; Hemorrhage ; Humans ; Ileus ; Leukemia ; Lung Diseases, Interstitial ; Metabolism, Inborn Errors ; Methylprednisolone ; Mortality ; Pneumonia ; Prednisolone ; Respiratory Insufficiency ; Siblings ; Skin ; Transplants

Primary cancer af the duodenum is rare. An extensive experience with fiberoptic eaophag-ogastro-duodenoscopy in our institution has indicated that duodenal cancer may be more frequent thsn suspeeted and readily diagnesed by this modality, With this in mied, clinical, pathological, diagnostic and therapeetie aspects of the 26 patients with primary duodenal cancer which had been endoscopically diugnosed were reviewed. The results obtained were as follows: 1) Ages ranged from 16 to 75, with the mean of 55.2 years. Seventeen patients were men and nine patients women being l. 9:1 of male-female ratio. 2) Hndoscoyic findings were 46. 1 percent ulcerative anld 34. 8 per cent ulcerative-infiltrative, 3) The diatribution within the duodenum was 50 per cent suprapapillary, 42. 4 per cent peri- papillary aecl 7. 6 per cent infrapapillary. 4) The twenty-three patients had adenocarcinoma, two patients leiomyosarcoma and 1 patient lymphoma. 5) Twenty patients were confirmed by duodenoseopic biipsies and six patients by operations. 6) The mast common presenting mmyliiints were abdominal pain, chronic blood lais leading anemia, jaundice, anorexia, indigestion, weight lass and palpable abdominal mass. 7) Of the 26 patients, eight patients underwent attempted curative resection, six patients underwent palliative bypasa procedures or exploratory laparotomy.
Abdominal Pain ; Adenocarcinoma ; Anemia ; Anorexia ; Diagnosis* ; Duodenal Neoplasms ; Duodenum ; Dyspepsia ; Female ; Humans ; Jaundice ; Laparotomy ; Leiomyosarcoma ; Lymphoma ; Male ; Ulcer

The purpose of this study was to collect basic data on the prevention of and education about diabetes mellitus for the nutritional management of a diabetes mellitus risk group. The study which took place in Kangbukgu, Seoul, involved a diabetes mellitus risk group (DMR > or = 110 mg/dL, 61), of males and females, aged 36 to 68 years, and a group of healthy people as a control group (< 110 mg/dL, 183), using luting blood sugar (FBS) levels. The proportion of people in the abnormal range was higher in the DMR than that of control group for total cholesterol, high-density lipoproteins-cholesterol (HDL-C), total protein, glutamic oxaloacetic transaminase (GOT), glutamic pyruvic transaminase (GPT) and creatinine. Particularly with respect to serum protein the proportion in the DMR in abnormal range (p<0.05) was significantly higher than that of the control group. The proportion in the DMR with a family history of disease was significantly higher than that of the control group (p<0.01). Using body mass index (BMI), waist-hip ratio (WHR) and relative body weight (RBW), the obesity indices in the DMR was higher than that of the control group. Comparing the DMR and the control group with respect to dietary habits, it seems that the DMR had more undesirable dietary habits than the control group. When the intake of each nutrient for the DMR and the control group was compared to the Korean recommended dietary allowances (RDA), the proportion of excess intake and deficient intake in the DMR was higher than that of the control group. The DMR showed a greater undesirable dietary intake pattern as compared to that of the control group, based on the RDA. With respect to the dietary diversity score (DDS) and the mean adequacy ratio (MAR) for quality estimation of the overall flood intake, the DMR showed a feater undesirable pattern than the control group. According to the above results, the DMR tended to have more undesirable eating habits when compared to the control group. Therefore, to provide a more efficient nutritional education program for the DMR we must conduct lurker studies on eating habits, so as to provide systematic nutritional management based on theme differences between the DMR and the control group.
Alanine Transaminase ; Aspartate Aminotransferases ; Blood Glucose ; Body Mass Index ; Body Weight ; Cholesterol ; Creatinine ; Diabetes Mellitus* ; Eating ; Education ; Female ; Food Habits ; Humans ; Male ; Nutritional Status* ; Obesity ; Recommended Dietary Allowances ; Seoul ; Waist-Hip Ratio