Intestinal malrotation is a constellation of a wide spectrum of embryologic failures of rotation and fixation of the gut, resulting in a narrow-based attachment of the mesentery and the presence of abnormal peritoneal (Ladd's) band. These abnormalities predispose to midgut volvulus, intestinal obstruction and internal herniation. We experienced a case of intestinal malrotation complicated by midgut volvulus in a 4-year-old girl who presente with abdominal pain, abdominal distension and melena. The diagnosis was made by abdominal CT scan, which showed "whirl sign." The diagnosis was confirmed by surgery. A brief review of literature ensues.
Abdominal Pain ; Acidosis ; Acidosis, Renal Tubular* ; Adolescent ; Alkalies ; Anemia, Hemolytic ; Atrophy ; Biopsy ; Chest Pain ; Child, Preschool ; Diagnosis* ; Exanthema ; Female ; Fever ; Fibrosis ; Hospitalization ; Humans ; Hypergammaglobulinemia ; Hypokalemia ; Intestinal Volvulus* ; Leukopenia ; Liver Diseases ; Lupus Erythematosus, Systemic* ; Lymphopenia ; Melena ; Mesentery ; Molecular Weight ; Multiple Myeloma ; Nephrotic Syndrome ; Neutrophils ; Potassium ; Prednisolone ; Proteinuria ; Thyroid Diseases ; Tomography, X-Ray Computed*

Intestinal malrotation is a constellation of a wide spectrum of embryologic failures of rotation and fixation of the gut, resulting in a narrow-based attachment of the mesentery and the presence of abnormal peritoneal (Ladd's) band. These abnormalities predispose to midgut volvulus, intestinal obstruction and internal herniation. We experienced a case of intestinal malrotation complicated by midgut volvulus in a 4-year-old girl who presente with abdominal pain, abdominal distension and melena. The diagnosis was made by abdominal CT scan, which showed "whirl sign." The diagnosis was confirmed by surgery. A brief review of literature ensues.
Abdominal Pain ; Acidosis ; Acidosis, Renal Tubular* ; Adolescent ; Alkalies ; Anemia, Hemolytic ; Atrophy ; Biopsy ; Chest Pain ; Child, Preschool ; Diagnosis* ; Exanthema ; Female ; Fever ; Fibrosis ; Hospitalization ; Humans ; Hypergammaglobulinemia ; Hypokalemia ; Intestinal Volvulus* ; Leukopenia ; Liver Diseases ; Lupus Erythematosus, Systemic* ; Lymphopenia ; Melena ; Mesentery ; Molecular Weight ; Multiple Myeloma ; Nephrotic Syndrome ; Neutrophils ; Potassium ; Prednisolone ; Proteinuria ; Thyroid Diseases ; Tomography, X-Ray Computed*

No abstract available.
Bartter Syndrome*

We conducted this cohort analytic study to determine whether women with unexplained elevations of maternal serum hCG at 15-18 weeks' gestation are at increased risk for pregnancy complications and adverse perinatal outcomes. The inclusion criteria were a singleton gestation, a confirmed gestational age, and an hCG level greater than 2.0 multiples of the median (MoM). The exclusion criteria were fetal anomalies, an abnormal karyotype, molar pregnancy, and an MSAFP level greater than 2.5 multiples of the median (MoM). A group of randomly selected women with hCG levels under 2.0 MoM served as controls. Patients with elevated levels of hCG had a significantly higher risk for PIH (17.9% versus 4.5%; P <.05) and preterm delivery (17.9% versus 3.5%; P<, 05) than control. But no significant differences were observed in the incidence of intrauterine growth restriction and low birth weight and in the newborn weight. We suggested that pregnancies with unexplained elevated hCG levels should be regarded as high-risk pregnancies. And these patients require careful monitoring with adequate obstetric management.
Abnormal Karyotype ; Chorionic Gonadotropin* ; Cohort Studies ; Female ; Gestational Age ; Humans* ; Hydatidiform Mole ; Incidence ; Infant, Low Birth Weight ; Infant, Newborn ; Pregnancy ; Pregnancy Complications ; Pregnancy Outcome* ; Pregnancy* ; Pregnancy, High-Risk

Since the first description of the trisomy 9p in 1970, over one hundred cases have been described with the advanced chromosomal banding technique. Clinical findings include growth and mental retardation and facial dysmorphism. Crucial determinants of the classical features of this syndrome lie within the distal half of the chromosome 9 short arm. But this syndrome has not been reported in Korea, we are reporting a boy diagnosed by clinical features and chromosomal study that is trisomic for a partial short arm of a chromosome 9. A brief review of the literature is included.
Arm ; Chromosomes, Human, Pair 9 ; Humans ; Intellectual Disability ; Korea ; Male ; Trisomy

No abstract available.

No abstract available.
Hematoma* ; Orbit*

The surgical intervention for secondary developmental cervical spinal stenosis that results in myelopathy and radiculopathy has been modified by technical improvement to obtain more reliable enlargement of the cervical spinal canal. The authours have experienced good results with the modified open door expansive laminoplasty, so we report this article. The patients were treated by surgical intervention during the period from October 1989 to February 1991. The results obtained were as follows : 1) The average age was 54.7 years, and the sex distribution ratio of female to male was 2 : 8. 2) In clinical symptoms and symptoms of both myelopathy and radiculopathy. 3) In morphological analysis of lesions, all patients except one case had lesions over 3 levels, The lesions were spondylosis, spondylosis combined with soft disc herniation, spondylosis combined with OPLL, spondylosis combined with OPLL and soft disc herniation, OPLL and OYL in the order of frequency. 4) The operation method was as follows : after the patient was positioned in modified Concorde position, open door lamina was anchored to faced joint capsule and surrounding tissue with nylon sutures. And then the raw epidural space was covered with Lyodura by the modified Hirabayashi method. By this method, reclosure of widened spinal canal was prevented. 5) The outcomes were evaluated by JOA score difference between preoperation and post operation times. The outcome of recovery rate was 100% in five cases. Therewere no aggravated or unimproved cases. The results were excellent 8 cases, good 1 case, unchanged 0 case, poor 0 case and expired 1 case.
Epidural Space ; Female ; Humans ; Joint Capsule ; Male ; Nylons ; Radiculopathy ; Sex Distribution ; Spinal Canal ; Spinal Cord Diseases ; Spinal Stenosis* ; Spondylosis ; Sutures

No abstract available.

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch