Retinopathy of Prematurity and Perinatal Risk Factors

PURPOSE: The incidence of retinopathy of prematurity(ROP) which is one of the most common cause of childhood blindness has not decreased despite the restricted use of oxygen. There may be other factors responsible for ROP which could not be explained solely by improved survival of very low birth weight infants. We tried to clarify perinatal risk factors that are nrelated to the occurrence of ROP. METHODS: We enrolled 239 infants with gestational ages less than 33 wks or with birth weight less than 1,800 gm who had received ophthalrnologic examination between Jan. 1995 and Dec. 1997. The patients were categorized into two groups,' "No ROP" group as control(n=185) and ROP group as study population(n=54), and we compared the perinatal risk factors between the two. RESULTS: 54(22.6%) out of 239 infants were diagnosed as having ROP. The annual incidence of ROP decreased but the incidence of treated ROP increased. The incidence of ROP decreased with longer gestation and higher birth weight. Mean gestational age was 30.3 weeks in the study group vs 31.0 weeks in the control group, and mean birth weight was 1,390 gm and 1,586 gm, respectively. Significant factors contributing to increased incidence of ROP included: duration of oxygen therapy, ventilator therapy, total parenteral nutrition and hospitalization, frequency of hyperoxia, hypercarbia, and transfusion and respiratory distress syndrome, umbilical artery catheterization, intraventricular hemorrhage, sepsis, bronchopulmonary dysplasia, use of dexamethasone and aminophylline, pneumonia, air leak syndrome, and hyperglycemia. The incidence of ROP and treated ROP was not influenced by the mode of surfactant treatment(prophylactic vs rescue) in RDS patients and the mode of dexamethasone treatment(short vs long) in BPD patients. CONCLUSION: The risk factors for retinopathy of prematurity included gestational age, birth weight and duration of oxygen, as well as other aforementioned perinatal factors. The occurrence of ROP can be decreased by preventing preterm birth, minimizing the use of oxygen, and further morbidity can be prevented by performing proper ophthalmologic examination and doing an appropriate follow-up.

A Case of Duplication 9q Syndrome

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.

Comparison of Combined Immune Suppression, Bone Marrow Transplantation and Androgen Treatment in Childhood Severe Aplastic Anemia / 대한소아혈액종양학회지

PURPOSE: The purpose of this study is to compare treatment response and survival according to treatment modalities, such as allogeneic bone marrow transplantation (BMT), combined immunosuppression and supportive care, and according to etiologies in children with severe aplastic anemia. METHODS: Thirty-four patients who had severe aplastic anemia received either BMT, combined immunosuppression, or androgen treatment at Chonnam University Hospital from Jan. 1990 to Mar. 1998. The clinical characteristics and response according to treatment modalities or etiologies were studied retrospectively. RESULTS: 1) Patients in Group 1 received combined immunosuppression of ALG/ATG+methylprednisolone+/-cyclosporin (N=18); Group 2, BMT (N=11); Group 3, supportive treatment of oxymetholone+prednisolone+transfusion (N=5). Clinical characteristics were not different among three groups. 2) In Group 1, the response rate was 50% with complete response (CR) in 4 (22.2%) and partial response (PR) in 5 (27.8%). CR was attained in 91% (10/11) of Group 2 patients, while all 5 in Group 3 were died. Relapse was found in 1 out of 9 (11.1%) in Group 1. Three of 10 in Group 2 who had initial response experienced late graft failure. The Kaplan-Meier 4-year-survival in each group was 75.5%, 81.8% and 20%, respectively (P = 0.019). 3) Among 5 patients were Fanconi's anemia, two survived who underwent HLA- matched sibling transplants. Three patients with secondary aplastic anemia, including posthepatitis (N=2) and post-rubella responded to either BMT or immune suppression. Idiopathic cases (N=26) showed response rate of 43.8% to immunosuppression [CR, 3/16; PR 4/16], 85.7% to BMT, and 0% to supportive care. 4) The relative risk of death was 0.26 (P = 0.052) in Group 1, and 0.15 (P = 0.03) in Group 2 in comparison with Group 3. CONCLUSION: The study showed that BMT and combined immunosuppression resulted in better response and survival than supportive care in the management of childhood severe aplastic anemia. Although BMT seemed to be better than immunosuppression, effective measure to reduce late graft failure and graft versus host disease should be pursued.

A Case of Intestinal Malrotation Complicated by Midgut Volvulus: Diagnosis with Abdominal CT Scan

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.

A Case of Intestinal Malrotation Complicated by Midgut Volvulus: Diagnosis with Abdominal CT Scan

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.