Clinical Study of Subarterial Ventricular Septal Defect

PURPOSE: The incidence of subarterial ventricular septal defect(SA VSD) ranges 25-30% among oriental patients with VSDs, which is greater than 5% reported in western. Natural history of the disease is characterized by progressive aortic valve prolapse(AVP), frequently subarterial VSD, we evaluated clinical characteristics emphasizing on the incidence of AVP and the degree of AI as aging. METHODS: Study subjects consisted of 140 patients, who were diagnosed as subarterial VSD and operated in Seoul paik Hospital during a 5 year period from Jan.1988 to Dec. 1992. The data were analyzed detrospectively as to clinical profiles, data of cardiac catheterization, frequencies of AVP, and AI in 5 each age group, operative methods, postoperative complications and mortality. RESULTS: The incidence of subarteial VSD was 34.6% of total operated VSD cases. Data of preoperative cardiac catheterization showed mean values of Qp/Qs and systolic pulmonary artery pressure, 1.43+/-0.47 and 33.8x16.4mmHg in each. Aortic valve prolapses and aortic insufficiencies were observed in 70.0% and 20.7% among patients, which showed increasing tendencies as ages increased. As operative methods, patch closures through main pulmonary artery were done mainly. In mild cases without AI or with grade I AI, simple VSD closures were performed but in more a advanced cases, 10 aortic valvuloplasties and additional 2 aortic valve replacements were performed. Total mortality rate was 2.1%. CONCLUSIONS: In the management of subarterial VSD, early elective closure regardless of shunt volume is important to prevent progressive aortic valve prolapse leading to aortic insufficiency.

A Case of Remission of Systemic Juvenile Rheumatoid Arthritis(Still's Disease) Treated with High-dose Intravenous Gammaglobulin

High dose intravenous gammaglobuline (IVLG) therapy is effective in some of the autoimmune diseases. Although the exact mechanism of action of IVIG is uncertain, the action as a neutralizing antibody against unknown etiologic agents, the action of blocking of Fc receptors of effector cells, or the action as a antiidiotypic antibody are suggested. We report a case of 12 year old girl with systemic juvenile rheumatoid arthritis who was treated with high dose IVIG and got a remission. In August 1990 she was admitted to our hospital. because of intermittent fever, transient rash and multiple arthralgia. Under the diagnosis of systemic juvenile rheumatoid arthritis, aspirin (4.0g/day) had been given with symptom improvement. She was readmitted in October 1990 because of aspirin intoxication and acute fulminant hepatitis. She was discharged after recovery and any medicine was not prescribed. In November 1990 she was admitted because of epigastric pain, vomiting, intermittent fever, multiple arthritis, and mild hepatomegaly. Total parenteral alimentation had been given under the diagnosis of superior mesenteric artery syndrome and gold sodium thiomalate (Myochrysine, 5 and 10 mg, two weekly IM injection) was given in conjunction with prednisolone (30 mg/day) and naproxen (375 mg/day). She was admitted again in February 1991 due to the fever, coughing, rash, and hepatosplenomegaly. Pneumonia and leukopenia (2100/mm(3)) were found and gold sodium thiomalate injection was discontinued. Gammaglobulin 1 g/kg/day was given intravenously for 2 consecutive days with dramatic symptom improvement. Five more monthly IV gammaglobulin had been given and the side reaction of injection were nausia, fever, and headache which were controlled by the decrease of infusion rate. Four months after the last IVIG injection she had no symtom of arthritis and the hepatosplenomegaly was decreased. Hemoglobin level was increase to 12.2 mg/dL form 6.2mg/dL and ESR was decrease to 15mm/h. The oral prednisolne and ibuprofen were stopped one year after th last IVIG injection. All the laboratory parameters of arthritis and physical examinations had been normal for more than two year after the stop of all the medications until March of 1994. We suggest that high dose intravenous gammaglobulin can be one of treatments for severe systemic juvenile rheumatoid arthritis.

The Effect of Cranial Irradiation of Growth of Children with Lymphatic Origin Tumors

A retrospective study was conducted to investigate the effects of CNS prophylaxis with 1,800 cGy cranial irradiation on standing height growth of children with acute lymphoblastic leukemia (ALL). Seventeen longterm survivors (DFS over 5 years ) of ALL and Non-Hodgkins Lymphoma who had been admitted at pediatric department of Inje University Seoul Paik Hospital from January 1982 to September 1988 were studied. The long term survivors were divided into two groups by prophylactic treatment modality; intrathecal chemotherapy alone (Group I, 7 cases), intrathecal chemotherapy with cranial irradiation (Group II, 10 cases). The heights of each groups were compared normal standard growth of the sane aged children by mean SD score (The SD score was calculated with the use of the following formula). SD score=(x-x)/SD (x; height measurement, x; mean height for age of the normal population SD; standard deviation). The results were as follows; 1) The mean SD scores of Group I and Group II at diagnosis were -0.30 and -0.17, which were smaller than height of normal child. 2) In Group 1, the mean SD scores after the end of treatment were -0.14 of 1 year, 0.03 at 2 years and 0.01 at 5 years, which suggests that were normal height velocity and catch-up growth in these patients (P<0.05). The height of 1 year after the beginning of treatment were particularly smaller than that of the normal children (P<0.05). 3) The mean SD scores 5 years after the end of treatment were 0.10 in Group I and -0.17 in Group II, which shows strong tendency to statistical difference among these two groups. If larger numbers of patients are evaluated, it, however, may turn significant. In conclusion, 1,800 cGy cranial irradiation in ALL children significantly affects on height growth. We recommend to evaluate growth hormone for patient with 1,800 cGy cranial irradiation and to treat them with growth hormone.