• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.428-431
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• 2010

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other is acquired and associated with anti-ADAMTS-13 autoantibodies. The measurement of ADAMTS-13 activity in plasma, identification of ADAMTS-13 circulating inhibitor, anti-ADAMTS-13 IgG, and ADAMTS-13 gene sequencing are crucial to the diagnosis of TTP. Plasma exchanges are the first-line treatment for acquired TTP, combined with steroids and immunosuppressive drugs. Here, we describe the case of an adolescent patient with TTP, confirmed by decreased level of ADAMTS-13 activity and an increased level of ADAMTS-13 inhibitor, who was successfully treated by plasma exchanges.

• Pediatric Infection and Vaccine
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• v.22 no.3
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• pp.210-215
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• 2015

Macrophage activation syndrome (MAS) is a rare complication in systemic lupus erythematosus (SLE) that can be triggered by infections. Due to the fact that MAS may mimic clinical features of underlying rheumatic disease, or be confused with an infectious complication, its detection can prove challenging. This is particularly true when there is an unknown/undiagnosed disease; and could turn into an even greater challenge if MAS and SLE are combined with a viral infection. A-14-year-old female came to the hospital with an ongoing fever for 2 weeks and a painful facial skin rash. Hepatomegaly, pancytopenia, increased aspartate aminotransferase, elevated serum ferritin and lactate dehydrogenase were reported. No hemophagocytic infiltration of bone marrow was reported. The patient was suspected for hemophagocytic lymphohistiocytosis. Her skin rashes were eczema herpeticum, which is usually associated with immune compromised conditions. With the history of oral ulcers and malar rash, positive ANA and low C3, C4 and the evidence of hemolytic anemia, she was diagnosed as SLE. According to the diagnostic guideline for MAS in SLE, she was diagnosed MAS as well, activated by acute HSV infection. After administering steroids and antiviral agent, the fever and skin rash disappeared, and the abnormal laboratory findings normalized. Therefore, we are reporting a rare case of MAS triggered by acute HSV infection as the first manifestation of SLE.

• Journal of Yeungnam Medical Science
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• v.11 no.1
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• pp.42-48
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• 1994

Among the erythrocyte membrane defects, hereditary spherocytosis is the most common. The erythrocyte membrane defect results from a deficiency of spectrin, the most important structural protein in red cell. Hereditary spherocytosis often presents with hemolytic anemia, jaundice, moderate splenomegaly. Diagnosis is established by the presence of spherocytes in the peripheral blood, reticulocytosis, an increased osmotic fragility, and a negative Coombs test. In children, splenectomy is usually performed after age 6 years but can be done at a younger age if warranted by the severity of the anemia and the need for frequent transfusions. In the period December 1987 to Agust 1993, 9 patients with hereditary spherocytosis underwent splenectomy and the following results were obtained. 1. Nine patients were comprised of five males and four females. 2. Five patients(55.6%) had been admitted to our hospital during age 6-10 years. 3. Four of the nine patients had autosomal dominant inheritance with variable expression. The other five patients had no known inheritance. 4. The diagnosis of the spherocytosis was based on the increased osmotic fragility and increased autohemolysis of the erythrocytes, as well as on the appearance of spherocytes in the peripheral blood smear. 5. In all cases splenectomy was performed. Two patients had concomitant gall stones and choledocholithiasis, respectively. One patient with concomitant gall stones underwent simultaneous cholecystectomy and splenectomy. The other patient associated with choledocholithiasis underwent splenectomy, cholecystectomy, choledocholithotomy, and T-tube drainage. 6. Complete hematologic recovery was obtained by the splenectomy in all cases. 7. Postoperative complication was not occurred.

• Journal of Chest Surgery
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• v.12 no.4
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• pp.383-394
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• 1979

Treatment of valvular heart disease with valve replacement has been one of the most popular procedures in cardiac surgery recently. Although, first effort was directed toward the prosthetic valve, it soon became popular that bioprosthesis, the valvular xenograft, was prefered in the majority cases. Valvular xenograft has some superiority to the artificial prosthetic valve in some points of thromboembolism and hemolytic anemia, and it also has some inferiority of durability, immunologic reaction and resistance to Infection. Tremendous efforts were made to cover the inferiority with several methods of collection, preservation, and valve mounting of the porcine valve or pericardium of the calf, and also with surgical technique of the valvular xenograft replacement. Auther has collected 320 porcine aortic valves immediately after slaughter, and aortic cusps were coapted with cotton balls in the Valsalva sinuses to protect valve deformity after immersion in the Hanks' solution, and oxidation, cross-linking and reduction procedures were completed after the proposal of Carpentier in 1972. Well preserved aortic valves were suture mounted in the hand-made tissue valve frame of 19, 21, and 23 mm J.d., and also in the prosthetic vascular segment of 19 mm Ld. with 4-0 nylon sutures after careful trimming of the aortic valves. Completed valves were evaluated with bacteriologic culture, pressure tolerance test with tolerane gauge, valve durability test in the saline glycerine mixed solution with tolerance test machine in the speed of 300 rpm, and again with pathologic changes to obtain following results: 1. Bacteriologic culture of the valve tissue in five different preservation method for two weeks revealed excellent and satisfactory result in view of sterilization including 0.65% glutaraldehyde preservation group for one week bacteriologic culture except one tissue with Citobacter freundii in 75% ethanol preserved group. 2. Pressure tolerance test was done with an apparatus composed of V-connected manometer and pressure applicator. Tolerable limit of pressure was recorded when central leaking jet of saline was observed. Average pressure tolerated in each group was 168 mmHg in glutaraldehyde, 128 mmHg in formaldehyde, 92 mmHg in Dakin's solution, 48 mmHg in ethylene oxide gas, and 26 mmHg in ethanol preserved group in relation to the control group of Ringer's 90 mmHg respectively. 3. Prolonged durability test was performed in the group of frame mounted xenograft tissue valve with 300 up-and-down motion tolerance test machine/min. There were no specific valve deformity or wearing in both 19, 21, and 23 mm valves at the end of 3 months (actually 15 months), and another 3 months durability test revealed minimal valve leakage during pressure tolerance test due to contraction deformity of the non-coronary cusp at the end of 6 months (actually 30 months) in the largest 23 mm group. 4. Histopathologic observation was focussed in three view points, endothelial cell lining, collagen and elastic fiber destructions in each preservation methods and long durable valvular tolerance test group. Endothel ial cell lining and collagen fiber were well preserved in the glutaraldehyde and formaldehyde treated group with minimal destruction of elastic fiber. In long durable tolerance test group revealed complete destruction of the endothelial cell lining with minimal destruction of the collagen and elastic fiber in 3 month and 6 month group in relation to the time and severity. In conclusion, porcine xenograft treated after the proposal of Carpentier in 1972 and preserved in the glutaraldehyde solution was the best method of collection, preservation and valve mounting. Pressure tolerance and valve motion tolerance test, also, revealed most satisfactory results in the glutaraldehyde preserved group.

• Journal of Yeungnam Medical Science
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• v.10 no.1
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• pp.68-76
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• 1993

Pregnancy induced hypertension is multifaceted syndrome with variable involvement of several key organ systems, so sensitive and specific laboratory tests for predicting severity and prognosis, and early diagnosis of this disease are required. Because heme catabolism results in equimolar production of carboxyhemoglobin, iron and bilirubin, a concomittant rise of these parameters would provide confirmation of increased heme catabolism. Microangiopathic hemolytic anemia may occurs in severe preeclampsia, but it is not known whether increased red cell turnover occurs with mild preeclampsia as complication. The purpose of this study was to confirm that increased heme catabolism also occurs in patients with mild preeclampsia. The analysis of data was done on 23 cases with mild preeclampsia and 35 normal pregnant women, who were admitted to Yeungnam University Hospital from October 1992 to March 1993. The results were as follows. 1. The mean antepartum serum iron concentration was significantly higher in the group with mild preeclampsia($86.5{\pm}6.1{\mu}g/dl$) than in the controls($53.2{\pm}5.3{\mu}g/dl$). 2. The mean antepartum and postpartum carboxyhemoglobin concentrations were significantly higher in the group with mild preeclampsia(antepartum : $2.55{\pm}0.42{\mu}g/dl$, postpartum : $1.21{\pm}0.4{\mu}g/dl$) than the controls(antepartum : $0.61{\pm}0.2{\mu}g/dl$, postpartum $0.53{\pm}0.2{\mu}g/dl$) 3. During postpartum, carboxyhemoglobin concentration in preeclampsia reduced significantly from antepartum level, but, there was no difference between antepartum and postpartum carboxyhemoglobin concentrations among controls. 4. Bilirubin concentrations were similar in both groups.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.401-408
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• 2008

Purpose : Transcatheter closure of atrial septal defects (ASD) is currently established therapy as an alternative to surgery. But rarely, complications are reported in some studies. We report early and intermediate term complications associated with transcatheter closure of atrial septal defects using the Amplatzer septal occluder (ASO). Methods : From June 2003 to May 2006, 64 patients underwent transcatheter closure of secundum ASD or patent foramen ovale using the ASO. The ratio of male to female was 1:2.4, the median age was 17 years (range: 2.6-64 years) and their median weight was 47.5 kg (range: 2.6-64 kg). Results : The median diameter of ASD measured with transthoracic or transesophageal echocardiography was 15 mm (range: 6-28 mm), the median balloon stretched diameter was 18 mm (range: 6.5-34 mm), and the median size of device was 19.5 mm (range: 6-36 mm), was little difference with balloon stretched diameter. There were 10 cases of complications: arrhythmia (2), device malformation (2), aorta to right atrial fistula (1), hemolytic anemia (1), mitral valve encroachment (1), malposition (1), residual shunt (1), and inferior vena cava perforation (1). Conclusion : Transcatheter closure of ASD using ASO is effective and safe therapy. However, significant complications such as aorta to atrial fistula, atrial erosion, or device embolization can happen, so an appropriate selection of patient and device in relevance to size and anatomy of ASD is important for successful closure.

• The Korean Journal of Nuclear Medicine
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• v.36 no.3
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• pp.185-194
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• 2002

Purpose: We studied clinical aspects and courses of patients with pulmonary radioactivity on liver scintigraphy and speculated the mechanism of pulmonary uptake of radiocolloids. Materials and Methods: Forty-nine patients with pulmonary radioactivity were classified into 5 disease groups-liver disease, infection, cancer, ischemic necrosis of liver, etc.- and their presence or absence of chronic liver disease (CLD), Child-Pugh class, serum levels of AST and ALT, results of follow-up liver scintigraphy and clinical course were checked. Results: Of total 49 patients 25 had CLD; there were 23 liver disease patients, 16 infection patients, 7 advanced cancer patients, 2 ischemic necrosis of liver patients, and 1 hemolytic anemia patient. Reversible rise of serum levels of AST and ALT was observed in all patients with liver disease and ischemic necrosis of liver; on one-way ANOVA, these rise were statistically significant (p<0.01). Serum level of ALT of liver disease group patients without CLD was significantly higher than that of infection group patients without CLD (p<0.05). Among 17 patients who underwent follow-up liver scintigraphy, 13 showed no pulmonary radioactivity. Total 12 patients died during follow-up and most of them were terminal cancer patients or CLD patients of Child-Pugh class C. Conclusion: Pulmonary radioactivity of radiocolloid liver scintigraphy could be attributed to the mobilization of reticuloendothelial system (RES) cells by the activation of RES cells in severe infection and terminal cancer, and also by the extensive liver destruction in liver diseases.

• Journal of Chest Surgery
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• v.32 no.9
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• pp.781-789
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• 1999

Background: Pulmonary artery banding(PAB) accompanies some risks in the aspect of band complications and mortality in the second-stage operation. To assess these risks of the second-stage operation after PAB, we reviewed the surgical results of the second-stage operation in the pediatric patients who had undergone PAB in infancy. Material and Method: From May 1988 to June 1997, a total of 29 patients with preliminary PAB underwent open heart surgery. Ages ranged from 2 to 45 months(mean 20.6$\pm$9.0 months). Preoperative congestive heart failure conditions were improved after PAB(elective operation group) in 27 patients, but early second-stage procedures were required in the remaining 2 patients due to sustaining congestive heart failure(early operation group). Preoperative surgical indications included 2 double outlet right ventricles(DORV group) and 27 ventricular septal defects as the main cardiac anomaly(VSD group). Result: The mean time interval from PAB to the second-stage operation was 15.5$\pm$8.7 months(range 5 days to 45 months). One patient in the DORV group underwent intraventricular tunnel repair and modified Glenn procedure in the other. In the VSD group, the VSD was closed with a Dacron patch in all patients. Concomitant procedures included a right ventricular infundibulectomy in 4 patients and a valvectomy of the dysplastic pulmonary valve in 1 patient. At the second-stage operations, pulmonary angioplasty was required due to the stenotic banding sites in 18 patients. One patient underwent complete ligation of the main pulmonary artery with the modified Glenn procedure. The mortality at the second-stage operation was 17.2%(5 patients). Causes of death were 4 low cardiac output, and 1 autoimmune hemolytic anemia. Diagnosis with DORV and the early operative group were the risk factors for operative death in this series. There was 1 late death. Conclusion: This study revealed the second-stage operation for pulmonary artery debanding and closure of VSD in children was complicated by the correction of the acquired lesions with a significantly high incidence of morbidity and early postoperative deaths. Primary repair is recommended for isolated VSD, if possible.