• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.57-59
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• 2016

Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and fludarabine reduced-toxicity myeloablative conditioning. Intravenous busulfan was administered once daily for 4 consecutive days (days -8 to -5), and the target area under the curve was $75,000{\mu}g{\cdot}hr/L$. Fludarabine ($40mg/m^2$) was administered once daily for 6 consecutive days from days -8 to -3. Antithymocyte globulin (2.5 mg/kg/day) was administered from days -4 to -2. The patient underwent successful engraftment and did not have any severe toxicity related to the transplantation. Conditioning with a targeted busulfan and fludarabine regimen could provide a better outcome for HSCT in CGD, with close regulation of the busulfan dose.

• Korean Journal of Veterinary Service
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• v.40 no.2
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• pp.155-159
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• 2017

Korean goral (Neamorhedus caudatus) is registered as a natural monument number 217 by South Korea Cultural Heritage Administration. It is also recognized as the endangered species I by Convention on International Trade in Endangered Species of Wild Fauna and Flora (CITES). In this study, blood samples of Korean goral were collected to make reference intervals. Blood sampling was conducted on 19 numbers of Korean gorals (ten females, nine males), which were reared in Korean Goral Restoration Center. For total samples, the reference intervals of hematological parameters were: white blood cell $7.69{\sim}10.87K/{\mu}{\Lambda}$; hematocrit 36.73~46.18%; red blood cell $10.72{\sim}12.86K/{\mu}{\Lambda}$; hemoglobin 12.79~15.14 g/dL; mean corpuscular volume 33.15~36.75 fL; mean corpuscular hemoglobin 11.53~12.23 pg; mean corpuscular hemoglobin concentration 32.64-35.91 g/dL and red blood cell distribution width 39.2~43.46%. For total samples of each parameters, the following results were obtained for serum biochemistry: glucose 111.81~153.77 mg/dL; blood urea nitrogen 22.35~28.91 mg/dL; creatine 1.22~1.84 mg/dL; phosphate 4.57~6 mg/dL; calcium 8.7~9.1 mg/dL; total protein 6.53~6.92 g/dL; albumin 3.1~3.48 g/dL; globulin 3.26~3.62 g/dL; alanine aminotransferase 56.7~158.56 U/L; aspartate aminotransferase 230.35~473.06 U/L; alkaline phosphatase 178.06~332.47 U/L; gamma-glutamyl transpeptidase 131.6-~181.24 U/L; total bilirubin 1.47~2.12 mg/dL; cholesterol 46.48~71.52 mg/dL; amylase 16.3~26.03 U/L; sodium 150.43~153.88 mmol/L; potassium 3.98~4.6 mmol/L and chlorine 109.48~113.26 mmol/L. The ranges of values were similar campared to previous studies except in the case of RDW value, which showed higher range than the RDW value of a previous study. The reference intervals from this study will be useful data for treatment and management of gorals.

• Pediatric Infection and Vaccine
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• v.9 no.1
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• pp.61-66
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• 2002

Purpose : To determine wether varicella can be prevented by administration of oral acyclovir(ACV) during the incubation period of the disease. Methods : Starting 9 days after exposure to the index case in their families, ACV(40 mg/kg/day in four divided doses) was given orally to 20 exposed children for 5 days. Their clinical features was compared with those of 20 control subjects. Antibody titers to VZV were measured in both group 1 week and 4 weeks after finishing the oral ACV administration. Results : The mean age of family members with varicella(51.4 months) were significantly high compared to that of ACV prophylaxis group(28.5 months) and control group(31 months) (P<0.05). Among the 12 children with ACV prophylaxis who completed follow up blood sampling, nine children were diagnosed as VZV infection on the serologic test(75%). Among them six children showed positive VZV IgM on the first blood sample and two children showed serocoversion to positive IgM on the second test after ACV prophylaxis. One child who was negative on both IgM and IgG, showed positive IgG on the second test. The incidence of fever and severity of skin rashes were significantly low in children received oral ACV than in the control group. No or reduced number of maculopapular eruption were observed in the oral ACV group compared to multiple vesicles of the control group. Conclusion : In the present study, we observed that oral ACV prophylaxis to the family contacts is effective in reducing severity of skin lesion. It is likely that oral ACV 9 days after contact prevents or reduces blood dissemination of VZV. Little is known about clinical effect and immunity to the virus in exposed children with no varicella symptom after treatment. We propose the checking up antibody to VZV some period after oral ACV, and considering vaccination to whom with no antibody. But further more studies are needed to practical application of oral ACV for the postexposure prophylaxis of varicella.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.155-168
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• 2002

Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.

• Journal of the Korean Society of Food Science and Nutrition
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• v.35 no.6
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• pp.683-689
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• 2006

This study was established to investigate the effect of germanium-fortified yeasts on the serum lipid composition and immune system of human body. All 50 subjects with the age range of $50{\sim}75$ were entered in this clinical trial for 6 months. The effects were determined by the proliferative responses of immune-mediated cells, T-cell, B-cell and NK-cell during daily supplementation with/without germanium-fortified yeast. The results of hematology and blood chemistry didn't show any significant differences during administration periods. Serum lipid compositions also didn't show any significant differences during administration periods except triglyceride (TG) and VLDL-cholesterol. TG and VLDL-cholesterol levels were increased significantly by the consumption of germanium-fortified yeast (p<0.05). Immune mediated T-celt and NK-cell didn't increased in both control and test group supplemented with germanium fortified yeast, while B-cell increased in the germanium fortified yeast group after 8 week (p<0.05). Also $TNF-{\alpha}$ increased in the group of germanium fortified yeast after 8 week (p<0.05) but not in the control group. From the above results, germanium fortified yeast is expected to be useful on the improvement of the cellular immune response and protection of organs from various chronic diseases.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1329-1339
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• 2006

Purpose : Failure of hematopoietic stem cell transplantation(HSCT) may be encountered in practice because of either relapse of the malignancy or dysfunction of the graft. Second HSCT may be the only option for some patients whose initial HSCT failed. Methods : From May, 1991 to December, 2004, 115 HSCTs were performed at the Pediatric Blood & Marrow Transplantation Center, Chonnam National University. This study was a retrospective analysis of the medical records of 15 patients who received the second HSCT after initial graft. Results : Among eight patients with nonmalignant diseases, two patients underwent the second HSCT because of primary graft failure and five because of late graft rejection. The remaining Fanconi anemia patient was re-transplanted due to development of AML. Two patients died and one experienced primary graft failure, but is still alive. The Kaplan-Meier 5-year overall survival rate was 75 percent and the disease free survival rate was 62.5 percent in nonmalignant diseases. All malignant patients underwent second transplants because of relapses. Four died of relapse and one of treatment-related complications. The Kaplan-Meier 2-year overall and event free survival rate was 28.6 percent each in malignant diseases. Conclusion : Second HSCT for graft dysfunction of nonmalignant disease seems to be feasible and should be considered as a standard practice. The relapse of malignant diseases remains a big obstacle even after the second HSCT, although a small portion of patients might be salvaged. Further investigation of novel therapeutic strategies, as well an the understanding of the biology should be explored.

• The Korean Journal of Nuclear Medicine
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• v.5 no.1
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• pp.49-64
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• 1971

The radioactive $^{131}I$-rose bengal serial scintiphotography was performed in 62 patients with the hepatobiliary diseases and in 20 normal subjects. This approach permitted visualization of the hepatic uptake of $^{131}I$-rose bengal from the circulation and its excretion into the biliary trees and the intestines. In some of these patients, gallbladder function was examined, using eggs as a gallbladder constrictor. The time of maximum hepatic uptake was well correlated to the conventional biochemical liver function tests. In addition to $^{131}I$-rose bengal scintiphotography, $^{198}Au$-colloid scintiphotography was also performed to make comparison of these two tests. The results obtained were as follows: 1. In normal subjects, the maximum hepatic uptake of $^{131}I$-rose bengal occurred at $23{\pm}2.9$ minutes, the initial hepatic excretion at $34{\pm}5.1$ minutes, the visualization of the gallbladder at $29{\pm}5.7$ minutes and the intestinal visualization at $54{\pm}25.8$ minutes. The radioactivity in the gallbladder decreased to $10.7{\pm}5.0%$ one hour after the ingestion of eggs. 2. In the patients with cirrhosis of the liver, there was a delayed and decreased hepatic uptake. The maximun hepatic upake occurred at $43{\pm}12.9$ minutes. The differences in the results of uptake between the cirrhotic and the normal group were statistically significant. The initial hepatic excretion occurred at $60{\pm}18.5$ minutes and had tendency of delaying compared with the normal controls. The gallbladder was visualized in 13 of 16 cases (81%) and its visualization occurred at $49{\pm}14.6$ minutes with a tendency to be delayed compared with the normal controls. The intestinal visualization occurred at $63{\pm}15.8$ minutes and its delaying tendency was somewhat more prominent. 3. In patients with hepatitis, the maximum hepatic uptake occurred at $59{\pm}21.4$ minutes and was significantly delayed. The initial hepatic excretion occurred at $82{\pm}34.3$ minutes and the results revealed a delaying tendency. The gallbladder was visualized in 15 of 20 cases (75%) at $57{\pm}18.7$ minutes, which was significantly delayed. The Intestinal visualization was noted in all cases with marked delay. 4. In patients with obstructive jaundice, the maximum hepatic uptake was noted at $83{\pm}14.7$ minutes, showing the most significant delay. The hepatic excretion into biliary trees and intestines was not entirely noted in all cases except the only one case with gallbladder visualization. 5. In patients with cholelithiasis, the maximum hepatic upake and the initial hepatic excretion were slightly delayed with mean times of $39{\pm}11.2\;and\;48{\pm}17.1$ minutes respectively. The visualization of the gallbladder was demonstrated in 10 of 17 cases (59%) and occurred at $52{\pm}25.6$ minutes with a slight delay. The intestinal visualization occurred at $67{\pm}47.7$ minutes and was slightly delayed. $^{131}I$-rose bengal in the gallbladder remained high, $49.3{\pm}21.3%$, which suggested quantitatively decreased power of gallbladder constriction. 6. The time of the maximum hepetic uptake was correlated well to BSP retention and serum alkaline phosphatase ativity. However, the maximum hepatic uptake had no definite correlation with serum albumin, serum globulin, TTT, serum cholesterol, SGPT or SGOT. 7. In the diagnosis of the hepatobiliary diseases with jaundice, $^{131}I$-rose bengel serial scintiphotography has proved to be more useful than $^{198}Au$-colloid scintiphotography. With these results, it could be justified that $^{131}I$-rose bengal scintiphotography is an excellent diagnostic aid for dynamic hepatobiliary function studies in the clinical practice.

• Journal of the Mineralogical Society of Korea
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• v.18 no.4 s.46
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• pp.277-288
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• 2005

Perlite, a hydrated volcanic glass, occurs mainly as a bed-like body, and is distributed intermittently along the unconformity surface between the Beomgockri Group and its lower formations, viz. Janggi Group. The perlite is intimately associated with surrounding pumiceous welded tuff and rhyodacites in space and time. Compared to the typical perlite, the perlite is rather silica-poor and impure, and thus, includes lots of phenocrysts and rock fragments. Nearly the perlite is compositionally rather close to a pitchstone than a perlite in water contents. Petrographic comparison between perlite and associated volcanic to volcaniclastic rocks indicates that pumiceous welded tuff and rhyodacite seem to be Protolith of the Perlite. A Zr/$TiO_{2}$-Nb/Y diagram and field occurrence of perlite and their protolithic rocks also conforms the above interpretation. Kn addition, remnant vesicles in perlite strongly reflect that the precursor of perlitic glass appeared to be pumice fragment as well as volcanic glass. The perlite was diagenetically formed by way of a pervasive water-rock interaction at the deposition of the Manghaesan Formation in lacustrine environment. During perlitization, $SiO_{2}$ and alkali tend to be consistently depleted. Preexisting system of the Beomgockri Group based on the perlite formation should be corrected, because the perlite was formed diagenetically without lateral persistence in its occurrence.

• Progress in Medical Physics
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• v.16 no.4
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• pp.214-219
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• 2005

As part of Korean retinal prosthesis project, we have provided preliminary experimental results regarding voltage parameters for the stimulation of chemically degenerated rabbit retina. Since our APB-treated chemically degenerated retina is only ON-pathway blocked, now we switch our experiments to more appropriate retinal degeneration model, genetically degenerated retina model (RD mouse: rd/rd (C3H/HeJ)). Before studying with RD mouse, we started control experiments with normal SD rat to understand characteristics of retinal ganglion ceil activity with postnatal maturation in rodents. Ganglion cell activities were recorded with 8${\times}$8 multi-electrode array. Moving spontaneous bursts appeared until postnatal day of 15. During pre-eye opening period, no light evoked response appeared. After postnatal day of 2 weeks (post-eye opening period), ON-, OFF- and ON/OFF response appeared. The fractional distributions of ON, OFF, and ON/OFF ganglion cell is about $40\%,\;50\%$, and $5\%$. The percentage ($\%$) of light evoked response in each dorso-temporal, ventral, and dorso-nasal area of eye is about $50\%,\;37.5\%$ and $12.5\%$, respectively. We concluded that the optimal period for experiment in rodent is about postnatal day of 2${\~}$3 weeks.