• Radiation Oncology Journal
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• v.19 no.2
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• pp.142-145
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• 2001

Purpose : Acute leukemia with hyperleukocytosis (more than $10^5/mm^3$) is at high risk of early sudden death, usually from intracerebral hemorrhage. Emergency cranial irradiation is a relatively simple approach to solve this the problem. We summarized our experience of cranial irradiation in 24 leukemic children who presented with hyperleukocytosis. Methods and Materials : Between 1990 and 1998, 40 children with acute leukemia presenting with hyperleukocytosis were referred for emergency cranial irradiation. Among these patients, 24 children were evaluable. There were 16 boys and eight girls, their ages ranged from 2 to 13 years (median 9.5 years). The initial leukocyte counts ranged $109,910/mm^3\;to\;501,000/mm^3$. Peripheral blood smear was peformed in all patients and noted the morphology of the blast. Introduction of emergency cranial irradiation was determined by the leukocyte counts (more than 100,000/mm) and the existence of the blast in peripheral blood smear. All patients were treated with intravenous hydration with alkaline fluid and oral allopurinol. Cranial irradiation started on the day of diagnosis. With 2 Gy in one fraction in 4 patients, 4 Gy in two fractions in 20 patients. Results : The WBC count had fallen in 19 patients (83%) and no intracerebral hemorrhage occurred after irradiation. There were five cases of early deaths. Four patients died of metabolic complications, and one patient with intracerebral hemorrhage. He died 5 hours after cranial irradiation. No patient had any immediate side effect from cranial irradiation. Conclusion : Our data suggest, that emergency cranial irradiation can be safely chosen and effective in childhood leukemic patients presenting with high leukocyte counts.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.18-24
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• 2015

Glycogen storage disease type Ib (GSD Ib) is one of the rare inherited metabolic disease caused by mutation of SLC37A4 gene. Clinical characteristics include hepatomegaly, hypoglycemia, lactic acidosis, hyperlipidemia and high serum uric acid concentration. The authors analyzed clinical and molecular characteristics of three Korean patients (one male and two females) with GSD Ib by retrospective review of medical records. Two patients were diagnosed in toddler period by hypoglycemia and hepatomegaly. One patient was diagnosed by growth retardation and short stature in puberty. c.412T>C (p.Trp138Arg) (3/6 alleles, 50.0%) was most frequently observed, following by p.Leu348Valfs*53 (1 allele), p.Pro191Leu (1 allele), p.Ala148Val (1 allele) in molecular analysis. Uncooked corn starch and allopurinol was administered. Because all three patients had neutropenia and recurrent infections, G-CSF was administered. Two patients had severe osteoporosis needing calcium supplement. The patient who diagnosed at puberty had relatively poor prognosis demonstrated by having severe infection and complications in liver and kidney.

• Journal of Applied Biological Chemistry
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• v.64 no.1
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• pp.5-11
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• 2021

The phenolics of Sambucus sieboldiana var. pendula leaves for functional resources were examined on inhibitory activity against biological enzyme and anti-microbial activity. The amount of phenolic compounds were 11.60±0.18 and 12.43±0.07 mg/g by water and 50% ethanol extraction, respectively. The antioxidative activity of phenolic in extracts was higher than solids. The inhibition activities on angiotensin converting enzyme were 92.08 and 78.33% at 200 ㎍/mL phenolic concentration in water and ethanol extracts, respectively. The xanthine oxidase inhibitory activity were 100% at 200 ㎍/mL phenolic in water and ethanol extracts, respectively. These result was higher than 70.37% of allopurinol as positive control at 200 ㎍/mL. The inhibitory activity against hyaluronidase were each 25.35 and 43.38% in water and ethanol extracts. The water extract from S. sieboldiana var. pendula leaves showed antibacterial activity on the Propionebacterium acnes, Staphylococcus aureus and Streptococcus mutans in water extract and S. mutans in ethanol extract. This result suggests that phenolic from S. sieboldiana var. pendula leaves are suitable as functional foods with anti-hypertension, anti-gout, anti-inflammation and anti-microorganism activities.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Journal of Applied Biological Chemistry
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• v.57 no.1
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• pp.53-59
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• 2014

To evaluate the inhibitory effect of xanthine oxidase (XO) and aldehyde oxidase (AO), and antihyperuricemic effect by Aspergillus oryzae fermented Smilax china L. leaf extracts and fractions, we observed extracted yield by each solvent, the content of total polyphenol and total flavonoid (TF), the activities of XO and AO, and serum uric acid level. Extracted yield (g/kg) by 80% ethanol (EtOH) was 13.56, those of n-hexane, dichloromethane (DICM), ethylacetate (EtOAc) and n-butanol fraction (BuOH) were 1.35-3.33. Furthermore, total polyphenol content (mg/g-extract) of EtOAc fraction, BuOH fraction, DICM fraction and EtOH fraction is 478.07-501.26, 259.49-289.02, 165.03-232.27, 134.02-196.54, respectively. Those of fermented EtOAc and DICM fraction was 4.85 and 40.74% higher than that of non-fermented fraction, respectively, while the other fermented fractions were lower than those of non-fermented fractions. And total flavonoid content (mg/g-extract) of EtOAc fraction was higher than those of other fractions. Additionally, TF of fermented EtOAc and BuOH fraction is 10.56 and 60.17% higher, than that of fermented fraction, respectively, although those of the other fermented fractions was lower than that of non-fermented fractions. On the other hand, XO inhibitory activities of all fermented fractions was significantly higher than that of all non-fermented fraction, while those of fermented EtOAc (75.02%) and BuOH fraction (65.59%) was markedly higher than that of non-fermented fraction (39.42 and 5.34%), respectively. In addition, AO inhibitory activities of DICM and EtOAc fraction was 81.82 and 77.93% higher, respectively, than those of the other fractions, and those of fermented fractions as with XO were significantly higher than that of non-fermented fractions. Meanwhile, serum uric acid level (SU) of hyperuricemic control mice (HC, 6.98 mg/dL) was 1.83 folds higher than that of normal control (NC, 3.82 mg/dL). Furthermore, SU in the group treated with EtOAc fraction decreased in a dose dependent manner compared with the allopurinol control group, although those of fermented fractions were significantly lower than those of non-fermented fractions. This study suggests that fermented Smilax china L. leaf extracts may regulate the XO and AO inhibitory activities and antihyperuricemic effect due to aglycone components from glycoside form flavonoids by fermentation of A. oryzae.

• Food Science and Preservation
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• v.23 no.1
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• pp.110-116
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• 2016

In this study, the antioxidative activity and functional food activities of water and ethanol extracts from Pinus densiflora root were examined. It was more effective to use ethanol than water when extracting phenolic compounds. The extracted phenolic compounds from Pinus densiflora root for biological activities were examined. The phenolic compounds extracted with water and 80% EtOH were $1.86{\pm}0.04mg/g$ and $6.85{\pm}0.16mg/g$, respectively. DPPH free radical scavenging activity of water and EtOH were each 86% and 85% at $100{\mu}g/mL$ phenolics, respectively. ABTS radical decolorization activity was 48% in water and 68% in EtOH at $200{\mu}g/mL$. Antioxidant Protection Factor (PF) were 1.74 PF in water and 1.96 PF in EtOH at $50{\mu}g/mL$. TBARs of water and EtOH were 93% and 98%, respectively at $100{\mu}g/mL$. The inhibition activity on xanthine oxidase was 83.7% in water extracts and 79.6% in ethanol extracts. Inhibition on xanthine oxidase of water and ethanol extracts showed a higher inhibition effect than allopurinol. The inhibition activity on ${\alpha}$-glucosidase was 14.8% in water extracts and 91.6% in ethanol extracts. The result suggests that P. densiflora root extracts may be useful as as functional food material.

• Journal of Life Science
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• v.22 no.12
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• pp.1621-1627
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• 2012

Cell motility plays an essential role in many physiological responses, such as development, immune reaction, and angiogenesis. In the present study, we showed that lysophosphatidic acid (LPA) modulates cancer cell migration by regulation of generation of reactive oxygen species (ROS). Stimulation of SKOV-3 ovarian cancer cells with LPA strongly promoted migration. but this migration was completely blocked by pharmacological inhibition of phosphatidylinositol 3-kinase (PI3K)/Akt signaling pathway. Inhibition of the ERK pathway had no effect on migration. Stimulation of SKOV-3 ovarian cancer cells with LPA significantly induced the generation of ROS in a time-dependent manner. LPA-induced generation of ROS was significantly blocked by pharmacological inhibition of PI3K or Akt, but inhibition of the ERK signaling pathway had little effect. LPA-induced generation of ROS was blocked by pretreatment of SKOV-3 ovarian cancer cells with an NADPH oxidase inhibitor, whereas inhibition of xanthine oxidase, cyclooxygenase, or mitochondrial respiratory chain complex I had no effect. Scavenging of ROS by N-acetylcysteine completely blocked LPA-induced migration of SKOV-3 ovarian cancer cells. Inhibition of NADPH oxidase blocked LPA-induced migration whereas inhibition of xanthine oxidase, cyclooxygenase, or mitochondrial respiratory chain complex I did not affect LPA-induced migration of SKOV-3 ovarian cancer cells. Given these results, we suggest that LPA induces ROS generation through the PI3K/Akt/NADPH oxidase signaling axis, thereby regulating cancer cell migration.