• Childhood Kidney Diseases
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• v.1 no.1
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• pp.67-72
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• 1997

Purpose : Congenital urinary tract anomaly is the most common anomaly in the childhood and progress to chronic renal failure and growth retardation. Therefore, early diagnosis arid treatment of urinary tract anomaly are important. Method : We reviewed medical records of 124 patients who had urinary tract anomalies on radiologic studies from Jan. 1986 to Dec. 1996. We analyzed demography and clinical characteristics of urinary tract anomalies. Results : 1) The age distributions were as follows ; 61 cases of 124 patients (49%) were under 1 year, 11 cases (8.8%) from 1 to 3 years, 20 cases (16%) from 4 to 6 years, 10 cases (8%) from 7 to 9 years, 9 cases (7.2%) from 10 to 12 years, 10 cases (8%) from 13 to 15 years, and 3 cases (2.4%) from 16 to 18 years. 2) Chief complaints in patients with urinary tract anomalies were fever, flank pain, prenatally diagnosed hydronephrosis, abdominal mass, dysuria and hematuria. 3) Of 124 patients, 68 cases(54.8%) were combined with urinary tract infection, and main causative organism was E.coli, and the most frequently associated anomaly was vesicoureteral reflux. 4) Most of the urinary tract anomalies were VUR, UPJ obstruction, congenital hydronephrosis and double ureter in order of sequence. 5) Whereas the frequency of simple urinary tract anomaly was 87.9%, that of complex anomaly was 12%. 6) Operative corrections were needed in 47 cases and 7 cases were progressed to renal insufficiency. Conclusion : We emphasize that early detection of urinary tract anomaly, appropriate treatment and regular follow-up are needed.

• Journal of the Korean Society of Food Science and Nutrition
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• v.36 no.5
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• pp.616-621
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• 2007

In order to improve the storage stability of steamed bread, fermented pine needle extract syrup potentially with several functional properties was added in manufacturing the products at levels of 8.3, 11 and 18% based on Brix degree of the dough. The manufactured bread was stored at ambient conditions and measured for quality attributes. Dough added with the fermented pine needle extract syrup maintained its pH and water activity at levels of $5.45{\sim}5.90\;and\;0.94{\sim}0.96$, which are normally suitable for yeast fermentation and for appropriate dough hardness. Addition of the extract syrup increased the bread volume by more than 20%. The bread with higher content of the pine needle extract syrup showed slower increase of bread hardness during storage, suggesting retardation of bread retrogradation. The addition of the pine needle extract syrup in bread dough also inhibited growth of the aerobic bacteria and molds on the bread surface (by $0.8{\sim}24$ in log (CFU/g) at 4 day storage). Use of higher than 11% concentration presented initially a strong fine needle flavor to the bread, which disappeared soon after 2 days. Generally addition of the pine needle extract syrup did not give negative effects on the bread quality including sensory quality. Therefore, the addition of the needle extract syrup could contribute to improving the storage stability and extending the shelf life of the bread.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1236-1240
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• 2008

Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with behavioral and learning problems to a lethal disease characterized by multiple malformations. Here, we describe a newborn male with ambiguous genitalia who was diagnosed to have type II SLOS during the neonatal period. A clinical examination revealed low levels of unconjugated estriol in the maternal serum, and a variety of fetal ultrasound anomalies, including prenatal growth retardation. After birth, the infant was diagnosed to have congenital heart disease (Tetralogy of Fallot with severe pulmonary artery stenosis), cleft lip and palate, micrognathia, postaxial polydactyly, ambiguous genitalia, and cataracts. Clinical investigation revealed extremely low plasma cholesterol levels and the presence of mutation (homozygote of p.Arg352Gln) in the DHCR7 gene. The patient underwent palliative heart surgery (to widen the pulmonary artery) and received intravenous lipid supplementation. Cholesterol levels increased slightly, but not to normal values. The patient died from cardiopulmonary failure and sepsis 72 days after birth. This report provides the first description of a Korean patient with SLOS confirmed by verification of DHCR7 gene mutation and illustrates the need for early recognition and appropriate diagnosis of this disease.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.141-147
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• 2016

Hypophosphatasia is caused by the mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). It can be inherited either in an autosomal dominant or recessive manner. Clinically, hypophophosphatasia is characterized by skeletal findings similar to those in rickets or osteomalacia, but serum alkaline phosphatase levels are decreased in the affected patients. Hypophosphatasia can be classified into six clinical forms according to age at diagnosis and severity of symptoms: perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and perinatal benign. As being a very rare disease, only one case has been reported in Korean population. Here we describe a case with perinatal benign hypophosphatasia with recessive ALPL mutations. Bowing of lower legs was detected in prenatal period and low serum alkaline phosphatase level was noted after birth. During the follow-up evaluation for 4.5 years, bone mineralization and legs bowing were improved but the growth retardation was persistent. As the recombinant bone-targeted human TNSALP became available, the clinical improvement of the affected patients is expected including the case described here with this treatment. More efforts are needed to identify the cases affected by hypophosphatasia.

• Korean Journal of Food Science and Technology
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• v.6 no.3
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• pp.147-157
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• 1974

This study was undertaken to observe the effect of sesame in the diet of rats (Splague Dowley). Comparisons were made of weight gains, organ weights and the cholesterol, phospholipid and triglyceride content of the blood serum and liver tissue. Rats weighing 67-80 g were used for the study, while rats weighing 160-165 g were used in the study. In the both studies, the experimental animals were fed on sesame mixtures for 24 weeks. The study was conducted to determine the effect of sesame hulls which are known to contain a high proportion of oxalic acid and phytic acid, which are toxic substances causing various physical disorders. The rats were divided into four groups, a control group which was fed on a standard diet and three groups fed with 20%, 10% and 5% mixtures of sesame seed hulls respectively. The study was designed to observe the same effect, but in this case the rats were divided into two groups, one fed with a 5% mixture of whole sesame seeds and the other with a 5% mixture of dehulled seeds. The results are as follows: 1) An examination of weight gains showed that group I and II which were fed on 20% and 10% mixture of sesame hulls were significantly retarded in comparison with the control group(P<0. 05). 2) Comparisons of organ weights, group I (20% mixture) showed relatively lower weights(P<0.05). 3) The cholesterol content of the blood serum and liver tissue of group I (20% mixture) and group II (10% mixture) were significantly higher than that of the control. 4) The group fed on the whole sesame seed diet and that fed on the dehulled seed diet differed significantly from each other. 5) With the results stated above, the investigators could observe that a high content of sesame hulls in the diet caused retardation in growth and might be the cause of many physical disorders. Though these effects are not important in Korea at present, the increasing intake of sesame seeds and oils indicates that it may become an important problems.

• Journal of Preventive Medicine and Public Health
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• v.37 no.4
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• pp.300-305
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• 2004

Objectives : The exposure to particulate air pollution during the pregnancy has reported to result in adverse pregnancy outcome such as low birth weight, preterm birth, still birth, and intrauterine growth retardation (IUGR). We aim to assess whether prenatal exposure of particulate matter less than 10 (m in diameter ($PM_{10}$) is associated with preterm birth in Seoul, South Korea. Methods : We included 382,100 women who delivered a singleton at 25-42 weeks of gestation between 1998 and 2000. We calculated the average PM10 exposures for each trimester period and month of pregnancy, from the first to the ninth months, based on the birth date and gestational age. We used three different models to evaluate the effect of air pollution on preterm birth; the logistic regression model, the generalized additive logistic regression model, and the proportional hazard model. Results : The monthly analysis using logistic regression model suggested that the risks of preterm birth increase with PM10 exposure between the sixth and ninth months of pregnancy and the highest risk was observed in the seventh month (adjusted odds ratio=1.07, 95% CI=1.01-1.14). We also found the similar results using generalized additive model. In the proportional hazard model, the adjusted odds ratio for preterm births due to PM10 exposure of third trimester was 1.04 (95% CI=0.96-1.13) and PM10 exposure between the seventh month and ninth months of pregnancy was associated with the preterm births. Conclusions : We found that there were consistent results when we applied the three different models. These findings suggest that air pollution exposure during the third trimester pregnancy has an adverse effect on preterm birth in South Korea.

• Biomolecules & Therapeutics
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• v.20 no.2
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• pp.226-233
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• 2012

Ethanol exposure during gestational period is related to growth retardation, morphological abnormality, and even in neurological abnormalities including attention deficit/hyperactivity disorder (ADHD)-like behaviors on offspring. However, relatively little is known about the effects of maternal ethanol consumption prior to conception on their offspring. In this study, we investigated whether maternal ethanol administration during preconceptional phase produces ADHD-like behaviors in the rat offspring. Sprague-Dawley (SD) female rats were administrated ethanol via intragastric intubation with dosing regimen of 6 g/kg daily for 10 consecutive days and treated female rats then mated with non-treated male SD rats after 8 weeks. Another group subjected to the same procedure as those conducted on ethanol treated group except the saline administration instead of ethanol. Offspring was tested for their ADHD-like behaviors using open field test, Y maze test and impulsivity test that is performed in the aversive electronic foot shock paradigm. Offspring of preconceptional ethanol treated (EtOH) group showed hyperlocomotive activity, attention deficit and impulsivity. And reduction of striatal dopamine transporter (DAT) level was observed by Western blot in the EtOH group, compared to control (Con) group, while the immunohistochemical analysis exhibited increased expression of norepinephrine transporter (NET) in the frontal cortex. These results suggest that maternal ethanol consumption in the preconceptional phase induces ADHD-like behaviors in offspring that might be related to the abnormal expression of DAT and NET in rat.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.40-43
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• 2015

Short-chain acyl-CoA dehydrogenase (SCAD) deficiency is an autosomal recessive mitochondrial disorder of fatty acid oxidation associated with mutations in the ACADS gene. While patients diagnosed clinically have a variable clinical presentation, patients diagnosed by newborn screening are largely asymptomatic. We describe here the case of a 1-year-old male patient who was detected by newborn screening and diagnosed as SCAD deficiency. Spectrometric screening for inborn errors of metabolism at 72hrs after birth showed elevated butyrylcarnitine (C4) level of 1.69 mol/L (normal, <0.83 mol/L), C4/C2 ration of 0.26 (normal, <0.09), C5DC+C60H level of 39 mol/L (normal, <0.28 mol/L), and C5DC/C8 ration of 7.36 (normal, <4.45). The follow-up testing at 18 days of age were performed: liquid chromatography tandem mass spectrometry (LC-MS/MS), urine organic acids, and quantitative acylcarnitine profile. C4 carnitine was elevated as 0.91; urine organic acid analysis showed elevated ethylmalonic acid as 62.87 nmol/molCr (normal, <6.5), methylsuccinate 6.81 nmol/molCr (normal, not detected). Sequence analysis of ACADS revealed a homozygous missense mutation, c.164C>T (p.Pro55Leu). He is growing well and no episodes of seizures or growth retardation had occurred.

• 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.

• Korean Journal of Weed Science
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• v.18 no.1
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• pp.63-68
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• 1998

This experiment was carried out to select some herbicides for edible wild greens, Album monanthum, Petasites japonicus, and Aster scaber. The herbicides tested were napropamide 21.8% EC, nitralin 50% WP, and pendimethalin 31.7% EC. Dorminant weeds in the field were Echinochloa crus-galli, Digitaria sanguinalis, Persicaria hydropiper, Chenopodium album, and Siegesbeckia pubescens. Simpson's index was calculated to 0.26~0.30, which showed that weed occurrence in the field was quite various. Control efficacy in the field treated with napropamide EC 872g(ai/ha)., nitralin WP 1,000g(ai/ha), and pendimethalin EC 634g(ai/ha) were 81.4%~85.6%, 79.4%~82.8%, and 86.8%~92.2%, respectively. The typical phytotoxic symptoms to herbicides were germination inhibition, growth retardation, and malformation.