Journal of the korean academy of Pediatric Dentistry
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v.45
no.2
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pp.257-264
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2018
Osteopetrosis is characterized by impaired osteoclast function and increased bone density. Infantile osteopetrosis is a severe form of the disease and has characteristics such as diffusely sclerotic skeleton, pancytopenia, cranial nerve entrapment, infection susceptibility, and abnormal craniofacial appearance. Patients with infantile osteopetrosis often experience developmental delay, and may have a short life span. A 14-month-old girl with osteopetrosis presented to the department of pediatric dentistry. Incipient caries on deciduous incisors were observed. The patient revisited 4 years of age. Besides medical problems, oral complications such as growth retardation, narrow upper arch, crowding, dental caries, and abnormal tooth development were observed. After consultation with her pediatrician, dental treatments were performed on the deciduous molars under sedation after a prophylactic antibiotic injection. At a periodic follow-up, multiple deciduous teeth were treated and extracted, and oralrehabilitation with a removable partial denture was initiated. Patient with osteopetrosis are highly susceptible to infection because of their compromised immune system and problems associated with wound healing that lead to osteomyelitis or sepsis development. Active participation in dental care for sugar intake management and proper oral hygiene are obligatory.
Purpose : Recent advances in the methods of treating cancer in young patients have led to both an increased frequency of CNS complications as well as prolonged life expectancy. We intend to analyze the clinical aspects and laboratory findings of patients with CNS complications during and after treatment. Methods : We reviewed the medical records of 174 childhood cancer patients treated with chemotherapy admitted to the Dept. of Pediatrics, Keimyung University Dongsan Hospital, from January 1995 to November 2002. Among them, 15 cases with CNS complications were investigated in this study. Results : CNS abnormalities were found in 13 patients by CT or MRI during treatment such as leukoencephalopathy(n=7), mineralizing microangiopathy(n=4), brain infarction(n=3), intracranial hemorrhage(n=1), and hypoxic ischemic encephalopathy(n=1). It was found that two patients had two or more CNS abnormalities. Two patients who had no imaging abnormalities had convulsions, possibly after the addition of intrathecal methotrexate. The patients with intracranial hemorrhage and brain infarction had rapid and fatal clinical courses. The hypoxic ischemic encephalopathy following electrolyte imbalance completely recovered after correction of electrolyte. Conclusion : The CNS complications that occur during and after chemotherapy influence prognoses significantly, and remain neurologic sequelae. Therefore early diagnosis and prophylaxis for CNS complications and regular physical examination of patients who have recieved cancer therapy are strongly recommended.
L-carnitine is found in high levels in muscle tissues. It has been developed as a nutrient and dietary supplement, and also used as a therapeutic supplement in various diseases including type II diabetes, osteoporosis and metabolic neuropathies. However, it is not fully understood how it affects cellular mechanisms in colorectal cancer. Therefore, we attempted to determine the effect of L-carnitine in HCT116 human colorectal cancer cells. First, the HCT116 cells were exposed to L-carnitine for 24 hours at 0-40 mM, and then analyzed for cellular proliferation, oxidative stress and related mechanisms. In a MTT assay, L-carnitine inhibited cellular proliferation and induced reactive oxygen species (ROS) in HCT116 by DCF-DA analysis. To analyze the mechanism of L-carnitine in colorectal cancer cells, we performed a western blot analysis for pERK1/2 and pp38 MAP kinase. The western blot showed that L-carnitine significantly increased protein levels of pERK1/2 and pp38 compared with control. Taken together, we found that L-carnitine has anti-proliferative function via increased ROS and activation of ERK1/2 and p38 pathway in HCT116. These findings suggest that L-carnitine may have an anti-proliferative role on colorectal cancer.
Moon, Il Hong;Ha, Kee Soo;Kim, Gui Sang;Choi, Byung Min;Eun, Baik-Lin;Yoo, Kee Hwan;Hong, Young Sook;Lee, Joo Won
Clinical and Experimental Pediatrics
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v.49
no.12
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pp.1308-1314
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2006
Purpose : This study aimed to evaluate the usefulness of auditory evoked potential (AEP) in clarifying neuronal development in premature small for gestational age (SGA), and appropriate for gestational age (AGA) infants. Methods : A total of 183 premature infants who were born from August 2002 to July 2005, were examined with AEP. They were divided into three groups; AGA, symmetric-SGA and asymmetric-SGA group. Results : Statistically significant differences in the head circumference were observed in three groups. Among the risk factors, prevalence of hypoglycemia and hypoalbuminemia between AGA and asymmetric SGA infants were significantly different. V absolute peak latency (APL) in the right side of AGA infants was delayed were than that of asymmetric SGA infants. III-V interpeak latency (IPL) of asymmetric SGA infants was delayed more than that of symmetric SGA infants. Moreover, I-V IPL on both sides of symmetric SGA infants was shortened more than that of AGA infants. However, all the results of AEP were within the reference range, according to gestational age. Birth weight of, only asymmetric SGA, was related to the III APL on both sides and the III-V IPL on right side. Conclusion : This study shows that the values of APL and IPL of premature SGA infants are different than that of premature AGA infants. These data could be an indicator in evaluating the neurologic functions of small for gestational age infants.
Kim, Gi Hwan;Kook, Hoon;Baek, Hee Jo;Han, Dong Kyun;Song, Eun Song;Jo, Young Kook;Choi, Ic Sun;Kim, Young Ok;Kim, Chan Jong;Woo, Young Jong;Yang, Soo Jin;Hwang, Tai Ju
Clinical and Experimental Pediatrics
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v.50
no.2
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pp.182-189
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2007
Purpose : The primary purpose of this study was to evaluate the growth and neuropsychologic function following treatments for pediatric hematologic and oncologic diseases. Healthy monozygotic twins served as ideal controls for comparison to exclude possible confounding factors. Methods : Seven children treated with various hematologic and oncologic diseases were included in the study: acute lymphoblastic leukemia (ALL; n=2), Diamond-Blackfan anemia twins (n=2), and aplastic anemia (n=3). The median age at the diagnosis was 5.2 (0.3-15) years. The median duration of follow-up was 7.2 (4.9-10) years. Controls were healthy monozygotic twins. Growth was measured and the percentile channels were evaluated sequentially for patients. The K-WISC III was applied and compared in 5 pairs of patients and controls. Results : Similar growth profiles were noted for the twins. The percentiles at diagnosis was 3-10 in 3, 25-50 in 2, and 50-75 in 2 cases. All patients stayed in their growth percentiles through follow-up, except for 1 patient who became obese. For IQ tests, the mean behavioral, verbal and full scale IQ scores of patients were 88.0, 93.8, and 89.8, respectively, and those from their corresponding controls were 92.2, 97.0, and 91.7 (P>0.05). However, 2 children who were treated for ALL had lower IQ scores. Conclusion : Similar growth profiles were observed in the monozygotic twins in terms of height and weight. The IQ scores of patients were similar to those of monozygotic twins. However, prophylactic CNS-directed therapy for leukemia might adversely affect the IQ scores. A further prospective study on larger number of twins is warranted.
Journal of the Korean Academy of Child and Adolescent Psychiatry
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v.9
no.1
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pp.54-66
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1998
Objectives:This study was to investigate the impact of parental psychiatric disorder on offspring's depression, anxiety, self concept, perception of familial relationship compared with offspring of normal control. In offsprings of parents with psychiatric disorder, this study explored whether their psychopathology, self concept, and perception of familial relationship were influenced by parent’s sex, onset time of parent’s psychiatric disorder and parent’s psychiatric diagnosis. Methods:52 offsprings aged 10-18 years of 39 psychiatric outpatient were surveyed from June, 1997 to April, 1998 and completed several questionnaaire, including Korean from of the Family Environment Scale, Korean form of the State-Trait Anxiety Inventory for Children, Korean form of Kovac’s Children’s Depression Inventory, and Korean form of Piers-Harris Children’s Self-Concept Scale. Their score was compared with offsprings’ of normals. In offsprings of parents with psychiatric disorder, they were compared according to parent’s sex, onset time of parent's psychiatric disorder and parent’s psychiatric diagnosis. Results:The results were as follows:1) Offsprings of parents with psychiatric disorder reported higher level of state anxiety and lower level of the FES expressive subscale than offsprings of normals(p<0.05). But they reported higher level of PHCSCS intellectual & school status subscale and popularity subscale than normals(p<0.05). 2) There were no differences in anxiety, depression, self concept, and perception of familial relationship between patient’s sex. 3) Offsprings less than 3 years old when parent’s psychiatric disorder had developed showed higher level of trait anxiety and lower level of FES control subscale than offsprings more than 3 years old (p<0.05). 4) There were no diferences in anxiety, depression, self concept, and perception of familial relationship between patient’ diagnostic groups(schizophrenia spectrum disorder-mood disoderneurosis). Conclusion:The finding indicated that self reported scale of anxiety and depression showed no significant difference between offsprings of psychiatric patients and offsprings of normals. In offsprings of parents with psychiatric disorder, parent’s sex and psychiatiric diagnosis had no influence on offspring’s psychopathology. But the offspring’s age(before 3 years old) when the parent’s psychiatriric disorder developed had influence on higher level of offspring’s trait anxiety. For further high risk group study, direct interview and evaluation of parent-child agreement or teacher-child agreement will be needed in longitudinal study.
The use of phonosurgery in the recent development of laryngomicrosurgery has enabled the restoration of a normal voice in respect to functional laryngeal surgery which in Korea in the past limited to simple removal of benign laryngeal tumor such as laryngeal polyp or nodules and cordal injection of $Teflon^{{\circledR}}$ for the treatment of recurrent nerve paralysis under the vision of suspension laryngoscopy. Performance of phonosurgery for the treatment of cord paralysis, mutational dysphonia, vocal cord atrophy, hyperkinetic dysphonia and sulcus vocalis is a happy event in the view point of development of phonosurgery in Korea. In this aspect thyroplasty to change the position and physical characteristics of the cord outside the glottis instead of the direct handling of the vocal cord through direct endoscopy is popular. Among the 4 types of thyroplasty, classified by Insshiki(1974), type I thyroplasty(1ateral compression of vocal cord) and type IV thyroplasty(lengthening of vocal cord) were effective in the treatment of unilateral vocal cord paralysis. Advantages of this operation are the fine adjustment of the degree of lateral compression under local anesthesia according to the phonation of the patient during operation and avoidance of dyspnea and intralaryngeal hemorrhage due to the manipulation outside the internal perichondrium of the thyroid cartilage. We did 7 cases of thyroplasty for the treatment of unilateral vocal cord paralysis in the 7 months from September 1981 to March 1982. Before the operation aerodynamic study, psychoacoustical evaluation, stroboscopy and sound spectrographic analysis were done. Two months after the operation the above procedures were performed again. Results of preoperative and postoperative examination were compared and the following results were obtained. 1) In the aerodynamic study, maximum phonation time increased to 158% of the preoperative value and the phonation quotient and the mean flow rate decreased to 58% and 54% of preoperative values. 2) The degree of hoarseness improved in the psychoacoustical evaluation and the glottic chink during phonation was decreased in the stroboscopic examiantion. 3) In the sound spectrographic analysis, periodicity was much restored and noise distribution decreased especially in the high frequency area.
Separating the patient from hypothermic cardiopulmonary bypass(CPB) before achieving adequate rewarming often results in afterdrop, which can predispose to electrolyte disturbances, arrhythmia, hemodynamic alterations, and shivering-induced increase of oxygen consumption. In an attempt to find an adequate end point temperature of rewarming after hypothermic CPB, 50 pediatric cardiac surgical patients were r ndomly assigned for end point temperature of rewarming of 35.5$^{\circ}C$ (Group 1) or 37t (Group 2), rectal temperature. Thereafter the rectal temperature was measured half, one, four, eight, and 16 hour after arrival to the intensive care unit(ICU), with heart rate and blood pressure. Additionally the rectal temperature was compared with esophageal temperature during CPB, and axillary temperature luring stay in the ICU. Nonpulsatile perfusion with a roller pump was used in all patients and a membrane or bubble oxygenator was used for oxygenation. Both groups were comparable with respect to age, sex, body surface area, total bypass time, and rewarming time. There was no afterdrop in both groups, and there were no statistical differences in the rectal temperatures between two groups. There were also no statistical dilyerences with respect to the heart rate and blood pressure between two groups. At the end of rewarming the esophageal temperature was higher than the rectal temperature. The axil ary temperature measured in ICU was always lower than the rectal temperature. No shivering was noted in all patients. In conclusion, with restoration of rectal temperature above 35.5$^{\circ}C$ at the end of CPB in pediatric patients, we did not observe an afterdrop.
Lee Ji-Suk;Rho Kwang-Sik;Kim Ji-Hong;Lee Jae-Seung;Kim Pyung-Kil
Childhood Kidney Diseases
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v.1
no.2
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pp.144-150
/
1997
Purpose : The kidney is one of the most common sites of cyst formation. Cystic diseases of the kidney are a diverse group of clinicopathologic entities and variable prognosis. They span a wide range of both age of presentation and severity of the renal disease. And many of them are systemic disorders, sharing similar process of cyst formation in other organs. Recently, development of imaging studies has been contributing widely to the diagnosis of the diseases. Treatment, however, is not established satisfactorily. We performed this study to evaluate the occurrence and treatment of cystic diseases of the kidney. Methods : We reviewed retrospectively the medical records of 44 patients with cystic diseases of the kidney in the Department of Pediatrics, during last 11 years. Results : In the 44 patients with cystic diseases of the kidney, 31 patients(71%) had multicystic dysplastic kidney and 11(35%) of them received nephrectomy due to differentiation from neoplasms or severe abdominal distension. Seven patients(16%) had polycystic kidney disease, and all of them were infantile type. Five patients(11%) were diagnosed as having a simple renal cysts. Progression to renal failure was noted in none of the cases. In 14(32%) out of total 44 patients, the diagnosis was made in neonatal or infantile pelted. Conclusion : The incidence of cystic diseases of the kidney appeared very low, but further investigation on their pathogenesis, classification, and indication of treatment is needed.
Polycystic ovary syndrome (PCOS) is hormonal imbalance condition as the endocrine and metabolic disorder that induces the infertility and various complications in reproductive age women. Estradiol valerate (EV) is used hormone replacement therapy in menopausal women and is reported that excessive administration of EV induces the PCOS. Nerve growth factor (NGF) is the factor to regulate the survival and maturation of developing neuronal cell and is also synthesized in ovary. And NGF is overexpressed in EV-induced polycystic ovary (PCO) as previously reported. Therefore, this study examined the possibility of NGF as can be used the biological marker in diagnosis of PCOS, the hormonal imbalance condition, using PCO and CHO (chinese hamster ovarian) cell lines. The concentration of EV treatment is optimized a 1 mg as not influence on the proliferation of CHO cell but 2 mg and 3 mg of EV treatment have the inhibition effect at initial stage. The morphological change was not observed in CHO cell after dose dependent manner treatment of EV. Expression of NGF mRNA and protein is significantly increased at 30 min after EV treatment in CHO cells compared to that of control. And NGF protein expression is strongly increased in PCO tissue, which observed many follicular cysts compared to normal ovary tissue. Taken together, overexpression of NGF may be act as a molecule to induce an abnormal development of follicle, suggesting that NGF can be used as a biological marker in diagnosis of PCOS.
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