Hong, Yong Hee;Ha, Sun Mi;Jeon, You Hoon;Yang, Hyeon Jong;Pyun, Bok Yang
Clinical and Experimental Pediatrics
/
v.51
no.5
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pp.506-511
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2008
Purpose : The results of pulmonary function test (PFT) in children are variable according to the patient's cooperation and comprehensiveness. This study has intended to figure out the effectiveness of pre-education and training with balloons on PFT in children. Methods : One hundred six children mean aged $9.35{\pm}2.92\;years$ were tested. All participants performed PFT twice in 30 minutes intervals. First PFT were performed after usual instruction and second PFT were performed according to randomly classified grouping; Group 1 : repeat PFT after training with balloons, Group 2 : repeat PFT after training and education, both, Group 3 : repeat PFT after education about objects and necessities of pulmonary function test, Group 4 : repeat PFT without any education and training. Results : There were no difference statistically on the results of percent of predicted $FEV_1$, $FEV_1/FVC$, $FEF_{25-75%}$ and PEFR in Group 1, 2 and 4 patients. In some cases, the average is decreased with repeated PFT. At the case of repeated PFT after education, the average of percent of predicted $FEV_1$, $FEV_1/FVC$, $FEF_{25-75%}$ and PEFR is increased. There was statistically significant difference on the value of $FEV_1$, $FEF_{25-75%}$ and PEFR between first and second PFT in Group 3. Conclusion : Training with balloons before PFT causes fatigueness and lowers concentration. Understanding of PFT makes results better than training. Therefore, enough explanation and education about PFT before examination is most effective for appropriate PFT in children.
Purpose: Liver function test abnormalities have been reported frequently in patients receiving total parenteral nutrition (TPN). In adults, it is known that liver complications decrease with the use of cyclic parenteral nutrition (CPN), especially if the shift to cycling was not too late. However, there are few studies about the effects of cycling on liver injury in children beyond the neonatal period. The aim of this study is to evaluate the effect of the early use of CPN on total parenteral nutrition induced hepatic dysfunction. Methods: Twelve sets of CPN in 11 children (2 months to 17 years) were included in this study. Data on underlying diseases, age, length of time on TPN, macronutrient intake, complications, and biochemical parameters were collected from clinical records. All children had received CPN in the early period of persistent transaminase elevation or cholestasis complicated by previous continuous PN. The duration of infusion off-time in CPN was 2 hours in patients less than 3 months of age and 4 hours in the older children. Results: All 12 cases showed elevated aminotransferase and 5 of them also showed cholestasis. Serum total bilirubin concentration was normalized in all 5 cases with median periods of 8 days (p<0.05) after initiation of CPN. ALT either decreased significantly or was normalized in all cases with median periods of 30 days (p<0.05) on CPN. The CPN was well tolerated without significant complication except for one case of hyperglycemia. Conclusion: The early use of cyclic parenteral nutrition had a beneficial effect in improving hepatic dysfunction complicated by TPN in children.
Purpose: Recently, after patent ductus arteriosus (PDA) ligation in preterm infants, unexplained hemodynamic instabilities are reported. To determine the incidence, risk factors and clinical manifestations of hemodynamic instability after PDA ligation in very low birth weight (VLBW) infants. Methods: This retrospective multicenter study enrolled 18 VLBW infants who underwent PDA ligation from January 2002 to February 2008. Hemodynamic instability defined as unexplained cardiopulmonary dysfunction with increased dependency on mechanical ventilation and decreased blood pressure. Results: The mean gestational age and birth weight (BW) of all infants were $27^{+6}{\pm}1^{+6}$ weeks and 951${\pm}$245 g. Hemodynamic instability group (HI) included seven infants (39%) and hemodynamic stability group (HS) included 11 infants (61%). Compared to HS, HI had lower BW (1,033${\pm}$285 g vs. 821${\pm}$126 g, P=0.048) and weight on operation day (1,195${\pm}$404 g vs. 893${\pm}$151 g, P=0.042), longer hospital days (105${\pm}$29 vs. 141${\pm}$39, P=0.038), more severe bronchopulmonary dysplasia (BPD), (no/mild/moderate/severe, 2/5/2/2 vs. 0/1/2/4, P=0.038) and higher preoperative $FiO_2$ (0.29${\pm}$0.06 vs. 0.38${\pm}$0.09, P=0.02). One case of mortality due to sepsis, which was not associated with ligation, was observed among HS. Conclusion: The incidence of hemodynamic instability after PDA ligation in VLBW infants was 39%. Low BW, low weight on operation day and preoperative high $FiO_2$ might be risk factors of hemodynamic instability after PDA ligation in VLBW infants. The hemodynamic instability could increase the severity of BPD and hospital days.
Purpose: SPECT/CT, a combination of SPECT and CT, is capable of expressing the results of attenuation correction on images biased by automatic program. As a result, this research evaluates the usefulness of images with CT attenuation correction, using various phantoms and images of patients. Materials and Methods: From July of 2012 to September of 2012, this research was conducted on the contrast, spatial resolution, and images of patients. We studied the contrast with IEC body phantom and Jaszczak phantom, while the spatial resolution was evaluated with NEMA triple line phantom. Further, a comparative study was carried out on the quality of the images, on the difference between the images before and after the CT attenuation correction. Results: Compared the differences between the contrast before and after the CT attenuation correction in IEC body phantom. The contrast was improved by 33.6% at minimum, 89.8% at maximum. In case of Jaszczak Phantom, the contrast was enhanced by 9.9% at minimum, 27.8% at maximum. In NEMA Triple line phantom, the resolution was raised by 4.5% in average: 4.4% in horizontal, 4.5% in vertical. In Anthropomorphic Torso Phantom, the perfusion score of the interior wall with the most severe attenuation was measured to be 29.4%. In the experiment carried out on myocardial perfusion SPECT/CT patients, 9% improvement was discovered in the interior wall, where the most dramatic attenuation occurred, after the CT attenuation correction. Conclusion: SPECT/CT proved its clinical usefulness by enabling the acquisition of images with enhanced contrast and spatial resolution compare to the ones resulted from SPECT.
Park, Su Jin;Ahn, Moon Bae;Jang, Woori;Cho, Won Kyung;Chae, Hyo Jin;Kim, Myung Shin;Suh, Byung Kyu
Journal of The Korean Society of Inherited Metabolic disease
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v.17
no.3
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pp.103-108
/
2017
Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.
Kim, Huyn-Ah;Lee, Sook-Hwan;Cho, Sung-Won;Jeong, Hye-Jin;Son, Soo-Min;Kang, Soo-Jin;Bae, Seong-Keun;Kim, Soo-Hee;Yoon, Tae-Ki
Clinical and Experimental Reproductive Medicine
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v.31
no.2
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pp.105-110
/
2004
Objectives: Despite severe oligospermia, males with Y chromosome microdeletion can achieve conception through ICSI (Intracytoplasmic Sperm Injection). However, ICSI may not only result in the transmission of microdeletions but also the expansion of deletion to the offspring. The purpose of this study was to screen vertical transmission, expansion of microdeletions and de novo deletion in male fetuses conceived by ICSI. Materials and Methods: A total of 32 ICSI treated patients with their 33 (a case of twin) male fetuses conceived by ICSI were used to make this study group. Sequence-tagged sites (STSs)-based PCR analyses were performed on genomic DNA isolated from peripheral blood of fathers and from the amniocytes of male fetuses. Ten primer pairs namely, sY134, sY138, MK5, sY152, sY147, sY254, sY255, SPGY1, sY269 and sY158 were used. The samples with deletions were verified at least three times. Results: We detected a frequency of 12.5% (4 of the 32 patients) of microdeletions in ICSI patients. In 4 patients with detected deletions, two patients have proven deletions on single STS marker and their male fetuses have the identical deletion in this region. Another two patients have two and three deletions, but their male fetuses have more than 3 deletions which include deletions to their father's. Meanwhile, seven male fetuses, whose fathers were analyzed to have all 10 STS markers present, have deletions present in at least one or more of the markers. Conclusions: Although the majority of deletions on the Y chromosome are believed to arise de novo, in some cases a deletion has been transmitted from the fertile father to the infertile patient. In other cases the deletion was transmitted through ICSI treatment, it is likely that one sperm cell is injected through the oocyte's cytoplasm and fertilization can be obtained from spermatozoa. Our tests for deletion were determined by PCR and our results show that the ICSI treatment may lead to vertical transmission, expansion and de novo Y chromosome microdeletions in male fetuses. Because the sample group was relatively small, one should be cautious in analyzing these data. However, it is important to counsel infertile couples contemplating ICSI if the male carries Y chromosomal microdeletions.
In 1965, Rosenberg reported that platinum compounds not only inhibit growth and cell division of E. coli but also has anti-tumor activity. Since then, through animal and clinical experiments by Welsch(1971), Speer(1972), Rossof(1972), Hill(1974), and Wittes(1975), it was proved that Cis-platinum has excellent supressive effects on malignant tumor, especially on head and neck cancer. Accordingly, Cis-platinum is now widely used, sometimes without any other durg, or sometimes with Bleomycin and Methotrexate etc. Inspite of the strong anticancer effect, the use of Cis-platinum is quite often discouraged because of the reports that Cis-platinum causes auditory impairment at high frequencies above the speech range due to inner ear damage and irreversible change in the renal tubules. Since Kohonen et al(1965), Standnicki et al(1974) reported that Cisplatinum has toxic effects at the basal turn of the cochlea using guinea pig, many studies on ototoxicity after infusion of Cis-platinum have been carried out using animals. But the studies on ototoxicity in human beings can hardly be found except in reports by Piel et al(1974) and Hong et al (1979). So the authors did a study which tried to clarify the ototoxic effect by comparing the hearing level after infusion of Cis-plastinum with the hearing level before infusion of Cis-plastinum in 30 patients who was treated with Cis-platinum and admitted to the dept. of otolaryngology of Yonsei University Hospital during 2 years and a half from July. 1979 to March. 1982 and the following results were obtained. 1) The results of auditory evaluation, using the pure tone average, hearing loss of 4kHz and 8kHz, Speech Reception Threshold, PB score, SISI showed that the difference of dosage does not change the hearing level after infusion of Cis-platinum and before infusion of Cis-platinum. 2) Cis-platinum had no effect on the hearing level of patients with conductive hearing loss, or with sensorineural hearing loss, as well as with normal hearing level. 3) The infusion of Cis-platinum did not cause any change in creatinine clearance, creatinine, uric acid, but only one case showed that Cis-platinum caused severe nephrotoxicity. 4) The infusion of Cis-plastinum did not cause any change in hemoglobin, leukocyte count, platelet count and there was no correlation with the amount of infusion. 5) To see the side effect of hydration practiced with the infusion of Cis-platinum, the electrolytes, particularly the K level in the serum was measured. But the results did not show any change. 6) Judging from the results of this study mentioned above, ototoxicity caused by infusion of Cis-platinum can be prevented by sufficient hydration. Also the results might say that the appropriate method of infusion of Cis-platinum might be effective in the patients with head and neck cancer who had sensorineural hearing loss for whom the infusion of Cis-platinum has been absolutely cotraindicated.
VCUG(Voiding Cystourethrography) study is being performed to check urinary reflex symptom from bladder to ureter or kidney in the method of filling the bladder with radiation opaque contrast agent. However, VCUG study have been performed impersonally, patients have to be naked and open their legs and then void. This method is so impersonal that it is immediately needed to improve the way of testing and crete new aid. Therefore, this study through producing underwear for VCUG, analysis and compares the test time of VCUG, patient radiation dose and patient satisfaction. The target of this study was 79 Male and Female patients who visited genitourinary clinic of PNUYH for their VCUG tests AXIUM Iconos R200(Siemens Medical System : Germany) and self-produced plastic underwears made by vinyl and plasic molding machine were used. The 79 patient were divided into two groups : experiment of patient and comparison group of we patient (using aid) in order to compare, testing time, DAP (dose area product) and patient satisfaction. There was time reduction, from 35.3min of comparison group to 27.8min experimentation group by 7.5min decrease. And comparison group of $4566.6{\mu}Gym^2$(DAP) decreased experimental group (using aid) of $3411.9{\mu}Gym^2$ by $1154.7{\mu}Gym^2$. In the case of patients satisfaction study, anxiety had a drop by 3.45 of comparison group to 2.51 experimentation group by 0.94 decrease. in experimental group. And shame had a drop by 4.02 of comparison group to 3.08 experimentation group by 0.94 increase. in experimental group. Difference of the smooth voiding had a drop by 1.90 of comparison group to 2.84 experimentation group by 0.94 increase. in experimental group. In addition satisfaction had a rise by 1.19 in experimental group. There were also other opinions of uncomfortableness on it and troublesome to wear. VCUG is one of the sensitive care required test. Therefore staff and the aid can give patients such comfortable and the aid can consentive on voiding. As a result test time and DAP can be reduced. It is needed to make testing at more comfortable for patients.
To investigate the maternal and child factors associated with early detection of cerebral palsy, 74 mothers of cerebral palsy children who were born since January 1, 1980 and being treated at Taegu Rehabilitation Center for the Handicapped, Rehabilitiation Center of Taegu University, St. Paul Children's House and Pusan Welfare Association of Cerebral Palsy Children were interviewed from February to April 1987. There is no association between age of child when parents noticed the child's abnormality and educational level of father but it tend to be detected earlier when education level of mother is college or above compared with high school or under. There is a trend of earlier detection of child's abnormality although statistically not significant in case father is professional or managerial worker, monthly income of father is over \610,000, child is first-born, age of the parents is 34 years or under, child is a boy, and child has periodic well-baby check-up. The child's abnormality is detected earlier when mothers had 7 prenatal visits or more compared with those who had 6 visits or less (p<0.05). Parents noticed the child's abnormality first in 85.1% of the cases whereas doctors detected it first in 2.7% and this percentage was not different whether the child had periodic well-baby check-up or not. The first physician's diagnosis of the children was cerebral palsy in 36.5% and the rest was normal, need for observation, uncertain, etc. Parents took the child to doctor for diagnosis 2-3 months after they noticed the child's abnormality and after the child was diagnosed as cerebral palsy parents either took no therapeutic measure or brought the child to physiotherapy or acupuncture or gave herb medicine before they started specific rehabilitative therapy. For early detection of the cerebral palsy children, teaching of evaluation method for child development should be reinforced both in medical school and clinical training course and should train the specialist for diagnosis and treatment of crippling conditions. Also, public education is needed for the importance of early detection of crippling conditions and currently available methods for diagnosis and treatment.
Background: Patients with mitral regurgitation (MR) in the setting of coronary artery disease have a dismal long-term prognosis whether treated medically or surgically. Moreover, the optimal management of moderate ischemic MR at the time of coronary artery bypass grafting (CABG) remains the subjects of controversy. Thus, the present retrospective study was undertaken to determine whether mitral valve surgery for moderate ischemic MR at the time of CABG would be preferable to CABG alone in terms of clinical outcome. Material and Method: Between January 1997 and December 2003, 34 patients with moderate (Gr 3/4) ischemic MR underwent CABG alone (Group I, n=23) or CABG plus mitral valve surgery (Group II, n=11). Operative mortality, long-term survival and echocardiographic parameters were used to evaluate the efficacy of mitral valve surgery in patients with moderate ischemic MR. The mean follow-up durations of each group were $69.3\pm4.3$ months and $53.1\pm4.9$ months respectively. Result: There was no hospital mortality in both groups. There was one case of late mortality in Group I. The mean number of bypass graft was similar ($3.8\pm1.2\;vs\;3.7\pm1.3$ respectively). Cardiopulmonary bypass time was longer in group II (p=0.014). In group II, all of the patients received mitral annuloplasty using ring. On immediate postoperative echo-cardiogram, mitral regurgitation was reduced more in group II (p=0.002). Echocardiogram performed at last follow-up state showed no difference except the grade of MR between the two groups. Actuarial survival of both groups at 5 years was similar ($95.5\%\;vs\;100\%$, p=0.48). Conclusion: This study shows that in selected patients with moderate ischemic MR, CABG without mitral valve surgery might be sufficient. However, patients with low EF and NYHA functional class pre-operatively had tendency of significant residual MR, so mitral valve surgery should be necessary in these patients, and moreover, MR severity and left ventricle volume decreased more in mitral valve surgery group. Therefore, more large-scale studies are necessary to determine these effects on the ventricular function and long-term survival.
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