• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.669-674
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• 2005

Nephrogenic diabetes insipidus (NDI) is a disorder in which the secretion of antidiuretic hormone is normal, but the response of the renal collecting tubules to vasopressin is impaired. Compared with acquired NDI (a-NDI), which is secondary to chronic bilateral incomplete urinary tract obstruction with hydronephrosis, congenital NDI (c-NDI) is a very rare heritable disorder that usually follows the X- linked recessive pattern. Clinical symptoms of c-NDI can be non specific, and often the disease ultimately results in failure to thrive, or mental retardation. Recently, the diagnosis can be confirmed by direct sequencing analysis of the peripheral blood specimens. The long-term results of treatment for c-NDI are not satisfactory. Reports on the follow up of c-NDI cases are rare and there is no report on the cases treated with combinations of three drugs. We report herein a case of severe c-NDI in an 8 year-old-boy with a severely dysconfigurated urinary tract system. The patient and his mother showed a frameshift mutation on the AVPR2 gene on chromosome Xq28:.847_851delTGCTG (p.C283fsX90). The patient showed normal growth and development by treatment with combinations of hydrochlorothiazide ($65mg/m^2$), amiloride (0.3 mg/kg/d) and indomethacin ($100mg/m^2$), yet after five years he needed adjuvant cystostomy to relieve him from the residual symptoms of urgency with polyuria.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.490-494
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• 2003

Purpose : We'd like to determine the incidence of congenital heart disease and arrhythmia in elementary school children in Busan, and to provide adequate prevention and treatment. Methods : A total of 23,802(male 12,909, female 10,893) 1st grade elementary school children living in Busan were studied. All children were 7-8 years old. We obtained their medical history by questionnaire and checked elecrocardiography(ECG). Subsequent screening tests including a 2nd ECG, chest X-ray, phonocardiogram and CBC for the students who had abnormal findings at the first screening test. The third screening test was done for students who had cardiac murmurs or abnormal ECG findings in the second screening test by echocardiogram, treadmill test and 24-hour Holter monitoring. Results : Among 23,802 children participants, 605(2.54%) had abnormal ECG findings at the first screening test. Q wave abnormality(0.58%) was observed most frequently, and complete right bundle branch block(RBBB)(0.26%), sinus tachycardia(0.24%), right axis deviation(0.22%) and ventricular premature contraction(VPC)(0.21%) followed in order. Four hundred and twenty four children participated in the second ECG screening test. Two hundred and two children(47.6%) had an abnormality such as sinus tachycardia(18.8%), VPC(17.8%), or complete RBBB(17.3%). After completing the third examination including echocardiogram, we couldn't find the students with ventricular tachycardia (VT) or SVT at the third arrhythmia screening test. Conclusion : A high incidence of arrhythmia was found in the 1st grade elementary school children in Busan despite their healthy appearances, although fatal heart diseases were not noted. Early diagnosis, adequate preventative measures and treatment will prevent and decrease the frequency of emergent situations like syncope and sudden death.

• Clinical and Experimental Pediatrics
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• v.52 no.9
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• pp.1035-1037
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• 2009

Patent ductus arteriosus (PDA) is a common congenital heart defect. All PDAs, regardless of size or degree of symptoms, require occlusion. Transcatheter PDA occlusion features fewer complications than trans-thoracic closure. It is also more cost-effective and has an excellent occlusion rate. Therefore, transcatheter PDA occlusion is accepted as the standard treatment option for PDA. However, tubular-type PDAs are difficult to close with ordinary detachable coils or the Amplatzer Duct Occluder; thus, these lesions remain a challenge for transcatheter closure. We attempted to occlude a tubular-type PDA by using an oversized Amplatzer Vascular Plug, which allowed intraluminal packing of the ductus. By using this treatment method, PDA occlusion was achieved safely with an excellent final outcome. We suggest that this approach may be a good option for transcatheter closure of a tubular-type PDA.

• Journal of Chest Surgery
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• v.25 no.7
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• pp.769-776
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• 1992

Total 632 cases of open heart surgery were performed in the department of thoracic and cardiovascular surgery, Pusan Paik Hospital, Inje University from october 1985 to december 1990. Among them, there were 503 cases of congenital heart disease and 129 cases of acquired heart disease. In the cases of 503 congenital heart disease surgically corrected, 122 cases over 15 years of age were reviewed and its results were summarized as follows ; l. In total 122 cases 65 were male and 52 female. 2. There were 54 patients under 20 years of age, 31 between 20 & 24 years, 20 between 25 & 29 and 17 over 30 years of age. The eldest was a 46-year-old female of atrial septal defect group. 3. There were 63 cases of VSD[51.7%], 38 of ASD[31.2%], 11 cases of TOF[9%] and so on. 4. In recovery cases, main post-operative complications were as follows; cardiac tamponade, arrhythmia, low cardiac output syndrome, pulmonary, edema pericarditis, mediastinitis, sepsis and DIC. 5. There were 3 cases of post-operative death in this series, so operative mortality rate was 2.5% compairing with 1.8% of pediatric cases below 15 years of age. 6. This reviewed series reveal that aggressive surgical approach in adult congenital heart disease can be justified with low operative mortality like as pediatric age group.

• Journal of the Korean Crystal Growth and Crystal Technology
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• v.8 no.2
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• pp.193-204
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• 1998

The single crystal of the $LiTaO_3$has large electro-optic effects, so it is applied to optical switch, acousto-optic deflector, and optical memory device as hologram using photorefractive effect. In this study, optic-grade undoped $LiTaO_3$and Fe:LiTaO$LiTaO_3$single crystals were grown by the Czochralski method and optical transmission and absorption spectrums were measured in the wavelength of UV-VIS range. The curie temperature was determined with DSC and by measuring capacitance for the grown undoped crystal and ceramic powder samples of various Li/Ta ratio. In case of having a 48.6 mol% $Li_2O$ as a starting Li/Ta ratio, the results of concentration variations were below 0.01 mol% $Li_2O$ all over the crystal, so it was confirmed that $LiTaO_3$single crystals were grown under congruent melting composition having optical homogeneity. The curie temperature of the Fe:$LiTaO_3$crystal was increased with increased with increased doped Fe concentrations;by the ratio of $7.5^{\circ}C$ increase per Fe 0.1 wt%. Also, the optical transmittance was about 78 %, which was sufficient for optical device.

• Neonatal Medicine
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• v.16 no.1
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• pp.64-70
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• 2009

Congenital diaphragmatic hernia (CDR) with severe pulmonary hypoplasia is associated with significant mortality. Recently, several new therapeutic methods have been suggested, such as high-frequency oscillatory ventilation and inhaled nitric oxide. For hypoxemic respiratory failure unresponsive to these advanced medical treatment options, extracorporeal membrane oxygenation (ECMO) serves as the last potentially effective treatment. An understanding of the pathophysiology of pulmonary hypertension associated with CDH led to a strategy involving preoperative stabilization and delayed surgical intervention with ECMO. We describe four cases of ECMO, including the first report of ECMO for neonatal CDH in Korea.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.583-588
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• 2005

Congenital tracheal stenosis can be a life-threatening disease, especially in cases involving the long-segment of the trachea. When patients are symptomatic immediately after birth or develop an accompanying complex cardiac anomaly, surgical repair can be a considerable challenge. We experienced a tracheoplasty in one early infant weighing 2.6 kg and one neonate who had ventilator dependency from long-segment congenital tracheal stenosis and congenital cardiac anomaly. One early infant, who had diffuse stenosis of distal trachea after ventricular septal defect closure, underwent resection and extended end to end anastomosis. One neonate who had diffuse stenosis of proximal trachea with tetralogy of Fallot (TOF), underwent slide tracheoplasty with total correction for TOF Postoperative chest computed tomography showed widely patent trachea. Both infants are now well without symptoms.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1270-1275
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• 1996

Pulmonary artery aneurysms are extremely rare conditions usually associated with congenital cardiac defects that cause increased pulmonary blood flow and pulmonary hypertension. The prognosis of pulmonary artery aneurysms is fatal due to the potential for rupture of the aneurysm and the underlying severe pulmonary hypertension. A 40-year old woman was admitted to our hospital with headache following traffic accident. On admission a continuous murmur was heard over the 2nd to 3rd intercostal space along left sternal border and a calcified cystic mass at left hilar portion was incidentally discoverd on chest reontgenogram. Cardiac catherization was diagnostic of a left to right shunt at main pulmoanry artery level and pulmonary hypertension. Pulmonary angiogram demonstrated a large aneurysm of main pulmonary artery extending into proximal left pulmonary artery. The pulmonary artery aneurysm associated with patent ductus arteriosus was diagnosed definitively and the patient was underwent resection of pulmonary artery aneurysm, closure of PDA and Dacron prosthetic graft replacement under cardiopulmonary bypass. The postoperative course was uneventful and the patient was discharged without any problem.

• Journal of Music and Human Behavior
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• v.2 no.2
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• pp.15-31
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• 2005

Terminal pediatric patient include congenital metabolic abnormalities, chromosome aberrations, congenital anomalies, neuromuscular diseases and other incurable conditions as well as malignant tumors. One third of these diseases are cancers, and two thirds of cancers are lymphadenoma and leukemia. Terminal pediatric patient may feel fear, anger and frustration against treatment process in hospitals, lose control of themselves and stay in helplessness due to restrictions within controlled hospital environment. This study examined the relationship between hospitalized childrens behavior and music using Contextual Support Model of Music Therapya theory stating therapeutic music environments possess three elements of Structure, Autonomy support and Involvement, and increase childrens active engagement. Focused on these three therapeutic elements, this study analyzed music therapy cases of terminally ill children to examine the relationship between childrens behavior and music as environmental supportive medium in the environment. This study is on a single case case subject, a 10-year-old girl with acute lymphocytic leukemia. Nine sessions of music therapy activities were conducted and analyzed using qualitative method. Focusing on three therapeutic elements of Contextual Support Model, analysis of sessions was made on the basis of activities specifically designed for this study. Main music therapy activities included singing while playing musical instruments, singing while listening to music through CD player, making up songs, and searching for song lines. The findings of this study are as follows: a) in terms of Structure, music therapy suggested a direction for effective musical activities for terminally ill children by providing environments where child can sing, play musical instruments and make up songs; b) in terms of Autonomy support, music therapy encouraged childs voluntary participation by giving her chances to make choice while singing or playing musical instruments; c) in terms of Involvement, child could gain positive approval of the therapist and conduct musical activities in cooperation with the therapist. Music therapy serves as supportive medium in the environment, develops spontaneity and self-reliance in emotionally depressed children, and promotes voluntary attitudes in the restricted hospital environment. Based on the positive effects of music therapy, more studies need to be conducted with an approach to more various terminal pediatric patients.

• Korean Journal of Clinical Laboratory Science
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• v.53 no.2
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• pp.151-157
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• 2021

Charcot-Marie-Tooth disease (CMT) is a slowly progressive hereditary degenerative disease and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated disorder characterized by weakness and sensory deficits. The purpose of this study was to analyze and compare the electrophysiological characteristics observed in sensory nerve conduction studies (SNCS) of both diseases. A retrospective study of 65 patients with a diagnosis of CIDP (N=35) and CMT type I (N=30) was performed. This study analyzed No potentials ratio, distal compound nerve action potential (dCNAP) of various nerve types, and a correlation coefficient analysis of the sensory nerve conduction velocity (SNCV). As a result, I found that CMT 1 was more severe systemic demyelinating and axonal polyneuropathy better than CIDP (P<0.05). In a quantitative analysis of dCNAP and SNCV, especially sural nerve was the most severe nerve injury observed in both diseases. In correlation and scatter plot analysis, CMT 1 showed relatively high correlations compared to CIDP based on the correlation coefficient analysis (Fisher's Z test) of SNCV. The results of this study suggested that CMT 1 showed the slowness in SNCV, one of the characteristics of demyelinating polyneuropathy, and this slowing had a uniform pattern. In conclusion, electrophysiological characteristic of SNCS may be useful in the diagnosis and research between patients with CMT 1 and CIDP.