• Title/Summary/Keyword: Onset age

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Total Parenteral Nutrition-associated Cholestasis in Premature Infants (미숙아에서의 전비경구적 영양 관련 담즙울체)

  • Park, Kyung Pil;Kim, Se Young;Kim, Heng Mi
    • Clinical and Experimental Pediatrics
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    • v.46 no.1
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    • pp.17-23
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    • 2003
  • Purpose : Cholestasis is a major complication in prolonged use of TPN, especially in the neonatal period, but there are few long-term reviews examining the clinical course in premature infants. Thus, in this study, we reviewed premature infants with TPN-associated cholestasis(TPNAC) to determine the incidence, clinical courses and possible risk factors. Methods : Retrospective review of 66 premature infants less than 2,000 gm of birth weight and on TPN for more than two weeks was performed. Cholestasis was defined as a serum direct bilirubin level greater than 2.0 mg/dL. The clinical course of cholestasis was described, and perinatal risk factors were evaluated. Results : TPNAC developed in 21 out of 66 infants(31.8%). The onset was $41.7{\pm}17.4days$ after receiving TPN, and the mean duration was $33.6{\pm}23.4days$. The incidence of TPNAC was significantly correlated with birth weight, and gestational age, and duration of TPN. But, possible etiologic factors, such as incidence of perinatal asphyxia or infection, showed no remarkable differences between infants with TPNAC and those without TPNAC(control). The enteral intake in the third postnatal week was significantly smaller in infants with TPNAC than in the control infants(P=0.033). Conclusion : The enteral intake in the third postnatal week was smaller in the infants with TPNAC than in the control infants. Thus, the incidence of TPNAC may be reduced by increasing the amount of oral intake during TPN in high risk infants.

Renovascular Hypertension in Children (소아의 신혈관성 고혈압)

  • Kang Byoung-Chul;Ha Il-Soo;Kim In-One;Cheong Hae-Il;Choi Yong;Ko Kwang-Wook
    • Childhood Kidney Diseases
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    • v.1 no.2
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    • pp.101-108
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    • 1997
  • Purpose : The clinical characteristics of renovascular hypertension (RVHT) in children were analyzed. Methods : Medical records of 16 children diagnosed as RVHT on the basis of angiography during Jan. '86 to Jun. 94 in our hospital were reviewed retrospectively. Results : The mean age at the onset was 8.5 yrs and the sex ratio(M:F) was 7:9. The causes of RVHT were Takayasu arteritis in 6, Moyamoya disease in 5, and fibromuscular dysplasia in 3 patients. Abdominal bruit was noted in 6 patients (38%). Peripheral renin activity was raised in all tested patients. Bilateral renal arterial involvemnent was found in 9 patients (56%). Captopril renal scans showed good correlation with angiographic findings. Five patients were treated with antihypertensives only, and blood pressure was controlled completely in 2 and incompletely in 3. Percutaneous transluminal angioplasty was performed in 10 patients with 50% of success rate. However, hypertension was recurred due to restenosis or accompaning aortic stenosis in 3 patients. Surgical treatment was performed in 4 patients, and the blood pressure was controlled partially in 1 and poorly in the remaining 3. Conclusions : Takayasu arteritis, Moyamoya disease and fibromuscular dysplasia are the major causes of childhood RVHT in our country. The diagnosis of RVHT in children should be based on a set of tests individually selected for case by case. For the low curability of the current treatment modalities available, RVHT in children should not be regarded as 'curable' so far. We expect, however, that the outcome will be improved by more extensive application of the newly developed surgical technique.

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Hemolytic-uremic Syndyome Associated with Bloody Diarrhea (혈변을 동반한 용혈성 요독 증후군 5례)

  • Kim Jung-Sim;Park Eun-Jung;Chung So-Hee;Ko See-Hwan;Uhm Mee-Ryung;Park Moon-Su;Lee Heung-Jea;Jin Dong-Kyu
    • Childhood Kidney Diseases
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    • v.1 no.2
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    • pp.170-175
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    • 1997
  • Purpose : Hemolytic uremic syndrome(HUS), known as a most common cause of childhood renal failure in western countries, has been a relatively rare disease in Korea. Although the reported cases were not related to any specific cause in Korea, there was an outbreak of HUS with bloody diarrhea in Japan last year. We report here that we experienced the several typical HUS last year. Patients : From Jan.1996 to Dec. 1996, five patients were diagnosed as HUS at Samsung Seoul Medical Center, Dept. Pediatrics. Results : 1) The age of onset was below 3 years in 3 cases and above 9 years in 2 cases. 2) All the cases happened between summer and autumn. Three patients had domestic travel and 4 patients drank well or spring water before the symptoms. 3) The clinical manifestation was generalized edema, oliguria, anuria and hematuria. The bloody diarrhea were present in 5 cases and 1 patient had operation with the impression of appendicitis. 4) There was no bacteria which was isolated from the blood or stool samples. 5) Renal biopsies were performed in 2 cases, because of protracted clinical course. One showed microthrombotic angiopathy and the other cortical necrosis with necrotizing glomeruli. 6) Complete recovery was the outcome in 4 cases and one case progressed to chronic renal failure. Conclusion : In conclusion, typical HUS associated with bloody diarrhea, epidemic and good prognosis can be found in Korea and careful surveillance of the pre-clinical cases will be necessary.

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Urological Manifestations and Laboratory Findings in Patients with Tethered Cord Syndrome (Tethered cord 증후군에서 비뇨기계 증상 및 검사 소견)

  • Jung Tae-Sung;Kim Eun-Jung;Lee Eun-Sil;Shin Son-Moon;Moon Han-Ku;Park Yong-Hoon
    • Childhood Kidney Diseases
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    • v.1 no.2
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    • pp.155-160
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    • 1997
  • Purpose : Tethered cord syndrome is characterized by progressive motor and sensory disturbances in lower extremities, foot deformities caused by a pathologic fixation of spinal cord resulting in excessive stretching of the spinal cord. It is also frequently associated with urological symptoms include urinary frequency, incontinence, enuresis, urgency and recurrent urinary tract infection. Because there is few report in the literature about urological manifestations of theterd cord syndrome, we conducted a retrospective study on the patients diagnosed as tethered cord syndrome to delineate the characteristics of urologic manifestations in tethered cord syndrome and to establish the policy to evaluate patients who is suspected of tethered cord syndrome. Method : A retrospective study was conducted by reviewing the medical records of nine patients who was diagnosed as tethered cord syndrome from November 1991 to July 1996 in Yeungnam University Hospital. Result : 1) The age distribution of nine patients was as follows; 5 patients were under 2 years, 1 case from 2 to 6 years and 3 cases from 6 to 10 years. 2) Of 9 patients 6 had voiding frequency, urinary incontinence, enuresis, urgency and loss of micturation sense. Radiologic urodynamic studies revealed neurogenic bladder in 5 patients, hydronephrosis and hydroureter in 3, vesicoureteral reflux in 3. 3) Of 6 patients with urological abnormal manifestations 4 underwent spinal cord surgery (detethering). In spite of surgical intervention, the urological manifestations improved in only one patient. Conclusion : Urological abnormalities were common in tethered cord syndrome. Abnormal laboratory findings including urodynamic study were found even before the onset of urological symptoms. We have to concern tethered cord syndrome as one of common causes of voiding frequency and enuresis and to evaluate urological abnormalities as thetered cord syndrome is diagnosed.

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A Study on Epidemiologic Characteristics of Recurrent Abdominal Pain in Elementary School Children (반복성 복통증 환아의 역학적 특징에 관한 조사)

  • Oh, Sang-Hyun;Yang, Eun-Seok;Park, Sang-Kee;Park, Young-Bong;Park, Jong;Park, Sang-Hak;Moon, Kyung-Rye
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.2 no.1
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    • pp.21-29
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    • 1999
  • Purpose: The aims of this study are to examine clinical characteristics, patterns of medical care utilization, and factors which determine medical care utilization of elementary school children with recurrent abdominal pain (RAP), to find posssible factors influencing the onset and the course of the disorder. Method: We performed questionnaires in Kwangju on children from two primary schools from June,1 1998 to June 30 and carried out statistical analysis. Result: 1) Total number of questionnaires were 1417. 715 were male and 702 were female and the ratio of male to female was 1.02:1. Average age was 10.3 years. 2) 268 children had RAP (18.9%), boys 132 (18.4%), girls136 (19.2%). 3) The duration of the pain within 10 minutes was 68.5%. 178 children with RAP (66.3%) visited the doctor. The utilization pattern of medical facilities of the pupils with RAP; the most frequently utilized medical facility was pediatrics (35.2%) and the order ran as internal medicine (31.5%), and pharmacy (29.25). The utilization pattern of medical facilities for the older students; the utilization rate of pediatrics decreased, but internal medicine increased. The major factors affecting the selection of the medical facility were geographic accessibility, kindness of the personnel, good results and traffic convenience. 4) Symptoms which were accompanied with abdominal pain were headache (44.5%), chest pain (28.2%), dizziness (26.6%), vomiting (9%), and 119 children (44.5%) had no accompanied symptoms. 5) In 95 children (35.3%) abdominal pain, occured at postprandial time, in 55 children (20.5%) before meal and in 39 children (14.7%) at school. The highest incidence rate of RAP was observed on Monday (21.4%), and the lowest on Saturday (8.7%). 6) The most frequent involved part of the abdomen was periumbrical area (38%) and the order ran as epigastrium and suprapubic area. The most frequent characteristics of abdominal pain were burning pain (36.9%) and the order ran as dull, cramping and colicky pain. Conclusion: RAP is a frequent disease entitiy in children. Too many times children with RAP are treated by other departments instead of Pediatrics. A child has a peculiar growth and development which is different to those from an adult with advancing years. So, it is necessary to choose special medical care and an adequate medical facility.

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Clinical Outcome and Long Term Follow-up of Chronic Functional Constipation in Children (소아 만성 기능성 변비의 치료 성적과 장기적 예후)

  • Ahn, Yoon Jin;Park, Jae Ock
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.9 no.2
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    • pp.200-209
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    • 2006
  • Purpose: The purpose of this study was to evaluate the long term outcome and the factors contributing to treatment outcome for chronic functional constipation in children. Methods: Sixty three children were enrolled who had chronic functional constipation and could be followed by telephone contact. They were treated at the Bucheon Soonchunhyang Hospital for more than 1 month and observed from March 2001 to June 2005. We analyzed the clinical features, symptoms and signs, as well as the course and results of treatment. Results: The male to female ratio was 35 (55.6%) : 28 (44.4%). The mean age at the onset of symptoms and diagnosis was $21.1{\pm}23.5$ (1.9~84.0) months and $47.1{\pm}34.2$ (6.9~138.0) months, respectively. The mean defecation frequency before treatment was $3.2{\pm}2.3$ (0.5~10.0) times per week. The symptoms associated with constipation were as follows: soiling 34 (54.0%) which was more common in males than females, large stools in 30 (47.6%), decreased bowel movements less than three times a week in 20 (31.7%), straining during defecation in 19 (30.2%) and retentive posturing 19 (30.2%). The mean duration of follow-up was $34.2{\pm}14.6$ (3.6~60.0) months and 44 (69.8%) patients had their symptoms resolve ("success") and 19 (30.2%) were not resloved ("fail") from the constipation. The time for recovery from soiling, straining during defecation and retentive posturing after treatment was $4.3{\pm}2.4$ (1.0~36.0), $5.0{\pm}1.4$ (0.8~36.0) and $5.0{\pm}3.1$ (1.0~36.0) months, respectively. A relapse of the constipation occurred in 15 (23.8%) patients, 9 (60%) boys and 6 (40%) girls. The time to relapse after cessation of treatment was $2.9{\pm}1.9$ (1.0~6.0) months and the only risk factor associated with relapse was the initial duration of treatment. Conclusion: Most of the patients had resolution of symptoms within five months after treatment; relapse occurred within three months after the interruption of treatment. The duration of treatment was important for recovery and for the prevention of relapse in the constipated children. Thus a long term maintenance of therapy and follow-up is necessary for chronic functional constipation in children.

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Supraclavicular Brachial Plexus block with Arm-Hyperabduction (상지(上肢) 외전위(外轉位)에서 시행(施行)한 쇄골상(鎖骨上) 상완신경총차단(上腕神經叢遮斷))

  • Lim, Keoun;Lim, Hwa-Taek;Kim, Dong-Keoun;Park, Wook;Kim, Sung-Yell;Oh, Hung-Kun
    • The Korean Journal of Pain
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    • v.1 no.2
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    • pp.214-222
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    • 1988
  • With the arm in hyperabduction, we have carried out 525 procedures of supraclavicular brachial plexus block from Aug. 1976 to June 1980, whereas block with the arm in adduction has been customarily performed by other authors. The anesthetic procedure is as follows: 1) The patient lies in the dorsal recumbent position without a pillow under his head or shoulder. His arm is hyperabducted more than a 90 degree angle from his side, and his head is turned to the side opposite from that to be blocked. 2) An "X" is marked at a point 1 cm above the mid clavicle, immediately lateral to the edge of the anterior scalene muscle, and on the palpable portion of the subclavian artery. The area is aseptically prepared and draped. 3) A 22 gauge 3.5cm needle attached to a syringe filled with 2% lidocaine (7~8mg/kg of body weight) and epineprine(1 : 200,000) is inserted caudally toward the second portion of the artery where it crosses the first rib and parallel with the lateral border of the muscle until a paresthesia is obtained. 4) Paresthesia is usually elicited while inserting the needle tip about 1~2 em in depth. If so, the local anesthetic solution is injected after careful aspiration. 5) If no paresthesia is elicited, the needle is withdrawn and redirected in an attempt to elicit paresthesia. 6) If, after several attempts, no paresthesia is obtained, the local anesthetic solution is injected into the perivascular sheath after confirming that the artery is not punctured. 7) Immediately after starting surgery, Valium is injected for sedation by the intravenous route in almost all cases. The age distribution of the cases was from 11 to 80 years. Sex distribution was 476 males and 49 females (Table 1). Operative procedures consisted of 103 open reductions, 114 skin grafts combined with spinal anesthesia in 14, 87 debridements, 75 repairs, i.e. tendon (41), nerve(32), and artery (2), 58 corrections of abnormalities, 27 amputations above the elbow (5), below the elbow (3) and fingers (17), 20 primary closures, 18 incisions and curettages, 2 replantations of cut fingers. respectively (Table 2). Paresthesia was obtained in all cases. Onset of analgesia occured within 5 minutes, starting in the deltoid region in almost all cases. Complete anesthesia of the entire arm appeared within 10 minutes but was delayed 15 to 20 minutes in 5 cases and failed in one case. Thus, our success rate was nearly 100%. The duration of anesthesia after a single injection ranged from $3\frac{1}{2}$ to $4\frac{1}{2}$, hours in 94% of the cases. The operative time ranged from 0.5 to 4 hours in 92.4% of the cases(Table 3). Repeat blocks were carried out in 33 cases when operative times which were more than 4 hours in 22 cases and the others were completed within 4 hours (Table 4). Two patients of the 33 cases, who received microvasular surgery were injected twice with 2% lidocaine 20 ml for a total of $13\frac{1}{2}$ hours. The 157 patients who received surgery on the forearms or hands had pneumatic tourniquets (250 torrs) applied without tourniquet pain. There was no pneumothorax, hematoma or phrenic nerve paralysis in any of the unilateral and 27 bilateral blocks, but there was hoarseness in two, Horner's syndrome in 11 and shivering in 7 cases. No general seizures or other side effects were observed. By 20ml of 60% urcgratin study, we confirm ed the position of the needle tip to be in a safer position when the arm is in hyperabduction than when it is in adduction. And also that the humoral head caused some obstraction of the distal flow of the dye, indicating that less local anesthetic solution would be needed for satisfactory anesthesia. (Fig. 3,4).

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X-linked Adrenoleukodystrophy in Childhood (X 염색체 연관 부신백질이영양증 환아들의 임상양상)

  • Yoo, Eun Jung;Kim, Eun Young;Kook, Hoon;Woo, Young Jong
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.5 no.1
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    • pp.33-43
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    • 2005
  • Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.

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DENTAL TREATMENT IN A PATIENT WITH FUKUYAMA TYPE MUSCULAR DYSTROPHY UNDER TOTAL INTRAVENOUS ANESTHESIA USING PROPOFOL (후쿠야마 선천성 근이영양증 환자의 프로포폴을 이용한 전정맥마취 하 치과치료)

  • Jin, Dallae;Shin, Teo-Jeon;Hyun, Hong-Keun;Kim, Young-Jae;Kim, Jung-Wook;Lee, Sang-Hoon;Kim, Chong-Chul;Jang, Ki-Taeg
    • Journal of the korean academy of Pediatric Dentistry
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    • v.40 no.1
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    • pp.66-71
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    • 2013
  • Muscular dystrophy is a genetically heterogeneous group of disorders characterized by progressive muscle weakness of variable distribution and severity. Fukuyama type congenital muscular dystrophy (FCMD) is an unusual form of muscular dystrophy with autosomal recessive inheritance and is clinically characterized by an early age of onset, severe central nervous system involvement, facial muscle weakness, and multiple joint contractures. Muscular dystrophy is susceptible to perioperative respiratory, cardiac and other complications. Patients with FCMD have upper airway muscle weakness, therefore general anesthesia is preferred to sedation regarding maintaining the airway when treating these patients. The development of malignant hyperthermia in general anesthesia for patients with muscular dystrophy is a concern. Total intravenous anesthesia should be used instead of inhaled anesthetics because of the risk of malignant hyperthermia. A 3-year-9-month old, 13kg girl with Fukuyama type congenital muscular dystrophy was scheduled for dental treatment under general anesthesia. She had multiple caries and 14 primary teeth needed caries treatment. Prior to general anesthesia, oral premedication with 9 mg midazolam was given. General anesthesia was induced and maintained with target controlled infusion of propofol $3{\sim}3.5{\mu}g/mL$. The patient with progressive muscular dystrophy was successfully treated under total intravenous anesthesia with a target controlled infusion of propofol. There were no complications related to anesthesia and dental treatment during or after the operation. This case suggests that target controlled infusion of propofol is a safe and appropriate anesthetic technique in FCMD patients for dental treatment.

THE EFFECTIVENESS OF TWO SEDATIVE REGIMEN USED FOR CHILD PATIENT WHO FAILED TO ORAL CHLORAL HYDRATE (Chloral hydrate 경구 진정에 실패한 소아환자를 대상으로 사용한 두 가지 진정요법의 효과)

  • Lee, Sang-Min;Kim, Jong-Soo;Kim, Yong-Kee
    • Journal of the korean academy of Pediatric Dentistry
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    • v.27 no.4
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    • pp.505-516
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    • 2000
  • Chloral hydrate is one of the most widely used sedative agents to control the difficult-to-treat young age group in the dental clinic. We are often frustrated to see the patient still awake and cry with agitation even after far more than the normal onset time of Chloral hydrate. In such a case, the patient has to be rescheduled for another sedation visit with different agents and/or routes which greatly disappoints the guardians. This study was designed to test the efficacy of one sedative regimen that can possibly help the clinician complete scheduled treatment without postponement. We have tried sleep induction with mixed gas of Enflurane(2vol%) and $N_2O(50%)-O_2(50%)$ for $60\sim120$ seconds to 35 patients of those who failed to respond properly to the dose(70mg/kg)of oral Chloral hydrate. The Result of this regimen was compare to those of two oral regimen of Chloral hydrate/Hydroxyzine and Chloral hydrate only Analyses of result on vital signs and behavior pattern were performed. The outcome of the study suggest that sleep induction by a short inhalation of low dose of $Enflurane/N_2O-O_2$ provide dentist with suitable condition for the completion of scheduled treatment in the patient who failed to oral Chloral hydrate. Evidence of adverse effect was not detected or reported during and/or after the procedures.

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