• Title/Summary/Keyword: DOWN SYNDROME

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A Clinical Analysis of the Intestinal Atresia (선천성 장 폐쇄증의 임상적 분석)

  • Park, Jin-Young
    • Advances in pediatric surgery
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    • v.10 no.2
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    • pp.99-106
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    • 2004
  • Intestinal atresia is a frequent cause of intestinal obstruction in the newborn. We reviewed the clinical presentation, associated anomalies, types of atresias, operative managements, and early postoperative complications in 36 cases of intestinal atresia treated at the Department of Surgery, Kyungpook National University Hospital between January 1994 and February 2003. Location of the lesion was duodenum in 17 patients, jejunum in 11 patients and ileum in 8 patients. The male to female ratio was 1:1.4 in duodenal atresia (DA), 2.7:1 in jejunal atresia (JA) and 7:1 in ileal atresia (IA). The most common type was type III (41.1 %) in DA, and type I (52.6 %) in JA and IA. The most common presenting symptoms was vomiting(88.2 %) in DA, but in jejunoileal atresia, vomiting(89.4 %) and abdominal distension(89.4 %) were the most common sign and symptom. All cases of DA were diagnosed by plain abdominal radiography. There were 6 cases of DA with congenital heart disease, 3 cases of DA with Down syndrome and 3 cases of JA with meconium peritonitis. Segmental resection was performed in 13 cases, duodenoduodenostomy in 11 cases, membrane excision in 7 cases, jejunojejunostomy in 2 cases, gastroduodenostomy in 2 cases and ileocolic anastomosis in 1 case. There were 9 postoperative complications including 3 each of anastomotic leakage, wound infection, and intestinal obstruction 3 cases. The mortality rate for DA was 11.8 %(2/17). Both deaths in DA were attributed to congenital heart disease. The mortality rate for JA was 18% (2/11). Both cases died with sepsis and short bowel syndrome.

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MULTIPLE CONGENITAL MISSING TEETH : CASE REPORT (선천성 다수 영구치 결손 환아의 증례보고)

  • Shin, Jeong-Geun;Kim, Jae-Gon;Yang, Yeon-Mi;Kim, Sung-Hee;Baik, Byeong-Ju
    • Journal of the korean academy of Pediatric Dentistry
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    • v.33 no.1
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    • pp.122-130
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    • 2006
  • Congenital Missing Teeth(hypodontia, oligodontia) is the developmental absence of one or more teeth. It has been reported as being the most common anomaly of dental development in human, relatively common in the permanent dentition. In a recent review, Vastardis has quoted incidence ranges of $1.6%{\sim}9.6%$ in the permanent dentition. Brook has quoted a prevalence of $3.5%{\sim}6.5%$ in most populations, with severe hypodontia, defined as the absence of six or more teeth, having a prevalence of $0.3{\sim}0.4%$. The most commonly affected teeth are third molars, followed by maxillary lateral incisor, and second premolars. The etiology is unknown, several hypotheses include trauma, nutritional deficiency, infection, metabolic abnormalities, systemic disease and genetic influence. The multiple congenital missing is commonly associated with specific syndrome or severe systemic abnormalities such as cleft lip & palate and Down's syndrome. These cases present that children have multiple congenital missing teeth in the permanent dentition, without any systemic disease. Management of this condition must be considered orthodontic and prosthodontic treatment comprehensively. In these cases, children were treated by space maintainer or orthodontic appliance and follow-up checked.

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Expression of Toll-like Receptors, Pro-, and Anti-inflammatory Cytokines in Relation to Gut Microbiota in Irritable Bowel Syndrome: The Evidence for Its Micro-organic Basis

  • Shukla, Ratnakar;Ghoshal, Ujjala;Ranjan, Prabhat;Ghoshal, Uday C
    • Journal of Neurogastroenterology and Motility
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    • v.24 no.4
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    • pp.628-642
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    • 2018
  • Background/Aims A Subset of patients with irritable bowel syndrome (IBS) may have mild inflammation due to immune activation. Toll-like receptors (TLRs) and cytokines may cause intestinal inflammation. We studied their expression in relation to gut microbiota. Methods Expression of TLRs and cytokines was assessed in 47 IBS patients (Rome III) and 25 controls using quantitative real-time polymerase chain reaction. Immunohistochemistry was further performed to confirm the expression of TLR-4 and TLR-5. Results Of 47 patients with IBS, 20 had constipation (IBS-C), 20 diarrhea (IBS-D), and 7 unclassified (IBS-U). The mRNA levels of TLR-4 and TLR-5 were up-regulated in IBS patients than controls (P = 0.013 and P < 0.001, respectively). Expression of TLR-4 and TLR-5 at protein level was 4.2-folds and 6.6-folds higher in IBS-D than controls. The mRNA levels of IL-6 (P = 0.003), C-X-C motif chemokine ligand 11 (CXCL-11) (P < 0.001) and C-X-C motif chemokine receptor 3 (CXCR-3) (P < 0.001) were higher among IBS patients than controls. Expression of IL-6 (P = 0.002), CXCL-11 (P < 0.001), and CXCR-3 (P < 0.001) were up-regulated and IL-10 (P = 0.012) was down-regulated in IBS-D patients than controls. Positive correlation was seen between TLR-4 and IL-6 (P = 0.043), CXCR-3, and CXCL-11 (P = 0.047), and IL-6 and CXCR-3 (P = 0.003). Stool frequency per week showed positive correlation with mRNA levels of TLR-4 (P = 0.016) and CXCR-3 (P = 0.005), but inversely correlated with IL-10 (P = 0.002). Copy number of Lactobacillus (P = 0.045) and Bifidobacterium (P = 0.011) showed correlation with IL-10 in IBS-C, while Gram-positive (P = 0.031) and Gram-negative bacteria (P = 0.010) showed correlation with CXCL-11 in IBS-D patients. Conclusions Altered immune activation in response to dysbiotic microbiota may promote intestinal inflammation in a subset of patients with IBS.

Hair Heavy Metal Contents in Mentally Retarded Children II - In Association with Cadmium and Zinc - (정신지체아 두발 중 중금속 함량 II - 카드뮴 및 아연과의 관련성 -)

  • Park, Soon-Woo;Lee, Jong-Young;Kim, Doo-Hie
    • Journal of Preventive Medicine and Public Health
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    • v.22 no.2 s.26
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    • pp.215-222
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    • 1989
  • The relationship between cadmium level and mental retardation was investigated. The 297 subjects with mental retardation were drawn from two schools providing special educational services, one, consisted of children living in an orphan home, another, children with parents. The 117 control subjects were drawn from whom had got average or above average academic achivement in a general elementary school. Hair sample was taken from the nape of the neck and the cadmium and zinc analysis were carried out on an atomic absorption spectrophotometer (IL 551). Children in the retarded group had significantly higher cadmium levels compared with control but not in zinc levels. There was no relationship between metal concentrations and age except control male group, which showed significant positive linear relationship in zinc, and there was no difference between sex in both metal except the male orphan group in cadmium. In the orphan group, there was relationship between severity of retardation and cadmium concentration in both sex but not in retarded children with parents. No difference in cadmium levels between the group with Down's syndrome, one of causes of mental retardation, and the control group suggested the cadmium as a possible cause of mental retardation. In the case of accompanying autism, zinc level was significantly lower than that of other accompanying diseases. Although not establishing an etiologic relationship, findings of this study suggest that there are some influeces of cadmium on mental retardation, and call for a continuing study.

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Moderate and Deep Hypothermia Produces Hyporesposiveness to Phenylephrine in Isolated Rat Aorta

  • Cho, Jun Woo;Lee, Chul Ho;Jang, Jae Seok;Kwon, Oh Choon;Roh, Woon Seok;Kim, Jung Eun
    • Journal of Chest Surgery
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    • v.46 no.6
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    • pp.402-412
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    • 2013
  • Background: Moderate and severe hypothermia with cardiopulmonary bypass during aortic surgery can cause some complications such as endothelial cell dysfunction or coagulation disorders. This study found out the difference of vascular reactivity by phenylephrine in moderate and severe hypothermia. Methods: Preserved aortic endothelium by excised rat thoracic aorta was sectioned, and then down the temperature rapidly to $25^{\circ}C$ by 15 minutes at $38^{\circ}C$ and then the vascular tension was measured. The vascular tension was also measured in rewarming at $25^{\circ}C$ for temperatures up to $38^{\circ}C$. To investigate the mechanism of the changes in vascular tension on hypothermia, NG-nitro-L-arginine methyl esther (L-NAME) and indomethacin administered 30 minutes before the phenylephrine administration. And to find out the hypothermic effect can persist after rewarming, endothelium intact vessel and endothelium denuded vessel exposed to hypothermia. The bradykinin dose-response curve was obtained for ascertainment whether endothelium-dependent hyperpolarization factor involves decreasing the phenylnephrine vascular reactivity on hypothermia. Results: Fifteen minutes of the moderate hypothermia blocked the maximum contractile response of phenylephrine about 95%. The vasorelaxation induced by hypothermia was significantly reduced with L-NAME and indomethacin administration together. There was a significant decreasing in phenylephrine susceptibility and maximum contractility after 2 hours rewarming from moderate and severe hypothermia in the endothelium intact vessel compared with contrast group. Conclusion: The vasoplegic syndrome after cardiac surgery might be caused by hypothermia when considering the vascular reactivity to phenylephrine was decreased in the endothelium-dependent mechanism.

A STUDY OF ORAL STATUS OF MENTAL RETARDED CHILDREN (정신(精神) 박약아(薄弱兒)의 구강(口腔) 상태(狀態)에 관(關)한 고찰(考察))

  • Jhee, In-Ae
    • Journal of the korean academy of Pediatric Dentistry
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    • v.8 no.1
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    • pp.77-88
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    • 1981
  • The purpose of this study was to make a comprehensive study & evaluation of the oral status of mental retarded children. The auther examined intraorally 486 (male; 311, female;175) mental retarded children. The result was as follows; (General mental retarded children means the children who live in their parent's home, & orphan mental retarded children means the children who live in orphanage.) 1. The dft rate was 31.6% in general mental retarded children (G.m.r.c.) & 20.7% in orphan mental retarded children (O. m. r. c.). The dft index was 3.73 in G.m.r.c. & 2.15 in O.m.r.c. 2. The DMFT rate was 24.6% in female G.m.r.c., 16.7% in male G.m.r.c., 12.7% in female O.m.r.c., 8.4% in male O.m.r.c. The DMFT index was 4.94 in female G.m.r.c., 4.01 in male G.m.r.c., 1.40 in male O.m.r.c., 2.75 in female O.m.r.c. 3. The malocclusion prevalence was 57.3%. the class I malocclusion was 14.2% Class II malocclusion 19.3%, Class III malocclusion 23.5%. The children with Down's syndrome had 60.0% of class III malocclusion prevalence. 4. The dental calculus index was 1.97 in male O.m.r.c., 1.81 in female O.m.r.c., 1.30 in male G.m.r.e., 1.13 in female G.m.r.c. 5. The dental plaque index was 3.06 in female G.m.r.c., 3.00 in male Gm.r.e. 2.70 in male O.m.r,c., 2.32 in female O.m.r.c.

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Two Cases of Stroke Patient's Pruritic Dermatoses Treated with Sopung-San(Xiaofeng-San) (중풍(中風) 환자(患者)의 피부(皮膚) 소양증(瘙痒症)에 소풍산(消風散) 투여(投與) 2례(例))

  • Kim, Tai-Kyung;Kim, Jung-Yul;Kang, Kyung-Suk;Ruy, Soon-Hyun;Bae, Hyung-Sub;Choi, Yo-Sub
    • The Journal of Internal Korean Medicine
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    • v.23 no.2
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    • pp.280-285
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    • 2002
  • Pruritus is [CHECK DEFN] itchy feeling of one's skin. We often face stroke patients who complain about Pruritus. They occasionally fail to fall asleep or have a secondary infection as a result of scratching. For these reasons, severe Pruritus brings down general condition and interferes with recovery. The causes of Pruritus are distributed to from skin diseases and from internal diseases. Especially among the skin diseases, Xerotic Eczema, which is called Senile Eczema causes the dry skin in the elderly, especially lower limb's extensor part. According to the epidemiology, 20% of the old have Xerotic Eczema. In oriental medicine, Pruritus is called Pungsoyang(風瘙痒), Pungyang(風痒), Yangpung(痒風), Sinyang(身痒). The cause of Pruritus is divided into two. One is endogenous factors and the other is exogenous factors. The former are deficiency of blood(血虛), blood fever(血熱), wind-heat due to internal damage(內傷風熱), damp-heat in the liver and gallbladder(肝膽濕熱), endogenous wind stirring in the liver(肝風內動), deticiency syndrome of the spleen(脾虛), deficiency of Yin of the liver and kidneys(肝腎陰虛) and deficiency of the Penetration and Conception Vessels(衝任不足). The latter are wind-cold due to exogenous affection(外感風寒) and wind-heat due to exogenous affection(外感風寒). We report two stroke patients who complained of severe Pruritus They were diagnosed as having Xerotic Eczema by a dermatologist. We regarded their Pruritus as blood fever(血熱) and wind-heat(風熱) and prescribed Sopung-San to these patients. These patients showed significant improvement.

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Complicated Wound Infection Following Transvenous Endocardial Pacemaker (경정맥(經靜脈) 내적(內的) Pacemaker 이식후(移植後) 발생(發生)한 복잡한 감염합병증(感染合?症)의 1치험례(治驗例))

  • Lee, D.Y.;Yoon, Y.J.;Cho, B.K.;Hong, S.N.
    • Journal of Chest Surgery
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    • v.9 no.2
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    • pp.265-270
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    • 1976
  • Implantation of a permanent pacemaker is a widely accepted procedure for the patient with complete heart block.As a result of these device, the prognosis for patients with Adams-Stokes syndrome caused by complete A-V block and other cardiac arrhythmia have become much more optimistic. Permanent pacemaker implantation by means of a transvenous approach has made the operative risk much less and the procedure simpler. However, a number of complications have been reported in the literature regarding transvenous endocardial pacemaker implantation during the last a decade. The patient presented in this paper is a 26-year old girl who was implanted with a permanent pacemaker at 14 years of age because of a congenital A-V block. Following first exchange of pulse generator, the electrode (lead) was fractured, so that by the pulse generator, a change to the transvenous technique of implantation was made, After this, there were episodes of recurrent wound infection on three occasions, even though the site of pulse generator implantation was exchanged to the contralateral side of chest wall, massive doses of antibiotics were administered and sensitivity tests for coagulase positive staphylococcal infection were performed. Though there was no definite evidence of blood stream infection by blood culture, we decided not to use the transvenous technique and not to implant the pulse generator in the chest wall because the venous system and the entire anterior chest wall appeared to be diseased or contaminated by virulent pyogenic organisms. Finally this intractable systemic and local wound infection was successfully controlled by myocardial lead implantation via a subxiphoid approach and implantation of the pulse generator far down in the abdominal wall. The causes and routes of recurrent wound infection and possible blood born infection in this particular patient are still obscure. We strongly believe that myocardial pacemaker implantation is much safer than transvenous endocardial pacemaker implantation & myocardial pacemaker implantation is a definite method for controlling such an intractable wound infection. following transvenous pacemaker implantation.

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Endoscopic Spinal Surgery for Herniated Lumbar Discs

  • Shim, Young-Bo;Lee, Nok-Young;Huh, Seung-Ho;Ha, Sang-Soo;Yoon, Kang-Joan
    • Journal of Korean Neurosurgical Society
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    • v.41 no.4
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    • pp.241-245
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    • 2007
  • Objective : So called "minimally invasive procedures" have evolved from chemonucleolysis, automated percutaneous discectomy, arthroscopic microdiscectomy that are mainly working within the confines of intradiscal space to transforaminal endoscopic technique to remove herniated epidural disc materials directly. The purpose of this study is to assess the result of endoscopic spinal surgery and favorable indication in the thoracolumbar spine. Methods : The records of 71 patients, 73 endoscopic procedures, were retrospectively analysed. Yeung Endoscopic Spine Surgery system with 7 mm working sleeve and $25^{\circ}$ viewing angle was used. The mean follow up period was 6 months [range, 3-9]. Results : Operated levels were from T12-L1 disc down to L5-L6 of S1 disc. Of 71 cases, 2 patients underwent transforaminal endoscopic surgery twice due to recurrence after initial operation. MacNab's criteria was used to assess the outcome. Favorable outcome, excellent of good, was seen in 78% [57 procedures] of the patients. Among 11 fair outcomes, only 1 procedure was followed by secondary open procedure, laminectomy with discectomy. Two of 5 poor outcomes were operated again by same procedure which resulted in fair outcomes. One patient with aggravated cauda equina syndrome remained poor and a lumbar fusion procedure was performed in other patient with poor outcome. There were 2 postoperative discitis that were treated with conservative care in one and anterior lumbar interbody fusion in the other. Conclusion : Evolving technology of mechanical, visual instrument enables minimal invasive procedure possible and effective. The transforaminal endoscopic spinal surgery can reach as high as T12-L1 disc level. The rate of favorable outcome is mid-range among reported endoscopic lumbar surgery series. Authors believe that the outcome will be better as cases accumulate and will be able to reach the fate of standard open microsurgery.

Segmental Dilatation of the Sigmoid Colon : A Rare Cause of Chronic Constipation (S상 결장 분절확장증)

  • Park, Woo-Hyun;Choi, Soon-Ok;Paik, Tae-Won;Lee, Hee-Jung;Suh, Soo-Jhi;Kim, Sang-Pyo
    • Advances in pediatric surgery
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    • v.1 no.1
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    • pp.68-72
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    • 1995
  • Segmental dilatation of the colon is a very rare disease entity of unknown etiology and may mimic Hirschsprung's disease. It is characterized by dilatation of a segment of the colon of variable length with obstruction due to lack of peristalsis in a normally innervated intestine. Recently authors experienced a case of segmental dilatation of the sigmoid colon in a 6 month-old male, who presented with severe constipation, abdominal distention, and abdominal mass since 2 months of age. Down's syndrome and congenital nystagmus were associated. Barium enema demonstrated focal dilatation of the sigmoid colon, but the rectum and descending colon proximal t o the affected colon were of normal caliber. Rectal suction biopsy with acetylcholinesterase staining was normal and anorectal manometry showed normal rectosphincteric reflex. At operation, there was a massively dilated and hypertrophied sigmoid colon with increased tortuous serosal vessels, measuring 15 cm in length and 10 cm in width. Teniae coli were identifiable in the affected segment. Frozen section biopsies at the proximal, affected, and distal colon showed ganglion cells. Descending loop colostomy was constructed initially and segmental resection and end to end colocolostomy were carried out 3 months later. Final histologic examination showed 1) normal colonic mucosa with ganglion cells, 2) prominent submucosal fibrosis and marked muscular hypertrophy, 3) unremarkable acetylcholinesterase activity and immunohistochemical findings against S-100 protein. On 8 months follow-up, he has been doing well and moves bowels 1-2 times daily.

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