• Title/Summary/Keyword: Syndrome

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Two Cases of Hemolytic Uremic Syndrome Associated with Pneumococcal Infection (폐렴구균 감염에 동반된 비전형적 용혈성 요독 증후군 2례)

  • Jo Seung-Heui;Park Kyung-Mi;Ha Il-Soo;Cheong Hae-Il;Choi Yong
    • Childhood Kidney Diseases
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    • v.3 no.2
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    • pp.227-231
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    • 1999
  • Hemolytic uremic syndrome is a clinical syndrome with various etiology and pathogenesis. And pneumococcal neuraminidase has been known to play a pathogenetic role in some cases with this syndrome. We experienced two children with hemolytic uremic syndrome complicated by pneumococcal infection. One was 21-month-old girl with pneumococcal pneumonia, and the other was 7-month-old girl with pneumococcal meningitis and sepsis. Both of them showed typical clinical manifestations of hemolytic uremic syndrome with prolonged anuria during the course of pneumococcal infection. The renal functions of both cases did not recovered after resolution of acute hemolytic episode and chronic renal failure developed.

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Epidemiology of the metabolic syndrome among Korean children and adolescents (소아청소년에서의 대사증후군 역학)

  • Park, Mi Jung
    • Clinical and Experimental Pediatrics
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    • v.51 no.6
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    • pp.564-568
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    • 2008
  • The prevalence of obesity in Korean children is estimated to be around 10%, and has increased significantly over the past 20 years. Metabolic syndrome, which includes central obesity, glucose intolerance, insulin resistance, dyslipidemia, and hypertension, is a well documented risk factor for cardiovascular diseases, but there is no general consensus concerning its definition for children. In this study, ablut 30-40% of overweight or obese children had metabolic syndrome compared to less than 10% in the general population. Dyslipidemia was the most common component and hyperglycemia was the least common component of metabolic syndrome. If the prevalence of childhood obesity continues to increase, it is highly likely that the prevalence of the metabolic syndrome will also increase. These findings emphasize the importance of preventing obesity in addition to the diagnosis and management of metabolic syndrome in Korean adolescents.

Radiological features of familial Gorlin-Goltz syndrome

  • Hegde, Shruthi;Shetty, Shishir Ram
    • Imaging Science in Dentistry
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    • v.42 no.1
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    • pp.55-60
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    • 2012
  • Gorlin-Goltz syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocystic odontogenic tumors, and skeletal anomalies. This syndrome may be diagnosed early by dentist because keratocystic odontogenic tumors are usually one of the first manifestations of the syndrome. Early diagnosis and treatment are of utmost importance in reducing the severity of long term sequelae of this syndrome. This report presents a rare event of Gorlin-Goltz syndrome occurring in a 39-year-old male and his 8-year-old daughter. The clinical and investigative features of this familial disorder has been described in detail.

Management of obstructive sleep apnea in a Treacher Collins syndrome patient using distraction osteogenesis of the mandible

  • Damlar, Ibrahim;Altan, Ahmet;Turgay, Berk;Kilic, Soydan
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.42 no.6
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    • pp.388-392
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    • 2016
  • In this study, we present the surgical treatment of obstructive sleep apnea in a child with Treacher Collins syndrome. A 10-year-old girl with a past history of Treacher Collins syndrome presented to our clinic with her parents for respiratory distress and insomnia. The patient was referred to a sleep laboratory where she was diagnosed with obstructive sleep apnea, which was a consequence of her Treacher Collins syndrome. The patient underwent mandibular distraction osteogenesis under general anesthesia. The mandible was expanded by 15 mm using internal bilateral distractors. After distraction osteogenesis, the patient's respiratory problems resolved, and she was able to sleep comfortably. Distraction osteogenesis was an effective method of advancing the mandible, increasing the upper airway space and ultimately preventing obstructive sleep apnea syndrome in patients with Treacher Collins syndrome.

Clinical Observation on 3 Cases of Dry Eye Syndrome (안구 건조증 환자의 치험 3례)

  • Choi, Eun-Hee;Jeon, Ju-Hyun;Kim, Young-Il
    • Journal of Acupuncture Research
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    • v.24 no.4
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    • pp.237-245
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    • 2007
  • Objective : The Dry Eye Syndrome is characterized by reduction of the stability of the tearfilm and a set of alterations of the eye surface which could relate to tear quality, normal makeup of tearfilm and alterations in blinking or regular closing of eyelids. We treated 3 patients who was suffering with severe Dry Eye Syndrome and assessed the effectiveness of Oriental Medicine in the treatment of Dry Eye Syndrome. Methods : The patients were treated by acupunture, Gi Guk Yang Hyeol-decoction(Qijuyangxie-decoction). The improvement of the symptom was judged the subjective symptoms by the change of Ocular Surface Disease Index. Result : We observed in 2 cases of the patient significant decrease in the frequency of subjective symptoms, 1 case was not treated. Conclusion: The Oriental Medicine had effective results in Dry Eye syndrome. We need to have more observation and examination of Dry Eye syndrome.

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Acute Respiratory Distress Syndrome as the Initial Clinical Manifestation of an Antisynthetase Syndrome

  • Kim, Seo-Hyun;Park, I-Nae
    • Tuberculosis and Respiratory Diseases
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    • v.79 no.3
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    • pp.188-192
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    • 2016
  • Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids.

Molecular genetics of congenital central hypoventilation syndrome and Haddad syndrome

  • Lee, Jae-Ho;Kim, Dae-Kwang
    • Journal of Genetic Medicine
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    • v.11 no.1
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    • pp.11-15
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    • 2014
  • Congenital central hypoventilation syndrome (CCHS) is a disorder of the autonomic nervous system characterized by a decreased response to hypercarbia. CCHS is frequently associated with congenital megacolon; the combination is called Haddad syndrome. CCHS is associated with dysfunction in respiratory features of the autonomic nervous system and with other disorders, including facial deformities, cardiovascular symptoms, and tumors. Patients with CCHS frequently have a mutation in the homeobox protein 2b (PHOX2B) gene. Most mutations involve heterozygous expansion of alanine repeats (GCN). Interestingly, a higher polyalanine repeat number is associated with a more severe clinical phenotype. To clarify the role of PHOX2B in disease pathogenesis, we introduce and review the clinical and molecular features of CCHS and Haddad syndrome.

A Study for Effects of Metabolic Syndrome Care Program with Korean Medicine in Public Health Center (한의약건강증진 사업 중 한방대사증후군 프로그램의 효과에 관한 연구)

  • Park, Eun-Seong
    • The Journal of Korean Medicine
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    • v.35 no.1
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    • pp.135-144
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    • 2014
  • Objectives: The purpose of this study is to evaluate the effects of Metabolic Syndrome care program with Korean Medicine in public health center. Methods: The data were collected from 28 participations who joined Korean Medicine care program. The program was started from April of 2013 and finished November of 2013. The effect of Metabolic Syndrome were evaluated with blood pressure check and blood test, physical test. Results: The number of Metabolic Syndrome patients decreased after the program(-24.9%). Specially TG decreased from 83.321 to 80.036(p<0.05) and FBS also decreased 8.607(p<0.01) after the program. HDL increased from 47.821 to 53.000(p<0.01) but blood pressure didn't change. BMI decreased 0.789(p<0.01) and abdominal obesity decreased 2.607(p<0.01) after the program significantly. Conclusions: From these study, the Metabolic Syndrome care program with Korean Medicine is worthy for treating Metabolic Syndrome.

Acromegaloid Facial Appearance Syndrome - A New Case in India

  • Rai, Arpita;Sattur, Atul P.;Naikmasur, Venkatesh G.
    • Journal of Genetic Medicine
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    • v.10 no.1
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    • pp.57-61
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    • 2013
  • Acromegaloid Facial Appearance syndrome is a very rare syndrome combining acromegaloid-like facial appearance, thickened lips and oral mucosa and acral enlargement. Progressive facial dysmorphism is characterized by a coarse facies, a long bulbous nose, high-arched eyebrows, and thickening of the lips, oral mucosa leading to exaggerated rugae and frenula, furrowed tongue and narrow palpebral fissures. We report a case of acromegaloid facial appearance syndrome in a 19-year-old male patient who presented with all the characteristic features of the syndrome along with previously unreported anomalies like dystrophic nails, postaxial polydactyly and incisal notching of teeth.

Neurophysiological and Clinical Features of the Pusher Syndrome: Review Article

  • Kim, Chung-Sun;Nam, Seok-Hyun
    • The Journal of Korean Physical Therapy
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    • v.22 no.3
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    • pp.45-48
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    • 2010
  • Patients with the pusher syndrome show severe misperception of their own upright body orientation although visual vestibular processing is almost intact. They recognize their body as oriented upright when it is actually tilted nearly 20 degrees to the affected side. These patients resist any attempts to passively correct their tilted body posture towards an earth vertical upright orientation. They use the non-affected side arm and/or leg to actively push towards the affected side. Pusher syndrome patients have different prognoses and symptoms than general stroke patients without pusher syndrome. Pusher syndrome patients have a poor prognosis, so they need a long duration of treatment. Therefore, accurate diagnosis and proper treatment are important. In this study, we reviewed the symptoms, causes, evaluation, and treatment for pusher syndrome.