• Title/Summary/Keyword: 신증후군

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Predictive Clinical Factors for the Treatment Response and Relapse Rate in Childhood Idiopathic Nephrotic Syndrome (소아 일차성 신증후군의 치료반응과 재발빈도에 관련된 인자)

  • Jeon, Hak-Su;Ahn, Byung-Hoon;Ha, Tae-Sun
    • Childhood Kidney Diseases
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    • v.10 no.2
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    • pp.132-141
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    • 2006
  • Purpose : This study was aimed to determine the predictive risk factors for the treatment response and relapse rate in children diagnosed with idiopathic nephrotic syndrome. Methods : We analyzed the medical records of children who were diagnosed and treated for childhood idiopathic nephrotic syndrome from November 1991 to May 2005. Variables selected in this study were age at onset, sex, laboratory data, concomitant bacterial infections, days to remission, and interval to first relapse. Results : There were 46 males and 11 females, giving a male:female ratio of 4.2:1. The age($mean{\pm}SD$) of patients was $5.8{\pm}4.1$ years old. Of all patients who were initially given corticosteroids, complete remission(CR) was observed in 54(94.7%). Of the 54 patients who showed CR with initial treatment, 40(70.2%) showed CR within 2 weeks and 14(24.6%) showed CR after 2 weeks. The levels of serum IgG were lower in the latter group who showed CR after 2 weeks(P=0.036). Of the 54 patients who showed CR with initial treatment, 47(82.5%) relapsed. Of these patients, 35.1% were frequent relapsers and 43.9% were infrequent relapsers. There was no significant correlation between the frequency of relapse and the following variables : sex, days to remission, and laboratory data. However, age at onset and interval to first relapse had a negative correlation with the frequency of relapse(Pearson's coefficient=-0.337, -0.433, P<0.012, P<0.01). Conclusion : The age at onset and the interval to first relapse were found to be predictive clinical parameters for the relapse rate, while the levels of serum IgG at initial presentation were a predictive laboratory factor for treatment response in childhood idiopathic nephrotic syndrome.

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A Case of Wiskott-Aldrich Syndrome with Novel Mutation in Exon 2 of the WASP Gene (WASP 유전자의 Exon 2에서 새로운 돌연변이를 가진 Wiskott-Aldrich 증후군의 1례)

  • Lee, Hyuk;Park, Jung-In;Kim, Sun Young;Moon, Kyeung Hee;Yi, Ho Keun;Hwang, Pyeong Han
    • Clinical and Experimental Pediatrics
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    • v.48 no.5
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    • pp.551-556
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    • 2005
  • Wiskott-Aldrich syndrome(WAS) is an X-linked recessive immunodeficiency characterized by thrombocytopenia with small platelet volume, eczema, and recurrent infections, and is also characterized by increased incidence of auto immune diseases and malignancies. The phenotype observed in this syndrome is caused by mutation in the Wiskott-Aldrich syndrome protein(WASP) gene localized to the proximal short arm of the X chromosome and recently isolated through positional cloning. The gene encodes a 502 amino acid protein, which contains 12 exons and spans 9 kb of genomic DNA. The function of the encoded protein is not well understood. The clinical diagnosis of WAS can be difficult and is usually confirmed by the detection of WASP gene mutations and the expression of WSAP in patient blood sample using genetic analysis. We reported a case of a 13-month old boy with WAS who was identified with the novel mutation in exon 2 of WASP gene by direct sequencing and the complete absence of WASP expression by immunoblotting.

Effect of Duloxetine in Functional Gastrointestinal Disorder : In the Perspective of 'Brain-Gut Axis' (기능성 위장관 장애에서 Duloxetine의 효과 : '뇌-장관 축' 모델을 중심으로)

  • Lee, Sang-Shin;Park, Si-Sung
    • Korean Journal of Psychosomatic Medicine
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    • v.20 no.2
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    • pp.135-138
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    • 2012
  • The pathophysiology of functional gastrointestinal disorder(FGID) is not completely understood, but the importance of the 'Brain-Gut Axis(BGA)' model in FGID is being increasingly recognized. The BGA model is a bidirectional, hard-wired and homeostatic relationship between the central nervous system(CNS) and the enteric nervous system(ENS) via neural, neurohormonal and neuroimmunological pathways. In addition, the BGA model would provide a rationale for the use of psychotropics on FGID. The authors experienced two cases in which duloxetine, a serotonin-norepinephrine reuptake inhibitor, was effective in relieving FGID symptoms as well as psychiatric symptoms such as depression and hypochondriacal anxiety. Therefore we discuss the vignettes from the perspective of BGA theory. Duloxetine showed efficacy in these two patients by reducing visceral hypersensivity (bottom-up regulation) and by relieving depression and anxiety(top-down regulation).

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Midazolam and Ketamine Intramuscular Premedication in Attention Deficit Hyperactivity Disorder Patient with Poor Cooperability (치과 진료에 비협조적인 주의력 결핍 과잉행동 장애 증후군 환자에서의 전처치로서의 미다졸람 케타민의 근육투여)

  • Kim, Young-Jae;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Jung-Wook;Jang, Ki-Taeg;Lee, Sang-Hoon;Kim, Chong-Chul;Kim, Hyun-Jeong;Seo, Kwang-Suk;Lee, Jung-Man;Shin, Soonyoung
    • Journal of The Korean Dental Society of Anesthesiology
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    • v.12 no.2
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    • pp.111-114
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    • 2012
  • Attention deficit hyperactivity disorder (ADHD) is characterized by inattention, impulsivity, and hyperactivity. Given high incidence of ADHD, many children with ADHD is likely to present for anesthesia. This case report suggests intramuscular premedication as an alternative method for anesthetic induction. A 9-year-old male patient with ADHD was transferred for dental treatment under general anesthesia. The patient refused to go into dental clinic office. Oral midazolam was given to the patient, however, he was resistant to take midazolam via oral route. Instead, we administer midazolam and ketamine via intramuscular route. After less than 10 miniutes, the patient became drowsy and was transferred to dental chair. Intravenous access and mask inhalation was possible. The patient received dental treatment under general anesthesia and recovered in a non-complicated way. In this case, intramuscular sedation with midazolam and ketamine was used as a premedication in highly uncoopearive patient refused to take oral sedative medication.

Alexithymia in Patients with Tension-Type Headache (긴장성 두통 환자에서의 감정표현 불능증)

  • Shin, Dong-In;Ham, Byung-Joo;Kwon, Ho-In;Park, Gun-Woo;Kim, Leen;Suh, Kwang-Yun
    • Sleep Medicine and Psychophysiology
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    • v.9 no.1
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    • pp.56-60
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    • 2002
  • Objective: Various psychological factors influence the occurrence of tension-type headaches. The aim of this study is to compare the level of alexithymia between tension-type headache patients and normal controls. Methods: Sixty-six subjects with tension-type headaches and 59 controls were studied. The Beck Depression Inventory and Toronto Alexithymia Scale (TAS-20K) were administered to the tension-type headache group and TAS-20K to the normal control group. Results: Compared with normal controls, the tension-type headache group had significantly higher alexithymia scores. There was also significant association between the level of alexithymia and the severity of the depression in tension-type headache patients. Conclusions: These findings suggest that patients with tension-type headaches have difficulty in expressing their emotions. And in patients with tension-type headaches, the more alexithymic they are, the more depressive.

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A Case of Central Diabetes Insipidus in Patient with Non-small Cell Lung Cancer (비소세포폐암에 합병된 중추성 요붕증 1예)

  • Hwang, Eun Mi;Oh, You Kyoung;Kim, Ki Jo;Kim, Yong Hyun;Yoon, Hyoung Kyu;Song, Jeong Sup
    • Tuberculosis and Respiratory Diseases
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    • v.57 no.3
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    • pp.284-288
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    • 2004
  • Central diabetes insipidus (DI) is a disease caused by insufficient release of antidiuretic hormone. Central DI with lung cancer is very rare. Most of them are caused by the pituitary metastasis, and rarely, by the paraneoplastic syndromes. Central DI is diagnosed by the water deprivation test. The treatment consists of surgical resection, radiotherapy and administration of desmopressin. We report an unusual case of central DI with non-small cell lung cancer. The diagnosis was confirmed by water deprivation test. After the administration of desmopressin, the urine osmolarity was increased. The patient's symptoms and urine osmolarity were improved by intranasal desmopressin.

DENTAL TREATMENT OF A PATIENT WITH HADDAD SYNDROME UNDER GENERAL ANESTHESIA: CASE REPORT (Haddad syndrome 환아의 전신마취 하 치아우식 치료: 증례보고)

  • Suh, Heewon;Song, Ji-Soo;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Young-Jae;Kim, Jung-Wook;Lee, Sang-Hoon;Jang, Ki-Taeg
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.13 no.1
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    • pp.47-51
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    • 2017
  • Haddad syndrome, the association of Congenital Central Hypoventilation Syndrome (CCHS) and Hirschsprung's disease (HD), is a disease caused by mutation of PHOX2B gene. This disease, which occurs very rarely worldwide, is characterized by adequate ventilation during wakefulness and hypoventilation during sleep due to decreased ventilatory sensitivity. In this case report, we report a case of dental treatment under general anesthesia in a child with Haddad syndrome. A 3-year-old child with Haddad syndrome visited Seoul National University Dental Hospital for dental treatment. Dental treatment was planned under general anesthesia because of his medical condition. Treatment was successfully done without any postoperative complications. This case suggests that general anesthesia may be advantageous for dental treatment due to the risk of respiratory depression in a child with Haddad syndrome.

INCONTINENTIA PIGMENTI : CASE REPORTS (색소실조증 환아의 치험례)

  • Shin, Hye-Sung;Kim, Seon-Mi;Choi, Nam-Ki;Yang, Kyu-Ho
    • Journal of the korean academy of Pediatric Dentistry
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    • v.36 no.4
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    • pp.563-567
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    • 2009
  • Incontinentia pigmenti(Bloch-Sulzberger syndrome) is a sex-linked hereditary disorder so girls are almost exclusively affected. The frequency rate is approximately 1:40,000 among girls. It is associated with skin, ocular, dental, skeletal and central nervous deformities. We reported 2 cases of medically diagnosed IP patients who were 4 and 5 years old girls. They had thin and sparse hair, and represented congenital missing of multiple primary and permanent teeth, accessory cusps and cone-shaped crowns. Therefore we report the dental manifestations and treatment progress.

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Nocturnal Eating Behavior Associated with Zolpidem (Zolpidem과 연관된 야간 식이 행동 증례)

  • Kim, Ji-Min;Paik, Kyoung-Won;Shin, Hong-Beom;Kim, Soo-In;Yun, Kyu-Wol;Lim, Weon-Jeong
    • Sleep Medicine and Psychophysiology
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    • v.12 no.2
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    • pp.144-147
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    • 2005
  • Objective: The authors would like to find the relationship between zolpidem and nocturnal eating episodes in diverse psychiatric patients. Method: We evaluated case series of 6 patients who showed nocturnal eating episodes after takine zolpidem. Results: We have experienced 6 cases who showed nocturnal eating behavior after taking zolpidem. They included 3 patients with schizoprenia, a patient with major depressive disorder, a patient with PTSD and a patient with bipolra I disorder. With reducing or discontiuation of zolpidem, their nocturanl eating resolved. Conclusion: This finding strongly suggests the relationship between zolpidem and the nocturnal eating episode. Physicians should be aware that zolpidem might induce nocturnal eating behaviors.

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Review of Clinical Research for Herbal Medicine Treatment on Refractory Nephrotic Syndrome in Children (소아의 난치성 신증후군의 한약 치료에 대한 최신 임상연구 동향)

  • Jang, Eun Ha;Min, Sang Yeon;Kim, Jang Hyun
    • The Journal of Pediatrics of Korean Medicine
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    • v.34 no.3
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    • pp.1-17
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    • 2020
  • Objectives The purpose of this study is to analyze clinical studies on effectiveness of herbal medicine in refractory nephrotic syndrome (RNS). Methods We searched the randomized controlled trials (RCTs) with herbal medicine treatment on RNS from the Pubmed, CNKI, OASIS, NDSL, J-stage, and CiNii. The demographic data, duration of illness, intervention, treatment period, outcome, adverse events, and composition of herbal medicine were analyzed for this study. Results 11 RCT studies were selected and analyzed. The children in the control group were given western medicine therapy, and the treatment group was given herbal medicine along with the same western medicine of the control group. The most commonly used herbal medicines were the prescriptions to treat 'Kidney Deficiency with Blood Stasis (腎虛兼瘀血)' which composed of 'Promoting blood circulation (化瘀)', 'Diuresis-inducing (利水)', or 'Heating Yang (溫陽)' medicine based on 'Replenishing Kidney or Spleen (補腎, 補脾)' medicine. In the treatment group, proteinuria and serum lipid was significantly decreased, serum albumin was significantly increased, and total effective rate was significantly higher than the control group. Hypercoagulation and relapse rate was also significantly reduced. Adverse events were significantly lower in the treatment group. Conclusions Herbal medicine treatment on pediatric RNS can be suggested as a new treatment for children who have less response to the conventional therapy. It can also supplement the limitations of the western medicine by reducing adverse events from the steroids and immuno-suppressive agents, and lower the relapse rate as well.