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Epidemiology and Clinical Manifestations of $Henoch-Sch\"{o}nlein$ Purpura in Children (소아 $Henoch-Sch\"{o}nlein$ 자반증의 역학 및 임상양상)

  • Kim Se-Hun;Lee Chong-Guk
    • Childhood Kidney Diseases
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    • 제7권2호
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    • pp.166-173
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    • 2003
  • Purpose : The cause and pathogenesis of $Henoch-Sch\"{o}nlein$ purpura has been studied for many years but the results are disappointing. Recently the hypothesis that abnormalities involving the glycosylation of the hinge region of immunoglobulin Al(IgAl) may have an important role in the pathogenesis of $Henoch-Sch\"{o}nlein$ purpura is being approved. $Henoch-Sch\"{o}nlein$ purpura is the most common vasculitis Ihat affects children and the prognosis is good. But if kidney invovement occurs, the course may be chronic and troublesome. So we evaluated children with $Henoch-Sch\"{o}nlein$ purpura especially from the point of epidemiology and clinical manifestations. Methods : Investigation of 124 children who were diagnosed with $Henoch-Sch\"{o}nlein$ purpura at Inje University Ilsan Paik Hospital from December 1999 to July 2003 was performed retrospectively through chart review. Efforts were made to get informations about the profile, epidemiology, clinical manifestations, progress of the disease and recurrence rate of patients. Results : The patients were 69 boys and 55 girls, with a mean age of $6.1{\pm}2.7$ years at the time of data collection. The male to female ratio was 1.25 : 1. The occurrence rate was much higher in autumn(from September to November, 31.5%) and winter(from December to February, 28.2%) than in spring and summer, with a peak in November. Joint involvement was shown in 66.9% of patients mostly on the foot/ankle(75.9%), knee(39.8%). Seventy(56.5%) out of 124 patients had abdominal pain and 10 patients(8.1%) showed bloody stools. Renal involvement was observed in 24 patients(19.4%) after 21.1 days on the average. IgA was elevated in 10 of 21 patients(47.6%). $C_3$ and $C_4$ levels were normal in 40 of 49 patients (81.7%) and 47 of 48 patients(97.9%), respectively Antistreptolysin-O(ASO) titer was elevated over 250 Todd units in 29 of 62 Patients(46.8%). Mycoplasma antibody titer was elevated in 21 of 49 patients(42.9%) equal or greater than 1:80. Radiologic studies were peformed in 23 patients. Seven patients(30.4%) showed bowel wall thickening and one of them received intestinal resection and anastomosis operation due to terminal ileum necrosis. Eighty four patients took steroid 1.4 mg/kg/day in average. Recurrence rate was 2.5 in 37 patients(29.8%). Conclusion : $Henoch-Sch\"{o}nlein$ purpura in childhood appears most in about 6 years of age. The occurrence rate is much higher in autumn and winter relatively. Diagnosis can be made through the perspective history taking and the inspection of clinical manifestations, but the laboratory findings are not of great help. A small portion of the patients might show abdominal pain or arthritis before purpura develops, therfore various diagnosis can be made. Radiologic evaluation should be performed to avoid surgical complications in cases accompanying abdominal pain, and long term follow up should be needed especially in patients suffering from kidney involvement. In about 30% of the patients $Henoch-Sch\"{o}nlein$ purpura would recur. Steroid can be used safely without side effects.

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Clinical Characteristics of precocious puberty girls and Comparison Analysis of GnRH Test results with Diagnosis type (성조숙증 여아들의 임상적 특징 및 진단별 성선자극호르몬 분비호르몬 GnRH (Gonado Tropin Releasing Hormone) 검사결과의 비교분석평가)

  • Kim, Jung-In;Kwon, Won-Hyun;Moon, Ki-Choon;Lee, In-Won
    • The Korean Journal of Nuclear Medicine Technology
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    • 제20권2호
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    • pp.54-61
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    • 2016
  • Purpose Precocious Puberty is defined as the development of secondary sexual characteristics in girls younger than 8 years, and boys 9 years. Cause premature closure of the epiphysis is a disease that eventually decreases the final adult height. In this study, we retrospectively analyzed to evaluate the diagnostic difference the GnRH (Gonado-tropin-releasing Hormone) stimulation test results with medical records of precocious puberty in girls. Materials and Methods From February 2015 to December 2015 it was enrolled in the girls 118 people who visited the Seoul National University Bundang Hospital, Pediatrics, Endocrinology Internal Medicine. True precocious puberty group (n=57), early puberty group (n=39), were divided into Premature thelarche (n=22) group. A Tanner stage, chronological age, bone age, height, body weight for each group was determined by examining the mean${\pm}$standard deviation. GnRH test result was compared LH (Basal, 30 min, 45 min, 60 min), FSH (Basal, 30 min, 60 min) for each group, Each group LH, FSH Peak value distribution, the mean${\pm}$standard deviation was calculated for the peak LH/LH basal ratio, peak LH/Peak FSH ratio. The significance probability (P-value) between the value of each third group was determined. Results The average height of the true precocious puberty group $131{\pm}14.85$, the mean weight was $28.80{\pm}4.93$, the average chronological age $7.1{\pm}0.81$, the mean bone age was $9.9{\pm}0.9$, The average height of early puberty group was $134{\pm}5.10$, the average weight $28.50{\pm}4.43$, the average chronological age $8.05{\pm}0.03$, the mean bone age was $10.0{\pm}0.62$, The average height of Premature thelarche $129{\pm}6,01$, the average weight was $28.65{\pm}5.98$, the average chronological age $7.02{\pm}0.58$, the mean bone age was $8.04{\pm}1.29$. There was no significant difference when compared to the height and weight. There was a significant difference between the groups in the chronologic age and bone age difference (P <0.0002) True precocious puberty group showed peak LH levels at 30'(82.5%), 45'(12.3%), 60'(5.3%), in Peak FSH 30'(8.8%), 60'(91.2%). Early Puberty group showed high values in Peak LH at 30'(79.5%), 45'(17.9%), 60'(2.6%), in peak FSH levels at 30'(7.7%), 60'(92.32%). In Premature thelarche Group it showed the Peak LH levels at 30'(30%), 45'(59%), 60'(9.09%), Peak FSH levels at 30'(0%) 60'(100%). When compared with the The Peak LH/basal LH ratio, True precocious puberty group was $19.09{\pm}17.15$, early puberty group was $15.23{\pm}10.88$, Premature thelarche group showed significant differences between the three groups as $4.93{\pm}4.36$.(P <0.0001) LH Peak/FSH Peak ratio, true precocious puberty group was $1.222{\pm}0.77$, early puberty group was $1.34{\pm}1.23$, Premature thelarche group showed significant differences between the three groups as $0.3{\pm}0.09$(P <0.0001) Conclusion In order to diagnose the true precocious puberty have a diagnostic value when the LH peak after GnRH stimulation is increased by more than two to three times compared to baseline or a predetermined level or more than 5~10 IU/L increases. GnRH Test is a test for a long time and the patient discomfort due to repeated blood sampling, but the hypothalamus-pituitary gland- gonad axis activity evaluate and is the most basic accurate test in the differential diagnosis of precocious puberty disorders.

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