Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.
Purpose: Membranoproliferative glomeulonephritis(MPGN) has been diagnosed in an increasing number of asymptomatic cases. These cases have been detected by school urinary screening test even though the total cases of MPGN show a decreasing trend. We have analyzed the clinical and pathological characteristics of children with MPGN according to the clinical manifestations at the time of disease presentation. Methods: A total of 18 patients who had been diagnosed with idiopathic MPGN by percutaneous renal biopsy from January 1990 to February 2004 were involved in our study. The patients were divided into 2 groups as the school urinary screening(A) group and the symptomatic(S) group according to the clinical manifestations at the time of disease presentation. Results: Out of the total 18 patients, 8(44.4%) were in the S group and 10(55.6%) were in the A group. The mean serum total protein, albumin and $C_3$ levels in the S group were significantly lower than those levels of the A group, respectively($4.9{\pm}1.2\;g/dL,\;vs\;7.0{\pm}0.5\;g/dL\;P=0.002,\;2.8{\pm}0.9\;g/dL\;vs.\;4.1{\pm}0.3\;g/dL\;P=0.002,\;63.9{\pm}36.4\;mg/dL\;vs.\;100.8{\pm}39.5\;g/dL\;P=0.041$). The mean total protein amount of 24 hour collected urine in the S group were significantly higher than that of the A group($3684.0{\pm}2601.3\;mg/m^2\;vs.\;559.4{\pm}4.6.9\;mg/m^2$, respectively, P=0.001). Hypocomplementemia was observed in 11(61.1%) out of 18 patients at the time of disease onset, 7(87.5%) in the S group and 4(40%) in the A group. However the hypocomplementemia was decreased in 6(33.3%) out of 18 patients at the time of final follow-up, 3(37.5%) in the S group and 3(30%) in the A group. According to the pathologic type, hypocomplementemia was observed 8 patients(61.5%) with type I disease, 1 patients (100%) with type II disease, 2 patients(50%) in type III disease at the disease onset, but 4 patients(30.8%) in type I disease, 1 patient(100%) in type II disease, 1 patient(33.3%) with type III disease at the time of last follow-up. The incidence of cellular crescent formation and tubular atropy. as observed on light microscopy, were higher in the S group compared to the A group. Mean grade of capillary wall thickening and, mesangial proliferation were significantly higher in the S group. Conclusion: MPGN, as diagnosed in patients with only asymptomatic urinary abnormalities, has been increasing, it is more frequent in asymptomatic patients than in patients with presenting symptoms. Our result suggests that MPGN should be considered in the renal biopsy diagnosis regardless of serum $C_3$ level when urinary abnormalities are found by school urinary screening test.
Purpose: Thyroglobulin (Tg) is a valuable and sensitive tool as a marker for diagnosis and follow-up for several thyroid disorders, especially, in the follow-up of patients with differentiated thyroid cancer (DTC). Often, clinical decisions rely entirely on the serum Tg concentration. But the Tg assay is one of the most challenging laboratory measurements to perform accurately owing to antithyroglobulin antibody (Anti-Tg). In this study, we have compared the degree of Anti-Tg effects on the measurement of Tg between availale Tg measuring kits. Materials and Methods: Measurement of Tg levels for standard Tg solution was performed with two different kits commercially available (A/B kits) using immunoradiometric assay technique either with absence or presence of three different concentrations of Anti-Tg. Measurement of Tg for patient's serum was also performed with the same kits. Patient's serum samples were prepared with mixtures of a serum containing high Tg levels and a serum containg high Anti-Tg concentrations. Results: In the measurements of standard Tg solution, presence of Anti-Tg resulted in falsely lower Tg level by both A and B kits. Degree of Tg underestimation by h kit was more prominent than B kit. The degree of underestimation by B kit was trivial therefore clinically insignificant, but statistically significant. Addition of Anti-Tg to patient serum resulted in falsely lower Tg levels with only A kit. Conclusion: Tg level could be underestimated in the presence of anti-Tg. Anti-Tg effect on Tg measurement was variable according to assay kit used. Therefore, accuracy test must be performed for individual Tg-assay kit.
Kim, Young-Hyo;Park, Ki-Chan;Bae, Seong;Lee, Sang-Hun;Chun, Myung-Ho;Lee, Sang-Ki;Jun, Kwang-Su;Lee, Chan-Se
Tuberculosis and Respiratory Diseases
/
v.39
no.5
/
pp.417-424
/
1992
Background: There were many reports about the clinical aspect and outcomes of pulmonary tuberculosis in health center but few in a medium sized general hospital. The purposes of this study were to find any characteristic differencies in the patients and the general outcomes of the treatments and also to give some suggestive points for the insurance policy making. Methods: We made a retrograde analysis of the medical records of 1981 patients (male 992 female 1,059) who attended our clinics of the 4th internal department, Daedong general hospital during two years from January 1989 to December 1990. Result: 1) Of 1981 patients, 96 were diagnosed as pulmonary tuberculosis taking relatively large proportion in the prevalence. The ratio of prevalence between male and female was 7.81% to 2.27%. The 61.46% were the first diagnosis & initial treatment cases and the remaining 38.54% were the retreatment cases with no statistical significance between sex. 2) The most prevalent age group was between 21~40 years old and the prevalence rate was 45.45% of male and 76.76% of female. The lowest age group in male patient was above 61 showed 3.03%, and there was no female patients above age 60 years old. This phenomena could be thought as the negligence for the treatment of pulmonary tuberculosis in the old age groups rather than true tuberculosis prevalence and it could be proved by the higher rates towards old age groups in the national tuberculosis prevalence survey. 3) There were 57.07% of the minimal case, 48.96% of the moderate, 18.75% of the far advanced. The sputum examination showed 37.07% were culture positive, 46.88% were the negative, and 15.63% of the patients had no stutum examination. Moreover, uncoperatives among the far advanced cases were notable showing 22.22% of the stutum examination, where 16.13% in the minimal cases. The stutum positive rate among the initial treatment cases were 41.07% and 55.00% for the retreatment cases. The sputum no examination rates were 17.86% and 12.50% respectively. 4) The classfication of the mode of disease onset showed 68.75% with gradual onset, 9.38% hemoptic, 3.13% acute pneumonic and 18.75% was found through the radiologic examination in various occasions. 5) The percentages of patients who continued their treatment for more than 8 month were 35.71% (for initial treatment), 25.00% (for retrement), 16.13% (for the minimal), 27.78% (for the far advanced). 6) The group of patients who were treated more than 8 months showed the negative conversion rate of 80% on sputum and marked improvement on chest x-ray in 56.67%. However, in far advanced or retreatment cases, the rate of negative conversion on sputum and the rate of improvement on chest x-ray were low being 60% and 20% for the former and 60% and 10% for the latter, each respectively. Conclusion: It would be strongly emphasized that the improvement of National medical insurance system and social welfare system in Korea must be definite to improve overall treatment and control of tuberculosis diseases as well as physician's devotious National tuberculosis control policy.
Purpose: To make objective standards of small intestinal mucosal changes in cow's milk-sensitive enteropathy (CMSE) we analyzed histological changes of endoscopic duodenal mucosa biopsy specimens from normal children and patients of CMSE. Methods: We review the medical records of patients who had been admitted and diagnosed as CMSE by means of gastrofiberscopic duodenal mucosal biopsy following cow's milk challenge and withdrawal. Thirteen babies with CMSE, ranging from 14 days to 56 days of age, were studied. Five non-CMSE patients were used as control, ranging from 22 days to 72 days of age. The morphometric parameters under study were villous height, crypt zone depth, ratio of villous height to crypt zone depth, total mucosal thickness and length of surface epithelium by using H & E stained specimens under the drawing apparatus attached microscope. In addition, the numbers of lymphocytes in the epithelium and eosinophil cells in the lamina propria and epithelium were measured. Results: In the duodenal mucosal biopsy specimens in CMSE we found partial and subtotal villous atrophy with an increased number of interepithelial lymphocytes. The mean villous height($135{\pm}59\;{\mu}m$), ratio of villous height to crypt zone depth ($0.46{\pm}0.28$), total mucosal thickness ($499{\pm}56\;{\mu}m$), length of surface epithelium of small intestinal mucosa ($889{\pm}231\;{\mu}m$) in CMSE was significantly decreased compared with the control (p<0.05). The mean crypt zone depth ($311{\pm}65\;{\mu}m$) was significantly greater than the control ($188{\pm}24\;{\mu}m$)(p<0.05). Infiltration of interepithelial lymphocytes ($34.1{\pm}10.5$) were significantly greater than the control ($13.6{\pm}3.6$)(p<0.05). The number of eosinophil cells in both lamina propria and epithelium was no significant differences between groups (p>0.05). The small intestinal mucosa in treated CMSE showed much improved enteropathy of villous height, crypt zone depth, interepithelial lymphocytes compared with the control as well as untreated CMSE. Conclusion: Quantitation of mucosal dimensions confirmed the presence of CMSE. It seems to be a limitation in the capacity of crypt cells to compensate for the loss of villous epithelium in CMSE. Specimens obtained by gastrofiberscopic duodenal mucosal biopsy were suitable for morphometric diagnosis of CMSE. Improvement of CMSE also can be confirmed histologically after the therapy of protein hydrolysate.
Lee Soon Min;Kim Ji Hong;Lee Jae Seung;Han Suk Joo
Childhood Kidney Diseases
/
v.9
no.2
/
pp.222-230
/
2005
Purpose : ARC syndrome refers to an association of arthrogryposis, renal tubular dysfunction, and cholestasis. The VPS33B gene was recently identified as the causative gene. So far, 41 cases of ARC syndrome have been reported worldwide, and it has rarely been reported in Korea. This study was conducted to report the clinical findings of seven ARC syndrome cases in Korean children, focusing especially on renal tubular dysfunction. Methods : The hospital records of 7 cases diagnosed as ARC syndrome at Severance Hospital between Mar. 1995 and Aug. 2005 were reviewed and analyzed. Results : Of the 7 cases, 4 were boys and 3 were girls. Six patients(85$\%$) were born with normal birth weight at term, and one was born at preterm. All cases presented with cholestasis and severe jaundice. According to the type of arthrogryposis described by Brown et al, type 3 and 4 were found in 2 patients and type,6, 7, and the undistributed type in one patient respectively. Other associated clinical findings were as follows : failure to thrive in 6(85$\%$), lax skin in 5(71$\%$), and gray platelet syndrome in 4(57$\%$). Urine analysis revealed 6 cases(85$\%$) with proteinuria, 3(43$\%$) with hematuria, 5(71$\%$) with glycosuria, 2(29$\%$) with phosphaturia and 2(29$\%$) with calciuria. Serum electrolytes showed 4 cases(57$\%$) with hyponatremia, 3(43$\%$) with hypokalemia, and 1(14$\%$) with creatinine elevation. Renal tubular dysfunctions were diagnosed as renal tubular acidosis in 6 cases(85$\%$), nephrogenic diabetes insipidus in 2(29$\%$), and Fanconi syndrome in 2(29$\%$). During the follow-up period, 2(29$\%$) had no treatment, 5(85$\%$) had continuous supplementation to correct the electrolyte imbalance and acidosis, and 1(14$\%$) had dialysis. Only one patient had a family history of ARE syndrome in a sibling. Four cases(57$\%$) were diagnosed at the mean age of 8.2 months, and one case was lost during follow-up. Ages of the survived 2 cases were 13 and 25 months, respectively. Conclusion : The rare disease of ARC syndrome Is associated with severe renal dysfunction. However, this study revealed that the renal manifestation of ARC syndrome in Korean children is relatively mild and survival rate is higher than that of previous studies. Contrary to previous reports, this study showed that familial cases are rare and sporadic occurence is possible in Korea. Thus, the diagnosis of this syndrome requires a careful evaluation of the venal function in cases of congenital arthrogryposis, and a mandatory genetic counseling of affected family for prevention of familial occurance. (J Korean Soc Pediatr Nephrol 2005;9:222-230)
Kim, So Young;Lim, Seong Joon;Yun, Sin Weon;Lee, Dong Keun;Choi, Eung Sang
Clinical and Experimental Pediatrics
/
v.45
no.6
/
pp.773-782
/
2002
Purpose : To identify the necessity of more reasonable diagnostic criteria and the possibility of early prediction of coronary involvement in the higher risk group, we investigated and compared clinical and laboratory findings in the acute phase and coronary involvements in those younger (n=17) and older(n=53) than one year of age in Kawasaki disease(KD). Methods : Retrospective chart reviews were performed on 70 patients with KD who were admitted to the Chung-Ang University Hospital from April 1997 to May 2001. Results : Male were significantly higher in the younger age group(M : F ratio 3.3 : 1 vs. 1.0 : 1, P=0.004). Fever durations before intravenous immunoglobulin(IVIG) and echocardiography were significantly shorter in the younger group($4.6{\pm}1.3$ vs. $6.2{\pm}2.5$, P=0.004 vs. 0.01, respectively). Cases meeting typical diagnostic criteria were significantly less in the younger group(P=0.006). In the laboratory findings, serum albumin, BUN and $K^+$ levels in the acute febrile phase were significantly higher in the younger group(P=0.002, 0.006, <0.001, respectively) and incidences of coronary artery dilatation in the acute phase were significantly higher in the younger group(P=0.01). Conclusion : Although less met the typical diagnostic criteria of KD, infants younger than one year of age are more susceptible to coronary artery change in the acute febrile phase. Therefore, KD should be entertained as a diagnostic possibility in young infants with prolonged fever without distinct fever focus, and echocardiography should be considered as part of the evaluation of these patients, and then early diagnosis and prompt IVIG should be conducted.
Purpose: In order to develop the national guide-lines for the standardization of radiotherapy we are planning to establish a web-based, on-line data-base system for laryngeal cancer. As a first step this study was performed to accumulate the basic clinical information of laryngeal cancer and to determine the items needed for the data-base system. Materials and Methods: We analyzed the clinical data on patients who were treated under the diagnosis of laryngeal cancer from January 1998 through December 1999 In the South-west area of Korea. Eligiblity criteria of the patients are as follows: 18 years or older, currently diagnosed with primary epithelial carcinoma of larynx, and no history of previous treatments for another cancers and the other laryngeal diseases. The items were developed and filled out by radiation oncologlst who are members of forean Southwest Radiation Oncology Group. SPSS vl0.0 software was used for statistical analysis. Results: Data of forty-five patients were collected. Age distribution of patients ranged from 28 to 88 years(median, 61). Laryngeal cancer occurred predominantly In males (10 : 1 sex ratio). Twenty-eight patients (62$\%$) had primary cancers in the glottis and 17 (38$\%$) in the supraglottis. Most of them were diagnosed pathologically as squamous cell carcinoma (44/45, 98$\%$). Twenty-four of 28 glottic cancer patients (86$\%$) had AJCC (American Joint Committee on Cancer) stage I/II, but 50$\%$ (8/16) had In supraglottic cancer patients (p=0.02). Most patients(89$\%$) had the symptom of hoarseness. indirect laryngoscopy was done in all patients and direct laryngoscopy was peformed in 43 (98$\%$) patients. Twenty-one of 28 (75$\%$) glottic cancer cases and 6 of 17 (35$\%$) supraglottic cancer cases were treated with radiation alone, respectively. The combined treatment of surgery and radiation was used in 5 (18$\%$) glottic and 8 (47$\%$) supraglottic patients. Chemotherapy and radiation was used in 2 (7$\%$) glottic and 3 (18$\%$) supraglottic patients. There was no statistically significant difference in the use of combined modality treatments between glottic and supraglottic cancers (p=0.20). In all patients, 6 MV X-ray was used with conventional fractionation. The iraction size was 2 Gy In 80$\%$ of glottic cancer patients compared with 1.8 Gy in 59$\%$ of the patients with supraglottic cancers. The mean total dose delivered to primary lesions were 65.98 ey and 70.15 Gy in glottic and supraglottic patients treated, respectively, with radiation alone. Based on the collected data, 12 modules with 90 items were developed or the study of the patterns of care In laryngeal cancer. Conclusion: The study Items for laryngeal cancer were developed. In the near future, a web system will be established based on the Items Investigated, and then a nation-wide analysis on laryngeal cancer will be processed for the standardization and optimization of radlotherapy.
Purpose: This study was designed to evaluate the effects on bone mineral density (BMD) and related factors according to the distance from the radiation field at different sites. This study was conducted on patients with uterine cervical cancer who received pelvic radiotherapy. Materials and Methods: We selected 96 patients with cervical cancer who underwent determination of BMD from November 2002 to December 2006 after pelvic radiotherapy at Kosin University Gospel Hospital. The T-score and Z-score for the first lumbar spine (L1), fourth lumbar spine (L4) and femur neck (F) were analyzed to determine the difference in BMD among the sites by the use of ANOVA and the post-hoc test. The study subjects were evaluated for age, body weight, body mass index (BMI), post-radiotherapy follow-up duration, intracavitary radiotherapy (ICR) and hormonal replacement therapy (HRT). Association between the characteristics of the study subjects and T-score for each site was evaluated by the use of Pearson's correlation and multiple regression analysis. Results: The average T-score for all ages was -1.94 for the L1, -0.42 for the L4 and -0.53 for the F. The average Z-score for all ages was -1.11 for the L1, -0.40 for the L4 and -0.48 for the F. The T-score and Z-score for the L4 and F were significantly different from the scores for the L1 (p<0.05). There was no significant difference between the L4 and F. Results for patients younger than 60 years were the same as for all ages. Age and ICR were negatively correlated and body weight and HRT were positively correlated with the T-score for all sites (p<0.05). BMI was positively correlated with the T-score for the L4 and F (p<0.05). Based on the use of multiple regression analysis, age was negatively associated with the T-score for the L1 and F and was positively correlated for the L4 (p<0.05). Body weight was positively associated with the T-score for all sites (p<0.05). ICR was negatively associated with the T-score for the L1 (p<0.05). HRT was positively associated with the T-score for the L4 and F (p<0.05). Conclusion: The T-score and Z-score for the L4 and F were significantly higher than the scores for the L1, a finding in contrast to some previous studies on normal women. It was thought that radiation could partly influence BMD because of a higher T-score and Z-score for sites around the radiotherapy field. We suggest that a further long-term study is necessary to determine the clinical significance of these findings, which will influence the diagnosis of osteoporosis based on BMD in patients with cervical cancer who have received radiotherapy.
Purpose: Gastrointestinal stromal tumorsm (GISTs) are the most common mesenchymal tumors that arise anywhere in the tubular GI tract. The prognosis for GSTIs is important because f GISTs may metastasiwx in the liver or the abdominal cavity in an early stage. For the reason we examined the tumor size, the mitotic number, ki 67, p53, and c-kit mutation as independent prognostic factor for GISTs. Materials and Methods: A retrospective study was conducted in 76 patients who had been re-evaluated for confirmation of diagnosis between Jan 1998 and Dec. 2001. at Catholic University of medicine. Results: There were significant difference between the turner size, mitotic indices, ki 67, c-kit mutations and the 5-years survival rates. Tumor size (${\geq}5\;cm$) and mitotic index (${\geq}5/50\;HPF$) were statistically related to a significantly poor prognosis (P=0.017 and P=0.042, respectively). c-kit mutations in exon 11 were found in 7 cases c-kit mutation was observed more frequently in high risk patients, and there was a significant difference between c-kit mutation and survival (P=0.037). Elevated ki 67 was noted in 34 out of the 76 cases. High risk patients showed elevated ki67 index more frequently and there was significant relation with the survival rate (P=0.0417). Conclusion: We think that tumor size, mitotic index, Ki 67 and c-kit mutation are as independent prognostic factors for GISTs, but more research is needed.
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