Natural course of childhood chronic immune thrombocytopenia using the revised terminology and definitions of the international working group: a single center experience

BACKGROUND: The immune thrombocytopenia (ITP) criteria were newly standardized by the International Working Group. Thus, we analyzed the natural course of childhood chronic ITP to predict the prognosis based on the revised criteria. METHODS: The medical records of children with chronic ITP from May 2000 to February 2013 in our institute were reviewed. RESULTS: Forty-seven children with chronic ITP who were not undergoing corticosteroid therapy were included. Their initial platelet count was 23+/-25x10(9)/L, and age at diagnosis was 6.3+/-4.1 years. The follow-up period was 5.4+/-3.7 years. Among them, 44.7% (21/47) showed spontaneous remission and maintained a platelet count > or =100x10(9)/L. And 66.0% (31/47) maintained a platelet count > or =50x10(9)/L until the last follow-up date. The time periods required for the platelet count to be maintained > or =50x10(9)/L and > or =100 x10(9)/L were 3.1+/-2.7 and 3.6+/-2.7 years. Age at diagnosis in the > or =50x10(9)/L group (5.7+/-4.4 years) was significantly lower than the age at diagnosis in the or =100x10(9)/L group and or =50x10(9)/L, relatively safe state. Age at diagnosis of ITP and follow-up period were the factors influencing prognosis in this study.

Progress of in vitro factor VIII coagulant activity from 0 to 8 hours after reconstitution

BACKGROUND: Continuous infusion of factor VIII (FVIII) is a more cost-effective method for treating hemophilia A than intermittent bolus injection. However, there is currently no specific data in Korea about the progress of in vitro FVIII coagulant activity (FVIII:C) after reconstitution from its lyophilized form. METHODS: Three commercial FVIII concentrate products (two recombinant FVIII and one plasma-derived) were used. In vitro FVIII:C was measured at 0, 2, 4, 6, and 8 hours following reconstitution in both the indoor light-exposed and light-shielded groups. RESULTS: For the three drugs, in vitro FVIII:C decreased over the 8 hours following reconstitution (P<0.001). The decline of FVIII:C was linear (P<0.001). In vitro FVIII:C for the indoor light-exposed groups was 95.3+/-1.9% and 90.6+/-2.5% after 4 and 8 hours following reconstitution, respectively, compared to baseline activity. In the light-shielded group, FVIII:C was 95.4+/-1.1% and 90.9+/-1.7% of the baseline activity after 4 and 8 hours, respectively. There was no statistical difference between FVIII:C in the indoor light-exposed and light-shielded groups (P=0.849). CONCLUSION: In vitro FVIII:C decreased after reconstitution, but activity was maintained at over 90% of the baseline value during 8 hours. Exposure to indoor light did not accelerate the loss of FVIII:C over the experimental time. This result indicates that CI with FVIII is available in 8-hour intervals, with no indoor light-exposure precautions needed.

Early Prediction of Chronic Childhood Immune Thrombocytopenic Purpura According to the Response of Immunoglobulin Treatment / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is a frequently observed bleeding disorder in children. High dose intravenous immunoglobulin G (IVIG) has been used for the treatment of ITP since 1981, and now several methods of IVIG infusion are used. Since 1983, we have treated ITP patients with short-term and low-dose IVIG according to the individual patient's daily response. This study aimed to evaluate individual patient's response after IVIG for the prediction of chronic ITP.METHODS: We evaluated 259 childhood ITP patients retrospectively who were newly diagnosed at the Department of Pediatrics, Kyungpook National University Hospital from 1983 to 2012. We analyzed the individual response to treatment and current state of disease. We evaluated the time to reach desired platelet counts after treatment of IVIG, relapse rate and diagnosis of chronic ITP. The patients were classified into 2 groups according to the time to reach desired platelet counts (50,000/microL) after daily treatment of IVIG, rapid (1 or 2 doses) and slow responder (more than 3 doses).RESULTS: Among 182 patients followed up over 6 months, 41 patients (22.5%) were eventually diagnosed with chronic ITP. Hundred and two patients (56.7%) belonged to rapid response group, and 17 of them (16.7%) were diagnosed with chronic ITP. Eighty patients (44.4%) belonged to the slow response group, and 24 of them (30%) were diagnosed with chronic ITP, which were higher than the early response group (P=0.033).CONCLUSION: Individual response rate of IVIG treatment could be a useful predictor of chronic ITP, but this finding needs support from further studies.

Various endocrine disorders in children with t(13;14)(q10;q10) Robertsonian translocation

PURPOSE: 45,XY,t(13;14)(q10;q10) karyotype can suggest infertility associated with more or less severe oligospermia in male adults. In addition, 45,XX,t(13;14)(q10;q10) karyotype carries reproductive risks such as miscarriage or infertility in female adults. However, reports on the phenotype of this karyotype in children are very rare. This study was done to observe various phenotypes of this karyotype in children. METHODS: Between January 2007 and December 2012, children diagnosed with 45,XY,t(13;14)(q10;q10) or 45,XX,t(13;14)(q10;q10) karyotype by chromosome analysis were analyzed retrospectively. RESULTS: Eight children (5 boys and 3 girls) were diagnosed with 45,XY,t(13;14)(q10;q10) or 45,XX,t(13;14)(q10;q10) karyotype. They ranged in age from 5 years and 6 months to 12 years and 4 months. The phenotypes of the study patients consisted of 1 hypogonadotrophic hypogonadism, 1 precocious puberty, 3 early puberty, 2 growth hormone deficiency (GHD) (partial) and 1 idiopathic short stature. As shown here t(13;14)(q10;q10) Robertsonian translocation shows a wide range of phenotypes. CONCLUSION: It can be said that t(13;14)(q10;q10) Robertsonian translocation shows various phenotypes from GHD to precocious puberty in children. Further large-scale studies are necessary.

Early Prediction of Chronic Childhood Immune Thrombocytopenic Purpura According to the Response of Immunoglobulin Treatment / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is a frequently observed bleeding disorder in children. High dose intravenous immunoglobulin G (IVIG) has been used for the treatment of ITP since 1981, and now several methods of IVIG infusion are used. Since 1983, we have treated ITP patients with short-term and low-dose IVIG according to the individual patient's daily response. This study aimed to evaluate individual patient's response after IVIG for the prediction of chronic ITP. METHODS: We evaluated 259 childhood ITP patients retrospectively who were newly diagnosed at the Department of Pediatrics, Kyungpook National University Hospital from 1983 to 2012. We analyzed the individual response to treatment and current state of disease. We evaluated the time to reach desired platelet counts after treatment of IVIG, relapse rate and diagnosis of chronic ITP. The patients were classified into 2 groups according to the time to reach desired platelet counts (50,000/microL) after daily treatment of IVIG, rapid (1 or 2 doses) and slow responder (more than 3 doses). RESULTS: Among 182 patients followed up over 6 months, 41 patients (22.5%) were eventually diagnosed with chronic ITP. Hundred and two patients (56.7%) belonged to rapid response group, and 17 of them (16.7%) were diagnosed with chronic ITP. Eighty patients (44.4%) belonged to the slow response group, and 24 of them (30%) were diagnosed with chronic ITP, which were higher than the early response group (P=0.033). CONCLUSION: Individual response rate of IVIG treatment could be a useful predictor of chronic ITP, but this finding needs support from further studies.

Early Prediction of Chronic Childhood Immune Thrombocytopenic Purpura According to the Response of Immunoglobulin Treatment / 임상소아혈액종양

BACKGROUND: Immune thrombocytopenic purpura (ITP) is a frequently observed bleeding disorder in children. High dose intravenous immunoglobulin G (IVIG) has been used for the treatment of ITP since 1981, and now several methods of IVIG infusion are used. Since 1983, we have treated ITP patients with short-term and low-dose IVIG according to the individual patient's daily response. This study aimed to evaluate individual patient's response after IVIG for the prediction of chronic ITP. METHODS: We evaluated 259 childhood ITP patients retrospectively who were newly diagnosed at the Department of Pediatrics, Kyungpook National University Hospital from 1983 to 2012. We analyzed the individual response to treatment and current state of disease. We evaluated the time to reach desired platelet counts after treatment of IVIG, relapse rate and diagnosis of chronic ITP. The patients were classified into 2 groups according to the time to reach desired platelet counts (50,000/microL) after daily treatment of IVIG, rapid (1 or 2 doses) and slow responder (more than 3 doses). RESULTS: Among 182 patients followed up over 6 months, 41 patients (22.5%) were eventually diagnosed with chronic ITP. Hundred and two patients (56.7%) belonged to rapid response group, and 17 of them (16.7%) were diagnosed with chronic ITP. Eighty patients (44.4%) belonged to the slow response group, and 24 of them (30%) were diagnosed with chronic ITP, which were higher than the early response group (P=0.033). CONCLUSION: Individual response rate of IVIG treatment could be a useful predictor of chronic ITP, but this finding needs support from further studies.

Changes of antithroglobulin antibody in children with congenital hypothyroidism

PURPOSE: It has been reported that antithroglobulin (anti-TG) antibody is increased in the sera of both children with transient congenital hypothyroidism and their mothers. And transplacental transport of thyroid autoantibody was proposed as the pathogenesis of transient congenital hypothyroidism. However this is not known in nontransient congenital hypothyroidism. This study was done to see changes of anti-TG antibody in children with nontransient congenital hypothyroidism. METHODS: Study patients consisted of 60 patients diagnosed as congenital hypothyroidism in the Department of Pediatrics, Kyungpook National University Children's Hospital, Daegu, Republic of Korea between January 2010 and March 2013. Healthy control were 45 children showing normal thyroid function. Anti-TG antibody and various laboratory tests were analyzed retrospectively, and compared in both children with congenital hypothyroidism and controls. RESULTS: Anti-TG antibody was significantly higher in children with congenital hypothyroidism compared to healthy controls, 119.4+/-34.7 U/mL versus 80.6+/-19.6 U/mL, respectively (P<0.001). There was no significant difference of anti-TG antibody in gender and age. CONCLUSION: We observed a significant increase of anti-TG antibody in children with nontransient congenital hypothyroidism compared to healthy controls. Further study focusing pathogenetic role of anti-TG antibody in nontransient congenital hypothyroidism is necessary. Furthermore, the clinical significance in the course of congenital hypothyroidism need to be known.

The Prevalence and Quality of Life of Overactive Bladder and Urinary Incontinence in Young Women / 대한비뇨기과학회지

PURPOSE: Urinary discomforts can affect the quality of life. However, little information is available on the relation between an overactive bladder (OAB) and urinary incontinence (UI) in young women. We conducted a questionnaire survey via internet to assess the effects that the symptoms of an overactive bladder and urinary incontinence have on the quality of life in young women. MATERIALS AND METHODS: A total of 3,372 (89.8%) women (mean age: 26.3+/-4.8years) among 3,757 participants completed the questionnaire via an internet web site. They were divided into three groups: those who had OAB, those who had UI, and the controls who had neither. The factors related to OAB and UI were analyzed by the Chi-square test. The risk ratios of OAB and UI on daily and sexual life were analyzed by a multiple logistic regression analysis. RESULTS: Of 3,372 respondents, the prevalence of OAB and UI in young women was 429 (12.7%) and 707 (21%), respectively. The factors related to OAB were history of urinary tract infection (x2=17.04, p<0.01) and family history (x2=9.95, p<0.01), and factors related to UI were age (x2=6.2, p<0.05), occupation (x2=11.0, p<0.05), history of urinary tract infection (x2=20.2, p<0.01), family history (x02=4.9, p<0.05), and parity (x2=8.6, p<0.01). The risk ratios of OAB on daily and sexual life were 5.0 and 4.3 times, respectively. The risk ratios of UI on daily and sexual life were 2.9 and 3.9 times, respectively. CONCLUSIONS: The symptoms that were suggestive of OAB and UI are highly prevalent and significantly affect the quality of life in young women. The reduction in mobility associated with OAB and UI symptoms may be especially distressing for these young and active women. Effective healthcare polices and prompt management of OAB and UI should be implemented.

The Prevalence of Overactive Bladder Syndrome and Urinary Incontinence in Young and Middle Aged Women

PURPOSE: We performed a pilot survey via internet to assess the prevalence of overactive bladder syndrome (OABs) and urinary incontinence(UI) in young and middle aged women. MATERIALS AND METHODS: From 22 April 2002 to 5 May 2002, a total of 3,372(89.8%) women(mean age: 26.3+/-4.8 years) among 3,757 participants completed a questionnaire via internet web site. They were divided into 3 groups: those who had OABs(group 1), who had UI(group 2), and controls who had neither. The factors related to OABs and UI were analyzed by multiple logistic regression analysis. RESULTS: Of 3,372 respondents, the prevalence of OABs and UI in young women was 429(12.7%) and 707 (21%), respectively. The factors related to OABs were history of urinary tract infection and family history. The factors related to UI were age, occupation, history of urinary tract infection, family history, and parity. CONCLUSIONS: The symptoms suggestive of OABs and UI are highly prevalent in young and middle aged women. So, effective healthcare polices and prompt management about OABs and UI should be concerned.

A Mixed Liposarcoma of the Paratesticular Tissue / 대한비뇨기과학회지

A liposarcoma of paratesticular origin is extremely rare, and preoperative diagnosis is difficult. Most are a low-grade malignancy. We report a case of a mixed liposarcoma of the paratesticular tissues in a 74-year-old man. Treatment consisted of a radical orchiectomy without retroperitoneal lymph node dissection. The patient has survived 18 months with no evidence of a recurrence.