ABSTRACT
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.
ABSTRACT
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±25×10(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 ≥100×10(9)/L. And 66.0% (31/47) maintained a platelet count ≥50×10(9)/L until the last follow-up date. The time periods required for the platelet count to be maintained ≥50×10(9)/L and ≥100 ×10(9)/L were 3.1±2.7 and 3.6±2.7 years. Age at diagnosis in the ≥50×10(9)/L group (5.7±4.4 years) was significantly lower than the age at diagnosis in the <50×10(9)/L group (7.4±3.3 years) (P=0.040). And follow-up period was the factor influencing prognosis between the ≥100×10(9)/L group and <50×10(9)/L group (P=0.022). CONCLUSION: Approximately 45% of children with chronic ITP recovered spontaneously about 3-4 years after the diagnosis and 2/3 of patients maintained a platelet count ≥50×10(9)/L, relatively safe state. Age at diagnosis of ITP and follow-up period were the factors influencing prognosis in this study.
ABSTRACT
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.
ABSTRACT
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.
