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Objective:To investigate the diagnosis and treatment of congenital leukemia and the main factors affecting its prognosis.Methods:Clinical data of children with congenital leukemia admitted to Zhengzhou Children′s Hospital from July 2007 to December 2017 were retrospectively collected.Their clinical characteristics, chemotherapy and factors affecting their long-term survival were analyzed.Results:A total of 23 cases of congenital leukemia were collected, who mostly complained of " increased white blood cells, skin nodules and skin hemorrhage" . Among them, 10 cases abandoned the treatment, who all died according to the follow-up results.Among the 13 treated cases, 7 cases were male, and 6 were female.One of the 13 cases was a twin.Eight cases were acute myeloid leukemia, and 5 cases were acute lymphoblastic leukemia.One case did not perform the fusion gene test. MLL gene was positive in 2 cases and negative in 10 cases.No other fusion genes were detected.Karyotype analysis showed that 1 case was 21-trisomy and 12 cases had a normal karyotype.All 13 patients received symptomatic and/or reduced chemotherapy.The median follow-up time was 4 years and 11 months.Six cases recovered and 7 cases died during the follow-up.One case was diagnosed with acute lymphoblastic leukemia on the 6 th day after the birth in another hospital.He was not treated timely and came to Zhengzhou Children′s Hospital with general contract blocks at the age of 32 days.After checking, he was diagnosed with center leukemia and testicular leukemia.He developed a severe infection in the lung and brain after strong chemotherapy, and died even after the remote consultation with Lurie Children′s Hospital of Chicago.The other 6 cases died of severe infection and multiple organ injuries after chemotherapy, and no one died of leukemia relapse. Conclusion:Reduction chemotherapy may be an important treatment to children with congenital leukemia.
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Objective To study the prognosis of hepatoblastoma (HB) in children.Methods Retrospective analysis on the clinical data and follow-up of children with HB admitted from September 2010 to February 2015 and sum up the clinical outcome and prognosis.Results Thirty-six children with HB (23 males and 13 females) were enrolled. The median age on diagnosiswas 20 months. Allthe36 patients received follow-up, the median follow-up time was 37 months (7~71 months).(1) Of the 36 patients, 8 died. The prognosis of patients with stageⅠorⅡwas signiflcantly better than those with stage Ⅲ or Ⅳ(P<0.05). The prognosis of patients with fetal type was signiflcantly better than those with other subtypes (P<0.05). (2) Serum AFP level of 35 newly diagnosed cases increased, AFP level of survivors returned to normal after treatment. Of the 8 died patients, 3 patients had AFP elevated when relapsed, 5 cases had a persistently high AFP level after adjuvant chemotherapy. The levels of LDH, CRP, and AST at diagnosis had signiflcant influence on the prognosis (P<0.05).Conclusions Serum AFP can be a monitoring indicator for recurrence and prognosis of hepatoblastoma. POG / CCG staging, pathology classiflcation are key points when assessing the prognosis of hepatoblastoma. The prognosis of patients with high levels of LDH, CRP, and AST might be worse.
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Objective To explore the clinical feature, treatment and prognosis of hepatitis associated aplastic anemia (HAAA) in children. Method The clinical data of 5 children with HAAA were retrospectively analyzed. Results There were 5 patients (4 males and 1 female) with median age of 10 years (7~13 years ). The detection of hepatitis A, B, C, and E were all negative in these 5 children during their acute hepatitis period. In 2 children, parvovirus B19 antibody and EB virus were negative and the proportion of CD4+T cells and the ratio of CD4+/CD8+ were decreased, and the proportion of CD8+T cells was increased by T-lymphocyte subsets analysis. Three children were treated with anti-thymocyte globulin combined with cyclosporine, 2 of whom achieved complete remission and 1 died of pulmonary fungal infection. One child was treated with cyclosporine only and achieved partial remission. One child lost follow-up after giving up treatment. Conclusion HAAA in children can be caused by viral seronegative hepatitis, and usually has T lymphocyte immune disorders. Immunosuppressive therapy is effective. .
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Objective To investigate the role of regulatory B cells (Breg)in children with newly diagnosed immune thrombocytopenia (ITP).Methods A total of 35 newly diagnosed ITP children admitted to the Pediatric De-partment,the First Affiliated Hospital of Zhengzhou University from January to December 201 4 were recruited in this study,and another 20 gender -and age -matched healthy children from the Department of Medical Examination Center of the same Hospital were recruited as controls during the same period.Peripheral blood samples (3 mL from each chil-dren)were collected from all the newly diagnosed ITP children and the normal controls.Breg cells were tested by Flow Cytometry,and the expression levels of interleukin -1 0 (IL -1 0)and transforming growth factor -β1 (TGF -β1 ) mRNA were measured by real time fluorescence quantitative polymerase chain reaction.Meanwhile,the correlation be-tween Breg cells and the expression levels of IL -1 0,TGF -β1 mRNA were analyzed by Pearson correlation.Results The percentages of Breg cells in the peripheral blood of the newly diagnosed ITP children [(2.37 ±0.67)%]were sig-nificantly lower than those of the normal controls [(4.92 ±1 .32)%],and there was a significant difference (t =-7.47,P =0.000);the expression levels of IL -1 0 mRNA in the newly diagnosed ITP children(0.202 ±0.059) were significantly decreased compared with those of the normal controls(0.41 5 ±0.21 2),and there was a significant difference(t =-5.1 75,P =0.000);while the expression levels of TGF -β1 mRNA in the newly diagnosed ITP chil-dren(1 .587 ±0.823)were significantly increased than those in the normal controls(0.61 9 ±0.322),and there was a significant difference(t =4.081 ,P =0.001 ).There was a significant positive correlation between Breg cells and the ex-pression levels of IL -1 0 mRNA(rs =0.828,P <0.05),but no correlation between Breg cells and expression level of TGF -β1 mRNA was found (rs =0.527,P =0.1 1 7).Conclusions The decrease expressions of Breg cells can be found in the newly diagnosed ITP children,and the abnormal expression of Breg cells may play a key role in the immu-nological pathogenesis of the newly diagnosed ITP children.
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<p><b>OBJECTIVE</b>To explore significance of serum soluble CD163(sCD163) and soluble CD25(sCD25) in diagnosis and guiding treatment of children with hemophagocytic lymphohistiocytosis (HLH).</p><p><b>METHOD</b>Data of 42 cases of children with HLH, 32 cases of non-HLH children with infection presented to First Affiliated Hospital of Zhengzhou University pediatric clinic and ward were collected from December 2013 to December 2014. Twenty-four healthy children were enrolled into a normal control group in the same period.Peripheral venous blood specimens (3 ml) were taken from the children with HLH after fasting before treatment, two weeks after treatment and eight weeks after treatment.Peripheral venous blood specimens (3 ml) were also taken from children of non-HLH infected group and normal control group after fasting at the initial visit. Serum sCD163 and sCD25 levels in the peripheral blood in three groups were determined by ELISA. According to cause of disease, children with HLH were divided into infection-related HLH, tumor-related HLH, primary HLH and others; relationship between serum sCD163 and sCD25 level and cause of disease was analyzed.</p><p><b>RESULT</b>Serum sCD163 of HLH group ((6 094 ± 2 769) µg/L) and serum sCD163 of non-HLH infection group ((2 174 ± 950) µg/L) were significantly higher than that of normal control group ((777 ± 256) µg/L), F=71.396, P<0.05), and the differences among groups were statistically significant (P<0.05); serum sCD25 of HLH group ((41 963 ± 31 821) ng/L) and serum sCD25 of non-HLH infection group ((6 700 ± 4 105) ng/L) were significantly higher than that of normal control group ((2 440 ± 1 870) ng/L, F=37.513, P<0.05).There was no statistically significant difference between the non-HLH infection group with the normal control group (P>0.05), and the difference between the remaining groups was statistically significant (P<0.05). And serum sCD163 and sCD25 level of HLH group had a positive linear correlation, and Pearson correlation coefficient r=0.742 (t=7.000, P<0.05). The difference of serum sCD163 and sCD25 level among the different cause of disease in HLH group was significant (P<0.05).Pairwise comparison showed that serum sCD163 and sCD25 level of tumor-associated HLH group significantly increased as compared with infection-associated HLH group (P<0.05), but the difference was not statistically significant between the other groups (all P>0.05). Serum sCD163 and sCD25 level of HLH group before treatment, 2 weeks and 8 weeks after treatment showed a statistically significant tendency of decrease (P<0.05). Seen from the ROC curve, when sCD163 cut-off point was 2 359.08 µg/L, the diagnostic sensitivity was 83.3%, and specificity was 83.9%.When sCD25 cut-off point was 14 901.024 ng/L, the diagnosis sensitivity was 76.2%, and specificity was 98.2%.</p><p><b>CONCLUSION</b>Serum sCD163 and sCD25 levels may be used for diagnosis of HLH.Dynamically monitoring of serum sCD163 and sCD25 level can help to determine deterioration of HLH and guide treatment.</p>
Subject(s)
Child , Humans , Antigens, CD , Blood , Antigens, Differentiation, Myelomonocytic , Blood , Case-Control Studies , Enzyme-Linked Immunosorbent Assay , Interleukin-2 Receptor alpha Subunit , Blood , Lymphohistiocytosis, Hemophagocytic , Blood , Diagnosis , Therapeutics , ROC Curve , Receptors, Cell Surface , Blood , Sensitivity and SpecificityABSTRACT
Objectives To investigate the rational treatment strategy of hepatoblastoma (HB) in children. Methods Clinical data and follow-up of 25 children with HB admitted from February 2009 to March 2013 were retrospectively analyzed. Results Twenty-five children with newly diagnosed HB (14 males and 11 females) were enrolled. The median age on diagnosis was 25 months (3-92 months);In 18 of 25 cases with complete resection of the primary tumor, 17 cases survived. Only 1 of 7 cases with incomplete resection survived. The survival rate in children with complete resection of primary tumor is significantly higher than those without complete resection (P<0.05). The survival rate in children of stage I or II is significantly higher than the children of stage III or IV (P<0.05). Conclusions Complete tumor resection is the cornerstone of therapy for long-term disease-free survival in HB patients. Treatment strategy remains to be further improved for children with recurrent and metastatic HB.
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Objective To analyze the serumα1-antitrypsin (α1-AT) and α1-glycoprotein (α 1-AGP) in children with Wilms tumor,and to explore the clinical application value.Methods Thirtyeight children with Wilms tumor who received treatment from January 2007 to December 2010 were designed as observation group,38 healthy children were designed as control group.Fluorescence quantitative PCR was performed to analyze α 1-AT andα 1-AGP mRNA transcription levels of two groups,and ELISA was used to measure the serum concentration of α 1-AT and α 1-AGP.Results The mRNA levels of α 1-AT and α 1-AGP in observation group were 3.50 ± 0.37 and 2.10 ± 0.41,which were significantly higher than those in control group (0.90 ± 0.45,0.50 ± 0.24) (P =0.000,0.002).The levels of α 1-AT and α 1-AGP in observation group were (4516.8 ± 102.4),(1316.0 ± 27.3) g/L,which were significantly higher than those in control group [(2467.4 ± 23.8),(728.6 ± 9.4) g/L] (P =0.015,0.008).Conclusion To detect the levels of α 1-AT and α 1-AGP have the potential value for the diagnosis of Wilms tumor,which can be applied to early screening.