Journal of Clinical Pediatrics

The immunotherapy progress of PD-1/PD-L1 inhibitors in children with refractory lymphoma

QIAO Xiaohong

Journal of Clinical Pediatrics. 2022, 40(1): 1. doi:10.12372/jcp.2022.21e1679

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Immune checkpoint inhibitors targeting programmed cell death protein- 1 (PD- 1 ) or programmed death ligand-1 (PD-L1) have shown certain efficacy in some clinical studies for refractory and recurrent children's malignant blood diseases and tumors. It has certain anti-tumor effect on refractory and recurrent Hodgkin lymphoma and primary mediastinal B-cell lymphoma, and is relatively well tolerated in children and adolescents. PD-1/PD-L1 inhibitors do not seem to increase the infection rate, but for glucocorticoid and/or TNF supplementation-伪 targeted drug immunosuppressive therapy suggests the use of preventive measures against Pneumocystis carinii. The combination of PD- 1 /PD-L 1 inhibitors with other types of immune checkpoint inhibitors, chemotherapy agents and histone deacetylase inhibitors is still in the clinical research stage. The dosage, course of treatment and scheme of PD- 1 /PD-L 1 inhibitors for children still need further research in multiple centers. Whether they can achieve lasting antitumor effect also needs further research.

Diagnosis and treatment of hemorrhagic cystitis after hematopoietic stem cell transplantation

HE Yunyan

Journal of Clinical Pediatrics. 2022, 40(1): 8. doi:10.12372/jcp.2022.21e1628

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Hemorrhagic cystitis (HC) is one of the common complications of allogeneic hematopoietic stem cell transplantation, which is the key point of transplantation prevention and treatment. This article introduces the etiology, pathogenesis, prevention and treatment of HC. The occurrence of HC is often related to the toxicity of high-dose radiotherapy and chemotherapy before transplantation, gene polymorphism of drug metabolism enzyme, viral infection, graft-versus-host disease, patient age, sex, donor type and transplantation method. Diagnosis of HC is not difficult, based on the history, typical clinical manifestations, and necessary auxiliary tests. Effective preventive measures are adequate hydration and alkalization of urine, diuresis and the application of Mesna. The treatment mainly includes antiviral therapy, immunotherapy, bladder irrigation, bladder perfusion, hyperbaric oxygen therapy and surgical treatment.

Expert consensus on GD2 antibody dinutuximab-β in the treatment of neuroblastoma

Collaborative Group on GD Monoclonal Antibody in the Treatment of Neuroblastoma

Journal of Clinical Pediatrics. 2022, 40(1): 14-20. doi:10.12372/jcp.2022.21e1685

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Clinical analysis of acute kidney injury after allogeneic hematopoietic stem cell transplantation in children

NIE Yingming, LIU Jing, QI Chang, et al

Journal of Clinical Pediatrics. 2022, 40(1): 21. doi:10.12372/jcp.2022.21e0892

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Objective To analyze the clinical characteristics and risk factors of acute kidney injury (AKI) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children. Methods The clinical data of children who received allo-HSCT in the Department of Hematology and Oncology, Wuhan Children's Hospital from August 2016 to March 2020 were retrospectively analyzed. Serum creatinine level (Cr) and creatinine clearance rate (Ccr) before and 100 days after transplantation were compared, and survival rate 1 year after transplantation was analyzed. The risk factors of AKI were analyzed by logistic regression. Results A total of 147 children ( 85 boys and 62 girls) with allo-HSCT were included in the study, with a median age of 5 . 5 years ( 0 . 7 months to 16 years) at the time of transplantation. AKI occurred in 101 patients ( 68 . 7 %) with a median time of 24 . 0 (- 5 . 0 - 91 . 0 ) days after transplantation. Binary logistic regression analysis showed that acute graft-versus-host disease (aGVHD) and sinusoidal obstruction syndrome (SOS) were independent risk factors for AKI after transplantation (Prate was ( 95 . 70 ±2 . 97 ) % in the non-AKI group and ( 84 . 00 ±3 . 86 ) % in the AKI group, and the difference between the two groups was statistically significant (P<0 . 05 ). Conclusions AKI is common in children with allo-HSCT, and aGVHD and SOS were the risk factors for AKI. AKI was associated with 1-year OS.

The in luence of chemotherapy on gonadal function in acute lymphoblastic leukemia children

CAO Ke, LUO Xiaojuan, WANG Ying, et al

Journal of Clinical Pediatrics. 2022, 40(1): 27. doi:10.12372/jcp.2022.21e0842

Abstract ( 392 )

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Objective To investigate the influence of different intensity of chemotherapy on hormonal markers of gonadal function in children with acute lymphoblastic leukemia (ALL). Methods Based on the chemotherapy regimens of South China children's leukemia Group (SCCLG-ALL- 2016 ), a total of 112 newly diagnosed ALL preadolescent were included. Serum anti-Müllerian hormone (AMH) and inhibin B (INHB) levels were detected by chemiluminescence assay before and 3 , 6 , 12 and 18 months after chemotherapy, and were compared according to gender and risk type. Results A total of 112 patients ( 58 boys and 54 girls) with ALL were enrolled, with a median age of 4 . 1 ( 2 . 5 - 6 . 1 ) years. There were 24 high-risk children, 58 medium-risk children, and 30 low-risk children. In the normal control group, there were 57 patients ( 28 boys and 29 girls), with a median age of 4 . 0 ( 2 . 5 - 6 . 3 ) years. Repeated measures analysis of variance showed that there were statistically significant differences in serum INHB levels among low-risk, medium-risk and high-risk boys (F= 3 . 60 , P= 0 . 036 ) at different times before and after chemotherapy (F= 81 . 67 , P< 0 . 001 ). Time of chemotherapy and risk type had an interaction effect on serum INHB level (F=5.12, P< 0 . 001 ). There was no statistically significant difference in serum AMH level between low-risk, medium-risk and high-risk girls (F= 0 . 62 , P= 0 . 551 ), and there was statistically significant difference in serum AMH level between different time before and after chemotherapy (F=21 . 32 , P< 0 . 001 ). Time of chemotherapy and risk type had an interaction effect on serum AMH level (F=2.33, P= 0 . 029 ). Conclusions Although the children with ALL were still prepubertal, SCCLG-ALL- 2016 chemotherapy could change the hormonal markers of gonadal function. Moreover, the influence degree of high-risk chemotherapy was higher than that of low and medium risk chemotherapy, suggesting that gonadal function of children with ALL was impaired to varying degrees during chemotherapy.

Efficacy and safety of neurokinin 1 receptor antagonists for the prevention of highly emetogenic chemotherapy-related nausea and vomiting in pediatric patients with cancer

YU Liting, WANG Zhuo, ZHOU Fen, et al

Journal of Clinical Pediatrics. 2022, 40(1): 34. doi:10.12372/jcp.2022.21e0959

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Objective To evaluate the clinical efficacy and safety of neurokinin 1 receptor antagonists (NK- 1 RA) for the prevention of nausea and vomiting induced by highly emetogenic chemotherapy (HEC) in children with cancer. Methods The pediatric patients who received NK-1RA to manage nausea and vomiting caused by HEC between May 2020 and December 2020 were enrolled in this study. The children treated with fosaprepitant in combination with ondansetron and dexamethasone were enrolled in the fosaprepitant group, and the children treated with aprepitant in combination with ondansetron and dexamethasone were included in the aprepitant group. The clinical efficacy and safety of the two groups were compared after treatment. Results A total of 122 children ( 83 boys and 39 girls) were included. The median age was 8 . 0 ( 4 . 8 ~ 11 . 0 ) years. Sixty-two patients were included in the fosaprepitant group and 60 in the aprepitant group. After NK- 1 RA was used to prevent chemotherapy-induced nausea and vomiting (CINV), the complete remission (CR) rate of acute, delayed, and overall vomiting (i.e., non-vomiting rate) was 88 . 5 %, 71 . 3 % and 65 . 6 %, respectively. In the stage of acute vomiting, there was significant difference in the distribution of vomiting severity between the fosaprepitant group and aprepitant group (P

Clinical and imaging characteristics of acute lymphoblastic leukemia with leukoencephalopathy in children

CHU Sijia, TANG Jihong, ZHANG Bingbing, et al

Journal of Clinical Pediatrics. 2022, 40(1): 40. doi:10.12372/jcp.2022.21e0975

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Objective To investigate the clinical and neuroimaging features of childhood acute lymphoblastic leukemia (ALL) complicated with leukoencephalopathy, so as to improve the understanding of it. Methods The clinical data of 28 children with ALL who developed leukoencephalopathy from May 2011 to April 2021 were retrospectively analyzed. Results Among the 28 patients, there were 18 boys and 10 girls. The median onset age of ALL was 6 . 63 ( 1 . 37 - 12 . 87 ) years, and the median onset age of leukoencephalopathy was 7 . 81 ( 1 . 45 - 14 . 01 ) years. Neurological manifestations were found in 24 children, and the main symptoms were epileptic seizure ( 13 cases), paralysis ( 8 cases), limb numbness ( 6 cases), visual impairment ( 6 cases) and slurred speech ( 4 cases). On neuroimaging, the lesions were mainly distributed in the parietal lobe ( 10 cases), periventricular and central white matter area of semicovale ( 9 cases), basal ganglia ( 6 cases), frontal lobe ( 4 cases) and occipital lobe ( 4 cases). The lesions were multiple and irregular, distributed in small or large patches. After the occurrence of ALL with leukoencephalopathy, chemotherapy drugs were stopped immediately and symptomatic treatment was given, and the clinical symptoms and imaging lesions gradually disappeared in most cases. During the 1 -year follow-up, 19 of the 28 cases were re-examined with head MRI, 16 cases ( 84 . 2 %) showed significant improvement in lesions compared with the first time, and 11 cases ( 57 . 9 %) showed complete disappearance of abnormal signals. Conclusions Leukoencephalopathy may be an important complication during the treatment of ALL. Although the clinical and radiographic manifestations of ALL with leukoencephalopathy are diverse, children with early diagnosis and treatment have better prognoses when neurological symptoms occur.

Hematopoietic stem cell transplantation with low intensity conditional regimen for Fanconi anemia in children: a report of 3 cases

CHEN Zhiwei, LIU Jian, WANG Yingjie, et al

Journal of Clinical Pediatrics. 2022, 40(1): 46. doi:10.12372/jcp.2022.21e0720

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Objective To investigate the efficacy of hematopoietic stem cell transplantation (HSCT) with low intensity conditional regimen in the treatment of Fanconi anemia (FA), and to provide basis for the optimization of FA transplantation. Methods The clinical data and follow-up results of patients with FA treated by HSCT in our hospital from January 2018 to January 2021 were retrospectively analyzed. Results There were 3 children ( 1 girl and 2 boys) with FA. The median age of onset was 6 . 1 ( 3 . 6 - 13 . 4 ) years, and the median age of transplantation was 7 .2 ( 5 . 7 - 14. 0) years. The 3 patients were treated with HSCT from HLA-mismatched unrelated donor ( 9 /10 ), HLA-matched sibling donor ( 10 /10 ) and HLA-matched unrelated donor ( 10 / 10 ), respectively. The conditional regimen consisted of busulfan, low dose of cyclophosphamide ( 40 mg/kg) and antithymocyte globulin. In the 3 children with FA, the median number of returned mononuclear cells was 9 . 0 ( 8 . 9 - 11 . 2 ) ×108 /kg, and the median number of peripheral CD 34 + cells was 4 . 5 ( 3 . 1 - 5 . 2 ) ×106 /kg. Hematopoietic function was reconstructed in all the 3 children with FA. The median time of neutrophils engraftment was 12 ( 12 - 14 ) days, and the median time of platelet engraftment was 13 ( 12 - 14 ) days. Follow-up was performed to April 2021 . Two patients survived without events, and 1 patient died due to the complications of severe infection and graft-versus-host disease (GVHD) grade Ⅲ. Conclusion HSCT with low intensity of conditional regimen is feasible for the treatment of FA, and it is necessary to continuously explore individualized HSCT regimens for FA.

Analysis of risk factors of nosocomial infection in children with congenital heart disease after surgery

LUO Runjiao, DU Xinwei, GONG Xiaolei, et al

Journal of Clinical Pediatrics. 2022, 40(1): 51. doi:10.12372/jcp.2022.21e0492

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: Objective To investigate the relationship between preoperative neutrophil lymphocyte ratio (NLR) and other factors and postoperative nosocomial infection in children with congenital heart disease (CHD). Methods A total of 11937 children who underwent cardiac surgery from 2006 to 2017 were collected. According to the inflection point of NLR distribution with age and whether nosocomial infection occurred, the children with the age of score and abnormal LWR were independent protective factors for postoperative nosocomial infection in children≥224 days (P<0 . 05 ). The area under the curve (AUC) of NLR for predicting nosocomial infection in children <224 days was 0 . 594 ( 95 %CI: 0 . 572 ~ 0 . 617 ). When NLR>0 . 542 , its sensitivity and specificity for diagnosing nosocomial infection were 41 . 8 % and 73 . 6 %. Conclusion The postoperative infection rate of CHD children has decreased in recent years. Younger age, low BMI-Z score, preoperative infection, complex surgery, long operation time and increased NLR are the risk factors for postoperative infection in CHD children.

Kawasaki disease with arthritis: a report of two cases and literature review

LIU Lei, SONG Xiaoxiang, FENG Qihua

Journal of Clinical Pediatrics. 2022, 40(1): 58. doi:10.12372/jcp.2022.21e0827

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Objective To analyze the clinical features of Kawasaki Disease (KD) with arthritis and to explore the differential diagnosis between KD with arthritis and systemic juvenile idiopathic arthritis (SJIA). Methods The clinical data of two KD children with arthritis were retrospectively analyzed and the relevant literature was reviewed. Results One child was admitted witha high fever and abdominal pain complicated with gastrointestinal bleeding and macrophage activation syndrome (MAS), which was improved after hormone therapy. The child developed joint symptoms during the convalescence, and was considered as KD with arthritis. The other patient presented with fever and joint swelling and pain as the first symptoms. After treatment with two intravenous immunoglobulin (IVIG), intravenous methylprednisolone and methotrexate (MTX), the joint symptoms were not relieved significantly, but improved after treatment with recombinant human type II tumor necrosis factor receptor-antibody fusion protein (rhTNFR:Fc), and the child was considered as KD with JIA. Conclusions KD with arthritis and SJIA have high similarities in clinical manifestations. At present, there is no specific laboratory examination to distinguish the two diseases. Long-term clinical observation is needed to establish more precise diagnostic criteria and develop more experimental methods to assist diagnosis.

Bernard-Soulier syndrome caused by compound heterozygous variations of GP1BA gene: a case report

WANG Yan, SHEN Diying, ZHANG Jingying, et al

Journal of Clinical Pediatrics. 2022, 40(1): 63. doi:10.12372/jcp.2022.21e0390

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Objective To analyze the clinical characteristics and genetic etiology of a case of Bernard-Soulier syndrome. Methods The clinical and molecular genetic data of a child diagnosed with BSS were reviewed. Results The patient was a 5 -year-old boy. Thrombocytopenia occurred in the newborn period, and the platelet count was maintained at ( 25 ~ 40 ) × 109 /L. Gamma globulin and hormone therapy were not effective. The expression level of platelet membrane glycoproteinⅠbα(GPⅠbα) was 42.4%, which was significantly lower than normal level. Whole exome sequencing detected two variations in the GP 1 BA gene of the child, c. 987 G>A and c. 523 _ 525 delAAC. The Sanger family verified that the variations were derived from their parents respectively and constituted a compound heterozygous variant. Conclusion The variation site of c.523 _ 525 delAAC has not been reported, which expands the BSS gene variation spectrum.

Research progress of pediatric sepsis caused by carbapenem-resistant Klebsiella pneumoniae

LI Jiashan

Journal of Clinical Pediatrics. 2022, 40(1): 67. doi:10.12372/jcp.2022.21e0627

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In recent years, the resistance of gram-negative bacteria to antibiotics has reached a critical level, and the detection rate of carbapenem-resistant Klebsiella pneumoniae (CRKP) in pediatric wards in many countries has been increasing year by year. CRKP sepsis has an insidious onset and lacks specific clinical manifestations. However, CRKP sepsis is highly pathogenic, progresses rapidly, and is prone to multiple organ failure, which can endanger the lives of children. Carbapenem antibiotics are still the main treatment for CRKP infection, and new antibiotics are also being studied. This article reviews the clinical research progress of CRKP sepsis.

Advances in molecular genetics of nephropathy associated with primary coenzyme Q10 deficiency

WAN Ling, CHEN Chaoying

Journal of Clinical Pediatrics. 2022, 40(1): 73. doi:10.12372/jcp.2022.21e0173

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Department of nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, Beijing 100020 , China)

2022 Contribution

Journal of Clinical Pediatrics. 2022, 40(1): 78.

Abstract ( 367 )

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