Journal of Clinical Pediatrics

Standardized treatment and management of immune thrombocytopenia in children

ZHANG Aijun, LIU Qinqin, PU Ting

Journal of Clinical Pediatrics. 2022, 40(2): 81-86. doi:10.12372/jcp.2022.21e1625

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Immune thrombocytopenia (ITP) is the most common bleeding disorde characterized by isolated thrombocytopenia in children. It is mediated by a variety of autoimmune mechanisms. Children with ITP usually have acute onset. A considerable proportion of patients can be traced back to the history of precursor infection or vaccination, which can make them immune intolerant, resulting in the occurrence of ITP. There is strong heterogeneity of ITP in children. At present, more and more children tend to relapse. The first-line treatment is not effective, and the protracted treatment leads to chronic ITP. Clinicians should make corresponding individualized treatment and give reasonable management according to the characteristics of each child to reduce treatment-related complications and improve the quality of life of children.

Inherited platelet function disorders: diagnosis, treatment and management

YANG Xiaoyan, BIAN Qiuhan, TUO Yuanyuan, WANG Dinghuan, HUANG Jing

Journal of Clinical Pediatrics. 2022, 40(2): 87-94. doi:10.12372/jcp.2022.21e1618

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Inherited platelet function disorders (IPFDs) is a rare disorder. The clinical manifestations were heterogeneous, mainly characterized by spontaneous cutaneous and mucosal hemorrhage, menorrhagia, difficulty in hemostasis after trauma, with or without thrombocytopenia. Its incidence has been underestimated due to difficulties in clinical diagnosis. Treatment and management of the disease are also challenging. This study summarized the classification, clinical manifestations, diagnosis, treatment and management of IPFDs, to improve the understanding of IPFDs and provide reference for diagnosis, treatment and management of IPFDs for front-line pediatricians.

Expression and clinical essential of chemokines CCL3 and CCL4 in children with ITP

CHEN Dongping, LUO Xi, HUANG Pei, GUO Yimin, WU Liusong, HE Zhixu, CHEN Yan

Journal of Clinical Pediatrics. 2022, 40(2): 95-100. doi:10.12372/jcp.2022.21e1233

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Objective To investigate expression and clinical significance of chemokines CCL3 and CCL4 in childhood immune thrombocytopenia (ITP). Methods Children with ITP diagnosed in Affiliated Hospital of Zunyi Medical University from December 2019 to April 2020 were selected as study subjects, and were divided into newly diagnosed group (n=37, disease duration ≤3 months) and non-newly diagnosed group (n=27, disease duration >3 months) according to the disease duration, and into pre-treatment group (n=29) and post-treatment group (n=35) according to the timing of specimen collection (at the first diagnosis and 7th day of hospital admission); and healthy children with outpatient health examination were used as the control group, and the expression of CCL3 and CCL4 and platelet-related indexes were compared between different groups. Results A total of 64 children with ITP, 42 males and 22 females, with a median age of 5.1 (3.0-8.0) years, and 19 healthy controls, 12 males and 7 females, with a median age of 4.2 (2.8-6.0) years, were included. The differences in PLT counts, MPV, P-LCR, PDW, PCT, and IgG levels between the pre-treatment group, post-treatment group, and control group were statistically significant (P<0.05); PLT counts and PCT were lower and MPV, P-LCR, PDW, and IgG were higher in the pre-treatment group. CCL3 and CCL4 concentrations were higher in the newly diagnosed and non-newly diagnosed groups than in the control group, with statistically significant differences (P<0.05). The CCL3 and CCL4 concentrations were higher in the pre-treatment and post-treatment groups than in the control group, and the CCL3 and CCL4 concentrations were lower in the post-treatment group than in the pre-treatment group, with statistically significant differences (P<0.05). The differences in CCL3 and CCL4 expression between different bleeding score groups of ITP were statistically significant (P<0.05). The expression levels of CCL3 and CCL4 increased gradually with the increase of bleeding score. The CCL3 and CCL4 expression levels in the treatment ineffective group were higher than those in the complete response group and the response group, while the CCL3 and CCL4 expressions in the response group were lower than those in the complete response group, and the differences were statistically significant (P<0.05). Conclusion CCL3 and CCL4 can be used as children's molecular markers of ITP to assess its severity and response to treatment.

Analysis of clinical characteristics of immune thrombocytopenia children with myocardial injury

QI Shan-shan, WANG Zhuo, LI Jianxin, CHEN Jingpei, YANG Shijia, CHEN Zhi, SUN Ming, CHEN Yan, XIONG Hao

Journal of Clinical Pediatrics. 2022, 40(2): 101-106. doi:10.12372/jcp.2022.21e1231

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Objective To investigate the clinical characteristics of immune thrombocytopenia (ITP) children with myocardial injury. Methods Newly diagnosed ITP children hospitalized in Wuhan Children's Hospital from December 2018 to April 2020 were retrospectively analyzed. According to the complication, children were divided into the non-myocardial injury group and myocardial injury group. The clinical characteristics, laboratory examination and prognosis of the two groups were compared. Results In this study, 360 newly diagnosed ITP children were analyzed, including 240 boys and 120 girls, with a median age of 3.0 (2.0~6.0) years. Of the 360 children, 294 were in the non-myocardial injury group and 66 were in myocardial injury group. Compared with the non-myocardial injury group, levels of CK, CK-MB and LDH-L in myocardial injury group were higher, the proportions of girls and younger children were higher, the values of thromboelastogram-maximal amplitude were larger, the proportions of children with PLT <10×10 ⁹/L / bleeding score >3 were lower, the counts of platelet, white blood cells, CD8 ⁺T cells and NK cells were higher, the levels of IgE were lower. These differences were statistically significant (P<0.05). Log-rank analysis showed that platelet recovery of the myocardial injury group was slower than the non-myocardial injury group after treatment (P<0.001). Conclusion It is not rare for ITP children with myocardial injury. Although platelet recovery is slower at initial diagnosis, myocardial injury does not promote the progression of newly diagnosed ITP to persistent ITP.

Diagnostic value of MRI in children with acute lymphoblastic leukemia misdiagnosed as juvenile idiopathic arthritis

XU Lin, SUN Aimin, HUANG Hua, PAN Huihong, ZHONG Yumin

Journal of Clinical Pediatrics. 2022, 40(2): 107-112. doi:10.12372/jcp.2022.21e0851

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Objective The aim of this study is to assess the diagnostic value of magnetic resonance imaging (MRI) in children with acute lymphoblastic leukemia (ALL) misdiagnosed as juvenile idiopathic arthritis (JIA). Methods Retrospective analysis was performed on children with lower extremity pains as the first symptom in our hospital from January 2018 to December 2020 who were initially diagnosed as JIA. Among them, fifteen patients were finally diagnosed as ALL confirmed by bone marrow aspiration, eighteen patients were JIA. All of them underwent blood routine and bilateral knee MRI examination before any treatment. The MRI were performed, including T1WI, spectral attenuated inversion recovery (SPAIR) T2WI and DWI (b value was 0 and 600 s/mm²). The MRI features of bone marrow were assessed by extent and location, the signal intensity, morphological features. The ADC value of all knee joints were measured and were compared between the two groups. The diagnostic value was calculated. Results There were no statistically significant differences in WBC count, hemoglobin (Hb), platelet count (PLT), C- creative protein (CRP) and erythrocyte sedimentation rate (ESR) between the case group and the control group (P>0.05). Abnormal signal intensity of bone marrow in bilateral bones of lower extremities was demonstrated in 13 patients with ALL patients which showed diffuse homogeneous iso- or low-signal intensity on T1W images and high signal intensity on T2 SPAIR images; other 2 patients had normal results of MRI. In 18 patients with JIA, 14 patients showed ill-defined patchy areas in the metaphysis and epiphysis with iso- or low-signal intensity on T1W images and high signal intensity on T2 SPAIR images; other 4 cases of JIA patients had normal results of MRI. The positive rate in the case group was higher than that in the control group. Compared with the control group, the MRI features of the case group were bilateral diffuse low signal intensity in T1W images and high signal intensity in T2W images (P<0.01). The ADC values in ALL group were lower than control group (t=-14.133, P<0.01). Taking 0.88×10^-3 mm² /s as the threshold, AUC of ROC curve in diagnosis of ALL was 0.895, with sensitivity, specificity and diagnostic accuracy was 86.7%, 83.3% and 84.9%. Conclusion MRI may serve as a noninvasive method to help discrimination of ALL from JIA in children presenting with lower extremity pains. MRI show diffuse abnormal signal intensity on bilateral lower extremities are more likely to be initial ALL.

Correlation between serum ferritin level and prognosis of childhood acute lymphoblastic leukemia

LIU Yu, YUE Yingbin, YAN Mei

Journal of Clinical Pediatrics. 2022, 40(2): 113-117. doi:10.12372/jcp.2022.21e1295

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Objective To explore the relationship between serum ferritin (SF) and the treatment response, clinical risk and prognosis in children with acute lymphoblastic leukemia (ALL). Methods Clinical data and SF levels and bone marrow examination at 4 time points, i.e., first diagnosis (T1), before early enhancement (T2), before consolidation therapy (T3), and before maintenance therapy (T4) of 87 children with ALL admitted from October 2014 to January 2021 were collected. Results Among the 87 patients, 68 (78.16%) survived without incident for 3 years, and 19 (21.84%) with poor prognosis; the SF level of children in the poor prognosis group (269.24±79.25) was significantly higher than that of the accident-free survival group (174.06±51.12), and the difference was statistically significant (P <0.05). The average SF level of children in the high-risk group (232.86±64.28) was higher than that of the non-high-risk (low risk and intermediate risk) group (172.73±53.87), and the difference was statistically significant (P<0.01). At T1 and T2, there was no statistically significant difference in SF levels between the high-risk group and the non-high-risk group (P>0.05). But at T3 and T4, the SF level of children in the high-risk group was significantly higher than that of the non-high-risk group, and the difference was statistically significant (P<0.01); At T3 and T4, the SF level of the children in the high-risk group was higher than that of the same group at T1, the difference was statistically significant (P<0.01). High-risk children with high level ferritin were less responsive to treatment than high-risk children with normal ferritin, that is, before maintenance treatment the total number of positive minimal residues, positive fusion genes, recurrences and deaths in the ferritin overdose group (72.72%) was significantly higher than those of ferritin normal group (28.57%), and the difference was statistically significant (P<0.05). Conclusion Ferritin excess is related to poor response to treatment in children with ALL, and it is one of the risk factors for poor prognosis of ALL. In order to improve the treatment effect of children with ALL, it is recommended to dynamically monitor SF levels during chemotherapy.

Clinical analysis of 51 cases of acute lymphoblastic leukemia with seizures in children

XU Min, ZHOU Wenjie, TANG Jihong, SHI Xiaoyan, ZHANG Bingbing, XIAO Xiao

Journal of Clinical Pediatrics. 2022, 40(2): 118-122. doi:10.12372/jcp.2022.21e1106

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Objective To analyze the clinical characteristics of seizures in children with acute lymphoblastic leukemia (ALL) during treatment. Methods The clinical data of children diagnosed with ALL complicated with seizures from January 2011 to June 2019 were analyzed retrospectively. Results A total of 978 children with ALL were admitted, of which 51 (5.2%) developed seizure symptoms after ALL treatment, 39 males and 12 females, with a median age of 7.4 years (3.7-11.1). Their common etiologies were chemical drug neurotoxicity (12 cases), intracranial hemorrhage (7 cases), hyponatremia (6 cases), white matter encephalopathy (5 cases), febrile convulsions (5 cases), and reversible posterior encephalopathy syndrome (4 cases). First seizures were relatively common with generalized seizures, and depending on the cause of the seizures, they were often accompanied by impaired consciousness, focal symptoms and other clinical features. A total of 39 children in this group underwent cranial CT or MRI after the first seizure, and 27 were abnormal. In the 30 cases with successful follow-up, 14 cases had abnormal imaging, including 8 cases with reversible foci and 4 cases with cerebral softening foci. Twenty-eight cases had abnormal EEG results after the first seizure, mainly with medium-high amplitude slow wave emission. Of the 30 children successfully followed up, 18 had no significant sequelae. Conclusion Seizures are common neurological complications in children with ALL during treatment. The etiology of the first seizure and its accompanying clinical characteristics are diverse. Timely neuroimaging and EEG examinations are helpful for early diagnosis and treatment.

Clinical features and prognosis of 175 children with hemophagocytic lymphohistiocytosis

MAO Shuting, WANG Xin, LIU Yufeng

Journal of Clinical Pediatrics. 2022, 40(2): 123-128. doi:10.12372/jcp.2022.21e1321

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Objective To investigate the clinical characteristics and prognostic factors of hemophagocytic lymphohistosis (HLH). Methods The clinical data of 175 children with HLH admitted from January 2015 to October 2020 were retrospectively analyzed. Clinical manifestations of the patients of HLH in the survival group and the deceased group were compared. Log-rank test and COX proportional riskregression model were used for survival analysis. Results Among the 175 cases, 91 were male and 84 were female, with a median age of 2.0 (1.0-8.0) years, and the most common onset age was in infancy (54.3%). There were 9 cases of primary HLH and 166 cases of secondary HLH. Infection-associated HLH was the commonest in secondary HLH (66.9% of them were EBV infection). The clinical manifestations were persistent fever (99.4%) and splenomegaly (72%). Laboratory examination showed that elevated serum LDH was the most significant (97.1%), followed by serum ferritin≥500μg/L (85.1%). Compared with the surviving group, children in the deceased group had lower WBC, lower HB, longer APTT, longer PT, lower FIB, higher central nervous system involvement, all with statistically significant differences (P<0.05). Central nervous system involvement and fibrinogen≤1.5g/L were independent prognostic factors of OS. Conclusions HLH is most often seen in infants, and the commonest clinical manifestation is fever with abnormally elevated serum LDH. Central nervous system involvement and FIB levels are important for the assessment of the outcome and prognosis of children with HLH.

Central nervous system post-transplant lymphoproliferative disorder after allogeneic hematopoietic stem cell transplantation in children: one case report and literature review

XIE Yingying, CHEN Xia, YANG Wenyu, LIU Fang, ZHAO Beibei, ZHANG Xiaoyan, REN Yuanyuan, ZHANG Ranran, ZHU Xiaofan, GUO Ye

Journal of Clinical Pediatrics. 2022, 40(2): 129-133. doi:10.12372/jcp.2022.21e0696

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Objective To investigate the clinical feature of central nervous system post-transplant lymphoproliferative disorder (CNS-PTLD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children. Methods Firstly, we retrospectively analyzed the clinical data of one case after transplantation, then summarized relevant literature. Results The child, a 13-year-old male, was diagnosed with acute myeloid leukemia M5 (positive for TLS-ERG fusion gene), achieved complete remission with remission chemotherapy, turned negative for TLS-ERG, and then turned positive for TLS-ERG after 2 courses of MAE regimen and high-dose cytarabine based regimen, showed molecular biological relapse and was eligible for transplantation, so allo-HSCT was performed (paternal donor, HLA 5/10). Fever developed at +162d after transplantation, with a higher fever peak at +170d than before, and apathy, lethargy, headache and vomiting. In combination with cranial CT and enhanced MRI findings, cerebrospinal fluid EBV DNA amount (14903 copies/mL), and cerebrospinal fluid second-generation sequencing results (human gamma herpesvirus type 4 (EBV) DNA sequences up to 13717), primary central nervous system lymphoproliferative disorder (PCNS-PTL) was considered, and ganciclovir antiviral and rituximab treatment was given. The child’s malaise, nausea and drowsiness improved significantly on +179d, but a sudden cerebral hemorrhage with brain herniation occurred on +186d, and he died in resuscitation. Conclusion The incidence of PCNS-PTLD is very low, and it is difficult to diagnose and treat. For patients with central nervous system symptoms after transplantation, early diagnosis and timely treatment are necessary.

Expression and significance of cadherin-11 in liver of children with biliary atresia

WU Bo, TIAN Xinbei, LU Ying, DU Jun, XIAO Yongtao

Journal of Clinical Pediatrics. 2022, 40(2): 134-138. doi:10.12372/jcp.2022.21e1058

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Objective To analyze the expression and significance of cadherin-11 (CDH11) in livers of children with biliary atresia (BA). Methods Fifty children with BA and eight children with choledochal cyst (CC) were selected as the research objects. The expression levels of CDH11 and liver fibrotic marker genes in the livers of the children were evaluated by quantitative real-time polymerase chain reaction. The correlation between CDH11 and liver fibrotic marker genes or serum liver function markers was analyzed. Results The mRNA levels of CDH11 were significantly elevated in livers of children with BA compared to children with CC (P< 0.01). Hepatic CDH11 mRNA levels were positively correlated with the expression levels of transforming growth factor beta 1, transforming growth factor beta 2, keratin 19, actin alpha 2, smooth muscle, collagen type I alpha 1 and collagen type IV alpha 1 in children with BA (r= 0.36 ~ 0.73, all P< 0.01). The mRNA levels of CDH11 in livers of BA patients with severe liver fibrosis significantly increased compared to BA patients with mild liver fibrosis (P< 0.01). Hepatic CDH11 mRNA levels were positively correlated with serum levels of γ-glutamyl transferase, total bilirubin, direct bilirubin and bile acid in children with BA (r= 0.26 ~ 0.37, all P< 0.05). Conclusions The mRNA levels of CDH11 were significantly increased in livers of BA patients compared to CC patients. The expression levels of CDH11 were associated with the progression of liver fibrosis and degree of liver injury in BA.

A retrospective clinical analysis of 21 cases of Castleman disease in children

LIU Zhichao, LI Changchun, WANG Shan, KONG Xiangru, ZHANG Jun, YANG Chao, ZHAO Zhenzhen

Journal of Clinical Pediatrics. 2022, 40(2): 139-143. doi:10.12372/jcp.2022.21e0963

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Objective To analyze clinical features of Castleman disease (CD) for improving clinicians' understanding of CD. Methods Data including clinical manifestation, diagnosis, therapy and prognosis of pediatric patients with CD diagnosed and treated in a single center in Children's Hospital of Chongqing Medical University from January, 2010 to June, 2020 were retrospectively analyzed. Results There are 21 children with CD (11 males and 10 females), the median age of patients was 10.90 (5.35, 12.1) years old. All patients have performed CT examinations. Among them, all unicentric CD (UCD) patients underwent complete surgical resection, and six patients of multicentric CD (MCD) were treated with chemotherapy, anti-IL-6 and hormones. The median follow-up time of 21cases is 37 months (27,71), 2 UCD patients died with paraneoplastic pemphigus and lung interstitial changes. Conclusion Castleman disease is rare in pediatric patients, and the overall prognosis is well if it was treated timely. For UCD patients, preferred surgical treatment for asymptomatic UCD children have high cure rate. Children with MCD treated with anti-IL-6, hormones, and chemotherapy have significantly improved long-term survival rates.

Clinical and endoscopic diagnosis and treatment of 3 cases of chronic pancreatitis

ZOU Biao, LIU Shengxuan, QIN Hua, HUANG Zhihua, SHU Sainan

Journal of Clinical Pediatrics. 2022, 40(2): 144-148. doi:10.12372/jcp.2022.21e0866

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Objective To evaluate the clinical characteristics of children with chronic pancreatitis caused by genetic and structural abnormalities and the clinical efficacy of endoscopic retrograde cholangiopancreatography (ERCP). Methods The clinical data of 3 children with chronic pancreatitis treated with ERCP from July to October in 2020 were retrospectively analyzed. Results There were 3 children (2 boys and 1 girl) with chronic pancreatitis. One patient was 11 years old and the other two were 12 years old. The main clinical symptoms were chronic abdominal pain without manifestation of pancreatic secretion insufficiency. Two patients were caused by gene variation, which were SPINK1 and LPL gene variation respectively. One patient was caused by structural abnormality, and the pancreatic duct was bifurcated in the body of the pancreas, which was considered as congenital abnormal development of the pancreatic duct. Three children were treated with ERCP after conventional treatment failed. By removing the stones, cleaning the pancreatic duct, placing stents, improving drainage and relieving the pressure of the pancreatic duct, the trypsin levels in 2 patients were restored to normal and the abdominal pain was relieved. The trypsin levels were increased in 1 patient after operation and returned to normal 3 days later. Conclusions ERCP is safe and effective in diagnosis and treatment of childhood chronic pancreatitis caused by genetic or structural abnormalities.

Research progress on diagnosis and treatment of congenital high airway obstruction syndrome

Reviewer: YAN Huiyuan, ZHOU Jianguo, Reviser: CHEN Chao

Journal of Clinical Pediatrics. 2022, 40(2): 149-154. doi:10.12372/jcp.2022.21e657

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Congenital high airway obstruction syndrome (CHAOS) is a rare syndrome caused by complete or nearly complete airway obstruction of the fetus, including any airway obstruction that interrupts the amniotic fluid circulation between the fetal lungs and the amniotic cavity. Its causes are various, most of them are congenital malformations, and airway obstruction can also be caused by external compression (such as primary cervical mass or double aortic arch). If prenatal disorders can be found in time and given corresponding treatments, it is of great help to reduce perinatal mortality. At present, most of the domestic studies on CHAOS are case reports and prenatal ultrasound screening, there are few studies related to gene defects, so it is particularly important to develop a more systematic diagnosis and treatment plan.

Diagnostic approach on childhood renal dysfunction

ZHANG Hongwen

Journal of Clinical Pediatrics. 2022, 40(2): 155-159. doi:10.12372/jcp.2022.21e1093

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Renal dysfunction refers to a variety of situations, including kidney diseases and other diseases, which cause the increase of creatinine and urea nitrogen, accompanied with edema, electrolyte disorder, hypertension and other multiple system related symptoms in some cases. A large proportion of patients were characterized by renal dysfunction as the primary symptom in children. The author summarized the diagnosis approach on childhood renal dysfunction, suggested that the stage on renal function should be based on the same classification standard as in chronic renal disease. Hereditary disease is the most common cause of renal dysfunction in children. Congenital anomalies of the kidney and urinary tract, which might be mainly results from developmental abnormalities, represent a broad spectrum of disorders.

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