Journal of Clinical Pediatrics

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Journal of Clinical Pediatrics. 2021, 39(5): 320. doi:10.3969/j.issn.1000-3606.2021.05.000

Abstract ( 170 )

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Clinical significance of minimal residual disease in pediatric patients with precursor B-acute lymphoblastic leukemia

XUE Yujuan, LU Aidong, WANG Yu, et al

Journal of Clinical Pediatrics. 2021, 39(5): 321. doi:10.3969/j.issn.1000-3606.2021.05.001

Abstract ( 425 )

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Objective To explore the predictive value of minimal residual disease (MRD) level at different time-points of treatment in children with precursor B- acute lymphoblastic leukemia (B-ALL) in the context of MRD-guided therapy. Methods Data of newly diagnosed 417 patients with B-ALL from September 2014 to November 2017 were reviewed. We used multiparametric flow cytometry to monitor the MRD level on day 15, 33, 90 and 180, and analyzed the relationship between MRD levels and prognosis. Results The 417 patients included 240 males and 177 females with a median age of 5-years-old (3.0 year to 10.0 years). With a median follow-up of 44.0(33.7-56.2) months, the 3-y overall survival (OS) and event-free survival (EFS) were (90.9±1.4)% and (85.2±1.7)%, respectively. Patients who reached good MRD level on day 15 ((<10.0% or ≥10.0%), day 33 (<0.1% or ≥0.1%), day 90/180 (<0.01% or ≥0.01%) had a significantly higher probability of estimated OS and EFS (P<0.05). Patients who reached the MRD negative at all 3 time-point (day 33, 90, 180) had a significantly higher probability of estimated OS (95.8% vs. 82.8%) and EFS (92.3% vs. 72.6%) compared to patients with MRD failure at least in one time-point (P<0.05). Multivariable analysis showed MRD≥0.1% on day 33 and MRD≥0.01% on day 180 were independent risk factors for OS and EFS (P<0.05). Conclusion MRD levels on day 33 and 180 have important prognostic implications even in the context of MRD guided therapy. Sequential MRD monitoring is warranted in the treatment of children with B-ALL.

Nutritional status and in uencing factors of children with malignant solid tumor

YAN Mei, TANG Weibing, HUANG Jie, et al

Journal of Clinical Pediatrics. 2021, 39(5): 327. doi:10.3969/j.issn.1000-3606.2021.05.002

Abstract ( 281 )

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Objective This study aimed to investigate the nutritional status of children newly diagnosed with malignant solid tumor and the changes during therapy, and to identify factors associated with malnutrition during therapy. Method We enrolled 104 children with malignant solid tumor received inpatient treatment at the Department of Hematology and Oncology in Nanjing Medical University Affiliated Children’s Hospital between March 2018 and September 2019 . STRONGkids was used for nutritional risk screening. Dietary intake was analyzed within 24 h, and nutritional status were assessed according to WHO 2006 child growth standards. The influencing factors of malnutrition after 3 months of treatment were analyzed. Results At diagnosis, there were 61 males and 43 females, with a median age of 4 . 5 years old, 27 . 9 % of the patients were malnourished, BMI-Z decreased significantly after treatment, and the difference was statistically significant (P< 0 . 05 ). During anticancer therapy, the incidence of malnutrition rose to 48 % after 3 months. Logistic regression showed that the nutritional status at diagnosis and STRONGkids nutritional risk score were positively associated with a higher proportion of malnutrition (P< 0 . 05 ). Conclusion Children with solid tumor are prone to nutritional deterioration during treatment. For children with malnutrition and high nutritional risk score, reasonable nutritional support intervention should be given as soon as possible.

CT and MRI findings of solid pseudopapillary tumor of pancreas in children

CUI Zhimeng, REN Gang, CAI Rong, et al

Journal of Clinical Pediatrics. 2021, 39(5): 332. doi:10.3969/j.issn.1000-3606.2021.05.003

Abstract ( 449 )

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Objective To investigate the imaging signs of CT and MRI for pediatric solid pseudopapillary tumor (SPTP) of the pancreas. Methods This study retrospectively reviewed 21 cases of SPTP confirmed by clinic surgery and pathology. Results Among the 21 cases, there were two males and 19 females. The median age was 11 years. Preoperative CT scan was performed in 19 patients, and preoperative MRI scan was performed in 7 patients. All the 21 cases were of single lesion and often occurred in the body/tail of pancreas, with a mean diameter of 5.80(5.00~7.31) cm. All tumors were cystic and solid masses. Nine cases were mainly composed of solid tissue. The other two cases mainly had cystic components. Ten cases were composed of cystic and solid tissue, and the proportion of cystic components was close to solid components. On precontrast CT scan, SPTPs were typically well demarcated, and equal/low density lesions comprising both solid and cystic components. MRI showed that the solid components of the tumor showed low signal in T 1 WI and slightly higher signal in T 2 WI, hemorrhage was showed high signal in T 1 WI. The solid portion was mild to obviously enhanced slightly in the arterial phase, with an enhancement degree lower than normal pancreas, and progressive enhancement in the portal venous phase. In this group, five cases were confirmed to be malignant SPTP by surgery and pathology, with all the lengths greater than 5 cm and incomplete capsule, among which 3 cases were lobulated, and 2 cases had mass calcification. Conclusion The characteristic imaging signs of CT and MRI for pediatric solid SPTP have significant value in the diagnosis correctly and evaluation the malignant risk of tumor before surgery

Clinical analysis of two cases with β-thalassemia complicated with α-globin gene triplication

REN Zhenmin, HUANG Lilan, LIU Sixi, et al

Journal of Clinical Pediatrics. 2021, 39(5): 338. doi:10.3969/j.issn.1000-3606.2021.05.004

Abstract ( 4187 )

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Objective To investigate the clinical diagnosis of thalassemia intermediate caused by β-thalassemia with α-globin gene triplication. Methods The clinical manifestations of two β-thalassemia heterozygotes were retrospectively analyzed, and the results of β-globin gene sequencing and α-globin gene triplication detection in peripheral blood were also analyzed. Results Case one is a 4 years old girl, and the routine thalassemia gene detection revealed that she was a βCD 41 - 42 heterozygote. Case two was a 13 years old boy, he was a βCD 17 heterozygote detected by routine thalassemia gene sequencing. The clinical manifestations of both cases were moderate to severe anemia with hepatosplenomegaly. No rare mutation was found in β-globin by sequencing. Triplication αααanti4 . 2 fragment was found in case one by Gap-PCR using specific primers. The αααanti3 . 7 fragment was positive in case two by qPCR relative quantification. Conclusion When the result of routine detection indicated beta heterozygote, but with moderate to severe anemia, if there is no rare mutation found by sequencing, the existence of α-globin gene triplication should be considered.

Epstein-Barr virus related post-transplant lymphoproliferative disorder after hematopoietic stem cell transplantation in children with Wiskott-Aldrich syndrome: a case report and literature review

XI Bixin, CHEN Jing, LUO Chengjuan

Journal of Clinical Pediatrics. 2021, 39(5): 341. doi:10.3969/j.issn.1000-3606.2021.05.005

Abstract ( 331 )

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Objective To investigate the clinical progress of Epstein-Barr virus related post-transplant lymphoproliferative disorder (PTLD) after allogeneic hematopoietic stem cell transplantation (HSCT) in children with Wiskott-Aldrich syndrome (WAS). Methods Firstly, we retrospectively analyzed the clinical diagnosis and treatment data of one case of WAS after transplantation, and summarized relevant literature of WAS and PTLD. Then the risk factors, diagnosis and treatment progress of PTLD were discussed. Results A 12 -year-old boy with WAS underwent HLA 10 /10 allogeneic HSCT, and EBV activation at day + 33 after transplant was occurred, and then bilateral cervical lymph node enlargement appeared on day + 46 . The nucleic acid sorting results of T/B/NK virus in peripheral blood was as follows: EBV-B DNA 1 . 39 × 106 copies/mL, EBV-T DNA 1 . 35 × 105 copies/mL, EBV-NK DNA 1 . 71 × 102 copies/mL. PET-CT showed multiple lymph node enlargement in the right neck with increased FDG metabolism, which lead to consideration of lymphoma involvement. Neck lymph node biopsy results showed diffused large B-cell lymphoma, plasma blast cell phenotype, EBER (+), which confirmed the diagnosis of Epstein-Barr virus-associated PTLD. The child was given comprehensive treatments including antivirals, reduction of immunosuppression, rituximab, and low-dose chemotherapy. The enlarged lymph nodes disappeared, and the peripheral blood EBV DNA was negative at day + 69 . Conclusion For children with WAS from Epstein-Barr virus associated lymphocyte cloning PTLD after HSCT, when rituximab-refractoriness occurs, low-dose chemotherapy can help to achieve complete remission.

GATA1-positive non-Down syndrome acute megakaryoblastic leukemia: a report of two cases with literature review

ZHANG Feng, LU Aidong, ZUO Yingxi, et al

Journal of Clinical Pediatrics. 2021, 39(5): 346. doi:10.3969/j.issn.1000-3606.2021.05.006

Abstract ( 366 )

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Objective To investigate clinical characteristics of GATA 1 -positive non-Down syndrome (DS) acute megakaryoblastic leukemia (AMKL). Methods Clinical data of two children with AMKL were retrospectively analyzed, and related literatures were reviewed. Results Case one was a 2 -year-old girl diagnosed as AMKL by bone marrow morphology, immunotyping, molecular and genetic analysis, with no special facial features or developmental delay. Bone marrow chromosome karyotype at the beginning showed 50, XX, +8, +10, +21, +21[7]/46, XX[13]. Gene test identified a mutation of c.52dupT in GATA1 gene. After induction chemotherapy, peripheral blood chromosome showed 46 , XX. Now she has a disease-free survival. Case two was a 1 -year-old boy diagnosed as acute AMKL without Down's special features. The karyotype of bone marrow at the beginning was 47 , XY, + 21 [ 10 ]/ 46 , XY[10 ]. Gene test identified a mutation in GATA1 , and chromosome reexamination after bone marrow remission chemotherapy showed 46 , XY. Continuous bone marrow remission was observed at a 30 -month follow-up. Conclusion These cases emphasize that the importance of GATA1 testing for the non-DS AMKL.

Effects of umbilical vein catheterization on intestinal lora of very low birth weight infants

SUN Binjie, WANG Jun, YANG Qianqian, et al

Journal of Clinical Pediatrics. 2021, 39(5): 350. doi:10.3969/j.issn.1000-3606.2021.05.007

Abstract ( 251 )

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Objective To study the effect of umbilical vein catheterization on intestinal flora of very low birth weight infants. Methods A total of 41very low birth weight infants hospitalized in NICU from June 2019 to June2020 were enrolled. According to the umbilical vein catheterization status, they were divided into umbilical vein catheterization group (n=24) and nonumbilical vein catheterization group (n=17). Fecal samples were collected on day 1, 3 and 7 after birth. In order to study the effect of umbilical vein catheterization on intestinal flora, the diversity and relative abundance of intestinal flora between two groups at different times, Illumina high-throughput sequencing technology was used to sequence 16S gene. Results The detection rate of intestinal flora at the first day was low, so we did not compare the difference between the two groups. At the third day, there was no significant difference between the two groups in the Shannon index and relative abundance of all bacteria (P>0.05). At the seventh day, the Shannon index in umbilical vein catheterization group was significantly lower than that of non-umbilical vein catheterization group (P

Effect of feeding intolerance on the short-term outcome of premature infants

HU Xiaoyan, CHANG Yanmei, LI Zailing

Journal of Clinical Pediatrics. 2021, 39(5): 355. doi:10.3969/j.issn.1000-3606.2021.05.008

Abstract ( 330 )

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Objective To investigate the effect of feeding intolerance (FI) on the short-term outcome of premature infants. Methods This is a retrospective study involving premature infants who were hospitalized from January to December, 2017 . According to the occurrence of FI, they were divided into the FI group and feeding tolerance group (FT group). The infants were followed up to 6 months of corrected age. Medical records and follow-up data were reviewed to investigate the effect of FI on the short-term outcome of premature infants. Results There were 612 eligible subjects with 182 ( 29 . 7 %) in the FI group and 430 ( 70 . 3 %) in the FT group. A total of 126 follow-up cases with 63 cases in each group were included for analysis. Multivariate logistic regression analysis showed that FI was an independent influencing factor for anemia (OR= 2 . 131 , 95 %CI: 1 . 293 - 3 . 514 , P= 0 . 003 ), electrolyte disorder (OR=1.750, 95%CI: 1 . 105 - 2 . 771 , P= 0 . 017 ) and cholestasis (OR= 2 . 143 , 95 %CI: 1 . 211 - 3 . 795 , P= 0 . 009 ) in premature infants one week after birth. Multiple linear regression analysis showed that FI was an independent influencing factor for the prolongation of hospitalization (β=5 .884 , P< 0 . 001 ), the delay of reaching total enteral nutrition age (β=7.339 , P< 0 . 001 ), the delay of reaching oral feeding age (β=7.339 , P< 0 . 001 ) and the increase of body weight at discharge (β= 100 . 237 , P= 0 . 001 ). There was no significant difference in length, weight, head circumference and Peabody motor development score between the two groups at 6 months of corrected age. Conclusions FI increases the incidence of anemia, electrolyte disorder and cholestasis in premature infants during hospitalization. FI leads to prolonged hospitalization time of premature infants, and it delays the infants to reach the age of total enteral nutrition and the age of oral feeding. Premature infants with FI need to grow to a larger weight to meet the discharge standard. FI had no significant effect on physical growth and motor development of premature infants at 6 months of corrected age.

Crohn's disease complicated with Takayasu arteritis: a case report and literature review

MENG Yingying, WANG Yuhuan, TANG Zifei, et al

Journal of Clinical Pediatrics. 2021, 39(5): 360. doi:10.3969/j.issn.1000-3606.2021.05.009

Abstract ( 363 )

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Objective To explore the understanding of Crohn's disease (CD) with Takayasu arteritis (TA) in children. Method The clinical data of CD with TA in a child were retrospectively analyzed, and the related literature was reviewed. Results A 10 -year-old girl was diagnosed with CD by colonoscopy and received infliximab (IFX) at follow-up. Three years later, the patient developed dizziness and syncope during the clinical remission phase, and TA was diagnosed by vascular ultrasonography and CT angiography. After stopping IFX treatment and starting glucocorticoid and mycophenolate mofetil, both CD and TA were relieved. Through literature review, it is found that CD combined with TA is more common in young women, and CD is more common in the first episode. The clinical manifestations of CD combined with TA are various, and the most common were nonspecific systemic symptoms and gastrointestinal symptoms. The clinical manifestations of TA in children are more atypical, and it is easy to be missed or misdiagnosed when combined with CD. The detection of blood pressure, pulse and vascular murmur is helpful to the diagnosis of TA. Once TA is suspected, vascular imaging should be performed as soon as possible. Most of the patients with CD and TA received combined therapy of mesalazine, glucocorticoid and immunosuppressive agents, some of them received biological agents, and more than 1 / 3 of them received surgical treatment. Conclusion CD combined with TA is rare. When there are differences in the inflammatory indexes and the clinical treatment effect among CD patients, it should be alerted whether they are complicated with other diseases.

Neck myoclonia with absence seizures: a report of 2 cases and literature review

KUANG Xiaojun, ZHANG Xiao, NING Zeshu, et al

Journal of Clinical Pediatrics. 2021, 39(5): 366. doi:10.3969/j.issn.1000-3606.2021.05.010

Abstract ( 275 )

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Objective To explore the clinical features of neck myoclonia with absence seizures in children. Method The clinical and electroencephalogram (EEG) features of neck myoclonia with absence seizures in 2 children were analyzed retrospectively, and the related literature was reviewed. Results Both cases were boys and the age at onset was 7 years and 9 months, 9 years and 10 months respectively. There was no abnormal birth history or growth history, and no family history of genetic diseases or epilepsy. Cranial imaging showed no abnormality. The video-EEG (VEEG) showed no abnormality in the background rhythm, and the anterior head or generalized spinous and slow spinous waves were recorded in the sleep period between attacks. All the episodes were characterized by rhythmic head shaking movement with lateral deflection of the head and neck, accompanied by disturbance of consciousness, lasting about 6 - 13 seconds. During the seizure, the EEG showed a spinous slow-wave rhythmic burst with wide and extremely high amplitudes (about 3 Hz). One side of sternocleidomastoid muscle had about 50 ms of rhythmic myoelectric bursts synchronized with spinous waves, accompanied by tonic potentials. Hyperventilation could induce attack without light sensitivity. There was no abnormality in gene detection. The seizures of the both children were controlled by sodium valproate. Literature search found 4 children reported abroad with neck myoclonus with absence seizures confirmed by VEEG. The seizure was controlled after 4 months to 3 years of treatment with one or two antiepileptic drugs. Conclusion Neck myoclonus with absence seizures is a type of generalized epilepsy with independent EEG clinical characteristics.

Clinical features and gene analysis of childhood purine nucleoside phosphorylase deficiency: a case report and literature review

ZHONG Zhijuan, JI Xunqi, CHEN Yuwen, et al

Journal of Clinical Pediatrics. 2021, 39(5): 370. doi:10.3969/j.issn.1000-3606.2021.05.011

Abstract ( 615 )

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Objective To explore the clinical characteristics and immunophenotype of childhood purine nucleoside phosphorylase deficiency (PNPD) induced by PNP gene mutation. Methods clinical data from a case with PNPD in Hainan People’s Hospital was retrospectively analyzed, and related literature was reviewed. Results A 21 -month-old boy was admitted to hospital because of “pallor for more than 2 months, fever and cough for one day”. He had suffered from recurrent respiratory infection and motor retardation. Laboratory examination showed that hemoglobin level was 88 g/L, reticulocyte hemoglobin percentage was 11 . 35 %, Coomb’s test was positive, CD 4+ /CD 8+ T cell ratio ( 0 . 32 ) was low, and blood uric acid was low. Chest CT indicated thymic dysplasia. Gene sequencing analysis identified a homozygous mutations of c. 722 T>c (p.I 241 T) in PNP gene which inherited from his parents. He was diagnosed with PNPD and died of a severe multi-organ infection at the age of 2 years and 3 months old. Literature search found 78 children with PNPD, prevalence had no significant difference between males and females. Of them, 53 had repeated infections, 51 had abnormal nerve function, 21 had autoimmune disease, and 6 had secondary neoplastic diseases. Conclusion Children with recurrent infection, neurological dysfunction, autoimmune diseases, decreased CD 4+ /CD 8+ T cell ratio, and low uric acid level should be considered the possibility of immunodeficiency caused by PNP gene deficiency. Gene sequence analysis could assist early diagnosis

Ayme-Gripp syndrome: a case report and literature review

WANG Jiandong, FANG Xiao, JIN Peina, et al

Journal of Clinical Pediatrics. 2021, 39(5): 373. doi:10.3969/j.issn.1000-3606.2021.05.012

Abstract ( 562 )

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Objective To summarize the clinical characteristics and genetic change in Ayme-Gripp syndrome (AYGRPS). Methods The clinical data of a child with AYGRPS were retrospectively analyzed and related literatures were reviewed. Results A 3 years and 3 months old female patient presented recurrent seizure since five months old. Her head circumference was 45.5 cm ( C (p.E 156 D) heterozygous missense mutation in MAF gene. Combined with the phenotype and variation, this child was diagnosed as AYGPRS caused by MAF gene mutation. A total of 21 cases of AYGRPS were reviewed in the literature, and all the 21 patients carried missense mutations in MAF gene. The AYGRPS is a congenital disease with multiple system involvement, characterized by congenital cataracts, sensorineural hearing loss, special facial features, skeletal abnormalities and neurodevelopmental abnormalities. More than half patients may have epilepsy. Conclusion Epilepsy is one of the common clinical features of AYGRPS, and a novel mutation in MAF was discovered.

Analysis on clinical and genetic characteristics of childhood lissencephaly-pachygyria

ZHOU Yunqing, WANG Cuijin, WANG Yingyan, et al

Journal of Clinical Pediatrics. 2021, 39(5): 377. doi:10.3969/j.issn.1000-3606.2021.05.013

Abstract ( 799 )

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Objective To analyze the clinical and genetic characteristics of lissencephaly-pachygyria in children, and to perform genotype-phenotype analysis. Methods The clinical data of 60 children diagnosed with lissencephaly-pachygyria from January 2014 to March 2020 were collected, and next generation sequencing analysis was performed in 45 children and their parents. Results The onset age of 60 cases ( 30 males and 30 females) ranged from 2 days to 14 years old. Among 60 cases, 45 cases ( 75 %) were pachygyria, 4 cases ( 6 . 7 %) were lissencephaly, and 11 cases (18 . 3 %) were pachygyria with lissencephaly. Ten patients ( 16 . 7 %) had motor or language retardation as the onset symptom, and 48 patients ( 80 %) had epilepsy. Among them, 31 cases had spasm. During the follow-up period from 1 . 5 months to 5 years, among 50 patients with seizure, two cases of febrile seizure were seizure-free without treatment, 12 cases were seizure-free by treatment with antiepileptic drugs and 4 cases were seizure-free after epilepsy operation; 31 cases still had recurrent seizures; one case died of severe pneumonia. Among the 60 children, 59 had different degrees of psychomotor retardation, and 1 child (with onset age of 14 years) had normal development with focal pachygyria. Four mutations in PAFAH 1 B 1 /LIS 1 gene and one mutation in TUBA 1 A gene were found in 45 pedigrees. All the mutations were de novo and classified as pathogenic according to the American College of Medical Genetics and Genomics classification. Conclusions Most lissencephaly-pachygyria patients had refractory epilepsy, developmental retardation and poor prognosis. The first-line medication of epileptic spasm and epilepsy surgery may be beneficial to some patients. PAFAH 1 B 1 /LIS 1 gene and TUBA 1 A gene mutations were found in a few patients.

Case report of a child with Dravet syndrome caused by a de novo heterozygous mutation in HCN1 gene mutation

YU Xiaohua, TIAN Maoqiang, LI Juan,et al

Journal of Clinical Pediatrics. 2021, 39(5): 382. doi:10.3969/j.issn.1000-3606.2021.05.014

Abstract ( 467 )

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Objective To explore the clinical manifestations and HCN 1 gene mutation in Dravet syndrome. Methods The clinical data and gene test results of Dravet syndrome in a child were retrospectively analyzed. Results The proband was a one year and 11 months old female who had recurrent febrile seizure onset from 4 months old, she presented with status epilepticus and various seizure types. Gene sequencing identified a de novo heterozygous mutation of c.1199 T>C (p.L400 P) in the HCN 1 gene. Conclusion The novel mutation of HCN 1 gene was classified as pathogenic, which enriched the mutation spectrum of Dravet syndrome.

Analysis on the clinical manifestations and gene mutations from 3 cases with neuronal ceroid lipofuscinosis

WANG Yao, ZHUO Zhihong, KONG Huimin, et al

Journal of Clinical Pediatrics. 2021, 39(5): 386. doi:10.3969/j.issn.1000-3606.2021.05.015

Abstract ( 466 )

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Objective To explore the clinical and genetic characteristics of children with neuronal ceroid lipofuscinosis (NCL). Methods Clinical data of three patients diagnosed with NCL was retrospectively analyzed. Genomic DNA from peripheral blood samples from 3 families of the neuronal ceroid lipofuscinosis were extracted, and whole exome sequencing (WES) was used to identify mutations. Results The affected members of the three families presented with cognitive and motor regression, seizures of various degrees and visual impairment. Compound heterozygous mutations of c. 124 + 1 G>A inherited from her father and c. 413 C>T inherited from her mother in PPT 1 gene were found by the WES in case one. In case two, a compound heterozygous mutation of c. 181 C>T and c. 536 + 1 G>A in PPT 1 gene were found in her and her brother, in which c.181C>T mutation was inherited from her father and c. 536 + 1 G>A mutation was inherited from her mother. Compound heterozygous mutations of c. 768 G>T inherited from her father and c. 209 G>T inherited from her mother in CLN8 gene were found in patient 3 . c.768 G>T and c.209 G>T mutation were new mutation site not reported before. Conclusion Patients with PPT 1 gene mutation may present with different clinical manifestation, even if in the individual with same mutation from the same family; the findings enriched the pathogenic mutation spectrum of CLN 8.

Research progress on enterovirus 71 vaccines

BI Fangchuan

Journal of Clinical Pediatrics. 2021, 39(5): 391. doi:10.3969/j.issn.1000-3606.2021.05.016

Abstract ( 378 )

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Hand, foot and mouth disease (HFMD) is a common infectious disease in infants and young children. Enterovirus 71 (EV-A 71 ) is one of the main pathogens of HFMD, which could cause serious complications and even death. Therefore, it is an urgent need to protect against EV-A 71 infection. Development of EV-A 71 vaccine would be the most effective approach to prevent and control EV-A 71 infection. The EV-A 71 vaccines under development mainly include inactivated vaccines, attenuated live vaccines, virus-like particle vaccines, recombinant epitope vaccines, and synthetic peptide vaccines. This article summarizes the progress and development of the EV-A 71 vaccine.

Recommendation for diagnosis and treatment of Epstein-Barr virus related post-transplant lymphoproliferative disorder following hematopoietic stem cell transplant in children

XI Bixin, HU Qun

Journal of Clinical Pediatrics. 2021, 39(5): 396. doi:10.3969/j.issn.1000-3606.2021.05.017

Abstract ( 342 )

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The Epstein-Barr virus related post-transplant lymphoproliferative disorder (EBV-PTLD) is one of the deadliest complications after hematopoietic stem cell transplantation in children. Multiple risk factors have been associated with the onset of EBV-PTLD such as reduced intensity conditioning (RIC), use of antithymocyte globulin (ATG), graft-versus-host disease (GVHD), cytomegalovirus reactivation, etc. There is no clear consensus on the treatment of EBV-PTLD in children transplant recipients due to few clinical trials and the rarity of the disease. This article aims to explore the entity of EBV-PTLD in childhood hematopoietic stem cell transplantation recipients, expanding on pathogenesis, risk factors, clinical diagnosis and recent treatment strategy, as such to highlight the gaps in knowledge needed for building a perfect treatment paradigm suitable for all pediatric patients.

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