Journal of Clinical Pediatrics

Interpretation of “2023 Children’s Renal Nutrition Working Group Clinical Practice Recommendations: Nutritional Management of Children with Acute Kidney Injury”

DING Yaping, XIA Shanshan, ZHANG Chenmei

Journal of Clinical Pediatrics. 2024, 42(8): 667-672. doi:10.12372/jcp.2024.23e0820

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Acute kidney injury is one of the most common multiple organ dysfunctions in critically ill children, which can cause protein-energy wasting, exacerbate renal impairment, and lead to extremely high mortality. Given the complexity of the nutritional and metabolic mechanisms of AKI and the lack of relevant basic research, and how to meet the nutritional needs of AKI substitutes faces more unique challenges. The Pediatric Kidney Injury Nutrition Working Group formulated the clinical practice recommendations of “Nutritional Management of Children with Acute Kidney Injury”. The purpose of this article is to interpret the recommendation, with a view to providing reference for the construction of a systematic nutritional management program for children with AKI in the in the domestic field of pediatrics.

Comparison of different doses of cytarabine for induction chemotherapy in children with acute myeloid leukemia in FLAG-IDA regimen

LI Yirong, LI Huiping, GAO Jingyu, XIAO Yuhua, CHEN Xiaomin, LU Yanling, ZHAO Nana, FENG Xiaoqin

Journal of Clinical Pediatrics. 2024, 42(8): 673-677. doi:10.12372/jcp.2024.23e0900

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Objective To explore the efficacy and safety of different doses of cytarabine (Ara-C) in FLAG-IDA regimen as induction therapy in children with acute myeloid leukemia (AML). Methods 121 children who newly diagnosed AML from January 2015 to October 2022 were enrolled in this study. Participants were divided into two groups according to the dose of cytarabine used in the FLAG-IDA induction chemotherapy regimen: the standard-dose (SD) group and the high-dose (HD) group. Therapeutic efficacy and toxicity and side effects of the two groups after the induction chemotherapy were compared. Result A total of 121 children with AML were included, 71 males and 50 females, with a median age at first diagnosis of 6.7 (2.6~10.9) years. There were 30 cases in the SD group and 91 cases in the HD group. Compared with the SD group, the CR rate of induction chemotherapy in the HD group was lower, the duration of neutropenia was shorter, and the levels of AST and ALT after chemotherapy were higher, and the difference was statistically significant (P<0.05). There was no statistically significant difference in toxic and side effects between the two groups (P>0.05). The median follow-up time was 37.0 (12.0~46.0) months. The 3-year OS and EFS of the SD group were (91.1±6.0) % and (79.5±8.3) % respectively, and the 3-year OS and EFS of the HD group were (64.5±11.5) %and (57.5±10.7) % respectively. There was no statistically significant difference in 3-year OS and EFS between the two groups (P>0.05). Conclusion Standard-dose cytarabine can obtain better efficacy and less side effects in induction chemotherapy.

Clinical characteristics of 348 children with infectious mononucleosis

HUANG Bo, DONG Yanying, SONG Linlan

Journal of Clinical Pediatrics. 2024, 42(8): 678-683. doi:10.12372/jcp.2024.23e0776

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Objective To analyze the clinical characteristics and laboratory findings of 348 children with infectious mononucleosis (IM). Methods Clinical features, complications and laboratory findings of children hospitalized with IM from January 2019 to February 2022 were included. The clinical characteristics, complications and laboratory indicators were retrospectively analyzed. Results A total of 348 children with IM were included, 185 male (53.2 %) and 163 female (46.8 %), aged 1-15 years old, mainly 4-6 years old (preschool group) (49.1 %) ; The season of onset was mainly in summer, with a high incidence in June-August. The main clinical symptoms were fever in 289 cases (83.0 %), eyelid edema in 192 cases (55.2 %), nasal obstruction 182 cases (52.3 %). Compared with the ≤3 years old and 4-6 years old groups, the >6 years old group had a lower proportion of fever and a higher proportion of pharyngitis (P<0.05). The incidence of angina and lymphadenopathy in school-age group was significantly higher than ≤3 years old group (infant group) (P<0.05), and the incidence of hepatosplenomegaly in preschool group was significantly higher than the other two groups (P<0.05). The symptom of nasal obstruction in infant group was significantly higher than other two groups (P<0.05). The differences in lymphocyte counts, CD4⁺/CD3⁺, CD8⁺/CD3⁺, CD4⁺/CD8⁺, glutamate aminotransferase, glutamyl transpeptidase, and lactate dehydrogenase between the ≤3, 4-6, and >6 year old groups were statistically significant (P<0.05). Complications in children with IM were predominantly liver injury in 133 cases (38.2 %), followed by neutropenia (53 cases, 15.22 %), and sepsis (27 cases, 7.75 %). Compared to the non-hepatic injury group, the hepatic injury group had a lower proportion of males, older age, higher lymphocyte counts, and lower neutrophil-lymphocyte ratios (NLR) and monocyte-lymphocyte ratios (MLR), with a statistically significant difference (P<0.05). Conclusion IM in children has a high incidence, predominantly in preschool children, with variable and atypical early clinical manifestations and liver injury as the most common complication. Gender, age, lymphocyte count, NLR and MLR may be associated with liver injury.

Clinical analysis of 38 cases of hematological malignancies complicated with tumor lysis syndrome in children

WANG Dan, SHAO Jingbo, LI Hong, ZHANG Na, ZHU Jiashi, FU Pan, WANG Zhen

Journal of Clinical Pediatrics. 2024, 42(8): 684-690. doi:10.12372/jcp.2024.23e0868

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Objective To study the clinical features and prognosis of tumor lysis syndrome (TLS) in children and provide clinical guidance for the prevention and treatment of TLS. Methods The clinical data of patients with TLS from December 2014 to March 2023 were retrospectively analyzed for their clinical features and prognosis. Results A total of 38 children with TLS were included, 27 males and 11 females, with a median age of 6.6 (2.9-9.9) years.. Among these children, 18 were diagnosed with acute lymphoblastic leukemia, 8 with acute myeloid leukemia, and 12 with Burkitt lymphoma, of which 12 cases (31.6 %) were combined with renal infiltration. 37 cases (97.4 %) developed TLS from 24 hours before chemotherapy to 72 hours after the start of chemotherapy, 21 cases were consistent with clinical TLS, and 17 cases with laboratory TLS. The main manifestations of TLS were acute kidney injury (AKI, 20 cases), nausea and vomiting (18 cases), fever (18 cases), chest tightness, and hypoxemia (12 cases). 21 children were transferred to the PICU for treatment of disease exacerbation, and a total of 3 TLS-related deaths occurred. Uric acid was lowered by allopurinol in 16 cases and by recombinant uric acid oxidase in 22 cases. Compared with the allopurinol group, the recombinant uric acid oxidase group had a higher proportion of laboratory TLS and a lower incidence of AKI and severe transfer to the PICU, with statistically significant differences (P<0.05). The LDH and uric acid levels in the allopurinol group were higher at the five time points of -3 d before the onset of TLS, the same day of TLS, and +1 d, +3 d, and +5 d after TLS, and the levels in the allopurinol group were higher at the five time points of -1 d, +1 d, +3 d, and +5 d after TLS. The difference between the LDH, uric acid and blood creatinine levels in the recombinant uric acid oxidase group was statistically significant (P<0.05). The uric acid levels in the recombinant uric acid oxidase group were lower than those in the allopurinol group at +1 d, +3 d and +5 d after the onset of TLS, and the difference was statistically significant (P<0.05). Conclusion Patients with high-load hematological tumors have a high risk of TLS at the early stage of treatment, and uric acid oxidase can effectively lower uric acid, reduce the occurrence of AKI, lower the rate of severe disease, and shorten the duration of TLS.

Genetic analysis of 11 patients with congenital adrenal cortical abnormalities in a single center

WU Qin, PAN Hairui, MA Panpan, WANG Yupei, ZHOU Bingbo, ZHENG Lei, TIAN Xinyuan, HUI Ling, HAO Shengju, SUN Bo, ZHANG Chuan, GUO Jinxian

Journal of Clinical Pediatrics. 2024, 42(8): 691-696. doi:10.12372/jcp.2024.23e0821

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Objective To investigate the genetic characteristics of 11 families with congenital adrenal cortex abnormalities. Methods From January 2019 to June 2023, 11 families of patients with congenital adrenal cortex abnormalities diagnosed in the Medical Genetic Center of Gansu Maternity and Child Health Hospital were enrolled. Exome sequencing was used to detect genetic variants in the proband, Sanger sequencing and MLPA were used for verify the variants and their family origin. Results 11 patient families were genetically diagnosed: 8 cases were congenital adrenal hyperplasia due to 21-hydroxylase deficiency caused by CYP21A2 variants, and 1 case was congenital adrenal hyperplasia due to 17-α hydroxylase deficiency caused by CYP17A1 variation Cortical hyperplasia, one case of lipocongenital adrenal hyperplasia caused by STAR variants, and one case of congenital adrenal hypoplasia caused by NR0B1 variants. A total of 7 different variants were detected in the CYP21A2 gene. Among the 7 variants, the site with the highest frequency was c.518T>A, followed by c.293-13C>G and c.1069C>T. The c.780dupG and c.397C>T variants of STAR are novel variants that have not been reported. According to the ACMG Genetic Variation Classification Standards and Guidelines, the c.780dupG was categorized as pathogenic (PVS1+PM2_Supporting+PP4), c.397C>T was categorized as uncertain significance (PM2_Supporting+PM3+PP3+PP4). The variant c.64_c.65insGAGCGCGAAGC of NR0B1 is a novel variant that has not been reported, which is categorized as Likely pathogenic (PVS1+PM2_Supporting+PP4). Conclusion Patients with adrenal cortex anomalies with overlapping clinical phenotypes cannot be reliably identified by symptoms and biochemical markers alone, and early precise genetic diagnosis is essential for diagnosis of the disease, interventional therapy, genetic counseling, and fertility guidance.

Analysis of etiological composition and clinical features of stage 5 chronic kidney disease in children in a tertiary hospital in western China

MA Yan, WEI Xingjiao, BAI Hua, ZHANG Yan, TIAN Xinmin, Aqsa Ahmad, LIANG Lijun

Journal of Clinical Pediatrics. 2024, 42(8): 697-703. doi:10.12372/jcp.2024.23e0655

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Objective To summarize the etiology and clinical features of 50 children with stage 5 chronic kidney disease (CKD) treated at a tertiary hospital in western China in the past 10 years and to establish a theoretical basis for the investigation of chronic kidney disease in children. Methods The clinical data of 50 children aged 0-18 years with CKD5 who visited the General Hospital of Ningxia Medical University from January 1, 2010 to June 30, 2021 were retrospectively collected, and their etiological composition and clinical features were analyzed. Results Among the 50 children with CKD stage 5, 31 (62.0 %) were male and 19 (38.0 %) were female. The age of onset was 0.5-18 years old, including 8 cases (16.0 %) aged 0-6 years, 17 cases (34.0 %) 7-12 years old, and 25 cases (50.0 %) aged 13-18 years. At the initial diagnosis, there were 9 cases (18.0 %) with no obvious symptoms, 15 cases (30.0 %) with primarily renal symptoms, and 26 cases (52.0 %) with mainly extrarenal symptoms. The primary cause of the disease was glomerular disease in 26 cases (52%) and congenital renal and urinary tract malformations in 12 cases (24.0 %). Complications observed were anemia in 49 cases (98.0 %), chronic kidney disease-mineral and bone abnormalities (CKD-MBD) included hyperphosphatemia in 49 cases (98.0 %), secondary hyperparathyroidism in 38 cases (76.0 %), hypocalcemia in 33 cases (66.0 %), vitamin D deficiency in 27 cases (54.0 %), water electrolyte acid-base disorders including hyperuricemia in 40 cases (80.0 %), hyperkalemia in 15 cases (30.0 %), hypertension in 28 cases (56.0 %), growth disorder in 18 cases (36.0 %), and refinement of nephropuncture biopsy in 7 cases (14.0 %), exoms sequencing in 5 cases (10.0 %). loss of follow-up in 2 cases (4.0 %), death in 11 cases (22.0 %), long-term hemodialysis in 15 cases (30.0 %), long-term peritoneal dialysis in 13 cases (26.0 %), and kidney transplantation in 11 cases (22.0 %). Conclusions The main cause of children with CKD stage 5 in this region is glomerular disease; the incidence rate is higher in males than females, and higher in older than younger children; the first symptom is more common with extra-renal manifestations, and the most common complications are anemia and CKD-MBD; the prognosis of children who have completed renal transplantation is better, but the vast majority of them opt for dialysis treatment, which is predominantly hemodialysis; and the main causes of death are severe infections and diseases of cardiovascular system.

Clinical analysis of six cases of Langerhans cell histiocytosis with the first manifestation of sclerosing cholangitis

WANG Xuelian, FANG Chunxiao, CHEN Minxia, YANG Huamei, SHE Lanhui, GONG Yu, XU Yi, AI Bin, HUANG Li, LI Xufang

Journal of Clinical Pediatrics. 2024, 42(8): 704-708. doi:10.12372/jcp.2024.23e0831

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Objective To explore the clinical features of Langerhans cell histiocytosis (LCH) with sclerosing cholangitis (SC) as the first manifestation, aiming to enhance the understanding of LCH in children with liver involvement. Methods Cases in the electronic medical record system of Guangzhou Women and Children’s Medical Center who were diagnosed with SC at the first visit and subsequently diagnosed with LCH were retrospectively analyzed. The clinical manifestations, lab examination, imaging, treatment process, and prognosis were summarized. Results There were six cases of LCH with SC as the first manifestation. The female-to-male ratio was 5:1, and the median age was 12 months (ranging from 12 to 22 months). All six patients present cholestasis as the first manifestation, which was characterized by abnormalities in liver function, mainly elevated γ-glutamyl transpeptidase and direct bilirubin levels. Their magnetic resonance cholangiopancreatography (MRCP) showed focal intrahepatic bile duct stenosis and dilatation, leading to the radiological diagnosis of SC. Five cases were diagnosed with LCH after immunohistochemical staining of the skin rash tissue, four of which presented with SC accompanied by skin rash; one case had SC alone, and the diagnosis was confirmed by a tissue biopsy of skin rash that developed over following two months. One presented with SC accompanied by diabetes insipidus caused by pituitary involvement, whose 2 Liver puncture biopsies only showed nodular cirrhosis, and LCH was confirmed by immunohistochemical staining of liver mass tissue after liver transplantation. Six cases received first-line chemotherapy protocol after LCH diagnosis. One showed systemic remission after liver transplantation with chemotherapy and symptomatic treatment. Four showed improvement in systemic condition but no significant improvement in the liver imaging examinations at the end of the follow-up. One died of liver failure. Conclusions SC is one of the advanced and severe form of LCH liver involvement, with or without other tissue and organ lesions. Diagnosis requires a combination of characteristic skin rash, osteolytic changes, and tissue involvement such as pituitary gland, as well as a pathological diagnosis.The prognosis of SC due to LCH is poor. SC in combination with cirrhosis, especially with portal hypertension, intractable itching of the skin, or growth retardation, may be considered for liver transplantation, and chemotherapy may be a therapeutic approach with a better prognosis after the operation.

Clinical analysis of 4 cases with primary intestinal lymphangiectasia in infants

ZHU Yan, SUN Yuguang, XIA Song, XIN Jianfeng, SHEN Wenbin

Journal of Clinical Pediatrics. 2024, 42(8): 709-713. doi:10.12372/jcp.2024.24e0080

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Objective To analyze the clinical characteristics and treatment methods of primary intestinal lymphangiectasia (PIL) in infants and to improve clinical diagnosis and treatment. Methods A retrospective analysis was conducted on the clinical manifestations, laboratory tests, nuclear imaging examinations and treatment methods of four cases of PIL in infants admitted to the Department of Lymphatic Surgery at Beijing Shijitan Hospital from October 2012 to December 2013. The diagnosis and treatment of infantile PIL were summarized based on long term follow-up results. Results The patients aged 4-9 months and are all female. All 4 patients presented with diarrhea as the initial symptom, accompanied by symmetrical edema of the lower limbs, and 3 cases were complicated with respiratory infections. Laboratory tests showed a decrease in absolute values of blood lymphocytes, albumin, and globulin. Four patients presented with mild anemia, hypocalcemia, and iron deficiency. Radionuclide imaging indicated loss of intestinal proteins. Four patients were treated with liver protection, protein supplementation, dieresis and total parenteral nutrition for 3-4 weeks. After discharge, personalized medium chain triglycerides (MCT) dietary treatment was administered for 3-30 months. Following up for 10 years, 4 patients resumed normal diet and had normal blood albumin levels rechecked, with no recurrence. Conclusions Infant PIL is relatively rare, with clinical manifestations mainly characterized by diarrhea and edema, it is often accompanied by respiratory infections. Electrolyte disorders, such as hypocalcemia and iron deficiency, are more common. In infant patients suspected of PIL, radioactive nuclide testing may serve as the first choice for definitive diagnosis A sufficient course of total parenteral nutrition in conjunction with personalized MCT diet is an effective treatment for PIL in infants.

Application of simplified lung ultrasound performed shortly after birth in the respiratory support needs of late preterm and term infants

ZHANG Ruijie, XI Guannan, WANG Xuefeng, LIN Xin’ao, DAI Jiale, FAN Xiaobo, WANG Jimei

Journal of Clinical Pediatrics. 2024, 42(8): 714-721. doi:10.12372/jcp.2024.23e0358

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Objective To investigate the application of a simplified 8-zone lung ultrasound examination method in predicting early postnatal respiratory support in late preterm and term neonates. Methods Late preterm and term infants who developed dyspnea within 4 hours after birth from June 1, 2019 to January 1, 2020 were included. The infants underwent lung ultrasound at any of the postnatal time points : 0.5 h, 1.0 h, 2.0 h, and 4.0 h. Among the lung ultrasound signs observed early postnatally, " isolated A-lines " and " scattered B-lines " were categorized as " low-risk " signs, while" coalescent B-lines " and " consolidation" were categorized as " high-risk " signs. The association between " high-risk " signs and lung disease was determined through logistic regression (odds ratio, OR). The accuracy of lung ultrasound and an existing symptom scoring tool (ACoRN score) in predicting respiratory support was compared using the receiver operating characteristic curve and area under the curve (AUC). The optimal sensitivity and specificity were calculated using the Youden index. Results A total of 310 newborns were included. Follow-up until 3 days postnatally found that 74 infants required respiratory support, while 236 did not. The AUC for " high-risk " signs predicting respiratory support in preterm infants was 0.93 (95 % CI: 0.85 - 1.00) with a cut-off value of 3; for full-term infants, it was 0.88 (95 % CI: 0.82 - 0.94) with a cut-off value of 2. Using clinical symptoms and signs (ACoRN score) for prediction, the AUC was 0.69 (95 % CI: 0.51 - 0.87) for preterm infants and 0.74 (95 % CI: 0.67 - 0.81) for full-term infants, significantly lower than the " high-risk " signs (P=0.006). Conclusion The simplified lung ultrasound method can be used to predict respiratory support in infants with early postnatal respiratory distress.

A case of clinical report of T-lymphoblastic lymphoma secondary to acute promyelocytic leukemia in children

WANG Ye, ZHANG Linlin, CHI Zuofei, SUN Ruowen, JIANG Zehui, XU Gang

Journal of Clinical Pediatrics. 2024, 42(8): 722-727. doi:10.12372/jcp.2024.23e1211

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Objective To summarize the clinical process of patients with non-Hodgkin 's lymphoma (NHL) secondary to acute leukemia after treatment, and to explore the mechanism of NHL secondary to acute leukemia after treatment. Methods The clinical data of child with T-lymphoblastic lymphoma (T-LBL) secondary to acute promyelocytic leukemia (APL) was retrospectively analyzed, and the literature reports of NHL secondary to acute leukemia after treatment were summarized. Results The patient was a 10-year-old boy who presented with "intermittent fever" and was diagnosed with APL. After treatment, bone marrow relapsed and a complete remission was achieved after adjusting the treatment regimen, however, T-LBL was diagnosed due to enlarged lymph nodes at the end of the leukemia treatment, and remission was achieved again after standardized chemotherapy. Searching the literature over the last 10 years, a total of 9 cases of non-Hodgkin's lymphoma secondary to acute leukemia treatment have been reported, all in adults, and 6 of these patients were disease-free survival up to the time of reporting. Conclusion The incidence of secondary NHL after acute leukemia treatment is low and the prognosis is favorable. In addition, for patients after chemotherapy for neoplastic diseases, it is crucial to monitor the incidence of secondary tumors and employ advanced diagnostic techniques to enhance understanding of the pathophysiology underlying these secondary malignancies.

Treatment and reflection of infective endocarditis caused by methicillin-resistant Staphylococcus aureus in children after complex congenital heart disease

HUANG Shiyu, WANG Wei, ZHU Diqi, SHEN Jie, CAO Qing

Journal of Clinical Pediatrics. 2024, 42(8): 728-736. doi:10.12372/jcp.2024.23e1225

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The patient, male, 6 years and 10 months old, was admitted to our hospital because of “repeated fever for 2 months and mental fatigue for half a day”. The patient had congenital heart disease in the past, and fever occurred after modified body to pulmonary circulation shunt. Multiple blood cultures indicated methicillin-resistant Staphylococcus aureus (MRSA), and echocardiography indicated that the tube was not smooth. The patient was diagnosed with infective endocarditis, and vancomycin, linezolid, meropenem and other anti-infective drugs were given. After the use of drugs, bone marrow suppression is obvious, infection control is poor. Emergency surgery was performed to remove the redundant organisms and recurrent fever after central shunt surgery. The patient’s condition improved and the infection was stably controlled, after switched to contizolamide anti-infective treatment with contezolid. Follow-up six months, the child has no recurrent infections, there is no organ damage and bone marrow suppression phenomenon, the echocardiogram showed that the blood flow is smooth. This case is intended to provide clinical ideas for clinicians to deal with this kind of complex infection, and to discuss the choice of drugs for anti-infection treatment of drug-resistant bacteria in children.

Progress of Clinical Research on IgA Vasculitis in Children

CHAI Yahui, YANG Dasheng, ZHANG He, BI Lingyun

Journal of Clinical Pediatrics. 2024, 42(8): 737-740. doi:10.12372/jcp.2024.23e0251

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IgA vasculitis (IgAV) is an autoimmune disease caused by IgA deposition in small blood vessels. It is characterized by non-thrombocytopenic purpura, abdominal pain, joint swelling and pain, hematuria or proteinuria. The disease is self-limited, with only a minority progressing to end-stage renal disease in later life. This paper reviews the clinical features of IgAV in children and the mechanisms of renal involvement, and discusses the treatment and prognosis of the disease.

Research progress of gut microbiota in allergic diseases in children

DENG Menglu, ZHANG Jie, SHENG Wenbin

Journal of Clinical Pediatrics. 2024, 42(8): 741-746. doi:10.12372/jcp.2024.23e1054

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In recent years, allergic diseases continue to pose a major threat and challenge to the health of a wide range of children, thereby calling for urgent intervention to prevent the progression of the disease. The human gut microbiota is established by the age of 3 years. The imbalance in the gut microbiota occurs due to factors, such as cesarean delivery and antibiotic use before the age of 3 years, is strongly associated with a higher risk of future onset of allergic diseases. Recent advancements in next-generation sequencing methods have revealed the presence of dysbiosis in patients with allergic diseases, which increases attention on the relationship between dysbiosis and the development of allergic diseases. A large number of researchers have conducted in-depth research on the correlation between gut microbiota and allergic diseases, and the research results have revealed that the imbalance of gut microbiota is associated with the high risk of children developing allergic diseases in the future, and the changes in gut microbiota may be used as therapeutic targets for allergic diseases. Therefore, the purpose of this paper is mainly to review the latest research progress of gut microbiota in children with allergic diseases, and try to further promote the treatment and prevention of clinical diseases by comparing the gut microbiota of children with allergic diseases and normal children.

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