Expert Consensus on Nutritional Support for Children with Congenital Heart Disease(2023 Edition)

The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the results of multi-center clinical nutrition research for congenital heart disease following thefirst Chinese consensus edition of 2016.The consensus was also shaped by the results of three discussion sessions and two questionnaires con-ducted by the 13-member collaboration group.This process was informed by both clinical guidelines and expert consensus.The quality of literature,both in English and Chinese,and the level of recommendations were evaluated using the Grading of Recommendations Assessment,Development,and Evaluations(GRADE)system.

Special electromyographic features in a child with paramyotonia congenita: A case report and review of literature

BACKGROUND Paramyotonia congenita(PMC)stands as a rare sodium channelopaty of skeletal muscle,initially identified by Eulenburg.The identification of PMC often relies on electromyography(EMG),a diagnostic technique.The child’s needle EMG unveiled trains of myotonic discharges with notably giant amplitudes,alongside irregular wave trains of myotonic discharges.This distinctive observation had not surfaced in earlier studies.CASE SUMMARY We report the case of a 3-year-old female child with PMC,who exhibited la-ryngeal stridor,muffled speech,myotonia from birth.Cold,exposure to cool water,crying,and physical activity exacerbated the myotonia,which was relieved in warmth,yet never normalized.Percussion myotonia was observable in bilateral biceps.Myotonia symptoms remained unchanged after potassium-rich food consumption like bananas.Hyperkalemic periodic paralysis was excluded.Cranial magnetic resonance imaging yielded normal results.Blood potassium remained within normal range,while creatine kinase showed slight elevation.Exome-wide genetic testing pinpointed a heterozygous mutation on chromosome SCN4A:c.3917G>A(p.G1306E).After a six-month mexiletine regimen,symptoms alleviated.CONCLUSION In this case revealed the two types of myotonic discharges,and had not been documented in other studies.We underscore two distinctive features:Giant-amplitude potentials and irregular waves.

Comparison of ethanol-soaked gelatin sponge and microspheres for hepatic arterioportal fistulas embolization in hepatic cellular carcinoma

Hepatic arterioportal fistulas(APFs)are common in hepatocellular carcinoma(HCC).Moreover,correlated with poor prognosis,APFs often complicate antitumor treatments,including transarterial chemoembolization(TACE).AIM To compare the efficacy of ethanol-soaked gelatin sponges(ESG)and microspheres in the management of APFs and their impact on the prognosis of HCC.METHODS Data from patients diagnosed with HCC or hepatic APFs between June 2016 and December 2019 were retrospectively analyzed.Furthermore,APFs were embolized with ESG(group E)or microspheres(group M)during TACE.The primary outcomes were disease control rate(DCR)and objective response rate(ORR).The secondary outcomes included immediate and first follow-up APF improvement,overall survival(OS),and progression-free survival(PFS).RESULTS Altogether,91 participants were enrolled in the study,comprising 46 in group E and 45 in group M.The DCR was 93.5%and 91.1%in groups E and M,respectively(P=0.714).The ORRs were 91.3%and 66.7%in groups E and M,respectively(P=0.004).The APFs improved immediately after the procedure in 43(93.5%)patients in group E and 40(88.9%)patients in group M(P=0.485).After 2 mo,APF improvement was achieved in 37(80.4%)and 33(73.3%)participants in groups E and M,respectively(P=0.421).The OS was 26.2±1.4 and 20.6±1.1 mo in groups E and M,respectively(P=0.004),whereas the PFS was 16.6±1.0 and 13.8±0.7 mo in groups E and M,respectively(P=0.012).CONCLUSION Compared with microspheres,ESG embolization demonstrated a higher ORR and longer OS and PFS in patients of HCC with hepatic APFs.

Hydrogen inhalation promotes recovery of a patient in persistent vegetative state from intracerebral hemorrhage:A case report and literature review

BACKGROUND Persistent vegetative state(PVS)is a devastating and long-lasting clinical condition with high morbidity and mortality;currently,there are no available effective interventions.CASE SUMMARY We report the case of an 11-year-old boy with PVS caused by severe intracerebral bleeding in the left hemisphere following anticoagulation treatment.The patient’s PVS severity showed no notable improvement after 2-mo neuroprotective treatment and rehabilitation,including nerve growth factor and baclofen,hyperbaric oxygen,and comprehensive bedside rehabilitation therapies.Daily inhalation treatment(4-6 h)of high-concentration hydrogen(H2)gas(66.6%H2+33.3%O2)was provided.Surprisingly,the patient’s orientation,consciousness,ability to speak,facial expressions,and locomotor function were significantly restored,along with improvements in essential general health status,after H2 gas inhalation treatment,which was consistent with stabilized neuropathology in the left hemisphere and increased Hounsfield unit values of computed tomography in the right hemisphere.The patient finally recovered to a near normal conscious state with a Coma Recovery Scale-Revised Score of 22 from his previous score of 3.CONCLUSION Phase 1 clinical trials are needed to explore the safety and efficacy of H2 gas inhalation in patients with PVS.

Application effect of safety hazard self-examination mode in nursing risk management in hepatobiliary surgery

Objective:To explore the application effect of safety hazard self-examination mode in nursing risk management in hepatobiliary surgery.Methods:Sixty patients underwent hepatobiliary surgery in two tertiary hospitals in Shandong Province from May 2021 to October 2021.According to the different implementation time,they were divided into 30 cases in the observation group and 30 cases in the control group.The control group adopts routine risk assessment,and the observation group adopts the self-examination mode of potential safety hazards on the basis of routine risk assessment to compare the incidence of nursing risk between the two groups.Results:The observation group had a significantly lower incidence of safety hazards in terms of missing surgical instruments,lack of aseptic operation,and postoperative pressure injuries than the control group(P<0.05).Conclusion:The appropriate hazard self-examination mode in the hepatobiliary operation room is of positive significance to improve the safety awareness of operating staff and the comprehensive ability of operating room nurses.

Surgical first aid and nursing care of a parturient with massive hemorrhage in natural delivery

Objective:To summarize the first aid and nursing of the operating room due to serious complications caused by postpartum massive bleeding.Methods:One case of emergency hemorrhage was ineffective in emergency hysterectomy.Results:The uterus was successfully removed and the bleeding was successfully stopped.It was transferred to the ICU under general anesthesia.Conclusion:For pregnant women with postpartum massive bleeding and hemorrhagic shock and diffuse intravascular coagulation(DIC),targeted surgical treatment and complete operating room emergency care are of great significance to save maternal lives.

Safety and efficacy of Yupingfeng granules in children with recurrent respiratory tract infection:A randomized clinical trial

Importance:Recurrent respiratory tract infection(RRTI)is common in children.Inappropriate RRTI treatment will lead to asthma and other diseases,thereby seriously affecting the growth and physical health of children.Immune function modulation can prevent and alleviate childhood RRTI.Yupingfeng(YPF),a patented traditional Chinese medicine(TCM),has immunomodulatory effects and is widely used in China to treat children with RRTI.Objective:To evaluate the safety and efficacy of YPF monotherapy in treating children with RRTI.Methods:This multicenter,randomized,double-blind,double-simulation,noninferiority clinical trial was conducted from January 2015 to August 2017,with an 8-week treatment period and 52-week follow-up after the drug withdrawal.Children aged 2–6 years with RRTI meeting the inclusion and exclusion criteria were enrolled in 13 hospitals in China and divided randomly into three groups(2:2:1 ratio)to receive YPF,pidotimod,or placebo.The primary outcome was the proportion of RRTI returning to normal standard level during the follow-up.The secondary outcomes were reduction in the number of RRTI recurrences,effect on clinical symptoms(in accord with TCM practice),effect per symptom,and safety.The trial was registered at the Chinese Clinical Trials Registry(www.chictr.org.cn)under the unique identifier ChiCTR-IPR-15006847.Results:Three hundred and fifty-one children were enrolled and randomly assigned to 3 groups;124,125,and 61 children in the YPF,pidotimod,and placebo groups,respectively,had completed the trial.During the follow-up,the proportion of RRTI returning to normal standard level was 73.13%,67.15%,and 38.81%with YPF,pidotimod,and placebo,respectively(P<0.0001).The proportion of cases who returned to normal standard level in the YPF group was 34.32%higher than that in the placebo group.The safety profile did not significantly differ among the groups.Interpretation:YPF granules were noninferior to the active control drug pidotimod oral solution for the treatment of RRTI in children,and were superior to placebo,with a high safety profile.

Leucine-rich glioma-inactivated protein 1 antibody-mediated autoimmune encephalitis in a 4-year-old girl:a case report

Background:Leucine-rich glioma-inactivated protein 1(LGI1)antibody-mediated encephalitis is a rare subtype of autoimmune encephalopathy,which is associated with autoimmunity against the neuronal plasma membrane proteins.The characteristic symptoms of this disease are memory dysfunction,seizures,faciobrachial dystonic seizures,cognitive deficits,neuropsychiatric disturbances,and intractable hyponatremia.The diagnosis of this disease mainly depends on the presence of anti-LGI1 antibody in serum or cerebrospinal fluid of patients.LGI1 antibody encephalitis has been reported mostly in adults,with rare occurrences in children.Case presentation:In this report,we described a 4-year-old girl with typical seizures.Seizure types included focal seizures and generalized tonic-clonic seizures.The electroencephalogram findings showed focal discharges.Brain magnetic resonance imaging(MRI)showed normal.The cerebrospinal fluid(CSF)levels of cells,glucose,and chloride were within the normal range,and the culture did not reveal growth of any pathogen.Test of serum LGI1-Ab was positive,while the tests for autoimmune encephalitis antibody series in CSF were negative.The seizures of the patient were completely controlled after the therapy of immunoglobulin,methylprednisolone and antiepileptic drugs(AEDs),and the mental state almost returned to normal.Conclusion:To our knowledge,the patient described here may be the youngest case of LGI1 antibody encephalitis reported to date.Children with the LGI1 antibody-associated encephalitis may present only with single symptoms such as epileptic seizures and have good response to the therapy of immunoglobulin,methylprednisolone and antiepileptic drugs.Our case report will provide hints for pediatricians in the diagnosis and treatment of LGI1-antibody encephalitis.

Correction to:Leucine-rich gliomainactivated protein 1 antibody-mediated autoimmune encephalitis in a 4-year-old girl:a case report

Correction to:Acta Epileptologica 3,4(2021)https://doi.org/10.1186/s42494-021-00039-z After publication of this article[1],it is reported the below corrections need to be made with it.1.The following sentence need to be added to the end of‘Background’section:“This study was approved by the Institutional Ethics Committee of Qilu Children’s Hospital of Shandong University and informed consent has been obtained from the guardian of patient prior to analysis.”

Genetic Architecture of Childhood Kidney and Urological Diseases in China

Kidney disease is manifested in a wide variety of phenotypes,many of which have an important hereditary component.To delineate the genotypic and phenotypic spectrum of pediatric nephropathy,a multicenter registration system is being imple-mented based on the Chinese Children Genetic Kidney Disease Database(CCGKDD).In this study,all the patients with kidney and urological diseases were recruited from 2014 to 2020.Genetic analysis was conducted using exome sequencing for families with multiple affected individuals with nephropathy or clinical suspicion of a genetic kidney disease owing to early-onset or extrarenal features.The genetic diagnosis was confirmed in 883 of 2256(39.1%)patients from 23 provinces in China.Phenotypic profiles showed that the primary diagnosis included steroid-resistant nephrotic syndrome(SRNS,23.5%),glomerulonephritis(GN,32.2%),congenital anomalies of the kidney and urinary tract(CAKUT,21.2%),cystic renal disease(3.9%),renal calcinosis/stone(3.6%),tubulopathy(9.7%),and chronic kidney disease of unknown etiology(CKDu,5.8%).The pathogenic variants of 105 monogenetic disorders were identified.Ten distinct genomic disorders were identified as pathogenic copy number variants(CNVs)in 11 patients.The diagnostic yield differed by subgroups,and was highest in those with cystic renal disease(66.3%),followed by tubulopathy(58.4%),GN(57.7%),CKDu(43.5%),SRNS(29.2%),renal calcinosis/stone(29.3%)and CAKUT(8.6%).Reverse phenotyping permitted correct identification in 40 cases with clinical reassessment and unexpected genetic conditions.We present the results of the largest cohort of children with kidney disease in China where diagnostic exome sequencing was performed.Our data demonstrate the utility of family-based exome sequencing,and indicate that the combined analysis of genotype and phenotype based on the national patient registry is pivotal to the genetic diagnosis of kidney disease.