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Childhood sleep:physical,cognitive,and behavioral consequences and implications

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World Journal of pediatrics 2024年第2期20卷 122-132页

作者： Jianghong Liu Xiaopeng Ji Susannah Pitt Guanghai Wang Elizabeth Rovit Terri Lipman Fan Jiang School of Nursing University of Pennsylvania418 Curie Blvd.Room 424Claire M.Fagin HallPhiladelphiaPA 19104USA College of Health Sciences University of DelawareNewarkDE 19716USA Geisinger Commonwealth School of Medicine ScrantonPA 18510USA pediatric Translational Medicine Institute Department of Developmental and Behavioral PediatricsSchool of MedicineShanghai Jiao Tong UniversityShanghaiChina

Background Sleep problems in children have been increasingly recognized as a major public health issue.Previous research has extensively studied and presented many risk factors and potential mechanisms for children’s sleep problems.In this paper,we aimed to identify and summarize the consequences and implications of child sleep problems.Data sources A comprehensive search for relevant English language full-text,peer-reviewed publications was performed focusing on pediatric sleep studies from prenatal to childhood and adolescence in a variety of indexes in PubMed,SCOPUS,and Psych Info published in the past two decades.Both relevant data-based articles and systematic reviews are included.Results Many adverse consequences are associated with child sleep deficiency and other sleep problems,including physical outcomes(e.g.,obesity),neurocognitive outcomes(e.g.,memory and attention,intelligence,academic performance),and emotional and behavioral outcomes(e.g.,internalizing/externalizing behaviors,behavioral disorders).Current prevention and intervention approaches to address childhood sleep problems include nutrition,exercise,cognitive–behavioral therapy for insomnia,aromatherapy,acupressure,and mindfulness.These interventions may be particularly important in the context of coronavirus disease 2019.Specific research and policy strategies can target the risk factors of child sleep as well as the efficacy and accessibility of treatments.Conclusions Given the increasing prevalence of child sleep problems,which have been shown to affect children’s physical and neurobehavioral wellbeing,understanding the multi-aspect consequences and intervention programs for childhood sleep is important to inform future research direction as well as a public health practice for sleep screening and intervention,thus improving sleep-related child development and health.

关键词： Behavior Child sleep Consequences Implications Neurocognitive Physical Prevention/intervention

Natural history and management of liver dysfunction in lysosomal storage disorders

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World Journal of Hepatology 2022年第10期14卷 1844-1861页

作者： Moinak Sen Sarma Parijat Ram Tripathi department of pediatric gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India department of pediatric gastroenterology Ankura Hospital for Women and ChildrenHyderabad 500072India

Lysosomal storage disorders(LSD)are a rare group of genetic disorders.The major LSDs that cause liver dysfunction are disorders of sphingolipid lipid storage[Gaucher disease(GD)and Niemann-Pick disease]and lysosomal acid lipase deficiency[cholesteryl ester storage disease and Wolman disease(WD)].These diseases can cause significant liver problems ranging from asymptomatic hepatomegaly to cirrhosis and portal hypertension.Abnormal storage cells initiate hepatic fibrosis in sphingolipid disorders.Dyslipidemia causes micronodular cirrhosis in lipid storage disorders.These disorders must be keenly differentiated from other chronic liver diseases and non-alcoholic steatohepatitis that affect children and young adults.GD,Niemann-Pick type C,and WD also cause neonatal cholestasis and infantile liver failure.Genotype and liver phenotype correlation is variable in these conditions.Patients with LSD may survive up to 4-5 decades except for those with neonatal onset disease.The diagnosis of all LSD is based on enzymatic activity,tissue histology,and genetic testing.Enzyme replacement is possible in GD and Niemann-Pick types A and B though there are major limitations in the outcome.Those that progress invariably require liver transplantation with variable outcomes.The prognosis of Niemann-Pick type C and WD is universally poor.Enzyme replacement therapy has a promising role in cholesteryl ester storage disease.This review attempts to outline the natural history of these disorders from a hepatologist’s perspective to increase awareness and facilitate better management of these rare disorders.

关键词： Lysosomal Gaucher Niemann-Pick Wolman Cholesteryl ester Children

Seronegative autoimmune hepatitis in childhood

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World Journal of Clinical pediatrics 2023年第3期12卷 77-85页

作者： Ali Islek Gokhan Tumgor department of pediatric gastroenterology Cukurova University School of Medicine

Comprehensive guidelines on seropositive autoimmune hepatitis have been published for both adults and children,although these guidelines comprise only limited knowledge about seronegative autoimmune hepatitis.Autoimmune hepatitis presents as an acute or chronic progressive disease and poor outcomes are inevitable if left untreated.The absence of autoantibody positivity,hypergammaglobulinemia and lack of comprehensive algorithms makes seronegative autoimmune hepatitis a mysterious disease.In general,seronegative autoimmune hepatitis often presents with acute hepatitis,and its treatment and prognosis similar to seropositive autoimmune hepatitis.The present review focuses on the known characteristics of seronegative autoimmune hepatitis in childhood,and those of which current knowledge is vague.

关键词： Seronegative autoimmune hepatitis Autoantibody negative Lymphocytopenia Aplastic anemia

Clinical significance of low transaminase levels in children with inflammatory bowel disease

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World Journal of pediatrics 2019年第2期15卷 143-147页

作者： Murat Cakir Elif Sag Guzide Dogan Fatih Unal Erhun Kasirga department of pediatric gastroenterology Hepatology and nutrition Faculty of MedicineKaradeniz Technical UniversityTrabzonTurkey department of pediatric gastroenterology Hepatology and nutrition Faculty of MedicineManisa Celal Bayar UniversityManisaTurkey department of pediatric gastroenterology Hepatology and nutrition BursaTurkey

Background Low alanine aminotransaminase (LALT) levels may be seen in patients with inflammatory bowel disease (IBD),but there has been no study about the frequency and its clinical significance.We aimed to analyze the frequency of LALT,and its clinical significance in children with IBD.Methods The study included the 89 patients with IBD without hepatobiliary involvement.LALT was defined as ALT levels < 5 U/L.Demographic and clinical findings and outcome of the patients with and without LALT were compared.Results LALT was found 47.1% of the patients.At initial examination,it was more common in female patients (92.3 vs.41.3%,P < 0.001) and patients with CD (57.7 vs.30.2%,P=0.01).75% of the patients with penetrating Crohn's disease (CD) had LALT (P=0.002).Hemoglobin (10.4±2.1 vs.11.7± 1.9 g/dL,P=0.01),folic acid (5.2±3.3 vs.8.6±5.9 ng/mL,P =0.02) and serum albumin levels (3.6 ± 0.8 vs.4.7 ± 5 g/dL,P =0.002) were significantly low in patients with LALT.LALT was associted with the disease relapse within 2 weeks in 12 of the 16 patients with LALT whereas it was seen in 16 of the 73 patients without LALT during the follow-up (75 vs.21.9%,P < 0.001).Additionally,steroid dependency was more common in patients with LALT during the follow-up (62.5 vs.12.3%,P<0.001).Conclusions LALT is common in children with IBD especially in CD and associated with low hemoglobin,albumin and folic acid levels.It may be a marker of relapse and steroid dependency.

关键词： Children Inflammatory bowel disease Low transaminase levels Vitamin B6

Gastric electrical stimulation: An emerging therapy for children with intractable gastroparesis

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World Journal of gastroenterology 2019年第48期25卷 6880-6889页

作者： Aniruddh Setya Priyanka Nair Sam Xianjun Cheng department of pediatric gastroenterology Hepatology and Nutrition University of Florida

Management of gastroparesis remains challenging,particularly in pediatric patients.Supportive care and pharmacological therapies for symptoms remain the mainstay treatment.Although they are effective for mild and some moderately severe cases,often time they do not work for severe gastroparesis.There are a few prokinetics available,yet the use of these drugs is limited by a lack of persistent efficacy and/or safety concerns.Currently,the only modality for adult patients with severe intractable gastroparesis is surgery,e.g.,pyloroplasty and partial gastrectomy,however,this option is generally considered too radical for a growing child.Novel therapeutic approaches,particularly those which are less invasive,are needed.This article explores gastric electrical stimulation(GES),a new therapy for gastroparesis.Unlike others,it neither needs medications nor gastrectomy;rather,it treats through the use of microelectrodes to deliver high-frequency low energy electric stimulation to the pacemaker area of the stomach.Thus,it is tolerated and safe in children.Like in adult patients,GES appears to work in releasing symptoms,improving nutrition,and enhancing the quality of life;it also helps wean off medications and eliminate many needs for hospitalization.Considering the transient nature of gastroparesis in children in many occasions,GES is considered a“bridging”therapy after failed medical interventions and before surgery.

关键词： Gastroparesis Gastric electrical stimulation Nausea Vomiting Prokinetics

Gaseous metabolites as therapeutic targets in ulcerative colitis

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World Journal of gastroenterology 2023年第4期29卷 682-691页

作者： Chu K Yao Chen Sarbagili-Shabat department of gastroenterology Monash UniversityMelbourne 3004Australia pediatric gastroenterology Unit PIBD Research CenterWolfson Medical CenterHolon 5822012Israel

Diet therapies are currently under-utilised in optimising clinical outcomes for patients with active ulcerative colitis(UC).Furthermore,existing dietary therapies are framed by poorly defined mechanistic targets to warrant its success.There is good evidence to suggest that microbial production of gaseous metabolites,hydrogen sulfide(H2S)and nitric oxide(NO)are implicated in the development of mucosal inflammation in UC.On a cellular level,exposure of the colonic epithelium to excessive concentrations of these gases are shown to promote functional defects described in UC.Hence,targeting bacterial production of these gases could provide an opportunity to formulate new dietary therapies in UC.Despite the paucity of evidence,there is epidemiological and clinical data to support the concept of reducing mucosal inflammation in UC via dietary strategies that reduce H2S.Several dietary components,namely sulphurcontaining amino acids and inorganic sulphur have been shown to be influential in enhancing colonic H2S production.More recent data suggests increasing the supply of readily fermentable fibre as an effective strategy for H2S reduction.Conversely,very little is known regarding how diet alters microbial production of NO.Hence,the current evidence suggest that a whole diet approach is needed.Finally,biomarkers for assessing changes in microbial gaseous metabolites in response to dietary interventions are very much required.In conclusion,this review identifies a great need for high quality randomised-controlled trials to demonstrate the efficacy of a sulphide-reducing dietary therapy for patients with active UC.

关键词： Diet Ulcerative colitis Hydrogen sulfide Nitric oxide Sulphide-reducing diet

Very early onset perinatal constipation:Can it be cow’s milk protein allergy?

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世界胃肠病学杂志:英文版 2023年第33期29卷 4920-4926页

作者： Rajalakshmy Arakoni Hebat Kamal Sam Xianjun Cheng department of pediatrics University of Florida Shands Children's HospitalGainesvilleFL 32608United States department of pediatric gastroenterology Hepatologyand NutritionUniversity of Florida Shands Children's HospitalGainesvilleFL 32608United States

Delayed passage of meconium or constipation during the perinatal period is traditionally regarded as a signal to initiate further work up to evaluate for serious diagnoses such as Hirschsprung’s disease(HD),meconium ileus due to Cystic Fibrosis,etc.The diagnosis of HD particularly warrants invasive testing to confirm the diagnosis,such as anorectal manometry or rectal suction biopsy.What if there was another etiology of perinatal constipation,that is far lesser known?Cow’s milk protein allergy(CMPA)is often diagnosed in infants within the first few weeks of life,however,there are studies that show that the CMPA allergen can be passed from mother to an infant in-utero,therefore allowing symptoms to show as early as day one of life.The presentation is more atypical,with perinatal constipation rather than with bloody stools,diarrhea,and vomiting.The diagnosis and management would be avoidance of cow's milk protein within the diet,with results and symptom improvement in patients immediately.Therefore,we discuss whether an alternative pathway to address perinatal constipation should be further discussed and implemented to potentially avoid invasive techniques in patients.This entails first ruling out CMPA with safe,noninvasive techniques with diet modification,and if unsuccessful,then moving forward with further diagnostic modalities.

关键词： Delayed passage of meconium Perinatal constipation Cow's milk protein allergy In-utero Alternate pathway Hirschsprung’s disease

Preliminary evidence in treatment of eosinophilic gastroenteritis in children:A case series

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World Journal of Clinical Cases 2022年第19期10卷 6417-6427页

作者： Ying Chen Mei Sun department of pediatric gastroenterology Shengjing Hospital of China Medical UniversityShenyang 110004Liaoning ProvinceChina

BACKGROUND Eosinophilic gastroenteritis is a rare inflammatory disorder in children.However,there is still no standard guideline in the treatment of pediatric eosinophilic gastroenteritis.AIM To report our experience with the diagnosis and treatment of children with eosinophilic gastroenteritis.METHODS From January 2017 to December 2019,a total of 22 children were diagnosed with eosinophilic gastroenteritis.RESULTS Endoscopic examination showed eosinophil infiltration in the duodenum[mean number of eosinophils/high-power field(HPF)=53.1±81.5],stomach(mean number of eosinophils/HPF=36.8±50.5),and terminal ileum(mean number of eosinophils/HPF=49.0±24.0).All 18 children with low eosinophil infiltration(14%)relapsed after initial methylprednisolone/montelukast treatment.In addition,children with high eosinophil infiltration(>14%)showed symptomatic relief and histological remission without further relapse after receiving budesonide/methylprednisolone as initial or relapse treatment.CONCLUSION Methylprednisolone/montelukast is still the best treatment for children with low eosinophil infiltration(14%).

关键词： Eosinophil gastroenteritis Children Budesonide Methylprednisolone Montelukast Absolute eosinophil count

Challenges and dilemmas in pediatric hepatic Wilson’s disease

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World Journal of Hepatology 2023年第10期15卷 1109-1126页

作者： Upasana Ghosh Moinak Sen Sarma Arghya Samanta department of pediatric gastroenterology Sanjay Gandhi Post graduate Institute of Medical SciencesLucknow 226014Uttar PradeshIndia department of pediatric gastroenterology Sanjay Gandhi Postgraduate Institute of Medical SciencesLucknow 226014India

Wilson disease is an autosomal recessive disorder affecting the ATP7B gene located on chromosome 13q.This leads to copper deposition in various organs,most importantly in the liver and brain.The genetic mutations are vast,well reported in the West but poorly documented in developing countries.Hence the diagnosis is made with a constellation of clinico-laboratory parameters which have significant overlap with other liver diseases and often pose a significant dilemma for clinicians.Diagnostic scoring systems are not fool-proof.The availability and affordability of chelators in developing countries impact the drug compliance of patients.While D-penicillamine is a potent drug,its side effects lead to drug discontinuation.Trientine is cost-prohibitive in developing countries.There is no single test to assess the adequacy of chelation.Exchangeable urinary copper is an essential upcoming diagnostic and prognostic tool.In the presence of cirrhosis,hypersplenism clouds the assessment of myelosuppression of drugs.Similarly,it may be difficult to distinguish disease tubulopathy from druginduced glomerulonephritis.Neurological worsening due to chelators may appear similar to disease progression.Presentation as fulminant hepatic failure requires rapid workup.There is a limited window of opportunity to salvage these patients with the help of plasmapheresis and other liver-assisted devices.This review addresses the challenges and clinical dilemmas faced at beside in developing countries.

关键词： Wilson’s disease Children Hepatic Wilson disease D-penicillamine Trientine Exchangeable copper