Neonatal magnesium sulphate for neuroprotection: A systematic review and meta-analysis.

AIM: To review the evidence of the effects of neonatal magnesium sulphate for neuroprotection in perinatal asphyxia and hypoxic-ischaemic encephalopathy (HIE). METHOD: This was a systematic review of randomized controlled trials (RCTs) (with meta-analysis) and non-RCTs assessing magnesium sulphate for treating perinatal asphyxia and HIE at 35 weeks or more gestation (primary outcomes: neonatal death and death or long-term major neurodevelopmental disability). RESULTS: Twenty-five RCTs (2099 infants) and four non-RCTs (871 infants) were included, 23 in low- and middle-income countries (LMICs). In RCTs, reductions in neonatal death with magnesium sulphate versus placebo or no treatment (risk ratio [RR] = 0.68; 95% confidence interval [CI] = 0.53-0.86; 13 RCTs), and magnesium sulphate with melatonin versus melatonin alone (RR = 0.74; 95% CI = 0.58-0.95; one RCT) were observed. No difference in neonatal death was seen for magnesium sulphate with therapeutic hypothermia versus therapeutic hypothermia alone (RR = 0.66, 95% CI = 0.34-1.26; three RCTs), or magnesium sulphate versus phenobarbital (RR = 3.00; 95% CI = 0.86-10.46; one RCT). No reduction in death or long-term neurodevelopmental disability (RR = 0.52; 95% CI = 0.14-1.89; one RCT) but reductions in several short-term adverse outcomes were observed with magnesium sulphate. Evidence was low- to very-low certainty because of risk of bias and imprecision. INTERPRETATION: Given the uncertainty of the current evidence, further robust neonatal magnesium sulphate research is justified. This may include high-quality studies to determine stand-alone effects in LMICs and effects with and after therapeutic hypothermia in high-income countries.

Peri-Transplant Inflammation and Long-Term Diabetes Outcomes Were Not Impacted by Either Etanercept or Alpha-1-Antitrypsin Treatment in Islet Autotransplant Recipients.

The instant blood-mediated inflammatory response (IBMIR) causes islet loss and compromises diabetes outcomes after total pancreatectomy with islet autotransplant (TPIAT). We previously reported a possible benefit of etanercept in maintaining insulin secretion 3 months post-TPIAT. Here, we report 2-year diabetes outcomes and peri-operative inflammatory profiles from a randomized trial of etanercept and alpha-1 antitrypsin (A1AT) in TPIAT. We randomized 43 TPIAT recipients to A1AT (90 mg/kg IV x6 doses, n = 13), etanercept (50 mg then 25 mg SQ x 5 doses, n = 14), or standard care (n = 16). Inflammatory cytokines, serum A1AT and unmethylated insulin DNA were drawn multiple times in the perioperative period. Islet function was assessed 2 years after TPIAT with mixed meal tolerance test, intravenous glucose tolerance test and glucose-potentiated arginine induced insulin secretion. Cytokines, especially IL-6, IL-8, IL-10, and MCP-1, were elevated during and after TPIAT. However, only TNFα differed significantly between groups, with highest levels in the etanercept group (p = 0.027). A1AT increased after IAT in all groups (p < 0.001), suggesting endogenous upregulation. Unmethylated insulin DNA ratios (a marker of islet loss) and 2 years islet function testing were similar in the three groups. To conclude, we found no sustained benefit from administering etanercept or A1AT in the perioperative period.

Clinical features associated with epilepsy occurrence, resolution, and drug resistance in children with cerebral palsy: A population-based study.

AIM: To investigate clinicoradiological features associated with epilepsy, its resolution, and drug resistance in children with cerebral palsy (CP). METHOD: Data were gathered from the New South Wales/Australian Capital Territory CP Register, encompassing children with CP born between 2003 and 2015 (n = 1916). Clinical features and the severity of impairments were compared among three groups: children with current epilepsy (n = 604), those with resolved epilepsy by age 5 years (n = 109), and those without epilepsy (n = 1203). Additionally, a subset of the registry cohort attending Children's Hospital Westmead (n = 256) was analysed to compare epilepsy and treatment characteristics between drug-responsive (n = 83) and drug-resistant groups (n = 147) using logistic regression and hierarchical cluster analysis. RESULTS: Manual Ability Classification System levels IV and V, intellectual impairment, and vision impairment were found to be associated with epilepsy in children with CP on multivariable analysis (p < 0.01). Moderate to severe intellectual impairment and bilateral spastic CP were independent positive and negative predictors of epilepsy persistence at the age of 5 years respectively (p < 0.05). Microcephaly and multiple seizure types were predictors of drug-resistant epilepsy (area under the receiver operating characteristic curve of 0.83; 95% confidence interval 0.77-0.9). Children with a known genetic cause (14%) and CP epilepsy surgery group (4.3%) formed specific clinical subgroups in CP epilepsy. INTERPRETATION: Our study highlights important clinical associations of epilepsy, its resolution, and treatment response in children with CP, providing valuable knowledge to aid in counselling families and identifying distinct prognostic groups for effective medical surveillance and optimal treatment.

The Sexual and Reproductive Health of Adolescents with Cerebral Palsy in Rural Bangladesh: A Qualitative Analysis.

Adolescents with disability in the Global South have unique sexual and reproductive health (SHR) experiences and needs; however, they are rarely included in SRH discourse. This qualitative study, conducted in rural Bangladesh, used semi-structured interviews to understand how adolescents with cerebral palsy (CP) experience their SRH. Participants were recruited from the Bangladesh Cerebral Palsy Register and included 24 adolescents with CP (n = 12 female; n = 12 male) and 76 parents (n = 56 mothers, n = 17 fathers, n = 3 other relatives). Data were analyzed using reflexive thematic analysis. Findings highlighted heterogeneity among adolescents with CP including differences for adolescent men versus women. For some adolescent men with CP, sexual maturity was viewed as bringing new opportunities, whereas for other men, adolescence affirmed exclusions and some transgressed sociocultural norms as they struggled to navigate their pubescent body alongside new privacy requirements. For adolescent women with CP, sexual maturity was associated with new domestic responsibilities, silence and secrecy regarding menstruation, and increased vulnerability to sexual violence and abuse. Adolescent men and women with CP spoke about marriage as something "everybody wants," however, was deemed "impossible" for those with more impairment-related support needs. Both adolescent men and women with CP lacked access to SRH information and support. Mothers positioned providing care to their adolescent child with CP after puberty as "shameful." Our findings suggest that disability, health, and education services in rural Bangladesh need to adopt a life-course approach that incorporates the SRH of adolescents with CP. We recommend the provision of SRH education that addresses the physical, cognitive, and social needs of adolescents with CP.

A randomized controlled pilot trial of etanercept and alpha-1 antitrypsin to improve autologous islet engraftment.

BACKGROUND: In total pancreatectomy with islet auto-transplantation, successful diabetes outcomes are limited by islet loss from the instant blood mediated inflammatory response. We hypothesized that blockade of the inflammatory response with either etanercept or alpha-1-antitrypsin would improve islet function and insulin independence. METHODS: We randomized 43 participants to receive A1AT (90 mg/kg x 6 doses, n = 13), or etanercept (50 mg then 25 mg x 5 doses, n = 14), or standard care (n = 16), aiming to reduce detrimental effects of innate inflammation on early islet survival. Islet graft function was assessed using mixed meal tolerance testing, intravenous glucose tolerance testing, glucose-potentiated arginine-induced insulin secretion studies, HbA1c, and insulin dose 3 months and 1 year post-TPIAT. RESULTS: We observed the most robust acute insulin response (AIRglu) and acute C-peptide response to glucose (ACRglu) at 3 months after TPIAT in the etanercept-treated group (p ≤ 0.02), but no differences in other efficacy measures. The groups did not differ overall at 1 year but when adjusted by sex, there was a trend towards a sex-specific treatment effect in females (AIRglu p = 0.05, ACRglu p = 0.06), with insulin secretion measures highest in A1AT-treated females. CONCLUSION: Our randomized trial supports a potential role for etanercept in optimizing early islet engraftment but it is unclear whether this benefit is sustained. Further studies are needed to evaluate possible sex-specific responses to either treatment. CLINICAL TRIAL NOTATION: This study was performed under an Investigational New Drug Application (IND #119828) from the Food and Drug Administration and was registered on clinicaltrials.gov (NCT#02713997).

Sib-pair subgroup familial type 1 diabetes mellitus in children in the state of Qatar.

BACKGROUND: Type 1 diabetes is the most common type of diabetes mellitus (DM) in children. It can be sporadic in onset or cluster in families, which comprises parent-offspring and sib-pair subgroups. The risk of developing DM in first-degree relatives of affected individuals is 8-15 fold higher. There is limited data about familial DM from the Gulf region. This study aims to describe the clinical, biochemical and genetic characteristics of sib-pair familial type 1 diabetes in Qatar. METHODS: Every child with DM following up at Sidra Medicine was recruited. Data was collected regarding clinical features, family history, type 1 diabetes autoantibodies and whole genome sequencing was performed. Genetic analysis for MODY genes and HLA association analysis was conducted. RESULTS: 44 families with sib-pair familial diabetes were identified. Of these, 2 families had 4 affected siblings and 5 families had 3 affected siblings. The majority are of Qatari ethnicity and the most common autoantibody was GAD65. The most common age of onset in the proband was 5-9 years while it was 10-14 years in subsequent siblings. The occurrence of DKA & HbA1c levels were lower in the second affected sibling. No relevant MODY gene variants were found. HLA analysis found 15 variants in at least 50% of the subjects. Most common were HLA-F*01*01*01G, HLA- DPA1*01*03*01G, HLA- DRB3*02*02*01G, HLA- E*01*01*01G & DRB4*03*01N. CONCLUSIONS: The prevalence of sib-pair diabetes is 3.64%. The second affected siblings were older. MODY is unlikely and Class I and II HLA genes was present in sib-pair diabetes.

Improving epilepsy control among children with cerebral palsy in rural Bangladesh: a prospective cohort-based study.

OBJECTIVE: To define the prevalence and seizure subtypes among children with cerebral palsy (CP) in rural Bangladesh and explore barriers to optimum epilepsy control. DESIGN: Prospective cohort study. SETTING: The study was conducted in Shahjadpur, a rural subdistrict of Bangladesh. PARTICIPANTS: Children (<18 years) with CP and epilepsy identified using the Bangladesh CP Register (BCPR) in the study site. METHODS: Assessments were conducted in three focused epilepsy clinics overseen by a paediatric neurologist between December 2016 and January 2018, with intervening phone and video-conference follow-ups. Details of event type, frequency and medication compliance were collected. Antiepileptic drugs (AEDs) were prescribed based on seizure type, family income, comorbidity and medication availability. RESULTS: 23.4% (170/726) of the BCPR cohort had a clinical diagnosis of epilepsy of whom 166 were assessed. Following the focused epilepsy clinics, 62.0% (103/166) children were clinically determined to have ongoing epileptic seizures. 62.1% (64/103) had generalised onset tonic clonic seizures, 27.2% (28/103) had focal onset seizures with impaired awareness and 10.7% (11/103) had other seizure types. None of the children with prolonged seizures (31/103) had an emergency seizure management plan. Non-epileptic events were being pharmacologically treated as seizures in 18.1% (30/166) children. Financial constraints were the main reason for non-compliance on follow-up. CONCLUSIONS: Gaps in optimum epilepsy management in rural Bangladesh are amenable to improvement anchored with local healthcare workers. Training and clinical care focused on recognition of common seizure types, seizure mimics and rationalising use of available AEDs can be facilitated by better referral pathways and telehealth support.

The epidemiology, clinical, biochemical, immunological and radiological features of youth onset type 2 diabetes mellitus in the state of Qatar.

Objectives: To describe the epidemiology, clinical, biochemical, immunological and radiological aspects of youth with type 2 diabetes. Methods: Patients under 18 year of age with type 2 diabetes were recruited from 2018 to 2020, clinical data collected, autoantibodies (GAD65, IAA, IA2 and ZnT8), insulin, ALT and c-peptide were measured. Hepatic ultrasound was performed for assessment of non-alcoholic fatty liver disease (NAFLD). Results: 104 patients were identified. The incidence in 2020 and prevalence per 100,000 was 2.51 and 23.7, respectively. The age of onset was between 8.5 and 18 years with 74% of the patients being of Qatari nationality. Males were more affected than females (1.5/1). Overweight/obesity was present in 98% of all the patients, a positive family history (either both parents or a single parent) in 71% and maternal gestational diabetes mellitus (GDM) in 60% of patients. More than 90% of the patients had acanthosis nigricans. 5 patients had 1 autoantibody positivity and hepatic ultrasound detected evidence of NAFLD in majority of patients. Conclusion: Obesity, maternal GDM and family history of diabetes were the key risk factors for the development of type 2 diabetes. Autoantibody positivity may be present in youth type 2 diabetes. As youth type 2 diabetes is associated with early onset microvascular and macrovascular complications, these findings have important social and health budget implications for Qatar. Tackling the burden of maternal GDM and childhood obesity and building programmes for early detection and intervention, are therefore, essential to reduce the risk of future complications.

Nutrition Interventions for Children with Cerebral Palsy in Low- and Middle-Income Countries: A Scoping Review.

BACKGROUND: Malnutrition is substantially higher among children with cerebral palsy (CP) in low- and middle-income countries (LMICs) when compared with the general population. Access to appropriate interventions is crucial for better management of malnutrition and nutritional outcomes of those children. We aimed to review the existing evidence on nutrition interventions for children with CP in LMICs. METHODS: Online databases, i.e., PubMed and Scopus, and Google Scholar were searched up to 10 January 2022, to identify peer-reviewed publications/evidence on LMIC focused nutritional management guidelines/interventions. Following title screening and abstract review, full articles that met the inclusion/exclusion criteria were retained for data charting. Information about the study characteristics, nutrition interventions, and their effectiveness were extracted. Descriptive data were reported. RESULTS: Eight articles published between 2008 and 2019 were included with data from a total of n = 252 children with CP (age range: 1 y 0 m-18 y 7 m, 42% female). Five studies followed experimental design; n = 6 were conducted in hospital/clinic/center-based settings. Four studies focused on parental/caregiver training; n = 2 studies had surgical interventions (i.e., gastrostomy) and n = 1 provided neurodevelopmental therapy feeding intervention. Dietary modification as an intervention (or component) was reported in n = 5 studies and had better effect on the nutritional outcomes of children with CP compared to interventions focused on feeding skills or other behavioral modifications. Surgical interventions improved nutritional outcomes in both studies; however, none documented any adverse consequences of the surgical interventions. CONCLUSION: There is a substantial knowledge gap on nutrition interventions for children with CP in LMICs. This hinders the development of best practice guidelines for the nutritional management of children with CP in those settings. Findings suggest interventions directly related to growth/feeding of children had a better outcome than behavioral interventions. This should be considered in planning of nutrition-focused intervention or comprehensive services for children with CP in LMICs.

Epidemiology of eye diseases among children with disability in rural Bangladesh: a population-based cohort study.

AIM: To describe the epidemiology of eye diseases among children with disability in rural Bangladesh. METHOD: We established a population-based cohort of children with disability using the key informant method. Children younger than 18 years with disability (i.e. physical, visual, hearing, speech, epilepsy) were included. We used detailed ophthalmological assessments following World Health Organization (WHO) protocols by a multidisciplinary team including an ophthalmologist, optometrist, physician, and physiotherapist. Visual impairment, blindness, and severe visual impairment (SVI) were defined by following WHO categories. RESULTS: Between October 2017 and February 2018, 1274 children were assessed (43.6% female; median [interquartile range] age 9y 10mo [6y -13y 7mo]). Overall, 6.5% (n=83) had blindness/SVI, and 5.6% (n=71) had visual impairment. In the group with blindness/SVI, 47% (n=39) had cortical blindness; of those, 79.5% (n=31) had cerebral palsy (CP). The other main anatomical sites of abnormalities in this group included lens (13.3%, n=11), cornea (10.8%, n=9), and optic nerve (9.6%, n=8). In the group with visual impairment, 90.1% (n=64) had refractive error. Overall, 83.1% (n=69) and 78.8% (n=56) of those with blindness/SVI and visual impairment had avoidable causes. Most children with blindness/SVI and visual impairment lacked access to education. INTERPRETATION: The burden of blindness/SVI/visual impairment is high among children with disability in rural Bangladesh, mostly due to avoidable causes. Overrepresentation of CP and cortical blindness in the group with blindness/SVI and refractive error in the group with visual impairment highlights the need for integration of ophthalmology assessment, eye care, and refraction services in comprehensive health care for children with disability including CP in rural Bangladesh.

Data on cerebral palsy in Vietnam will inform clinical practice and policy in low and middle-income countries.

PURPOSE: To document known risk factors, clinical severity, associated impairments and rehabilitation status of children presenting with cerebral palsy (CP) to the National Children's Hospital (NCH) in Hanoi, Vietnam. MATERIALS AND METHODS: Active prospective ascertainment of cases of CP presenting between June and November 2017 to the NCH using surveillance modelled on the Paediatric Active Enhanced Disease Surveillance system in Australia. RESULTS: Data were collected on 765 children with CP (mean age: 2 years 7 months (SD 2 y 6 mo). Mean age at diagnosis was 1 year 8 months (SD 1 y 9 mo). Children predominantly had spastic CP (95.2%, n = 729), most were quadriplegic (69.6%, n = 532) and 60.3% (n = 454) were Gross Motor Functional Classification System level III-V. Of the children 76.2% (n = 583) had one/more associated impairments. 36.3% (n = 276) had presumed perinatal asphyxia, 26.5% (n = 202) were preterm. Physiotherapy (94.3%, n = 663) was the most common form of intervention used. Only 2.6% (n = 12) of the children who would have benefitted from assistive devices had wheelchairs. CONCLUSION: We established hospital-based surveillance of CP in Hanoi and confirmed a high burden and severity of CP with potentially preventable risk factors. These data will inform clinician training and health policy and identify need for evidence-based care and assistive devices.IMPLICATIONS FOR REHABILITATIONWe identified a high number of children with severe forms of cerebral palsy (CP) in Hanoi, Vietnam through hospital-based surveillance.There is an urgent need for clinician training and access to and use of evidence-based interventions including assistive technology.This study will inform local capacity building and health policy for improved diagnosis and care of children with CP in Vietnam and other low and middle-income countries.

Clinical features, epidemiology, autoantibody status, HLA haplotypes and genetic mechanisms of type 1 diabetes mellitus among children in Qatar.

To describe the clinical features, epidemiology, autoantibody status, HLA haplotypes and genetic mechanisms of type 1 diabetes mellitus (T1DM). Patients (0-18 years) with diabetes were recruited. Clinical data was collected, autoantibodies and c-peptide were measured. Whole Genome Sequencing was performed. Genomic data analysis was compared with the known genes linked with T1DM and HLA alleles were studied. 1096 patients had one or more antibody positivity. The incidence of T1DM in 2020 was 38.05 per 100,000 children and prevalence was 249.73. GADA was the most common autoantibody followed by IAA. Variants in GSTCD, SKAP2, SLC9B1, BANK1 were most prevalent. An association of HLA haplotypes DQA1*03:01:01G (OR = 2.46, p value = 0.011) and DQB1*03:02:01G (OR = 2.43, p value = 0.022) was identified. The incidence of T1DM in Qatar is the fourth highest in the world, IA2 autoantibody was the most specific with some patients only having ZnT8 or IA2 autoantibodies thus underlining the necessity of profiling all 4 autoantibodies. The genes associated with T1DM in the Arab population were different from those that are common in the Caucasian population. HLA-DQ was enriched in the Qatari patients suggesting that it can be considered a major risk factor at an early age.

Outcome of Community-Based Early Intervention and Rehabilitation for Children with Cerebral Palsy in Rural Bangladesh: A Quasi-Experimental Study.

We evaluated the outcome of a community-based early intervention and habilitation for children with cerebral palsy (CP) in Bangladesh. Children registered on the Bangladesh CP Register (BCPR) were recruited in two groups for this study: Group A received a comprehensive six-month long community-based caregiver-led intervention program at the "Shishu Shorgo" (Bengali title, which translates to 'Children's Heaven') Early Intervention and Rehabilitation Centres developed to support participants from the BCPR. Group B received standard care. A quasi-experimental study was conducted. Data were obtained at baseline, at the end of the program (i.e., 6 months), and at a 12-month follow-up. Outcome measures for children included gross motor functional measure (GMFM-66), Communication Function Classification System (CFCS), and Viking Speech Scale (VSS) and, for adult caregivers, the depression, anxiety, and stress scale (DASS 21). Between October 2016 and March 2017, 156 children with CP were recruited (77 in Group A and 79 in Group B). The total score of GMFM-66, CFCS level, and VSS level significantly improved statistically in Group A (p < 0.05 for all) and deteriorated in Group B (p < 0.001, p = 0.095, p = 0.232). The intervention showed promising outcomes particularly for children with CP under five years of age. There is a need for caregiver-led community-based programs for children with CP in LMICs.

The Epidemiology and Genetic Analysis of Children With Idiopathic Type 1 Diabetes in the State of Qatar.

CONTEXT: Idiopathic type 1 diabetes is characterized by the absence of autoantibodies and the underlying mechanisms are not clear. OBJECTIVE: We aimed to study the epidemiology, describe the clinical characteristics, and report results of genetic studies in pediatric patients with idiopathic type 1 diabetes. METHODS: This was a prospective study of type 1 diabetes patients attending Sidra Medicine from 2018 to 2020. Autoantibodies (GAD65, IAA, IA-2A, and ZnT8) were measured and genetic testing was undertaken in patients negative for autoantibodies to rule out monogenic diabetes. Demographic and clinical data of patients with idiopathic type 1 diabetes were compared with patients with autoimmune type 1 diabetes. RESULTS: Of 1157 patients with type 1 diabetes, 63 were antibody-negative. Upon genome sequencing, 4 had maturity onset diabetes of the young (MODY), 2 had Wolfram syndrome, 1 had H syndrome, and 3 had variants of uncertain significance in MODY genes; 53 patients had idiopathic type 1 diabetes. The most common age of diagnosis was 10 to 14 years. C-peptide level was low but detectable in 30 patients (56.6%) and normal in 23 patients (43.4%) The average body mass index was in the normal range and 33% of the patients had a history of diabetic ketoacidosis (DKA). CONCLUSION: Four percent of the children had idiopathic type 1 diabetes. There were statistically significant differences in the C-peptide level and insulin requirement between the 2 groups. DKA was less common in the idiopathic group. Mutations in MODY genes suggest the importance of autoantibody testing and genetic screening for known causes of monogenic diabetes in idiopathic type 1 diabetes. The mechanism of idiopathic type 1 diabetes is unknown but could be due to defects in antibody production or due to autoantibodies that are not yet detectable or discovered.

The prevalence, immune profile, and clinical characteristics of children with celiac disease and type 1 diabetes mellitus in the state of Qatar.

OBJECTIVES: Children with antibody positive type 1 diabetes mellitus (type 1 diabetes) are at an increased risk of developing celiac disease (CD) which suggests a common autoimmune basis with both high-risk human lymphocyte antigen (HLA) and non-HLA factors playing a role in the pathophysiology. We aim to describe the prevalence, immune profile, and clinical characteristics of children with CD who have type 1 diabetes mellitus in Qatar. METHODS: All children (aged 0-18 years) attending a regional diabetes clinic with antibody positive type 1 diabetes were screened for CD. Measurement of tissue transglutaminase IgA and IgG as well as anti-endomysial antibody, was done, clinical details about the birth history, family history of diabetes and CD, age of onset, and ethnicity were collected. RESULTS: Out of the 1,325 children with antibody positive type 1 diabetes, 54 were identified to have CD on screening and then confirmed on small bowel biopsy. The prevalence of CD in the type 1 diabetes childhood population in Qatar is 4.07%. CD and type 1 diabetes were more prevalent in the Qatari children (n=32) as compared to non-Qatari (n=22) and occurred mostly in the age group 6-10 years. The most common type 1 diabetes antibodies in children with CD were glutamic acid decarboxylase and insulin autoantibody. Twelve subjects were asymptomatic for CD symptoms and picked up only on screening. CONCLUSIONS: The prevalence of CD in children with type 1 diabetes in Qatar is comparable to reports from around the world. Many children were asymptomatic and thus routine screening is recommended.

Depression, anxiety and stress among caregivers of adolescents with cerebral palsy in rural Bangladesh.

PURPOSE: Prior studies indicate high risk of mental health problems among caregivers of adolescents with cerebral palsy although limited consideration is given to caregivers in low- and middle-income countries. This study aimed to compare the burden of depression, anxiety and stress among caregivers of adolescents with cerebral palsy to caregivers of adolescents without disability in rural Bangladesh; and to identify factors unique to low- and middle-income countries that predict caregiver's mental health. METHODS: Observational study comparing caregivers of adolescents with cerebral palsy identified through the Bangladesh Cerebral Palsy Register and caregivers of adolescents without disability from neighboring dwellings. Caregiver mental health was assessed using the Depression, Anxiety and Stress Scale-21, adolescent mental health using the Strengths and Difficulties Questionnaire and adolescent health-related quality of life using Kidscreen-27. Hierarchical multivariable regression analysis was performed. RESULTS: Participants were 154 caregivers of adolescents with cerebral palsy and 173 caregivers of adolescents without disability, matched on adolescent age and sex. Caregivers of adolescents with cerebral palsy reported significantly higher risk of depression and stress than caregivers of adolescents without disability (Effect Size 0.1 to 0.2, p < 0.05) although no difference on anxiety. Caregiver age, adolescent mental health, household overcrowding and adolescent hearing impairment were significant predictors of depression, anxiety and/or stress (0.1 to 2.2, p < 0.05). CONCLUSIONS: Caregivers of adolescents with cerebral palsy in rural Bangladesh are at high risk of depression and stress. Initiatives to improve caregiver mental health are required; we recommend initiatives address adolescent mental health problems and include poverty reduction measures to improve social and economic capital. Improved understanding of the factors predicting caregiver depression, anxiety and stress unique to low and middle-income countries are necessary to guide policies and public health infrastructure development.Implications for rehabilitationCaregivers of adolescents with cerebral palsy in rural Bangladesh are at significantly higher risk of depression and stress than caregivers of adolescents without disability.We recommend interventions to improve caregiver mental health give specific consideration to older caregivers, those whose adolescent report mental health problems, families living in crowded households, and/or whose child has hearing impairment.We recommend interventions include poverty reduction measures to improve social and economic capital and target both caregivers and adolescents with CP to enhance long term outcomes.

Epidemiology, genetic landscape and classification of childhood diabetes mellitus in the State of Qatar.

AIMS/INTRODUCTION: To study the epidemiology, genetic landscape and causes of childhood diabetes mellitus in the State of Qatar. MATERIALS AND METHODS: All patients (aged 0-18 years) with diabetes mellitus underwent biochemical, immunological and genetic testing. American Diabetes Association guidelines were used to classify types of diabetes mellitus. The incidence and prevalence of all the different types of diabetes mellitus were calculated. RESULTS: Total number of children with diabetes mellitus was 1,325 (type 1 n = 1,096, ≥1 antibody; type 2 n = 104, type 1B n = 53; maturity onset diabetes of the young n = 20; monogenic autoimmune n = 4; neonatal diabetes mellitus n = 10;, syndromic diabetes mellitus n = 23; and double diabetes mellitus n = 15). The incidence and prevalence of type 1 diabetes were 38.05 and 249.73 per 100,000, respectively, and for type 2 were 2.51 and 23.7 per 100,000, respectively. The incidence of neonatal diabetes mellitus was 34.4 per 1,000,000 live births, and in indigenous Qataris the incidence was 43.6 per 1,000,000 live births. The prevalence of type 1 diabetes and type 2 diabetes in Qatari children was double compared with other nationalities. The prevalence of maturity onset diabetes of the young in Qatar was 4.56 per 100,000. CONCLUSIONS: This is the first prospective and comprehensive study to document the epidemiology and genetic landscape of childhood diabetes mellitus in this region. Qatar has the fourth highest incidence of type 1 diabetes mellitus, with the incidence and prevalence being higher in Qatari compared with non-Qatari. The prevalence of type 2 diabetes mellitus is also higher in Qatar than in Western countries. The incidence of neonatal diabetes mellitus is the second highest in the world. GCK is the most common form of maturity onset diabetes of the young, and a large number of patients have type 1B diabetes mellitus.

Nutritional status of children with cerebral palsy in remote Sumba Island of Indonesia: a community-based key informants study.

AIM: To assess the burden and underlying factors of malnutrition among children with cerebral palsy (CP) in the remote Sumba Island, Indonesia. METHODS: This is a community-based key informant (KI) method survey of children with CP in the southwest regency of Sumba Island, Indonesia (child population∼152 471). Children with suspected CP identified by trained community volunteers (KIs) underwent a detailed neurodevelopmental assessment by a multidisciplinary medical assessment team to confirm diagnosis of CP. Anthropometric measurements were taken, z scores were calculated, and nutritional status was determined following the World Health Organization guideline. Descriptive analyses were performed, multivariable linear regression model was fitted to identify potential predictors of malnutrition. RESULTS: One hundred and thirty children with CP aged <18 years were included in the study. The majority were severely underweight (78.8%) and severely stunted (85.9%). Severe malnutrition was overrepresented among young children, children who had spastic tri/quadriplegia, Gross Motor Function Classification System levels III-V, had at least one associated impairment, speech impairment and/or swallowing difficulties. Age and presence of visual impairment were found to be significantly associated with weight-for-age z score (i.e., underweight) whereas, epilepsy was significantly associated with the height-for-age z score (i.e., stunting) when adjusted for other covariates. CONCLUSIONS: The substantial-high burden of malnutrition among children with CP in remote Sumba Island highlights their vulnerability to poor health-related outcomes. There is an urgent need for nutritional rehabilitation services to avert such consequences among children with CP in low and middle-income countries like Indonesia.Implications for rehabilitationMalnutrition among children with cerebral palsy (CP) is alarmingly high in the remote Sumba island of Indonesia.Children with severe gross motor function limitations or spastic tri/quadriplegia had the most severe malnutrition.There is an urgent need for rehabilitation services including nutrition and feeding interventions for children with CP in low and middle-income countries like Indonesia.

Epidemiology of cerebral palsy in Sumba Island, Indonesia.

AIM: To define the epidemiology, clinical characteristics, and rehabilitation status of children with cerebral palsy (CP) in Sumba Island, Indonesia. METHOD: A community-based key informant method survey among children (aged <18y) with CP was conducted between March and August 2017. Children with suspected CP underwent detailed neurodevelopmental assessment by a multidisciplinary medical team. Socio-demographic characteristics, aetiology, motor type, motor severity, associated impairments, educational, and rehabilitation status were documented. RESULTS: There were 130 children with clinically confirmed CP. The mean age at assessment was 8 years 11 months and 43.8% (n=57) of the children were female. The mean age at CP diagnosis was 6 years 5 months. Of these children, 46.9% (n=61) had post-neonatally acquired CP, most frequently because of vaccine-preventable infectious encephalopathy (73.8%, n=45). In total, 80.8% (n=105) had a predominantly spastic motor type of CP and 83.8% (n=109) were classified in Gross Motor Functional Classification System levels III to V. A total of 77.7% (n=101) had at least one associated impairment (speech 77.5%, intellectual 29.2%, visual 13.8%, hearing 20.0%, and epilepsy 13.5%). And 66.2% (n=86) had never received rehabilitation services. INTERPRETATION: Post-neonatally acquired CP was common in this setting. Addressing preventable post-neonatally acquired risk factors for CP should be a public health priority. Earlier identification and diagnosis of CP would also provide new opportunities for early intervention and targeted rehabilitation services.

What predicts the proxy-reported health-related quality of life of adolescents with cerebral palsy in Bangladesh?

BACKGROUND: The health-related quality of life (HRQoL) of adolescents with CP in low and middle-income countries is often poor, as is the case in Bangladesh. This exploratory study examined what factors predict the proxy-reported HRQoL of adolescents with CP in rural Bangladesh, a typical low- and middle-income country (LMIC). METHODS: Adolescents with CP (10 to 18y) were identified using the Bangladesh Cerebral Palsy Register. HRQoL was assessed using the Cerebral Palsy Quality of Life-Teens proxy-report questionnaire (CPQoL-Teens), adolescent mental health using the Strengths and Difficulty Questionnaire (SDQ) and caregiver mental health using the Depression, Anxiety and Stress Scale (DASS-21). Theoretical and statistical interests (i.e. bivariate analysis, p < 0.05) identified potential predictors which were entered into hierarchical multiple linear regression (HMLR) models in order of clinical significance; HMLR related adolescent clinical characteristics, adolescent and caregiver mental health and proxies of socioeconomic status to CPQoL-Teens dimensions. RESULTS: One hundred fifty-four adolescents with CP (mean age 15y 1mo, SD 1y 8mo, female 31.2%) participated in this study. Twenty-four factors were identified to explore for relationship to adolescent proxy-reported HRQoL. Fifteen of the factors correlated to one or more CPQoL-Teens dimension; strongest correlation was between 'feelings about functioning' and motor impairment (r = 0.545). Nine were predictive of CPQoL-Teens dimensions; adolescent sex, school attendance, severity of motor impairment, hearing and speech impairment, mother's education, primary caregiver depression and stress, and having a sanitary latrine at home resulting in score changes of between 0.79 (95% CI 0.24 to 1.35) to 35.1 (95% CI 6.03 to 64.22). CONCLUSIONS: Many of the factors predicting the proxy-reported HRQoL of adolescents with CP are amenable to intervention, and have the potential to improve adolescent wellbeing. Several determinants are priorities of the sustainable development goals (SDGs); these findings should inform resource prioritization to improve the wellbeing of adolescents with CP in Bangladesh and other LMICs.