Life Expectancy Of Children Research Articles

Pediatric limbal stem cell deficiency: An overview of a rarely studied pathology.

The cornea is the outermost transparent layer of the eye, and the continuous renewal of the corneal epithelium is important for its transparency. This process is primarily facilitated by corneal stem cells, most of which are found at the limbus. However, any deterioration or damage in this region leads to corneal conjunctivalization and consequent limbal stem cell deficiency (LSCD), which compromises corneal transparency. LSCD is an important condition, especially in the pediatric population, as it can lead to corneal vascularization, opacity and ultimately loss of vision and subsequent amblyopia, unlike adults. Although pediatric LSCD is often due to chemical injuries, as in adults, it is also caused by conditions such as congenital aniridia, Steven Johnson Syndrome and various other immunological disorders. Appropriate and timely treatment in pediatric LSCD is of particular importance in preventing progression to amblyopia, unlike adults. Accurate staging of the disease is essential for the necessary medical and surgical treatment decision. While medical approaches are at the forefront to eliminate the causative agent and improve the ocular surface in reversible cases, it is essential to replace the limbal stem cells lost in advanced disease. For these replacement procedures, it is noteworthy that there is a tendency for minimally invasive methods compared to adults to avoid possible complications due to long life expectancy in children. In conclusion, although there are various reviews on limbal stem cell deficiency (LSCD) in adults, we believe that this review for childhood LSCD will make an important contribution to the literature, since the relevant literature currently reported for the pediatric population is limited.

Clinical case of a live-born child with triploidy

Theoretical data on etiopathogenesis, clinical manifestations of triploidy, life expectancy of children in case of live-birth with this chromosomal anomaly are presented. A clinical case of a triploidy in a child is described. The girl was born from the first pregnancy from young healthy parents. Parents are not close relatives. Pregnancy was complicated with anemia, recurrent threats of miscarriage, progressive lack of water, progressive violation of uteroplacental blood flow and delay in fetal development. A congenital heart defect was diagnosed prenatally. The girl was born at 38 weeks of pregnancy with low indicators of physical development by caesarean section. Multiple developmental anomalies and malformations were revealed after birth. Combined congenital heart disease was confirmed by instrumental examination. Karyotyping revealed karyotype 69, ХХХ. Dynamic observation revealed that the child's age-related skills were not formed. Epileptic seizures occurred from the age of 4 months. The seizures had a status course. Surgical correction of congenital heart disease was performed in two stages: at 4.5 months and at 9.5 months in this patient. A fatal outcome occurred with an increase in multiple organ failure at the age of 10 months. The features of the clinical case, the possibilities of prenatal diagnosis of triploidy are analyzed.

Optimization of pediatric brain CT protocols

PurposeDue to the higher radiosensitivity of the tissues and the larger life expectancy in children than in adults, the present study presented the process of optimizing pediatric brain CT protocols, for the age groups 0 to 2 years, 3 to 5 years, and 6 to 12 years, evaluating the parameters of image acquisition and dose reduction, without loss of quality in image diagnostics. Methods: The CTDIvol index and DLP were verified in samples of routinely used protocols for patients in the age groups previously specified, as well as the image acquisition parameters. Based on these data, experimental protocols were developed and the contrast-to-noise ratios of the images produced were analyzed. The figure of merit (FOM) was used as a quality metric for quality analysis in comparison with CTDIvol and DLP levels. Results: The results showed a reduction in the CTDIvol index in the three studied protocols: 10.5% in the 0 to 2 years protocol, 33% in the 3 to 6 years protocol, and 16% in the 6 to 12 years protocol. DLP was also reduced by 14%, 41% and 25%, respectively, for the three age groups, in relation to previously used protocols. With regard to FOM, there were increases in quality of 6%, 19%, and 43%, respectively, for the three age groups. Conclusion: Considering the alteration of the image acquisition parameters, it was possible to achieve a dose reduction, without loss of diagnostic quality, according to the FOM metric.

Parenteral Nutrition in Pediatric Patients with Neurodisability: Current Perspectives.

Pediatric neurodisability describes functional limitations in children with varied severity and complexity often attributed to brain or neuromuscular abnormalities. The life expectancy of children with neurodisability is improving, but many will require significant medical support. The gastrointestinal tract is usually affected in children with neurodisability and can lead to a wide range of symptoms. In gastrointestinal (GI) dystonia, a newly coined term, feeding will trigger a distressing dystonia and symptoms can improve with cessation of feed. Parenteral nutrition (PN) is often viewed as a viable option in severe GI dystonia or when enteral feeding does not support sufficient nutrition. The use of PN in children with severe neurodisability is complex. It involves an intricate interplay between medical, psychological and ethical factors. In the absence of a universally agreed guidance on the use of PN in this cohort, paediatricians should maintain the individual need of the child at the centre of the decision-making process and work closely with families and other healthcare professionals before initiating or withholding PN in children with severe neurodisability. In this article, we discuss the complex and multifaceted approach to the use of PN in children with severe neurodisability and aimed to explore the medical, psychological and ethical aspect dilemmas facing clinicians looking after children with declining gut function who may require PN support.

Burden in primary informal caregivers of children and adolescents with type 1 diabetes: Is it associated with depression, family dysfunction, and glycemic control?

The requirement of a chronic treatment and the increase in life expectancy in children with type 1 diabetes (T1D) leads to the possibility of caregiver burden. The aim of our study was to evaluate the burden in primary informal caregivers (PIC) of children and adolescents with type 1 diabetes and its association with depression, family dysfunction, and glycemic control. A retrospective study was performed in PIC of children and adolescents with T1D. Zarit Burden Interview Scale (ZBIS) was used to evaluate caregiver burden. Beck Depression Inventory (BDI-II) was used to evaluate depression in PIC, and the Family APGAR questionnaire was used to evaluate the family functionality. A total of 100 PIC of children and adolescents with T1D were included. Caregiver burden was found in 33% of caregivers. The total score of the Zarit scale was 41 (34-49); 19% had mild caregiver burden, and 14% had severe caregiver burden. According to the BDI-II, 82% had minimal depression, 11% mild depression, 5% moderate depression, and 2% severe depression. Family function was good in 69%; 13% had moderate dysfunction, and 18% had severe dysfunction. A positive correlation between caregiver burden and BDI-II score (r = 0.84; p = 0.001) and the grade of depression (r = 0.87; p = 0.001) was found. A logistic regression model showed that BDI-II score was associated with caregiver burden (OR 1.14; 95% CI 1.061-1.23; p = 0.001). A BDI-II cut off of 9 or more had a sensibility and specificity of 58% and 28%, respectively, for caregiver burden [AUC 0.751 (0.64-0.85); p = 0.001]. A BDI-II score ≥9 was a predictor of caregiver burden (OR 3.4; 95% CI 1.4-8.1; p = 0.008). Caregiver burden is present in more than one third of the PIC of patients with T1D and is associated with depression. A BDI-II score ≥9 is a predictor of caregiver burden which may be a point to take into account in the integral approach to the patient with T1D and his or her family nucleus.

Identification of behavioral disorders using the Achenbach questionnaire in children with tumors of the posterior cranial fossa after completion of special treatment (pilot research)

Background. The increase in life expectancy of children who survived cancer leads to new tasks for doctors, psychologists and rehabilitation specialists to assessing the consequences of the experienced disease and its treatment. The most common disorders in children who have survived oncological diseases are behavioral disorders, a decrease in mood background, as well as chronic fatigue.Aim. To identify predictors of behavioral disorders in children who have survived central nervous system oncological diseases.Materials and methods. The study involved 52 children with central nervous system tumors aged 6 to 17 years. The median time after completion of therapy in this group of patients was 18 (3–117) months.Results. As a result of the study, it was shown that such treatment parameters as the degree of tumor malignancy and the radiation therapy volume are associated with behavioral disorders in children who have survived cancer. In such children, a reduced mood background was revealed, and the older the child, the higher the probability of a reduced mood background. A reduced mood background is also associated with the use of vincristine preparation. Children who have a residual tumor are more likely to complain of unpleasant sensations in the body. All children, despite the specifics of their treatment, complain of constant fatigue, which affects their daily activity.Conclusion. Thus, factors that are associated with behavioral disorders in children who have survived oncological diseases in the central nervous system were identified.

Newborn screening for severe combined immunodeficiency: The results of the first pilot TREC and KREC study in Ukraine with involving of 10,350 neonates.

Severe combined immunodeficiency (SCID) is a group of inborn errors of immunity (IEI) characterized by severe T- and/or B-lymphopenia. At birth, there are usually no clinical signs of the disease, but in the first year of life, often in the first months the disease manifests with severe infections. Timely diagnosis and treatment play a crucial role in patient survival. In Ukraine, the expansion of hemostatic stem cell transplantation and the development of a registry of bone marrow donors in the last few years have created opportunities for early correction of IEI and improving the quality and life expectancy of children with SCID. For the first time in Ukraine, we initiated a pilot study on newborn screening for severe combined immunodeficiency and T-cell lymphopenia by determining T cell receptor excision circles (TRECs) and kappa-deleting recombination excision circles (KRECs). The analysis of TREC and KREC was performed by real-time polymerase chain reaction (RT-PCR) followed by analysis of melting curves in neonatal dry blood spots (DBS). The DBS samples were collected between May 2020 and January 2022. In total, 10,350 newborns were screened. Sixty-five blood DNA samples were used for control: 25 from patients with ataxia-telangiectasia, 37 - from patients with Nijmegen breakage syndrome, 1 – with X-linked agammaglobulinemia, 2 – with SCID (JAK3 deficiency and DCLRE1C deficiency). Retest from the first DBS was provided in 5.8% of patients. New sample test was needed in 73 (0.7%) of newborns. Referral to confirm or rule out the diagnosis was used in 3 cases, including one urgent abnormal value. CID (TlowB+NK+) was confirmed in a patient with the urgent abnormal value. The results of a pilot study in Ukraine are compared to other studies (the referral rate 1: 3,450). Approbation of the method on DNA samples of children with ataxia-telangiectasia and Nijmegen syndrome showed a high sensitivity of TRECs (a total of 95.2% with cut-off 2000 copies per 106 cells) for the detection of these diseases. Thus, the tested method has shown its effectiveness for the detection of T- and B-lymphopenia and can be used for implementation of newborn screening for SCID in Ukraine.

8-14 Yaş Grubu Çocukların COVID-19 Pandemi Döneminde Sağlıkla İlgili Yaşam Kalitelerinin ve Etkileyen Faktörlerin Belirlenmesi

Amaç: Araştırmada çocukların pandemi döneminde sağlıkla ilgili yaşam kalitesi düzeyleri ve ilişkili faktörlerin belirlenmesi amaçlanmıştır.Materyal ve Metot: Kesitsel tasarım tipindeki araştırma üç ayrı ilde belirlenen altı okulda 8-14 yaş arası çocuğu olan gönüllü 1221 aile üyesi ile yürütülmüştür. Çalışmada sosyo-demografik özellikler soru formu, COVID-19 pandemi sürecinde çocuğun sağlıklı yaşam biçimi davranışları soru formu ve KIDSCREEN-27 ölçeği kullanılmıştır.Bulgular: Çalışmada; erkek, 12-13 yaş grubu, dört ve daha fazla kardeşe sahip, ailesi düşük ekonomik düzeyde olan, sessiz-sakin, duygusal-alıngan, içe dönük kişilik özelliğine sahip olan çocukların yaşam kalite puanları daha düşük bulunmuştur. Ayrıca, pandemi sürecinde uyku alışkanlığı, sosyal ilişkileri, kişisel hijyen alışkanlıkları ve sağlıklı besinleri tüketim davranışları olumsuz olarak etkilenen, dersleri için kullanımları dışında ekran maruziyeti süresi artış gösteren, kitap okumaya, hobilerine, spor ve egzersize ayırdığı süre azalan çocukların yaşam kalitesi puanları da düşük bulunmuştur.Sonuç: Çalışma sonuçları düşük yaşam kalitesi riski altında olan çocuklar için planlanacak müdahale programlarına rehber olması açısından önemlidir.

Parents' Perspectives on Diagnosis and Decision-Making regarding Ventilator Support in Children with SMA Type 1.

Spinal muscular atrophy (SMA) is a rare neuromuscular disorder with a broad clinical spectrum. The most severe phenotype-SMA type 1-is characterized by marked muscle weakness also affecting bulbar and respiratory function. Life expectancy of children with SMA type 1 is expected to be less than 2 years without ventilator support or disease-specific drug treatment. The aim of this study was to evaluate parents' perspectives on the process of decision-making regarding ventilator support in children with SMA type 1. Fourteen semi-structured interviews were performed with parents of children with SMA type 1 that decided either for or against ventilator support for their child. All children were diagnosed prior to the approval of SMA-specific drug treatment. Interviews were recorded and transcribed verbatim. Data analysis was performed using a qualitative content analysis approach according to Mayring. Parents experienced that they were not adequately informed about the disease and treatment options in first informed consent discussions. Especially regarding ventilator support, parents perceived that they were not offered ventilator support as an actual option for treatment. Regarding the decision of whether or not to offer ventilator support, parents reported that their attitude toward ventilator support and contact with other affected families or patient advocacy groups were more likely to influence the decision than the content of informed consent discussions with physicians. Our results underline the importance of an interdisciplinary team not only to provide parents with relevant information but also to consider the criteria of a patient-centered medicine.

Upaya Peningkatan Pengetahuan melalui Promosi Kesehatan Penyakit Keganasan pada Anak di SMAN Titian Teras Jambi

Malignant disease, or more commonly known as cancer, is a disease characterized by the growth and spread of abnormal cells in the body. Malignant disease in children, although rare, is the second leading cause of death in children in developed countries after accidents. Data from the Union for International Cancer Control (UICC), there are about 176,000 children worldwide who are diagnosed with cancer each year. In Indonesia, there are about 11,000 cases of childhood cancer every year. Recognizing the signs of malignancy at an early age can increase the cure rate and life expectancy of children who experience it. Therefore, it is necessary to provide knowledge to children about early detection and prevention of cancer in children. This activity is carried out by the method of providing health promotion in the form of counseling at SMAN Tititan Teras Jambi. This activity is carried out by the method of counseling. The activity has been carried out in October 2021. The output of this activity is an increase in student knowledge. The results of the activity evaluation, it was found that there were differences in the level of knowledge of students who were given counseling with students who were not given counseling (scores of 7.03 and 5.63, respectively). Increasing knowledge is expected to improve student attitude and behavior to recognize early signs & symptoms of malignancy, so that diagnosisand management can be carried out quickly & precisely

Integrated care to address child and adolescent health in the 21st century: A clinical review.

Increasing specialisation and technical sophistication of medical tools across the 21st century have contributed to dramatic improvements in the life-expectancy of children and adolescents with complex physical health problems. Concurrently, there is growing appreciation within the community of the extent that children and adolescents experience mental disorders, which are more prevalent in those with complex chronic, serious or life-limiting health conditions. In this context, there are compelling reasons for paediatric services to move to a model of care that promotes greater integration of child psychiatry within the medical, somatic teams that care for children and adolescents in children's hospitals. In this article, we discuss the range of medical disorders managed by contemporary paediatrics. We conducted a broad review of the literature and existing services, and use individual accounts to illustrate adolescents' healthcare preferences in the context of the challenges they experience around their mental health. Relevant disorders include life-limiting disorders, such as cancer; disorders involving the brain, such as epilepsy; common chronic disorders, such as asthma and diabetes; psychiatric emergencies, such as deliberate self-harm; and conditions that most commonly present to paediatric services, but where psychiatric input is required, such as severe eating disorders, somatic symptom disorders and gender dysphoria. The persisting legacy of the historical separation of physical and mental health services is described. Yet there are many models of service integration that can promote more collaborative care between psychiatrists and medical specialists, including some which have been taken to scale. In essence, clinical teams in children's hospitals require more collaborative approaches that facilitate early recognition and treatment of the psychological aspects of illness as an integral part of patient-centred, family-focussed paediatric care, rather than as something that is bolted on when things go wrong. Whilst trust and goodwill between services and providers will be required for novel models of care to be implemented, evaluation of these new models and incorporation of young people's healthcare preferences is needed.

Changes in lipid-synthesizing function of the liver in congenital cytomegalovirus infection in death newborns

Aim. To assess the change in the lipid-synthesizing function of the liver in congenital cytomegalovirus infection (CMVI) in dead newborns.Materials and methods. The study of the lipid spectrum of umbilical cord blood in the first minutes of life was carried out in 59 full-term newborns with antenatal ontogenesis, unburdened and burdened with congenital CMVI. The first group consisted of 25 newborns of early neonatal age (control group) from mothers without moderate and severe obstetric and somatic pathology, as well as respiratory viral diseases and sexually transmitted infections. The second group included 18 children of the same age (comparison group), whose mothers suffered exacerbation of CMVI in the second trimester of pregnancy, which did not lead to antenatal viral aggression. The third group was represented by 16 newborns with congenital CMVI. The life expectancy of children was 2-5 days. In the first group, the main cause of death of children was prolonged ante- and intranatal hypoxia, atelectasis and hyaline membranes of the lungs, in the second group – prolonged antenatal hypoxia, intranatal hypoxia and atelectatic, hyaline and edematous-hemorrhagic pneumopathies, and in the third group – congenital CMVI, which manifested itself as cerebral ischemia of moderate and severe degree, meningoencephalitis, ventriculomegaly, pseudocysts of the vascular plexus and subarachnoid hemorrhages, monocytosis, vesiculopustulosis, hepatitis and pneumonia. In the blood serum from the umbilical vein during biochemical analysis, the concentration (mmol/L) of total cholesterol, high-density lipoproteins, low-density lipoproteins and triglycerides was estimated.Results. In newborns of the second group in the blood serum from the umbilical cord vein, the concentration of total cholesterol was 1.90±0.04 mmol/L, high-density lipoproteins – 1.49±0.06 mmol/L, low-density lipoproteins - 0.26±0,02 mmol/L and triglycerides – 0.49±0.03 mmol/L (in the first group, respectively, 1.93±0.06 mmol/L, p>0.05; 1.37±0.06 mmol/L, p>0.05; 0.43±0.02 mmol/L, p<0.001; 0.45±0.02 mmol/L, p>0.05). In the third group, compared with the second one, there was a decrease in the level of total cholesterol to 1.79±0.04 mmol/L (p<0.05) and high-density lipoproteins – to 1.28±0.06 (p><0.05) against the background of an increase in triglyceride concentration up to 0.59±0.03 mmol/L (p><0.05). Conclusion. The above changes in the lipid-synthesizing function of the liver indicate a direct and indirect effect of congenital CMVI on the morphological structure of hepatocytes and the activity of enzyme systems. Key words: full-term newborns, congenital cytomegalovirus infection, total cholesterol, high-density lipoproteins, lowdensity lipoproteins, triglyceride>˂ 0.05) and high-density lipoproteins – to 1.28±0.06 (p<0.05) against the background of an increase in triglyceride concentration up to 0.59±0.03 mmol/L (p><0.05). Conclusion. The above changes in the lipid-synthesizing function of the liver indicate a direct and indirect effect of congenital CMVI on the morphological structure of hepatocytes and the activity of enzyme systems. Key words: full-term newborns, congenital cytomegalovirus infection, total cholesterol, high-density lipoproteins, lowdensity lipoproteins, triglycerides>˂ 0.05) against the background of an increase in triglyceride concentration up to 0.59±0.03 mmol/L (p˂ 0.05).Conclusion. The above changes in the lipid-synthesizing function of the liver indicate a direct and indirect effect of congenital CMVI on the morphological structure of hepatocytes and the activity of enzyme systems.

Chronic hepatitis B in children carried out of the hemochromatosis gene HFE

Purpose: to establish the frequency and clinical significance of mutant variations of the HFE gene polymorphism in chronic hepatitis B (CHB) in children with iron overload syndrome (IOS).Materials and methods: 60 children with chronic hepatitis B with iron overload syndrome (IOS) were examined. When distributing children into groups, we took into account the criteria we developed for assessing the degree of life expectancy in children with CHB: CST>0.5 – mild degree of life expectancy (43,3% of children), CST˃0.5 – mild degree of life expectancy (43,3% of children), CST˂0,5 – moderate severity of life expectancy (31,7% of children) and CST˂0,2 – severe degree of SPL (25,0%). Virological verification of HBV was performed by ELISA and PCR. Using PCR Real Time and molecular genetic analysis, HFE gene C282Y, H63D, S65C mutations were detected from amplified DNA using the PRONTO Hemochromatosis reagent kit (Israel). The transferrin saturation coefficient (CST) was calculated using the formula CST = sTfR / log10.Ft. Results:Results: The study of the hemochromatosis gene HFE showed that the overwhelming majority (84,0%) of children with CHB with IOS were carriers of heterozygous, phenotypically different, mutant types. And only 16,0% of sick children were homozites of the wild (normal) HFE gene. Analysis of the phenotypic polymorphism of the hemochromatosis gene HFE revealed the presence of three point heterozygous mutations: H63D, S65C and combined variations in H63D / S65C, the latter of which is associated with severe forms of CHB and severe IOS.Conclusion. Children with CHB with IOS are characterized by a high incidence of heterozygous mutations in the HFE gene, the phenotypic manifestations of which were S65C, H63D, H63D / S65C. The comparability of the heterozygous combined mutation H63D / S65C with severe forms of CHB and a severe degree of IOS gives grounds to consider this phenotype of the HFE gene as a factor in the progression of the disease.

Issues of COVID-19-related distance learning for children with neuronopathic mucopolysaccharidoses

The COVID-19 pandemic has impacted the education of children around the world, forcing a large proportion of teaching to be carried out remotely. The implications of this disruption have yet to be fully elucidated, but initial assessments suggest that COVID-19-related school closures and reliance on virtual learning may have a long-term negative impact on educational attainment and future earnings as well as life expectancy of children in the United States. Among children with neurodegenerative disorders, such as neuronopathic mucopolysaccharidoses (MPS disorders), the effects of the pandemic are likely to be even greater. We aim to shine a spotlight on the impact of COVID-19 on the education, treatment and general wellbeing of children and families affected by MPS disorders by highlighting the important role that educators and therapists play in supporting the neurocognitive function and quality of life of children with neuronopathic MPS disorders. This article will serve as a resource that caregivers, educators, clinicians and therapists can use when considering how best to advocate for children with neuronopathic MPS disorders in circumstances where in-school teaching or in-clinic treatment is compromised or not possible. Given that the current pandemic is likely to have a prolonged course and impact and that similar epidemics and pandemics are a near certainty in the future, it is essential that steps are taken to support the learning and care of children with neuronopathic MPS disorders. We must prioritize strategies to safely resume this fragile community's access to in-person education and supportive care, and to address gaps that have emerged during prolonged pauses in access, whenever possible.

Survival of South African children with cerebral palsy.

The South African (SA) public healthcare sector has experienced a surge in birth injury claims in recent years, particularly in respect of cerebral palsy (CP). The lump sum settlements in these matters are a function of the expected survival curve of the individual concerned. It is known from international studies that the life expectancy of children with CP is shorter than that of the general population, and depends on the pattern and severity of their disabilities. However, empirical estimates of survival for children with CP in SA are not available. To construct survival curves according to the pattern of gross motor skills for CP children in SA and compare these with international studies. We collected data on mortality and functional status for 339 CP childrenon whose behalf claims for medical negligence had been instituted. Motor disabilities were classified according to the five-level Gross Motor Function Classification System (GMFCS). Children who were unable to walk unaided were further classified according to more basic motor skills, including the ability to lift their heads or chests in the prone position, rolling and sitting. Mortality rates were calculated and survival curves were estimated using the Kaplan-Meier method. No deaths were observed among 119 children in GMFCS levels I - IV. Among the 220 children in GMFCS V, there were 20 observed deaths. The proportions surviving to ages 10 and 15 years were 85% (standard error (SE) 5%) and 55% (SE 11%), respectively. The former is comparable to what has been reported for children in California and Sweden, but the survival to age 15 is lower. Among 82 children who could not lift their heads in the prone position, there were 11 observed deaths for a mortality rate of 48.5 (95% confidence interval (CI) 24.2 - 86.9) deaths per 1 000 person-years. Among 72 children who could lift their heads but not their chests, there were 6 observed deaths for a mortality rate of 33.5 (95% CI 12.3 - 73.0) deaths per 1 000 person-years. These mortality rates are 22% and 15% higher than the corresponding figures documented for children with comparable abilities and disabilities in California. Life expectancy of children with CP in SA is lower than that of children with comparably severe disabilities in high-income countries.

СОСТОЯНИЕ СЕРДЕЧНО-СОСУДИСТОЙ СИСТЕМЫ У ДЕТЕЙ РЕЦИПИЕНТОВ ПОЧЕЧНОГО ТРАНСПЛАНТАТА

Damage of the cardiovascular system (cardiovascular disease, CVD) is the main cause of reduced life expectancy in children with chronic kidney disease (CKD). In the development of damage of the heart and blood vessels, both traditional factors and caused by impaired renal function, which appear already in the early stages of kidney disease, play a role. Purpose of the study: assessment of markers of the structure, function and metabolism of the heart and study of their changes in children, kidney transplant recipients. Materials and methods. 54 children, a kidney transplant recipients (Tx), who were under observation and treatment at the National Center for Pediatric Nephrology and Renal Replacement Therapy, Minsk 2nd Children's City Clinical Hospital, aged 3 to 17 years, were included in the study. The analysis of the data of the Tx group and conditionally divided subgroups: 1) with glomerular disease leading to the end stage of CKD (ESRD), n = 26; 2) with non-glomerular pathology, n = 27, in one patient the cause of ESRD was not specified. The control group consisted of healthy children from cardiology department without kidney pathology (n = 86). Results. Anamnestic, clinical, laboratory, immunological (serum concentration of T- and B-lymphocyte activation markers (RANTES and BAFF), proinflammatory (caspase 1, IL1fi and TNFa), vascular (VEGF) and tissue (TGF1p) growth factors), metabolic status (adyponectin, leptin, obestatin, vitamin D 25(OH)D), cardiospecific molecules (highly sensitive C-reactive protein (hsCRP), proBNP, transferrin, TSAT index), instrumental changes. Conclusion. Changes in the cardiovascular system in Tx are ambiguous. On the one hand, there is a significant improvement in the geometry of the myocardium and arterial hypertension, on the other hand, the atherogenic direction of metabolic changes and biochemical markers of CVD remains.

Excessive premature mortality among children with cerebral palsy in rural Uganda: A longitudinal, population-based study.

Studies from high-income countries reported reduced life expectancy in children with cerebral palsy (CP), while no population-based study has evaluated mortality of children with CP in sub-Saharan Africa. This study aimed to estimate the mortality rate (MR) of children with CP in a rural region of Uganda and identify risk factors and causes of death (CODs). This population-based, longitudinal cohort study was based on data from Iganga-Mayuge Health and Demographic Surveillance System in eastern Uganda. We identified 97 children (aged 2-17 years) with CP in 2015, whom we followed to 2019. They were compared with an age-matched cohort from the general population (n = 41 319). MRs, MR ratios (MRRs), hazard ratios (HRs), and immediate CODs were determined. MR was 3952 per 100 000 person years (95% CI 2212-6519) in children with CP and 137 per 100 000 person years (95% CI 117-159) in the general population. Standardized MRR was 25·3 in the CP cohort, compared with the general population. In children with CP, risk of death was higher in those with severe gross motor impairments than in those with milder impairments (HR 6·8; p = 0·007) and in those with severe malnutrition than in those less malnourished (HR = 3·7; p = 0·052). MR was higher in females in the CP cohort, with a higher MRR in females (53·0; 95% CI 26·4-106·3) than in males (16·3; 95% CI 7·2-37·2). Age had no significant effect on MR in the CP cohort, but MRR was higher at 10-18 years (39·6; 95% CI 14·2-110·0) than at 2-6 years (21·0; 95% CI 10·2-43·2). Anaemia, malaria, and other infections were the most common CODs in the CP cohort. Risk of premature death was excessively high in children with CP in rural sub-Saharan Africa, especially in those with severe motor impairments or malnutrition. While global childhood mortality has significantly decreased during recent decades, this observed excessive mortality is a hidden humanitarian crisis that needs to be addressed.

Effectiveness of immunosuppression minimisation, conversion or withdrawal strategies in paediatric solid organ and haematopoietic stem cell transplantation: a protocol of a systematic review and meta-analysis

IntroductionPaediatric transplantation is the only curative therapeutic procedure for several end-stage rare diseases affecting different organs and body systems, causing altogether great impact in European children’s health and quality of...

Natural History of Type 1 Spinal Muscular Atrophy in a Series of Argentinian Children.

SMA1 natural history is characterized by early development of chronic respiratory failure. Respiratory interventions in type 1 SMA infants are subject to great practice variability. Nusinersen, has been recently approved in Argentina. The advent of novel treatments has highlighted the need for natural history studies reporting disease progression in type 1 SMA. To analyze the progression, respiratory interventions and survival based on the type of respiratory support in type 1SMA patients, in a third level pediatric hospital in Argentina. Cohort of SMA1 patients followed at the Interdisciplinary Program for the Study and Care of Neuromuscular Patients (IPNM). Patient survival was analyzed by using the Kaplan-Meier method. Log-rank test was performed to compare the survival curve for three respiratory intervention groups. 59 patients. Mean age of symptom onset was 2.19 (±1.4) months, age at diagnosis was 3.9 (±2.1) months. Patients developed respiratory failure at 5.82 months (±2.32) and 13.8 months (±5.6) in Type 1B and Type 1C, respectively (p < 0.001) 53 p were SMA1B. Three copies were found in 1/6 SMA1C. Respiratory interventions: SRC 23 p (56.1%); SRC + NIV 8 p (19.5%); SRC + IV 10 p (24.4%). 8 patients were already on invasive ventilation when included in the IPNM. Patients with invasive ventilation showed longer survival. This series provides valuable information on respiratory intervention requirements and life expectancy in children with SMA1 before the implementation of novel treatments that increase the expression of the SMA protein.

Bariatric interventions in obesity treatment and prevention in pediatric acute lymphoblastic leukemia: a systematic review and meta-analysis.

Most children are surviving acute lymphoblastic leukemia (ALL) today. Yet, the emergence of cardiometabolic comorbidities in this population may impact long-term outcomes including the quality of life and lifespan. Obesity is a major driver of cardiometabolic disorders in the general population, and in ALL patients it is associated with increased risk of hypertension, dysglycemia, and febrile neutropenia when compared with lean ALL patients undergoing therapy. This systematic review aims to assess the current evidence for bariatric interventions to manage obesity in children with ALL. The primary outcome for this systematic review was the change in BMI z-score with implementation of the interventions studied. Literature searches were conducted in several databases. Ten publications addressing the study question were included in this review, and five studies were used in the meta-analysis to assess the impact of the bariatric interventions on obesity. The BMI z-score did not change significantly with the interventions. However, the quality of evidence was low, which precluded the recommendation of their use. In conclusion, prospective, rigorous, adequately powered, and high-quality longitudinal studies are urgently needed to deliver effective lifestyle interventions to children with ALL to treat and prevent obesity. These interventions, if successful, may improves cardiometabolic health outcomes and enhance the quality of life and life expectancy in children with ALL.