Background & aims
Caregivers of children with chronic diseases included in a home enteral nutrition (HEN) programme are at risk of experiencing a feeling of burden, high level of anxiety and ...psychological distress. The aims of this study were: first, to examine the prevalence of symptoms of anxiety–depression in caregivers of children with neurological diseases requiring HEN by gastrostomy tube (GT); second, to compare the characteristics of caregivers with high or low risk of exhibiting symptoms of anxiety–depression; and third, to investigate possible associations to child disease severity and nutrition support mode.
Methods
A cross‐sectional observational study was performed in 58 caregivers of children (31 boys, aged 0.3–18 years) with neurological diseases and GT feeding. The characteristics of caregivers with high or low risk of presenting symptoms of anxiety–depression were compared regarding the following variables: socio‐demographic characteristics, the primary caregiver's intrapsychic factors, anthropometric parameters of the child, length of HEN, type of nutrients delivered by GT and infusion regime.
Results
All primary caregivers were mothers. Fifty‐three per cent of them showed high risk of exhibiting symptoms of anxiety–depression. Mothers with high or low risk of presenting symptoms of anxiety–depression were comparable in age and family socio‐economic status. They were also similar in terms of age, anthropometric conditions and length of HEN in their children.No differences were found between the two groups of mothers according to the level of the child's motor function impairment, type of nutrients delivered by GT and infusion regime. Higher levels of psychological distress and perception of burden overload were found in mothers with high risk of exhibiting symptoms of anxiety–depression.
Conclusions
This study found a high prevalence of symptoms of anxiety–depression, perception of burden overload and psychological distress in caregivers of children with HEN. Thus, greater practical and emotional support is required for these families.
Background
To analyse the psychometric properties of the structured Satisfaction Questionnaire with Gastrostomy Feeding (SAGA‐8) in parents/caregivers of children with home enteral nutrition (HEN) by ...gastrostomy tube (GT).
Methods
Eighty‐six caregivers (mothers) of paediatric patients with HEN by GT were recruited. Patients suffered from neurological disease (61.6%) and other chronic diseases. The SAGA‐8 scale, a structured questionnaire to explore satisfaction with HEN by GT, and the Caregiver Burden Inventory (Zarit) were completed. The discriminating power of each of the SAGA‐8 items, internal consistency and external validity were evaluated. An exploratory factor analysis and Kaiser–Meyer–Olkin (KMO) was performed as well.
Results
Eighty‐four percent of the families expressed high satisfaction with GT feeding. All eight items of SAGA‐8 gave additional information. The exploratory factor analysis revealed that a significant part of the items’ variability could be explained by two independent factors: Factor 1 (direct benefit), which compiled the variables related to the perception of children's overall improvement by GT feeding; Factor 2 (indirect benefit), which grouped the variables related to a decrease in respiratory infections, feeding time and institutional support. Results from KMO (0.628) indicated the high adequacy of the items assessed in the factorial analysis. Moreover, the questionnaire presented high internal consistency (0.76), and the external validation analysis confirmed the correlation between SAGA‐8 and Zarit, thereby emphasising the approptiate use of the SAGA‐8 to detect carers’ satisfaction.
Conclusions
The SAGA‐8 questionnaire has a high discriminatory power to assess the degree of satisfaction experienced by parents/caregivers of children with HEN by GT and, subsequently, the patients’ wellbeing.
Identification of very long-chain acyl-CoA dehydrogenase deficiency is possible in the expanded newborn screening (NBS) due to the increase in tetradecenoylcarnitine (C14:1) and in the C14:1/C2, ...C14:1/C16, C14:1/C12:1 ratios detected in dried blood spots. Nevertheless, different confirmatory tests must be performed to confirm the final diagnosis. We have revised the NBS results and the results of the confirmatory tests (plasma acylcarnitine profiles, molecular findings, and lymphocytes VLCAD activity) for 36 cases detected in three Spanish NBS centers during 4 years, correlating these with the clinical outcome and treatment. Our aim was to distinguish unambiguously true cases from disease carriers in order to obtain useful diagnostic information for clinicians that can be applied in the follow-up of neonates identified by NBS.
Increases in C14:1 and of the different ratios, the presence of two pathogenic mutations, and deficient enzyme activity in lymphocytes (<12% of the intra-assay control) identified 12 true-positive cases. These cases were given nutritional therapy and all of them are asymptomatic, except one. Seventeen individuals were considered disease carriers based on the mild increase in plasma C14:1, in conjunction with the presence of only one mutation and/or intermediate residual activity (18–57%). In addition, seven cases were classified as false positives, with normal biochemical parameters and no mutations in the exonic region of ACADVL. All these carriers and the false positive cases remained asymptomatic. The combined evaluation of the acylcarnitine profiles, genetic results, and residual enzyme activities have proven useful to definitively classify individuals with suspected VLCAD deficiency into true-positive cases and carriers, and to decide which cases need treatment.
Short stature has been reported in congenital ichthyoses (CI), but few data exist on patients' nutritional status.
To describe the nutritional status at the first evaluation of children and young ...adults with CI.
Prospective observational study of patients assessed at a multidisciplinary clinic. Clinical variables and ichthyosis severity were collected. Anthropometric assessment was made by measuring weight and height, and nutritional status was classified based on the World Health Organization definitions for malnutrition. Analytical assessment included markers of nutritional status, fat-soluble vitamins, and micronutrients.
We included 50 patients with a median age of 5 years (IQR, 1.6-10.3). Undernutrition was found in 32% of patients, and 75% of the undernourished children presented growth impairment. Younger children and those with severe ichthyoses were the most affected. Micronutrient deficiencies were found in 60% of patients. Deficiencies of selenium (34%), iron (28%), vitamin D (22%), and zinc (4%) were the most frequent findings.
Our small sample includes a heterogeneous group of ichthyoses.
Children with CI appear to be at risk of undernutrition, especially at younger ages. Nutritional deficiencies are common and should be monitored. Growth failure in children with ichthyosis could be caused by undernutrition and aggravated by nutritional deficiencies.
Antecedentes, objetivos y metodología Pedrón Giner, Consuelo
Nutrición hospitalaria : organo oficial de la Sociedad Española de Nutrición Parenteral y Enteral,
2017, Letnik:
34, Številka:
3
Journal Article
Odprti dostop
La nutrición artificial ha experimentado un desarrollo muy importante en los últimos años debido, fundamentalmente, al aumento de la supervivencia de los pacientes con enfermedades agudas y crónicas, ...al mejor conocimiento de las necesidades de energía y nutrientes, a la disponibilidad de nuevos sustratos y a la existencia de nuevos materiales para llevarla a la práctica. El paciente pediátrico que necesita nutrición parenteral (NP) no constituye un grupo homogéneo no solo por las peculiaridades de cada enfermedad, sino también debido a la edad ya que, en función de esta, varían las características fisiológicas y las necesidades para el crecimiento y, por tanto, la composición de la NP.Además, existe una gran variabilidad en la práctica del soporte artificial y en concreto de la NP y más todavía en el niño debido a sus características biológicas.
Home enteral nutrition is used increasingly in pediatric populations. Our objective was to describe the profile of pediatric patients requiring this treatment.
All patients under 18 years old ...requiring treatment with home enteral nutrition between January 1995 and December 2004 were analyzed retrospectively.
304 patients were studied (157 boys). The mean age at the start of treatment was 4.02 ± 4.09 years, median of 2.5 years; 28% of all patients were under 1 year. The main indications were oncological disease in 91 patients (29.9%) and digestive diseases in 84 (27.6%). There were significant differences depending on the clinical diagnosis for the start age, type of access, infusion regime and formula prescribed. Nutrients were delivered by nasogastric tube in 218 patients (71.7%). Overnight enteral nutrition was the preferred infusion regime in 155 patients (51%). Adult or pediatric polymeric formulas were mostly prescribed in 190 patients (62.5%). The mean treatment duration was 306 ± 544 days.
In our series, enteral support usually begins at an early age. Its characteristics varied depending on patient pathology. Knowledge of the pediatric patient profile is important to design the most effective strategy for home enteral nutrition.
The home enteral nutrition (HEN) provides nutritional support to children with chronic diseases who are nutritionally compromised and allows them to be discharged more quickly from hospitals. In ...2003, a web-based registry (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition -NEPAD-) was created with the objective of gathering information about pediatric HEN practices in Spain.
The aim of this study was to report the implementation of the NEPAD (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition) registry of pediatric HEN in Spain and to analyze data evolution trends from 2003 to 2010.
The data from the Spanish NEPAD registry were analyzed according to the following variables: demographic data, diagnosis, indication for HEN, nutritional support regime and administration route.
Over the study period, 952 patients (1048 episodes) from 20 Spanish hospitals were included in the NEPAD registry. The most frequent indication for HEN was decreased oral intake (64%), and neurological disease was the most prevalent illness. HEN was delivered via a nasogastric tube in 573 episodes (54.7%), by gastrostomy in 375 episodes (35.8%), oral feeding in 77 episodes (7.3%) and by jejunal access in 23 episodes (2.2%). Significant differences in the mode of administration were observed based on the pathology of the child (χ(2), P<0.0001). The cyclic feeding was the most widely used technique for the administration of HEN. Most of the patients used a pump and a polymeric formula. Transition to oral feeding was the primary reason for discontinuation of this type of support.
Since the NEPAD registry was established in Spain, the number of documented patients has increased more than 25-fold. Many children with chronic illness benefit from HEN, mainly those suffering from neurological diseases.
To assess how the current practice of newborn screening (NBS) for homocystinurias compares with published recommendations.
Twenty-two of 32 NBS programmes from 18 countries screened for at least one ...form of homocystinuria. Centres provided pseudonymised NBS data from patients with cystathionine beta-synthase deficiency (CBSD, n = 19), methionine adenosyltransferase I/III deficiency (MATI/IIID, n = 28), combined remethylation disorder (cRMD, n = 56) and isolated remethylation disorder (iRMD), including methylenetetrahydrofolate reductase deficiency (MTHFRD) (n = 8). Markers and decision limits were converted to multiples of the median (MoM) to allow comparison between centres.
NBS programmes, algorithms and decision limits varied considerably. Only nine centres used the recommended second-tier marker total homocysteine (tHcy). The median decision limits of all centres were ≥ 2.35 for high and ≤ 0.44 MoM for low methionine, ≥ 1.95 for high and ≤ 0.47 MoM for low methionine/phenylalanine, ≥ 2.54 for high propionylcarnitine and ≥ 2.78 MoM for propionylcarnitine/acetylcarnitine. These decision limits alone had a 100%, 100%, 86% and 84% sensitivity for the detection of CBSD, MATI/IIID, iRMD and cRMD, respectively, but failed to detect six individuals with cRMD. To enhance sensitivity and decrease second-tier testing costs, we further adapted these decision limits using the data of 15,000 healthy newborns.
Due to the favourable outcome of early treated patients, NBS for homocystinurias is recommended. To improve NBS, decision limits should be revised considering the population median. Relevant markers should be combined; use of the postanalytical tools offered by the CLIR project (Collaborative Laboratory Integrated Reports, which considers, e.g. birth weight and gestational age) is recommended. tHcy and methylmalonic acid should be implemented as second-tier markers.
Home enteral nutrition (HEN) is a type of enteral nutrition (EN) which is becoming progressively more widespread in pediatrics due to the benefits it affords to patients, their families and to ...reducing hospital costs. However, the true extent of its use is unknown in Spain as the data-base set up for this purpose is still underused (Registro de Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria -NEPAD-). More thorough registration of patients in the NEPAD online register will provide information about the characteristics of HEN in Spain: prevalence, diagnosis, the population sector being administered HEN, complications and developments. Likewise, forecast and planning of the necessary resources could be made while those in use could be analysed.
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