Fat-soluble Vitamin Supplementation Research Articles

Current Diagnosis and Management of Familial Hypobetalipoproteinemia 1.

Familial hypobetalipoproteinemia (FHBL) 1 is a rare genetic disorder with an autosomal codominant mode of inheritance and is caused by defects in the apolipoprotein (apo) B (APOB) gene that disable lipoprotein formation. ApoB proteins are required for the formation of very low-density lipoproteins (VLDLs), chylomicrons, and their metabolites. VLDLs transport cholesterol and triglycerides from the liver to the peripheral tissues, whereas chylomicrons transport absorbed lipids and fat-soluble vitamins from the intestine. Homozygous or compound heterozygotes of FHBL1 (HoFHBL1) are extremely rare, and defects in APOB impair VLDL and chylomicron secretion, which result in marked hypolipidemia with malabsorption of fat and fat-soluble vitamins, leading to various complications such as growth disorders, acanthocytosis, retinitis pigmentosa, and neuropathy. Heterozygotes of FHBL1 are relatively common and are generally asymptomatic, except for moderate hypolipidemia and possible hepatic steatosis. If left untreated, HoFHBL1 can cause severe complications and disabilities that are pathologically and phenotypically similar to abetalipoproteinemia (ABL) (an autosomal recessive disorder) caused by mutations in the microsomal triglyceride transfer protein (MTTP) gene. Although HoFHBL1 and ABL cannot be distinguished from the clinical manifestations and laboratory findings of the proband, moderate hypolipidemia in first-degree relatives may help diagnose HoFHBL1. There is currently no specific treatment for HoFHBL1. Palliative therapy including high-dose fat-soluble vitamin supplementation may prevent or delay complications. Registry research on HoFHBL1 is currently ongoing to better understand the disease burden and unmet needs of this life-threatening disease with few therapeutic options.

Effect of vitamin blend supplementation on the oxidative stability and beef quality parameters of Nellore cattle

This study was conducted to investigate the effects of supplementation of B vitamins, fat-soluble vitamins (ADE), and combination of B+ADE on beef quality parameters and antioxidant status. Forty young Nellore bulls were allocated randomly across four treatments: no vitamin supplementation (Control); B vitamins supplementation (B); fat-soluble vitamins supplementation (ADE); and ADE + B supplementation. At the end of a 140-day trial, samples were collected to perform analysis of meat quality parameters and status activity of antioxidant enzymes at 1 h, 24 h, 192 h and 360 h post mortem. Treatments had no significant on pH24h, sarcomere length, meat color (L*, b*, chroma) or fat color (L*, a*, b*) of the longissimus lumborum muscle at 24 h, 192 h and 360 h post mortem between treatments B, ADE or ADE+B in relation to the control treatment. Furthermore, no significant changes were observed in the redox form of myoglobin (OMb, DMb, MMb) 24 h post mortem. However, the ADE and ADE+B treatments resulted in a higher percentage of intramuscular fat in the longissimus lumborum muscle when compared to the control treatment. Greater myofibrillar fragmentation index at 24 h, 192 h and 360 h, higher levels of oxymyoglobin at 192 h and 360 h, lower levels of metmyoglobin at 360 h were detected in the ADE and ADE+B treatments when compared to the control treatment. Furthermore, ADE increased superoxide dismutase (SOD) at 192 h and ferric reducing antioxidant power (FRAP) at 360 h compared to the control treatment, while ADE and ADE+B treatments increased FRAP at 24 h and 192 h, and catalase (CAT) at 192 h Moreover, ADE and ADE+B treatments decreased glutathione peroxidase (GPx) at 24 h and decreased malondialdehyde (MDA) content at 24 h and 360 h when compared to the control treatment. The use of ADE and ADE+B favored the formation of oxymyoglobin, prevented the oxidation of myoglobin, increased the ferric reducing antioxidant power and impaired lipid oxidation at different periods of ageing.

Nutrition support in the pediatric total pancreatectomy with islet autotransplantation recipient.

For children with diminished quality of life and chronic pain caused by acute recurrent or chronic pancreatitis who are undergoing total pancreatectomy with islet autotransplantation, postoperative nutrition support has several unique characteristics. Surgical complications may lead to delays in nutrition support initiation or require modifications to the regimen. Early postoperative dysmotility requires the use of temporary enteral nutrition until this improves. The resultant complete exocrine pancreatic insufficiency necessitates lifelong pancreatic enzyme replacement therapy and fat-soluble vitamin supplementation. A low-oxalate diet is recommended to prevent kidney stones. Carbohydrate counting is needed for the provision of short-term insulin dosing and possibly long-term as well, depending on the transplanted islet yield. Children should have careful nutrition assessment and monitoring at several follow-up visits during the first year, then annually, and at any time with concerns.

Evaluating Risk: Benefit Ratio of Fat-Soluble Vitamin Supplementation to SARS-CoV-2-Infected Autoimmune and Cancer Patients: Do Vitamin-Drug Interactions Exist?

Simple SummaryThis review brings attention to a crucial yet under-investigated subject which is vitamin–drug interactions. Fat-soluble vitamins such as vitamins A, D, E, and K have been proven to possess many beneficial effects in the treatment and prevention of COVID-19. Moreover, it has been reported that deficiencies of fat-soluble vitamins have been directly linked to COVID-19 infection-related morbidity and mortality, especially in high-risk populations such as cancer and autoimmune patients. However, many vitamin–drug interactions exist between most of the newly COVID-19 FDA-approved medications and fat-soluble vitamins. Hence, this mandates personalizing the COVID-19 treatment protocols, especially for patients who have any deficiency in any of these vital vitamins. Weighing the risk-to-benefit ratio of supplementing any of these fat-soluble vitamins with COVID-19 medications is considered crucial to maximize the therapeutic benefit and decrease the side effects of these drugs. COVID-19 is a recent pandemic that mandated the scientific society to provide effective evidence-based therapeutic approaches for the prevention and treatment for such a global threat, especially to those patients who hold a higher risk of infection and complications, such as patients with autoimmune diseases and cancer. Recent research has examined the role of various fat-soluble vitamins (vitamins A, D, E, and K) in reducing the severity of COVID-19 infection. Studies showed that deficiency in fat-soluble vitamins abrogates the immune system, thus rendering individuals more susceptible to COVID-19 infection. Moreover, another line of evidence showed that supplementation of fat-soluble vitamins during the course of infection enhances the viral clearance episode by promoting an adequate immune response. However, more thorough research is needed to define the adequate use of vitamin supplements in cancer and autoimmune patients infected with COVID-19. Moreover, it is crucial to highlight the vitamin–drug interactions of the COVID-19 therapeutic modalities and fat-soluble vitamins. With an emphasis on cancer and autoimmune patients, the current review aims to clarify the role of fat-soluble vitamins in SARS-CoV-2 infection and to estimate the risk-to-benefit ratio of a fat-soluble supplement administered to patients taking FDA-approved COVID-19 medications such as antivirals, anti-inflammatory, receptor blockers, and monoclonal antibodies.

Influence of Fat-Soluble Vitamin Intramuscular Supplementation on Kinematic and Morphometric Sperm Parameters of Boar Ejaculates.

Ejaculate quality can be regarded as multifactorial, with nutrition being a factor that could directly influence sperm parameters. The present study aimed to evaluate seminal quality associated with seasonal fat-soluble vitamin supplementation of boars. Seven sexually mature boars were randomly allotted to one of the three groups, and fed one of the three supplementary diets for 32 weeks: (1) control treatment (COD), without supplementation of fat-soluble vitamins, (2) treatment containing 100% fat-soluble vitamin supplementation administered intramuscularly, which was based on fat soluble vitamin supplementation (A, D3, E) (FVD1), and (3) treatment containing 50% of fat-soluble vitamin supplementation (FVD). Semen was collected at 7-day intervals. Semen samples were analyzed to assess several sperm parameters using the Computer-Assisted Semen Analysis (CASA) ISAS®v1 system. Results showed that groups receiving FVD1 and FVD supplementation had an increased semen volume. The percentages of motile and progressively motile sperm were increased by FVD1 treatment. A statistically significant interaction between treatment and season was found in the percentage of motility and progressive motility (p < 0.05). Sperm concentrations showed significant differences (p < 0.05) between treatments. Velocity variables (VSL, VCL, and VAP) were higher (p < 0.05) in boars that received fat-soluble vitamin supplementation in comparison to controls receiving no supplementation. The FVD1 treatment presented spermatozoa with greater head size and more elongated heads (p < 0.05). Overall, the utilization of dietary fat-soluble vitamin supplementation significantly improved the semen quality of boar ejaculates. This highlights the importance of fat-soluble vitamin supplementation in sexually active boars.

Effect of vitamin blend supplementation on the ingestive, digestive, and ruminal parameters of Nellore cattle

Rumen cannulated Nellore bulls (n = 4) were used in a 4 × 4 Latin square design, with a 2 × 2 factorial scheme to evaluate the effects of vitamin supplementation on: (1) ingestive, digestive, and ruminal parameters; (2) serum concentrations of 25-hydroxyvitamin D (25(OH)D); and (3) in situ degradability of complete diets. The factors consisted of two fat-soluble vitamin blend (ADE) supplementation levels (ADE− or ADE+) and two B vitamin blend (biotin, niacin, and thiamine) supplementation levels (B-blend− or B-blend+). Thus, the treatments evaluated consisted of no vitamin supplementation (ADE− B-blend−), supplementation of a B vitamin blend (ADE− B-blend+), supplementation with a fat-soluble vitamin blend (ADE+ B-blend−), or a combination of these two blends (ADE+ B-blend+). Nutrient intake and DM, as well as ruminal digestibility were not affected (P > 0.05) by any of the vitamin supplementations. The apparent total-tract digestibility of DM, organic matter (OM), neutral detergent fiber corrected for residual ash and protein (apNDF), crude protein (CP), and non-fiber carbohydrates (NFC) were not influenced by the experimental diets (P > 0.05). Furthermore, an increase in starch total-tract digestibility was verified with ADE supplementation in the diets (P = 0.031). None of the in situ parameters were affected (P > 0.05) by vitamin supplementation. Further, the treatments did not affect ruminal kinetics (ki, kp, and kd,) of DM, OM, and starch (P > 0.05). The omasal flow of total N, NH3-N, non-ammonia N, microbial N, and non-ammonia non-microbial N (P > 0.05) or the efficiency of microbial protein synthesis (P > 0.05) were not affected by vitamin blend supplementation. The total fat volatile acid (VFA) concentrations, as well lactate concentration were not affected (P > 0.05) by vitamin blend supplementation. Bulls that consumed the combination of ADE and B-blend supplementation had lower ruminal NH3-N concentrations (P = 0.008). B-blend vitamin supplementation decreased ruminal pH (P = 0.003). As expected, concentrations of serum 25(OH)D increased (P = 0.001) in Nellore bulls fed diets with the combination of fat-soluble vitamin (ADE) and B-blend supplementation. Supplementing Nellore bulls with a vitamin blend containing 25(OH)D3 was a successful strategy for increasing circulating concentrations of 25(OH)D. The B vitamin blend (biotin, niacin, and thiamine) supplementation did not improve ruminal fermentation, ingestive or digestive parameters. However, the fat-soluble vitamin blend (ADE) supplementation increased total-tract starch digestibility.

Vitamins A, D, E status as related to supplementation and lung disease markers in young children with cystic fibrosis.

The variable response to fat-soluble vitamin supplementation in young children with cystic fibrosis (CF), and factors contributing to this variability, remain under-investigated. To determine if recommended supplement doses normalize serum vitamins A (retinol), D (25-hydroxy-vitamin D, 25OHD), and E (α-tocopherol), and identify factors predictive of achieving sufficiency, in children with CF in the first 3 years of life. We studied 144 infants born during 2012-2017 and diagnosed with CF through newborn screening. Serum retinol, 25OHD, α-tocopherol and plasma cytokines interleukin (IL)-6, IL-8, IL-10, and tumor necrosis factor (TNF)-α were measured in early infancy and yearly thereafter. Vitamin supplement intakes and respiratory microbiology were assessed every 1-2 months in infancy and quarterly thereafter. The prevalence of vitamin D insufficiency (<30 ng/ml) at all ages combined was significantly higher (22%) compared to vitamin A (<200 ng/ml, 3%) and vitamin E (<5 µg/ml, 5%). All children were vitamin A sufficient by age 2 years. Vitamin E insufficiency was rare. Only 42% were early responders of vitamin D and 17% remain insufficient despite high supplement intakes. IL-6 was positively correlated, while IL-8, IL-10, and TNF-α were negatively correlated, with retinol and 25OHD. Multiple regression analysis revealed that supplement dose, season, α-tocopherol, pancreatic insufficiency, respiratory infections and IL-10 were significant predictors of 25OHD. Diagnosis through newborn screening coupled with supplementation normalized serum retinol and α-tocopherol in almost all infants with CF by age 3 years. However, response to vitamin D supplements in young children with CF occurred later and variably despite early and sustained supplementation.

Sebelipase alfa enzyme replacement therapy in Wolman disease: a nationwide cohort with up to ten years of follow-up

BackgroundWolman disease (WD), the rapidly progressive phenotype of lysosomal acid lipase (LAL) deficiency, presents in neonates with failure to thrive and hepatosplenomegaly, and leads to multi-organ failure and death before 12 months of age. In clinical trials, enzyme replacement therapy (ERT) with sebelipase alfa led to improved survival, growth and biological parameters in WD patients followed up to 5 years. Long-term follow-up and health-related quality of life (HRQoL) evaluation are lacking.ResultsWe performed a nationwide, retrospective study of sebelipase alfa in WD patients. Five patients with abolished LAL activity and bi-allelic LIPA mutations were included with a median follow-up of 7 years (1–10). ERT was initiated at a median age of 1 month (0–4). Infusion tolerance was excellent on the long-term with only one patient requiring systematic pre-medication. Cholestyramine, fat-soluble vitamin supplements and a specific diet (high in medium-chain triglycerides and low in long-chain fatty acids) were prescribed. Liver function tests, plasma lipid profiles, fat-soluble vitamin levels and growth parameters improved. Three patients transiently exhibited a neuromyopathic phenotype (footdrop gait, waddling walk or muscle fatigue) but electromyography and muscle strength testing were normal. At last follow-up, all patients were alive with normal growth parameters and a satisfactory HRQoL, no patient had special education needs, and one patient required parenteral nutrition since an acute gastroenteritis.ConclusionsEarly ERT initiation allowed 100% survival with positive outcomes. Very long-term follow-up and hematopoietic stem cell transplantation while on ERT should be evaluated to strengthen the benefits of sebelipase alfa.

Neonatal hepatitis in a term newborn: sequelae to in utero COVID-19 infection

Neonatal hepatitis is a common disease entity seen among newborns. It is known as inflammation of liver parenchyma in newborns, specifically in first month of life. Most common cause of neonatal hepatitis is viral infection which infects liver in utero or in postnatal period. Newborns carrying neonatal hepatitis mostly present with jaundice, failure to gain weight and hepatosplenomegaly. Neonatal hepatitis due to in utero COVID infection is diagnosed by conjugated hyperbilirubinemia, raised SGOT and SGPT levels and raised COVID antibody levels. Treatment includes addition of fat-soluble vitamins, phenobarbitone and supportive care. Here author is reporting a rare case of 11 days old newborn with neonatal hepatitis, who presented with severe jaundice (TSB/DB- 44/22) hepatosplenomegaly and hypoxemia with mild respiratory distress. Maternal history revealed high grade fever with low cell count during 5th month of gestation, though COVID RAT was negative and RT-PCR was not done. Baby was managed in NICU with IV antibiotics, IV fluids, oxygen by prongs, oral fat-soluble vitamin supplementation, adequate calorie intake and phenobarbitone. Baby responded very well to the treatment and discharged in healthy condition after 7 days. Paucity of data documenting in utero COVID-19 infection as cause of neonatal hepatitis led us to report this rare case.

Fat-soluble vitamin assessment, deficiency and supplementation in infants with cholestasis.

Infants with cholestasis are at risk of fat-soluble vitamin deficiency. The present study amied to review practice relating to the assessment, deficiency and supplementation of fat-soluble vitamins in infants with cholestasis. The medical records of all newly diagnosed infants with cholestasis (conjugated bilirubin >17 mmolL-1 />20% total bilirubin) at King's College Hospital between 2017 and 2019 were reviewed. Data extracted included bilirubin, serum vitamin concentrations (A, D, E), international normalised ratio and evidence of supplementation at initial assessment, as well as at 3 and 6 months. Rates of vitamin assessment, deficiency and supplementation were compared using chi-squared or Fisher's exact test. In total, 136 infants (87 male) with idiopathic neonatal cholestasis (n = 62), biliary atresia (n = 40) and other aetiology (n = 34) were included. Assessment of serum vitamins (A, D, E) was low (33.3%-52.2%) and deficiency was initially high for vitamin D (60.6%) and vitamin E (70.9%). Supplementation prevalence at initial assessment was high (A, E, K), but dropped significantly at 3 and 6 months for vitamin E (p = 0.003) and vitamin K (p = 0.001), whereas vitamin D supplementation was consistently low throughout (25%-33.3%). Infants with biliary atresia were more likely to have vitamins assessed (3 months), be deficient initially (D, E) and supplemented (E, K) throughout. Supplementation continued in up to 80% of infants despite cholestasis resolving. Supplementation was generally high and continued in many despite cholestasis resolving. Deficiency of vitamin D and vitamin E was high at initial assessment, although lower at follow-up. Actual prevalence of deficiency of all vitamins is unknown because monitoring was not consistently performed.

Chylomicron retention disease caused by a new pathogenic variant in sar1b protein: a rare case report from Syria

BackgroundChylomicron retention disease (Anderson disease) is a result for variant of the SAR1B gene. It is a rare autosomal recessive hereditary disorder with most incidence in infant. It is characterized by lipid malabsorption syndrome with fatty, chronic diarrhea, and growth retardation.Case presentationWe report a case of a 19-month Syrian boy who presented with vomiting, growth failure, and chronic, fatty diarrhea. Upper gastrointestinal endoscopy showed whitish appearing duodenal mucosa and small intestinal biopsies revealed steatosis of enterocytes. Genetic testing confirmed chylomicron retention disease with the first description of variant located in the fourth helix of sar1b protein. The patient is treated with nutritional supplements and fat-soluble vitamin supplementation resulting in significant improvement.ConclusionEarly endoscopy is recommended in infants with persistent vomiting and failure to thrive due to high suspicion for a disorder of hypocholesterolemia. Early diagnosis and treatment are essential to avoid serious clinical complications, especially neurological impairment.

A Rare Cause of Fat Malabsorption in Children: Chylomicron Retention Disease: A Case Report

Background: Chylomicron retention disease is an autosomal recessive disorder causing malabsorption of intestinal fat. It is extremely rare. This report is presented because although the condition is rare in cases presenting with fatty diarrhea, inability to gain weight, and abdominal distension, it should nevertheless be considered. Case Presentation: A four-month-old girl presented due to diarrhea, inability to gain weight, and lack of appetite since birth at Cukurova University, Pediatric gastroenterology outpatient clinic, Adana, Turkey. No blood or mucus was present in stool. Her general condition was average, she appeared pale and lethargic, the abdomen was distended, and no organomegaly was present. Duodenal pathology was normal but electron microscopy revealed that the enterocytes were filled with fat globules. Causes leading to fat malabsorption were investigated, and homozygous mutation was determined in the SAR1B, chylomicron retention disease, gene. Conclusion: The patient was treated with a low-fat diet and fat-soluble vitamin supplementation resulting in significant improvement. Although it is rarely seen in patients presenting with chronic diarrhea, chylomicron retention disease should be kept in mind in the differential diagnosis.

New Classification and Management of Abetalipoproteinemia and Related Disorders

New Classification and Management of Abetalipoproteinemia and Related Disorders

Fat-Soluble Vitamin Supplementation Levels in Diets for Laying Hens from 28 to 44 Weeks of Age

ABSTRACT This study aimed to examine the impact of levels of dietary supplementation with fat-soluble vitamins on the production performance and egg quality of laying hens. Three hundred Hy-Line White W-36 laying hens were evaluated from 28 to 44 weeks of age. The birds were allotted to one of six treatments in a randomized block design with 10 replicates with five birds each. Performance and egg quality parameters were evaluated in four 28-day periods. A corn and soybean meal-based basal diet was formulated so as to meet the nutritional requirements of the animals, with the exception of fat-soluble vitamins. The treatments consisted of dietary supplementation with 0%, 33.3%, 66.7%, 100.0%, 133.3% or 166.7% of fat-soluble vitamins (100% supplementation consisted of 7500 IU, 2000 IU, 10 IU and 1.8 mg of vitamins A, D3, E and K per kilogram of diet, respectively). Eggshell weight, shell thickness, shell strength, feed intake, egg weight, feed conversion per egg mass and feed conversion per dozen eggs showed a quadratic response (p≤0.05) to the treatments, whereas egg mass responded linearly. Optimal results were obtained at an average fat-soluble vitamin supplementation level of 109%, which corresponds to 8175 IU of vitamin A, 2180 IU of vitamin D3, 10.9 IU of vitamin E and 1.96 mg of vitamin K per kilogram of diet.

Growth and Nutrition in Cystic Fibrosis.

Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

Primary intestinal lymphangiectasia in a 23-month- old girl

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy which is defined as dilation of existing mucosal, submucosal, or subserosal lymphatics within the gastrointestinal tract. That causes loss of lymph fluid into the gastrointestinal tract, leading to the development of hypoproteinemia, edema, lymphocytopenia, hypogammaglobinemia, and immunologic anomalies. It is usually diagnosed in patients younger than 3 years old and is rarely first diagnosed in adulthood. Here we have a case report in a 23-month- old female presented with the complaint of peripheral edema and diarrhea. The diagnosis of PIL was made through upper gastrointestinal endoscopy and pathology histologic analysis. Patient placed on oral supplements of medium-chain triglycerides, a high protein diet, supplements of fat-soluble vitamins and responded well.

Effects of Fat-soluble Vitamins Supplementation on Common Complications and Neural Development in Very Low Birth Weight Infants

Effects of Fat-soluble Vitamins Supplementation on Common Complications and Neural Development in Very Low Birth Weight Infants

Nutritional Status in the First 2 Years of Life in Cystic Fibrosis Diagnosed by Newborn Screening.

To evaluate nutritional status and associated factors in a cystic fibrosis (CF) cohort diagnosed by newborn screening and followed up to month 24. A prospective longitudinal multicenter study assessing nutritional status according to pancreatic status, feeding modalities, prescriptions, pulmonary outcome, and biological nutritional parameters. One hundred and five infants were recruited and 99 completed the study. Nutritional care management prevented undernutrition and stunting in those with exocrine pancreatic sufficiency (EPS), but affected (13/87) 15% and (21/86) 24%, respectively, of infants with exocrine pancreatic insufficiency (EPI). The logistic regression model found a positive association between both weight and length z scores "at risk" at month 24, and initial pulmonary symptoms (odds ratio [OR] 0.06, P < 0.01 and OR 0.08, P < 0.01, respectively); these symptoms were less frequent when age at first visit was earlier than 1.2 months (33% vs 67%, P = 0.02); stunting was also associated with high-calorie density intake and Staphylococcus aureus (OR 0.05, P = 0.01 and OR 0.17, P < 0.01). Pulmonary outcome did not differ according to pancreatic status; breast-feeding for at least 3 months delayed first acquisition of Pseudomonas aeruginosa. Despite sodium and fat-soluble vitamin supplementation, half of both cohorts had low urinary sodium output and half of the EPI cohort had low vitamin D levels. Our data shed light on the fact that stunting was more frequent than undernutrition, while both parameters involved only patients with pancreatic insufficiency. Modalities of feeding were not associated with nutritional status; breast-feeding may provide some protection against acquisition of P aeruginosa.

309 Prospective randomized open label comparison of fat soluble and water soluble vitamin supplementation in children with CF

309 Prospective randomized open label comparison of fat soluble and water soluble vitamin supplementation in children with CF

Caracterización de los pacientes con Fibrosis Quística diagnosticados por cloruros en Sudor

Antecedentes y Objetivo: La Fibrosis Quística (FQ) es una enfermedad genética de herencia autosómica recesiva, caracterizada por disfunción de las glándulas de secreción exocrina. En Honduras no hay ningún tipo de revisión sobre este tema, por lo que se realizó este estudio con el objetivo de caracterizar los pacientes diagnosticados con FQ mediante prueba de Cloruros en Sudor.Pacientes y Métodos: Estudio descriptivo con enfoque cuantitativo, se revisaron expedientes clínicos y se aplicó un instrumento de 20 preguntas a pacientes en control médico, durante enero 2010 a junio 2015.Resultados: Se presentan cinco pacientes con diagnóstico de fibrosis quística. La edad al momento del diagnóstico varió de Recién nacido a 5 años; 2 de ellos con antecedentes familiares para dicha enfermedad. Al inicio de la enfermedad 3 tenían sintomatología respiratoria y digestiva. En relación a talla/edad al momento del diagnóstico, 4 se encontraban por debajo del percentil Z-3. La totalidad reportó cultivos positivos para Pseudomona aureginosa. Respecto al manejo terapéutico de los pacientes en la totalidad de ellos se indicó nebulizaciones con Salino hipertónico al 7%, Beta-2-agonistas, antibióticos sistémicos y suplementación enzimática y de vitaminas liposoluble. En 2 pacientes, se encontró la mutación ΔF508 en el gen CFTR.Conclusiones: La Fibrosis Quística todavía es una patología en gran medida desconocida. Se recomienda implementar el tamizaje neonatal para ayudar al diagnóstico temprano y la prueba de cloruros en sudor en pacientes de mayor edad y con alta sospecha clínica de esta enfermedad.Acta Pediátrica Hondureña, Vol. 6, No. 2 /octubre 2015 a marzo 2016: 486-492