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1.
JAMA ; 332(5): 430, 2024 08 06.
Artigo em Inglês | MEDLINE | ID: mdl-38990558
2.
Curr Opin Clin Nutr Metab Care ; 23(2): 138-144, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31977336

RESUMO

PURPOSE OF REVIEW: This is a review of the research on the effectiveness of vitamin supplementation for alcoholism and alcohol-related illnesses. The focus is on research, both clinical and basic on alcohol treatment and nutritional effectiveness of these vital nutrients. RECENT FINDINGS: Most of the research involves basic experiments exploring the impact of vitamin depletion or deficits on physiological systems, especially liver and brain, in rodents. These often include behavioral measures that use cognitive, learning/memory and motivation experiments that model clinical studies. These provide support for hypotheses concerning the impact of such deficiencies in clinical populations. Clinical studies are rare and involve evaluation of the outcome of supplementation usually in the context of a treatment program. Specific vitamins, dosages and treatment programs vary. Deficiencies in retinoids (vitamin A), thiamine (B1) and niacin (B3) are the most frequently investigated. However, there is a greater need for further research on other vitamins, and for more uniform supplementation and treatment procedures. SUMMARY: The literature is primarily basic research on specific vitamins. There are very significant findings with individual vitamin supplementation and combinations that show promise of our understanding of the role of vitamins in the disease of alcoholism and its treatment.


Assuntos
Alcoolismo/terapia , Deficiência de Vitaminas/terapia , Suplementos Nutricionais , Vitaminas/uso terapêutico , Alcoolismo/complicações , Animais , Deficiência de Vitaminas/etiologia , Modelos Animais de Doenças , Humanos , Niacina/uso terapêutico , Estado Nutricional , Tiamina/uso terapêutico , Resultado do Tratamento , Vitamina A/uso terapêutico
3.
Nutr Clin Pract ; 35(1): 50-62, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31840874

RESUMO

Micronutrients are essential components of the diet and are required to maintain fundamental bodily functions. Liver disease has a profound effect on nutrient intake, metabolism of nutrients, and nutrition status, often resulting in some degree of malnutrition, including micronutrient deficiency. Vitamin and mineral deficiencies can impair metabolic processes at the cellular and biochemical level even before clinical and physical alterations are seen. It is essential that micronutrient status is evaluated as part of a comprehensive nutrition assessment for all patients with chronic or advanced liver disease. Early intervention to correct suspected or confirmed deficiencies may minimize symptoms and improve clinical outcomes and quality of life. In this narrative review, different types of liver disease and associated micronutrient abnormalities are outlined, and methods of micronutrient assessment and supplementation are discussed.


Assuntos
Hepatopatias/epidemiologia , Hepatopatias/terapia , Micronutrientes/deficiência , Micronutrientes/uso terapêutico , Deficiência de Vitaminas/epidemiologia , Deficiência de Vitaminas/terapia , Dieta , Suplementos Nutricionais , Ingestão de Energia , Humanos , Hepatopatias/fisiopatologia , Desnutrição/epidemiologia , Desnutrição/terapia , Estado Nutricional , Qualidade de Vida , Fatores de Risco , Oligoelementos/deficiência , Vitaminas/uso terapêutico
4.
JAAPA ; 33(1): 28-32, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31880647

RESUMO

Type 2 diabetes is primarily managed with lifestyle modifications, self-monitoring of blood glucose, and medication. The goal is to maintain A1C less than 7% in most patients and prevent damage to other organs such as the kidneys and heart. Patients who are obese and cannot achieve normal blood glucose levels despite diet, exercise, and multiple medications may be considered for bariatric surgery. The Roux-en-Y gastric bypass and laparoscopic sleeve gastrectomy have been shown to improve A1C, reduce weight, and reduce the number of medications patients need for diabetes management. Comorbidities such as hyperlipidemia and hypertension also may improve. This article describes types of bariatric surgery, proper selection of surgical candidates, patient education, and the postoperative patient management necessary for long-term success in improving blood glucose control.


Assuntos
Cirurgia Bariátrica , Diabetes Mellitus Tipo 2/metabolismo , Obesidade/cirurgia , Deficiência de Vitaminas/epidemiologia , Deficiência de Vitaminas/prevenção & controle , Deficiência de Vitaminas/terapia , Diabetes Mellitus Tipo 2/complicações , Hemoglobinas Glicadas/metabolismo , Humanos , Hipoglicemia/epidemiologia , Hipoglicemia/prevenção & controle , Hipoglicemia/terapia , Hipoglicemiantes/uso terapêutico , Estilo de Vida , Síndromes de Malabsorção/epidemiologia , Síndromes de Malabsorção/prevenção & controle , Síndromes de Malabsorção/terapia , Obesidade/complicações , Obesidade/metabolismo , Educação de Pacientes como Assunto , Seleção de Pacientes , Cuidados Pós-Operatórios , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/terapia , Redução de Peso , Programas de Redução de Peso
5.
Curr Neurol Neurosci Rep ; 19(12): 101, 2019 11 26.
Artigo em Inglês | MEDLINE | ID: mdl-31773293

RESUMO

PURPOSE OF REVIEW: The goal of this chapter is to educate clinicians on the neurologic manifestations of certain nutritional deficiencies in order to promptly identify and appropriately treat these patients. RECENT FINDINGS: Many vitamin and nutritional deficiencies have been described dating back to the early days of neurology and medicine. Some are very rare and thus, there are no randomized controlled studies to assess supplementation or dosage; however, there are reviews of case reports that can assist clinicians in choosing treatments. While endemic vitamin and nutritional deficiencies may be rarely encountered in many countries, vulnerable populations continue to be at risk for developing neurologic complications. These populations include those with diseases causing malabsorption, the elderly, chronic alcohol users, as well as pregnant mothers with hyperemesis gravidarum to name a few. It is important to recognize syndromes associated with these nutritional deficiencies, as prompt identification and treatment may prevent permanent neurologic damage.


Assuntos
Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/epidemiologia , Distúrbios Nutricionais/diagnóstico , Distúrbios Nutricionais/epidemiologia , Deficiência de Vitaminas/diagnóstico , Deficiência de Vitaminas/epidemiologia , Deficiência de Vitaminas/terapia , Humanos , Desnutrição/diagnóstico , Desnutrição/epidemiologia , Desnutrição/terapia , Doenças do Sistema Nervoso/terapia , Neurologia , Distúrbios Nutricionais/terapia
6.
Gastroenterol Clin North Am ; 48(4): 483-498, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31668178

RESUMO

Pediatric intestinal failure occurs when gut function is insufficient to meet the growing child's hydration and nutrition needs. After massive bowel resection, the remnant bowel adapts to lost absorptive and digestive capacity through incompletely defined mechanisms newly targeted for pharmacologic augmentation. Management seeks to achieve enteral autonomy and mitigate the development of comorbid disease. Care has improved, most notably related to reductions in blood stream infection and liver disease. The future likely holds expansion of pharmacologic adaptation augmentation, refinement of intestinal tissue engineering techniques, and the development of a learning health network for efficient multicenter study and care improvement.


Assuntos
Síndrome do Intestino Curto/terapia , Adaptação Fisiológica , Deficiência de Vitaminas/etiologia , Deficiência de Vitaminas/terapia , Cateterismo Venoso Central/efeitos adversos , Criança , Endoscopia do Sistema Digestório , Nutrição Enteral , Fármacos Gastrointestinais/uso terapêutico , Humanos , Hepatopatias/etiologia , Hepatopatias/terapia , Micronutrientes/deficiência , Nutrição Parenteral , Equipe de Assistência ao Paciente , Síndrome do Intestino Curto/etiologia
7.
Rev Assoc Med Bras (1992) ; 65(9): 1151-1155, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31618329

RESUMO

This report describes the post-bariatric-surgery evolution of an obese patient who had low adherence to the diet and micronutrient supplementation. Four years after two bariatric surgeries, the patient was admitted due to transient loss of consciousness, slow thinking, anasarca, severe hypoalbuminemia, in addition to vitamin and mineral deficiencies. She had subcutaneous foot abscess but did not present fever. Received antibiotics, vitamins A, D, B12, thiamine, calcium, and parenteral nutrition. After hospitalization (twenty-eight days), there was a significant body weight reduction probably due to the disappearance of clinical anasarca. Parenteral nutrition was suspended after twenty-five days, and the oral diet was kept fractional. After hospitalization (weekly outpatient care), there was a gradual laboratory data improvement, which was now close to the reference values. Such outcome shows the need for specialized care in preventing and treating nutritional complications after bariatric surgeries as well as clinical manifestations of infection in previously undernourished patients.


Assuntos
Cirurgia Bariátrica , Desnutrição Proteico-Calórica/complicações , Infecções Estreptocócicas/complicações , Adulto , Deficiência de Vitaminas/complicações , Deficiência de Vitaminas/terapia , Cirurgia Bariátrica/efeitos adversos , Feminino , Humanos , Nutrição Parenteral , Complicações Pós-Operatórias , Desnutrição Aguda Grave/complicações , Desnutrição Aguda Grave/etiologia , Desnutrição Aguda Grave/terapia , Cooperação e Adesão ao Tratamento
8.
Rev. Assoc. Med. Bras. (1992, Impr.) ; Rev. Assoc. Med. Bras. (1992, Impr.);65(9): 1151-1155, Sept. 2019. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1041071

RESUMO

SUMMARY This report describes the post-bariatric-surgery evolution of an obese patient who had low adherence to the diet and micronutrient supplementation. Four years after two bariatric surgeries, the patient was admitted due to transient loss of consciousness, slow thinking, anasarca, severe hypoalbuminemia, in addition to vitamin and mineral deficiencies. She had subcutaneous foot abscess but did not present fever. Received antibiotics, vitamins A, D, B12, thiamine, calcium, and parenteral nutrition. After hospitalization (twenty-eight days), there was a significant body weight reduction probably due to the disappearance of clinical anasarca. Parenteral nutrition was suspended after twenty-five days, and the oral diet was kept fractional. After hospitalization (weekly outpatient care), there was a gradual laboratory data improvement, which was now close to the reference values. Such outcome shows the need for specialized care in preventing and treating nutritional complications after bariatric surgeries as well as clinical manifestations of infection in previously undernourished patients.


RESUMO Este relato descreve a evolução pós-cirurgia bariátrica de uma paciente obesa que apresentou baixa adesão à dieta e suplementação de micronutrientes. Quatro anos após duas cirurgias bariátricas, a paciente foi internada por perda transitória de consciência, raciocínio lento, anasarca, hipoalbuminemia grave, além de deficiências vitamínicas e minerais. Apresentava abscesso subcutâneo no pé, mas não apresentava febre. Recebeu antibióticos, vitaminas A, D, B12, tiamina, cálcio e nutrição parenteral. Após a internação (28 dias) houve redução significativa do peso corporal, provavelmente devido ao desaparecimento clínico da anasarca. A nutrição parenteral foi suspensa após 25 dias e a dieta oral foi mantida fracionada. Após a internação (atendimento ambulatorial semanal) houve uma melhora gradativa dos dados laboratoriais, que estavam próximos dos valores de referência. Tal desfecho mostra a necessidade de cuidados especializados na prevenção e tratamento de complicações nutricionais após cirurgias bariátricas, bem como manifestações clínicas de infecção em pacientes previamente desnutridos.


Assuntos
Humanos , Feminino , Adulto , Infecções Estreptocócicas/complicações , Desnutrição Proteico-Calórica/complicações , Cirurgia Bariátrica/efeitos adversos , Complicações Pós-Operatórias , Deficiência de Vitaminas/complicações , Deficiência de Vitaminas/terapia , Nutrição Parenteral , Desnutrição Aguda Grave/complicações , Desnutrição Aguda Grave/etiologia , Desnutrição Aguda Grave/terapia , Cooperação e Adesão ao Tratamento
9.
World J Gastroenterol ; 25(10): 1171-1184, 2019 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-30886501

RESUMO

Symptoms of gastroenteropancreatic located neuroendocrine neoplasms (GEP-NENs) are often related to food intake and manifest as abdominal pain or diarrhoea which can influence patients nutritional status. Malnutrition is common in cancer patients and influences quality of life, treatment options and survival but is also present in up to 40% of patients with GEP-NENs. As part of malnutrition there are often deficiencies in fat-soluble vitamins, mainly vitamin D. Little knowledge exists on trace elements. Several factors influence the development of malnutrition such as size and localisation of the primary tumour as well as metastases, side effects from treatment but also hormone production of the tumour itself. One of the main influencing factors leading to malnutrition is diarrhoea which leads to dehydration and electrolyte disturbances. Treatment of diarrhoea should be guided by its cause. Screening for malnutrition should be part of routine care in every GEP-NEN patient. Multidisciplinary treatment including dietician support is necessary for all malnourished patients with GEP-NENs.


Assuntos
Deficiência de Vitaminas/etiologia , Neoplasias Gastrointestinais/complicações , Desnutrição/etiologia , Tumores Neuroendócrinos/complicações , Estado Nutricional/fisiologia , Deficiência de Vitaminas/fisiopatologia , Deficiência de Vitaminas/terapia , Diarreia/etiologia , Diarreia/fisiopatologia , Diarreia/terapia , Neoplasias Gastrointestinais/mortalidade , Neoplasias Gastrointestinais/fisiopatologia , Humanos , Desnutrição/diagnóstico , Desnutrição/fisiopatologia , Desnutrição/terapia , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/fisiopatologia , Prognóstico , Qualidade de Vida , Oligoelementos/deficiência , Vitaminas/fisiologia
10.
Clin Liver Dis ; 22(3): 533-544, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-30259851

RESUMO

Patients with primary biliary cholangitis (PBC) are at risk for various harmful consequences of chronic cholestasis. These include fat-soluble vitamin deficiency, even in the setting of macronutrient sufficiency, as well as metabolic bone disease, including osteoporosis with fractures. Hyperlipidemia is often present and less commonly associated with risk of cardiovascular event; however, the long-term effect of new emerging therapies for PBC remains to be determined. Patients with PBC also have infrequent but notable risk of portal hypertension despite early-stage disease. This review discusses the background, evaluation, and practical management of these complications of chronic cholestasis.


Assuntos
Deficiência de Vitaminas/diagnóstico , Carcinoma Hepatocelular/diagnóstico , Hiperlipidemias/diagnóstico , Hipertensão Portal/diagnóstico , Hipertensão Portal/terapia , Cirrose Hepática Biliar/terapia , Neoplasias Hepáticas/diagnóstico , Osteoporose/diagnóstico , Absorciometria de Fóton , Deficiência de Vitaminas/etiologia , Deficiência de Vitaminas/terapia , Conservadores da Densidade Óssea/uso terapêutico , Doenças Ósseas Metabólicas/diagnóstico , Doenças Ósseas Metabólicas/tratamento farmacológico , Doenças Ósseas Metabólicas/etiologia , Carcinoma Hepatocelular/etiologia , Carcinoma Hepatocelular/terapia , Colestase/complicações , Colestase/terapia , Dieta Saudável , Detecção Precoce de Câncer , Varizes Esofágicas e Gástricas/diagnóstico , Varizes Esofágicas e Gástricas/etiologia , Varizes Esofágicas e Gástricas/terapia , Exercício Físico , Ácidos Fíbricos/uso terapêutico , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/terapia , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Hiperlipidemias/etiologia , Hiperlipidemias/terapia , Hipertensão Portal/etiologia , Cirrose Hepática Biliar/complicações , Neoplasias Hepáticas/etiologia , Neoplasias Hepáticas/terapia , Programas de Rastreamento , Osteoporose/tratamento farmacológico , Osteoporose/etiologia , Fraturas por Osteoporose/prevenção & controle , Deficiência de Vitamina A/diagnóstico , Deficiência de Vitamina A/etiologia , Deficiência de Vitamina A/terapia , Deficiência de Vitamina D/diagnóstico , Deficiência de Vitamina D/etiologia , Deficiência de Vitamina D/terapia , Deficiência de Vitamina E/diagnóstico , Deficiência de Vitamina E/etiologia , Deficiência de Vitamina E/terapia , Deficiência de Vitamina K/diagnóstico , Deficiência de Vitamina K/etiologia , Deficiência de Vitamina K/terapia
12.
Pediatr Rev ; 39(4): 161-179, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29610425

RESUMO

The published literature supports the high prevalence of supplement use in children and adolescents in the United States. Pediatricians today are faced with questions from parents and patients about the benefits, safety, efficacy, and correct dose of vitamins and minerals. In this article, we review 7 vitamins with the most clinical relevance as judged by abundance in food, risks and symptoms of deficiency, and potential for toxicity. Specifically, we focus on possible clinical scenarios that can be indicative of nutritional deficiency. We synthesize and summarize guidelines from nutrition experts, various medical societies, the World Health Organization, and the American Academy of Pediatrics.


Assuntos
Deficiência de Vitaminas , Vitaminas/efeitos adversos , Adolescente , Deficiência de Vitaminas/diagnóstico , Deficiência de Vitaminas/etiologia , Deficiência de Vitaminas/terapia , Aleitamento Materno , Criança , Dieta/efeitos adversos , Dieta/métodos , Suplementos Nutricionais , Humanos , Lactente , Pediatria , Relações Profissional-Família , Recomendações Nutricionais , Medição de Risco , Fatores de Risco , Vitaminas/uso terapêutico
13.
Obes Surg ; 28(1): 234-241, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28861696

RESUMO

BACKGROUND: Vitamin and mineral deficiencies are a major concern after biliopancreatic diversion (BPD) and BPD with duodenal switch (BPD/DS). Evidence-based guidelines how to prevent or how to treat deficiencies in these patients are currently lacking. The aim of the current study is to give an overview of postsurgical deficiencies and how to prevent and treat these deficiencies. METHODS: Retrospective evaluation of a 1-year structured monitoring and treatment schedule for various deficiencies in 34 patients after BPD or BPD/DS. RESULTS: Patients were introduced into the program 12-90 months after surgery. Vitamin B1, B6, B9, and B12 deficiencies could be prevented by mean daily doses of 2.75 mg, 980 µg, 600 µg, and 350 µg, respectively. However, many patients continued to develop deficiencies of vitamin A, D, iron, calcium, and zinc despite major dose adjustments. Current observations suggest that at least total daily doses of 200 mg Fe in premenopausal women and 100 mg in men, 100 mg of Zinc, 3000 mg of calcium, and weekly doses of at least 50,000 IU solubilized vitamin A and vitamin D are needed to prevent the occurrence of major deficiencies. CONCLUSION: Exceptionally high supplementation doses are needed to prevent and treat vitamin and mineral deficiencies in patients after BPD or BPD/DS. Further refinement and simplification of treatment schedules is needed. Focus on improvement of compliance to treatment is recommended.


Assuntos
Desvio Biliopancreático/efeitos adversos , Desnutrição/etiologia , Desnutrição/terapia , Minerais/metabolismo , Obesidade Mórbida/cirurgia , Adulto , Anastomose Cirúrgica/efeitos adversos , Anastomose Cirúrgica/métodos , Deficiência de Vitaminas/etiologia , Deficiência de Vitaminas/metabolismo , Deficiência de Vitaminas/terapia , Desvio Biliopancreático/métodos , Deficiências Nutricionais/etiologia , Deficiências Nutricionais/terapia , Duodeno/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Desnutrição/metabolismo , Pessoa de Meia-Idade , Estudos Retrospectivos
14.
J Clin Gastroenterol ; 51(10): 878-889, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28858940

RESUMO

BACKGROUND: Vitamin deficiency is frequently associated with inflammatory bowel disease (IBD). Supplementation of vitamins could thus serve as an adjunctive therapy. The present meta-analysis reviews the deficiencies and alterations in serum fat-soluble vitamins (A, D, E, and K) reported in IBD patients. MATERIALS AND METHODS: PubMed database search was performed to identify all primary studies up to January 2015 that evaluated the serum concentrations of fat-soluble vitamin levels in IBD patients compared with healthy individuals. We estimated pooled mean differences between groups and estimated their relations with some compounding variables (age, disease duration, C-reactive protein, albumin), using a meta-regression analysis. RESULTS: Nineteen case-control studies met selection criteria. In patients with Crohn's disease (CD), vitamin A, D, E, K status was lower than in controls [D=212 µg/L.92; 95% confidence interval (CI), 95.36-330.48 µg/L, P=0.0002; D=6.97 nmol/L, 95% CI, 1.61-12.32 nmol/L, P=0.01; D=4.72 µmol/L, 95% CI, 1.60-7.84 µmol/L, P=0.003; D=1.46 ng/mL, 95% CI, 0.48-2.43 ng/mL, P=0.003, respectively]. Patients with ulcerative colitis had lower levels of vitamin A than controls (D=223.22 µg/L, 95% CI, 44.32-402.12 µg/L, P=0.01). Patients suffering from CD for a longer time had lower levels of vitamins A (95% CI=7.1-67.58 y, P=0.02) and K (95% CI, 0.09-0.71 y, P=0.02). Meta-regression analysis demonstrated statistically significant associations between the levels of inflammatory biomarkers: C-reactive protein (P=0.03, 95% CI, -9.74 to -0.6 mgl/L) and albumin (P=0.0003, 95% CI, 402.76-1361.98 g/dL), and vitamin A status in CD patients. CONCLUSION: Our meta-analysis shows that the levels of fat-soluble vitamins are generally lower in patients with inflammatory bowel diseases and their supplementation is undoubtedly indicated.


Assuntos
Deficiência de Vitaminas/complicações , Colite Ulcerativa/etiologia , Doença de Crohn/etiologia , Deficiência de Vitaminas/terapia , Colite Ulcerativa/sangue , Colite Ulcerativa/terapia , Doença de Crohn/sangue , Doença de Crohn/terapia , Humanos , Vitamina A/administração & dosagem , Vitamina A/sangue , Vitamina D/administração & dosagem , Vitamina D/sangue , Vitamina E/administração & dosagem , Vitamina E/sangue , Vitamina K/administração & dosagem , Vitamina K/sangue
15.
Rev Neurol ; 64(s03): S25-S28, 2017 May 17.
Artigo em Espanhol | MEDLINE | ID: mdl-28524215

RESUMO

INTRODUCTION: Individually, neurometabolic diseases are ultra rare, but for some of them there is an effective treatment. DEVELOPMENT: Several recent therapeutic advances are reviewed. Today, the possibilities of treatment for lysosomal diseases have improved. In recent years the use of enzyme replacement therapy has become more widely extended to treat mucopolysaccharidosis type IVA (Morquio A), mucopolysaccharidosis type VII (Sly syndrome), lysosomal acid lipase deficiency and alpha-mannosidosis. It has been proven that very early treatment of mucopolysaccharidoses can change their natural course. Intrathecal enzyme replacement therapy is being tried in some mucopolysaccharidoses with cognitive involvement, in an attempt to halt neurodegeneration. Very positive results have been obtained with genetically modified autotransplants in late-onset infantile metachromatic leukodystrophy and research is being conducted on other pathologies (mucopolysaccharidosis type III, X-linked adrenoleukodystrophy). Novel outcomes are also being achieved in the treatment of some encephalopathies that are sensitive to vitamins or cofactors: triple therapy in pyridoxine dependency, treatment with thiamine for some subacute encephalopathies with involvement of the basal ganglia, treatment with folinic acid for children with cerebral folate deficiency, or treatment with cyclic pyranopterin monophosphate in molybdenum cofactor deficiency type A. CONCLUSIONS: As neuropaediatricians we must update our knowledge, especially in the case of treatable neurometabolic pathologies, since early treatment can change their prognosis significantly.


TITLE: Terapias novedosas en enfermedades neurometabolicas: importancia de una intervencion precoz.Introduccion. Las enfermedades neurometabolicas son individualmente ultrarraras, pero algunas de ellas tienen un tratamiento eficaz. Desarrollo. Se revisan algunas novedades terapeuticas. Las enfermedades lisosomales tienen actualmente mejores posibilidades de tratamiento. En los ultimos años se ha extendido el uso de la terapia enzimatica sustitutiva a la mucopolisacaridosis tipo IVA (Morquio A), a la mucopolisacaridosis tipo VII (enfermedad de Sly), al deficit de lipasa acida lisosomal y a la alfa-manosidosis. Se ha constatado que un tratamiento muy precoz de las mucopolisacaridosis puede cambiar su historia natural. Se esta probando la terapia enzimatica sustitutiva intratecal en algunas mucopolisacaridosis con afectacion cognitiva, en el intento de frenar la neurodegeneracion. Se han obtenido resultados muy positivos con autotrasplante modificado geneticamente en leucodistrofia metacromatica infantil tardia y se esta trabajando en otras patologias (mucopolisacaridosis tipo III, adrenoleucodistrofia ligada a X). Tambien hay novedades en la terapia de algunas encefalopatias sensibles a vitaminas o cofactores: la triple terapia en la dependencia de piridoxina, el tratamiento con tiamina de algunas encefalopatias subagudas con afectacion de ganglios basales, el tratamiento con acido folinico de niños con deficiencia de folato cerebral, o el tratamiento con monofosfato de piranopterina ciclico en los defectos de cofactor de molibdeno de tipo A. Conclusiones. Los neuropediatras debemos actualizar nuestro conocimiento especialmente en aquellas patologias neurometabolicas tratables, dado que una terapia precoz puede cambiar de forma significativa su pronostico.


Assuntos
Encefalopatias Metabólicas Congênitas/terapia , Intervenção Médica Precoce , Doenças do Sistema Nervoso/terapia , Terapias em Estudo , Deficiência de Vitaminas/terapia , Encefalopatias Metabólicas Congênitas/diagnóstico , Encefalopatias Metabólicas Congênitas/genética , Proteínas de Transporte/metabolismo , Criança , Ensaios Clínicos como Assunto , Coenzimas/deficiência , Coenzimas/uso terapêutico , Diagnóstico Precoce , Terapia de Reposição de Enzimas , Epilepsia/genética , Epilepsia/metabolismo , Epilepsia/terapia , Terapia Genética , Humanos , Recém-Nascido , Doenças por Armazenamento dos Lisossomos do Sistema Nervoso/tratamento farmacológico , Doenças por Armazenamento dos Lisossomos do Sistema Nervoso/genética , Doenças do Sistema Nervoso/diagnóstico
19.
Obes Surg ; 26(10): 2324-30, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-26843085

RESUMO

INTRODUCTION: After all bariatric procedures, multivitamin supplements are uniformly prescribed to minimize eventual deficiencies. These supplements are usually maintained long time, even during the whole life after malabsorptive techniques, while these are maintained at least during 1 year after restrictive procedures. Given that sleeve gastrectomy does not alter intestinal absorption, the supplements are possibly unnecessary, once the patient can take an adequate diet. PATIENTS AND METHODS: A prospective randomized study of patients undergoing a laparoscopic sleeve gastrectomy was performed. Patients were randomized into two groups: those patients receiving the multivitamin supplement (Multicentrum, Pfizer, 1 tablet/day) during 3 months (group 1) and those receiving the supplement during 12 months (group 2). Laboratory data were recorded: vitamins (D, B12 and folic acid) and oligoelements (calcium, iron, phosphorus, magnesium and zinc) at 3, 6 and 12 months after surgery. RESULTS: Eighty patients were included, 40 in each group. At 3 months, 7.5 % of the patients presented iron deficiency and 2.5 % ferritin one, similarly in both groups, that was corrected with specific extra iron supplements. At 6 months, one patient (2.5 %) in group 1 presented iron deficiency and one in group 2 vitamin D deficiency (NS). At 12 months, only one patient in group 2 presented vitamin D deficiency, treated with specific supplements. CONCLUSION: The maintenance of multivitamin supplements more than 3 months postoperatively seems to be of no benefit. It is preferable monitoring laboratory values and adding specific supplements when necessary. CLINICALTRIALS. GOV IDENTIFIER: NCT02620137.


Assuntos
Deficiência de Vitaminas/terapia , Suplementos Nutricionais , Gastrectomia/efeitos adversos , Obesidade Mórbida/cirurgia , Vitaminas/administração & dosagem , Adulto , Deficiência de Vitaminas/sangue , Deficiência de Vitaminas/etiologia , Deficiência de Vitaminas/prevenção & controle , Dieta , Feminino , Gastrectomia/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Obesidade Mórbida/sangue , Estudos Prospectivos
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