Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System.

Vitamin A is a fundamental micronutrient that regulates various cellular patterns. Vitamin A deficiency (VAT) is a worldwide problem and the primary cause of nocturnal blindness especially in low income countries. Cystic fibrosis (CF) is a known risk factor of VAD because of liposoluble vitamin malabsorption due to pancreatic insufficiency. We describe a case of a 9-year-old girl who experienced recurrent episodes of nocturnal blindness due to profound VAD. This little girl is paradigmatic for the explanation of the key role of the gut-liver axis in vitamin A metabolism. She presents with meconium ileus at birth, requiring intestinal resection that led to a transient intestinal failure with parenteral nutrition need. In addition, she suffered from cholestatic liver disease due to CF and intestinal failure-associated liver disease. The interaction of pancreatic function, intestinal absorption and liver storage is fundamental for the correct metabolism of vitamin A.

Redefining short bowel syndrome in the 21st century.

In 1968, Wilmore and Dudrick reported an infant sustained by parenteral nutrition (PN) providing a potential for survival for children with significant intestinal resections. Increasing usage of TPN over time led to some patients developing Intestinal Failure Associated Liver Disease (IFALD), a leading cause of death and indication for liver/intestinal transplant. Over time, multidisciplinary teams called Intestinal Rehabilitation Programs (IRPs) began providing meticulous and innovative management. Usage of alternative lipid emulsions and lipid minimization strategies have resulted in the decline of IFALD and an increase in long-term and transplant-free survival, even in the setting of ultrashort bowel (< 20 cm). Autologous bowel reconstructive surgeries, such as the serial tapering enteroplasty procedure, have increased the likelihood of achieving enteral autonomy. Since 2007, the number of pediatric intestinal transplants performed has sharply declined and likely attributed to the newer innovations healthcare. Recent data support the need for changes in the listing criteria for intestinal transplantation given the overall improvement in outcomes. Over the last 50 y, the diagnosis of short bowel syndrome has changed from a death sentence to one of hope with a vast improvement of quality of life and survival.

Study of teduglutide effectiveness in parenteral nutrition-dependent short-bowel syndrome subjects.

Loss of intestinal absorptive capacity from congenital defect, surgical resection or mucosal disease results in short bowel syndrome (SBS)-associated intestinal failure. In the past, few medical management options were available besides dietary modification, controlling diarrhea or high stomal output, and providing parenteral fluid, electrolyte and nutrient support (parenteral support). Recent research on strategies to enhance the intestinal absorptive capacity focused on glucagon-like peptide-2, an intestinotrophic hormone that has been shown to increase the villus height and crypt depth, and decrease gastric motility and intestinal secretory losses. STEPS is a Phase III randomized double-blinded controlled trial in which teduglutide, a recombinant analog of glucagon-like peptide-2, or placebo was given subcutaneously to SBS patients for 24 weeks. A clinically meaningful response, defined as a 20-100% reduction in parenteral support volume, was achieved in 63% of the treatment group compared with 30% in the placebo group (p = 0.002) without an increase in serious side effects. Teduglutide offers a new targeted approach to SBS-associated intestinal failure management. Its specific role in clinical practice remains to be evaluated.

[A case of hypomagnesemia linked to refractory hypokalemia and hypocalcemia with short bowel syndrome].

We report a case of a 59-year old Japanese woman with short bowel syndrome, whose hypokalemia and hypocalcemia were successfully treated with magnesium (Mg) supplementation. Two years previously, she underwent Mile's operation for advanced rectal cancer, which could have been the cause of subsequent extensive resection of the small intestine by strangulation. After serial resection, she gradually developed chronic diarrhea and anorexia. Three weeks before admission, she developed general fatigue and tetany, and was hospitalized at another hospital. On admission, her serum K and Ca were 2.5 mEq/L and 4.3 mg/dL, respectively, hence regular fluid therapy containing potassium (K) and calcium (Ca) was provided following admission. However, her hypokalemia and hypocalcemia persisted, and she also displayed renal dysfunction and thereafter was transferred to our department for further evaluation and treatment. Since the laboratory tests revealed severe hypomagnesemia (0.4 mg/dL), we started intravenous Mg supplementation together with fluid therapy containing K and Ca. After the combination therapy, her clinical symptoms and electrolyte disorders were remarkably improved within a week. As Mg is essential for PTH secretion in response to hypocalcemia and to inhibit the K channel activity that controls urinary K excretion, hypomagnesemia can cause hypocalcemia and hypokalemia, which is refractory to repletion therapy unless Mg is administered. Therefore, for patients who present with signs of Mg deficiency, early and accurate diagnosis of Mg deficiency should be made and corrected.

Reversal of severe parenteral nutrition-associated liver disease in an infant with short bowel syndrome using parenteral fish oil (Omega-3 fatty acids).

Total parenteral nutrition is an important adjunct in the care of neonates with surgical disorders. Cholestasis is at present the most worrisome complication of this technique; it is difficult to treat and may progress to eventual cirrhosis and liver failure. This article reviews the pertinent clinical and nutritional data in a surgical patient with short bowel syndrome who developed parenteral nutrition-associated liver disease successfully treated with fish-oil based lipids.

Safety and efficacy of a fish-oil-based fat emulsion in the treatment of parenteral nutrition-associated liver disease.

BACKGROUND: Parenteral nutrition-associated liver disease can be a progressive and fatal entity in children with short-bowel syndrome. Soybean-fat emulsions provided as part of standard parenteral nutrition may contribute to its pathophysiology. METHODS: We compared safety and efficacy outcomes of a fish-oil-based fat emulsion in 18 infants with short-bowel syndrome who developed cholestasis (serum direct bilirubin level of > 2 mg/dL) while receiving soybean emulsions with those from a historical cohort of 21 infants with short-bowel syndrome who also developed cholestasis while receiving soybean emulsions. The primary end point was time to reversal of cholestasis (3 consecutive measurements of serum direct bilirubin level of < or = 2 mg/dL). RESULTS: Among survivors, the median time to reversal of cholestasis was 9.4 and 44.1 weeks in the fish-oil and historical cohorts, respectively. Subjects who received fish-oil-based emulsion experienced reversal of cholestasis 4.8 times faster than those who received soybean emulsions and 6.8 times faster in analysis adjusted for baseline bilirubin concentration, gestational age, and the diagnosis of necrotizing enterocolitis. A total of 2 deaths and 0 liver transplantations were recorded in the fish-oil cohort and 7 deaths and 2 transplantations in the historical cohort. The provision of fish-oil-based fat emulsion was not associated with essential fatty acid deficiency, hypertriglyceridemia, coagulopathy, infections, or growth delay. CONCLUSIONS: Parenteral fish-oil-based fat emulsions are safe and may be effective in the treatment of parenteral nutrition-associated liver disease.

Hyperbaric oxygen as a treatment for malabsorption in a radiation-damaged short bowel.

Radiation enteritis can be challenging to diagnose and treat. We report the case of a 44-year-old woman who was diagnosed with a squamous cell carcinoma of the cervix in 1978 and treated with hysterectomy and post-operative radiotherapy. Over the next 20 years she required multiple intestinal operations resulting in short bowel syndrome. She became symptomatic of severe hypomagnesaemia which could not be corrected with oral supplementation and which required intravenous magnesium sulfate every 5-7 days for an 11-month period. However, following 25 sessions of hyperbaric oxygen therapy, she was able to discontinue intravenous magnesium and maintain her serum magnesium level with oral treatment. Her weight and stoma output improved. For over 4 years subsequent to this therapy she has not required further intravenous magnesium although has needed temporary nutritional support. Her case is complicated by vitamin A, B and D deficiencies.

Síndrome de intestino corto en adultos. Tratamiento nutricional / The short bowel syndrome in adults. Nutritional treatment

El síndrome de intestino corto es un cuadro clínico caracterizado por hipoabsorción grave que aparece fundamentalmente tras resecciones intestinales extensas. Como complicaciones fundamentales, produce diarrea, desnutrición, pérdida de peso, deshidratación y déficit de electrolitos, vitaminas y oligoelementos. En el intestino se producen cambios adaptativos para aumentar la absorción de nutrientes. Mientras tanto, los enfermos precisarán un soporte nutricional adecuado, inicialmente con nutrición parenteral total, y algunos la necesitarán toda la vida. Los avances en el campo de la nutrición han mejorado el pronóstico de estos pacientes. Se están estudiando también diversas técnicas quirúrgicas. La más desarrollada es el trasplante intestinal, aunque, debido a su alta morbimortalidad, todavía está indicado sólo en los pacientes con síndrome de intestino corto que precisen mantenerse con nutrición parenteral domiciliaria y hayan desarrollado complicaciones importantes relacionadas con ésta o con el fallo intestinal (AU)

Terapia nutricional enteral na síndrome do intestino curto com fórmula infantil elementar / Enteral nutrition therapy in short bowel syndrome with infantile elemental diet

A Síndrome do Intestino Curto (SIC) se caracteriza por importante estado de má absorçäo de nutrientes, que resulta de uma reduçäo substancial do intestino delgado. Este estudo é um relato de caso de um paciente com SIC, com história de ampla rececçäo intestinal. Paciente do sexo masculino, 23 meses, negro, com desnutriçäo grave, peso de internaçäo 6460 g. Avia de acesso para a nutriçäo foi nasogástrica, sonda de polietileno, infusäo contínua e a dieta utilizada foi uma formula infantil elementar (NEOCATE). A oferta calórica inicial foi de 100 kcal/kg/dia e a propéica foi de 3,0 g/kg/dia, com evoluçäo dependente do ganho ponderal e da readaptaçäo intestinal, alcançando oferta calórica final de 200 kcal/kg/dia e protóica de 6 kcal/kg/dia. O paciente foi mantido em balanço metabólico, com acompanhamento diário de peso, ingestäo e perdas. Cuidados das lesöes infectadas nas ostomias, avaliaçäo nutricional bioquímica, antropométrica e clínica constante foram realizadas. Apesar do paciente ter sido encaminhado inicialmwente para Nutriçäo Parenteral Total (NPT), a via enteral foi utilizada com o uso de uma dieta elementar como última escolha antes da NPT. O paciente foi abordado com sucesso, exclusivamente com nutriçäo enteral. Apresentou curva enteral ascendente, com recuperaçäo do estado geral e nutricional, alcançando no quinquagésimo quarto DHI o peso de 8540 g, que possibilitou um teto cirúrgico necessário para a reconstruçäo do transito intestinal. Apesar do encaminhamento inadequado e do manejo inicial incorreto, o estabelecimento de uma terapia nutricional eficaz foi capaz de proporcionar a sobrevida de um paciente absolutamente viável.(au)

In vivo measurement of intestinal absorption using 3-0 methylglucose in short bowel syndrome.

PURPOSE: The management of patients with short bowel syndrome is complicated by the paucity of methods to assess in vivo the absorptive capacity of the remaining bowel. The purpose of this experiment was to assess the feasibility of using urinary recovery of 3-0 methylglucose (3-0 MG) as a quantitative measure of carbohydrate absorptive capacity, comparing it with in vivo absorption and in vitro glucose transport studies. METHODS: Male Sprague Dawley rats underwent either a 90% proximal small bowel resection or sham resection (n = 8 in each group). Animals were pair fed, weighed, and followed up for 14 days. A 3-day balance study was done, measuring feed intake and fecal output for percentages of fat and energy absorption. Animals were gavaged with 3-0 MG/Mannitol solution, and 4-hour urinary recovery of sugars was assessed using high-performance liquid chromatography (HPLC). On different days these studies were repeated with increasing amounts of added normal glucose (1 mol/L, 1.25 mol/L, and 1.5 mol/L) in the gavage solution given to compete for 3-0 MG transport, and thus increase the "sensitivity" of the test. Animals were then killed, and sections of intestine taken for in vitro assessment of glucose transport using radiolabeled 3-0 MG in Ussing chambers. RESULTS: Total energy, carbohydrate, and fat absorption all were reduced significantly in the resected animals, as was 3-0 MG urinary recovery (62.9 +/- 10.5%) in controls versus (35.8 +/- 17.5%) in resected animals (P <.05). 3-0 MG urinary recovery correlated well with dietary carbohydrate absorption (r = 0.74), and with Ussing chamber measures of glucose flux (r = 0.97). Adding exogenous glucose to the test solution to "compete" for 3-0 MG transport sites did not improve sensitivity. CONCLUSIONS: These results show that 3-0 MG is useful in measuring nutrient absorption capacity in rats after massive small bowel resection. Further studies to validate these methods in human patients with short bowel syndrome are suggested.