Comprehensive nutrition guidelines and management strategies for enteropathy in children.

Protein-losing enteropathy (PLE) describes a syndrome of excessive protein loss into the gastrointestinal tract, which may be due to a wide variety of etiologies. For children in whom the protein loss is associated with lymphangiectasia, medical nutrition therapy focused on restricting enteral long-chain triglycerides and thus intestinal chyle production is an integral component of treatment. This approach is based on the principle that reducing intestinal chyle production will concurrently decrease enteric protein losses of lymphatic origin. In patients with ongoing active PLE or those who are on a fat-restricted diet, particularly in infants and young children, supplemental calories may be provided with medium-chain triglycerides (MCT). MCT are absorbed directly into the bloodstream, bypassing intestinal lymphatics and not contributing to intestinal chyle production. Patients with active PLE or who are on dietary fat restriction should be monitored for associated micronutrient deficiencies. In this paper, we seek to formally present recommended nutrition interventions, principles of dietary education and patient counseling, and monitoring parameters in pediatric populations with PLE based on our experience in a busy clinical referral practice focused on this population.

Dietary and Nutritional Approaches to the Management of Chronic Enteropathy in Dogs and Cats.

Nutrition can influence those functions of the gastrointestinal tract that can be adversely affected in chronic enteropathy, such as microbiota, mucosal immune system, intestinal permeability, and motility. Diet serves as a possible risk factor in disease pathogenesis and as a target for treatment in chronic enteropathy. Malnutrition is prevalent in people with inflammatory bowel disease and negatively affects outcome. Approximately two-thirds of dogs with protein-losing enteropathy due to chronic enteropathy or lymphangiectasia are underweight. Commercial diets and home-prepared diets have been used successfully in the management of chronic enteropathy. Fat restriction is the main dietary strategy for intestinal lymphangiectasia.

Protein-Amino Acid Metabolism Disarrangements: The Hidden Enemy of Chronic Age-Related Conditions.

Proteins are macro-molecules crucial for cell life, which are made up of amino acids (AAs). In healthy people, protein synthesis and degradation are well balanced. However, in the presence of hypercatabolic stimulation (i.e., inflammation), protein breakdown increases as the resulting AAs are consumed for metabolic proposes. Indeed, AAs are biochemical totipotent molecules which, when deaminated, can be transformed into energy, lipids, carbohydrates, and/or biochemical intermediates of fundamental cycles, such as the Krebs' cycle. The biochemical consequence of hyper-catabolism is protein disarrangement, clinically evident with signs such as sarcopenia, hypalbuminemia, anaemia, infection, and altered fluid compartmentation, etc. Hypercatabolic protein disarrangement (HPD) is often underestimated by clinicians, despite correlating with increased mortality, hospitalization, and morbidity quite independent of the primary disease. Simple, cheap, repeatable measurements can be used to identify HPD. Therefore, identification and treatment of proteins' metabolic impairment with appropriate measurements and therapy is a clinical strategy that could improve the prognosis of patients with acute/chronic hypercatabolic inflammatory disease. Here, we describe the metabolism of protein and AAs in hypercatabolic syndrome, illustrating the clinical impact of protein disarrangement. We also illustrate simple, cheap, repeatable, and worldwide available measurements to identify these conditions. Finally, we provide scientific evidence for HPD nutritional treatment.

Dietary management of presumptive protein-losing enteropathy in Yorkshire terriers.

OBJECTIVES: To describe the clinical outcome of dietary management of Yorkshire terriers with protein-losing enteropathy without immunosuppressive/anti-inflammatory medications. METHODS: Records were searched for Yorkshire terriers with hypoalbuminaemia and a clinical diagnosis of protein-losing enteropathy that were managed with diet and without immunosuppressive/anti-inflammatory medications. Serum albumin changes were compared using a one-way repeated measures ANOVA. Canine chronic enteropathy clinical activity index scores were compared using a Wilcoxon signed-rank test. RESULTS: Eleven cases were identified. Clinical signs were variable including: diarrhoea, respiratory signs, vomiting, lethargy and weight loss. Diets fed included home cooked (n=5); Royal Canin Gastrointestinal Low Fat (n=4); Hill's Prescription Diet i/d Low Fat (n=1); or Purina HA Hypoallergenic (n=1). Clinical signs resolved completely in eight dogs, partially resolved in two dogs and failed to respond in one dog. In dogs that responded, albumin significantly improved from baseline (mean 14·9 g/L, sd ±3·7), at 2 to 4 weeks (mean 24·2 g/L, sd ±5·5, P=0·01), and at 3 to 4 months (mean 27·0 g/dL, sd ±5·9, P=0·01). CLINICAL SIGNIFICANCE: These results indicate that dietary management of protein-losing enteropathy is a potential management strategy in Yorkshire terriers. Randomised clinical trials in Yorkshire terriers with protein-losing enteropathy are necessary to compare success rate, survival and quality of life with dietary management versus combined dietary and immunosuppressive/anti-inflammatory therapy.

[Protein-losing enteropathy due to intestinal lymphangiectasis: a rare disease. Report of two cases]. / Enteropatía perdedora de proteínas por linfangiectasia intestinal: una enfermedad rara. Presentación de 2 casos.

Congenital intestinal lymphangiectasis (LIP) is a protein-losing enteropathy that appears sporadically in children. It begins with edema due to hypoproteinemia and hypoalbuminemia, and in some cases with ascites, immunodeficience and hypocalcemic tetania. The purpose of this report is to present two patients with LIP which appeared during the first year of life. The diagnosis was certificated by upper gastrointestinal videoendoscopy and histological findings. Both patients were treated with a new formula containing mean chain triglycerides with an adequate response, not obtained before with a common semielemental formula.

Protein-losing enteropathy following the Fontan procedure in a child with intestinal lactase deficiency treated with lactose-free diet.

UNLABELLED: A 12-year-old girl presented with a protein-losing enteropathy. Symptoms started 4 weeks after undergoing the Fontan procedure at the age of 1.5 years for mitral atresia, ventricular septal defect, and double-outlet right ventricle. Upon referral for 3 weeks of rehabilitation after multiple interventional measures and drug treatments, she appeared in a dystrophic state, with decreased plasma protein and electrolyte levels along with occasional tetanic convulsions. Blood glucose levels after a lactose tolerance test were markedly reduced. The introduction of a lactose-free diet was quickly effective, with plasma protein and electrolyte levels raised to normal levels, and the girl's body weight increased without ascites or oedema. Molecular genetic examination revealed a homozygous C/C13910 polymorphism in the LCT gene. CONCLUSION: Protein-losing enteropathy in the Fontan circulation may be provoked by lactase deficiency and should therefore be ruled out to exclude this rather common condition.

Focus on nutrition: Nutritional management of protein-losing nephropathy in dogs.

Optimal treatment of protein-losing nephropathy (PLN) should address both medical and nutritional issues. In nonazotemic dogs with PLN, the main nutrients of concern are protein, calories, omega-3 fatty acids, and sodium. In azotemic dogs with PLN, requirements for additional nutrients should be addressed. The amount of protein and the specific diet must be individualized for every patient with PLN because commercial dog foods differ greatly in protein and other nutrients. It is critical to avoid excessive dietary protein restriction, which may contribute to loss of lean body mass. A thorough diet history must be obtained to account for the animal's entire daily intake of protein and other nutrients.

Importancia de la nutrición en enfermos con encefalopatía hepática / Importance of nutritional support in patients with hepatic encephalopathy

La desnutrición es una complicación frecuente que influye negativamente en el pronóstico del enfermo con cirrosis hepática. La disminución de la ingesta junto con la aparición de diversas alteraciones endocrino-metabólicas condicionan un estado hipercatabólico que precisa de un mayor aporte energético. Una de las complicaciones que puede aparecer en la fase de cirrosis descompensada es la encefalopatía hepática. El reconocido papel del amonio en la patogenia de la encefalopatía hepática ha condicionado durante muchos años una restricción en el aporte de proteínas de estos enfermos. Sin embargo, no existe evidencia de que una dieta baja en proteínas mejore el curso de la encefalopatía hepática y sí de que empeore el estado nutricional y favorezca la aparición de distintas complicaciones relacionadas con la desnutrición. En este trabajo, se revisa el uso de aminoácidos ramificados y de proteínas de diferente origen, probióticos y simbióticos, antioxidantes, L-Ornitina-L-Aspartato, acetil-L-carnitina en enfermos con encefalopatía hepática (AU)

Protein calorie malnutrition is frequently a complication in the chronic liver disease patient and is considered to be a negative prognostic factor. Anorexia and several other endocrine metabolic complications produce an hypermetabolic state that needs more caloric intake. Hepatic encephalopathy is one of the developments possible in patients with descompensated cirrhosis. The wellknown role of ammonia in the pathogenesis of hepatic encephalopathy has determined a restriction in dietary protein along many decades. Nevertheless, there is no evidence about a low protein diet being better in the outcome of hepatic encephalopathy, it worsens, moreover, the nutritional status and helps in the development of many nutritional related complications. This article reviews the use of oral branched-chain amino acids and proteins of different sources, probiotics, synbiotics, antioxidants, oral L-Ornithine L-Aspartate and acetyl-L-carnitine in patients with hepatic encephalopathy (AU)

Digital clubbing in primary intestinal lymphangiectasia: a case report.

Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. The symptoms usually start in early infancy. We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. A low-fat diet associated with medium-chain triglyceride supplementation was clinically effective as medical management in reducing diarrhea and leg edema, and promoting weight gain. Double-balloon enteroscopy and small bowel biopsy histopathology confirmed dilated intestinal lacteals. Digital clubbing associated with primary intestinal lymphangiectasia which may causally be related to chronic platelet excess has not been reported before.

[Influence of physiological disturbances on treatment success of dietary therapy in dogs with chronic enteropathies]. / Einfluss körperlicher Mangelzustände auf den diätetischen Behandlungserfolg bei Hunden mit chronischen Enteropathien.

The aim of this retrospective study in 40 dogs with chronic inflammatory enteropathies was to investigate a possible influence of immunologic and metabolic deficiencies on the success of dietary treatments. At the time of initial presentation, routine clinical and laboratory methods were used to evaluate various metabolic (i.e., weight loss, measurement of serum total protein, albumin, and cobalamin concentrations) and immunological parameters (i.e., serum globulin concentration, immunoglobulin-fractionation by serum electrophoresis, and serum C-reactive protein concentration as measured by a species-specific ELISA). The dogs were classified as food-responsive (FR-group, n = 11), antibiotic-responsive (AR-group, n = 12), steroid-responsive (SR-group, n = 11), or dogs with various combinations of therapies due to protein-losing enteropathy (PLE-group, n = 6). Differences among the four treatment groups were evaluated by statistical analysis. Compared to dogs in the AR, SR, and PLE groups, dogs in the FR-group showed a significantly milder weight loss (p < 0.01 for each). Dogs in the FR-group also had significantly higher serum concentrations of total protein and albumin compared to the PLE-group (p < 0.001 for each). The FR-group had significantly higher median concentrations of total globulin and gamma-globulin fractions compared to the AR- and PLE-groups (p <0.005 and p < 0.01, respectively). Lower gamma-globulin concentrations correlated with increased weight loss (Spearman r = -0.53; p < 0.005), serum cobalamin (Spearman r = 0.38; p < 0.05), and albumin (Spearman r = 0.45; p < 0.01). Increased serum concentrations of C-reactive protein correlated inversely with serum concentrations of cobalamin (Spearman r = -0.58; p < 0.05) and gamma-globulins (Spearman r = -0.6; p < 0.005). This study provides evidence for a possible association between the severity of immunological disturbances and the development of deficiencies in dogs with chronic enteropathies. This study provides further evidence that less severe physiological disturbances, such as milder forms of weight loss and higher serum concentrations of albumin, globulin, and gamma-globulin are possibly associated with a more favorably response to dietary treatment alone.

Protein-losing enteropathy during highly active antiretroviral therapy in a patient with AIDS-related disseminated Mycobacterial avium complex infection.

Protein-losing enteropathy (PLE) is defined as a condition in which excess protein loss into the gastrointestinal lumen, due to various causes, is severe enough to produce hypoproteinemia and hypoalbuminemia. We report a 28-year-old Japanese woman with PLE. She had been diagnosed with AIDS and disseminated Mycobacterium avium complex (MAC) infection at age 26. Although highly active antiretroviral and antimycobacterial treatments helped her overcome this critical situation, 2 years after initiation of the treatments, she was readmitted to our hospital because of hypoalbuminemia and edema of the lower extremities, and she was diagnosed, by the use of double-balloon enteroscopy, with PLE due to intestinal lymphangiectasia (IL). The etiology was thought to be obstruction of the mesenteric and retroperitoneal lymphatic drainage systems by MAC lymphadenitis. Even with intensive antimycobacterial treatment, octreotide treatment as a long-acting somatostatin analogue, and a low-fat diet enriched with medium-chain triglyceride, IL was not cured during the follow-up period. In patients with AIDS, complete clinical remission of MAC (especially disseminated MAC) infection is very difficult.

Cows milk protein enteropathy and granulomatous duodenitis in a newborn.

Cow's milk protein enteropathy is a symptom complex that composed of severe diarrhoea and malnutrition. This disorder is caused by non-immunoglobulin E-mediated food allergy. Its clinical features and natural course have been explained in many reports, of different types of cow's milk and soy reactions. In the present article, we describe a newborn patient who presented with chronic diarrhoea and failure to thrive diagnosed as cow's milk protein enteropathy. The duodenal biopsy revealed granulomatous duodenitis which has not been described before. Her clinical and pathological findings responded well to cow's milk elimination. We suggest that food allergies should be considered in differential diagnosis of patients with chronic diarrhoea and failure to thrive.

A case of recurrent gastrointestinal bleeding and protein-losing gastroenteropathy.

BACKGROUND: A 40-year-old male with pentalogy of Fallot (a congenital heart defect with five anatomical components) presented with recurrent gastrointestinal bleeding. He had recently recovered from a heart operation, which was performed to reconstruct the right ventricular outflow tract. INVESTIGATIONS: Laboratory tests and absorption tests, esophagogastroduodenoscopy, capsule endoscopy, human serum albumin scintigraphy, lymphoscintigraphy, CT and abdominal lymph-node histology. DIAGNOSIS: Intestinal lymphangiectasia with concurrent protein-losing gastroenteropathy and recurrent gastrointestinal bleeding. MANAGEMENT Despite a low-fat diet and surgical suturing of multiple small-bowel ulcerations the gastrointestinal bleeding continued. Serum albumin levels remained very low and severe lymphedema occurred. Unfortunately, the patient developed severe sepsis and died of multiple organ failure.

Acquired intestinal lymphangiectasia successfully treated with a low-fat and medium-chain triacylglycerol-enriched diet in a patient with liver transplantation.

Intestinal lymphangiectasia is defined as a dilatation of small bowel lymphatic capillaries and a loss of lymph into the bowel lumen. Clinically it is characterized by hypoproteinaemia and oedema. We present here a case of protein-losing enteropathy due to intestinal lymphangiectasia after liver transplantation in a 57-year-old man who was transplanted for hepatitis C virus. Four years after liver transplantation, the patient developed hypoalbuminaemia and ascites associated with recurrence of cirrhosis. The sudden fall in serum albumin led us to look for a cause of reduction other than or in addition to cirrhosis. Duodenal biopsies showed tall villi with dilated lymphatic vessels and widening of the villi caused by oedema, demonstrating intestinal lymphangiectasia. In this case a low-fat diet supplemented with medium-chain triacylglycerols achieved an early clinical improvement with increased serum albumin levels and ascites disappearance. Intestinal lymphangiectasia should be suspected in liver-transplanted patients developing hypoproteinaemia and hypoalbuminaemia after the recurrence of cirrhosis.

[Congenital intestinal lymphangiectasia: a rare differential diagnosis in hypoproteinemia in infants]. / Kongenitale intestinale Lymphangiektasie: Eine seltene Differerenzialdiagnose hypoproteinämischer Odeme im Säuglingsalter.

BACKGROUND: Congenital intestinal lymphangiectasia is a rare disease in childhood, which may already cause protein-losing enteropathy in newborns. PATIENT, METHODS AND RESULTS: This is a case report of an infant with generalized edema and protein-losing enteropathy, in whom intestinal lymphangiectasia was diagnosed at the age of two months. Following repetitive intravenous albumin und gamma globulin infusions, the elimination of long-chain fats from the diet and the substitution with medium-chain triglycerides (MCT) led to an improvement of the protein-losing enteropathy. CONCLUSION: In newborns with low level of serum protein and edema protein-losing enteropathy caused by congenital lymphangiectasia might be considered as a differential diagnosis.

High-resolution real-time compound ultrasound imaging of transient protein-losing gastropathy of childhood.

We report the case of a 1.5-year-old girl with transient protein-losing gastropathy with hypertrophic gastric folds (PLGH). The diagnosis of PLGH was made by abdominal ultrasound (US) and not by an upper gastrointestinal (UGI) study as reported in many previous publications. Real-time compound ultrasound imaging showed in high detail the echogenic thickening of the mucosal gastric layer and associated hyperaemia on colour Doppler US. These ultrasonic findings highly correlated with the endoscopic US findings and microscopic changes of the gastric wall in PLGH. An awareness of the high-resolution abdominal ultrasound appearances of PLGH may facilitate earlier diagnosis and obviate the need for an upper GI contrast series.