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.

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)

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.

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.

Treatment of iron deficiency anemia and associated protein-losing enteropathy in children.

PURPOSE: The aims of this study were to evaluate the response of oral iron treatment in children with iron deficiency anemia (IDA) fed whole cow's milk (WCM) or soy formula; to compare the incidence of fecal blood loss in infants fed WCM and soy formula; and to evaluate the incidence and relation of protein-losing enteropathy (PLE) and IDA by testing serum albumin, fecal blood loss, and fecal alpha1-antitrypsin (alpha1AT). METHODS: Twenty-four children with nutritional IDA were randomly assigned to receive either 16 oz WCM or soy formula daily. Both groups were treated with daily therapeutic oral iron during 12 weeks. Stool specimens for hemoglobin losses were collected at weeks 0, 3, 6, and 12. Levels of serum albumin and fecal alpha1AT were tested at diagnosis and when IDA was corrected. RESULTS: Anemia was corrected in 21 of the 24 children by week 6 or 12. Median fecal hemoglobin losses were not increased in either group at diagnosis or during treatment. Seven of 24 children had PLE at diagnosis with elevated fecal alpha1AT levels of 72 to 381 mg/dL that returned to normal after correction of IDA. Their initial fecal alpha1AT levels averaged 170 mg/ dL at diagnosis and 21 mg/dL after the IDA was corrected. Excessive WCM intake of 30 oz/day or more was present in 63% of the infants. CONCLUSIONS: Treatment of nutritional IDA with oral iron was just as effective with a limited quantity of either WCM or soy formula. Fecal hemoglobin losses were uncommon and did not differ in children at diagnosis or during treatment of IDA. PLE associated with IDA resolves when the IDA is corrected, but differences between children fed WCM or soy formula could not be detected.

[Idiopathic intestinal lymphangiectasis. Evolution with M.C.T. (author's transl)]. / Linfangiectasia intestinal idiopática. Evolución con triglicéridos de cadena media

A case of idiopathic intestinal lymphangiectasis is reported in a three month old child. Clinical course and laboratory findings are given in relation to administration of three diets containing different concentrations and types of fat. Short term improvement was only noticed with diets containing low concentrations of long chain triglycerides supplement with medium chain triglycerides. Clinical manifestations related to fat malabsortion improved greatly but there was no relationship with serum protein level. No effect on low level of gamma-globulins and lymphatic displasia was found as sawn in an intestinal biopsy performed after three months of treatment. Nevertheless, long-term results were poor and only were evident in a diminution of steatorrhea and normalization of stools.

Intestinal lymphangiectasia. Long-term results with MCT diet.

The clinical course of 6 children with primary intestinal lymphangiectuasia who have been treated with low fat medium chain triglyceride-supplemented diets for between 3 and 8 years (4 for longer than 5 years) is described. Though laboratory findings indicate continuing chyle leak, evidence for long-term benefit from dietary treatment is provided by symptomatic relief while on the diet, clinical relapse upon relaxation of t,e regimen, and improvement in growth rates. In most patients the underlying lymphatic defect, and thus the need for dietary treatment, appears to be permanent.