Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Metabolic bone disease has been recognized as an important complication of chronic liver disease particularly in cholestatic disorders [primary biliary cirrhosis (PBC) and primary sclerosing cholangitis] and after liver transplantation. It includes osteoporosis and more rarely osteomalacia, which is more frequent in severe malabsorption and advanced liver disease. The pathogenesis of this disorder is complex and is likely to be multifactorial. Regardless of the etiology of osteoporosis in PBC patients, they have an increased risk of spontaneous or low-trauma fracturing leading to significant patient morbidity, deterioration of quality of life, and even patient mortality. The development of bone densitometry has allowed assessment of bone mass and then contributed in estimating the fracture risk. The gold standard of bone mineral density measurement is currently the dual- energy X-ray absorptiometry. Recommendations formulated by the World Health Organization have reported the diagnostic ranges of osteoporosis based on the t-score: patient with osteoporosis has a t-score less than -2.5 SD, osteopenia has a t-score between -1.0 and -2.5 SD and a normal individual has a t-score more than -1.0 SD. The risk of fracture shows a correlation with bone mineral density but no fracture threshold was determined and the best site of characterizing the hip fracture risk is the measure of the bone mineral density of the proximal femur. The treatment of osteoporosis in patients with PBC is largely based on trials of patients with postmenopausal osteoporosis as there are a few and smaller studies of osteoporotic patients with PBC. Bisphosphonates seem to be effective in biliary disease and are more tolerated.
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PMID:Osteoporosis in patients with primary biliary cirrhosis. 2092 53

Growth failure is a common problem in many children with chronic diseases. This article is an overview of the most common causes of growth failure/growth retardation that affect children with a number of chronic diseases. We also briefly review the nutrition considerations and treatment goals. Growth failure is multifactorial in children with chronic conditions, including patients with cystic fibrosis, chronic kidney disease, chronic liver disease, congenital heart disease, human immunodeficiency virus, inflammatory bowel disease, short bowel syndrome, and muscular dystrophies. Important contributory factors to growth failure include increased energy needs, increased energy loss, malabsorption, decreased energy intake, anorexia, pain, vomiting, intestinal obstruction, and inflammatory cytokines. Various metabolic and pathologic abnormalities that are characteristic of chronic diseases further lead to significant malnutrition and growth failure. In addition to treating disease-specific abnormalities, treatment should address the energy and protein deficits, including vitamin and mineral supplements to correct deficiencies, correct metabolic and endocrinologic abnormalities, and include long-term monitoring of weight and growth. Individualized, age-appropriate nutrition intervention will minimize the malnutrition and growth failure seen in children with chronic diseases.
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PMID:Growth failure and nutrition considerations in chronic childhood wasting diseases. 2537 56

Malnutrition is common in patients with chronic liver disease and is associated with poor outcomes. Inadequate intake, poor quality diet, maldigestion, malabsorption, altered macronutrient metabolism, and hypermetabolism all contribute to the development of malnutrition in this patient population. Although it is generally easy to detect, clinicians often overlook malnutrition and its measurement is complicated by the lack of a simple, standardized diagnostic method. Early detection of malnutrition and multidisciplinary treatment approaches greatly increase the probability for successful outcomes.
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PMID:Importance of malnutrition in patients with cirrhosis. 2603 97

Malnutrition is prevalent in individuals with chronic liver disease and occurs as a result of inadequate nutrient intake, altered metabolism, and malabsorption. Although limited data show benefits of enteral nutrition (EN) in this population, patients with chronic liver disease often have inadequate oral intake and are potential candidates for EN. The goals of the EN, type and severity of liver disease, and access for EN will influence the decision to initiate EN. This paper summarizes EN studies in patients with liver disease and provides practical tips regarding patient selection, EN access, and EN formula choices. Two case studies illustrate the principles and challenges of providing EN to patients with cirrhosis. The paper concludes with suggested parameters for an EN feeding protocol and recommendations for future research.
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PMID:Enteral Nutrition in Chronic Liver Disease: Translating Evidence Into Practice. 2611 62

Loss-of-function mutations in the Notch ligand, Jagged1 (Jag1), result in multi-system developmental pathologies associated with Alagille syndrome (ALGS). ALGS patients present with skeletal manifestations including hemi-vertebrae, reduced bone mass, increased fracture incidence and poor bone healing. However, it is not known whether the increased fracture risk is due to altered bone homeostasis (primary) or nutritional malabsorption due to chronic liver disease (secondary). To determine the significance of Jag1 loss in bone, we characterized the skeletal phenotype of two Jag1-floxed conditional knockout mouse models: Prx1-Cre;Jag1(f/f) to target osteoprogenitor cells and their progeny, and Col2.3-Cre;Jag1(f/f) to target mid-stage osteoblasts and their progeny. Knockout phenotypes were compared to wild-type (WT) controls using quantitative micro-computed tomography, gene expression profiling and mechanical testing. Expression of Jag1 and the Notch target genes Hes1 and Hey1 was downregulated in all Jag1 knockout mice. Osteoblast differentiation genes were downregulated in whole bone of both groups, but unchanged in Prx1-Cre;Jag1(f/f) cortical bone. Both knockout lines exhibited changes in femoral trabecular morphology including decreased bone volume fraction and increased trabecular spacing, with males presenting a more severe trabecular osteopenic phenotype. Prx1-Cre;Jag1(f/f) mice showed an increase in marrow mesenchymal progenitor cell number and, counterintuitively, developed increased cortical thickness resulting from periosteal expansion, translating to greater mechanical stiffness and strength. Similar alterations in femoral morphology were observed in mice with canonical Notch signaling disrupted using Prx1-Cre-regulatable dominant-negative mastermind like-protein (dnMAML). Taken together, we report that 1) Jag1 negatively regulates the marrow osteochondral progenitor pool, 2) Jag1 is required for normal trabecular bone formation and 3) Notch signaling through homotypic Jag1 signaling in osteochondral progenitors, but not mature osteoblasts, inhibits periosteal expansion. Therefore, Jag1 signaling within the osteoblast lineage regulates bone metabolism in a compartment-dependent manner. Moreover, loss of Jag1 function in osteoblast lineage cells may contribute to the skeletal phenotype associated with ALGS.
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PMID:Jagged1 expression by osteoblast-lineage cells regulates trabecular bone mass and periosteal expansion in mice. 2741 9

Acrodermatitis enteropathica syndrome (AE) is a clinical entity that results in severe zinc deficiency. It can be genetic or acquired. Acquired AE has been reported in patients with chronic liver disease, malabsorption syndrome, sickle cell anemia, and chronic renal failure. We present a kidney transplant recipient with skin rash and watery diarrhea. The patient had low serum zinc levels, which quickly resolved after zinc supplementation. Skin biopsy showed cytoplasmic pallor and vacuolization and ballooning degeneration of keratinocytes within the superficial epidermis, which may have led to confluent necrosis of keratinocytes. Large amounts of keratinosome-derived lamellae were found in the intercellular spaces in the keratinized area, probably related to disturbance of keratinosome metabolism due to zinc deficiency.
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PMID:Acquired Acrodermatitis Enteropathica Syndrome in a Kidney Transplant Receipt: A Case Report. 2834 Aug 43

The liver is a major organ and an essential component in maintaining an appropriate nutritional status in healthy individuals through metabolism of protein, carbohydrates, and fat. In individuals with chronic liver disease (CLD), along with a number of other essential functions that the liver serves, its role in nutrition maintenance is severely impaired. Common causes of CLD include hepatitis C, alcoholic liver disease, and non-alcoholic liver disease. Amongst this population, the most common manifestation of impaired nutritional maintenance is protein-calorie malnutrition. Aside from inherent abnormalities in metabolism, such as malabsorption and maldigestion, CLD can be associated with anorexia as well as increased metabolic requirements, all of which contribute to a state of malnutrition. Given the systemic implications and impact on prognosis of malnutrition, proper nutritional assessment is essential and can be achieved through a thorough history and physical, as well as biochemical investigations and anthropometry as needed. Following an appropriate assessment of a patient's nutritional status, an approach to management can be decided upon and is based on the extent of malnutrition which directly reflects the severity of disease. Management options can be grossly separated into enteral and parenteral nutrition. The former is usually sufficient in the form of oral supplements in less severe cases of malnutrition, but as the CLD worsens, parenteral nutrition becomes necessary. With appropriate assessment and early intervention, many of the complications of CLD can be avoided, and ultimately better outcomes can be achieved.
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PMID:Nutritional support in chronic liver disease and cirrhotics. 3038 61

More than one-half of children with chronic liver disease suffer from malnutrition, which leads not only to a poor quality of life and even possibly catastrophic complications, but also to poor outcomes after a liver transplantation. These children have increased metabolic demands but often decreased intake with malabsorption and altered nutrient utilization, all of which make it difficult to keep up with nutritional demands. Assessment of a patient's nutritional status should be timely, and it should be performed routinely and proactively. When specific nutritional needs are identified, these should be addressed with a multidisciplinary team approach and with the close guidance of an experienced pediatric dietician. The assessment includes anthropometric and laboratory assessments, in addition to a careful physical examination and a detailed patient history. The specific nutritional needs vary, but generally dietary intervention focuses on increasing caloric intake, supplementation with medium-chain triglycerides, and prevention of essential fatty acid and fat-soluble vitamin deficiencies. [Pediatr Ann. 2018;47(11):e445-e451.].
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PMID:Nutrition in Pediatric Chronic Liver Disease. 3042 87

Malnutrition is prevalent in patients with hepatic failure and remains an independent risk factor for morbidity and mortality in these patients. Factors that contribute to malnutrition in this patient population include altered metabolic rate, fat malabsorption, and impaired gastric emptying, all in the setting of an acute and potentially prolonged hospitalization. Acute liver failure (ALF), different from cirrhosis or chronic liver disease, is an uncommon but dramatic clinical syndrome that demonstrates severe and rapid decline in hepatic metabolic function. ALF has a significant risk of mortality. There are >10 cases per million persons per year in developed countries, but ALF presents with unique challenges in clinical management related to heterogeneity in severity and etiology. Patients with ALF by definition should not have a prior history of liver disease, and liver disease is subsequently defined by the onset of liver injury, the presence of hepatic encephalopathy (HE), and coagulopathy as defined by an international normalized ratio > 1.5. HE usually develops within 1-4 weeks of the onset of liver injury but may occur within 26 weeks of the initial presentation. Rates of survival from ALF have improved over recent years, but the rarity and severity of presentation have resulted in traditionally limited evidence to guide basic supportive care. Over time, advances in critical care management and the use of emergency liver transplantation have improved. In this article, we will review current nutrition considerations for patients with ALF.
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PMID:Acute Hepatic Failure and Nutrition. 3187 3

Vitamin A (VA) is an essential nutrient often lacking in the diets of people in developing countries. Accurate biomarkers of VA status are vital to inform public health policy and monitor interventions. The relative dose-response (RDR) and modified-RDR (MRDR) tests are semi-quantitative screening tests for VA deficiency that have been used in Demographic and Health Surveys and VA intervention studies. A systematic review and meta-analysis of sensitivity and specificity were conducted to summarize the physiological evidence to support the RDR tests as methods to assess VA status and investigate the impact of different pathological and physiological states on the tests. A total of 190 studies were screened for inclusion, with 21 studies comparing the RDR tests with the gold-standard biomarker, liver VA concentration (68% and 80% sensitivity and 85% and 69% specificity for the RDR and MRDR, respectively). Nearly all studies with VA interventions in VA-deficient populations demonstrated a response of the tests to VA intake that would be expected to improve VA status. The impacts of chronic liver disease, protein malnutrition, age, pregnancy and lactation, infection and inflammation, and various other conditions were examined in 51 studies. The RDR and MRDR tests were reported to have been used in 39 observational studies, and the MRDR has been used in at least 6 national micronutrient surveys. The RDR and MRDR are sensitive tests for determining population VA status and assessing VA interventions. Although they are robust to most physiological and pathological states, caution may be warranted when using the tests in neonates, individuals with chronic liver disease, and those with protein or iron malnutrition. Research on further improvements to the tests to increase accessibility, such as sampling breast milk instead of blood or using intramuscular doses in subjects with malabsorption, will allow wider adoption. This review was registered with PROSPERO as CRD42019124180.
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PMID:Systematic Review and Meta-Analysis of the Relative Dose-Response Tests to Assess Vitamin A Status. 3313 Aug 84


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