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)

Alagille syndrome (AGS) is frequently associated with growth failure, which has been attributed to concurrent congenital anomalies, cholestasis, and malabsorption and/or malnutrition. However, the underlying cause of the growth failure is not well understood. Our objective is to analyze the growth pattern in 26 patients with AGS and the possible effect that orthotopic liver transplantation (OLT) may have on this pattern. The standardized height, weight, and growth velocity of 26 pair-matched patients with AGS were compared. Thirteen patients underwent OLT. Repeated-measure ANOVA methods were used for the statistical analysis. The overall mean standardized height (z score) was -2.92 in the OLT group versus -1.88 in the non-OLT group (P =.03). The overall mean standardized weight was -1. 21 in the non-OLT group and -1.67 in the OLT group (P =.23). In 15 patients, birth weight was 2.82 +/- 0.4 kg, for a mean standardized weight of -0.95, and weight at diagnosis was 4.53 +/- 2.12 kg, for a mean standardized weight of -1.56. Bone age was delayed in the 9 patients who underwent bone-age analysis. Growth hormone therapy administered to 2 patients did not improve growth. Patients with AGS had growth failure secondary to other factors in addition to liver disease. Growth failure beginning in the prenatal period supports a genetic basis for this feature. Growth improvement up to normal levels should not be expected as a benefit of OLT in these patients. Growth failure as a primary indication for OLT should be cautiously examined in patients with AGS.
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PMID:Does liver transplantation affect growth pattern in Alagille syndrome? 1098 57

Growth failure is one of the most common problems in children with thalassemia with multiple etiologies. We present a case of celiac disease, an underdiagnosed cause of growth failure in a child with beta-thalassemia major. A 10-year-old boy on a hypertransfusion regimen was referred for early onset growth failure. Serology for hepatitis B, hepatitis C, and HIV was negative. Serum zinc levels were normal. Thyroid function tests and growth hormone secretion, evaluated with clonidine stimulation test were normal. Malabsorption syndrome was suspected, even in the absence of gastrointestinal symptoms. Tissue transglutaminase were highly raised >300 IU/mL (normal values <15 U/L). Characteristic mucosal lesions on jejunal biopsy confirmed the diagnosis of celiac disease. Institution of a gluten-free diet resulted in rapid gain in weight and improvement in height velocity.
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PMID:Celiac disease in a child with beta-thalassemia major: a need for improved screening and awareness. 1913 78

Nutrition plays a role in inflammatory bowel disease (IBD) primarily in prevention and treatment of malnutrition and growth failure. Furthermore, in Crohn disease (CD), nutrition can induce remission, maintain remission, and prevent relapse. Malnutrition is common in IBD and the mechanisms involved include decreased food intake, malabsorption, increased nutrient loss, increased energy requirements, and drug-nutrient interactions. At the time of diagnosis, up to 85% of pediatric patients with CD and 65% of those with ulcerative colitis (UC) have weight loss. Growth failure occurs in 15% to 40% of children with IBD and is less common in UC compared with CD, both at diagnosis and during follow-up. In CD, nutritional therapy with enteral formulas induces remission at a rate comparable with that achieved with steroids. In adults with CD, limited information suggests that enteral nutrition (EN) may play a role in maintenance of remission. In children with CD colitis, one study suggested that children without colitis respond better to EN than children with colitis, and another study found no such difference but reported a trend toward earlier relapse in those with isolated colonic involvement. Finally, nutrition may play a role in IBD via the possible protective effect of breastfeeding against UC and CD. In summary, although only CD may benefit from nutrition as primary therapy for remission induction and possibly maintenance of remission, nutrition plays an important role in the prevention and treatment of malnutrition in IBD, and may have a protective role, via the effect of breast-feeding on disease occurrence.
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PMID:Nutritional aspects in inflammatory bowel disease. 1930 Jan 35

Improved survival of orthotopic liver transplantation (OLT) has shifted the focus of patient care to quality of life, including prevention and treatment of pre- and post-transplant complications. End-stage liver failure affects bone length and strength, causing growth failure and hepatic osteodystrophy. Growth failure affects 60% of children assessed for OLT. Optimization of nutrition may prevent further stunting of growth before OLT but is rarely successful. Catch-up growth is observed following steroid withdrawal usually from 18 months post OLT. Whether growth hormone treatment would benefit the 20% of children who fail to regain normal height needs to be tested in randomized controlled trials. Hepatic osteodystrophy in children comprises vitamin D deficiency rickets, low bone mass and fractures caused by malnutrition and malabsorption. Vitamin D deficiency should be treated aggressively with cholecalciferol (D2) or ergocalciferol (D3). The active vitamin D metabolites alphacalcidol or calcitriol are used to increase calcium absorption from the gut but do nothing to replace vitamin D stores. Children before and after OLT have an increased prevalence of fractures of 10-13% and 12-38%, respectively. Most fractures are vertebral, and are related to low spine BMD. They often occur asymptomatically but may also cause chronic pain and later scoliosis. The main risk groups are infants with cholestatic liver disease, and adolescents with later OLT and greater BMI. Fracture prediction in these children is limited. OLT also bears the risk of avascular bone necrosis (4%), and development of scoliosis (13-38%). This paper reviews the literature and presents preventative and therapeutic strategies to improve bone length and strength.
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PMID:Growth and bone health in chronic liver disease and following liver transplantation in children. 2052 40

With improved survival of orthotopic liver transplantation (OLT) in children, prevention and treatment of pre- and posttransplant complications have become a major focus of care. End-stage liver failure can cause endocrine complications such as growth failure and hepatic osteodystrophy, and, like other chronic illnesses, also pubertal delay, relative adrenal insufficiency, and the sick euthyroid syndrome. Drug-induced diabetes mellitus post-OLT affects approximately 10% of children. Growth failure is found in 60% of children assessed for OLT. Despite optimisation of nutrition, rarely can further stunting of growth before OLT be prevented. Catch-up growth is usually observed after steroid weaning from 18 months post-OLT. Whether growth hormone treatment would benefit the 20% of children who fail to catch up in height requires testing in randomised controlled trials. Hepatic osteodystrophy in children comprises vitamin D deficiency rickets, low bone mass, and fractures caused by malnutrition and malabsorption. Vitamin D deficiency requires aggressive treatment with ergocalciferol (D2) or cholecalciferol (D3). The active vitamin D metabolites alphacalcidol or calcitriol increase gut calcium absorption but do not replace vitamin D stores. Prevalence of fractures is increased both before OLT (10%-28% of children) and after OLT (12%-38%). Most fractures are vertebral, are associated with low spine bone mineral density, and frequently occur asymptomatically, but they may also cause chronic pain. Fracture prediction in these children is limited. OLT in children is also associated with a greater risk of developing avascular bone necrosis (4%) and scoliosis (13%-38%). This article reviews the literature on endocrine and skeletal complications of liver disease and presents preventive screening recommendations and therapeutic strategies.
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PMID:Endocrine and bone metabolic complications in chronic liver disease and after liver transplantation in children. 2206 32

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

Impairment of growth is recognized as one of the most significant complications of inflammatory bowel disease (IBD) in pediatric patients. The reported incidence of growth failure at diagnosis is 15-40% in pediatric onset Crohn's disease (CD) and 3-10% in ulcerative colitis (UC). Growth failure is associated with decreased appetite, abdominal symptoms, malabsorption due to mucosal inflammation, growth hormone (GH) resistance due to inflammation, and even genetic factors. Several population-based studies and cohort studies suggest that patients with pre-pubertal onset CD have a higher risk of growth failure at disease onset. Final adult height is still lower than that of healthy controls; however, its prevalence is generally lower than that at the disease onset. Several IBD treatments were reported to improve patients' growth. In addition to enteral nutrition therapy, treatment with anti-tumor necrosis factor (TNF) agents was reported to have favorable effects on growth of patients with pre-pubertal onset CD. Avoiding corticosteroids (CS) and achieving deep remission seems to be important to maintain optimal growth in patients with pediatric onset IBD.
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PMID:Growth failure in pediatric onset inflammatory bowel disease: mechanisms, epidemiology, and management. 3088 94