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Carbohydrate and glycosylation disorders
Published in Steve Hannigan, Inherited Metabolic Diseases: A Guide to 100 Conditions, 2018
Hereditary fructose intolerance is an inherited disorder that is characterised by the inability to digest fructose (a natural fruit sugar used in foods, including many baby foods) or sucrose (cane sugar or brown sugar). This intolerance is due to a deiciency of an enzyme known as fructose-1-phosphate aldolase, which results in a build-up of fructose-1-phosphate in the kidney, liver and small intestine. The gene responsible for this disorder is located on the long arm of chromosome 9.
Drug profiles: generic names A-Z
Published in Jerome Z. Litt, Neil H. Shear, Litt's Drug Eruption & Reaction Manual, 2017
Note: Risk of serious adverse reactions in patients with hereditary fructose intolerance due to sorbitol excipient. SkinIrritation [2]Central Nervous SystemDelirium (7%)Fever (6%)Headache [2]Neuromuscular/SkeletalBack pain [2]Gastrointestinal/HepaticConstipation (7%)RespiratoryNasopharyngitis [2]Pneumonia (6%)Endocrine/MetabolicHypokalemia (7%)
Antihistamines-refractory chronic pruritus in psoriatic patients undergoing biologics: aprepitant vs antihistamine double dosage, a real-world data
Published in Journal of Dermatological Treatment, 2022
Giovanni Damiani, Khalaf Kridin, Alessia Pacifico, Piergiorgio Malagoli, Paolo D. M. Pigatto, Renata Finelli, Fabio S. Taccone, Lorenzo Peluso, Rosalynn R. Z. Conic, Nicola L. Bragazzi, Marco Fiore
Exclusion criteria comprehended: (i) pediatric patients (<18 years) or pregnant woman, (ii) different type of psoriasis (i.e. erythrodermic psoriasis, guttate psoriasis, impetigo herpetiformis, pustular psoriasis, drug-induced psoriasis), (iii) the presence of acute or chronic infections (HIV, hepatitis B and C, tuberculosis), (iv) renal, hepatic or metabolic conditions able to induce pruritus, (v) other concomitant autoimmune/auto-inflammatory conditions except psoriatic arthritis or psoriatic spondylitis, (vi) concomitant dermatoses, atopic background, or even history of positive patch test, (vii) drugs recently introduced (<3 months) or capable to trigger pruritus, (viii) use of medical contraceptives, (ix) hereditary fructose intolerance, glucose-galactose malabsorption or sucrase-isomaltase insufficiency, (x) ongoing therapies with pimozide, terfenadine, astemizolo, cisapride, or ergot derivates, (xi) active disease or even history of psychiatric diagnoses, (xii) VAS <6mm.
Special diet in type 1 diabetes: do gender and BMI-SDS differ?
Published in Child and Adolescent Obesity, 2021
Alena Gerlinde Thiele, Nicole Prinz, Monika Flury, Melanie Hess, Daniela Klose, Thomas Meissner, Klemens Raile, Ilona Weis, Sabine Wenzel, Sascha Tittel, Thomas Kapellen, Reinhard Holl
Patient characteristics are shown in Table 2. Most patients with a special diet were younger than 10 years (41.7%) or between 10–20 years (48.7%). In the majority of patients (93.2%), only one type of diet was documented, in 6.5% two diets, and in 0.3% ≥3 diets. The three most common diets or diagnoses requiring dietary treatment were: 1. gluten-free diet due to celiac disease (51.6%), 2. low-protein diet (14.4%), and 3. lactose-restricted diet due to lactose intolerance (11.9%) (Figure 2). The most common combination of two diagnoses requiring dietary treatment was fructose and lactose intolerance (n = 44, 1.7% of the entire diet group). A gluten-free diet was also recorded in patients without the documented clinical diagnosis of celiac disease (n = 85). Other diets were found in less than 10% of the patients. The following diets and clinical diagnoses requiring a special diet were documented in less than 10 patients, respectively: Hereditary fructose intolerance (n = 3), gluten-free diet due to non-celiac gluten/wheat sensitivity (n = 4), cow milk allergy (n = 3), and purine-restricted diet (n = 2). None of the following diets were recorded: FODMAP diet (fermentable oligo-, di-, monosaccharides, and polyols), Paleo diet, lactose-free diet due to congenital lactose intolerance, or potassium-restricted diet.
Symptoms and Problems in Children with Inherited Metabolic Diseases and Factors Affecting the Caregiver Burden of Mothers
Published in Comprehensive Child and Adolescent Nursing, 2020
Tuba Arpaci, Naime Altay, Ebru Kilicarslan Toruner, Mehmet Gunduz
The three most common diagnoses were glycogen storage disease (19.1%), phenylketonuria (14.9%), and hereditary fructose intolerance (12.8%) (Table 1), followed by familial hyperlipidemia (10.6%), mucopolysaccharidosis (8.5%), maple syrup urine disease (6.4%), familial hypercholesterolemia (4.3), methylmalonic acidemia (4.3%), galactosemia (4.3%), Gaucher disease type 1 (4.3%), and others (10.6%). In this study, 14.9% of children had additional anomalies unrelated to their disease and 38.3% of children were hospitalized at least once in the last year. Of children, 85.3% and 36.6% had difficulty following their diet and managing their medication, respectively. Only two children received their nutrition through gastrostomy feeding tubes.