Hereditary Fructose Intolerance (HFI) is an autosomal recessive inborn error of metabolism characterised by the deficiency of the hepatic enzyme aldolase B. Its treatment consists in adopting a fructose-, sucrose-, and sorbitol (FSS)-restrictive diet for life. Untreated HFI patients present an abnormal transferrin (Tf) glycosylation pattern due to the inhibition of mannose-6-phosphate isomerase by fructose-1-phosphate. Hence, elevated serum carbohydrate-deficient Tf (CDT) may allow the prompt detection of HFI. The CDT values improve when an FSS-restrictive diet is followed; however, previous data on CDT and fructose intake correlation are inconsistent. Therefore, we examined the complete serum sialoTf profile and correlated it with FSS dietary intake and with hepatic parameters in a cohort of paediatric and adult fructosemic patients. To do so, the profiles of serum sialoTf from genetically diagnosed HFI patients on an FSS-restricted diet (n = 37) and their age-, sex- and body mass index-paired controls (n = 32) were analysed by capillary zone electrophoresis. We found that in HFI patients, asialoTf correlated with dietary intake of sucrose (R = 0.575, p < 0.001) and FSS (R = 0.475, p = 0.008), and that pentasialoTf+hexasialoTf negatively correlated with dietary intake of fructose (R = −0.386, p = 0.024) and FSS (R = −0.400, p = 0.019). In addition, the tetrasialoTf/disialoTf ratio truthfully differentiated treated HFI patients from healthy controls, with an area under the ROC curve (AUROC) of 0.97, 92% sensitivity, 94% specificity and 93% accuracy.
Background: Periodontal diseases (PD), including periodontitis, are chronic inflammatory pathologies caused by bacteria in the subgingival biofilm which affect the periodontal tissues. PD is now considered a localized, chronic, oral infection that activates the host immuno-inflammatory responses both locally and systemically, and also constitutes a source of bacteraemia. It is a known fact that periodontal diseases exercise an important influence on the pathogenesis of numerous systemic diseases, including diabetes mellitus (DM). In the mid-nineties, sufficient scientific evidence emerged to confirm an association between DM and periodontitis, which then began to be regarded as the sixth complication of DM. Current scientific evidence points to a two-way relationship between DM and periodontal disease, whereby DM is associated with an increase in the incidence and progression of periodontitis, while periodontal infection is associated with worsening glycemic control in diabetic patients. This two-way relationship points to a need to promote oral health in DM patients, and to implement a joint management protocol between endocrinologist and dentist that aims to create adequate conditions for early diagnosis and the effective treatment of both diseases.
We suggest that it should be necessary to relate GH secretion to body mass in prepubertal children before defining their secretion as normal or pathological.
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