The Noble Method in the dairy sector as a sustainable production system to improve the nutritional composition of dairy products: A review

Sustainable production systems in line with consumer expectations are attractive for the dairy sector. The objective of this review is to examine the benefits of an Italian method, named the Noble Method (‘Metodo Nobile ’), in order to improve the nutritional properties of milk and environmental sustainability. The prohibition of silage and the use of polyphite pastures are some of the rules established by the Noble Method. The greater amount of unsaturated fatty acids and other beneficial compounds found in milk and dairy products produced by using milk from animals fed on well-managed pasture could have positive implications on consumers’ health

A ‘Mediterranean ice-cream’: Sensory and nutritional aspects of replacing milk cream with extra virgin olive oil

In this study, we explored the sensory and nutritional properties of innovative ice-creams designed totally replacing cow’s milk cream with extra virgin olive oil (EVOO). Milk and chocolate flavored ice-creams containing 14.1% and 10.2% milk cream or 5.1% and 3.6% of EVOO were produced. In a triangle test, only the milk-flavored ice-cream with EVOO was distinguished from its traditional counterpart whereas in a quantitative descriptive sensory analysis EVOO ice-creams showed an increased intensity of the descriptor “grassy” that was supported by the greater concentrations of specific volatile organic compounds coming from EVOO, compared to traditional ice-creams. Moreover, EVOO ice-creams contained less saturated fatty acids and more mono- and poly- unsaturated fatty acids along an increased concentration of bioaccessible polyphenols and antioxidant activity measured after an in vitro digestion, which was more pronounced in milkflavored sample. Data showed that EVOO can be a functional fat replacer in ice-cream recipes to produce healthier products

Sensory and electronic nose analysis of Noble goat’s milk from Cilento (Italy)

The interest in extensive farming which is sustainable for the environment and animal welfare has recently increased. In such context, an approach that arises from these assumptions, aimed at enhancing the quality of dairy products, is the “Noble method”, applied in Cilento area (PSR Campania 2014/2020-Measure 16.1) for zootechnical productions. The disciplinary of the “Noble method” prohibits the use of silage in the livestock feeding system, provides quantitative limits for the use of energy concentrates and dictates specific rules for the botanical composition of pasture forage (at least 5 different cultivars in the ration). Due to constraints related to the feasibility of extensive grazing, the “Noble method” appears to be a promising strategy especially for small ruminants, such as sheep and goat farms. In this investigation, sensory analysis was applied to test the differences between noble goat’s milk and non-noble standard goat’s milk. Then, E-nose, consisting of ten metal oxide semiconductor sensors was used to classify the two types of goat’s milk, in terms of rearing and feeding system, and to test the discrimination feasibility of the device. The milk samples were taken in five different times over a period of six months (lactation period effect) from 18 individual goats. In terms of sensory differences, Noble milk scored higher for descriptors such as grassy and sweet aromatic, typical of an extensive pasture farmed milk, while regular milk scored higher for descriptors such as silage and malty. E-nose resulted capable of classifying the two milk types in terms of animal feed (87.78% correct classification of milk samples according to the feeding system). Since the “Nobile method” follows a production specification, it is necessary to control and guarantee the authentic quality of the dairy products, and the E-nose has proved to be a quick, reproducible and simple method for routine control analyses

Glycogen storage disease type Ia (GSDIa) but not Glycogen storage disease type Ib (GSDIb) is associated to an increased risk of metabolic syndrome: Possible role of microsomal glucose 6-phosphate accumulation

Background: In GSDIa, glucose 6-phosphate (G6P) accumulates in the endoplasmic reticulum (ER); in GSDIb, G6P levels are reduced in ER. G6P availability directly modulates the activity of 11β-hydroxysteroid dehydrogenase type 1 (11βHSD1), an ER-bound enzyme playing a key role in the development of the metabolic syndrome (MS). Objective: To evaluate the prevalence of MS and Insulin Resistance (IR) in GSDIa and GSDIb patients. Patients and Methods: This was a prospective study. All the enrolled patients were followed at the Department of Pediatrics "Federico II" University of Naples for 10 years. Clinical and biochemical parameters of MS and the presence of IR were recorded. The results were correlated with the biochemical parameters of GSDI-related metabolic control. 10 GSDIa patient (median age 12.10 ± 1.50), 7 GSDIb patients (median age 14.90 ± 2.20 were enrolled in the study. They were compared to 20 and 14 age and sex matched controls, respectively. 10 GSDIa patients (median age 24.60 ± 1.50) and 6 GSDIb patients (median age 25.10 ± 2.00) completed the 10-year-follow-up. At the end of the study the patients' data were compared to 10 and 6 age and sex matched controls, respectively. Results: At study entry, 20 % GSDIa patients had MS and 80 % showed 2 criteria for MS. GSDIa patients showed higher HOMA-IR than controls and GSDIb patients (p < 0.001, p < 0.05), respectively. Baseline ISI was lower in GSDIa than controls (p < 0.001). QUICKI was significantly lower in GSDIa than in controls (p < 0.001). At the end of the study 70 % of GSDIa patients had MS and 30 % showed 2 criteria for MS. HOMA-IR was higher in GSDIa than controls (p < 0.01). Baseline ISI was higher in GSDIb than controls (p < 0.005) and GSD1a (p < 0.05). QUICKI was lower in GSD1a patients than in controls (p < 0.03). VAI was higher in GSDIa patients than controls (p < 0.001) and GSDIb patients (p = 0.002). Conclusions: Our data showed high prevalence of IR and MS in GSDIa patients. We speculate a possible role of 11βHSD1 modulation by G6P availability. We suggest a routine metabolic assessment in GSDIa patients

Myasthenia gravis in a patient affected by glycogen storage disease type Ib: A further manifestation of an increased risk for autoimmune disorders?

Glycogen storage disease type Ib (GSD Ib, OMIM 232220) is an inborn disorder of glucose metabolism, caused by mutations in the G6PT gene, encoding a glucose 6-phosphate transporter (G6PT). GSD Ib is mainly associated with fasting hypoglycaemia and hepatomegaly. Most GSD Ib patients also show neutropenia and neutrophil dysfunction and therefore are at risk of developing severe infections and inflammatory bowel disease (IBD). An increased risk for autoimmune disorders, such as thyroid autoimmunity and Crohn-like disease, has also been demonstrated, but no systematic study on the prevalence of autoimmune disorders in GSD Ib patients has ever been performed. We describe a 25-year-old patient affected by GSD Ib who developed 'seronegative' myasthenia gravis (MG), presenting with bilateral eyelid ptosis, diplopia, dysarthria, severe dysphagia, dyspnoea and fatigue. The repetitive stimulation of peripheral nerves test showed signs of exhaustion of neuromuscular transmission, particularly evident in the cranial area. Even in the absence of identifiable anti-acetylcholine receptor antibodies, seronegative MG is considered an autoimmune disorder and may be related to the disturbed immune function observed in GSD Ib patients

The growth hormone-insulin-like growth factor axis in glycogen storage disease type 1: evidence of different growth patterns and insulin-like growth factor levels in patients with glycogen storage disease type 1a and 1b.

OBJECTIVES: To investigate the growth hormone (GH)-insulin-like growth factor (IGF) system in patients with glycogen storage disease type 1 (GSD1). STUDY DESIGN: This was a prospective, case-control study. Ten patients with GSD1a and 7 patients with GSD1b who were given dietary treatment and 34 sex-, age-, body mass index-, and pubertal stage-matched control subjects entered the study. Auxological parameters were correlated with circulating GH, either at basal or after growth hormone releasing hormone plus arginine test, insulin-like growth factors (IGF-I and IGF-II), and anti-pituitary antibodies (APA). RESULTS: Short stature was detected in 10.0% of patients with GSD1a, 42.9% of patients with GSD1b (P = .02), and none of the control subjects. Serum IGF-I levels were lower in patients with GSD1b (P = .0001). An impaired GH secretion was found in 40% of patients with GSD1a (P = .008), 57.1% of patients with GSD1b (P = .006), and none of the control subjects. Short stature was demonstrated in 3 of 4 patients with GSD1b and GH deficiency. The prevalence of APA was significantly higher in patients with GSD1b than in patients with GSD1a (P = .02) and control subjects (P = .03). The GH response to the provocative test inversely correlated with the presence of APA (P = .003). Compared with levels in control subjects, serum IGF-II and insulin levels were higher in both groups of patients, in whom IGF-II levels directly correlated with height SD scores (P = .003). CONCLUSION: Patients with GSD1a have an impaired GH secretion associated with reference range serum IGF-I levels and normal stature, whereas in patients with GSD1b, the impaired GH secretion, probably because of the presence of APA, was associated with reduced IGF-I levels and increased prevalence of short stature. The increased IGF-II levels, probably caused by increased insulin levels, in patients with GSD1 are presumably responsible for the improved growth pattern observed in patients receiving strict dietary treatment