Dietary fiber for treating patients suffering from methylmalonic acidemia and propionic acidemia

A technology of methylmalonic acidemia and dietary fiber, applied in the direction of acid-containing food ingredients, applications, food science, etc., can solve problems such as inability to infer

Pending Publication Date: 2021-05-14
NV NUTRICIA
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  • Summary
  • Abstract
  • Description
  • Claims
  • Application Information

AI Technical Summary

Problems solved by technology

In general, results of dietary fiber digestion cannot be extrapolated from infants to older subjects

Method used

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  • Dietary fiber for treating patients suffering from methylmalonic acidemia and propionic acidemia
  • Dietary fiber for treating patients suffering from methylmalonic acidemia and propionic acidemia
  • Dietary fiber for treating patients suffering from methylmalonic acidemia and propionic acidemia

Examples

Experimental program
Comparison scheme
Effect test

Embodiment 1

[0109] fermentation

[0110] In the high-throughput anaerobic colon model (I-screen TM Platform, TNO, Zeist, Netherlands) after incubation for 24 hours, the effect of the fiber mixtures in Table 1 on SCFA production was measured. The platform mimics gut microbiota conditions and enables the determination of the effects of compounds on microbiota composition over time under strictly anaerobic conditions. Inoculate I-screen with standard healthy human adult gut flora TM Model. Gut microbiota were obtained by pooling stool samples from 6 healthy adult volunteers and incubating the pooled stool samples in fed-batch fermenters for 40 h to generate standardized microbiota. The pooled stool samples were then cultured in vitro in modified standard ileal efflux medium (SIEM) with pH adjusted to 5.8. This standard adult gut flora was stored at -80°C in 12% glycerol until further analysis. This pooled approach aims to limit inter-individual variability and increase the likelihood ...

Embodiment 2

[0123] Example 2: Compositions of the invention

[0124] The following compositions of the present invention are compositions suitable for the treatment of MMA and PA by modulating intestinal flora such that propionic acid production is reduced. The supplement comprises the following ingredients in % by weight, based on the total weight of the composition:

[0125] 1.5-28.4% fructooligosaccharides

[0126] 1.5-25.5% inulin

[0127] 1.5-50% resistant starch

[0128] 1-15% gum arabic

[0129] 0-0.5% soybean polysaccharide

[0130] 0-3% guar gum

[0131] ·5-79.5% galacto-oligosaccharides

[0132] • Optionally supplemented with lactic acid producing bacteria, preferably Bifidobacteria and / or Lactobacilli.

Embodiment 3

[0133] Example 3: Compositions of the invention

[0134] The following compositions of the present invention are compositions suitable for the treatment of MMA and PA by modulating intestinal flora such that propionic acid production is reduced. The supplement comprises the following ingredients in % by weight, based on the total weight of the composition:

[0135] ·5-100% galacto-oligosaccharide

[0136] 0-70% resistant starch

[0137] • Optionally supplemented with lactic acid producing bacteria, preferably Bifidobacteria and / or Lactobacilli.

[0138] • Optionally a source of lipids and a source of digestible carbohydrates.

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PUM

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Abstract

The present invention concerns a composition comprising dietary fiber for use in the dietary management of methylmalonic acidemia (MMA) and / or propionic acidemia (PA), and / or for use in treating and / or reducing MMA and / or PA, and / or treating, reducing and / or preventing symptoms associated therewith.

Description

technical field [0001] The present invention relates to the field of dietary management of methylmalonic acidemia (MMA) and propionic acidemia (PA), and to the field for the treatment of symptoms associated therewith. Background technique [0002] Patients with organic aciduria (OA) suffer from a rare inborn error of metabolism. The largest disorders in OA are methylmalonic acidemia (MMA) and propionic acidemia (PA). Both disorders share biochemical and clinical features: Propionyl-CoA and / or methylmalonate (MMA) due to deficiency of propionyl-CoA carboxylase or methylmalonyl-CoA mutase, respectively and accumulation of other metabolites. Initial symptoms of PA and MMA include poor eating, vomiting, loss of appetite, weak muscle tone (hypotonia), and low energy (lethargy). These symptoms can sometimes develop into more serious medical problems, including heart abnormalities (cardiomyopathy), pancreatitis, seizures, coma and possibly death. [0003] Propionate is the prec...

Claims

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Application Information

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Patent Type & Authority Applications(China)
IPC IPC(8): A61K31/702A61K31/715A23L33/00A23L33/21A61P3/00
CPCA23L33/40A61K31/702A61K31/715A61P3/00A23L33/125A23L33/135A23L29/212A23L29/244A23L29/25A23L29/262A23L29/206A23L29/238A23L29/281A23L33/17A23L33/15A23L33/16A23L33/115A23L33/12A23V2002/00A23V2200/32A23V2200/3202A23V2250/06A23V2250/156A23V2250/18A23V2250/50A23V2250/5028A23V2250/506A23V2250/5062A23V2250/5116A23V2250/5118A23V2250/54A23V2250/60A23V2250/70A23V2200/3204A23L33/24A23L33/18A23L33/22A23L33/175A23L33/155A61P7/08A61K31/202A61K31/59A61K31/717A61K31/718A61K31/733A61K31/736A61K33/06A61K35/745A61K35/747
Inventor M·库恩M·拉赫尚德鲁
Owner NV NUTRICIA
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