(Q36501312)

14 August 2017

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title

Nutrition therapy of organic acidaemias with amino acid-based formulas: emphasis on methylmalonic and propionic acidaemia (English)

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14 August 2017

Steven Yannicelli

1

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14 August 2017

1 April 2006

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Journal of Inherited Metabolic Disease

14 August 2017

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14 August 2017

29

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14 August 2017

2-3

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14 August 2017

281-287

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https://scigraph.springernature.com/pub.10.1007/s10545-006-0267-2

14 August 2017

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cites work

Organic acids in urine of patients with congenital lactic acidoses: an aid to differential diagnosis

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS10545-006-0267-2

Assessment of energy expenditure in metabolic disorders

21 January 2018

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12 December 2020

Contribution of gut bacterial metabolism to human metabolic disease.

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12 December 2020

The management and outcome of propionic and methylmalonic acidaemia

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12 December 2020

Clinical outcome of long-term management of patients with vitamin B12-unresponsive methylmalonic acidemia

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12 December 2020

Long-term follow-up of 77 patients with isolated methylmalonic acidaemia

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12 December 2020

Dietary reference intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids

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12 December 2020

Resting energy expenditure in disorders of propionate metabolism.

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12 December 2020

A patient with propionic acidemia managed with continuous insulin infusion and total parenteral nutrition

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12 December 2020

Protein insufficiency and linear growth restriction in phenylketonuria

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12 December 2020

Parenteral nutrition in propionic and methylmalonic acidemia

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12 December 2020

Inborn errors of metabolism with a protein-restricted diet: effect on polyunsaturated fatty acids

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12 December 2020

L-Carnitine Insufficiency in Disorders of Organic acid Metabolism: Response to L-Carnitine by Patients with Methylmalonic Aciduria and 3-Hydroxy-3-methylglutaric Aciduria

1 reference

12 December 2020

Dependence of the utilization of a phenylalanine-free amino acid mixture on different amounts of single dose ingested. A case report

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12 December 2020

Increased urinary metabolite excretion during fasting in disorders of propionate metabolism

1 reference

12 December 2020

Protein and amino acid requirements and the composition of complementary foods

1 reference

12 December 2020

Propionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency.

1 reference

12 December 2020

The digestion rate of protein is an independent regulating factor of postprandial protein retention

1 reference

12 December 2020

Clinical outcome and long-term management of 17 patients with propionic acidaemia.

1 reference

12 December 2020

Acrodermatitis enteropathica-like cutaneous lesions in organic aciduria

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12 December 2020

Trace element nutrition status and dietary intake of children with phenylketonuria

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12 December 2020

Methylmalonic and propionic acidaemias: management and outcome

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12 December 2020

The effects of dietary protein insufficiency and excess on skeletal health

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12 December 2020

Body composition of reference children from birth to age 10 years

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12 December 2020

Acidosis inhibits osteoblastic and stimulates osteoclastic activity in vitro

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12 December 2020

Catch-up growth in children treated with home enteral nutrition

1 reference

12 December 2020

Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food

1 reference

12 December 2020

Sources of propionate in inborn errors of propionate metabolism

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12 December 2020

Comparison of an elemental and polymeric enteral diet in patients with normal gastrointestinal function

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12 December 2020

Apparent decreased energy requirements in children with organic acidemias: preliminary observations

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12 December 2020

Behavior management of feeding disturbances in urea cycle and organic acid disorders

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12 December 2020

Neonatal-onset propionic acidemia: neurologic and developmental profiles, and implications for management.

1 reference

12 December 2020

Nutrient intakes and physical growth of children with phenylketonuria undergoing nutrition therapy

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12 December 2020

Effect of simultaneous ingestion of L-amino acids and whole protein on plasma amino acid and urea nitrogen concentrations in humans.

1 reference

12 December 2020

Trace element status of children with PKU and normal children

1 reference

12 December 2020

Decreased selenium intake and low plasma selenium concentrations leading to clinical symptoms in a child with propionic acidaemia

1 reference

12 December 2020

Glutathione deficiency as a complication of methylmalonic acidemia: Response to high doses of ascorbate

1 reference

12 December 2020

Malnutrition at age 3 years and lower cognitive ability at age 11 years: independence from psychosocial adversity

1 reference

12 December 2020

Plasma amino acid response to ingestion of L-amino acids and whole protein

1 reference

12 December 2020

Primary treatment of propionic acidemia complicated by acute thiamine deficiency

1 reference

12 December 2020

The relationship of dietary and lifestyle factors to bone mineral indexes in children

1 reference

12 December 2020

10.1007/S10545-006-0267-2

Identifiers

DOI

1 reference

14 August 2017

1 reference

PubMed ID

16763889