pyruvate dehydrogenase complex deficiency disease/carbohydrate

Tautan disimpan ke clipboard

Pilih jenis pengurutan

Halaman 1 dari 20 hasil

Sensitivity to carbohydrate in a patient with familial intermittent lactic acidosis and pyruvate dehydrogenase deficiency.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

A 9-year old boy with severe mental and growth retardation and diffuse neurologic damage had minimal elevation of blood pyruvate (0.21 mM) and lactate (2.1 mM) on a normal diet but developed life-threatening lactic acidosis (pH 7.14; lactate 21.0 MM) on a diet containing 65% carbohydrate and 15%

Energy substrate metabolism in pyruvate dehydrogenase complex deficiency.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

Pyruvate dehydrogenase (PDH) deficiency is an inherited disorder of carbohydrate metabolism, resulting in lactic acidosis and neurological dysfunction. In order to provide energy for the brain, a ketogenic diet has been tried. Both the disorder and the ketogenic therapy may influence energy

Urinary lactate excretion in normal children and in children with enzyme defects of carbohydrate metabolism.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

Urinary lactate was analyzed in 53 normal children, 7 children with glucose-6-phosphatase-deficient glycogenosis, 1 child with fructose-1,6-diphosphatase deficiency and 1 child with pyruvate dehydrogenase deficiency. Lactate in 24-h urine was expressed as concentration, total excretion, excretion

Anaesthesia and pyruvate dehydrogenase deficiency.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

A ten-month-old infant with pyruvate dehydrogenase deficiency received anaesthesia on two occasions, once for a laparotomy and once for a tracheostomy. During both anaesthetics (different techniques) she developed an increase in arterial lactate levels and a metabolic acidosis. Pyruvate

Pyruvate dehydrogenase deficiency and epilepsy.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

The pyruvate dehydrogenase complex (PDHc) is a mitochondrial matrix multienzyme complex that provides the link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the conversion of pyruvate into acetyl-CoA. PDHc deficiency is one of the commoner metabolic disorders of lactic

Pyruvate dehydrogenase complex deficiency and its relationship with epilepsy frequency--An overview.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

The pyruvate dehydrogenase complex (PDHc) is a member of a family of multienzyme complexes that provides the link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the physiologically irreversible decarboxylation of various 2-oxoacid substrates to their corresponding acyl-CoA

Liver-specific pyruvate dehydrogenase complex deficiency upregulates lipogenesis in adipose tissue and improves peripheral insulin sensitivity.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

The pyruvate dehydrogenase complex (PDC) plays a critical role in lipid synthesis and glucose homeostasis in the fed and fasting states. The central role of the liver in the maintenance of glucose homeostasis has been established by studying changes in key enzymes (including PDC) and the carbon-flux

Enzymologic studies and therapy of Leigh's disease associated with pyruvate decarboxylase deficiency.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

An 11-month-old boy with muscle hypotonia and neurologic deteriorations had lactic acidosis, pyruvic acidemia and alaninemia due to deficiency of the pyruvate dehydrogenase complex in his platelets and of pyruvate decarboxylase in his muscle. When placed on a low carbohydrate-high fat diet for 6

Caveats when considering ketogenic diets for the treatment of pyruvate dehydrogenase complex deficiency.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

OBJECTIVE We conducted a critical assessment of the use of diets high in fat and low in carbohydrate ("ketogenic") in the treatment of children with congenital lactic acidosis caused by mutations in the mitochondrial pyruvate dehydrogenase complex (PDC). METHODS The dietary composition of 18

Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets. Studies in patients with identical mutations.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

Inborn errors of the pyruvate dehydrogenase complex (PDC) are associated with lactic acidosis, neuroanatomic defects, developmental delay, and early death. PDC deficiency is a clinically heterogeneous disorder, with most mutations located in the coding region of the X-linked alpha subunit of the

Impact of selected inborn errors of metabolism on prenatal and neonatal development.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

In general, data regarding maturational processes of different metabolic pathways in the very vulnerable fetal and neonatal period are rare. This review is to substantiate the impact of selected inborn errors of metabolism on this critical period of life and their clinical manifestation. Significant

Pyruvate dehydrogenase-E1α deficiency presenting as recurrent acute proximal muscle weakness of upper and lower extremities in an 8-year-old boy.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

The mitochondrial pyruvate dehydrogenase enzyme complex (PDHC) plays an important role in aerobic energy metabolism and acid-base equilibrium. PDHC contains of 5 enzymes, 3 catalytic (E1, E2, E3) and 2 regulatory, as well as 3 cofactors and an additional protein (E3-binding protein) encoded by

A cognitively normal PDH-deficient 18-year-old man carrying the R263G mutation in the PDHA1 gene.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

Pyruvate dehydrogenase (PDH) is a crucial multienzyme system linking glycolysis to the tricarboxylic acid cycle by catalysing the decarboxylation of pyruvate to acetyl-CoA. Deficiency in pyruvate dehydrogenase is most commonly secondary to mutations in the X-linked PDHA1 gene encoding the E1 alpha

Introduction of a ketogenic diet in young infants.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

The ketogenic diet is a rational treatment for pyruvate dehydrogenase complex deficiency (McKusick 312170) and GLUT1 deficiency syndrome (McKusick 138140). An increasing number of patients are diagnosed in early infancy, but few data are available on the introduction of a ketogenic diet in this age

Controlled clinical trial of dichloroacetate for treatment of congenital lactic acidosis in children.

Hanya pengguna terdaftar yang dapat menerjemahkan artikel

Masuk daftar

OBJECTIVE Open-label studies indicate that oral dichloroacetate (DCA) may be effective in treating patients with congenital lactic acidosis. We tested this hypothesis by conducting the first double-blind, randomized, control trial of DCA in this disease. METHODS Forty-three patients who ranged in

Sebelumnya
1
2
Lanjut

Bergabunglah dengan
halaman facebook kami

Herbal & Natural Medicine

Database tanaman obat terlengkap yang didukung oleh sains

Bekerja dalam 55 bahasa
Pengobatan herbal didukung oleh sains
Pengenalan herbal melalui gambar
Peta GPS interaktif - beri tag herba di lokasi (segera hadir)
Baca publikasi ilmiah yang terkait dengan pencarian Anda
Cari tanaman obat berdasarkan efeknya
Atur minat Anda dan ikuti perkembangan berita, uji klinis, dan paten

Ketikkan gejala atau penyakit dan baca tentang jamu yang mungkin membantu, ketik jamu dan lihat penyakit dan gejala yang digunakan untuk melawannya.
* Semua informasi didasarkan pada penelitian ilmiah yang dipublikasikan