Defects in long chain fatty acid oxidation presenting as severe cardiomyopathy and cardiogenic shock in infancy

被引：5

作者：

Dereddy, Narendra R. ^{[1
]}

Kronn, David ^{[2
]}

Krishnan, Usha ^{[3
]}

机构：

[1] Maria Fareri Childrens Hosp, Westchester Med Ctr, Dept Pediat, Valhalla, NY USA

[2] Maria Fareri Childrens Hosp, Westchester Med Ctr, Dept Metab & Genet Disorders, Valhalla, NY USA

[3] Maria Fareri Childrens Hosp, Westchester Med Ctr, Dept Pediat Cardiol, Valhalla, NY USA

来源：

CARDIOLOGY IN THE YOUNG | 2009年 / 19卷 / 05期

关键词：

Heart failure; lipid metabolism; pericardial effusion; COA DEHYDROGENASE-DEFICIENCY;

D O I：

10.1017/S104795110999134X

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Inborn errors of fatty acid metabolism are important causes of reversible cardiomyopathy in infancy. Disorders in long chain fatty acid oxidation can lead to cardiomyopathy, as fatty acid beta oxidation is the major source of myocardial energy after birth. We present 2 cases of such disorders with cardiac manifestations during infancy, which responded well to a diet low in long chain fatty acids.

引用

页码：540 / 542

页数：3

共 50 条

[41] MECHANISM OF ACTION OF HYPOGLYCIN ON LONG-CHAIN FATTY ACID OXIDATION
ENTMAN, M
BRESSLER, R
MOLECULAR PHARMACOLOGY, 1967, 3 (04) : 333 - +
[42] Mitochondrial long chain fatty acid oxidation: Man versus mouse
Chegary, M.
te Brinke, H.
Ruiter, J. P. N.
Wijburg, F. A.
Stoll, M.
Schulz, H.
Hoppel, C.
Wanders, R. J. A.
Houten, S. M.
JOURNAL OF INHERITED METABOLIC DISEASE, 2008, 31 : 36 - 36
[43] RATE-CONTROLLING FACTORS OF LONG CHAIN FATTY ACID OXIDATION
PANDE, SV
JOURNAL OF BIOLOGICAL CHEMISTRY, 1971, 246 (17) : 5384 - +
[44] Outcomes of mitochondrial long chain fatty acid oxidation and carnitine defects from a single center metabolic genetics clinic
Anastasia Ambrose
Melissa Sheehan
Shalini Bahl
Taryn Athey
Shailly Ghai-Jain
Alicia Chan
Saadet Mercimek-Andrews
Orphanet Journal of Rare Diseases, 17
[45] Normal acylcarnitine levels during confirmation of abnormal newborn screening in long-chain fatty acid oxidation defects
Browning, MF
Larson, C
Strauss, A
Marsden, DL
JOURNAL OF INHERITED METABOLIC DISEASE, 2005, 28 (04) : 545 - 550
[46] COMPROMISED MITOCHONDRIAL OXYGEN CONSUMPTION AND INCREASED REACTIVE OXYGEN SPECIES PRODUCTION IN LONG CHAIN FATTY ACID OXIDATION DEFECTS
Seminotti, Bianca
Wang, Yudong
Roginskaya, Vera
Leipnitz, Guilhian
Mohsen, Al-Walid
Van Houten, Bennett
Vocldey, Jerry
MOLECULAR GENETICS AND METABOLISM, 2016, 117 (03) : 285 - 285
[47] Outcomes of mitochondrial long chain fatty acid oxidation and carnitine defects from a single center metabolic genetics clinic
Ambrose, Anastasia
Sheehan, Melissa
Bahl, Shalini
Athey, Taryn
Ghai-Jain, Shailly
Chan, Alicia
Mercimek-Andrews, Saadet
ORPHANET JOURNAL OF RARE DISEASES, 2022, 17 (01)
[48] Frequency of the association between maternal liver disease in pregnancy and fetal defects in medium and long chain fatty acid oxidation
Yang, Z
Viola, J
Ibdah, JA
GASTROENTEROLOGY, 2002, 122 (04) : A645 - A645
[49] Cardiogenic Shock Secondary to Stress Induced Cardiomyopathy Precipitated by Severe Diabetic Ketoacidosis
Gurreri, R. A.
Poommipanit, P.
Yamamuro, M.
Alghamdi, A.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2022, 205
[50] Long-chain polyunsaturated fatty acid supplementation in infancy and cognitive function in later childhood
Willatts, P
Forsyth, JS
Agostoni, C
Bissenden, J
Casaear, P
Boehm, G
JOURNAL OF REPRODUCTIVE AND INFANT PSYCHOLOGY, 2003, 21 (03) : 257 - 258

← 1 2 3 4 5 →