Cardiac septal hypertrophy in hyperinsulinemic infants

Breitweser, James A.; Meyer, Richard A.; Sperling, Mark A.; Tsang, Reginald C.; Kaplan, Samuel

doi:10.1016/s0022-3476(80)80862-6

Cited by 140 publications

(67 citation statements)

References 18 publications

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“…Cardiac involvement in CGL has been reported in the literature [9][10][11][12][13][14][15] but there are no cases describing early onset cardiac failure, as in our case. Bjornstad et al [9] reported 6 cases of BSCL presenting myocardial hypertrophy, and Rheuban et al [10] described 4 other similar cases.…”

Section: Discussionmentioning

confidence: 57%

“…The mechanism causing hypertrophic cardiomyopathy in patients with genetic insulin resistance syndrome as BSCL, and therefore with hyperinsulinemia, may result from the insulin action in the receptors of Insulin-like growth factor I (IGF-1) present in the myocardial tissue [10,12]. This is based on the occurrence of myocardial hypertrophy in children of diabetic mothers [13]. Hypertension was also present in a few patients with BSCL and myocardial hypertrophy at second or third decade of life [10,15].…”

Section: Discussionmentioning

confidence: 99%

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Severe cardiac phenotype of Berardinelli-Seip congenital lipodystrophy in an infant with homozygous E189X BSCL2 mutation

Friguls

Coroleu

Alcázar

et al. 2009

European Journal of Medical Genetics

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a b s t r a c tBerardinelli-Seip congenital lipodystrophy (BSCL) is a rare autosomal recessive condition associating insulin resistance, absence of subcutaneous fat and muscular hypertrophy. Disease-causing mutations have been described in AGPAT2 and BSCL2 genes. Hypertrophic cardiomyopathy is a classical late (third decade) complication which has only been occasionally described in childhood. We report on a 4-month -old Chinese male infant who presented with a severe BSCL ''cardiac'' phenotype comprising heart failure, hypertension and hypertrophic cardiomyopathy.

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Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

Severe cardiac phenotype of Berardinelli-Seip congenital lipodystrophy in an infant with homozygous E189X BSCL2 mutation

Friguls

Coroleu

Alcázar

et al. 2009

European Journal of Medical Genetics

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show abstract

“…Prenatal dönemde yapılan fetal EKO incelemesinde, interventriküler septum kalınlığının 4.5 mm ve üzerinde, interventriküler septumun sol ventrikül miyokard duvar kalınlığına oranının 1.18'in altında olmasının, postnatal dönemde önemli HKMP gelişiminin bir göstergesi olabileceği bildirilmiştir 20 . Diyabetik anne bebeğinde hipoglisemi olması, ASH görülme riskini artırmaktadır 32 . Diyabetik anne adaylarında, fetal HKMP nedeniyle fetüsün kaybedildiği olgular da bildirilmiştir.…”

Section: Asimetrik Septal Hipertrofiunclassified

Diyabetik Anne Bebeğinde Görülen Kardiyovasküler Komplikasyonlar

Güvenç¹,

Güzeltaş²

2017

Arşiv Kaynak Tarama Dergisi

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Diabetes mellitus is a condition that has negative effects on fetal development throughout pregnancy and causes metabolic disorders and congenital abnormalities in neonates. The most frequent congenital abnormalities are seen in cardiovascular, gastrointestinal, genitourinary system and central nervous system. The cardiac abnormalities that may develop include ventricular and atrial septal defect, patent ductus arteriosus, asymmetrical septal hypertrophy, large artery transposition, tricuspid atresia and aortic contraction. The infants of diabetic mothers need to be followed up also in respect of congenital heart diseases as of the intrauterine period. In this study, we aimed to evaluate the problems of infants of diabetic mothers and to determine risk factors of mothers during pregnancy.

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“…Indeed, congenital cardiac defects, myocardial hypertrophy, and cardiorespiratory dis- 1. Abbreviations used in this paper: 2,3-DPG, 2,3-diphosphoglycerate; EMD, electromechanical delay; ET, ejection time; Hb, hemoglobin; ICT, isovolumetric contraction time; P50, the oxygen pressure at 50% of the Hb saturation; PEP, preejection period; STI, systolic time intervals. tress are known complications in infants of diabetic mothers (3)(4)(5). There have also been several controversial reports suggesting that hyperglycemia could influence the metabolism of the erythrocytes and produce changes in 02 delivery (6,7).…”

Section: Introductionmentioning

confidence: 99%

Myocardial function and hemoglobin oxygen affinity during hyperglycemia in the fetal lamb.

Bard¹,

Fouron²,

Muylder³

et al. 1986

J. Clin. Invest.

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To determine the effects of maternal hyperglycemia on fetal hemodynamic and cardiac function, a study was carried out on nine chronically catheterized fetal sheep. In six fetuses, glucose was infused intravenously with an initial dose of 5 mg/kg per min. Data were compared with controls. This dose was gradually increased to 16 mg/kg per min by the fifth day. The initial blood glucose was 14.7±3.0 mg/dl and increased to 54.6±16.4 mg/dl by the last day of the infusion period (P < 0.001). The P02 decreased from a baseline of 20.25±3.40 to 15.88±5.24 mmHg (P < 0.01). Similarly significant decreases were also observed for the blood 02 content and 02 hemoglobin saturation: 8.5±1.7 to 6.4±2.2 ml/dl and 62.3±13.6 to 46.1±17.6%, respectively, during hyperglycemia (P < 0.01). The duration of the preejection period (PEP) before the start of the experiment was 45±4 ms; a final value of 57±10 ms was obtained (P < 0.01). However, the electromechanical delay and ejection time (ET) showed no significant variation. The ratio of the PEP/ET increased from 0.31±0.04 to 0.38±0.07 (P < 0.01) during hyperglycemia. The reticulocytes increased from 1.4±1.8 to 3.1±2.9% (P < 0.05) and the 2,3-diphosphoglycerate decreased from 4.4±1.1 to 2.8±1.2 ,mol/g hemoglobin (P < 0.005). This study demonstrated that fetal hyperglycemia depresses myocardial function in the fetal lamb. The changes in cardiac function could not be explained by the small drop in 02 saturation.

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Cardiac septal hypertrophy in hyperinsulinemic infants

Cited by 140 publications

References 18 publications

Severe cardiac phenotype of Berardinelli-Seip congenital lipodystrophy in an infant with homozygous E189X BSCL2 mutation

Severe cardiac phenotype of Berardinelli-Seip congenital lipodystrophy in an infant with homozygous E189X BSCL2 mutation

Diyabetik Anne Bebeğinde Görülen Kardiyovasküler Komplikasyonlar

Myocardial function and hemoglobin oxygen affinity during hyperglycemia in the fetal lamb.

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