[Strawberry and raspberry anaphylaxis]. / Allergie immédiate à la fraise et à la framboise.

Strawberry IgE-mediated hypersensitivity is often reported rarely confirmed. Only a few cases are described in medical literature, unlike other fruits of the rosacea family. Strawberry is rich in histamine. It can cause histamine release syndrome, especially when eaten in large quantities. However IgE-mediated hypersensitivity exists. We reported the case of a 9-year-old boy with a history of strawberry and raspberry anaphylaxis.

L'allergie à la fraise est souvent rapportée, rarement confirmée. Il existe peu de cas décrits dans la littérature, contrairement aux autres fruits de la famille des rosacées. La fraise est un fruit riche en histamine et peut donc être responsable de fausses allergies, surtout en cas d'ingestion en grandes quantités. Cependant, de réelles allergies, médiées par les IgE, existent. Nous rapportons le cas d'un garçon, âgé de 9 ans, qui a présenté, à deux reprises, une anaphylaxie à la fraise et à la framboise.

Cardiac autonomic dysfunction in diabetic children.

OBJECTIVE: Adults with type 1 diabetes may have abnormal alterations in heart rate variability (HRV) due to cardiac autonomic neuropathy. This prospective study was performed to determine whether HRV can be used to detect subclinical autonomic neuropathy in diabetic children. RESEARCH DESIGN AND METHODS: We examined five time domain and three frequency domain HRV indices determined from 24-h Holter recordings in 73 diabetic children and adolescents aged 3-18 years (mean 12.1 years) with a mean duration of diabetes of 55 months. The measures were compared with normal ranges. Z scores were established for each parameter and were compared with classic risk factors of other diabetic complications. RESULTS: Most HRV indices were significantly depressed in children aged > or = 11 years, and the levels of HRV abnormalities were significantly correlated with long-term metabolic control (mean GHb for 4 years) in that age-group. In younger patients, HRV indices were within the normal range and were not correlated with the level of metabolic control. Illness duration and microalbuminuria but not short-term metabolic control (most recent GHb) were also independently predictive of HRV abnormalities. CONCLUSIONS: These results suggest that early puberty is a critical period for the development of diabetic cardiac autonomic dysfunction. Therefore, all type 1 diabetic patients should be screened for this complication by HRV analysis beginning at the first stage of puberty regardless of illness duration, microalbuminuria, and level of metabolic control.

[Pompe disease or type 2 glycogenosis]. / La maladie de pompe ou glycogénose de type 2.

We report the case of a rapidly progressing respiratory failure of a three-month old infant, who shows a cardiomyopathy with left ventricule hypertrophy leading to a Pompe's disease diagnosis. This type 2 glycogenosis will be confirmed by the enzymatic study of the hepatocytes. It is a genetic pathology associated with a deficient activity of the acid maltase resulting in a intralysosomial accumulation of glycogen. The most generally responsible mutations are: delta 18 et delta 525. Prognosis is quite bad with a progressive deterioration of the heart, brain and muscular functions causing death at around 4 to 8 months. Type 2 glycogenosis may be diagnosed before birth through enzymatic study of a material collected through an amniocentesis or a biopsy on chorionic villi, and through DNA analysis. Curative treatment is still under study, but two main research orientations are being developed: genic therapy using viral vector and the correction of the enzymatic deficiency thanks to the synthesis of alpha glucosidase modified to specially get affixed to the heart and muscular cells. Both techniques have already show some encouraging results even though the clinical applications are not presently valid yet.

Correlations between indices of heart rate variability in healthy children and children with congenital heart disease.

Heart rate variability, as determined from 24-hour Holter recordings, represents a noninvasive parameter for studying the autonomic control of the heart. It decreases with certain disease states characterized by autonomic dysfunction such as congestive heart failure. No study in healthy or cardiac children has been performed to determine the correlations between and within time and frequency domain indices of heart rate variability. We examined five time domain (SDNN, SDNNi, SDANNi, rMSSD and pNN50) and five frequency domain measures (ULF, VLF, LF, HF and balance LF/HF) in 200 healthy children and 200 children with congenital heart disease, aged 3 days to 14 years. All measures were significantly correlated with each other. However, the strength of correlation varied greatly. Our data show that variables strongly dependent on vagal tone (rMSSD, pNN50 and HF) were highly correlated (r value > 0.90), as well as SDNN and SDANNi. We conclude that certain time and frequency domain indices correlate so strongly with each other that they can act as surrogates for each other.

Heart rate behavior in children with atrial septal defect.

The aim of this study was to assess heart rate variability, a noninvasive parameter for studying the neurohumoral control of heart rate, in children with atrial septal defect. We examined five time-domain and three frequency-domain indices of heart rate variability determined from 24-hour Holter recordings in 20 children, aged 3-14 years, with secundum atrial septal defect who were about to undergo operation. The measures were compared to normal ranges and to the hemodynamic data of the preoperative cardiac catheterization. Most indices of heart rate variability were moderately depressed in children with atrial septal defect. Our data revealed negative correlations between indices and the mean right atrial pressure and the enddiastolic right ventricular pressure, respectively. The study demonstrates the importance of the right ventricular filling pressures for the autonomic control of the heart in children with atrial septal defect.