La ventilation à l''Ambu chez le nouveau-né : une simple question de doigté ?

Insuflation pressures were measured during manual ventilation using a neonatal rebreathing bag (Ambu^®) on a manikin. Maximal insufflation pressures were greater than that published or given by the manufacturer, theoretically limited to 30 cm of water at open valve, and that wathever the number of fingers used for the compression of the bag. These results indicate that Ambu^® ventilation, often mandatory for newborn resuscitation, does not simply rely upon the finger-touch of the operator and that it always has a risk of baro and/or volotraumatism.     

Association d''une dysgénésie gonadique mixte et d''une forme non classique de bloc de la 21-hydroxylase

Background.

The rare association of mixed gonadal dysgenesis and non classical congenital hyperplasia by 21-hydroxylase deficiency poses the problem of their respective responsability in the development of sexual ambiguity.

Case report.

In a newborn with ambiguous genitalia, blood 17-OH progesterone was moderately elevated (3.9 to 14.1 ng/mL) leading to the diagnosis of non-classical 21 hydroxylase deficiency. Molecular studies later confirmed this diagnosis. However, the presence of a palpable gonad and the caryotype (45 X/46 XY mosaicism) indicated a mixed gonadal dysgenesis as the cause of sexual ambiguity. Histological examination revealed the presence of a testis and a streak gonad.

Conclusion.

This observation emphasizes the need for a complete clinical and biological analysis in all newborns with sexual ambiguity.     

Le test aux androgènes : comparaison d''un test faible et d''un test fort sur le développement du pénis dans les pseudohermaphrodismes masculins

Background.

The androgen sensitivity test used in male pseudohermaphroditism for clinical assessment of the androgen sensitivity and prediction of penile development is an important element in choice of gender. However, there is a wide range of testosterone dosage and no standardized test.

Methods and patients.

Two doses (25 mg and 100 mg) of testosterone heptylate were used in six cases of male pseudohermaphrodism with sexual ambiguity and small penis (ages 6 to 18 months). The clinical results were compared with those of the study of androgen receptors.

Results.

In two cases, both low-dose and high-dose tests resulted in only minimal changes in the penis. In two cases, the low-dose test gave a good result which was confirmed by the high-dose test; on the other hand, in two cases, the low-dose test was considered to be negative whereas the high-dose test led to the development of a normal-sized penis. In all cases except one, there was good concordance between the results of study of androgen receptors and those of the clinical test.

Conclusion.

The high-dose androgen test is thus useful in both diagnosis and treatment and facilitates the gender assignment.     

Ischémie de la main révélant une maladie de KawasakiDistal arm ischemia revealing Kawasaki disease.

An aneurysm of the medium arteries is one of the major complications of Kawasaki syndrome. Eleven cases of giant coronary aneurysm have been reported. Case report. – We report a new case in a nine-month-old child. Ischemia of the left hand was the first symptom. Diagnosis was made 30 days later. Anticoagulant and fibrinolytic treatments did not prevent necrosis of four fingers. Conclusion. –The prognosis of Kawasaki disease depends on early diagnosis and management. Infants younger than seven months of age are more susceptible to developing coronary and mid-artery aneurysms, which therefore justifies a close observation during the acute phase and later on. In the case of a coronary artery aneurysm, Doppler echography of the upper and lower limbs is mandatorily recommended. Anticoagulant therapy should be started once the diagnosis of mid-artery aneurysms is made. Ischemia of the extremities can lead to necrosis and amputation. Until now, anticoagulants, vasodilators and fibrinolytic agents were not proven to be effective.