Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
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Case Reports
[Hereditary neuralgic amyotrophy: a paediatric and familial presentation of Parsonage-Turner syndrome].
Hereditary neuralgic amyotrophy is a rare disorder, characterized by recurrent attacks of pain in a brachial plexus distribution. We report the case of a 12-year-old boy with several attacks of pain and atrophy of the muscles of the shoulders. The age of onset of this disease is variable, most frequently in the second or third decade. ⋯ The analysis of several families has shown that hereditary neuralgic amyotrophy phenotype is heterogeneous and two different clinical courses can be discerned. Recent evidence indicates that HNA is genetically heterogeneous. Pathophysiology of the disease remains unclear, so the treatment is not clearly established.
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The modalities of an inhaled treatment depends on the characteristics of the aerosol, on the age of the child, and mainly on the inhalatory system. Before the age of 8 years, the pressurised metered dose inhaler must be used together with a spacer device. Above this age, the use of a breath-actuated inhaler or of a dry powder inhaler can be proposed. Demonstration and educational instructions will increase the probability of compliance and efficiency of the treatment.
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Preterm babies born before the 33rd week of gestation often exhibit primary surfactant deficiency responsible for the respiratory distress syndrome or hyaline membrane disease. In that situation, there is a limited and insufficient production of surfactant by type II alveolar cells of the lung due to immaturity. Secondary surfactant deficiencies occur in patients with prior normal surfactant synthesis and can be related to sepsis, hypoxia, ventilator induced lung injury or surfactant inhibition by a variety of substances reaching the alveolar spaces. ⋯ Treatment with exogenous surfactant may be beneficial. There is a need for randomized controlled studies for evaluation of this treatment. Next generation of surfactants containing recombinant surfactant protein or synthetic peptides appear as promising agents in these situations of secondary surfactant deficiencies.