Developmental outcome of children with Robin sequence: How does the question arise?

Robin sequence (RS) is a heterogeneous congenital condition characterized by retrognathia, glossoptosis, upper airway obstruction, and very often, posterior U-shape cleft palate. Half the children with RS have an underlying syndrome, either identified (syndromic RS) or not (RS+). Long-term intellectual developmental outcome first depends on the underlying diagnosis and is often poor in syndromic cases. On the contrary, the rare studies that analysed the long-term developmental outcome of children with isolated RS who received effective treatment of their respiratory and feeding difficulties early in life, showed intellectual and academic results close to or within the normal range. Speech outcome in RS is often delayed with phonation disorders. Speech difficulties depend on intellectual level, hearing and velar function after palate repair. It affects most children with RS and deserves active monitoring and care.

[Severe human parechovirus-3 sepsis in a 6-week-old infant]. / Infection sévère à paréchovirus de type 3 chez un nourrisson de 6semaines.

Febrile infants under 3 months of age are often treated with broad-spectrum intravenous antibiotics while awaiting culture results, to prevent mother-to-child bacterial infections. Human parechoviruses (HPeV) have recently been described as etiologic agents of meningitis and severe sepsis in neonates and young infants. They are rarely investigated and are therefore probably underestimated. They cause acute clinical symptoms that can incorrectly suggest a bacterial infection. In the present case, a 6-week-old infant infected with HPeV developed severe sepsis, complicated by hepatic cytolysis, meningitis, acute renal failure, and mild hemophagocytic lymphohistiocytosis. HPeV type 3 was found by routine specific RT-PCR in cerebrospinal fluid, stools, and plasma. The outcome was spontaneously favorable after 4 days. Early diagnosis of the HPeV infection by routine specific RT-PCR reduces unnecessary antibiotic use and extended hospitalization in febrile young infants.

[Afebrile seizures in gastroenteritis: a Japanese peculiarity]. / Convulsions sans fièvre et gastroentérite aiguë: une spécialité japonaise.

Febrile seizures appearing during acute gastroenteritis have been described in japanese populations. These convulsions are not related to clinical signs of dehydration or electrolyte disorder. This entity was called CwG, benign Convulsions with mild Gastroenteritis. We report the case of a 19 month-old japanese boy who presented with a CwG. We described the characteristic clinical features of this entity and we reviewed the cases reported in literature. The evolution of the CwG is always simple without relapse or side effects. Better understanding will help pediatricians make more accurate diagnosis and avoid treatment even though initial signs might be severe.