Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
-
Langerhans cell histiocytosis (LCH) is characterized by inflammatory lesions containing abundant CD1a+ CD207+ histiocytes that lead to the destruction of affected tissues. This disease has a remarkable pleiotropic clinical presentation and most commonly affects young children. Although the current mortality rate is very low for childhood LCH patients (<2%), reactivation frequently occurs after a long period of disease control and the rates of permanent complications and sequelae remain high. ⋯ Quantification of the plasmatic BRAFV600E load for this group of patients can precisely monitor response to therapy. Finally, targeted therapies, such as BRAF inhibitors, are new therapeutic options especially designed for refractory multisystemic LCH involving risk organs. However, the long-term efficacy, long-term tolerance, optimal protocol scheme, and appropriate modalities of administration for these innovative therapies for children still need to be defined, a huge challenge.
-
Review Case Reports
[Perineal groove: Case report and review of the literature].
Perineal groove is a rare anoperineal congenital malformation, usually occurring in girls, unknown to many pediatricians. We report the case of a 17-day-old girl admitted for urinary infection, who presented a wet sulcus with mucous membrane, extending from the vaginal fourchette to the anterior border of the anus. ⋯ This benign malformation tends to be self-epithelialized in the 1st year of life, and no surgical treatment is recommended, except for cosmetic reasons or for recurrent infections. This diagnosis must be known so as to avoid unnecessary explorations and anxiogenic misdiagnosis.
-
In the last 20years, culture-independent DNA-based techniques ("shotgun sequencing") demonstrated that complex microbial communities reside on most epithelial surfaces, including the lower airways. Until the amniotic sac ruptures, a fetus is considered to be essentially sterile. Many factors affect the composition of the lung microbiota: inheritance, mode of delivery, diet, and age-related changes in adults. ⋯ Animal models show that these interactions play a role in innate pulmonary immunity and modulation of the inflammatory response. The microbial composition of the airway microbiota differs between healthy children and those with chronic lung disease. The advances in the comprehension of microbiome changes have resulted in new approaches concerning the microbiota for treatment and prevention of disease.
-
Postinfectious erythema multiforme is an uncommon skin disease in childhood that can have a strong impact, especially in infants if there is involvement of the mucous membranes. The lesion is targeted (central bullous lesions with three concentric circles). Its diagnosis is typically made clinically. ⋯ More recently, postvaccination erythema multiforme has been reported with the majority of vaccines used in pediatric practice. The prognosis is usually good but requires observation of the mucosal involvement because of the risk of serious complications. The treatment of erythema multiforme is essentially symptomatic, with etiology-related treatment.
-
Cannabis is the most widely used illicit drug in the world. In France, cannabis use has been increasing among youth since 2011, in both experimental use and regular consumption. A distinct syndrome, characterized by recurrent vomiting associated with abdominal pain and compulsive bathing, has been increasingly recognized in adult chronic users. ⋯ To date, the specific etiology of CHS is unknown as is the pathophysiology of improvement with hot baths. All youth presenting with cyclic vomiting should be questioned about cannabis use and compulsive hot bathing. The early recognition of this syndrome will save unnecessary and invasive investigations.