Translation and validation of the French version of the adolescent idiopathic scoliosis Truncal Anterior Asymmetry Scoliosis Questionnaire (TAASQ).

PURPOSE: Scoliosis is an abnormality which causes anterior trunk asymmetry. The Truncal Anterior Asymmetry Scoliosis Questionnaire-adolescent idiopathic scoliosis (AIS) (TAASQ) measures the feeling about anterior trunk appearance in girls with AIS. It comprises 14 questions which evaluate frontal asymmetry of breasts, shoulders, and waist as well as behavioral attitude. There is currently no validated questionnaire in French to evaluate truncal asymmetry in AIS girls. The aim of this study was to translate and validate the French version of the TAASQ (TAASQ-VF). METHODS: The study reports the translation and transcultural adaptation of the TAASQ into French. The translation was tested on 20 patients to verify comprehension and modify if necessary. To assess the reliability of the translated version, each domain as well as the total measure were tested for internal consistency. Convergent validity was evaluated on 63 patients and test-retest on a sample of 15. RESULTS: The translation and content validation process resulted in a French version of the TAASQ. Internal consistency was over 0.80 for each item, over 0.70 for each domain, and 0.88 for the total scores. SAQ were used to perform convergent validity with TAASQ-VF. The TAASQ-FV correlated well with many of the SAQ domains (p < 0.05) and every TAASQ-FV domain correlated with at least one SAQ domain (p < 0.05). Test-retest reliability for the total score and for each domain was good. CONCLUSION: The translation and cross-cultural adaptation of the TAASQ questionnaire provides a French version that can measure the feeling about frontal appearance in girls with idiopathic scoliosis.

Is Infantile Hemangioma a Neuroendocrine Tumor?

Infantile hemangioma (IH) is the most common infantile tumor, affecting 5-10% of newborns. Propranolol, a nonselective ß-adrenergic receptor (ADRB) antagonist, is currently the first-line treatment for severe IH; however, both its mechanism of action and its main cellular target remain poorly understood. Since betablockers can antagonize the effect of natural ADRB agonists, we postulated that the catecholamine produced in situ in IH may have a role in the propranolol response. By quantifying catecholamines in the IH tissues, we found a higher amount of noradrenaline (NA) in untreated proliferative IHs than in involuted IHs or propranolol-treated IHs. We further found that the first three enzymes of the catecholamine biosynthesis pathway are expressed by IH cells and that their levels are reduced in propranolol-treated tumors. To study the role of NA in the pathophysiology of IH and its response to propranolol, we performed an in vitro angiogenesis assay in which IH-derived endothelial cells, pericytes and/or telocytes were incorporated. The results showed that the total tube formation is sensitive to propranolol only when exogenous NA is added in the three-cell model. We conclude that the IH's sensitivity to propranolol depends on crosstalk between the endothelial cells, pericytes and telocytes in the context of a high local amount of local NA.

Who needs prenatal counselling with a pediatric surgeon? Experience from a large tertiary care university hospital.

OBJECTIVES: To describe our experience with prenatal counselling for surgical anomalies in a large volume center. The secondary aim is to suggest a list of prenatal abnormalities warranting counselling by a pediatric surgeon. METHODS: We reviewed all prenatal counselling consultations performed by the pediatric surgery team between January 1st, 2015 and December 31st, 2016. RESULTS: A total of 169 patients or couples had a prenatal consultation with a pediatric surgeon. Prenatal work-up included a fetal MRI in 26% of cases, mainly for digestive and thoracic pathologies (56.1% of cases). Consultation with the pediatric surgeon led mainly to recommendations concerning the place of delivery. Induction for reasons related to the fetal anomaly occurred in 22.2% of cases. Most children were surgically treated within the first year of life (63.5%). Correlation between predicted prognosis and actual status at four years of life was 96.9%. Correlation between prenatal and postnatal diagnosis was 87.4%. CONCLUSIONS: Prenatal counselling by a pediatric surgeon allows couples to obtain clear information on the pathology of their unborn child, giving them greater autonomy in their decision to continue the pregnancy.

Critical role of Aquaporin-1 and telocytes in infantile hemangioma response to propranolol beta blockade.

Propranolol, a nonselective ß-adrenergic receptor (ADRB) antagonist, is the first-line therapy for severe infantile hemangiomas (IH). Since the incidental discovery of propranolol efficacy in IH, preclinical and clinical investigations have shown evidence of adjuvant propranolol response in some malignant tumors. However, the mechanism for propranolol antitumor effect is still largely unknown, owing to the absence of a tumor model responsive to propranolol at nontoxic concentrations. Immunodeficient mice engrafted with different human tumor cell lines were treated with anti-VEGF bevacizumab to create a model sensitive to propranolol. Proteomics analysis was used to reveal propranolol-mediated protein alteration correlating with tumor growth inhibition, and Aquaporin-1 (AQP1), a water channel modulated in tumor cell migration and invasion, was identified. IH tissues and cells were then functionally investigated. Our functional protein association networks analysis and knockdown of ADRB2 and AQP1 indicated that propranolol treatment and AQP1 down-regulation trigger the same pathway, suggesting that AQP1 is a major driver of beta-blocker antitumor response. Examining AQP1 in human hemangioma samples, we found it exclusively in a perivascular layer, so far unrecognized in IH, made of telocytes (TCs). Functional in vitro studies showed that AQP1-positive TCs play a critical role in IH response to propranolol and that modulation of AQP1 in IH-TC by propranolol or shAQP1 decreases capillary-like tube formation in a Matrigel-based angiogenesis assay. We conclude that IH sensitivity to propranolol may rely, at least in part, on a cross talk between lesional vascular cells and stromal TCs.

Congenital haemangiomas: a single-centre retrospective review.

OBJECTIVE: Congenital haemangiomas (CHs) are rare, benign vascular tumours that are fully developed at birth. Three subtypes of CHs have been described based on clinical behaviour: rapidly involuting CHs (RICHs), non-involuting CHs (NICHs) and partially involuting CHs (PICHs). We explore in our study clinical, evolutionary and paraclinical characteristics of the three CH subtypes. DESIGN: Children with CH attending our department of paediatric dermatology at Bordeaux University Hospital over a 13-year period were retrospectively included. Epidemiological, clinical and evolutionary data, photographs and imaging results were reviewed. All available tissue samples were histologically examined. RESULTS: We included 57 patients: 22 with RICH, 22 with NICH and 13 with PICH. Males predominated (ratio 1.7); the most common CH location was on the limbs. RICH, NICH and PICH exhibited overlapping characteristics; all were single telangiectatic lesions with pale peripheral halos. At birth, NICHs were flat but RICHs and PICHs bulky. The median age at complete RICH involution was 12 months. One-third of CHs that appeared RICH-like at birth underwent incomplete involution to become PICHs. Heart failure and thrombocytopenia were rare complications. PICHs were frequently ulcerated. Pain was common for NICH and PICH. The imaging and histological data of the three CH subtypes were rather similar. CONCLUSIONS: We describe the characteristics and evolution of the three CH subtypes using a case series. Certain overlapping features were apparent, reinforcing the hypothesis that RICH, NICH and PICH lie on the same pathological spectrum.

Management of congenital nevi at a dermatologic surgical paediatric outpatient clinic: consequences of an audit survey 1990-1997.

BACKGROUND: Since 1987 we have run a Dermatologic Surgical Paediatric Outpatient Clinic (DSPOC) within the Children's Hospital in Bordeaux. OBJECTIVE: We analyse the consequences of an audit survey concerning the management of patients with congenital nevi (CN) seen at this clinic. METHODS: We reviewed the cases of 192 children examined and photographed at the DSPOC during the period January 1990-December 1997. Patients were contacted for a reassessment of their status. The management options chosen at the DSPOC were reviewed as well as the satisfaction of the patients or parents of young children. RESULTS: Of 192 children pre-recruited, 56 girls and 52 boys could be included in the survey. They were mostly European whites and 67% were <6 months of age at the first DSPOC visit. 65/108 (61%) had been operated following the first DSPOC visit. The mean follow-up based on the 1997-1998 survey was 33 months (8 months to 10 years). The size of the nevus, independently of location, influenced decision for early surgery. Another important factor was the estimated disfigurement risk (15% of decisions) mostly related to CN of the face. There was a significant risk of pigmentary recurrence around the scar in children operated before the age of 2, but long-term follow-up indicated a spontaneously regressive course. CONCLUSIONS: Nevus recurrence in cases operated early suggests a time-dependent phenomenon in nevogenesis. Early counselling is important. Early surgery seems associated with a better scar quality. Explanations concerning risks and outcome are best given with the cooperation of a surgeon and a dermatologist.