Bronchopulmonary dysplasia and pulmonary hypertension: a meta-analysis.

OBJECTIVE: Pulmonary hypertension (PH) is a complication of bronchopulmonary dysplasia (BPD) but the true impact of PH in patients with BPD remains unclear. We sought to systematically review and meta-analyze incidence of PH in BPD and compare clinical outcomes of BPD patients with PH to those without PH in preterm infants. STUDY DESIGN: Medline, Embase, PsychINFO and CINAHL were searched from January 2000 through December 2015. Cohort, case-control and randomized studies were included. Case-reports, case-series and letters to editors and studies with high risk of bias were excluded. Study design, inclusion/exclusion criteria, diagnostic criteria for BPD and PH and outcomes were extracted independently by two co-authors. RESULTS: The pooled incidence of PH in patients with BPD (any severity) was 17% (95% confidence interval (CI) 12 to 21; 7 studies) and 24% (95% CI 17 to 30; 9 studies) in moderate-severe BPD. Patients with BPD have higher unadjusted odds of developing PH compared to those without BPD (odds ratio (OR) 3.00; 95% CI 1.18 to 7.66; 4 studies). Patients with BPD and PH were at higher odds of mortality (OR 5.29; 95% CI 2.07 to 13.56; 3 studies) compared with BPD without PH, but there was no significant difference in duration of initial hospitalization, duration of supplemental oxygen requirement or need for home oxygen. No studies included in this review reported on long-term pulmonary or neurodevelopmental outcomes. CONCLUSIONS: PH occurs in one out of 4 to 5 preterm neonates with BPD. Patients with BPD and PH may have higher odds of mortality; however, there is urgent need for high quality studies that control for confounders and provide data on long-term outcomes.

The German national registry for primary immunodeficiencies (PID).

In 2009, a federally funded clinical and research consortium (PID-NET, http://www.pid-net.org) established the first national registry for primary immunodeficiencies (PID) in Germany. The registry contains clinical and genetic information on PID patients and is set up within the framework of the existing European Database for Primary Immunodeficiencies, run by the European Society for Primary Immunodeficiencies. Following the example of other national registries, a central data entry clerk has been employed to support data entry at the participating centres. Regulations for ethics approvals have presented a major challenge for participation of individual centres and have led to a delay in data entry in some cases. Data on 630 patients, entered into the European registry between 2004 and 2009, were incorporated into the national registry. From April 2009 to March 2012, the number of contributing centres increased from seven to 21 and 738 additional patients were reported, leading to a total number of 1368 patients, of whom 1232 were alive. The age distribution of living patients differs significantly by gender, with twice as many males than females among children, but 15% more women than men in the age group 30 years and older. The diagnostic delay between onset of symptoms and diagnosis has decreased for some PID over the past 20 years, but remains particularly high at a median of 4 years in common variable immunodeficiency (CVID), the most prevalent PID.

[Congenital nasal pyriform aperture stenosis: an uncommon, potentially fatal cause of inhibition of nasal breathing in neonates]. / Kongenitale Stenose der Apertura piriformis: Seltene, potenziell lebensbedrohliche Ursache einer Nasenatmungsbehinderung bei Neugeborenen.

Congenital nasal pyriform stenosis can be a life threatening emergency in neonates which must be included in the differential diagnosis of nasal airway obstruction. We describe the case of a male child who developed symptoms 36 h after birth. As the conservative therapy was not successful surgery became necessary.

[Traumatic amputation of the lower limb and traumatic hemipelvectomy. Report of 2 cases in children and review of the literature]. / Amputation traumatique du membre inférieur et de l'hémi-bassin, Présentation de deux cas chez l'enfant et revue de la littérature.

Traumatic hemipelvectomy is rarely observed, mostly because very few patients survive the initial trauma. We describe 2 cases of children who survived this trauma. The first was a 12 year-old boy who accidentally fell between a metro train and the platform. He had a severe open trauma of the pelvis with important hemorrhage. After a first operation to stop hemorrhage and do a cystotomy and a colostomy, ischemia of the lower limb led to an inter-ilio-abdominal amputation. Two months were necessary to heal this. A prosthesis was made 12 months after the accident, postponed by urinary problems. The boy also had a traumatic amputation of the left arm and an upper metaphyseal fracture of the humerus. The second case was that of an 8 year-old girl who suffered a complete traumatic amputation of the hemipelvis after a road accident. A secondary colostomy was performed because of a local infection. Once healing was achieved, the child was low to follow-up. In the literature, we found 36 survivors to this dreadful lesion (4 of them children). We analysed the different problems for the treatment of this trauma caused, and their short term consequences. An early evaluation of the lesions, rapid and intensive resuscitation, early decision to complete the traumatic amputation if necessary, with urinary and digestive derivation, are important elements towards eventual successful outcome of the treatment.