[Bronchopulmonary dysplasia-associated pulmonary arterial hypertension of very preterm infants]. / Hypertension artérielle pulmonaire des anciens grands prématurés bronchodysplasiques.

Bronchopulmonary dysplasia (BPD) of very preterm infants is a multifactorial chronic lung disease and its incidence has not decreased despite improvements in neonatal intensive care, including lung protective strategies. Pulmonary hypertension (PH) can complicate the course of BPD. Mortality in infants with BPD-associated PH is thought to be very high, but its incidence is unknown and a standard diagnostic and therapeutic strategy has not been well defined. In this article, we will first describe the current knowledge on the BPD-associated PH and the current treatments available for this pathology. We will then present the HTP-DBP Study, carried out in Paris (France) starting in 2012. The diagnosis of PH is suspected on echocardiographic criteria, but cardiac catheterization is considered the gold standard for diagnosis and evaluation of the severity of PH. Moreover, pulmonary vasoreactivity testing is used to guide the management of patients with PH. The pathogenesis of BPD-associated PH is poorly understood and even less is known about appropriate therapy. Today, optimizing ventilation and reducing the pulmonary vascular tone with specific pulmonary vasodilatator drugs are the main goals in treating HTP-associated DBP. Animal studies and a few clinical studies suggest that medications targeting the nitric oxide (NO) signaling pathway (NO inhalation, oral sildenafil citrate) could be effective treatments for BPD-associated PH, but they have not been approved for this indication. The HTP-DBP study is a French multicenter prospective observational study. The objective is to evaluate the frequency of BPD-associated PH, to describe its physiopathology, its severity (morbidity and mortality), and the effectiveness of current treatments.

[Coagulase-negative staphylococcal osteomyelitis in preterm infants: a proposal for a diagnostic procedure]. / Ostéomyélites à staphylocoque coagulase négative chez le prématuré : proposition de démarche diagnostique.

Acute osteomyelitis, although a rare complication in neonates, is a diagnostic and therapeutic challenge. Successful treatment to avoid functional sequelae depends on early recognition of infection and rapid initiation of therapy. Although Staphylococcus aureus is the most common causative agent, coagulase-negative Staphylococcus (CONS), well known for bloodstream infection, can be involved in neonatal osteomyelitis. Risk factors of osteomyelitis include prematurity and invasive procedures, such as long-term central venous catheterization. We report on 3 cases of acute CONS osteomyelitis in preterm infants presenting with prolonged CONS bacteremia. Bacteremia persisted despite antibiotic treatment in accordance with antibiograms and despite removal of the intravascular device. All catheter cultures were negative and osteomyelitis was not located on the limb where the central catheter had been inserted in all cases. Osteomyelitis diagnosis may be difficult in neonates because of the paucity of clinical signs. In our observations, (99m)Tc scintigraphy was the key investigation for diagnosis and detection of multiple sites of bone infection. The place of this investigation is discussed in relation to other imaging techniques. These observations suggest that in the context of persisting CONS bacteremia, a secondary bone infection should be considered. Scintigraphy is a discriminating diagnostic tool.

Idiopathic male pseudohermaphroditism: variations in presentation and management.

UNLABELLED: Male pseudohermaphroditism (MPH) is the abnormal development of genitalia in an individual with a 46,XY chromosome complement and testicular tissue. The etiology of MPH is unknown in most cases, which are defined as idiopathic. OBJECTIVE: To analyze the data for cases of idiopathic MPH. PATIENTS AND METHODS: A retrospective study of 29 patients with idiopathic MPH and no uterus. RESULTS: Four patients had a family history of abnormal sexual development and five had low birth weight. The initial manifestations were sexual ambiguity (26), microphallus and hypospadias (2), and primary amenorrhea (1). Basal and/or stimulated testosterone concentrations showed insufficient testosterone secretion in three patients. Genitography showed a vagina in 13 patients. Male genitoplasties were performed on 21 out of the 24 patients reared as males and female genitoplasties on five patients. Histological studies of the gonads of these showed streak gonads in one, normal gonads in one and signs of testicular dysgenesis in three others. Molecular studies on the SRY gene (17) showed no mutation. CONCLUSIONS: Idiopathic male pseudohermaphroditism is a heterogeneous condition, even within families with a history of this condition. We propose a set of guidelines for the management of these patients.

[Preventing dehydration with oral rehydration therapy (ORT) in less than 15-day-old newborns: safety, efficiency and limits]. / Soluté de réhydratation orale (SRO) et hydratation entérale du nouveau-né dans les 15 premiers jours de vie: tolérance, efficacité et limites.

OBJECTIVES: We conducted a retrospective evaluation of enteral infusion with a marketed hypoosmolar oral rehydration solution (HORS), as an alternative to intravenous infusion. POPULATION AND METHODS: Premature infants, with difficult venous condition, 30 weeks or more during HORS infusion. Enteral ORS started after well-tolerated milk gastric gavage. Gradual increase of enteral feeding. RESULTS: January 1999 to April 2001, 105 neonates 28 weeks to 36 weeks, birth weight 1050 to 2700g, including 71.5% eutrophic newborns 30 to 34 weeks; 13.3% hypotrophic<10th P. More than 90% had a physiological weight curve: weight loss vs birth<15%, back to birth weight at day 15. No significant pathology during ORS. Failure of ORS for 7/105 children. Relative risk increased 8 fold if term was less than 30 weeks, 7 folds in the event of enteropathy before ORS. In 26.7% of the infants, gastric enteral residuals exceeded 1/3 of intake, vomiting and/or abdominal ballooning lasted less than 48 hours. There were 4 deaths during follow-up (periventricular leucomalacia, myocardial infarctus) and 1 necrotizing enterocolitis. At theoretical birth date, 25% of the neonates were hypotrophic<10th P. At one and 2 years of age, less than 5% were still hypotrophic: relative risk increased 18 fold when birth weight was<5th P. CONCLUSION: HORS is an efficient, well-tolerated, low-cost and less invasive alternative to intravenous infusion. It must be reserved for eutrophic neonates born>30 weeks gestation due to risk of failure and insufficient growth. Validation with a multicentric clinical trial is in progress.