[Vestibular neuronitis in a teenager with sickle cell disease. Treatment is urgent]. / Syndrome vestibulaire chez une adolescente drépanocytaire SC. Le traitement est urgent.

Vestibular syndrome is not frequently described in patients with sickle cell disease. We report the case of a teenager with sickle cell disease who had a vestibular syndrome with vertigo that successfully responded to exchange transfusion. We discuss guidelines and review the literature in view of this case report. Sensorineural disorders should be considered as stroke syndromes. They require urgent treatment consisting of exchange transfusion or maintaining optimal hydration associated with blood withdrawal. Treatment of vestibular syndrome in sickle cell disease is urgent.

Gastroschisis and omphalocele: retrospective study of initial postoperative management in the ICU.

AIM OF THE STUDY: The purpose of this study is to describe the management of infants with gastroschisis (G) and omphalocele (O) during the first 7 days after surgery. METHODS: A retrospective review of all cases of O or G managed at the ICU of the Robert Debré Teaching Hospital between January 1993 and July 2000 was carried out. PATIENTS: 29 infants with G, 15 with O (12 unruptured O [UO] and 3 ruptured O [RO]). RESULTS: Ventilatory support consisted of conventional mechanical ventilation (46 %) and/or in high-frequency oscillatory ventilation (61 %). After day 4, ventilatory requirements evaluated by mean airway pressure (MAP) differed significantly between G (n = 10/29) and O (n = 7/15; group vs. day of life, p = 0.04). The average of MAP measured on days 5, 6, and 7 was significantly higher in O than in G (14.7 +/- 3.0 versus 10.9 +/- 2.8, p < 0.01, respectively). Volume expansion was required at least once in 90 % of patients. Mean fluid requirements were significantly lower in UO than in G and in RO (41 +/- 31 ml/kg, 91 +/- 73 ml/kg, and 137 +/- 25 ml/kg, respectively; p = 0.02 for each comparison). Patients with G were significantly more likely to receive norepinephrine (59 % vs. 20 %, p = 0.027) than patients with O. Twenty-six infants with G (90 %) and 11 with O (73 %) were discharged alive from ICU. CONCLUSIONS: Haemodynamic instability can be expected in patients with G or RO, and ventilatory requirements were higher in infants with O than in infants with G during the first week after surgery.

[Antenatal corticosteroids: benefits and risks]. / Corticothérapie anténatale: bénéfices et risques.

A favorable benefit-to-risk ratio is well established for a single dose of antenatal corticosteroids in women at risk of preterm delivery. The efficacy is real with an important decrease of mortality and morbidity. No adverse effects were described after one course. Possible beneficial effects of repeated courses include lower rates of RDS and a decrease in oxygen use, whereas an increasing body of evidence raises the concern of multiple short and long term adverse consequences, principally neurological. It seems rational to prescribe one course of corticosteroids. The indication for a second course should be discussed but multiples courses of this treatment should not be prescribed. We prefer betamethasone over dexamethasone because of the better side profile. Further work is needed to understand the long-term effects of this treatment.

[Erythropoietin and anemia in premature infants]. / Erythropoïétine et anémie du prématuré.

Anemia of prematurity is characterized by low reticulocyte counts and inadequate erythropoietin response, for which many premature infants receive multiple blood transfusions. To reduce the number of those transfusions, treatment with EPO and iron supplementation is routinely used in premature infants. Even if the efficacy of this treatment is demonstrated, the results are not so good in the very low birth weight infants or very small gestational age infants and the need of transfusion is still important. This is due for a large part to blood loss in these very small infants. But there are also other explanations. Thus the pharmacokinetics of EPO is different in premature infants and newborn than in adults. Best dose, best way of administration (i.v. or subcutaneous), best number of injections per week are not already known. Further study has to be done to achieve a better use and efficacy of this treatment.

[A case of atrial flutter after umbilical venous catheterization]. / Une observation de flutter auriculaire après pose de cathéter veineux ombilical.

UNLABELLED: Isolated atrial flutter is an extremely rare form of supraventricular tachycardia in the neonatal period. It may be initiated by central venous catheterization. CASE REPORT: A male infant was born at 35 weeks by cesarean section for placenta praevia. He was eutrophic. Apgar score was 10 at 1 and 5 minutes. He secondary developed a respiratory distress syndrome. He was then ventilated by nasal CPAP. Immediately after an umbilical venous catheterization, a tachycardia appeared without preexistent cardiac dysfunction. An intravenous dose of adenosine (Striadyne) showed a characteristic sawtooth pattern of P waves on inferior leads. The cardiac-US examination was normal. This atrial flutter was converted to normal sinus rhythm by transoesophageal pacing, without adjunction of antiarrhythmic drugs. The newborn was weaned from mechanical ventilation 48 hours later and discharged from hospital at seven days post natal age. His development and clinical examination were normal two months later. CONCLUSION: The isolated atrial flutter is rare in the neonate. It may be triggered by a venous catheterization. Transoesophageal atrial pacing is safe and effective for conversion.

[Severe neonatal bacterial infections]. / Infections bactériennes graves du nouveau-né.

The prognosis of septicemic forms of early and late neonatal sepsis is severe with a high rate of mortality especially in premature infants. The evaluation of severity is difficult because of the non specificity of the clinical signs and mortality seems to be a good means of evaluation. A study was conducted in France on the mortality due to infection in neonatal intensive care units and neonatology wards during the third trimester 2000. Among 18 units, the mortality rate was 9.3% of admissions, corresponding to 11 early onset sepsis and 17 nosocomial infections. Death in primitive infections is essentially due to group B streptococci and E. coli with a more important risk in low gestational age infants. The nosocomial infections arise almost only in premature infant. Prognosis of infections due to Staphylococcus coagulase negative staphylococci, most frequent pathogens is good but mortalities rate is higher for enterobacteriacae--40% and for Pseudomonas, 62%.

[Prolonged length of stays in pediatric intensive care. Retrospective study of 100 stays]. / Les séjours prolongés en réanimation pédiatrique. Analyse rétrospective de 100 séjours.

UNLABELLED: New issues have arisen in pediatric intensive care units, especially concerning long-stay patients. The aims of the present study were to describe the etiologic factors of these long-stay patients and to recognize the comorbidities. MATERIAL AND METHODS: Ninety-five patients who had a total of 100 hospitalizations of more than 30 days were admitted to the pediatric intensive care unit at Robert-Debre Hospital during a 3-year period (1993-1995); this accounted for 9.1% of total admissions. We retrospectively reviewed these 100 long-stay hospitalizations. RESULTS: Most of these patients were newborns (65%). Patients with severe congenital anomalies (44 patients) and very premature infants (26 patients) constituted the majority of long-stay patients. The mean duration of mechanical ventilation for the 95 patients was 110 days (ranges 17-789 days). Two factors of comorbidity were found: gastroesophageal reflux (41% of cases) and nosocomial infections (89% of cases). CONCLUSION: In order to prevent long stays, pediatric intensive care units must be directed toward these factors.

Improvement of mesenteric superior artery Doppler velocimetry by amnio-infusion in fetal gastroschisis.

This study aimed to analyse the influence of amnio-infusion on superior mesenteric Doppler velocimetry and to correlate Doppler data with outcome in fetuses presenting with gastroschisis. Umbilical and intra- and extra-abdominal superior mesenteric artery Doppler velocimetry was measured prospectively before and after amnio-infusion in fetuses with gastroschisis. Doppler index values and changes were correlated with the following outcome parameters: importance of fibrous coating, duration of hospitalization in the neonatal intensive care unit, total duration of hospitalization, duration of parenteral feeding, duration of ventilatory assistance, and the interval to initiation of oral feeding. A significant improvement of diastolic flow was observed in the extra-abdominal superior mesenteric artery with amnio-infusion. No similar change was seen in the intra-abdominal superior mesenteric or in the umbilical artery. Pre-amnioinfusion extra-abdominal mesenteric Doppler index was correlated with maximal diameter of exteriorized bowel and slightly with duration of hospitalization in the neonatal intensive care unit, but no other relation between Doppler index and outcome parameter could be ascertained. Amnio-infusion induces modifications in the vascularization of exteriorized bowel in gastroschisis. This could partly explain the beneficial effect of this procedure on fetal gut.

Dypiridamole, a cGMP phosphodiesterase inhibitor, transiently improves the response to inhaled nitric oxide in two newborns with congenital diaphragmatic hernia.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) remains a frustrating cause of respiratory failure associated with persistent pulmonary hypertension of the newborn (PPHN). Although inhaled nitric oxide (iNO) is effective in many infants with PPHN, it often fails to improve oxygenation in infants with CDH. As the increase in vascular smooth muscle cyclic guanosine monophosphate (cGMP) in response to iNO may be impeded by increased phosphodiesterase type-V (PDE-V) activity, it has been suggested that PDE-V blockade potentiates the efficiency of iNO. CASE REPORTS: We used dypiridamole (Persantine), a specific PDE-V inhibitor, in two patients with CDH. Prenatal diagnosis showed a left-sided CDH at 23 weeks of gestation (GA) with intrathoracic stomach and left heart underdevelopment in the one infant and a right-sided CDH at 26 weeks GA with intrathoracic liver in the other. After antenatal corticoids, planned delivery was performed by the vaginal route at 38 weeks GA. Preoperative stabilization was achieved by high frequency oscillation, iNO and inotropic support over 24 h. Both had early pneumothorax drained by a chest tube. Despite optimization of ventilatory and hemodynamic support with surfactant replacement, iNO and adrenaline, oxygenation worsened progressively. Dypiridamole was introduced intravenously at 27 and 40 h, respectively, and improved oxygenation over the next 12 h. However, oxygenation again deteriorated and both patients died. CONCLUSION: Dypiridamole enhanced the response to iNO in PPHN associated with CDH, although this effect was transient. Combined therapy of iNO with PDE-V inhibitors may improve pulmonary vasodilation in some forms of PPHN which do not respond to iNO, thereby reducing the need for extracorporeal membrane oxygenation (ECMO) and improving outcome.

[Congenital diaphragmatic hernia. II. Is pulmonary hypoplasia an indefinable obstacle?]. / Hernie diaphragmatique congénitale II. L'hypoplasie pulmonaire, obstacle incontournable?

Despite major insights into the pathogenesis and pathophysiology of congenital diaphragmatic hernia, and despite the availability of an antenatal diagnosis and continuous progress in neonatal intensive care, little improvement has been obtained in the prognosis of this malformation. Thus obstetricians, neonatologists and pediatric surgeons are still facing a several dilemma: dilemma before birth to predict the prognosis, i.e., to evaluate the severity of the associated pulmonary hypoplasia in order to decide whether or not to interrupt pregnancy; dilemma after birth in case of severe respiratory failure to decide how far to go in life support. Based on a review of the literature and their own experience, the authors attempt to recapitulate the perinatal management and outcome of this severe malformation.

Acute respiratory distress syndrome in children.

The adult (acute) respiratory distress syndrome is a significant cause of morbidity in children. The mortality rates remain elevated, greater than 50%, and even greater than 80% in patients with underlying malignancies. The therapeutic interventions remain mainly supportive. Strategies of conventional mechanical ventilation are directed toward the use of high positive end-expiratory pressures, low positive inspiratory pressure, and permissive hypercapnia. High-frequency oscillatory ventilation and tracheal insufflation are not yet used extensively, although they should contribute to less aggressive ventilation. Surfactant replacement, nitric oxide inhalation, and partial liquid ventilation seem to be promising technologies, but controlled clinical studies are necessary before their wide-spread use. Extracorporeal membrane oxygenation remains the alternative technology in case of failure of conventional support.

Mycobacterial lung disease in cystic fibrosis: a prospective study.

BACKGROUND: Patients with cystic fibrosis (CF) may be predisposed to airway infections with unusual organisms, such as mycobacteria. The aim of the study was to determine the incidence and clinical picture of mycobacterial infection in CF children. METHODS: At least 2 acid-fast bacillus (AFB) smears and mycobacterial cultures were performed on a prospective basis on 682 sputum specimens from 106 patients during a 1-year period. RESULTS: Thirty-three percent of the cultures were contaminated with other bacteria. Seven children had at least one sputum culture positive for one mycobacterium. Five children had only one positive AFB culture. Their clinical status and lung function remained stable during follow-up. Two teenagers with severe lung disease had several positive AFB smears and cultures for Mycobacterium chelonae and Mycobacterium abscessus. The isolation of M. chelonae and M. abscessus was associated with a clinical and functional decline. Clarithromycin treatment resulted in temporary improvement with the disappearance of the mycobacteria after 6 months of treatment. This prospective study shows an incidence of 2.3% for positive cultures. The prevalence was 6.6% for mycobacterial colonization but only 1.9% for mycobacterial lung disease in our pediatric population. CONCLUSIONS: We recommend performing AFB smears and cultures in CF children with severe lung disease and/or during a lung exacerbation. In these patients persistence of M. chelonae or M. abscessus in sputum should lead to consideration of treatment with clarithromycin.