[Radiation doses received by premature babies in the neonatal intensive care unit]. / Doses d'irradiation reçues par les prématurés en service de réanimation.

PURPOSE: Because of frequent radiological investigations performed in neonatal intensive care unit, a dosimetry study was carried out to assess the level of doses received by premature babies. MATERIALS AND METHODS: In vivo measurements were performed and effective doses were evaluated for single radiographs. Individual cumulative doses received over the period of stay were then estimated, for each premature baby entering the intensive care unit in 2002, taking into account the number of radiographs they underwent. RESULTS: On average, babies stayed for a week and more than one radiograph was taken per day. Results showed that, even if average doses per radiograph were relatively low (25 microSV), cumulative doses strongly depended on the length of stay, and can reach a few mSv. CONCLUSION: Even if doses per radiograph are in agreement with European recommendations, optimisation of doses is particularly important because premature babies are more sensitive to radiation than adults and because they usually undergo further radiological examinations in other services. On the basis of the results of this dosimetry study, the implementation of a larger study is being discussed.

Perinatal-lethal Gaucher disease.

Gaucher disease is a lysosomal storage disease caused by glucocerebrosidase deficiency. Although purely visceral in most cases, some Gaucher disease patients have neurological signs. Signs of Gaucher disease appear after a symptom-free period, except in rare cases with fetal onset. The description of such cases was based mainly on single reports and siblings. We report here a series of perinatal-lethal Gaucher disease cases highlighting the specificity of this phenotype. We retrospectively studied eight original cases of proven Gaucher disease with fetal onset. Non-immune hydrops fetalis was present in all cases but one, and associated with hepatosplenomegaly, ichthyosis, arthrogryposis, and facial dysmorphy. The similarities between our cases and 33 previously described cases allow us to better delineate the perinatal-lethal Gaucher disease phenotype. Hydrops fetalis, in utero fetal death and neonatal distress are prominent features. When hydrops is absent, neurological involvement begins in the first week and leads to death within three months. Hepatosplenomegaly is a major sign, and associated with ichthyosis, arthrogryposis, and facial dysmorphy in some 35-43% of cases. Perinatal-lethal Gaucher disease is a specific entity defined by its particular course and signs that are absent in classical type 2 Gaucher disease. Our study provides clues to the diagnosis of this likely underdiagnosed condition, which must be biochemically confirmed in order to propose appropriate genetic counselling.

[Extracorporeal oxygenation in newborn infant born to diabetic mother with severe hypertrophic myocardiopathy]. / Oxygénation extracorporelle chez un nouveau-né de mère diabétique avec une myocardiopathie hypertrophique sévère.

UNLABELLED: Hypertrophic cardiomyopathy is a common complication in infants born to diabetic mothers but it is rarely life threatening. CASE REPORT: A neonate born to a diabetic mother with a severe hypertrophic cardiomyopathy with life threatening respiratory and haemodynamic distress was successfully supported by extracorporeal membrane oxygenation. CONCLUSION: Extracorporeal membrane oxygenation can be an efficient transitory therapy for very severe hypertrophic cardiomyopathy in infants of diabetic mothers.

[Clinical and respiratory function follow-up of 39 infants treated with neonatal respiratory extracorporeal assistance]. / Suivi clinique et fonctionnel respiratoire de 39 nourrissons traités par assistance respiratoire extracorporelle néonatale.

UNLABELLED: The aim of this prospective study was to evaluate the consequences of neonatal treatment with a venovenous extracorporeal respiratory assistance. POPULATION AND METHODS: Thirty nine neonates (28 boys) with acute respiratory failure were included. Extracorporeal respiratory assistance consisted of an apnoeic oxygenation with low frequency positive pressure ventilation and extracorporeal membrane CO2 removal through a venous single canula perfusion circuit. The causes of respiratory distress were: 15 meconium aspiration syndrome, 12 respiratory distress syndrome, six hyaline membrane disease, three sepsis, two diaphragmatic hernia, and one post-surgery Mendelson syndrome. The mean duration of mechanical ventilation was 18 days, including 5 days of extracorporeal respiratory assistance. The prospective follow-up included physical examination, chest radiographs, scintigraphy and pulmonary function tests. These tests studied the following parameters: functional residual capacity by helium dilution technique, lung resistance and dynamic lung compliance by the esophageal balloon technique and blood gases with arterialized blood samples. RESULTS: The mean duration of the follow-up was 21.3 months (6 months to 5 years). Results showed in the first year 33% of children with chronic obstructive pulmonary disease and chest (X-ray abnormalities, such as bronchopulmonary dysplasia in 23% of children. Data of pulmonary function test at the end of the first year: lung resistance and functional residual capacity were within limits of predicted values for height, and dynamic lung compliance was slightly decreased; according to the analysis of the functional profile: 31% without abnormality, 41% of obstructive syndrome and 26% with restrictive pattern. Blood gases were normal in 37 children. At the end of the second year, we noticed normal functional residual capacity, an increase of lung resistance while lung compliance was normalized; functional profile was quite different: with a decrease of the number of patients without abnormality (22%) and increase of those with obstructive syndrome (56%). CONCLUSION: The percentage of abnormalities is high but these are moderate in most cases, especially if we compare with the initial seriousness of the pulmonary pathology. We suggest a regular follow-up to study the respective incidence of pulmonary disease and/or extracorporeal respiratory assistance over these abnormalities.

[Myocardiopathy and isolated glucocorticoid deficit with ACTH resistance: a fortuitous association?]. / Myocardiopathie et déficit isolé en glucocorticoïdes par résistance à l'ACTH: une association fortuite?

UNLABELLED: Hereditary syndrome of unresponsiveness to ACTH is a rare autosomal recessive disorder characterized by an isolated glucocorticoid deficiency which is exceptionally associated to regressive cardiomyopathy. CASE REPORT: A male newborn had iterative episodes of hypoglycemia since the first hours of life. Acute bronchiolitis at the age of 14 days was associated with transitory dilated cardiomyopathy. Hypoglycemia was due to glucocorticoid deficiency secondary to ACTH insensitivity. Molecular biology showed a composite heterozygotism for the ACTH receptor gene. CONCLUSION: Any congenital glucocorticoid deficiency should lead to search for cardiomyopathy.

Neuropathy in two cobalamin-deficient breast-fed infants of vegetarian mothers.

We describe the electrophysiological findings in 2 infants with deficient cobalamin intake. After normal development, psychomotor regression appeared after the 6th month, leading to severe hypotonia and apathy before the 12th month. Electrodiagnostic evaluation showed sensory neuropathy in both cases, associated with motor neuropathy in 1 case. Thus, in an acquired floppy infant syndrome, electrophysiological signs of peripheral neuropathy contributed to the diagnosis of a curable metabolic disorder.

Simultaneous administration of antithrombin III and protein C concentrates for the treatment of a devastating coagulopathy in a child.

Severe disseminated intravascular coagulation (DIC) is a rare event in childhood. We report here a young body with a devastating DIC unresponsive to heparin, fresh frozen plasma and platelet support. This prompted the use of antithrombin III and protein C concentrates and the effects of this combination were temporarily spectacular. We suggest that the simultaneous administration of two inhibitors of blood coagulation could be of interest and should be evaluated in appropriate trials for the treatment of a devastating coagulopathy.

[Cytomegalovirus, pregnancy and occupational risk]. / Cytomégalovirus, grossesse et risque professionnel.

BACKGROUND: Female health professionals are not more likely to contract cytomegalovirus (CMV) infection than the general population. CASE REPORT: Generalized congenital infection was diagnosed in a neonate. His mother was a nurse working the 2 first trimesters of her pregnancy in close-contact with AIDS patients chronically infected with CMV. CONCLUSION: Preventive measures to avoid CMV transmission among health-care professionals are controversial. The only guideline actually receiving universal agreement consists of standard hospital measures of hygiene.

[Pneumopathies in immunosuppressed children]. / Pneumopathies des enfants immunodéprimés.

Pneumonia in immunodeficient child is a frequent and dramatic event, with a high risk of mortality. Pneumonia requires immediate management. Multiplicity of causes makes diagnosis difficult. Broncho-alveolar lavage must be the first investigation to carry out while waiting for the results of investigations, rapid and efficient treatment is based on probabilistic reasoning which must take the type of immunodeficiency into account. Prophylaxis is still the best way to improve prognosis of pneumonia in immunodeficient children.

[Cytomegalovirus pneumopathies in pediatric intensive care units]. / Pneumopathies à cytomégalovirus en réanimation pédiatrique.

BACKGROUND: Cytomegalovirus (CMV) infection can result in major complications in immunocompromised infants and children. CMV pneumonia may be difficult to diagnose and the true pathogenic role of the virus in the disease is not always clear. This report describes a cohort of 20 children who suffered from CMV pneumonia. POPULATIONS AND METHODS: Twenty children aged 1 month to 11 years 10 months were admitted to our intensive care unit between 1981 and 1990 because of pneumonia with evidence of CMV infection. They were classified into three groups: group I (cases 1-10) with hemopathy or cancer, group II (cases 11-14) with AIDS, and group III (cases 15-20): non immunodeficient or immunosuppressed children. CMV infection was diagnosed after isolation of CMV from bronchoalveolar lavage (BAL) fluid (15 patients), lung biopsy revealing intranuclear inclusions or CMV antigens, or CMV-positive cultures (four patients), CMV-positive urine cultures (one patient). RESULTS: Clinical manifestations and X-rays findings were unspecific; interstitial pneumonia was found only in immunodeficient patients. CMV pneumonia was diagnosed only in two patients on post mortem examination. Concomitant pneumocystis carinii was found on BAL in two patients (group I) and two others (group II). Thirteen patients required ventilation. Eleven patients were given ganciclovir for 2 or 3 weeks; one of them was given a single dose. This treatment was well tolerated. Mortality was 90% in group I, 100% in group II and 33% in group III. CONCLUSION: Ganciclovir did not appear to benefit the immunocompromised patients with CMV pneumonia. Future treatment should include hyperimmune CMV immunoglobulins plus ganciclovir. Careful hand washing is important for all those caring for these patients to prevent contamination as is the use of CMV-negative blood products.

[A retrospective survey on the respiratory course of severe bronchiolitis]. / Enquête rétrospective sur l'évolution respiratoire des bronchiolites graves.

BACKGROUND: Respiratory syncytial virus (RSV) infection in infancy can induce bronchial reactivity and virus-specific IgE production; these responses may favor the later development of asthma. This retrospective study examines the influences of early and severe forms of bronchiolitis on such an outcome. POPULATION AND METHODS: The files of 43 infants who had spent 1 to 24 days (mean 7.8 days) in intensive care between 1986-1990 for severe bronchiolitis with apnea and/or hypercapnia and/or hypoxemia were studied. The parents of all these children agreed to answer a standard questionnaire covering perinatal events, gestational age, number and frequency of recurrent episodes of wheezing, personal and familial history of allergy and environmental conditions. 15 of the children were preterm and 12 of them were given respiratory support during the neonatal period; 5 of these patients developed broncho-pulmonary dysplasia. RSV was isolated in 20 of 39 patients. RESULTS: 26 of the 41 surviving patients developed at least 3 episodes of wheezing over periods ranging from 5 to 54 months (mean 27.6 months): 15 of them were admitted at least once for asthma and 17 were given long-term bronchodilators and/or corticosteroids. Of the 8 patients with histories of allergy, 6 developed asthma, as did 7 of the 9 that had been in close contact with other children, and 9 of the 15 patients who had been exposed to tobacco. CONCLUSIONS: Not only is the development of asthma associated with risk factors (RSV infection, bronchopulmonary dysplasia, history of allergy, close contact with other children and exposure to tobacco) but the severity of the bronchiolitis per se is also correlated with this risk.

Venovenous single lumen cannula extracorporeal lung support in neonates. A five year experience.

The authors have developed a venovenous extracorporeal lung support technique with an original single lumen cannula to avoid the carotid ligation of venoarterial extracorporeal membrane oxygenation (ECMO). During a 5 year period, the authors have used the technique in 107 neonates (weight: 3.045 +/- 0.6 1 kg; gestational age: 38.1 +/- 2.2 weeks). All of the neonates had severe respiratory failure despite maximal conventional treatment and the same indications as those for ECMO. The venovenous technique associates extracorporeal CO2 removal and apneic oxygenation. The system includes a single lumen cannula, an alternating clamp that generates a tidal flow, and an original non-occlusive roller pump that avoids the use of a venous bladder. The PaCO2 was normal (34.6 +/- 3.9 mmHg) with a blood flow of 40-50% of the total cardiac output. Under apneic oxygenation, PaO2 improved rapidly, allowing a decrease in FiO2 and mean airway pressure, minimizing barotrauma. The mean duration of bypass was 117.8 +/- 83.9 hr, and 91 of the 107 (85%) neonates were weaned from AREC. The technical complications were less important than those associated with venoarterial ECMO. The authors conclude that AREC is as effective as venoarterial ECMO and is easier to use.

[Extracorporeal oxygenation and extracorporeal respiratory assistance in newborn infants]. / Oxygénation extracorporelle et assistance respiratoire extracorporelle chez le nouveau-né.

The authors present the principle and discusses the indications and contraindications of two technics of extracorporeal membrane oxygenation in the treatment of neonatal respiratory failure, respectively veno-arterial and veno-venous. The two technics appear to give comparable results. They are indicated in neonates with refractory hypoxemia after failure of other methods such as surfactant, high frequency oscillatory ventilation and nitrous monoxyde.

Detection of Mycoplasma pneumoniae in clinical samples from pediatric patients by polymerase chain reaction.

The polymerase chain reaction (PCR) technique was used to detect Mycoplasma pneumoniae DNA in clinical samples (nasopharyngeal aspirations or bronchoalveolar lavages) obtained from 100 children, 1 month to 16 years old. PCR allowed the detection of M. pneumoniae DNA from 20 out of the 100 patients studied. In 16 cases, PCR positivity was associated with acute respiratory symptomatology. For five PCR-positive patients, a positive culture or a serological response evidenced acute M. pneumoniae infections. A lack of antibody response was observed particularly with immunocompromised children and infants less than 12 months old. The amount of M. pneumoniae DNA in the PCR was estimated in a semiquantitative way by comparison of its hybridization signal with those obtained for 100, 10, and 1 color-changing unit (CCU) of the M. pneumoniae FH strain. Small amounts (less than or equal to 10(2) CCU/ml) of M. pneumoniae were found in samples from asymptomatic patients, while larger amounts (greater than or equal to 10(2) to greater than or equal to 10(4) CCU/ml) were found for 8 out of 10 patients with acute pneumonia.

Miliary tuberculosis with acute respiratory failure and histiocytic hemophagocytosis. Successful treatment with extracorporeal lung support and epipodophyllotoxin VP 16-213.

A 14-year-old girl with high fever, dyspnea and bilateral miliary nodules on chest X-ray, developed a rapidly progressive respiratory failure associated with histiocytic hemophagocytosis. Histologic examination of bone marrow biopsy revealed tuberculous granulomas with caseating necrosis. We report a pediatric case in which treatment with extracorporeal lung support and epipodophyllotoxin VP 16-213 was successful.