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Biomedical subjects

J Costil

Publications and source records attributed to J Costil.

At least 19 recordsLinked to original sources

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.

Action Potentials↗

[Clinical and respiratory function follow-up of 39 infants treated with neonatal respiratory extracorporeal assistance].

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.

Acute Disease↗

[Myocardiopathy and isolated glucocorticoid deficit with ACTH resistance: a fortuitous association?].

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.

Adrenal Insufficiency↗

[Cytomegalovirus, pregnancy and occupational risk].

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.

AIDS-Related Opportunistic Infections↗

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.

Antithrombin III↗

[Pneumopathies in immunosuppressed children].

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.

Child↗

[Cytomegalovirus pneumopathies in pediatric intensive care units].

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.

Child↗

[Extracorporeal oxygenation and extracorporeal respiratory assistance in newborn infants].

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.

Extracorporeal Membrane Oxygenation↗

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.

Adolescent↗

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.

Adolescent↗

Interleukin-2 and lymphokine-activated killer cells in 15 children with advanced metastatic neuroblastoma.

A phase II trial using interleukin-2 (IL2) and lymphokine-activated killer (LAK) cells was carried out in an attempt to treat children with end-stage neuroblastoma. Fifteen patients (median age, 7 years) were enrolled in the study. Twelve were in relapse after massive chemotherapy and autologous bone marrow transplantation (ABMT), and three had a primary refractory disease after conventional chemotherapy. IL2 was administered as an 18 x 10(6) IU/m2/d continuous infusion. One course consisted of a double 5-day treatment period separated by a 6-day break. Cytapheresis to harvest LAK progenitor cells was performed during the rest period. After a 4-day in vitro culture, LAK cells were reinjected during the second cycle of therapy. A phenotypic and functional analysis of immunologic parameters was conducted along with the therapeutic protocol. Toxicity was significant with two toxic deaths (cardiotoxicity and respiratory distress). The reinfusion of large amounts of LAK cells was clearly involved in one case, but this particularly severe toxicity has to be related to the patient's status (ie, heavy pretreatment). No significant clinical response was seen. The immunologic monitoring showed phenotypic and functional modifications in these patients before initiation of treatment and an unexpected absence of evolution of these parameters during IL2 therapy. Although the origin of these immune dysfunctions is not clear, they could be involved in the failure of IL2 therapy. Future studies of IL2 therapy in neuroblastoma should be undertaken earlier in the course of the disease.

Antibodies, Monoclonal↗

Preliminary report: extracorporeal lung support for neonatal acute respiratory failure.

A technique for ventilatory support of life-threatening neonatal acute respiratory failure by use of apnoeic oxygenation and low-frequency positive-pressure ventilation, with extracorporeal membrane CO2 removal through a single-cannula perfusion circuit, is described. 20 severely ill babies with respiratory failure were treated with this technique, 17 of whom survived with no clinical evidence of pulmonary handicap or neurological deficit at discharge from hospital. All 10 patients followed up at 6 months showed normal growth and development.

Acute Disease↗

[Cystic fibrosis in infants revealed by severe respiratory distress. Mid-term course].

We report the follow-up of 13 infants aged 2.5 to 24 months in whom cystic fibrosis disease presented as respiratory distress. All infants received an intensive treatment. Mean clinical, radiological and microbiological follow-up was 3.1 years. Ten children aged 1.5 to 8.5 years are still alive. One of them presents with serious respiratory failure, with a Shwachman score of 50. The Shwachman score is between 85 and 70 for 8 of 13 and 65 for one. The prognosis of this group did not differ from the one usually observed in the disease. It depends partially on early intensive care management and is not altered by mechanical ventilation.

Child, Preschool↗

[Etiologic diagnosis of severe acute pneumopathies in infants and children].

In infants and children suffering from severe acute lung disease the problem of aetiological diagnosis arises in two sets of circumstances. In cases of diffuse primary pneumonitis, which occurs mainly in immunocompromised subjects, broncho-alveolar lavage is the ideal technique to discover opportunistic infections; besides, the technique is little invasive and well tolerated. It is only when its results are negative that lung biopsy still has a few indications. In hospital-acquired lung diseases occurring in children under mechanical ventilation the results of broncho-alveolar lavage are more difficult to evaluate owing to possible contamination by tracheal organisms. The various ways of solving this problem (bacterial counts, sampling by means of a protected brush operated by remote control) are discussed.

Acute Disease↗