Efficiency of prostaglandin E1 in a tiny baby with coarctation of the aorta and ligated ductus arteriosus.
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Biomedical subjects
Publications and source records attributed to F Didier.
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Gelatinase was purified from DFP treated human neutrophils which have been stimulated by the chemotactic peptide, N-formyl-methionyl-leucylphenylalanine. The secreted gelatinase was purified in two major steps: a gelatinase enriched fraction was recovered after a specific immunoadsorption of contaminant proteins from cytosol and immunoadsorption of proteins from specific or azurophilic granules; then gelatinase was isolated by affinity chromatography and FPLC gel filtration. Some kinetic properties of the Mr 94,000 purified enzyme were investigated.
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In a patient with clinical manifestations suggestive of brain malformation, CT showed lissencephaly with absent opercularization. The child had seizures but not a typical EEG of hypsarhythmia. MRI confirmed the diagnosis and showed heterotopic grey matter and abnormal basal ganglia. High grey-white matter contrast and the possibility of imaging the brain in sagittal, coronal and transverse planes make MRI the methode of choice for the evaluation of lissencephaly and other brain malformations.
Based on our experience with 13 in utero diagnoses we report the changes that may occur in the ultrasonic appearance of a multicystic dysplastic kidney. Macrocysts appear obvious only in the early third trimester of pregnancy. After reaching a maximum size the cysts start to involute either in utero or after birth, which may lead to a small noncystic mass, the so-called aplastic kidney, or even to complete disappearance of the entire dysplastic kidney. The dysplastic kidney seems vulnerable to anoxia or infection, and necrosis may supervene. The multicystic dysplastic kidney is a progressive and changing disorder. If its radiological appearance is typical management may be conservative with ultrasonic monitoring. Nephrectomy should be done if there is any abnormal clinical or ultrasonic change.
Two cases of fetal perirenal uriniferous pseudocyst were diagnosed in utero with ultrasound studies. The perirenal collections resolved spontaneously in utero. Follow-up examination showed growth impairment of the affected kidney. At birth, no renal function was demonstrated in one case and only slight function in the other case. Pathologic examination showed a highly dysplastic kidney with extravasated urine in the case in which surgery was performed. Some hypodysplastic kidneys apparently result from acute in utero obstruction with secondary pyelocalyceal rupture. Renal dysplasia could render the kidney more vulnerable to such phenomena.
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Pulmonary sequestrations are congenital abnormalities where nonfunctioning lung tissue receives its vascular supply from the systemic circulation (thoracic or abdominal aorta). It is necessary to establish the diagnosis in childhood when the lesions are uncomplicated. The authors present three cases of sequestration of the apex (2 extralobar and 1 atypical) with the main clinical and radiological features. Sequestrations in the upper lobe are rare, and the usual site is the left lower lobe. Plain x-rays show a dense opacity, sometimes air-filled and sometimes with an air-fluid level: angiography is currently the best mean for definitive diagnosis; however, computed tomography will probably be very useful in the future. Differential diagnosis includes tumours of the superior mediastinum (neurogenic tumours, digestive duplication, bronchogenic cysts, pheochromocytoma and hydatid cysts).
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A method for bone age calculation for neonates is reported. Criteria for maturation and growth are separately analysed. The chosen X ray charts were thorax including mandibula and profile of the right leg. 126 neonates 28 to 41 weeks of gestational age were studied. A linear regression is given. An area of confidence for growth and maturation for each gestational age has been designed. When gestational age is known, it gives the opportunity to appreciate the impact of a given pathology on these 2 data.
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A total of 1,197 diarrheic children less than 15 years old were investigated for parasitic, bacterial, and viral enteropathogens from March 1981 through February 1982 in the Central African Republic. One or more pathogens were identified from 49.4% of the patients. Rotavirus was the most frequently identified pathogen among children less than 18 months old. Enteropathogenic Escherichia coli was the second most frequently isolated pathogen (12.1%) in children less than 2 years of age. Campylobacter jejuni was also isolated frequently from diarrheic children less than 5 years of age (10.9%). Entamoeba histolytica was identified in very young children and was found to be the most frequent enteropathogen associated with diarrhea in children over the age of 2 years. Enterotoxigenic Escherichia coli was rarely isolated (ca. 2%). There was a peak in the incidence of rotavirus during the dry season and in the incidence of Campylobacter jejuni during the rainy season.
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Four boys of the same family (three first cousins and their uncle) had the same syndrome of multiple malformations. They had hypertelorism, antimongolian slant of the palpebral fissures, low-set ears, and Pierre Robin syndrome. The ossification of the skull vault was defective, with broad sutures and fontanelles. The ribs were sinuous, the clavicles were long, thin, and sloping, and the vertebrae were flattened. There were abnormal bowing with hyperostosis of the long bones, faulty ossification of the bones of hand and feet, and "fanned-out" toes. The disorder was lethal within a few weeks. The karyotype was normal. The mothers of affected boys had a mild form of the same abnormal facies. Transmission of this disorder appears to be linked to the X chromosome.
The hypoplastic left heart syndrome is a malformative complex which unfortunately can not be treated surgically. This cardiopathy is the result of a serious embryogenic error and takes into consideration the different anatomic varieties described here. Borderline cases are also noted especially isthmic coarctation of the aorta or aortic stenosis in the neonatal period. Though several years ago, the diagnosis of the hypoplastic left heart syndrome was made only on pathology, it is now possible to make this diagnosis using clinical, laboratory and radiologic examinations. However, they are cases that remain difficult to label precisely, especially in minor forms and we feel that these deserve hemodynamic investigations.
The neonatal pulmonary hypertension occuring with fetal distress can be evaluated by the echographic study of the sigmoide pulmonary valves. This non-invasive method can appreciate the severity of the hemodynamics disturbances and their evolution. In this preliminary study the authors report their experience through 14 cases.
Giant emphysema of a lung lobe has distinctive features. Classically there is overdistension of the affected lung lobe, with one lobe only being involved, and, 50% of cases occur in the newborn infant [1, 4, 8]. The authors describe a particularly severe example with marked mediastinal shift and initially the hemithorax on the side of the lesion was opaque. Angiography was carried out and followed by resection when the infant was 4 months old.
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