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Biomedical subjects

P Fleury

Publications and source records attributed to P Fleury.

At least 37 records · Page 2Linked to original sources
PubMed1989

Neonatal behavior after drug dependent pregnancy.

Neurobehavioural development of 35 infants of drug dependent mothers was compared with the development of 37 reference infants as part of a prospective longitudinal research project. Infants of drug dependent mothers had more poor responses than the other children on neurological examination. This difference is significant only when data of infants of drug dependent mothers born at full term are analysed. Two tailed testing indicated that significantly more infants of drug dependent mothers than reference children had electro-encephalograms rated as suspect or abnormal. By the end of the first month the infants of drug dependent mothers tended to be more active, and they had worse scores than the reference children on the neonatal behavioural assessment scale. Analysing data only of infants born at full term, the groups differ significantly on the interactive items. The results of this study show that even after treatment for the neonatal abstinence syndrome, infants of drug dependent mothers seem to differ from comparison children, which could indicate later developmental problems.

Child↗
PubMed1989

Behaviour in first year after drug dependent pregnancy.

Neurobehavioural development of a group of 35 infants of drug dependent mothers and of a reference group of 37 infants was compared. Two tailed testing showed that at the age of 3 months the infants of drug dependent mothers seemed to be more active than the reference group; at 6 months, however, no difference was found. On the infant behaviour temperament questionnaire, infants of drug dependent mothers tended to have slightly better scores for 'duration of orienting' at the age of 9 months; five other dimensions of behaviour did not differ between the groups. The Bayley scales of infant development and neurological examination according to the method of Touwen did not show any significant differences among the groups at the ages of 6 or 12 months. At 12 months infants of drug dependent mothers had slightly but not significantly worse results on electroencephalography. Studied with multidisciplinary measurements, the development of infants of drug dependent mothers does not seem to diverge particularly from the development of the reference group.

Child Behavior↗
PubMed1989

Evidence for genetic heterogeneity in tuberous sclerosis.

The question of genetic heterogeneity in tuberous sclerosis (TSC) was addressed by genetic linkage studies in eight affected families using nine polymorphic markers (EFD126.3, MCT136, ABO, ABL, AK1, and MCOA12 from distal 9q, and PBGD, MCT128.1, and 1CJ52.208M from distal 11q). The data as a whole supported a TSC locus on distal 9q, the peak lod score on multipoint analysis being 3.77 at 6 cM proximal to the Abelson oncogene locus (ABL). However, analysis of two point lod scores using the HOMOG programs showed significant evidence for genetic heterogeneity (p = 0.01), linkage to ABL being unlikely in one family. After exclusion of the unlinked family, multipoint analysis gave a peak lod score of 6.1 in the vicinity of ABL. The family unlinked to ABL showed no recombinants with two chromosome 11 probes, but was too small to provide significant evidence for linkage. Genetic heterogeneity in TSC will complicate efforts to clone the causative genes and severely limit the use of linked probes for carrier detection and prenatal diagnosis.

Chromosome Mapping↗
PubMed1989

[Neurofibromatosis in pediatrics].

The diagnostic criteria and complications of neurofibromatosis (Von Recklinghausen's neurofibromatosis or peripheral neurofibromatosis) are summarized. Patients and their relatives were studied in pediatric departments of the University Hospitals at Amsterdam (n = 23) and Rotterdam (n = 39) using a multidisciplinary approach and a standardized protocol. This comprehensive evaluation enables improved detection of serious complications and their management, and information to the parents about this highly variable disorder. Also, more insight is obtained into the natural history of the disease. Genetic counseling and family studies will become more precise in the near future, now the gene for NF I has been localised on chromosome 17, and for NF II (central NF) on chromosome 22, enabling DNA-marker studies.

Adolescent↗
PubMed1989

[Tuberous sclerosis, changes in the diagnostic and therapeutic approach].

In the last 15 years the diagnostic possibilities in tuberous sclerosis have considerably improved. Some new signs of this disorder have recently been described. The meaning of other signs became more evident. Investigation of large groups of patients made it possible to indicate which clinical features of tuberous sclerosis may be classified according to their diagnostic importance as pathognomonic or definitively diagnostic or as presumptive or provisionally diagnostic. It is sometimes difficult to determine the significance of a single nonspecific lesion. It is very important to know the meaning of the signs for those parents or more distant relatives who ask for genetic counseling. As long as the gene defect in tuberous sclerosis had not been determined it will remain possible that formes frustes will not be detected despite the most elaborate examination. Connected with the improvement of the diagnosis by imaging technics of especially the cerebral and renal features, the therapeutic possibilities have been changed. On one side this concerns neurosurgical removing of giant cell astrocytoma situated near the foramen of Monro and of cortical or subcortical tubers that cause therapy resistant partial epilepsy. On the other side it concerns embolisation of symptomatic renal angiomyolipomas or renal transplantation in the case of renal insufficiency due to cysts with or without angiomyolipomas.

Combined Modality Therapy↗
PubMed1989

[Localization of the gene defect in tuberous sclerosis].

Elucidation of the genetic defect in tuberous sclerosis (TS) awaits a precise chromosomal localization. At present two chromosomal regions, 9q34 and 11q23, are candidates for the site of a TS locus. Family studies using polymorphic DNA markers are carried out in other laboratories and in ours and are expected to disclose the existence of one TS gene that is localized on either chromosome 9 or 11, or the involvement of two genes, one on #9 and one on #11. Early postnatal and potentially prenatal diagnosis by means of DNA analysis may be offered to a family with TS after identification of the gene defect, but also after the identification of very closely linked DNA markers.

Chromosomes, Human, Pair 11↗
PubMed1987

Gliomatosis cerebri, report of a clinically diagnosed and histologically confirmed case.

Pathological criteria for diffuse gliomatosis are clearly demarcated but its clinical diagnosis has always been hampered by the imaging techniques used. Although with the advent of CT scanning clinical possibilities were improved, isodense brain lesions are difficult to detect and in these cases the diagnosis gliomatosis cerebri can only be suspected from the clinical signs and the absence of radiological signs. We have studied a young patient with a peculiar clinical history, in whom comparison of the CT-scan and NMR scan yielded remarkable results. Our clinical diagnosis of (diffuse) gliomatosis was confirmed by biopsy.

Adolescent↗
PubMed1987

The incidence of hepatic hamartomas in tuberous sclerosis. Evaluation by ultrasonography.

Our series of tuberous sclerosis patients consisted of 23 children between 6 and 16 years of age and of 13 patients between 16 and 48 years of age. In the former group the incidence of multiple hepatic haemangiomas, estimated by grey-scale ultrasonography, is 13%, whereas this incidence is 23% in the group of older patients. The sign might be important for genetic counselling in formes frustes.

Adolescent↗
PubMed1987

Cerebral thromboembolism due to antithrombin III deficiency in two children.

Despite numerous well-described causes of stroke in infancy and childhood, a significant proportion remains unexplained. Venous thromboembolism is a common complication in adult patients undergoing surgery, and after severe trauma, but not in otherwise healthy children less than 10 years old. However, it may also occur spontaneously without recognizable cause. It has been known for a long time that some patients are particularly prone to venous thrombosis and in recent years great efforts have been made to identify the risk factors. The attention of haematologists has been focused on the possibility that certain abnormalities of coagulation may be associated with a tendency to thrombosis, but only in a few instances a clear causal relationship has been established. One such example is a deficiency of antithrombine III, but such a deficiency has hitherto been recognized as a cause for thrombosis in children only in very particular circumstances. We present two young children with stroke of which one was purely ischemic and the other ischemic with secondary hemorrhage. Both our patients showed an AT III deficiency. Patient one also had a cyanotic congenital heart disease with right to left shunting which made cerebral embolism originating from a thrombus in the iliac vein possible to occur. We consider her hematocrit values too low to be a predisposing factor for this thrombosis. AT III deficiency may be caused by several different mechanisms. Either it exists as a congenital (hereditary) or as an acquired disorder. In patient two the family history was positive for hereditary AT III deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Antithrombin III Deficiency↗
PubMed1986

Ito's hypomelanosis (incontinentia pigmenti achromians). A review of four cases.

In 1952 Ito described the occurrence of a bilateral systematized depigmented nevus in a 22-year-old Japanese woman. He used the term incontinentia pigmenti achromians. The condition has been described under various designations, such as for instance Ito's hypomelanosis. Till now 71 patients with this syndrome are described. We will report 4 cases, 2 boys and 2 girls, 2 Caucasian, 1 Indonesian and 1 Caribean child. The cutaneous signs in these 4 patients fit in with the syndrome of Ito's hypomelanosis. Of these 4 children 3 are mentally retarded, 2 have epilepsy. Congenital malformations are seen in 3 children. Electronmicroscopy of skin biopsies of the hypomelanotic nevus and of the normal skin were performed. In the biopsy of the normal skin of one patient interruption of the basement membrane is seen. Anomalies of the central nervous system as seen in our patients occur in about 40% of the cases. Abnormalities of skin derivatives next to other ectodermal anomalies are described. Affection of other germ layers also occur to a varying degree. In our 4 patients some of these abnormalities exist also. These 4 cases are presented to underline the fact that this syndrome seems not to be as extremely rare as is proposed.

Abnormalities, Multiple↗
PubMed1986

[Our postoperative care and surveillance in surgery of stable sequelae of chronic otitis].

After a brief summary of the technique used for myringoplasty (enlarged endaural approach, large flattened aponeurotic graft applied to deep surface of tympano-meatal flap after a "request" for boring) emphasis is given to the need for strict surveillance and postoperative care by the surgeon. Immediate postoperative care starts in the operating room and involves a deep intra-meatal dressing (1 or 2 Popes oto-wicks placed on a thin film of silastic or simply surrounded by Gelfoam) and a superficial dressing. The dressing is renewed on the 3rd day with the patient in bed and involves only the superficial one. Secondary postoperative care extends to the 10th or 15th day and includes exeresis of the deep pope-Gelfoam assemblage under careful binocular microscope control followed by audiometric examination. Hospital stay is a mean of 5 days and medical treatment (antibiotics, vasodilators, corticoids) is given as required. Late postoperative care can be summarized as involving regular surveillance and the application of certain rules that cause little disturbance in the patients life. Various rare and even exceptional incidents, benign in the majority of cases, are reviewed.

Chronic Disease↗
PubMed1985

An infant with Marfanoid phenotype and congenital contractures associated with ocular and cardiovascular anomalies, cerebral white matter hypoplasia and spinal axonopathy.

An infant is presented with a Marfanoid phenotype and congenital contractures. In addition to this she showed severe neurological and ocular abnormalities. Cardiac insufficiency due to mitral and tricuspidal valve prolapse caused her death at the age of 6 months. Postmortem examination showed axonal pathology of the anterior horns and roots of the spinal cord, and white matter hypoplasia of the brain.

Abnormalities, Multiple↗
PubMed1985

[Unusual zones of osteitis detected during salvage tympanoplasty].

Three patients were noted to have unusual zones of osteitis during salvage myringoplasty. The first case involved the whole upper wall of the bony canal and was camouflaged by a weeping mucopolypoid carpet. In the second case there was a wide zone of osteitis of the posterosuperior angle of the canal and another smaller zone in the anteroinferior tympanic region. The osteitis in the third case had invaded the total mastoid cortex producing a vast plaque of superficial osteitis which could be removed without difficulty. Diffuse osteomatous reaction of the wall of the canal had resulted. In all three cases a salvage myringoplasty was possible after elimination of the osteitic lesions, although a glue ear persisted with a punctiform perforation in one patient, suggesting tubal dysfunction.

Adult↗