[Standard values during adolescence].
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Biomedical subjects
Publications and source records attributed to E Gautier.
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To realize the new concept of biological internal fixation the limited contact dynamic compression plate was developed. It minimizes vascular damage to the plated bone segment. It should lead to a more versatile and efficient application of internal fixation using plates.
A family is described with two siblings who suffered at different times from a single episode of meningococcal meningitis by Neisseria meningitidis groups B and C, respectively. In the two subjects, hemolytically active fifth component of complement (C5) was not detectable and antigenic C5 was less than 0.05% and less than 0.7% of normal, respectively. Repletion of sera by purified human C5 (70 micrograms/ml) restored total complement hemolytic activities. The asymptomatic first degree family members had C5 levels compatible with a heterozygous state of C5 deficiency. C4 allotyping revealed an inherited partial deficiency (Q0) of C4A and C4B in the family with a combined C4AQ0 and C4BQ0 heterozygous condition in one and C4BQ0 heterozygosity in the other C5 deficient (C5D) subject. To our knowledge, this is the first human kindred with recognized combined C5 and C4 deficiency. No other defect of the humoral and cellular immune system was found in this family, including specific immune response to tetravalent meningococcal vaccine. The effect of partial C4 deficiency on classical pathway function was assessed by inhibition of immune precipitation (IIP) of forming bovine serum albumin (BSA)/anti-BSA immune complexes. Sera from all family members showed normal IIP values, with exception of the subject with combined partial deficiency in C4A, C4B, and complete deficiency in C5. Despite undetectable functional C5 in the C5D sera, the titration of the alternative pathway indicated intact but deficient hemolytic activities when rabbit erythrocytes (EC) were used as indicator cells in the presence of Mg2+ and EGTA in an end-point or kinetic assay. Preincubation of the two sera at 0 degrees C for 60 min with rabbit ECs reduced alternative pathway hemolytic activity by 24 and 100%, respectively. When rabbit ECs were replaced by guinea pig ECs no alternative pathway function could be measured. The results indicate that the apparent functional activity of the alternative pathway in C5D sera strongly depends on a factor(s) present in such serum and/or on the detection system used. We conclude that the two C5D individuals of the family reported here may not have sufficient C5 activity to provide efficient protection against Neisserial infections in conditions where complement functions beyond C3 opsonic activity are required in vivo.
The problems related to infections of hip- and knee-prostheses are generally known. The aim of the operation- a mobile and painless joint is jeopardized. The best therapeutic measure has to be evaluated individually by a differentiated diagnostic procedure. The health condition of the patient, type and localization of the infection, bacteriology and resistance of the microbes as well as the biomechanical condition of the artificial hip joint and the trophic quality of bone and soft tissue play an important role. The different therapeutic concepts we can dispose of should take these factors into account, so that a successful healing may be expected in 80 to 90% of the cases.
Nine hundred and thirty-six prenatal chromosomal analyses were performed by four cytogenetic centres after ultrasound diagnosis of fetal abnormalities, amniotic fluid disorders, fetal growth retardation, and fetal or placental abnormalities. During the same period, 6515 fetal karyotypes were analysed because of maternal age. Frequencies of chromosomal aberrations in each case were respectively 4.4, 6.7 and 15.8 per cent, compared with 3.18 per cent when the fetal karyotype was performed because of maternal age. High rates of chromosomal aberrations are observed in cases of cervical hygroma, limb abnormalities, omphaloceles, duodenal stenosis, hydrocephalus, and facial abnormalities. In the case of polymalformations, this rate was 29.2 per cent. When malformations were seen together with an amniotic fluid disorder or growth retardation, 21.5 per cent chromosomal aberrations were observed. This frequency was 10.4 per cent when growth retardation was associated with an amniotic fluid disorder. Trisomy 13, 18, 21 and monosomy X accounted for 4/5 of all abnormalities in which we observed a high rate of triploidies (4.9 per cent) and balanced (3.3 per cent) or unbalanced (9.8 per cent) non-Robertsonian structural abnormalities. Sonographic ascertainment of these aberrations and prenatal characteristics of major anomalies are discussed.
Complex injuries to the hand may cause considerable problems in the aftertreatment. Two possibilities are described, which may facilitate and optimize the postoperative care of the severely injured hand: 1. In case of an inguinal pedicle flap, the temporary stabilization of the hand against the pelvis by means of external fixation is advocated. 2. After multiple extensor tendon repair, the temporary immobilization of the wrist, leaving the fingers free to move, by means of a small fixateur externe is suggested.
The authors describe a case of botulism in a 3-month-old infant infected with Clostridium botulinum type A. Symptomatology developed within four days, persisted for two weeks, then regressed. Symptoms were paresis of face muscles, hyporeactive pupils, loss of succion and deglutition, axial hypotonia, weakness of peripheral muscles, lability of the autonomic nervous system with acute episodes of bradycardia and constipation. Anomalies of the electroen-cephalogram and of the auditory evoked responses suggest that the toxin penetrated the central nervous system. Treatment was symptomatic, without need for assisted ventilation. It was not possible to detect the source of infection.
The authors describe a patient who presented from birth on a severe involvement of connective tissues with pathological fractures, lack of auricular cartilage, hyperlaxity of fingers and cutis laxa with deep folds, all suggestive of derangements of collagen and elastin. Hypothermia at 24 hours of age should have already indicated the possibility of Menkes' syndrome. From the 3rd month on, the patient presents a neurological deterioration and a myoclonic epilepsy which is resistant to treatment. Craniocerebral tomodensitometry revealed, with time, a cerebral atrophy and subdural hematomas. Angiodysplasia of a coronary artery was seen at cardiac echocardiography. Undetectable levels of serum copper and ceruloplasmin, and an increased uptake of copper by fibroblasts in vitro confirmed the diagnosis of Menkes' syndrome. Electron microscopy of a skin biopsy disclosed a desmosomal anomaly in the epidermis. Desmosomes stay apart suggesting an alteration of the interdesmosomal cement.
Stabilization of the fracture using implants requires contact surfaces between implant and bone. Such contact has been observed to induce bone porosis first seen at one month after surgery. Bone loss in the vicinity of implants has hitherto been explained as being induced by mechanical unloading of the bone (stress protection). Experiments in sheep, dogs, and rabbits combining intravital staining of blood circulation and polychrome fluorescent labeling of bone remodeling leads to the conclusion that early bone porosis in the vicinity of the implants is the result of internal remodeling of cortical bone and is induced by necrosis rather than by unloading. This theory is favored by the evidence that (1) the bone porosis is of a temporary nature, an intermediate stage in internal bone remodeling; (2) the pattern of the remodeling zone is closely related to that of the disturbed circulation, and not to that of unloading; (3) plastic plates may produce more porosis than steel plates; and (4) improved blood circulation using modified plates resulted in reduced porosis. The clinical relevance of these findings is related first to the temporary weakening of the bone, and second to the possibility of sequestration. Sequestration may be the result of intensified remodeling activity in the presence of inflammation or infection.
Two micro enzyme immunoassays (microEIA) for circulating immune complexes (CICs) are described. The Raji cell microEIA was similar in sensitivity, reproducibility and specificity to the Raji radioimmunoassay. The F(ab')2 anti-C3 microEIA was comparable to the 2 Raji cell assays. The 2 microEIAs for CICs were used to analyze sera from patients with systemic lupus erythematosus (SLE), the acquired immune deficiency syndrome (AIDS) and from hemophiliacs with AIDS-like symptoms. The microEIAs were very sensitive in detecting CICs in pathologic sera. In contrast, only 3% of normals (n = 30) were positive in the Raji microEIA while none were positive in the F(ab')2 anti-C3 microEIA. The initial high positivity of some normals (9/30) in the F(ab')2 microEIA was due to bovine serum albumin (BSA)-anti-BSA immune complex formation in vitro and was corrected when human albumin was used in buffer preparation instead of BSA. The reagents required for the microEIAs are more stable and less expensive than those required for the RIAs and the need for facilities to deal with 125-labeling and disposal is avoided.
The coexistence in two sisters, born to related parents, of a corticoresistant nephrotic syndrome, lymphopenia, an immune deficit, short stature, and photophobia is described. The immune deficit is mainly cellular; studies of lymphocyte markers demonstrate a pronounced deficiency of T lymphocytes and Fc-mu receptor-bearing cells. It is suggested that a thorough examination of number and function of T cells should be performed in patients with a familial corticoresistant nephrotic syndrome and recurrent infectious episodes before considering immunosuppressive treatment.
Myelin basic protein (BP) is a specific constituent of the myelin sheath. This structural protein cannot be detected in the cerebrospinal fluid (CSF) unless myelin is acutely degraded. In order to detect active demyelinating diseases, BP was measured in CSF samples of radioimmunoassay. The assay is specific and sensitive to as little as 1.5 to 2.5 ng/ml BP. A moderate non-parallelism between the standard curve and various dilutions of CSF samples indicates that in CSF BP is present in an altered state. Over 1000 CSF samples have been measured in a double-blind study, in which 100 patients were selected and their clinical records evaluated. Twenty-eight patients without demyelinating disease had BP levels lower than 2.5 ng/ml. 72 patients had values higher than 2.5 ng/ml. Among them, the most frequent causes of demyelination were multiple sclerosis (19 cases), brain tumors (22 cases) and cerebral or spinal vascular accidents (12 cases). During a single acute demyelinating episode, BP levels revert to background levels within a few days. In contrast to immunological anomalies observed in the CSF, the presence of BP is concomitant with the breakdown of myelin. The size and location of the lesion influence the level of BP in the CSF. Thus, the assay is useful for the detection of active demyelination in the central nervous system and in following the course of the disease, although normal values do not rule out the presence of demyelinating lesions. For the time being, therefore, this assay should be restricted to specialized neurological centers and selected patients.
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In a prospective, nonrandomized trial clinical (initial WBC and chest film) and immunological (surface immunoglobulin and rosetting with pretreated sheep red blood cells) criteria were used to stratify 69 children with previously untreated acute lymphoid leukemia (ALL). Forty of 61 evaluable patients had low-risk ALL (initial WBC less than or equal to 20,000/mm3, no mediastinal mass) and were treated less intensively. Twenty-one of 61 patients had high-risk ALL (initial WBC greater than 20,000/mm3 and/or mediastinal mass) and were treated more intensively. Of the high-risk patients 15 had non-T non-B and 6 T ALL. Sixty of 61 patients went into complete remission. After a median observation period of 27 months, 32 of 40 low-risk, 7 of 14 high-risk non-T non-B, and none of 6 high-risk T ALL patients were in continuous first remission. Thirty-six of 40 low-risk, 9 of 15 high-risk non-T non-B, and none of 6 T ALL patients were alive. Despite more intensive treatment, the duration of remission and the survival were significantly shorter in the high-risk than in the low-risk patients. Among the high-risk ALL, non-T non-B ALL did better than T ALL.
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The clinical value of plasma trypsin determination by radioimmunoassay has been investigated in children. In newborns (1-7 days), plasma trypsin levels are significantly higher than in older children, whose values do not differ from the adults' age. Variation between 15 days and 18 years is negligible. In ten cases of mumps without pancreatic symptomatology, results are within the normal range, even when amylase levels are high. In cystic fibrosis plasma trypsin has been found undetectable or very low in 13, normal in 2 and high in 1 of 15 cases. Plasma trypsin levels have also been found undetectable in 18 of 29 diabetic children, some of whom had been diagnosed for less than a year. We conclude that plasma trypsin determination by radioimmunoassay is of interest in pancreatic function testing of children.
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