CNS involvement in lymphomatoid granulomatosis.
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Biomedical subjects
Publications and source records attributed to M Dumont.
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The authors report a case where hypertonicity resulted in extension of the cerebral spine in a fetus. They think that they are reporting a case of congenital hyperextension of the lumbar spine, which is otherwise called "the newborn stiff-man syndrome". The outlook for this child is good in spite of the gross abnormality that is revealed on the X-rays of the fetus. Caesarean section is therefore indicated.
The authors have tried to look at the graphs of ultrasound growth in their population in order to establish a base pattern. The infants were divided into groups according to their frame and a study of each diameter was made for each group. The authors show that all babies that start off from the same point arrive at their final stature which is different from all others because of their own rates of growth. The curve which is suggested fits in with this spread. The way it is built up has been explained as well as the follow-up studies that show that it is valid. The discussion points out that the rates of growth can be measured; and the value of the diameter of the thorax.
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The purpose of this study was to find out whether the administration of corticosteroids to mothers with threatening premature labour reduced the risk of respiratory distress syndrome (RDS) in premature babies. Between XOU4 and 1979, the mothers of 62 children received 4 doses of a long-acting betamethasone preparation intramuscularly at 12-hour intervals followed, if required by 2 booster doses 15 days later. Salbutamol injections were sometimes given to delay labour. During the same period, the mothers of 81 other children (control group) with unavoidable premature delivery were left untreated. The incidence of RDS was similar in both groups. A study of the various factors likely to modify the incidence of RDS showed the same lack of effectiveness of the treatment. Neonatal mortality and morbidity did not appear to be altered.
It has been reported that biliary secretion is the limiting step in the hepatic transport of bile acids by the hepatocyte from plasma to canalicular bile. The aim of the present study was to examine the role of conjugation in the transport process using ursodeoxycholate (UDCA) and tauroursodeoxycholate (TUDCA), two bile acid with low liver toxicity. Rats were given constant intravenous infusions of cholate (C), taurocholate (TC), UDCA, or TUDCA at progressively increasing rates. The biliary maximum secretory rate (SRm), in nmol . min-1 . 100 g body wt-1, for TC (1,835.2 +/- 135.5, mean +/- SE) was not significantly different from that of C (1,749.4 +/- 85.6). In contrast, the SRm for TUDCA (5,909.4 +/- 304.4) was approximately sevenfold that of UDCA (802.1 +/- 134.2), the difference being statistically significant (P less than 0.001). The SRm of UDCA in the presence of a taurine infusion (1,367 +/- 84.4) was higher than that of UDCA infused alone but still much lower than that of TUDCA. Phenobarbital sodium pretreatment did not increase SRm of UDCA alone or in the presence of a taurine infusion. These results suggest that in the rat 1) conjugation is the rate-limiting step in the overall transport of UDCA (and perhaps other bile acids) by the liver, and 2) the conjugation process itself is limiting, rather than the availability of taurine. They support the view that, although not mandatory for secretion into bile, conjugation of bile acids confers a biological advantage, possibly by increasing the solubility of the bile acid.
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Dihydroxydibutyl ether (DDE) induces a choleresis and a diuresis in the rat. The stimulation of bile flow is immediate and dose-dependent; DDE appears to stimulate bile acid-independent flow of canalicular origin, because erythritol clearance increased in parallel to bile flow and choleresis occurs in the absence of an increased bile acid secretion. Increased bile flow may be accounted for by the osmotic activity of DDE and DDE metabolites excreted into bile, with an average increase in bile flow of 16 microliters/mumol of DDE (or DDE metabolites). Biliary secretion of DDE is limited with a maximal rate (biliary Tm) of 773 nmol.min-1.100 g b.wt.-1. The metabolic products of DDE in bile appear to be its mono- and diglucuronide conjugates inasmuch as DDE can be recovered after beta-glucuronidase incubation. DDE and a DDE glucuronide conjugate are also detected in the urine: there is an apparently linear relation between DDE (and DDE metabolites) excretion rate and increase in urinary flow (3.5 microliters/mumol of DDE or DDE metabolites). It is concluded that DDE stimulates choleresis and diuresis in the rat because the molecule and its glucuronide conjugates are secreted and concentrated in bile and urine.
Measurements are reported of NMR relaxation times (T2) on hydrogen in water of isotonic NaCl solutions and of normal human amniotic fluid in which known concentrations of meconium (fetal feces) were dissolved. Magnetic fields for resonance of 23.5 X 10(3) and 0.6 oersteds were used. From the measured T2 values, reliable estimates of meconium concentrations could be obtained that might be considered estimates of fetal distress. Observed minor variation of T2 values was probably due to the mucopolysaccharides comprising 80% of the meconium weight. Advantages of this technique for possible clinical use in obstetrics include small volume of sample required (0.1 ml), lack of necessity for sample filtration or centrifugation, and shortness of time required for analysis (less than 30 min).
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The authors present 6 observations of in utero detected abdominal wall defect : 2 laparoschisis and 4 omphaloceles. In three cases the diagnosis have been done prior week 20, by systematic AFP assay for amniocentesis performed for cytogenetic or metabolic reasons; the pregnancy was terminated. In three other cases, the pregnancy was complicated by hydramnios after week 30; amniocentesis for AFP and echography were performed to detect fetal malformations after associated with hydramnios. The detected abdominal wall anomaly was not alone: two fetus had an abnormal caryotype (trisomy 18), three other presented a polymalformative syndrome, a Beckwith-Wiedemann syndrome was discussed for the last child. The in utero diagnosis of omphalocele or laparoschisis implicates difficulties for genetic counselling, particularly if the diagnosis is done prior week 20. These anomalies can be treated with surgical management, but frequency of associated malformations must be underlined. it is important for genetic counselling to know the family history, the amniotic fluid cells caryotype, and an ultrasound scanning performed to reveal any other malformations.
In 68 pregnant women, blood samples were obtained at 12, 20, 25, 29, 32 and 36 weeks. Plasma renin activity (PRA) was measured in 10 of them, who developed "pregnancy induced hypertension" ("PIH") and in 10 normal pregnant women, matched with the hypertensive patients for age, parity and body weight. Towards the end of pregnancy, PRA showed a tendency to increase in the "PIH" group, and to decrease in the control group. However, these variations were not statistically significant. There was no significant difference between the two groups, except for the last measurement, obtained at the 36th week of gestation. At this time, PRA was more elevated in the PIH group. We conclude that PRA measurements early in pregnancy are of no value in predicting if women are going to develop PIH.
The authors report two new cases of primary amenorrhoea due to the presence of a dicentric Y with a chromosome formula of 45,X/46,X,dic(Y). These two patients show how variable the clinical manifestations of these gonosome anomalies can be, since one had a Turner's syndrome and the other is a woman who looks completely normal but only has primary amenorrhoea. A review of the literature has brought to light only 45 cases similar to ours and shows the different clinical aspects of these patients who are 45,X/46,Xdic(Y). Finding that one of our patients had a gonadoblastoma has confirmed the need to carry out bilateral castration when there is a dysgenetic gonad with a Y chromosome, in order to treat or prevent a dysgenetic tumour from arising.