Pathogeny and natural history of congenital dislocation of the hip.

Based on a review of the literature, the authors have made a critical study of several etiological factors. Endogenous factors such as acetabular dysplasia, increased anteversion of the femoral neck, and capsular laxity support the genetic theory but are neither constant nor necessary and are only facilitating factors. The major factor seems to be a mechanical one linked to the position in the uterus: hyperflexion with adduction and external rotation constituting the dislocating foetal posture combined with abnormal pressure on the greater trochanter and leading to expulsion of the head upward and backward. This theory can explain the natural history of C D H which is first, at birth a hip instability followed by two possible evolutions: either persistent luxation becoming irreducible or spontaneous stabilisation leading sometimes to complete healing or to residual abnormalities (subluxation or dysplasia). This concept suggests practical conclusions: the importance of an early diagnosis, the selection of the signs of the hip at risk, the pattern of prevention, the role for non-clinical investigations, the principles of the treatment based on postures, the indications for the different types of treatment.

Evolution of genetic variability in a population of the edible snail, Helix aspersa Müller, undergoing domestication and short-term selection.

The evolution of genetic variability is studied in six successive generations of a population originating from wild Helix aspersa. During the first three generations (G1 to G3), no artificial selection was applied. During the next three generations (G4 to G6), two lines were reared: a control line (C) and a line (S) selected for increased adult weight. Genetic variability is described by genealogical parameters (inbreeding, number of founders, effective number of founders and ancestors, effective number of remaining genomes) and by the additive genetic variance in adult weight. A large decrease in all parameters was observed between G1 and G2, suggesting strong natural selection: additive genetic variance in adult weight (transformed data) decreased from 0.0119 +/- 3.8 x 10(-3) to 0.0070 +/- 1.7 x 10(-3) (P < 0.05) and effective number of ancestors from 97.4 to 67.0. Selection also caused a large decrease during the first generation: additive genetic variance was 0.0079 +/- 2.1 x 10(-3) in G3 and 0.0040 +/- 1.1 x 10(-3) after the first selection cycle (P < 0.02). At the same time, the effective number of ancestors decreased from 59.2 to 29.5 and 24.2. This decrease is consistent with the theory of selection and the Bulmer effect.

Direct and correlated responses to individual selection for large adult weight in the edible snail Helix aspersa Muller.

A selection experiment for large adult weight based on individual performance was conducted for three generations in Helix aspersa aspersa. A second line was kept as an unselected control line. Direct response measured as deviation from the control line was 3.55 g after three generations of artificial selection, which averaged 13%. Realized heritability was 0.38 +/- 0.04. Correlated responses to selection showed a significant increase in weight after hibernation, mean egg weight and mean weight of newly-hatched snails with selection. For adult age, egg number, and hatching rate, no significant change correlated to selection was found, but this is to be confirmed.

[Congenital asymmetrical pelvis. Clinical, radiological study and outcome]. / Le bassin asymétrique congénital. Etude clinique, radiologique et évolution.

In congenital pelvic obliquity, there is a restricted abduction in one hip and an abduction contracture in the other. This association has been considered as a cause of acetabular dysplasia with progressive subluxation of the hip opposite to the abduction contracture. The clinical and radiological study of 120 children with an average follow-up of 3 years and 3 months permitted to demonstrate that two different types exist: the simple types (93 cases) without dysplasia, which never developed progressive subluxation and must not be treated, and the pelvic obliquities with dysplasia-subluxation-dislocation (27 cases) which have to be treated if the decentration of the hip opposite to the abduction contracture is marked. Distinguishing between these two types may be difficult because the X-ray is nearly never strictly correct because of the pelvic obliquity. This work leads to a better comprehension of the acetabular dysplasias who are subdivided into pseudodysplasias, secondary dysplasias and primary dysplasias. In the screening for the detection of congenital dislocation of the hip, it is essential that a pelvic obliquity be recognised because it constitutes a sign of hip at risk. Then, the clinical research of instability could be completed by echographic study in the two or three months or by radiographic study after 3 months in order to have a very precise diagnosis and apply the best treatment.

Minor tibial duplication: a new cause of congenital bowing of the tibia.

Five infants presented with a congenital anterolateral, unilateral bowing of the tibia. The fibula was normal but a bifid homolateral great toe was seen, associated with hand malformations. Plain films and CT disclosed a short tibial duplication, with distinct corticals. We do think that this abnormality represents a new entity, and may be related to a genetic anomaly. The long term follow up of our patients shows a spontaneously favourable prognosis without any association with pseudarthrosis. The only therapeutic problem is a limb length discrepancy.

Submaximal oxygen consumption in liver cirrhosis. Evidence of severe functional aerobic impairment.

We studied maximal aerobic work capacity in 24 cirrhotic patients whose clinical condition was well compensated. Nineteen of our patients had no heart or lung disease, and five had mild mechanical ventilatory impairment. The patients performed incremental exercise on a treadmill until subjective exhaustion. The maximal exercise levels reached by all were relatively low and led to a lower observed maximal oxygen uptake (VO2 max), than predicted uptake (19.6 +/- 0.5 vs. 37.9 +/- 0.6 ml/kg; p less than 0.001). Observed VO2 max values correlated strongly with the Pugh score, which reflects the degree of liver failure (r = -0.571; p less than 0.01). Since there were no clear cardiac or pulmonary causes to explain the decrease in work capacity, these observations suggest that liver cirrhosis might induce or be accompanied by muscular impairment. VO2 max, which seems to decline with the functional severity of the disease, may be a useful index for evaluating the capacity of patients for physical rehabilitation.

Hormonal and metabolic changes during exercise in cirrhotic patients.

The metabolic response to exercise was compared in 10 cirrhotic patients (P) in a stable clinical condition and in 6 sedentary, age-matched, normal subjects (C) performing 32 minutes of treadmill exercise with the same constant workload corresponding to three to four times their resting oxygen uptake. Taking indirect calorimetry as reference, respiratory exchanges indicated that cirrhotic patients consumed carbohydrates almost exclusively, unlike the normal controls, who consumed lipids and glucids in about the same proportions (RQ: 0.98 +/- 0.04 v 0.87 +/- 0.04, P less than .0001). In the patients, this carbohydrate path of exercise metabolism lowered glycemia from the resting value of 5.23 +/- 0.16 mmol/L to 4.03 +/- 0.37 mmol/L (P less than .0001) and raised the plasma lactate concentration from 2.08 +/- 0.24 mmol/L at rest to 3.48 +/- 0.32 mmol/L at the eighth minute of exercise (P less than .001), thus suggesting defective liver glyconeogenesis. Fatty free acids and glycerol remained almost constant during exercise, whereas catecholamines increased. Insulin levels were high in patients at rest (67.1 +/- 14.5 U/mL v 15.1 +/- 3.5 U/mL); they declined sharply at the onset of exercise but nevertheless remained high compared to those observed in the controls (P less than .0001). Glucagon increased in exercising patients from 88.3 +/- 21.3 pg/mL to 127.4 +/- 30.6 pg/mL (NS). Esterified plasma carnitine declined in the patients from 13.0 +/- 2.2 mumol/L to 8.6 +/- 1.5 mumol/L (P less than .05).(ABSTRACT TRUNCATED AT 250 WORDS)

[Pathogeny and natural history of congenital dislocation of the hip]. / Pathogénie et histoire naturelle de la luxation congénitale de la hanche.

Based on a review of the literature, the authors have made a critical study of several etiological factors. Endogenous factors such as acetabular dysplasia, increased anteversion of the femoral neck, capsular laxity support the genetic theory but they are neither constant nor necessary and are only facilitating factors. The major factor seems to be a mechanical one linked to the position in uterus: hyperflexion with adduction and external rotation causing an abnormal pressure on the greater trochanter leading to an expulsion of the head upward and backward. This theory can explain the natural history of C D H which is first, at birth a hip unstability followed by to possible evolutions: either a persistent luxation becoming irreducible or a spontaneous stabilisation leading sometimes to complete healing or to residual abnormalities (subluxation or dysplasia). This concept suggests practical conclusions: the importance of an early diagnosis, the selection of the signs of the hip at risk the pattern of prevention, the importance of non clinical investigations, the principles of the treatment based on postures, the indications for the different types of treatment.

[Treatment of acute pyogenic arthritis of major joints of the limbs]. / Traitement des arthrites aiguës à pyogènes des grosses articulations des membres.

The authors have treated 52 cases of septic arthritis of major joints. When aspiration contain an opaque fluid, conservative treatment with antibiotic therapy and immobilisation obtained healing in half of the patients. Surgical lavage of the joint was needed on 21 occasions with healing in 15. Synovectomy was performed in 8 cases and was indicated only at the stage of pyogenic synovitis before the development of pyogenic arthritis. When the latter stage was reached, arthrodesis was indicated. It was performed primarily on 6 occasions and secondarily once. Seven patients died. The average age was seventy years. The importance of early treatment is stressed.

[The first Zancolli procedure in the treatment of cubital and mediocubital claw hand. Apropos of 32 cases]. / L'intervention de Zancolli première manière dans le traitement des griffes cubitales et médio-cubitales. A propos de 32 cas.

The authors have reviewed 32 cases of claw hand due to lesions of the ulnar nerve only in 25 cases or associated lesions of the ulnar and median nerve in 7 cases treated by the first Zancolli procedure. This procedure consists of advancement of the pulleys and anterior capsulorraphy of the metacarpo-phalangeal joints. One case had to be operated on twice. In 25 instances there was a complete division of the nerve. One 17 occasions the procedure was done on the ring and little fingers only and on 12 occasions on the other finger. The results were excellent or good in 23 cases. An associated tendon transfer in the thumb was done in all cases in which there was an associated median lesion.

[Fatigue fracture of both femoral necks ina 19-year-old woman (author's transl)]. / Fracture de fatigue de deux cols fémoraux chez une femme de 19 ans. A propos d'un cas.

A case of slow fracture of both femoral heads in a 19-year-old woman is reported. The radiological characteristics, clinical context and onset in a histologically normal skeleton were in favour of a diagnosis of fatigue fracture. Data in the literature show the rarity of this condition affecting the neck of the femur in the young woman and rarely mention the existence of morphological abnormalities as a favouring factor.

[A device for studying the dynamic response of a gas analyser (author's transl)]. / Dispositif d'étude de la réponse dynamique d'un analyseur de gaz.

A mechanical device has been built, enabling one to produce a step change in gas concentration at the inlet of gas analyser. It is made of two chambers filled each with a different gas mixture and sliding rapidly in a cylinder in such a way that the probe from the gas analyser is exposed sequentially to the two gas mixtures. The total translation time is 6 ms. The devise has been used to study the response of a quadrupole mass spectrometer. Both the delay and the response time vary with the gas mixtures and/or the direction of the concentration changes.

[The effect on gas mixing of a He-O2 mixture in chronic obstructive lung diseases (author's transl)]. / Influence du mélange He-O2 sur la mixique dans les bronchopneumopathies obstructives chroniques.

In order to estimate the role played by gaseous diffusion in the mixing disorders of chronic obstructive lung disease (COLD), the effect of breathing a gas mixture lighter than air has been studied. Twenty four patients with severe airflow obstruction have been tested in the following way: in a random order they breathed two different mixtures with the same PO2 : air and helium-oxygen (heliox) for 20 min. Ventilation was monitored during the whole of each run; during the 2 last min arterial blood was sampled. While breathing heliox a slight, non-significant, increase in ventilation has been observed with a slight but statistically significant decrease of PaO2 (p less than 0.01), of PaCO2 (p less than 0.05) and increase of pH (p less than 0.01). These changes suggest a slight increase of distribution disorders with alveolar hyperventilation. For these results to be consistent with stratification, improvement of the diffusion due to low density should have been masked by other phenomena; the possible effects of ternary diffusion, increased viscous resistances and change of transfer factor have been looked at. No conclusive evidence has been found of such counter-effects. Therefore it looks unlikely that stratification be the major factor in distribution impairment in COLD.