Autosomal recessive anhidrotic ectodermal dysplasia in a large Moroccan family.

We studied a large Moroccan family in which anhidrotic ectodermal dysplasia is transmitted as an autosomal recessive trait. Fourteen family members, both males and females, were affected and they all had a common ancestor. Linkage analysis by homozygosity mapping in this family will permit the gene localisation of this rare form of anhidrotic ectodermal dysplasia.

Chronopharmacokinetics of Cyclosporine A in the Wistar rat following oral administration.

The pharmacokinetics of Cyclosporine A (CsA) was studied in male Wistar rats weighting 300 +/- 50 g trained to a 12:12 light-dark cycle. Oral administration (40 mg/kg) was performed at 1 of 4 different temporal stages: 09.00 h, 15.00 h, 21.00 h or 03.00 h (local time) i.e. 0200, 0800, 1400 or 2000 HALO (hours after light on). Blood samples were collected over 72-96 h after dosing, plasma was separated by centrifugation at 37 degrees C and stored frozen until assay, using radioimmunoassay (RIA). Two experiments were performed: the first with 4 groups of 48 rats and a non-specific polyclonal antibody (P-RIA); and the second with only 2 groups of 48 rats and a more specific monoclonal antibody (M-RIA). Plasma concentration data were evaluated with model-based linear pharmacokinetic concepts, with apparent zero-order or first-order absorption and n-exponential disposition (n = 1, 2 or 3): models MN0 or MN1. A compartment-independent approach was also conducted and led to area under the plasma concentration-time curve (AUC) and mean residence time (MRT) determinations. A comparison of the pharmacokinetic profiles across time of administration indicates that absorption, first-pass metabolism and tissue distribution of CsA in the rat are circadian-dosing stage dependent.

Cyclosporine A dosing-time-dependent effects on plasma creatinine and body weight in male Wistar rats treated for 3 weeks.

Cyclosporine A (CsA) nephrotoxicity was assessed in 120 male Wistar rats (350 +/- 50 g) entrained to a 12-h cycle (light-dark 12:12); plasma creatinine level and body weight were examined in controls and in rats that had been treated daily with oral CsA or vehicle alone (olive oil-ethanol 90:10) for 21 days; daily dosing (40 mg/kg) was at one of six equally spaced given times during the 24-h cycle. The variations observed in both indexes were shown to be circadian dosing stage dependent. Nephrotoxicity was present as early as the third day of treatment with CsA; plasma creatinine level was enhanced by about 50% in rats dosed around the time of the change from darkness to light: at 22 HALO, 146.7 +/- 4.5 mumol/L, against 92.0 +/- 2.8 mumol/L for controls (p less than 0.05); and at 2 HALO, 148.3 +/- 10.0 mumol/L, against 95.0 +/- 4.3 mumol/L for controls (p less than 0.05). Thereafter, a remission episode was observed between days D5-D9. The more drastic effects were seen on days D16 and D21, in animals dosed in the beginning of the dark span (14 HALO): 185 +/- 10 mumol/L for CsA and 98.0 +/- 5.3 mumol/L for controls (p less than 0.01) and, to a lesser extent, in rats treated at the early resting phase (2 HALO): 152.4 +/- 31 mumol/L for CsA and 95.0 +/- 4 mumol/L for controls (p less than 0.05). The normal increase in body weight during the 21-day period (about 14 +/- 8% in controls) was impeded in CsA-administered rats, especially those dosed at the beginning of the activity span (14 HALO) that even suffered weight reduction. Differences in percentages of survivors were noticed, depending on dosing stage. About 40% of the animals in every time CsA-treatment group died, except for those dosed at the end of the resting period (10 HALO), when all animals died. In surviving rats, the cessation of CsA dosing resulted in a reversible effect on the study variables.

Immunoreactive somatomedin C in children from Morocco: a biological marker of nutritional growth retardation?

Somatomedin C (Sm-C) is supposed to be a sensitive marker of malnutrition. Its relationship to growth and protein intake was investigated in 47 Moroccan children. The children (mean age: 7.5 +/- 2 yr) had no endocrine abnormality. Anthropometric parameters were measured, together with blood levels of Sm-C and growth hormone (GH). Lower values of Sm-C were found in the following situations: (a) growth retardation (more than -2 Sd, N = 23; Sm-C = 3.61 +/- 0.42 nmol/l vs. 13.38 +/- 1.52, P less than 0.02); (b) body weight less than 80% of expected weight for height (N = 8; Sm-C = 4.84 +/- 0.97 vs. 10.33 +/- 1.19, P less than 0.02); (c) boys with meat consumption less than 3 times per wk (N = 15; Sm-C = 4.03 +/- 0.71 vs. 12.35 +/- 2.01, P less than 0.002). Thus, lower values of Sm-C are found in association with weight insufficiency and lower protein intake. As has previously been assumed, serum Sm-C, which is regulated by nutrition, may be a sensitive tool in the assessment of nutritional status in developing countries such as Morocco. Furthermore, it could explain some cases of growth retardation in states of malnutrition.

[Treatment of post-poliomyelitis paralytic scoliosis of more than 80 by skeletal traction followed by posterior vertebral arthrodesis fixed with Luque's segmental spinal device]. / Traitement des scolioses paralytiques post-poliomyélitiques de plus de 80 degrees par traction squelettique, puis arthrodèse vertébrale postérieure fixée par instrumentation spinale segmentaire selon Luque.

We present 45 consecutive cases of post-poliomyelitic paralytic scoliosis, with an angle ranging from 90 degrees to 145 degrees (mean 117 degrees), seen in subjects aged between 12 and 16 years, and treated by arthrodesis using a Luque-type segmental spinal apparatus with, in the cases showing an oblique pelvis, anchoring to the pelvis using the Galveston technique. Post-operative contention was not performed. Neurological complications were not seen in any patient. There were no cases of rupture of either rods or wires. The loss of correction at one year was between 5 and 10 degrees. The technique was shown to be effective when obliqueness of the pelvis was not present. In rigid forms, with an oblique pelvis, a circumferential arthrodesis is indicated provided that a rigid apparatus has not previously been inserted thereby preventing supplementary correction via the posterior route.

[Spinal fractures in children. Diagnostic and therapeutic problems. Apropos of 67 cases]. / Fractures du rachis chez l'enfant. Problèmes diagnostiques et thérapeutiques. A propos de 67 observations.

Between 1960 and 1980 the authors have observed 67 cases of spinal traumatism for children under 15 years old. Neurological deficit was present for 25 of these patients. At the first examination for 17 and after for 8. 5 children have total paraplegia without osteoligamentary lesions. The cervical traumatisms were without other injuries and were the result of minor trauma. The thoracolumbar traumatisms were accompanied by other injuries and were the result of violent trauma. Most of these lesions have been reduced by orthopedic method; only 4 children were operated because the dislocation was incoercible. The 15 children who presented total paraplegia had no recovery. For all the others, the recuperation was good.

[Conservative treatment of injuries of the spleen in children]. / Expérience du traitement conservateur des traumatismes de la rate chez l'enfant.

Resolutely convinced of the necessity to preserve the spleen in all circumstances, the authors detail the indications and possibilities of non operative management and conservative surgery that allow the injured spleen to be salvaged. Of 55 cases observed from 1978 to 1982, only 22 required splenectomy because of our new experience; 13 of the remaining cases were treated non operatively and 20 had a conservative surgery. It is concluded that unless the child prognosis is directly and immediately involved, by the splenic injury (such cases require splenectomy or better still partial resection) every effort should be made to preserve a traumatized spleen.

Indications for halo immobilization of the cervical spine.

In the United States, cervical spine injuries are routinely immobilized with the halo apparatus. In Europe, this device is seldom used except in the treatment of spinal deviations. Based on studies of five cases the authors discuss the indications for use of the halo device in cervical spine injuries.