From his first years a child showed signs of a primary and rapidly developing muscular dystrophy. The diagnosis was established by an increased serum CK level and by electromyography and muscle biopsies. Afterwards this child developed a severe renal deficiency which needed binephrectomy and the graft of a normal kidney. During the few months just after the graft, the disability increased and the patient could not stand upright by himself. Later on, he gradually became able to walk on his own and without bracing. He could climb stairs and stand up from the floor. The CK activity returned to normal. At present, 4 years after the graft (the patient is 16 years), the improvement of his functional abilities is constant, although the CK activity has increased again. In this article we give evidence that this patient suffers from a primary muscular dystrophy. We discuss the type of dystrophy concerned. We believe that it is the graft of a normal kidney which was responsible for the improvement observed, and not the physiotherapy or the drugs administered after the graft.

Adolescent↗

PubMed1976

Variations in muscle cell protein of severely uremic children.

Alkali-solution protein (ASP) was determined in the muscle of 17 controls and 32 uremic children including 9 patients receiving intermittent hemodialysis. With regard to fat-free solids content (FFS), ASP values were found to be lower in uremic children than in normal subjects (59.2 +/- 1.6 vs. 76.4 +/- 1.9 g/100 g of FFS). This decrease was greater in hemodialyzed children (53.8 +/- 1.6 g/100 g of FFS). ASP depletion was found also when the results were expressed in relation to muscle potassium and phosphorus and therefore appeared to be due to an actual decrease in the amount of proteins per cell. ASP values were especially low (52.2 +/- 2.8 g/100 g of FFS) in seven nondialyzed children receiving a low protein diet (less than 0.165 g of N/kg of body wt/day) and in patients with a height below 2 SD (56.3 +/- 3.1 g/100 g of FFS). In the nine cases of glomerulonephritis, loss of muscle protein sfollowed plasma protein depletion. A relationship was found between plasma transferrin concentration and ASP. ASP depletion seemed to be related to the level of renal insufficiency. However, the most important factor in ASP depletion was probably an inadequate protein intake associated with the possible metabolic effects of uremix toxins which are currently unknown.

Adolescent↗

PubMed1976

Effects of 25-hydroxycholecalciferol on bone lesions of children with terminal renal failure.

Quantitative histology was performed on serial iliac crest biopsies obtained from 14 children with terminal renal failure. A long-term study on the comparative effects of vitamin D2 and 25-hydroxycholecalciferol [25-(OH)D3], in five patients with severe lesions of osteomalacia and/or osteitis fibrosa, demonstrated the efficiency of 25 to 200 mug/day of 25-(OH)D3 and the lack of therapeutic action of 345 to 685 mug/day of vitamin D2. In nine subjects with normal roentgenograms or minimal skeletal alterations, the first biopsy taken at the beginning of intermittent hemodialysis showed evidence of defective mineralization and/or lesions of resorption. Four of these children were treated with 25-(OH)D3 (25 to 50 mug/day) and calcium supplementation orally (0.5 to 1.5 g/day); five children received calcium orally (0.5 to 0.75 g/day) alone. Aggravation of bone lesions during intermittent hemodialysis was observed in patients treated with calcium supplements alone. In subjects who were given 25-(OH)D3, mineralization improved and marrow fibrosis disappeared. However, as the two groups of patients were different in composition and in the manner in which they were treated, it is difficult to state whether the beneficial effects observed were solely attributable to 25-(OH)D3 administration. 25-(OH)D3 therapy induced severe intoxication in two patients. A rise in plasma calcium concentration to 11.0 to 11.5 mg/100 ml was observed in two other patients. It is concluded that: a) pharmacologic doses of 25-(OH)D3 are highly effective in healing bone lesions of children with terminal renal failure; b) such treatment requires strict clinical surveillance as 25-(OH)D3 intoxication may occur even in anephric patients.

Adolescent↗

PubMed1976

Abnormal TSH, PRL and GH response to TSH releasing factor in chronic renal failure.

TSH, PRL and GH response to TSH releasing factor as well as basal T4 and T3 were evaluated in a group of patients with chronic renal failure undergoing chronic hemodialysis. Serum T4 and T3 were lower than normal. Basal TSH was normal as compared to control, but did not rise after TRF stimulation. Larger dosages of TRF did not correct this abnormal response. Basal PRL was higher than control and remained at the same level during the test. GH was stimulated by the TRF with a peak occurring 20 min after injection. This abnormal secretion was not blunted by T3 administration. TRF half-life measured in 3 patients was 4 min. These data indicate that 1) there is an abnormal response to TRF in chronic renal failure which does not seem to be due to an altered sensitivity to, or metabolism of TRF; and 2) there is an abnormal TSH secretion which may be responsible for the low T4 and T3 measured in these patients.

Adolescent↗

PubMed1976

Anaphylactoid purpura nephritis in childhood: natural history and immunopathology.

Explore the source record for details and available documents.

Adolescent↗

PubMed1976

[Preliminary psychological evaluation of a group of children before and after renal transplantation].

Eighteen children who have received kidney transplants within the last three years at the Hopital des Enfants Malades in Paris provide material for this survey. We try to find out how these transplants effect the way the children live, and the main psychological problems that they create. This new way of life is characterized by an explosion of vitality, a certain liberation from prior restraint and the possibility of activities which up to then had been difficult or forbidden. However, the necessity for the child to be watched regularly, to attend the hospital for examination, to follow a special diet, to take immunodepressive drugs remind him that he is not cured. Consequences of corticotherapy and the growth retardation remain essential problems for some of them. The child will have to cope with this complex situation which also creates difficulties to acquire independence.

Adolescent↗

PubMed1976

Renal transplantation in children: a psychological survey.

Psychological tests were carried out on 24 children with renal disease during the time they were hemodialyzed and after a minimum of one year after their transplant. These investigations included regular interviews with children and families, drawings and their commentaries, and projective techniques: Rohrschach tests. The results of these investigations indicated that the psychological status of the transplanted child could not be singled out and isolated from the effects of being ill, the different therapeutic procedures, and the personal history. Beyond the appearance adolescents give of a satisfactory social adjustment and beyond the explosion of vitality frequent amongst younger children, an impoverishment of their personality and major difficulties manifest themselves in coping with personal problems and identity crises especialy as far as agressiveness and sexuality are concerned. This neurotic mechanism appears mainly due to the traumatic experiences these children have experienced and their lack of support in mastering them through the verbalization process.

Activities of Daily Living↗

PubMed1975

Growth in experimental renal failure: role of calorie and amino acid intake.

In order to evaluate the role of calorie and protein intake in growth impairment due to chronic renal failure (CRF), a subtotal nephrectomy was performed in weanling Wistar rats. A two-thirds reduction of renal function was obtained, which induced a marked growth retardation. Growth retardation was identical in nephrectomized and in pair-fed controls, and thus appeared to be entirely due to a deficient food intake. Using low protein diets, administration of a supplement with small amounts of essential amino acids (EAA) resulted in accelerated growth associated with a higher calorie intake, a better utilization of ingested calories for growth and a greater fall of blood urea nitrogen concentration.

Amino Acids, Essential↗

PubMed1974