Search

While a diet deficient in phosphorus has a protective effect against kidney deterioration in uraemic rats, this effect, in fact, is more closely related to the reduction of food intake than tho the phosphorus load itself, even though the latter influenced the pattern of the renal lesions.

C. was used in 18 Steroid-Resistant (SR) N and in 27 Steroid-Sensitive (SS)N. Two regimens were used: in the former a total dose of 0.8to1/mg/kg was given alone using 12 IV injections over one month. In the latter a total dose of 0.8 mg/kg was given over two periods of 4 days separated by one month, and was associated with Prednisone (2 mg/kg/day) and anti-emetic agents. In SRN, only one patient remitted, whereas 2 had a transient ( 2 weeks) and one a partial remission. The others were un changed. SSN were all steroid dependent and 13/27 had severe steroid toxicity signs. C. was given 0.5 to 11 years after onset. A remission was obtained in all but two patients treated with the first regimen. Remission lasted less than one year in 10, 1 to 2 years in 5. In the remainder, follow-up was less than 2 years, except for 4 children: one had a relapse 4 years later, and 3 had sustained remission 2,2 1/2 and 13 years after therapy. Of the 16 patients who relapsed, 6 received other nitrogen mustards and 10 were controlled using non toxic doses of alternate-day Prednisone therapy. Side effects were leucopenia (20%) including pancytopenia in 3 children and gastrointestinal disorders (50%). Only one patient was studied in adulthood and had a normal spermcount. In conclusion: C. was ineffective in SRN, and resulted mainly in short remissions in SSN. In SSN however, C. when associated with Prednisone seemsbeneficial in decreasing the dose of Prednisone required to maintain remission.

32 children with FN were studied, after exclusion of finnish type. They belonged to 17 families totalizing 39 affected subjects. When compared to sporadic nephrosis (N) FN showed a higher incidence of infantile onset (11/32 vs 9/249: p < 0.001) of males affected (81 vs 71%), of steroid resistance (16/32 vs 65/249 p < 0.01) and poor outcome, but appeared as a heterogeneous entity. Two groups were separated according to steroid (S) response: 16 children from 9 families were S-responders, 16 from 8 families were S-resistant. No discordance was found in any of the families. S-responsive FN showed the same age and sex distribution as sporadic N and similar good prognosis. It affected both sexes in 5 families, two generations in 3 and 2 siblings in 6 families. S-resistant FN affected exclusively siblings (3 in 3 families, 2 in the others), boys except for 2 sisters, and children under 3 (11/16 under 1 year). Two pairs of siblings had microcephaly and dysmorphy. Three died from complications, 6 deteriorated to renal failure after 1 to 14 years and 7 had normal renal function after 1 to 15 years. Renal biopsy showed in S-responders 6 minimal changes (MC) and one focal lesion (FL), and in S-resistant 8 MC, 1 FL and 4 mesangial sclerosis (including 3 siblings). In conclusion: S-sensitive FN do not differ from sporadic N. The facilitating role of environmental or genetic factors is not prominent but cannot be excluded. S-resistance FN appears as (a) different entities(y) and is consistent with a heriditary disease suggesting a recessive transmission.

312 children with SSN were followed for 2 to 24 years (y). Age at onset was under 1 in 8 who never relapsed over 2 to 14y, and between 1 and 8y in 80 % of cases. 77 children were unselected: of them, 32 (42 %) never relapsed, 8 recovered after 1 or 3 relapses, 36 were steroid (S) dependent (D). Among the 312 children representing a highly selected series, 4 children died from complications and 8 developed renal failure (RF): five were SD for high doses, remained untreated and nephrotics for at least 15y: 3 reached terminal RF (TRF), 19, 20 and 21y after onset. Three children with initially SSN became resistant and were referred for TRF 2, 5 and 6y after onset. The 300 other patients had normal renal function after 5 to 10y in 118, 10 to 15y in 51, 15 to 24y in 23. 62 patients never relapsed, 24 recovered after 1 to 3 relapses, and 214 were SD. Of the latter, 104 received immunosuppressants (IS) resulting in prolonged remission in 74. Interrupted or alternate day S therapy was used for respectively 1 to 7y in 38 children, and 1 to 6y in 35. The latter allowed a good control of the disease with little toxicity and normal growth. In SD, therapy could be discontinued without IS in 26 children after 8 to 5y, in 15 after 5 to 10y, between 10 and 20y in 13, and remained necessary after 10 to 18y in 17 children, all of them with normal renal function. The high incidence of a single attack in unselected series, the unpredictable duration of disease in SD patients, the possibility of poor outcome in untreated patients and the good prognosis in the others may be stressed out.

Binding sites for human growth hcrmone (hGH) were studied in liver membranes of female rats with chronic renal insufficiency (CRI) associated to marked growth retardation. One month after a subtotal nephrectomy the animals with a plasma creatinine level > 3 times that of controls were studied ; their mean statural gain was 56 % that of controls. The specific binding of 125I-hGH to microsomal membranes of rats with CRI was low (62 % that of controls). The number of binding sites rather than the affinity of the binding was affected ; both the lactogenic and the somatotropic sites were decreased as judged on the binding of 125I-oPRL and 125I-bGH. The binding sites of the plasma membranes as well as those of Golgi fractions were reduced. In plasma membranes of rats with CRI the specific binding of glucagon was low and the specific binding of insulin was elevated ; these modifications were associated with a high plasma glucagon level and a decreased insulinemia in rats with CRI. But no modification of the plasma GH and PRL was found; the hormone level does not appear to regulate the GH binding sites in this system.The link between the growth defet and the decreased number of GH binding sites in the liver membranes of rats with CRI remains to be established.