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The purpose of the present study was to assess the effect of intelligence, schooling, psychomotor, emotional, and social status on renal graft survival in children. Sixty-two cadaver renal transplant recipients were evaluated retrospectively and the influence of sex, age, weight, and the use of cyclosporin A (CyA) on the success rate of the graft from 1 to 5 years later was analyzed. Psychological and social scores were devised and included as factors predictive of survival of the graft. Univariate analysis showed that the following variables predicted renal graft survival: the use of CyA (P = 0.0002), pre-transplant dialysis (P = 0.04), weight at the time of transplantation (P = 0.072), and psychological scores (P = 0.064). Association analysis demonstrated that pre-transplantation dialysis was only a chance association and therefore the parameter was discarded. Multivariate analysis showed that the predictive parameters were the use of CyA, sex, weight in kilograms, and the psychological score. An equation was then derived from variables that predict the probability that a specific patient's graft will survive more than t months. This equation is the estimated survival distribution function and is as follow: S (t) = Exp {-Exp[-(0.8882x1 - 1.827x2 + 0.037x3 - 0.1746x4) + ln t - 4.7862]} where S (t) = the survival at t months post transplantation, x1 = sex (male 1, female 2), x2 = CyA (yes 1, no 2), x3 = weight in kilograms, and x4 = psychological score. The major impact of psychological factors on renal graft survival was surprising.

Erythrocyte cation fluxes were measured in fresh cells, on two different occasions, in 90 children and adolescents suffering from essential hypertension who were followed for a prolonged period. The purpose of the present study was to track the stability of erythrocyte cation fluxes with time, and to determine whether a known sodium transport abnormality can predict the severity of essential hypertension. The patients with an increased Na—Li countertransport were the most severely hypertensive in three different ways. Clinically, they presented a stable rather than a labile form of hypertension. Hemodynamically, the mean arterial pressure was higher than that of the other subgroups. Finally, in those children followed for more than 2 years, this subgroup remained hypertensive with time. The patients with a decreased Na,K cotransport activity were second in severity; however, blood pressure was often labile, mean arterial pressure was slightly lower than that of the previous subgroup and all patients remained hypertensive with time but in four out of six patients the hypertension was labile. The patients with increased passive sodium permeability presented a mild form of hypertension. The patients with normal erythrocyte cation fluxes seemed to form a heterogeneous group. A few were severely hypertensive, but in most the hypertension was borderline and transient. We conclude that determination of erythrocyte cation fluxes may be reproducible, and may be a useful index of the severity of essential hypertension in children and adolescents.

Based on the histologic aspects of the lesions, primary nephrosis with focal glomerular sclerosis has been classified into focal segmental glomerulosclerosis (FSG) and focal global glomerulosclerosis (FGG). Several reports [1–14] define its clinical presentation and attempt to identify the initial signs that indicate a poor prognosis. The criteria indicating poor prognosis were reported to be age of onset [15, 16], the presence of a nephrotic syndrome [10, 15, 17], mesangial proliferation [14], the presence of focal segmental glomerulosclerosis on early biopsies [17], the association of glomerulotubular imbalance [18], the severity of the lesions on an early biopsy [1–3, 5, 6, 8, 10, 12, 13, 17], and especially the resistance of these patients to corticosteroid and immunosuppressive therapy [1–3, 6, 8, 9, 11–13, 16, 17]. The long-term followup of children with focal glomerular sclerosis has been analyzed in detail, however, only in a few articles [2, 3, 17], and these reports have concluded that the prognosis is generally poor. The purpose of our study is to report the outcome of observing 32 children with primary nephrosis and focal glomerular sclerosis over a period of several years. We observed that the long-term prognosis in our series is more favorable than that described by other reports. We believe that this discrepancy is due to the referral profile of our institution and that our population of patients more accurately reflects the full spectrum of the natural history of the disease.

Erythrocyte Na+ and K+ transport systems in children with Bartter syndrome: Increase in passive sodium permeability. Na+ and K+ intracellular content was studied in five children with Bartter syndrome and their age and race-paired controls. Na+ and K+ pump (ouabain sensitive) fluxes, Na+-K+ co-transport (furosemide sensitive), and rate constants of passive Na+ and K+ permeability were determined in each patient and control and also in six parents. The results show that in Bartter syndrome, there is a significant increase in the rate constant of passive Na+ permeability without any change in passive K+ permeability. This increase in the rate constant of passive permeability might explain at least partially the increased intracellular Na+ concentration also found in these patients. Moreover, the maximal rate of ouabain sensitive Na+ efflux was increased slightly, and co-transport fluxes were variable. Parents of patients had normal erythrocyte fluxes. Etude de divers systemes de transport du Na+ et du K + a travers la membrane erythrocytaire chez des enfants souffrant du syndrome de Bartter: Augmentation specifique de la permeabilite passive du sodium. La concentration intra-erythrocytaire en Na+ et K+ a ete mesuree chez cinq enfants souffrant du syndrome de Bartter couples a des temoins du meme âge et de la meme race. Les flux de la pompe a sodium (sensibles a la ouabaine), le co-transport du Na+ et du K+ (sensible a la furosemide) et le taux de permeabilite passive du Na+ et du K+ ont ete determines chez chaque patient et leur temoin ainsi que chez six parents de patients. Nos resultats demontrent que dans le syndrome de Bartter, le taux de la permeabilite passive du Na+ est eleve par rapport aux temoins, sans elevation toutefois du taux de la permeabilite passive du K+. Cette elevation du taux de la permeabilite passive du Na+ peut expliquer en partie les concentrations intra-erythrocytaires elevees de Na+ retrouvees chez nos patients. Quant aux autres systemes de transport, nos patients presentaient un taux maximal d'efiiux de Na+ sensible a la ouabaine legerement eleve et des flux des au co-transport variables. Les flux de Na+ et K+ chez les parents de patients etaient normaux.

This study presents an analysis of the role of genetic and household environment in explaining the familial aggregation of height (HT), weight (WT), and body mass indices (WT/HT1.2 and WT/HT2.0). The biologic model used for the analysis partitions the covariances between family members into the contributions of genetic and environmental variability shared within and across generations, including a variance component shared only by a mother and her natural children. Tests of hypotheses suggest that shared genes and shared household environmental factors make significant contributions to the familial aggregation of WT (adjusted for age and sex) and of HT (adjusted for age and sex), whereas family resemblance of WT adjusted for age and HT can be attributed mostly to the effects of shared genes. The familial aggregation of selected WT/HT indices is attributed to the effects of shared household environment only, suggesting that these variables measure a characteristic of stature that is independent of height and weight.

In contrast to the three previously recorded patients with cystathioninuria, the infantdescribed here was mentally normal and had thrombocytopenia and urinary calculi. Studies were carried out to determine possible interrelationship of these manifestations with the disturbed metabolism of cystathionine.

Seven children and one adult are described who presented with signs of fixed urine specific gravity, anemia and renal insufficiency. Histologically, the kidneys of these patients showed periglomerular and interstitial fibrosis with dilated and/or atrophic tubules. Two of the patients also had cysts in the kidneys, chiefly in the medulla. These patients are thought to suffer from nephronophthisis, an uncommon familial disease of unknown etiology. Examples of this disease have also been recorded in the American literature under the term medullary cystic disease of the kidney. Evidence is presented which suggests that this disorder might represent a toxic nephropathy.

Amoxicillin was used in treatment of 20 children suffering from recurrent urinary tract infection. Patients were divided into three groups: those without obstruction, those with obstruction, and those with renal parenchymal damage. Amoxicillin eradicated organisms from over 80% of urine samples within 48 hr. Three weeks after onset of therapy, most of the patients were again infected either with the same organism (which had become resistant to therapy) or with a new organism. Amoxicillin was well tolerated by the children; diarrhea, the only side effect of the drug, was observed in two instances.

The long-term damaging potential of remnant nephron hyperperfusion was investigated in patients who had undergone unilateral nephrectomy in childhood. 27 such patients were examined after a mean of 23·3 years postnephrectomy. The average creatinine clearance was 83·9±16·5 ml/min/1·73 m 2 or 74·3% of that in healthy controls with two kidneys; it was a value similar to that reported 3 to 6 months postnephrectomy in kidney donors. Age at the time of nephrectomy, duration of follow-up, or sex had no influence on the residual creatinine clearance. None of these patients had clinically important hypertension or proteinuria. Since so little evidence of kidney damage could be documented after such a long observation period, hyperperfusion would seem to be seldom of clinical importance in man unless other factors were present.

Body weight and weight/height were measured in 535 children adopted at the median age of 3 months, and in 250 natural children in French-Canadian origin living in 374 Montreal homes, to determine whether the shared environment contributed to the familial resemblance of weight in children aged one to 21. The mid-parent vs natural children's correlation ( r2 X 100) was 9.55% for body weight and 6.60% for W/H (p less than 0.01), whereas the mid-parent vs adopted children's correlation was 0.00% for both characteristics. The sib-sib correlation in 80 homes with greater than 1 natural child was 15.2% for weight and 13.48% for W/H (p less than 0.001), whereas in 138 homes with greater than 1 adopted child, the adoptee-adoptee correlations were, respectively, 0.00% and 0.07%. It is concluded that heredity explains most of the familial aggregation of patterns of weight and weight/height in children. This conclusion does not necessarily apply to obesity, since weight indices in children do not accurately reflect excess fat tissue, and half of the adoptees were adopted after the age of three months.

This paper presents a review of the genetic transmission of normal blood pressure and of essential hypertension. Familial aggregation of normal blood pressure has been reported in adults, in children and even in newborns. Blood pressure aggregation phenomenon, however, is the result of both a genetic component and shared environmental factors. More specific for each etiological factor were the studies of blood pressure aggregation in twins and in adopted children. Attention was focused on the Montreal Adoption Study. In essential hypertension, a Japanese study is reviewed showing the occurrence of hypertension in the offspring of hypertensive parents. The heterogeneity of essential hypertension is underlined and two of the multiple etiological factors are particularly considered for their genetic component: the response to salt intake and erythrocyte cation fluxes. The conclusion from the literature reviewed is that essential hypertension is a polygenic disease transmitted by polygenic systems.

A population at risk for a certain disease is usually the target population to consider for detection and prevention. Predicting the occurrence of the disease depends of the importance of genes in its transmission, along with the various environmental factors favouring its occurrence. In the case of hypertension, the various epidemiological aspects must be considered which affect normal blood pressure and hypertension, either primary or secondary.

The Montreal Adoption Survey was conducted as a cross-sectional epidemiologic study of cardiovascular risk factors in French Canadian families. Analysis included blood pressure readings of 756 adopted and 445 natural children as well as 1176 parents. A genetic model was applied to the analysis of our data. Interindividual variability of blood pressure was studied and observed correlatives of systolic and diastolic pressure of parental and non parental subjects were calculated. Based on the maximum likelihood estimates presented in the models the explanation of the parent offspring and the between sibs expected population correlatives for systolic blood pressure was the following: 61% was due to shared genes and 39% to environment shared by both parents and children. For diastolic blood pressure the explanation between parents and offspring was the following: 58% was due to shared genes and 42% due to shared environment across generation. The explanation between sibs was estimated to be the following: 33% due to generation, 24% to shared environment across generation and 43% to shared environment within generation.

We report on a 2-year-old child who presented with urinary tract infection and a large solitary bladder calculus. Dietary history indicated an intake of 2 gm. calcium with supplemental vitamin D daily. Urinary amino acid analysis revealed cystinuria. The high dietary intake of calcium and vitamin D may have precipitated early cystine calcium stone formation.

The well known existence of familial clustering of blood pressure among adults raises two important questions: At what age does this aggregation become significant? Are either shared environment or heredity or both the responsible components?

Families with at least one adopted child constitute a useful epidemiological unit in which to study the relative contribution of genes and environment to the familial clustering of blood pressure, already observed in natural children aged 2 to 14 by Zinner's group in Boston.2 Provided that adoption occurs shortly after birth, both adopted and natural children are exposed to the same household living conditions such as dietary salt and calories, exercise habits, and psychological atmosphere, to mention a few.

Of renal biopsies from 300 children with idiopathic nephrotic syndrome (INS), 32 showed histological lesions of focal glomerular sclerosis. From this latter group, 25 had segmental and focal hyalinosis (FSH) with or without mesangial hypercellularity, and 7 focal glomerular obsolescence (FGO). The overall outcome after a mean observation of 7 years (0.5-18) was as follows: 11 patients (34%) were in complete remission, 12 (38%) had persistent proteinuria and/or recurrent nephrotic syndrome, 6 (19%) were in chronic renal failure and 3 patients (9%) died from non renal disease. Patients with FGO had a lower incidence of hematuria, hypertension and tubular defects, a better response to steroid and cyclophosphamide therapy and a better overall outcome. These results are similar to those reported by Habib and Gubler (Ped. Nephrology, Ed. Rubin and Barratt, p. 499-515) and indicate a more favorable outcome than that reported by Cameron et al. (Clin. Nephrol. 6:213, 1978). A progressive decrease in GFR was observed in some patients with persisting proteinuria or recurring nephrosis indicating that ultimate outcome of FGS in children cannot be established from pediatric age population studies.