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Elevated levels of serum prolactin (PRL) are common and well described in patients with chronic renal failure. We report the case of a 4-year-old girl who also presented with premature thelarche and transient galactorrhea. Neither peritoneal dialysis nor hemodialysis reduced her extremely elevated levels of PRL, which fluctuated from time to time, probably reflecting variations in lactotroph secretion rate. Bilateral nephrectomy (BN) was eventually followed by a progressive and significant rise in PRL levels, suggesting that even uremic kidneys can eliminate PRL through tubular breakdown. Kidney transplantation was responsible for a very abrupt normalization of PRL serum levels, much faster than that observed for creatinine. This confirms animal studies suggesting that elimination of PRL occurs both through glomerular filtration and tubular breakdown. We hypothesized that the seemingly precocious puberty may have resulted from a combination of growth hormone therapy, elevated PRL and a rise in estrogens through the aromatization of adrenal androgens. This case illustrates the impact of dialysis, BN and kidney transplantation on PRL, providing new knowledge on renal PRL metabolism.

As bibliometric indicators are objective, reliable, and cost-effective measures of peer-reviewed research outputs, they are expected to play an increasingly important role in research assessment/management. Recently, a bibliometric approach was developed and integrated within the evaluation framework of research funded by the National Cancer Institute of Canada (NCIC). This approach helped address the following questions that were difficult to answer objectively using alternative methods such as program documentation review and key informant interviews: (a) Has the NCIC peer-review process selected outstanding Canadian scientists in cancer research? (b) Have the NCIC grants contributed to increasing the scientific performance of supported researchers? (c) How do the NCIC-supported researchers compare to their neighbors supported by the U.S. National Cancer Institute? Using the NCIC evaluation as a case study, this article demonstrates the usefulness of bibliometrics to address key evaluation questions and discusses its integration, along complementary indicators (e.g., peer ratings), in a practice-driven research evaluation continuum.

The management and optimal care for the pediatric patient with chronic kidney disease requires attention not only to medical management, but also special focus on the psychosocial and developmental factors of children which is complicated by the presence of other disease-related complications. In recent years, specialized chronic kidney disease and predialysis clinics have been set up to facilitate and improve the quality of care of these patients with a multidisciplinary organisation and coordinated management approaches of a renal team. We present our experience in establishing such a renal management clinic named "Prevoir" for children with chronic kidney disease at Sainte-Justine Hospital.

Le mouvement homéopathique est généralement présenté dans l'historiographie comme un phénomène opposé aux principales transformations du XIXe siècle médical. Pourtant, l'étude du cas québécois fait apparaître un groupe de praticiens dont la stratégie de reconnaissance sociale et le mode de développement sont tout à fait comparables à ceux des médecins de l'école régulière. Les homéopathes se présentent en fait comme porteurs des progrès d'une médecine « moderne », « scientifique » et « professionnelle » et bâtissent des institutions très semblables à celles des médecins réguliers. La persistance de la controverse concernant la validité de la doctrine de Hahnemann et la concurrence entre les deux groupes de praticiens ne doivent pas masquer le fait que, du moins au Québec, le développement de l'homéopathie s'inscrit dans le mouvement plus général de reconnaissance sociale d'une médecine professionnelle., The homeopathic movement is often portrayed by historians as being opposed to the main transformations of nineteenth-century medicine. Nevertheless the Quebec case study shows a group of practitioners whose strategy for social recognition and mode of development were, in fact, similar to those of physicians of the regular school. Indeed, not only did homoepaths present themselves as "modern", "scientific" and "professionnal" representatives of medicine, but also, they built institutions almost identical to those of regular physicians. The persistence of the controversy about the validity of Hahnemann's doctrine and the competition between the two groups of practitioners must not obscure the fact that, at least within Quebec, the development of homeopathy was part of the more general movement toward the social recognition of professional medicine.

Hyperhomocysteinemia, a risk factor for vascular disease, is commonly found in adult patients with end-stage renal disease. Major determinants of elevated plasma homocysteine levels in these patients include deficiencies in folate and vitamin B12, methylenetetrahydrofolate reductase (MTHFR) genotype and renal function. Little information is available for children with chronic renal failure (CRF). The prevalence and the factors that affect plasma homocysteine concentration were determined in children. Twenty-nine children with various degrees of CRF (15 were dialyzed, 14 were not dialyzed) were compared with 57 age- and sex-matched healthy children. Homocysteine concentrations were higher in patients than controls (17.3 micromol/l vs 6.8 micromol/l, P0.0001) and hyperhomocysteinemia (95th percentile for controls: 14.0 micromol/l) was seen in 62.0% of patients and 5.2% of controls. Folate concentrations were lower in patients (9.9 nmol/l) than controls (13.5 nmol/l), P0.01. Vitamin B12 was similar in patients (322 pmol/l) and controls (284 pmol/l). Dialyzed patients have a higher prevalence of hyperhomocysteinemia than nondialyzed patients (87% vs 35%). Dialyzed patients with MTHFR mutation have higher plasma homocysteine (28.5 micromol/l) than nondialyzed patients with the mutation (10.7 micromol/l), P0.002. In our study, differences between controls and patients in plasma homocysteine concentrations are observed when age is greater then 92 months, folate less than 21.6 nmol/l and vitamin B12 less than 522 pmol/l. Our study shows that hyperhomocysteinemia is common in children with CRF and is associated with low folate and normal vitamin B12 status, compared to normal children. Among the patients, the dialyzed patients with the MTHFR mutation are particularly at risk for hyperhomocysteinemia. Further studies are needed to investigate therapeutic interventions and the potential link with vascular complications in these patients.

Background Recent experimental data suggest that the inflammatory response of the host to verotoxin and/or lipopolysaccharides of Escherichia coli is involved in the pathophysiology of verotoxin-producing E. coli (VTEC) infections. Methods We measured the circulating concentrations of cytokines [TNF-alpha, interleukin (IL)-1-beta, IL-1 receptor antagonist (Ra), IL-6, IL-8, IL-10] and soluble leukocyte adhesion molecules (L-selectin, P-selectin, E-selectin, intracellular cell adhesion molecule-1, vascular cell adhesion molecule-1) by sandwich enzyme-linked immunosorbent assay among (1) normal controls (n = 12), (2) disease controls with hemorrhagic colitis (HC) not associated with VTEC infections (n = 57), (3) patients with uncomplicated HC caused by E. coli O157:H7 (n = 30), and (4) children with hemolytic-uremic syndrome (HUS) (n = 28). Patients with HUS were matched with children who presented an uncomplicated HC caused by E. coli O157:H7 for the time interval elapsed between the onset of HC and that of blood sample collection. Results Concentrations of TNF-alpha and IL-1-beta were undetectable. Children with HUS were characterized by increased amounts of IL-6 and IL-8, lower values of soluble L-selectin as well as increased levels of IL-10 and IL-1Ra. The circulating concentrations of IL-1Ra were higher among children with O157:H7 HC who subsequently developed HUS. Conclusions Increased pro- and antiinflammatory cytokine responses are produced by the host during the development of HUS among children with VTEC infections. Further studies are needed to determine their relative contribution to the pathophysiology of classic HUS.

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.

This article describes two sisters with type III Bartter syndrome (BS) due to a novel missense variant of the CLCNKB gene. The phenotypic expression of the disease was very different in these two siblings. In one sister, the disease followed a very severe course, especially in the neonatal period and as a toddler. Both the classic symptoms and the biochemical features of the syndrome were striking. In addition, she presented with sensorineural deafness, a complication yet unreported in this subtype of BS In contrast, the least affected sister was symptom free and the biochemical features of the disease although present remained discrete throughout the prolonged follow-up. It is suggested that such a difference in the phenotypic expression of the disease is possibly secondary to the modifier effect of a gene and/or results from environmental factor(s).

Atypical hemolytic uremic syndrome (aHUS) is a rare, chronic, life-threatening disease due to complement dysregulation. The use of early-onset plasma therapy is recommended, but optimal long-term treatment regimen is not well defined. Eculizumab, a monoclonal humanized anti-C5 antibody, has shown success in patients with aHUS. We report a 7-year-old girl with aHUS associated with factor H mutations successfully treated with eculizumab. Weekly plasma infusion (PI) of 25–30 ml/kg with short-term intensified PI during aHUS exacerbations was effective for 4.3 years. Progressive mild renal failure (stage 2) was attributed to chronic glomerular lesions. Subsequently, she exhibited aHUS exacerbation unresponsive to intensified PI. Eculizumab was initiated at 600 mg, resulting in immediate and complete inhibition of terminal complement activation. During the week following treatment, we observed a complete reversal of aHUS activity. She has been receiving 600 mg eculizumab every 2 weeks for the last 12 months. She had no aHUS exacerbation, and serum creatinine level returned to normal. In this patient, eculizumab led to control of PI-resistant aHUS exacerbation and chronic microangiopathic hemolytic activity. Clinical trials are ongoing to assess the safety and efficacy of this drug in the management of aHUS.

Atypical hemolytic uremic syndrome (aHUS) frequently results in end-stage renal failure and can be lethal. Several studies have established an association between quantitative or qualitative abnormalities in complement factor H and aHUS. Although plasma infusion and exchange are often advocated, guidelines have yet to be established. Long-term outcome for patients under treatment is still unknown. We describe a patient who, at 7 months of age, presented with aHUS associated with combined de novo complement factor H mutations (S1191L and V1197A) on the same allele. Laboratory investigations showed normal levels of complements C4, C3 and factor H. Plasma exchanges and large-dose infusion therapy resulted in a resolution of hemolysis and recovery of renal function. Three recurrences were successfully treated by intensification of the plasma infusion treatment to intervals of 2 or 3 days. This patient showed good response to large doses of plasma infusions and her condition remained stable for 30 months with weekly plasma infusions (30 ml/kg). Long-term tolerance and efficacy of such intensive plasma therapy are still unknown. Reported secondary failure of plasma therapy in factor H deficiency warrants the search for alternative therapeutic approaches.

The case of an infant with both Bartter syndrome and cholelithiasis is presented. We felt that this association of two rare diseases might not be coincidental, thus prompting us to publish this report. Typically, patients affected with Bartter exhibit marked urinary excretion of electrolytes associated with polyuria and, consequently, are prone to develop dehydration [5]. On the other hand, cholelithiasis is a rarely reported condition in infants [4], and several predisposing factors for this condition have been identified such as prematurity, prolonged fasting, parenteral nutrition, use of furosemide, sepsis and dehydration to name a few [3]. This male Caucasian infant was born after 38 weeks of gestation. Presence of severe polyhydramnios had first been noted at 32 weeks of pregnancy. Physical examination of the infant at birth was entirely normal. His serum electrolytes and creatinine were normal during the first two days. Renal ultrasound examination was also unremarkable. On day four it was observed that he had become polyuric and the typical serum electrolyte profile of Bartter had developed. (Serum sodium 135 mmol/L, potassium 2.8 mmol/L, bicarbonate 28.5 mmol/L and chloride 93 mmol/L). Serum creatinine was normal at 32 μmol/L, as well as serum magnesium at 0.83 mmol/L whereas serum calcium was slightly elevated at 2.82 mmol/L. Urinary osmolality remained low at approximately 130–140 mOsm/Kg H2O and urinary electrolyte concentrations done on several samples averaged 30 mm/L for sodium, 20 mm/L for potassium and 40 mm/L for chloride. At the same time, plasma renin activity was extremely elevated at 123 ng/ml per hour (normal 7.1 to 23.8) and plasma aldosterone 954 ng/dl (normal 30 to 201). At six months he was admitted for fever, vomiting and passage of discoloured stools. Presence of a gallbladder stone was clearly visible on ultrasound examination (Fig. 1). Fortunately, the child was able to evacuate his gallstone spontaneously. Presence of risk factors for cholelithiasis such as hemolytic disease and hyperlipidemia were not present. In addition, it is noteworthy that the infant had not been administered third generation cephalosporins and he was not fed by hyperalimention nor in a prolonged fasting state. Fig. 1 Ultrasound examination showing presence of a 4 mm cholelithiasis in the gallbladder neck On follow-up, he proved to be very difficult to manage, requiring enormous electrolyte supplementation in addition to indomethacin and aldactone administration. An adequate state of hydration was obtained through tube feeding by a gastro-jejunostomy. Upon transfer to another medical center in a different city at age 22 months, he was extremely growth retarded at 78 cm (below the third percentile). Bartter syndrome and cholelithiasis in infants are both very rare diseases. An association between these two conditions was sought by retrospectively examining abdominal ultrasounds available in six of our patients with Bartter syndrome but no gallstones were found. The association of these two conditions has never been published before. However, we asked families of patients with Bartter syndrome if they were aware of the simultaneous occurrence of these two diseases on a web site where patients affected with Bartter can exchange information (http://www.health.groups.yahoo.com/group/barttersyndrome/message). Surprisingly, several patients with Bartter replied having had cholelithiasis at a young age. Most striking was the case of a female baby born at term with the neonatal variant of Bartter syndrome, where the diagnosis of cholelithiasis was made at three months of age. This suggested that the association is most probably more frequent than it would be expected if there were no connections between the two conditions. In fact, infants with Bartter typically present with metabolic alkalosis, dehydration and urinary electrolyte losses. It is well established that serum and bile bicarbonate levels are in equilibrium and that alkalinization of bile favours precipitation of calcium carbonate, a key constituent of gallstones [2]. Conversely, systemic acidification induced by acetazolamide has the potential to prevent cholelithiasis formation [6]. Furthermore, dehydration which increases bile viscosity and electrolyte urinary losses such as occurs in pseudohypo-aldosteronism [1] may also promote gallstone formation. Thus factors predisposing to gallstone formation are present in patients with Bartter syndrome and routine ultrasonic screening for gallstones should be performed in these patients who often present bouts of vomiting without obvious explanations.

3 páginas, 4 figuras, 2 apéndices -- PAGS nros. 292-293, Many of the complement regulatory genes within the RCA cluster (1q32) have arisen through genomic duplication and the resulting high degree of sequence identity is likely to predispose to gene conversion events. The highest degree of identity is between the genes for factor H (CFH) and five factor H-related proteins – CFHL1, CFHL2, CFHL3, CFHL4, and CFHL5. CFH mutations are associated with atypical hemolytic uremic syndrome (aHUS). In the Newcastle cohort of 157 aHUS patients we have identified CFH mutations in 25 families or individuals. Eleven of these 25 independent mutations are either c.3226C>G,Q1076E; c.3572C>T,S1191L; c.3590T>C,V1197A or combined c.3572C>T,S1191L/c.3590T>C,V1197A. Sequence analysis shows that all four of these changes could have arisen as a result of gene conversion between CFH and CFHL1. Analysis of parental samples in two patients with S1191L/V1197A has shown that the changes are de novo thus providing conclusive evidence that gene conversion is the mutational mechanism in these two cases. To confirm that S1191L and V1197A are disease predisposing we examined their functional significance in three ways – analysis of the C3b/C3d binding characteristics of recombinant mutant S1191L/V1197A protein, heparin affinity chromatography and haemolytic assays of serum samples from aHUS patients carrying these changes. The results showed that these changes resulted in impaired C3b binding and a defective capacity to control complement activation on cellular surfaces. We, therefore, provide conclusive evidence that gene conversion is responsible for functionally significant CFH mutations in aHUS, Kidney Research UK, the Northern Counties Kidney Research Fund, the Foundation for Children with atypical HUS and the Robin Davies Trust

A small 5 mm renal renin-secreting tumor is described in a child. Suggestions are made to circumvent the difficulties in investigating such cases. It is stressed that the diagnosis is probably often missed especially in the case of very small tumors.

The case of a 13-month-old boy with fibromuscular dysplasia (FMD) presenting with a large saccular aneurysm of the left renal artery and renovascular hypertension is reported. Renal and intrarenal arteries showed numerous small aneurysms alternating with stenoses. All arterial lesions were localized to the left kidney. After left nephrectomy, the patient’s blood pressure normalized. Histopathologic examination of the arteries disclosed changes typical of medial fibroplasias, the most frequently described form of FMD in children. This diagnosis is rewarding as it represents a surgically curable cause of severe hypertension.

Experimental data suggest that the host's inflammatory response is involved in the pathophysiology of verotoxin-producing Escherichia coli (VTEC)-associated hemolytic uremic syndrome (HUS). We compared the circulating levels of pro- [interleukin (IL)-6, IL-8] and anti-inflammatory [IL-10 and IL-1 receptor antagonist (Ra)] mediators on enrollment among children with HUS due to E. coli O157:H7, according to the severity of renal dysfunction. The latter was evaluated by the occurrence of oligoanuria, the requirement for dialysis, and a glomerular filtration rate (GFR)/=80 ml/min per 1. 73 m(2) measured 1 year later. Increased levels of IL-6 (P0.0001), IL-10 (P0.0001), and IL-1Ra (P0.07) were found among patients with HUS compared with normal controls. Children with severe renal dysfunction also had tenfold increased levels of IL-6 and higher concentrations of IL-10 and IL-1Ra. Both the IL-6/IL-10 (4.9+/-8.3 vs. 0.5+/-0.4, P=0.01) and the IL-6/IL-1Ra ratios (0.10+/-0.20 vs. 0. 01+/-0.01, P=0.04) were significantly increased. GFR correlated well with IL-6 levels, IL-6/IL-10 and IL-6/IL-1Ra ratios. Our data demonstrate that the inflammatory response of the host is associated with the severity of renal dysfunction during classic HUS. An imbalance between the pro- and the anti-inflammatory responses may be involved in the pathophysiology of VTEC-associated HUS.

We report a 13-year-old girl with nephropathic cystinosis on chronic peritoneal dialysis who presented with two episodes of stroke. Laboratory evaluation showed severe hyperhomocysteinemia (108 mumol/l). Further testing revealed that she was homozygous for the thermolabile variant of the methylenetetrahydrofolate reductase (MTHFR) gene. Treatment with folic acid and vitamin B12 lowered plasma homocysteine to less than 20 mumol/l. No further episodes of stroke occurred over a follow-up of 12 months. Homocysteine levels should be measured in patients with chronic renal failure, since simple and safe treatment with folic acid and vitamin B12 is effective in lowering the plasma homocysteine level in patients with the thermolabile MTHFR allele.

We describe two infants with bilateral renal artery stenoses and severe hypertension. Adequate control of blood pressure was achieved by medical management. Endoluminal balloon dilatation of the renal arteries had been deferred because of their small size. On follow-up it was noticed that blood pressure had become progressively easier to control, with lesser amounts of antihypertensive medication. In fact, medication could be completely discontinued and blood pressure remained normal. On repeat arteriogram, bilateral renal artery stenoses had almost resolved in both patients. The etiology of the stenoses was not established in these patients. However, regardless of etiology, the transient nature of hypertension and renal artery stenoses in these two cases demonstrates that patient medical management of hypertension in infants can be a valid therapeutic option and occasionally obviate the need for unnecessary risky procedures.

To the Editor. We read with great interest the article by Cabrera et al1 regarding hemolytic uremic syndrome (HUS) associated with invasive Streptococcus pneumoniae infection. The authors carried out a retrospective study of 7 children who had invasive infections attributable to S pneumoniae associated with hemolytic anemia, schistocytosis, thrombocytopenia, and acute renal failure. We are concerned, however, as to how many of these patients definitely presented with HUS. First, it should be recognized that the hallmark of HUS, thrombotic microangiopathy (TMA), was not demonstrated in any of these patients.2 This is in contrast to most other reports in the literature.3–8 Moreover, activation of Thomsen-Friedenreich antigen on red blood cells (positive hemagglutination with Arachis hypogea ) was not demonstrated, although this was usually done in previously reported cases in whom pathologic material was unavailable.9,,10 Secondly, Cabrera et al1 noted that S pneumoniae -associated HUS represented 23% of all HUS cases noted during that 3-year study period. The authors suggested that this high prevalence may have represented the referral patterns for more severe critically ill patients. This remains mainly speculative unless it can be shown that the typical diarrhea-associated HUS cases were more frequently treated in other hospitals. Indeed, the authors paradoxically recognized that “pediatric nephrologists only admitted patients to the 3 participating centers, and it is unlikely that children with HUS would have been admitted to other facilities.”1 On the contrary, Erickson et al11 identified only 12 cases of S pneumoniae -associated HUS, published by 8 different groups, from 1977 to 1989. Similarly, based on our HUS record list, we found only 2 children with S pneumoniae -associated HUS among 268 cases …

In a retrospective review of 241 cases with hemolytic-uremic syndrome, there was evidence of pancreatic involvement in 2 patients, abnormal ultrasounds in 4 patients, and pancreatic injury in 3 of 8 patients undergoing autopsy. Pancreas ultrasound examination, available in 134 cases, was very valuable in identifying those with pancreatic injury.

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.

Eleven uremic children with osteodystrophy aged 3 to 17 years were studied during administration of l, 25-(OH)2D, for periods up to 21 months. Nine children presented with pure hyperparathyroidism, one with osteomalacia and one with mixed bone disease. Bone biopsies were performed before initiation of therapy and after 6 to 21 months of treatment following double tetracycline labeling. Skeletal lesions were improved but not cured in 5 of 9 children with hyperparathyroidism. In three instances lesions remained unchanged and worsened in one. No significant change was observed in the child with osteomalacia. Moderate improvement was noted in the patient with mixed bone disease. The propensity to develop hypercalcemia was the major factor associated with treatment failure since it precluded administration of adequate amounts of medication. Therapy with l, 25-(OH)2D3 was associated with a spectacular improvement in growth velocity in two of six children under age twelve.

Forty canine kidneys were the subject of this pilot study where control groups perfused with Perfudex plus DMSO (1.4 m ), modified Collins' solution with DMSO (1.4 m ) and modified Sacks' solution with DMSO (1.4 m ) showed little toxicity and life-sustaining conservation. In the experimental group, 16 kidneys were frozen for 15 min to −80 °C, thawed by microwave illumination, and reimplanted. Of the 16 dogs, eight survived 2–14 months on their single kidney. The technique of inducing freezing by using intra-arterial cold helium and thawing with high-power microwave illumination gave an overall success rate of 50% long-term life-sustaining survival.

Subacute sclerosing panencephalitis (SSPE) is a progressive neurologic disease associated with chronic measles infection. We have assessed renal function parameters and renal biopsy details in 14 patients with SSPE. All had normal renal function. However, 9 had microscopic hematuria. 9 patients had focal segmental mesangial proliferation and 5 had capillary wall thickening with deposits visible by light microscopy. 13 patients had glomerular C3 deposition and 4 had IgG deposits. SSPE is a disease caused by chronic viral infection, in which nephritogenic complex formation may occur.

10 children underwent cadaveric renal transplantation between the ages of 8.0 and 12.5 years for uremia secondary to infantile cystinosis. 6 children are doing well 6-62 months after-transplantation. 3 of the 4 other recipients required a second graft and eventually died of uremia or fulminant viral encephalitis, the other lost her first graft due to accelerated acute rejection and is now on maintenance hemodialysis. No further systemic complications of cystinosis have been observed in the patients with functioning grafts. Our experience confirms that kidney transplantation is the treatment of choice for uremic children with infantile cystinosis.

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.

Blood flow rates were measured using an isotopic dilution method in the main drainage segments of Brescia-Cimino arteriovenous fistulas in 9 pediatric patients who had undergone successful renal transplantation, 3 patients on hemodialysis and 2 patients with end-stage renal disease. Blood flow rates varied from 68 to 882 ml/min. The possibility that such high flow rates may have deleterious effects on limb growth, cosmetic appearance and cardiac function without any benefit to the patient is discussed.

The purpose of the study was to evaluate the efficacy and the toxicity of levamisole given for 1 year to 16 children suffering from minimal change primary nephrosis who were relapsing frequently. The overall results showed that under levamisole the relapse rate decreased by 55% and the steroid requirements by 50%. After cessation of levamisole administration, most children (11/16) relapsed again. A second course of levamisole treatment reduced the relapse rate significantly once again. At the dosage of 2.5 mg/kg given every other day for 1 year or more, no side effects of levamisole were observed. It is concluded that levamisole does not cure nephrosis but reduces the incidence of relapses for the period it is administered. It also reduces the steroid requirements without any important side effects.

A 38-month-old apparently healthy male has been followed for three years because of a massive glutamic and aspartic aminoaciduria detected shortly after birth in a neonatal screening program. Amino acid clearance studies revealed the presence of renal wastage of dicarboxylic amino acids. Intestinal transport and in vitro oxidation of dicarboxylic amino acids were found to be intact. Clinical and metabolic data obtained on a previously described patient and the present case suggest that some patients with dicarboxylic aminoaciduria might have a selective renal conservation defect without clinical abnormalities, whereas others might demonstrate an additional defect in intestinal transport associated with fasting hypoglycemia.

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.

A soluble preparation of nucleoprotein (sNP), a complex of native deoxyribonucleic acid (DNA) and histones, was isolated from calf thymus nuclei and labeled with [(125)I]iodide. Isotope-labeled antigen ([(125)I]sNP) was used in a primary binding radioimmunoassay method to detect antibodies to both sNP and native DNA. Sera with antibody to native DNA reacted with the DNA moiety of sNP and bound [(125)I] sNP, but this binding was completely inhibited by addition of unlabeled native DNA. Antibody to sNP which reacted with DNA-histone complex was not inhibited in the radioimmunoassay by addition of unlabeled DNA. Thus, antibodies to sNP and native DNA could be detected and differentiated by use of a single isotopically labeled antigen. In systemic lupus erythematosus (SLE), sera with binding to [(125)I]sNP was present in 21/36 (58%) patients. The majority (18/21) had antibodies to sNP and native DNA present simultaneously, one had antibody only to sNP and two had antibody only to DNA. In contrast, patients with other connective tissue diseases rarely showed binding to [(125)I]sNP. Serial studies on SLE patients showed that high serum binding to [(125)I]sNP paralleled renal disease activity as reflected by the degree of proteinuria. A fall in binding was observed with subsidence of renal disease and reappearance of increased binding coincided with exacerbation. In these patients, antibodies to sNP and DNA appeared or disappeared pari passu suggesting that in addition to the previously demonstrated role of antibody to native DNA, antibody to sNP might also be implicated in the pathogenesis of immunologically-mediated tissue lesions such as SLE nephritis.

Soluble nucleoprotein (sNP) antigen was extracted from calf thymus nuclei and labeled with 125iodide. The labeled antigen (125I-sNP) was used in a sensitive radioimmunoassay to detect either sNP antigen or sNP antibody. Synovial fluids from 54 patients with various rheumatic diseases were examined by this technique. sNP antigen was detected in 18 of 31 (58%) in rheumatoid arthritis (RA) patients and 14 of 23 (61%) in non-RA patients. The important finding was that 8 of 31 (26%) RA patients' synovial fluids contained antibody whereas with only one exception, antibody could not be detected in 23 non-RA patients. Presence of free sNP antigen or homologous antibody in synovial fluids of RA patients suggests that at certain times immune complexes might form and contribute to synovial injury.

Infusion of ketone bodies to ammonium chloride-loaded acidotic dogs was found to induce significant reduction in urinary excretion of ammonia. This effect could not be attributed to urinary pH variations. Total ammonia production by the left kidney was measured in 25 animals infused during 90 min with the sodium salt of D,L-beta-hydroxybutyric acid adjusted to pH 6.0 or 4.2. Ketonemia averaged 4.5 mM/liter. In all experiments the ammonia content of both urine and renal venous blood fell markedly so that ammoniogenesis was depressed by 60% or more within 60 min after the onset of infusion. Administration of equimolar quantities of sodium acetoacetate adjusted to pH 6.0 resulted in a 50% decrease in renal ammonia production. Infusion of ketone bodies adjusted to pH 6.0 is usually accompanied by a small increase in extracellular bicarbonate (3.7 mM/liter). However infusion of D,L-sodium lactate or sodium bicarbonate in amounts sufficient to induce a similar rise in plasma bicarbonate resulted in only a slight decrement in ammonia production (15%). The continuous infusion of 5% mannitol alone during 90-150 min failed to influence renal ammoniogenesis. Infusion of pure sodium-free beta-hydroxybutyric acid prepared by ion exchange (pH 2.2) resulted in a 50% decrease in renal ammoniogenesis in spite of the fact that both urinary pH and plasma bicarbonate fell significantly. During all experiments where ketones were infused, the renal extraction of glutamine became negligible as the renal glutamine arteriovenous difference was abolished. Renal hemodynamics did not vary significantly. Infusion of beta-hydroxybutyrate into the left renal artery resulted in a rapid decrease in ammoniogenesis by the perfused kidney. The present study indicates that ketone bodies exert their inhibitory influence within the renal tubular cell. Since their effect is independent of urinary or systemic acid-base changes, it is suggested that they depress renal ammoniogenesis by preventing the transformation of glutamine and glutamate into alpha-ketoglutarate in the mitochondria of the renal tubular cell.

Henoch-Schonlein’s purpura is a systemic vasculitis involving mainly skin, joints, intestine and kidney. We report on 2 patients who presented with clinical signs and symptoms suggestive of testicular torsion. In 1 case purpuric areas after leg cast removal indicated a diagnosis of Henoch-Schonlein’s purpura. Surgical exploration of the other case revealed congestion of the hydatid of Morgagni, which was followed by typical systemic manifestations of Henoch-Schonlein’s purpura. These and another case indicate that orchitis accompanying Henoch-Schonlein’s purpura may mimic testicular torsion.

Polycystic kidneys may rarely be a feature of the tuberous sclerosis complex in adults. A 3 month old female presented at the emergency room with a 2 day history of diarrhoae and vomiting. Physical examination revealed bilateral renal masses which by pyelography and ultrasonography were polycystic kidneys. The mother was noted to have adenomata sebacium on her forehead. At age 5 months the patient developed myoclonic seizures. Computerized tomography demonstrated the presence of paraventricular calcifications. Over the following 2 years other features of tuberous sclerosis have been observed. A 3 month old male presented for routine immunization and was noted to have bilateral renal masses. At age 5 months he developed myeloclonic seizure activity and computerized tomography demonstrated paraventricular calcifications. A biopsy of renal cystic lesions was typical of that described for renal cysts of tuberous sclerosis. Since that time both children have had progressive mental deterioration and have been extremely hypertensive though renal function have remained satisfactory. The cases serve to illustrate that though not a previously recognized phenomenon tuberous sclerosis can present as renal polycystic disease in infancy and indicates that care should be taken to outrule the presence of this entity when genetic advice is sought in cases of polycystic renal disease in infancy.

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.