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Biomedical subjects

A Drukker

Publications and source records attributed to A Drukker.

At least 91 records · Page 5Linked to original sources

Hypertrophic cardiomyopathy in children with end-stage renal disease and hypertension.

Cardiac function was evaluated by echocardiography (echo) in ten paediatric patients, 2.5 to 15 years of age, maintained on chronic dialysis. All had moderate to severe hypertension despite intensive antihypertensive medication. Four of ten patients showed, on echo, asymmetric septal hypertrophy (ASH) typical of hypertrophic cardiomyopathy (HCM). Two also had signs suggestive of outflow obstruction. Primary cardiomyopathy was excluded by a family survey of first-degree relatives. The incidence of HCM in our series is impressive (40%). The pathogenesis of this cardiac lesion in uraemia is unknown; long-standing severe hypertension may play a role. Early recognition of this form of cardiomyopathy has important clinical considerations. Echo should become a routine examination in the diagnosis, follow-up, and treatment of children with end-stage renal disease.

Adolescent↗

Orthotopic renal autotransplantation in the treatment of renovascular hypertension.

Severe hypertension non-responsive to medical therapy was discovered in a 27-year-old woman in the third trimester of pregnancy. Renal arteriography after delivery revealed bilateral renal artery stenosis. The right kidney was smaller than the left one. The differential renal function studies, rather than the renal vein renin measurements, identified which side was causing the hypertension. An attempt at primary repair of the right renal artery failed. Orthotopic renal autotransplantation, using saphenous vein grafts that were interposed between the renal veins and the inferior vena cava, and between the segmental renal arteries and the abdominal aorta, reduced the blood pressure to normal values.

Adult↗

The renin angiotensin system in newborn dogs: developmental patterns and response to acute saline loading.

Plasma renin (PRC) and aldosterone concentrations are known to be high during early postnatal life. Whether this is related to the low rates of renal blood flow or to sodium homeostasis remains unknown. Measurement of PRC, renal blood flow, and its intrarenal distribution were performed in 1- to 3-wk-old puppies subjected to maneuvers known to stimulate or inhibit renin release. In the awake state, PRC was observed to be higher in 2-wk-old puppies than in older or younger dogs, (P less than 0.0001). Significant differences in PRC were also found between litters (P less than 0.0001), but they did not account for the age-related changes. Anesthesia resulted in a 3- to 5-fold rise in PRC, whereas saline expansion suppressed PRC at all ages, the fall tending to become progressively greater with age (P less than 0.09). There was no significant correlation between the age-related changes in PRC and those in renal blood flow or its intrarenal distribution. The results of these experiments demonstrate that in the newborn from a qualitative point of view, PRC changes appropriately in response to various stimuli. However, quantitative age-related differences exist in this regard, reflecting an initial immaturity of the feedback system.

Age Factors↗

Hemodynamic and excretory response of the neonatal canine kidney to acute volume expansion.

It is generally recognized that developing animals retain sodium due to an enhanced reabsorption in distal tubule segements, even when the amount administered is in excess of their needs. This study was designed to test the relationship between this relative inability to dispose of a saline load and the functional characteristics of the kidney during postnatal maturation. In addition, we explored the role played by some of the factors known to affect natriuresis in the adult subject. Measurements of sodium excretion, glomerular filtration rate (GFR), and renal blood flow (RBF) and its intrarenal distribution were made in three age groups of puppies and in adult dogs. During expansion the GFR rose rapidly and to a similar extent at all ages, but it fell thereafter, the rate of decline being much slower in adult than in developing animals (P less than 0.001). RBF and its intrarenal distribution were not altered by volume expansion. The degree of natriuresis did not reflect either the age-related or the expansion-induced changes in GFR. Fractional and absolute sodium excretion were substantially higher in 2-wk-old puppies than in either 1- or 3-wk-old animals (P less than 0.002). These findings demonstrate that the blunted renal response of the maturing animal to saline loading is due to the persistence of an enhanced tubular reabsorption rather than to a limitation in glomerular filtration.

Animals↗

Recurrent hemolytic-uremic syndrome: a case report.

A girl who developed HUS at 2 years of age had four further episodes of the disease during the next 2 1/2 years. No renal or hematologic abnormalities were detected during or between the attacks. Reduced levels of serum complement were found during three of the episodes.

Child, Preschool↗

Acute osteolytic lesions following pancreatitis in a dialysis patient.

A 42-year-old man undergoing maintenance hemodialysis suffered an attack of acute pancreatitis. Convential treatment resulted in quick recovery. 10 days after its onset his hands and feet became swollen, hot, red and painful. Multiple intramedullary osteolytic lesions of the metatarsals, metacarpals and phalanges, with cortical destruction and a number of fractures were found. These lesions subsided over many weeks and did not recur. We believe that such acute osteolytic lesions following pancreatitis are not 'renal osteodystrophy' as such, but should be recognized as a possible complication in renal failure patients.

Acute Disease↗

Hematopoietic progenitors in children with end-stage renal disease.

Bone marrow and circulating erythroid progenitors (BFU-E) in six anemic children with end-stage renal disease (ESRD) were 2.0 and 1.9 times as abundant, respectively, as in six age-matched normal controls and were significantly more responsive in vitro to low concentrations of recombinant human erythropoietin (rHuEpo) than those from the controls. After 4 weeks of rHuEpo therapy, both the number and the in vitro rHuEpo response of circulating BFU-E in the ESRD patients returned to normal control values. The numbers of bone marrow and circulating granulocyte-monocyte progenitors in the ESRD patients before and after rHuEpo therapy were comparable to those of normal controls. There was no inhibition of in vitro erythropoiesis by either the patients' serum or medium conditioned by their mononuclear cells. These results demonstrate a significant abundance and an increased rHuEpo sensitivity of BFU-E in anemic children with ESRD with no evidence of the presence of uremic inhibitors to erythropoiesis.

Adolescent↗

Retinal lesions in Alport's syndrome.

We described a family in which two siblings were suffering from Alport's syndrome. Both had identical ocular lesions: whitish-grey dots in the superficial layers of the retina mainly in the posterior pole. The visual acuity, the visual field, and the fundus fluorescein angiogram were normal. These lesions were associated with an impaired ERG. The ocular finding seems to be characteristic of the Alport's syndrome.

Adolescent↗

Recurrence of oxalate deposition in a renal transplant during ciclosporin A therapy.

A 14.5-year-old boy with end-stage renal failure due to familial primary hyperoxaluria underwent cadaver donor renal transplantation. The graft function was stable for a period of 22 months, while he was on conventional immunosuppressive therapy (prednisolone and azathioprine) and additional oral pyridoxine, phosphorus, and magnesium supplementation. When ciclosporin A was introduced instead of azathioprine, the blood levels of oxalate rose, and oxalate deposition in the renal tubuli became evident. These observations suggest that ciclosporin A interferes with oxalate metabolism and, therefore, should be given with utmost caution in patients with primary hyperoxaluria.

Adolescent↗

Plasma vitamin E levels in uremic children and adolescents.

We studied plasma vitamin E levels in children/adolescents 3-19 years of age (10.9 +/- 4.5; mean +/- 1 SD) with chronic renal failure treated conservatively, on hemodialysis, on continuous ambulatory peritoneal dialysis and after renal transplantation. In all 4 groups of patients vitamin E levels (0.66-0.90 mg/dl) were within the normal range (0.76 +/- 0.19 mg/dl). The lowest levels of vitamin E (0.59 +/- 0.1 mg/dl) were found in nonuremic patients with a good functioning renal transplant. These data do not support the notion that uremia is accompanied by vitamin E malabsorption.

Adolescent↗