Alfred Drukker

Acute renal failure in a burn patient: the advantages of continuous peritoneal dialysis

An 8-year-old girl sustained 65-70 per cent body surface area burns when her clothes caught fire. Two weeks later, she developed acute renal failure (ARF) and needed dialysis therapy. The extensive, infected burn area prevented the establishment of a safe vascular access for haemodialysis. Continuous peritoneal dialysis proved to be a valid alternative form of treatment and even had advantages over haemodialysis. Large amounts of fluids, calories and electrolytes could be administered via the peritoneal route. This facilitated the treatment of the catabolic state of the uraemic burn patient and served to correct the electrolyte losses via the skin. Peritoneal dialysis is a valuable adjunct to the treatment of acute renal failure in burn patients who need renal replacement therapy.

Hyperhomocysteinemia in children on renal replacement therapy

Abstract. Hyperhomocysteinemia is an independent risk factor for the development of atherosclerosis in adult patients on dialysis or after kidney transplantation. There are few data on homocysteine (Hcy) concentrations in children under these circumstances. The aim of our study was to evaluate plasma Hcy levels and their determining factors in children on renal replacement therapy. In 29 children and adolescents on chronic dialysis therapy and in 34 children after renal transplantation (Tx) fasting total plasma Hcy, red blood cell (RBC) folate, and serum vitamin B12 levels were measured. The plasma Hcy levels were expressed as number of standard deviations (SD) from mean level in age- and gender-matched controls. In dialysis patients the mean plasma Hcy level was elevated (4.4±0.8xa0SDs), without significant difference between patients on hemodialysis or continuous cycling peritoneal dialysis. In the dialysis patients a negative correlation (r=–0.49) between plasma Hcy levels and RBC folate concentrations was found. Oral folate supplementation was given to 8 of 21 dialysis patients, resulting in high RBC folate levels (>800xa0µg/ml) and normalization of the plasma Hcy levels (0.4±0.5xa0SDs). In Tx patients the mean plasma Hcy level was 5.6±1.4xa0SDs. Multivariate regression analysis revealed that the main factor determining Hcy level after kidney Tx was creatinine clearance. Patients with normal kidney function had a mean Hcy concentration of 1.69±0.86 compared with 10.0±2.2 in children with decreased function. Folate and cyclosporine levels had less significant effects on Hcy concentrations. Seven patients who were evaluated while on dialysis and after a successful kidney Tx demonstrated a significant reduction in Hcy levels. Children and adolescents on dialysis therapy and with impaired renal function after renal Tx have significant hyperhomocysteinemia. Oral folate supplementation normalizes the increased plasma Hcy levels and should be added to the medical treatment of all children with impaired renal function.

Impaired intestinal fat absorption in chronic renal failure.

We performed oral fat loading tests in 10 patients with chronic renal failure (CRF) on hemodialysis (5 children and 5 adults). Fat absorption was measured by hourly determination of serum triglycerides (TG), cholesterol (CHOL), and lipoproteins (LP) after oral administration of a milkshake containing 50 g of fat of dairy origin. 10 age-matched healthy volunteers with normal fasting serum TG levels and 10 subjects with fasting hypertriglyceridemia served as controls. Mean fasting serum TG levels in CRF patients were elevated compared to normal controls (177.6 +/- 14.6 mg/dl, 2.0 +/- 17 mmol/l vs. 91.0 +/- 10.5 mg/dl, 1.03 +/- 12 mmol/l). 6 patients (4 adults, 2 children) had type IV LP patterns and 2 patients (both children) showed type IIb hyperlipidemia. In only 2 patients, 1 child and 1 adult were TG, CHOL and LP electrophoresis all normal. The oral fat loading test in all CRF patients showed delayed appearance of TG and chylomicrons (CHYL) in the bloodstream i.c. impaired or slow absorption of fat from the gut. In contrast to normal and hypertriglyceridemic controls, TG and CHYL levels in CRF did not decrease by 5 h after the oral fat load. This study demonstrates impaired intestinal fat absorption in children and adults with CRF.

Hypothyroidism in primary hyperoxaluria type 1

We describe 4 patients, aged 3 months to 23 years, with end-stage renal disease and severe, symptomatic hypothyroidism. All 4 had primary hyperoxaluria type 1 (PH1) with diffuse tissue (kidneys, skeleton, eyes, heart) calcium-oxalate deposition, a condition known as oxalosis. The hypothyroidism responded to thyroid hormone replacement therapy. Clinical hypothyroidism within the framework of PH1/oxalosis was probably caused by thyroid tissue damage from an abundance of calcium oxalate. We recommend that thyroid function be monitored in patients with PH1 and oxalosis.

Dialysis-induced hypoxemia: continuous monitoring of blood oxygen and carbon dioxide tension in children

We report our experience with continuous, transcutaneous monitoring of capillary oxygen and carbon dioxide tension (PtcO2, PTlcCO2 ) during the entire length of hemodialysis treatment in 8 pediatric patients. The relative changes in PtcO2 and PtcCO2 observed with this method were in accordance with those reported in the literature, obtained with frequent arterial blood sampling. There was a temporary fall of PtcO2, averaging 26.2% after 2h of dialysis, with a concomitant rise of PtcCO2 of 9.9%. The noninvasive, transcutaneous measurement of PtcO2 and PtcCO2 is a reliable and valuable adjunct for the monitoring of the dialysis patient who is prone to develop hypoxemia. It is particularly useful in the very young.

Novel HGPRT 293 A>G point mutation presenting as neonatal acute renal failure

We report on a rare case of hypoxanthine guanine phosphoribosyl transferase (HGPRT) deficiency that presented in the newborn period with acute renal failure (ARF). The clinical diagnosis was made on the basis of non-oliguric ARF and evidence of crystal nephropathy on renal biopsy. HGPRT deficiency was eventually confirmed by enzymatic and genetic testing, showing a novel point mutation, 293 A>G. Immediate treatment consisted of peritoneal dialysis with, initially, lactate- then bicarbonate-buffered 1.36% glucose solution together with oral administration of allopurinol. Follow-up after more than 4xa0years continued to show hyper-echogenic kidneys with almost normal renal glomerular function. There continues to be no neurobehavioural abnormalities.

Lipid Metabolism in Chronic Renal Failure

Disturbances of lipid metabolism in chronic renal disease have been known since the first half of the 19th century. In 1836, Bright(1) noticed that the serum of some patients with ‘nephritis’ appeared ‘milky and opaque’. More than 75 years later, in 1913, Munck(2) first described the double refractile lipid bodies in the urine of patients suffering from ‘lipoid nephrosis’.

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.

Meeting of the European Working Group on Psychosocial Aspects of Children with Chronic Renal Failure, Jerusalem, Israel, 8-12 November 1987.

The European Working Group on Psychosocial Aspects of Children with Chronic Renal Failure was established in 1979 by Barbara Korsch (Los Angeles) and Ginette Raimbault (Paris) and other workers in the behavioural sciences connected with units treating children with end-stage renal disease (ESRD). The composition of the working group meetings is unique in that the entire spectrum of the paediatric renal team is represented: nurses, dieticians, play-therapists, teachers, social workers, paediatric psychologists/psychiatrists and paediatric nephrologists. The 1987 meeting in Jerusalem was attended by some 70 participants from England, Holland, Federal Republic of Germany, Switzerland and Israel. Unfortunately, few paediatric nephrologists were present. More than 25 lectures were given in plenary sessions and simultaneous workshops which obviously cannot be summarized in detail here; special emphasis is given here to the material presented in the workshops. In the opening lecture Kaplan-Dinur (Jerusalem), a pioneer of adult psychonephrology, stressed that in the last decade the quality of life of (adult) ESRD patients has not improved significantly. The major problem for ESRD patients on renal replacement therapy (RRT) remains their levels of anxiety, which are far greater than in any other group of patients with chronic disease, including oncology patients. One of the reasons for this phenomenon is the fact that denial is very difficult if at all possible in patients on RRT. To improve the overall rehabilitation of ESRD patients the many personal, family and environmental factors contributing to the continuous maladjustment of these patients have to be taken into account. More objective hard psychosocial data are urgently needed to achieve that goal.

Xanthogranulomatous pyelonephritis in a child: Preoperative X-ray diagnosis

A 5-year-old boy with xanthogranulomatous pyelonephritis (XGP) is reported. This case is unusual since retrograde pyelography established the diagnosis preoperatively.