Alexander Kagan

Occurrence of hereditary nephritis, pretibial epidermolysis bullosa and beta-thalassemia minor in two siblings with end-stage renal disease.

Occurrence of Hereditary Nephritis, Pretibial Epidermolysis bullosa and Beta-Thalassemia minor in Two Siblings with End-Stage Renal Disease A. Alexander Kagan S. Steven Feld J. Juan Chemke Y. Yaacov Bar-Khayim Division of Nephrology, Department of Internal Medicine B, and Clinical Genetics Unit, Kaplan Hospital (affiliated to the Medical School of the Hebrew University and Hadassah, Jerusalem), Rehovot, Israel

Heterogeneity in peritoneal transport during continuous ambulatory peritoneal dialysis and its impact on ultrafiltration, loss of macromolecules and plasma level of proteins, lipids and lipoproteins.

We investigated the effect of heterogeneity in the peritoneal transport of plasma proteins on dialysis efficiency and plasma levels of proteins, lipids and lipoproteins in 32 patients undergoing long-term continuous ambulatory peritoneal dialysis (CAPD; 9 females and 23 males, 18-76 years old). Eleven patients were studied on several occasions (at 0-42 months) and the remainder at 6-60 months on CAPD (n = 49). We have divided our patients arbitrarily into two equal groups according to their protein concentration in the peritoneal effluent at the end of an 8-hour cycle. Patients with a high peritoneal protein concentration (> or = 2 g/l/1.73 m2) have lower ultrafiltration capacity, higher glucose absorption rate and increased loss of most plasma proteins [including albumin, immunoglobulins (Ig), complement components and high-density lipoproteins (HDL)] compared to patients with a low peritoneal protein concentration (< 2 g/l/1.73 m2). Consequently, plasma levels of albumin, IgM and HDL were lower in patients with a high peritoneal protein concentration. The latter had also increased levels of plasma triglycerides and very-low-density lipoproteins. The difference observed in peritoneal transport between the groups could be ascribed only in part to the duration of CAPD treatment, and hence to the number of peritonitis episodes but not to medications. Therefore, we suggest that inherent constitutional factors may be responsible for some of the observed heterogeneity in the peritoneal transport of these patients which is already evident at the start of CAPD treatment. Patients with high peritoneal transport are exposed to an augmented atherogenic plasma lipid profile in addition to a reduction in dialysis efficiency (ultrafiltration failure). These patients may become prone also to nutritional and immunological disturbances. Therefore, we suggest taking these effects into consideration before choosing the appropriate dialysis modality in patients with increased peritoneal transport for plasma proteins.

Role of Peritoneal Loss of Albumin in the Hypoalbuminemia of Continuous Ambulatory Peritoneal Dialysis Patients: Relationship to Peritoneal Transport of Solutes

The effects of peritoneal albumin loss and the consequences of heterogeneous peritoneal solute transport on serum albumin levels were investigated in 25 adult patients on standard continuous ambulatory peritoneal dialysis (0-58 months). The patients were divided into three groups according to their albumin concentrations (g/l/l.73 m2) in 8-hour overnight effluents: group 1 ( < 0.6, n = 5), group 2 (0.6-1.1; n = 14), and group 3 ( > 1.1;n= 6). Significant differences (mean +/- SD) were observed in serum albumin levels (4.4 +/- 0.2, 3.7 +/- 0.3, and 3.1 +/- 0.5 g/dl, respectively) and in net ultrafiltration (0.37 +/- 0.13, 0.19 +/- 0.21, and -0.06 +/- 0.20 liters/8 h/1.73m2, respectively). The serum albumin levels were strongly correlated with 8-hour peritoneal mass transfer, clearance of albumin, 8-hour effluent concentrations of protein and glucose, and ultrafiltration rate. Moreover, the serum albumin levels showed significant negative correlations with dialysate-to-serum ratios of small solutes (urea, creatinine, and uric acid) and macromolecules (IgG, IgA, and IgM) estimated from 8-, 4-, and 1-hour dwell times. In addition, an overnight dialysate glucose-to-protein ratio < 1.0 was highly predictive of low serum albumin levels ( < or = 3.5 g/dl) and poor ultrafiltration. From the results of this study we conclude that peritoneal loss of albumin as well as peritoneal transport of other solutes of wide size (permeability) contribute to the low serum albumin levels during continuous ambulatory peritoneal dialysis, especially in patients with a high peritoneal permeability.

RECOMBINANT HUMAN ERYTHROPOIETIN FOR ANEMIA OF END-STAGE RENAL FAILURE IN BETA THALASSEMIA TRAIT

A. Kagan, MD, Division of Nephrology, Kaplan Hospital, Rehovot, 76100 (Israel) Dear Sir, Recently, Steinberg [1] reported the successful use of recombinant human erythropoietin in the treatment of 2 patients with sickle cell anemia and renal failure. We have used erythropoietin therapy for severe anemia in 2 siblings with ß thalassemia minor and end-stage renal failure, in whom a unique association of hereditary nephritis and ep-idermolysis bullosa was previously reported [2]. The hematological characteristics of the patients and their response to treatment are shown in figure 1. Blood transfusion requirements for 6 months prior to therapy were 14 blood units for R.Y., a 21-year-old male, and 12 blood units for I.Y., his 19-year-old sister. In the first 4 months following initiation of erythropoietin treatment, R.Y. required 7 units and I. Y. 5 units; thereafter, blood transfusions were not needed. No problems with vascular access or increase in blood pressure were observed. Maintenance doses of erythropoietin in 15 other adult patients on dialysis are shown in table 1. These doses in the 2 siblings were higher than those required in the other 6 patients with ß thalassemia trait, probably due to the differences in the ß globin synthesis. ß Thalassemia is characterized by a decreased amount of ß globin as well as typically

Delayed Decubitus Perforation of the Bowel by Long-Term Peritoneal Dialysis Catheter

Delayed perforation of the bowel is a rare complication of long-term peritoneal dialysis (LTPD), carrying high morbidity and mortality. We report a case of delayed perforation of the sigmoid colon by