Recently, involvement of remnant-like particle cholesterol (RLP-C) in atherosclerosis was reported, but this parameter has not been adequately investigated in hemodialysis (HD) patients. The present study investigated the relationship between the RLP-C level and total cholesterol (TC), triglycerides (TG), low density lipoprotein cholesterol (LDL-C), high density lipoprotein cholesterol (HDL-C), lipid peroxides (malone dialdehyde, MDA), apolipoprotein (Apo) A-I, and ApoB. In addition, the fractions of very low density lipoprotein (VLDL), intermediate density lipoprotein (IDL), LDL, and HDL in serum lipoproteins were determined by disk polyacrylamide gel electrophoresis. The relationship between the RLP-C level and three atherogenic indices was also studied. The RLP-C level in HD patients (8.2 ± 6.7 mg/dl) was significantly higher than that in normal controls (2.7 ± 1.3 mg/dl). The RLP-C level showed a significant positive correlation with the levels of TC, TG, LDL-C, MDA, ApoB, VLDL(%, and IDL(%, as well as a negative correlation with HDL(%. However, there was no correlation with age or the duration of HD. RLP-C also showed significant positive correlations with the (TC – HDL-C)/HDL-C ratio and the (VLDL + LDL)/HDL ratio, as well as a negative correlation with the ApoA-I/ApoB ratio. These results suggest that RLP-C may be a potential indicator of atherogenic risk in HD patients.

We report on a 22-year-old female with focal glomerular sclerosis and hyperlipidemia who did not respond to long-term steroid or immunosuppressant therapy. When low-density lipoprotein (LDL) apheresis was performed, her total daily protein excretion decreased, serum albumin increased, total cholesterol decreased from 1,052 mg/dl to 148 mg/dl after 3 months, and the serum lipoprotein(a) level also decreased from 117.8 mg/dl to 9.1 mg/dl. After this therapy, her clinical course was well maintained. By controlling hyperlipidemia, including oxidized low-density lipoprotein and lipoprotein(a), low-density lipoprotein apheresis may produce clinical improvement in focal glomerular sclerosis.

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A 25-year-old man developed renal dysfunction and his family showed autosomal dominant inheritance of endstage renal disease. The proband had trace albuminuria and a slightly raised serum creatinine level. Renal biopsy showed tubulointerstitial nephritis, which was compatible with the nephronophthisis-medullary cystic disease complex (N-MCD). His older brother (aged 31 years) had progressed to endstage renal failure by the age of 23 years after renal biopsy revealed N-MCD when he was 20 years old. Human leukocyte-associated (HLA) antigen typing was performed for the proband, the older brother, the mother (who showed endstage renal failure at the age of 50 years), and the sister (who showed no signs of renal disease at the age of 28 years). The HLA loci A24(9), B62(15), and DR2 were common to all 4 family members, while Cwl was only found in the 3 with renal disease. These findings suggested the possible participation of the Cwl locus in autosomal dominant N-MCD.Clin Exper Nephrol 1997; 1:234-236Key words: nephronophthisis-medullary cystic disease complex, HLA typing, CwlMedullary cystic disease is a hereditary cystic renal disease, and is characterized by the relatively late onset of symptoms and renal dysfunction, as well as by cyst formation at the corticomedullaryjunction in the bilateral kidneys. 1 Most patients with this disease show autosomal dominant inheritance. 2,3 Like familial juvenile nephronophthisis, this is a hereditary cystic renal disease that progresses to endstage renal failure. 4-6 Both diseases are almost indistinguishable on renal biopsy, but can be separated by the pattern of inheritance and by the age of onset. 7 However, because it is widely accepted that these 2 conditions form a single disease entity, s,9 the term "nephronophthisis-medullary cystic disease complex (N-MCD)" is often used. i,l~ We present a family with N-MCD, suggesting the possible participation of the human leukocyte-associated (HLA) antigens.Received Sep. 20, 1996; accepted for publication in revised form May 2, 1997. *Correspondence and requests for reprints to: Second Department of Internal Medicine, Hiroshima University School of Medicine, 1-2-3 Kasumi Minami-ku, Hiroshima 734, Japan. CASE REPORTA 25-year-old man without any history of renal disease showed trace albuminuria in an annual health examination at the age of 22 years, but underwent no follow-up. Five months prior to renal biopsy, slightly high levels of blood urea nitrogen (BUN, 4.26 mmol/L), and serum (s-) creatinine (141 pmol/ L), as well as a slightly low creatinine clearance (60.0 mL/min) were observed. At the time of renal biopsy, he had no polydypsia, polyuria, pallor, lethargy, or hypertension. Urinalysis showed trace albuminuria (< 150 mg/d) without glucosuria or occult blood, and no casts in the sediment. His laboratory data were as foll6ws: BUN, 5.16 mmol/L; s-creatinine, 150 ttmol/ L; s-sodium, 142 mEq/L; s-potassium, 4.7 mEq/L; schloride, 108 mEq/L; s-calcium, 4.6 mEq/L; s-phosphorus, 1.3 mmol/L; urinary (u-) sodium, 148 mEq/d; u-potassium, 59...

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