No abstract

No abstract

Eighty-six patients (59 males and 27 females) diagnosed with steroid-responsive nephrotic syndrome during childhood were identified. The patients were 20–40 years of age (mean 27.0 ± 5.0) with a mean follow-up period of 19.5 ± 5.9 years. All patients had been treated with a long-term tapering corticosteroid therapy. Thirty patients had also received a course of cyclophosphamide (2 mg/kg/day for 12 weeks). Sixty-six had achieved sustained remission off corticosteroids, while 20 were still receiving corticosteroids to maintain remission. None of the 86 patients had proteinuria or renal insufficiency at the time of the study. Mean final heights in males and females were similar (–0.51 ± 1.21 and –0.23 ± 1.16 standard deviation score). Mean final height of 20 steroid-dependent patients was significantly less than that of 66 in remission off corticosteroids (p < 0.005). Ten cyclophosphamide-treated patients got married and 9 had at least 1 healthy child. In children with steroid-responsive nephrotic syndrome, the need for corticosteroid therapy to maintain remission may be associated with decreased adult height. Patients who received a 12-week course of cyclophosphamide are likely to be normally fertile as adults.

Sirs, Compression of the left renal vein (LRV) between the aorta and the superior mesenteric artery (SMA), known as the nutcracker phenomenon (NCP), can cause gross or microscopic hematuria, flank pain, proteinuria, or a combination of these clinical features [1, 2, 3]. The phenomenon causes hypertension of the LRV, consequently causing LRV compression, left gonadal vein varices, and unilateral hematuria [2,4]. A recent report documented orthostatic proteinuria associated with the NCP [1, 5, 6, 7]. We describe two siblings with microscopic hematuria caused by NCP. These patients were a 3-year-old brother and a 5-year-old sister born to healthy non-consanguineous parents. Their family history manifested no renal diseases. Microscopic hematuria was first indicated in both patients by an annual screening urinalysis at their kindergarten. Their respective blood chemistries and urinary calcium/creatinine ratios were normal. Urine was normal except for sediment containing 5-20 red cells per high-power field. Repeated urine cultures showed no pathological organisms. Urinary red cell morphology revealed predominantly (>90%) isomorphic cells. Serum complement, IgA, IgG, and IgM concentrations, as well as antistreptolysin O titer, antinuclear antibody, antidouble-stranded DNA antibody, and rheumatoid factor were in the normal range. Ultrasonography of the kidneys showed marked dilatation of the LRV in the hilar portion and severe compression of the LRV between the aorta and the SMA, which was an indirect finding that is typical of NCP.

No abstract