Spinal epidural lipomatosis in children with renal diseases receiving steroid therapy.

Spinal epidural lipomatosis (SEL) in patients on steroid therapy may be explained by two hypotheses: (1) steroids induce SEL and (2) steroids cause the growth of a pre-existing SEL, especially in obese children. Steroid-induced SEL (SSEL) is rarely described in children, with only six cases reported elsewhere. However, we have already reported four nephrotic children with SSEL, including one child in another hospital, in addition to the six cases. We investigated the frequency of SSEL in 125 children with renal diseases treated with glucocorticoids in a single hospital over 16 years, and examined the risk factors in 62 patients with SSEL reported in the literature. When patients complained of symptoms at the early stage of SSEL, i.e., back pain or numbness, and patients were obese, we performed spinal magnetic resonance imaging (MRI). SSEL was detected in 5 of 125 children (4.0%). Of the 5 patients with SSEL, 3 had vertebral compression fractures, and all 5 patients were on methylprednisolone pulse therapy. Our study suggests that many more patients can be detected at the early stage of SSEL by performing spinal MRI for patients with early symptoms, obesity, and those who have received methylprednisolone pulse therapy.

Effectiveness of high trough levels of cyclosporine for 5 months in a case of steroid-dependent nephrotic syndrome with severe steroid toxicity.

Glucocorticoid treatment for steroid-dependent nephrotic syndrome (NS) is associated with severe adverse effects, such as bone fractures and epidural lipomatosis. Furthermore, a high trough level of cyclosporine (CsA) over an extended period of time is known to induce CsA nephropathy. We present a girl with steroid-dependent NS and steroid-induced vertebral compression fractures and epidural lipomatosis who was treated with a high-dose of prednisolone after experiencing several relapses. A high CsA trough level (between 147 and 225 ng/mL) over a period of only 5 months was effective in improving the vertebral compression fractures, alleviating the epidural lipomatosis by enabling the discontinuation of prednisolone treatment. Thus, high trough levels of CsA over a short period of time may enable prednisolone to be discontinued in cases of steroid-dependent NS without causing any clinical, histological, serum and/or urinary CsA-related adverse effects.

A patient with membranoproliferative glomerulonephritis diagnosed by the third biopsy via endocapillary proliferative glomerulonephritis and focal membranoproliferative glomerulonephritis.

We present a girl with type I membranoproliferative glomerulonephritis (MPGN) diagnosed by the third renal biopsy. The first renal biopsy was performed at age 11.2 years after microscopic hematuria (which was revealed by school urinary screening) had persisted for 3 months, along with a low level of serum C3. Pathological examination of the biopsied specimen revealed endocapillary proliferative glomerulonephritis with multiple humps. The serum C3 level increased to within the normal range 2 months after the first renal biopsy, and the microscopic hematuria disappeared at age 12.3. However, microscopic hematuria, proteinuria, and the low serum complement level reappeared at age 12.8. Pathological examination of a further renal biopsy that was performed at age 13.2 revealed focal MPGN with humps. Prednisolone therapy was subsequently initiated. Fluvastatin was added to her treatment regime when she developed hypercholesterolemia at age 13.6 and was continued even after normal cholesterol levels were reestablished. Pathological examination of the third renal biopsy, which was performed at age 15.2, revealed type I MPGN with humps. Serum C3 normalized 6 months after the cessation of prednisolone at age 15.9. It is clinically important that patients with nontypical acute glomerulonephritis should be observed over a long period and repeated renal biopsies should be performed.