Changes in bone turnover markers after discontinuing long-term glucocorticoid administration in children with idiopathic nephrotic syndrome: a multicenter retrospective observational study.

BACKGROUND: Glucocorticoids affect bone turnover. Little is known about how bone turnover changes when glucocorticoids are discontinued following long-term administration. METHODS: This retrospective observational study was conducted on the relationship between discontinuation of long-term administration of glucocorticoid and bone turnover markers (BTMs) in patients with childhood-onset idiopathic nephrotic syndrome. Serum bone alkaline phosphatase (BAP), intact procollagen type 1 N-terminal propeptide (P1NP), and tartrate-resistant acid phosphatase-5b (TRACP-5b) were evaluated as BTMs. RESULTS: Thirty-eight pairs of BTMs at glucocorticoid administration and after discontinuation were analyzed in 29 patients. The median age at baseline was 12.4 (interquartile range, 9.0-14.5) years, and the median time from the onset of nephrotic syndrome was 5.9 (3.3-9.7) years. The mean period from prednisolone discontinuation to the measurement of BTMs after glucocorticoid discontinuation was 3.5 ± 1.0 months. Changes in BTMs after glucocorticoid discontinuation were modest when the daily prednisolone dose was < 0.25 mg/kg/day (ln BAP standard deviation [SD] score, p = 0.19; log intact P1NP SD score, p = 0.70; TRACP-5b, p = 0.95). When the daily prednisolone dose was ≥ 0.25 mg/kg/day, all BTMs increased significantly after glucocorticoid discontinuation (ln BAP SD score, p < 0.01; log intact P1NP SD score, p < 0.01; TRACP-5b, p < 0.01). CONCLUSIONS: Decreased BTMs can rise within a few months of discontinuing long-term glucocorticoid administration. When the administered glucocorticoid dose is low, changes in BTMs may be small. A higher resolution version of the Graphical abstract is available as Supplementary information.

Growth hormone therapy for a patient with idiopathic Fanconi syndrome and growth hormone deficiency.

Idiopathic Fanconi syndrome (FS) is characterized by a generalized dysfunction of the renal proximal tubules. Patients with FS often exhibit growth retardation due to complex factors, such as hypophosphatemia, metabolic acidosis, disturbed vitamin D metabolism and hypokalemia. To date, one FS patient has been reported to exhibit growth failure due to growth hormone deficiency (GHD), but the long-term clinical course of recombinant human GH (rhGH) therapy has not been reported. At 10 months of age, the patient was admitted to our hospital due to growth failure. Blood and urinary biochemical abnormalities, such as hypophosphatemia, metabolic acidosis, glycosuria and low-molecular-weight proteinuria, indicated a generalized dysfunction of the renal proximal tubules. The presence of cystinosis, collagen diseases, toxic agents and metabolic diseases were excluded. These features are compatible with idiopathic FS. Treatment with high-dose alkali, potassium citrate, phosphate buffer, hydrochlorothiazide and vitamin D supplement was initiated. The biochemical abnormalities achieved nearly normal values, and the patient's height was within -2.5 SD at the age of 2 years. However, his height did not continue to increase at the same rate and gradually declined to -2.9 SD at 4 years of age. GH stimulation test demonstrated GHD. After initiation of rhGH therapy, his height improved to -2.0 SD at the age of 9 years with no adverse effects. In conclusion, we report the case of a patient with FS and GHD who continued rhGH therapy for 5 years. The differential diagnosis of GHD should also be considered for FS patients with short stature.

[Optimization of exposure conditions for amorphous selenium direct conversion DR-based mammography system].

A new direct-conversion detector for DR mammography has improved the detectability of microcalcifications and masses. Each optimized exposure condition (target/filter combination and tube voltage) was defined through comparison of physical values and visual evaluation on breast specimens using the innovative DR mammography. The contrast-to-noise-ratios (CNRs) of PMMA phantoms of various thicknesses were obtained under a variety of exposure conditions whose average glandular doses (AGDs) were made consistent. Fifty breast specimens were irradiated under these combinations. Visual evaluation was conducted on the images, whose histograms were controlled for consistency. In the phantoms with thicknesses of 20 mm or more, tungsten/rhodium had the highest CNRs of the targets/filters such as molybdenum/molybdenum and molybdenum/rhodium. For visualizing microcalcifications and masses on breast specimens of thicknesses of 35 mm and below, molybdenum/molybdenum was the best. Nevertheless, to obtain better image quality, molybdenum/rhodium was superior for 35-55 mm thickness, and tungsten/rhodium was superior for 55 mm and above under the same AGD, enabling accurate and efficient diagnosis. The study showed that the exposure conditions differ for obtaining the highest CNR using phantoms and those under which breast specimen images allow the most accurate and efficient diagnosis. In addition, image evaluations of the breast specimens allowed optimization of exposure conditions that are closer to those of the actual diagnosis using mammography.