Mineral and bone disorder in chronic kidney disease (CKD-MBD) is a triad of biochemical imbalances of calcium, phosphate, parathyroid hormone and vitamin D, bone abnormalities and soft tissue calcification. Maintaining optimal bone health in children with CKD is important to prevent long-term complications, such as fractures, to optimise growth and possibly also to prevent extra-osseous calcification, especially vascular calcification. In this review, we discuss normal bone mineralisation, the pathophysiology of dysregulated homeostasis leading to mineralisation defects in CKD and its clinical consequences. Bone mineralisation is best assessed on bone histology and histomorphometry, but given the rarity with which this is performed, we present an overview of the tools available to clinicians to assess bone mineral density, including serum biomarkers and imaging such as dual-energy X-ray absorptiometry and peripheral quantitative computed tomography. We discuss key studies that have used these techniques, their advantages and disadvantages in childhood CKD and their relationship to biomarkers and bone histomorphometry. Finally, we present recommendations from relevant guidelines-Kidney Disease Improving Global Outcomes and the International Society of Clinical Densitometry-on the use of imaging, biomarkers and bone biopsy in assessing bone mineral density. Given low-level evidence from most paediatric studies, bone imaging and histology remain largely research tools, and current clinical management is guided by serum calcium, phosphate, PTH, vitamin D and alkaline phosphatase levels only.

A 6 year old boy who presented with steroid unresponsive nephrotic syndrome is reported. He was found to have focal segmental glomerulosclerosis and associated hypoparathyroidism and sensorineural deafness. The child progressed to end stage renal failure and was successfully managed by dialysis and cadaveric renal transplantation. He later developed progressive neurological deterioration and mitochondrial myopathy and neuropathy was diagnosed. (Postgrad Med J 2001;77:523-526) Keywords: mitochondrial myopathy; neuropathy; focal and segmental glomerulosclerosis; renal transplantationThe coexistence of hypoparathyroidism and sensorineural deafness is well recognised. An associated renal anomaly has been reported in some individuals but a review of the literature indicates there is no consistent renal lesion. We report the clinical course of a boy with hypoparathyroidism and sensorineural deafness who was found to have focal and segmental glomerulosclerosis and who was successfully treated by cadaveric renal transplantation. This is the first reported case of long term survival in a child with this constellation of symptoms. Four years after transplant he presented with weakness of limbs and mitochondrial myopathy and neuropathy was diagnosed.Case report A 6 year old white boy was admitted to his local hospital with a two week history of cough, coryzal symptoms, and oedema. On admission he was normotensive and was noted to have generalised oedema, marked proteinuria, hypoalbuminaemia, and hypercholesterolaemia. A diagnosis of nephrotic syndrome was made. Investigations revealed a glomerular filtration rate of 75 ml/min/1.73 m 2 calculated from height and plasma creatinine, C 3 0.90 g/l (normal range 0.75-1.75 g/l), C 4 0.15 g/l (0.14-0.54 g/l), negative antinuclear antibodies and double stranded DNA antibodies, and no evidence of previous hepatitis B or C infection. His nephrotic state did not remit despite oral steroid therapy 2 mg/kg/day for five weeks. He was transferred to this hospital where he was found to be grossly oedematous with ascites and scrotal oedema and he was hypertensive and oliguric. He was started on daily albumin infusions with frusemide because of persisting oliguria and to reduce the severity of the oedema. He remained nephrotic despite daily infusions of methylprednisolone at a dose of 10 mg/kg/day. A percutaneous renal biopsy was undertaken and revealed typical features of advanced focal, segmental, and global glomerular sclerosis (fig 1). A diagnosis of hypoparathyroidism was made after investigation of persistent hypocalcaemia and hyperphosphataemia.Following the histological findings he was maintained on prednisolone 2 mg/kg/day and cyclophosphamide 2.5 mg/kg/day for eight weeks; on completion he was treated with cyclosporin A 5 mg/kg/day. He rapidly progressed to end stage renal failure and was established on continuous cycle overnight peritoneal dialysis. Twenty months after presentation he received a successful cadaveric renal transplant with excellent renal function four ye...

Milk bolus obstruction of the neonatal bowel apparently related to the use of artificial powder milk, particularly full cream cows milk, was first recognised in the late 1960s. The syndrome almost disappeared with the use of highly modified cows milk formulae but has now re-emerged in the low birth weight population with the early use of high calorific feeds specifically designed for these infants. We describe four cases of obstruction and perforation of the bowel seen on our neonatal intensive care unit in 1 calendar year and discuss the possible aetiology of this condition.

HDC HRG criteria identify children who consume significantly greater staff resources. Revision of the definition has resulted in a large reduction of cases meeting the criteria but identifies a group consuming greater staff resources.