The implications of complexity, systems thinking and philosophy for pediatricians.

National service systems in child healthcare are characterized by diversity and complexity. Primary, secondary, tertiary and quaternary healthcare services create complex networks covering pediatric subspecialties, psychology, sociology, economics and politics. Can pediatrics exist without philosophy? Does the absence of integrating philosophical perspectives during conceptualization of pediatric care contribute to deficiencies in the service systems structuring child healthcare? Philosophy offers new ways of complex systems thinking in scientific and clinical pediatrics. Philosophy could improve coping strategies on different levels when dealing with ethics of research projects, individual child healthcare and crises of healthcare service systems. Boundary and ultimate situations experienced by severely sick children require help, hope and resilience. Patients and families as well as pediatricians and other caregivers must act in concert. All of them may benefit from consulting with philosophers. The aim of this article is to point out the risks of a strict separation of scientific insight and sensory experience affecting child healthcare in our modern society, which is dominated by technology, competition and lack of equity and time.

Late diagnosis of primary hyperoxaluria after failed kidney transplantation.

Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive inborn error of the glyoxylate metabolism that is based on absence, deficiency or mislocalization of the liver-specific peroxisomal enzyme alanine:glyoxylate aminotransferase. Hyperoxaluria leads to recurrent formation of calculi and/or nephrocalcinosis and often early end-stage renal disease (ESRD) accompanied by systemic calcium oxalate crystal deposition. In this report, we describe an adult female patient with only one stone passage before development of ESRD. With unknown diagnosis of PH, the patient received an isolated kidney graft and developed an early onset of graft failure. Although initially presumed as an acute rejection, the biopsy revealed calcium oxalate crystals, which then raised a suspicion of primary hyperoxaluria. The diagnosis was later confirmed by hyperoxaluria, elevated plasma oxalate levels and mutation of the AGXT gene, showing the patient to be compound heterozygous for the c.33_34InsC and c.508G > A mutations. Plasma oxalate levels did not decrease after high-dose pyridoxine treatment. Based on this case report, we would recommend in all patients even with a minor history of nephrolithiasis but progression to chronic renal failure to exclude primary hyperoxaluria before isolated kidney transplantation is considered.

Management of renal osteodystrophy in children.

Prevention and treatment of renal osteodystrophy (ROD) are great challenges for pediatric nephrologists. The strategies for prevention and treatment of ROD in children with chronic renal failure (CRF) should be created on an individual basis. The following factors should be considered: age, type of primary disease, rate of progression of CRF, nutrition, acidosis, type of dialysis, and drugs (corticosteroids, growth hormone, etc). The treatment should start very early in the course of renal insufficiency with close monitoring of serum calcium, phosphate, alkaline phosphatase and parathormone (PTH) levels. Maintenance of serum phosphate within age- appropriate limits is essential for prevention of secondary hyperparathyroidism. PTH levels should be kept within normal limits in predialysis children and 2-3 times over upper normal limit in those on dialysis. Aggressive treatment with calcium-based phosphate binders and vitamin D derivates should be avoided to prevent PTH oversuppression and development of adynamic bone disease. The advantage in this respect is the development of calcium- and aluminum-free phosphate binders, of which there is limited pediatric experience with sevelamer hydrochloride. Paricalcitol is a non-hypercalcemic vitamin D analogue, and preliminary favorable experience has been reported in children. Calcimimetics like cinacalcet hydrochloride, which directly stimulate calcium sensing receptor and potently suppress PTH secretion without increasing plasma calcium in adults, are very promising agents, but pediatric experience is lacking.