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Culver City, CA, United States

Wilne S.,SteelHouse
Archives of disease in childhood. Education and practice edition | Year: 2010

Only 2% of childhood tumours occur in the spine and spinal cord; yet these tumours account for a disproportionate degree of morbidity in children with cancer. Spine and spinal cord tumours frequently initially present with non-specific symptoms such as back pain and clumsiness and are therefore often associated with a prolonged period between symptom onset and diagnosis. Many children present repeatedly to healthcare services before a diagnosis is made. and appropriate imaging is often only instigated once a child has developed neurological deficits. Unfortunately, despite treatment, these deficits are often only at best partially reversible. This article reviews the pathology and presentation of spine and spinal cord tumours in children and advises on the appropriate assessment of a child who may have a spine or spinal cord tumour. The principles underlying the management of these tumours are discussed and the management strategies for individual tumour types summarised. Source


Shaw N.J.,SteelHouse | Mughal M.Z.,Royal Manchester Childrens Hospital
Archives of Disease in Childhood | Year: 2013

The first part of this review focused on the skeletal aspects of vitamin D. This second part reviews some of the available evidence that vitamin D may have a physiological extraskeletal role beyond its traditional effect on the skeleton. This aspect has influenced the definition of vitamin D deficiency and what level of vitamin D should be regarded as optimal. The recognition of the prevalence of vitamin D deficiency and insufficiency has led to debate as to whether and how we should be treating asymptomatic individuals. This review discusses the potential extraskeletal effects of vitamin D, the definition of vitamin D deficiency and our thoughts on indications for measurement and treatment. Source


Wong T.,SteelHouse
Current Opinion in Clinical Nutrition and Metabolic Care | Year: 2012

Purpose of Review: Since the 1970s, it has been known that the supplementation of trace elements with parenteral nutrition is required in order to avoid the clinical manifestations of their deficiencies. However, the correct level of requirements of these trace elements, particularly in paediatrics, has provided some debate. The recent developments might help revise some of the current recommendations, particularly in short-term parenteral nutrition provision. Recent Findings: Parenterally fed preterm neonates require routine addition of zinc. Provision of chromium and manganese in parenteral nutrition should be limited, particularly for short-term patients. Newer parenteral iron preparations provide the opportunity for a safer and larger dose of administration. Summary: Clinicians should prescribe according to the individual requirements and revise the routine practice of providing 'all in one' parenteral trace elements preparations, as these products do not reflect or allow tailored provision in paediatrics and may increase the risk of toxicity. © 2012 Wolters Kluwer Health | Lippincott Williams & Wilkins. Source


Parulekar M.V.,SteelHouse
Early Human Development | Year: 2010

Retinoblastoma is the commonest primary ocular malignancy of childhood. There are two forms - heritable and non heritable. Heritable retinoblastoma is a cancer susceptibility syndrome. Presentation is in the first few years of life, sometimes in the neonatal period. Early detection and prompt treatment can give cure rates up to 95% for intraocular tumours, but extraocular disease carries a very high mortality. The diagnosis is essentially clinical and biopsy is contraindicated due to the risk of extraocular spread. Treatment requires significant multidisciplinary input, with local ophthalmic treatment, systemic chemotherapy and external beam or plaque radiotherapy, or surgery to remove the affected eye. Screening of family members is essential for early detection. Lifelong surveillance of mutation carriers is needed due to the risk of second cancers. Newer treatment modalities including intra-arterial chemotherapy have been added to the therapeutic armamentarium in recent years. © 2010 Elsevier Ireland Ltd. Source


Kelly D.A.,SteelHouse
Early Human Development | Year: 2010

Parenteral nutrition liver disease (PNLD) develops in 40-60% of infants who require long-term PN for intestinal failure. The clinical spectrum includes hepatic steatosis, cholestasis, cholelithiasis, and hepatic fibrosis. Progression to biliary cirrhosis and the development of portal hypertension and liver failure occurs in a minority who require combined liver and intestinal transplantation.The pathogenesis is multifactorial and is related to prematurity, low birth weight, duration of PN, short bowel syndrome requiring multiple laparotomies and recurrent sepsis. Other important mechanisms include lack of enteral feeding which leads to reduced gut hormone secretion, reduction of bile flow and biliary stasis which leads to the development of cholestasis, biliary sludge and gallstones, which exacerbate hepatic dysfunction, especially in premature neonates with immature hepatic function.The use of lipid emulsions, particularly soy bean emulsions have been associated with hepatic cholestasis in children, although there are little data now to support toxicity from other PN components.Management strategies for the prevention of parenteral nutrition liver disease include consideration of early enteral feeding, a multidisciplinary approach to the management of parenteral nutrition with a specialized nutritional care team and aseptic catheter techniques to reduce sepsis. The use of specialized lipid emulsions such as fish oil emulsions and or SMOF (Soy bean/Medium Chain Triglyceride/Olive Oil/Fish oil) improves established cholestasis and may prevent the onset.Oral administration of ursodeoxycholic acid may improve bile flow and reduce gall bladder stasis, although there is little data to suggest that prophylactic use prevents the onset of PNLD.Survival following either isolated small bowel or combined liver and small bowel transplantation is approximately 50% at 5. years making this an acceptable therapeutic option in children with irreversible liver and intestinal failure. © 2010. Source

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