ABSTRACT. Background. Difficulties with ambulation in patients with myelomeningocele often lead to physical inactivity, osteoporosis, and subsequent development of pathologic fractures.Objective. The purpose of this study was to examine bone mineral density and biochemical markers of bone metabolism in patients with myelomeningocele.Design and Methods. A total of 35 patients between 6 and 19 years of age with myelomeningocele (ambulatory and nonambulatory) were randomly chosen at the Texas Scottish Rite Hospital for Children. We measured bone mineral density of the distal radius in these patients using single photon absorptiometry and measured the biochemical markers of bone metabolism including parathyroid hormone, 1,25 vitamin D, osteocalcin, urinary pyridinolines/deoxypyridinolines, and urinary calcium excretion.Results. Bone mineral density of the distal radius in the patients with myelomeningocele was ϳ1 to 2 standard deviation units below the mean of the normal population. There were no significant differences between ambulators and nonambulators. However, bone mineral density of the 8 patients who suffered multiple fractures (19) was significantly lower than that for those remaining patients without fractures. Elevated urinary pyridinoline levels, which indicate elevated bone reabsorption, were found more frequently in both non-and limited ambulators than in full-time ambulators. Urinary calcium excretion also was greater than twofold higher in nonambulatory patients versus ambulatory patients. There were no other differences in the biochemical markers of bone metabolism (osteocalcin, parathyroid hormone, 1,25 vitamin D, and urinary deoxypyridinolines) between ambulators and nonambulators. Bone mineral density rises in normal growing children 6 to 19 years of age. When the boys and girls were considered separately, bone mineral density rises with age in boys, but not in girls.Conclusion. Patients with myelomeningocele have decreased bone mineral density and are at risk of suffering pathologic bone fractures. The measurement of bone mineral density may help to identify those patients at greatest risk of suffering multiple fractures. The urinary calcium excretion of nonambulators was higher than that of ambulators and likely contributes to their decreased bone mineral density. Bone mineral density increases with age in boys, but not in girls. Pediatrics 1998;34(102). URL: http://www.pediatrics.org/cgi/content/full/34/102/ e34; spina bifida, bone densitometry, osteoporosis, pathologic fractures.ABBREVIATIONS. FTWC, full-time wheelchair patient; LA, limited ambulator; FTA, full-time ambulator; PTH, parathyroid hormone; OSTEO, serum osteocalcin; PYR, urinary pyridinolines; dPYR, urinary deoxypyridinolines. L ower extremity sensory and motor deficits are found frequently in patients with myelomeningocele. These deficits can impair normal ambulation and lead to variable degrees of physical inactivity, osteoporosis, and development of pathologic fractures.1-6 Such fractures lead to a vicious cycle in which furthe...
Children with myelomeningocele experience difficulty with ambulation, which leads to immobilization and secondary loss of bone mineral density (BMD). In addition, non-ambulatory myelomeningocele patients have higher urinary calcium losses than their ambulatory counterparts. Hydrochlorothiazide (HCTZ) is known to reduce urinary calcium loss and increase BMD in non-myelomeningocele patients with hypercalciuria. This study examines the effect of HCTZ on urinary calcium and BMD in non-ambulatory children with myelomeningocele. Thirteen of 20 non-ambulatory patients with myelomeningocele completed the year-long randomized double-blinded study (placebo = 7 and HCTZ = 6). Evaluation included electrolytes, PTH, osteocalcin, 1, 25-OH vitamin D, urinary pyridinolines/deoxypyridinolines (U(pyr/dpyr)), urinary calcium/creatinine (U(Ca/Cr)), and forearm BMD (dual X-ray absorptiometry). Follow-up electrolytes were obtained at 1-2, 6, and 12 months and U(Ca/Cr) and BMD was obtained again at 12 months. There were no initial differences between the placebo and HCTZ groups. U(Ca/Cr) decreased in the HCTZ group after treatment (0.20+/-0.09 vs. 0.04+/-0.02, p<0.05). However, forearm BMD ( z-scores) after 1 year remained unchanged in both the HCTZ (-5.95+/-0.98 to -5.86+/-0.92) and placebo (-7.19+/-0.69 to -6.67+/-0.63) groups. While use of HCTZ for 1 year did not affect BMD, it reduced urinary calcium excretion in non-ambulatory children with myelomeningocele.
Serum creatinine is used to estimate the glomerular filtration rate (GFR). The serum creatinine, however, may not accurately reflect the GFR in spina bifida patients, who often have decreased overall muscle mass resulting from spinal cord abnormalities. The relationship between the serum creatinine and GFR (obtained by [125I]iothalamate clearance) was examined in a population of spina bifida patients. Age‐matched patients without spina bifida were used as controls. Results demonstrate that, for serum creatinines above 0.5 mg/dL, serum creatinine is a very poor predictor of GFR. Two patients with serum creatinines of 2.2 mg/dL are near end‐stage renal disease with GFRs of 12.5 and 13 mL/minute per 1.73mz and two patients were initiated on dialysis at the conclusion of the study. It is concluded that obtaining a GFR from a clearance study and not serum creatinine is the only reliable method to assess renal function in spina bifida patients once the serum creatinine is greater than 0.5 mg/dL.
Bowel continence is one of the most difficult challenges for patients with spina bifida. Incontinence acts as a social stigma for children and a barrier for adults seeking employment. We present an algorithm for stepwise decision-making in construction of personalized continence programs for greater likelihood of success. The protocol contains 13 assessment points including; stool consistency, frequency and amount; mobility; level of paraplegia: diet; medication; anal/rectal canal tone; prior programs attempted; family routines; age; accessibility; and learning issues. Based on outcomes of these assessments, an individualized bowel program is constructed. The algorithm helps the practitioner and patient decide on components and indicators of a successful continence program. The recommended program might include timed toileting, suppository, continence enema, and ACE procedure, or a combination. Evaluation and patient education address adequate fluid/fiber, appropriate toileting equipment, and use of stool softeners/laxatives. Descriptions are available. Key elements in monitoring a continuing plan for continence include: the degree of constipation and its etiology; changing age; family availability for assistance until interdependence is optimal; wheelchair accessibility of the toilet; and ability to transfer to and from the toilet. Use of the algorithm allows for careful decision-making based on information from the patient and family. This has led to greater success in bowel continence in children with spina bifida.
The purpose of this article is to provide an overview of skin issues in children with spina bifida. Included in the discussion below is a review of the etiology of pressure ulcers and the updated 2007 pressure ulcer definition and pressure ulcer staging system as defined by the National Pressure Ulcer Advisory Panel (NPUAP). Pediatric risk factors for skin breakdown are presented including risk factors unique to children with spina bifida. Pediatric pressure ulcer risk assessment scales are described. The 5 Million Lives Kids' Campaign which has a focus on preventing hospital-acquired pressure ulcers in children is also reviewed along with evidence based prevention strategies. The key to preventing skin breakdown and pressure ulcers in children with spina bifida is early identification of the child's individual risk factors so that a prevention protocol can be implemented in all settings: hospital, home and the community. Options for wound management, dressing selection and pain management are included.
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