Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
International Urology and Nephrology
Published in: International Urology and Nephrology 3/2012

01-06-2012 | Nephrology – Review

Prevention of chronic kidney disease in spina bifida

Authors: Guido Filler, Mireille Gharib, Shelley Casier, Petra Lödige, Jochen H. H. Ehrich, Sumit Dave

Published in: International Urology and Nephrology | Issue 3/2012

Login to get access

Abstract

Objective

The prevalence of progressive chronic kidney disease (CKD) in children and adults with spina bifida is considerable, rising, and entirely preventable.

Removing the cause: prevention of spina bifida

The best prevention of CKD in spina bifida is prevention of spina bifida itself through strategies that include folate supplementation, ideally before pregnancy.

The cause of CKD

Dysfunctional bladder outlet causes febrile Urinary Tract Infections (UTI), even with clean intermittent catheterization (CIC), and subsequent renal scarring. The development of secondary vesicoureteric reflux (VUR) increases the risk of renal scarring and CKD.

Finding the ideal marker for measurement of renal function in spina bifida

Creatinine-based methods are insensitive because of low muscle mass and underdeveloped musculature in the legs. Only Cystatin C–based eGFR can reliably assess global renal function in these patients. However, unilateral renal damage requires nuclear medicine scans, such as 99mTc DMSA.

(Video)Urodynamics studies (UDS)

Early treatment is recommended based on UDS with anticholinergics, CIC, and antibiotic prophylaxis when indicated. Overnight catheter drainage, Botox, and eventually augmentation cystoplasty are required for poorly compliant bladders. A continent child or one rendered continent following surgery is at a higher risk of renal damage.

Conclusion

A multidisciplinary approach is required to reduce the burden of CKD in patients with spina bifida. The right tools have to be utilized to monitor these patients, particularly if recurrent UTIs occur. Cystatin C eGFR is preferred for monitoring renal damage in these patients, and 99mTc DMSA scans have to be used to detect unilateral renal scarring.
Literature
1.
2.
Muralikrishna GS, Rodger RS, Macdougall AI, Boulton-Jones JM, Allison ME, Kyle KF, Junor BJ, Briggs JD (1989) Renal replacement treatment in patients with spina bifida or spinal cord injury. BMJ 299:1506PubMedCrossRef
3.
Lapides J, Diokno AC, Silber SJ, Lowe BS (1971) Clean, intermittent self-catheterization in the treatment of urinary tract disease. Trans Am Assoc Genitourin Surg 63:92–96PubMed
4.
Edelstein RA, Bauer SB, Kelly MD, Darbey MM, Peters CA, Atala A, Mandell J, Colodny AH, Retik AB (1995) The long-term urological response of neonates with myelodysplasia treated proactively with intermittent catheterization and anticholinergic therapy. J Urol 154:1500–1504PubMedCrossRef
5.
Lee CY, Lee CY (2010) Long-Term Renal Outcome in Patients with Lumbar Meningomyelocele. Abstract# 864. Pediatr Nephrol 25:1967
6.
Muller F, O’Rahilly R (2004) The primitive streak, the caudal eminence and related structures in staged human embryos. Cells Tissues Organs 177:2PubMedCrossRef
7.
Alvarez IS, Schoenwolf GC (1992) Expansion of surface epithelium provides the major extrinsic force for bending of the neural plate. J Exp Zool 261:340PubMedCrossRef
8.
Muller F, O’Rahilly R (1987) The development of the human brain, the closure of the caudal neuropore, and the beginning of secondary neurulation at stage 12. Anat Embryol 176:413PubMedCrossRef
9.
Juranek J, Salman MS (2010) Anomalous development of brain structure and function in spina bifida myelomeningocele. Dev Disabil Res Rev 16(1):23–30PubMedCrossRef
10.
Frey L, Hauser WA (2003) Epidemiology of neural tube defects. Epilepsia (suppl 44) 3:4
11.
MacHenry JCRM, Nevin NC, Merrett JD (1979) Comparison of central nervous system malformations in spontaneous abortions in Northern Ireland and south-east England. Br Med J 1:1395–1397PubMedCrossRef
12.
13.
Nicolaides KH, Campbell S, Gabbe SG, Guidetti R (1986) Ultrasound screening for spina bifida: cranial and cerebellar signs. Lancet 2:72PubMedCrossRef
14.
Wald N, Cuckle H, Nanchahal K (1989) Amniotic fluid acetylcholinesterase measurement in the prenatal diagnosis of open neural tube defects. Prenat Diagn 9:813PubMedCrossRef
15.
Berry RJ, Li Z, Erickson JD et al (1999) Prevention of neural-tube defects with folic acid in China. N Engl J Med 341:1485PubMedCrossRef
16.
Goh YI, Bollano E, Einarson TR, Koren G (2006) Prenatal multivitamin supplementation and rates of congenital anomalies: a meta-analysis. J Obstet Gynaecol Can 28:680PubMed
17.
Shaw GM, Schaffer D, Velie EM et al (1995) Periconceptional vitamin use, dietary folate, and the occurrence of neural tube defects. Epidemiology 6:219PubMedCrossRef
18.
Antal R, Siffel C, Czeizel E (1996) Prevention of anencephaly-spina bifida. Orv Hetil 137:100PubMed
19.
Oakley GP Jr, Erickson JD, James LM, Mulinare J, Cordero JF (1994) Prevention of folic acid-preventable spina bifida and anencephaly. Ciba Found Symp 181:212–223 Discussion 223–231PubMed
20.
Hernandez-Diaz S, Werler MM, Walker AM, Mitchell AA (2000) Folic acid antagonists during pregnancy and the risk of birth defects. N Engl J Med 343:1608PubMedCrossRef
21.
Blencowe H, Cousens S, Modell B, Lawn J (2010) Folic acid to reduce neonatal mortality from neural tube disorders. Int J Epidemiol 39(Suppl 1):i110–i121PubMedCrossRef
22.
Alasfoor D, Elsayed MK, Mohammed AJ (2010) Spina bifida and birth outcome before and after fortification of flour with iron and folic acid in Oman. East Mediterr Health J 16:533–538PubMed
23.
De Wals P, Tairou F, Van Allen MI, Uh SH, Lowry RB, Sibbald B et al (2007) Reduction in neural-tube defects after folic acid fortification in Canada. N Engl J Med 357:135–142PubMedCrossRef
24.
de Jong TP, Chrzan R, Klijn AJ, Dik P (2008) Treatment of the neurogenic bladder in spina bifida. Pediatr Nephrol 23:889–896PubMedCrossRef
25.
Almodhen F, Capolicchio JP, Jednak R, El Sherbiny M (2007) Postpubertal urodynamic and upper urinary tract changes in children with conservatively treated myelomeningocele. J Urol 178(4 Pt 1):1479–1482PubMedCrossRef
26.
27.
Kaefer M, Pabby A, Kelly M et al (1999) Improved bladder function after prophylactic treatment of the high risk neurogenic bladder in newborns with myelomentingocele. J Urol 162:1068PubMedCrossRef
28.
Müller T, Arbeiter K, Aufricht C (2002) Renal function in meningomyelocele: risk factors, chronic renal failure, renal replacement therapy and transplantation. Curr Opin Urol 12(6):479–484PubMedCrossRef
29.
Panicker JN, de Sèze M, Fowler CJ (2010) Rehabilitation in practice: neurogenic lower urinary tract dysfunction and its management. Clin Rehabil 24:579–589PubMedCrossRef
30.
Dwoskin JY, Perlmutter AD (1973) Vesticoureteric reflux in children: a computerized review. J Urol 109:888–890PubMed
31.
Jafri RA et al (1988) 99Tcm-MAG-3: a comparison with I-123 and I-131 orthoidohippurate in patients with renal disorder. J Nucl Med 19:994–1000
32.
Ottolini MC, Shaer CM, Rushton HG, Majd M, Gonzales EC, Patel KM (1995) Relationship of asymptomatic bacteriuria and renal scarring in children with neuropathic bladders who are practicing clean intermittent catheterization. J Pediatr 127:368–372PubMedCrossRef
33.
Schlager TA, Clark M, Anderson S (2001) Effect of a single use sterile catheter for each void on the frequency of bacteriuria in children with neurgenic bladder on intermittent catheterization for bladder emptying. Pediatrics 108:e71PubMedCrossRef
34.
Joseph DB, Bauer SB, Colodny AH, Mandell J, Retik AB (1989) Clean intermittent catheterization in infants with neurogenic bladder. Pediatrics 84:78PubMed
35.
Galloway A, Green HT, Windsor JJ, Menon KK, Gardner BP, Krishnan KR (1986) Serial concentrations of C-reactive protein as an indicator of urinary tract infection in patients with spinal injury. J Clin Pathol 39:851–855PubMedCrossRef
36.
Muller T, Arbeiter K, Aufricht C (2002) Renal function in meningomyelocele: risk factors, chronic renal failure, renal replacement therapy and transplantation. Curr Opin 12:479
37.
van Gool JD, Dik P, de Jong TP (2001) Bladder-sphincter dysfunction in myelomeningocele. Eur J Pediatr 160(7):414–420PubMedCrossRef
38.
Bauer SB, Hallet M, Khoshbin S, Leowitz RL, Winston KR, Gibson S, Colodny AH, Retik AB (1984) Predictive value of urodynamic evaluation in newnborns with myerlodysplasia. JAMA 252:650PubMedCrossRef
39.
Tepmongkol S, Chotipanich C, Sirisalipoch S, Chaiwatanarat T, Vilaichon AO, Wattana D (2002) Relationship between vesicoureteral reflux and renal cortical scar development in Thai children: the significance of renal cortical scintigraphy and direct radionuclide cystography. J Med Assoc Thai 85(Suppl 1):S203–S209PubMed
40.
Lee JH, Son CH, Lee MS, Park YS (2006) Vesicoureteral reflux increases the risk of renal scars: a study of unilateral reflux. Pediatr Nephrol 21:1281–1284PubMedCrossRef
41.
Morgan C, Senthilselvan FB, Hoskinson M, Gowrishankar M (2008) Correlation between cystatin C- and renal scan- determined glomerular filtration rate in children with spina bifida. Pediatr Nephrol 23:329–332PubMedCrossRef
42.
Rahn KH, Heidenreich S, Bruckner D (1999) How to assess glomerular function and damage in humans. J Hypertens 17:309PubMedCrossRef
43.
Schwartz GJ, Furth SL (2007) Glomerular filtration rate measurement and estimation in chronic kidney disease. Pediatr Nephrol 22:1839–1848PubMedCrossRef
44.
Filler G, Browne R, Seikaly MG (2003) Glomerular filtration rate as a putative ‘surrogate end-point’ for renal transplant clinical trials in children. Pediatr Transplant 7:18–24PubMedCrossRef
45.
Stevens LA, Levey AS (2005) Measurement of kidney function. In: Singh AK (ed) Medical clinics of North America. W.B. Saunders, Philadelphia, p 457
46.
Schwartz GJ, Haycock GB, Edelman CM, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263PubMed
47.
Sharma AP, Filler G (2009) Monitoring kidney function and renal disease in children after transplant. Paediatr Health 3:155–163CrossRef
48.
49.
Doolan PD, Alpen EL, Theil GB (1962) A clinical appraisal of the plasma concentration and endogenous clearance of creatinine. Am J Med 32:65PubMedCrossRef
50.
51.
Petri M, Bochenstedt L, Colman J et al (1988) Serial assessment of glomerular filtration rate in lupus nephropathy. Kidney Int 34:832PubMedCrossRef
52.
Pham-Huy A, Leonard M, Lepage N, Halton J, Filler G (2003) Measuring glomerular filtration rate with cystatin C and beta-trace protein in children with spina bifida. J Urol 169:2312–2315PubMedCrossRef
53.
Filler G, Bokenkamp A, Hofmann W, Le Bricon T, Martinez-Bru C, Grubb A (2005) Cystatin C as a marker of GFR- history, indications, and future research. Clin Biochem 38:1–8PubMedCrossRef
54.
Jung P (1987) Low molecular mass proteins in serum and their relationship to the glomerular filtration rate. Nephron 47:160–168PubMedCrossRef
55.
Grubb A, Simonsen O, Sturfelt G, Truedsson L, Thysell H (1985) Serum concentration of cystatin C, factor D and b2-microglobulin as a measure of glomerular filtration rate. Acta Med Scand 218:499–503PubMedCrossRef
56.
Bokenkamp A, Domanetzki M, Zinck R, Schumann G, Brodehl J (1998) Reference values for cystatin C serum concentrations in children. Pediatr Nephrol 12:125–129PubMedCrossRef
57.
Randers E, Krue S, Erlandsen EJ, Danielsen H, Hansen LG (1999) Reference interval for serum cystatin C in children. Clin Chem 45:1856–1858PubMed
58.
Norlund L, Fex G, Lanke J et al (1997) Reference intervals for the glomerular filtration rate and cell proliferation markers: serum cystatin C and serum b2-microglobulin/cystatin C-ratio. Scand J Clin Lab Invest 57:463–470PubMedCrossRef
59.
Harmoinen A, Ylinen E, Ala-Houhala M, Janas M, Kaila M, Kouri T (2000) Reference intervals for cystatin C in pre- and full-term infants and children. Pediatr Nephrol 15:105–108PubMedCrossRef
60.
Fischbach M, Graff V, Terzic J, Bergere V, Oudet M, Hamel G (2002) Impact of age on reference values for serum concentration of cystatin C in children. Pediatr Nephrol 17:104–106PubMedCrossRef
61.
Bokenkamp A, Domanetzki M, Zinck R, Schumann G, Brodehl J (1998) Reference values for cystatin C serum concentrations in children. Pediatr Nephrol 12:125–129PubMedCrossRef
62.
Vinge E, Lindergard B, Nilsson-Ehle P, Grubb A (1999) Relationships among serum cystatin C, serum creatinine, lean tissue mass and glomerular filtration rate in healthy adults. Scand J Clin Lab Invest 59:587–592PubMedCrossRef
63.
Woitas RP, Stoffel-Wagner B, Flommersfeld S et al (2000) Correlation of serum concentrations of cystatin C and creatinine to inulin clearance in liver cirrhosis. Clin Chem 46:712–715PubMed
64.
Thomassen SA, Johannesen IL, Erlandsen EJ, Abrahamsen J, Randers E (2002) Serum cystatin C as a marker of the renal function in patients with spinal cord injury. Spinal Cord 40:524–528PubMedCrossRef
65.
Abrahamsson K, Jodal U, Sixt R, Olsson I, Sillen U (2008) Estimation of renal function in children and adolescents with spinal dysraphism. J Urol 179:2407–2409PubMedCrossRef
66.
Ozel SK, Dokumcu Z, Akyildiz C, Avanoglu A, Ulman I (2007) Factors affecting renal scar development in children with spina bifida. Urol Int 79:133–136PubMedCrossRef
67.
Dik P, Klijn AJ, van Gool JD, van Steenwijk CC, de Jong TP (2006) Early start to therapy preserves kidney function in spina bifida patients. Eur Urol 49:908–913PubMedCrossRef
68.
Coulthard MG, Verber I, Jani JC, Lawson GR, Stuart CA, Sharma V, Lamb WH, Keir MJ (2009) Can prompt treatment of childhood UTI prevent kidney scarring? Pediatr Nephrol 24:2059–2063PubMedCrossRef
69.
Dawrant MJ, Mohanan N, Puri P (2006) Endoscopic treatment for high grade vesicoureteral reflux in infants. J Urol 176(4 Pt 2):1847–1850PubMedCrossRef
70.
Montini G, Hewitt I (2009) Urinary tract infections: to prophylaxis or not to prophylaxis? Pediatr Nephrol 24:1605–1609PubMedCrossRef
71.
Ziessman HA, Majd M (2009) Importance of methodology on (99m)technetium dimercapto-succinic acid scintigraphic image quality: imaging pilot study for RIVUR (Randomized Intervention for Children With Vesicoureteral Reflux) multicenter investigation. J Urol 182:272–279PubMedCrossRef
72.
Brandström P, Nevéus T, Sixt R, Stokland E, Jodal U, Hansson S (2010) The Swedish reflux trial in children: IV. Renal damage. J Urol 184:292–297PubMedCrossRef
73.
Shiroyanagi Y, Suzuki M, Matsuno D, Yamazaki Y (2009) The significance of 99mtechnetium dimercapto-succinic acid renal scan in children with spina bifida during long-term followup. J Urol 181:2262PubMedCrossRef
74.
Leonardo CR, Filgueiras MF, Vasconcelos MM, Vasconcelos R, Marino VP, Pires C, Pereira AC, Reis F, Oliveira EA, Lima EM (2007) Risk factors for renal scarring in children and adolescents with lower urinary tract dysfunction. Pediatr Nephrol 22(11):1891–1896PubMedCrossRef
75.
DeLair SM, Eandi J, White MJ, Nguyen T, Stone AR, Kurzrock EA (2007) Renal cortical deterioration in children with spinal dysraphism: analysis of risk factors. J Spinal Cord Med 30:30
76.
McGuire EJ, Woodside JR, Borden TA, Weiss RM (1981) Prognostic value of urodynamic testing in myelodysplastic patients. J Urol 126(2):205–209PubMed
77.
Cho SY, Yi JS, Oh SJ (2009) The clinical significance of poor bladder compliance. Neurourol Urodyn 28:1010–1014PubMedCrossRef
78.
Khoury AE, Dave S, Peralta-Del Valle MH, Braga LH, Lorenzo AJ, Bägli D (2008) Severe bladder trabeculation obviates the need for bladder outlet procedures during augmentation cystoplasty in incontinent patients with neurogenic bladder. BJU Int 101:223–226PubMed
79.
Zermann DH, Löffler U, Reichelt O, Wunderlich H, Wilhelm S, Schubert J (2003) Bladder dysfunction and end stage renal disease. Int Urol Nephrol 35:93–97PubMedCrossRef
80.
Nguyen MT, Pavlock CL, Zderic SA, Carr MC, Canning DA (2005) Overnight catheter drainage in children with poorly compliant bladders improves post-obstructive diuresis and urinary incontinence. J Urol 174(4 Pt 2):1633–1636 discussion 1636PubMedCrossRef
81.
Gamé X, Mouracade P, Chartier-Kastler E, Viehweger E, Moog R, Amarenco G, Denys P, De Seze M, Haab F, Karsenty G, Kerdraon J, Perrouin-Verbe B, Ruffion A, Soler JM, Saussine C (2009) Botulinum toxin-A (Botox) intradetrusor injections in children with neurogenic detrusor overactivity/neurogenic overactive bladder: a systematic literature review. J Pediatr Urol 5(3):156–164PubMedCrossRef
82.
Duel BP, Gonzalez R, Barthold JS (1998) Alternative techniques for augmentation cystoplasty. J Urol 159:998–1005PubMedCrossRef
83.
Buffa P, Torre M, Scarsi PL, De Gennaro M, Battaglino F, Beseghi U, Di Lorenzo F, Cama A (2002) Caudal regression syndrome: an online multicentre survey. Urological long-term results. Eur J Pediatr Surg 12(Suppl 1):526–528
84.
Dave S, Pippi Salle JL, Lorenzo AJ, Braga LH, Peralta-Del Valle MH, Bägli D, Khoury AE (2008) Is long-term bladder deterioration inevitable following successful isolated bladder outlet procedures in children with neuropathic bladder dysfunction? J Urol 179(5):1991–1996PubMedCrossRef
Metadata
Title
Prevention of chronic kidney disease in spina bifida
Authors
Guido Filler
Mireille Gharib
Shelley Casier
Petra Lödige
Jochen H. H. Ehrich
Sumit Dave
Publication date
01-06-2012
Publisher
Springer Netherlands
Published in
International Urology and Nephrology / Issue 3/2012
Print ISSN: 0301-1623
Electronic ISSN: 1573-2584
DOI
https://doi.org/10.1007/s11255-010-9894-5

Other articles of this Issue 3/2012

International Urology and Nephrology 3/2012 Go to the issue

Urology – Original Paper

Utility of the transition zone index for identification of prostate cancer in Chinese men with intermediate PSA levels

Nephrology – Case Report

An unusual cause of membranous glomerulonephritis in a patient with HIV

Nephrology – Original Paper

Serum and urine responses to the aquaretic agent tolvaptan in hospitalized hyponatremic patients

Nephrology – Original Paper

Effect of sirolimus on the regression of peritoneal sclerosis in an experimental rat model

Urology – Original Paper

Extracorporeal shock wave lithotripsy as first line treatment for urinary tract stones in children: outcome of 500 cases

Urology - Original Paper

Long-term safety, tolerability, and efficacy of α1-adrenergic blocker in young men with primary bladder neck obstruction: Results from a single centre in China
Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Reserve your place

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin
Prof. Florian Limbourg
Prof. Anoop Chauhan
Developed by: Springer Medicine
Obesity Clinical Trial Summary

At a glance: The STEP trials

Read more

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Pediatric Cardiology Video

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

Cardiopulmonary Comorbidity Video

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Valvular Heart Disease Video

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Heart Failure Video

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Heart Failure Video

Watch this official video from ACC.24. Dr. Biykem Bozkurt discusses last year's major advances in heart failure and cardiomyopathies.

Watch now

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

16-04-2024 Type 2 Diabetes News

Incentivised to exercise

11-04-2024 Lifestyle Modifications News

New intervention for STEMI-related cardiogenic shock

10-04-2024 Myocardial Infarction News

» View more highlights on the ACC 2024 congress hub page

Image Credits