Home About us Current issue Back issues Submission Instructions Advertise Contact Login   

Search Article 
  
Advanced search 
 
Saudi Journal of Kidney Diseases and Transplantation
Users online: 3424 Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size 
 

CASE REPORT Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 1  |  Page : 135-137
Nephrotic syndrome and Guillan-barre syndrome: A rare association in child


1 Pediatric's Department, Children's Hospital of Tunis, Tunisia
2 Nephrology Department, Military Hospital, Tunis, Tunisia
3 Pediatric's Department, Charles Nicole Hospital of Tunis, Tunisia

Click here for correspondence address and email

Date of Web Publication8-Jan-2010
 

   Abstract 

Only few cases of nephrotic syndrome associated with Guillain-Barre Syndrome (GBS) have been reported in the adult and pediatric literature. A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of renal or neurological disease. Cerebro­spinal fluid studies showed a protein level of 92 mg/dL and a white cell count of 1 per high-power field. The diagnosis of GBS was verified with a nerve conduction velocity test as well as. The GBS symptoms improved gradually on intravenous immunoglobulin. Three weeks later, he deve­loped severe proteinuria and edema; laboratory investigation showed nephrotic syndrome which responded to steroid therapy. Renal biopsy showed minimal change glomerulonephritis. He re­mained free of proteinuria during his 20 months of follow-up.

How to cite this article:
Bouyahia O, Khelifi I, Gharsallah L, Harzallah K, Mrad SM, Ghargah T, Boukthir S, Azza SE. Nephrotic syndrome and Guillan-barre syndrome: A rare association in child. Saudi J Kidney Dis Transpl 2010;21:135-7

How to cite this URL:
Bouyahia O, Khelifi I, Gharsallah L, Harzallah K, Mrad SM, Ghargah T, Boukthir S, Azza SE. Nephrotic syndrome and Guillan-barre syndrome: A rare association in child. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2020 Sep 28];21:135-7. Available from: http://www.sjkdt.org/text.asp?2010/21/1/135/58789

   Introduction Top


Guillain-Barre syndrome (GBS) is an acute demyelinating disease of peripheral nerves, which usually follows viral illness or immuni­zation. It is the commonest form of peripheral neuropathy affecting children. [1],[2]

There is considerable evidence that GBS is an immune-mediated disease. However, the exact pathogenesis remains unclear. [1],[3] The involve­ment of both sympathetic and parasympathetic fibers in GBS is usually observed as extra neu­rological manifestations such as cardiovascular, gastrointestinal, respiratory, and other systems. [1],[3] Glomerulonephritis (GN) with or without neph­rotic syndrome (NS) is unusual in a patient with GBS and only few cases have been reported. [1],[3],[4]


   Case Report Top


A 3-year-old boy was initially admitted to our hospital following five days of progressive weakness of his extremities, fatigue, right leg pain and numbness. There was no past history of renal or neurological disease. The parents denied any history of fever or sore throat, ex­posure to heavy metals, toxins or sick contacts. His neurological examination revealed mild bulbar palsy and flaccid paraplegia. Tendon re­flexes were totally absent. He was able to obey commands and attempted to speak. All cranial nerves were intact. A chest X-ray and electro­cardiogram were normal. Cerebrospinal fluid study showed a protein level of 92 mg/dL and a white cell count of 1 per high-power field. The diagnosis of GBS was verified with a nerve conduction velocity test. The GBS symp­toms improved gradually on intravenous immu­noglobulin at the dose of 1 g/kg/day in two days. By day 17 after admission, abnormal neu­rological signs improved and the patient was transferred to continue physical therapy. Three weeks after admission, he developed periphe­ral edema and ascites. Urinalysis by dipstick showed proteinuria > 300 mg/dL without blood. Microscopic examination of urine revealed neither white cells nor red cells. The 24 hours urine protein was 209 mg/kg. The serum albu­min concentration was low at 11.5 g/L and se­rum cholesterol was 366 mg/dL. The serum crea­tinine concentration and blood urea were nor­mal. Cytomegalovirus, Epstein Barr virus, hepa­titis B, hepatitis C and human immunodeficiency virus were negative. Antinuclear antibodies were negative, C3 and C4 complement fractions were normal. Percutaneous renal biopsy showed mi­nimal change glomerulonephritis. The patient received 2 mg/kg/day of prednisone, proteinu­ria decreased to 105 mg/kg/day by 11th day and NS went into remission within two weeks. Prednisone was decreased progressively to be stopped four months later. He remained free of proteinuria after his 20 months of follow-up.


   Discussion Top


Our patient presented with NS 3 weeks after onset of GBS while he was still recovering from GBS. Viral infection, history of exposure to solvents, toxins, drugs and recent vaccina­tion were excluded. Immunological investiga­tions were negative. The time course of pro­teinuria and GBS, suggests that this occurrence is more than chance and that a causal rela­tionship can be suspected.

The association of GBS and GN has been reported, the exact prevalence of renal involve­ment during GBS is probably underestimated because glomerular lesions can be asymptoma­tic. [5] Proteinuria is usually mild and transient; it usually subsides during motor recovery. True NS, reported in our patient, is uncommon in GBS. [2],[4]

Although most of the systemic disorders asso­ciated with GBS are due to immobilization, respiratory failure, dysautonomia and preceding infection, the primary inflammatory mechanism plays a part in the pathogenesis of renal in­volvement. In fact, circulating antibodies against not only the nervous system, but also the kid­ney, brain, liver and cardiac striated muscles have been documented. [2],[6] Both diseases are cell mediated witch supports the possibility that the association may not be coincidental. [3] The pathogenesis of GBS seems to involve lymphocytes and macrophages which are rich sources of cytokines. Cytokines appear to induce the damage of Schwann cells, myelin and axons. This lymphocyte-mediated disturbance is pro­bably implicated in glomerular lesions also in GBS.[2],[7]

Membranous GN is the most frequent histo­logical finding with other presentation as acute and post infectious GN.[4],[5] Minimal change NS, reported in our patient, was only described in one child and rest of them in adults.[1],[4],[8],[9] Re­ported cases of GBS associated with NS are represented in [Table 1].

Remission of GN occurred in almost 60% of cases with recovery of GBS. Nephrotic syn­drome is usually reversible on oral cortico­steroid treatment, immunosuppressive drugs are rarely indicated.[1],[8],[9]


   Conclusion Top


In conclusion, this case report indicates that cell mediated immune response represent probab­ly the main pathogenic mechanism of Guillan­Barre syndrome. Large prospective studies of renal involvement during Guillan-Barre syn­drome can contribute to understanding the exact pathogenesis of both diseases.

 
   References Top

1.Kitamura H, Nakano T, Kakihara M, et al. A case of Guillain-Barre syndrome developed minimal change nephrotic syndrome simultaneously. Am J Nephrol 1998;18:151-4.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Dhib M, Moulin B, Godin M, Gourmelen O, Senant J, Le Loet X, Fillastre JP. Guillain­Barre syndrome and extramembranous glome­rulopathy. Apropos of a case. Ann Med Interne (Paris) 1989;140(6):523-4.  Back to cited text no. 2      
3.Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with Guillain-Barre syndrome. Pediatr Nephrol 2004;19:105-6.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Olbricht CJ, Stark E, Helmchen U, Schulze M, Brunkhorst R, Koch KM. Glomerulonephritis associated with inflammatory demyelinating polyradiculoneuropathy: a case report and re­view of the literature. Nephron 1993;64:139-41.  Back to cited text no. 4  [PUBMED]    
5.Rodriguez-Iturbe B, Garcia R, Rubio L, Zabala J, Moros G, Torres R. Acute glomeruloneph­ritis in the Guillain-Barre-Strohl syndrome. Report of nine cases. Ann Intern Med 1973; 78:391-5.  Back to cited text no. 5      
6.Van Doorn PA, Brand A, Vermeulen M. Cli­nical significance of antibodies against peri­pheral nerve tissue in inflammatory polyneuro­pathy. Neurology 1987;37:1798-802.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Penny MJ, Boyd RA, Hall BM. Role of T cells in the mediation of Heymann nephritis. ii. Identification of Th1 and cytotoxic cells in glomeruli. Kidney Int 1997;51:1059-68.  Back to cited text no. 7      
8.Froelich CJ, Searles RP, Davis LE, Goodwin JS. A case of Guillain-Barre syndrome with immunologic abnormalities. Ann Intern Med 1980;93:563-5.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Chen JK, Wu MS, Yang CW, et al. Guillain­Barre syndrome associated with minimal change glomerulopathy and tubular dysfunction re­lated to acetone-based organic solvent? Am J Nephrol 2002;22:560-5.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  

Top
Correspondence Address:
Olfa Bouyahia
Pediatric's Department, Children's Hospital Tunis, Tunis
Tunisia
Login to access the Email id


PMID: 20061709

Rights and Permissions



 
 
    Tables

  [Table 1]

This article has been cited by
1 Inflammatory demyelinating polyneuropathy with nephrotic syndrome: Report of a case and review of the literature
Tateyama, M. and Nakashima, I. and Suzuki, N. and Kanaoka-Suzuki, C. and Takano, R. and Sato, H. and Kusunoki, S. and Fujihara, K. and Itoyama, Y. and Aoki, M.
Clinical and Experimental Neuroimmunology. 2013; 4(1): 79-88
[Pubmed]



 

Top
 
 
    Similar in PUBMED
    Search Pubmed for
    Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  
 


 
    Abstract
    Introduction
    Case Report
    Discussion
    Conclusion
    References
    Article Tables
 

 Article Access Statistics
    Viewed4205    
    Printed120    
    Emailed0    
    PDF Downloaded738    
    Comments [Add]    
    Cited by others 1    

Recommend this journal