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| Year : 2010 | Volume
: 21
| Issue : 1 | Page : 135-137 |
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| Nephrotic syndrome and Guillan-barre syndrome: A rare association in child |
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Olfa Bouyahia1, Ibtissem Khelifi1, Lamia Gharsallah1, Kais Harzallah2, Sonia Mazigh Mrad1, Tahar Ghargah3, Samir Boukthir1, Sammoud El Gharbi Azza1
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 Publication | 8-Jan-2010 |
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 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 2021 Jul 24];21:135-7. Available from: https://www.sjkdt.org/text.asp?2010/21/1/135/58789 |
| Introduction | |  |
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 | | |
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 | | |
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 | | |
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 | | |
| 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.  [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. |
| 3. | Ilyas M, Tolaymat A. Minimal change nephrotic syndrome with Guillain-Barre syndrome. Pediatr Nephrol 2004;19:105-6. [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. [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. |
| 6. | Van Doorn PA, Brand A, Vermeulen M. Clinical significance of antibodies against peripheral nerve tissue in inflammatory polyneuropathy. Neurology 1987;37:1798-802. [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. |
| 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. [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. [PUBMED] [FULLTEXT] |
Correspondence Address:
Olfa Bouyahia
Pediatric's Department, Children's Hospital Tunis, Tunis
Tunisia
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PMID: 20061709 
[Table 1] |
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| This article has been cited by | | 1 |
Inflammatory demyelinating polyneuropathy with nephrotic syndrome: Report of a case and review of the literature |
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| 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] | |
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