|Year : 2022 | Volume
| Issue : 1 | Page : 76-77
Bilateral posterior subcapsular lens deposits associated with membranoproliferative glomerulonephritis in a 6-year-old child: A case report
Rym Maamouri1, Abir Boussetta2, Ouederni Meriem1, Sassi Hela1
1 Department of Ophtalmology, Habib Thameur Hospital; University of Tunis El Manar, Faculty of Medecine of Tunis, Tunis, Tunisia
2 University of Tunis El Manar, Faculty of Medecine of Tunis; Department of Pediatric Nephrology, Charles Nicolle hospital, Tunis, Tunisia
|Date of Submission||15-Feb-2021|
|Date of Acceptance||29-Jun-2021|
|Date of Web Publication||07-Jan-2022|
Dr. Rym Maamouri
Department of Ophthalmology, Habib Thameur Hospital, 3, rue Ali ben Ayed, Montfleury - 1089, Tunis
Source of Support: None, Conflict of Interest: None
A 6-year-old girl with membranoproliferative glomerulonephritis (MPGN) was referred for a systematic ophthalmological examination. Her visual acuity was 20/20 in both eyes, and detailed slit examination showed bilateral posterior subcapsular lens deposits. She was treated with corticosteroid and immunosuppressive therapy. Ophthalmological examination 10 months later showed a significant decrease of the lens deposits. To the best of our knowledge, this is the first case reporting lens deposits in MPGN. It warns us to perform a detailed examination of the anterior segment and correct management of the pathology in order to be able to reduce these deposits.
Keywords: Membranoproliferative glomerulonephritis, posterior subcapsular lens deposits, young patient
|How to cite this article:|
Maamouri R, Boussetta A, Meriem O, Hela S. Bilateral posterior subcapsular lens deposits associated with membranoproliferative glomerulonephritis in a 6-year-old child: A case report. Indian J Ophthalmol Case Rep 2022;2:76-7
|How to cite this URL:|
Maamouri R, Boussetta A, Meriem O, Hela S. Bilateral posterior subcapsular lens deposits associated with membranoproliferative glomerulonephritis in a 6-year-old child: A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Jan 23];2:76-7. Available from: https://www.ijoreports.in/text.asp?2022/2/1/76/334949
Membranoproliferative glomerulonephritis (MPGN) is a rare glomerular disease that causes up to 4% of all primary nephrotic syndromes in children. Diagnosis is primarily made based on histopathology and immunohistochemistry. Classification differentiates between immune-complex MPGN (IC-MPGN) and complement-mediated C3-glomerulopathy (C3G) based on immunoglobulin (Ig) or complement deposits in the glomeruli, respectively. The disease has been traditionally classified into types I, II, and III based on electron microscopy findings. A previous diagnosis of MPGN type I reflects either C3G or IgG/IC-mediated glomerulonephritis (GN), depending on the C3 staining. Patients with MPGN are reclassified as C3G. Type II, also called DDD for dense-deposit disease, is well known to be associated with drusen-like deposits and associated with choroidal neovascularization and idiopathic central serous chorioretinopathy. We report the first case of bilateral and posterior subcapsular lens deposits in a 6-year-old child with IC-MPGN.
| Case Report|| |
A 6-year-old girl was admitted to the pediatric nephrology department for rapidly progressive GN. Physical examination of the patient revealed the following: Height 110 cm, weight 16.8 kg, gross hematuria and normal blood pressure, and heart rate was 80 with a regular rhythm and normal heart auscultation. Examination of her respiratory system revealed no abnormality. She had face and lower limbs edema and oliguria. Investigations revealed hemoglobin 9.7 g/dL, total leukocyte count 11.08 × 109/L, and platelet count 600 × 109/L. Blood urea was 39.2 mg/dL, serum creatinine 1.205 mg/dL, sodium 143 mmol/L, potassium 3.6 mmol/L, total protein 4.4 g/dL, albumin 1.7 g/dL, and daily urinary excretion of protein was 244 mg/kg/day; direct Coombs test was negative. Serological tests for antinuclear antibody and anti-double-stranded DNA were negative, as well as for cryoglobulinemia. Serum C3 and C4 levels were in normal range. Immunoglobulin electrophoresis, serum cholesterol, lactate dehydrogenase, angiotensin-converting enzyme, and α1-antitrypsin were normal. Tests for HCV (hepatitis C virus), HBV (hepatitis B virus), HIV (human immunodeficiency virus), and blood culture were negative. Kidney biopsy showed diffuse mesangial cell proliferation and thickening of the capillary walls with a double-contour appearance of the glomerular basement membranes. Immunofluorescence microscopy shows positive staining for IgM, IgA, C3, C1q, kappa light chains, and lambda light chains.
The diagnosis of IC-mediated MPGN was confirmed. She was then referred for a systematic ophthalmological examination. Her visual acuity was 20/20 in both eyes. A detailed slit-lamp examination showed bilateral posterior subcapsular yellowish lens deposits with a clear lens fiber behind [Figure 1] and ruled out the presence of anterior segment uveitis. The posterior segment was unremarkable, and macular optical coherence tomography showed the absence of vitritis and retinal deposit. Treatment with corticosteroids was then started with a partial response, and the ophthalmologic control examination showed the persistence of the same lens deposits. Immunosuppression therapy with mycophenolate mofetil was then started after 2 months of corticosteroids treatment, with complete renal remission. The control of her ophthalmological examination 10 months later showed a stable visual acuity at 20/20 in each eye and a significant decrease of the posterior subcapsular lens deposits in both eyes [Figure 2].
|Figure 1: (a) Right eye slit lamp photograph showing a posterior subcapsular lens deposit surrounded by a clear lens. (b) High magnification slit lamp photograph highlighting a yellowish rounded different sized deposits with well defined limits|
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|Figure 2: A high-magnification slit-lamp photograph showing a decrease in the number of the posterior subcapsular lens deposits becoming whitish, respectively, in the right eye (a) and in the left eye (b) 10 months after the beginning of the corticosteroid associated with the immunosuppressive therapy|
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| Discussion|| |
MPGN is a rare and progressive renal disease that can cause end-stage renal failure. It usually presents in early adulthood, and patients with DDD often develop drusen-like deposits in the second decade of life, but visual loss can also occur at older ages. The systemic nature of type II MPGN is illustrated by the fact that dense deposits on electron microscopy may also be found in the spleen, choriocapillaris, and Bruch's membrane of the eye, and by the fact that the disease has a tendency to recur in allografts., This could be explained by the fact that the eye is composed of different basement membranes (BMs) that are at the level of the cornea, the lens capsule, and the retina. The lens capsule BM has been identified as one of the thickest BM in the body. It is therefore very likely that deposits found on the glomerular BM could be found in one or more of these structures as well. The most frequent ocular manifestations reported are at the posterior pole, represented mainly by the drusens especially in complement-mediated MPGN., Association between drusen and DDD has been known for decades, but that with Ig-mediated MPGN has been very rarely reported. Anterior segment involvement is unusual; one case described a cloudy cornea and arcus juvenilis in a DDD due to dyslipidemia induced by the nephrotic syndrome related to the MPGN.
To the best of our knowledge, this is the second case report of ophthalmological involvements at the anterior segment in an Ig-mediated MPGN, but the first case report of capsular lens deposits in a child with Ig-mediated MPGN that responded to immunosuppressive treatment. All patients with complement-mediated MPGN should be examined by an ophthalmologist for a baseline dilated eye examination at the time of diagnosis. Although ophthalmologic involvement in Ig-mediated MPGN seems very rare with a much lower risk of vision loss than those with complement-mediated MPGN, ophthalmologists should continue to examine carefully all patients with MPGN for ophthalmologic pathologic features.
| Conclusion|| |
Although drusens are by far the most common ocular manifestation of MPGN reported mostly in older patients compared with our case, anterior segment examination should be considered because it may be possible to intervene and prevent deterioration of visual acuity.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]