Umbilical cord-derived mesenchymal stem cell implantation in patients with optic atrophy.

BACKGROUND: Optic nerve cells can be irreversibly damaged by common various causes. Unfortunately optic nerve and retinal ganglion cells have no regenerative capacity and undergo apoptosis in case of damage. In this study, our aim is to investigate the safety and efficacy of suprachoroidal umbilical cord-derived MSCs (UC-MSCs) implantation in patients with optic atrophy. METHODS: This study enrolled 29 eyes of 23 patients with optic atrophy who were followed in the ophthalmology department of our hospital. BCVA, anterior segment, fundus examination, color photography, and optical coherence tomography (OCT) were carried out at each visit. Fundus fluorescein angiography and visual field examination were performed at the end of the first, third, sixth months, and 1 year follow-up. RESULTS: After suprachoroidal UC-MSCs implantation there were statistically significant improvements in BCVA and VF results during 12 months follow-up (p < 0.05). When we evaluate the results of VF tests, the mean deviation (MD) value at baseline was -26.11 ± 8.36 (range -14.18 to -34.41). At the end of the first year it improved to -25.01 ± 8.73 (range -12.56 to -34.41) which was statistically significant (p < 0.05). When we evaluate the mean RNFL thickness measurements at baseline and at 12 month follow-up the results were 81.8 ± 24.9 µm and 76.6 ± 22.6 µm, respectively. There was not a significant difference between the mean values (p > 0.05). CONCLUSION: Stem cell treatment with suprachoroidal implantation of UCMSCs seems to be safe and effective in the treatment for optic nerve diseases that currently have no curative treatment options.

Six-month results of suprachoroidal adipose tissue-derived mesenchymal stem cell implantation in patients with optic atrophy: a phase 1/2 study.

PURPOSE: This prospective clinical case series aimed to investigate the safety and efficacy of suprachoroidal adipose tissue-derived mesenchymal stem cell (ADMSC) implantation in patients with optic nerve diseases. METHODS: This prospective, single-center, phase 1/2 study enrolled 4 eyes of 4 patients with optic atrophy of various reasons who underwent suprachoroidal implantation of ADMSCs. The best-corrected visual acuity (BCVA) in the study was HM at 1 m. The worse eye of the patient was operated. Patients were evaluated on the first day, first week, first month, third and sixth months postoperatively. BCVA, anterior segment and fundus examination, color photography, optical coherence tomography (OCT) and visual field examination were carried out at each visit. Fundus fluorescein angiography and multifocal electroretinography (mfERG) recordings were performed at the end of the first, third and sixth months and anytime if necessary during the follow-up. RESULTS: All 4 patients completed the six-month follow-up. None of them had any systemic or ocular complications. All of the patients experienced visual acuity improvement, visual field improvement and improvement in the mfERG recordings. We found choroidal thickening in OCT of the 4 patients. CONCLUSION: Even though the sample size is small, the improvements were still encouraging. Stem cell treatment with suprachoroidal implantation of ADMSCs seems to be safe and effective in the treatment for optic nerve diseases that currently have no curative treatment options.

Stem Cell Treatment in Retinal Diseases: Recent Developments.

Stem cells are undifferentiated cells which have the ability to self-renew and differentiate into mature cells. They are highly proliferative, implying that an unlimited number of mature cells can be generated from a given stem cell source. On this basis, stem cell replacement therapy has been evaluated in recent years as an alternative for various pathologies. Degenerative retinal diseases cause progressive visual decline which originates from continuing loss of photoreceptor cells and outer nuclear layers. Theoretically, this therapy will enable the generation of new retinal cells from stem cells to replace the damaged cells in the diseased retina. In addition, stem cells are able to perform multiple functions, such as immunoregulation, anti-apoptosis of neurons, and neurotrophin secretion. With recent progress in experimental stem cell applications, phase I/II clinical trials have been approved. These latest stem cell transplantation studies showed that this therapy is a promising approach to restore visual function in eyes with degenerative retinal diseases such as retinitis pigmentosa, Stargardts' macular dystrophy, and age-related macular degeneration. This review focuses on new developments in stem cell therapy for degenerative retinal diseases.

Suprachoroidal Adipose Tissue-Derived Mesenchymal Stem Cell Implantation in Patients with Dry-Type Age-Related Macular Degeneration and Stargardt's Macular Dystrophy: 6-Month Follow-Up Results of a Phase 2 Study.

This prospective clinical case series aimed to investigate the safety and efficacy of suprachoroidal adipose tissue-derived mesenchymal stem cell (ADMSC) implantation in patients with dry-type age-related macular degeneration (AMD) and Stargardt's macular dystrophy (SMD). This study included four patients with advanced-stage dry-type AMD and four patients with SMD who underwent suprachoroidal implantation of ADMSCs. The best-corrected visual acuity (BCVA) in the study was 20/200. The worse eye of the patient was operated on. Patients were evaluated on the first day, first week, and first, third, and sixth months postoperatively. BCVA, anterior segment and fundus examination, color photography, fundus autofluorescence, optical coherence tomography, and visual field examination were carried out at each visit. Fundus fluorescein angiography and multifocal electroretinography (mf-ERG) recordings were performed at the end of the first, third, and sixth months and anytime if necessary during the follow-up. All eight patients completed the sixth month follow-up. None of them had any systemic or ocular complications. All of the eight patients experienced visual acuity improvement, visual field improvement, and improvement in mf-ERG recordings. Stem cell treatment with suprachoroidal implantation of ADMSCs seems to be safe and effective in the treatment of dry-type AMD and SMD.

Subretinal adipose tissue-derived mesenchymal stem cell implantation in advanced stage retinitis pigmentosa: a phase I clinical safety study.

BACKGROUND: This prospective clinical case series aimed to investigate the safety of subretinal adipose tissue-derived mesenchymal stem cell (ADMSC) implantation in advanced stage retinitis pigmentosa (RP). METHODS: This study included 11 patients with end-stage RP who received subretinal implantation of ADMSCs. All patients had a total visual field defect and five of them only had light perception. The best corrected visual acuity (BCVA) in the study was 20/2000. All patients had undetectable electroretinography (ERG). The worst eye of the patient was operated on and, after total vitrectomy with a 23 gauge, ADMSCs were injected subretinally. Patients were evaluated at day 1, at weeks 1-4, and then once a month for 6 months, postoperatively. BCVA, anterior segment and fundus examination, color photography, and optical coherence tomography (OCT) were carried out at each visit. Fundus fluorescein angiography (FFA), perimetry, and ERG recordings were performed before treatment and at the end of month 6, and anytime if necessary during the follow-up. RESULTS: All 11 patients completed the 6-month follow-up. None of them had systemic complications. Five patients had no ocular complications. One of the patients experienced choroidal neovascular membrane (CNM) at the implantation site and received an intravitreal anti-vascular endothelial growth factor drug once. Five patients had epiretinal membrane around the transplantation area and at the periphery, and received a second vitrectomy and silicon oil injection. There was no statistically significant difference in BCVA and ERG recordings from baseline. Only one patient experienced an improvement in visual acuity (from 20/2000 to 20/200), visual field, and ERG. Three patients mentioned that the light and some colors were brighter than before and there was a slight improvement in BCVA. The remaining seven patients had no BCVA improvement (five of them only had light perception before surgery). CONCLUSIONS: Stem cell treatment with subretinal implantation of ADMSCs seems to have some ocular complications and should be applied with caution. The results of this study provide the first evidence of the short-term safety of ADMSCs in humans, and clarifies the complications of the therapy which would be beneficial for future studies. To optimize the cell delivery technique and to evaluate the effects of this therapy on visual acuity and the quality of life of these patients, future studies with a larger number of cases will be necessary.

Crescentic glomerulonephritis in children: a single centre experience.

BACKGROUND: Crescentic glomerulonephritis (CsGN) is characterized by crescents in 50% or more of glomeruli and clinically by a sudden and progressive decline in renal function. METHODS: We evaluated the etiology, clinical features, prognostic factors and long-term outcome of CsGN. Between January 2000 and December 2010, 45 children (26 girls, 19 boys) with biopsy-proven CsGN (>50% crescents) were investigated retrospectively. RESULTS: The mean age of the patients was 130.86±33.77 months. The mean duration of symptoms prior to diagnosis was 26±12 days (4-40 days). Most of the children had hypertension (62.2%), macroscopic hematuria (73.3%), oligoanuria (44.4%), edema (51.1%) and purpuric rash (40%) at presentation. The final clinical status of the patients was complete remission (n=21), partial remission (n=5) or chronic kidney disease (n=19). Adverse outcomes were significantly associated with a long duration between the onset of symptoms and treatment (P=0.038), the presence of oligoanuria (P=0.006), a severe decreased glomerular filtration rate (GFR <30 mL/min/1.73m²) and the need for dialysis (P=0.003) on admission, the ratio of crescents (>75%) (P=0.03), and the ratio of fibrous crescents (P=0.015). CONCLUSION: The outcome of CsGN in children continues to be poor, and it should be treated as a renal emergency.

Long-term prognosis of idiopathic nephrotic syndrome in children.

BACKGROUND: To investigate the demographic, clinical and laboratory data of the children with idiopathic nephrotic syndrome (INS), and to determine prognostic factors that affect the clinical outcome of the patients. METHODS: Medical charts of 372 patients diagnosed to have INS and followed up at least 5 years between January 1990 and December 2008 were evaluated, respectively. After initial demographic, clinical and laboratory findings of the patients were documented, therapeutic protocols, prognosis and prognostic factors were investigated. RESULTS: 299 of the patients (80.4%) were steroid responsive and 73 (19.6%) were not. Focal segmental glomerulosclerosis (FSGS) was observed in 57%, minimal change disease (MCD) in 20.6% and diffuse mesengial proliferation in 21.9% renal biopsy materials. Steroid sensitivity was higher in patients with MCD and under the age of five years. Resistance to steroids was higher in children with FSGS. Complete remission was achieved in 96% of patients who were sensitive to steroids and in 46.6% who were resistant. 15% of patients who were steroid resistant developed chronic kidney disease (CKD). CONCLUSION: Intercurrent infections and response to steroid therapy are the most important factors affecting the prognosis of the disease.

Effects of chronic smoking on color vision in young subjects.

AIM: To evaluate the effects of chronic smoking on color vision in young subjects. METHODS: This study included 91 smokers and 88 non-smokers (a total of 179 volunteers) without any ophthalmologic and systemical disorders. The subjects were between 18-40 years of age with a best corrected visual acuity (BCVA) of 20/20, normal anterior and posterior segment examinations and normal intraocular pressure. The color vision of the subjects were evaluated with Farnsworth-Munsell 100 Hue test (FMHT). The total error scores and axis calculation were performed for each subject and the results correlated. RESULTS: Mean age and the standard deviation was 28±5y in the smokers group, and 26.7±5.5y in the control group (P=0.101). Sex distribution was similar in the two groups (P=0.365). There was no significant correlation between age and FMHT total error scores (P=0.069). Median of FMHT total error scores of smokers and non-smokers were 65 and 50.50, respectively. FMHT total error scores was found significantly higher in smokers than non-smokers (P=0.004). There was no statisticaly significant difference between smoker and non-smoker groups with respect to axis ratio calculation (P=0.611). There was no significant correlation with FMHT total error scores with neither smoking duration nor number of cigarettes smoked per day (P=0.405, P=0.454, respectively). CONCLUSION: This study suggested that chronic smoking affects the color vision of young smokers but this may not be sector selective.

The relation between treatment and prognosis of childhood membranoproliferative glomerulonephritis.

BACKGROUND: The prognostic factors, the outcome and the most favorable treatment regimen are not entirely known for children with membranoproliferative glomerulonephritis (MPGN). MPGN is a rarely observed disease more prevalent in adolescents, so we aimed to review the clinical and histological properties, treatments and the outcome of our patients who were diagnosed as MPGN. METHODS: Fifty-one children - diagnosed with MPGN - were selected from biopsy records in Dr. Sami Ulus Maternity and Children's Hospital Pediatric Nephrology Department from January 1999 to January 2011. A retrospective analysis was made of 33 regularly followed children. RESULTS: Thirty-three patients were identified, 13 female and 20 male. Their age groups at presentation ranged from 4 to 15 years. The following duration was 26-144 months (mean 74). Following the initial treatment, 20 (60%) patients achieved complete remission. Six patients with nephrotic syndrome and one with non-nephrotic proteinuria showed partial remission. The condition of one patient with nephrotic syndrome was unchanged with the persisting symptoms. The one patient with nephrotic syndrome and four others with non-nephrotic proteinuria did not respond to initial treatment as their renal functions decreased gradually. CONCLUSION: We concluded that only degree of tubulointerstitial damage on the initial biopsy is determinative for prognosis of childhood MPGN. If the patient receives high doses of steroid therapy in the early stages, their treatment is more likely to be successful. The effect of immunosuppressive treatment on MPGN is not clear.

Overexpression of vascular endothelial growth factor receptor 2 in pterygia may have a predictive value for a higher postoperative recurrence rate.

BACKGROUND: To investigate the expression levels of vascular endothelial growth factor (VEGF), vascular endothelial growth factor receptor 1 (VEGFR-1) and VEGFR-2 in pterygium and to clarify the prognostic significance of these expressions in pterygia. METHODS: A total of 40 surgically excised pterygia and 9 normal conjunctivae were immunohistochemically studied applying the streptavidin-biotin method in paraffin-embedded tissue sections. Monoclonal antibodies were targeted against VEGF, VEGFR-1 and VEGFR-2 proteins. At the sixth postoperative month, the recurrence rate was graded on a scale of 1-4. RESULTS: The mean percentage of VEGF-positive epithelial cells was comparable in pterygium and normal conjunctivae. However, the pterygium group presented higher expression levels of VEGF in pterygia endothelial cells (p=0.05). In terms of VEGFR-1 expression in epithelial cells, no statistically significant difference was found between two groups (p=0.658). However, normal conjunctivae exhibited higher expression levels of VEGFR-1 in endothelial cells (p=0.002). Epithelial cells in pterygium presented higher combined scores of VEGFR-2 (87.5% and 22.2%, respectively) (p=0.013). While higher expression levels of VEGFR-2 were documented in pterygia endothelial cells, no VEGFR-2 immunoreactivity was observed in the endothelial cells of normal conjunctivae (p<0.001). Expression levels of VEGFR-2 in epithelial cells and endothelial cells were positively correlated with the postoperative recurrence grading system (p<0.001 and τ=0.627, p=0.001 and τ=0.508, respectively). CONCLUSIONS: The results suggest that VEGF may play a key role through VEGFR-2 in the pathogenesis of pterygium. Moreover, overexpression of VEGFR-2 in pterygia may have a predictive value for a higher postoperative recurrence rate.

Obstructive sleep apnoea prevalence in non-arteritic anterior ischaemic optic neuropathy.

AIMS: The aim of this study was to show the prevalence of obstructive sleep apnoea (OSA) in non-arteritic anterior ischaemic optic neuropathy (NAION). METHODS: 20 patients diagnosed with NAION were included in the study. 20 age and sex matched subjects with similar risk factors for NAION, such as diabetes mellitus (DM) and hypertension (HT), constituted the control group. All cases underwent polysomnography for investigation of the presence of OSA. Cases with an Apnoea-Hypopnoea Index >5 were accepted as having OSA. RESULTS: Mean ages of the patients and controls were 60.90±8.14 and 61.15±7.23 years, respectively. There were no significant differences between the patient and control groups in terms of age, gender, body mass index, smoking/alcohol consumption or systemic diseases. In the patient group, 85% were diagnosed with OSA compared with 65% in the control group (p>0.05). CONCLUSIONS: We found a high prevalence of OSA in patients with NAION but it was also high in the control group (p>0.05). This may be due to the fact that the two groups were matched for the same risk factors for NAION. The study indicates that OSA is not a risk factor for NAION in itself but is the contributing factor as it has effects on the vascular endothelium in DM, HT and atherosclerosis.

Orbital apex syndrome in herpes zoster ophthalmicus.

Orbital apex syndrome is a rare manifestation of Herpes Zoster Ophthalmicus. Herein we report on a case of orbital apex syndrome secondary to Herpes Zoster Ophthalmicus. A 75 year-old male complained of vision loss, conjunctival hyperemia and proptosis on the left eye, was referred to our clinic. Visual acuity was 5/10 Snellen lines and he had conjunctival hyperemia, chemosis, minimal nuclear cataract and proptosis on the left eye. A diagnosis of orbital pseudotumor was demonstrated firstly. The patient received oral and topical corticosteroids, antiinflammatory and antibiotic agents. On day 2, vesiculopustular lesions were observed, Herpes Zoster Ophthalmicus was diagnosed and corticosteroid treatment stopped, oral acyclovir treatment initiated. Two days later, total ophthalmoplegia, ptosis and significant visual loss were observed on the left. The diagnosis of orbital apex syndrome was considered and the patient commenced on an intravenous acyclovir treatment. After the improvement of acute symptoms, a tapering dose of oral cortisone treatment initiated to accelarate the recovery of ophthalmoplegia. At 5-month follow-up, ptosis and ocular motility showed improvement. VA did not significantly improve because of cataract and choroidal detachment on the left. We conclude that ophthalmoplegia secondary to Herpes Zoster Ophthalmicus responds favourably to intravenous acyclovir and steroids.

The change in ocular higher-order aberrations after pterygium excision with conjunctival autograft: a 1-year prospective clinical trial.

PURPOSE: To investigate the effects of pterygium excision with conjunctival autograft plus corneal polishing on ocular higher-order aberrations (HOAs) and to monitor the changes throughout the first postoperative year. METHODS: A total of 18 eyes of 16 patients with primary pterygium who had an indication for surgical excision were included in the study. All patients underwent a detailed ophthalmologic examination preoperatively and at the third and twelfth postoperative months. Pterygium excision with conjunctival autograft plus corneal polishing was performed by the same surgeon (K.G.). Corneal topography and ocular HOAs were measured by NIDEK optical path difference scanning system ARK-10000 refractive power/corneal analyzer. RESULTS: Root mean square values of total aberrations and total HOAs were found to be significantly decreased both at 3 and 12 months after an uneventful pterygium surgery. Although solely root mean square values of coma and trefoil revealed a statistically significant reduction in the third postoperative month (P=0.043 and P=0.018, respectively), after 1 year, all parameters (total coma: P<0.001; total trefoil: P=0.004; total quatrefoil: P=0.010; and total high astigmatism: P=0.016) except for total spherical aberration (P=0.236) were found to be lower when compared with the preoperative values. CONCLUSION: Uneventful pterygium excision with conjunctival autograft plus corneal polishing can significantly reduce most of the pterygium-induced HOAs. Moreover, the reduced postoperative aberrations continue to change beyond the early postoperative period even into the 1-year postoperative period. Therefore, any refractive procedures should be postponed at least 1 year after the pterygium surgery until corneal stability occurs.

Respiratory-chain deficiency presenting as diffuse mesangial sclerosis with NPHS3 mutation.

Renal manifestations of mitochondrial cytopathies have been described, but nephrotic syndrome with respiratory-chain disorders have been described extremely rarely. We report a 9-month-old boy with a mitochondrial cytopathy preceded by a 2-month history of steroid-resistant nephrotic syndrome. Percutaneous renal biopsy revealed diffuse mesangial sclerosis, and mutational analysis was compatible with PLCE1 mutation. However, electron microscopic findings of renal tissue, sensorineural hearing loss, and other ocular and neurologic findings led us to suspect mitochondrial cytopathy. Muscle tissue analysis showed a deficiency of the respiratory chain complex IV. The clinical presentation of our patient is not typical for primary cytochrome oxidase (COX) deficiency but showed similarities with patients carrying AR mutations in COX10. This was the first case in the literature with both PLCE1 mutation and COX deficiency. We could not identify pathogenic mutations in the COX10 gene, suggesting that PLCE1 deficiency could be the cause of the secondary deficiency of COX. Another, more likely, possibility is that the mitochondriopathy phenotype is caused by another mutation homozygous by descent in a yet unidentified recessive gene.

[Evaluation of visual functions in elderly patients with femoral neck fracture]. / Femur boyun kirigi olan ileri yas olgularda görsel fonksiyonlarin degerlendirilmesi.

OBJECTIVES: We aimed at assessing the visual functions in elderly patients with femoral neck fracture and to compare the results with age-matched controls in this three-year prospective study. PATIENTS AND METHODS: Seventy-one patients with a history of fall related hip fracture (39 females, 32 males; mean age 76.3+/-9.7 years; range 64 to 90 years) and who were diagnosed with femoral neck fracture after direct graphy were treated by means of bipolar partial prosthesis and they were contacted postoperatively or prior to discharge to participate in the study. Visual acuity, depth perception, the presence of cataract in the red reflex were evaluated. A dilated fundus and slit-lamp examination were performed if possible. On completion of the examination, the ophthalmologist documented the causes of any visual impairment found. Control group was comprised of age-matched 40 subjects (22 females, 18 males; mean age 73.2+/-7.6 years; range 62 to 90 years) who applied to ophtalmology clinic for routine examination. RESULTS: The visual acuity was significantly decreased in the patient group as was stereopsis (p<0.05). We found no difference between the study group and the controls when we evaluate the distribution of self reported eye disease and eye disease found on ocular examination. The rate of cases who reported not usually wearing glasses was 35% while it was 5% in the control group. When we evaluate the time since last examination, 38% of cases had not had an eye examination for over four years, as compared with 22.5% of controls. CONCLUSION: This study shows that elderly people should have their eyes tested at least once every two years, refractive errors should be corrected and eye diseases should be treated to decrease the risk of fall-related femoral neck fractures.

Evaluation of ocular surface damage and dry eye status in chronic hepatitis C at different stages of hepatic fibrosis.

PURPOSE: The purpose of this study was to explore changes in ocular surface and tear function parameters in chronic hepatitis C at different stages of hepatic fibrosis. METHODS: Fifty-four patients with biopsy-proven chronic hepatitis C and 54 age- and sex-matched healthy control subjects without systemic hepatitis C infection were examined with the Ocular Surface Disease Index questionnaire, Schirmer with and without anesthesia, tear film breakup time, and scoring of ocular surface Lissamine green staining using modified Oxford and van Bijsterveld scoring systems and corneal fluorescein staining. RESULTS: Patients with chronic hepatitis C scored significantly worse than the control subjects on all parameters: modified Oxford scores of Lissamine green staining (5.5/3.0; P <0.001), Oxford and van Bijsterveld scores (4.0/2.0; P <0.001), and corneal fluorescein staining (1.5/0.0; P = 0.001). The chronic hepatitis C group also had higher Ocular Surface Disease Index scores than the control subjects (22.3/13.7; P = 0.001). Schirmer with and without anesthesia and tear film breakup time scores were found to be lower in patients with chronic hepatitis C (P <0.001). Moreover, patients with advanced stages of hepatic fibrosis (stages 4-6) had significantly lower values of tear film breakup time and worse Ocular Surface Disease Index scores and ocular surface vital dye staining than those with initial stages of hepatic fibrosis (stages 0-3). CONCLUSION: Patients with chronic hepatitis C, especially those with advanced stages of hepatic fibrosis, were more likely to exhibit severe ocular surface damage and signs of dry eye.

Pattern electroretinographic results after photodynamic therapy alone and photodynamic therapy in combination with intravitreal bevacizumab for choroidal neovascularization in age-related macular degeneration.

PURPOSE: To evaluate the changes in pattern electroretinography (PERG) 1 month after photodynamic therapy alone and photodynamic therapy in combination with intravitreal bevacizumab for choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD). METHODS: This is a prospective series of 45 eyes with subfoveal CNV secondary to AMD. Twenty eyes were treated with photodynamic therapy (PDT) with verteporfin and 1.25 mg of intravitreal bevacizumab, and 25 patients were treated with PDT alone. Visual acuities and serial PERG recordings were performed both before and 1 month after therapy. RESULTS: Following the 1-month therapy period, visual acuity improved in 56% of patients in the PDT group and 76% of patients in the combination group. No significant ocular or systemic adverse effects were observed in either group. According to the PERG results, the mean P50 amplitude was 1.5 +/- 0.9 microV before PDT and improved to 2.1 +/- 1.1 microV at 1 month after PDT. The mean P50 amplitudes in the combination therapy group before and after therapy were 1.6 +/- 0.8 microV and 2.7 +/- 1.2 microV, respectively, and the difference was statistically significant between the groups. CONCLUSIONS: In this small series of eyes with limited follow-up, the combined treatment of PDT with verteporfin and intravitreal bevacizumab seems to be associated with improvement in VA and pattern electroretinographic results when compared to those in the PDT group.

Is the urinary protein excretion pattern compatible with renal morphological findings in renal amyloidosis?

The aims of this study are to compare urinary protein excretion pattern with renal morphological findings and to find out whether urinary protein excretion pattern is a prognostic indicator of renal amyloidosis. Fifteen children with renal amyloidosis secondary to familial Mediterranean fever were included in the study. The patients were classified into three groups according to the degree of tubulointerstitial injury in renal biopsy (group 1, <25%; group 2, 25-50%; and group 3, >50%). In all patients, urinary protein electrophoresis were performed. Levels of urinary beta(2)-microglobulin, retinol binding protein, and beta.N-acetyl-D glucosaminidase were measured as markers for tubular injury, and urinary excretions of protein and albumin and plasma albumin levels were measured as markers of glomerular injury. While urinary excretions of protein and albumin and plasma albumin levels were not different between groups, higher urinary beta(2-)microglobulin and retinol binding protein values and lower creatinine clearance values were found in group 3 than in groups 1 and 2 (p < 0.05). We concluded that analysis of urinary protein excretion pattern is a non-invasive and reliable method to detect the degree of tubulointerstitial injury as the most important prognostic factor in renal amyloidosis and may be used to determine the changes during the follow-up period of the patients.