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urea 51mg&#47;dl&#59; uric acid 2&#46;1mg&#47;dl&#59; phosphorus 4&#46;4mg&#47;dl&#59; mild metabolic acidosis with C-reactive protein 29mg&#47;l and globular sedimentation velocity 62mm &#40;Table 1&#41;&#46;</p><p class="elsevierStylePara">The urine analysis showed glycosuria &#40;0&#46;8g&#47;l&#41;&#59; non-nephrotic range proteinuria &#40;13mg&#47;kg&#47;day&#44; microalbuminuria 148mg&#47;g Cr&#41;&#59; hyperphosphaturia with low tubular phosphate reabsorption &#40;78&#37;&#41;&#59; hyperuricosuria with a high uric acid index &#40;0&#46;78 100mg&#47;100ml&#41;&#59; normal glomerular filtration and calcium levels &#40;1&#46;1mg&#47;kg&#47;day&#41;&#59; fractional excretion of sodium 0&#46;3&#37;&#44; and fractional excretion of potassium 19&#37;&#46; Urinary sediment showed granular and hyaline casts&#44; leukocyturia without eosinophils and a negative urine culture&#46;</p><p class="elsevierStylePara">These findings are compatible with multiple dysfunctions of the proximal tubule &#40;PT&#41;&#44; or Fanconi syndrome&#46;</p><p class="elsevierStylePara">Liver&#44; thyroid and parathyroid functions were normal&#46; The immunological study revealed low-level positivity for antinuclear antibodies &#40;1&#47;80&#41;&#46; The anti-streptolysin O &#40;ASLO&#41; titre was high &#40;2175IU&#47;ml&#41; with a negative oropharyngeal culture&#46; Immunoglobulin levels were normal&#44; but IgG was high 1 week later &#40;1820mg&#47;dl&#41;&#46; An IgM test was run for Epstein-Barr&#44; cytomegalovirus&#44; parvovirus B19&#44; toxoplasmosis and hepatitis B and C&#46; All were negative&#46; Renal ultrasound was normal&#46;</p><p class="elsevierStylePara">Given the PT dysfunction and mild renal failure&#44; we suspected a case of TIN&#46; We adopted a watchful waiting approach and kidney function and blood pressure normalised in less than 4 weeks&#46; No kidney biopsy was performed &#40;Figure 1&#41;&#46;</p><p class="elsevierStylePara">One month after renal symptoms began&#44; he presented with acute anterior uveitis&#44; which evolved favourably under treatment with topical steroids and cycloplegic agents&#46;</p><p class="elsevierStylePara">According to Mandeville&#8217;s criteria&#44;<span class="elsevierStyleSup">1</span> we diagnosed tubulointerstitial nephritis associated with uveitis &#40;TINU&#41;&#46; After 18 months&#44; renal function remains normal&#44; although there have been various relapses of uveitis&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The aetiology and pathogenesis of this disease are unknown&#44; but hypotheses suggest mechanisms mediated by cellular immunity&#46; It has been linked to infections&#44; systemic diseases and previous use of medications&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">In our case&#44; the high ASLO titres suggested a streptococcus infection&#44; but there were no associated symptoms and the oropharyngeal culture was negative&#46; It seems unlikely to have originated with the desmopressin as no causal relationship has been established&#46; We have not found any cases linking TIN to the use of that drug&#46;</p><p class="elsevierStylePara">Clinical manifestations are variable&#44; with non-specific systemic symptoms and renal symptoms that include flank pain&#44; sterile pyuria&#44; haematuria&#44; non-nephrotic range proteinuria and different stages of renal failure&#46; Proximal and distal tubular defects may also be associated&#46;</p><p class="elsevierStylePara">Our patient presented glycosuria with general PT dysfunction&#44; moderate renal dysfunction and mild hypertension&#46;</p><p class="elsevierStylePara">We diagnosed TINU syndrome according to Mandeville&#39;s criteria&#46;<span class="elsevierStyleSup">1</span> Differential diagnosis was performed to rule out other entities involving both renal and ocular disease&#44;<span class="elsevierStyleSup">2</span> such as sarcoidosis&#44; Sj&#246;gren&#39;s syndrome&#44; Wegener&#39;s granulomatosis&#44; Beh&#231;et&#8217;s disease&#44; toxoplasmosis&#44; tuberculosis&#44; histoplasmosis and brucellosis&#46;</p><p class="elsevierStylePara">Renal dysfunction tends to be self-limiting&#44;<span class="elsevierStyleSup">3</span> but becomes persistent in some 10&#37; of patients&#46; Oral steroids are the treatment of choice&#44;<span class="elsevierStyleSup">4</span> although immunomodulators may also be used in patient with a poor response&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">Uveitis may develop prior to &#40;in 20&#37;&#41; or after &#40;in 65&#37;&#41; renal symptoms&#44; and is rarely concomitant&#46;<span class="elsevierStyleBold"> </span>Cases of uveitis presenting as much as 2 months before or 14 months after renal symptoms have been observed&#46;<span class="elsevierStyleSup">2</span> Uveitis generally has a favourable prognosis&#44; but some articles report complications in up to 20&#37; of patients<span class="elsevierStyleSup">2</span> and relapses are frequent&#46;</p><p class="elsevierStylePara">Since the manifestations of TINU syndrome are non-specific&#44; its true incidence rate may be underestimated&#46; Both nephrologists and ophthalmologists must be aware of this entity and check renal function in patients with uveitis &#40;and vice versa&#41;&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The authors affirm that they have no conflicts of interest related to the content of this article&#46;</p><p class="elsevierStylePara"><a href="grande&#47;11238&#95;19157&#95;29365&#95;en&#95;t1&#95;&#46;11238&#46;jpg" class="elsevierStyleCrossRefs"><img src="11238_19157_29365_en_t1_.11238.jpg" alt="Laboratory blood and urine analyses at onset of symptoms and during syndrome progression "></img></a></p><p class="elsevierStylePara">Table 1&#46; Laboratory blood and urine analyses at onset of symptoms and during syndrome progression </p><p class="elsevierStylePara"><a href="grande&#47;11238&#95;19157&#95;29366&#95;en&#95;f1&#95;&#46;11238&#46;jpg" class="elsevierStyleCrossRefs"><img src="11238_19157_29366_en_f1_.11238.jpg" alt="Creatinine clearance&#44; microalbuminuria&#44; tubular reabsorption of phosphate and glycosuria"></img></a></p><p class="elsevierStylePara">Figure 1&#46; Creatinine clearance&#44; microalbuminuria&#44; tubular reabsorption of phosphate and glycosuria</p>"
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Tubulo-interstitial nephritis and uveitis with Fanconi syndrome
Nefritis del túbulo intersticial y uveítis con síndrome de Fanconi
Belén Llorente-Gómeza, Cristina González-Menchéna, Carmen de Lucas-Collantesa
a Departamento de Pediatría, Hospital Universitario Puerta de Hierro, Madrid,
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urea 51mg&#47;dl&#59; uric acid 2&#46;1mg&#47;dl&#59; phosphorus 4&#46;4mg&#47;dl&#59; mild metabolic acidosis with C-reactive protein 29mg&#47;l and globular sedimentation velocity 62mm &#40;Table 1&#41;&#46;</p><p class="elsevierStylePara">The urine analysis showed glycosuria &#40;0&#46;8g&#47;l&#41;&#59; non-nephrotic range proteinuria &#40;13mg&#47;kg&#47;day&#44; microalbuminuria 148mg&#47;g Cr&#41;&#59; hyperphosphaturia with low tubular phosphate reabsorption &#40;78&#37;&#41;&#59; hyperuricosuria with a high uric acid index &#40;0&#46;78 100mg&#47;100ml&#41;&#59; normal glomerular filtration and calcium levels &#40;1&#46;1mg&#47;kg&#47;day&#41;&#59; fractional excretion of sodium 0&#46;3&#37;&#44; and fractional excretion of potassium 19&#37;&#46; Urinary sediment showed granular and hyaline casts&#44; leukocyturia without eosinophils and a negative urine culture&#46;</p><p class="elsevierStylePara">These findings are compatible with multiple dysfunctions of the proximal tubule &#40;PT&#41;&#44; or Fanconi syndrome&#46;</p><p class="elsevierStylePara">Liver&#44; thyroid and parathyroid functions were normal&#46; The immunological study revealed low-level positivity for antinuclear antibodies &#40;1&#47;80&#41;&#46; The anti-streptolysin O &#40;ASLO&#41; titre was high &#40;2175IU&#47;ml&#41; with a negative oropharyngeal culture&#46; Immunoglobulin levels were normal&#44; but IgG was high 1 week later &#40;1820mg&#47;dl&#41;&#46; An IgM test was run for Epstein-Barr&#44; cytomegalovirus&#44; parvovirus B19&#44; toxoplasmosis and hepatitis B and C&#46; All were negative&#46; Renal ultrasound was normal&#46;</p><p class="elsevierStylePara">Given the PT dysfunction and mild renal failure&#44; we suspected a case of TIN&#46; We adopted a watchful waiting approach and kidney function and blood pressure normalised in less than 4 weeks&#46; No kidney biopsy was performed &#40;Figure 1&#41;&#46;</p><p class="elsevierStylePara">One month after renal symptoms began&#44; he presented with acute anterior uveitis&#44; which evolved favourably under treatment with topical steroids and cycloplegic agents&#46;</p><p class="elsevierStylePara">According to Mandeville&#8217;s criteria&#44;<span class="elsevierStyleSup">1</span> we diagnosed tubulointerstitial nephritis associated with uveitis &#40;TINU&#41;&#46; After 18 months&#44; renal function remains normal&#44; although there have been various relapses of uveitis&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The aetiology and pathogenesis of this disease are unknown&#44; but hypotheses suggest mechanisms mediated by cellular immunity&#46; It has been linked to infections&#44; systemic diseases and previous use of medications&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">In our case&#44; the high ASLO titres suggested a streptococcus infection&#44; but there were no associated symptoms and the oropharyngeal culture was negative&#46; It seems unlikely to have originated with the desmopressin as no causal relationship has been established&#46; We have not found any cases linking TIN to the use of that drug&#46;</p><p class="elsevierStylePara">Clinical manifestations are variable&#44; with non-specific systemic symptoms and renal symptoms that include flank pain&#44; sterile pyuria&#44; haematuria&#44; non-nephrotic range proteinuria and different stages of renal failure&#46; Proximal and distal tubular defects may also be associated&#46;</p><p class="elsevierStylePara">Our patient presented glycosuria with general PT dysfunction&#44; moderate renal dysfunction and mild hypertension&#46;</p><p class="elsevierStylePara">We diagnosed TINU syndrome according to Mandeville&#39;s criteria&#46;<span class="elsevierStyleSup">1</span> Differential diagnosis was performed to rule out other entities involving both renal and ocular disease&#44;<span class="elsevierStyleSup">2</span> such as sarcoidosis&#44; Sj&#246;gren&#39;s syndrome&#44; Wegener&#39;s granulomatosis&#44; Beh&#231;et&#8217;s disease&#44; toxoplasmosis&#44; tuberculosis&#44; histoplasmosis and brucellosis&#46;</p><p class="elsevierStylePara">Renal dysfunction tends to be self-limiting&#44;<span class="elsevierStyleSup">3</span> but becomes persistent in some 10&#37; of patients&#46; Oral steroids are the treatment of choice&#44;<span class="elsevierStyleSup">4</span> although immunomodulators may also be used in patient with a poor response&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">Uveitis may develop prior to &#40;in 20&#37;&#41; or after &#40;in 65&#37;&#41; renal symptoms&#44; and is rarely concomitant&#46;<span class="elsevierStyleBold"> </span>Cases of uveitis presenting as much as 2 months before or 14 months after renal symptoms have been observed&#46;<span class="elsevierStyleSup">2</span> Uveitis generally has a favourable prognosis&#44; but some articles report complications in up to 20&#37; of patients<span class="elsevierStyleSup">2</span> and relapses are frequent&#46;</p><p class="elsevierStylePara">Since the manifestations of TINU syndrome are non-specific&#44; its true incidence rate may be underestimated&#46; Both nephrologists and ophthalmologists must be aware of this entity and check renal function in patients with uveitis &#40;and vice versa&#41;&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The authors affirm that they have no conflicts of interest related to the content of this article&#46;</p><p class="elsevierStylePara"><a href="grande&#47;11238&#95;19157&#95;29365&#95;en&#95;t1&#95;&#46;11238&#46;jpg" class="elsevierStyleCrossRefs"><img src="11238_19157_29365_en_t1_.11238.jpg" alt="Laboratory blood and urine analyses at onset of symptoms and during syndrome progression "></img></a></p><p class="elsevierStylePara">Table 1&#46; Laboratory blood and urine analyses at onset of symptoms and during syndrome progression </p><p class="elsevierStylePara"><a href="grande&#47;11238&#95;19157&#95;29366&#95;en&#95;f1&#95;&#46;11238&#46;jpg" class="elsevierStyleCrossRefs"><img src="11238_19157_29366_en_f1_.11238.jpg" alt="Creatinine clearance&#44; microalbuminuria&#44; tubular reabsorption of phosphate and glycosuria"></img></a></p><p class="elsevierStylePara">Figure 1&#46; Creatinine clearance&#44; microalbuminuria&#44; tubular reabsorption of phosphate and glycosuria</p>"
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