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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">Dear Editor&#44;</span></p><p class="elsevierStylePara">Focal segmental glomerulosclerosis &#40;FSGS&#41; is characterised by the presence of nephrotic syndrome&#44; hypertension and progressive deterioration of kidney function&#46; Although in many cases its aetiology is unknown&#44; it has been associated with inherited disorders&#44; viral infections&#44; induced by toxic substances and with hyperfiltration situations&#46;<span class="elsevierStyleSup">1</span></p><p class="elsevierStylePara">Polycythaemia vera &#40;PV&#41; is a myeloproliferative disorder of unknown aetiology characterised by excessive production of normal erythrocytes&#44; leukocytes and platelets&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">Glomerular involvement in PV is rare&#46; We present a patient diagnosed with PV with nephrotic syndrome secondary to GSF and progressive kidney disease&#46;</p><p class="elsevierStylePara">An 83 year-old woman&#44; diagnosed with PV 4 years earlier&#44; was admitted for the study of nephrotic syndrome and kidney disease of 2 years evolution&#46;</p><p class="elsevierStylePara">There was no family history of polycythaemia&#46; Among her background was&#58; left nephrectomy due to hypernephroma at 63 years&#44; bronchiectasis with recurrent bacterial superinfections&#44; hypertension controlled with medication and celiac disease well controlled through diet&#46; Four years earlier she was diagnosed with PV after a bone marrow biopsy&#44; including details of polycythaemia and thrombocytosis&#44; well controlled with hydroxyurea treatment &#40;500mg&#47;day&#41;&#46;</p><p class="elsevierStylePara">In March 2007&#44; she began with nephrotic range proteinuria &#40;4&#46;2g&#47;day&#41; with Crs 1&#46;1mg&#47;dL&#59; antiproteinuric treatment was started with telmisartan 80mg&#47;day and spironolactone 50mg&#47;day&#46; A month later the proteinuria dropped to 1&#46;2g&#47;day with no change in the creatinine&#46; She was admitted to hospital in June 2007 for severe hyponatraemia &#40;107mEq&#47;L&#41;&#44; symptomatic&#44; with hyperkalaemia &#40;5&#46;7mEq&#47;L&#41; and metabolic acidosis &#40;pH 7&#46;34&#41; secondary to treatment with spironolactone&#46; Due to the persistence of nephrotic proteinuria and her history of bronchiectasis&#44; a biopsy of rectal and abdominal fat was performed which discarded the existence of amyloidosis&#46; Throughout its evolution&#44; the proteinuria varied between 4-10g&#47;day and started with lower limb oedema&#44; decreased total protein and albumin &#40;5&#46;4&#47;2&#46;7g&#47;dL&#41; and a progressive decline in the glomerular filtration rate &#40;Crs 1&#46;6-1&#46;7mg&#47;dL&#41;&#46;</p><p class="elsevierStylePara">In May 2009 she was admitted to hospital due to a deterioration in her general condition&#44; oedema&#44; Crs 3&#46;6mg&#47;dL and proteinuria 8&#46;4g&#47;day despite treatment with ARB&#46; On&#160; admission&#44; her blood pressure was 137&#47;82mmHg&#59; in the physical examination her systolic ejection murmur was heard II&#47;VI in the left sternal border&#44; and she had bilateral pitting oedema up to the root of her thigh&#46; CBC&#58; haemoglobin 16&#46;8g&#47;dL&#44; haematocrit 56&#37;&#44; RBC 6&#44;750&#44;000&#47;&#181;L&#44; 11&#44;690&#47;&#181;l leukocytes with normal formula and platelets 460&#44;000&#47;&#181;l&#46; Crs 4&#46;3&#44; urea 102 &#40;mg&#47;dL&#41;&#46; Total protein 5&#46;9&#44; albumin 2&#46;5g&#47;dL&#44; cholesterol 188&#44; triglycerides 260&#44; uric acid 9&#46;8mg&#47;dL&#46; Proteinuria 6&#46;9g&#47;d&#59; 1&#46;4 sediment erythrocytes&#47;field and 5-10 leukocytes&#47;field&#46; Immunology&#58; ANA&#44; anti-DNA&#44; ENA&#44; ANCA and anti-GBM negative&#46; Complement&#58; C3 79&#44; C4 30mg&#47;dL&#46; CRP 0&#46;37mg&#47;dL&#46; Rheumatoid factor negative&#46; IgG 820&#44; IgA 185&#44; IgM 188mg&#47;dL&#46; Kappa 635&#44; lambda 515mg&#47;dL&#46; Kidney biopsy was performed with 8 glomeruli of which two were completely sclerosed&#44; and the remaining six&#44; one global mesangial expansion with increased mesangial cells could be seen and the other five had segmental proliferative lesions without necrosis accompanied by moderate epithelial proliferation&#46; The interstitium showed moderate fibrosis with tubular atrophy and occasional chronic inflammatory infiltrates&#46; Arterial and arteriolar vessels with hyperplastic lesions with occasional hyaline lesions&#46;&#160; These findings were indicative of <span class="elsevierStyleItalic">cellular variant segmental proliferative glomerulonephritis</span>&#46;</p><p class="elsevierStylePara">Treatment was initiated with three shocks of 125mg of 6-methylprednisolone followed by prednisone &#40;1mg&#47;kg&#47;day&#41; and mycophenolate mofetil &#40;360mg&#47;12 hours&#41;&#46; No evidence of a decrease in proteinuria was seen and kidney function deteriorated progressively with clinical uraemia&#46; So it was decided to make the right jugular catheter permanent and initiate treatment with periodic haemodialysis&#46; A progressive improvement in the symptoms was&#160; seen&#46; Treatment with mycophenolate mofetil was discontinued and prednisone was gradually withdrawn&#46;</p><p class="elsevierStylePara">The patient developed a nephrotic syndrome secondary to FSGS 4 years after detection of the polycythaemia&#46; It met the WHO criteria for diagnosis of PV2 &#40;Hb greater than 16&#46;5g&#47;dL in women&#44; no cases of secondary polycythaemia&#44; decreased EPO levels&#44; splenomegaly&#44; thrombocytosis &#62; 400&#44;000&#47;&#181;l and leukocytosis &#62; 12&#44;000&#47;&#181;L&#41;&#46; Despite good control of the PV with hydroxyurea and treatment with high dose steroids and immunosuppressive agents&#44; the nephrotic syndrome did not improve&#44; and was accompanied by a progressive deterioration of kidney function with uremic symptoms and initiation of replacement therapy&#46;</p><p class="elsevierStylePara">The kidney biopsy showed the existence of a focal segmental glomerulosclerosis cell variant that had a worse prognosis than the other FSGS &#40;perihilar hyalinosis&#44; tip variant and classical variant&#41; and better than collapsing variant&#46;<span class="elsevierStyleSup">3</span></p><p class="elsevierStylePara">The joint presence of FSGS and PV is a rare combination&#46; There have only been 6 published cases confirmed histologically in the literature&#46;<span class="elsevierStyleSup">4-8 </span>Table 1 summarises the clinical data of the 6 cases reported and of the current one&#46;</p><p class="elsevierStylePara">From this we can get some interesting data&#46; The average age at the time of kidney biopsy was 60&#46;3 years &#40;range 41-83&#41;&#46; FSGS was diagnosed up to 4 years after the detection of polycythaemia in 5 of the 7 cases &#40;71&#46;4&#37;&#41;&#46; Four patients &#40;57&#46;1&#37;&#41; developed nephrotic range proteinuria&#44; 3 patients were treated with steroids and immunosuppressive agents&#44; of whom only one had improvement of kidney function&#46; The other two were given haemodialysis due to progressive deterioration of plasma creatinine with onset or worsening of uremic symptoms&#46;</p><p class="elsevierStylePara">The presence of FSGS in these patients could be directly related to the PV&#46; As noted by Sharma et al&#44;<span class="elsevierStyleSup">4</span> the haemodynamic changes in the PV&#44; as well as kidney vasodilation and increased effective kidney blood flow could trigger kidney glomerulosclerosis&#46; Ferrario et al<span class="elsevierStyleSup">9</span> suggested that the haemodynamic glomerular injury induces the activation of the mesangial cells&#44; leading to overproduction of extracellular matrix&#46; Furthermore&#44; damaged endothelial cells facilitate platelet activation by releasing platelet activating factor&#44; which induces proliferation of endothelial and mesangial cells&#46; These alterations may contribute to the development of glomerular sclerotic changes&#46;</p><p class="elsevierStylePara">To summarise&#44; we recommend regular testing for the presence of kidney damage in patients with PV&#46; Ifthese patients develop proteinuria&#44; association with FSGS could be considered as a possible complication&#44; although it is rare&#46; The combination of steroids and immunosuppressive drugs administered early in patients with nephrotic range proteinuria may slow the progression of kidney damage&#46;</p><p class="elsevierStylePara"><a href="grande&#47;1022518078&#95;t1&#95;pag139&#46;jpg" class="elsevierStyleCrossRefs"><img src="1022518078_t1_pag139.jpg" alt="GSF cases associated with PV"></img></a></p><p class="elsevierStylePara">Table 1&#46; GSF cases associated with PV</p>"
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Focal segmental glomerulosclerosis associated with polycythaemia vera
Glomerulosclerosis segmentaria y focal asociada a policitemia vera
J.. Sánchez Martína, L.. García-Puente Suáreza, Florencio García Martína
a Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, Madrid, España,
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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">Dear Editor&#44;</span></p><p class="elsevierStylePara">Focal segmental glomerulosclerosis &#40;FSGS&#41; is characterised by the presence of nephrotic syndrome&#44; hypertension and progressive deterioration of kidney function&#46; Although in many cases its aetiology is unknown&#44; it has been associated with inherited disorders&#44; viral infections&#44; induced by toxic substances and with hyperfiltration situations&#46;<span class="elsevierStyleSup">1</span></p><p class="elsevierStylePara">Polycythaemia vera &#40;PV&#41; is a myeloproliferative disorder of unknown aetiology characterised by excessive production of normal erythrocytes&#44; leukocytes and platelets&#46;<span class="elsevierStyleSup">2</span></p><p class="elsevierStylePara">Glomerular involvement in PV is rare&#46; We present a patient diagnosed with PV with nephrotic syndrome secondary to GSF and progressive kidney disease&#46;</p><p class="elsevierStylePara">An 83 year-old woman&#44; diagnosed with PV 4 years earlier&#44; was admitted for the study of nephrotic syndrome and kidney disease of 2 years evolution&#46;</p><p class="elsevierStylePara">There was no family history of polycythaemia&#46; Among her background was&#58; left nephrectomy due to hypernephroma at 63 years&#44; bronchiectasis with recurrent bacterial superinfections&#44; hypertension controlled with medication and celiac disease well controlled through diet&#46; Four years earlier she was diagnosed with PV after a bone marrow biopsy&#44; including details of polycythaemia and thrombocytosis&#44; well controlled with hydroxyurea treatment &#40;500mg&#47;day&#41;&#46;</p><p class="elsevierStylePara">In March 2007&#44; she began with nephrotic range proteinuria &#40;4&#46;2g&#47;day&#41; with Crs 1&#46;1mg&#47;dL&#59; antiproteinuric treatment was started with telmisartan 80mg&#47;day and spironolactone 50mg&#47;day&#46; A month later the proteinuria dropped to 1&#46;2g&#47;day with no change in the creatinine&#46; She was admitted to hospital in June 2007 for severe hyponatraemia &#40;107mEq&#47;L&#41;&#44; symptomatic&#44; with hyperkalaemia &#40;5&#46;7mEq&#47;L&#41; and metabolic acidosis &#40;pH 7&#46;34&#41; secondary to treatment with spironolactone&#46; Due to the persistence of nephrotic proteinuria and her history of bronchiectasis&#44; a biopsy of rectal and abdominal fat was performed which discarded the existence of amyloidosis&#46; Throughout its evolution&#44; the proteinuria varied between 4-10g&#47;day and started with lower limb oedema&#44; decreased total protein and albumin &#40;5&#46;4&#47;2&#46;7g&#47;dL&#41; and a progressive decline in the glomerular filtration rate &#40;Crs 1&#46;6-1&#46;7mg&#47;dL&#41;&#46;</p><p class="elsevierStylePara">In May 2009 she was admitted to hospital due to a deterioration in her general condition&#44; oedema&#44; Crs 3&#46;6mg&#47;dL and proteinuria 8&#46;4g&#47;day despite treatment with ARB&#46; On&#160; admission&#44; her blood pressure was 137&#47;82mmHg&#59; in the physical examination her systolic ejection murmur was heard II&#47;VI in the left sternal border&#44; and she had bilateral pitting oedema up to the root of her thigh&#46; CBC&#58; haemoglobin 16&#46;8g&#47;dL&#44; haematocrit 56&#37;&#44; RBC 6&#44;750&#44;000&#47;&#181;L&#44; 11&#44;690&#47;&#181;l leukocytes with normal formula and platelets 460&#44;000&#47;&#181;l&#46; Crs 4&#46;3&#44; urea 102 &#40;mg&#47;dL&#41;&#46; Total protein 5&#46;9&#44; albumin 2&#46;5g&#47;dL&#44; cholesterol 188&#44; triglycerides 260&#44; uric acid 9&#46;8mg&#47;dL&#46; Proteinuria 6&#46;9g&#47;d&#59; 1&#46;4 sediment erythrocytes&#47;field and 5-10 leukocytes&#47;field&#46; Immunology&#58; ANA&#44; anti-DNA&#44; ENA&#44; ANCA and anti-GBM negative&#46; Complement&#58; C3 79&#44; C4 30mg&#47;dL&#46; CRP 0&#46;37mg&#47;dL&#46; Rheumatoid factor negative&#46; IgG 820&#44; IgA 185&#44; IgM 188mg&#47;dL&#46; Kappa 635&#44; lambda 515mg&#47;dL&#46; Kidney biopsy was performed with 8 glomeruli of which two were completely sclerosed&#44; and the remaining six&#44; one global mesangial expansion with increased mesangial cells could be seen and the other five had segmental proliferative lesions without necrosis accompanied by moderate epithelial proliferation&#46; The interstitium showed moderate fibrosis with tubular atrophy and occasional chronic inflammatory infiltrates&#46; Arterial and arteriolar vessels with hyperplastic lesions with occasional hyaline lesions&#46;&#160; These findings were indicative of <span class="elsevierStyleItalic">cellular variant segmental proliferative glomerulonephritis</span>&#46;</p><p class="elsevierStylePara">Treatment was initiated with three shocks of 125mg of 6-methylprednisolone followed by prednisone &#40;1mg&#47;kg&#47;day&#41; and mycophenolate mofetil &#40;360mg&#47;12 hours&#41;&#46; No evidence of a decrease in proteinuria was seen and kidney function deteriorated progressively with clinical uraemia&#46; So it was decided to make the right jugular catheter permanent and initiate treatment with periodic haemodialysis&#46; A progressive improvement in the symptoms was&#160; seen&#46; Treatment with mycophenolate mofetil was discontinued and prednisone was gradually withdrawn&#46;</p><p class="elsevierStylePara">The patient developed a nephrotic syndrome secondary to FSGS 4 years after detection of the polycythaemia&#46; It met the WHO criteria for diagnosis of PV2 &#40;Hb greater than 16&#46;5g&#47;dL in women&#44; no cases of secondary polycythaemia&#44; decreased EPO levels&#44; splenomegaly&#44; thrombocytosis &#62; 400&#44;000&#47;&#181;l and leukocytosis &#62; 12&#44;000&#47;&#181;L&#41;&#46; Despite good control of the PV with hydroxyurea and treatment with high dose steroids and immunosuppressive agents&#44; the nephrotic syndrome did not improve&#44; and was accompanied by a progressive deterioration of kidney function with uremic symptoms and initiation of replacement therapy&#46;</p><p class="elsevierStylePara">The kidney biopsy showed the existence of a focal segmental glomerulosclerosis cell variant that had a worse prognosis than the other FSGS &#40;perihilar hyalinosis&#44; tip variant and classical variant&#41; and better than collapsing variant&#46;<span class="elsevierStyleSup">3</span></p><p class="elsevierStylePara">The joint presence of FSGS and PV is a rare combination&#46; There have only been 6 published cases confirmed histologically in the literature&#46;<span class="elsevierStyleSup">4-8 </span>Table 1 summarises the clinical data of the 6 cases reported and of the current one&#46;</p><p class="elsevierStylePara">From this we can get some interesting data&#46; The average age at the time of kidney biopsy was 60&#46;3 years &#40;range 41-83&#41;&#46; FSGS was diagnosed up to 4 years after the detection of polycythaemia in 5 of the 7 cases &#40;71&#46;4&#37;&#41;&#46; Four patients &#40;57&#46;1&#37;&#41; developed nephrotic range proteinuria&#44; 3 patients were treated with steroids and immunosuppressive agents&#44; of whom only one had improvement of kidney function&#46; The other two were given haemodialysis due to progressive deterioration of plasma creatinine with onset or worsening of uremic symptoms&#46;</p><p class="elsevierStylePara">The presence of FSGS in these patients could be directly related to the PV&#46; As noted by Sharma et al&#44;<span class="elsevierStyleSup">4</span> the haemodynamic changes in the PV&#44; as well as kidney vasodilation and increased effective kidney blood flow could trigger kidney glomerulosclerosis&#46; Ferrario et al<span class="elsevierStyleSup">9</span> suggested that the haemodynamic glomerular injury induces the activation of the mesangial cells&#44; leading to overproduction of extracellular matrix&#46; Furthermore&#44; damaged endothelial cells facilitate platelet activation by releasing platelet activating factor&#44; which induces proliferation of endothelial and mesangial cells&#46; These alterations may contribute to the development of glomerular sclerotic changes&#46;</p><p class="elsevierStylePara">To summarise&#44; we recommend regular testing for the presence of kidney damage in patients with PV&#46; Ifthese patients develop proteinuria&#44; association with FSGS could be considered as a possible complication&#44; although it is rare&#46; The combination of steroids and immunosuppressive drugs administered early in patients with nephrotic range proteinuria may slow the progression of kidney damage&#46;</p><p class="elsevierStylePara"><a href="grande&#47;1022518078&#95;t1&#95;pag139&#46;jpg" class="elsevierStyleCrossRefs"><img src="1022518078_t1_pag139.jpg" alt="GSF cases associated with PV"></img></a></p><p class="elsevierStylePara">Table 1&#46; GSF cases associated with PV</p>"
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