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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">Dear Editor&#44; </span></p><p class="elsevierStylePara">Amyloidoses are a group of life-threatening diseases characterized by deposition of fibrillar misfolded proteins with an antiparallel beta-sheet structure&#46;<span class="elsevierStyleSup">1</span> Renal Amyloidosis is determined by extracellular deposition of amyloid fibrils within kidney compartments&#46; Clinically evident renal involvement is presented as proteinuria or nephrotic syndrome&#46;<span class="elsevierStyleSup">2</span> Systemic reactive &#40;AA&#41; amyloidosis&#44; leading to renal failure&#44; is a severe complication of most hereditary periodic fever syndromes&#46; We report the occurrence of renal AA amyloidosis causing severe nephrotic syndrome in a young Italian man affected with Hyper-IgD Syndrome &#40;HIDS&#41;&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE REPORT</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">A 29-year-old man of southern Italian ancestry&#44; was admitted to the hospital&#46; Since the first months of life he had experienced characteristic febrile attacks associated with sore throat&#44; myalgias&#44; vomiting and diarrhoea&#46; Later the attacks of fever reduced their frequency but in the last year there was an increased frequency of febrile episodes with proteinuria in nephrotic range&#46; On admission his blood pressure was 150&#47;80mmHg and temperature 36&#46;5&#176;C&#46; Heart sounds were clear with regular sinus rhythm and pulse rate was 88 beats&#47;min&#46; Physical examination showed normal findings and no symptoms suggestive of respiratory&#44; abdominal or urinary infection were apparent&#46; Chest radiography and abdominal ultrasonography scan revealed no abnormalities&#46; Laboratory investigations showed proteinuria 9&#46;17g&#47;day&#44; a raised erythrocyte sedimentation rate&#44; a normal C-reactive protein&#44; a total leukocyte count of 12&#46;500&#47;mm<span class="elsevierStyleSup">3</span>&#44; serum amyloid 3&#46;67mg&#47;L&#44; serum IgD 233UI&#47;ml &#40;normal range 0-100&#41;&#44; creatinine 1&#46;09mg&#47;dl&#46; Immunological tests and other laboratory parameters resulted negative or within normal limits&#46; Kidney biopsy&#58; among 28 glomeruli&#44; 18 showed a weakly eosinophilic amorphous material infiltrating the mesangium &#40;Figure 1 and Figure 2&#41;&#46; After positive Congo red staining&#44; the deposits revealed apple-green birefringence under polarized light&#44; consistent with the presence of amyloid&#46; On immunochemistry&#44; the amyloid deposits were negative for antibodies against kappa and lambda chains &#40;Figure 3&#41;&#46; Clinical and laboratory findings suggested diagnosis of systemic reactive amyloidosis&#46; The research for MEFV&#44; for Familial Mediterranean Fever &#40;FMF&#41; and TNFRSF1A&#44; for Tumor Necrosis Factor Receptor-Associated Periodic Syndrome &#40;TRAPS&#41; mutations was negative&#59; the research for 2 mevalonate kinase &#40;MVK&#41; mutations was positive in heterozygosis&#44; diagnosing the HIDS&#46; The patient started therapy with Anakinra&#44; Interleukin -1 antagonist&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">Renal amyloidosis comprise a spectrum of vascular&#44; glomerular&#44; and tubulointerstitial deposition&#46; The reason for the preferential localization to one or the other compartment is not well established&#46; It seems very likely that the varying chemicophysical properties of the amyloid fibrils determine the tropism&#46; Currently&#44; the distribution patterns do not aid in the management of the patients&#46;<span class="elsevierStyleSup">3</span> At least 25 different precursor proteins are known and are associated with a variety of inflammatory&#44; immune&#44; infectious&#44; and hereditary conditions&#46; Most renal amyloidosis is either the result of primary fibrillar deposits of immunoglobulin light chains known as amyloid L &#40;AL&#41; or secondary to fibrillar deposits of serum amyloid A &#40;AA&#41; protein fragments&#46; Renal involvement can be found in some monogenic diseases&#44; the hereditary periodic fever syndromes&#44; which present with recurrent inflammation and unexplained fevers as part of their phenotype&#46; Familial Mediterranean Fever &#40;FMF&#41; is the most widely known and the most prevalent of these inherited disorders&#46;<span class="elsevierStyleSup">4</span> The most dreaded complication of untreated FMF is amyloidosis&#44; which eventuates in renal failure in as many as 20&#37; of patients in some populations&#46; HIDS&#44; which mimics FMF&#44; is a much rarer disease&#59; it has been reported mainly in families of European ancestry&#44; most of whom are clustered in The Netherlands&#46; It is inherited in autosomal recessive manner&#46; Mutations in the gene encoding MVK constitute the molecular defect in HIDS&#46; The defective gene resides on chromosome 12q&#46; MVK is a key enzyme in the biosynthesis of cholesterol and isoprenoid&#46; HIDS is caused by a defect in the isoprenoid pathway&#59; presumably&#44; intermediary metabolites of the isoprenoid pathway &#40;or a shortage of certain metabolites&#41; influence the immune system in such a way that high levels of IgD are produced&#46;<span class="elsevierStyleSup">5</span> HIDS is characterized by recurrent&#44; self-limiting attacks of fever occurring since early childhood&#46; Febrile episodes usually last 3&#8211;7 days and are variably associated with headache&#44; arthralgias&#44; lymphadenopathy&#44; abdominal pain&#44; diarrhoea&#44; vomiting&#44; and skin lesions&#46; The diagnosis of HIDS is based on clinical criteria and elevated serum immunoglobulin D &#40;IgD&#41; levels &#40;100IU&#47;ml&#41;&#46; Amyloidosis has been reported only rarely in HIDS&#46; In 2006 Obici et al&#46; did the first report to describe the occurrence of renal AA amyloidosis causing severe nephrotic syndrome in a young Italian man affected with HIDS&#46;<span class="elsevierStyleSup">6</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflict of interest</span></p><p class="elsevierStylePara"><br></br>The authors declare that there is no conflict of interest associated with this manuscript&#46;</p><p class="elsevierStylePara"><a href="grande&#47;11660&#95;108&#95;34387&#95;en&#95;f119&#46;jpg" class="elsevierStyleCrossRefs"><img src="11660_108_34387_en_f119.jpg" alt="Kidney biopsy"></img></a></p><p class="elsevierStylePara">Figure 1&#46; Kidney biopsy</p><p class="elsevierStylePara"><a href="grande&#47;11660&#95;108&#95;34390&#95;en&#95;f2e19&#46;jpg" class="elsevierStyleCrossRefs"><img src="11660_108_34390_en_f2e19.jpg" alt="Kidney biopsy"></img></a></p><p class="elsevierStylePara">Figure 2&#46; Kidney biopsy</p><p class="elsevierStylePara"><a href="grande&#47;11660&#95;108&#95;34392&#95;en&#95;f3e19&#46;jpg" class="elsevierStyleCrossRefs"><img src="11660_108_34392_en_f3e19.jpg" alt="Kidney biopsy"></img></a></p><p class="elsevierStylePara">Figure 3&#46; Kidney biopsy</p>"
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Renal amyloidosis due to hyper-IgD syndrome
Gioacchino Li Cavolia, Daniela Passantinob, Calogera Tortoricia, Luisa Bonoa, Angelo Ferrantellia, Carlo Giammarresia, Ugo Rotoloa
a Division of Nephrology and Dialysis, Civic and Di Cristina Hospital, Palermo, Italy,
b Anatomical Pathology Services, Civic and Di Cristina Hospital, Palermo, Italy,
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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">Dear Editor&#44; </span></p><p class="elsevierStylePara">Amyloidoses are a group of life-threatening diseases characterized by deposition of fibrillar misfolded proteins with an antiparallel beta-sheet structure&#46;<span class="elsevierStyleSup">1</span> Renal Amyloidosis is determined by extracellular deposition of amyloid fibrils within kidney compartments&#46; Clinically evident renal involvement is presented as proteinuria or nephrotic syndrome&#46;<span class="elsevierStyleSup">2</span> Systemic reactive &#40;AA&#41; amyloidosis&#44; leading to renal failure&#44; is a severe complication of most hereditary periodic fever syndromes&#46; We report the occurrence of renal AA amyloidosis causing severe nephrotic syndrome in a young Italian man affected with Hyper-IgD Syndrome &#40;HIDS&#41;&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE REPORT</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">A 29-year-old man of southern Italian ancestry&#44; was admitted to the hospital&#46; Since the first months of life he had experienced characteristic febrile attacks associated with sore throat&#44; myalgias&#44; vomiting and diarrhoea&#46; Later the attacks of fever reduced their frequency but in the last year there was an increased frequency of febrile episodes with proteinuria in nephrotic range&#46; On admission his blood pressure was 150&#47;80mmHg and temperature 36&#46;5&#176;C&#46; Heart sounds were clear with regular sinus rhythm and pulse rate was 88 beats&#47;min&#46; Physical examination showed normal findings and no symptoms suggestive of respiratory&#44; abdominal or urinary infection were apparent&#46; Chest radiography and abdominal ultrasonography scan revealed no abnormalities&#46; Laboratory investigations showed proteinuria 9&#46;17g&#47;day&#44; a raised erythrocyte sedimentation rate&#44; a normal C-reactive protein&#44; a total leukocyte count of 12&#46;500&#47;mm<span class="elsevierStyleSup">3</span>&#44; serum amyloid 3&#46;67mg&#47;L&#44; serum IgD 233UI&#47;ml &#40;normal range 0-100&#41;&#44; creatinine 1&#46;09mg&#47;dl&#46; Immunological tests and other laboratory parameters resulted negative or within normal limits&#46; Kidney biopsy&#58; among 28 glomeruli&#44; 18 showed a weakly eosinophilic amorphous material infiltrating the mesangium &#40;Figure 1 and Figure 2&#41;&#46; After positive Congo red staining&#44; the deposits revealed apple-green birefringence under polarized light&#44; consistent with the presence of amyloid&#46; On immunochemistry&#44; the amyloid deposits were negative for antibodies against kappa and lambda chains &#40;Figure 3&#41;&#46; Clinical and laboratory findings suggested diagnosis of systemic reactive amyloidosis&#46; The research for MEFV&#44; for Familial Mediterranean Fever &#40;FMF&#41; and TNFRSF1A&#44; for Tumor Necrosis Factor Receptor-Associated Periodic Syndrome &#40;TRAPS&#41; mutations was negative&#59; the research for 2 mevalonate kinase &#40;MVK&#41; mutations was positive in heterozygosis&#44; diagnosing the HIDS&#46; The patient started therapy with Anakinra&#44; Interleukin -1 antagonist&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">Renal amyloidosis comprise a spectrum of vascular&#44; glomerular&#44; and tubulointerstitial deposition&#46; The reason for the preferential localization to one or the other compartment is not well established&#46; It seems very likely that the varying chemicophysical properties of the amyloid fibrils determine the tropism&#46; Currently&#44; the distribution patterns do not aid in the management of the patients&#46;<span class="elsevierStyleSup">3</span> At least 25 different precursor proteins are known and are associated with a variety of inflammatory&#44; immune&#44; infectious&#44; and hereditary conditions&#46; Most renal amyloidosis is either the result of primary fibrillar deposits of immunoglobulin light chains known as amyloid L &#40;AL&#41; or secondary to fibrillar deposits of serum amyloid A &#40;AA&#41; protein fragments&#46; Renal involvement can be found in some monogenic diseases&#44; the hereditary periodic fever syndromes&#44; which present with recurrent inflammation and unexplained fevers as part of their phenotype&#46; Familial Mediterranean Fever &#40;FMF&#41; is the most widely known and the most prevalent of these inherited disorders&#46;<span class="elsevierStyleSup">4</span> The most dreaded complication of untreated FMF is amyloidosis&#44; which eventuates in renal failure in as many as 20&#37; of patients in some populations&#46; HIDS&#44; which mimics FMF&#44; is a much rarer disease&#59; it has been reported mainly in families of European ancestry&#44; most of whom are clustered in The Netherlands&#46; It is inherited in autosomal recessive manner&#46; Mutations in the gene encoding MVK constitute the molecular defect in HIDS&#46; The defective gene resides on chromosome 12q&#46; MVK is a key enzyme in the biosynthesis of cholesterol and isoprenoid&#46; HIDS is caused by a defect in the isoprenoid pathway&#59; presumably&#44; intermediary metabolites of the isoprenoid pathway &#40;or a shortage of certain metabolites&#41; influence the immune system in such a way that high levels of IgD are produced&#46;<span class="elsevierStyleSup">5</span> HIDS is characterized by recurrent&#44; self-limiting attacks of fever occurring since early childhood&#46; Febrile episodes usually last 3&#8211;7 days and are variably associated with headache&#44; arthralgias&#44; lymphadenopathy&#44; abdominal pain&#44; diarrhoea&#44; vomiting&#44; and skin lesions&#46; The diagnosis of HIDS is based on clinical criteria and elevated serum immunoglobulin D &#40;IgD&#41; levels &#40;100IU&#47;ml&#41;&#46; Amyloidosis has been reported only rarely in HIDS&#46; In 2006 Obici et al&#46; did the first report to describe the occurrence of renal AA amyloidosis causing severe nephrotic syndrome in a young Italian man affected with HIDS&#46;<span class="elsevierStyleSup">6</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflict of interest</span></p><p class="elsevierStylePara"><br></br>The authors declare that there is no conflict of interest associated with this manuscript&#46;</p><p class="elsevierStylePara"><a href="grande&#47;11660&#95;108&#95;34387&#95;en&#95;f119&#46;jpg" class="elsevierStyleCrossRefs"><img src="11660_108_34387_en_f119.jpg" alt="Kidney biopsy"></img></a></p><p class="elsevierStylePara">Figure 1&#46; Kidney biopsy</p><p class="elsevierStylePara"><a href="grande&#47;11660&#95;108&#95;34390&#95;en&#95;f2e19&#46;jpg" class="elsevierStyleCrossRefs"><img src="11660_108_34390_en_f2e19.jpg" alt="Kidney biopsy"></img></a></p><p class="elsevierStylePara">Figure 2&#46; Kidney biopsy</p><p class="elsevierStylePara"><a href="grande&#47;11660&#95;108&#95;34392&#95;en&#95;f3e19&#46;jpg" class="elsevierStyleCrossRefs"><img src="11660_108_34392_en_f3e19.jpg" alt="Kidney biopsy"></img></a></p><p class="elsevierStylePara">Figure 3&#46; Kidney biopsy</p>"
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