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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">To the Editor&#58;</span></p><p class="elsevierStylePara">Autosomal dominant polycystic kidney disease &#40;ADPKD&#41; is the most common hereditary cause of terminal chronic renal failure &#40;CRF&#41;&#44; with an incidence of 1 in 500-1000&#46; This disease is produced by mutations to the genes <span class="elsevierStyleItalic">PKD1</span> &#40;16p13&#46;3&#44; 85&#37;&#41; or <span class="elsevierStyleItalic">PKD2</span> &#40;4q22&#46;1&#44; 15&#37;&#41;&#46; In ADPKD&#44; the growth of renal cysts produces a progressive increase in renal volume and destruction of the parenchyma&#44; leading to terminal CRF at approximately 50-60 years of age &#40;in PKD1 mutations&#41;&#46; Although ADPKD is bilateral&#44; renal involvement may be asynchronous and asymmetrical&#44;<span class="elsevierStyleSup">2</span> and in PKD2 mutations&#44; terminal CRF may be delayed by as many as 20 years with respect to PKD1&#46; On the other hand&#44; congenital anomalies of the kidney and urinary tract &#40;CAKUT&#41; are the most common cause of CRF in children&#44; constituting half of all cases&#46;<span class="elsevierStyleSup">3-6</span> Although many cases of CAKUT are caused by unique genetic defects&#44; mutations have been identified in only a few genes&#46; These unique mutations can produce a wide phenotypic spectrum of CAKUT&#44; which ranges from vesicoureteral reflux to renal agenesis&#46;<span class="elsevierStyleSup">5-7</span> Unilateral renal agenesis is a congenital anomaly that occurs in 1 in 3000 births&#44;<span class="elsevierStyleSup">3</span> for which several candidate genes exist&#46; The coexistence of ADPKD and unilateral renal agenesis occurs in approximately 1 in 1&#160;500&#160;000-3&#160;000&#160;000 individuals&#46; As such&#44; the coincidence of both anomalies in a single individual is very rare&#44; with only 7 cases reported&#46;<span class="elsevierStyleSup">8-12</span></p><p class="elsevierStylePara">In a cohort of 205 patients with ADPKD&#44; we identified a new case of ADPKD with unilateral renal agenesis&#44; which is the 8<span class="elsevierStyleSup">th</span> such case reported in the world and only the 2<span class="elsevierStyleSup">nd</span> in Spain&#46; Our patient was a 57-year old male who was diagnosed with right ADPKD and left renal agenesis by ultrasound at age 41&#46; At this moment&#44; the patient&#8217;s serum creatinine &#40;Crs&#41; value was 1&#46;2mg&#47;dl&#46; The right ADPKD and left renal agenesis were confirmed by isotopic renogram and magnetic resonance angiography &#40;MRA&#41;&#46; The MRA revealed hepatic cysts&#44; a large right polycystic kidney&#44; and absent renal tissue on the left side &#40;Figure 1&#41;&#46; Sixteen years later&#44; sCr was 5&#46;9mg&#47;dl and creatinine clearance was 12ml&#47;min&#47;1&#46;73m<span class="elsevierStyleSup">2</span> &#46; In the last 6 years&#44; the patient had suffered a mean annual loss in glomerular filtration rate of 7&#46;6ml&#47;min&#47;1&#46;73m<span class="elsevierStyleSup">2</span>&#44; requiring peritoneal dialysis starting at age 57&#46; In the patient&#8217;s family background&#44; the mother&#44; two siblings&#44; and one child all had ADPKD &#40;Figure 2&#41;&#46; The family genetic analysis ruled out the possibility of unilateral renal agenesis in other family members&#46;</p><p class="elsevierStylePara">Renal agenesis is produced by a lack of interaction between the ureteric bud and the metanephric mesenchyme&#44; leading to the absence of ureter and kidney&#46; Induction of the ureteric bud from the nephric duct is mediated by glia cell-derived neurotrophic factor &#40;GDNF&#41;&#44; which is secreted by the metanephric mesenchyme and interacts with the tyrosine kinase c-Ret receptor expressed in the ureteric bud in order to induce branching of the nephric duct&#46;<span class="elsevierStyleSup">13</span> Several candidate genes have been isolated&#58; <span class="elsevierStyleItalic">BMP4</span>&#44; <span class="elsevierStyleItalic">RET</span>&#44;<span class="elsevierStyleItalic"> GDNF&#44;</span><span class="elsevierStyleItalic">FREM2</span> &#40;FRAS1-<span class="elsevierStyleItalic">related extracellular matrix protein</span><span class="elsevierStyleItalic"> </span><span class="elsevierStyleItalic">2</span>&#41;&#44; and<span class="elsevierStyleItalic"> FRAS1</span> &#40;Fraser syndrome 1&#41;&#46;<span class="elsevierStyleSup">13-16</span> FRAS1 and FREM2 are responsible for maintaining the integrity of several renal epithelial structures&#44; and are also implicated in the initiation of metanephric kidneys&#46; The gene FRAS1 codes for the protein FRAS1&#44; which contains repeats of the primary domain of sulphate chondroitin proteoglycan&#44; whose function is to maintain the integrity of the epithelial cells&#46;<span class="elsevierStyleItalic"> </span>The protein FRAS1 was detected in several tissues under development&#46; In the metanephros&#44; FRAS1 was detected in the extracellular matrix&#44; covering the basal surface of the ureter during growth and the basal membrane of the collecting tubule&#46; In individuals with Fraser syndrome&#44; all <span class="elsevierStyleItalic">FRAS1</span> mutations reported were mixed heterozygotes or homozygotes&#44; indicating a recessive genetic trait&#46; In contrast&#44; in non-syndromic unilateral renal agenesis&#44; unique <span class="elsevierStyleItalic">FRAS1</span> heterozygotic mutations have been observed&#46;<span class="elsevierStyleSup">16</span> On the other hand&#44; unilateral renal agenesis is associated with congenital anomalies of the reproductive system and urinary tract &#40;cysts in the seminal vesicles&#41;&#46;<span class="elsevierStyleSup">17</span> Approximately 40&#37;-50&#37; of children with renal agenesis also have other urogenital defects&#44; such as vesicoureteral reflux&#44; ectopic kidney&#44; pelvicalyceal dilatation&#44; ureteral duplication&#44; neurogenic bladder&#44; cryptorchidism&#44; and structural defects of the vagina or uterus&#46; In addition&#44; 15&#37; of these children have cardiovascular defects&#46;<span class="elsevierStyleSup">18</span></p><p class="elsevierStylePara">The importance of the association between ADPKD and contralateral renal agenesis is due to the possible causal relationship between these two processes and due to the influence that a single polycystic kidney may have on the early development of CRF&#46; Of the 8 cases of ADPKD and unilateral renal agenesis &#40;including our case&#41; recorded in the medical literature&#44; only 2 underwent genetic analyses to differentiate between PKD1 and PKD2&#44;<span class="elsevierStyleSup">12</span> and each case was associated with a different mutation of the <span class="elsevierStyleItalic">PKD1</span> gene&#46; When unilateral renal agenesis occurs as an isolated anomaly&#44; it is asymptomatic&#44; tends to be discovered incidentally during routine ultrasound tests&#44; and normally produces a compensatory hypertrophy of the contralateral kidney&#46; In the case of association with ADPKD&#44; this compensatory hypertrophy may not occur&#44; thus accelerating the development of CRF in the single polycystic kidney&#46; In this manner&#44; unilateral ADPKD would represent a model for analysing the consequences of reducing the number of available nephrons in ADPKD&#46; The cases of ADPKD in a single kidney do not provide sufficient information regarding kidney volume or rates of progression&#44;<span class="elsevierStyleSup">8-12 </span>and several already had advanced CRF &#40;Table 1&#41;&#46; In addition&#44; these cases of ADPKD in a single kidney cannot be compared to those of patients with two polycystic kidneys&#46; The 2 patients described by Poster<span class="elsevierStyleSup">12</span> who had unilateral ADPKD had higher renal volumes and rates of volume increase than the mean values recorded in a control group&#46; With the exception of the case reported by Sirvent&#44;<span class="elsevierStyleSup">11</span> which involved severe uncontrolled hypertension&#44; the 7 cases reported of unilateral ADPKD did not experience accelerated rates of disease progression&#46; Our patient progressed towards terminal CRF&#44; with an annual loss in renal function of 7&#46;6ml&#47;min&#47;1&#46;73m<span class="elsevierStyleSup">2</span> during the last 6 years&#46; As such&#44; the available data indicate that unilateral ADPKD kidneys do not appear to retain the capacity to compensate for the reduction in the number of available nephrons&#46; Unilateral renal agenesis also occurs most commonly on the left side of the body&#44;<span class="elsevierStyleSup">19</span> since in 6 out of the 8 cases reported to date of ADPKD and contralateral renal agenesis&#44; the absent kidney was the left kidney &#40;75&#37; of cases&#41;&#46; No clear explanation has been put forth for this predominance&#44; highlighting our lack of understanding of embryogenesis and nephrogenesis in particular&#46;</p><p class="elsevierStylePara">To conclude&#44; this case illustrates the coincidence of two hereditary renal diseases&#58; one autosomal dominant&#44; ADPKD&#44; and the other recessive&#44; contralateral renal agenesis&#46; Although ADPKD and unilateral renal agenesis can be due to alterations to 2 different and independent genes&#44; we cannot rule out the possibility of a causal relationship between these two anomalies&#46;<span class="elsevierStyleSup">20</span> More cases will need to be analysed in order to come to a conclusion in this regard&#46;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">Acknowledgements</span>&#160;</p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">This study was financed in part by the Carlos III Institute of Health&#44; the Ministry of Science and Innovation &#40;EC08&#47;00236&#41; and the programme for intensifying research activities &#40;IdiPAZ and Agencia Lain-Entralgo&#47;CM&#41; to R&#46;P&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The authors state that they have no potential conflicts of interest related to the content of this article&#46;</p><p class="elsevierStylePara"><a href="grande&#47;11658&#95;108&#95;39751&#95;en&#95;11658t1&#46;jpg" class="elsevierStyleCrossRefs"><img src="11658_108_39751_en_11658t1.jpg" alt="Patients with autosomal dominant polycystic kidney disease and contralateral renal agenesis as reported in the medical literature"></img></a></p><p class="elsevierStylePara">Table 1&#46; Patients with autosomal dominant polycystic kidney disease and contralateral renal agenesis as reported in the medical literature</p><p class="elsevierStylePara"><a href="grande&#47;11658&#95;108&#95;39752&#95;en&#95;11658f1&#46;jpg" class="elsevierStyleCrossRefs"><img src="11658_108_39752_en_11658f1.jpg" alt="Coronal magnetic resonance image demonstrating a large right polycystic kidney and absent left kidney&#46; The liver also has multiple cysts"></img></a></p><p class="elsevierStylePara">Figure 1&#46; Coronal magnetic resonance image demonstrating a large right polycystic kidney and absent left kidney&#46; The liver also has multiple cysts</p><p class="elsevierStylePara"><a href="grande&#47;11658&#95;108&#95;39753&#95;en&#95;11658f2&#46;jpg" class="elsevierStyleCrossRefs"><img src="11658_108_39753_en_11658f2.jpg" alt="Genealogical tree of the family with autosomal dominant polycystic kidney disease and unilateral renal agenesis&#46; Numbers indicate date of birth"></img></a></p><p class="elsevierStylePara">Figure 2&#46; Genealogical tree of the family with autosomal dominant polycystic kidney disease and unilateral renal agenesis&#46; Numbers indicate date of birth</p>"
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                0 => array:3 [
                  "referenciaCompleta" => "Masoumi A, Reed-Gitomer B, Kelleher C, Bekheirnia MR, Schrier RW. Developments in the management of autosomal dominant polycystic kidney disease. Ther Clin Risk Manag 2008;4:393-407. <a href="http://www.ncbi.nlm.nih.gov/pubmed/18728845" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
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              "identificador" => "bib2"
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                0 => array:3 [
                  "referenciaCompleta" => "Shiroyanagi Y,\u{A0}Suzuki M,\u{A0}Matsuno D,\u{A0}Mochizuki K,\u{A0}Kitagawa N,\u{A0}Tanaka M,\u{A0}et al. Asymmetric development of tumor-like cysts in a child with autosomal dominant polycystic kidney disease.\u{A0}J Pediatr Surg 2008;43:e21-3. <a href="http://www.ncbi.nlm.nih.gov/pubmed/19040915" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
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                  "host" => array:1 [
                    0 => null
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            ]
            2 => array:3 [
              "identificador" => "bib3"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Wilson RD, Baird PA. Renal agenesis in British Columbia. Am J Med Genet 1985;21:153-69. <a href="http://www.ncbi.nlm.nih.gov/pubmed/4003440" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            3 => array:3 [
              "identificador" => "bib4"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Costantini F. Renal branching morphogenesis: concepts, questions, and recent advances. Differentiation 2006;74:402-21. <a href="http://www.ncbi.nlm.nih.gov/pubmed/16916378" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            4 => array:3 [
              "identificador" => "bib5"
              "etiqueta" => "5"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Dressler GR. Advances in early kidney specification, development and patterning. Development 2009;136:3863-74. <a href="http://www.ncbi.nlm.nih.gov/pubmed/19906853" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            5 => array:3 [
              "identificador" => "bib6"
              "etiqueta" => "6"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Reidy KJ, Rosenblum ND. Cell and molecular biology of kidney development. Semin Nephrol 2009; 29:321-37. <a href="http://www.ncbi.nlm.nih.gov/pubmed/19615554" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            6 => array:3 [
              "identificador" => "bib7"
              "etiqueta" => "7"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Yosypiv IV.\u{A0}Renin-angiotensin system in ureteric bud branching morphogenesis: insights into the mechanisms.\u{A0}Pediatr Nephrol 2011;26:1499-512. <a href="http://www.ncbi.nlm.nih.gov/pubmed/21359618" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            7 => array:3 [
              "identificador" => "bib8"
              "etiqueta" => "8"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Bear RA. Solitary kidney affected with polycystic disease: A report of 2 cases. J Urol 1974;111:566-7. <a href="http://www.ncbi.nlm.nih.gov/pubmed/4823963" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            8 => array:3 [
              "identificador" => "bib9"
              "etiqueta" => "9"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Todorov VV. The diagnostic dilemma of the unilateral cystic kidney-ADPKD with aplasia of one kidney. Nephrol Dial Transplant 1999;14:2775. <a href="http://www.ncbi.nlm.nih.gov/pubmed/10534805" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            9 => array:3 [
              "identificador" => "bib10"
              "etiqueta" => "10"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Jeong GH, Park BS, Jeong TK, Ma SK,\u{A0}Yeum CH,\u{A0}Kim SW, et al. Unilateral autosomal dominant polycystic kidney disease with contralateral renal agenesis: Acase report. J Korean Med Sci 2003; 18:284-6. <a href="http://www.ncbi.nlm.nih.gov/pubmed/12692431" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            10 => array:3 [
              "identificador" => "bib11"
              "etiqueta" => "11"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Sirvent AE, Enríquez R, Ardoy F, Amorós F, González C, Reyes A. Autosomal dominant polycystic kidney disease with congenital absence of contralateral kidney. Int Urol Nephrol 2006;38:773-4. <a href="http://www.ncbi.nlm.nih.gov/pubmed/17171426" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            11 => array:3 [
              "identificador" => "bib12"
              "etiqueta" => "12"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Poster D, Kistler AD, Krauer F.\u{A0}Kidney function and volume progression in unilateral autosomal dominant polycystic kidney disease with contralateral renal agenesis or hypoplasia: a case series.\u{A0}Am J Kidney Dis 2009;54:450-8. <a href="http://www.ncbi.nlm.nih.gov/pubmed/19515475" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            12 => array:3 [
              "identificador" => "bib13"
              "etiqueta" => "13"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Song R, Yosypiv IV. Genetics of congenital anomalies of the kidney and urinary tract. Pediatr Nephrol 2011;26:353-64. <a href="http://www.ncbi.nlm.nih.gov/pubmed/20798957" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            13 => array:3 [
              "identificador" => "bib14"
              "etiqueta" => "14"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Skinner MA, Safford SD, Reeves JG, Jackson ME, Freemerman AJ. Renal aplasia in humans is associated with RET mutations. Am J Hum Genet 2008;82:344-51. <a href="http://www.ncbi.nlm.nih.gov/pubmed/18252215" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            14 => array:3 [
              "identificador" => "bib15"
              "etiqueta" => "15"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Rozen EJ,\u{A0}Schmidt H,\u{A0}Dolcet X,\u{A0}Basson MA,\u{A0}Jain S,\u{A0}Encinas M. Loss of sprouty1 rescues renal agenesis caused by Ret mutation. J Am Soc Nephrol 2009;20:255-9. <a href="http://www.ncbi.nlm.nih.gov/pubmed/19056869" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            15 => array:3 [
              "identificador" => "bib16"
              "etiqueta" => "16"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Saisawat P,\u{A0}Tasic V,\u{A0}Vega-Warner V, Kehinde EO,\u{A0}Günther B,\u{A0}Airik R, et al. Identification of two novel CAKUT-causing genes by massively parallel exon resequencing of candidate genes in patients with unilateral renal agenesis.\u{A0}Kidney Int\u{A0}2012;81:196-200."
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            16 => array:3 [
              "identificador" => "bib17"
              "etiqueta" => "17"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Nakanishi K, Yoshikawa N. Genetic disorders of human congenital anomalies of the kidney and urinary tract (CAKUT). Pediatr Int 2003;45:610-6. <a href="http://www.ncbi.nlm.nih.gov/pubmed/14521546" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            17 => array:3 [
              "identificador" => "bib18"
              "etiqueta" => "18"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Szmigielska A, Roszkowska-Blaim M, Werner B, Kami¿ska H, Brzewski M.\u{A0}Hypertension in a girl with severe coarctation of the aorta and renal agenesis.\u{A0}J Pediatr 2012;160:705-6. <a href="http://www.ncbi.nlm.nih.gov/pubmed/22082948" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            18 => array:3 [
              "identificador" => "bib19"
              "etiqueta" => "19"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Schreuder MF. Unilateral anomalies of kidney development: why is left not right? Kidney Int 2011;80:740-5. <a href="http://www.ncbi.nlm.nih.gov/pubmed/21716256" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
            19 => array:3 [
              "identificador" => "bib20"
              "etiqueta" => "20"
              "referencia" => array:1 [
                0 => array:3 [
                  "referenciaCompleta" => "Kerecuk L,\u{A0}Long DA,\u{A0}Ali Z,\u{A0}Anders C,\u{A0}Kolatsi-Joannou M,\u{A0}Scambler PJ,\u{A0}et al. Expression of Fraser syndrome genes in normal and polycystic murine kidneys.\u{A0}Pediatr Nephrol 2012; 27:991-8. <a href="http://www.ncbi.nlm.nih.gov/pubmed/21993971" target="_blank">[Pubmed]</a>"
                  "contribucion" => array:1 [
                    0 => null
                  ]
                  "host" => array:1 [
                    0 => null
                  ]
                ]
              ]
            ]
          ]
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