First Case Described in the Medical Literature: Male Urethral Sarcoidosis. Description of a Case and Review of the Literature

Objective: Although the sarcoidosis is a multisystemic disease that theoretically can affect almost any organ, the presence of sarcoidosis in the male urethra has not been described in the medical literature. We present the first male case of urethral sarcoidosis. Method: A 46 years old male undergoing follow up due to lower urinary tract symptoms was diagnosed of endobronchial sarcoidosis during the preoperative study for internal urethrotomy. After surgery, he presented clinical improvement for one year. Given the worsening, a new internal urethrotomy was tried. As it was impossible due to complexity they took a biopsy of the urethra. The pathology report described non-caseating granulomas compatible with sarcoidosis. After that, medical and endoscopic management of the urethral sarcoidosis was attempted. As it didn't achieve an adequate control, the patient was derivate to the "complex urethral unit" of the Cruces University Hospital. Once it was valuated, it was decided to start immunotherapy and subsequently an urethroplasty with a double oral mucosa graft was performed. Outcomes: During the postoperative period, a urethral catheter was maintained for two weeks. It was removed after no urinary leakage was observed in de cystourethrography. After that the patient remains with good evolution until today. Conclusions: Urethral affectation by sarcoidosis is a therapeutic challenge itself. For a better symptom control and to reduce the recurrences, a dual approach using systemic treatment in combination with local surgical treatment seems necessary (AU)

Objetivo: Pese a que la sarcoidosis es una enfermedad multisistémica que teóricamente puede afectar a casicualquier órgano, no ha sido descrita en la literatura médicala presencia de sarcoidosis en uretra masculina. Presentamos el diagnóstico y tratamiento del primer caso masculinode sarcoidosis uretral.Método: Varón de 46 años en seguimiento por clínicamiccional de vaciado al que se le diagnosticó de sarcoidosis endobronquial durante el estudio preoperatorio para larealización de una uretrotomía interna. Tras la intervenciónpresentó mejoría clínica durante un año. Ante el empeoramiento, se intentó repetir una nueva uretrotomía internasiendo esta imposible y decidiéndose realizar una biopsia.El informe anatomopatológico describió granulomas no caseificantes compatibles con sarcoidosis. Posteriormente seintentó un manejo médico y endoscópico de la sarcoidosis uretral que no logró un adecuado control por lo que sederivó a la unidad de uretra compleja del Hospital Universitario Cruces. Ante los hallazgos se decidió comenzar coninmunoterapia y posteriormente se realizó una uretroplastiacon doble injerto de mucosa oral.Resultado: Durante el postoperatorio se mantuvo unasonda uretral durante dos semanas. Posteriormente se retirótras la realización de una cistouretrografía miccional seriada donde no se apreciaba fuga urinaria permaneciendo elpaciente con buena evolución hasta hoy en día.Conclusiones: La afectación uretral por sarcoidosissupone actuar ante una patología que de por sí es un retoterapéutico. Para un mejor control sintomatológico y reducir las recidivas parece necesario un abordaje doble mediante un tratamiento sistémico farmacológico en combinación con tratamiento quirúrgico local (AU)

Preoperative Computed Tomography Parameters and Deterioration of Remaining Kidney Function in Living Donors.

INTRODUCTION: After living kidney donation, a decrease of kidney function (described as estimated glomerular filtration rate [eGFR]) is observed in majority of donors. However, the loss is more significant in some patients without an explicable reason. The aim of this study was to identify quantitative parameters in computed tomography (CT) of the abdomen that would predict greater eGFR reduction after kidney removal. MATERIAL AND METHODS: One hundred and ten preoperative multiphase CT examinations of the abdomen of kidney donors were analyzed for the following renal parameters: cortex, parenchyma and pyramids volume, scarring thickness (low grade: <1 cm, high grade: >1 cm), cortical gaps, vascularisation, and cortex-to-aorta enhancement index (CAEI). The radiologic and biometric (eg, donor weight) parameters were correlated with eGFR (CKD-EPI formula) change between baseline and at discharge. RESULTS: Donor weight was correlated with a loss of eGFR (P < .001). Kidney volumetric parameters including renal cortex and parenchyma volume, as well as renal artery cross-section area were associated with donor weight (r = 0.50 P < .001 and r = 0.39 P < .001). CAEI was correlated with a loss of eGFR (P = .003) and was related to the donor's sex in favor of men. Forty-one (37%) donors had an additional renal artery, which did not influence kidney function. No influence of cortical gaps or scarring on eGFR was observed. CONCLUSIONS: CAEI may be a helpful tool in predicting greater short-term kidney function decrease after living kidney donation. Male sex is the strongest risk factor of greater eGFR loss after kidney donation.

Epidemiological factors related to hospitalization due to influenza in children below 6 months of age.

The aim of this study was to address the epidemiological factors associated to hospital admissions due to influenza in infants younger than 6 months. A case-control study was performed in a tertiary hospital in Spain. Cases were infants under 6 months of age without comorbidities who were admitted due to influenza between October 2010 and March 2015. Controls were healthy infants younger than 6 months who were hospitalized due to non-respiratory illness or non-infectious diseases (urinary tract infection was included as controls). Data were retrospectively collected from medical records and phone interviews. A total of 88 cases and 122 controls we included. From univariate analysis, differences were found in relation to maternal age (43.1 ± 4.95 vs 32 ± 5.3), paternal age (37 ± 6.4 vs 34.5 ± 6.1), having siblings (79 vs 24%), siblings below 4 years old (54 vs 15%), and having vaccinated grandparents (18 vs 39%) (p < 0.05). After logistic regression, having vaccinated grandparents was an independent protective factor (OR 0.22 [CI95%; 0.05-0.91]), while having siblings was a risk factor (OR 15.8 [CI95% 3.15-79.5]). Vaccination during pregnancy was highly uncommon (3.5 vs 8.3%; p = 0.3). CONCLUSION: This study underlines the importance of increasing influenza immunization among household contacts of infants below 6 months to prevent their influenza admission. What is Known: • Infants younger than 6 months old are considered a high-risk population. • Vaccination against influenza is not licensed in infants below 6 months. What is New: • Increasing vaccination coverage in elderly people could reduce infants' hospitalization rates. • Cocoon immunization strategy may reduce the admission of infants.

Septic pulmonary emboli detected by 18F-FDG PET/CT in children with S. aureus catheter-related bacteremia.

PURPOSE: The role of 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT) in the diagnosis of metastatic infectious foci in children with catheter-related blood stream infection has been hardly studied, although some authors have reported it benefit in the screening of metastatic foci in adult population. Septic pulmonary emboli are among the most difficult to identify, because many cases do not present pulmonary complaints or abnormal chest radiography. However, diagnosis of these foci has important therapeutic consequences. The purpose of this article is to describe the role of 18F-FDG PET/CT in the diagnosis of septic pulmonary embolism in children with S. aureus catheter-related bacteremia. METHODS: We report 3 children with S. aureus catheter-related bacteremia and normal chest X-ray at admission, in whom 18F-FDG PET/CT led to the diagnosis of unsuspected septic pulmonary emboli, with an impact on clinical management. RESULTS: All patients had hemophilia and implantable venous access ports and presented with fever and normal lung auscultation. Only 1 reported non-specific symptoms (undifferentiated left chest pain). All patients had normal chest X-ray on admission. Catheters were removed within 48 h after admission in 2 cases, and 5 days after admission in the last case, subsiding fever. In 2 children, paired blood cultures were not able to identify bacteremia. However, in all cases catheter tip and subcutaneous port cultures yielded S. aureus and PET/CT detected unsuspected pulmonary metastatic emboli. CONCLUSIONS: 18F-FDG PET/CT should be considered as a useful tool to diagnose septic pulmonary embolism in S. aureus catheter-related bacteremia, especially if conventional diagnostic imaging techniques have failed to reveal possible metastatic foci. Further studies are needed to clarify the usefulness of PET/CT performance in children with CRBSI.

Pentatricopeptide repeat 336 as the candidate gene for paternal sorting of mitochondria (Psm) in cucumber.

KEY MESSAGE: Pentatricopeptide repeat (PPR) 336 was identified as the candidate gene for Paternal Sorting of Mitochondria ( Psm ), a nuclear locus that affects the predominant mitochondria transmitted to progenies. Cucumber (Cucumis sativus L.) is a useful plant to study organellar-nuclear interactions because its organelles show differential transmission, maternal for chloroplasts and paternal for mitochondria. The mitochondrial DNA (mtDNA) of cucumber is relatively large due in part to accumulation of repetitive DNAs and recombination among these repetitive regions produces structurally polymorphic mtDNAs associated with paternally transmitted mosaic (MSC) phenotypes. The mitochondrial mutant MSC16 possesses an under-representation of ribosomal protein S7 (rps7), a key component of the small ribosomal subunit in the mitochondrion. A nuclear locus, Paternal Sorting of Mitochondria (Psm), affects the predominant mitochondria transmitted to progenies generated from crosses with MSC16 as the male parent. Using single nucleotide polymorphisms, Psm was mapped to a 170 kb region on chromosome 3 of cucumber and pentatricopeptide repeat (PPR) 336 was identified as the likely candidate gene. PPR336 stabilizes mitochondrial ribosomes in Arabidopsis thaliana and because MSC16 shows reduced transcription of rps7, the cucumber homolog of PPR336 (CsPPR336) as the candidate for Psm is consistent with a nuclear effect on ribosome assembly or stability in the mitochondrion. We used polymorphisms in CsPPR336 to genotype progenies segregating at Psm and recovered only one Psm -/- plant with the MSC phenotype, indicating that the combination of the Psm- allele with mitochondria from MSC16 is almost always lethal. This research illustrates the usefulness of the MSC mutants of cucumber to reveal and study unique interactions between the mitochondrion and nucleus.

Características de la rotación en Atención Primaria de los residentes de Pediatría / Characteristics of Primary Care rotation of pediatric residents

Objetivo: determinar si en la Comunidad de Madrid (CM) la formación en Atención Primaria (AP) del médico interno residente (MIR) de Pediatría se adecúa a lo establecido en la Orden Ministerial SCO 3148/2006: rotación obligatoria y duración mínima de tres meses, siendo aconsejable su distribución en dos periodos (R1-R2 y R3- R4). Material y métodos: se realiza una encuesta telefónica o por correo electrónico, cumplimentada por un MIR de Pediatría y supervisada por un médico adjunto tutor de residentes. Se incluyen todos los hospitales de la CM con formación acreditada en dicha especialidad. Resultados: en la CM existen 19 hospitales con formación MIR en Pediatría, siendo en todos ellos la rotación por AP obligatoria. En un 58% (11/19) de ellos la duración es de tres meses, siendo inferior en el resto. En un 42% (8/19) de los casos la rotación se divide en dos periodos, aunque solo en cuatro centros según lo recomendado en la Orden Ministerial. Conclusiones: solo uno de cada cinco hospitales de la CM presenta una formación en AP de acuerdo a lo que aconseja la Orden Ministerial (AU)

Objective: to determine whether training in Primary Care (PC) during pediatric residency in the Autonomous Community of Madrid (ACM) follows the recommendations established in the Ministerial Order SCO 3148/2006: mandatory rotation with a minimum length of 3 months, being advisable to distribute it in two periods (R1-R2 and R3-R4). Material and methods: a survey by telephone or e-mail was carried out and answered by a pediatric resident and supervised by an attending physician, tutor of residents. All ACM hospitals with accredited training in this speciality were included. Results: in the ACM there are 19 hospitals with training in Pediatrics, and in all of them the rotation in PC is mandatory. In 58% (11/19) of them the rotation lasts for three months, being shorter in the rest. In 42% (8/19) of the cases, the rotation is divided into two periods, but only in 4 centers as recommended by the Ministerial Order. Conclusions: only in one out of five hospitals in the ACM is PC training organized according to the Ministerial Order (AU)

Resección de metástasis hepáticas en cáncer de colon: nuevas perspectivas / Resection of liver metástasis in colon cancer: new perspectives

Liver metastasis of colon cancer is a very common clinical entity. Resective surgery can be used to improve the overall survival and disease-free. The problem is that major resections are associated with hepatic failure caused by an inadequate residual volume. Chemotherapy with diagrams as FOLFOX, FOLFIRI associated with antibodies such as bevacizumab, cetuximab or panitumumable added an important role in the conversion of unresectable to resectable tumors. Another widely used technique is portal vein ligation in a first surgical procedure, that generates left hepatic growth, to perform the resection in a second surgical procedure. The liver hypertrophy is achieved in a period of 2 months. The latest new technical procedure is the association of the ligation portal to the liver partition of the hepatic parenchyma without resection, which allows a segmental hypertrophy of the liver remnant between 7 to 10 days. This technique is called ALPPS. Radiofrecuency also has a role in the treatment of liver metastases. However,it is secondary.

La metástasis hepáticas del cáncer de colon es una entidad clínica muy frecuente. La cirugía resectiva permite mejorar la sobrevida global y libre de enfermedad. El problema es que grandes resecciones se asocian a insuficiencia hepática por un inadecuado volumen residual. La quimioterapia con esquemas como FOLFOX, FOLFIRI asociada a anticuerpos como bevacizumab, cetuximab o panitumumable agregan un rol importante en la conversión de tumores irresecables a resecables. Otra técnica utilizada es la ligadura portal en un primer tiempo, que genera crecimiento hepático izquierdo, para realizar la resección en un segundo tiempo quirúrgico. La hipertrofia hepática se logra en un período de 2 meses. La última novedad técnica es la asociación de la ligadura portal a la transección del parénquima hepático sin resección, lo que permite una hipertrofia segmentaria del hígado remanente entre 7 a 10 días. Esta técnica se denomina ALPPS. La radiofrecuencia también tiene un rol en el tratamiento de las metástasis hepáticas, aunque este es secundario.

Ictericia post-operatoria / Post-operative jaundice

The occurrence of postoperative jaundice should encourage the surgeon to review the wide spectrum of possible causes of jaundice and perform detailed history, physical examination and laboratory tests to define the causes, and take the measures to treat the patient properly. We also emphasize the need to prevent the presence of residual stones in the bile duct and bile duct iatrogenic injuries. This paper describe the possible causes of postoperative jaundice and current recommendations for adequate treatment.

La aparición de ictericia en el postoperatorio debe alertar al cirujano y llevarlo a revisar el gran espectro de causas posibles del cuadro. Se debe realizar una anamnesis y examen físico detallado y complementarlo con exámenes de laboratorio e imágenes según el caso. Además, se insiste en la necesidad de prevenir la presencia de cálculos residuales en colédoco y lesiones iatrogénicas de vía biliar. Se revisan las causas posibles de ictericia postoperatoria y las recomendaciones actuales de manejo.

Localized primary ureteral amyloidosis.

OBJECTIVE: To analyze a new case of primary localized amyloidosis of the ureter, describing the differential diagnosis with ureteral tumors and its pathogenic mechanism. METHOD: Description of the case of a male patient who presented clinical symptoms of renoureteral pain and hematuria and an image suspected of ureteral tumour undergoing laparoscopic nephroureterectomy. The pathological study confirmed amyloidosis of the ureter, AL by immunohistochemistry. RESULTS: This report further extends the slightly more than 50 published cases of primary localized amyloidosis of the ureter the clinical signs of which, pain and haematuria, are similar, showing stenosis and enlargement in an area of the ureteral wall on imaging techniques. In the majority of cases the diagnosis was obtained postoperatively, being necessary to exclude generalized and secondary affectation. CONCLUSIONS: Emphasis must be placed on the differential diagnosis of tumors of the ureter, especially in single-kidney patients, by using ureteroscopy and biopsy or preferably a careful cytological study. In our case a local inflammatory phenomenon could have been the basis of its pathogenesis.

Amiloidosis ureteral primaria localizada / Localized primary ureteral amyloidosis

OBJETIVO: Analizar un nuevo caso de amiloidosis ureteral primaria localizada, ahondando en el diagnóstico diferencial con los tumores ureterales y en su mecanismo patogénico.MÉTODO: Describimos el caso de un paciente varón, al que por clínica de dolor renoureteral y hematuria y sospecha por imagen de tumor ureteral, se practicó nefroureterectomía laparoscópica, confirmando el estudio histológico amiloidosis ureteral, AL por inmunohistoquímica. RESULTADOS: Viene a engrosar los escasos 50 casos publicados de amiloidosis ureteral primaria localizada, cuya manifestación clínica de dolor y hematuria es similar, encontrando estenosis y engrosamiento en una zona de la pared ureteral en técnicas de imagen. En la mayoría el diagnóstico fue postoperatorio, debiendo excluir afectación generalizada y secundaria.CONCLUSIONES: Hay que incidir en el diagnóstico diferencial con los tumores de uréter, sobre todo en pacientes monorrenos, mediante ureteroscopia y biopsia o preferible estudio exhaustivo citológico. En nuestro caso un fenómeno inflamatorio local, pudo ser la base de su patogénesis(AU)

OBJECTIVE: To analyze a new case of primary localized amyloidosis of the ureter, describing the differential diagnosis with ureteral tumors and its pathogenic mechanism.METHOD: Description of the case of a male patient who presented clinical symptoms of renoureteral pain and hematuria and an image suspected of ureteral tumour undergoing laparoscopic nephroureterectomy. The pathological study confirmed amyloidosis of the ureter, AL by immunohistochemistry.RESULTS: This report further extends the slightly more than 50 published cases of primary localized amyloidosis of the ureter the clinical signs of which , pain and haematuria, are similar, showing stenosis and enlargement in an area of the ureteral wall on imaging techniques. In the majority of cases the diagnosis was obtained postoperatively, being necessary to exclude generalized and secondary affectation.CONCLUSIONS: Emphasis must be placed on the differential diagnosis of tumors of the ureter, especially in single-kidney patients, by using ureteroscopy and biopsy or preferably a careful cytological study. In our case a local inflammatory phenomenon could have been the basis of its pathogenesis(AU)

[Secondary amyloidosis of the bladder and massive hematuria]. / Amiloidosis vesical secundaria y hematuria masiva.

OBJECTIVE: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. MATERIALS AND METHODS: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. RESULTS: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massive, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor. CONCLUSIONS: Despite its rarity, as shown by the few cases reported, secondary amyloidosis of the bladder should be considered in patients already diagnosed with systemic amyloidosis and/or the conditions reported who require simple urethral catheterization.

Amiloidosis vesical secundaria y hematuria masiva / Secondary amyloidosis of the bladder and massive hematuria

Objetivo: Dar a conocer cuatro nuevos casos de amiloidosis vesical secundaria, cuya manifestación clínica es extraordinariamente rara, si atendemos a los escasos casos publicados que no llegan a la treintena. Material y métodos: Describimos los cuatro casos clínicos, manifestados todos ellos por hematuria, siendo en tres masiva y fulminante, con evolución fatal. Resultados: La amiloidosis vesical secundaria corresponde al tipo AA, más frecuente en mujeres y secundaria sobre todo a artritis reumatoide, pero también a espondilitis anquilopoyética y procesos inflamatorios crónicos de larga evolución. La hematuria es el síntoma fundamental, prácticamente único. El estudio patológico e inmunohistoquímico confirma el diagnóstico. Se da la circunstancia de que los tres casos de hematuria masiva y fatal presentaron una patología intercurrente que precisó practicar un sondaje uretral, siendo este el desencadenante. Conclusiones: A pesar de su rareza, confirmada por los pocos casos publicados, habrá que pensar en ella ante pacientes ya diagnosticados de amiloidosis sistémica y/o con las patologías descritas, que precisen un simple sondaje uretral(AU)

Objective: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. Materials and methods: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. Results: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massiva, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor. Objective: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. Materials and methods: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. Results: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massiva, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor(AU)

[Charles II: From spell to genitourinary pathology]. / Carlos II: del hechizo a su patología génito-urinaria.

OBJECTIVES: We attempt to unravel the complex condition of the last king of the Hapsburg dynasty in Spain, Charles II, called The Bewitched, in whom a genitourinary disorder was preponderant, preventing him from fulfilling one of the objectives of the monarchial institution, engendering a heir, and causing a series of complications that led to his death. METHODS: We review the works describing the life of the King, with special emphasis on his bloodline, the doubts about his sex at birth, his pathological processes, the repercussion among European Courts, but above all on his marriages and the inability to engender an heir. We also emphasize the thought of 17th century Spanish society which led to the belief that he was bewitched. The nickname he passed into history with. RESULTS: It was deduced that he could have presented posterior hypospadias which, together with monorchism and atrophic testicle, led to the belief that he presented an intersexual state with ambiguous genitals. The physical phenotype leans more towards true hermaphro-ditism and above all a XX male, rather than the more often attributed Klinefelter's syndrome. This is probably also associated with a fragile X syndrome. Very possibly congenital monorenal, death was due to chronic kidney failure caused by glomerulopathy or interstitial nephro-pathy as a consequence of renal lithiasis plus recurrent infections of the urinary tract. CONCLUSIONS: As a result of a reiterated endogamic matrimonial policy, the Hapsburg dynasty died out in Spain in 1700, represented by Charles II, a pluripatho-logical king who can only be freed from speculation by chromosomal and genetic studies of his remains buried in El Escorial monastery.

Carlos II: Del hechizo a su patología génito-urinaria / Charles II: From spell to genitourinary pathology

OBJETIVO: Tratar de desentrañar la compleja patología del último rey de la dinastía de los Austrias, Carlos II, apodado El Hechizado, dentro de la cual la urogenital fue preponderante, impidiéndole cumplir con una de las finalidades de la institución monárquica cual es la transmisión a un descendiente y produciéndole una serie de complicaciones que le llevaron al fallecimiento.MÉTODOS: Revisamos las obras en las cuales se describe la vida del Rey, haciendo hincapié en sus antecedentes consanguíneos, en las dudas sobre su sexo en el momento del nacimiento, sus procesos patológicos, la repercusión en las Cortes Europeas, pero sobre todo en sus matrimonios y en la incapacidad para generar un heredero. Resaltamos como, siguiendo el pensamiento de la sociedad española del siglo XVII, hizo pensar que se encontraba hechizado. Sobrenombre con el que pasó a la historia.RESULTADO: Deducimos que pudo presentar un hipospadias posterior que junto con la monorquia y testículo atrófico, hace pensar que presentó un estado intersexual con genitales ambiguos. Su fenotipo físico inclina más hacia un hermafroditismo verdadero y sobre todo un varón XX, que hacia un síndrome de Klinefelter que ha sido el más atribuido. Es probable su asociación con un síndrome X frágil. Monorreno congénito muy posiblemente, su muerte se debió a una insuficiencia renal crónica producida por una glomerulopatía o una nefropatía intersticial a consecuencia de una litiasis renal más infecciones del tracto urinario recidivantes.CONCLUSIONES: Fruto de una reiterada política matrimonial endogámica, feneció en 1700 la dinastía de los Habsburgo en España encarnada en Carlos II, un monarca pluripatológico que sólo se libraría de especulaciones si se efectuaran estudios cromosómicos y genéticos de sus restos presentes en el monasterio de El Escorial(AU)

OBJECTIVES: We attempt to unravel the complex condition of the last king of the Hapsburg dynasty in Spain, Charles II, called The Bewitched, in whom a genitourinary disorder was preponderant, preventing him from fulfilling one of the objectives of the monarchial institution, engendering a heir, and causing a series of complications that led to his death. METHODS: We review the works describing the life of the King, with special emphasis on his bloodline, the doubts about his sex at birth, his pathological processes, the repercussion among European Courts, but above all on his marriages and the inability to engender an heir. We also emphasize the thought of 17th century Spanish society which led to the belief that he was bewitched. The nickname he passed into history with.RESULT: It was deduced that he could have presented posterior hypospadias which, together with monorchism and atrophic testicle, led to the belief that he presen-ted an intersexual state with ambiguous genitals. The physical phenotype leans more towards true hermaphro-ditism and above all a XX male, rather than the more often attributed Klinefelter’s syndrome. This is probably also associated with a fragile X syndrome. Very possibly congenital monorenal, death was due to chronic kidney failure caused by glomerulopathy or interstitial nephro-pathy as a consequence of renal lithiasis plus recurrent infections of the urinary tract.CONCLUSIONS: As a result of a reiterated endogamic matrimonial policy, the Hapsburg dynasty died out in Spain in 1700, represented by Charles II, a pluripatho-logical king who can only be freed from speculation by chromosomal and genetic studies of his remains buried in El Escorial monaster(AU)