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.

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)

[Socioeconomic justification of kidney transplantation]. / Justificación socioeconómica del trasplante renal.

OBJECTIVES: In recent years, the socioeconomic aspects of renal transplantation have focused the attention of the managers of the health care system and the professionals involved in transplant programs. The present study analyzed, within the context of renal transplantation, the two fundamental concepts of health care: quality of life and health costs. METHODS: A survey was conducted in 427 patients who underwent renal transplantation to evaluate the improvement in their quality of life. RESULTS: 93% referred physical improvement, 85% emotional, and 85% subjective improvement in their family relationship. Considering 9% of the kidneys were not viable, 15% of the grafts were lost in the first year and 4% thereafter, the cost of transplantation for the first year (in million pesetas) was 3,597 versus 2,961 for hemodialysis and 905 for the second year, with hemodialysis costs practically unchanged. CONCLUSIONS: Renal transplantation not only increases survival but also improves quality of life. It is less costly and the savings in health costs can be allocated to other fields of health care.

[Non-instrumental traumatism of the urethra: our experience]. / Traumatismos no instrumentales de la uretra: nuestra experiencia.

OBJECTIVES: To report our experience in the diagnosis and treatment of this type of urologic trauma. The results are analyzed in order to determine the best therapeutic approach and the literature reviewed. METHODS: The series comprised 9 males; 6 had rupture of the posterior urethra and 3 had anterior urethral trauma. Four cases underwent suprapubic drainage and delayed definitive treatment; early realignment of the urethra was performed in 3 cases, and the only patient with a penetrating injury immediately underwent a repair procedure. Urethral urethrotomy sufficed in one patient with partial rupture of the anterior urethra. Delayed treatment consisted of end-to-end anastomosis; one patient required pubic partial resection. RESULTS: Good results were achieved by end-to-end anastomosis; 3 patients have been discharged and one required internal urethrotomy. Two of these patients have remained impotent. The patients submitted to early realignment of the urethra are managed well with regular dilatation and internal urethrotomy in some cases. CONCLUSIONS: We found suprapubic drainage and delayed repair superior to early realignment in those cases with posterior urethral injury. In our view, delayed end-to-end anastomosis, with or without pubic resection can be performed in virtually all cases. We advocate immediate repair of penetrating injuries to the anterior urethra.

[Renal cystic mass: the diagnostic dilemma]. / Masa quística renal: el dilema diagnóstico.

OBJECTIVE: To determine the best diagnostic approach and management of cystic renal mass. METHODS: Four cases are described and the literature reviewed. RESULTS: Of the 4 cases of cystic renal mass, 2 had a previous history of renal trauma. Puncture and aspiration of the cystic content were performed in three cases; cytology was positive for malignancy in one, which was confirmed by the histologic analysis. Two cases underwent nephrectomy although one of the post-traumatic cases had a preoperative diagnosis of pararenal pseudocyst. The literature review showed a significant controversy exists concerning the diagnostic approach and treatment. CONCLUSIONS: We advocate an approach free from tenets, where all the appropriately chosen diagnostic tools may be valid. If doubts persist after diagnostic evaluation, nephrectomy should be performed.

[Rectal involvement secondary to prostatic adenocarcinoma]. / Afectación rectal secundaria a adenocarcinoma prostático.

Rectal involvement secondary to adenocarcinoma of the prostate is uncommon and carries a poor prognosis. Distinguishing secondary from primary rectal tumors is mandatory, since each condition warrants a completely different treatment. There are at least five forms of clinicopathological involvement, depending on the rectal wall invasion and its consequences. We report on 6 cases, 4 had been diagnosed when rectal invasion was present, the enteric involvement appeared during the process in 1 patients and the other had prostate carcinoma coexisting with squamous cell carcinoma of the anus.

[Verrucous carcinoma of the penis: our caseload]. / Carcinoma verrucoso de pene: nuestra casuística.

Verrucous carcinoma, a tumor that encompasses approximately 5% of all penile neoformations, is a variant of squamous cell carcinoma which is benign and has a peculiar histological behaviour. We report on three patients aged 57, 80 and 84 years with this tumor type who underwent surgery (circumcision in one case and partial penectomy in the remaining two cases). The literature is briefly reviewed and the epidemiological, clinical, diagnostic and therapeutic aspects are discussed.

[Medico-surgical management of renal leiomyosarcoma]. / Manejo médico-quirúrgico del leiomiosarcoma renal.

Because renal leiomyosarcoma is extremely rare, the experience in the management of this tumor type is necessarily scant. Our surgical and chemotherapeutic approach in a 17-year-old male patient with renal leiomyosarcoma achieved partial remission of tumor recurrence and a survival of 31 months following diagnosis.

[2 new cases of ++urethral diverticuli in women]. / Dos nuevos casos de divertículos uretrales femeninos.

The first two cases of diverticulum of the female urethra seen in our Service are described. The secretion of purulent material from the urethra on vaginal compression prompted us to suspect this condition, which could not be confirmed radiologically in one case and a urethroscopy procedure was required. The etiopathogenesis of this disease is briefly reviewed and the therapeutic possibilities depending on the location of the urethral diverticulum are discussed.

[Bilateral renal lymphoma]. / Linfoma renal bilateral.

Lymphomas can present as a solid tumor mass in one or both kidneys or compromise renal function directly or indirectly. Lymphoma should be discarded before performing surgical excision of a rapidly growing renal mass, particularly if involvement is bilateral. Reducing the renal mass may lead to difficulty in instituting specific chemotherapy for this tumor type.

[Leydig cell tumor in a patient with type I multiple endocrine neoplasm: study of a case and review of the literature]. / Tumor de células de Leydig en paciente con neoplasia endocrina múltiple tipo I: estudio de un caso y revisión de la literatura.

Presentation of the case of a 42-year old male patient with Type I Multiple Endocrine Neoplasia, known as the Wermer Syndrome, who developed Leydig's cells tumour. No association was found between both processes and therefore a fortuitus, though worthwhile mentioning given the characteristics and hormonal implications of both pathologies, can be presumed.

[Lymphatic involvement predominating and revealing prostatic carcinoma]. / Afectación linfática predominante y reveladora en el carcinoma prostático.

The lymphatic system is the most common site of metastasis of prostatic carcinoma. However, carcinoma of the prostate presenting as lymphadenopathy is rare. It can present at any site and may sometimes mimic lymphoreticular tumors. The diagnosis is based on the histological findings. Histochemical techniques using immunoperoxidase demonstration of acid phosphatase and prostate specific antigen are fundamental in detecting the occult tumor of the prostate. Treatment is palliative and enlargement may occasionally remit with hormone therapy. The prognosis is invariably poor. Survival is less than 5 years irrespective of the response to treatment. Three cases are described herein: 2 presented as retroperitoneal mass and 1 as left lateral cervical lymphadenopathy.