223Ra-dichloride spectrometric characterization: Searching for the presence of long-lived isotopes with radiological protection implications.

223Ra-dichloride was approved with the commercial name of Xofigo in 2014 for treatment of metastatic castration-resistant prostate cancer. 223Ra is obtained by neutron irradiation of 226Ra yielding 227Ac, which decays to 227Th and 223Fr, both decaying to 223Ra. Since 223Ra is predominantly (95.3%) an alpha emitter with a 11.42days long half-life, the radiopharmaceutical, its remnants, the patient, and waste material can be managed and disposed with low radiation protection requirements. 227Ac is a long-lived (T1/2=21.77years) beta emitter that demands strong radiation protection measures. In particular waste disposal has to follow the International Atomic Energy Agency (IAEA) and European Commission (EC) regulations. Since 227Ac is involved in the production of 223Ra, an impurity analysis of each batch is required after production. Due to time restrictions, the manufacturer's detection limit (<0.001%) exceeds the one required to assure that 227Ac concentrations are below direct disposal levels. To improve the detection limit, long-term accurate spectroscopy is required. Alpha and gamma spectroscopy measurements were carried out at the Complutense University Nuclear Physics Laboratory. After twelve months follow up of a sample, 227Ac concentration was found to be smaller than 10-9. This allows for direct waste disposal and no additional radiation protection restrictions than those required for 223Ra. The presence of contamination by other radioisotopes was also ruled out by this experiment. Specifically 226Ra, involved in 223Ra production as the original parent and with a very long-lived (T1/2=1577years) alpha emitter, was also below the experimental detection limit.

[Short cold ischaemia time optimises transplant results for kidneys from expanded criteria donors]. / El tiempo de isquemia fría corto optimiza los resultados de los trasplantes renales efectuados con donantes con criterios expandidos.

INTRODUCTION: Outcome of renal transplant from expanded criteria donors (ECD) is usually inferior than those from standard criteria donors (SCD) and may be improved decreasing cold ischemia time (CIT) and minimizing preservation injury. We compare the results obtained with CIT <15 hours in kidney transplants from ECD vs SCD. SUBJECTS AND METHODS: Prospective, single center study of kidney transplants performed since June 2003 to December 2007. Minimum follow-up period was 12 months. Data of donors, receptors and transplant outcome from ECD and SCD are compared. RESULTS: CIT (mean +/- SD) was 9.3+/-2.5 hours in transplants from ECD (n=24) and 8.3+/-3.3 hours in those from SCD (N=50), p=0.18. We did not find significant differences among recipients of grafts from ECD and those from SCD regarding: primary non-function (4.2% vs 2%, respectively), delayed graft function (16.7% vs 10%), surgical complications (25% vs 16%) or acute rejection episodes (8.3% vs 2%). Glomerular filtration rate at one year follow-up was 65.8+/-14.9 ml/min in ECD recipients and 49.4+/-12.5 ml/min (p<0.0001). One year graft survival was 95.8% in ECD recipients and 94% in SCD recipients (p=0.75). CONCLUSIONS: Short CIT in kidney transplant from ECD leads to similar outcome than that obtained from SCD, although renal function is inferior in ECD grafts.

El tiempo de isquemia fría corto optimiza los resultados de los trasplantes renales efectuados con donantes con criterios expandidos / No disponible

Introducción: Los resultados de los trasplantes efectuados condonantes con criterios expandidos (DCE) son inferiores a los obtenidos con donantes con criterios estándar (DCS). Para optimizar su evolución, se podría reducir su tiempo de isquemiafría (TIF) reduciendo su daño de preservación. Comparamoslos resultados obtenidos al aplicar TIF <15 horas tanto a DCE como a DCS. Material y métodos: Realizamos un estudio unicéntrico, de cohortes, prospectivo, de casos incidentes de trasplante renal de cadáver entre junio de 2003 y diciembre de2007. El tiempo mínimo de seguimiento fue de 12 meses. Comparamos los datos de los donantes, de los receptores y de la evolución de los trasplantes efectuados con DCE frente a los de los DCS. Resultados: El TIF para los DCE (N = 24) y para los DCS (N = 50) fue, respectivamente, de 9,3 ± 2,5 y 8,3± 3,3 horas (p = 0,18). No encontramos diferencias significativas entre los receptores de DCE y DCS en cuanto a: no función primaria del injerto 4,2 vs. 4%, retardo en la función del injerto 16,7 vs. 10%, complicaciones quirúrgicas 25 vs. 16% y rechazos agudos 8,3 vs. 2%. El filtrado glomerular estimado al año para los DCS fue de 65,8 ± 14,9 ml/min y para los DCE de 49,4 ± 12,5 ml/min (p <0,0001). La supervivencia renal al año fue del 95,8% para los receptores de DCE y del 94% para los DCS (p = 0,75). Conclusiones: La aplicación de TIF cortos a los DCE permite conseguir una evolución similar a la de los DCS, aunque su función renal sea en todo momento inferior (AU)

Introduction: Outcome of renal transplant from expanded criteria donors (ECD) is usually inferior than those from standard criteria donors (SCD) and may be improved decreasing cold ischemia time (CIT) and minimizing preservation injury. We compare the results obtained with CIT <15 hours in kidney transplants from ECD vs. SCD. Subjects and Methods: Prospective, single center study of kidney transplants performed since June 2003 to December 2007. Minimum follow-up period was 12months. Data of donors, receptors and transplant outcome from ECD and SCD are compared. Results: CIT (mean ± SD)was 9.3 ± 2.5 hours in transplants from ECD (n = 24) and8.3 ± 3.3 hours in those from SCD (N = 50), p = 0.18. We did not find significant differences among recipients of grafts from ECD and those from SCD regarding: primary non-function (4.2% vs. 2%, respectively), delayed graft function (16.7% vs. 10%), surgical complications (25% vs.16%) or acute rejection episodes (8.3% vs. 2%).Glomerular filtration rate at one year follow-up was 65.8± 14.9 ml/min in ECD recipients and 49.4 ± 12.5 ml/min (p<0.0001). One year graft survival was 95.8% in ECD recipients and 94% in SCD recipients (p = 0.75).Conclusions: Short CIT in kidney transplant from ECD leads to similar outcome than that obtained from SCD, although renal function is inferior in ECD grafts (AU)

Fracaso renal agudo por microangiopatía trombótica asociado a enfermedad de Castleman / Acute renal failure due to thrombotic microangiopathy associated with Castleman´s disease

Publicamos un caso de un paciente de 30 años que acude al servicio de Urgencias con dolor abdominal, fiebre, inestabilidad hemodinámica, hepatoesplenomegalia, fracaso renal agudo anúrico, linfadenopatías latero-cervicales, anemia y trombocitopenia. El paciente fue tratado con antibióticos empíricos, esteroides, gammaglobulinas, noradrenalina y hemodiálisis intermitente diaria con buena respuesta. La biopsia renal mostró datos de microangiopatía trombótica y tras la biopsia de una adenopatía se diagnosticó de enfermedad de Castleman. La enfermedad de Castleman, también conocida como hiperplasia nodular linfática gigante o angiofolicular, es una entidad clínico-patológica de etiología desconocida. La afectación renal és heterogénea y frecuente (AU)

We report a case of a 30-year-old man presenting with abdominal pain, fever, homodynamic instability, hepatosplenomegaly, acute renal failure, cervical lymph nodes, anaemia and thrombocytopenia. The patient was treated with empiric antibiotics, high dose corticosteroids, gammaglobulins, noradrenalin and diary intermittent haemodialysis, with an excellent response. The renal biopsy showed a thrombotic microangiopathy, the lymph node biopsy showed a Castleman’s disease.Castleman’s disease (also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia) is a clinicopathological entity of unknown aetiology. A number of renal alterations have been described in association with the Castleman’s disease (AU)

[Acute renal failure due to thrombotic microangiopathy associated with Castleman's disease]. / Fracaso renal agudo por microangiopatía trombótica asociado a enfermedad de Castleman.

We report a case of a 30-year-old man presenting with abdominal pain, fever, homodynamic instability, hepatosplenomegaly, acute renal failure, cervical lymph nodes, anaemia and thrombocytopenia. The patient was treated with empiric antibiotics, high dose corticosteroids, gammaglobulins, noradrenalin and diary intermittent haemodialysis, with an excellent response. The renal biopsy showed a thrombotic microangiopathy, the lymph node biopsy showed a Castleman s disease. Castleman s disease (also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia) is a clinicopathological entity of unknown aetiology. A number of renal alterations have been described in association with the Castleman s disease.

Pubertad precoz. Actualización en el diagnóstico y tratamiento / Precocious puberty. An update on the diagnosis and treatment

El término pubertad precoz define el inicio del desarrollode los caracteres secundarios antes de los 8años en niñas y antes de los 9 años en niños. Eldiagnóstico precoz es fundamental para conseguiruna talla adulta final adecuada. Es importante distinguirla pubertad precoz verdadera de otras situacionesclínicas conocidas como variantes normales deldesarrollo puberal, que únicamente requieren uncontrol clínico periódico. Tras el diagnóstico de pubertadprecoz hay que individualizar la indicación detratamiento, e identificar a aquellos pacientes que sepuedan beneficiar del mismo. En los casos en losque esté indicado, para el tratamiento de la pubertadprecoz central idiopática disponemos de análogosde GnRH, que usados de forma crónica producenuna desensibilización de la hipófisis al estímulode la GnRH, reduciendo la liberación de gonadotropinas

The term Precocious puberty defines the beginningof the development of secondary sexual characteristicsbefore age of 8 in girls and before 9 inboys. Early diagnosis is fundamental in order toachieve full adequate final adult height. It is importantto distinguish true Precocious puberty from otherclinical situations known as normal variations ofdevelopment, which only need regular clinical control.After the diagnosis of Precocious puberty onemust individualize the treatment indications, andidentify those patients who may benefit from the same.In the cases in which it should be indicated, wehave GnRH agonists available, which when used chronicallyinhibits gonadotripin secretion

[Rapid recurrence of ANCA-MPO positive vasculitis after non-heart beating donor renal transplantation]. / Recurrencia rápida de una vasculitis ANCA-MPO positivo tras trasplante renal de donante en asistolia.

We report a patient with end stage renal disease with lesions compatibles with renal vasculitis antineutrophil cytoplasmic autoantibody (ANCA)-associated in phase of sclerosis that underwent renal transplantation from a non-heart beating donor after one year of haemodialysis treatment, without evidence of active vasculitis. Post-transplantation management was performed according to our protocol in this kind of donors with immunosuppressive treatment based on daclizumab, half-doses of tacrolimus, mycophenolate mofetil and steroids. In the third week the renal biopsy showed an acute necrotizing vasculitis associated with crescent glomerulonephritis. The patient was initially diagnosed of acute vascular rejection and initiated treatment with 6-metilprednisolone and anti-CD3 monoclonal anti-bodies. Two days later he developed a cutaneous purpura and the skin biopsy showed an acute necrotizing vasculitis. The determination of circulating ANCA-anti-myeloperoxidase (MPO) was positive. We initiated treatment with oral cyclophosphamide plus mycophenolate mofetil discontinuation with rapid improvement of cutaneous lesions and initiation of renal function recovery.

Recurrencia rápida de una vasculitis ANCA-MPO positivo tras trasplante renal de donante en asistolia / Rapid recurrence of ANCA-MPO positive vasculitis after non-heart beating donor renal transplantation

Publicamos un caso de un paciente con insuficiencia renal avanzada con lesionescompatibles con vasculitis renal asociada a anticuerpos contra citoplasmade neutrófilos (ANCA) en fase de esclerosis que recibió un injerto renal de cadáveren asistolia tras un año en programa de hemodiálisis con ausencia de sintomatologíavasculítica. El tratamiento inmunosupresor se realizó acorde a nuestroprotocolo en este tipo de donantes basado en daclizumab, dosis media detacrolimus, micofenolato mofetil y esteroides.En la tercera semana post-trasplante la biopsia renal mostró una glomerulonefritisnecrotizante con semilunas. El paciente fue inicialmente diagnosticado de rechazoagudo vascular y recibió tratamiento con 6-metilprednisolona y anticuerposmonoclonales anti-CD3. Dos días más tarde el paciente desarrolló una púrpuracutánea cuya biopsia mostró una vasculitis aguda necrotizante. La determinaciónde ANCA circulante anti mieloperoxidasa (MPO) fue positiva. Se inició tratamientocon ciclofosfamida oral retirándose el micofenolato mofetil, con rápida desapariciónde las lesiones purpúricas y mejoría de la función renal

We report a patient with end stage renal disease with lesions compatibles withrenal vasculitis antineutrophil cytoplasmic autoantibody (ANCA) - associated inphase of sclerosis that underwent renal transplantation from a non-heart beatingdonor after one year of haemodialysis treatment, without evidence of active vasculitis.Post-transplantation management was performed according to our protocolin this kind of donors with immunosuppressive treatment based on daclizumab,half-doses of tacrolimus, mycophenolate mofetil and steroids. In the third week the inmurenalbiopsy showed an acute necrotizing vasculitis associated with crescent glomerulonephritis.The patient was initially diagnosed of acute vascular rejection and initiated treatmentwith 6-metilprednisolone and anti-CD3 monoclonal anti-bodies. Two dayslater he developed a cutaneous purpura and the skin biopsy showed an acute necrotizingvasculitis. The determination of circulating ANCA-anti-myeloperoxidase(MPO) was positive. We initiated treatment with oral cyclophosphamide plus mycophenolatemofetil discontinuation with rapid improvement of cutaneous lesionsand initiation of renal function recovery

Recurrencia rápida de una vasculitis ANCA-MPO positivo tras trasplante renal de donante en asistolia / Rapid recurrence of ANCA-MPO positive vasculitis after non-heart beating donor renal transplantation

Publicamos un caso de un paciente con insuficiencia renal avanzada con lesiones compatibles con vasculitis renal asociada a anticuerpos contra citoplasma de neutrófilos (ANCA) en fase de esclerosis que recibió un injerto renal de cadáver en asistolia tras un año en programa de hemodiálisis con ausencia de sintomatología vasculítica. El tratamiento inmunosupresor se realizó acorde a nuestro protocolo en este tipo de donantes basado en daclizumab, dosis media de tacrolimus, micofenolato mofetil y esteroides. En la tercera semana post-trasplante la biopsia renal mostró una glomerulonefritis necrotizante con semilunas. El paciente fue inicialmente diagnosticado de rechazo agudo vascular y recibió tratamiento con 6-metilprednisolona y anticuerpos monoclonales anti-CD3. Dos días más tarde el paciente desarrolló una púrpura cutánea cuya biopsia mostró una vasculitis aguda necrotizante. La determinación de ANCA circulante anti mieloperoxidasa (MPO) fue positiva. Se inició tratamiento con ciclofosfamida oral retirándose el micofenolato mofetil, con rápida desaparición de las lesiones purpúricas y mejoría de la función renal

We report a patient with end stage renal disease with lesions compatibles with renal vasculitis antineutrophil cytoplasmic autoantibody (ANCA) - associated in phase of sclerosis that underwent renal transplantation from a non-heart beating donor after one year of haemodialysis treatment, without evidence of active vasculitis. Post-transplantation management was performed according to our protocol in this kind of donors with immunosuppressive treatment based on daclizumab, half-doses of tacrolimus, mycophenolate mofetil and steroids. In the third week the inmurenal biopsy showed an acute necrotizing vasculitis associated with crescent glomerulonephritis. The patient was initially diagnosed of acute vascular rejection and initiated treatment with 6-metilprednisolone and anti-CD3 monoclonal anti-bodies. Two days later he developed a cutaneous purpura and the skin biopsy showed an acute necrotizing vasculitis. The determination of circulating ANCA-anti-myeloperoxidase (MPO) was positive. We initiated treatment with oral cyclophosphamide plus mycophenolate mofetil discontinuation with rapid improvement of cutaneous lesions and initiation of renal function recovery

[Meningoencephalitis by Listeria in the lupus disease]. / Meningoencefalitis por Listeria en el lupus.

We present a patient with lupus nephropathy of 20 years of evolution in treatment with oral steroids who developed a meningoencephalitis associated to bacteraemia by Listeria monocytogenes. The patient was treated successfully with gentamicin and ampicillin for 6 weeks. Infection by Listeria monocytogenes occurs more frequently in individuals with some form of immunodeficiency like lupus disease, with a mortality around 30%.

Meningoencefalitis por Listeria en el lupus / Meningoencephalitis by Listeria in the lupus disease

Presentamos un caso de una paciente con nefropatía lúpica de 20 años de evolución en tratamiento con esteroides que desarrolló una meningoencefalitis asociada a bacteriemia por Listeria monocytogenes. La paciente recibió tratamiento antibiótico con ampicilina y gentamicina durante 6 semanas con excelentes resultados. La infección por Listeria monocytogenes afecta predominantemente a pacientes con cierto grado de inmunosupresión, como pacientes con lupus eritematoso sistémico, con una mortalidad alrededor del 30%

We present a patient with lupus nephropathy of 20 years of evolution in treatment with oral steroids who developed a meningoencephalitis associated to bacteraemia by Listeria monocytogenes. The patient was treated successfully with gentamicin and ampicillin for 6 weeks. Infection by Listeria monocytogenes occurs more frequently in individuals with some form of immunodeficiency like lupus disease , with a mortality around 30%

[Nephropathia caused by hantavirus puumala: a case report]. / Nefropatía por hantavirus puumala: a propósito de un caso.

We present a patient from Germany with Hantavirus infection, admitted in the Emergency room of our hospital, with fever, thrombocytopenia, acute renal failure, oliguria, mild proteinuria and hematuria. Percutaneous renal biopsy revealed an acute interstitial nephritis without medulla haemorrhages. The virus infection confirmation was made by detection of IgM against Hantavirus Puumala. This infection should be considered in patients with thrombocytopenia, fever and acute renal failure, over all if they are from North and Central Europe.

Nefropatía por hantavirus puumala: a propósito de un caso / Nephropathia caused by hantavirus puumala: a case report

Presentamos un caso de un paciente alemán con infección por Hantavirus, queacudió al Servicio de Urgencias de nuestro hospital con fiebre, trombocitopenia,fracaso renal agudo oligúrico, proteinuria y hematuria. La biopsia renal percutaneamostró una nefritis intersticial aguda sin hemorragias medulares. El diagnósticose realizó por la positividad de IgM frente al virus Puumala. La infección porHantavirus debe tenerse en cuenta en pacientes con fracaso renal agudo y fiebrecon trombocitopenia, sobre todo si proceden del Centro y Norte de Europa

We present a patient from Germany with Hantavirus infection, admitted in theEmergency room of our hospital, with fever, thrombocytopenia, acute renal failure,oliguria, mild proteinuria and hematuria. Percutaneous renal biopsy revealedan acute interstitial nephritis without medulla haemorrhages. The virus infectionconfirmation was made by detection of lgM against Hantavirus Puumala. This infectionshould be considered in patients with thrombocytopenia, fever and acuterenal failure, over all if they are from North and Central Europe

[Porphyria and inappropriate antidiuretic hormone syndrome]. / Síndrome de secreción inadecuada de hormona antidiurética y porfiria.

We report the case of a 37-years-old woman with inappropriate antidiuretic hormone syndrome due to an attack of acute porphyria. The patient was admitted to our hospital for abdominal pain, sleepiness and pink urine. Family and personal history were normal. Seven days before the admission the patient had a laparoscopy operation for endometriosis in her left ovary. The patient had had two normal pregnancies. The physical examination was normal, the skin turgor was good and no edema was present, the blood pressure was 140/90 mmHg. Her serum sodium was 114 mEq/L, serum osmolality 243 mOsm/kg, urine sodium 146 mEq/L and urine osmolality 457 mOsm/kg. Values from laboratory examination revealed a normal peripheral haematogram, a normal kidney function, normal liver, adrenal and thyroid function. The urine tested for amino-levulinic acid, coproporphyrin and uroporphyrin was strongly positive. These findings are compatible with Porphyria Variegata or Coproporphyria Hereditary. A diagnosis of Porphyria acute with SIADH was made, and water fluid restriction, i.v. hypertonic saline infusion and furosemide to correct the hyponatremia was begun. In 1966, lesions of the median eminence of the hypothalamus and both hypothalamic -hypophyseal tracts were described in a patient with Porphyria acute intermittent and SIADH. It was suggested that SIADH occurred because of damage to these areas of the brain from excessive exposure to porphyrins.

[Primary amyloidosis associated to severe factor X deficiency]. / Amiloidosis primaria (AL) asociada a déficit severo de factor X.

Amyloidosis is a systemic disease characterized by generalized deposition of beta-organized proteic fibrillar material with green birefringence under polarized light, in different tissues and organs, the most frequent kidney, liver and heart, with important clinical repercussion. Primary or AL amyloidosis is the most common subtype of amyloidosis (1), confirmed by biopsy-proved amyloid deposition in abdominal fat pad, rectum, kidney or liver, if necessary, in which fragments of monoclonal light chains are deposited. Cases with factor X (Stuart factor) of coagulation deficiency associated are described, due to adsorption of this factor to amyloid fibrills. Normally, evolution is fatal, with only few months of survival. We report a case of primary amyloidosis with nephrotic syndrome, severe factor X deficiency (without bleeding complications), possible heart affection and short-term good response to chemotherapic treatment.

Síndrome de secreción inadecuada de hormona antidiurética y porfiria / Porphyria and inappropriate antidiuretic hormone síndrome

Describimos el caso de una mujer de 37 años que ingresa en el Servicio de Cirugía General por un cuadro de dolor abdominal y niveles hidroaéreos en la radiografía simple de abdomen con el diagnóstico de Suboclusión Intestinal. La paciente era madre de dos hijos y no tenía antecedentes de interés personales ni familiares. Siete días previos al ingreso había sido sometida a una intervención vía laparoscópica por una Endometriosis ovárica izquierda. Al ingreso la paciente refería dolor abdominal intenso y continuo acompañado de orinas rosadas, sin síndrome miccional y con cierta tendencia al sueño. Analíticamente destacaba una hiponatremia de 114 mEq/l con osmolalidad plasmática de 243 mOsm/kg, sodio en orina de 146 mEq/l, osmolalidad urinaria de 457 mOsm/kg con función renal y sedimento urinario normal. En el examen físico la paciente estaba normohidratada con buen estado general y sin signos de irritación peritoneal, con ruidos hidroaéreos disminuidos. Se diagnostica de Síndrome de secreción inadecuada de hormona antidiurética (SIADH) por lo que se trata con restricción hí- drica, suero salino hipertónico y furosemida. Con la presencia de un SIADH y ante el cuadro que presentaba la paciente se sospecha una porfiria aguda, siendo los niveles de uroporfirinógeno, ácido delta amino-levulínico y coproporfirinas en orinas altos, compatible con Porfiria Variegata o Coproporfiria hereditaria (AU)

We report the case of a 37-years-old woman with inappropriate antidiuretic hormone syndrome due to an attack of acute porphyria. The patient was admitted to our hospital for abdominal pain, sleepiness and pink urine. Family and personal history were normal. Seven days before the admission the patient had a laparoscopy operation for endometriosis in her left ovary. The patient had had two normal pregnancies. The physical examination was normal, the skin turgor was good and no edema was present, the blood pressure was 140/90 mmHg. Her serum sodium was 114 mEq/L, serum osmolality 243 mOsm/kg, urine sodium 146 mEq/L and urine osmolality 457 mOsm/kg. Values from laboratory examination revealed a normal peripheral haematogram, a normal kidney function, normal liver, adrenal and thyroid function. The urine tested for amino-levulinic acid, coproporphyrin and uroporphyrin was strongly positive. This findings are compatible with Porphyria Variegata or Coproporphyria Hereditary. A diagnosis of Porphyria acute with SIADH was made, and water fluid restriction, i.v. hypertonic saline infusion and furosemida to correct the hyponatremia was begun. In 1966, lesions of the median eminence of the hypothalamus and both hypothalamic –hypophyseal tracts were described in a patient with Porphyria acute intermittent and SIADH. It was suggested that SIADH occurred because of damage to these areas of the brain from excessive exposure to porphyrins (AU)