El enigma de la enfermedad de Enrique IV, rey de Castilla: ¿padeció síndrome de McCune-Albright/displasia fibrosa? / The enigma of Henry IV's disease: Did he suffer from McCune-Albright syndrome/fibrous dysplasia?

Introducción: Enrique IV Rey de Castilla, último rey de la dinastía Trastámara, era hermano de Isabel la Católica. Se le conoce como «el impotente». Basándose en las descripciones previas de los historiadores y biógrafos, Gregorio Marañón en 1922 lo catalogó de «Displásico eunucoide con reacción acromegálica y con netos rasgos esquizoides». Métodos: En 1946 se realizó una inspección post mortem del cadáver momificado hallado en el Monasterio de Guadalupe. Se dejó constancia de un documento escrito y algunas fotografías. Hemos recogido los signos y síntomas descritos y aplicado la clasificación internacional de las enfermedades recomendada por la Organización mundial de la Salud, CIE11-2023. También nos hemos apoyado en las monedas emitidas en el monetario de Enrique IV, en las que hemos identificado aumento de la glándula tiroides. Resultados: Con los datos que están accesibles hasta este momento, sugerimos que Enrique IV padeció de forma altamente probable: displasia ósea facial y poliostótica, cifosis, cojera de una extremidad, alteraciones endocrinas múltiples, acromegalia con macrognatia, enfermedad nodular tiroidea, diaforesis maloliente, disfunción eréctil, hipospadias, desarrollo sexual anómalo, «pelvis feminoide», cólicos abdominales, oligodoncia y desplazamientos dentales. Es posible que también padeciera: pubertad precoz, litiasis renal con fosfaturia debilitante, túnel carpiano, trombocitopenia e hiperplasia o adenoma hipofisario productor de hormona de crecimiento. Conclusión: Sugerimos que Enrique IV pudo sufrir un síndrome de McCune-Albrigth asociado a Displasia fibrosa, una enfermedad rara debida a mutaciones activadoras de función en el gen GNAS.(AU)

Background: Henry IV King of Castile, last king of the Trastámara dynasty, was the brother of Isabella the Catholic. He is known as “the impotent”. Based on previous descriptions by historians and biographers, Gregorio Marañón in 1922 described him as “eunuchoid dysplastic with acromegalic reaction and clear schizoid features”. Methods: In 1946, a post-mortem inspection was carried out on the mummified corpse found in the Monastery of Guadalupe. A written document and some photographs were recorded. We have collected the signs and symptoms described and applied the international classification of diseases recommended by the World Health Organisation, ICD11-2023. We have relied on the coins issued in the money of Henry IV, on which we have identified enlargement of the thyroid gland. Results: With the data available at this time, we suggest that Henry IV most probably suffered from: facial and polyostotic bone dysplasia, kyphosis, limb limping, multiple endocrine disorders, acromegaly with macrognatia, nodular thyroid disease, malodorous diaphoresis, erectile dysfunction, hypospadias, abnormal sexual development, “feminoid pelvis”, abdominal colic, oligodontia and dental displacement. It is possible that he also suffered from: precocious puberty, renal lithiasis with debilitating phosphaturia, carpal tunnel, thrombopenia and growth hormone-producing pituitary hyperplasia or adenoma. Conclusion: We suggest that Henry IV may have suffered from McCune–Albrigth syndrome associated with fibrous dysplasia, a rare disease due to gain-of-function mutations in the GNAS gene.(AU)

[The enigma of Henry IV's disease: Did he suffer from McCune-Albright syndrome/fibrous dysplasia?] / El enigma de la enfermedad de Enrique IV, rey de Castilla: ¿padeció síndrome de McCune-Albright/displasia fibrosa?

BACKGROUND: Henry IV King of Castile, last king of the Trastámara dynasty, was the brother of Isabella the Catholic. He is known as "the impotent". Based on previous descriptions by historians and biographers, Gregorio Marañón in 1922 described him as "eunuchoid dysplastic with acromegalic reaction and clear schizoid features". METHODS: In 1946, a post-mortem inspection was carried out on the mummified corpse found in the Monastery of Guadalupe. A written document and some photographs were recorded. We have collected the signs and symptoms described and applied the international classification of diseases recommended by the World Health Organisation, ICD11-2023. We have relied on the coins issued in the money of Henry IV, on which we have identified enlargement of the thyroid gland. RESULTS: With the data available at this time, we suggest that Henry IV most probably suffered from: facial and polyostotic bone dysplasia, kyphosis, limb limping, multiple endocrine disorders, acromegaly with macrognatia, nodular thyroid disease, malodorous diaphoresis, erectile dysfunction, hypospadias, abnormal sexual development, "feminoid pelvis", abdominal colic, oligodontia and dental displacement. It is possible that he also suffered from: precocious puberty, renal lithiasis with debilitating phosphaturia, carpal tunnel, thrombopenia and growth hormone-producing pituitary hyperplasia or adenoma. CONCLUSION: We suggest that Henry IV may have suffered from McCune-Albrigth syndrome associated with fibrous dysplasia, a rare disease due to gain-of-function mutations in the GNAS gene.

Tratamiento médico del hiperparatiroidismo primario / Medical treatment of primary hyperparathyroidism

El diagnóstico de hiperparatiroidismo primario en pacientes sin criterio quirúrgico es cada vez más frecuente. Aunque la evidencia de calidad es escasa en algunos casos, cada vez se dispone de más datos que nos permiten conocer el efecto de los distintos fármacos sobre la calcemia, la afectación ósea y renal en pacientes sometidos a ellos durante periodos prolongados de tiempo

The diagnosis of primary hyperparathyroidism in patients without surgical criteria is increasingly frequent. Although quality evidence is scarce in some cases, last years there are more data available that allow us to know the effect of different drugs on calcemia, bone and kidney involvement in patients undergoing them for prolonged periods of time

Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy: results from the von Willebrand Disease Prophylaxis Network.

Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed in severe haemophilia A, may also be observed. The bleeding patterns of VWD can affect quality of life, and may be life-threatening. The von Willebrand Disease Prophylaxis Network is an international study group formed with the goal of investigating the role of prophylaxis in clinically severe VWD. The objective of the present study is to investigate the response to prophylaxis focusing primarily on epistaxis, joint bleeding, gastrointestinal bleeding, and heavy bleeding associated with menses. Data from 105 subjects, 10 enrolled in a prospective study and 95 in a retrospective study between 2008 and 2013, were available for analysis. The median annualized rate reductions in bleeding were significant for epistaxis (P < 0.0001), gastrointestinal bleeding (P = 0.0003), joint bleeding (P < 0.0001), and menorrhagia (P = 0.008). Doses on a group level were approximately the same prior to and during prophylaxis, but more patients with gastrointestinal bleeding had prophylaxis three or more times per week as well as higher dosages. Our study, which primarily used retrospective data, indicates that prospective studies are needed to better delineate the doses and dose intervals that should be used for prophylactic treatment of VWD.

Equinococosis alveolar humana. Presentación de un caso / Human alveolar echinococcosis. A case report

Introducción: La equinococosis está causada por diversasespecies de Echinococcus, parásitos céstodos de la familiade las tenias. La especie más frecuentemente observadaes el Echinococcus granulosus, agente causal del quistehidatídico. Otras especies de Echinococcus se observan conpoca frecuencia en nuestro medio. Caso clínico: Presentamosel caso de un varón de 83 años con equinococosis alveolarhumana. Discusión: La equinococosis alveolar humanaes una enfermedad rara, causada por el E. multilocularis. Enpacientes que no reciben tratamiento o que son tratados deforma inadecuada, la mortalidad es muy alta. En Europa, esendémica en Bélgica, Luxemburgo, Holanda, Francia, Alemania,Suiza, Austria, Polonia y la república checa. En lasúltimas dos décadas, como consecuencia del aumento de lapoblación de zorros, se ha observado la propagación de esteparásito a áreas no endémicas. Por ello, el personal médicodebería conocer esta grave enfermedad y saber cómo diagnosticarlay tratarla (AU)

Introduction: Echinococcosis is caused by severalspecies of Echinococcus, cestode parasites in the familyTaeniidaea. The most widely observed specie is E. granulosus,the causative agent of the cystic hydatid disease. Otherspecies of Echinococcus are seldom encountered in ourenvironment. Case report: The case of an 83-year-old malewith human alveolar echinococcosis is presented. Discussion:Human alveolar echinococcosis is a rare zoonotic diseasecaused by E. multilocularis. It has a high mortality ratein untreated or inadequately treated patients. In Europe, it isendemic in Belgium, Luxembourg, Holland, France, Germany,Switzerland, Austria, Poland and the Czech Republic.As a consequence of the increase in fox population, thespread of this parasite has been observed in non-endemicareas over the last two decades. Therefore, medical personnelshould be familiar with this serious disease, and knowhow to diagnose and treat it (AU)

[Streptococcus agalactiae highly resistant to fluoroquinolones]. / Streptococcus agalactiae altamente resistente a fluoroquinolonas.

BACKGROUND: Streptococcus agalactiae remains susceptible to penicillin; nevertheless, an increase in the resistance to other antimicrobial families, such as macrolides and more rarely fluoroquinolones, has been described. METHODS AND RESULTS: From 2003 to 2004, two fluoroquinolone-resistant S. agalactiae strains were detected. The strains presented one mutation in parC (Ser79 --> Phe) and an additional mutation in gyrA (Glu85 --> Ala or Glu85 --> Lys). CONCLUSION: This study describes the first S. agalactiae strains highly resistant to fluoroquinolones in Spain.

Streptococcus agalactiae altamente resistente a fluoroquinolonas / Streptococcus agalactiae highly resistant to fluoroquinolones

Antecedentes. Streptococcus agalactiae se mantiene sensible a la penicilina, si bien se ha descrito un incremento en la resistencia a otras familias de antimicrobianos como macrólidos y más raramente fluoroquinolonas. Métodos y resultados. Entre 2003 y 2004 se detectaron dos cepas de S. agalactiae resistentes a fluoroquinolonas, que presentaron mutaciones en parC (Ser79 → Phe) y gyrA (Glu85 → Ala o Glu85 → Lys). Conclusión. Se describen por primera vez en España cepas de S. agalactiae altamente resistentes a fluoroquinolonas (AU)

Background. Streptococcus agalactiae remains susceptible to penicillin; nevertheless, an increase in the resistance to other antimicrobial families, such as macrolides and more rarely fluoroquinolones, has been described. Methods and results. From 2003 to 2004, two fluoroquinolone-resistant S. agalactiae strains were detected. The strains presented one mutation in parC (Ser79 → Phe) and an additional mutation in gyrA (Glu85 → Ala or Glu85 → Lys). Conclusion. This study describes the first S. agalactiae strains highly resistant to fluoroquinolones in Spain (AU)

[Basic cardiopulmonary resuscitation program for high school students (PROCES). Results from the pilot program]. / Programa de Reanimación cardiopulmonar Orientado a Centros de Enseñanza Secundaria (PROCES): análisis de los resultados del estudio piloto.

BACKGROUND AND OBJECTIVE: The PROCES (Programa de Reanimació Cardiopulmonar Orientat a Centres d'Ensenyament Secundari) program is aimed at teaching basic cardiopulmonary resuscitation (b-CPR) to teenagers within high school. Our aim was to analyze the results obtained from the pilot program. SUBJECTS AND METHOD: PROCES was splitted in 7 sessions: 5 of them (5 hours) were taught by teachers at high school and 2 of them (4 hours, including how to perform b-CPR) were taught by emergency physicians. To assess the degree of students' learning, they were administered a 20-question test before and after the program. Epidemiological characteristics and students' opinions (all them were requested to rate the program from 0 to 10) were also collected. RESULTS: Students were 14 years-old in 38%, 15 in 38% and 16 or more in 24%. Before PROCES, the mean mark (over 20 points) was 8.5 (2.4). After PROCES, marks improved up to 13.5 (3.2) (p < 0.001). Participants who had previously taken a first-aid course or were in the 4th course obtained significantly better marks than the rest. These differences disappeared after PROCES completion. Students rated the theoretical part as 7.9 (1.1), the skill part as 8.2 (1.2), and the emergency physicians classes as 8.4 (1.1). CONCLUSIONS: PROCES is an useful tool for teaching and improving teenagers' knowledge and skills in b-CPR, with no exceptions associated with teenagers' characteristics.

Programa de reanimación cardiopulmonar orientado a centros de enseñanza secundaria (PROCES): análisis de los resultados del estudio piloto / Basic cardiopulmonary resuscitation program for high school students (PROCES). Results from the pilot program

FUNDAMENTO Y OBJETIVO: Valorar los primeros resultados del Programa de Reanimació cardiopulmonar Orientat a Centres d’Ensenyament Secundari (PROCES), encaminado a universalizar la difusión de las maniobras de reanimación cardiopulmonar básica (RCP-B) en la población general mediante su enseñanza en las escuelas de Enseñanza Secundaria Obligatoria (ESO). SUJETOS Y MÉTODO: El PROCES se desarrolla durante 7 sesiones: 5 (5 h) impartidas por los propios profesores del centro y 2 (4 h, incluyen las prácticas de RCP-B) por personal sanitario. Se recogen los datos epidemiológicos, las respuestas de un cuestionario de conocimientos específicos (realizado antes y después de realizar el PROCES) y una encuesta de opinión de los 250 primeros alumnos que han participado en el PROCES. RESULTADOS: El 38% de los participantes tenía 14 años; el 38%, 15 años, y el 24%, ≥ 17 años. La puntuación media (DE) total obtenida (sobre 20 puntos) antes del PROCES fue de 8,5(2,4). Tras la realización del PROCES, esta puntuación aumentó hasta 13,5 (3,2) (p < 0,001). Los alumnos que habían realizado algún curso de socorrismo con anterioridad y los de 4.º cursode ESO obtuvieron puntuaciones previas al PROCES significativamente más altas que el resto, diferencias que desaparecieron tras la realización del mismo. La valoración (sobre 10 puntos)que los participantes en el PROCES hicieron del desarrollo de los conceptos teóricos fue de 7,9(1,1); de los de RCP básica, 8,2 (1,2), y de las prácticas, 8,4 (1,1). CONCLUSIONES: El PROCES es un instrumento válido para incrementar los conocimientos en RCP-B en la población general, sin que haya ninguna limitación asociada a las características del alumno

BACKGROUND AND OBJECTIVE: The PROCES (Programa de Reanimació Cardiopulmonar Orientat a Centres d’Ensenyament Secundari) program is aimed at teaching basic cardiopulmonary resuscitation (b-CPR) to teenagers within high school. Our aim was to analyze the results obtained from the pilot program. SUBJECTS AND METHOD: PROCES was splitted in 7 sessions: 5 of them (5 hours) were taught by teachers at high school and 2 of them (4 hours, including how to perform b-CPR) were taught by emergency physicians. To assess the degree of students’ learning, they were administered a 20-question test before and after the program. Epidemiological characteristics and students’ opinions (all them were requested to rate the program from 0 to 10) were also collected. RESULTS: Students were 14 years-old in 38%, 15 in 38% and 16 or more in 24%. Before PROCES,the mean mark (over 20 points) was 8.5 (2.4). After PROCES, marks improved up to 13.5 (3.2) (p < 0.001). Participants who had previously taken a first-aid course or were in the 4th course obtained significantly better marks than the rest. These differences disappeared after PROCES completion. Students rated the theoretical part as 7.9 (1.1), the skill part as 8.2(1.2), and the emergency physicians classes as 8.4 (1.1). CONCLUSIONS: PROCES is an useful tool for teaching and improving teenagers’ knowledge and skills in b-CPR, with no exceptions associated with teenagers’ characteristics