[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.

[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.

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.

[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.