[Lower glenohumeral recurrent luxation (luxatio erecta) arthroscopic treatment, literature review and case report]. / Luxación glenohumeral inferior recurrente (luxatio erecta) tratamiento artroscópico, revisión de la literatura y reporte de caso.

It is extremely important to take into account the complications of inferior gelnohumeral dislocation, which, although infrequent, may become incapacitating in the medium and long term, even in some cases reported, endangering the life of the same. Material and methods: We present the case of a young adult patient diagnosed with inferior rectal glenohumeral dislocation (luxatio erecta), with Bankart lesion and Hill Sachs lesion, treated by arthroscopy with repair of the Bankart lesion, plication of the lower capsular recess and remplissage. Results: We evaluated the functionality of the patient at 18 months with the UCLA scale, obtaining a final score of 30 points with good functional results.

Es de suma importancia tener en cuenta las complicaciones de la luxación glenohumeral inferior que, aunque poco frecuentes, pueden llegar a ser incapacitantes a mediano y largo plazo, incluso en algunos casos reportados ponen en riesgo la vida del paciente. Material y métodos: Se presenta el caso clínico de un joven paciente adulto con diagnóstico de luxación glenohumeral inferior (luxatio erecta) recurrente derecha, con lesión de Bankart y lesión de Hill Sachs, tratado mediante artroscopía con reparación de la lesión de Bankart, plicatura del receso capsular inferior y remplissage. Resultados: Evaluamos la funcionalidad del paciente al cabo de 18 meses con la escala de UCLA obteniendo una puntuación final de 30 puntos con buenos resultados funcionales.

Determination of phosphate concentration in glaucoma eye drops commercially available in Spain. / Determinación de la concentración de fosfatos en los colirios antiglaucomatosos comercializados en España.

OBJECTIVES: To identify and analyze the phosphate concentration in glaucoma eye drops available in Spain. MATERIAL AND METHODS: Glaucoma medications containing phosphates were identified according to the 2013 Vademecum and the website of the Spanish Agency for Medicines and Medical Devices. Phosphate concentration was determined in these eye drops using ultraviolet molecular absorption spectrophotometry, and pH was determined using scan image analysis algorithms of pH strips. RESULTS: A total of 37 phosphate containing glaucoma eye drops were identified. The mean phosphate concentration was 97.72±75.52mM. The group with higher concentration of active substance was timolol (204.85±42.38mM) followed by brimonidine/timolol (200.9mM). No statistically significant difference was found between brand name (95.65±71.11mM) and generic eye drops (99.14±80mM, P=.892). Although no statistically significant difference was found between products containing preservatives (99.24±76.78mM) and those without preservatives (85.17±72.86mM) (P=.730), a lower phosphate concentration was observed in the preservative-free Timolol and Latanoprost. Single dose samples showed a lower phosphate concentration than multi-dose ones (102.04±75.39 vs. 22.24±2.98mM, P<.001). The mean pH was 7.13±0.63. No statistical correlation was found between phosphate concentration and pH (r: 0.07). CONCLUSION: The phosphate concentration in glaucoma eye drops exceeded the tear film physiological level (1.45mM). No difference was observed between brand names and generic eye drops. Lower phosphate concentration was observed in preservative-free single dose eye drops.

Determination of phosphate concentration and pH in artificial tear drops. / Determinación de la concentración de fosfatos y pH en los colirios de lágrima artificial.

OBJECTIVE: To determine phosphate concentration and pH in artificial tear eye drops commercially available in Spain. MATERIALS AND METHODS: A total of 71 examples of artificial tear preparations were identified in a search of Vademecum 2014 and the Spanish Medicines Agency website. In the 24 artificial tear products containing phosphates, quantification of these was performed by ultraviolet molecular absorption spectrophotometry, and the determination of pH was performed using scan image analysis algorithms of pH strips. RESULTS: Of the 71 artificial tears tested, 24 contained phosphate among their excipients in the data sheet, three of which had a concentration level below detection limit (<0.1mM). The mean phosphate concentration was 17.91±23.87mM. The artificial tear sample containing a higher concentration was Colircusi Humectante (87.1mM). Lubricants based on hypromellose showed the highest phosphate concentration (41.59±32.1mM), showing statistically significant differences compared to povidone (P=.0196) and hyaluronate (P=.0067). Statistically significant differences were found between products containing preservatives (32.39±20.91mM), and preservative free ones (8.49±11.98mM) (P=.0498). However, no difference was found between multidose (20.21±26.91mM) and unidose (9.31±14.39mM) samples, or between brand name (15.44±23.3mM) and generic eye drops (20.81mM). The mean pH was 6.93±0.26 (6.2-7.22). No statistical correlation was detected between phosphate concentration and pH (Spearman's Rho -0.1089 and P=.6125). CONCLUSION: A total of 24 (33.8%) of the 71 artificial tears contained phosphate. We believe identifying the phosphate concentration of artificial tears is useful information in order to avoid complications in high-risk patients.

Golpe de calor relacionado con el uso de topiramato. La importancia de la prevención / Heat stroke related to the use of topiramate. The importance of prevention

Entre las entidades patológicas relacionadas con el calor, el golpe de calor es el más grave. Se define como una elevación de la temperatura corporal central acompañada de signos de disfunción neurológica, presentando una elevada tasa de mortalidad si no se establece un tratamiento precoz. El topiramato es uno de los fármacos más utilizados en el tratamiento de la epilepsia y en la prevención de las migrañas. Entre sus efectos secundarios, se ha descrito la hipohidrosis, produciendo riesgo de hipertermia y favoreciendo la aparición del golpe de calor. Presentamos el caso de una paciente que presentó un golpe de calor por ejercicio potenciado por el uso de topiramato

Heat stroke is the most severe pathology related to heat. It is defined as an increase in core body temperature accompanied by signs of neurological dysfunction. In the absence of an early treatment, it has a very high mortality rate. Topiramate is a well known drug widely used in epilepsy treatment and migraine prevention. Oligohydrosis has been described amongst topiramate side effects, favouring the risk of hyperthermia and heatstroke. We present the case of a patient who developed heat stroke due to physical exercise while under topiramate treatment

[Heat stroke related to the use of topiramate. The importance of prevention]. / Golpe de calor relacionado con el uso de topiramato. La importancia de la prevención.

Heat stroke is the most severe pathology related to heat. It is defined as an increase in core body temperature accompanied by signs of neurological dysfunction. In the absence of an early treatment, it has a very high mortality rate. Topiramate is a well known drug widely used in epilepsy treatment and migraine prevention. Oligohydrosis has been described amongst topiramate side effects, favouring the risk of hyperthermia and heatstroke. We present the case of a patient who developed heat stroke due to physical exercise while under topiramate treatment.

Análisis del control metabólico de pacientes con diabetes mellitus tipo 1 que acuden a campamentos de verano en Castilla-La Mancha / Analysis of metabolic control of patients with type 1 diabetes attending summer camps in Castilla-La Mancha

Objetivo: Describir el grado de control metabólico en jóvenes con diabetes mellitus tipo 1 (DM1) y analizar los factores que pueden influir en él. Material y métodos: Se realizó un estudio descriptivo y observacional, con una recogida de datos retrospectiva, en el que se incluyó a pacientes con DM1 que acudieron a los campamentos de verano organizados por la Fundación Sociosanitaria de Castilla-La Mancha durante los años 2009 y 2010. Se determinó la hemoglobina glucosilada (HbA1c) en sangre capilar (método DCA 2000+). Se llevó a cabo un análisis estadístico mediante el programa SPSS. Resultados: Se incluyeron en el estudio 85 pacientes, con una media edad de 13,5 años. El 100% de los pacientes recibía una pauta de insulinoterapia intensiva: infusión subcutánea continua de insulina (8,2%), insulina detemir (10,6%), insulina glargina (70,6%) e insulina NPH (neutral protamine Hagedorn) (10,6%). Se realizó una media de 5,4 autoanálisis diarios (rango: 3-12). El valor medio de HbA1c era del 7,6% (rango: 5,7-13,7), presentando el 33% una HbA1c ≤7%, el 32% una HbA1c >7% y ≤8%, y un 35% una HbA1c >8%. No se encontraron diferencias significativas en función de la consulta de procedencia ni de la pauta de insulina empleada, y se observaron valores de HbA1c significativamente menores en los pacientes con menos de 2 años de evolución.Conclusiones: El factor que más influye en la HbA1c de los pacientes analizados es el tiempo de evolución de la enfermedad, sin diferencias significativas en función de la pauta de insulinoterapia (AU)

Objective: To describe the degree of metabolic control in youth with type 1 diabetes mellitus (DM1) and analyze the factors that influence in this control. Material and methods: We performed a descriptive, observational and retrospective study which includes patients with DM1 attending summer camps organized by the Fundación Sociosanitaria de Castilla-La Mancha during the years 2009 and 2010. Glycosylated hemoglobin (HbA1c) is measured in capillary blood (method DCA 2000+). Statistical analysis was performed using SPSS. Results: We collected 85 patients with mean age of 13.5 years. 100% of patients receiving intensive insulin regimen: continuous subcutaneous insulin infusion (8.2%), detemir (10.6%), glargine (70.6%) and NPH (10.6%). The mean HbA1c is 7.6% (5.7 to 13.7%), with 33% HbA1c ≤7%, 32% HbA1c >7% and ≤8%, and 35% HbA1c ≥8%. No significant differences were found depending on the consultation of origin or the pattern of insulin used, with values of HbA1c significantly lower in patients with less than 2 years of evolution. Conclusions: The factor that most influences the HbA1c of the patients analyzed is the time to disease progression, with no differences according to the pattern of insulin (AU)

Resilience of Mediterranean shrubland to a severe drought episode: the role of seed bank and seedling emergence.

Extreme climate events, such as severe drought episodes, may induce changes in vegetation if they induce species-specific adult mortality and changes in the seedling recruitment pattern. In 2005 a severe drought occurred in Doñana National Park (south Spain) causing extensive shrubland mortality. Over the following years we monitored the soil seed bank and seedling emergence via a gradient of canopy dieback induced by the drought episode. The canopy dieback corresponded to an increase in emergence of seedlings of woody species in 2007, probably because of the reduced competition induced by canopy loss. The soil seed bank of woody species sampled in 2008 was less abundant on plots with a higher proportion of dead vegetation, probably because of depletion of the seed bank as a result of the increased germination in the previous year and also as a result of a reduction in seed supply in these sites. Accordingly, in 2009 we detected reduced emergence of woody species on plots that had suffered the greatest shrub mortality. We failed to find any significant changes in patterns of the soil seed bank and seedling emergence of short-lived herbaceous species, indicating greater resilience in these types of species. This study highlights the resilience of Mediterranean shrublands to climate fluctuations at one extreme of the variability characteristic of these ecosystems. An increase in the frequency of severe drought episodes - increasingly probable under the new climate conditions - does have the potential, however, to induce changes in vegetation, especially in woody communities that need more time to replenish their seed banks.

Mating system of the Amazonian cichlid angel fish, Pterophyllum scalare.

The species, Pterophyllum scalare distinguishes itself by its breeding behavior, involving competition for territory, sexual partners, courtship and parental care. The purpose of this study was to identify the mating system adopted by this species of fish. Twenty males and twenty females were observed under semi-natural and experimental conditions to test the hypothesis of serial monogamy. Under semi-natural conditions, after the third breeding cycle, the couples changed mates. Under experimental conditions, the couples changed partners after the first breeding cycle. Under experimental conditions, mate recognition was investigated through the preference of the females, indicated by the time they spent with the males. The females were available or not for courtship from new males, depending on their aggressiveness or submission. The larger and more aggressive males obtained new mating opportunities while the submissive males were rejected by the females. The mated fish were aggressive towards intruders in the presence of the mate, protecting their pair bond. In the interval between breeding cycles, the couples did not display aggression towards intruders, confirming the hypothesis of serial monogamy. Best mate selection by the females and the opportunity of new matings for both sexes influenced the reproductive success of this species.

Flotillin-1 localization on sporozoites oF Eimeria tenella.

In an attempt to identify parasite surface components involved in the interaction with the host cell, the present research focuses on the rafts of Eimeria tenella that might be involved in the host cell invasion process. To that end, this study was undertaken to investigate the expression of flotillin-1, which is an important component and marker of lipid rafts at the plasma membrane of sporozoites of E. tenella. The expression of this plasma membrane protein was identified by an antibody that specifically reacts with flotillin- and was studied by electron microscopy. Flotillin-1 was found to occur in patches on the surface of E. tenella sporozoites. Immunoblot analysis of the total proteins of the sporozoites showed only 1 band of approximately 48 kDa. This indicates that the antibody exclusively recognized the molecules of flotillin-1 expressed on the surface of E. tenella sporozoites. The presence of flotillin-1 on the cellular membrane of sporozoites predominantly at the apical tip suggests that flotillin-1 belongs to the invasion machinery of E. tenella.

Mating system of the amazonian cichlid angel fish, Pterophyllum scalare

The species, Pterophyllum scalare distinguishes itself by its breeding behavior, involving competition for territory, sexual partners, courtship and parental care. The purpose of this study was to identify the mating system adopted by this species of fish. Twenty males and twenty females were observed under semi-natural and experimental conditions to test the hypothesis of serial monogamy. Under semi-natural conditions, after the third breeding cycle, the couples changed mates. Under experimental conditions, the couples changed partners after the first breeding cycle. Under experimental conditions, mate recognition was investigated through the preference of the females, indicated by the time they spent with the males. The females were available or not for courtship from new males, depending on their aggressiveness or submission. The larger and more aggressive males obtained new mating opportunities while the submissive males were rejected by the females. The mated fish were aggressive towards intruders in the presence of the mate, protecting their pair bond. In the interval between breeding cycles, the couples did not display aggression towards intruders, confirming the hypothesis of serial monogamy. Best mate selection by the females and the opportunity of new matings for both sexes influenced the reproductive success of this species.

A espécie Pterophyllum scalare se destaca pela complexidade de seu comportamento reprodutivo, envolvendo competição por território e parceiros sexuais, corte, acasalamento e cuidado parental. Este trabalho teve como objetivo identificar o tipo de sistema de acasalamento adotado pelos peixes desta espécie. Vinte machos e vinte fêmeas foram observados nas situações seminatural e experimental para testar a hipótese de monogamia serial. Em situação seminatural, após o terceiro ciclo reprodutivo, os casais mudaram de parceiros, enquanto que, em situação experimental, os casais mudaram de parceiros a partir do primeiro ciclo reprodutivo. Em situação experimental foi investigado o reconhecimento do parceiro, pela permanência das fêmeas que se mostraram disponíveis ou não à corte de novos machos, em função da agressividade e submissão deles. Machos maiores e mais agressivos obtiveram novas oportunidades de acasalamento e machos submissos foram rejeitados pelas fêmeas. Os peixes acasalados foram agressivos a intrusos na presença do parceiro, protegendo a ligação do par. No intervalo entre os ciclos reprodutivos, os pares não mostraram agressão a intrusos, confirmando a hipótese de monogamia serial. A escolha do melhor parceiro pelas fêmeas e a oportunidade de novos acasalamentos para ambos os sexos influenciaram o sucesso reprodutivo desta espécie.

Sulfonamida: el grupo mágico / Sulfonamide: the magic group

En este trabajo se establece el papel protagonista que el grupo sulfonamida ha tenido en el diseño de nuevos fármacos a lo largo de la historia. La sulfonamida es un grupo funcional que contiene el radical –SO2NH2. Así, la sulfonamida puede ser considerada como un derivado de un ácido sulfónico, donde el grupo hidroxilo se ha sustituido por una amina. La aplicación de las sulfamidas en el tratamiento de enfermedades infecciosas ha sido el primer fruto de la combinación de la observación inteligente con la «serendipity»; sólo así se puede entender el descubrimiento de esta serie de fármacos, que surge a partir de la extracción de la anilina del alquitrán de la hulla, que realizó Runge, y que daría lugar posteriormente a la potente industria de los colorantes azoicos. En la actualidad existen numerosos medicamentos donde está presente este grupo mágico, entre los que se encuentran antibióticos, antimaláricos, antimicrobianos, diuréticos, hipoglucemiantes, antiinflamatorios, antihipertensivos, etc., poniendo de manifiesto el amplio espectro terapéutico de las sulfonamidas, como grupo funcional de interés terapéutico

The significant role of sulfonamide in the development of new drugs is shown in this article, through an overview of its history. Sulfonamide is an organic sulfur compound containing the radical –SO2NH2. Then, any sulfonamide can be considered as derived from a sulfonic acid by replacing a hydroxyl group with an amine group. The treatment of infectious diseases with sulfamides has been the result of a combination of intelligent research and «serendipity»; this is the only way to explain the development of this drugs, starting from the aniline extraction by Runge and its evolution to give the azoic dyes manufacturing. Nowadays, this magic group is present in many drugs as antibiotics, antimalaric drugs, antimicrobial agents, diuretics, hypoglycemics, anti-inflammatory drugs, antihypertensive drugs, etc. showing the broad therapeutic field of sulfonamides as therapeutic function

Determination of the sensory attributes of a Spanish dry-cured sausage.

A lexicon for describing the sensory attributes of a Spanish dry-cured sausage (salchichón) was developed in order to characterise this product. A highly trained, descriptive sensory panel generated, defined, selected and referenced the main sensory characteristic of commercial salchichón elaborated from meat of white pig. This language was not only descriptive but also discriminative. Panellists initially produced a vocabulary of 108 terms that were later modified to 15 attributes: four for appearance (luminance, presence of crust, fat/lean connection and exudate); four for odour (black pepper, lactic acid, mould and other spices); two for texture (hardness and initial juiciness) and five for flavour (black pepper aroma, mould aroma, other spices aroma, acid taste and salty taste). The Kruskal-Wallis test showed that all attributes were significantly different between samples (p<0.001). Three different groups of Salchichones were separated by principal component analyses: group I was characterised by strong other spices smell and aroma and higher juiciness than the other groups. Salchichones from group II had a high mould smell and aroma, a notable fat/lean connection and exhibited a great amount of exudate. Salchichones from group III were characterised by an acid and salty taste.

[Treatment of renal anemia with darbepoetin alfa administered once every other week in predialysis patients with chronic kidney disease and previously treated with epoetin alfa]. / Tratamiento de la anemia renal con administración una vez cada dos semanas de darbepoetina alfa en pacientes con insuficiencia renal crónica prediálisis previamente tratados con epoetina alfa.

Darbepoetin alfa is an erythropoiesis-stimulating glycoprotein with up to 3 times longer half-life than recombinant human erythropoietin (rHuEPO) in humans. The aim of this study was to assess the efficacy and safety of darbepoetin alfa given once every other week as treatment of anemia in predialysis patients with chronic renal failure (CRF) previously treated with once-weekly epoetin alfa. A total of 42 CRF patients were included, all of whom had previously been treated with epoetin alfa and showed stable hemoglobin (Hb) levels without dose changes during the last 8 weeks prior to enrolment in this study. All patients received s.c. darbepoetin alfa once every other week at doses calculated from the protein mass equivalence between rHuEPO and darbepoetin alfa. Follow-up lasted for 24 weeks. Dose adjustments were conducted to preserve target Hb levels between 11 and 13 g/dl. Thirty-nine patients completed the 24 weeks of study. Hb levels increased during follow-up [mean values of 0.39 (p < 0.002), 0.58) (p < 0.001), and 0.83 g/dl (p < 0.001) at 8, 16 and 24 weeks, respectively] despite reducing the darbepoetin alfa dose up to 15% at 24 weeks [from 0.192 microg/kg body weight to 0.185, 0.178 and 0.163 at 8, 16, and 24 weeks, respectively (p < 0.001)]. No adverse events related to darbepoetin alfa were reported. In conclusion, these results show s.c. administration of darbepoetin alfa once every other week was superior to weekly epoetin alfa as a maintenance treatment for anemia in predialysis CRF patients, since the former provided higher Hb levels. Moreover, darbepoetin alfa administration was safe in these patients.

Intercalators as anticancer drugs.

Intercalators are the most important group of compounds that interact reversibly with the DNA double helix. Some of them are valuable drugs currently used for the treatment of ovarian and breast cancers and acute leukemias, while many others are in different phases of clinical trials. Intercalating agents share common structural features such as the presence of planar polyaromatic systems which bind by insertion between DNA base-pairs, with a marked preference for 5'-pyrimidine-purine-3' steps. The chromophores are linked to basic chains that might also play an important role in the affinity and selectivity shown by these compounds. Bisintercalators have two potential intercalating ring systems connected by linkers which can vary in length and rigidity. Nowadays it is well accepted that the antitumor activity of intercalators is closely related to the ability of these compounds to stabilize the DNA-intercalator-topoisomerase II ternary complex. In this work we have carried out a revision of small organic molecules that bind to the DNA molecule via intercalation, and exert their antitumor activity through a proven topoisomerase II inhibition. We have tried to give a general overview of the most recent results in this area, paying special attention to compounds that are currently under clinical trials. Among those are naphthalimides, a group of compounds that has been developed in our laboratory since the 70's.

Nuevos agentes antitumorales: algunos enfoques actuales / New antitumor agents: some novel approaches

En la presente comunicación se describen algunas de las estructuras potencialmente antitumorales que estamos desarrollando en nuestro laboratorio. Entre ellas se han seleccionado nuevas naftalimidas en las que se ha ampliado el cromóforo mediante un ciclo de imidazol o se ha poliramificado la cadena básica para llegar a estructuras de dendrímeros buscando un incremento de las constantes de conjugación con el DNA. También serán citadas nuevas estructuras relacionadas con jaspamida, así como fármacos potenciales inhibidores de la angiogénesis tumoral (AU)

Cytologic features of pulmonary hamartoma. Report of a case diagnosed by fine needle aspiration cytology.

BACKGROUND: Pulmonary hamartoma (PH) is the most common benign tumor of the lung. It is usually composed of cartilage, fat, smooth muscle and respiratory epithelium. Its diagnosis is based on imaging methods (radiography, computed tomography) and cytohistomorphologic study by means of fine needle aspiration cytology (FNAC). CASE: A 59-year-old female had a productive cough and lung mass on chest radiography. Fine needle aspiration of the nodule showed a fusiform tumor cell, which was diagnosed as consistent with PH. The patient underwent surgery for the tumor. Histopathologic study confirmed the diagnosis of PH. CONCLUSION: The fluoroscopically guided FNAC specimen was adequate in achieving a diagnosis. Cytologic features consisted of a serosanguineous background in which scant cellular elements of spindle and stellate cells, as well as fibromyxoid material, enabled us to make a definitive diagnosis. Since this technique is relatively noninvasive, it is very useful in diagnosing PH before a preoperative biopsy.

CDKs y cáncer: nuevas dianas terapéuticas / CDKs y cáncer: nuevas dianas terapéuticas

Las quinasas ciclino-dependientes (CDKs) juegan un papel trascendental en la regulación del ciclo celular y constituyen, por tanto, una de las dianas moleculares de mayor actualidad en el desarrollo de nuevos fármacos con actividad antineoplásica. En el presente trabajo se muestran algunos aspectos del mecanismo de acción de estas enzimas, y se describen los inhibidores químicos de pequeño tamaño molecular que han mostrado, hasta el momento, mayor actividad y selectividad frente a CDKs, y que por lo tanto pueden considerarse como modelos para el diseño de nuevos fármacos (AU)

Comparison between MEM and Nott dynamic retinoscopy.

PURPOSE: The aim of this study was to compare MEM dynamic retinoscopy with the Nott method, to discover whether there were different results in the accommodative response and whether a relation exists between the two techniques. METHODS: We performed MEM and Nott dynamic retinoscopy in 50 visually normal university students. Both methods were performed first on the basis of static retinoscopy and second with the result of the subjective refractive exam (binocular balancing). RESULTS: A statistically significant difference existed between the methods. Nott retinoscopy assessed on basis of the subjective refractive exam was the method that obtained the lowest amounts of accommodative lag (+0.42 D), whereas MEM method performed through the static retinoscopy result showed the highest accommodative lag (+0.94 D). Furthermore, correlation analysis showed that a linear relation existed between both methods, so that the Nott value was about one-half the value of the MEM retinoscopy. CONCLUSIONS: MEM dynamic retinoscopy showed greater lag than Nott retinoscopy.

[Apert syndrome: clinico-epidemiological analysis of a series of consecutive cases in Spain]. / Síndrome de Apert: análisis clínico-epidemiológico de una serie consecutiva de casos en España.

OBJECTIVE: Apert syndrome is one of the five craniosynostosis syndromes caused by allelic mutations of the fibroblast growth-factor receptor 2 (FGFR2). It is characterized by symmetrical cutaneous and bony syndactyly of the hands and feet and a variety of pleiotrophic features of the skeleton, central nervous system, skin and internal organs. PATIENTS AND METHODS: We show the clinical and epidemiological characteristics of the 17 cases of Apert syndrome identified in a consecutive series of 26,956 malformed liveborn infants detected among 1,502,639 livebirths surveyed by the Spanish Collaborative Study of Congenital Malformations (CEMC) between April 1976 and March 1998. RESULTS AND CONCLUSIONS: The estimated frequency of Apert syndrome in Spain is 0.11 per 10,000 liveborn infants. All of the cases were sporadic and were associated with an increased paternal age. The clinical manifestations of our cases are concordant with the variable expression of the syndrome, with the cardinal features of acrocephaly secondary to craniosynostosis and syndactyly of hands and feet present in all cases, and other anomalies, including cardiovascular (23.5%), cleft palate (23.5%), urinary (5.9%) and central nervous system (5.9%), in some of the patients.