[Ontogenic peculiarities of the human tympanic ossicular chain]. / Peculiaridades en la organización de la cadena osicular timpánica humana a lo largo de su ontogenia.

We have studied the development of the tympanic ossicles in 40 embryo-foetal human series aged between 32 days (6 mm) and newborn. Once performed the measurements to date chronologically embryos and foetuses, we did a meticulous dissection of temporal bones. After fix in 10% formol, decalcified with 2% nitric acid, embedded in Paraplast, sectioned in a sequence of 7 mm, and stained with Martin's trichrome. The tympanic ossicles are developed in the mesenchyme of the two first pharyngeal archs. The head of the malleus, the body and the short limb of the incus arise from the first arch while the handle of the malleus, the long limb of the incus and the mass of the stapes arise from the second arch. The vestibular side of the stapedial footplate develops in the otic capsule. The tympanic ossicles develop from endochondral ossification, while anterior process of the malleus has the membranous ossification. In their ontogenia 6 stages are observed. First stage, the formation of their sketch by mesenchimal condensation, in the second stage, "pre-cartilaginous", the cells of the primordia are differentiated into condroblasts, in the third stage "cartilaginous" the ossicles show a cartilaginous structure, in the forth stage the primary ossification centers are developed, in the fifth stage the ossicles arise in the periostic annulus and inside the endochondral bone, and in the last stage the osseous tissue grows until it acquires a compact osseous structure.

[Severe fulminant form of neonatal citrullinemia. Report of a case]. / Forma grave fulminante de citrulinemia neonatal. Comunicación de un caso.

INTRODUCTION: Citrullinemia is an autosomal recessive disease, which is caused by a deficiency of the argininosuccinate synthetase. The neonatal forms are serious and many times are associated with a high level of mortality. CASE REPORT: A newborn that came in again on her third day of life due to a apneic episodes which required mechanical ventilation. Previously, she rejected feeding, had poor suction, lethargy and remarkable hypoactivity. During the following hours, she showed serious neurologycal deterioration with multifocal convulsions and coma, passing away 20 hours after admission due to endocraneal hypertension. The metabolic evaluation confirmed very significant hyperammonemia, with important increase of citrullin and glutamin, and arginine in the low limits of normality. She was treated with sodium benzoate and arginine and she also needed exanguinotransfusion. It was not possible to put her on hemodyalisis. The findings of the autopsy confirmed massive cerebral edema and characteristic hystological changes in the liver. The determination of the enzymatical activity in liver tissue showed a partial deficiency, with a residual activity of 25% of the average control. CONCLUSIONS: This is a case of fulminant neonatal citrullinemia that we considered of interest in order to draw the attention of the clinical on this type of diseases. The prognosis depends on early diagnosis, witch is based on clinical suspicion and analytical determination of ammonia in every newborn with unexplained vomiting, lethargy or other symptoms of encephalopathy.

Forma grave fulminante de citrulinemia neonatal. Comunicación de un caso / Severe fulminant form of neonatal citrullinemia. Report of a case

Introducción. La citrulinemia es una afección autosómica recesiva, debida a una deficiencia de la argininsuccinato sintetasa (ASS); las formas neonatales son graves y se asocian a un alto índice de mortalidad. Caso clínico. Recién nacida que reingresa al tercer día de vida por episodios apneicos, que requirieron intubación y ventilación mecánica. Previamente, presentó rechazo de las tomas, mala succión, letargia e hipoactividad marcada. En las horas siguientes presentó grave deterioro neurológico, con convulsiones multifocales y coma; falleció a las 20 horas de su ingreso por un cuadro de hipertensión endocraneal. La evaluación metabólica confirmó una hiperamonemia muy significativa, con importante aumento de citrulina y glutamina, adsí como arginina en los límites bajos de la normalidad. Se trató con benzoato sódico y arginina, y se le realizó una exanguinotransfusión de doble volumen; no fue posible practicarle hemodiálisis. Los hallazgos de la autopsia confirmaron un edema cerebral masivo y cambios histológicos característicos en el hígado. La medida de la actividad enzimática en el tejido hepático reveló una deficiencia parcial, con una actividad residual del 25 por ciento de la media control. Conclusión. Se trata de una observación de citrulinemia neonatal de curso fulminante que consideramos de interés, con la finalidad de alertar al clínico sobre este tipo de patología, ya que el pronóstico se va a relacionar con el diagnóstico precoz, basado en la sospecha clínica y determinación del amonio en todo recién nacido con vómitos inexplicables, letargia u otros signos de encefalopatía (AU)

Introduction. Citrullinemia is an autosomal recessive disease, which is caused by a deficiency of the argininosuccinate synthetase. The neonatal forms are serious and many times are associated with a high level of mortality. Case report. A newborn that came in again on her third day of life due to a apneic episodes which required mechanical ventilation. Previously, she rejected feeding, had poor suction, lethargy and remarkable hypoactivity. During the following hours, she showed serious neurologycal deterioration with multifocal convulsions and coma, passing away 20 hours after admission due to endocraneal hypertension. The metabolic evaluation confirmed very significant hyperammonemia, with important increase of citrullin and glutamin, and arginine in the low limits of normality. She was treated with sodium benzoate and arginine and she also needed exanguinotransfusion. It was not possible to put her on hemodyalisis. The findings of the autopsy confirmed massive cerebral edema and characteristic hystological changes in the liver. The determination of the enzymatical activity in liver tissue showed a partial deficiency, with a residual activity of 25% of the average control. Conclusions. This is a case of fulminant neonatal citrullinemia that we considered of interest in order to draw the attention of the clinical on this type of diseases. The prognosis depends on early diagnosis, witch is based on clinical suspicion and analytical determination of ammonia in every newborn with unexplained vomiting, lethargy or other symptoms of encephalopathy (AU)

Peculiaridades en la organización de la cadena osicular timpánica humana a lo largo de su ontogenia / Ontogenic peculiarities of the human tympanic ossicular chain

Hemos estudiado el desarrollo de los osículos timpánicos en 40 series embrio-fetales humanas de edades comprendidas entre los 32 días (6 mm) y el nacimiento. Una vez realizadas las mediciones para datar cronológicamente los embriones y fetos, se llevó a cabo una minuciosa y cuidada disección de los temporales en bloque; posteriormente fueron fijados en formol al 10 por ciento, descalcificados con ácido nítrico al 2 por ciento, deshidratados en alcoholes a concentraciones progresivamente crecientes, incluidos en parafina, cortados en serie a 7 mm, y teñidos en serie con la técnica del Tricrómico de Martins. Los osículos timpánicos se desarrollan en el mesénquima de los dos primeros arcos faríngeos. La cabeza del martillo, el cuerpo y la apófisis corta del yunque se originan del primer arco mientras que el mango del martillo, la apófisis larga del yunque y la masa del estribo lo hacen del segundo. La lámina vestibular de la platina se desarrolla en la cápsula ótica. Los osículos timpánicos se desarrollan por osificación endocondral con excepción de la apófisis anterior del martillo que lo hace por osificación membranosa. En su ontogenia se observan seis estadios. En el primero se produce la formación de sus esbozos por condensación del mesénquima, en el segundo "precartilaginoso" las células de los primordios se diferencian a condroblastos, en el tercero "cartilaginoso" los osículos presentan una estructura cartilaginosa, en el cuarto se forman los centros de osificación primarios, en el quinto aparecen en los osículos el collar perióstico y en su interior hueso endocondral, y en el último estadio el tejido óseo se va extendiendo progresivamente hasta adquirir una estructura ósea compacta (AU)

We have studied the development of the tympanic ossicles in 40 embryo-foetal human series aged between 32 days (6 mm) and newborn. Once performed the measurements to date chronologically embryos and foetuses, we did a meticulous dissection of temporal bones. After fix in 10% formol, decalcified with 2% nitric acid, embedded in Paraplast, sectioned in a sequence of 7 mm, and stained with Martin's trichrome. The tympanic ossicles are developed in the mesenchyme of the two first pharyngeal archs. The head of the malleus, the body and the short limb of the incus arise from the first arch while the handle of the malleus, the long limb of the incus and the mass of the stapes arise from the second arch. The vestibular side of the stapedial footplate develops in the otic capsule. The tympanic ossicles develop from endochondral ossification, while anterior process of the malleus has the membranous ossification. In their ontogenia 6 stages are observed. First stage, the formation of their sketch by mesenchimal condensation, in the second stage, "pre-cartilaginous", the cells of the primordia are differentiated into condroblasts, in the third stage "cartilaginous" the ossicles show a cartilaginous structure, in the forth stage the primary ossification centers are developed, in the fifth stage the ossicles arise in the periostic annulus and inside the endochondral bone, and in the last stage the osseous tissue grows until it acquires a compact osseous structure (AU)

[Contribution to the development of the union between the manubrium of the malleus and the tympanic membrane in human fetus]. / Contribución al desarrollo de la unión del mango del martillo y la membrana del tímpano en fetos humanos.

The development of the union between the manubrium of the malleus and the tympanic membrane was studied in human embryo. For that purpose 25 temporal bones of human foetus, aged from 36 days (14 mm) to 29 weeks (270 mm) were analyzed. Samples were fixed in a 10% formaldehyde solution, decalcified with 2% nitric acid, embedded in Paraplast, cut in sections of 7 microns thick and stained with Martin's trichrome method. During the development a pseudojoint between the malleus and the tympanic membrane several stages were seen. In the first stage, the manubrium was adhered to membrane mesenchyme of primitive tympanum, in the second one this mesenchyme was loose and there appeared capillars in it, in the third one there were collagen fibers in a radial disposition, and in the forth stage, the hollow in the meatal plug gave independence to the tympanic membrane for the external acoustic meatus. The distal portion of the manubrium, included in tympanic membrane, has remained with a cartilaginous structure during end of the time it has been studied by us. The collagen fibers at the level of the umbo surround the manubrium, while the rest of it remains in its anterior edge.

Contribución al desarrollo de la unión del mango del martillo y la membrana del tímpano en fetos humanos / Contribution to the development of the union between the manubrium of the malleus and the tympanic membrane in human fetus

Se ha estudiado el desarrollo de la unión entre el mango del martillo y la membrana del tímpano en embriones y fetos humanos.Para ello se han utilizado 25 huesos temporales de fetos humanos, cuyas edades cronológicas van desde los 36 días (14 mm) hasta las 29 semanas (270 mm). Las muestras fueron fijadas en formol al 10 por ciento, descalcificadas con ácido nítrico al 2 por ciento, incluidas en parafina, cortadas en serie a 7 µm, y teñidas con el método Tricrómico de Martins. Se han identificado varias etapas en el desarrollo de la pseudoarticulación entre el martillo y la membrana del tímpano. En la primera el mango se adhiere íntimamente al mesénquima de la membrana del tímpano primitiva, en la segunda dicho mesénquima se hace laxo y en él aparecen capilares, en la tercera aparecen fibras de colágeno de disposición radial, y en la cuarta el ahuecamiento del tapón meatal da independencia a la membrana del tímpano del conducto auditivo externo. La porción distal del mango, incluida en la membrana del tímpano, ha permanecido con una estructura cartilaginosa durante todo el tiempo estudiado. Las fibras de colágeno a nivel del umbo rodean al extremo terminal del mango mientras que en el resto de él lo hacen solamente en su arista anterior (AU)

The development of the union between the manubrium of the malleus and the tympanic membrane was studied in human embryo. For that purpose 25 temporal bones of human foetus, aged from 36 days (14 mm) to 29 weeks (270 mm) were analyzed. Samples were fixed in a 10% formaldehyde solution, decalcified with 2% nitric acid, embedded in Paraplast, cut in sections of 7 microns thick and stained with Martin's trichrome method. During the development a pseudojoint between the malleus and the tympanic membrane several stages were seen. In the first stage, the manubrium was adhered to membrane mesenchyme of primitive tympanum, in the second one this mesenchyme was loose and there appeared capillars in it, in the third one there were collagen fibers in a radial disposition, and in the forth stage, the hollow in the meatal plug gave independence to the tympanic membrane for the external acoustic meatus. The distal portion of the manubrium, included in tympanic membrane, has remained with a cartilaginous structure during end of the time it has been studied by us. The collagen fibers at the level of the umbo surround the manubrium, while the rest of it remains in its anterior edge (AU)

[Parotid metastases of a neuroendocrine cutaneous carcinoma]. / Metástasis parotídea de un carcinoma neuroendocrino cutáneo.

A case of metastatic Merkel-cell carcinoma to lymph nodes on the left side of the neck nad left parotid is described. Neuroendocrine cutaneous carcinoma, also called Merkel cell carcinoma (MCC), is an uncommon primary skin tumor most often seen in elderly. Literature is reviewed and comment about more important features of these lesions, like differential diagnosis, prognosis and treatment.

Metástasis parotídea de un carcinoma neuroendocrino cutáneo / Parotideal metastasis of a cutaneous neuroendocrine carcinoma

Presentamos un caso de carcinoma neuroendocrino cutáneo con metástasis cervicales y en glándula parótida izquierda. El carcinoma neuroendocrino cutáneo, también llamado carcinoma de células de Merkel (MCC), es un raro tumor de la piel que aparece en edades avanzadas de la vida. Una vez revisada la literatura, discutimos sobre las características más importantes de estas lesiones, como sonel diagnóstico diferencial, pronóstico y tratamiento (AU)

A case of metastatic Merkel-cell carcinoma to lymph nodes on the left side of the neck nad left parotid is described. Neuroendocrine cutaneous carcinoma, also called Merkel cell carcinoma (MCC), is an uncommon primary skin tumor most often seen in elderly. Literature is reviewed and comment about more important features of these lesions, like differential diagnosis, prognosis and treatment (AU)

[Duodenal Crohn's disease: diagnostic and therapeutic complexity]. / Enfermedad de Crohn duodenal. Complejidad diagnóstica y terapéutica.

Crohn's disease may involve any area of the digestive tract, but its localization in the duodenum is rare. The clinical, radiological or endoscopic findings may simulate those of peptic ulcer and histologic examination is not diagnostic. In contrast with Crohn's disease of any other localization, the formation of fistulas is exceptional. The authors present one case of duodenal Crohn's disease the unspecific symptomatology of which deviated the original orientation towards the more common diseases. Steroid treatment was not sufficient to control the disease which led to complications with the formation of an enterocutaneous fistula. The association of azathioprine was very effective not only in achieving remission of the process, but also in resolving the complications.

[Hypernephroma metastasis in frontal sinus]. / Metástasis de hipernefroma en seno frontal.

PURPOSE: We present a case of metastatic neoplasia in the right frontal sinus derived from and hypernephroma, with a clinical picture that initially was interpreted as a mucocele. PATIENTS AND METHODS: Sixty years old male. Personal history: prostatectomy and left nephrectomy because of hypernephroma three years before. In december 92 the patient reported a protrusion in the right frontal sinus. CT scans and NMR revealed a breakage of the external and internal walls of the right frontal sinus and the right orbital roof. In february 93 a surgical exploration of the frontal sinus revealed the metastatic nature of the lesion. DISCUSSION: Metastatic tumors in paranasal sinuses are infrequent since most of the tumors in this localization are primary. Metastatic affectation of the frontal sinus is extremely rare. We recommend the systematic exploration of paranasal sinuses in patients affected by hypernephroma.

[Pleomorphic adenoma of the nasal wall. Revision]. / Adenoma pleomorfo de tabique nasal. Revisión.

The pleomorphic adenoma is the most common neoplasm of the salivary glands, especially the parotid. It can also be originated from minor salivary glands and may appear in nasal cavities and paranasal cavities. We showed a case of pleomorphic adenoma of nasal septum and, at the same time, we reviewed all related bibliography.

[Juvenile nasopharyngeal angiofibroma. Apropos of a case]. / Angiofibroma nasofaríngeo juvenil. A propósito de un caso.

We present the case of a 14 years old male with juvenile nasopharyngeal angiofibroma, treated in our Department with radiotherapy due to the patient having declined surgery. In the control carried out 2 years after finishing radiotherapy the tumour had disappeared. Up to now, we have not found important secondary effects of the treatment. We go over what has been published during the last few years about this disease.

[Mixed tumor of the external auditory canal]. / Tumor mixto de conducto auditivo externo.

Ceruminoma is a collective term applied to tumors arising from the modified apocrine (ceruminous) glands of the external auditory canal. Four distinctive histopathologic patterns are recognized: adenoma, pleomorphic adenoma, adenoid cystic carcinoma, and adenocarcinoma. We report a case of pleomorphic adenoma. It manifested as a soft mass covered with epithelium, filling the right external auditory canal. Wide local excision was the treatment, but local recurrence appeared four years later.

[Cancer of the nasopharynx: our experience with 34 cases]. / Cáncer de nasofaringe: nuestra experiencia con 34 casos.

Thirty four cases of patients with nasopharyngeal cancer, all white people, are reviewed. The more frequent clinical signs are, in order of frequency: nasal obstruction, which is presented in 65% of patients, transmission deafness, in 59%; adenomegalies in 44%; epistaxis in 26% and migraine in 21% of cases. The mean time evolution between the first clinical symptom and the treatment was four and a half months. The pathologic study was: epidermoid carcinoma in 31 cases (91.9% and non-Hodgkin lymphoma in 3 cases 8.9%). The 31 cases of epidermoid carcinoma were treated with radiotherapy and the 5 years survival was 38%. In 62% of cases, the tumor recurred locally, in lymph nodes or were distant metastasis.

[Massive lower digestive hemorrhage caused by typhoid fever]. / Hemorragia digestiva baja de carácter masivo por fiebre tifoidea.

A case is presented of massive lower intestinal bleeding due to typhoid fever. Because of uncontrollable bleeding, emergency right hemicolectomy and resection of the last 50 cm of the distal ileum was performed after endoscopy and arteriography, both of which were negative. The histology of the lesions typical of this disease is described.

[Malignant oncocytoma of the parotid gland. Study with the optical and the electron microscope]. / Oncocitoma maligno de glándula parótida. Estudio con microscopio óptico y electrónico.

Salivary gland oncocytomas are very rare tumors with very few malignant cases reported in the literature. We present in this work a malignant oncocytoma of the parotid gland, and make a summary of all the accepted criteria in the reviewed publications as definitive of oncocytoma and malignant oncocytoma of the salivary gland, and furthermore we make a differential diagnosis with other neoplasms.