Manejo del dolor en la fibrodisplasia osificante progresiva / Treatment of pain crisis in fibrodysplasia ossificans progressiva

La fibrodisplasia osificante progresiva es una enfermedad de escasa prevalencia, 1 caso cada 2 millones de habitantes, hereditaria, severamente incapacitante, caracterizada por un proceso de osificación en músculos esqueléticos, fascias, tendones y ligamentos, sin tratamiento eficaz y que evoluciona por empujes, que se acompañan de dolor intenso, dando lugar a posteriori, después de varios empujes, a la instalación de dolor basal permanente. Analizamos las características más salientes de la enfermedad, su historia habitual, su forma de presentación, su evolución, el dolor acompañante y su tratamiento. Este se realiza para combatir el dolor, ya que no existe tratamiento en el momento actual, para la enfermedad de fondo, y será variable de acuerdo al momento evolutivo de la misma, comenzándose con paracetamol y AINE en etapas iniciales, para dar lugar luego al uso de opioides. En lo que se refiere a las crisis de dolor, secundarias a brotes de osificación heterotópicos, existe consenso actualmente en cuanto a la utilización de corticoides de alta potencia antiinflamatoria. Siendo el dolor un hecho relevante en esta patología, no hemos encontrado ninguna publicación que lo aborde específicamente, por lo cual entendemos oportuna esta comunicación. Presentamos el caso de persona de 27 años, sexo femenino, portadora de fibrodisplasia osificante progresiva, controlada desde hace años en nuestra Unidad por sus crisis dolorosas severas, así como también por dolor basal instalado en los últimos años. Se relata la presentación de la enfermedad, su evolución, su estado actual, los tratamientos que se han realizado, así como el manejo de su última, reciente y más severa crisis de dolor, en base a dexametasona en altas dosis, y al uso de pregabalina como coadyuvante. Se concluye con recomendaciones para tratar los empujes dolorosos, basadas en el uso de corticoides, así como el manejo del dolor basal siempre presente en estos pacientes, fundamentado en el uso de opioides (AU)

The fibrodysplasia ossificans progressiva is a disease of low prevalence, 1 case per 2 million inhabitants, hereditary, severely disabling, characterized by a process of ossification in skeletal muscles, fascia, tendons and ligaments, without effective treatment, evolving by thrusts which are accompanied by severe pain, resulting in a posteriori, after several thrusts, to installing permanent baseline pain. We analyze the most salient features of the disease, common history, its presentation, its evolution, the accompanying pain and its treatment. This is done to combat the pain, since there is no treatment at present, for the underlying disease, and will vary according to the time course of the same, beginning itself with paracetamol and NSAIDs in early stages, giving rise then to the use of opioids. In regard to pain crises, buds secondary heterotopic ossification, currently there is consensus regarding the use of high potency anti- inflammatory corticosteroids. With pain as a relevant in this disease, we found no publication that specifically addresses; therefore we consider this presentation timely. We report the case of a person of 27 years, female gender, fibrodysplasia ossificans progressiva carrier controlled for years in our unit, for its severe painful crises and baseline pain also installed in recent years. We report the presentation of the disease, its evolution, its current status, as well as treatments that have been made, well as the handling of her latest, recent and more severe pain thrust, based on high-dose dexamethasone, and the use of pregabalin as an adjuvant. It concludes with recommendations for treating painful crises, based on the use of corticosteroids, and baseline pain management always present in these patients, based on the use of opioids (AU)

Malignant melanomas of the nasolacrimal duct.

Two patients with a history of epistaxis who were both found to have a nasolacrimal duct melanoma are presented. A literature review revealed that no previous cases of primary nasolacrimal duct melanoma have been reported. Current therapeutic modalities are discussed.

[Results of treatment of retinal angiomatous proliferation with photodynamic therapy]. / Resultados del tratamiento de la proliferación angiomatosa retiniana mediante terapia fotodinámica.

PURPOSE: To determine the effects of phothodynamic therapy using verteporfin in the treatment of patients with retinal angiomatous proliferation (RAP) and the incidence of this retinal disease in our area. METHODS: We performed a retrospective study of 11 cases of RAP who were treated with photodynamic therapy using verteporfin (PDT). RESULTS: The incidence of RAP in the group of eyes with minimally classic or occult subfoveal choroidal neovascularization was 8%. The mean follow-up time after treatment was 15 months. The visual acuity improved in 3, remained the same in 4, and decreased in 4. In 4 patients, angiomatous lesions were observed in both eyes. CONCLUSIONS: There is no method proven to be effective for the treatment of retinal angiomatous proliferation. Our results suggest that PDT may be useful therapy in patients with RAP as it appeared to reduce the risk of visual loss.

[Submacular hemorrhage following photodynamic therapy in the treatment of choroidal neovascularization]. / Hemorragia submacular tras terapia fotodinámica en el tratamiento de la neovascularización coroidea.

PURPOSE: To describe the incidence, clinical features and evolution of submacular hemorrhage (SMH) occurring after photodynamic therapy (PDT) with verteporfin in the treatment of choroidal neovascularization (CNV). METHODS: A retrospective analysis of the patients treated with PDT in our hospital between July 2002 and May 2005 was undertaken. RESULTS: 8 out of 504 eyes treated with PDT (1.58%) developed SMH; 4 of them (0.79%) required surgical attack. The incidence of SMH for every application of PDT was 0.65% (8/1221). The underlying disorder defined was age-related macular degeneration (AMD) in 7 cases (87.5%), and high myopia in one case (12.5%). Regarding the type of lesion, 5 were occult (62.5%; p=0.01), 1 predominantly classic, 1 minimally classic, and the last one was not classified. The average final visual acuity (VA) was 0.057, with 25% of patients having a VA >or= 0.1. Patients lost 4 Snellen lines on average. CONCLUSIONS: SMH after PDT was an event of unknown etiology and low frequency. The incidence in our series (1.58%) was comparable with that described in the world literature (0.24-9.0%). The greatest incidence of AMD was in the occult group with no classic type of CNV, suggesting a possible higher risk for SMH in this type of lesion. It is mandatory to inform patients of the possibility of this complication, which can compromise the visual result of the PDT, and sometimes require surgery. The low risk of SMH related to the PDT justifies its application when it is indicated.

[Sarcoidosis following combined ribavirin and interferon therapy: a case report and review of the literature]. / Sarcoidosis tras tratamiento con interferón y ribavirina. Presentación de un caso y revisión de la bibliografía.

Treatment of active chronic viral hepatitis type C with interferon alpha has proved effective and therefore its use is being extended to a large number of patients. Common side effects include respiratory manifestations. One side effect attributable to the immunomodulatory effect of interferon is the possible triggering or exacerbation of systemic or cutaneous sarcoidosis. We report a new case and offer an exhaustive review of the literature. A 49-year-old man with type C chronic, active hepatitis developed new respiratory symptoms and pulmonary infiltrates with hilar and mediastinal adenopathy after 4 months of treatment with pegylated interferon and ribavirin. The transbronchial biopsy showed multiple sarcoid granulomas. When the patient was diagnosed, he had already taken the total dose of interferon and no specific treatment was started. His hepatitis did not respond to therapy and his viral load and transaminase levels remained high.

[Herpes simplex virus type 1 acute retinal necrosis two years after presumably herpetic meningoencephalitis]. / Necrosis retiniana aguda por virus herpes simplex tipo 1 dos años después de meningoencefalitis presuntamente herpética.

CASE REPORT: We report the case of a 53-year-old woman with uveitis in her right eye. She suffered from meningoencephalitis two years before. In the ophthalmic examination she showed no light perception, mild anterior uveitis and severe vitritis, which prevented from visualizing the retina. We suspected herpetic acute retinal necrosis (ARN), so therapy with intravenous acyclovir was started and a diagnostic vitrectomy was performed. Peripheral retinal necrosis and pallor of the optic disc were observed. PCR of the vitreous was positive for herpes simplex virus type I. DISCUSSION: This is probably a case of brain-to-eye virus transmission. According to this, the ARN would support the etiologic suspicion of the previous encephalitis.

Sarcoidosis tras tratamiento con interferón y ribavirina. Presentación de un caso y revisión de la bibliografía / Sarcoidosis following combined ribavirin and interferon therapy: a case report and review of the literature

El tratamiento con interferón alfa en la hepatitis crónica activa por el virus C tiene una eficacia demostrada, por ello se está generalizando su uso a gran número de pacientes.Entre los efectos secundarios, las manifestaciones respiratorias banales son muy frecuentes. Un efecto secundario atribuible al efecto inmunomodulador del interferón es la posibilidad de agravar o desencadenar una sarcoidosis cutánea o sistémica. Presentamos un nuevo caso y hacemos una exhaustiva revisión de la bibliografía.Se comunica el caso de un varón de 49 años con hepatitis crónica activa por el virus C que a los 4 meses de tratamiento con interferón pegilado más ribavirina presentó sintomatología respiratoria que no tenía previamente e infiltrados pulmonares con adenopatías hiliares y mediastínicas. En la biopsia transbronquial se apreciaron abundantes granulomas sarcoideos. Cuando se diagnosticó, el paciente había finalizado toda la dosis de interferón y no se instauró ningún tratamiento específico. La hepatitis no respondió a la terapia, permaneciendo elevada la carga viral y las transaminasas (AU)

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[Hypopharynx and larynx basaloid squamous carcinoma: our experience with 6 cases]. / Carcinoma basaloide escamoso de laringe e hipofaringe: presentación de una serie de 6 casos.

INTRODUCTION: Basaloid squamous carcinoma (BSC) of the head and neck is an uncommon, recently described variant of epidermoid carcinoma. BSC appears biologically with a greater propensity to local aggressiveness and metastatic potential than the conventional type. OBJECTIVES: To determine the incidence of BSC of the larynx and hypopharynx in our area, and to evaluate its behavior and prognosis. PATIENTS & METHODS: 272 cases of squamous carcinoma of the larynx and hypopharynx were reviewed and 6 diagnosis of BSC were made, corresponding to 6 tumors in 5 patients. Their clinical records were analyzed. An immunohistochemical study was performed, including antibodies against cytokeratins (CK 7, CK 8, CK 19, CK 20, CK 1, 10, 5, 14, y CK 10), epithelial markers (EMA, CEA, Ber-EP4), vimentin and three neuroendocrine markers: neuron-specific enolase, chromogranin and synaptophisin. Bcl-2 expression was also investigated. RESULTS: Most tumors were supraglottic. 5 out of 6 tumors were clinically benign, without any metastases or recurrence after exclusive surgical resection and a follow-up ranging 42 to 142 months. We detected a trend toward a primitive pattern of keratin expression. All tumors were stained with EMA and CEA MoAbs, but we could not find Ber-EP4 nor endocrine markers staining in any of them. However, BCL-2 expression was widely found in 4 out of 5 tumors immunostained. CONCLUSION: It seems to be a progressive dedifferentiation of the basaloid cell component, probably a subpopulation with more primitive features responsible of the aggressiveness reported in the literature. However, the surprising good prognosis in most of our cases makes necessary more studies and wider series to confirm our data.

Carcinoma basaloide escamoso de laringe e hipofaringe: presentación de una serie de 6 casos / Hypopharynx and larynx basaloid squamous carcinoma: our experience with 6 cases

Introducción: El carcinoma basaloide escamoso (CBE) de cabeza y cuello es una variante infrecuente de carcinoma epidermoide que se ha descrito muy recientemente, apuntándose que posee mayor agresividad y capacidad metastásica que el tipo convencional. Objetivos: El propósito de esteestudio ha sido establecer la incidencia del CBE de laringe e hipofaringe en nuestro medio en relación con las otras formas de carcinoma epidermoide, así como analizar su comportamiento biológico para determinar si representa una forma clínica más agresiva como se ha descrito en la literatura. Pacientes y métodos: Se revisaron 272 pacientes afectos de carcinoma epidermoide de laringe e hipofaringe, extrayéndose de la base de datos aquéllos con el diagnóstico histopatológico de variantes de carcinoma epidermoide. Se estudiaron las preparaciones en los casos sugerentes, dada la reciente descripción del CBE que impedía su diagnóstico en los casos más antiguos. Se extrajeron un total de 6 casos de carcinoma basaloide escamoso en 5 pacientes, de los que se revisó la historia clínica. Hemos realizado un estudio inmunohistoquímico de los cinco primeros casos, empleando una batería decitoqueratinas (CK 7, CK 8, CK 19, CK 20, CK 1, 10, 5, 14, y CK 10), marcadores epiteliales (EMA, CEA, Ber-EP4), vimentina y tres marcadores neuroendocrinos (enolasa neuronoespecífica, cromogranina y sinaptofisina). También se incluyó la expresión del gen Bcl-2. Resultados: Los tumores asentaron mayoritariamente en la región supraglótica. El comportamiento clínico en 4 de los 5 pacientes fue muy benigno, con crecimiento excrecente, poco infiltrante y sin metastatizar. Se detecta una tendencia a la expresión de un patrón de citoqueratinas más primitivo. Todos los tumores marcaron de forma variable con EMA y CEA. No se apreció expresión de Ber-EP4, ni tampoco de los marcadores neuroendocrinos. En cambio, el anticuerpo dirigido contra la proteína del bcl-2 marcó muy considerablemente cuatro de los cinco casos en que se envió. Conclusión: Pensamos que estos resultados indican una progresiva desdiferenciación del componente basaloide, el cual representaría una fracción constituida por elementos más primitivos, y conferiría posiblemente la mayor agresividad que aparece recogida en la mayor parte de las series informadas hasta el momento. Sin embargo, el sorprendente buenpronóstico de nuestros casos hace que sean necesarios ulteriores estudios con mayores series para confirmar los datos publicados (AU)

INTRODUCTION: Basaloid squamous carcinoma (BSC) of the head and neck is an uncommon, recently described variant of epidermoid carcinoma. BSC appears biologically with a greater propensity to local aggressiveness and metastatic potential than the conventional type. OBJECTIVES: To determine the incidence of BSC of the larynx and hypopharynx in our area, and to evaluate its behavior and prognosis. PATIENTS & METHODS: 272 cases of squamous carcinoma of the larynx and hypopharynx were reviewed and 6 diagnosis of BSC were made, corresponding to 6 tumors in 5 patients. Their clinical records were analyzed. An immunohistochemical study was performed, including antibodies against cytokeratins (CK 7, CK 8, CK 19, CK 20, CK 1, 10, 5, 14, y CK 10), epithelial markers (EMA, CEA, Ber-EP4), vimentin and three neuroendocrine markers: neuron-specific enolase, chromogranin and synaptophisin. Bcl-2 expression was also investigated. RESULTS: Most tumors were supraglottic. 5 out of 6 tumors were clinically benign, without any metastases or recurrence after exclusive surgical resection and a follow-up ranging 42 to 142 months. We detected a trend toward a primitive pattern of keratin expression. All tumors were stained with EMA and CEA MoAbs, but we could not find Ber-EP4 nor endocrine markers staining in any of them. However, BCL-2 expression was widely found in 4 out of 5 tumors immunostained. CONCLUSION: It seems to be a progressive dedifferentiation of the basaloid cell component, probably a subpopulation with more primitive features responsible of the aggressiveness reported in the literature. However, the surprising good prognosis in most of our cases makes necessary more studies and wider series to confirm our data (AU)

Cambio de tipo de células de Paneth en el adenocacinoma de próstata: a propósito de un caso / Change in type of Paneth´s granular cells in adenocarcinoma of the prostate: A case report

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Primary esophageal lymphoepithelioma.

Lymphoepitheliomas are extremely rare outside the nasopharynx. Extranasopharyngeal lymphoepithellomas may be located in the stomach or digestive tract, and only one single case of esophageal location has been found in our review. In many cases the tumor is related to Epstein-Barr virus infection. We report a case of undifferentiated carcinoma of the distal third of the esophagus in a 79 year-old man. Pathological examination of the esophagus revealed an undifferentiated carcinoma with lymphold stroma (lymphoepithelioma). In situ hybridization of the neoplastic cells was negative for Epstein-Barr virus. To our knowledge, the present case is the first documented esophageal lymphoepithelioma in Western countries.

Sarcoidosis, sclerosing cholangitis, and chronic atrophic autoimmune gastritis: a case of infiltrative sclerosing cholangitis.

We report a patient in whom sarcoidosis coexisted with sclerosing cholangitis and chronic atrophic autoimmune gastritis. There are some autoimmune diseases associated with primary sclerosing cholangitis; the difference between sarcoidosis and all other autoimmune diseases associated with primary sclerosing cholangitis is the ability of the former to damage the biliary tree. Moreover, when sarcoidosis behaves like cholestasis it can damage the biliary tree, mimicking primary sclerosing cholangitis, with high immunoglobulin M but without inflammatory bowel disease and p-ANCAs negative. We believe that it should be regarded as a single disease "infiltrative sclerosing cholangitis" because this is not a primary disease and sarcoidosis would be responsible for a beaded biliary tree.

Phacoemulsification cataract surgery in vitrectomized eyes.

PURPOSE: To determine the problems, safety, and results of phacoemulsification cataract surgery in previously vitrectomized eyes. SETTING: Department of Ophthalmology of Galdácano Hospital, Galdacano, Spain. METHODS: In this prospective study, phacoemulsification was performed in 23 eyes that had had vitrectomy between February 1992 and May 1994. Surgical and postsurgical difficulties and complications and visual acuity results were analyzed. Follow-up ranged from 12 to 24 months. RESULTS: The incidence of surgical problems and complications was higher than usual: 7 eyes presented small pupil size (< 3.0 mm); sudden changes in anterior chamber depth and pupil size during surgery occurred in 6 eyes; unusual mobility and flaccidity of the posterior capsule was observed in 6 cases, which was associated with posterior subcapsular cataract and young age (< 50 years); posterior capsule tear occurred in 2 eyes, 1 of which required anterior vitrectomy. Postoperatively, best visual acuity improved two Snellen lines or more in 17 eyes (73.91%), did not change in 3 (13.04%), and worsened in 3. CONCLUSION: Phacoemulsification in vitrectomized eyes presented more problems and complications than usual. However, it seems to be safer than manual extracapsular surgery because it minimized the risk of intraoperative eye hypotony or collapse.

Nucleolar organizer regions in pigmented skin lesions. Value in the differential diagnosis of Spitz nevi.

Forty-one cases of typical melanocytic skin lesions (15 intradermal nevi, 14 Spitz nevi and 12 malignant melanomas) were used to investigate the value of staining of nucleolar organizer regions (NORs) in the differential diagnosis of such pigmented lesions. Histologic sections were stained by the silver colloid (Ag) method, with and without the prior use of a melanin blocking agent. There were statistically significant differences in the mean numbers of AgNORs per nucleus between the groups of lesions studied (1.658 for intradermal nevi, 3.0042 for Spitz nevi and 6.669 for malignant melanomas). Sections treated with potassium permanganate (melanin blocking agent) prior to staining showed an obvious increase in the AgNOR scores in all groups; this increase was highest for Spitz nevi. Although AgNOR staining allows a distinction to be made between intradermal nevi and malignant melanomas, the striking overlap between the counts for Spitz nevi and malignant melanomas precludes the use of this technique as the sole method for establishing the diagnosis of malignancy. Other clinical and morphologic data are especially required to make the diagnosis of Spitz nevi.

[Malignant primary lymphoma of the bladder]. / Linfoma maligno primitivo de vejiga.

We report a case of primary malignant lymphoma of the bladder in a 20-year-old female patient who consulted for recurrent urinary tract infections. Tumor type was that of a poorly-differentiated lymphocytic lymphoma with a predominantly diffuse growth pattern. The patient was put on a combined treatment modality of external radiotherapy and polychemotherapy which achieved good results. Patient follow up 20 months after treatment revealed no local recurrence or distant spread of the tumor.

Dexamethasone-prepared Escherichia coli-induced disseminated intravascular coagulation. Animal model.

We have developed a new model of disseminated intravascular coagulation in rats based on the induction of immunosuppression by prolonged high-dose dexamethasone treatment. Most models of disseminated intravascular coagulation are based on the generalized Shwartzman reaction, which is observed characteristically in experimental animals after two separate inoculations of bacterial endotoxins. These produce massive deposition of thrombi in the microcirculation and significant hemorrhagic and ischemic phenomena. We have demonstrated that the administration of glucocorticosteroids at the specific doses and intervals can adequately replace the first (preparatory) injection of endotoxin. For this reason, we have attempted to experimentally simulate a frequent clinical situation, such as sepsis secondary to peritonitis, by intraperitoneal inoculation of Escherichia coli and hog gastric mucin into rats pretreated with dexamethasone. This inoculation was equivalent to the second injection of endotoxin in the Shwartzman model (triggering inoculation). A typical picture of disseminated intravascular coagulation induced by bacterial endotoxins developed, as demonstrated by the anatomopathologic, microbiologic, and hematologic studies performed. These results were then compared to those obtained in rats treated exclusively with dexamethasone or given, in addition, an effective antibiotic therapy.

Karyometry and histometry of renal-cell carcinoma.

In an attempt to more objectively predict the outcome of renal cancers, karyometric and histometric studies were performed using an interactive computer-based system for the quantitative analysis of tissue sections. Analysis showed a significant relationship between patient survival and metastases and the histometric parameters of nuclear elongation, nuclear crowding and mitotic density, as well as tumor grade. Patients who died tended to have a high mitotic density, elongated and crowded nuclei and high-grade tumors. Ploidy showed no significant correlation with prognosis while nuclear elongation and crowding did. Differences in histologic grade were significantly associated with several histometric variables, including nuclear area, shape, crowding, elongation and mitotic density.