Hemofilia adquirida / Acquired hemophilia

Resumen La hemofilia adquirida (HA) es un trastorno hemostático autoinmune ocasionado por autoanticuerpos dirigidos contra el factor VIII: C. En 52 % de los casos, la causa se desconoce o no se asocia con otra entidad patológica; en el resto, existen factores concomitantes: lupus, artritis reumatoide, cáncer, embarazo y medicamentos. En México no existe registro ni conciencia de la enfermedad entre el personal de salud. Los grupos de mayor incidencia son las mujeres en edad reproductiva y los individuos mayores de 70 años. Se caracteriza por hemorragia grave, sobre todo posterior a traumatismos y parto o cesárea, y equimosis grandes en tronco y extremidades. La sospecha es simple, basta que concurran hemorragia súbita, grave y un TTPa prolongado que no se corrige con plasma. El tratamiento consiste en lograr la hemostasia y erradicar el anticuerpo; lo primero se logra con el factor VII activado recombinante o concentrado del complejo de protrombínico activado. La ciclofosfamida, prednisona o rituximab sirven para erradicar el anticuerpo. La mayoría de los casos no son diagnosticados y la mortalidad es alta. Ya que los médicos desconocen el problema, no se sospecha, no se diagnostica y no se trata. Este documento revisa los datos más recientes de la HA y abunda en el diagnóstico y tratamiento.

Abstract Acquired hemophilia (AH) is an autoimmune hemostatic disorder mediated by autoantibodies directed against factor VIII: C. In 52% of cases, the cause is unknown or is not associated with other pathological entities; in the rest, there are concomitant factors: lupus, rheumatoid arthritis, cancer, pregnancy, and medications. In Mexico, there is not a registry of AH, and awareness of the disease among health personnel is low. The groups with the highest incidence are women of childbearing age and individuals older than 70 years. It is characterized by severe bleeding, especially after trauma and normal childbirth or cesarean delivery, and large ecchymoses in the trunk and extremities. The suspicion is simple, it just takes for sudden, severe hemorrhage and a prolonged activated partial thromboplastin time that is not corrected with plasma to concur in an individual. Treatment involves achieving hemostasis and eradicating the antibody. The former is achieved with recombinant activated factor VII or activated prothrombin complex concentrate. Cyclophosphamide, prednisone or rituximab are used to eradicate the antibody. Most cases of AH are not diagnosed, which translates into a high mortality rate. Given that awareness about the disease among physicians is low, it is not suspected, neither diagnosed, and nor is it treated. This document reviews the most recent data on AH and expands on its diagnosis and treatment.

Hepatic Hemangioma with Kasabach-Merritt Syndrome in an Adult Patient / 대한소화기학회지

Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of Kasabach-Merritt syndrome cases occur within the first year of life. However, there are few reports of Kasabach-Merritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of Kasabach-Merritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.

Complicaciones postoperatorias asociadas a la cirugía del tercer molar inferior retenido / Postoperative complications associated with impacted lower third molar surgery

Antecedentes: La cirugía del tercer molar inferior retenido es unprocedimiento de rutina en la práctica de la cirugía bucal. Varias complicaciones surgen como resultado de esta intervención quirúrgica. Entre las más frecuentes se encuentran: dolor, infl amación, trismo, hemorragia, equimosis, alveolitis, infección, parestesia y dificultad para comer. Objetivos: El propósito de este estudio fue evaluar la incidencia de complicaciones postoperatorias en la cirugía del tercer molar inferior retenido en pacientes de la Unidad Académica deOdontología de la Universidad Autónoma de Nayarit a fin de tenerlas en cuenta y tomar las respectivas precauciones, ya sea para tratar de evitarlas en lo sucesivo o bien, para reconocerlas y darles tratamiento. No se incluye dolor, infl amación ni trismo. Material y métodos: Este estudio fue longitudinal y prospectivo de siete días consecutivos y de 30 días en total en 38 pacientes entre 16 y 38 años de edad. Se evaluaron las complicaciones postoperatorias relacionadas con la cirugía del tercer molar inferior. Resultados: Participaron 38 pacientes; 29 del género femenino (76.3 por ciento) y 9 del masculino (23.7 por ciento) de entre 16 y 38 años, con un promedio de edad de 23.16 ± 5.2 años. La complicación más frecuente fue la difi cultad para masticar seguida de equimosis...

Background: Impacted lower third molar surgery is a routine pro-cedure in the practice of oral surgery. However, there are various complications associated with the extraction of impacted mandibular third molars, the most common being pain, swelling, trismus, hemor-rhaging, ecchymosis, dry socket, infection, paresthesia, and diffi culty with chewing. Objectives: The aim of this study was to evaluate the incidence of postoperative complications following impacted lower third molar surgery in patients at the Academic Unit of Dentistry of the Autonomous University of Nayarit, so as to ensure these are taken into account and that the respective precautions are taken, either by attempting to avoid them in the future or to recognize and treat them. Pain, infl ammation and trismus are not analyzed in this paper. Material and methods: A longitudinal prospective study of 38 patients between the ages of 16 and 38 years old (with an average age 23.1) was carried out over seven consecutive days, with follow up examination performed at 15 and 30 days. The postoperative complications associated with lower third molar surgery were assessed. Results: 38 patients took part; 29 females (76.3%) and 9 males (23.7%) between the ages of 16 and 38 years, with a mean age of 23.16 ± 5.2 years old. The most common complication was diffi culty with chewing followed by ecchymosis...

Estimation of salivary amylase and total proteins in leukemia patients and its correlation with clinical feature and radiographic finding.

Background: Leukemia is a fatal disease. The oral manifestations of the leukemias occur early in the course of the disease and these oral features can at times act as a diagnostic indicator. Saliva has been used as a diagnostic aid in a number of systemic diseases. Materials and Methods: In our study, samples of unstimulated saliva of 30 leukemia patients who were not on chemotherapy were collected and analyzed for salivary amylase and total protein. The oral manifestations and radiographic changes (OPG) were recorded. The correlation between the oral manifestations and the salivary components (salivary amylase and total protein) was assessed for prognostic significance. Results: In the present study when the mean values of salivary amylase (1280±754 U/ml) and total protein (647.2±320.7 mg%) were compared with that in control subjects. There was a statistically significant difference for amylase levels (P<.05). On intraoral examination the study subjects showed pallor, gingivitis, gingival enlargement, petechiae, and ecchymosis. On the OPG, the radiographic features included generalized rarefaction of bone (20%), thinning of lamina dura (3.4%), generalized alveolar crest bone resorption (30%), thinning of walls of alveolar crypts (6.7%), besides others, e.g., periapical abscess (10%). Conclusions: The saliva of leukemic patients demonstrated obvious changes in composition. A rise in salivary amylase and total protein levels was evident, with the increase in amylase levels being statistically significant.

El caso de una niña maloliente / A case of a child with bad odor

El olor emitido por un paciente puede ser uno de los signos guía para el diagnóstico en algunas enfermedades. Ciertas condiciones producen olores característicos y muchas enfermedades se asocian a olores específicos. Presentamos un caso ilustrativo, el de una niña de tres años y medio con intolerable olor a materia fecal en todo el cuerpo causado por un cuerpo extraño alojado en la nariz. Revisamos sumariamente la fisiología del olfato, el valor semiológico del olfato en el examen físico, y los diagnósticos diferenciales de las enfermedades que presentan olores inusuales.

Ecchymoses: an unusual manifestation of toxocariasis in children.

OBJECTIVE: To investigate the aetiology of multiple ecchymoses associated with eosinophilia. SETTING: Professorial Paediatric Unit (PPU) at Lady Ridgeway Hospital (LRH) for children, Colombo. DESIGN: Prospective study from July 1998 to April 2000. MATERIALS AND METHODS: Patients admitted to the PPU with multiple ecchymoses associated with an absolute eosinophilia (> 1 x 10(9)/1) were investigated further to determine the possible aetiology; bleeding time, clotting time, platelet count, platelet function tests, stools examination and toxocara antibody tests were performed. Patients who were positive for toxocara were treated either with thiabendazole or albendazole. Patients who had geohelminth infestations were treated with mebendazole. RESULTS: 14 children (11 boys) were studied. 7 were between 1 and 5 years, and 7 were above 5 years of age. The mean eosinophil count was 4.3 x 10(9)/1. All patients had a prolonged bleeding time, but clotting time and platelet counts (mean value 220 x 10(9)/1) were normal. Platelet function tests were done in 5 patients. Twelve patients were positive for toxocariasis and the other 2 were positive for ascariasis. All children who were positive for toxocariasis had contact with pet cats or dogs. Six patients were treated with thiabendazole and 6 with albendazole. The two patients who had ascariasis were treated with mebendazole. Post-treatment mean eosinophil count was 0.63 x 10(9)/1, and the bleeding time was 4 minutes. CONCLUSION: Toxocariasis could present with ecchymoses due to an acquired bleeding disorder. Awareness of this would help to investigate, diagnose and treat early, and lessen parental anxiety.

Complicaçöes da cirurgia microendoscópica naso-sinusal / Complications of microendoscopic naso-sinusal surgery

Os autores apresentam uma atualizaçäo sobre as complicaçöes da cirurgia microendoscópica naso-sinusal

Fracturas de base de cráneo en niños: pronóstico / Skull base fractures in children: pronostic

El presente trabajo retrospectivo tiene como objetivo analizar la morbimortalidad de 500 niños con fracturas de la base de cráneo, hospitalizados en el servicio de Neurocirugía del Hospital del Niño, Lima-Perú. Al momento del alta encontramos que los pacientes tuvieron: A) Buena recuperación, 450 pacientes, 90 por ciento, B) Con secuelas, 27 pacientes, 5,4 por ciento y C) Fallecidos, 23 pacientes, 4,6 por ciento. El cuadro clínico que predominó fué la contusión cerebral. Se concluye que los niños presentan fracturas de la base de cráneo tienen un buen pronóstico y que la mortalidad es del 4.6 por ciento

Púrpura trombocitopénica y hemorrágia de tubo digestivo proximal: informe de 7 pacientes / Trombocytopenic purpura an upper gastrointestinal tract bleeding: report of seven patients

El pacientes con trombocitopenia, independientemente de la etiología de la misma, presenta diátesis hemorrágica a diferentes niveles incluyendo la mucosa de tubo digestivo superior. El presente informe comprende el estudio retrospectivo de siete pacientes con trombocitopenia grave, seis de ellos con púrpura trombocitopénia idiopática crónica y uno con trombocitopenia asociada a hemoglobinuria paroxística nocturna. A todos se les realizó estudio endoscópico que permitió la identificación de lesiones purpúricas sangrantes, en todos los pacientes se localizaron las lesiones en estómago y en tres de ellos también se encontraron en esófago y/o duodeno. La cuenta plaquetaria fue inferior a 39.000 por c.c y se encontró una correlación directa entre la gravedad de la trombocitopenia y la extensión y número de las lesiones en tubo digestivo