Hypertrophic pachymeningitis in a patient with ANCA negative granulomatosis with polyangiitis: A case report / Paquimeningitis hipertrófica en un paciente con granulomatosis con poliangeítis ANCA negativo: reporte de un caso

ABSTRACT Granulomatosis with polyangiitis (GPA) is a vasculitic disease with an infrequent involvement of the central nervous system. This can lead, in rare cases, to hypertrophic pachymeningitis (HP), which is characterized by inflammation and fibrosis that cause a thickening of dura mater. At present, it is crucial to consider GPA in the differential diagnosis of elderly patients with intracranial hypertension. The case is presented of a 60-year-old male with progressive severe headache, vomiting, and wasting syndrome. Physical examination showed pallor, weight loss, and unilateral papilloedema. A gadolinium-enhanced brain MRI scan showed sinusitis, chronic otomastoiditis, and hypertrophic pachymeningitis. Finally, a meningeal biopsy concluded a necrotising granulomatous vasculitis compatible with GPA. However, PR3- and MPO-ANCA were negative. After corticosteroid therapy was initiated, the patient had a favorable outcome during his hospital stay.

RESUMEN La granulomatosis con poliangeítis (GPA) compromete excepcionalmente el sistema nervioso central, conllevando en raras ocasiones a una paquimeningitis hipertrófica (PH), caracterizada por inflamación y fibrosis, que originan un engrosamiento de la duramadre. Actualmente, su consideración es crucial en el diagnóstico diferencial de pacientes ancianos con hipertensión endocraneana. Presentamos el caso de un adulto de 60 anos con cefalea severa progresiva, vómitos, papiledema unilateral y síndrome consuntivo en donde la resonancia magnética cerebral contrastada con gadolinio muestra sinusitis, otomastoiditis y PH. Finalmente, la biopsia de meninges reveló vasculitis granulomatosa necrosante de pequenos y medianos vasos compatible con GPA. Empero, PR3- y MPO-ANCA resultaron negativos. Se inició terapia con corticoides, presentando una evolución clínica favorable durante su hospitalización.

Rupus y hepatitis autoinmune: una asociación infrecuente / Rhupus and autoimmune hepatitis: a rare association

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Tetralogy of Fallot and pheochromocytoma in a situs inversus totalis: An unusual association.

Introduction: Situs inversus totalis is an uncommon anomaly which exist a complete transposition of organs and it's occasionally associated with congenital heart diseases, such as tetralogy of fallot. Pheochromocytoma is a rare neuroendocrine tumor with an annual incidence of 2-8 cases per million people and for years has been studied its relationship with the hypoxic pathway. Case Report: A 29 year old male with a history of tetralogy of fallot corrected at 10 years and situs inversus totalis. He was admitted to hospital with a progressive story of four months of constipation, palpitations, headache, dyspnea and sweating. Physical examination revealed a thinned man with peripheral cyanosis, clubbing and signs of decompensated congestive heart failure as hepatomegaly, legs edema, multifocal systodiastolic murmurs, abdominal distension and jugular venous distention. The echocardiogram shows severe right ventricular dysfunction and severe pulmonary hypertension. Furthermore, abdominal computed tomography shows right adrenal mass. Elevated metanephrines and catecholamines confirmed the diagnosis of pheochromocytoma. Surgical removal is decided and preoperative management begins with alpha-adrenergic blockade, however the patient had a hemodynamic decompensation with an unfavorable evolution. Discussion: In conclusion, there are few reports of cyanotic congenital heart disease with pheochromocytoma. Several studies show a significant association between both of them due to chronic hypoxia leads sustained hyperresponsiveness in adrenal medulla and it would cause the tumor. Special preoperative management of pheochromocytoma is recommended when there underlying heart disease and congestive heart failure. We present the first international report of tetralogy of fallot and pheochromocytoma in a patient with situs inversus totalis.

[Dermatomiositis and evans syndrome associated with HTLV-1 infection]. / Dermatomiositis y síndrome de evans asociado a infección por HTLV-1.

A 55-year-old female patient, born in Ayacucho, with a history of dermatomyositis for 3 years, who received irregular treatment with prednisone. Two months prior to admission, she presented with autoinmune hemolytic anemia and idiopathic thrombocytopenic purpura. The patient received methylprednisolone pulse therapy and packed red blood cells transfusions. Upon admission, she was drowsy, with a poor overall status, marked weight loss, dehydration, with presence of livedo reticularis in her lower extremities, and onychodystrophy and onycholysis on the toes of both feet. Western blot test was positive for human T-lymphotropic virus type 1 (HTLV-1). The patient evolved with recurrent hypoglycemia. Therefore, we report a case of dermatomyositis and Evans syndrome in the context of an HTLV-1 infection.

Dermatomiositis y síndrome de Evans asociado a infección por HTLV-1 / Dermatomiositis and Evans syndrome associated with HTLV-1 infection

Paciente mujer de 55 años de edad, natural de Ayacucho, con antecedente de dermatomiositis desde hace 3 años, recibió tratamiento irregular con prednisona. Dos meses antes del ingreso presenta anemia hemolítica autoinmune y púrpura trombocitopénica idiopática, recibió pulsos de metilprednisolona y transfusión de paquetes globulares. Al ingreso, soporosa, mal estado general, marcada pérdida ponderal, deshidratada, livedo reticularis en miembros inferiores, onicodistrofia y onicolisis múltiple en los dedos de ambos pies. Western Blot positivo para HTLV-1. Evoluciona con hipoglicemia recurrente. Reportamos un caso de dermatomiositis y síndrome de Evans presentados en el contexto de una infección por virus linfotrópico humano tipo 1...

A 55-year-old female patient, born in Ayacucho, with a history of dermatomyositis for 3 years, who received irregular treatment with prednisone. Two months prior to admission, she presented with autoinmune hemolytic anemia and idiopathic thrombocytopenic purpura. The patient received methylprednisolone pulse therapy and packed red blood cells transfusions. Upon admission, she was drowsy, with a poor overall status, marked weight loss, dehydration, with presence of livedo reticularis in her lower extremities, and onychodystrophy and onycholysis on the toes of both feet. Western blot test was positive for human T-lymphotropic virus type 1 (HTLV-1). The patient evolved with recurrent hypoglycemia. Therefore, we report a case of dermatomyositis and Evans syndrome in the context of an HTLV-1 infection...

Pénfigo foliáceo endémico / Endemic pemphigus foliaceus (fogo selvagem)

El pénfigo foliáceo endémico (P F E) o fuego salvaje es una dermatosis ampollar auto inmune presente en áreas de la Amazonia peruana. Se caracteriza por vesículas intraepidérmicas acantolíticas, localizadas en la región subcorneal y en cuya etiología participan anticuerpos contra desmogleína 1, una glicoproteína encargada de la unión intercelular de las células epidérmicas. Se presenta el caso de en una mujer de 52 años con PFE grave, de evolución fulminante y desenlace fatal por sepsis...

The endemic pemphigus foliaceus (EPF) or fogo selvagem is an autoimmune bullous dermatosis located in areas of our Amazon jungle .It is characterized by intraepidermic acantholytic vesicles in the subcorneal region and its etiology involves the presence of antibodies to desmoglein 1, a glycoprotein responsible for the intercellular junctions of the epidermal cells. We present the case of a 52-year-old woman with severe PFE with fulminant evolution and fatal outcome due to sepsis...

Esclerosis tuberosa asociada a síndrome nefrótico y falla renal aguda / Tuberous Sclerosis complex associated to Nephrotic Syndrome and Acute Kidney Injury

El complejo de esclerosis tuberosa es una facomatosis, caracterizada por lesiones cutáneas, epilepsia y retardo mental; de variable afectación a: cerebro, riñones, corazón y otros órganos. De carácter autosómico dominante, existe entre un 60 a 70% de mutaciones de novo. Las lesiones renales se pueden identificar hasta en un 57.5% de pacientes. Se reporta el caso de un paciente varón de 20 años de edad en el Hospital Nacional Arzobispo Loayza. Se presentó con angiofibromas cutáneos, placas de Chagrin, calcificaciones subependimarias, angiomiolipomas renales y leve retardo mental. Con un intenso síndrome nefrótico asociado a insuficiencia renal aguda. Sin antecedentes familiares.Se revisó la literatura y las diversas formas de presentación clínica-patológica de la esclerosis tuberosa.

The tuberous sclerosis complex is a phakomatosis, characterized by cutaneous lesions, epilepsy and mental retardation; with variable affectation in brain, kidneys, heart and other organs. With dominant autosomic character, there are between 60 to 70% de novo mutations. Renal lesions can be identified in up to 57.5% of patients. We report the case of a 20 years old male patient from the Hospital NacionalArzobispoLoayza. He presents cuteanousangiofibromas, Shagreen patches, subependymal calcifications, renal angiomyolipomas and mild mental retardation; along with an intense nephrotic syndrome associated with acute renal injury. No family history of importance. Literature is revised along with the diverse forms of clinical-pathologic presentations of tuberous sclerosis complex.

Hashimoto's encephalopathy presenting with neurocognitive symptoms: a case report.

INTRODUCTION: Hashimoto's encephalopathy is a neurological disorder of unknown cause associated with thyroid autoimmunity. The disease occurs primarily in the fifth decade of life and may present in two types - a sudden vasculitic type or a progressive subacute type associated to cognitive dysfunction, confusion and memory loss. CASE PRESENTATION: We report the case of a 62-year-old Hispanic woman, previously healthy, who developed a subacute onset of declining upper brain function. Serologic studies demonstrated high levels of antithyroid antibodies. Electroencephalographic and magnetic resonance image findings were consistent with Hashimoto's encephalopathy. CONCLUSION: Hashimoto's encephalopathy is a diagnosis of exclusion. This unusual disorder is often under-recognized because of the multiple and protracted neurocognitive manifestations; therefore, it is important to be aware of the clinical manifestations to make a correct diagnosis.

[Endometriosis of the pancreas]. / Endometriosis pancreática.

The case of a 23-year old woman with a history of epigastric pain, a palpable tumor that covered the epigastrium and the left hypochondrium, and an episode of acute pancreatitis was reported. The computerized tomography revealed a pancreatic cyst. The CA-125 increased significantly. An exploratory laparotomy was performed, finding an endometrioma. The pathological anatomy showed necrotic tissue, mucus and blood, with a presence of macrophages with hemosiderin phagocytosis. The symptoms and signs, pathogenesis and treatment of the endometriosis of the pancreas are discussed.

Endometriosis Pancreática / Endometriosis of the Pancreas

Reportamos el caso de una mujer de 23 años de edad con historia de dolor epigástrico, tumoración palpable que abarca el epigastrio y el hipocondrio izquierdo y un episodio de pancreatitis aguda. La tomografía computarizada reveló un quiste pancreático. El CA-125 se elevó notablemente. Se efectuó una laparotomía exploratoria encontrándoseun endometrioma. La anatomía patológica mostró tejido necrótico, moco y sangre, con presencia de macrófagos con fagocitosis de hemosiderina. Se discute el cuadro clínico, la patogénesis y el tratamiento de la endometriosis pancreática.

The case of a 23-year old woman with a history of epigastric pain, a palpable tumor that covered the epigastrium and the left hypochondrium, and an episode of acute pancreatitis was reported. The computerized tomography revealed a pancreatic cyst. The CA-125 increased significantly. An exploratory laparotomy was performed, finding an endometrioma. The pathological anatomy showed necrotic tissue, mucus and blood, with a presence of macrophages with hemosiderin phagocytosis. The symptoms and signs,pathogenesis and treatment of the endometriosis of the pancreas are discussed.

Neumonía adquirida en la comunidad en pacientes adultos hospitalizados en el HNDAC durante el 2003 / Pneumonia acquired in the community in adult patients hospitalized in the HNDAC during 2003

OBJECTIVE: to Know the clinical epidemic profile and microbiológico of the NAC in hospitalized mature patients. MATERIAL AND METHODS: I Study observational analytic retrospective. 123 Clinical Histories of mature patients were evaluated hospitalized by NAC in the National Hospital Daniel A. Carrión, in the 2003. RESULTS: the population's 65.8 per cent was bigger than 50 years, 50.4 per cent was women and 49.6 per cent males. The average of hospital stay was smaller than 10 días. The opposing more frequent symptoms were: disnea (18.2 per cent ), cough (17.2 per cent), and fever (14.4 per cent ). In relation to the x-ray of Thorax, in 103 (83.7 per cent) he/she was condensation. Among the factors of risk for specific patógenos of NAC was: age bigger than 65 years 25 per cent, alcoholism 10.6 per cent, Illness structural lung 10.2 percent, Comorbilidad multiple 7.9 per cent. In relation to the antibiotic treatment: more Ceftriaxona Claritromicina (39.8 per cent ), more ceftriaxona Doxiciclina (17.9 per cent). CONCLUSION: The affected group is the one of bigger than 50 years. The presence of factors of risk for specific patógenos is frequent. A low frequency of cultivations exists for determination etiológica. The evolution of these patients, in most of the cases is favorable, for the speed with which he/she is carried out the diagnosis and the therapy begins, keeping in mind the clinical symptoms and the radiological discoveries.

OBJETIVO: Conocer el perfíl clínico, epidemiológico y microbiológico de la NAC en pacientes adultos hospitalizados. MATERIAL y MÉTODOS: Estudio observacional analítico retrospectivo. Se evaluaron 123 Historias Clínicas de pacientes adultos hospitalizados por NAC en el Hospital Nacional Daniel A. Carrión, en el 2003. RESULTADOS: El 65.8 por ciento de la población fue mayor de 50 a±os, 50.4 por ciento fueron mujeres y 49.6 por ciento varones. El promedio de estancia hospitalaria fue menor de 10 días. Los síntomas mßs frecuentes encontrados fueron: disnea (18.2 por ciento), tos (17.2 por ciento), y fiebre (14.4 por ciento). En relación a la radiografía de Tórax, en 103 (83.7 por ciento) se encontró condensación . Entre los factores de riesgo para patógenos específicos de NAC se encontró: edad mayor de 65 a±os 25 por ciento, alcoholismo 10.6 por ciento, Enfermedad estructural pulmonar 10.2 por ciento, Comorbilidad múltiple 7.9 por ciento. En relación al tratamiento antibiótico: Ceftriaxona mßs Claritromicina (39.8 por ciento), ceftriaxona mßs Doxiciclina(17.9 por ciento). CONCLUSIONES: El grupo mßs afectado es el de mayores de 50 a±os. La presencia de factores de riesgo para patógenos específicos es frecuente. Existe una baja frecuencia de cultivos para determinación etiológica. La evolución de estos pacientes, en la mayoría de los casos es favorable, por la rapidez con que se realiza el diagnóstico y se inicia la terapéutica, teniendo en cuenta los síntomas clínicos y los hallazgos radiológicos.

Acidosis láctica severa y leucemia aguda / Severe lactice acidosis and acute leukemia

Reportamos el caso de una paciente de 27 años de edad con leucemia linfoblástica aguda, quien presentó acidosis láctica severa como complicación metabólica. Ella acudió con desnutrición severa, anemia marcada y síndrome consuntivo. No había compromiso del sistema reticuloendotelial y un mielograma inicial fue normal. Estos factores retardaron el diagnóstico y obligaron a ampliar el diagnóstico diferencial. La sospecha de neoplasia hematológica asociada a acidosis láctica sin causa aparente permitió reevaluar el caso con un nuevo mielograma y establecer el diagnóstico.

We report the case of a 27 year-old patient with acute lymphoblastic leukemia who presented severe lactic acidosis as a metabolic complication. She presented with severe malnutrition, marked anemia and consuming syndrome. There was no reticuloendothelial system compromise and an initial myelogram was normal. These factors delayed the diagnosis and forced the extent of the differential diagnosis. The suspicion of lactic acidosis associated hematologic neoplasia needed reassessing the case with new myelogram to establish the diagnosis.

[Hepatic subcapsular hematoma caused by fascioliasis]. / Hematoma hepático subcapsular por fasciola.

UNLABELLED: The case of a 22 year old woman from Huaraz is presented herein. She suffered from pain at right hypocondrium, associated to nausea and vomits, which intensified three days prior to admission. Upon examining her, a faded gallbladder murmur was found on the base of the right hemithorax. There is pain in the abdomen when touched at the epigastrium and right hypocondrium. The liver is perceived 3 cm beneath the costal edge: White blood count with severe eosinophilia. Mild cholestasis is observed. Abdominal scan: Heterogeneous hepatic mass, with a 13 cm diameter in the right lobe. CAT scan: Subcapsular 14x8 cm mass. Scintiscan: Liver with a low absorption area showing absence of perfusion to the vascular pool. She undergoes an exploratory laparotomy and an 800 cc subcapsular hematoma is found in segment 6, 7 and 8, which is drained. Evolution evidences the persistence of eosinophilia and positive Arc-2 is obtained for Fasciola. She was administered Triclabendazol and is currently asymptomatic. CONCLUSION: The invasive stage of human fascioliasis may cause hepatic hematoma as a rare complication. The triad of persisting eosinophilia, painful hepatomegalia and prolonged fever leads to insist in the search of fascioliasis in endemic areas.

Hematoma hepático subcapsular por fasciola / Hepatic subcapsular hematoma by fascioliasis

Presentamos el caso de una mujer de 22 años de edad, natural de Huaraz con historia de dolor en hipocondrio derecho asociado a náuseas y vómitos que se intensifican tres días antes de su admisión. Al examen se encuentra disminución del murmullo vesicular en la base del hemitórax derecho. El abdomen es doloroso a la palpación en el epigastrio e hipocondrio derecho. El hígado se palpa a 3 cm debajo del reborde costal; Hemograma con eosinofilia severa. Se aprecia leve colestasis. Ecografía abdominal: Masa hepática heterogénea de 13 cm de diámetro en el lóbulo derecho. Tomografía axial computarizada: Masa subcapsular de 14x8 cm. Gammagrafía: Hígado con área hipocaptadora que muestra ausencia de perfusión al pool vascular. Se somete a laparotomía exploratoria y se encuentra hematoma subcapsular de 800 cc en segmento 6, 7 y 8, el cual se drena. En la evolución persiste la eosinofilia y se obtiene Arco 2 positivo para Fasciola. Recibió triclabendazol, actualmente asintomática. Conclusión: La fase invasiva de la fasciolosis humana puede ocasionar hematoma hepático como una complicación rara. La triada de eosinofilia persistente, hepatomegalia dolorosa y fiebre prolongada, orienta a insistir en la búsqueda de fasciolasis en zonas endémicas.

Síndrome de Rapunzel: tricobezoar gastroduodenal / Rapunzel syndrome: gastroduodenal trichobezoar

El Síndrome de Rapunzel es una forma inusual de tricobezoar gástrico con extensión al intestino. Está compuesto de pelo, moco y fragmentos de alimentos en descomposición. Reportamos el caso de una adolescente de 18 años de edad que es admitida en nuestra institución con antecedente de depresión mayor, en tratamiento irregular en el año previo e historia de 5 meses de náuseas, vómitos y pérdida ponderal de 15 kg. Los hallazgos del examen incluyeron distensión y dolor abdominal. Tenía anemia severa y en la serie gastroduodenal mostró defecto de relleno gástrico. El diagnóstico fue confirmado con la gastroscopia. La gastrotomía reveló un gran tricobezoar con extensión duodenal, el que fue extraído. Fue dada de alta sin complicaciones.