Absceso periamigdalino bilateral. Reporte de un caso y revisión de las controversias en el diagnóstico y tratamiento / Bilateral peritonsillar abscess. A case report and review of controversies surrounding diagnosis and management

La presentación bilateral del absceso periamigdalino es poco frecuente. Su abordaje es controversial y se discute si realizar amigdalectomía en caliente versus diferida. Se presenta el caso de un paciente de sexo masculino, de 14 años, con odinofagia, trismo y fiebre. Presentaba hipertrofia amigdalina bilateral, pilares abombados y edema de paladar blando. Tomografía computada: hipertrofia amigdalina bilateral, con realce poscontraste, ambas con colección, edema con moderada estenosis faríngea. Se decidió internación para tratamiento endovenoso y amigdalectomía con drenaje bilateral. Resolución completa del cuadro con alta a las 48 horas. Ante la presencia de un absceso periamigdalino, debe considerarse la posibilidad de un absceso contralateral oculto. Debe ser diagnosticado y tratado adecuadamente para prevenir complicaciones. La amigdalectomía en caliente podría ser un tratamiento seguro y debería ser considerado en pacientes que serán sometidos a anestesia para drenaje. La decisión final debe ser determinada para cada caso en particular.

The bilateral presentation of peritonsillar abscess is uncommon. Its management is controversial and it has been argued whether a quinsy tonsillectomy or an interval tonsillectomy should be performed. Here we describe the case of a 14-year-old boy with sore throat, trismus, and fever. He had bilateral tonsillar hypertrophy, convex arches, and soft palate edema. Computed tomography: bilateral tonsillar hypertrophy, with post-contrast enhancement, both with collection, edema with moderate pharyngeal stenosis. The patient was hospitalized for intravenous therapy and tonsillectomy with bilateral drainage resulting in a complete resolution of his condition and discharge at 48 hours. In the presence of a peritonsillar abscess, an unsuspected contralateral abscess should be considered. It should be diagnosed and managed adequately to prevent complications. Quinsy tonsillectomy could be safe and should be considered in patients who will undergo anesthesia for abscess drainage. The final decision should be made for each patient on an individual basis.

Evaluation of Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum

ABSTRACT Purpose: To assess Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum and correlate with ocular surface changes. Methods: This cross-sectional study evaluated patients with xeroderma pigmentosum. All patients underwent a comprehensive and standardized interview. The best-corrected visual acuity of each eye was determined. Detailed ophthalmic examination was conducted, including biomicroscopy examination of the ocular surface, Schirmer test type I, and meibography, and fundus examination was also performed when possible. Meibomian gland dysfunction was assessed by non-contact meibography using Oculus Keratograph® 5M (OCULUS Inc., Arlington, WA, USA). Saliva samples were collected using the Oragene DNA Self-collection kit (DNA Genotek Inc., Ottawa, Canada), and DNA was extracted as recommended by the manufacturer. Factors associated with abnormal meiboscores were assessed using generalized estimating equation models. Results: A total of 42 participants were enrolled, and 27 patients underwent meibography. The meiboscore was abnormal in the upper eyelid in 8 (29.6%) patients and in the lower eyelid in 17 (62.9%). The likelihood of having abnormal meiboscores in the lower eyelid was 16.3 times greater than that in the upper eyelid. In the final multivariate model, age (p=0.001), mutation profile (p=0.006), and presence of ocular surface malignant tumor (OSMT) (p=0.014) remained significant for abnormal meiboscores. For a 1-year increase in age, the likelihood of abnormal meiboscores increased by 12%. Eyes with OSMT were 58.8 times more likely to have abnormal meiboscores than eyes without ocular surface malignant tumor. Conclusion: In the final model, age, xeroderma pigmentosum profile, previous cancer, and clinical alterations on the eyelid correlated with a meiboscore of ≥2. Meibomian gland dysfunction was common in patients with xeroderma pigmentosum, mainly in the lower eyelid. The severity of Meibomian gland dysfunction increases with age and is associated with severe eyelid changes.

Partial regression of peripapillary myelinated nerve fibers after non-arteritic anterior ischemic optic neuropathy / Regressão parcial das fibras nervosas peripapilares mielinizadas após neuropatia óptica isquêmica anterior não arterítica

ABSTRACT A 71-year-old woman presented a non-arteritic anterior ischemic optic neuropathy in an optic nerve with previously registered superonasal peripapillary myelinated nerve fibers. Her past medical history was significant for controlled systemic hypertension, hyperlipidemia, and diabetes mellitus. The physiologic cup was absent in both optic discs. Non-arteritic anterior ischemic optic neuropathy mainly affected the temporal and inferior sectors of the peripapillary retinal nerve fiber layer, as could be demonstrated by retinal nerve fiber layer optical coherence tomography and optic disc optical coherence tomography angiography. Unlike other published reports, just a slight regression of the myelinated nerve fibers was observed after 1 year of follow-up. This occurred because ischemia mainly affected the temporal and inferior peripapillary sectors, whereas myelinated nerve fibers were superonasal to the optic disc.

RESUMO Uma mulher de 71 anos de idade apresentou neuropatia óptica isquêmica anterior não arterítica no nervo óptico com fibras nervosas peripapilares mielinizadas previamente registradas. Seu histórico médico foi significativo para hipertensão arterial sistêmica controlada, hiperlipidemia e diabetes mellitus. Em ambos os discos ópticos, a tacícula fisiológica esteve ausente. A neuropatia óptica isquêmica anterior não arterítica afetou principalmente os setores temporal e inferior da camada de fibras nervosas da retina peripapilar, como demonstrado pela tomografia de coerência óptica da camada de fibras nervosas da retina e pela angiotomografia de coerência óptica do disco óptico. Ao contrário de outros relatórios publicados, apenas uma ligeira regressão das fibras nervosas mielinizadas foi observada após um ano de acompanhamento. Isto pode ser explicado pelo fato da isquemia ter afetado principalmente os setores temporal e inferior peripapilares, enquanto as fibras nervosas de mielina eram nasal superior ao disco óptico.

Artéria ulnar superficial diagnosticada por ultrassom vascular: relato de caso / Superficial ulnar artery diagnosed by vascular ultrasound: case report

Resumo O maior ramo da divisão terminal da artéria braquial é a artéria ulnar, que se origina após a fossa cubital. Essa artéria usualmente tem trajeto profundo aos músculos do antebraço anterior e é responsável pela vascularização da musculatura superficial e profunda da região ulnar do antebraço e hipotênar da mão, sendo a principal responsável pela formação do arco palmar superficial após o retináculo dos flexores. Reportamos uma variação anatômica após diagnóstico com ultrassom vascular na qual a artéria ulnar situava-se em posição superficial no antebraço. A ocorrência da artéria ulnar superficial é rara, porém de grande importância para clínicos, cirurgiões e profissionais de enfermagem.

Abstract The largest branch of the terminal division of the brachial artery is the ulnar artery, which arises after the cubital fossa. This artery usually has a deep path in the muscles of the anterior forearm and is responsible for vascularization of the superficial and deep musculature on the ulnar side of the forearm and hypothenar area of the hand. We report an anatomical variant diagnosed by Doppler ultrasound in which the ulnar artery had a superficial position in the forearm. Occurrence of a superficial ulnar artery is rare, but it is an important fact for clinicians, surgeons, and nursing professionals.

Simultaneous unilateral central retinal vein occlusion and branch retinal artery occlusion after Coronavirus Disease 2019 (COVID-19) mRNA vaccine / Oclusão unilateral simultânea da veia retiniana central e artéria retiniana ramificada após vacina mRNA COVID-19

ABSTRACT A 51-year-old non-obese woman presented with a one-week history of progressive blurry vision within the inferior visual field of her left eye. Her only relevant past medical history was long-standing hypothyroidism and recent vaccination against Coronavirus Disease 2019 (COVID-19) with an mRNA vaccine 12 days before the onset of symptoms. At examination, the anterior segment was unremarkable, but the retinal fundus revealed a central retinal vein occlusion associated with a branch retinal artery occlusion of the superior temporal branch in her left eye. Ancillary tests to rule out thrombophilia, hyperviscosity, hypercoagulability, or inflammation were negative. Ultrasound tests were also negative for a cardiac or carotid origin of the branch retinal artery occlusion. At two-month follow-up, no new retinal vascular occlusive events were observed. Although the best-corrected visual acuity at presentation was 8/10 in the left eye, the final best-corrected visual acuity remained 3/10.

RESUMO Uma mulher de 51 anos, não obesa, apresentou história de uma semana de visão embaçada progressiva no campo visual inferior do olho esquerdo. Seu único histórico médico anterior relevante era hipotireoidismo de longa data e uma recente vacinação contra a Doença de Coronavírus 2019 (COVID-19), com vacina de mRNA, 12 dias antes do início dos sintomas. O exame mostrou segmento anterior normal, mas o fundo da retina revelou uma oclusão da veia central da retina associada a uma oclusão de ramo arterial da retina do ramo temporal superior no olho esquerdo. Testes auxiliares para descartar trombofilia, hiperviscosidade, hipercoagulabilidade ou inflamação apresentaram resultados negativos. Testes de ultrassom também foram negativos quanto a uma origem cardíaca ou da carótida da oclusão do ramo da artéria da retina. Após dois meses de acompanhamento, nenhum novo evento vascular oclusivo retiniano foi observado. Embora, a acuidade visual melhor corrigida na apresentação tenha sido de 8/10 no olho esquerdo, a acuidade visual final melhor corrigida permaneceu em 3/10.

Conjunctival leiomyosarcoma in a patient with xeroderma pigmentosum: 5-year follow-up without recurrence / Leiomiossarcoma da conjuntiva em paciente com xeroderma pigmentoso: acompanhamento de 5 anos sem recidivas

ABSTRACT Conjunctival leiomyosarcoma is a very rare soft tissue malignancy. Herein, we describe a conjunctival leiomyosarcoma case in a patient with another rare disease, xeroderma pigmentosum. The 27-year-old single-eyed xeroderma pigmentosum patient complained of exophytic mass covering the ocular surface in her left eye. A vascular, hemorrhagic mass covering the entire ocular surface of the left eye was identified on the examination. Thus, total mass excision surgery was performed. The pathological diagnosis was compatible with conjunctival leiomyosarcoma. Additional chemotherapy, radiotherapy, or surgery were not accepted by the patient. No recurrence or metastasis was observed during the 5-year follow-up. Both primary conjunctival leiomyosarcoma and xeroderma pigmentosum are very rare diseases. Conjunctival masses in xeroderma pigmentosum patients should be approached carefully, and histopathological examination is warranted. For conjunctival leiomyosarcoma, early diagnosis, localized, unspread disease, and complete resection provide the best prognosis.

RESUMO O leiomiossarcoma da conjuntiva é um tumor ma­ligno de tecidos moles muito raro. Aqui é descrito um caso de leiomiossarcoma da conjuntiva em um paciente com xeroderma pigmentoso, que também é uma doença rara. Um paciente de 27 anos de idade com xeroderma pigmentoso de olho único queixou-se de uma massa exofítica cobrindo a superfície ocular do olho esquerdo. Ao exame, foi observada uma massa vascular hemorrágica cobrindo toda a superfície ocular do olho esquerdo. Foi realizada uma cirurgia de excisão total dessa massa. O diagnóstico patológico foi compatível com leiomiossarcoma da conjuntiva. O paciente recusou qualquer quimioterapia, radioterapia ou cirurgia adicional. Nenhuma recidiva ou metástase foi observada durante o acompanhamento de 5 anos. Tanto o leiomiossarcoma primário da conjuntiva quanto o xeroderma pigmentoso são doenças muito raras. Massas conjuntivais em pacientes com xeroderma pigmentoso devem ser abordadas com cuidado e deve-se realizar um exame histopatológico. Para o leiomiossarcoma conjuntival, o diagnóstico precoce, uma doença localizada e não disseminada e a ressecção completa proporcionam o melhor prognóstico.

Retinal vein and artery occlusion as the first manifestation of primary antiphospholipid syndrome in a pediatric patient / Oclusão arterial e venosa como primeira manifestação da síndrome antifosfolipide primária em criança

ABSTRACT Antiphospholipid syndrome is an acquired autoimmune disease characterized by hypercoagulability associated with recurrent venous and arterial thromboembolism in the presence of antiphospholipid antibodies. Herein, we report a case of rapid sequential retinal vein and artery occlusion as the first manifestation of a primary antiphospholipid syndrome triggered by an acute Mycoplasma infection in a previously healthy 11-year-old patient. On day 1, ophthalmoscopy revealed a central retinal vein occlusion. The patient developed temporal branch retinal artery occlusion the next day. On day 3, a central retinal artery occlusion was observed. Serum lupus anticoagulant, immunoglobulin (Ig) G anticardiolipin, IgG anti-β2-glycoprotein 1 antibody, and Mycoplasma pneumoniae IgM antibody levels were increased. Thus, retinal vascular occlusions can be the first manifestation of primary antiphospholipid syndrome. Although it may not improve visual prognosis, prompt diagnosis and treatment are essential to avoid further significant morbidity.

RESUMO A síndrome antifosfolipide é uma doença autoimune adquirida caracterizada por hipercoagulabilidade associada a tromboembolismo venoso e arterial recorrente na presença de anticorpos antifosfolipídicos. Aqui, relatamos um caso clínico de oclusão sequencial de veia e artéria da retina como primeira manifestação de uma síndrome antifosfolipíde primária desen­cadeada por uma infeção aguda por Mycoplasma num paciente de 11 anos previamente saudável. No primeiro dia, a oftalmoscopia revelou uma oclusão da veia central da retina. No dia seguinte, o paciente desenvolveu uma oclusão do ramo temporal da artéria central da retina. No terceiro dia, uma oclusão da artéria central da retina foi diagnosticada. Os níveis de anticoagulante lúpico sérico, anticorpos IgG anticardiolipina e IgG anti-β2-glicoproteína 1 e anticorpos IgM para Mycoplasma pneumoniae estavam aumentados. As oclusões vasculares retinianas podem ser a primeira manifestação da síndrome antifosfolipíde primária. Apesar do prognóstico visual ser reservado, o seu diagnóstico e o tratamento imediatos são essenciais para evitar outras morbilidades associadas.

Urolitiasis en lactantes: un reto diagnóstico y terapéutico. Reporte de un caso

La litiasis urinaria en niños obedece a cambios nutricionales, ambientales, climáticos, predisposición genética, infecciones del tracto urinario, anomalías metabólicas y/o anatómicas subyacentes, su incidencia es de 1-3%, la cual se ha incrementado en la última década. Se presenta lactante masculino de 22 meses, con presencia de cálculos a nivel uretral con proceso infeccioso asociado, quien requirió procedimiento invasivo para la extracción de un cálculo y documentaron hiperuricosuria, hipomagneseuria e hipercalciuria, sin antecedente familiar de litiasis renal. La urolitiasis en lactantes es relevante dado el papel que puede tener en la etiología de la infección urinaria y en el deterioro progresivo de la función renal, abarca un manejo integral multidisciplinario debido a su carácter recurrente y que aún no existe un tratamiento curativo, es importante establecer los factores de riesgo, definir estrategias para el diagnóstico temprano y acciones para su prevención y procedimientos menos invasivos para la extracción de los cálculos.

Urinary lithiasis in children are due to nutritional, environmental, climatic changes, genetic predisposition, urinary tract infections, underlying metabolic and/or anatomical anomalies. Its incidence is 1-3%, which has increased in the last decade. We present the case of a 22-month-old male infant, who presented with stones at the urethral level with an associated infectious process, and who required an invasive procedure for the extraction of a stone. The patient had documented hyperuricosuria, hypomagneseuria and hypercalciuria, without a family history of kidney stones. Urolithiasis in infants is relevant given the role it can play in the etiology of urinary infection and the progressive deterioration of kidney function. Its treatment encompasses comprehensive multidisciplinary management due to its recurrent nature and the fact that there is still no curative treatment. It is important to identify risk factors, define strategies for early diagnosis, preventive measures and less invasive procedures for stone extraction.

Diagnosis of Celiac Disease after Starting Antitubercular Medication: A Case Report

We present the case of a young patient from Argentina living in a rural area without any relevant medical history. He consulted the emergency department after blunt chest trauma, and during trauma studies, images compatible with pulmonary tuberculosis were found, a diagnosis made incidentally. After starting treatment, he exhibited gastrointestinal symptoms such as diarrhea, abdominal pain, and weight loss, which were initially considered an adverse effect of treatment with rifampin/isoniazid/pyrazinamide/ethambutol (RHZE). Upon completing the first phase of treatment and suspending the medication, the symptoms improved, and the bacilloscopies were negative. Subsequently, the severity of the symptoms drew attention. Additional paraclinical tests were performed with malabsorptive diarrhea results, considering the patient's origin and the fact that his diet included products that he grew himself without prior exposure to gluten. Celiac disease is suspected, and antibodies and biopsy results compatible with this entity were obtained. When reviewing the association of symptom onset with the RHZE/pyridoxine treatment, we found these medications may have wheat-based excipients, which explains the worsening of symptoms, not due to the gastrointestinal adverse effects of the antibiotic but its excipients. Finally, the case was analyzed, tuberculosis was ruled out, and treatment was suspended, refocusing the therapeutic effort on recovering the patient's nutritional status. Subsequently, no other hospital admissions were recorded, and he remained respiratorily asymptomatic, with weight gain and nutritional recovery.

Se presenta el caso de un paciente joven, proveniente de Argentina y residente de un área rural, sin ningún antecedente médico a resaltar. Consultó al servicio de urgencias después de un trauma cerrado de tórax y durante los estudios de trauma se encontraron imágenes compatibles con tuberculosis pulmonar, diagnóstico realizado de manera incidental. Luego del inicio de tratamiento presentó síntomas gastrointestinales dados por diarrea, dolor abdominal y pérdida de peso, lo que inicialmente se consideró un efecto adverso al tratamiento con rifampicina/isoniazida/pirazinamida/etambutol (RHZE). Al completar la primera fase del tratamiento y suspender la medicación mejoraron los síntomas, además de que las baciloscopias fueron negativas. Posteriormente, llamó la atención la gravedad de los síntomas, por lo que se realizaron paraclínicos adicionales con resultados a favor de diarrea malabsortiva; teniendo en cuenta la procedencia del paciente y el hecho de que basaba su dieta en productos que él mismo cultivaba, sin haber exposición previa al gluten. Se sospecha enfermedad celíaca, y se obtuvieron anticuerpos y resultados en la biopsia compatibles con esta entidad. Al revisar la asociación de inicio de síntomas con el uso de tratamiento de RHZE/piridoxina, se observa que estos medicamentos pueden tener excipientes a base de trigo, lo que explica el empeoramiento de los síntomas, no por efectos adversos gastrointestinales del antibiótico sino por sus excipientes. Finalmente, se analizó el caso, se descartó tuberculosis y se suspendió el tratamiento, redirigiendo el esfuerzo terapéutico hacia la recuperación del estado nutricional del paciente. Posteriormente, no se registran otros ingresos hospitalarios, continúa asintomático respiratorio, con ganancia de peso y recuperación nutricional.

Use of intravenous lidocaine in a patient with refractory chest pain secondary to Takayasu arteritis. Case report / Uso da lidocaína endovenosa em paciente com dor torácica refratária secundária à arterite de Takayasu. Relato de caso

ABSTRACT BACKGROUND AND OBJECTIVES: Takayasu's arteritis (TA) is a rare form of chronic inflammatory disease involving large vessels, with uncertain etiology, with chest pain as a common and challenging symptom, resulting from inflammation in the aortic root or arch, pulmonary artery or coronary arteries. The objective of this study was to describe the use of intravenous lidocaine to treat severe and refractory chest pain secondary to TA. CASE REPORT: A 33-year-old female patient diagnosed with TA, with severe chest pain that was difficult to manage, was admitted after consulting an emergency department. The pain was unresponsive to traditional treatment after a week of drug adjustments. As a therapeutic option, a Sympathetic Venous Blockade (SVB) with lidocaine was chosen, achieving a reduction in pain from 10 to 3 on the Visual Analog Scale. Infliximab was administered before discharge. The patient was re-evaluated at an outpatient appointment after 30 days. CONCLUSION: This strategy for the treatment of severe chest pain allowed for pain reduction and relief.

RESUMO JUSTIFICATIVA E OBJETIVOS: A arterite de Takayasu (AT) é uma forma rara de doença inflamatória crônica envolvendo grandes vasos, com etiologia incerta, tendo a dor torácica como um sintoma comum e desafiador, consequente à inflamação na raiz ou arco aórtico, artéria pulmonar ou coronárias. O objetivo deste estudo foi relatar a utilização da lidocaína por via endovenosa na abordagem da dor torácica intensa e refratária secundária à AT. RELATO DO CASO: Paciente do sexo feminino, 33 anos, com diagnóstico de AT, dor torácica intensa de difícil manejo, internada após consulta em serviço de emergência. Dor não responsiva ao tratamento tradicional após uma semana de ajustes em fármacos. Como opção terapêutica, foi escolhido o Bloqueio Simpático Venoso (BSV) com lidocaína, obtendo redução da dor de 10 para 3 na Escala Analógica Visual. Antes da alta hospitalar foi administrado infliximabe. Paciente foi reavaliada em consulta ambulatorial após 30 dias. CONCLUSÃO: Esta estratégia fora tratamento da dor torácica intensa permitiu redução e alívio da dor.

Apicectomía de Primer Molar Inferior. Revisión de Literatura y Reporte de Caso

La apicectomía consiste en la amputación de la fracción apical de la raíz de un diente lesionado y se considera la última opción terapéutica para mantener el diente afectado en boca. Con el objetivo de dilucidar el potencial terapéutico de la técnica quirúrgica, en el presente estudio presentamos el reporte de caso de un paciente con cuadro de hiperestesia asociada al nervio alveolar inferior debido a sobreobturación de la raíz mesial de un primer molar inferior izquierdo. Para resolver el caso realizamos apicectomía mediante abordaje convencional acompañado de medicación empírica enfocada a resolver cuadros de parestesia asociada al procedimiento. Durante los controles posteriores al procedimiento quirúrgico se pesquisa ausencia de parestesia y sintomatología dolorosa, por lo que consideramos la apicectomía como una buena opción de tratamiento en casos de sobreobturación apical que no pueden solucionarse mediante tratamiento no quirúrgico.

Apicoectomy consists of the amputation of the apical fraction of the root of an injured tooth and is considered the last therapeutic option to keep the affected tooth in the mouth. With the aim of elucidating the therapeutic potential of the surgical technique, in this study we present the case report of a patient with hyperesthesia associated with the inferior alveolar nerve due to overfilling of the mesial root of a left lower first molar. To solve the case we performed apicoectomy by conventional approach accompanied by empirical medication focused on resolving paresthesia associated to the procedure. During the controls after the surgical procedure we found absence of paresthesia and painful symptomatology, so we consider apicoectomy as a good treatment option in cases of apical overfilling that cannot be solved by non-surgical treatment.

Pancreatitis aguda en el escenario de hipercalcemia por hiperparatiroidismo primario / Acute pancreatitis in the setting of hypercalcemia due to primary hyperparathyroidism

Introducción: describir el caso de un paciente con pancreatitis aguda secundaria a hipercalcemia por hiperparatiroidismo prImario. Esta es una causa poco frecuente de pancreatitis, asociada a morbimortalidad significativa en caso de no ser diagnosticada oportunamente Caso clínico: un hombre de 44 años, con antecedente de pancreatitis de presunto origen biliar que había requerido previamente colecistectomía, consultó por dolor abdominal y náuseas. Los estudios complementarios fueron compatibles con un nuevo episodio de pancreatitis aguda. Presentaba hipercalcemia y hormona paratiroidea (PTH) elevada, configurando hiperparatiroidismo primario. La gammagrafía informó hallazgos compatibles con adenoma paratiroideo. Se inició tratamiento con reanimación hídrica y analgesia con adecuada disminución de calcio sérico y resolución de dolor abdominal. Después de la paratiroidectomía se logró normalizar los niveles de calcio y PTH. Discusión: la pancreatitis aguda es una condición potencialmente fatal, por lo que la sospecha de causas poco frecuentes como la hipercalcemia debe tenerse en cuenta. El tratamiento de la hipercalcemia por adenoma paratiroideo se basa en reanimación hídrica adecuada y manejo quirúrgico del adenoma, con el fin de evitar recurrencia de pancreatitis y mortalidad. (AU)

Introduction: we describe the case of a patient with acute pancreatitis secondary to hypercalcemia due to primary hyperparathyroidism. This is a rare cause of pancreatitis associated with significant morbidity and mortality if not diagnosed in time. Clinical case: a 44-year-old man with a history of pancreatitis of presumed biliary origin, which had previously required cholecystectomy, consulted for abdominal pain and nausea. The laboratory findings were compatible with a new episode of acute pancreatitis. He presented hypercalcemia and an elevated parathyroid hormone (PTH), configuring primary hyperparathyroidism. Scintigraphy was performed, yielding findings compatible with parathyroid adenoma. Treatment with fluid resuscitation and analgesia was started, resulting in an adequate decrease in serum calcium and resolution of abdominal pain. After parathyroidectomy, calcium and PTH levels were normalized. Discussion: acute pancreatitis is a potentially fatal condition; therefore the suspicion of rare causes, such as hypercalcemia, should be considered. The treatment of hypercalcemia due to parathyroid adenoma is based on adequate fluid resuscitation and surgical management of the adenoma, to avoid recurrence of pancreatitis and death. (AU)

Proteinuria en la infancia: a propósito de un caso de enfermedad de Dent

Introducción: la proteinuria en la edad pediátrica es una entidad relativamente frecuente, la cual puede ser fisiológica o patológica. La segunda, por una alteración a nivel glomerular con pérdida de proteínas de gran tamaño o a nivel tubular, caracterizada por pérdida de proteínas de bajo peso molecular y alteraciones en la excreción de iones. Entre las enfermedades hereditarias que cursan con proteinuria tubular, se ha descrito la enfermedad de Dent, una patología ligada al cromosoma X. Esta enfermedad se manifiesta principalmente en varones, pero las mujeres pueden ser portadoras y tener manifestaciones clínicas leves de la enfermedad. La primera descripción de esta enfermedad fue hecha por Dent y Friedman en 1964. La mayoría de los casos recientemente reportados han sido en China y Alemania. Objetivo: realizar una revisión general de la enfermedad de Dent y del enfoque diagnóstico de la proteinuria en la infancia con base en nuestro caso, para así, sospechar de esta enfermedad. Descripción del caso: se presenta el caso de un paciente masculino sin antecedentes prenatales ni personales de importancia, quien presenta proteinuria persistente desde los primeros meses de vida y a quien, a los 7 años de edad, se le documenta la presencia de una variante ya conocida en el gen CLCN5, causante de la enfermedad de Dent tipo 1. Discusión: la proteinuria persistente patológica en la infancia debe ser estudiada debido a su posible relación con patologías que pueden afectar la función renal. Además de la diferenciación de la proteinuria persistente, de origen glomerular y tubular, la evaluación de alteraciones en la excreción de electrolitos, puede guiarnos hacia la realización de estudios genéticos y, por ende, al diagnóstico de patologías infrecuentes como la enfermedad de Dent. Conclusión: el enfoque diagnóstico de causas poco frecuentes de proteinuria tubular en la infancia, como la enfermedad de Dent, requiere de la valoración conjunta entre nefrología pediátrica y genética clínica.

Background: In pediatric patients, proteinuria is a relatively frequent entity that can be physiological or pathological. The second one, due to an alteration at the glomerular level with the loss of large proteins or at the tubular level, characterized mainly by the loss of low molecular weight proteins and changes in the excretion of ions. Among the hereditary diseases that present with tubular proteinuria, Dent disease is a disease linked to the X chromosome. Therefore, it manifests essentially in males, but women can be carriers and have minor clinical manifestations of the disease. Dent and Friedman made the first description of this disease in 1964. Recently, most of the cases have been reported in China and Germany. Objective: To perform a revision of Dent disease, as well as the diagnostic approach of childhood proteinuria based in our case in order to suspect this disease. Case description: This is the case of a masculine patient, without relevant prenatal and personal antecedents, the son of a father with polycystic renal disease, who presents persistent proteinuria from the first months of life, and who, at seven years old, the presence of a variant in the CLCN5 gene -causing of type 1 Dent disease- was documented. Discussion: The persistent pathological proteinuria in childhood must be studied due to its possible relation with pathologies that could affect renal function. Moreover, the differentiation among glomerular and tubular proteinuria can guide us to perform additional studies, including genetic tests to diagnose infrequent pathologies like Dent disease. Conclusion: The diagnostic approach to rare causes of tubular proteinuria in childhood, such as Dent's disease, requires joint assessment between pediatric nephrology and clinical genetics.

Absceso esplénico en un paciente adulto. Reporte de caso

El absceso esplénico es una entidad poco común. Se define como la presencia de una lesión ocupante de espacio en el bazo asociada a infección. Ha sido descrito en 0,14-0,7 % de los estudios de autopsias. Se presenta el caso de un paciente de 60 años, de color de piel blanca, que acudió al hospital por presentar fiebre y diarreas, con dolor en fosa lumbar izquierda. Fue ingresado y trasladado a Unidad de Cuidados Intensivos debido a fallo renal agudo por deshidratación. Al trasladarse a sala abierta se diagnosticó un absceso esplénico mediante ultrasonido y tomografía. No se decidió operar en ese momento porque no tenía criterio. Posteriormente sufrió de abdomen agudo y shock séptico por ruptura del absceso hacia cavidad abdominal y fue intervenido de urgencia; se realizó esplenectomía y toilette de la cavidad. Su evolución posterior fue favorable. Por lo poco común de esta entidad, se decidió la publicación del caso.

The splenic abscess is a rare entity. It is defined as the presence of a space-occupying lesion in the spleen associated with infection. It has been described in 0.14-0.7% of autopsy studies. The case of a 60-years-old patient, white-skinned, who came to the hospital due to fever and diarrhea, with pain in the left lumbar fossa is presented. He was admitted and transferred to the Intensive Care Unit due to acute renal failure due to dehydration. When transferred to the open room, a splenic abscess was diagnosed by ultrasound and tomography. It was not decided to operate at that time because there are no criteria. Subsequently, he presented acute abdomen and septic shock due to rupture of the abscess into the abdominal cavity and underwent emergency surgery, performing splenectomy and the cavity's toilette. His subsequent evolution was positive. Due to the unusual nature of this entity, it was decided to publish the case.

Angioedema por enalapril. Presentación de un caso

Los inhibidores de la enzima convertidora de angiotensina son utilizados por más de 40 millones de personas en todo el mundo para el tratamiento de enfermedades cardiovasculares. Son considerados seguros, aunque pueden producir angioedema severo en el 0,1 a 0, 5 % de los pacientes. Se presenta el caso de un paciente del sexo masculino, de 67 años de edad, con diagnóstico de diabetes mellitus e hipertensión arterial, tratado con metformina, hidroclorotiazida y enalapril desde hacía aproximadamente cuatro años, que ingresó en cuerpo de guardia con edema severo del tercio anterior de la lengua, sin compromiso respiratorio. Se indicó hidrocortisona y difenhidramina y evolucionó satisfactoriamente, por lo que fue dado de alta y se prescribió prednisona y difenhidramina por vía oral; se suspendió el enlapril y a las 48 horas se reevaluó y estaba asintomático. El mecanismo por el que estos medicamentos producen angioedema no está claro, pero probablemente sería por la acumulación tisular de bradiquinina y puede presentarse en cualquier momento del tratamiento. La correcta anamnesis, el diagnóstico precoz y el tratamiento inmediato con hidrocortisona por vía endovenosa son aspectos a considerar ante casos similares. El análisis del evento mediante la farmacovigilancia, permitió clasificarlo como severo, probablemente relacionado con el consumo de enalapril. Esto genera alertas para informar al personal de salud y tomar decisiones relacionadas con los medicamentos, que permitan la actuación inmediata con la finalidad de reducir la morbimortalidad.

Angiotensin converting enzyme inhibitors are used by more than 40 million people worldwide for the treatment of cardiovascular diseases. They are considered safe, although they can cause severe angioedema in 0.1 to 0.5% of patients. The case of a 67-years-old male patient diagnosed with diabetes mellitus and arterial hypertension, treated with metformin, hydrochlorothiazide and enalapril for approximately four years, who was admitted to the emergency room with severe edema of the third anterior of the tongue, without respiratory compromise is presented. Hydrocortisone and diphenhydramine were indicated and he evolved satisfactorily, for which he was discharged and prednisone and diphenhydramine were prescribed orally; he discontinued enlapril, 48 hours later he was reassessed and was asymptomatic. The mechanism by which these drugs produce angioedema is not clear, but it would probably be due to the tissue accumulation of bradykinin and can occur at any time during treatment. The correct history, early diagnosis and immediate treatment with intravenous hydrocortisone are aspects to consider in similar cases. Analysis of the event through pharmacovigilance allowed it to be classified as severe, probably related to the enalapril consumption. This generates alerts to inform health staff and make decisions related to medications, which allow immediate action in order to reduce morbidity and mortality.

Immunoglobulin-resistant Kawasaki disease / Enfermedad de Kawasaki refractaria a tratamiento con inmunoglobulina

Abstract Background: Kawasaki disease is a systemic vasculitis that affects small and medium-sized vessels, primarily the coronary arteries. First-line treatment includes intravenous immunoglobulin (IVIG) and acetylsalicylic acid; however, 20% do not respond adequately despite treatment. We describe a case treated with etanercept after initial IVIG failure, showing a good response. Case report: A 5-year-old female was diagnosed with classic Kawasaki disease. Echocardiography and angiotomography revealed giant and fusiform aneurysms in the coronary arteries. A first dose of IVIG therapy was administered without improvement; after the second dose, the fever persisted, so etanercept was administered, and the fever subsided. There were no new lesions in medium-caliber vessels and the previously identified coronary lesions did not progress. Conclusions: The use of etanercept in Kawasaki disease has demonstrated a clinically favorable response. Controlled clinical trials of this drug are needed to establish it as a formal therapy in cases of initial IVIG failure.

Resumen Introducción: La enfermedad de Kawasaki es una vasculitis sistémica que afecta los vasos de pequeño y mediano calibre con predominio de las arterias coronarias. El tratamiento de primera línea incluye inmunoglobulina intravenosa (IGIV) y ácido acetilsalicílico; a pesar del tratamiento, el 20% de los pacientes no responden adecuadamente. Se presenta un caso tratado con etanercept debido a la falla inicial a IGIV, con buena respuesta. Caso clínico: Se trata de una paciente de 5 años de edad, a quien se diagnosticó con enfermedad de Kawasaki clásica. En ecocardiografía y angiotomografía se evidenciaron aneurismas gigantes y fusiformes en las coronarias. Se administró una primera dosis con IGIV, sin mejoría; después de la segunda dosis, la paciente persistió con fiebre, por lo que se administró etanercept, tras lo cual esta cesó. No aparecieron nuevas lesiones en vasos de mediano calibre y las lesiones coronarias previas no progresaron. Conclusiones: Con el uso de etanercept se presentó una respuesta favorable clínicamente en la enfermedad de Kawasaki. Se requieren ensayos clínicos controlados con este fármaco para establecerlo como terapia formal en los casos de falla inicial a IGIV.

Pentalogía de Cantrell diagnosticada en el primer trimestre: reporte de caso / Cantrell's pentalogy diagnosed in the first trimester: case report

La pentalogía de Cantrell es una rara anomalía congénita caracterizada por la asociación de ectopia cordis con defectos en la pared toracoabdominal, el diafragma, el esternón y pericárdicos, y anomalías cardíacas intrínsecas. En diagnóstico prenatal, la ecografía se utiliza sistemáticamente entre las 11 y 14 semanas de gestación, evaluando marcadores de alteraciones cromosómicas como la sonolucencia nucal, el hueso nasal y la morfología patológica del ductus venoso, entre otros. Además, permite examinar la anatomía fetal y diagnosticar anomalías mayores, como acrania-anencefalia, holoprosencefalia, defectos de la pared abdominal y toracoabdominal, entre los que se incluye la pentalogía de Cantrell. Se reporta un feto con los hallazgos clásicos de pentalogía de Cantrell, que fue expulsado a las 13 semanas de gestación bajo protocolo de interrupción voluntaria del embarazo. Madre de 23 años, G1P0, sin exposiciones teratogénicas, en cuyo feto se encontró ectopia cordis, asas intestinales e hígado por fuera de la cavidad abdominal en las 10 y 12 semanas de gestación. El objetivo de este estudio es aportar a la literatura un reporte de pentalogía de Cantrell, siendo el primero reportado en Colombia en el primer trimestre de gestación, mostrando la importancia de la ecografía sistemática durante este periodo, en el marco de la posibilidad de interrupción voluntaria del embarazo.

Cantrells pentalogy is a rare congenital anomaly characterized by the association of ectopia cordis with intrinsic cardiac anomalies and various anatomical defects found in the thoracoabdominal wall, diaphragm, sternum and pericardium. Ultrasound is used routinely between 11 and 14 weeks of gestation during prenatal diagnosis. It evaluates markers of chromosomal alterations such as nuchal sonolucency, the nasal bone, and the pathological morphology of the ductus venosus, among others. Furthermore, it allows the diagnosis of altered fetal anatomy and major abnormalities such as acrania-anencephaly, holoprosencephaly, abdominal and thoraco-abdominal wall defects including Cantrells pentalogy. In this case report, we present a fetus with the classic findings of Cantrells pentalogy, which was expelled during the 13th week of gestation under the protocol of voluntary interruption of pregnancy. The mother, a 23-year-old woman, G1P0, without teratogenic exposures, in whom during the routine ultrasound of the 10th and 12th weeks of gestation ectopia cordis, intestinal loops and liver outside the abdominal cavity were found on the fetus. The main objective of this study is to contribute to the literature a case report of pentalogy of Cantrell, diagnosed through prenatal ultrasound, being the first reported in Colombia during first trimester of gestation, showing the importance of routine ultrasound, in the context of access to a voluntary termination of pregnancy.

Neumotórax espontaneo y enfisema bulloso en un paciente joven? A propósito de un caso

Introducción: El neumotórax es una patología prevalente en accidentes de tipo traumático en tórax, pero que también se puede encontrar de forma espontánea por causas atribuibles enfermedad bullosa, neumonía, obstrucción de las vía aérea, malignidad, entre otras. Caso clínico: Paciente masculino joven quien debuta con neumotórax espontaneo requirió manejo en unidad de cuidados intensivos con posterior aparición de bullas en imágenes diagnósticas y patología. Discusión: El neumotórax espontaneo primario (PSP) en población joven, se ve influenciada por factores psicosociales como la edad, genero, hábitos, entorno y factores genéticos, en el 80% de los pacientes con PSP se ha demostrado presencia de bullas o blebs apicales, además de la porosidad de la pleura que suele ocurrir en adolescentes altos con cuerpos atléticos; pero también se observa que el consumo de tabaco que puede aumentar el riesgo. Conclusiones: El neumotórax espontaneo por enfisema bulloso ha incrementado en los jóvenes por el uso de sustancias psicoactivas, vapeadores, cigarrillos.

Introduction: Pneumothorax is a pathology prevalent in traumatic accidents in the thorax, but it can also be found spontaneously due to causes attributable to bullous disease, pneumonia, airway obstruction, malignancy, among others. Case report: Young male patient who debuted with spontaneous pneumothorax who required management in the intensive care unit with subsequent appearance of bullae in diagnostic images and pathology. Discussion: Primary spontaneous pneumothorax (PSP) in the young population is influenced by psychosocial factors such as age, gender, habits, environment, and genetic factors. In 80% of patients with PSP, the presence of bullae or apical blebs has been demonstrated, in addition from the porosity of the pleura that usually occurs in tall adolescents with athletic bodies; but it is also observed that tobacco use can increase the risk. Conclusions: Spontaneous pneumothorax due to bullous emphysema has increased in young people due to the use of psychoactive substances, vapers, and cigarettes.

Plasmocitoma extramedular mamario. Reporte de caso y revisión de la literatura / Extramedullary plasmacytoma of the breast: case report and literature review / Plasmocitoma extramedular mamário: relato de caso e revisão da literatura

El plasmocitoma extramedular es una neoplasia infrecuente y el compromiso mamario es en extremo raro, con alrededor de 83 casos reportados desde 1928. Se presenta el caso de una paciente de 63 años con antecedente de mieloma múltiple, con cuadro clínico de un mes de evolución de masa blanda de 4.5 cm en la mama derecha, indolora, con progresión rápida. Se realizó ecografía mamaria con reporte Bi-RADS 4, por sospecha de malignidad se realizó biopsia y fue remitida al servicio de mastología. El estudio básico de patología e inmunohistoquímica concluye que la lesión corresponde a un plasmocitoma extramedular y luego de múltiples líneas de tratamiento oncológico el plasmocitoma mamario presenta una excelente respuesta clínica e imagenológica. Se hace la descripción del caso y la revisión de literatura.

Extramedullary plasmacytoma is a rare neoplasm, and involvement of the breast is extremely uncommon, with approximately 83 cases reported since 1928. We present the case of a 63-year-old patient with a history of multiple myeloma, who presented with a one-month history of a rapidly progressing, painless, soft mass measuring 4.5 cm in the right breast. Breast ultrasound showed a Bi-RADS 4 lesion, and due to suspicion of malignancy, a biopsy was performed and the patient was referred to the mastology department. Basic pathological and immunohistochemical studies concluded that the injury corresponded to an extramedullary plasmacytoma. After multiple lines of oncological treatment, the breast plasmacytoma showed an excellent clinical and imaging response. The case is described, and a literature review is presented.

O plasmocitoma extramedular é neoplasia pouco frequente e o acometimento mamário extremamente raro, com cerca de 83 casos relatados desde 1928. Apresentamos o caso de uma doente de 63 anos com antecedentes de mieloma múltiplo, com quadro clínico com um mês de evolução de uma massa mole de 4,5 cm na mama direita, indolor, de rápida evolução. Foi realizada ultrassonografia mamária com laudo Bi-RADS 4, realiza-da biópsia por suspeita de malignidade e encaminhada ao serviço de mastologia. O estudo básico de patologia e imuno-histoquímica conclui que a lesão corresponde a um plasmocitoma extramedular e após múltiplas linhas de tratamento oncológico, o plasmocitoma mamário apresenta uma excelente resposta clínica e imagiológica. É feita a descrição do caso e revisão da literatura.

Dexamethasone intravitreal implant as an adjuvant treatment for pediatric patients with Coats' disease / Implante intravítreo de dexametasona como tratamento adjuvante em pacientes pediátricos com doença de Coats

ABSTRACT We report two cases of stage 3A unilateral Coats' disease in pediatric patients. In both cases, disease control was achieved using a dexamethasone intravitreal implant in addition to other treatments. The treatment improved visual acuity in one patient and prevented the worsening of the decline in visual acuity in the other patient during follow-up periods of 7 and 3 years, respectively. One of the patients presented an increase in intraocular pressure, which was controlled with topical antiglaucoma medication, but developed a cataract that required surgery. In conclusion, dexamethasone intravitreal implant may be a useful adjuvant treatment to consider in some pediatric cases with Coats' disease.

RESUMO Relatamos dois casos de doença de Coats em estágio 3A unilateral em pacientes pediátricos. Em ambos os casos, o controle da doença foi obtido com implante intravítreo de dexametasona, além de outros tratamentos, com melhora da acuidade visual em um caso e sem piora da visão no outro, durante um período de acompanhamento de 7 e 3 anos. Um dos casos apresentou elevação da pressão intraocular controlada com medicação antiglaucoma tópica e desenvolveu catarata que exigiu cirurgia. Em conclusão, o implante intravítreo de dexametasona pode ser um tratamento adjuvante útil a ser considerado em alguns casos pediátricos com doença de Coats.