Congenital Knee Dislocation at Birth - An Extraordinary Case of Spontaneous Reduction / Luxação congênita do joelho no nascimento - Um caso extraordinário de redução espontânea

Abstract Congenital knee dislocation (CKD) is a rare malformation characterized by hyperextension deformity of the knee with anterior tibia displacement, present at birth. Rarely reported, CKD might occur as an isolated deformity or commonly associated with musculoskeletal abnormalities, with the most common ones being developmental dysplasia of the hip (DDH) and clubfoot. The etiology is unknown, but CKD has been associated with certain intrinsic and extrinsic factors. Treatment with conservative methods at an early stage is most likely to yield successful results. We report here a rare case of successful spontaneous reduction of CKD in an infant within 24 hours of life.

Resumo A luxação congênita do joelho (LCJ) é uma malformação rara caracterizada por deformidade de hiperextensão do joelho com deslocamento anterior da tíbia, presente ao nascimento. Raramente relatada, a LCJ pode ocorrer como uma deformidade isolada ou comumente associada a anormalidades musculoesqueléticas, sendo as mais comuns a displasia do desenvolvimento do quadril (DDQ) e o pé torto congênito (PTC). A etiologia é desconhecida, mas a LCJ foi associada a certos fatores intrínsecos e extrínsecos. O tratamento com métodos conservadores em um estágio inicial tem maior probabilidade de produzir resultados bem-sucedidos. Relatamos aqui um caso raro de redução espontânea bem-sucedida de LCJ em um bebê nas suas primeiras 24 horas de vida.

Linfadenopatía cervical y supraclavicular ipsilateral a la vacunación de refuerzo con Pfizer-BioNTech COVID-19 / Cervical and supraclavicular lymphadenopathy after Pfizer-BioNTech COVID-19 vaccination

La COVID-19 es la enfermedad causada por el nuevo coronavirus conocido como SARS-CoV-2. Para finales del 2020, la FDA de los Estados Unidos aprobó la primera vacuna para su uso de emergencia contra el COVID-19, desarrollada por Pfizer y BioNTech (BNT162b2). Este nuevo tipo de vacuna utiliza ARN mensajero modificado, el cual le da instrucciones al organismo para generar un fragmento de la proteína espiga de la superficie del virus, y que por sí sola desencadena una respuesta inmunitaria que ayuda a proteger el organismo contra una infección por COVID-19. Dentro de los eventos adversos menos comunes reportados en los estudios clínicos iniciales está la linfadenopatía (0.3 %). Objetivo: reportar el caso de paciente masculino que acude a evaluación sonográfica por preocupación de nódulo palpable en región supraclavicular. Resultados: a la evaluación sonográfica se observa cadena ganglionar reactiva compatible con una linfadenopatía. Paciente reporta vacunación de refuerzo con la vacuna Pfizer 8 días antes de la evaluación, subsecuente a dos vacunas Coronavac, corroborando de que se trata de una linfadenopatía reactiva, secundaria a una respuesta inmune robusta al refuerzo con la vacuna Pfizer. Se realiza una medición de Anti-SARS-CoV-2 TrimericS IgG cuantitativa a los 15 días del refuerzo con Pfizer, reportando valores elevados de 10,600 BAU/mL. Se orientó al paciente a regresar en una semana para seguimiento ecográfico, el cual evidenció resolución espontánea sin secuelas. Conclusiones: los hallazgos de adenopatía axilar o supraclavicular unilateral subsecuentes a la vacunación por COVID-19 deben ser informados tanto a médicos como pacientes, como un efecto secundario temporal producto de la respuesta inmunológica post vacuna. Este hallazgo benigno no requiere seguimiento adicional de imágenes y mucho menos de procedimientos invasivos como biopsias, los cuales generan mucha ansiedad al paciente, además de ser muy costosos para los mismos

COVID-19 is a disease caused by a new coronavirus identified as SARS-CoV-2. Towards the end of 2020, the FDA of the United States approved the first vaccine for emergency use against COVID-19, which was developed by Pfizer and BioNTech (BNT162b2). This new type of vaccine uses a modified RNA Messenger, which gives instructions to the host cells of the vaccinated person to produce a fragment of the spike protein of the virus, which then generates an inmune response and protects the recipient of the vaccine against COVID-19. Among the adverse events less frequently reported in the initial clinical studies of the vaccine is lymphadenopathy which was reported by 0.3% of the participants. Objective: Presentation of a case report of a male subject that came to a ultrasound evaluation due to concern of a palpable nodule in the supraclavicular región. Results: Ultrasound exam showed reactive unilateral cervical and supraclavicular lymphadenopathy. Patient reports a third dose booster with the Pfizer-BioNTech COVID-19 (BNT162b2) vaccine, 8 days prior to the evaluation, after completing a two-dose vaccination schedule with the Coronavac/Sinovac vaccine, confirming a vigorous immune response to the mRNA anti-COVID vaccines. This response was validated by elevated Anti-SARS-CoV-2 TrimericS IgG (10,600 BAU/mL). Patient was informed to return in a week for an echography follow-up which showed spontaneous resolution without leaving sequelae. Conclusions: It is of great importance to inform this benign finding of supraclavicular or axillar adenopathy subsequent to COVID vaccination to the medical community and patients, to avoid unnecessary medical interventions such as imaging or biopsies, which generate anxiety to the patient as well as additional costs

Hematoma subgaleal espontáneo en lactantes, una enfermedad subestimada, serie de casos / Spontaneous subgaleal hematoma in infants, an underestimated disease, case series / Hematoma subgaleal espontâneo em bebês, uma doença subestimada, série de casos

Introducción. Los hematomas subgaleales son patologías poco frecuentes que suelen originarse y resolverse espontáneamente. En neonatos se asocian con distocias y en lactantes o niños mayores con historial de traumatismo, incluso trivial, que puede pasar inadvertido. Hay un pequeño grupo donde no hay antecedente de trauma ni de otros factores como discrasias sanguíneas, se conforma principalmente por lactantes. El objetivo del presente artículo es mostrar una serie de casos de hematomas subgaleales espontáneos en lactantes, por su poca frecuencia, dificultad diagnóstica y diferencias de acuerdo con el tipo neonatal. Casos clínicos. Se presentan cuatro lactantes, entre 4 y 12 meses de edad, diagnosticados con hematomas subgaleales espontáneos (sin relación directa con traumatismo previo), de diferente severidad y atendidos en el servicio de urgencias. Se les realizó una evaluación diagnóstica dirigida a confirmar el hematoma y descartar trastornos de coagulación asociados. Todos los casos mostraron un curso clínico autolimitado sin complicaciones. Discusión. Los hematomas subgaleales espontáneos en lactantes suelen ser benignos, aunque es de vital importancia descartar discrasias sanguíneas. Dentro de éstas, los trastornos plaquetarios no suelen tenerse en cuenta y deben estudiarse. Su manejo es conservador y se reabsorberán solos en pocas semanas. Conclusiones. se debe establecer la causa de los hematomas subgaleales en niños pequeños y contemplar su posible aparición espontánea o por causa de discrasias sanguíneas.

Introduction. Subgaleal hematomas are rare pathologies that usually arise and resolve spontaneously. In neonates they are associated with dystocia and in infants or older children with a history of trauma, even trivial, which may go unnoticed. There is a small group where there is no background of trauma or other factors such as blood dyscrasias, it is made up mainly of infants. The objective of this article is to show a case series of spontaneous subgaleal hematomas in infants, due to their infrequency, diagnostic difficulty, and differences according to neonatal type. Clinical cases. Four infants are presented, between 4 and 12 months of age, diagnosed with spontaneous subgaleal hematomas (not directly related to previous trauma), of different severity and treated in the emergency unit. They underwent a diagnostic test aimed at confirming the hematoma and ruling out associated coagulation disorders. All cases showed a self-limited clinical course without complications. Discussion. Spontaneous subgaleal hematomas in infants are usually benign, although it is vitally important to rule out blood dyscrasias. Within these, platelet disorders are not usually taken into account and should be studied. Its management is conservative and they will be reabsorbed on their own in a few weeks.

Introdução. Os hematomas subgaleais são patologias raras que geralmente surgem e se resolvem espontaneamente. Em recém-nascidos estão associados a distocia e em bebês ou crianças mais velhas com um historial de trauma, mesmo trivial, que pode passar despercebido. Existe um pequeno grupo onde não há historial de traumas ou outros fatores como discrasias sanguíneas, é constituído principalmente por bebês. O objetivo deste artigo é mostrar uma série de casos de hematomas subgaleais espontâneos em bebês, devido à sua infrequência, dificuldade diagnóstica e diferenças de acordo com o tipo neonatal. Casos clínicos. São apresentados quatro bebês, entre 4 e 12 meses de idade, com diagnóstico de hematoma subgaleal espontâneo (não diretamente relacionado a traumas anteriores), de gravidade variável e atendidos no pronto-socorro. Eles foram submetidos a uma avaliação diagnóstica com o objetivo de confirmar o hematoma e descartar distúrbios de coagulação associados. Todos os casos apresentaram evolução clínica autolimitada e sem complicações. Discussão. Os hematomas subgaleais espontâneos em bebês geralmente são benignos, embora seja de vital importância descartar discrasias sanguíneas. Nestas, os distúrbios plaquetários geralmente não são considerados e devem ser estudados. Seu manejo é conservador e serão reabsorvidos por conta própria em algumas semanas. Conclusões. Deve ser estabelecida a causa dos hematomas subgaleais em crianças pequenas e, se considerar seu possível aparecimento espontâneo ou devido a discrasias sanguíneas.

Occurrence and remission of loss of sensitivity of inferior alveolar nerve in mandibular fractures

ABSTRACT Purpose: to evaluate the occurrence of loss of sensitivity of the inferior alveolar nerve, and to monitor the remission of this change in patients with mandibular fractures subjected to surgical treatment. Methods: patients with mandibular fractures, surgically treated within one year, were prospectively evaluated. Data regarding etiology, fracture location, type and displacement, and surgical access, were obtained. The tactile and thermal tests were applied at eighteen points in the mental region, within a period of six months. Statistical tests were applied to compare the categories of variables and the period of observation (p ≤ 0.050). Results: during the study, 37 patients were included. There were 24 bilateral and 13 unilateral fractures. Sensory changes occurred in 56.8% of the patients in the preoperative period, in 83.8% of the patients, in the postoperative period, and 35.1% of the patients presented complete remission during the final period of the study. Conclusion: sensory changes occurred in about half of the patients, due to the fracture, increasing greatly in the postoperative period, with complete remission in about one third of the cases. The fracture type, degree of displacement and surgical access type influenced the occurrence of sensory alterations.

Spontaneous remission of follicular bronchiolitis with nonspecific interstitial pneumonia: A case report and literature review / 北京大学学报(医学版)

A 41-year-old female patient was admitted in Department of Respiratory and Critical Care Medicine, Peking University Third Hospital because of having cough for a year. Multiple subpleural ground grass and solid nodules could be seen on her CT scan. Four months before admission, she began to experience dry mouth and eyes, blurred vision, finger joints pain, muscle pain and weakness in both lower limbs and weight loss. At the time of admission, the patient's vital signs were normal, no skin rash was seen, breath sounds in both lungs were clear, no rales or wheeze, no deformities in her hands, no redness, swelling, or tenderness in the joints. There was no edema in both lower limbs. Some lab examinations were performed. Tumor markers including squamous cell carcinoma (SCC) antigen, neuron-specific enolase (NSE), carcinoembryonic antigen (CEA), Cyfra21-1, pro-gastrin-releasing peptide (proGRP), carbohydrate antigen 125 (CA125) and carbohydrate antigen 199 (CA199) were all normal. The antinuclear antibody, rheumatoid factor, antineutrophil cytoplasmic antibody, anti-dsDNA antibody, anti-Sm antibody, anti-SSA/SSB antibody, anti-ribonucleoprotein (RNP) antibody, anti-Jo-1 antibody, anti-SCL-70 antibody and anti-ribosomal antibody were all negative. The blood IgG level was normal. The blood fungal β-1.3-D glucose, aspergillus galactomannan antigen, sputum bacterial and fungal culture, and sputum smear test for acid-fast staining were all negative. Lung function was normal. Bronchoscopy showed the airways and mucosa were normal. To clarify the diagnosis, she underwent thoracoscopic lung biopsy, the histopathology revealed follicular bronchiolitis (FB) with nonspecific interstitial pneumonia (NSIP). She did not receive any treatment and after 7 months, the lung opacities were spontaneously resolved. After 7 years of follow-up, the opacities in her lung did not relapse. To improve the understanding of FB, a literature research was performed with "follicular bronchiolitis" as the key word in Wanfang, PubMed and Ovid Database. The time interval was from January 2000 to December 2018. Relative articles were retrieved and clinical treatments and prognosis of FB were analyzed. Eighteen articles concerning FB with complete records were included in the literature review. A total of 51 adult patients with FB were reported, including 18 primary FB and 33 secondary FB, and autoimmune disease was the most common underlying cause. Forty-one (80.4%) patients were prescribed with corticosteroids and/or immunosuppressive agents, 6 (11.8%) patients were treated with anti-infective, 5 (9.8%) patients did not receive any treatment. The longest follow-up period was 107 months. Among the 5 patients without any treatment, 1 patients died of metastatic melanoma, the lung opacities were unchanged in 1 patient and getting severe in 3 patients. In conclusion, FB is a rare disease, the treatment and prognosis are controversial. Corticosteroid and immunosuppressive agents could be effective. This case report suggests the possibility of spontaneous remission of FB.

Necrosis grasa del recién nacido / Fat necrosis of the newborn

Resumen: Introducción: Las paniculitis constituyen un grupo de enfermedades que afectan al tejido graso subcutáneo y se manifiestan clínicamente como nódulos. Su patogenia no es del todo clara, por lo general es asintomático. El diagnóstico confirmatorio es histológico. Objetivo: describir las características clínicas e histopatológicas de un caso de necrosis grasa, una forma especifica de paniculitis en el recién nacido (RN). Caso Clínico: RN femenino de 40 semanas, parto cesárea de urgencia por taqui cardia fetal más meconio, APGAR 7-8-9. Requirió oxigeno y presión positiva por 5 min. Al quinto día de vida presentó aumento de volumen en tronco posterior, de coloración eritematosa - violácea, remitente a la palpación, no doloroso. Ecotomografría de piel y partes blandas informó aumento de la ecogenecidad del tejido celular subcutáneo y pérdida de definición de las celdillas adiposas de aproximadamente 42.3 x 9.7 x 20.1 mm, sin presencia de vascularización. Biopsia de piel: epidermis con acantosis irregular y ortoqueratosis en cestas; dermis papilar con infiltrado inflamatorio y dermis reticular y tejido adiposo con presencia de infiltrado linfohistocitiario con tendencia a la formación de nódulos, sin compromiso vascular, leves depósitos de colesterol; compatible con necrosis grasa del recién nacido (NGRN). Paciente tuvo regresión completa de la lesión a los tres meses de vida. Conclusiones: se describe un caso compatible clínica e histológicamente con NGRN; sin complica ciones durante su observación. Por lo general esta patología tiene buen pronóstico, con resolución espontánea, como en nuestro caso.

Abstract: Introduction: Panniculitis is a group of diseases that affect subcutaneous fat tissue and clinically manifest as nodules. Its pathogenesis is not entirely clear, and it is usually asymptomatic. The confirma tory diagnosis is histological. Objective: To describe the clinical and histopathological characteristics of a case of fat necrosis, a specific form of panniculitis in the newborn (NB). Clinical Case: 40-week female NB, born by emergency cesarean section due to fetal tachycardia with meconium, Apgar score 7-8-9. She required oxygen and positive pressure for five minutes. On the fifth day of life, she presen ted an increased volume in the posterior trunk region, with an erythematous - purplish discoloration, which is soft and non-tender to palpation. Skin and soft tissues ultrasound showed increased echo genicity of the subcutaneous cellular tissue and loss of definition of the adipocytes of 42.3 x 9.7 x 20.1 mm approximately, without vascularization. Skin biopsy showed epidermis with irregular acanthosis and basket-weave orthokeratosis; papillary dermis with inflammatory infiltrate, and reticular dermis and adipose tissue with presence of lymphohistiocytic infiltrate with a tendency to form nodules, without vascular involvement, and small cholesterol deposits, compatible with subcutaneous fat ne crosis (SBFN) of the newborn. The patient at three months of age had complete regression of the lesion. Conclusions: a clinically and histologically compatible case with SBFN is described, that did not present complications during observation. In general, this pathology has a good prognosis, with spontaneous resolution as in our case.

Natural History of Chronic Urticaria in Korea

Spontaneous outer retinal layer recovery in a case of hypertensive choroidopathy secondary to pre-eclampsia: a multimodal evaluation / Recuperação espontânea das camadas externas da retina em um caso de coroidopatia hipertensiva secundária a pré-eclâmpsia: avaliação multimodal

ABSTRACT A 26-year-old woman presented at 28 weeks gestation with hypertensive choroidopathy associated with pre-eclampsia. Fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed in both eyes in the immediate postoperative period. SD-OCT images were obtained before delivery and during a 3-month follow-up. Fundus autofluorescence exhibited patchy hyper- and hypoautofluorescent lesions; fluorescein and indocyanine green angiography revealed areas of choroidal ischemia; and SD-OCT showed disorganization of the outer retinal layers and disruption of the ellipsoid zone. After her blood pressure was stabilized, progressive recovery of the outer retinal layer was monitored on SD-OCT.

RESUMO Uma mulher de 26 anos de idade, com 28 semanas de gestação apresentando coroidopatia hipertensiva associada à pré-eclâmpsia. Retinografia, autofluorescência, tomografia de coerência óptica de domínio espectral, angiofluoresceínografia e angiografia com indocianina verde foram realizadas em ambos os olhos no período pós-operatório imediato do parto. Imagens da tomografia de coerência óptica de domínio espectral foram obtidas antes do parto e durante o seguimento de 3 meses. A autofluorescência apresentou lesões heterogêneas hiper e hipoautofluorescentes, a angiofluoresceínografia e angiografia com indocianina verde revelaram áreas de isquemia de coroide, e a tomografia de coerência óptica de domínio espectral apresentou desorganização das camadas externas da retina e interrupção da zona elipsóide. Após a estabilização da pressão sanguínea, a recuperação progressiva da camada externa da retina foi monitorada pela tomografia de coerência óptica de domínio espectral.

Síndrome de flutter ocular e ataxia de tronco pós-infecciosa / The post-infectious ocular flutter syndrome and truncal ataxia

OBJETIVO: Descrever um quadro clínico subagudo pós-infeccioso caracterizado por ataxia de tronco e flutter ocular. RELATO DE CASO: Um homem de 37 anos previamente hígido, pouco após um quadro sistêmico inespecífico que se resolveu espontaneamente, deu início a movimentos involuntários hipercinéticos dos olhos, da cabeça e desequilíbrio importante. Os sintomas evoluíram em poucos dias. Ao exame, apresentava ataxia de tronco e presença de flutter ocular. O líquor mostrou pleocitose discreta. Ressonância magnética (RM) de crânio e exames laboratoriais normais. Houve melhora espontânea e total dos sintomas em cerca de três semanas. CONCLUSÃO: A síndrome de flutter ocular e ataxia de tronco é rara e é destacado a benignidade do quadro.

OBJECTIVE: To describe a post-infectious subacute clinical picture characterized by truncal ataxia and ocular flutter. CASE REPORT: A healthy 37-year-old man, a few days after spontaneously resolved nonspecific systemic disease, initiated involuntary hyperkinetic movements of the eyes, head, and major imbalance. The symptoms progressed within a few days. He presented ataxia of the trunk and presence of ocular flutter. CSF showed pleocytosis. Magnetic resonance of the skull and laboratory tests were normal. There was spontaneous and total improvement of symptoms in about three weeks. CONCLUSION: The ocular flutter syndrome and truncal ataxia is rare and the benignity of the condition is highlighted.

Identification and management of inverted or everted edges of traumatic tympanic membrane perforations / Identificação e conduta nas perfurações traumáticas da membrana timpânica com bordas invertidas ou evertidas

Abstract Introduction: Most of traumatic tympanic membrane perforations have inverted or everted edges, however, the effects of inverted and everted edges on the spontaneous healing of the eardrum remain controversial. Objective: We investigated the influence of inverted or everted edges on the spontaneous healing of traumatic tympanic membrane perforations. Methods: The clinical records of patients with a traumatic tympanic membrane perforations who met the study criteria were retrieved and categorized into two groups, based on whether the eardrum was inverted or everted. The features along the edge of each inverted or everted eardrum were described using 30º and 70º endoscopes. Results: In total, 196 patients (196 ears) met the inclusion criteria; of these, 148 had inverted or everted eardrums while 48 did not. Of the 148 patients with inverted or everted eardrums, the perforation edges were everted in 77 patients, inverted in 44 patients, drooping in 17 patients, and both inverted and everted in 10 patients. The perforation shape was triangular in 18.9% of patients, sector-shaped in 11.5%, kidney-shaped in 14.2%, ovoid in 20.3%, and irregularly shaped in 35.1% of patients. The difference was not significant between the with and without inverted/everted eardrum edges groups in terms of the closure rate or closure time. Similarly, the difference was not significant between the with and without edge approximation groups in terms of the closure rate or closure time at the end of the 12-month follow-up period. Conclusion: This study suggests that endoscopic inspection can clearly identify inverted/everted eardrum edges using 30º and 70º endoscopes. The edge is glossy in inverted/everted eardrums, whereas the edge is rough and irregular in non-inverted/everted cases. The inverted/everted eardrums gradually became necrotic, but this did not affect the healing process. Additionally, edge approximation did not improve the healing outcome of traumatic tympanic membrane perforations.

Resumo Introdução: A maioria das perfurações de membrana timpânica traumáticas apresenta bordas invertidas ou evertidas; no entanto, os efeitos dessas configurações sobre a cicatrização espontânea do tímpano continuam a ser uma questão controversa. Objetivo: Investigar a influência de bordas invertidas ou evertidas sobre a cicatrização espontânea de perfurações traumáticas de membrana timpânica. Método: Os prontuários clínicos de pacientes com perfuração traumática de membrana timpânica que preencheram os critérios do estudo foram recuperados e categorizados em dois grupos, baseados na configuração invertida ou evertida das bordas da membrana timpânica. As características de configuração da borda de cada membrana foram descritas com o uso de endoscópios de 30º e 70º. Resultados: No total, 196 pacientes (196 orelhas) preencheram os critérios de inclusão; desses, 148 apresentavam bordas de membranas timpânicas invertidas ou evertidas, enquanto 48 não. Dos 148 pacientes, as bordas da perfuração estavam evertidas em 77 pacientes, invertidas em 44 pacientes, caídas em 17 pacientes e ambas invertidas e evertidas em 10 pacientes. O formato da perfuração era triangular em 18,9% dos pacientes, em forma de fatia de pizza em 11,5%, em forma de rim em 14,2%, ovoide em 20,3% e de forma irregular em 35,1% dos pacientes. A diferença não foi significante entre os grupos com e sem membrana timpânica invertida/evertida em termos de taxa ou tempo de fechamento. Da mesma forma, a diferença não foi significativa entre os grupos com e sem aproximação das bordas em termos de taxa de fechamento ou tempo de fechamento no fim do período de seguimento de 12 meses. Conclusões: Este estudo sugere que a avaliação com endoscópios de 30º e 70º pode identificar claramente as bordas invertidas/evertidas das perfurações de membranas timpânicas. A borda da perfuração timpânica em casos invertidos/evertidos é brilhante, enquanto a borda é áspera e irregular em casos não invertidos/evertidos. O rebordo timpânico invertido/evertido gradualmente torna-se necrótico, mas isso não afetou o processo de cicatrização. Além disso, a aproximação das bordas não melhorou o resultado da cicatrização.

Pênfigo neonatal em filho de mãe com pênfigo vulgar: relato de caso / Neonatal pemphigus in an infant born to a mother with pemphigus vulgaris: a case report

RESUMO Objetivo: Relatar um caso de pênfigo neonatal em paciente que manifestou lesões cutâneas extensas e críticas ao nascimento. Descrição do caso: Recém-nascido do sexo masculino com lesões vesicobolhosas extensas em região anterior do tórax e abdome, desde o nascimento. Admitido na ala pediátrica de um hospital para diagnóstico etiológico e tratamento. Com base na história materna e na avaliação clínica, concluiu tratar-se de pênfigo vulgar neonatal. O paciente apresentou evolução satisfatória, sem a necessidade de intervenção farmacológica. Comentários: Os casos descritos na literatura e as referências avaliadas revelam o pênfigo neonatal como uma doença de ocorrência rara, porém cujo conhecimento e diagnóstico precoce têm grande relevância clínica, considerando-se que geralmente se manifesta com lesões epidérmicas extensas e de aspecto crítico, embora apresente curso clínico transitório e benigno, sem necessidade de tratamento específico e sem relação com doença futura.

ABSTRACT Objective: To report on the case of a patient with neonatal pemphigus that had extensive and critical skin lesions at birth. Case description: A newborn male with extensive vesico-bullous lesions on the anterior side of his chest and abdomen at birth. He was admitted to the pediatric ward of a hospital for an etiological diagnosis and for treatment. Based on maternal history and a clinical evaluation, the patient was diagnosed with neonatal vulgar pemphigus. His progression was satisfactory and, in the end, he did not need pharmacological interventions. Comments: The cases reported in the literature and the references evaluated reveal that neonatal pemphigus is rare, but that knowledge about the disease allows for an early diagnosis to be made. This has great clinical relevance considering that the disease usually manifests itself in the form of extensive epidermal lesions, even though it is transient and benign, it does not require specific treatment, and it does not have any relation with possible future diseases.

A Case of Recurrent Rosai-Dorfman Disease Successfully Treated with 2-Chlorodeoxyadenosine (Cladribine) / 임상소아혈액종양

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.

Frequency and clinical relevance of anti-Mi-2 autoantibody in adult Brazilian patients with dermatomyositis

Abstract Background: To analyze the frequency and clinical relevance of anti-Mi-2 autoantibody in a representative sample of patients with dermatomyositis. Methods: This longitudinal inception cohort study, from 2001 to 2017, included 87 definite adult dermatomyositis. Anti-Mi-2 analysis was performed using a commercial kit. Results: Seventeen patients (19.5%) had anti-Mi-2 and 70 (80.5%) did not have this autoantibody. The following parameters were equally distributed between the patients with versus without anti-Mi-2: mean age at the disease diagnosis onset, median follow-up time, constitutional symptoms (baseline), cutaneous cumulative lesions, dysphagia, joint and pulmonary involvement. There was also no difference between the groups in relation to follow-up time, disease relapsing, treatment, disease status, deaths and occurrence of neoplasia. In contrast, patients with anti-Mi2 antibodies had higher frequency of elevated serum levels of muscle enzymes at disease onset (median: creatine phosphokinase 6240 [3800-9148] U/L and aldolase 60.0 [35.0-138.0] U/L), lower frequency of pulmonary involvement at disease onset (5.9%), less current glucocorticoid dose (median: 0 [0-10] mg/day), and higher frequency of disease remission during follow-up (58.8%) in comparison with patients without anti-Mi-2 autoantibody (484 [115-4880] and 12.1 [6.3-70.0] U/L, 40.0%, 0 [0-10] mg/day, 27.1%, respectively). Conclusion: The anti-Mi-2 autoantibody was found in one fifth of patients with dermatomyositis. This autoantibody was associated with a lower occurrence of pulmonary involvement, a higher frequency of disease in remission, and elevated levels of muscle enzymes. There was also no correlation regarding the frequency of disease relapsing or neoplasia development.

Remisión espontánea de acromegalia y enfermedad de Cushing en adultos mayores: casos clínicos / Spontaneous remission of acromegaly and Cushing's disease in older patients: report of two cases

Pituitary diseases such as acromegaly and Cushing's disease require surgical or medical therapy. In some functioning pituitary tumors, a spontaneous remission of hormonal hypersecretion is observed, mainly associated to apoplexy or pituitary infarction. We report the evolution of two female patients older than 70 years at the time of diagnosis, with multiple comorbidities. In case 1, acromegaly was diagnosed at 74 years of age. Sellar CT scan showed a 10-mm adenoma. During her follow-up, IGF1 levels remained normal. Nine years later, a magnetic resonance (MR) showed a 7-mm adenoma. In case 2, clinical and biochemical diagnosis of Cushing's disease was done being 71 years old. Sellar MR showed a 6-mm adenoma. Three years later, urinary cortisol normalized with no changes in adenoma at MR. Seven years later, she remains without clinical or biochemical signs of hypercortisolism. In both cases, no signs of hemorrhage were observed at MR.

Evaluation of a birth-related foveal hemorrhage in an infant using optical coherence tomography / Avaliação de uma hemorragia foveal relacionada ao nascimento de uma criança usando tomografia de coerência óptica

ABSTRACT During the routine ophthalmologic examination of a 38-day old female infant, indirect ophthalmoscopy revealed a dense hemorrhage on the fovea and a couple of superficial hemorrhages in the nasal retina of the left eye. No hemorrhage was observed in the right eye. A hand-held spectral domain optical coherence tomography (SD-OCT) was used at the time of diagnosis. The hemorrhage at the nasal retina resolved in the first week of follow-up, and the foveal hemorrhage resolved 12 weeks after birth. Spectral domain optical coherence tomography was repeated once the foveal hemorrhage had resorbed, and it showed that the foveal contour had reformed without any sequelae. This case suggests that birth-related foveal hemorrhages do not cause any disturbance in the foveal architecture.

RESUMO Durante o exame oftalmológico de rotina de uma criança do sexo feminino de 38 dias, a oftalmoscopia indireta revelou uma hemorragia densa na fóvea e algumas hemorragias superficiais na retina nasal do olho esquerdo. Nenhuma hemorragia foi observada no olho direito. Foi utilizada uma tomografia de coerência óptica de domínio espectral no momento do diagnóstico. A hemorragia na retina nasal foi resolvida na primeira semana de acompanhamento e a hemorragia foveal foi resolvida em 12 semanas após o nascimento. A tomografia de coerência óptica de domínio espectral foi repetida uma vez que a hemorragia foveal foi reabsorvida, e mostrou que o contorno foveal havia se reformado sem sequelas. Este caso sugere que as hemorragias foveais relacionadas ao nascimento não causam qualquer distúrbio na arquitetura foveal.

Atypical Gianotti-Crosti syndrome

Abstract: We report the case of a male 22-month-old child, with atypical presentation of Gianotti-Crosti syndrome after infection with Epstein-Barr virus.

Oxidative stress in patients with endemic pemphigus foliaceus and healthy subjects with anti-desmoglein 1 antibodies

Abstract: Background: Previous studies have shown oxidative stress in pemphigus vulgaris and pemphigus foliaceus, nevertheless, it remains unknown whether a similar response is characteristic of endemic pemphigus foliaceus in Peru. Objectives: To determine the oxidative stress response in endemic pemphigus foliaceus patients and subjects with positive for anti-desmoglein1 antibodies (anti-dsg1) from endemic areas of Peru. Subjects and Methods: This is a cross-sectional study. The study population included 21 patients with Endemic Pemphigus foliaceus and 12 healthy subjects with anti-dsg1 antibodies from the Peruvian Amazon (Ucayali), as well as 30 healthy control subjects. Malondialdehyde, an indicator of lipid peroxidation by free radicals, was measured in serum. Results: We collected 21 cases of endemic pemphigus foliaceus, 15 of them with active chronic disease and 6 in clinical remission. Serum malondialdehyde values in patients with chronic active evolution and healthy subjects with anti-dsg1 antibodies were statistically higher than those of healthy controls (p<0.001). There was no significant difference between serum values of localized and generalized clinical forms. Study limitations: The main limitation of this present study is the small number of patients with endemic pemphigus and healthy subjects positive for desmoglein 1 antibodies. Conclusions: The increased serum levels of malondialdehyde in patients with chronic active endemic pemphigus foliaceus and healthy subjects from endemic areas with anti-dsg1 antibodies may suggest a contribution of systemic lipid peroxidation in the pathogenesis of endemic pemphigus foliaceus.

Anti-phospholipase A2 receptor antibody as a prognostic marker in patients with primary membranous nephropathy

BACKGROUND: Phospholipase A2 receptor (PLA2R) has been identified as a major autoantigen in primary membranous nephropathy (MN). We evaluated the association between anti-PLA2R antibodies and clinical outcome in Korean patients with primary MN. METHODS: A total of 66 patients with biopsy-proven MN were included. Serum level of anti-PLA2R antibodies was measured by enzyme-linked immunosorbent assay. Biochemical parameters were estimated initially and at follow-up. RESULTS: Anti-PLA2R antibodies were detected in 52.1% and 27.8% of patients with primary and secondary MN, respectively. Forty-eight patients with primary MN were grouped based on presence or absence of anti-PLA2R antibodies. Proteinuria was more severe in anti-PLA2R-positive patients than in anti-PLA2R-negative patients (urine protein/creatinine ratio 7.922 ± 3.985 g/g vs. 4.318 ± 3.304 g/g, P = 0.001), and anti-PLA2R antibody level was positively correlated with proteinuria. The incidence of chronic kidney disease stage ≥ 3 was higher in anti-PLA2R-positive patients compared with anti-PLA2R-negative patients (P = 0.004). The probabilities of spontaneous remission were higher in anti-PLA2R-negative patients compared with anti-PLA2R-positive patients (P < 0.001). Multivariate analysis demonstrated that anti-PLA2R antibodies are an independent risk factor for developing chronic kidney disease stage ≥ 3 and for not reaching spontaneous remission. CONCLUSION: Detection of anti-PLA2R antibodies at diagnosis in patients with primary MN can predict prognosis and guide treatment decisions.