Browsing by Author "Ferreira, R"
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- Bowel-associated dermatosis-arthritis syndrome in an adolescent with short bowel syndromePublication . Pereira, E; Estanqueiro, P; Almeida, S; Ferreira, R; Tellechea, O; Salgado, MBowel-associated dermatosis-arthritis syndrome (BADAS) is a neutrophilic dermatosis, characterized by the occurrence of arthritis and skin lesions related to bowel disease with or without bowel bypass. We report an unusual case of BADAS in a 15-year-old white male with congenital aganglionosis of the colon and hypoganglionosis of the small intestine and multiple bowel surgeries in childhood complicated by short bowel syndrome. He presented with recurrent peripheral polyarthritis, tenosynovitis, and painful erythematous subcutaneous nodules located on the dorsolateral regions of the legs and on the dorsa of the feet. Histological examination disclosed a neutrophilic dermatosis confirming the diagnosis of BADAS.Although an uncommon disease, especially at pediatric age, it is important to evoke the diagnosis of BADAS in children and adolescents with bowel disease, because treatment options and prognosis are distinct from other rheumatologic conditions.
- Embolização percutânea de fístula coronária: caso clínicoPublication . Rodrigues, D; Sá e Melo, A; Marinho da Silva, A; Ferreira, R; Martins, FM
- Encephalopathy Associated with Autoimmune Thyroid Disease: A Potentially Reversible ConditionPublication . Correia, I; Marques, I; Ferreira, R; Sousa, LAutoimmune thyroid disease may occasionally associate with unspecific neurological symptoms, which are more commonly insidious, include cognitive or behavioural symptoms, and may associate with tremor, myoclonus, or ataxia. We report a 61-year-old female patient who presented with chronic headache, insidious mood, and cognitive disturbance which evolved in a few months to dementia associated with exuberant limb myoclonus. Diagnostic workup revealed high anti-thyroid peroxidase antibody titers and an inflammatory CSF profile, and it was negative for other possible etiologies. Treatment with steroids induced significant improvement. The diagnosis of encephalopathy associated with autoimmune thyroid disease is still controversial given the fact that the clinical presentation and diagnostic workup are unspecific, the pathophysiology is still undetermined, and the diagnosis is mostly of exclusion. No direct correlation is found between anti-thyroid antibody titers and clinical presentation, and it is currently speculated that other still unrecognized antibodies may be responsible for this clinical entity. It is extremely important to recognize this entity because it is potentially treatable with immunotherapies. It is also increasingly recognized that clinical improvement with first-line treatment with steroids may be absent or incomplete, and other immunotherapies as immunosuppressants, intravenous immunoglobulin, or plasma exchange must be attempted in the clinical suspicion of EEAT.
- HorrendoplastiasPublication . Freitas, J; Casanova, J; Moura, DL; Ferreira, R; Judas, F; Fonseca, FA infeção e reações alérgicas associadas a grandes perdas de tecido ósseo em artroplastias protéticas da anca e joelho ou a infeção em reconstruções ósseas com aloenxertos estruturais de grandes dimensões pós resseções ósseas por patologia tumoral, correspondem a grandes desafios cirúrgicos de salvamento de membros. São casos clínicos submetidos a múltiplas cirurgias, anos de evolução, fistulas profusamente produtivas, tratamentos antibióticos múltiplos, pessoas socialmente e profissionalmente destruídas. Muitos destes casos são propostos para cirurgia mutiladora com amputações de membros ou extração de material protético sem reconstrução estrutural, condenando o doente a incapacidades funcionais marcantes. Apresentam-se 9 casos clínicos de doentes, 8 com artroplastias primárias, de revisão ou tumorais, de anca e joelho, complicadas com infeção (6 doentes) e/ou reação alérgica a metais/iodo (2 doentes) e 1 doente com aloenxerto estrutural do fémur de grandes dimensões infetado, a maioria com perda de osso e proposta de amputação, nomeadamente desarticulação pela anca. Na maioria dos casos tratava-se de infeção por St. aureus meticilino resistente, havendo 1 doente com flora polimicrobiana hospitalar resistente, submetidos a múltiplas cirurgias e oriundos de vários hospitais do país. Os doentes foram operados entre Novembro de 2013 e Abril de 2016 tendo o mais novo 23 anos e o mais velho 76 anos. O tratamento cirúrgico foi composto por 2 tempos operatórios. Primeiro tempo (7 a 9h): extração de material protético; excisão de osso infetado e sem viabilidade; excisão em bloco de tecidos moles desvitalizados/necrosados e com exsudato em toda a periferia do material protético e tecido ósseo infetado, numa espessura com o mínimo de 4 mm; lavagem pulsátil do leito cirúrgico sangrante desbridado com betadine/H2O2 e soro fisiológico; reconstrução articular temporária com espaçador de grandes dimensões em metilmetacrilato com gentamicina. Todos os doentes foram submetidos a terapia antibiótica tripla endovenosa durante 8 a 9 semanas, com controlo analítico rigoroso, tendo alguns deles deambulado com apoio de ortóteses. 2º tempo cirúrgico (4 a 7h) após valores de PCR normais, fez-se conversão dos espaçadores utilizando material protético de revisão e do foro tumoral revestido a prata (potencial bactericida), 8 dos casos com próteses tumorais da anca (5 com reconstrução de ½ fémur proximal e 3 com fémures totais protéticos). Os doentes tiveram alta autónomos e com apoio de canadianas após +- 3 semanas com triterapia antibiótica e.v. e PCR normal ou “borderline”, com passagem a antibióticoterapia dupla oral em ambulatório, no mínimo durante 3 meses até normalização de PCR em 3 avaliações analíticas espaçadas em 15 dias. O doente mais antigo tem +- 29 meses com PCR normal (avaliações analíticas frequentes) e clinicamente todos se apresentam com PCR normais e sem sinais de infeção e/ou fistulas ativas. Funcionalmente, estão autónomos, os mais novos deambulam sem apoio de canadianas enquanto os mais velhos, por vezes, necessitam do apoio de 1 ou 2 canadianas, mas todos negam dores. Todas estas situações clinicas devem ser tratadas de forma agressiva, do ponto de vista de antibióticoterapia alargada e com conjugação de complexas técnicas cirúrgicas do âmbito de revisão protética e do foro tumoral, para se poder obter os melhores resultados possíveis no salvamento de membros, livres de infeção e com capacidade funcional. Os doentes estão satisfeitos com o tratamento, apesar de extremamente exigente do ponto de vista físico e psíquico, por manterem os membros inferiores, referindo que se tivessem de voltar atrás passariam por tudo de novo. Não se pode dizer que estejam curados mas aqui apresenta-se uma metodologia médica e cirúrgica, como uma forma de ir mais além, na esperança da cura de situações clinico-cirúrgicas que muitos cirurgiões designam de horrendoplastias.
- Implantação de Dispositivo de Amplatzer® Após Desinserção do PrimeiroPublication . Andrade, H; Rodrigues, D; Marinho da Silva, A; Ferreira, R; Martins, FM; Sá e Melo, A
- Implantação de Dispositivo de Amplatzer® Após Desinserção do PrimeiroPublication . Andrade, H; Rodrigues, D; Marinho da Silva, A; Ferreira, R; Martins, FM; Sá e Melo, A
- Intoxicações Agudas num Serviço de Medicina Intensiva: Anos 2002 a 2014Publication . Ferreira, R; Cunha, B; Ferreira, DM; Devesa, N; Pimentel, J
- Juvenile polyposis of infancy in a child with deletion of BMPR1A and PTEN genes: Surgical approachPublication . Oliveira, PH; Cunha, C; Almeida, S; Ferreira, R; Maia, S; Saraiva, JM; Lopes, MFJuvenile polyposis of infancy is the most severe and life-threatening form of juvenile polyposis. This disease typically presents in the first two years of life with gastrointestinal bleeding, diarrhea, inanition, and exudative enteropathy. In very few reports concerning this entity, a large deletion in the long arm of chromosome 10 (10q23), encompassing the PTEN and BMPR1A genes, was found. The authors report a case of delayed diagnosis of juvenile polyposis of infancy at 6years of age. A 3.34Mb long de novo deletion was identified at 10q23.1q23.31, encompassing the PTEN and BMPR1A genes. The disease course was severe with diarrhea, abdominal pain, inanition, refractory anemia, rectal bleeding, hypoalbuminemia, and exudative enteropathy. A sub-total colectomy, combined with intraoperative endoscopic removal of ileal and rectal stump polyps, was required for palliative disease control.
- Limb salvage surgery in extreme situations of prosthetic complicationsPublication . Freitas, J; Moura, DL; Fonseca, R; Ferreira, R; Casanova, J; Judas, F; Fonseca, FThe treatment of periprosthetic hip and knee infection associated with loss of bone substance, as well as the treatment of the infection of large structural allografts used in tumoral reconstructive surgery, are a major challenge to the orthopaedic surgeon. Indeed, these are chronic conditions which are submitted to multiple surgeries and prolonged antibiotic therapy in socially and professionally vulnerable patients. Many of these cases receive proposals for limb amputation/disarticulation or extraction of the prosthesis without structural reconstruction aggravating, even more, their suffering and functional disability. The aim of this study is to show the results of a treatment of complex hip and knee periprosthetic infections and of a structural allograft, in the context of limb salvage surgery. 9 patients were treated, minimum age of 22 years and maximum of 76 years with multiple surgeries and from different national hospitals. Six of these patients had periprosthetic infection of the hip and knee (primary, revision and tumoral prostheses) and two of the patients showed an apparent allergic reaction to metal/iodine. The remaining case, an infection of a large femoral structural allograft, used in tumoral surgery. The main cause of the infection was the St. aureus multiresistant. One of the patients showed multimicrobial multiresistant flora. Treatment consisted in 2 different operative stages. First stage (7 to 9h): Extraction of the prosthesis or allograft; debridement and extensive excision of the periprothetic infected and devitalized bone and soft tissue, a minimum thickness of 4 mm; pulsatile lavage of the bleeding “surgical bed” with betadine / H2O2 and saline; implantation of large methyl methacrylate with gentamicin spacer. Triple intravenous antibiotic therapy was made for 8 to 9 weeks, with rigorous analytical control, and some of the patients were able to walk with the support of axillary support crutches. The second surgical stage (5 to 7h) takes place after normal levels of PCR: spacer excision with prosthetic joint reconstruction in 8 cases and in one case a silver coated knee arthrodesis prosthetic implant (bactericidal effect). So, 3 silver coated total femoral prostheses and 5 silver coated total hip prostheses, with the reconstruction of the proximal half of the femur, were applied. The interventions took place between July 2014 and April 2016. Patients were discharged after being able to walk with the help pf crutches and kept taking oral antibiotics until the normalization of PCR in 3 consecutive analytical assessments spaced by 15 days. After a clinical/ analytical evaluation, all patients showed a normal PCR, without pain and without signs of infection and/or active fistulas. Currently 5 patients walk without external support. The oldest case has 28 months of follow-up and all have a minimum follow-up of 7 months, average of 17,5 months. All patients expressed great satisfaction with the outcome of the surgical procedures for the preservation of their lower limbs. Chronic relapsing, multidrug-resistant, periprosthetic infection should be treated aggressively in a combination of surgical techniques of prosthetic revision and tumoral surgery, first through an extended debridement of devitalized tissue and the extraction of the prosthesis with the appropriate antibiotic therapy; second, with the reconstruction of the bone loss with silver coated modular prostheses, which are indicated in order to prevent the mutilating surgery and provide the restoration, as much as possible, of the functional capacity. The results obtained have been very satisfactory, although the follow-up time is insufficient to draw definitive conclusions about the infectious relapse. Such limb salvage surgery is indicated for the treatment of complex clinical situations as an alternative to the disarticulation / limb amputation, i.e. supports the hope of curing clinical and surgical situations that many surgeons designate by horrendoplasties.
- Malignant transformation in chronic osteomyelitisPublication . Moura, DL; Ferreira, R; Garruço, AIntroduction: Carcinomatous degeneration is a rare and late complication developing decades after the diagnosis of chronic osteomyelitis. Objectives: To present the results from a retrospective study of six cases of squamous cell carcinoma arising from chronic osteomyelitis. Methods: Six cases of chronic osteomyelitis related to cutaneous squamous cell carcinoma were identified. The cause and characteristics of the osteomyelitis were analyzed, as well as time up to malignancy, the suspicion signs for malignancy, the localization and histological type of the cancer, and the type and result of the treatment. Results: The mean time between osteomyelitis onset and the diagnosis of malignant degeneration was 49.17 years (range: 32-65). The carcinoma resulted from tibia osteomyelitis in five cases and from femur osteomyelitis in one. The pathological examination indicated cutaneous squamous cell carcinoma in all cases. All the patients were staged as N0M0, except for one, whose lomboaortic lymph nodes were affected. The treatment consisted of amputation proximal to the tumor in all patients. No patient presented signs of local recurrence and only one had carcinoma metastasis. Conclusion: Early diagnosis and proximal amputation are essential for prognosis and final results in carcinomatous degeneration secondary to chronic osteomyelitis.