Análise Retrospectiva do Protocolo de Tratamento Utilizado na Consulta Multidisciplinar de Úlcera de Perna do Hospital dos Capuchos no Período entre 2002 e 1º Semestre 2006

As úlceras de perna constituem uma importante patologia causando uma diminuição da qualidade de vida, hospitalizações frequentes e aumento da mortalidade e morbilidade. Têm uma incidência de 1% na população adulta, sendo que esta incidência atinge níveis de 10% nos escalões etários superiores a 70 anos. Cerca de 95% das úlceras são venosas, arteriais, mistas ou diabéticas, sendo as mais frequentes as úlceras venosas (70 a 80%). Com o objectivo de optimizar o tratamento e acompanhamento dos doentes com esta patologia, foi criada em 2002 uma Consulta de Referência Multidisciplinar de Úlcera de Perna, no Hospital dos Capuchos. Simultaneamente foi estabelecido um protocolo de referenciação/ tratamento com os Centros de Saúde da Unidade B da Sub-região de Saúde de Lisboa. Neste protocolo o doente é observado no contexto de uma equipa multidisciplinar. Os autores fizeram um estudo retrospectivo dos doentes observados nesta consulta no período entre 2002 e 1º semestre de 2006. Foram observados e acompanhados 294 novos doentes, tendo 80% idade superior a 60 anos. Em relação à etiologia das úlceras, 51,3% (n=151) eram venosas, 35,4% (n=104) eram diabéticas e 6,8% (n=20) eram arteriais. A área média das úlceras foi 23,9cm2 e o número médio de úlceras foi 1,6. A duração das úlceras tinha em 42,3% dos casos um período superior a 6 meses. Das 199 culturas positivas, 40,2% apresentavam Staphylococcus aureus, sendo 21,2% destes MRSA. Com o protocolo instituído, foi obtida uma taxa de cicatrização de 72,2%. 45,9% dos doentes tiveram uma cicatrização total da úlcera em menos de 2 meses, resultados estes que são muito positivos face às taxas de cicatrização de 6 meses referidas na literatura.

In Vitro Methods for Specific IgE Detection on Cow’s Milk Allergy

Background: A new method for determining serum specific IgE (IMMULITE“ 2000 3gAllergy) has recently become available. Objective: To evaluate the clinical performance of IMMULITE 2000 in the diagnosis of cow’s milk allergy compared with that of UniCAP“. Additionally, we verified the behavior of both methods at two diagnostic decision points proposed by other authors. Methods: The study population consisted of 31 children with cow’s milk allergy (group A) and a control group of 19 atopic children without food allergy(group B). A blood sample from each child was tested using both methods and the results were compared. Results: In group A, the values for cow’s milk IgE ranged from 0.35 kU/L (the lowest common detection limit) to above 100 kU/L. In group B, the values were less than 1.1 kU/L for IMMULITE 2000 and less than 1.6 kU/L for UniCAP. An agreement of 90 % in IgE classes was obtained. Both methods demonstrated exactly the same diagnostic performance(sensitivity: 100 %; specificity: 78.9 %; negative predictive value: 100%; positive predictive value: 84.6%;efficiency: 90.2 %). The evaluation of the two methods at the two different decision points proposed in the literature showed a better positive predictive value with UniCAP, but we obtained equivalent performance with IMMULITE 2000 by choosing higher cutoff values. Conclusions: We conclude that IMMULITE 2000 is as effective as UniCAP in the diagnosis of cow’s milk allergy. Both methods can be used to obtain site-specific decision points that are population, age and disease dependent.

Octreótido - Uma Terapêutica Opcional para Quilotórax Pós-Cirúrgico em Crianças com Cardiopatia Congénita

INTRODUCTION: Chylothorax is a rare but serious postoperative condition in children with congenital heart disease. Conventional medical treatment consists of specific long-term dietary modification, and surgical reintervention, such as lymphatic duct ligation, may be indicated in refractory cases. In recent years, an additional conservative treatment, octreotide, a synthetic analog of somatostatin, has been used in management of congenital and postoperative chylothorax. METHODS: The objective of this work was to analyze the efficacy and safety of this treatment for chylothorax after congenital heart surgery. We reviewed the records of sixteen patients with chylothorax after surgery for congenital heart disease between January 1999 and December 2007, and collected the following data: demographic information; type of surgical procedure; onset, duration and management of chylothorax and treatment; and duration of hospital stay. To analyze efficacy we compared these parameters in children receiving conventional treatment only with those receiving octreotide. To analyze safety we compared the adverse effects of both treatments. Octreotide was administered at a dose of 4 to 10 microg/kg/hour, with monitoring of side effects. RESULTS: The incidence of chylothorax in our population was 1.6%. It occurred more often after Glenn and Fontan procedures (8 patients). Octreotide was begun three days after diagnosis of chylothorax and continued for a median of seventeen days (ranging from 4 to 26 days), until complete resolution. Side effects were frequent (in 3 of the 8 patients) but of no clinical relevance. All patients responded to the therapy and there was no indication for further surgical intervention. DISCUSSION AND CONCLUSIONS: Octreotide is safe and effective in the treatment of postoperative chylothorax in children with congenital heart disease. It is a useful adjunctive therapy to the conventional treatment of this complication.

Cirurgia Cardíaca em Doentes de Muito Alto Risco

Purpose: To assess the results obtained in very high-risk patients, which are those patients with an EUROSCORE greater than 13 points. Material and methods: From September 2001 to September 2003, thirty-three very high-risk patients were operated on in our department, which represents 1.6% of all the surgical activity during that period of time, being 17 male and 16 female, with an average of 69 years old (maximum 86 and minimum 32). Diagnosis includes: post infarction CIV 5, coronary insufficiency 11, aortic dissection 3, mitral prosthesis 3, valvular disease 9, aortic prosthesis disfunction 2. Fifteen patients underwent an emergency procedure, 12 were urgent and the remaining 6 were electively operated on. Results: Overall post-operative mortality was 12 patients (36%), being 6 emergent, 5 urgent and 1 elective patient. Patients who survived the operation had longer intensive care and hospital admission periods, which will be analyzed in detail. Conclusion: Surgery can be justified in very high risk patients. Despite the high perioperative mortality and longer periods of hospital stay, they will be otherwise condamned to death, if surgery would not be performed.

Prostatic Arterial Supply: Anatomic and Imaging Findings Relevant for Selective Arterial Embolization

PURPOSE: To describe the anatomy and imaging findings of the prostatic arteries (PAs) on multirow-detector pelvic computed tomographic (CT) angiography and digital subtraction angiography (DSA) before embolization for symptomatic benign prostatic hyperplasia (BPH). MATERIALS AND METHODS: In a retrospective study from May 2010 to June 2011, 75 men (150 pelvic sides) underwent pelvic CT angiography and selective pelvic DSA before PA embolization for BPH. Each pelvic side was evaluated regarding the number of independent PAs and their origin, trajectory, termination, and anastomoses with adjacent arteries. RESULTS: A total of 57% of pelvic sides (n = 86) had only one PA, and 43% (n = 64) had two independent PAs identified (mean PA diameter, 1.6 mm ± 0.3). PAs originated from the internal pudendal artery in 34.1% of pelvic sides (n = 73), from a common trunk with the superior vesical artery in 20.1% (n = 43), from the anterior common gluteal-pudendal trunk in 17.8% (n = 38), from the obturator artery in 12.6% (n = 27), and from a common trunk with rectal branches in 8.4% (n = 18). In 57% of pelvic sides (n = 86), anastomoses to adjacent arteries were documented. There were 30 pelvic sides (20%) with accessory pudendal arteries in close relationship with the PAs. No correlations were found between PA diameter and patient age, prostate volume, or prostate-specific antigen values on multivariate analysis with logistic regression. CONCLUSIONS: PAs have highly variable origins between the left and right sides and between patients, and most frequently arise from the internal pudendal artery.

Variantes Genéticas Pró-Trombóticas como (Improváveis?) Factores de Risco para as Grandes Crises Vaso-Oclusivas na Drepanocitose

CONTEXTO: A drepanocitose é uma anemia hemolítica hereditária com características pró-adesivas, pró-inflamatórias e pró-coagulantes, incluindo alterações na hemostase e activação da cascata da coagulação. PLANO DO ESTUDO: Neste estudo analisaram-se, em 140 drepanocíticos africanos e 126 indivíduos sem hemoglobina S também de origem africana, variantes genéticas polimórficas em quatro loci envolvidos na coagulação (F2 20210G>A e F5 R506Q), na fibrinólise (PAI-1 5G>4G), ou no metabolismo da homocisteína (MTHFR 677C>T). Estratificaram-se os pacientes em dois grupos de acordo com a ocorrência ou não de, pelo menos, uma complicação vaso-oclusiva (CVO) grave até à data da sua participação no estudo. RESULTADOS: Não se observou uma associação estatisticamente significativa entre a ocorrência de uma CVO grave e a herança do alelo predisponente à trombose, 4G no locus PAI-1 ou 677T no locus MTHFR. Nenhum drepanocítico apresentava os alelos F2 20210A ou F5 506Q (factor V Leiden). Visando excluir a possibilidade de que genuínas diferenças inter-grupos fossem mascaradas pela presença de indivíduos mais jovens no grupo sem-CVO, dividiu-se este num sub-grupo de pacientes mais novos e num sub-grupo de pacientes cuja idade não diferia significativamente do grupo com-CVO grave. Mesmo assim, não foi encontrada associação significativa. No entanto, pode observar-se, no grupo de doentes com o alelo PAI-1 4G (cuja expressão resulta numa diminuição da actividade fibrinolítica), uma tendência (OR=1,6) para um risco acrescido de CVO. Esta tendência era ligeiramente maior (OR=2,1) se se considerasse apenas a CVO síndrome torácica aguda. CONCLUSÕES: O alelo 4G no promotor do PAI-1 poderá ser um factor de risco para CVO na drepanocitose, uma hipótese a testar numa série maior de doentes, idealmente oriunda de uma população homogénea e com alta prevalência de drepanocitose.

Clinical Outcome and Morphologic Analysis after Endovascular Aneurysm Repair Using the Excluder Endograft

OBJECTIVE: Long-term follow-up after endovascular aneurysm repair (EVAR) is very scarce, and doubt remains regarding the durability of these procedures. We designed a retrospective cohort study to assess long-term clinical outcome and morphologic changes in patients with abdominal aortic aneurysms (AAAs) treated by EVAR using the Excluder endoprosthesis (W. L. Gore and Associates, Flagstaff, Ariz). METHODS: From 2000 to 2007, 179 patients underwent EVAR in a tertiary institution. Clinical data were retrieved from a prospective database. All patients treated with the Excluder endoprosthesis were included. Computed tomography angiography (CTA) scans were retrospectively analyzed preoperatively, at 30 days, and at the last follow-up using dedicated tridimensional reconstruction software. For patients with complications, all remaining CTAs were also analyzed. The primary end point was clinical success. Secondary end points were freedom from reintervention, sac growth, types I and III endoleak, migration, conversion to open repair, and AAA-related death or rupture. Neck dilatation, renal function, and overall survival were also analyzed. RESULTS: Included were 144 patients (88.2% men; mean age, 71.6 years). Aneurysms were ruptured in 4.9%. American Society of Anesthesiologists classification was III/IV in 61.8%. No patients were lost during a median follow-up of 5.0 years (interquartile range, 3.1-6.4; maximum, 11.2 years). Two patients died of medical complications ≤ 30 days after EVAR. The estimated primary clinical success rates at 5 and 10 years were 63.5% and 41.1%, and secondary clinical success rates were 78.3% and 58.3%, respectively. Sac growth was observed in 37 of 142 patients (26.1%). Cox regression showed type I endoleak during follow-up (hazard ratio, 3.74; P = .008), original design model (hazard ratio, 3.85; P = .001), and preoperative neck diameter (1.27 per mm increase, P = .006) were determinants of sac growth. Secondary interventions were required in 32 patients (22.5%). The estimated 10-year rate of AAA-related death or rupture was 2.1%. Overall life expectancy after AAA repair was 6.8 years. CONCLUSIONS: EVAR using the Excluder endoprosthesis provides a safe and lasting treatment for AAA, despite the need for maintained surveillance and secondary interventions. At up to 11 years, the risk of AAA-related death or postimplantation rupture is remarkably low. The incidences of postimplantation sac growth and secondary intervention were greatly reduced after the introduction of the low-permeability design in 2004.

Five Cases of Atypical Rickettsial Infections

Background: Rickettsia conorii is the most frequent species of RickettsiaI causing disease in Portugal. In general the disease manifests itself by fever, exanthema, headaches and the presence of an eschar. However atypical forms can be present and physicians should be aware. Aims: Analyse the atypical presentation of rickettsiosis. Material and Methods: Children admitted at the CHLC Hospital from 2000 to 2010 with atypical presentation of rickettsiosis. Clinical diagnosis was confirmed by serology and molecular techniques (PCR). Results: Five cases of children with a median age of 2 years, 1 of which female, were admitted between June and August. The diagnoses were: myositis (1), synovitis (1), cholecystitis (1), orchiepididymitis (1) and meningitis (1). Myositis developped with functional disability, CPK 9600 U/L, lower limbs’ edema, hypoalbuminemia (1,6 g/dL) and arterial hypertension. Synovitis developped with functional disability, synovial fluid increase and CRP 16,2 mg/dL. The child with cholecystitis had abdominal pain, intraabdominal fluid increase, leukopenia (1900/μL), thrombocytopenia (75000/μL) and CRP 15,3 mg/dL. Orchiepididymitis developped with testicle’s inflammatory signs, leukopenia (2900/μL), thrombocytopenia (90000/μL) and CRP 14,45 mg/dL. The patient with meningitis, who had pleocytosis (320 cells/μL), hyperproteinorrachia (284 mg/dL), hypoglicorrachia (36 mg/dL), presented only with fever and headaches. The tache noire and the classical triad were present in 3/5 cases. The clinical course was favourable in all cases. Antibodies against Rickettsia of spotted fever group were detected in 3/5 cases. In one patient Rickettsia conorii Malish strain was identified by PCR and sequencing. Conclusions: Rickettsial infection may present itself unusually. In a country of high prevalence, especially during summer months and in the presence of an inoculation eschar, it is of the uttermost importance to study the atypical presentations for a possible rickettsial infection.

Velhos e Novos Anticoagulantes Orais. Perspectiva Farmacológica

A terapêutica anticoagulante oral está em mudança. A varfarina e o acenocumarol constituem ainda o padrão anticoagulante oral em muitos contextos clínicos. Mas, face às limitações dos antivitamínicos K, resultantes do lento início de ação farmacológica, da janela terapêutica estreita, do metabolismo variável dependente do citocromo P450, das múltiplas interações com fármacos e com alimentos e do risco potencial de complicações hemorrágicas, nos últimos anos tem-se procurado afirmar, na terapêutica anticoagulante oral, novos grupos farmacológicos capazes de superar estes problemas. Atualmente, a investigação farmacológica centra-se no desenvolvimento de novas moléculas, não peptídicas, inibidoras de instantes fulcrais do sistema de coagulação (trombina e fator Xa), com um padrão farmacodinâmico e farmacocinético previsível e consistente, administradas por via oral. Destes compostos, três(dabigatrano, rivaroxabano e apixabano) têm já indicações terapêuticas definidas (ou a definir), assentes em largos estudos de fase III de intervenção, enquanto muitos outros compostos estão em fases menos avançadas do seu desenvolvimento. Nesta revisão, sumariamos e discutimos a farmacologia da varfarina/acenocumarol e dos novos inibidores diretos da trombina e do fator Xa, objetivando semelhanças e assinalando diferenças que ajudam a fundamentar as nossas opções terapêuticas.

Perspectivas do Estudo da Diabetes Ocular numa Consulta Pediátrica

A duração da diabetes mellitus é um factor de risco no aparecimento de complicações oculares. A prevalência da retinopatia diabética é praticamente nula antes dos 10 anos de idade, independentemente da duração da doença, atingindo 95% após 20-30 anos de evolução. Na Consulta de Oftalmologia Pediátrica do Hospital D. Estefânia avaliámos retrospectivamente 62 crianças com diagnóstico de diabetes meliitus tipo I, no intervalo d etempo compreendido entre 1 de Janeiro de 1999 e 31 de Junho de 2000. As idades oscilavam entre os 3 e os 17 anos(média 11,8 anos), tendo sido 29 casos do sexo feminino e 33 do sexo masculino. O período de evolução da doença variava entre 6 meses e 16 anos (média 6,2 anos). Na população estudada constatámos uma incidência de 6,4% de lesões oculares. Vericámos 1 caso (1,6%) de retinopatia de fundo, numa adolescente de 17 anos de idade e 11 anos de evoluão da doença, e 3 casos (4,8%) de catarata bilateral. Na diabetes juvenil a probabilidade de ocorrência de lesões oculares precoces é baixa. Exceptuando a catarata que pode ocorrer com alguma precocidade, a retinopatia clínica é raramente demosntrável antes do inicio da puberdade.