Gravidez e Diálise

A gravidez nas mulheres insuficientes renais crónicas tratadas com diálise é pouco frequente, mas tem aumentado nos últimos anos e o seu prognóstico tem melhorado. Uma gravidez bem-sucedida é mais frequente nas mulheres com função residual, sobretudo se a concepção ocorre antes de iniciar diálise ou se estão em diálise há menos de 1 ano. No entanto, numa doente com insuficiência renal moderada a grave, há risco de deterioração acelerada da função renal. Os resultados são semelhantes com ambas as modalidades de substituição da função renal, hemodiálise ou diálise peritoneal. A prescrição dialítica deve visar uma ureia inferior a 100mg/dl. As complicações maternas, que incluem hipertensão, anemia, perturbações do metabolismo fósfo-cálcico e deficits nutricionais, devem ser prevenidas e/ou tratadas atempadamente. A prematuridade e as perdas fetais são frequentes. A sobrevivência do recém-nascido e o bem-estar da mãe impõem a cooperação estreita entre médicos, enfermeiros e nutricionistas das áreas da obstetrícia, nefrologia e neonatologia.

Mesotelioma Peritoneal Maligno. Dificuldades Diagnósticas e Terapêuticas

O Mesotelioma Peritoneal Maligno (MPM) é um tumor raro, de apresentação clínica inespecífica, colocando dificuldades diagnósticas particularmente na diferenciação com carcinomatose peritoneal. O diagnóstico tardio e a ineficácia da terapêutica convencional – cirurgia, radioterapia, quimioterapia – conferem-lhe um mau prognóstico com sobrevida média de 6-12 meses. Os autores descrevem um caso de MPM diagnosticado durante investigação de ascite inaugural, através de biópsia peritoneal laparoscópica. Submetido a quimioterapia sistémica, o doente encontra-se em remissão parcial aos 42 meses.

Insuficiência Renal Crónica – Casuística de 16 Anos (1986-2001) da Unidade de Nefrologia do Hospital de Dona Estefânia

Entre Janeiro 1986 e Dezembro de 2001 foram seguidos na nossa Unidade 100 doentes com Insuficiência Renal Crónica com idade igual ou inferior a 15 anos (M:F = 54:46; idade ≥ 0 ≤15 anos). A idade de detecção da doença foi inferior aos dois anos em 35% dos casos. Cinquenta e nove doentes (59%) atingiram a fase de Insuficiência Renal Terminal no decurso destes 16 anos, tendo sido transplantados 41 (69,5%), encontrando-se no final do estudo 12 (20,3%) doentes em hemodiálise, três (5,1%) em diálise peritoneal e um em preparação para indução de diálise peritoneal. Registaram-se cinco óbitos, dois dos quais ocorreram após transplantação renal. Durante o período em estudo a hemodiálise foi a primeira forma de terapêutica de substituição da função renal em 33 casos (55,9%) dos doentes que atingiram a insuficiência renal terminal. Contudo a diálise peritoneal, instituida em 24 doentes (42,1%) foi a primeira escolha em 10 ( 100%) das crianças com menos de seis anos , submetidas a terapêutica de substituição da função renal. Dos 41 doentes transplantados registaramse dois óbitos e oito rejeições, com necessidade de instituição de hemodiálise em dois casos, de diálise peritoneal noutro e de duas retransplantações.

Primary Malignant Peritoneal Mesothelioma Associated with Renal Cell Carcinoma. A Concise Review Based on a Clinical Case Baseada num Caso Clínico

O Mesotelioma peritoneal maligno é um tumor maligno relacionado frequentemente com exposição prolongada a fibras de amianto, de mau prognóstico, de diagnóstico geralmente tardio, face à pouca expressão clínica na fase inicial da doença. Como o mesotelioma evolui geralmente só na cavidade peritoneal, doentes seleccionados poderão ter maior sobrevivência se for possível a peritonectomia extensa e quimioterapia hipertérmica intraperitoneal intraoperatória. Os autores referem a sincronicidade ainda não descrita, de mesotelioma peritoneal maligno primário e carcinoma de Grawitz. São revistos concisamente: a clínica destes tumores, síndromes paraneoplásicos (disfunção bioquímica hepática, emagrecimento extremo); etiopatogenia da acção cancerígena das fibras de amianto; mecanismos de disseminação intraperitoneal; avaliação tomodensitométrica; importância da imunohistoquímica no diagnóstico histopatológico; estadiamento; importância do tratamento multidisciplinar destes tumores.

Enterocolite Necrosante com Perfuração e Drenagem Peritoneal no Recém-Nascido de Muito Baixo Peso: Um Caso Clínico de Evolução Favorável

A enterocolite necrosante (ECN) constitui o problema gastrointestinal mais grave e mais frequente no recém-nascido (RN) de baixo peso. A melhoria na taxa de sobrevivência tem sido atribuída ao diagnóstico mais precoce e à experiência adquirida no tratamento do recém-nascido pré-termo em estado crítico. Desde 1977 que a drenagem peritoneal como actuação prioritária nos quadros de ECN tem sido preconizada nos recém- -nascidos de peso inferior a 1500 g com perfuração intestinal, e nos de peso superior a 1500 g com instabilidade hemodinâmica. Neste artigo relata-se o caso de um recém-nascido, com 1473 g de peso e 30 semanas de idade gestacional, ECN, sinais de perfuração intestinal e de instabilidade hemodinâmica, o qual foi submetido a drenagem peritoneal com evolução favorável e sem sequelas. Na discussão faz-se referência especial, de acordo com dados de literatura, aos mecanismos que explicam os bons resultados do procedimento em cerca de 2/3 dos casos de ECN com perfuração, os quais estão relacionados com as características particulares da cicatrização nos tecidos imaturos. Em conclusão, admite-se que a drenagem peritoneal deverá constituir a forma de actuação prioritária nos casos de ECN com perfuração e instabilidade hemodinâmica em RN pré-termo de muito baixo peso.

Fibromioma Extra-Peritoneal do Ligamento Redondo. Caso Clínico

Fibromyoma of the round ligament should be considered as a possible etiology for a large preperitoneal tumor. We report a case of a 44-year-old female who was found to have a fibroleiomyoma that measured 14 cm, weighed 3599 gr and had grown in the abdominal wall between the muscle and the peritoneum, taking as its starting point the right round ligament.

O Rim Revisitado

O autor faz a recapitulação do percurso histórico que conduziu ao conhecimento moderno da fisiologia renale refere-se ao fascínio que esse conhecimento exerceu sobre os internistas no início da década de 60. Depois de realçar a importância do aparecimento da diálise no tratamento e no prognóstico da insuficiência renal, faz algumas considerações acerca do tratamento das glomerulopatias e dos mistérios que o rim ainda persiste em nos ocultar.

The Arterial Switch Operation in Europe for Transposition of the Great Arteries: a Multi-Institutional Study from the European Congenital Heart Surgeons Association

OBJECTIVES: This study analyzes the results of the arterial switch operation for transposition of the great arteries in member institutions of the European Congenital Heart Surgeons Association. METHODS: The records of 613 patients who underwent primary arterial switch operations in each of 19 participating institutions in the period from January 1998 through December 2000 were reviewed retrospectively. RESULTS: A ventricular septal defect was present in 186 (30%) patients. Coronary anatomy was type A in 69% of the patients, and aortic arch pathology was present in 20% of patients with ventricular septal defect. Rashkind septostomy was performed in 75% of the patients, and 69% received prostaglandin. There were 37 hospital deaths (operative mortality, 6%), 13 (3%) for patients with an intact ventricular septum and 24 (13%) for those with a ventricular septal defect (P < .001). In 36% delayed sternal closure was performed, 8% required peritoneal dialysis, and 2% required mechanical circulatory support. Median ventilation time was 58 hours, and intensive care and hospital stay were 6 and 14 days, respectively. Although of various preoperative risk factors the presence of a ventricular septal defect, arch pathology, and coronary anomalies were univariate predictors of operative mortality, only the presence of a ventricular septal defect approached statistical significance (P = .06) on multivariable analysis. Of various operative parameters, aortic crossclamp time and delayed sternal closure were also univariate predictors; however, only the latter was an independent statistically significant predictor of death. CONCLUSIONS: Results of the procedure in European centers are compatible with those in the literature. The presence of a ventricular septal defect is the clinically most important preoperative risk factor for operative death, approaching statistical significance on multivariable analysis.

Effects of Sevelamer Hydrochloride and Calcium Carbonate on Renal Osteodystrophy in Hemodialysis Patients

Disturbances in mineral metabolism play a central role in the development of renal bone disease. In a 54-wk, randomized, open-label study, 119 hemodialysis patients were enrolled to compare the effects of sevelamer hydrochloride and calcium carbonate on bone. Biopsy-proven adynamic bone disease was the most frequent bone abnormality at baseline (59%). Serum phosphorus, calcium, and intact parathyroid hormone were well controlled in both groups, although calcium was consistently lower and intact parathyroid hormone higher among patients who were randomly assigned to sevelamer. Compared with baseline values, there were no changes in mineralization lag time or measures of bone turnover (e.g., activation frequency) after 1 yr in either group. Osteoid thickness significantly increased in both groups, but there was no significant difference between them. Bone formation rate per bone surface, however, significantly increased from baseline only in the sevelamer group (P = 0.019). In addition, of those with abnormal microarchitecture at baseline (i.e., trabecular separation), seven of 10 in the sevelamer group normalized after 1 yr compared with zero of three in the calcium group. In summary, sevelamer resulted in no statistically significant changes in bone turnover or mineralization compared with calcium carbonate, but bone formation increased and trabecular architecture improved with sevelamer. Further studies are required to assess whether these changes affect clinical outcomes, such as rates of fracture.

Evaluation of Bone Remodeling in Hemodialysis Patients: Serum Biochemistry, Circulating Cytokines and Bone Histomorphometry

BACKGROUND: To optimize the noninvasive evaluation of bone remodeling, we evaluated, besides routine serum markers, serum levels of several cytokines involved in bone turnover. METHODS: A transiliac bone biopsy was performed in 47 hemodialysis patients. Serum levels of intact parathyroid hormone (iPTH; 1-84), total alkaline phosphatases (tAP), calcium, phosphate and aluminum (Al) were measured. Circulating levels of interleukin-6 (IL-6), IL-1 receptor antagonist (IL-1Ra) and soluble IL-6 receptor (sIL-6r) were determined using ELISA. Circulating IL-1beta, IL-6, IL-8, IL-10, IL-12p70 and tumor necrosis factor-alpha (TNF-alpha) were simultaneously quantified by flow cytometric immunoassay. RESULTS: Patients with low/normal bone formation rate (L/N-BFR) had significantly lower serum iPTH (p<0.001) and tAP (p<0.008) and significantly higher Al (p<0.025) than patients with high BFR. Serum calcium and phosphorus, however, did not differ (p=NS). An iPTH >300 pg/mL in association with tAP >120 U/L showed low sensitivity (58.8%) and low negative predictive value (44.0%) for the diagnosis of high BFR disease. An iPTH <300 pg/mL in association with normal or low tAP, <120 U/L, was associated with low sensitivity (66.7%) but high specificity (97.1%) for the diagnosis of L/N-BFR. Serum IL-1, IL-6, IL-12p70 and TNF-alpha were positively correlated with BFR, serum IL1-Ra and IL-10 with bone area, and by multiple regression analysis, tAP and IL-6 were independently predictive of BFR. CONCLUSIONS: Significant associations were found between several circulating cytokines and bone histomorphometry in dialysis patients. The usefulness of these determinations in the noninvasive evaluation of bone remodeling needs to be confirmed in larger dialysis populations.

Appendiceal Mucocele – Unexpected Diagnosis in a Gynecology Department

Appendiceal mucocele is a rare entity, occuring in < 1% among appendicectomies, with a female predominance 4/1 (F/M) and a mean age of more than 50 years. The preoperative diagnosis is difficult; in most cases, it´s an intraoperative finding. In such work, we describe the two clinical cases occurring in last 10 years in our Department. Case 1 - 56 years old, posmenopausal, referred to our Department (02/2004) because an asymptomatic right adnexal septated cystic image, 53x48mm, with hipovascularized septa and a vascularised capsule with low flow resistance (IR 0,57). CA 125 elevated (71,3 U/mL).Exploratory laparotomy: an ovary increased, with a gelatanious consistency and an appendicular enlargement. Extemporaneous examination: a pseudomixoma peritonei, associated with a mucinous appendicular and an ovary tumor. It was performed a radical surgery. The histo-pathological analysis showed a mucinous cystadenoma of the appendix with peritoneal mucinous dissemination involving the ovary. Expectant attitude since the surgery, without clinical and imaging signs of recurrence. Case 2- 62 years old posmenopausal and asymptomatic woman, with a large adnexal mass detected on routine pelvic ultrasound: heterogeneous, 94x84mm without vascularisation signs in its interior. CEA was elevated (41,47U/ml). Exploratory laparotomy (02/2010): enlarged appendix and macroscopically normal pelvic organs. An appendicectomy was performed. The histo-pathological analysis showed a 10cm mucinous cystadenoma of the appendix and signs of localized (visceral peritoneal surface) pseudomyxoma peritonei. Currently she’s clinically well, in an expectant attitude. Despite mucoceles of the appendix are rare, they should be considered in women presenting with abnormal quadrant masses.

Demographic and Clinical Characteristics of Human Immunodeficiency Virus-Infected Patients Receiving Dialysis in Portugal: a Nationwide Multicentre Survey

Background: Data on human immunodeficiency virus (HIV) infected patients receiving dialysis in Portugal is scarce. Methods: This nationwide epidemiological survey retrospectively evaluates HIV-infected patients on chronic dialysis in Portugal between 1997 and 2002. Results: Sixty-six patients were evaluated (mean age: 39.1±1.6 years, 47 men, 35 black African). Sixty-two patients started dialysis and 4 patients who were receiving dialysis had HIV seroconversion. Eighty-five percent of patients were treated in Lisbon. The annual incidence of HIV-infected patients on chronic dialysis was 0.5% in 1997 and 0.9% in 2002. Seventy-eight percent of patients were HIV-1 infected , 13% had hepatitis B and 31% hepatitis C. Sexual contact was the mode of transmission of HIV in 53% of cases. Four patients had biopsy-proved HIV-associated nephropathy. Ninety-five percent of patients were on chronic hemodialysis. Fifty percent of patients had acquired immunodeficiency syndrome. At follow-up, 12 patients died. HIV-infected CKD patient survival after starting dialysis was 80% at 3 years. Conclusion: The incidence of HIV-infected patients on chronic dialysis in Portugal has almost doubled. Widespread use of highly active antiretroviral therapy and the increasing number of black Africans from former overseas Portuguese colonies now living in Portugal are possible reasons for this large increase.