Cerolipofuscinose: Estudo Ultrastrutural de 8 Casos

Os autores apresentam as principais características clínicas e descrevem os achados ultrastruturais de 8 casos de cerolipofuscinose (CLF)dos tipo infantil tardio (5 casos) e juvenil (3 casos) cujo diagnóstico clínico foi confirmado pela observação em microscopia electrónica de microbuffycoats de linfócitos do sangue periférico, e de biópsias de músculo estriado, pele, conjuntiva palpebral e mucosa rectal. A observação ultrastrutural confirmou o predomínio de agrupamentos de perfis paralelos de membranas e figuras paracristalinas nas células dos casos do tipo juvenil, e de corpos curvilineares nas células dos casos de tipo infantil tardio. Nestes dois tipos não se verificou preferência pela observação de um determinado tecido para a confirmação ultrastrutural do diagnóstico clínico. Em dois casos foram observados, nos linfócitos do sangue periférico, feixes de tubos paralelos associados no mesmo citosoma às inclusões típicas das CLF, o que pode sugerir uma relação daquelas estruturas com a alteração metabólica destas doenças. A sensibilidade e a especificidade reveladas pelo exame ultrastrutural de linfócitos do sangue periférico, e a facilidade de execução técnica, recomendam o seu uso para a confirmação do diagnóstico clínico de cerolipofuscinose.

Incontinência Fecal. O que Fazer?

Várias são as patologias orgânicas ou funcionais que podem interferir com os mecanismos da defecação. Os AA apresentam 4 casos clínico com incontinência fecal: - 1 falsa encoprese - 2 mielomeningocelos - 1 anastomsose ileo-anal (colectomia total em D. de Behçet). Todos os casos foram avaliados manométricamente, e operados com miorrafia dos levantadores do recto, plastia dos músculos glúteos, com aproximação e sutura mediana, sem dissecção circunferencial do recto, solidarizando músculos contíguos de inervação independente. Na incontinência por anastomose ileo-anal foi préviamente feita ileostomia e reconstruída uma bosa rectal. Nos outros casos não foi feita qualquer derivação intestinal. Em 3 casos os pós-operatórios decorreram sem incidentes tendo havido uma nítida melhoria da continência. No outro caso (mielomeningocelo) verificou-se uma complicação abcesso com deiscência de sutura - o que contribuiu muito provávelmente para um resultado menos satisfatório. Depois de claramente despistados os casos de incontinência, a técnica utilizada é de simples execução, usando para a reconstrução anatómica e funcional grupos musculares vizinhos capazes de melhorar os mecanismos de continência.

Obstipação Funcional e Encopresis - Estudo Clínico e Manométrico

A obstipação é um problema frequente na idade pediátrica. Os autores propuseram-se analisar os dados clínicos e os registos da manomatria anorectal de um grupo de crianças com obstipação funcional para encontrar as características preditivas de risco de desenvolvimento de encopresis. Foram analisados os processos de 46 crianças com características clínicas de obstipação funcional e agrupadas em dois grupos. o grupo A (n=20) constítuido por crianças obstipadas mas sem encopresis e o grupo B (n=26) por crinaças com história de encopresis. Todas as crianças efecturam manometria anorectal. A duração da obstipação foi mais prolongada no grupo das crianças encopréticas. A análise dos traçados manométricos não demonstrou diferenças significativas entre os dois grupos, no que diz respeito ao tónus anal mínimo e máximo e à contracção anal voluntária. O reflexo recto anal inibidor (RRAI) esteve presente em todos os doentes, para volumes de distensão elevados, mas semelhantes. O limiar da sensibilidade à distensão foi mais elevado no grupo B. A relação entre os volumes de distensão desencadeando a sensaibilidade rectal e o RRAI foi semelhante nos dois grupos e em 50% dos casos o RRAI precedeu a sensibilidade para a distensão rectal. Os factores preditivos de aparecimento de encopresis, forma o sexo masculino e o tempo de duração da obstipação.

Cirurgia Obliterativa do Prolapso dos Órgãos Pélvicos - uma Opção ainda Válida?

Objective: To investigate the results of vaginal obliterate surgery in elderly women with pelvic organ prolapse. Design: observational retrospective study. Material and Methods: a total of 69 women with the diagnosis of pelvic prolapse were submitted to obliterative surgery in the urogynecology unit of a tertiary care hospital centre over the course of 8 years (2001 to 2008). The following data were collected from their clinical records: age, number of vaginal births, body mass index (BMI), hormone therapy, other existing diseases, type of prolapse and stage, anaesthetic risk score, duration of surgery, length of hospital stay, and short-term complications. Results: Of the 69 women studied, 31 were submitted to colpocleisis and the remaining 38 were managed by the LeFort technique. Mean age was 74.8 years with a standard deviation (sd) of 7.14 years. Average BMI was 26.2 (sd =3.76). Vaginal births were recorded in all patients. Only three patients were taking hormone therapy at the time of surgery. Sixty-three women were classified as having and anesthetic risk of II or III and 55 underwent local-regional anesthesia. Complications were reported in five cases, four of which in the first days after surgery. Nearly all were mild and resolved within the first 6 weeks. Conclusion: Complication rates appear to be low after obliterative surgery for pelvic organ prolapse in elderly women.

The Oncological and Functional Risks of the Organ Preservation Experimental Treatment Approach

We increasingly face conservative surgery for rectal cancer and even the so called ‘wait and see’ approach, as far as 10–20% patients can reach a complete pathological response at the time of surgery. But what can we say to our patients about risks? Standard surgery with mesorectal excision gives a <2% local recurrence with a post operative death rate of 2–8% (may reach 30% at 6 months in those over 85), but low AR has some deterioration in bowel function and in low cancer a permanent stoma may be required. Also a long-term impact on urinary and sexual function is possible. Distant metastasis rate seem to be identical in the standard and conservative approach. It is difficult to evaluate conservative approach because a not clear standardization of surgery for low rectal cancer. Rullier et al tried to clarify, and they found identical results for recurrence (5–9%), disease free survival (70%) at 5y for coloanal anastomosis and intersphinteric resection. Other series have found local recurrence higher than with standard approach and functional results may be worse and, in some situations, salvage therapy is compromised or has more complications. In this context, functional outcomes are very important but most studies are incomplete in measuring bowel function in the context of conservative approach. In 2005 Temple et al made a survey of 122/184 patient after sphinter preserving surgery and found a 96.9% of incomplete evacuation, 94.4% clustering, 93.2% food affecting frequency, 91.8% gas incontinence and proposed a systematic evaluation with a specific questionnaire. In which concerns ‘Wait and see’ approach for complete clinical responders, it was first advocated by Habr Gama for tumors up to 7cm, with a low locoregional failure of 4.6%, 5y overall survival 96%, 72% for disease free survival; one fifth of patients failed in the first year; a Dutch trial had identical results but others had worse recurrence rates; in other series 25% of patients could not be salvaged even with APR; 30% have subsequent metastatic disease what seems equal for ‘wait and see’ and operated patients. In a recent review Glynne Jones considers that all the evaluated ‘wait and see’ studies are heterogeneous in staging, inclusion criteria, design and follow up after chemoradiation and that there is the suggestion that patients who progress while under observation fare worse than those resected. He proposes long-term observational studies with more uniform inclusion criteria. We are now facing a moment where we may be more aggressive in early cancer and neoadjuvant treatment to be more conservative in the subsequent treatment but we need a better stratification of patients, better evaluation of results and more clear prognostic markers.

Hiperplasia Benigna da Próstata

Os autores fazem a revisão da clínica, do registo da sintomatologia e impacto na qualidade de vida, dos meios auxiliares ao diagnóstico e das atitudes terapêuticas da Hiperplasia benigna da Próstata. Fundamentados nestes dados propõem Orientações Terapêuticas e Recomendações, para quando e como enviar os doentes portadores desta patologia a Urologia. Assim, segundo os critérios definidos neste trabalho, devem ser enviados à Urologia os doentes com sintomatologia importante ou grave, idade inferior a 50 anos, antecedentes de diabetes ou alterações neurológicas, micção francamente alterada, globo vesical, toque rectal com palpação prostática suspeita de neoplasia, hematúria, infecção urinária, insuficiência renal, ou PSA> 4 nglml para idade inferior a 70 anos.

Serratia marcescens bacteremia associated with schistosomiasis mansoni

The case of a 21 -year-old man coming from rural Paraíba, northeastern Brazil, with schistosomiasis mansoni associated with Serratia marcescens bacteremia, is reported. His main complaints on admission were fever, diaphoresis and chills for ten days, and diarrhoea that lasted for four days. On physical examination he hadjaundice and hepatosplenomegaly. Diagnosis of S. marcescens bacteremia was made by isolation of the bacterium in blood culture, and schistosomiasis was diagnosed by rectal and liver biopsies. This is the first time that the association of S. marcescens bacteremia and schistosomiasis mansoni is recognized. Although our case does not fit into the classic definition of prolonged bacteremia associated with schistosomiasis, it can be considered as a mild form of this association. With the improvement of medical assistance and laboratory facilities, early diagnosis of this association will be made more frequently, cases with short duration will be diagnosed few days after the start of the symptoms, and classic prolonged cases will become rarer.

Antischistosomal activity of acridanone- hydrazones in Cebus monkeys experimentally infected with the SJ strain of Schistosoma mansoni

In this study, four compounds were utilized at the dose of 12.5mg/kg body weight, p.o., to treat Cebus monkeys experimentally infected with about 200 cercariae of Schistosoma mansoni (SJ strain), via transcutaneous route. The oograms performed with rectal snips, as well as stool examinations carried out periodically, showed no viable eggs of the parasite, from day 29 to 226post-treatment. The perfusion undertaken after killing the animals showed absence of worms in the treated monkeys, whereas 83 worms were recovered from the control, thus corroborating the results obtained by means of oograms and coproscopy. These results confirm the efficacy of 9-acridanone- hydrazones previously tested against the LE strain of S. mansoni. The low curative dose and apparent absence of toxicity render these dmgs an important therapeutic reserve, taking into consideration the reports on the resistance of S. mansoni to the modern drugs oxamniquine and praziquantel.

A longitudinal study on enteropathogenic infections of livestock in Trinidad

A longitudinal study was conducted on selected livestock farms to determine the prevalence of enteropathogens in diarrhoeic and non-diarrhoeic animals. The enteropathogens assayed from faecal samples and rectal swabs were bacteria (Escherichia coli, Campylobacter spp. Salmonella spp. and Yersinia enterocolitica), parasites (coccidia, gastrointestinal nematodes and Cryptosporidium spp.) and viruses (group A rotavirus and parvovirus). The prevalence of the enteropathogens in various animal species was related to age and month of the year. Generally, younger animals presented a higher prevalence of infection by enteropathogens than older animals while most infections occurred between the months of January and April.

Pyoderma gangrenosum and ulcerative colitis in the tropics

Pyoderma gangrenosum is a rare inflammatory skin condition, characterized by progressive and recurrent skin ulceration. There may be rapidly enlarging, painful ulcers with undermined edges and a necrotic, hemorrhagic base. Disorders classically associated with pyoderma gangrenosum include rheumatoid arthritis, inflammatory bowel disease, paraproteinemia and myeloproliferative disorders. There have been some reports of the occurrence of pyoderma gangrenosum in Africa, and in Nigeria, but only one specifically reported pyoderma gangrenosum in association with ulcerative colitis. We report on a 45-year-old man who presented with pyoderma gangrenosum associated with ulcerative colitis; the second report in Nigeria. The skin lesions were managed with daily honey wound dressings. Oral dapsone and prednisolone were started. The frequency of the bloody diarrhea decreased, and was completely resolved by the second week after admission. The ulcers also showed accelerated healing. The goal of therapy is directed towards the associated systemic disorder, if present.

Outbreak of neonatal infection by an endemic clone of Serratia marcescens

INTRODUCTION: The outbreak occurred between February and June 2006 and included identification of the cases, analysis of medical records, cultures from environmental sources, resistance analyses and genotyping profile of Serratia marcescens. METHODS: The cultures were composed of 13 blood isolates, 17 rectal and hand swabs and air sampling. RESULTS: The data obtained by pulsed-field gel electrophoresis exhibited three strains that contaminated 24 patients. Systemic infection was the most common in neonates with lower weight, long periods of hospitalization, premature delivery and the use of mechanical ventilation. CONCLUSIONS: This investigation revealed the multifactorial nature of the outbreak. An endemic clone of S. marcescens was detected.

Anal polyp caused by Schistosoma mansoni

We describe a schistosomal polyp in the anus of a 24-year-old patient, born in Viçosa, State of Minas Gerais, and living in Belo Horizonte, State of Minas Gerais. From 8 to 13 years of age, he swam in the rivers that bathe Viçosa. The histopathological examination has shown a lesion, lined by a keratinized squamous epithelium, ulcerated, with granulomas, centered or not by Schistosoma mansoni egg, laid, in loco, by the female present in the vascular lumen of a vein of the hemorrhoidal plexus. There was also a diffuse, nonspecific inflammation in the dermis. The patient was treated with praziquantel. Four months after the treatment, sigmoidoscopy showed a normal rectal mucosa, and negative oogram and stool tests. Ultrasound of abdomen was normal.

Conservative therapies for hemorrhagic radiation proctitis: a review

Chronic radiation proctitis represents a challenging condition seen with increased frequency due to the common use of radiation for treatment of pelvic cancer. Hemorrhagic radiation proctitis represents the most feared complication of chronic radiation proctitis. There is no consensus for the management of this condition despite the great number of clinical approaches and techniques that have been employed. Rectal resection represents an available option although associated with high morbidity and risk of permanent colostomy. The effectiveness of nonoperative approaches remains far from desirable, and hemorrhagic recurrence represents a major drawback that leads to a need for consecutive therapeutic sessions and combination of techniques. We conducted a critical review of published reports regarding conservative management of hemorrhagic chronic radiation proctitis. Although prospective randomized trials about hemorrhagic radiation proctitis are still lacking, there is enough evidence to conclude that topical formalin therapy and an endoscopic approach delivering an argon plasma coagulation represent available options associated with elevated effectiveness for interruption of rectal bleeding in patients with chronic radiation proctitis.

Anorectal leiomyomas: report of two cases with different anatomical patterns and literature review

Gastrointestinal mesenchymal tumors comprise a rare group of gastrointestinal tract wall tumors that have long been a source of confusion and controversy, especially in terms of pathological classification, preoperative diagnosis, management strategies, and prognosis. This report describes the clinical manifestations and management of 2 rectal leiomyomas and reviews the pertinent literature. Case 1: A 44-year-old woman was admitted reporting a nodule in the right para-anal region for the previous 2 years. At proctological examination, a 4-cm diameter fibrous mass situated in the para-anal region that produced an arch under the smooth muscle on the right rectal wall just above the anorectal ring was noted. Computed tomography and magnetic resonance imaging of the abdomen and pelvis showed the lesion and detected no other abnormalities. Surgical treatment consisted of wide local resection of the tumor through a para-anal incision, with no attempts to perform lymphadenectomy. Case 2: A 40-year-old male patient was admitted reporting constant anal pain for 4 months. He presented a 3-cm submucosal nodule at the anterior rectal wall just above the dentate line. After 2 inconclusive preoperative biopsies, transanal resection of the tumor was performed. Histological analysis of the specimen showed a benign leiomyoma. A review of the literature is presented, emphasizing some clinical and therapeutic aspects of this unusual rectal tumor.