The dilution evaluation as a corrective measure for interference in the white blood cell scattergram in Beckman Coulter DxH 900.

BACKGROUND: The Beckman Coulter DxH 900 is a haematological analyser capable of counting and sizing blood cells, and obtaining a complete blood cell count (CBC). This analyses different parameters of red blood cells (RBC), platelets and white blood cells/leukocytes. Some automated CBC counters present limitations due to specimen characteristics, abnormal cells or both factors. In the presence of abnormalities, the DxH 900 has a flagging system, warning the laboratory technician that something needs to be verified. In the present work, we evaluated samples from oncologic patients, presenting a population erroneously perceived as being lymphocytes. The most common explanations for this situation are RBC resistant to lysis or serum hyperbilirubinaemia. METHODS: In an attempt to solve and understand what the cause of this problem might be, we diluted our samples (1:3) and analysed the serum total bilirubin. To identify cells' abnormalities, the samples were also analysed by manual DLC counts. During the study, we also checked the different flags presented by the equipment. RESULTS: The results evidenced that the major interference was due to RBC lysis resistance, corresponding to 94.7% of the cases, while hyperbilirubinaemia was only present in 73.4%. Besides, we determined that some samples with normal bilirubin levels also presented interference, suggesting that hyperbilirubinaemia was not the main cause of the error. The most recurrent flag observed was "High event rate". CONCLUSION: The dilution solved all of the observed interferences. The results between diluted and manual counts showed a strong correlation, leading us to introduce dilution in our laboratory routine.

A rare acute thoracic manifestation of a chronic digestive disease: pancreaticopleural fistula.

Pancreaticopleural fistula is a rare complication of chronic pancreatitis. The diagnosis requires a high suspicion degree and its management frequently involves a multidisciplinary approach between gastroenterology and surgery.

Hepatocellular carcinoma presenting with Budd-Chiari syndrome, right atrial thrombus and pulmonary emboli.

A 47-year-old patient presented with a two-week history of right upper quadrant pain, abdominal distention and new onset of shortness of breath. He had a history of intravenous drug abuse, no alcohol consumption and denied any known liver disease. On physical examination, he was tachypneic and had dullness in the flanks. His blood analysis at admission was as follows: hemoglobin, 12.9 g/dL; leukocyte count, 6,800/uL; platelet count, 63,000/uL; INR, 2.1; serum creatinine, 1.27 mg/dL; liver biochemistry tests were notable for marginal derangement, HBsAg was negative, anti-HCV was positive, HCV RNA was 367,498 IU/ml and alpha-fetoprotein was 992 mg/dL. Abdominal ultrasound showed a right liver lobe mass (13 cm in diameter) with inferior vena cava (IVC) thrombosis and mild peri-hepatic ascites. A 2D echocardiogram showed a presumed right atrial tumor thrombus. A multiphasic contrast-enhanced abdominal tomography (CT) confirmed a hepatocellular carcinoma (HCC) with IVC obstruction and extensive tumoral thrombus to the right atrium (14 cm long).

Serratia marcescens Endocarditis: A Case Report and Literature Review.

Serratia marcescens is a rarely implicated agent in endocarditis. We describe a case of a patient that underwent aortic and mitral valve replacement for Streptococcus agalactiae endocarditis. Four months later, he was readmitted with an ischemic stroke and fever. Physical examination and repetitive transthoracic echocardiogram were unremarkable. The initial blood cultures were negative. Due to sustained fever, vancomycin, gentamicin and piperacillin-tazobactam were initiated. On subsequent blood cultures, Serratia marcescens was isolated and antibiotics switched to ertapenem and gentamicin. In addition to cerebral emboli, a splenic embolus was found. The PET/CT revealed an abnormal hypercaptation in the mitral bioprosthesis. The patient was treated for six weeks. There are no current specific recommendations regarding the treatment of Serratia marcescens endocarditis. It is widely accepted that treatment should be prolonged and include a combination of antimicrobial agents. Morbidity and mortality are high, particularly when there's the need for surgical replacement. In this case, however, the patient ended-up only requiring medical treatment due to the favourable response.

A Serratia marcescens é um agente raro de endocardite. Descrevemos o caso de um doente submetido a substituição das válvulas aórtica e mitral por endocardite causada por Streptococcus agalactiae. Quatro meses após, é readmitido por evento cerebral isquémico e febre. Ao exame objetivo não evidenciava alterações e os ecocardiogramas transtorácicos eram normais. As hemoculturas colhidas à admissão foram estéreis. O doente manteve-se febril, iniciando-se empiricamente vancomicina, gentamicina e piperacilina-tazobactam. Após isolamento de Serratia marcescens em hemoculturas subsequentes, a antibioterapia foi ajustada para ertapenem e gentamicina. Para além de um êmbolo cerebral, foi encontrada embolia esplénica e hipercaptação anormal na prótese mitral biológica em PET. Foi efetuado tratamento durante seis semanas. Não existem recomendações específicas sobre o tratamento de endocardite por Serratia marcescens, mas deve ser prolongado e com terapêutica combinada. A morbimortalidade é elevada, sobretudo quando há necessidade de cirurgia. Neste caso, a evolução clínica favorável do doente permitiu o tratamento médico exclusivo.

A Rare Cause of Low Back Pain: A Challenging Diagnosis.

One of the rarest causes of low back pain is septic arthritis of a lumbar facet joint. We report the case of a 92-year-old diabetic woman with a history of four days of back pain, dysuria, and fever. Due to a sudden worsening of lumbar pain, she went to the emergency department. Physical exam revealed pain with pressure over the D12 vertebral apophyses and the lower-left paraspinal musculature. Laboratory data showed a normochromic normocytic anemia with a hemoglobin of 9.3 g/dL, white cell count of 14.61x10e3/µL (83.1% neutrophils), serum creatinine 1.46 mg/dL and C-reactive protein of 32.11 mg/dL. In urinalysis, nitrites and leukocyturia were identified. CT scan showed an acute D12 fracture and fat stranding at L5, with no irregularities in the discs or in other lumbar spaces. Escherichia coli was isolated in blood culture. Lumbar MRI confirmed the diagnosis of septic arthritis of an L5-S1 facet joint and L5 vertebrae osteomyelitis. The patient was successfully treated with intravenous ceftriaxone for eight weeks. As far as we know, this is the second report of septic arthritis of the facet joint caused by Escherichia coli.

Portuguese validation of the Adult Separation Anxiety-Questionnaire (ASA-27).

Adult separation anxiety disorder (ASAD) is characterized by developmentally inappropriate and excessive fear or anxiety concerning separation from those to whom the individual is attached. Despite the high rates of this diagnosis among Portuguese adults, there is a lack of measures to assess it. In this study, we assessed the psychometric properties of a Portuguese adaptation of the Adult Separation Anxiety questionnaire (ASA-27) on a sample of 267 adults (72.7% women) aged 18-80 years (M = 40.5, SD = 13.1). Factor structure, internal consistency, and convergence validity were examined. This study confirmed the single-factor structure of the Portuguese version of ASA-27. Consistency was high for the total sample (ω = .92) and by gender (ω = .93 and 92, men and women groups, respectively). The scale was positively related to the Portuguese version of State-Trait Anxiety Inventory (STAI) (r = .57, p< .001, for both State and trait anxiety scales) and Composite Codependency Scale total score (r = .29, p< .001). In addition, the ASA-27 total score showed incremental validity in the explanation of anxiety measured by STAI. In conclusion, results show that the Portuguese version of the ASA-27 is a reliable and valid measure of ASAD.

Mandibular distraction in a tracheostomized patient with Pierre-Robin sequence.

The triad of glossoptosis, micrognathia, and cleft palate characterizes the Pierre Robin sequence (PRS). Complications can be severe and the clinical manifestations are very heterogeneous, but airway obstruction is almost present and may be life threatening. To avoid tracheostomy or allow for early decannulation in severely affected children, external or bioresorbable internal distraction devices can be applied to repair mandibular deficiencies. We report a case of a children submitted to bilateral mandibular distraction with external nonresorbable devices. After optimal lengthening of mandibles, laryngotracheoplasty was performed to close the tracheostomy with improvement of respiratory pattern and quality of life this child. Bioresorbable internal distraction devices offers some advantages, but complications include device failure to support resistance. Distraction osteogenesis has become a safe, less-invasive procedure with a negligible risk of infection making it effective to substitute tracheostomy as first choice in treatment of patients with Pierre Robin Sequence and life threatening airway obstruction.

Reconstruction of middle third defects of the face with the temporal flap.

The temporal flap is of great interest in head and neck reconstruction when a skin graft or a local flap cannot be used. It has shown important results on the facial reanimation and in oncological surgery. We describe our experience with the pedicled flap in reconstruction of the middle third face defects in 8 oncologic patients. This flap allows the covering of bone and noble structures such as the periorbital, auricular, frontal, or parotid gland areas. Few major or minor complications were seen and good aesthetic results were achieved. It also can be done with or without adjuvant radiotherapy. The patient prognosis however, depends on the stage of the tumor.

The use of forearm free fillet flap in traumatic upper extremity amputations.

BACKGROUND: Complete traumatic upper extremity avulsions are an infrequent but devastating injury. These injuries are usually the result of massive blunt trauma to the upper limb. Intact issue from amputated or nonsalvageable limbs may be transferred for reconstruction of complex defects resulting from trauma when the indications for replantation are not met. This strategy allows preservation of stump length or coverage of exposed joints, and provides free flap harvest for reconstruction without additional donor-site morbidity. METHODS: A retrospective review at São João Hospital was performed on seven patients who had undergone immediate reconstruction with forearm free fillet flaps between 1992 and 2007. RESULTS: There were six men and one woman, with patient age ranging from 17 to 74 years (mean, 41 years). Amputation sites were at the humeral neck (n = 1), at the humeral shaft (n = 5), and below the elbow (n = 1). The area of the forearm free fillet flap skin paddle was 352.14 +/- 145.48 cm (mean +/- SD). The two major complications were the flap loss and the patient death on postoperative day 3 in other case. The postoperative course in the remaining five cases was uneventful with good healing of the wounds. Minor complications included two small residual defects treated by split-thickness skin grafting and one wound infection requiring drainage and revision. CONCLUSIONS: The forearm free fillet flap harvested from the amputated limb provides reliable and robust tissue for reconstruction of large defects of the residual limb without additional donor-site morbidity. Microsurgical free fillet flap transfer to amputation sites is valuable for achieving wound closure, improving stump durability, and maximizing function via preservation of length.

[Fournier's gangrene: from urological emergency to plastic surgery]. / Gangrena de Fournier: de urgencia urológica hasta el departamento de cirugía plástica.

Fournier's gangrene (FG) is a rare and potentially fatal infectious disease characterised by necrotic fasciitis of the perineum and abdominal wall in addition to the scrotum and penis in men and the vulva in women. Skin loss can be very damaging and difficult to repair. This condition must be treated aggressively. Several techniques are used to reconstruct lost tissue: skin grafts, transposition of the testicles and spermatic cords to a subcutaneous pocket in the upper thigh, scrotal musculocutaneous flaps, fasciocutaneous flaps and several other types of pediculated myocutaneous flaps are all employed. The supero-medial thigh skin flap is a likely arterial flap and has been shown to be an effective method for reconstructing large scrotal defects.

A Rare Presentation of Breast Cancer.

Breast cancer is the most common tumour in women. Only 5-15% of breast cancer patients have distant metastases at diagnosis. Here we describe the case of a previously healthy 43-year-old woman with nausea, postprandial epigastric pain, and constipation as the first clinical presentation of breast cancer. Total colonoscopy revealed colon metastases and the immunohistochemical profile favoured primitive invasive lobular carcinoma of the breast. Gastrointestinal tract metastases are more frequent in lobular histology of breast cancer. Stomach and small intestine are the most common metastatic sites, while colon metastases are a rare occurrence.

Outcomes of Single-Operator Cholangioscopy-Guided Lithotripsy in Patients with Difficult Biliary and Pancreatic Stones.

BACKGROUND AND AIMS: Endoscopic retrograde cholangiopancreatography is the preferred strategy for the management of biliary and pancreatic duct stones. However, difficult stones occur, and electrohydraulic (EHL) and laser lithotripsy (LL) have emerged as treatment modalities for ductal clearance. Recently, single-operator cholangioscopy was introduced, permitting the routine use of these techniques. We aimed to evaluate the clinical effectiveness of cholangioscopy-guided lithotripsy using LL or EHL in patients with difficult biliary or pancreatic stones. METHODS: This is a prospective clinical study - conducted at two affiliated university hospitals - of 17 consecutive patients with difficult biliary and pancreatic stones who underwent single-operator cholangioscopy-guided lithotripsy using two techniques: holmium laser lithotripsy (HL) or bipolar EHL. We analyzed complete ductal clearance as well as the impact of the location and number of stones on clinical success and evaluated the efficacy of the two techniques used for cholangioscopy-guided lithotripsy and procedural complications. RESULTS: Twelve patients (70.6%) had stones in the common bile duct/common hepatic duct, 2 patients (17.6%) had a stone in the cystic stump, and 3 patients (17.6%) had stones in the pancreas. Sixteen patients (94.1%) were successfully managed in 1 session, and 1 patient (5.9%) achieved ductal clearance after 3 sessions including EHL, LL, and mechanical lithotripsy. Eleven patients were successfully submitted to HL in 1 session using a single laser fiber. Six patients were treated with EHL: 4 patients achieved ductal clearance in 1 session with a single fiber, 1 patient obtained successful fragmentation in 1 session using two fibers, and 1 patient did not achieve ductal clearance after using two fibers and was successfully treated with a single laser fiber in a subsequent session. Complications were mild and were encountered in 6/17 patients (35.2%), including fever (n = 3), pain (n = 1), and mild pancreatitis (n = 1). Conclusions: Cholangioscopy-guided lithotripsy using LL or EHL in patients with difficult biliary or pancreatic stones is highly effective with transient and minimal complications. There is a clear need to further compare EHL and HL in order to assess their role in the success of cholangioscopy-guided lithotripsy.

INTRODUÇÃO: A CPRE é o exame preferencial para a remoção de cálculos biliares e pancreáticos. Em situações de cálculos difíceis foram propostas novas modalidades terapêuticas como a litotrícia electro-hidráulica (LEH) e a litotrícia por laser (LL). Recentemente a disponibilidade da colongioscopia de operador-único tornaram estas técnicas mais acessíveis e fáceis de realizar. Procuramos avaliar a eficácia clínica de litotrícia guiada por colangioscopia recorrendo á LEH ou á LL em doentes com cálculos biliares e pancreáticos difíceis. MÉTODOS: Estudo prospetivo, conduzido em 2 Hospitais associados á Universidade e englobando 17 doentes consecutivos com cálculos difíceis biliares e pancreáticos, estes doentes foram tratados com litotrícia guiada por colangioscopia recorrendo a LEH ou LL. Analisamos a limpeza completa dos ductos, bem como o impato do número de pedras e localização no sucesso clínico, associada á avaliação das 2 técnicas de litotrícia e complicações desta abordagem terapêutica. RESULTADOS: Doze doentes (70.6%) tinham cálculos no colédoco/hepático comum, 2 doentes (17.6%) tinham um cálculo único no coto do cístico e 3 doentes (17.6%) apresentavam cálculos pancreáticos. Dezasseis (94.1%) doentes foram tratados com sucesso numa única sessão e o restante (5.9%) doente necessitou de 3 sessões incluído LEH, LL e litotrícia mecânica para obter limpeza dos ductos. Onze doentes foram tratados com LL e obtiveram sucesso clínico numa única sessão com uma fibra única de laser. Seis doentes foram tratados com LEH: 4 doentes obtiveram sucesso clínico numa única sessão com 1 fibra; 1 doente necessitou de 2 fibras para obter limpeza ductal numa sessão única. O último doente falhou a limpeza dos ductos com duas fibras de LEH e necessitou de sessão adicional com Laser (uma fibra) para obter fragmentação adequada dos cálculos. As complicações foram ligeiras em 6/17 (35.2%) doentes e incluíram febre (n = 4), dor (n = 1) e pancreatite ligeira (n = 1). Conclusões: A litotrícia guiada por colangioscopia com recurso a LEH ou LL em doentes com cálculos difíceis biliares e pancreáticos é muito eficaz e está associada a complicações transitórias e ligeiras. Existe clara necessidade de realizar estudos comparativos entre LEH e LL.

Endoscopy Timing in Patients with Acute Upper Gastrointestinal Bleeding.

BACKGROUND/AIMS: The role of very early (≤12 hours) endoscopy in nonvariceal upper gastrointestinal bleeding is controversial. We aimed to compare results of very early and early (12-24 hours) endoscopy in patients with upper gastrointestinal bleeding demonstrating low-risk versus high-risk features and nonvariceal versus variceal bleeding. METHODS: This retrospective study included patients with nonvariceal and variceal upper gastrointestinal bleeding. The primary outcome was a composite of inpatient death, rebleeding, or need for surgery or intensive care unit admission. Endoscopy timing was defined as very early and early. We performed the analysis in two subgroups: (1) high-risk vs. low-risk patients and (2) variceal vs. nonvariceal bleeding. RESULTS: A total of 102 patients were included, of whom 59.8% underwent urgent endoscopy. Patients who underwent very early endoscopy received endoscopic therapy more frequently (p=0.001), but there was no improvement in other clinical outcomes. Furthermore, patients at low risk and with nonvariceal bleeding who underwent very early endoscopy had a higher risk of the composite outcome. CONCLUSION: Very early endoscopy does not seem to be associated with improved clinical outcomes and may lead to poorer outcomes in specific populations with upper gastrointestinal bleeding. The actual benefit of very early endoscopy remains controversial and should be further clarified.

Successful treatment of severe perianal Crohn's disease with infliximab in an HIV-positive patient.

We present the case of a woman infected with the HIV type 1, controlled with highly active antiretroviral therapy. In the meantime, she developed a severe perianal disease, with complex fistulae and chronic anal fissures. After developing a severe chronic diarrhea, a total ileocolonoscopy with biopsies was performed, showing multiple ileal and segmental colonic erosions. Histology favoured a Crohn's disease diagnosis. Despite the limited experience of anti-tumour necrosis factor agents in the HIV-infected population, infliximab was started in this patient, due to her severe and symptomatic Crohn's disease, with a controlled HIV infection. No side effects were reported and her bowel movements and perianal disease improved right after induction regimen with infliximab. 1 year after starting this therapy she is in clinical and endoscopic remission. The CD4+ T-cell count remained stable, the HIV-RNA undetectable and no opportunistic infections were reported during follow-up period. Data concerning the use of anti-tumour necrosis factor drugs is limited in patients with both inflammatory bowel disease and HIV infection. Only three cases of Crohn's disease and concomitant HIV infection treated with infliximab were reported in the literature. This case report might help future decisions in patients with a similar clinical situation.

Recurrent Gastrointestinal Bleeding from Dieulafoy's Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association.

Von Willebrand disease (vWD) is the most prevalent hereditary bleeding disorder, affecting 0.6-1.3% of the population. While gastrointestinal bleeding from angiodysplasia is a well-known complication of vWD, the same is not true for Dieulafoy's lesions (DLs). We report the case of a 21-year-old black male with type 1 vWD and 2 previous hospital admissions for severe anemia with no visible blood loss. In both episodes, DLs were identified and treated endoscopically, one in the stomach and another in the duodenum. The patient presented to the emergency department in September 2016 with dizziness, fatigue, and again no visible blood loss. He was hemodynamically stable, and laboratory workup showed a hemoglobin level of 3.4 g/dL. After transfusion of packed red blood cells, intravenous iron, and von Willebrand factor/factor VIII concentrate infusions, the patient underwent upper endoscopy and colonoscopy, which were normal. Small-bowel capsule endoscopy showed dark blood and a fresh clot in the proximal jejunum. At this site, push enteroscopy identified a pulsatile vessel with an overlying minimal mucosal defect, consistent with a DL, type 2b of the Yano-Yamamoto classification, which was successfully treated with adrenaline and 2 hemoclips. The patient remains stable after 18 months of follow-up, with a hemoglobin level of 13.2 g/dL. This is a case of recurrent severe occult gastrointestinal bleeding from multiple DL in a young patient with vWD who is otherwise healthy. Three other cases of DL bleeding in the setting of vWD have been reported in the literature, suggesting a possible association between these 2 entities.

A doença de von Willebrand é a perturbação hemorrágica hereditária mais frequente, afetando 0.6 a 1.3% da população. A hemorragia por angiectasias do tubo digestivo é uma complicação bem estabelecida desta doença. Contudo, o mesmo não é verdade para as lesões de Dieulafoy. Apresentamos o caso de um doente de 21 anos, melanodérmico, com doença de von Willebrand tipo 1 e dois internamentos prévios por anemia grave sem perdas hemáticas visíveis. Em ambos os episódios foram identificadas lesões de Dieulafoy que foram tratadas endoscopicamente, uma das quais no estômago e outra no duodeno. O doente foi admitido no serviço de urgáncia em Setembro de 2016 por quadro de tonturas e cansaço, novamente sem perdas visíveis. Apresentava-se hemodinamicamente estável e a avaliação laboratorial mostrou hemoglobina de 3.4 g/dL. Após transfusão de concentrados eritrocitários, terapáutica com ferro endovenoso e concentrados de fator de von Willebrand/fator VIII, foram realizadas endoscopia digestiva alta e colonoscopia, sem alterações. A enteroscopia por cápsula detetou a presença de sangue digerido e um coágulo fresco no jejuno proximal. A enteroscopia de pulsão identificou nessa topografia uma solução de continuidade da mucosa milimétrica sobre lesão vascular pulsátil procidente, compatível com lesão de Dieulafoy tipo 2b da Classificação de Yano-Yamamoto, que foi tratada eficazmente com adrenalina e dois hemoclips. Após 18 meses, o doente mantém-se clinicamente estável e com Hb 13.2 g/dL. Este é um caso particular de hemorragia gastrointestinal oculta recorrente por múltiplas lesões de Dieulafoy num jovem com doença de von Willebrand, sem outras patologias. Há trás casos semelhantes descritos na literatura, sugerindo uma possível associação entre estas duas entidades.