Management of an Anticipated Difficult Airway in a Pediatric Patient With Stickler Syndrome.

Stickler syndrome (SS) is a congenital autosomal dominant condition that affects the formation of collagen. Of primary importance to the anesthesia provider is the propensity for difficulties in managing the airway due to orofacial abnormalities associated with this syndrome. The authors present a two-year-old infant with SS who required anesthetic care for a cleft palate repair. The potential anesthetic implications of this syndrome are discussed and the importance of proper planning and preparation and the usefulness of ultrasound as an airway evaluation tool are highlighted.

Anesthesia strategy for factor X deficiency coagulopathy: case report

Abstract Factor X deficiency ranks among the rarest coagulopathies and has a variable presentation spectrum. We intend to present a proposal for anesthesia protocol for individuals with the coagulopathy. The excision of an ovarian neoplasm was proposed for a 26-year-old, female, ASA II patient, with congenital Factor X deficiency. Physical examination and lab tests were normal, except for Prothrombin Time (PT) 22.1s (VR: 8-14s), International Normalized Ratio (INR) 1.99 (VR: 0.8-1.2) and Activated Partial Thromboplastin Time (aPTT) 41.4s (VR: 25-37s). We concluded that a history of bleeding should always be investigated, along with a pre-anesthetic coagulation study.

Anesthesia strategy for factor X deficiency coagulopathy: case report.

Factor X deficiency ranks among the rarest coagulopathies and has a variable presentation spectrum. We intend to present a proposal for anesthesia protocol for individuals with the coagulopathy. The excision of an ovarian neoplasm was proposed for a 26-year-old, female, ASA II patient, with congenital Factor X deficiency. Physical examination and lab tests were normal, except for Prothrombin Time (PT) 22.1s (VR: 8-14s), International Normalized Ratio (INR) 1.99 (VR: 0.8-1.2) and Activated Partial Thromboplastin Time (aPTT) 41.4s (VR: 25-37s). We concluded that a history of bleeding should always be investigated, along with a pre-anesthetic coagulation study.

Use of Peritoneal Catheters for Patients Receiving Palliative Care.

Background: Recurrent ascites is a late manifestation of several diseases, including cancer, cirrhosis, and heart failure, invariably associated with very poor prognosis. Hence, every effort must be aimed at reducing discomfort and side effects of its management. Objective: To evaluate if peritoneal catheters (PCs) are a viable alternative treatment approach in palliative patients who fail medical management of ascites. Design: Case study. Results: We report the case of a terminal patient with cirrhosis and hepatocellular carcinoma who presented refractory ascites despite serial large-volume paracentesis. A Tenckhoff percutaneous catheter was inserted, allowing for ascites' control and with no complications noted. Conclusions: PC placement was successful in controlling the patient's symptoms and ultimately improved comfort and well-being during the final stage of his life. This option should be assessed in selected patients to elevate palliative standards of care.

Hemodialysis Catheter-Related Thrombi: A Challenging Patient.

Patients requiring renal replacement therapy remain a significant burden on the healthcare system. A substantial amount of hospitalization costs for these patients are related to vascular complications, especially catheter-related thrombosis, which is associated with a significant increase in morbidity and mortality. We report the case of a male patient with multiple myeloma (MM) and dialysis-dependent renal failure due to light-chain cast nephropathy, who presented recurrent early catheter dysfunction. A large thrombus was detected, extending from the superior vena cava (SVC) to the right atrium (RA) and later at the inferior vena cava (IVC), ultimately leading to exhaustion of vascular capital. To this date, there is limited evidence regarding the best approach to catheter-related thrombosis, which frequently leads to treatment dilemmas in clinical practice and demands careful evaluation and individualized decisions. In patients with a highly thrombotic profile, peritoneal dialysis may be considered earlier to prevent further vascular capital damage.

An unexpected cause of residual renal function loss.

The residual renal function (RRF) in a peritoneal dialysis (PD) patient is clinically important because it contributes to dialytic adequacy, quality of life and mortality. We present the case of a patient in PD with a marked decrease in RRF. Even after the increase of dialysis, the patient maintained asthenia and anorexia, was prostrate and showed no improvement analytically. The study revealed hypothyroidism, iatrogenic due to the use of amiodarone. After suspension of the drug and replacement with levothyroxine, there was a normalization of thyroid function and recovery of RRF to baseline values. A thyroid dysfunction is associated with several changes in renal function, in most cases reversible after obtaining euthyroid state. The association between thyroid dysfunction and loss of RRF continues to be under-recognized. We should consider monitoring thyroid function annually as routine in this group of patients.

Outcomes of acute kidney injury in a nephrology ward.

Acute kidney injury (AKI) is a global problem which predicts immediate and long-term adverse outcomes. We evaluated the AKI progression to end-stage renal disease, as well as the mortality associated with AKI and the in-hospital readmission rate because of a cardiovascular event in AKI patients admitted in a nephrology ward. A 5-year retrospective study was set in a nephrology department, with a follow-up period of up to 8 years. In a total of 191 patients, mean age was 73.83 ± 12.49 years, and 137 (71.7%) patients had history of chronic kidney disease. One hundred and twenty-four (65%) patients needed RRT and two (1%) needed surgery. Upon discharge, 107 (56%) patients had recovered the renal function, 41 (21.6%) patients had partial recovery, 25 (13%) patients were RRT dependent, 16 (8.4%) died, and two (1%) patients had outcomes unknown to us, because they were transferred to other hospitals. The median survival time free of RRT was 74 months. The median survival time of the followed patients was 34 months (95% CI 23.3-44.7). The mortality rate in the follow-up period in this sample was 18 deaths/100 patients-years, and the incidence of a composite cardiovascular endpoint of heart failure, acute coronary syndrome, and stroke was 6 events/100 patients-years. The mortality rate in the follow-up period was higher than usually described for patients outside intensive care unit, probably because our patients were old and had many comorbidities.

Tradução, adaptação e validação do conteúdo da secçãoi da escala: ôassessment of peer relationsõ para o idioma português / Translation, adaptation and content validation of section i of the scale ôassessment of peer relationsõ into the portuguese language

Objetivo: realizar a adaptação e validação do conteúdo da secção I da escala ôAssessment of PeerRelationsõ û Avaliação das Relações com Pares para o idioma Português. Método: a secção I foi traduzidae retro traduzida por tradutores experientes. A versão de consenso resultante desta tradução foi utilizadaem dois estudos piloto que indicaram a necessidade de melhoramentos linguísticos. Realizadas asalterações necessárias, reuniu-se um painel com oito peritos û investigadores na área da intervençãoprecoce e das interações sociais û que procedeu à discussão aprofundada de cada um dos itens secção I.Resultados: todo o processo inerente a este estudo revelou-se de extrema importância para a exploraçãoaprofundada da secção I e para a realização de todas as alterações consideradas necessárias para quea escala possa ser funcional e aplicável na Língua e Cultura Portuguesas. Conclusões: o processo deinvestigação permitiu atingir o objetivo delineado e, assim, foi possível adaptar e validar, para o idiomaportuguês, o conteúdo da secção I da escala Assessment of Peer Relations û Avaliação das Relações comPares.(AU)

Translation, adaptation and content validation of section i of the scale "assessment of peer relations" into the portuguese language: "assessment of peer relations"para o idioma português / Tradução, adaptação e validação do conteúdo da secção i da escala

OBJECTIVE: adaptation and content validation of Section I of the scale "Assessment of Peer Relations" to the Portuguese language. METHODS: section I was translated and back translated by experienced translators. The version of consensus was used in two pilot studies that indicated the necessity of linguistic improvements. After realizing these modifications, it was possible to gather an expert panel - composed by 8 researchers in early intervention and social interaction - who thoroughly discussed each item of section I. RESULTS: this research process was essential to deeply explore section I allowing, consequently, to perceive which modifications should be done in order to operationalize its use in Portuguese's language and culture. CONCLUSIONS: the objective of this research was achieved and, consequently, it was possible to do the adaptation and the content validation to the Portuguese language concerning Section I of the scale "Assessment of Peer Relations".(AU)

OBJETIVO: realizar a adaptação e validação do conteúdo da secção I da escala "Assessment of Peer Relations" - Avaliação das Relações com Pares para o idioma Português. MÉTODO: a secção I foi traduzida e retro traduzida por tradutores experientes. A versão de consenso resultante desta tradução foi utilizada em dois estudos piloto que indicaram a necessidade de melhoramentos linguísticos. Realizadas as alterações necessárias, reuniu-se um painel com oito peritos - investigadores na área da intervenção precoce e das interações sociais - que procedeu à discussão aprofundada de cada um dos itens secção I. RESULTADOS: todo o processo inerente a este estudo revelou-se de extrema importância para a exploração aprofundada da secção I e para a realização de todas as alterações consideradas necessárias para que a escala possa ser funcional e aplicável na Língua e Cultura Portuguesas. CONCLUSÕES: o processo de investigação permitiu atingir o objetivo delineado e, assim, foi possível adaptar e validar, para o idioma português, o conteúdo da secção I da escala Assessment of Peer Relations - Avaliação das Relações com Pares.(AU)

Tradução, adaptação e validação do conteúdo da secçãoi da escala: ôassessment of peer relationsõ para o idioma português / Translation, adaptation and content validation of section i of the scale ôassessment of peer relationsõ into the portuguese language

Objetivo: realizar a adaptação e validação do conteúdo da secção I da escala ôAssessment of PeerRelationsõ û Avaliação das Relações com Pares para o idioma Português. Método: a secção I foi traduzidae retro traduzida por tradutores experientes. A versão de consenso resultante desta tradução foi utilizadaem dois estudos piloto que indicaram a necessidade de melhoramentos linguísticos. Realizadas asalterações necessárias, reuniu-se um painel com oito peritos û investigadores na área da intervençãoprecoce e das interações sociais û que procedeu à discussão aprofundada de cada um dos itens secção I.Resultados: todo o processo inerente a este estudo revelou-se de extrema importância para a exploraçãoaprofundada da secção I e para a realização de todas as alterações consideradas necessárias para quea escala possa ser funcional e aplicável na Língua e Cultura Portuguesas. Conclusões: o processo deinvestigação permitiu atingir o objetivo delineado e, assim, foi possível adaptar e validar, para o idiomaportuguês, o conteúdo da secção I da escala Assessment of Peer Relations û Avaliação das Relações comPares.