A 74-year-old man presented with recurrent syncope 3 months after definitive surgery for hypopharyngeal cancer. The patient experienced dizziness and severe hypotension on the movement of the neck and head. CT revealed disease recurrence with masses encasing the left internal carotid artery. The patient was diagnosed with vasodepressor type of tumour-induced carotid sinus syndrome (tiCSS) and was referred for palliative intensity-modulated radiotherapy (IMRT). Ten days after the commencement of IMRT (25 Gy in five fractions), the symptoms of tiCSS improved, and there was no re-exacerbation of the symptoms till the patient died 56 days after the commencement of RT. Palliative IMRT was feasible and effective for recurrent malignant tiCSS. Given the fact that palliative IMRT is minimally invasive, this option could be widely adapted for patients with such poor general condition and prognosis.
This meta-analysis synthesizes the results of 14 independent studies conducted in the U.S. (N = 6979 participants) that examined sex differences in internalized, externalized, and PTSD symptoms associated with grief during adolescence. The mean age of participants was 12.22 years (SD = 2.31) with 50% male and 50% female sex assigned at birth. While no mean-level differences were found between adolescent females and males in externalizing behaviors associated with grief (d = 0.03), on average, females reported higher levels of internalized grief responses (d = 0.18) and higher levels of PTSD symptoms (d = 0.36) than their male counterparts. Findings suggest the need for additional, more nuanced research to investigate possible sex differences in externalized behaviors relating to grief. In addition, research should examine whether tailored therapeutic and intervention measures and resources are needed for adolescents experiencing internalized grief and PTSD symptoms given sex differences in these reactions.
Background: Third-degree atrioventricular (AV) block can result in sudden cardiac death if no reliable escape rhythm is present. Here, we report a case of an 86-year-old female patient who developed a third-degree AV block leading to cardiac arrest. Surprisingly, sinus rhythm returned after 4 min of asystole, and she showed complete neurological recovery.
Case summary: Emergency services were contacted by the husband of an 86-year-old woman after she was found unconscious. Ambulance personnel diagnosed a third-degree AV block without an escape rhythm and transcutaneous pacing was started. At arrival on the emergency ward, pacing was inadequate, resulting in absence of circulation for ~10 min. After consultation with the family, the patient turned out to have signed a ‘do not resuscitate’ order. Given the impression that the considerable delay deemed favourable neurological recovery unlikely, it was decided together with the family to stop the resuscitation. Subsequently, she had an intermittent junctional escape rhythm but eventually developed a documented asystole of more than 4 min. Against all expectations, she regained sinus rhythm and fully recovered. Eventually, a pacemaker was implanted and she was discharged home without neurological sequalae of the cardiac arrest.
Discussion: Autoresuscitation, also known as the Lazarus syndrome, is the spontaneous return of circulation after cardiac arrest and is incidentally seen after failed cardiopulmonary resuscitation (CPR). Autoresuscitation in the absence of CPR is highly unusual, but could, in this case, be due to the total AV block as the cause of the cardiac arrest.
The potential association between serotonin syndrome and tapentadol is not well described in the literature. This study aimed to review the literature and identify methodological issues that could lead to inaccurately reported rates of serotonin syndrome associated with tapentadol use. A systematic review of English articles using MEDLINE, Cochrane Controlled Trials Register, and Scopus was performed. Additional studies were identified by cross-referencing article bibliographies. Original research that examined the safety of tapentadol in patients with nonconfounding indications were examined. In total, 22 studies met inclusion criteria. There were 13 randomized clinical trials, 7 open-label trials, and 2 observational studies. All studies either did not mention whether serotonergic medication use was prohibited or disallowed use. Frequently reported adverse events were nausea, diarrhea, constipation, fatigue, vomiting, and somnolence. No studies reported serotonin syndrome development. No included trials differentiated between the development of adverse events in patients taking serotonergic drugs and those who were not. This differentiation is necessary to evaluate the increased risk of adverse events in patients prescribed tapentadol concomitantly with other serotonergic medications. Therefore, the current tapentadol literature has important limitations that prevent the adequate characterization of the potential association between tapentadol and serotonin syndrome.
OBJECTIVES: Incident depression, occurring after an acute coronary syndrome (ACS) in never depressed patients, exerts a negative effect on the cardiac prognosis. Nonetheless only a few studies have evaluated the risk factor for incident depression and, particularly, no study have investigated the role of personality disorders. Therefore, the aim of this study is to verify if personality disorders represent a risk for incident depression in patients at their first ACS.
METHOD: The study sample was selected among never depressed patients who were consecutively admitted to the Coronary Intensive Care Unit, from January 2009 to March 2012, for the first ACS. The study sample included 262 patients. The presence of depressive disorder was assessed with the Primary Care Evaluation of Mental Disorders (DSM-IV criteria), whereas its severity was evaluated with the Hospital Anxiety and Depression Scale. Evaluations were collected at baseline and at 1, 2, 4, 6, 9, 12 and 24 months of follow-up. Moreover, at baseline personality disorders were investigated with the Structured Clinical Interview for DSM-IV Axis II disorders.
RESULTS: Out of 262 subjects, a depressive disorder was diagnosed in 56 patients (21%). At baseline risk factors for incident depression were being widowed, having a distress reaction and narcissistic personality traits.
CONCLUSION: Clinicians should keep in mind these characteristics when facing patients at their first ACS, given the detrimental effect of depression on cardiac prognosis. A psychological support should prevent the onset of incident depression in these patients.
AIM: Vascular Blowout Syndrome (VBOS) is a life-threatening condition secondary to direct tumor encasement or invasion in advanced stage malignancies. Endovascular management can be an alternative to surgical treatment in this fragile patient population providing a minimally invasive measure both acutely and prophylactically.
MATERIALS AND METHODS: Three patients with peripheral VBOS secondary to advanced stage malignancies underwent successful endovascular treatment. Technical success was obtained in all patients with non-significant perioperative complications.
RESULTS: Endovascular management controlled immediately life-threatening haemorrhage and enabled these high-risk patients to undergo other adjunctive therapeutic modalities.
CONCLUSION: Endovascular treatment can offer a safe and effective palliative measure of peripheral VBOS secondary to neoplastic erosion in patients with advanced-stage malignancies.
Sudden cessation of baclofen can produce a withdrawal syndrome even if it was previously orally administered. We present the case of a man who exhibited signs of baclofen withdrawal syndrome during palliative sedation. Attempts were made to induce muscle relaxation with ever-increasing doses of benzodiazepine. Ultimately, control over the withdrawal syndrome was regained by using a continuous subcutaneous infusion (CSCI) of dexmedetomidine, a highly selective a2 adrenergic agonist. Very limited published reports concerning CSCI of dexmedetomidine exist. To our knowledge, this is the first case to report its use as an adjunctive agent to treat baclofen withdrawal syndrome through the subcutaneous route in the palliative care setting.
Pena-Shokeir syndrome (PSS) type 1, also known as fetal akinesia deformation sequence, is a rare genetic syndrome that almost always results in intrauterine or early neonatal death. It is characterized by markedly decreased fetal movements, intrauterine growth restriction, joint contractures, short umbilical cord, and features of pulmonary hypoplasia. Antenatal diagnosis can be difficult. Ultrasound features are varied and may overlap with those of Trisomy 18. The poor prognosis of PSS is due to pulmonary hypoplasia, which is an important feature that distinguishes PSS from arthrogryposis multiplex congenital without pulmonary hypoplasia, which has a better prognosis. If diagnosed in the antenatal period, a late termination of pregnancy can be considered following ethical discussion (if the law allows). In most cases, a diagnosis is only made in the neonatal period. Parents of a baby affected with PSS require detailed counseling that includes information on the imprecise recurrence risks and a plan for subsequent pregnancies.
OBJECTIVES: The aim of the present study was to evaluate the association between sarcopenia, diagnosed by different muscle mass measurement techniques, with nutritional status and overall survival in patients with advanced cancer under palliative care.
AIM: To investigate the association of sarcopenia, according to distinct muscle mass measurement methods, with nutritional status and overall survival (OS).
METHODS: This observational and prospective study, including 334 patients, defined sarcopenia as reduced muscle mass and strength. Muscle mass was evaluated adopting 3 different methods, mid-upper arm muscle area (MUAMA), calf circumference (CC) and appendicular skeletal muscle mass (ASMI) described by Baumgartner (1998) and adjusted for height. Strength was defined using a handgrip dynamometer and OS was established based on a 90 days follow-up after inclusion date. Kaplan-Meier curves were conducted for survival analyzes and the association between sarcopenia and OS was evaluated by Cox regression model RESULTS: Prevalence of sarcopenia varied from 27-65% according to the method used to evaluate muscle mass. Malnutrition assessed by different parameters was significantly higher in patients with sarcopenia. Patients considered sarcopenic by MUAMA (43 versus 67 days, p<0.001), CC (44 versus 77 days, p<0.001) and ASMI (48 versus 75 days, p<0.001) had significantly lower OS compared to non-sarcopenic patients. Sarcopenia evaluated by MUAMA (HR, 1.57; 95% CI, 1.12-2.18) and CC (HR, 2.00; 95% CI, 1.45-2.76) showed a higher risk of mortality.
CONCLUSION: Sarcopenia diagnosed by MUAMA and CC could predict mortality and CC proved to be the best prognostic method for estimating OS in patients with advanced cancer in palliative care.
Background: Heterotaxy is a condition of abnormal lateralization of organs across the body's left-right axis, causing multiple congenital malformations. The anatomic manifestations of heterotaxy syndrome generally follow one of two patterns, referred to as right atrial isomerism (with two similar right atria and duplication of right-sided features of multiple organs) and left atrial isomerism (with two similar left atria and duplication of left-sided features of multiple organs). Cardiac surgical intervention for patients with heterotaxy syndrome depends on ventricular physiology and circulatory balance. For patients with single-ventricle physiology, a Fontan operation, which directs systemic venous return to the pulmonary arteries, is the definitive intervention. Prior to a Fontan operation, many patients require one or more palliative surgeries (eg, a Blalock-Taussig-Thomas shunt or bidirectional Glenn/Kawashima procedure) to prepare them for definitive correction.
Case Report: We present the case of a term female neonate who was transferred to our pediatric cardiovascular intensive care unit for management of suspected congenital cardiac disease. Echocardiography confirmed the diagnosis of heterotaxy syndrome with left atrial isomerism, an interrupted inferior vena cava with azygos continuation, and a hypoplastic left ventricle with single-ventricle physiology. At 11 months of age, she underwent a Kawashima procedure with subtotal pulmonary artery ligation. She tolerated the procedure well and is anticipated to remain stable for the near future, possibly without the need for further cardiac surgery.
Conclusion: Patients with heterotaxy syndrome have congenital malformations in several organ systems, requiring lifelong coordination of care among health providers across multiple disciplines.
Trousseau syndrome was first described by Armand Trousseau in 1865 and is characterized by hypercoagulation resulting from malignant tumors. This complication can markedly impact quality of life (QOL). This is the first report of a terminally ill patient who developed large-vessel occlusion stroke from Trousseau syndrome and underwent mechanical thrombectomy. A 75-year-old woman presented with stage IV ovarian cancer. Goals of care were transitioned to palliative care. The patient was hospitalized with vertebral compression fracture, and suddenly developed right hemiparesis and total aphasia during admission. Magnetic resonance imaging and angiography showed occlusion of segment 1 of the left middle cerebral artery. We administered tissue-plasminogen activator, but symptoms remained unimproved. We performed mechanical thrombectomy based on medical indications and with the consent of her family. Thrombectomy improved symptoms dramatically. She was able to walk and talk with her family at discharge. She eventually died of respiratory failure on postoperative day 98, but QOL remained high for those 98 days. Mechanical thrombectomy has the potential to markedly improve QOL in terminally ill patients with large-vessel occlusion associated with Trousseau syndrome.
Background: Congenital syndrome of Zika virus (CSZV) is associated with neuromotor and cognitive developmental disorders, limiting the independence and autonomy of affected children and high susceptibility to complications, so palliative care needs to be discussed and applied.
Aim: To identify factors associated with emergency visits and hospitalizations of patients with CSZV and clinical interventions performed from the perspective of palliative care.
Design: This is a cross-sectional study with bidirectional longitudinal component. Data were collected between May and October 2017 through the review of medical records and interviews with relatives of patients hospitalized.
Setting/Participants: The study was developed in a tertiary care hospital involving patients with confirmed CSZV born as of August 2015 and followed up until October 2017. Patients under investigation were excluded.
Results: 145 patients were followed up at the specialized outpatient clinic, 92 (63.5%) were consulted at least once in the emergency room, and 49% had already been hospitalized, with the main reason being neurological causes, while 24.1% had never required any emergency visit or hospitalization. No risk factors were associated with the occurrence of consultations or hospitalizations. Such events happened at an early age and were accompanied by a high number of invasive procedures and interventions. An approach in palliative care was only identified in two hospitalized patients.
Conclusions: For the patient with known severe malformations caused by congenital infection by the Zika virus with indication of palliative care, this approach could be used in order to allow life without suffering and disproportionate invasive method.
Identification of individuals with inherited predispositions to cancer, including Lynch syndrome, can help prevent cancer and cancer-related death by allowing for the uptake of specific cancer prevention and screening as well as the use of therapies directed toward the underlying neoplastic process for individuals with advanced cancer. In the 25 years since the discovery of microsatellite instability (MSI) and the first recognition of germline mismatch repair (MMR) gene variants as the etiologic basis of Lynch syndrome, there has been tremendous progress in the understanding of the spectrum of cancer risk associated with Lynch syndrome as well as in cancer prevention and risk-reduction strategies. The past few years, in particular, have brought transformative changes in the treatment of Lynch syndrome-associated cancers with immune checkpoint inhibitors. In parallel, advances in next-generation sequencing (NGS) technologies now allow rapid and scalable somatic and germline sequencing that promises to help identify Lynch syndrome in individuals who otherwise lack classic phenotypes. Last, real progress is being made to understand more sophisticated methods of precision cancer prevention, including chemotherapeutic prevention agents (e.g., aspirin) and strategies that leverage the immune system to facilitate primary cancer prevention in otherwise-healthy Lynch syndrome carriers.
Purple urine bag syndrome (PUBS) is a rare condition characterised by urine discolouration. The management of PUBS remains controversial. Four females (mean age 84.5±9.7 years) with palliative conditions (two cancer and two non-cancer cases) presenting PUBS were identified. Urine bags were changed in all cases. Urinary catheters were changed in three cases. Oral antibiotics were prescribed in two cases and used in one case. Urine discolouration was resolved in all cases. One patient (without antibiotic treatment) died on day 5 after presentation of PUBS. Three patients (one out of three cases used oral antibiotics) were clinically stable after the management of PUBS. There was no recurrence of PUBS. Caring for patients with PUBS should be based on clinical decisions, patient status and the goals of care. Palliative care teams should focus on the prevention of PUBS by shortening the duration of catheterisation and minimising modifiable risk factors for this condition.
This article presents a review of the literature on Von Hippel-Lindau (VHL) syndrome and recommendations for healthcare professionals working with VHL patients. Implications from the literature may be extrapolated to people living with hereditary cancers in general, which account for 2 or 3 in every cancers. Palliative care in VHL syndrome involves managing the distressing psychological symptoms that occur alongside the myriad and varied physical problems.
The case of Charlie Gard, an infant who was hospitalized in England due to a mitochondrial DNA depletion syndrome that led to an epileptic encephalomyopathy, was highly publicized. Though Charlie's parents lobbied for him to receive experimental nucleoside replacement therapy as a desperate effort to save him, this request was denied, and after a lengthy legal battle, he died in late July 2017. We discuss the ethical considerations and consequences of this case.
INTRODUCTION: In Denmark, first trimester screening has a very high uptake (>90%). If Down syndrome is diagnosed, termination rates are high (>95%). The aim of this study was to investigate the timing of the decision of termination of pregnancy following a Down syndrome diagnosis and the factors influencing this decision
MATERIAL AND METHODS: Semi-structured, qualitative interview study with 21 couples who had received a prenatal diagnosis of Down syndrome and decided to terminate the pregnancy. Participants were recruited from obstetric departments between February 2016 and July 2017. Data were analysed using thematic analysis
RESULTS: Five themes were identified: 'initial decision-making', 'consolidating the decision' 'reasons and concerns shaping the termination of pregnancy decision', 'the right decision is also burdensome' and, 'perceived influences in decision-making'. For most couples, an initial termination of pregnancy decision was made before or during the diagnostic process, but it was re-addressed and consolidated following the actual diagnosis. Imagining a family future with a severely affected Down syndrome child was the main factor influencing the termination of pregnancy decision. The decision was articulated as 'right' but also as existentially burdensome for some due to fear of regret and concern about ending a potential life. The termination of pregnancy decision was considered a private matter between the couple, but was refined through interactions with clinicians and social network
CONCLUSION: All couples made an initial decision prior to receiving the Down syndrome diagnosis. Knowledge of the couple's initial decision may facilitate patient-centred communication during and after the diagnostic process. Couples may benefit from counselling to deal with grief and existential concerns.
Myhre syndrome is a rare connective tissue disorder. Signs and symptoms include fibrosis of the skin and internal organs (heart, lungs, gastrointestinal system), intellectual disability, distinctive facial features, and skeletal abnormalities.Myhre syndrome is caused by a mutation in the SMAD4 gene. It typically occurs for the first time in an affected person. A clinical case is described in a child whose family received a diagnosis of Myhre syndrome after he died. Although for some families the specific diagnosis is important, this mother did not feel that the information would have changed the course of her child’s life. We outline the benefits of palliative care supporting the child and family with attention to individualized symptom management, improved communication, and support making difficult decisions.
Tumour lysis syndrome (TLS) is a rare oncological emergency in solid tumours. Because it is associated with bad short-term prognosis, early recognition and treatment are mandatory. This case refers to a middle-aged woman who presented with stage IV colon cancer, with massive hepatic involvement. After three cycles of first-line FOLFOX (folinic acid, 5-fluorouracil and oxaliplatin), she developed acute kidney injury and hyperkalaemia that did not respond to standard measures. High suspicion of TLS prompted further corroborating investigations and early intensive care unit admission. With vigorous hydration and allopurinol, TLS completely resolved and the patient was discharged. Prophylaxis of subsequent TLS recurrence was complicated by biopsy-proven neutrophilic vasculitis secondary to allopurinol. Prevention of TLS with hydration and rasburicase was performed prior to each subsequent cycle of chemotherapy. This case report is intended to highlight risk factors for TLS in solid tumours and focus on treatment and secondary prophylaxis of TLS.
OBJECTIVE: Family members of critically ill patients can suffer symptoms of post-intensive care syndrome-family (PICS-F), including anxiety, depression, and posttraumatic stress disorder (PTSD) with a diminished quality of life. Our aim was to examine the relationship between coping strategies used by family decision-makers (FDMs) of critically ill patients and the severity of PICS-F symptoms and to examine the relationship between FDM PICS-F symptoms and health-related quality of life (HRQOL).
METHOD: A single-center, prospective, longitudinal descriptive study was undertaken of FDMs of intensive care unit (ICU) patients admitted to a large tertiary care hospital. PICS-F symptoms and coping strategy use were measured upon ICU admission (T1), 30 days (T2) after ICU admission, and 60 days (T3) after ICU admission. HRQOL was measured by the Short Form-36 version 2 at T1 and T3.
RESULTS: We found a significant prevalence of anxiety (45.8%), depression (25%), and PTSD (11.1%) symptoms among FDMs over the course of the study. The patient mortality rate in our sample was 50%. The HRQOL mental summary score in FDMs was low at T1 and decreased to M = 41.72 (standard deviation = 12.47) by T3. Avoidant coping demonstrated moderate relationships with PTSD symptoms and anxiety at T3. A previous history of anxiety, depression, or PTSD was a significant predictor of PICS-F symptom severity and prevalence. PICS symptom severity at T3 explained 75% of the variance in HRQOL mental summary score.
Significance of results: This study describes a significant prevalence of PICS-F symptoms in FDMs with a diminished mental HRQOL.