Craniofacial Microsomia: New Updates in Spinal Anomalies.

INTRODUCTION: Craniofacial microsomia (CFM) involves anomalies of the first and second pharyngeal arches, mainly of the mandible, maxilla, ears, and vertebral spine. This study aimed to identify the frequency and morphology of spinal anomalies of individuals with clinical diagnoses of CFM. In addition, the correlation between spinal anomalies and craniofacial involvement was performed. MATERIALS AND METHODS: This study was a retrospective review of individuals with a clinical diagnosis of craniofacial microsomia. The inclusion criteria were a clinical diagnosis of CFM with no overlap with any other syndromes of first and second pharyngeal arches and radiograph availability in the hospital's database. Prevalence and morphology of spinal anomalies were calculated and clinical details were recorded: types of spinal anomalies and correlations according to OMENS score. RESULTS: The sample consisted of 46 individuals with a clinical diagnosis of CFM, 24 (52,2%) female and 22 (47,8%) male (1M:1F). Twenty-one (45,7%) had unilateral craniofacial involvement and 25 (54,3%), bilateral. Twenty-eight (60,9%) individuals presented spinal anomalies. Those with unaltered spinal morphology showed a slight preference toward OMENS scores under 5: 7 patients did, only one of which had spinal alterations (14,3%); 68,8% (22) in the group with scores 5 to 9 (n=32) and 71,4% (5) in the 10 to 15 group (n=7) did as well. DISCUSSION AND CONCLUSIONS: Spinal anomalies in individuals with CFM are more common than usually reported in medical literature, mainly when associated with radial anomalies and correlate with statistical significance to facial features, mainly the OMENS score.

Pain management in cancer patients in the main hospitals in Mozambique.

BACKGROUND: Pain is the most feared and distressing symptom in palliative care. In advanced stages of cancer, its incidence is 70-80%. In Mozambique there is little published information concerning to the prevalence, intensity, and pain's management in cancer patients. METHODS: A cross-sectional observational study was conducted between August 2018 and January 2019, in Mozambique's main hospitals, and in the only hospital with an isolated provision of palliative care service. The analyzed data included demographic data, pain intensity and its treatment. The Pain Management Index was used to calculate the adequacy of the analgesia. RESULTS: A total of 294 patients were included. The mean patients' age was 46.1 years old. Concerning to pain, 83.7% of the patients had pain, most of them moderate to severe pain. The prevalence of pain was frequent in women mainly in cervical cancer (84.3%) and in men with Kaposi sarcoma (80%). The main analgesic used for severe pain was paracetamol, and it was used alone in 40.9% of the patients. Morphine was used in 8.1% and adjuvants less than 10%. Pain Management Index was negative for 68.7% of the sample, meaning an inadequate analgesia. Significant differences were found in Pain Management Index levels between hospitals. CONCLUSIONS: The prevalence of pain in the main health institutions in Mozambique is high. Paracetamol was the analgesic most used in severe pain. Further studies are needed to understand the main reasons of patients' suffering.

Palliative care in Mozambique: Physicians' knowledge, attitudes and practices.

BACKGROUND: Palliative care is an essential part of medical practice but it remains limited, inaccessible, or even absent in low and middle income countries. OBJECTIVES: To evaluate the general knowledge, attitudes, and practices of Mozambican physicians on palliative care. METHODS: A cross-sectional observational study was conducted between August 2018 and January 2019 in the 3 main hospitals of Mozambique, in addition to the only hospital with a standalone palliative care service. Data was collected from a self-administered survey directed to physicians in services with oncology patients. RESULTS: Two hundred and seven out of 306 physicians surveyed answered the questionnaire. The median physician age was 38 years. Fifty-five percent were males, and 49.8% residents. The most common medical specialty was surgery with 26.1%. Eighty percent of physicians answered that palliative care should be provided to patients when no curative treatments are available; 87% believed that early integration of palliative care can improve patients' quality of life; 73% regularly inform patients of a cancer diagnosis; 60% prefer to inform the diagnosis and prognosis to the family/caregivers. Fifty percent knew what a "do-not-resuscitate" order is, and 51% knew what palliative sedation is. Only 25% of the participants answered correctly all questions on palliative care general knowledge, and only 24% of the participants knew all answers about euthanasia. CONCLUSIONS: Mozambican physicians in the main hospitals of Mozambique have cursory knowledge about palliative care. Paternalism and the family-centered model are the most prevalent. More interventions and training of professionals are needed to improve palliative care knowledge and practice in the country.

Palliative care in Mozambique: Physicians' knowledge, attitudes and practices

Background Palliative care is an essential part of medical practice but it remains limited, inaccessible, or even absent in low andmiddle income countries. Objectives Toevaluate the general knowledge, attitudes, and practices of Mozambican physicians on palliative care. Methods Across­sectional observational study was conducted between August 2018 and January 2019 in the 3mainhospitals of Mozambique, in addition to the only hospital with a standalone palliative care service. Data was collected from a self-administered survey directed to physicians in services with oncology patients. Results Twohundredandsevenoutof306physicians surveyed answered the questionnaire. The median physician age was 38years. Fifty-five percent were males, and 49.8% residents. Themostcommonmedicalspecialty was surgery with 26.1%. Eighty percent of physicians answered that palliative care should be provided to patients when no curative treatments are available; 87% believed that early integration of palliative care can improve patients' quality of life; 73% regularly inform patients of a cancer diagnosis; 60% prefer to inform the diagnosis and prognosis to the family/caregivers. Fifty percent knew what a "do-not-resuscitate" order is, and 51% knew what palliative sedation is. Only 25% of the participants answered correctly all questions on palliative care general knowledge, and only 24% of the participants knew all answers about euthanasia. Conclusions Mozambican physicians in the main hospitals of Mozambique have cursory knowledge about palliative care. Paternalism and the family-centered model are the most prevalent. Moreinterventions and training of professionals are needed to improve palliative care knowledge andpractice in the country.

Deprescription in Advanced Cancer Patients.

The use of multiple drugs is often referred to as polypharmacy, although this term has not been precisely defined. Frequently, drugs are used unwisely in multiple combinations increasing the risk of adverse reactions, or for the long-term prevention of diseases in patients with a short life expectancy who, therefore, will not benefit from them. The reflection on this has led to the introduction of the concept of deprescription. There are many reasons for the inappropriate drug prescription and barriers to reduce medications. Tools were developed to help prescribers to limit the number of prescribed drugs that patients are taking. Several studies have shown that deprescription of some drugs is possible and safe and can even have a positive influence on wellbeing, cognitive function, falls, and admission to a hospital. Deprescription should be individualized and carried out, as far as possible, in agreement with patients and their families. A six-step method for deprescription is proposed.

A Protocol for the Control of Agitation in Palliative Care.

BACKGROUND: Agitation is a distressing and dangerous behavior for all involved. OBJECTIVE: To study a protocol effectiveness and safety. METHODS: The time when the protocol was initiated and when the agitation was controlled, the number of doses needed and the complications observed were recorded. RESULTS: One hundred and thirty-five inpatients of a palliative care service were included. The most frequent diagnosis was head and neck cancer, 37 (27%). The protocol was used 584 times, from 1 to 31 times on each patient, median of 3 times. Five hundred and thirty-four (91%) agitation episodes were controlled with only the first dose of the protocol, without significant complications. CONCLUSION: From those results, it can be said that this protocol is effective and safe.

Symptoms other than pain in palliative care in Portugal.

OBJECTIVE: To study the burden of patients' symptoms other than pain and their treatment by Portuguese palliative care teams. METHODS: Of the 21 Portuguese palliative care teams identified, 10 accepted to participate. Data from all patients observed on the 18th week of 2011 were collected. RESULTS: One hundred and sixty-four patients were included in this study. One hundred and fifty-one patients (92%) had cancer. The patients' median age was 71 years (16-95) and 84 (51%) were females. The main symptoms were fatigue (116 [85%]) and depression/sadness (107 [65%]). Many different drugs were used for symptom control and other aims. Symptoms and drugs used here are similar to those practiced in other countries. CONCLUSION: The practice of palliative care in Portugal seems to be similar to those in other countries.

Interleukin-1ß genotype and circulating levels in cancer patients: metastatic status and pain perception.

OBJECTIVES: Proinflammatory cytokines released during inflammation can cause hyperexcitability in pain transmission neurons, leading to hyperalgesia and allodynia. Polymorphisms in interleukin 1 (IL-1) family of genes (IL1A, IL1B) and in IL-1 receptor antagonist (IL-1Ra, coded by IL1RN) may therefore induce alterations in cytokine levels/effects and pain related response. Our purpose was to investigate the influence of polymorphisms in IL1A/B/RN on cytokine serum levels and its correlation with pain intensity, performance status, adverse effects, metastases and breakthrough pain in Caucasian cancer patients. DESIGN AND METHODS: Serum IL-1α/ß levels of 74 cancer patients were measured by competitive enzyme immunosorbent assay. All patients were also genotyped for the polymorphisms in IL1A (rs17561), IL1B (rs1143634) and IL1RN (rs419598) with Real-Time PCR. Results were then correlated to the appearance of bone or CNS metastases and several pain-related parameters. RESULTS: IL-1ß rs1143634 homozygous for T allele were associated with lower levels of IL1-ß (p=0.032, Mann-Whitney test) and presented a trend for lower levels of pain (p=0.06, Fisher's Exact Test). Also, IL1-ß levels were related with cancer onset status, since a four-fold increase probability of metastatic disease was observed in high IL-1ß individuals (OR=4.074, p=0.010, Pearson χ(2) test). Among the female patients presenting metastatic disease and carriers of the TT genotype we observed a trend to lower levels of IL1-ß (p=0.053, Pearson χ(2) test). CONCLUSIONS: Our results indicate that genetic variation at IL1-ß gene may influence serum levels of IL1-ß, with proportional consequences in cancer-related pain.

Initial assessment of patients without cognitive failure admitted in palliative care.

AIM: To develop a screening tool that was short, not time consuming but able to detect the patients' main problems at admission. METHODS: A list of 106 symptoms/problems derived from a review of the literature was created and shortened using a Delphi process. RESULTS: Thirteen experts scored each item with a numeric rating scale of 0 to 10 for relevance. After 3 rounds, the list was shortened to 14 items: general question--what bothers you the most? symptoms/problems--pain, lack of appetite, vomiting, tiredness/fatigue, nausea, constipation, shortness of breath, depression, anxiety, difficulty sleeping; activity (dressing, washing, etc); support from family/friends; and well-being. CONCLUSION: The final tool is short and seems to include the relevant items that would make it useful for clinical practice.

A cross-sectional survey of pain in palliative care in Portugal.

OBJECTIVE: The purpose of this work is to study the prevalence, intensity, and treatment of pain in Portuguese palliative care teams. METHODS: Twenty-one palliative care teams were invited to participate in a cross-sectional survey. Ten of these accepted and were included in the study. Data of all patients observed on the 18th week of 2011 were collected. The data collected concerning pain were: demographic data, pain intensity, drugs prescribed, and invasive techniques. The intensity of pain was rated using a five-point verbal rating scale from none to maximum. The Pain Management Index (PMI) was used to calculate the adequacy of the analgesia. RESULTS: A total of 164 patients were included in this study. One hundred fifty-one (92 %) had cancer. The median age was 71 years (16 to 95). Eighty-four (51 %) were females. Pain was directly assessed in 136 (83 %) of the patients, whereas 27 patients could not report pain because of cognitive failure. Of those directly assessed, 77 (57 %) had pain when they were assessed: 42 (55 %) mild, 25 (32 %) moderate, 9 (12 %) severe, and 1 (1 %) maximum. Non-opioid analgesics were used: paracetamol in 61 (37 %) and NSAID in 20 (12 %). Tramadol was the only opioid for mild to moderate pain used in 25 (15 %) patients. The opioids most used for moderate to intense pain were: morphine 74 (45 %), transdermal (TD) fentanyl 32 (20 %), and buprenorphine TD 28 (17 %). The adjuvants most used were: corticosteroids 38 (23 %), gabapentin 37 (23 %), and amitriptyline 15 (9 %). Only five (4 %) patients had a negative PMI, meaning an inadequate analgesia. CONCLUSION: The general prevalence of pain is similar to that reported by other. The prevalence of moderate to severe pain is also similar to that reported in other studies, although severe pain is somewhat lower than indicated in most reports. According to the PMI, pain control was acceptable to good.

A cross-sectional survey of the activity of palliative care teams in Portugal.

Of the 21 Portuguese teams identified, 10 accepted to participate in the study. A total of 164 patients were included with a median of 15.5 per team (4-32). Of all the patients included, 60 (37%) were identified as inpatients in palliative care units; 59 (36%) by an intrahospital support team; 26 (16%) as outpatients; and 19 (12%) at home. The median age was 71 years (16-95). Fifty-one percent were females. The diagnosis was cancer in 151 (92%) patients. The most common cancer was colorectal in 22 (15%) patients, followed by gastric 17 (11%), head and neck 17 (11%), breast 15 (10%), and lung cancers 14 (9%). All patients were treated by doctors and nurses experienced in palliative care.

Variation in the incidence of agitated delirium during the day in a palliative care unit: a preliminary report.

In the literature regarding delirium and agitation in palliative care, there are references of their worsening as the hours of the day flows from afternoon on, with an inversion of the awake-sleep cycle. We studied the frequency of the use of our protocol for the control of agitation. From June 18, 2007, to January 4, 2010, 27 (5.3%) patients from the 509 admitted were sedated intermittently 86 times, in strict compliance with the protocol, because of episodes of agitation. We verified that from 8 pm until 6 am, the number of observed cases clearly exceeds the expected cases, if the distribution was uniform. The reverse situation occurs in all other hours of the day. The chi-square goodness of fit test proves that the differences are statistically significant (P < .001).

A survey of the sedation practice of Portuguese palliative care teams.

AIM: The purpose of this study is to study the practice of sedation by Portuguese palliative care teams. METHODS: The teams included on the website of the Portuguese Association for Palliative Care were invited to participate. Data from all the patients sedated between April and June 2010 were recorded. Sedation was defined as the intentional administration of sedative drugs for symptom control, except insomnia, independently of the consciousness level reached. RESULTS: Of the 19 teams invited only 4 actually participated. During the study period, 181 patients were treated: 171 (94 %) were cancer patients and 10 non-cancer patients. Twenty-seven (16 %) patients were sedated: 13 intermittently, 11 continuously, and 3 intermittently at first then continuously. The rate of sedation varied substantially among the teams. Delirium was the most frequent reason for sedation. Midazolam was the drug used in most cases. In 21 cases of sedation, the decision was made unilaterally by the professionals; in 16 (76 %) of those, the situation was deemed to be emergent. From the patients on continuous sedation, 9 (64 %) patients maintained oxygen, 13 (93 %) hydration, and 6 (43 %) nutrition. Two patients who had undergone intermittent sedation were discharged home and one was transferred to another institution; the reason for sedation in the three cases was delirium. CONCLUSION: There is a substantial variation in the sedation rate among the teams. One of the most important aspects was the decision-making process which should be object of reflection and discussion in the teams.

A protocol for the acute control of agitation in palliative care: a preliminary report.

Agitation is one of the most frequent causes for palliative sedation. It often requires urgent control to avoid negative consequences and even endangerment of all involved, including the patients themselves. A protocol for the control of episodes of agitation was developed, based on a previous experience. The protocol includes a combination of haloperidol and midazolam. The protocol was used 86 times in 27 patients. Each patient was sedated from 1 to 12 times, median 2 times. The median time from the beginning of sedation to the control of agitation was 15 minutes with a range from 1 minute (2 cases) to 3 hours and 5 minutes (only 1 case). In 71 cases (83%), only the first dose was needed. There were no significant complications.

Thalidomide for the control of severe paraneoplastic pruritus associated with Hodgkin's disease.

A 22-year-old woman with nodular sclerosis type II Hodgkin lymphoma diagnosed in June 2001. She initially underwent chemotherapy with 6 cycles of ABVD (adriamycin, bleomycin, vincristine, dacarbazine) regimen, leading to clinical remission. As it relapsed, she was again treated with 2 different chemotherapy regimens. In November 2003, she underwent bone marrow autotransplantation, but it relapsed after 2 months. After that, she was treated with chemotherapy in monotherapy until November 2005. In December 2005, she was referred to palliative care. Her main symptom was very severe pruritus that interfered with all aspects of her life, making her scratch continuously and interfering in all aspects of her life. She was treated with loratadine, hydroxyzine, prednisolone, paroxetine, mirtazapine, cimetidine, and ondansetron, individually and in various combinations. She also underwent ultraviolet phototherapy. All trials failed and her pruritus remained at level 8 of 10 most of the time. In April 2006, she started on thalidomide, 200 mg at night. The pruritus significantly improved to a level of 3 of 10 but did not disappear completely. She was at last able to sleep properly at night. She remained with a low level of pruritus until her death in July 2008, at the same dose of thalidomide.

Attitudes toward assisted death amongst Portuguese oncologists.

BACKGROUND: The attitudes and practise of doctors concerning euthanasia and assisted suicide have been the subject of studies performed in many countries. However, these issues have not been studied properly in Portugal. MATERIALS AND METHOD: This study is a survey of 450 Portuguese oncologists by postal means and personal contact. RESULTS: The response rate was 33% (143). Only 13% would practise euthanasia with the present law in force forbidding such practise, and 24% would do so if it were legalised; 39% favoured its legalisation and 36% would like to have the option of euthanasia if they had a terminal disease. About assisted suicide, 15% would do it with the current law in force forbidding such action and 25% would do so if it were made legal; 32% favoured its legalisation and 24% would like to have that option if they had a terminal disease. There was one case of euthanasia and no cases of assisted suicide. The most important factor related with the acceptance of euthanasia and assisted suicide was religion, with non-practising Catholics accepting such practises more often than practising Catholics. The Portuguese oncologists have a very positive view on the potential role of palliative care in preventing many requests for euthanasia and assisted suicide. CONCLUSION: Portuguese oncologists are mainly against the practise of euthanasia and assisted suicide and the number of requests is also relatively low; consequently, the number of episodes of assisted death is also apparently very low.

Gastrostomies in palliative care.

OBJECTIVE: In palliative care, gastrostomies are used to provide nutritional support or to decompress the bowel. To evaluate what happened to the patients monitored at our palliative care unit (PCU) who underwent gastrostomy between October 1994 and January 2005, a retrospective audit was made. METHOD: The charts of 154 patients were reviewed. RESULTS: The most frequent reason why a patient underwent a gastrostomy was dysphagia due to head and neck and/or esophageal cancer. Only one patient underwent a drainage gastrostomy because of intestinal obstruction. Interventional radiology performed 96% of the gastrostomies. Early complications occurred in 53 patients (34%) who underwent the gastrostomy for feeding and the most common was local pain, usually mild. However, there was one death due to peritonitis, probably related with the procedure. Late complications also occurred in 53 patients and major complications occurred in 22 patients, the most common was extrusion. The median survival after the performance of the gastrostomy was 61 days (range 1 to 551 days). Nineteen patients (12%) survived 1 week or less, 28 (18%) between 8 and 30 days, 51 (33%) from 31 to 90 days, 53 (35%) 91 days or more, and one unknown. The patient who underwent a gastrostomy for bowel obstruction survived for only 7 days. One hundred and twenty-five patients (81%) died at the PCU, 26 (17%) at home, and four (3%) at other places.

Breaking bad news: experiences and preferences of advanced cancer patients at a Portuguese oncology centre.

The disclosure of information to patients about diagnosis and prognosis and other aspects of care is still a matter of debate. We have conducted a study in advanced cancer patients about their experiences and preferences concerning this issue. A questionnaire was developed and completed during the first appointment in the outpatient clinic of an oncology centre's palliative care unit, before the patient had any contact with the staff of the unit. The study was conducted on a convenience sample of 47 patients. We found that 34 (72%) of those patients thought they had been informed of their diagnosis, most of them by the hospital doctor. However, not all stated the diagnosis in a manner clearly showing that they were aware of the nature of their disease. Most patients were with a family member when the diagnosis was disclosed, which is what the majority had preferred. Of the 13 uninformed patients, only one preferred to remain uninformed. Most patients (89%) participated in decisions concerning treatment, although only 68% thought they should have participated. Some 39 patients (83%) were informed that they were being referred to the palliative care unit, but surprisingly only eight had received an explanation of the unit's function. We concluded that, although most patients had been informed of their disease, there remains many problems, the most important of which, in our view, is the difference between the information provided and the patients' needs.