A novel veno-arteriovenous extracorporeal membrane oxygenation with double pump for the treatment of Harlequin syndrome.

OBJECTIVES: The Harlequin syndrome is a complication observed in patients receiving peripheral venoarterial extracorporeal membrane oxygenation. This condition is defined as a critical variation in the oxygen saturation between the upper and the lower part of the body deriving from a poor lung function. METHODS: Between July 2018 and November 2019, a total of 60 patients (42 men and 18 women; mean age 57.4 ± 10.0 years; range = 28-71 years) underwent peripheral venoarterial extracorporeal membrane oxygenation in our center. Harlequin syndrome was identified in eight cases (six men and two women; 13.3%) of the 60 venoarterial extracorporeal membrane oxygenation-supported patients. As a result of the Harlequin syndrome, all these patients required conversion to veno-arteriovenous extracorporeal membrane oxygenation. Control and monitoring of the blood flows of the return cannulae were performed using two centrifugal pumps, one for each inlet line, according to the patient requirements to achieve optimum hemodynamic and oxygenation. RESULTS: Mean duration of veno-arteriovenous extracorporeal membrane oxygenation support was 5.3 ± 1.4 days. Seven patients (87.5%) were switched to venovenous extracorporeal membrane oxygenation, and after 13.5 ± 2.7 days, those patients were totally weaned from extracorporeal membrane oxygenation support. One patient (12.5%) had an improvement in the pulmonary function, but the cardiac function was poor. This patient was switched to venoarterial extracorporeal membrane oxygenation, and after 10 days, the patient was completely weaned from extracorporeal membrane oxygenation support. CONCLUSION: The use of a secondary centrifugal pump to manage the blood flow directed to the internal jugular vein, in the veno-arteriovenous extracorporeal membrane oxygenation setup, allows the reduction in the risk of blood clot formation, clotting factor consumption, and pulmonary embolism when compared to the use of an external clamp.

Child Neurology: An infant with episodic facial flushing: A rare case and review of congenital harlequin syndrome.

Congenital harlequin syndrome is rare dysautonomia of the face most often reported in adults and rarely in infants and children. It is a diagnosis of exclusion and a seemingly benign condition. We report a case of a 6-month-old girl with episodic unilateral and bilateral facial flushing provoked upon awakening and resolved with sleeping with associated autonomic features consistent with harlequin syndrome. This is followed by a review of cases identified regarding this condition in infants and children.

MANAGEMENT OF ENDOCRINE DISEASE: Flushing: current concepts.

OBJECTIVE: Flushing can be defined as a sensation of warmth accompanied by erythema that most commonly is seen on the face and which occurs in episodic attacks. Such a problem can be clinically problematic, since many conditions and drugs can be related to flushing, and while often there appears to be no underlying organic disease, it is important to exclude disorders since they may be life-threatening conditions. DESIGN AND METHODS: We performed a search in MEDLINE using the terms 'flushing' in combination with 'carcinoid syndrome', 'pheochromocytoma', 'mastocytosis', 'menopausal hot flush' and 'treatment'. European and American guidelines relating to neuroendocrine tumours, mastocytosis and menopause were reviewed. RESULTS: In this review, we discuss the main causes of flushing and propose an algorithm based on pathogenesis, which can be used to guide the clinical evaluation process. We also review recent significant developments in the assessment and treatment of the carcinoid syndrome and menopausal hot flushes, which should guide the clinical practice regarding this common but sometimes confusing condition. CONCLUSIONS: When evaluating flushing, a precise systematic approach is needed to exclude potentially serious underlying causes, although despite this, the cause of the disorder is not always found. If symptoms are not progressive, the patient should be advised about its apparently benign nature in order to avoid unnecessary studies or initiating treatments of minimal benefit.

Venous Cannula Positioning in Arterial Deoxygenation During Veno-Arterial Extracorporeal Membrane Oxygenation-A Simulation Study and Case Report.

Venoarterial extracorporeal membrane oxygenation (VA-ECMO) is indicated in reversible life-threatening circulatory failure with or without respiratory failure. Arterial desaturation in the upper body is frequently seen in patients with peripheral arterial cannulation and severe respiratory failure. The importance of venous cannula positioning was explored in a computer simulation model and a clinical case was described. A closed-loop real-time simulation model has been developed including vascular segments, the heart with valves and pericardium. ECMO was simulated with a fixed flow pump and a selection of clinically relevant venous cannulation sites. A clinical case with no tidal volumes due to pneumonia and an arterial saturation of below 60% in the right hand despite VA-ECMO flow of 4 L/min was described. The case was compared with simulation data. Changing the venous cannulation site from the inferior to the superior caval vein increased arterial saturation in the right arm from below 60% to above 80% in the patient and from 64 to 81% in the simulation model without changing ECMO flow. The patient survived, was extubated and showed no signs of hypoxic damage. We conclude that venous drainage from the superior caval vein improves upper body arterial saturation during veno-arterial ECMO as compared with drainage solely from the inferior caval vein in patients with respiratory failure. The results from the simulation model are in agreement with the clinical scenario.

Urotherapy for tear production with painless urination.

We report a rare case of a 4-year-old girl with involuntary watering of eyes and facial redness during painless voiding since early infancy. Here, we describe her response to urotherapy with improvement of involuntary tearing during micturition, which although neither life threatening nor debilitating, may be quite bothersome for children and their parents, leading to anxiety, emotional burden, or embarrassment. We hypothesize that the lacrimal glands and the bladder and bowel have common central nervous system pathways and that bladder retraining and bowel management might be a potential treatment for this rare condition.

¿Tiene tratamiento la ereutofobia? / Is there a treatment for ereuthophobia?

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Practical guide to supportive care of patients with functional neuroendocrine tumors.

Supportive care of patients with functional neuroendocrine tumors (NETs) has evolved to include the use of multiple targeted agents to control paraneoplastic states and newer surgical and interventional radiologic techniques to reduce tumor bulk. Challenges encountered by the clinician are the recognition of specific symptom complexes, selecting the relevant laboratory tests and radiologic/scintigraphic scans, and the timing of intervention(s). Individual variables such as the severity of symptoms in the context of primary and metastatic disease sites, tumor bulk, comorbidities, and previous treatment are factors determining the prioritization of specific treatment regimens for patients with functional NETs. Symptoms such as flushing, secretory diarrhea, hypercalcemia, hyper /hypoglycemia, hypercortisolism, and peptic ulcers should improve with decreasing the elevated amino acid and/or peptide levels produced by NETs. These paraneoplastic symptoms may be accompanied by complaints related to tumor burden such as fatigue, pain, early satiety, anorexia, weight loss, night sweats, and/or symptoms secondary to adverse drug effects such as mucositis, dysgeusia, diarrhea, rash, hypertension, and myelosuppression. Developing a comprehensive continuum of care plan early in disease management assists in controlling the presenting signs and symptoms, and in minimizing disease- and/or treatment-related side effects. This guide serves as a framework to manage the signs and symptoms of metastatic functional neuroendocrine tumors.

Topical niacin cream-assisted 595-nm pulsed-dye laser treatment for facial flushing: retrospective analysis of 25 Korean patients.

BACKGROUND: Flushing is defined clinically as a transient reddening of the face and other areas. Due to the transient nature of flushing, a patient may not show signs of flushing during laser treatment. OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of 595-nm pulsed-dye laser treatment of flushing or erythema after provocation of flushing by topical niacin cream. METHODS: We retrospectively reviewed a total of 25 Korean patients with facial flushing who were treated with three sessions of 595-nm pulsed-dye laser after the application of topical niacin cream. RESULTS: Follow-up results revealed that 12 of the 25 patients demonstrated marked (51-75%) clinical improvement of baseline facial erythema. Eight patients had moderate (26-50%) improvement and three demonstrated near total (≥ 75%) improvement. Two patients showed minimal to no (0-25%) improvement. We observed that the reactivity to topical niacin cream was markedly reduced in 64% of our patients after 595-nm pulsed-dye laser treatments. Minimal post-therapy facial oedema was noted in most of the patients, which usually resolved spontaneously within 2 days. Pronounced facial swelling was observed in four patients. CONCLUSION: We suggest that 595-nm pulsed-dye laser treatment after provocation of flushing by topical niacin cream may provide a new treatment algorithm for facial flushing in Asians.

Niacin-a critical component to the management of atherosclerosis: contemporary management of dyslipidemia to prevent, reduce, or reverse atherosclerotic cardiovascular disease.

Niacin (nicotinic acid) is the most effective agent for raising high-density lipoprotein cholesterol levels and can improve the entire lipid panel in patients with dyslipidemia. Niacin-containing regimens are among the few treatments studied for dyslipidemia that have both elicited significant reductions in atherosclerotic progression (by angiography or imaging) and also significantly reduced (by approximately 90% vs control) the incidence of cardiovascular events in a single clinical trial. However, cutaneous flushing-an uncomfortable but typically transient adverse effect of niacin-often results in patient nonadherence with this potentially life-saving therapy. Effective counseling regarding the highly favorable benefit-risk ratio for niacin and management strategies such as careful dose escalation, follow-up monitoring, regimen adjustments, and the use of treatment adjuncts (eg, aspirin) can improve patient adherence with niacin therapy. Clinicians are uniquely positioned to provide such counseling to appropriate patients for niacin treatment and hence encourage wider use of this important and necessary cardioprotective medication.

Nena de 20 mesos amb episodis de rubicundesa generalitzada (flushing) / No disponible

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Revisión del tratamiento actual de los sofocos inducidos por deprivación androgénica en el carcinoma prostático / Review of current treatment for hot flushes induced by androgen deprivation in prostate carcinoma

Dada la mayor incidencia, prevalencia y supervivencia del carcinoma prostático en la actualidad, el manejo de los sofocos derivados de su tratamiento con análogos LH-RH ha de ser muy tenido en cuenta. El tratamiento más utilizado y a la vez el más eficaz, es la sustitución hormonal pero este tipo de terapia no esta exenta de riesgos. Hoy por hoy es factible el abordaje de los sofocos de estos pacientes mediante un variado arsenal terapéutico en el cual el tratamiento hormonal puede quedar relegado al último lugar, dado el riesgo de recidiva o progresión tumora lal tratarse de un tumor hormonosensible. El objetivo de este trabajo es revisar los tratamientos utilizados actualmente y las medidas higiénico-dietéticas que pueden ayudar a disminuir la sintomatología. Se revisarán tanto los tratamientos hormonales como los no hormonales basados en su evidencia científica. Fármacos como los nuevos antidepresivos, la gabapentina y la clonidina podrían jugar un papel destacado en el manejo. Sus mecanismos de actuación aunque dispares, se enmarcan en el complejo sistema de retroalimentación ejercido por los niveles de hormonas sexuales sobre la secreción hipotalámica de noradrenalina, causa principal en la génesis de los sofocos (AU)

Considering the currently increased incidence, prevalence and survival of prostate cancer, the management of hot flushes associated with LH-RH analog treatment must be taken into account. The most widely used and effective treatment is hormone replacement, though the latter is not without risks. It is presently possible to address hot flushes in these patients based on a broad range of treatment options in which hormone therapy may constitute a last option, due to the risk of tumor relapse or progression – since prostate cancer is hormone sensitive. The present study reviews the currently used treatments and hygiene-dietary measures that may help reduce the symptoms. A review is made of both hormone and non-hormone therapies, based on the existing scientific evidence. Drugs such as the new antidepressants, gabapentin and clonidine may play an important role in the management of hot flushes. While the underlying mechanisms of action are varied, they are related to the complex feedback exerted by the sexual hormones upon the hypothalamic secretion of noradrenalin – this being the principal etiological factor of hot flushes (AU)

A study to look at the effects of a hydrolat spray on hot flushes in women being treated for breast cancer.

Women undergoing treatment for breast cancer may experience hot flushes, which greatly impact on quality of life. The use of water sprays or moist wipes to lower skin temperature is often recommended. A peppermint and neroli hydrolat spray was compared to a plain water spray to assess which was preferred, in a single-blind randomised control crossover trial. Only 18 of the 44 patients (41%) preferred the hydrolat spray to a plain water spray, which was less than the 80% required to offer this spray as a standard suggestion for hot flush management. However a small number of those choosing it found it extremely helpful. Both sprays appeared to lessen hot flush annoyance. Previous chemotherapy appeared to be a factor influencing the choice of spray.

Resultados de la simpaticotomía videotoracoscópica en el tratamiento del rubor y la hiperhidrosis facial en 41 pacientes / Results of videothorascopic sympathicotomy in the treatment of facial redness and hyperhidrosis in 41 patients

Objetivo. Valorar los resultados y complicaciones de la simpaticotomía torácica videotoracoscópica (VATS) en el tratamiento de los pacientes diagnosticados de rubor facial incontrolable y/o hiperhidrosis facial. Pacientes y método. Entre mayo de 2000 y agosto de 2006, llevamos a cabo 82 simpaticotomías torácicas por VATS en 41 pacientes diagnosticados de rubor facial incontrolable y/o hiperhidrosis facial. Dos de ellos habían sido intervenidos anteriormente sin resultados satisfactorios. La técnica empleada fue la simpaticotomía bilateral, variando los niveles en fun- ción de la presencia de hiperhidrosis palmar y/o axilar asociada y de las limitaciones anatómicas. Los resultados fueron evaluados a la semana y a los 3-6 meses en los 41 pacientes y al año de la intervención en 34 pacientes. Resultados. Había 22 varones y 19 mujeres, con una media de edad de 33,7 (intervalo, 18-56) años. En 17 (41,5%) pacientes el síntoma principal era la hiperhidrosis facial y en 24 (58,5%), el rubor facial incontrolable. Todos los pacientes fueron dados de alta antes de las 24 h de la intervención, 14 de ellos en un programa de cirugía sin ingreso. Se obtuvo mejoría de la hiperhidrosis facial en todos los casos 17/17 (100%). En el grupo de rubor la técnica fue eficaz en 20/24 (83,3%) casos. Se obtuvo resultados satisfactorios con la segunda intervención. Se constató sudoración compensadora en 16/41 (39%) pacientes, que fue grave en 6 (14.6%). Conclusiones. La simpaticotomía torácica por VATS es un procedimiento seguro y efectivo para el manejo de la hiperhidrosis facial y el rubor incontrolable. La sudoración compensadora permanece como el principal efecto secundario (AU)

Objective. To evaluate the results and complications of thoracic sympathectomy in the treatment of patients with uncontrollable blushing and facial hyperhidrosis. Patients and method. Between May 2000 and August 2006 we performed 82 VATS sympathectomies on 41 patients with the diagnosis of uncontrollable blushing and/or facial hyperhidrosis. Two of them had been previously operated on without good results. The technique employed was bilateral VATS sympathectomy varying the levels depending on the presence of palmar and/or axillary hyperhidrosis and the anatomical limitations. The results were evaluated one week after the procedure and 3-6 months later in 41 patients; and also one year later in 34 patients. Results. Twenty-two men and 19 women, with mean age of 33.7 years (range, 18-56). In 17 patients (41.5%) main symptom was facial hyperhidrosis and in 24 (58.5%) uncontrollable blushing. All the patients were discharged before 24 hours after surgery, 14 of them in an ambulatory surgery program. There was improvement of the symptoms in all the cases of facial hyperhidrosis 17/17 (100%). In the blushing group the procedure was effective in 20/24 cases (83.3%). Results were good with the second surgery. Compensatory sweating was observed in 16/41 patients (39%), and was severe in 6 (14.6%). Conclusions. Sympathectomy is a safe and effective procedure in the management of facial hyperhidrosis and uncontrollable blushing. Compensatory sweating remains as the main secondary effect (AU)

Diagnosing, treating and managing carcinoid tumours.

Carcinoid tumours are slow-developing, rare, malignant, hormone-secreting tumours. This article discusses their diagnosis, treatment and symptom management and highlights the need for a multidisciplinary approach to patient care.

The homeopathic approach to symptom control in the cancer patient: a prospective observational study.

The aims of this study were to describe a population of patients with cancer referred for complementary therapies to an NHS homeopathic hospital, and to explore the homeopathic approach to symptom control and its impact on mood disturbance and quality of life. One hundred consecutive patients attending a designated research cancer clinic were seen for a consultation, lasting up to 60 min, and prescription of a homeopathic remedy. A maximum of three symptoms were identified and rated by the patient as a problem, using a numerical self-rating scale. The effect these symptoms have on daily life and overall sense of well being were recorded using similar scales. Patients completed the Hospital Anxiety and Depression Scale (HADS) and the European Organization for Research and Treatment in Cancer--Quality of Life Questionnaire--Core 30 (EORTC QLQ-30) at the initial consultation and at four to six consultations later. After this time, the patients completed a final assessment questionnaire asking about satisfaction with the homeopathic approach, how helpful they had found the approach for the targeted symptoms and what factors they felt may have contributed to the changes perceived. One hundred patients were entered into the study. Thirty-nine patients had metastatic disease. Nine patients were refusing conventional cancer treatments. The most common symptoms were pain, fatigue and hot flushes. Symptom scores for fatigue and hot flushes improved significantly over the study period but not for pain scores. Side effects included a transient worsening of symptoms in a few cases, which settled on stopping the remedy. Fifty-two patients completed the study, and in those patients satisfaction was high, and 75% (n=38) rated the approach as helpful or very helpful for their symptoms. Results suggest that further research is warranted to explore the management of hot flushes in women with breast cancer and fatigue in the cancer diagnosis.

Integration of acupuncture into the oncology clinic.

PURPOSE/OBJECTIVE: Patients with cancer or symptoms referable to cancer therapy were offered acupuncture as potential palliation of their symptoms. This paper describes the physical integration of the discipline into the Oncology Clinic, and patient perspectives on its availability and efficacy. PATIENTS AND METHODS: Between August 1999 and May 2000, 123 patients with varying symptoms received acupuncture in our Center's Radiation and Medical Oncology Clinics and Breast Health Center. These patients had 823 visits during this time period. A practice outcome analysis was performed on patients receiving therapy between 1 January 2000 and 30 April 2000. The 89 patients treated during this interval had 444 total visits. In June and July 2000, a questionnaire was administered by phone to 79 of these patients (89%). Standard allopathic care continued while patients were receiving acupuncture. RESULTS: Major reasons for referral included pain (53%), xerostomia (32%), hot flashes (6%) and nausea/loss of appetite (6%). Patients had a mean of five acupuncture visits (range 1-9). Most patients (60%) showed at least 30% improvement in their symptoms. About one-third of patients had no change in severity of symptoms. There were no untoward effects reported related to the acupuncture. When analysed by diagnosis, these values persist. Irrespective of response to therapy, 86% of respondents considered it 'very important' that we continue to provide acupuncture services. CONCLUSION: Acupuncture may contribute to control of symptoms for cancer patients. Expansion of providers, continued patient follow-up, optimization of techniques and prospective objective measurement of response continue in our clinic.