[Harlequin syndrome after scoliosis surgery]. / Harlekinsyndrom nach Skoliosechirurgie.

Harlequin syndrome is a rare combination of symptoms, characterized by unilateral facial anhidrosis and paleness on the affected side, becoming obvious by contralateral flushing mainly during sports activity. The syndrome is mostly idiopathic, however it is also described as a complication of thoracic surgery, i.e. superior lobectomy. Here, we report on two cases of Harlequin syndrome following scoliosis surgery at the cervicothoracic junction.

Pure autonomic failure presenting as Harlequin syndrome.

Pure autonomic failure (PAF) is a progressive syndrome of neurogenic orthostatic hypotension, widespread anhidrosis, urinary retention, and constipation without other neurologic manifestations. It is generally considered a peripheral ganglionic synucleinopathy. Natural history studies have described risk factors for the conversion of PAF to Parkinson's disease, multiple system atrophy, or dementia with Lewy bodies, yet the early stages of PAF are not well characterized. We present a patient with unilateral anhidrosis, contralateral facial flushing and hyperhidrosis consistent with Harlequin syndrome that, over 6 years, progressed to PAF, suggesting that PAF may present with focal autonomic impairment prior to generalized autonomic failure.

Risks of substance uses, alcohol flush response, Helicobacter pylori infection and upper digestive tract diseases-An endoscopy cross-sectional study.

This study examines the effects of environmental hazards, including tobacco, alcohol/alcohol flush response, areca nut, and Helicobacter pylori (H pylori) infection on upper digestive diseases. This is a multi-hospital-based endoscopy-survey cross-sectional study. Subjects were received upper endoscopies in outpatient clinics at four hospitals in Taiwan between 2008 and 2013. Biopsy-based methods or urea breath test were used confirm the status of H pylori infection. In total, 8135 subjects were analyzed. Higher cumulative amounts of alcohol consumption were at higher risk of Barrett's esophagus and esophageal squamous cell carcinoma (ESCC), higher cumulative amounts of tobacco consumption were at higher risk of peptic ulcer, and higher cumulative amounts of areca nut consumption were at higher risk of duodenitis. Alcohol flush response was significant risk for reflux esophagitis and Barrett's esophagus (adjusted odds ratio [aOR] = 1.18 and 1.32, 95% confidence interval [CI] = 1.07-1.31 and 1.06-1.65, respectively). H pylori infection was inversely associated with ESCC risk (aOR = 0.20, 95% CI = 0.10-0.40). In addition, H pylori infection was consistently and significantly risk factors for gastrointestinal diseases, including peptic ulcer, gastric adenocarcinoma, and duodenitis (aOR = 5.51, 1.84, and 2.10, 95% CI = 4.85-6.26, 1.03-3.26, and 1.71-2.56, respectively). Besides the cumulative risk of alcohol, tobacco, and areca nut for Barrett's esophagus, ESCC, and peptic ulcer, respectively, presence of facial flushing was the significant risk for reflux esophagitis and Barrett's esophagus. H pylori infection was positively associated with peptic ulcer, gastric adenocarcinoma, and duodenitis, but inversely associated with ESCC.

Exploring the utility of alcohol flushing as an instrumental variable for alcohol intake in Koreans.

Previous studies have indicated an association of higher alcohol intake with cardiovascular disease and related traits, but causation has not been definitively established. In this study, the causal effect of alcohol intake on hypertension in 2,011 men and women from the Ansan-Ansung cohort was estimated using an instrumental variable (IV) approach, with both a phenotypic and genotypic instrument for alcohol intake: alcohol flushing and the rs671 genotype (in the alcohol dehydrogenase 2 [ALDH2] gene), respectively. Both alcohol flushing and the rs671 genotype were associated with alcohol intake (difference in alcohol intake with alcohol flushers vs. non-flushers: -9.07 g/day; 95% confidence interval [CI]: -11.12, -7.02; P-value: 8.3 × 10-18 and with the rs671 GA + AA vs. GG genotype: -7.94 g/day; 95% CI: -10.20, -5.69; P-value: 6.1 × 10-12). An increase in alcohol intake, as predicted by both the absence of alcohol flushing and the presence of the rs671 GG genotype in the IV analyses, was associated with an increase in blood pressure in men from this Korean population. In conclusion, this study supports a causal effect of alcohol intake on hypertension and indicated that alcohol flushing may be a valid proxy for the ALDH2 rs671 polymorphism, which influences alcohol intake in this Korean population.

Diarrea crónica, síndrome constitucional e insuficiencia cardiaca. ¿Manifestaciones de una misma entidad? / Chronic diarrhea, weight loss and heart failure. Features of the same disease?

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Congenital Cases of Concomitant Harlequin and Horner Syndromes.

We report three pediatric cases of concomitant congenital Horner and Harlequin syndromes. This association suggests a lesion at the superior cervical ganglion or just inferior. Often, no underlying lesion is documented.

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.

Linfedema de parénquima de mama. A propósito de 2 casos clínicos / Breast lymphoedema. A propos of 2 cases

El linfedema de mama (LM) secundario a cirugía tras un proceso neoplásico se manifiesta con edema o rubefacción en la zona de la mama. Presentamos 2 casos de pacientes intervenidas de cáncer de mama izquierda, hemimastectomía y linfadedectomía, que recibieron quimioterapia (QT) y radioterapia (RT) adyuvante. Fueron remitidas al Servicio de Rehabilitación (RHB) por aumento de volumen y sensación de dureza en extremidad superior izquierda (ESI) y mama. Tras objetivar el edema en la mama, tratamos el LM con taping linfático. Se las citó para revisión a la semana, y refirieron mejoría en la sensación de dureza e hinchazón en mama en la escala visual analógica (EVA). El LM es una dolencia infradiagnosticada aunque relativamente frecuente, de difícil cuantificación, y no hay descrito en la literatura un tratamiento específico. El taping linfático es una opción de tratamiento para el LM, ya que mejora la sensación subjetiva de dureza e hinchazón así como la clínica de induración y edema (AU)

Breast lymphoedema after oncological surgery is characterized by ipsilateral chest swelling and/or redness. We report the cases of 2 patients who underwent surgery for left breast cancer. Hemimastectomy and lymphadenectomy were performed, and postoperative therapy included adjuvant chemotherapy and radiotherapy. After discharge the patients were sent to our Rehabilitation Department complaining of swelling and heaviness in the left upper extremity and breast. The patients underwent a physical examination of the breast, and the lymphoedema of the upper extremity was measured. Lymphatic taping was applied to treat the breast lymphedema. One week later, both patients reported improvement in heaviness and swelling quantified by the visual analog scale (VAS). Breast lymphoedema is a relatively common but underdiagnosed condition and is difficult to quantify. There is no reported standard treatment for this entity in the literature. Lymphatic taping is an optional treatment for breast lymphoedema as it improves the subjective feeling of heaviness and swelling, as well as induration and oedema (AU)

[Harlequin phenomenon associated with neurological abnormalities: A case report]. / Phénomène d'Arlequin associé à des anomalies neurologiques : un cas.

BACKGROUND: Harlequin phenomenon consists of facial flush and erythrosis with unilateral sweating and pallor, associated with contralateral anhidrosis. We present the case of a child in whom the syndrome was associated with Horner's syndrome, epilepsy, mental and psychomotor retardation. PATIENTS AND METHODS: A 9-year-old boy presented with right unilateral hemifacial erythema on effort, with normal colouring and Horner's syndrome on the left side of the face. His medical history revealed generalized myoclonic epilepsy, psychomotor delay and mental retardation. No underlying anomalies were identified. Harlequin phenomenon was diagnosed. DISCUSSION: Despite its stereotypical clinical features, Harlequin phenomenon is a poorly known disease. However, clinicians must be aware of it in order to determine the diagnosis and investigate for causes and any associated abnormalities. The underlying mechanism is an autonomic neuropathy affecting the sympathetic vasodilator neurons. To our knowledge, there have been no previous reports of Harlequin phenomenon in association with Horner syndrome, psychomotor delay and mental retardation.

Harlequin syndrome in a patient with putative autoimmune autonomic ganglionopathy.

The author presents a patient with Harlequin and Horner syndromes as part of an autoimmune autonomic ganglionopathy and suggests implication for work-up and management. In general, Harlequin and Horner syndromes are reported to be caused by either a structural lesion of the sympathetic pathway or, when no structural lesion is found, are presumed to be idiopathic. In this paper, a 76 year old man developed a Harlequin and Horner syndromes in the setting of subacute autonomic failure and other systemic features. The patient's symptoms improved with a short course of intravenous methylprednisolone. An autoimmune etiology should be considered in patients with Harlequin syndrome and immunomodulatory treatment could be attempted, especially when there is evidence of a more generalized autoimmune autonomic ganglionopathy.

Concurrent Horner's and Harlequin syndromes.

Horner's syndrome and Harlequin syndrome are both caused by disruptions to the sympathetic supply to the face. They have a varied aetiology, including intraneural dysfunction, extra- or intraneural compression, and idiopathic as well as iatrogenic causes. Horner's syndrome can occur as a rare complication of thyroid surgery and the Harlequin sign has only been documented as a complication of cervical surgery in a handful of paediatric patients. We present a patient who developed both conditions subsequent to excision of a papillary carcinoma with a neck dissection. We illustrate the anatomical basis for this presentation and highlight the need for its appreciation. This is of particular interest as it impacts on several specialities operating in the cervical and thoracic fields.

Flushing and pruritus secondary to prescription fish oil ingestion in a patient with allergy to fish.

BACKGROUND: A brand of fish oil capsules contains omega-3 fatty acids obtained from several fish sources. Although the manufacturer calls for caution in patients with fish hypersensitivity, insufficient data is available to make a definitive recommendation regarding its use in this population. CASE PRESENTATION: A patient with documented seafood allergy presented to the emergency department 4 days after the initiation of prescription brand name fish oil capsules complaining of chest tightness, shortness of breath, tingling of upper extremities, flushing, and pruritus that was minimally relieved by excessive nonprescription diphenhydramine administration. During subsequent follow-up, the patient reported that all symptoms had resolved within 5 days of discontinuing the medication and 3 days of disposing of her pillbox and all medications that had come in contact with the fish oil capsules. CONCLUSION: Due to the patient's allergic history, timing of onset/offset of the reaction, laboratory evidence, and the use of the Naranjo probability scale, prescription fish oil capsules were deemed the probable cause of this patient's pruritus and flushing of the face and trunk. Practitioners and patients should always ensure they have an updated list of allergies within the patient's medical record that includes medications as well as foods and food additives.

Association between gastric cardia adenocarcinoma risk and alcohol flushing response, but not alcohol consumption.

The relationship between the development of gastric cardia cancer (GCA) and alcohol consumption remains unclear. Alcohol flushing response reflects an accumulation of the carcinogenic acetaldehyde and has been proved to be a risk factor for many cancers. The main objective of the present study was to assess the impact of alcohol flushing response on GCA risk in a Chinese population in conjunction with lifetime alcohol consumption. The study subjects consist of 281 male patients [130 with GCA and 151 with esophageal squamous cell carcinoma (ESCC)] and 160 non-cancer male controls, matched with respect to age. Conditional logistic regression was used to calculate odds ratios (ORs) and 95% confidence intervals (95% CI). There is no significant association between GCA risk and alcohol consumption. However, the adjusted ORs for flushing response of GCA and ESCC was 2.03 (95% CI 1.15-3.56, p=0.014) and 2.32 (95% CI 1.34-4.03, p=0.003), respectively, compared with those reporting no flushing response. Furthermore, compared with non-drinkers, the heavy drinkers and moderate drinkers with current/former flushing response experienced significant GCA risk (heavy drinkers: OR 2.59, 95% CI 1.06-6.31, p=0.037; moderate drinkers: OR 3.11, 95% CI 1.17-8.26, p=0.023), while drinkers without flushing response did not have increased risk. The drinkers with flushing response also had a higher ESCC risk than those without flushing response. In conclusion, alcohol flushing response is a clinically useful biomarker of susceptibility to GCA and ESCC risk from alcohol.

[Dysautonomic syndrome of the face with Harlequin sign and syndrome: Three new cases and a review of the literature]. / Syndromes dysautonomiques du visage avec signe et syndrome d'Harlequin : étude de trois cas et revue de la littérature.

INTRODUCTION: Harlequin phenomenon is characterized by a strictly unilateral erythrosis of the face with flushing and hyperhydrosis, and controlaterally a pale anhydrotic aspect. This syndrome can occur alone or associated to other dysautonomic phenomena such as Horner syndrome, Adie syndrome or Ross syndrome. PATIENTS AND METHODS: We report three cases: two patients presented a Harlequin sign, associated with Horner syndrome for one and Ross syndrome for the second. The etiologic investigation was normal, allowing recognizing the idiopathic nature of the disorder. For the third patient, Harlequin syndrome was observed in a neoplastic context due to breast cancer, metastatic dissemination, and bone metastases involving the right side of the T2 body. DISCUSSION: We reviewed the literature: 108 cases have been described. This syndrome occurred alone in 48 patients and was associated with other dysautonomic syndromes such as Horner syndrome in 38 patients, Holmes Adie syndrome in six, and Ross syndrome in six; both Ross and Holmes Adie syndrome were associated five cases and associations were not reported in five patients. The pathophysiological mechanisms of this autonomic cranial neuropathy, the possible etiologies, and therapeutic management were discussed. CONCLUSION: Harlequin phenomenon with flushing and unilateral hyperhydrosis is rare, occurring alone or in combination with other autonomic syndromes of the face. Idiopathic in two-thirds of cases, Harlequin phenomenon does not require specific treatment; sympathectomy may be discussed in the severe cases with a significant social impact.