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Acta Medica Iranica Jul 2017Prepartum or postpartum right ventricular thrombosis (RVT) is an exceedingly rare and potentially lethal phenomenon in pregnancy. We here report a case of a pregnant...
Prepartum or postpartum right ventricular thrombosis (RVT) is an exceedingly rare and potentially lethal phenomenon in pregnancy. We here report a case of a pregnant patient with near term pregnancy admitted for dyspnea, amniotic fluids discharge and labor pain in a gynecology center that an eight-month dead fetus was diagnosed and delivered vaginally by induction. A post delivery period was complicated by aggravation of her dyspnea and pleuritic chest pain that she referred for further evaluation in our cardiac center. Physical exam revealed normal head and neck exam, and history taking revealed that her fetus had intra-uterine growth failure as reported by her gynecologist. Chest exam except to left lung crackle was normal. Lower and upper left extremities were normal. However, acrocyanosis was found in tips of 4 and 5th right-hand digits. Chest x-ray revealed some linear consolidation in left lower lung lobes, and the precordial exam was normal. ECG was normal. Post delivery transthoracic echocardiography (TEE) showed a 1.5×1.5 cm mobile right ventricular clot. C-T angiography revealed obstruction of left upper lung pulmonary artery branches. Complete thrombophilia assay showed the presence of high titer of antiphospholipid, anticardiolipin antibody, and β1 glycoprotein antibody. However, others test were normal. The patient was scheduled for cardiac surgery, and her hemodynamic was monitored by left radial artery line and central pressure venous line, and thrombus was removed from the right ventricle (RV), and subsequent anticoagulation therapy constituted. Six-month follow-up revealed no recurrence of thrombus and recovery of patient's symptoms.
Topics: Angiography; Cardiac Surgical Procedures; Echocardiography; Female; Fetal Death; Humans; Pregnancy; Pregnancy Complications; Thrombosis; Young Adult
PubMed: 28918617
DOI: No ID Found -
Reviews in Cardiovascular Medicine Jan 2022After reading with great interest the article entitled: "Exploring the impact of the COVID-19 pandemic on provision of cardiology services: a scoping review" redacted by...
After reading with great interest the article entitled: "Exploring the impact of the COVID-19 pandemic on provision of cardiology services: a scoping review" redacted by Farah Yasmin ., published by Reviews in Cardiovascular Medicine, we would like to add the following thoughts. Acute respiratory distress syndrome (ARDS) in Coronavirus disease 2019 (COVID-19) and pulmonary insufficiency reduces blood oxygen saturation and results in hypoxia. Therefore, the determining factor in the survival of patients with COVID-19 is their resistance to hypoxia. At the same time, it is the cardiovascular system that is an important and very sensitive link in the human adaptation to hypoxia. That is why it is necessary to carefully study the relationship between diseases of the heart, blood vessels, the reactivity of the cardiovascular system to hypoxia, and mortality in patients who develop ARDS with COVID-19.
Topics: COVID-19; Cardiology; Cardiovascular System; Humans; Hypoxia; Pandemics; Respiratory Insufficiency; SARS-CoV-2
PubMed: 35092213
DOI: 10.31083/j.rcm2301021 -
The American Journal of Emergency... Feb 2021Cold environments can trigger a variety of conditions, which, in their acute phase often present to the Emergency Department. Primary acrocyanosis is a distinct, rare...
Cold environments can trigger a variety of conditions, which, in their acute phase often present to the Emergency Department. Primary acrocyanosis is a distinct, rare condition which may be missed resulting in misdiagnosis and mismanagement. Primary acrocyanosis is a peripheral vascular disorder defined by painless, symmetrical discoloration of the distal appendages and uniquely characterized by persistence of the skin color changes after cold exposure. We present a case of a 24-year-old female who presented to the Emergency Department with peripheral cyanosis after cold exposure and was eventually diagnosed with primary acrocyanosis by Rheumatology. The prognosis for primary acrocyanosis is quite good in comparison to other acrosyndromes and once secondary causes of acrocyanosis have been ruled, out can be managed conservatively with lifestyle modifications and potential follow-up with Rheumatology.
Topics: Cold Temperature; Cyanosis; Diagnosis, Differential; Female; Fingers; Humans; Peripheral Vascular Diseases; Young Adult
PubMed: 32800682
DOI: 10.1016/j.ajem.2020.07.064 -
BMC Rheumatology May 2022POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud's phenomenon is an overlooked skin...
Raynaud's phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report.
BACKGROUND
POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud's phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis.
CASE PRESENTATION
A 47-year-old man presented with color changes on fingertips consistent with biphasic Raynaud's phenomenon; an antinuclear antibody test was positive (at 1:320 titers in a speckled pattern) with normal nailfold capillaroscopy. Clinical features of systemic sclerosis were absent. Twenty-four months later, the patient presented symmetric sensorimotor demyelinating polyneuropathy, and he was diagnosed with Guillain-Barre syndrome; treatment with intravenous gammaglobulin had an incomplete response. Raynaud's phenomenon persisted associated with acrocyanosis, white nails, and positive ANA (1:1280 in a nucleolar pattern). POEMS syndrome was suspected, and serum protein electrophoresis (SPEP) was done. The SPEP revealed polyclonal gammopathy, and serum immunofixation showed monoclonal (M)-protein (IgG lambda). Serum vascular endothelial growth factor concentration showed increased levels. The patient was diagnosed with POEMS syndrome, and treatment with lenalidomide and dexamethasone improved the Raynaud's phenomenon, acrocyanosis, and white nails, but the neurological response was partial.
CONCLUSIONS
POEMS syndrome may mimic clinical manifestations of systemic sclerosis v.g. Raynaud's phenomenon, skin thickening, telangiectasia, and positive ANA. Raynaud's phenomenon may precede other clinical manifestations of POEMS syndrome by several months. It is necessary to have a high index of suspicion for the diagnosis, especially in patients with peripheral polyneuropathy and monoclonal paraprotein. The significance of positive ANA in this condition is unknown and deserves further investigation.
PubMed: 35585632
DOI: 10.1186/s41927-022-00258-y -
Case Reports in Vascular Medicine 2015Introduction. Digital ischemia is a rare complication of several chemotherapeutic medications. We aimed to present a patient with digital ischemia, secondary to a new...
Introduction. Digital ischemia is a rare complication of several chemotherapeutic medications. We aimed to present a patient with digital ischemia, secondary to a new generation chemotherapeutic drug, oxaliplatin. Case Report. 62-year-old woman presented to our department with severe pain, paresthesia, and distal acrocyanosis on her right hand fingertips. Her complaints started five days after the third cycle of a chemotherapy protocol consisting of 5-fluorourasil (5-FU), folinic acid, and oxaliplatin due to advanced colon carcinoma. On physical examination, hemorrhagic and partly ulcerative lesions were detected at her right hand fingertips. Radial and ulnar pulses were absent at affected side. Digital subtraction angiography revealed severe vascular resistance in the affected extremity. Iloprost trometamol treatment was started with the dosage of 1 ng/kg/min. In addition, low-molecule-weight heparin was used for preventing possible microemboli. Symptomatic relief was provided after five days, and patient was discharged on 7th day of treatment. Discussion. The pathogenesis of oxaliplatin induced vascular toxicity remains unclear. Endothelial damage, increased adherence of platelets, deposition of immune complexes as an immunologic effect of oxaliplatin, and hypercoagulable state may be the reason for arterial thrombosis, digital microemboli, possible digital ischemia, and their several consequences.
PubMed: 26064768
DOI: 10.1155/2015/248748 -
Frontiers in Endocrinology 2022Hypoglycemia is uncommon in people who are not being treated for diabetes mellitus and, when present, the differential diagnosis is broad. Artifactual hypoglycemia...
BACKGROUND
Hypoglycemia is uncommon in people who are not being treated for diabetes mellitus and, when present, the differential diagnosis is broad. Artifactual hypoglycemia describes discrepancy between low capillary and normal plasma glucose levels regardless of symptoms and should be considered in patients with Raynaud's phenomenon.
CASE PRESENTATION
A 46-year-old female patient with a history of a sleeve gastrectomy started complaining about episodes of lipothymias preceded by sweating, nausea, and dizziness. During one of these episodes, a capillary blood glucose was obtained with a value of 24 mg/dl. She had multiple emergency admissions with low-capillary glycemia. An exhaustive investigation for possible causes of hypoglycemia was made for 18 months. The 72h fasting test was negative for hypoglycemia. A Raynaud's phenomenon was identified during one appointment.
CONCLUSION
Artifactual hypoglycemia has been described in various conditions including Raynaud's phenomenon, peripheral arterial disease, Eisenmenger syndrome, acrocyanosis, or hypothermia. With this case report, we want to reinforce the importance of being aware of this diagnosis to prevent anxiety, unnecessary treatment, and diagnostic tests.
Topics: Awareness; Blood Glucose; Diabetes Mellitus; Female; Humans; Hypoglycemia; Memory Disorders; Middle Aged
PubMed: 35966055
DOI: 10.3389/fendo.2022.951377 -
Emergencias : Revista de La Sociedad... Jun 2016
Topics: Aged, 80 and over; Cyanosis; Diagnostic Errors; Female; Fingers; Humans; Hypoglycemia
PubMed: 29105461
DOI: No ID Found -
Zhongguo Dang Dai Er Ke Za Zhi =... Mar 2021A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed....
A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed. Laboratory examinations showed an increase in hemoglobin (162 g/L) and a reduction in arterial partial pressure of oxygen (61.5 mm Hg). Lung CT showed irregular slightly high-density nodules in the middle lobe of the right lung, and contrast-enhanced CT scan showed obvious enhancement with thick vascular shadow locally. An investigation of medical history revealed that the girl's mother had a history of epistaxis and resection of pulmonary mass and the girl presented with tongue telangiectasia. The girl was diagnosed with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformation. she was given interventional embolization therapy. Transcutaneous oxygen saturation reached 98% without oxygen inhalation on the day after surgery. Pulmonary angiography at 3 months after surgery showed the recurrence of pulmonary vascular malformation, and embolization of pulmonary arterial fistula was not performed since the guide wire could not enter the branch artery. There was still a need for long-term follow-up.
Topics: Adolescent; Arteriovenous Fistula; Arteriovenous Malformations; Female; Humans; Multiple Pulmonary Nodules; Neoplasm Recurrence, Local; Pulmonary Artery; Seizures
PubMed: 33691924
DOI: 10.7499/j.issn.1008-8830.2010094 -
Clinical Autonomic Research : Official... Dec 2021In neuropathic postural tachycardia syndrome, peripheral sympathetic dysfunction leads to excessive venous blood pooling during orthostasis. Up to 84% of patients report...
PURPOSE
In neuropathic postural tachycardia syndrome, peripheral sympathetic dysfunction leads to excessive venous blood pooling during orthostasis. Up to 84% of patients report leg pain and weakness in the upright position. To explore possible pathophysiological processes underlying these symptoms, the present study examined muscle excitability depending on body position in patients with neuropathic postural tachycardia syndrome and healthy subjects.
METHODS
In ten patients with neuropathic postural tachycardia syndrome and ten healthy subjects, muscle excitability measurements were performed repeatedly: in the supine position, during 10 min of head-up tilt and during 6 min thereafter. Additionally, lower leg circumference was measured and subjective leg pain levels were assessed.
RESULTS
In patients with neuropathic postural tachycardia syndrome, muscle excitability was increased in the supine position, decreased progressively during tilt, continued to decrease after being returned to the supine position, and did not completely recover to baseline values after 6 min of supine rest. The reduction in muscle excitability during tilt was paralleled by an increase in lower leg circumference as well as leg pain levels. No such changes were observed in healthy subjects.
CONCLUSIONS
This study provides evidence for the occurrence of orthostatic changes in muscle excitability in patients with neuropathic postural tachycardia syndrome and that these may be associated with inadequate perfusion of the lower extremities. Insufficient perfusion as a consequence of blood stasis may cause misery perfusion of the muscles, which could explain the occurrence of orthostatic leg pain in neuropathic postural tachycardia syndrome.
Topics: Heart Rate; Humans; Leg; Muscles; Pain; Postural Orthostatic Tachycardia Syndrome; Tachycardia; Tilt-Table Test
PubMed: 34674068
DOI: 10.1007/s10286-021-00830-5 -
American Journal of Medical Genetics.... Jun 2019Ethylmalonic encephalopathy (EE) is a rapidly progressive autosomal recessive mitochondrial disease caused by biallelic pathogenic variants in the ETHE1 gene that...
Ethylmalonic encephalopathy (EE) is a rapidly progressive autosomal recessive mitochondrial disease caused by biallelic pathogenic variants in the ETHE1 gene that encodes the mitochondrial sulfur dioxygenase. It is characterized by neurodevelopmental delay and regression, pyramidal and extrapyramidal signs, recurrent petechiae, chronic diarrhea, and orthostatic acrocyanosis. Laboratory findings include elevated serum levels of lactate and C4-C5 acylcarnitines, and elevated urinary excretion of ethylmalonic acid and C4-C6 acylglycines, notably isobutyrylglycine and 2-methylbutyrylglycine. These findings are attributed to deficiency of the mitochondrial sulfur dioxygenase resulting in toxic accumulation of hydrogen sulfide metabolites in vascular endothelium and mucosal cells of the large intestine. Medical management has thus far been directed toward decreasing the accumulation of hydrogen sulfide metabolites using a combination of metronidazole and N-acetylcysteine. More recently, orthotopic liver transplant (OLT) has been reported as a new therapeutic option for EE. Here, we report two additional cases of EE who achieved psychomotor developmental improvement after 7- and 22-months following OLT. The second case serves as the longest developmental outcome follow-up reported, thus far, following OLT for EE. This report provides additional evidence to validate OLT as a promising therapeutic approach for what was considered to be a fatal disease.
Topics: Biomarkers; Brain Diseases, Metabolic, Inborn; Consanguinity; Female; Humans; Infant; Liver Transplantation; Magnetic Resonance Imaging; Male; Mitochondrial Proteins; Mutation; Nucleocytoplasmic Transport Proteins; Phenotype; Purpura; Treatment Outcome
PubMed: 30864297
DOI: 10.1002/ajmg.a.61104