Prevalence and Contributing Factors of Orthostatic Hypotension in the Cardiology Department of the CHU Ignace Deen in Conakry

Introduction: Orthostatic Hypotension (OH) is a frequent situation during consultation in hypertensive patients. The prevalence in the general population increases with age and it is recommended to systematically search for it in patients with multiple comorbidities. The objective of this study was to determine the prevalence of orthostatic hypotension;describe their socio- demographic profile, the various clinical and paraclinical aspects and the predisposing factors of orthostatic hypotension in treated hypertensives, controlled or not. Method: This was a descriptive cross-sectional study, from January 2 to June 30, 2022 in the cardiology department of the Ignace Deen National Hospital. Included in this study were all patients who presented with orthostatic hypotension under antihypertensive treatment, regardless of age and sex, and who agreed to participate in the study. The non-inclusion criteria were hypertensive patients without OH and those who had not agreed to participate in the study. Each patient had blood pressure and heart rate measured in the supine position at room temperature and with an empty bladder. Then the measurement is taken again three (3) minutes after the switch to orthostatism. We retained the diagnosis of OH if the SBP drops by at least 20 mmHg and/or the PAD by at least 10 mmHg three (3) min after the transition to orthostatism. Our data obtained were analyzed in the Epi-info 7.4.0 software. Results: During our study period, we investigated 385 presented with OH, 12.2%. The mean age of the patients was 60.83 years ± 10.01 years and hypertensive patients, 47 of whom the M/F sex ratio was 0.81. In our study, blood pressure was not controlled in 36.2% of our patients with a predominance of grade 3 hypertension, a rate of 55.32%. Renal failure, anemia, heart failure and stroke were the main comorbidities associated with the occurrence of HO. The most incriminated factors were age with a frequency of 74.47%, followed by obesity at 44.68% and diabetes at 27.66%. BP was normally controlled with a rate of 68.8% in treated hypertensive patients with OH. It is found much more in patients using triple therapy, a rate of 61.71%. Conclusion: The prevalence of OH is high in our department. It is found much more in patients using triple therapy. The most incriminated factors were age followed by obesity and diabetes.

The association between orthostatic blood pressure changes and subclinical target organ damage in subjects over 60 years old

Background Although recent studies have indicated that both orthostatic hypotension and orthostatic hypertension independently predict cardiovascular events,the underlying mechanisms are still controversial.The aim of the study was to investigate the relationships between orthostatic changes and organ damage in subjects over 60 years old.Methods This is a prospective observational cohort study.One thousand nine hundred and ninety-seven subjects over 60 years old were enrolled.Participants were grouped according to whether they had a drop>20 mmHg in systolic or>10 mmHg in diastolic BP(orthostatic hypotension),an increase in mean orthostatic systolic blood pressure>20 mm Hg(orthostatic hypertension),or normal changes within 3 min of orthostatism.Multiple regression modeling was used to investigate the relationship between orthostatic hypotension,orthostatic hypertension and subclinical organ damage with adjustment for confounders.Results Orthostatic hypotension and orthostatic hypertension were found in 461(23.1%)and 189(9.5%)participants,respectively.Measurement of carotid intima-media thickness(IMT),brachial-ankle pulse wave velocity(baPWV),clearance of creatinine,and microalbuminuria were associated with orthostatic hypotension;measurement of IMT and baPWV were associated with orthostatic hypertension in a cruse model.After adjustment,IMT[odds ratio(OR),95%confidence interval(CI)per one-SD increment:1.385,1.052-1.823;P=0.02],baPWV(OR=1.627,95%CI:1.041-2.544;P=0.033)and microalbuminuria(OR=1.401,95%CI:1.002-1.958;P=0.049)were still associated with orthostatic hypotension,while orthostatic hypertension was only associated with IMT(OR=1.730,95%CI:1.143-2.618;P=0.009).Conclusions Orthostatic hypotension seems to be independently correlated with increased carotid atherosclerosis,arterial stiffness and renal damage in subjects over 60 years old.Orthostatic hypertension correlates with carotid atherosclerosis only.

What is the relationship between frailty and orthostatic hypotension in older adults?

Background Frailty and orthostatic hypotension (OH),which is common in older adults,is associated with morbidity and mortality.The relationship between them remains unclear.The aim of the study is to determine whether there is a relationship between frailty and OH.Methods A total of 496 patients who were admitted to the geriatric clinic and underwent comprehensive geriatric assessment were retrospectively reviewed.In a cross-sectional and observational study,OH was measured by the Head-up Tilt Table test at 1,3,and 5 min (respectively,OH1,OH3,and OH5) and the frailty was measured by the Fried’s frailty scale.Results The mean age of all patients was 75.4 ± 7.38.The prevalence of females was 69.8%.When the frail people were compared with the pre-frail and the robust ones,the frailty was associated with OH1.There was no relationship between the groups in terms of OH1 when the pre-frail group was compared with the robust group.OH3 were higher in the frail group than in the pre-frail group (P < 0.05) and the OH5 were higher in the frail group than in the pre-frail and robust group (P < 0.05),but OH3 and OH5 were not associated with frailty status when they were adjusted for age (P > 0.05).Slowness and weakness were associated with OH1 (P < 0.05),whereas the other components of the Fried’s test were not.Conclusions Frailty may be a risk factor for OH1.The 1^st min measurements of OH should be routinely evaluated in frail older adults to prevent OH-related poor outcomes.

Orthostatic hypotension,an often-neglected problem in community-dwelling older people:discrepancies between studies and real life

Orthostatic hypotension (OH)is a common condition in older persons.According to the 2011criteria,OH is defined by a sustained reduction of systolic blood pressure of 20 mmHg or a 10mmHg-fall of diastolic blood pressure,[1] Its prevalence increases with age and according to a recent systematic review the pooled prevalence in community dwelling older people is 22.2%.[2]Although OH is largely explained by concurrent clinical conditions (in particular, hypertension,diabetes,malnutrition,vascular encephalopa- thy,anemia,Parkinson's disease)[3],aging per se determines a series of physiological dysfunctions potentially representing its biological substratum (e.g.,reduction in barore- flex-mediated cardiovascular function,altered salt and water balance at renal level,impaired cardiac diastolic filling).It is also noteworthy that age-related changes in pharmacokinetic and pharmacodynamic mechanisms of drugs (especially in the context of polypharmacy)also increase the risk of OH.

Neurogenic orthostatic hypotension with Parkinson's disease as a cause of syncope:A case report

BACKGROUND Syncope presents with diagnostic challenges and is associated with high healthcare costs.Neurogenic orthostatic hypotension(nOH)as one cause of syncope is not well established.We review a case of syncope caused by nOH in a patient with Parkinson's disease.CASE SUMMARY We describe a case of syncope caused by nOH in Parkinson's disease and review the literature.A 70-year-old man with Parkinson's disease had uncontrolled blood pressure for 1 mo,with blood pressure ranging from 70/40 to 220/112 mmHg,and once lost consciousness lasting for several minutes after getting up.Ambulatory blood pressure monitoring indicated nocturnal hypertension(up to 217/110 mmHg)and morning orthostatic hypotension(as low as 73/45 mmHg).Seated-to-standing blood pressure measurement showed that the blood pressure dropped from 173/96 mmHg to 95/68 mmHg after standing for 3 min from supine position.A diagnosis of nOH with supine hypertension was made.During the course of treatment,Midodrine could not improve the symptoms.Finally,the patient's blood pressure stabilized with simple strategies by strengthening exercises,reducing the duration of lying in bed in the daytime,and consuming water intake before getting up.CONCLUSION nOH is one of the causes of syncope.Ambulatory blood pressure monitoring is a cost-effective method for its diagnosis,and non-pharmacological measures are still the primary management methods.

Reversible postural orthostatic tachycardia syndrome

Postural orthostatic tachycardia syndrome(POTS) is a relatively rare syndrome recognised since 1940. It is a heterogenous condition with orthostatic intolerance due to dysautonomia and is characterised by rise in heart rate above 30 bpm from base line or to more than 120 bpm within 5-10 min of standing with or without change in blood pressure which returns to base line on resuming supine position. This condition present with various disabling symptoms such as light headedness, near syncope, fatigue, nausea, vomiting, tremor, palpitations and mental clouding, etc. However there are no identifiable signs on clinical examination and patients are often diagnosed to have anxiety disorder. The condition predominantly affects young female between the ages of 15-50 but is rarely described in older people. We describe an older patient who developed POTS which recovered over 12 mo. Recognising this condition is important as there are treatment options available to alleviate the disabling symptoms.

Fu's subcutaneous needling for orthostatic hypotension due to Guillain-Barré syndrome: A case report

A 28-year-old female developed symptoms of frequent dizziness and occasional syncope while standing up,accompanied by paresthesia in her extremities and occasional constipation.She was diagnosed with Guillain-Barré syndrome(GBS).Her upright blood pressure was undetectable,and cerebrospinal fluid analysis showed protein-leukocyte separation.A nerve conduction study showed that the sensory nerves of her lower limbs had been injured,but the motor nerves were almost normal.She was treated with immunotherapy and hormonotherapy,which partially relieved her paresthesia and constipation.However,her orthostatic hypotension(OH) persisted after two months of treatment.After three courses of treatment consisting of Fu’s subcutaneous needling,OH significantly improved,with a much less pronounced decrease(<20 mmHg) in systolic blood pressure in the upright position than observed be fore treatment.She was sympto m-free and able to stand and walk for short distances after 3 months of follow-up.This case indicates that Fu’s subcutaneous needling treatment is effective for OH caused by GBS,and it may be a suitable alternative when conventional treatment does not produce ideal results.

Quantification of the Beneficial Effects of Compression Stockings on Symptoms of Exercise and Orthostatic Intolerance in Chronic Fatigue Syndrome/Myalgic Encephalomyelitis Patients

Chronic fatigue syndrome and myalgic encephalomyelitis (CFS/ME) are, amongst others, characterized by exercise intolerance, pain, post exertional malaise and orthostatic intolerance. It has been shown in venous disease and sport participation that compression stockings may improve exercise performance and reduce post exercise muscle soreness. Moreover, its use is advocated in orthostatic hypotension. Therefore, it was hypothesized that compression stockings may reduce symptomatology in CFS/ME patients. Methods: 100 patients used compression stockings class II for minimally 3 weeks and thereafter filled in a questionnaire, based on the Rand 36 physical activity questions (n = 9), whether compression stockings changed perceived symptoms or not. Moreover, 7 questions referring to prolonged standing and sitting, to recovery post exercise, muscle pain during or immediately post exercise, and to dizziness/light-headedness during or immediately post exercise, while standing and during prolonged sitting were added. Questions were scored as 1: able to perform activity much less while wearing the stockings, 2: perform activity somewhat less, 3: no perceived change in activity, 4: perform activity slightly better, 5: able to perform activity much better while wearing the stockings. Results: In patients able to answer the question, all mean scores per activity were significantly higher than 3, being no perceived change in activity while wearing the stockings. Subgroup analysis showed that patients with orthostatic intolerance reported higher effects than patients without orthostatic intolerance. Conclusion: This pilot study suggests that compression stockings may be useful to reduce symptomatology of physical activities in CFS/ME patients, especially in patients with orthostatic intolerance. Larger prospective studies with hard endpoints are warranted.

Value of Orthostatic Hypotension as a Prognostic Bed-Side Test in Heart Failure

Background: Neurohumoral compensatory mechanisms play an important role in stabilizing the functional activity of patients with heart failure using the arms of autonomic nervous system. Orthostatic Hypotension (OH) is one of the most incapacitating symptoms of Cardiac Autonomic Dysfunction (CAD). OH can include sympathetic withdrawal which in turn leads to marked disability and deterioration of heart failure symptoms. Progressive Autonomic Dysfunction (AD) associated with progressive deterioration and impact on mortality of many diseases as hypertension, diabetes and other chronic diseases. The idea of using (OH) as a bed-side simple test expecting the risk of deterioration of cardiac function and furthermore on mortality open a gateway for preventive medicine and care to these group of patients. For more confidential prove, studying subjective and objective factors in heart failure patients became necessary to support these idea. Methods and Results: Sixty-Four patients with known history of heart failure were collected. All patients taking the fixed regiment of 4 drugs (diuretic, ACE inhibitor, Digitalis and B-blocker) in appropriate tolerated doses for two weeks prior to the study. History taking and all routine investigations were done for all patients. Grouping is based upon wither they have (OH) or not. Group-A found to have normal Bp response to standing;they were 24 patients (18 male and 6 female) of mean Age (45 ± 8 years). Group-B discovered to have significant (OH) and was 22 patients (16 males and 6 females) of mean Age (43 ± 4 years). The first Clinical and Echocardiographic examination was done and considered as a base-line characteristic. Then, a Call-back after 6 months for follow-up and second visit examination is recorded. Furthermore, every patient was advised to report changes in his clinical symptoms in a note-book describing five main items to answer a questionnaire at the end of the study involves [times of admission to hospital, need for treatment modification, numbers of paroxysmal nocturnal dysnea, numbers of arrhythmic episodes and manifest lower limb edema]. At the end of the study, statistical methods are used to clarify the changes in their data and detect deterioration of cardiac functions by Echocardiographic results and their answers to the questionnaire. In the first visit, comparison of demographic, clinical and Echocardiographic data revealed no significant variations—odd values are excluded and the remaining 46 patients are then tested for their Bp response to standing and classified into two groups. Group-A (normal Bp response) and Group-B (having orthostatic hypotension). Group-A were 24 patients (18 male and 6 female) of mean Age (45 ± 8 years). Group-B were 22 patients (16 males and 6 females) of mean Age (43 ± 4 years). In the second visit (6-month later), divergence of data is observed and was statistically significant. Group-B was found to have a lower EF% and FS% (p = 0.01), a lower Dp/Dt (p = 0.01) and a higher Tie-Index and MR-jet area (p = 0.01). This means that, patients having orthostatic hypotension underwent significant deterioration of cardiac functions after a period of six-months. Indeed, the questionnaire proved frequent times of hospital admissions, paroxysmal nocturnal dysnea, need for treatment modification, arrhythmias and lower limb edema in group-B. Discussion: The present study conclude that, heart failure-patients having

Orthostatic Intolerance and Coagulation Abnormalities:An Update

Orthostatic intolerance(OI)is a series of clinical symptoms that develop during long-term standing in the upright position,with clinical manifestations of frequent,recurrent,or persistent dizziness,fatigue,and heart palpitations with or without syncope.

Alcohol intolerance and myalgic encephalomyelitis/chronic fatigue

BACKGROUND The literature is mixed about the occurrence of alcohol intolerance among patients with myalgic encephalomyelitis/chronic fatigue syndrome(ME/CFS).Surveys that asked respondents with ME/CFS whether they experienced alcohol intolerance within a recent time frame might produce inaccurate results because respondents may indicate that the symptom was not present if they avoid alcohol due to alcohol intolerance.AIM To overcome this methodologic problem,participants in the current study were asked whether they have avoided alcohol in the past 6 mo,and if they had,how severe their alcohol intolerance would be if they were to drink alcohol.METHODS The instrument used was a validated scale called the DePaul symptom questionnaire.Independent t-tests were performed among the alcohol intolerant or not alcohol intolerant group.The alcohol intolerant group had 208 participants,and the not alcohol intolerant group had 96 participants.RESULTS Using specially designed questions to properly identify those with alcohol intolerance,those who experienced alcohol intolerance vs those who did not experience alcohol intolerance experienced more frequent/severe symptoms and domains.In addition,using a multiple regression analysis,the orthostatic intolerance symptom domain was related to alcohol intolerance.CONCLUSION The findings from the current study indicated that those with ME/CFS are more likely to experience alcohol intolerance.In addition,those with this symptom have more overall symptoms than those without alcohol intolerance.

Cardiac autonomic neuropathy: Risk factors, diagnosis and treatment

Cardiac autonomic neuropathy(CAN)is a serious complication of diabetes mellitus(DM)that is strongly associated with approximately five-fold increased risk of cardiovascular mortality.CAN manifests in a spectrum of things,ranging from resting tachycardia and fixed heart rate(HR)to development of"silent"myocardial infarction.Clinical correlates or risk markers for CAN are age,DM duration,glycemic control,hypertension,and dyslipidemia(DLP),development of other microvascular complications.Established risk factors for CAN are poor glycemic control in type 1 DM and a combination of hypertension,DLP,obesity,and unsatisfactory glycemic control in type 2DM.Symptomatic manifestations of CAN include sinus tachycardia,exercise intolerance,orthostatic hypotension(OH),abnormal blood pressure(BP)regulation,dizziness,presyncope and syncope,intraoperative cardiovascular instability,asymptomatic myocardial ischemia and infarction.Methods of CAN assessment in clinical practice include assessment of symptoms and signs,cardiovascular reflex tests based on HR and BP,short-term electrocardiography(ECG),QT interval prolongation,HR variability(24 h,classic24 h Holter ECG),ambulatory BP monitoring,HR turbulence,baroreflex sensitivity,muscle sympathetic nerve activity,catecholamine assessment and cardiovascular sympathetic tests,heart sympathetic imaging.Although it is common complication,the significance of CAN has not been fully appreciated and there are no unified treatment algorithms for today.Treatment is based on early diagnosis,life style changes,optimization of glycemic control and management of cardiovascular risk factors.Pathogenetic treatment of CAN includes:Balanced diet and physical activity;optimization of glycemic control;treatment of DLP;antioxidants,first of allα-lipoic acid(ALA),aldose reductase inhibitors,acetylL-carnitine;vitamins,first of all fat-soluble vitamin B1;correction of vascular endothelial dysfunction;prevention and treatment of thrombosis;in severe cases-treatment of OH.The promising methods include prescription of prostacyclin analogues,thromboxane A2 blockers and drugs that contribute into strengthening and/or normalization of Na^+,K^+-ATPase(phosphodiesterase inhibitor),ALA,dihomo-γ-linolenic acid(DGLA),ω-3 polyunsaturated fatty acids(ω-3 PUFAs),and the simultaneous prescription of ALA,ω-3 PUFAs and DGLA,but the future investigations are needed.Development of OH is associated with severe or advanced CAN and prescription of nonpharmacological and pharmacological,in the foreground midodrine and fludrocortisone acetate,treatment methods are necessary.

Acute complications of spinal cord injuries

The aim of this paper is to give an overview of acute complications of spinal cord injury(SCI). Along with motor and sensory deficits, instabilities of the cardiovascular, thermoregulatory and broncho-pulmonary system are common after a SCI. Disturbances of the urinary and gastrointestinal systems are typical as well as sexual dysfunction. Frequent complications of cervical and high thoracic SCI are neurogenic shock, bradyarrhythmias, hypotension, ectopic beats, abnormal temperaturecontrol and disturbance of sweating, vasodilatation and autonomic dysreflexia. Autonomic dysreflexia is an abrupt, uncontrolled sympathetic response, elicited by stimuli below the level of injury. The symptoms may be mild like skin rash or slight headache, but can cause severe hypertension, cerebral haemorrhage and death. All personnel caring for the patient should be able to recognize the symptoms and be able to intervene promptly. Disturbance of respiratory function are frequent in tetraplegia and a primary cause of both short and long-term morbidity and mortality is pulmonary complications. Due to physical inactivity and altered haemostasis, patients with SCI have a higher risk of venous thromboembolism and pressure ulcers. Spasticity and pain are frequent complications which need to be addressed. The psychological stress associated with SCI may lead to anxiety and depression. Knowledge of possible complications during the acute phase is important because they may be life threatening and/ or may lead to prolonged rehabilitation.

Tilt table test today-state of the art

A tilt table test(TTT) is an inexpensive, noninvasive tool for the differential diagnosis of syncope and orthostatic intolerance and has good diagnostic yield. The autonomic system malfunction which underlines the reflex syncope is manifested as either hypotension or bradycardia, while an orthostatic challenge is applied. The timing of the response to the orthostatic challenge, as well as the predominant component of the response help to differentiate between various forms of neurocardiogenic syncope, orthostatic hypotension and non-cardiovascular conditions(e.g., pseudosyncope). Medications, such as isoproterenol and nitrates, may increase TTT sensitivity. Sublingual nitrates are easiest to administer without the need of venous access. TTT can be combined with carotid sinus massage to evaluate carotid sinus hypersensitivity, which may not be present in supine position. TTT is not useful to access the response to treatment. Recently, implantable loop recorders(ILR) have been used to document cardioinhibitory reflex syncope, because pacemakers are beneficial in many of these patients, especially those over 45 years of age. The stepwise use of both TTT and ILR is a promising approach in these patients. Recently, TTT has been used for indications other than syncope, such as assessment of autonomic function in Parkinson's disease and its differentiation from multiple system atrophy.

Atypical neurological symptoms associated with CGG expansions of the FMR1 gene

More than 40 CGG expansions in the 5' noncoding region of the fragile X mental retardation 1(FMR1)gene of the X chromosome give rise to several distinct clinical phenotypes, depending on the size of the expansion. First, more than 200 CGG expansions(full mutation) cause an inherited mental retardation called fragile X syndrome. Second, CGG expansions between55 and 199(premutation) cause a disorder called fragile X-associated tremor/ataxia syndrome(FXTAS) which typically includes intention tremor, ataxia and specific magnetic resonance imaging(MRI) findings. Indeed, it could develop parkinsonism although it usually shows features of postsynaptic parkinsonism. Finally, CGG expansions between 41 and 54 CGG(gray zone) are not consider normal but rarely develops abnormal neurological conditions. In this sense, the aim of this study is to report two atypical cases associated with CGG expansions of the FMR1 gene. First, a FMR1 premutation alleles carrier with an unusual phenotype, such as a presynaptic parkinsonism indistinguishable from Parkinson disease(PD) and a FMR1 gray zone alleles carrier presented with neurological features, namely hand tremor, parkinsonism and ataxia, usually described in FXTAS, as well as orthostatic tremor. We conclude that,on the one hand, FMR1 premutation alleles might cause two phenotypes of parkinsonism, such as a presynaptic phenotype, indistinguishable from PD, and a postsynaptic phenotype, associated with clinical features of FXTAS. On the other hand, although FMR1 gray zone alleles carriers were believed to have no abnormal neurological conditions, our study supports that they could develop FXTAS and other neurological disorders such as orthostatic tremor which has not been reported before associated with the FMR1 gene.

Blood Volume Status in Patients with Chronic Fatigue Syndrome: Relation to Complaints

Four studies have compared a possible decrease in circulating blood volume in Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) patients when compared to a healthy population. A more recent study has proven a correlation between RBC volume and OI in chronic OI patients without being diagnosed ME/CFS. The aim of the present study was to relate measured blood, RBC and plasma volumes (absolute and percent normalized) with the orthostatic intolerance complaints in ME/CFS patients. In the included 11 female ME/CFS patients, percentage decrease in normalized blood, RBC and plasma volume was similar for all three components: 83% &plusmn;12%, 83% &plusmn;12% and 83% &plusmn;11%, respectively. In patients with a clinical suspicion of OI (n = 7) all 3 volume components were significantly lower compared to patients without clinical suspicion of OI (n = 4). The difference percentage to normalized Blood volume was 77(7) vs 94(10) (p-value < 0.02), difference percentage to normalized RBC volume was 76(7) vs 96(10) (p-value < 0.01) and difference percentage to normalized plasma volume was 77(7) vs 93(10) (p-value < 0.05) in OI present versus absent. Plasma volumes were plotted against RBC volumes: the relation found was RBC volume = 0.99* Plasma volume + 1.55;p < 0.001;r = 0.90. In line with literature data, this pilot study shows that total blood volume and its components: RBC and plasma volume may be reduced in ME/CFS patients, especially in the presence of a clinical suspicion of OI.

Perioperative Care in Patients with Ehlers Danlos Syndromes

Background: There is an increasing recognition of patients with Ehlers Danlos Syndromes. The laxity of the ligaments and the weakness of the connective tissue has resulted in increasing number of patients requiring surgical intervention. Ehlers Danlos Syndromes are not about hypermobile joints only, they are associated with multiple co-existing conditions such as Chiari malformation, Tethered Cord Syndrome, spinal instability, abdominal pain, Dysautonomia and Mast Cell Activation Syndrome. The combined incidence of Ehlers Danlos Syndromes is 1 in 5000 people. Most experts believe that the actual incidence is much higher. Many of these cases are under-diagnosed. Nevertheless, patients with Ehlers Danlos Syndromes, diagnosed or undiagnosed often require surgical intervention. This review article has been written to shed light on the need for special consideration during anesthesia. Objectives: Our objective was to conduct a review of anesthetic considerations in patients with Ehlers Danlos Syndromes. Study Design: We used a narrative review design. Methods: This review was done using searches of PubMed, MEDLINE/OVID, SCOPUS, and manual searches of the bibliographies of known primary and review articles from inception to 2019. Other data sources included hand searches of publications driven by manuscript authors. Search terms included concepts of “Ehlers Danlos Syndrome”, “EDS”, “pain”, “anesthesia”, “surgery” and combination of terms. Search method was not restricted to any one language. Results: Articles were screened by title, abstract, and full article review. They were then analyzed by specific clinical indications and appropriate data was presented based on critical analysis of those articles. Limitations: More studies about the effect of anesthetic techniques and Ehlers Danlos Syndromes are required. Conclusions: Patients with Ehlers Danlos Syndromes may present with an array of coexisting medical conditions such as Dysautonomia, Mast Cell Activation Syndrome, Chiari Malformation, Tethered Cord Syndrome, Craniocervical instability, Gastroparesis, altered sensitivity to local anesthetics. Anesthetic techniques need to be modified according to the presentation. Preoperatively, a note should be made of the joints that sublux most often, craniocervical instability. They are prone to temporomandibular joint subluxation, obstructive sleep apnea and tracheomalacia, spontaneous pneumothorax, POTS, MCAS, insensitivity to local anesthetics and coagulation disorders. Intraoperative considerations should include fiberoptic intubation, IV fluid loading for POTS, avoid drugs that release histamine, unpredictable response to opioids, precautions for gastroparesis. Patient should be positioned to avoid joint subluxation and over stretching nerves. Postoperative considerations include maintaining hemodynamic stability, avoid exacerbation of symptoms of MCAS, high suspicion of occult bleeding, high risk of post dural puncture headache in patients undergoing spinal procedure or neuroaxial block.

Spontaneous Intracranial Hypotension: An Interesting Cause of Intractable Headache

Intracranial hypotension (ICH) is a benign syndrome which is often under-diagnosed. It is characterized by orthostatic headache which is predominantly occipital. ICH is diagnosed in the presence of a typical history and characteristic imaging findings. Further confirmation by lumbar puncture to document low CSF pressure might be necessary in some cases. Treatment is mainly conservative in the form of bed rest and intravenous saline infusion. However, surgical intervention may be required if conservative measures fail. In this report we presented a case of 42-year-old male patient who presented with symptoms of orthostatic occipital headache of three-month duration and was subsequently diagnosed with intracranial hypotension based on characteristic MRI findings of pachymeningeal enhancement on gadolinium enhanced MRI of the brain with sagging of the mid-brain.

A Case Report of Hepatopulmonary Syndrome

Hepatopulmonary syndrome (HPS) is a severe complication of end-stage hepatic disease. However, the majority of HPS patients present with non-specific clinical manifestations and imaging features. They are constantly misdiagnosed as interstitial pneumonia, pulmonary infection or heart failure and appropriate treatment is thus delayed. In this report, clinical manifestations, diagnosis and treatment of one case of HPS were retrospectively analyzed and literature review was conducted. When the heapatic failure liver disease patients present with hypoxemia, pulmonary angiography, contrast echocardiography for right-sided heart (contrast enhanced echocardiogram) and lung perfusion scan (99mTc-MAA) should be done to exclude HPS. Orthotopic liver transplantation serves as the unique effective therapy of HPS.

Body Fluid Changes, Cardiovascular Deconditioning and Metabolic Impairment Are Reversed 24 Hours after a 5-Day Dry Immersion

Dry immersion is an effective and useful model for research in physiology and physiopathology. The focus of this study was to provide integrative insight into renal, endocrine, circulatory, autonomic and metabolic effects of dry immersion. We assessed if the principal changes were restored within 24 h of recovery, and determined which changes were mainly associated with immersion-induced orthostatic intolerance. Five-day dry immersion without countermeasures, and with ad libitum water intake, standardized diet and a permitted short daily rise was performed in a relatively large sample for this experiment type (14 healthy young men). Reduction of total body water derived mostly from extracellular compartment, and stabilized rapidly at the new operating point. Decrease in plasma volume was estimated at 20% - 25%. Five-day immersion was sufficient to impair metabolism with a decrease in glucose tolerance and hypercholesterolemia, but was not associated with pronounced autonomic changes. Five-day immersion induced marked cardiovascular impairment. Immediately after immersion, over half of the subjects were unable to accomplish the 20-min 70° tilt;during tilt, heart rate and total peripheral resistance were increased, and stroke volume was decreased. However, 24 hours of normal physical activity appeared sufficient to reverse orthostatic tolerance and all signs of cardiovascular impairment, and to restitute plasma volume and extracellular fluid volume. Similarly, metabolic impairment was restored. In our study, the major factor responsible for orthostatic intolerance appeared to be hypovolemia. The absence of pronounced autonomic dysfunction might be explained by relatively short duration of dry immersion and daily short-time orthostatic stimulation.