Life-threatening Hypertension Research Articles

THU578 Concept: Role Of Spiral Steroids (SS) During Pregnancy

Abstract Disclosure: F.I. Chasalow: None. Background: Earlier, my laboratory reported 3 novel endogenous steroids (SS) that cross react with digoxin-specific antibodies (DLM). The SS are readily distinguished from all other steroids by 3 characteristics: [1] each is a phosphocholine diester, [2] each has more than 21 carbon atoms in the steroid fragment and [3] the extra carbons form an E-ring with a cyclic lactone. The discovery included a biosynthetic scheme with known enzymes and no need for a ‘magic’ step. Function: NaK-ATPase has 3 subunits, designated FXYD, α, and β. Affinity for Na+ and K+ ions is regulated by FXYD proteins. Each SS matches a tissue-specific α-subunit. Each α-subunit has 2 different binding sites for steroids. One site binds a SS; the ligand of the other site is unknown. For example, Ionotropin is specific for the α subunit present in cardiac and renal tissue. Kaleotropin is isolated from gonads, but the α subunit which matches each specific ovarian cell type is unknown. Galotropin (G-trop): One of the SS, G-trop, was detected as a DLM in human breast cyst fluids, specifically cysts with high K+ ion levels. Solubility properties distinguished it from previously known cardiac glycosides and, like other DLM, it inhibited NaK-ATPase. G-trop was also detected in milk from cows, sheep and goats. Ions derived from Ionotropin (m/z=341 Da) or Kaleotropin (m/z=369 Da) were not present in milk extracts. On LC-MS, G-trop fragmented to an ion at m/z= 381 Da. Trial and error analysis suggests C25O4H34. No other formula is consistent with m/z=381 Da. G-trop: G-trop wasn’t present in serum from children. At 22-26 weeks of gestation, whether or not a pregnant woman had pre-eclampsia, serum had low levels of G-trop. Maternal G-trop increases during the 3rd trimester, coincidentally with initiation of milk production; simultaneously, K+ ion levels in milk increased. Pre-eclampsia. In a pilot study, 65% of patients (n=20) with pre-eclampsia had elevated levels of Ionotropin precursor and 30% had elevated levels of Kaleotropin precursor. 25% of patients with pre-eclampsia develop life-threatening hypertension or seizures, typical of the expected response to elevated Ionotropin levels. Therapy includes immediate delivery of the infant at 30-36 weeks of gestation. Maternal G-trop synthesis has not yet begun and mother’s milk has low K+ ion levels. Elevated maternal Kaleotropin is not the cause of maternal hypertension, suggesting a specific gestational maternal α-subunit is its target. Kaleotropin from placenta may be the signal to respond to maternal hypokalemia. Necrotizing enterocolitis (NEC):In utero the fetus does not absorb K+ from the GI tract because fetal need for K+ ion is provided via the placenta. Concept: After premature delivery, without G-trop in milk, the infant can’t absorb K+ from the GI tract; the GI tract fails to grow and tight junctions do not form. As a result, infants are at high risk for developing NEC. Presentation Date: Thursday, June 15, 2023

Coats plus syndrome: a rare cause of severe gastrointestinal tract bleeding in children \u2013 a case report

BackgroundCoats plus syndrome, cerebroretinal microangiopathy with calcifications and cysts, is a rare disease with autosomal recessive pattern occurring due to a mutation in CTC1, encoding conserved telomere maintenance component 1, gene. Besides retinal involvement, abnormalities in brain and osteopenia, serious life-threatening bleeding in gastrointestinal tract and portal hypertension can be observed.Case presentationA 6-year-old girl with Coats plus syndrome presented to the pediatric emergency department with vomiting blood and blood in stool. An upper and lower gastrointestinal endoscopy revealed esophageal varices and vascular telangiectasia in the pyloric antrum, duodenum, and colon. She received palliative care and the bleeding was stopped after receiving intravenous octreotide. She then was followed in the pediatric gastroenterology, neurology, and ophthalmology clinics. She was later hospitalized and admitted to the intensive care unit as she continued to have intermittent gastrointestinal system bleeding. She eventually died due to severe gastrointestinal system bleeding.ConclusionsCoats plus syndrome can lead to life-threatening gastrointestinal bleeding and portal hypertension. As Coats plus syndrome is quite rare, there is little published data on this syndrome. This report presents a case of Coats plus syndrome as a rare cause of gastrointestinal bleeding and portal hypertension.

Bilateral adrenal pheochromocytomas in a 14 year-old boy

Abstract Introduction Pheochromocytomas are catecholamine-secreting tumors arise from chromaffin cells of the adrenal medulla. Classical symptoms include palpitations, headaches, sweating and paroxysmal hypertension as a result of elevated catecolamine production. This case report aims to describe the clinical presentation, management and outcome of the phechromocytomas. Case A 14 year-old boy came with hypertensive crisis (blood pressure 220/140 mmHg), recurrent tension headache, excessive sweating, palpitation and weight loss during the last 1 year. Twenty four hour urinary catecholamine level was normal. Abdominal CT scan confirmed the suspicion of pheochromocytoma. After having preoperative treatment with nicardipine infusion, oral nifedipine and captopril, bilateral cortical sparing adrenalectomy was done. Nicardipine infusion was administered to maintain the blood pressure during surgery. Histopathology confirmed the diagnosis of adrenal pheochromocytoma Adrenocortical was preserved. Complete resolution of hypertension was achieved gradually after one month. Conclusion Pheochromocytomas is a rare disease which may lead to a life-threatening hypertension as a result of elevated catecolamine production. Initial recognition and appropriate management of pheochromocytomas are important. Preoperative management to normalize blood pressure followed by tumor removal has proved to be the ideal choice for good prognosis. Multidisciplinary team during preoperative, intraoperative and postoperative are required for successful outcome.

MON-LB065 A Norepinephrine-Only Secreting Pheochromocytoma in the Absence of an Identifiable Genetic Defect

Introduction - Pheochromocytoma is a rare catecholamine-secreting tumor with an incidence as low as 0.8 per 100,000 individuals. Despite the rarity of pheochromocytoma, the availability of definitive treatment makes early diagnosis crucial to avoid life-threatening hypertension. The objective of this report is to describe a case of norepinephrine-only-secreting pheochromocytoma. Case Description - A 33-year-old female with a medical history of hypertension presented with complaints of acute, left-sided chest pain and palpitation. She also reported intermittent dizziness and headache for previous one week. Vital signs pertinent for blood pressure 160/80mmHg and physical exam was unremarkable. Initial evaluation with chest radiograph showed an 8.8x8.3cm peripherally calcified mass in the left upper quadrant. Subsequent computed tomography of the abdomen revealed a 10cm heterogeneous left adrenal mass, size concerning for malignancy, with central necrosis and calcification. Hormonal workup for adrenal mass resulted with normal serum testosterone, DHEA-S, morning cortisol, ACTH, aldosterone, plasma epinephrine, and dopamine levels, but profoundly elevated plasma norepinephrine 4124 (80-520) pg/mL. Her 24-hour urinary metanephrine levels were normal but with elevated normetanephrine 24,364 (88-444) ug/24hr, confirming a pheochromocytoma. The normal metanephrine level in the setting of profoundly elevated normetanephrine raised concern for Von Hippel Lindau (VHL). Ultrasound-neck showed benign, bilateral cystic nodules and her thyroid function tests, serum calcitonin were unremarkable. Oncologic workup showed confinement of tumor to the left adrenal. She was started on high salt and high fluid diet. After adequate alpha blockage, the beta-blocking drug was initiated and she underwent robotic laparoscopic removal of the left adrenal gland. The surgical pathology report confirmed benign, 9.8cm pheochromocytoma confined to left adrenal. She was started on corticosteroid postoperatively given relative adrenal insufficiency, but with anticipation of eventual steroid wean. The patient had complete resolution of symptoms and remains clinically stable. Conclusion - Pheochromocytomas arise from chromaffin cells of the adrenal medulla. Although commonly sporadic in nature, a myriad of familial syndromes are associated with pheochromocytoma. The characteristic feature of VHL-associated pheochromocytoma is almost exclusive secretion of normetanephrine and high normetanephrine to metanephrine ratio as seen in this case. Gene testing and confirmation helps with appropriate surveillance and follow up. This case is unique due to the benign pathology despite the large size and negative paraganglioma-pheochromocytoma genetic panel, despite the presence of characteristic features of VHL-associated pheochromocytoma. Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. s presented at a news conference are embargoed until the date and time of the news conference. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.

A custom made interface to integrate recording of pulse rate and blood pressure during urodynamics investigations

Patients with a spinal cord injury above the 6th thoracic vertebrae may be prone to autonomic dysreflexia (AD) in response to bladder stimulus associated with a urodynamics investigation. It is essential that these patients are managed carefully in the urodynamics clinic in order to prevent life-threatening hypertension and bradycardia. Part of this management is the measurement and manual recording of pulse rate (PR) and non-invasive blood pressure (NIBP), alongside the standard urodynamics data set. The purpose of recording these additional data is to identify the characteristic drop in PR and rise in NIBP that indicates the onset of AD. This technical note describes the development of a novel, in-house constructed interface that allows PR and NIBP to be recorded alongside the standard urodynamics data set, using a commonly available vital signs monitor and urodynamics workstation.

INCREASE IN BLOOD PRESSURE: THE UNUSED RESOURCES AMBULANCE

91104 calls were analyzed for the period of 10 years, the occurrence of hypertension increased from 324,3 to 423,8 cases for 10000 inhabitance/year. The majority of calls were multiple and undertaken to the same patients suffered from moderate, not life-threatening hypertension. To decrease the number of such callswe considered the possibility to implement self-care programs for these group of patients. Prehospital advanced treatments included moxodonine administration, and its combination with furosemide, or nifedipine, gives the possibility to develop the treatment plan exactly “on the field”, with the evidence of safety and efficacy for health care provider and his patient.

Management of life-threatening hypertension in a 12-year-old bichon frise undergoing an adrenalectomy for phaeochromocytoma excision

A bichon frise, previously diagnosed with a phaeochromocytoma, underwent phenoxybenzamine treatment 17 days before adrenalectomy. Preoperative haematology, biochemistry and oscillometric blood pressure readings for the 12 year old were typical....

Analysis of the management of hypertension complications in patients during emergency care

To analyze the management of patients with associated complications from hypertension to improve prehospital therapeutic and diagnostic care. The accounts and records (125,905 calling cards over 2012-2014) of the Statistics Division, Karaganda Regional First Aid Station, were used. The methods of clinical and mathematical analysis were applied. There was an annual increase in the number of calls for all cases of hypertension complications in relation to the higher rate of hospitalization by 2014. The causes of non-100% hospitalization for life-threatening hypertension complications were revealed. Incomplete diagnostic data were one of the reasons for delayed diagnosis and development of prehospital complications from hypertension. When emergency care was rendered, differentiation therapy for hypertension complications was not always withstood; the use of drugs was noted to be unwarranted and contrary to the data of evidence-based medicine. The management of patients with associated complications from hypertension does not fully comply with the Diagnosis and Treatment Protocols of the Ministry of Health of the Republic of Kazakhstan.

Pheochromocytoma with Ventricular Tachycardia as the Presenting Symptom

To the Editor: Although the triad of palpitations, headaches, and sweating is generally recognized as the classic presentation for pheochromocytoma, we reported the case of a patient with pheochromocytoma who first presented with ventricular tachycardia (VT). This case shows the importance of a broad differential diagnosis when the clinical presentation is unusual. A 17-year-old woman presented with episodic palpitation and headaches during physical activities for 4 years. The symptoms resolved after 3–5 min of rest. Two days before admission, she was admitted to the emergency department with persistent palpitations and headaches, and the blood pressure (BP) was 180/90 mmHg. The admission echocardiography (ECG) revealed a sinus rate of 92 beats/min. There was no family or personal history of syncope, cardiac arrest, and sudden death. The physical examination was unremarkable. The admission and peak creatine kinase-MB was 153 U/L and 157 U/L, respectively. During follow-up, the patient presented with recurrent palpitation and headaches when sitting or standing. During these episodes the BP was 160–200/90–100 mmHg and the pulse rate was elevated at 148 beats/min. Cardiac monitoring showed VT with a heart rate (HR) of 150 beats/min [Figure 1a]. Three episodes of VT were recorded during persistent hypertension. The symptoms were relieved after maintaining a recumbent position for 1–2 min. The 24 h urinary catecholamines and urinary vanillylmandelic acid levels were elevated. Abdominal ultrasound revealed a right adrenal mass with a diameter of 6 cm which was confirmed on abdominal computed tomography [Figure 1b]. A pheochromocytoma was diagnosed. Figure 1 (a) Echocardiography showing ventricular tachycardia. (b) Contrast-enhanced computed tomography scan of the upper abdomen revealing a tumor of the right adrenal gland. The patient was hemodynamically stabilized in the supine position with the administration of phentolamine (a nonspecific α-adrenergic blocker) and metoprolol (a selective β-antagonist). A right adrenalectomy was performed, yielding a 6.0 cm nodule confirmed to be a pheochromocytoma by histology. The hypertension was controlled and no arrhythmias were documented during 12 months of follow-up. Pheochromocytoma is a catecholamine-producing tumor that originates from the adrenal medulla or extra-adrenal chromaffin cells. The main clinical manifestation of pheochromocytoma is paroxysmal or persistent hypertension, accompanied by arrhythmias, such as sinus tachycardia, paroxysmal supra VT, atrial fibrillation, or ventricular premature contractions. We have reported a rare case of pheochromocytoma with VT as the presenting symptom. Frequent episodes of VT are almost always preceded by vital sign fluctuation (elevated HR and BP). Paulin et al.[1] reported that the sudden release of catecholamine from the pheochromocytoma is associated with the mechanism underlying VT. Excessively elevated catecholamine levels could lead to abnormal myocardial electrical activity, such as over opening of ion channels, enhanced ion exchange pump function, thus causing extremely large amounts of sodium, potassium, and calcium ion flow through the membranes, resulting in intensive myocardial autorhythmicity, conductivity, and triggered activities. Furthermore, increased catecholamine levels can reduce the cardiac ventricular fibrillation threshold, predispose to ventricular fibrillation, lead to sudden death,[2] and contribute to abnormal repolarization, thus causing all types of rapid arrhythmia. Ceremuzynski et al.[3] have shown that plasma catecholamine concentrations are increased in dogs during acute myocardial ischemia and a relationship between the blood catecholamine concentration and the severity of ventricular arrhythmias. Cardiac abnormalities caused by pheochromocytoma includes myocarditis, acute myocardial infarction, transient left ventricular systolic dysfunction, and cardiomyopathy. Bravo et al.[4] reported that catecholamines cause myocardial necrosis, focal myofibrillar degeneration, and subsequent fibrous scar formation. Left ventricular systolic dysfunction may resemble stress-induced cardiomyopathy, also known as takotsubo cardiomyopathy and is reversible in patients with pheochromocytoma.[5] Moreover, long-term high catecholamine levels can lead to myocardial hypertrophy and heart failure. The presence of sustained VT can be treated with radio-frequency ablation but such treatment would not abolish the systemic arrhythmogenic mechanism and would not resolve the symptoms or prevented the potential onset of other tachyarrhythmias. This case illustrates the importance of a complete differential diagnosis when the clinical presentation is unusual. In a hypertensive patient with a typical presentation consisting of palpitations and headache, and an ECG demonstrates VT or other tachyarrhythmias, a pheochromocytoma should be included in the differential diagnosis. In addition, biochemical testing and imaging studies will guide the diagnosis and allow complete resolution of symptoms following resection of the pheochromocytoma. The preoperative use of phentolamine, a nonspecific α-adrenergic blocker has a theoretical pharmacologic basis. The β-blocker propranolol is used when tachycardia or catecholamine-induced arrhythmias are present and should only be started after α-blocker has been established to avoid life-threatening hypertension. In conclusion, this case shows that VT is an unusual and initial cardiovascular presenting symptom in patients with a pheochromocytoma. Pheochromocytoma must be considered in patients with frequent VT plus sudden changes in BP. Resection of chromaffin cell tumor will completely relieve the symptoms. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Passive Hind-Limb Cycling Reduces the Severity of Autonomic Dysreflexia After Experimental Spinal Cord Injury

Background. Spinal cord injury (SCI) induces alterations in cardio-autonomic control of which autonomic dysreflexia (AD), a condition characterized by life-threatening hypertension, is arguably the most insidious. Passive hind-limb cycling represents a low-cost therapeutic intervention with demonstrable cardiovascular, sensory, and motor benefits. Objective. To investigate the effect of passive hind-limb cycling on AD in rodents with T3 SCI. Methods. Forty-five male Wistar rats were evenly assigned to either uninjured control (CON), SCI, or SCI plus hind-limb cycling exercise (SCI-EX). At the end of the experimental period (day 32), rats were randomly assigned to stream 1 (n = 24) or stream 2 (n = 21). Stream 1 rats were assessed for AD severity (pressor response to colorectal distension) and were then perfused for tissue dissection and immunohistochemistry. Stream 2 rats underwent excision of the superior mesenteric artery for in vitro myography assessments. Results. From 2 weeks post-SCI onwards, SCI-EX rats exhibited a significant reduction in the pressor response to colorectal distension versus SCI (P < .001). Reduced AD severity in SCI-EX rats was accompanied by a prevention of the SCI-induced increase in density of CGRP+ afferents in the dorsal horn (P = .001). Conversely, both SCI and SCI-EX rats exhibited a similar degree of mesenteric endothelial dysfunction and α-adrenoceptor hypersensitivity versus CON. Conclusion. Passive hind-limb cycling reduces the severity of AD in SCI, and is correlated with changes in primary afferent morphology, but has limited effects on the peripheral vasculature.

SURGICAL MANAGEMENT OF TYPE B AORTIC DISSECTION AND PHEOCHROMOCYTOMA

Arterial hypertension is the most important risk factor for aortic dissection. Arterial hypertension has been found in approximately 80% of aortic dissection patients. Typically, arterial hypertension is long-standing and poorly regulated in aortic dissection patients. A pheochromocytoma is a rare, catecholamine-secreting tumour that may precipitate life-threatening hypertension. The tumour is malignant in 10% of cases but may be cured completely by surgical removal. We present the case of a 49-year-old patient, heavy smoker, hypertensive, who addressed the hospital in the context of acute interscapulovertebral pain (November 2014). After a contrast agent CT scan of the thorax + upper abdomen + pelvis, a type B thoracic aortic dissection was observed, without subdiaphragmatic extension or extension to the supraaortic trunks, without mediastinal effraction, and a right adrenal tumour of approximately 3.6 cm was also detected. The patient was directed to the cardiovascular surgery service, where an endoprosthesis with Vaillant prosthesis of the descending aorta and subclavian carotid bypass were performed, with good postoperative evolution. The patient was then referred to The National Institute of Endocrinology "C.I. Parhon" for further investigations required prior to the adrenal surgery. The suspicion of pheochromocytoma was confirmed by urinary metanephrine and normetanephrine dosing, and adrenal surgery was recommended. The patient was placed under alpha and beta blocker treatment for the prevention of hypertensive flares and any potential arrhythmias. Surgical intervention was performed for adrenal tumour resection. Histopathological examination of the surgical sample confirmed the diagnosis of pheochromocytoma. The particularity of the case is the discovery of an adrenal tumour due to an aortic dissection, the patient having no other symptoms and presenting a recorded history of hypertension.

The Role of Autonomic Function on Sport Performance in Athletes With Spinal Cord Injury

Devastating paralysis, autonomic dysfunction, and abnormal cardiovascular control present significant hemodynamic challenges to individuals with spinal cord injury (SCI), especially during exercise. In general, resting arterial pressure after SCI is lower than with able-bodied individuals and is commonly associated with persistent orthostatic intolerance along with transient episodes of life-threatening hypertension, known as "autonomic dysreflexia." During exercise, the loss of central and reflexive cardiovascular control attenuates maximal heart rate and impairs blood pressure regulation and blood redistribution, which ultimately reduces venous return, stroke volume, and cardiac output. Thermoregulation also is severely compromised in high-lesion SCI, a problem that is compounded when competing in hot and humid conditions. There is some evidence that enhancing venous return via lower body positive pressure or abdominal binding improves exercise performance, as do cooling strategies. Athletes with SCI also have been documented to self-induce autonomic dysreflexia before competition with a view of increasing blood pressure and improving their performance, a technique known as "boosting." For health safety reasons, boosting is officially banned by the International Paralympics Committee. This article addresses the complex issue of how the autonomic nervous system affects sports performance in athletes with SCI, with a specific focus on the potential debilitating effects of deranged cardiovascular control.

Special Considerations for Exercise Testing and Programming for Individuals With Spinal Cord Injury

Special Considerations for Exercise Testing and Programming for Individuals With Spinal Cord Injury

Claims analysis of hypertension occurrence, severity changes and patterns of antihypertensive use in cancer patients receiving vascular endothelial growth factor inhibitors.

Vascular endothelial growth factor inhibitors such as bevacizumab, sorafenib, and sunitinib are utilized in the treatment of multiple cancers. Although these agents are associated with hypertension, there is a lack of evidence describing patterns of antihypertensive use in patients with vascular endothelial growth factor inhibitor-associated hypertension in a non-trial, "real-world" setting. To describe the occurrence and severity of vascular endothelial growth factor inhibitor-associated hypertension, patterns of antihypertensive use and occurrence of cardiovascular complications in a non-trial population, and to describe patterns of initial antihypertensive therapy in patients developing hypertension during treatment with a vascular endothelial growth factor inhibitor. This retrospective cohort study utilized claims data from the Medstat MarketScan Commercial Claims and Encounter database to identify patients with claims for a vascular endothelial growth factor inhibitor and a diagnosis of cancer using International Classification of Diseases, 9th Revision, Clinical Modification codes, Healthcare Common Procedure Coding System J-codes and National Drug Codes. The study period encompassed claims from one year before the patient's first claim for a vascular endothelial growth factor inhibitor, and continued through one year after the initial vascular endothelial growth factor inhibitor claim. Patients meeting study criteria were classified into cohorts A1, patients with no hypertension throughout the study period; A2, patients without hypertension at baseline who developed hypertension after starting a vascular endothelial growth factor inhibitor; and cohort B, patients with hypertension prior to receiving a vascular endothelial growth factor inhibitor. We utilized medical and pharmacy claims data to describe the presence of hypertension, its severity, and the occurrence of cardiovascular complications throughout the study period. Initial antihypertensive use in cohort A2 was described. In all, 2177 patients met study criteria and were categorized into cohorts A1 (n = 708), A2 (n = 333) and B (n = 1136). Approximately 32% of patients without hypertension at baseline had claims suggestive for hypertension during the study period. Life-threatening (Grade 4) hypertension increased throughout the study period for cohorts A1, A2, and B, to 3.4%, 10.2%, and 16.4%, respectively (p < 0.001 for all). Claims suggestive of Grade 3 hypertension occurred in more patients in cohort B (45.8%) than in cohort A2 (32.7%, p < 0.001). Cardiovascular complications occurred in 4.7%, 15.6%, and 22.7% of patients in cohorts A1, A2, and B, respectively. Initial antihypertensive agent selection did not impact the occurrence of cardiovascular complications in cohort A2. Our study provides valuable insight into non-trial patterns of vascular endothelial growth factor inhibitor-associated hypertension occurrence and severity, and is consistent with prior claims analysis. Identification of optimal strategies to manage vascular endothelial growth factor inhibitor-associated hypertension remain to be clarified with the advent of more comprehensive data sets.

Intraoperative Use of Clevidipine in a Patient With von Hippel-Lindau Disease With Associated Pheochromocytoma

PHEOCHROMOCYTOMAS and paragangliomas are rare neuroendocrine tumors. Pheochromocytoma is the nomenclature used for a catecholamine-secreting tumor occurring anywhere along the sympathetic chain, most commonly in the adrenal medulla. It is known also as a chromaffin tumor because of the brown-black color of the cells created when the catecholamine-rich tumor cells react with the chromogranin A stain during histologic sectioning. In contrast, paragangliomas are extra-adrenal tumors arising from the sympathetic or parasympathetic paraganglia. 1 Waguespack S.G. Rich T. Grubbs E. et al. A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 2010; 95: 2023-2037 Crossref PubMed Scopus (159) Google Scholar Because of excessive catecholamine secretion, pheochromocytomas may precipitate life-threatening hypertension or cardiac arrhythmias. 2 Kazic M.R. Zivaljevic V.R. Milan Z.B. et al. Perioperative risk factors, morbidity, and outcome of 145 patients during phaeochromocytoma resection. Acta Chir Belg. 2011; 111: 223-227 PubMed Google Scholar In particular, significant hemodynamic instability with hypertension may occur intraoperatively during tumor resection. The authors report for the first time the use of clevidipine to control intraoperative blood pressure (BP) during resection of a pheochromocytoma in a pediatric patient. Previous reports on the use of clevidipine in this pediatric population are reviewed and its utility in the clinical scenario is discussed.

Autonomic Dysreflexia

To present the complex issues of the impact of spinal cord injury (SCI) in sport, with a specific focus on autonomic dysreflexia (AD) and the potential debilitating effects of unstable blood pressure control among athletes. A literature review based on a key word electronic literature search of articles, practice guidelines, and review articles pertaining to AD was conducted using MEDLINE, SportDiscus, and EMBASE. Spinal cord injury results not only in devastating paralysis; it also commonly is associated with a range of autonomic dysregulation that can interfere with cardiovascular, bladder, bowel, temperature, and sexual function. Individuals with a cervical or high-thoracic SCI face lifelong abnormalities in systemic arterial pressure control. In general, their resting arterial pressure is lower than that in able-bodied individuals and is commonly associated with persistent orthostatic intolerance. In addition, they experience transient episodes of life-threatening hypertension, known as "AD," which often is associated with disturbances in heart rate and rhythm. Autonomic dysreflexia occurs in up to 90% of individuals with a cervical or high-thoracic SCI and requires prompt intervention. It also is known that, during athletic activities, self-induced AD is used by some individuals to improve their performance, a technique known as "boosting." For health safety reasons, boosting is officially banned by the International Paralympic Committee. Devastating paralysis, a variety of autonomic dysfunctions, and abnormal cardiovascular control after SCI present significant challenges in terms of individuals remaining active in competitive sports. Medical practitioners who are involved in the care of wheelchair athletes should be aware of the unique cardiovascular dysfunction that results from SCI and may occur at any time, even with seemingly innocuous triggers. Prompt recognition and appropriate management of these conditions, including episodes of AD, could be life saving.

A Large Pheochromocytoma With Invasion of Multiple Local Organs

A Large Pheochromocytoma With Invasion of Multiple Local Organs

Targeted ablation of mesenteric projecting sympathetic neurons reduces the hemodynamic response to pain in conscious, spinal cord-transected rats.

Individuals with spinal cord injuries above thoracic level 6 (T(6)) experience episodic bouts of life-threatening hypertension as part of a condition termed autonomic dysreflexia. The paroxysmal hypertension can be caused by a painful stimulus below the level of the injury. Targeted ablation of mesenteric projecting sympathetic neurons may reduce the severity of autonomic dysreflexia by reducing sympathetic activity. Therefore, cholera toxin B subunit (CTB) conjugated to saporin (SAP; a ribosomal inactivating protein that binds to and inactivates ribosomes) was injected into the celiac ganglion to test the hypothesis that targeted ablation of mesenteric projecting sympathetic neurons reduces the pressor response to pain in conscious, spinal cord-transected rats. Nine Sprague-Dawley male rats underwent a spinal cord transection between thoracic vertebrae 4 and 5. Following recovery (5 wk), all rats were instrumented with a radio telemetry device for recording arterial pressure and bilateral catheters in the gluteus maximus muscles for the infusion of hypertonic saline (hNa(+)Cl(-)). Subsequently, the hemodynamic responses to intramuscular injection of hNa(+)Cl(-) (100 microl and 250 microl, in random order) were determined. Following the experiments in the no celiac ganglia injected condition (NGI), rats received injections of CTB-SAP (n = 5) or CTB (n = 3) into the celiac ganglia. CTB-SAP rats, compared with NGI and CTB rats, had reduced pressor responses to hNa(+)Cl(-). Furthermore, the number of stained neurons in the celiac ganglia and spinal cord (segments T(6)-T(12)), was reduced in CTB-SAP rats. Thus, CTB-SAP retrogradely transported from the celiac ganglia is effective at ablating mesenteric projecting sympathetic neurons and reducing the pressor response to pain in spinal cord-transected rats.

Metoclopramide stimulates catecholamine- and granin-derived peptide secretion from pheochromocytoma cells through activation of serotonin type 4 (5-HT4) receptors

The gastroprokinetic agent metoclopramide is known to stimulate catecholamine secretion from pheochromocytomas. The aim of the study was to investigate the mechanism of action of metoclopramide and expression of serotonin type 4 (5-HT(4)) receptors in pheochromocytoma tissues. Tissue explants, obtained from 18 pheochromocytomas including the tumor removed from a 46-year-old female patient who experienced life-threatening hypertension crisis after metoclopramide administration and 17 additional pheochromocytomas (9 benign and 8 malignant) were studied. Cultured pheochromocytoma cells derived from the patient who previously received metoclopramide were incubated with metoclopramide and various 5-HT(4) receptor ligands. In addition, total mRNAs were extracted from all the 18 tumors. Catecholamine- and granin-derived peptide concentrations were measured in pheochromocytoma cell incubation medium by HPLC and radioimmunological assays. In addition, expression of 5-HT(4) receptor mRNAs in the 18 pheochromocytomas was investigated by the use of reverse transcriptase-PCR. Metoclopramide and the 5-HT(4) receptor agonist cisapride were found to activate catecholamine- and granin-derived peptide secretions by cultured tumor cells. Metoclopramide- and cisapride-evoked catecholamine- and granin-derived peptide productions were inhibited by the 5-HT(4) receptor antagonist GR 113808. 5-HT(4) receptor mRNAs were detected in the patient's tumor and the series of 17 additional pheochromocytomas. This study shows that pheochromocytomas express functional 5-HT(4) receptors that are responsible for the stimulatory action of metoclopramide on catecholamine- and granin-derived peptide secretion. All 5-HT(4) receptor agonists must therefore be contraindicated in patients with proven or suspected pheochromocytoma.

Spontaneous renal artery dissection: three cases and clinical algorithms

Spontaneous renal artery dissection (SRAD) is rare. Clinical manifestations vary from minimal symptoms to life-threatening hypertension. We analysed three cases from our institution and conducted a literature review in order to design diagnostic and treatment algorithms for SRAD.