Cause Of Malignant Hypertension Research Articles

PSAT014 Pheochromocytoma in a Pregnant Woman with VHL leading to First Trimester Fetal Demise

Abstract Introduction Pheochromocytomas (PCCs) are rare neuroendocrine tumors that secrete catecholamines. They may be associated with familial syndromes such as Multiple Endocrine Neoplasia type 2 (MEN 2), von Hippel-Lindau disease (VHL), and Neurofibromatosis type 1 (NF 1). PCCs are a common cause of malignant hypertension in pregnancy and can lead to adverse maternal and fetal outcomes if left undiagnosed or untreated. Case Description A 39-year-old Latin American primigravid woman at 8 weeks 4 days gestation was referred to the endocrinology clinic for labile blood pressure (160/90 mmHg at recent ER visit) and a lab finding of elevated catecholamines (plasma norepinephrine 4720 pg/ml; normal 0-874 pg/ml). She reported chronic headaches and 2-lb unintentional weight loss during pregnancy. Past medical history was significant for right adrenal PCC diagnosed at age 13 (currently status post adrenalectomy), and a cerebrovascular accident at age 29. Family history was relevant for PCC in the patient's sister and mother. The patient's home medications were nifedipine 30 mg daily and prenatal vitamins. On physical exam, her blood pressure was 126/76 mm Hg, heart rate was 67/min and a scar of previous right adrenalectomy was noted. Doxazosin 1 mg daily was added to her regimen and nifedipine was discontinued. The patient was initially planned for an elective cesarean section at term. However, at 10 weeks 5 days of gestation, she had a missed abortion. Further genetic testing of syndromic PCC revealed a heterozygous mutation in the VHL gene. Abdominal MRI showed a left adrenal mass (3.6×2.6×3.6 cm3) and retroperitoneal lymphadenopathy. The patient underwent left adrenalectomy and is currently hemodynamically stable on maintenance hydrocortisone and fludrocortisone. Discussion We report a case of PCC complicating pregnancy in a 39-year-old primigravida who had a family history of PCC. In females of reproductive age with a known PCC diagnosis, we suggest early genetic testing as it can further advise family planning, antepartum monitoring, and management. Medically, these patients can be managed with α-adrenergic blockers and calcium-channel blockers, followed by β-blockers. This can improve maternal and fetal outcomes primarily by minimizing paroxysmal elevations in catecholamines. Definitive treatment of PCC is surgical excision, usually performed during the second trimester or postpartum. Management of patients with VHL requires lifelong clinical, laboratory, and imaging surveillance for other manifestations of the disease. References Bancos, Irina et al. Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature. Lancet Diabetes Endocrinol 2021; 9: 13–21 Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

POST PARTUM HYPERTENSIVE EMERGENCY REVEALING ATYPICAL HEMOLYTIC AND URAEMIC SYNDROME IN TWO SISTERS

Objective: Hemolytic uremic syndrome (HUS) is a rare and severe form of thrombotic microangiopathy (TMA) associated with a poor renal prognosis. Atypical hemolytic uraemic syndrome (aHUS) is a form of HUS that has been clearly associated with genetic susceptibility factors related to complement regulation. Pregnancy- and postpartum-associated HUS is now considered as a part of spectrum of complement-mediated HUS. AHUS is a rare cause of malignant hypertension. For this reason we report 02 cases of postpartum aHUS observed in nephrology department. Design and method: We analyzed retrospectively the presentation and outcome of 02 sisters who had a malignant hypertension in the period of post-partum with features of hemolytic and uremic syndrome. Results: In the two cases, it was a clinical presentation of malignant hypertension needing intravenous treatment with nicardipine associated with severe acute kidney injury (AKI) requiring dialysis at presentation (table). We underwent plasma exchanges as first-line therapy then relayed by plasma infusions. Kidney biopsy performed in the first sister whose diagnosed in 2018 (with normal platelet count) showed typical features of thrombotic microangiopathy.Complement component assays showed low Factor H (<10%) in 02 cases. They evolved to chronic kidney disease and they have always a severe blood pressure although the combination of 5 antihypertensive medications. Conclusions: Post partum -related atypical HUS is a very severe disease, with a poor kidney prognosis and without specific efficacious treatment. Familial aHUS is very rare entity. We reported 02 cases of malignant hypertension which is highly prevalent in this population and it dominates the clinical presentation.

Entrapped renal artery in the left crux of diaphragm: a rare cause of malignant hypertension.

Entrapped renal artery in the left crux of diaphragm: a rare cause of malignant hypertension.

Pheochromocytoma and Von Hippel–Lindau in Pregnancy

Pheochromocytoma is an infrequent but well-acknowledged primary cause of malignant hypertension in pregnancy. Although the majority of pheochromocytomas are sporadic, those that present as bilateral or multifocal tumors may be a manifestation of a rare cancer susceptibility syndrome, such as Von Hippel-Lindau (VHL). Gravidae with unrecognized pheochromocytoma are at risk for recurrent paroxysmal hypertensive crises with ensuant maternal and fetal risks. To further illustrate the challenges of management of pheochromocytoma and VHL in pregnancy, we present two illustrative cases. In the first, a multigravida presented with an intrauterine fetal demise and malignant hypertension and a concurrent diagnosis of bilateral pheochromocytomas. A missense mutation in exon 3 of the VHL gene was identified, confirming the diagnosis of VHL type 2C. In the second case, a multigravida with a prior diagnosis of VHL syndrome but sporadic follow-up underwent renal and adrenal imaging surveillance as part of her prenatal care. Although she was normotensive and clinically asymptomatic, such imaging enabled the detection of bilateral pheochromocytomas. In summary, in this report we discuss our management in gravidae with pheochromocytoma and VHL, emphasizing current recommendations pertaining to obstetric management, genetic testing, and long-term follow-up.

Juxtaglomerular Cell Tumor in Pregnancy

In Brief BACKGROUND Although rare, two thirds of juxtaglomerular cell tumors of the kidney occur in young women in their reproductive years. CASE A primigravid woman with a 6-year history of chronic hypertension was evaluated for the sudden onset of uncontrolled hypertension, proteinuria, and hypokalemia at 16 weeks' gestation. An abdominal sonogram revealed a left flank mass, and magnetic resonance imaging confirmed that the mass was of renal origin. The worsening hypertension was not controlled with labetolol, methyldopa, nifedipine, or hydralazine, and required a nitroglycerine drip. The patient had left nephrectomy and subsequently miscarried at 19 weeks' gestation. Her blood pressure gradually decreased and normalized within 6 months. A pathologic examination of the renal mass confirmed that it was a juxtaglomerular cell tumor. CONCLUSION This tumor should be considered in the differential diagnosis as a cause of severe hypertension in pregnancy. Juxtaglomerular cell tumors are a rare cause of malignant hypertension during pregnancy.

Malignant hypertension in children in India

Malignant hypertension is now an uncommon entity in the western world but still remains a significant problem in India. Therefore we studied the aetiological spectrum, management, and outcome of these patients. Forty consecutive children (<16 years) with malignant hypertension were admitted and investigated to exclude or confirm the secondary causes of hypertension. For acute control of blood pressure sublingual nifedipine was used in dosage of 0.3-0.6 mg/kg, failing which intravenous nitroglycerin was used. In patients with aortoarteritis with active disease, steroids were used. Angioplasty was carried out for renal artery stenosis whenever possible. Renoparenchymal disease was the commonest cause of malignant hypertension, and was seen in 25 cases, renovascular hypertension in 13 cases (11 aortoarteritis and two fibromuscular dysplasia) and two had essential hypertension. For acute control of severe hypertension, sublingual nifedipine was effective in 92.5% of patients. Of the patients with renoparenchymal disease five became normotensive with treatment of the underlying disease, four received renal allograft, seven died, and nine are stable on antihypertensive drugs. Renal angioplasty was carried out in seven patients with renovascular hypertension (4 cured, 3 improved) and six are controlled on drugs. We conclude that apart from renoparenchymal disease, aortoarteritis is a common cause of malignant hypertension in children. Sublingual nifedipine is effective for the rapid control of severe hypertension, and angioplasty is effective in aortoarteritis for short-term preservation of renal function and control of hypertension.

POLYARTERITIS NODOSA PRESENTING WITH HYPERTENSIVE ENCEPHALOPATHY

SUMMARYPolyarteritis nodosa (PAN) is a rare cause of malignant hypertension.1 As far as we are aware, hypertensive encephalopathy has not been described in the literature as a presenting feature of PAN.

Malignant hypertension in women of childbearing age and its relation to the contraceptive pill.

Eleven of 34 women aged 15-44 with malignant phase hypertension were taking oral contraceptives at presentation. All had had normal blood pressure before starting to take the pill. In four the interval between the start of oral contraception and the diagnosis of malignant hypertension was less than four months, and in eight no other cause for the hypertension was found. Underlying renal disease and renal failure were less common among pill users than among non-users with malignant hypertension who were of similar age. No pill user became normotensive after withdrawal of the pill, but blood pressure was well controlled (diastolic less than 90 mm Hg) in three patients taking only one drug. By contrast, all 23 non-users needed two or more antihypertensive drugs to control blood pressure. Ten year survival was 90% among pill users and 50% among non-users. These results suggest that oral contraceptives may be a common cause of malignant hypertension in women of child-bearing age. If the pill is stopped and underlying renal disease excluded the long term prognosis for such patients is excellent.

Adenocarcinoma of the Kidney and Hypertension: Report of 2 Cases with Special Emphasis on Renin

Renin studies were done on 2 patients with adenocarcinoma of the kidney and hypertension. In 1 case plasma renin activity was high in the peripheral and renal veins, with a renal vein ratio of 1.7 favoring the side of the tumor. Nephrectomy cured the hypertension and renin values became normal. Tissue renin was elevated in the tumor and surrounding parenchyma. Acidification studies of tissue extracts failed to demonstrate the existence of big renin. In case 2 all renin values were normal and the blood pressure remained elevated after the operation. Although renin-secreting tumors remain an uncommon cause of malignant hypertension the condition should be recognized because it is potentially curable.

Medical management of hypertension.

The panelists discuss the relation of atherosclerosis to hypertensive disease, the cause of malignant hypertension, the treatment of mild hypertension, and the effects of guan-ethidine and methyldopa in the management of patients with hypertension.