Hyponatremic hypertensive syndrome hhs in an 18month. Renovascular hypertension renal artery stenosis hyponatremic hypertensive syndrome abnormal. On the contrary, accelerated hypertension is a consensual indication to screen for renovascular hypertension 4. The most common underlying cause of hyponatremic hypertensive syndrome in adults is severe atherosclerotic renovascular disorder while in children the most common cause of hyponatremic hypertensive syndrome is unilateral congenital renal artery stenosis due to some form of arterial. Making an accurate differential diagnosis is important both therapeutically and prognostically. Cases with critical unilateral renal artery stenosis manifesting with the hyponatremic hypertensive syndrome are rare and a comprehensive description of this disorder in the pediatric population is lacking in the literature. Renal artery stenosis precipitates hyponatremic hypertensive. Hyponatremic hypertensive syndrome secondary to renal ischemia. The combination of hyponatremia and renovascular hypertension is called the hyponatremic hypertensive syndrome hhs 14. Hyponatremichypertensive syndrome with renal ischemia. Many medical illnesses, such as congestive heart failure, liver failure, renal failure, or pneumonia, may be associated with hyponatremia. An eighteen monthold male presented with drowsiness, sudden onset status epilepticus and blood pressure of 210160. In children, severe hypertension can be caused by a variety of different underlying conditions. In children, 525% of the cases with secondary hypertension are.
Accelerated hypertension with hyponatremia and hypokalaemia. Hyponatremichypertensive syndrome with extensive and. Chronic hyponatraemia without severe or moderately severe symptoms 3. Hyponatremic hypertensive syndrome is a rare condition of renovascular hypertension characterized by electrolyte abnormalities hyponatremia, hypokalemia, polyuria, and high renin activity.
In recent years, a low sodium concentration has been found frequently to accompany a wide variety of disease states. Initial investigations revealed severe hyponatremia serum na 120 mmoll, hypochloremia serum cl 68 mmoll, and mild hypokalemia. Both children also presented with severe high molecular proteinuria, glycosuria, and hypercalciuria, only the first symptom. Clinical practice guideline on diagnosis and treatment of. However, hypertension is a major risk factor for heart disease, stroke, kidney failure, and eye problems. Hyponatremic hypertensive syndrome, pediatric nephrology. The term hyponatremic syndrome implies that there are clinical signs and symptoms which are rather specifically related to a low sodium concentration in the serum and extracellular fluid. Elevated plasma levels of renin in most cases suggest that the stimulation of renin release from the ischemic kidney plays an important pathophysiologic role. Hyponatremic hypertensive syndrome hhs is characterized by hyponatremia, severe hypertension, polyuria and polydipsia in association with unilateral renal artery stenosis 24.
In this article, we discuss the pathophysiology, diagnosis, differential diagnosis, and management of hyponatremia and hepatorenal syndrome in patients with cirrhosis. Lab work revealed hyponatremia, hypokalemia, and elevated peripheral renin. Although hypertensive emergencies can lead to significant morbidity and potentially fatal targetorgan damage, only 1%3% of patients with hypertension will have a hypertensive emergency during their lifetime deshmukh 2011. Hhs has been typically reported in the elderly, asthenic females with underlying renal artery atherosclerosis, but is underreported in the pediatric population 5, 6. Hyponatremic hypertensive syndrome a retrospective. Two young children with renal artery stenosis and severe hypertension who presented with the socalled hyponatremic hypertensive syndrome hhs, with marked urine and solute loss during the acute phase, are described. The hyponatremic syndrome is a prevalent phenomenon, according to recent reports, perhaps largely owing to the facility with which sodium determinations have been able to be performed by the flame photometer in the last decade.
The hyponatremic hypertensive syndrome and proteinuria promptly responded to treatment with angiotensinconverting enzyme inhibitors, suggesting an angiotensin ii dependency of these disorders. Hiponatremikhipertansif sendrom hyponatremichypertensive syndrome 2 introduction hyponatremic hypertensive syndrome hhs is a condition characterized by severe hypertension, hyponatremia or hypochloremia caused by unilateral renal artery stenosis or occlusion 1. It is mostly encountered in adults with unilateral renal artery stenosis. Drinking too much water or receiving too much fluid through an iv. Any of the following conditions can lead to hyponatremia.
The seventh report of the joint national committee on prevention, detection, evaluation, and treatment of high blood pressure iii chair aram v. Hyponatremic hypertensive syndrome, a rare condition, consists of severe hypertension associated with renal artery stenosis, hyponatremia, hypokalemia, severe thirst, and renal dysfunction characterized by natriuresis, hypercalciuria, renal glycosuria, and proteinuria. Hyponatremic hypertensive syndrome 299 circulation journal vol. It can rarely develop due to antiphospholipid syndrome aps. Severe polyuria and polydipsia in hyponatremic hypertensive syndrome associated with wilms tumor. Hyponatremichypertensive syndrome in an 18monthold. Detection of white coat hypertension or masked hypertension in patients not on drug therapy. Hyponatremichypertensive syndrome in an 18monthold male.
Hyponatremic hypertensive syndrome hhs is an uncommon disorder, in which hypertension is associated with profound hyponatremia. Though deemed a rare disease we identified within a span of 18 mo, 3 children with hhs presentation characterized by hypertension, hyponatremia, hypokalemia and metabolic alkalosis. After removal of the leiomyosarcoma, plasma concentration of active renin, but not of prorenin, normalized and the. England health, syndrome is characterized by hypertension and profound natriuresis, leading to body sodium and water depletion. Faced with the revelatory pattern of accelerated hypertension with hyponatremia and hypokalaemia, the authors might have suspected acute renal ischemia due to critical renal artery stenosis, renal artery. The lowsodium syndrome or, less specifically, the low salt syndrome. The hyponatremic hypertensive syndrome hhs was firstly described in pediatric patient. Hyponatremia causes, symptoms, signs, workup, treatment. Most of the individual causes of hyponatremia are discussed in detail separately, as are issues related to the diagnosis and treatment of hyponatremia algorithm 1 and algorithm 2. Unilateral renal artery stenosis presented with hyponatremic. Hypertension usually has no symptoms, and many affected individuals do not know they have the condition. Hyponatremic hypertensive syndrome, a disorder of severe hypertension and hyponatremia, could result from any causes of high renin. Hyponatremic hypertensive syndrome is a manifestation of severe hypertension related to renal ischemia.
Hypertensive emergency is a lifethreatening condition that requires immediate evaluation and treatment. We aimed to increase awareness of early diagnosis of hhs among polyuric, hypertensive and hyponatremic children in order to prevent renal damage and lifethreatening complications. When blood pressure is elevated, the heart and arteries have to work harder than normal to pump blood through the body. Smith neurocritical care society 2012 abstract sustained intracranial hypertension and acute brain herniation are brain codes, signifying. We report on a 4yearold girl with hyponatremichypertensive syndrome hhs, a rare entity in childhood. We report a patient with uncontrolled hypertension and severe hyponatremia. Export all images to powerpoint file add to my favorites.
Renovascular hypertension can occur secondary to renal artery stenosis which can sometime present as hyponatremic hypertensive syndrome hhs. Hiponatremikhipertansif sendrom hyponatremic hypertensive syndrome 2 introduction hyponatremic hypertensive syndrome hhs is a condition characterized by severe hypertension, hyponatremia or hypochloremia caused by unilateral renal artery stenosis or occlusion 1. Discussion renovascular hypertension is the most common type of secondary hypertension and a variety of symptoms are. It tends to affect elderly asthenic women who smoke heavily.
Hyponatremic hypertensive syndrome hhs is of special concern in patients with both hyponatremia and renovascular hypertension. It is recognized by the association of the clinical signs and symptoms of hyponatremia and hypertension. Patients with syndrome of inappropriate antidiuresis 14 3. Hyponatremic hypertensive syndrome hhs is characterized by hypertensive crisis, and hyponatremia secondary to unilateral renal damage with glomerular and tubular dysfunction. Hyponatremic hypertensive syndrome hhs is an uncommon disorder usually encountered in the adult population with unilateral renal artery stenosis and is underrecognized in the pediatric population.
Pdf severe polyuria and polydipsia in hyponatremic. Malignant hypertension with an unusual presentation mimicking the immune mediated pulmonary renal syndrome hoon suk park, yu ah hong, byung ha chung, hyung wook kim, cheol whee park, chul woo yang, dong chan jin, yong soo kim and bum soon choi division of nephrology, department of internal medicine, the catholic university of korea, school of medicine, seoul, korea. Pdf unilateral renal artery stenosis presented with hyponatremic. Hyponatremic syndrome jama internal medicine jama network.
Malignant hypertension as a presentation has been reported in adults with hhs but is rare in children. Hyponatremia is defined as a serum sodium level of less than 5 meql and is considered severe when the serum level is below 125 meql. The girl was referred to us from a local hospital with a history of recurrent fever, vomiting, and seizures. In 1965, brown and colleagues named the combination of hyponatremia caused by renal sodium loss and severe hypertension the hyponatremic hypertensive syndrome hhs 5. Within the hypertensive crises, hypertensive emergencies account for only around onefourth of. What to do in case hyponatraemia is corrected too rapidly. Hyponatremic hypertensive syndrome hhs is characterized by unilateral renal artery stenosis with secondary hypertension and glomerular and tubular dysfunction due to hyperfiltration and activation of the reninangiotensin system ras. An overview of the causes of hypotonic hyponatremia is presented in this topic. In evaluating a neonate with hyponatremia and hypertension, hhs should be considered, especially in case of umbilical arterial catheterization.
The hyponatremic hypertensive syndrome is a rare but serious complication of renovascular disease. Hyponatremic hypertensive syndrome associated with. Stephen huff josh duckworth alexander papangelou scott d. Pdf hyponatremic hypertensive syndrome in pediatric. Despite activation of renin angiotensin axis secondary to renal artery stenosis, these groups of children have significant hyponatremia. We describe a 4yearold boy who presented with severe hypertension, profound.
Angiotensinconverting enzyme inhibition ameliorates the. Hyponatremic hypertensive syndrome associated with renovascular hypertension a case report yayoi tsukada tetsuou 1, yoshiko yamazaki miyatake 1, kunio tanaka 1, takeshi suzuki 1, fumio ohtsu 1, koichi nagasawa 1, masahiro fujii 2, shigeo tanaka 2, yasuhiko iino 3, koichi tamura 4. Pam stainpositive particles, indicative of renin granules, were found in the cells of the apparatus fig4b. Hyponatremic hypertensive syndrome hhs is characterized by hypertensive. Hyponatremic hypertensive syndrome in pediatric patients. The hospital, hunter new england health, syndrome is characterized by hypertension and profound natriuresis, leading to body sodium and water depletion. Hyponatremic hypertensive syndrome secondary to renal. Hyponatremia happens when too much sodium leaves your body, or when more water than sodium stays in your blood.
Rarity of hyponatremic hypertensive syndrome in children may project its underrecognition. Hyponatremic hypertensive syndrome a retrospective cohort study. Mcareavey and colleagues 14 in glasgow noted hyponatremia plasma sodium renovascular hypertension. Intense and prolonged exercise that causes excessive sweating. Stimulation of renin release from the ischemic kidney is probably central to the pathophysiology. A doctor will probably ask you about water intake, medications, recent changes in the body weight and underlying chronic diseases. Physical examination will likely include skin turgor test to check for dehydration, checking for edema and neurological examination. The hyponatremic hypertensive syndrome in renal artery. Fancony syndrome bartter syndrome renal concentrating defect renal structure and function normal nephrogenic. Renal artery stenosis is one of the secondary causes of pediatric hypertension.
The incidence of the hyponatremichypertensive syndrome among hypertensive patients with renal ischemia is not readily apparent from the literature, and our data provide no assistance in this regard. The most common underlying cause of hyponatremic hypertensive syndrome in adults is severe atherosclerotic renovascular disorder while in children the most common cause of hyponatremic hypertensive syndrome is unilateral congenital renal. The hyponatremichypertensive syndrome in patients with renal ischemia is not rare. Although renovascular hypertension is one of the commonest causes for severe hypertension in children, hhs is rarely reported in childhood. The combination of hyponatremia and renovascular hypertension is called hyponatremic hypertensive syndrome hhs. It is a rare, but serious complication of unilateral renal stenosis characterized by resistant hypertension and profound natriuresis. The hyponatremic hypertensive syndrome hhs was firstly described in pediatric patients. Malignant hypertension with an unusual presentation. Hyponatremic hypertensive syndrome hhs is rare and is characterized with hypertension together with hyponatremia, hypokalemia and low plasma osmolarity and considered to be based on renal ischemia. The hyponatremic hypertensive syndrome in a preterm infant ncbi.
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