Screening for secondary hypertension is not recommended for all hypertensive patients, but missing these cases is critical. We aimed to (i) assess hypertension causes in a cohort of hypertensive children, (ii) determine the costs and contributions of an extended diagnostic work-up to screen for secondary hypertension, and (iii) compare the performance of a "short diagnostic work-up" with the protocols of the American Academy of Pediatrics (AAP) and European Society of Hypertension (ESH).

We conducted a retrospective, single-center study of 70 hypertensive patients aged 1-18 years. All underwent an extended work-up to exclude secondary hypertension. Diagnostic findings, test counts, and costs were analyzed. A short work-up (serum creatinine, fasting glucose, electrolytes, urinalysis, kidney ultrasound (US), and renal artery Doppler US), as well as the AAP and ESH protocols, was evaluated for performance and costs.

Secondary hypertension was identified in 29 patients (41.4%). The extended protocol identified or excluded secondary causes in all patients. Kidney US had the highest diagnostic yield (37.1%). The short work-up and ESH protocol identified all secondary cases, whereas the AAP protocol missed 15 diagnoses. The extended protocol cost € 17,715.60 (€ 253.08 per patient). Direct cost savings were 64.3% with the short work-up, 92.4% with the AAP protocol, and 76.2% with the ESH protocol.

Primary is more common than secondary hypertension in children, with kidney parenchymal disease being the leading secondary cause. As recommended by guidelines, a simplified, focused work-up may offer a cost-effective alternative to extensive screening while maintaining diagnostic accuracy.

Hyponatremic hypertensive syndrome (HHS) is a rare disorder, particularly in adults. A 76-year-old woman presented with vomiting and severe hyponatremia. The patient had been hospitalized four times over the past four years due to hyponatremia and recurrent vomiting. On admission, magnetic resonance angiography revealed left renal artery stenosis, which led to a diagnosis of HHS. Treatment with enalapril followed by tolvaptan improved the patient's condition, without the need for renal artery angioplasty. HHS should therefore be considered in patients with hyponatremia, hypertension, or recurrent vomiting. In addition to angiotensin-converting enzyme inhibitors and renal artery angioplasty, tolvaptan may also be a new effective treatment option for HHS.

Moyamoya disease, a cause of pediatric stroke, has been shown to affect furthermore extra-cranial districts, mostly the kidney arterial site, resulting in steno-occlusive changes. Unilateral renal artery stenosis accounts for 8%-10% out of cases of renovascular hypertension in childhood, however it rarely underlies a hyponatremic-hypertensive syndrome (HHS).

We describe an 18-month-old boy with a recent history of polyuria and polydipsia, who presented an acute febrile gastroenteritis with neurological impairment, severe dehydration, hyponatremia, hypokalemia, kidney tubular dysfunction, and elevated aldosterone and renin even with a normal blood pressure. Fluid and electrolytes correction was performed, with complete recovery. An abdominal ultrasound displayed a smaller right kidney. A brain magnetic resonance and an electroencephalogram did not show any relevant abnormalities. Five months later, the child experienced a left-side hemiparesis after a traumatic concussion, and a severe hypertension. A brain tomography documented a cerebral ischemia. Brain and kidney angiographic studies displayed puff of smoke findings of internal right carotid artery branches and a steno-occlusive pattern of right renal artery, respectively. Hence, moyamoya disease with HHS secondary to unilateral renal artery stenosis was diagnosed. After an unsuccessful antiplatelet and antihypertensive pharmacological treatment, the boy underwent a renal angioplasty and a cerebral STA-MCA bypass (direct superficial temporal artery-to-middle cerebral artery bypass), resulting in a significant improvement of both neurological and kidney disease.

Although the association between unilateral renal artery stenosis and HHS has been previously shown, this is the first report of atypical HHS, with hypertension preceded by tubular dysfunction, recognized in the framework of moyamoya disease.

Hyponatremic-hypertensive syndrome (HHS) is an uncommon disorder rarely seen in children. Herein, we report a 19-month-old boy with HHS. He had severe hypertension, polyuria, polydipsia, vomiting, and seizure at presentation. Laboratory findings revealed hyponatremia, hypokalemia, metabolic alkalosis, proteinuria, hypercalciuria, high levels of renin and aldosterone, and renal artery stenosis. All symptoms resolved after nephrectomy. Clinicians should be aware of this syndrome because prompt recognition can be lifesaving.

Hyponatremic hypertensive syndrome is characterized by hypertension, hyponatremia, and hypokalemia due to unilateral renal artery stenosis. We herein report a 1-year-old hyponatremic hypertensive syndrome infant without persistent hypertension in the acute phase. On the ninth hospital day, his systolic and diastolic blood pressure increased up to 154-160 and 70-84 mmHg, respectively. Acute gastroenteritis and dehydration might transiently mask his hypertension. By percutaneous transluminal balloon angioplasty for right renal artery, his blood pressure finally normalized without antihypertensive drugs. We reviewed 23 previously reported pediatric patients with hyponatremic hypertensive syndrome under the age of 15 years. Including our patient, there are only three reports on hyponatremic hypertensive syndrome without persistent hypertension in the acute phase. Hyponatremic hypertensive syndrome is curable with proper diagnosis and timely intervention. Therefore, pediatricians should pay attention to the signs and symptoms associated with hyponatremic hypertensive syndrome, even if persistent hypertension was absent in the acute phase.

Background: Fibromuscular dysplasia (FMD) is one of the important etiologies of renovascular hypertension in children. It is usually resistant to multiple antihypertensive agents and can cause extreme elevation in blood pressures, which can lead to end organ damage if not promptly diagnosed and treated. Treatment options include medical management with antihypertensive agents, balloon or stent angioplasties, surgical revascularization, and nephrectomy. The aim of the study was to review the efficacy of antihypertensive therapy only in the management of FMD in a very young child. Methods: This is a retrospective chart study with review of literature. Results: Here, we report a 22-month-old toddler who presented with severe resistant hypertension and cardiomyopathy who was found to have focal FMD of the right renal artery. She also presented with proteinuria, hyponatremia that was probably secondary to pressure natriuresis, hypokalemia, hyperaldosteronism, and elevated plasma renin activity. The stabilization of blood pressures was done medically with the usage of antihypertensive medications only, without the need for angioplasty or surgical revascularization. Conclusions: We demonstrate that surgical intervention may not always be necessary in the treatment of all cases of FMD, especially in a small child where such intervention may be technically challenging and lead to potential complications. Hence, medical management alone may be sufficient, at least for the short-term, in small children with controlled hypertension and normal renal function, with surgical intervention reserved for FMD with medication-refractory hypertension and/or compromised renal function.

Hypertensive crisis can be a source of morbidity and mortality in the pediatric population. While the epidemiology has been difficult to pinpoint, it is well-known that secondary causes of pediatric hypertension contribute to a greater incidence of hypertensive crisis in pediatrics. Hypertensive crisis may manifest with non-specific symptoms as well as distinct and acute symptoms in the presence of end-organ damage. Hypertensive emergency, the form of hypertensive crisis with end-organ damage, may present with more severe symptoms and lead to permanent organ damage. Thus, it is crucial to evaluate any pediatric patient suspected of hypertensive emergency with a thorough workup while acutely treating the elevated blood pressure in a gradual manner. Management of hypertensive crisis is chosen based on the presence of end-organ damage and can range from fast-acting intravenous medication to oral medication for less severe cases. Treatment of such demands a careful balance between decreasing blood pressure in a gradual manner while preventing damage end-organ damage. In special situations, protocols have been established for treatment of hypertensive crisis, such as in the presence of endocrinologic neoplasms, monogenic causes of hypertension, renal diseases, and cardiac disease. With the advent of telehealth, clinicians are further able to extend their reach of care to emergency settings and aid emergency medical service (EMS) providers in real time. In addition, further updates on the evolving topic of hypertension in the pediatric population and novel drug development continues to improve outcomes and efficiency in diagnosis and management of hypertension and consequent hypertensive crisis.

Hypertensive crisis is a relatively rare condition in children. However, if not treated, it might be life-threatening and lead to irreversible damage of vital organs. Clinical presentation of patients with hypertensive crisis can vary from very mild (hypertensive urgency) to severe symptoms (hypertensive emergency) despite similarly high blood pressure (BP). Individualized assessment of patients presenting with high BP with emphasis on the evaluation of end-organ damage rather than on the specific BP number is a key in guiding physician's initial management of a hypertensive crisis. The main aim of the treatment of hypertensive crisis is the prevention or treatment of life-threatening complications of hypertension-induced organ dysfunction, including neurologic, ophthalmologic, renal, and cardiac complications. While the treatment strategy must be directed toward the immediate reduction of BP to reduce the hypertensive damage to these organs, it should not be at a too fast rate to cause hypoperfusion of vital organs by an excessively rapid reduction of BP. Thus, intravenous continuous infusions rather than intravenous boluses of antihypertensive medications should be the preferable mode of initial treatment of children with hypertensive emergency.

To ascertain the frequency of hyponatremic hypertensive syndrome (HHS) in a cohort of children with hypertensive emergency in a tertiary pediatric hospital.

A retrospective review was undertaken among children with hypertensive emergency admitted in our tertiary children hospital between June 2014 and December 2015 with an aim to identify any children with HHS. Three children with HHS were identified during this period.

The 3 patients with HHS presented with hypertensive emergency. They were initially managed with Labetalol infusion and thereafter switched to oral anti-hypertensives (combination of Nifedipine sustained release, Hydralazine and Beta Blocker). All 3 were diagnosed to have unilateral renal artery stenosis. One child was lost to follow up, whereas the other 2 underwent renal angioplasty which was followed with normalization of blood pressure.

Despite activation of renin angiotensin axis secondary to renal artery stenosis, these groups of children have significant hyponatremia. Renal re-vascularisation produces excellent results in most of them.

Secondary hypertension is the most frequent form of hypertension in children. Renovascular disease accounts for 5-10% of all childhood hypertension and should be suspected in the presence of severe hypertension found difficult to manage with medical therapy. Uncontrolled hypertension can lead to severe target organ damage. We describe the case of a 13-month-old baby boy with failure to thrive, recent muscular weakness of the lower extremities and irritability. Hypertension was detected and he was admitted to the paediatric intensive care unit with a refractory hypertensive emergency, despite multiple antihypertensive therapies. Bilateral renal artery stenosis was diagnosed through renal angiography and balloon dilation was performed, leading to lower blood pressure. He is currently withdrawing from antihypertensive medication, and slowly gaining weight and recovering from target organ damage. However, weakness of the lower extremities persists and he has been diagnosed with a neurogenic bladder.