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The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17- mo- old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic- range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic polaryteritis nodosa (c- PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non- aneurismal changes such as arterial cutoff, arterial tapering stenosis and nephrogram perfusion defect. A decrease of blood pressure by antihypertensive treatment resulted in the normalization of HHS abnormalities. However, c- PAN became well controlled only after 4 mo of immunosuppressive therapy. Conclusion: The main interest of this case was the uncommon presentation of systemic polyarteritis nodosa in a very young child. Renal ischaemia from intrarenal vessel disease may have been the trigger event for HHS in our case. Management of PAN- associated severe arterial hypertension is based on immunosuppressive and antihypertensive treatment.
The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17-mo- old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic - range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic polaryteritis nodosa (c-PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non- aneurismal changes such as arterial cutoff, arterial tapering stenosis A decrease of blood pressure by antihypertensive treatment resulted in the normalization of HHS abnormalities. However, c-PAN became well controlled only after 4 mo of immunosuppressive therapy. Conclusion: The main interest of this case was the uncommon presentation of systemic polyarteritis nodosa in a very young child. Renal i Management of PAN-associated severe arterial hypertension is based on immunosuppressive and antihypertensive treatment.