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 pr...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.展开更多
文摘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.
