Infantile polyarteritis nodosa presenting as hyponatraemic hypertensive syndrome.
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 polyarteritis nodosa (c-PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non-aneurismal changes such as arterial cut-off, 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.


CONCLUSIONS
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.
Peco-Antić
Amira
A
University Children's Hospital, Belgrade, Serbia and Montenegro. amirapecoantic@yahoo.com
Perisić
Vojislav
V
Bonaći-Nikolić
Branka
B
Djukić
Milan
M
Kruscić
Divna
D
Kostić
Mirjana
M
D005260:Female; D006801:Humans; D006974:Hypertension, Malignant / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D007010:Hyponatremia / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D007223:Infant; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D013577:Syndrome