Abnormal Ambulatory Blood Pressure and Early Renal Dysfunction in Sickle Cell Disease.

OBJECTIVES: There is sparsity of studies evaluating blood pressure in children with sickle cell disease (SCD), which have shown inconsistent results. Few of the studies have documented lower office blood pressure (BP) in SCD patients, whereas, others have shown presence of masked hypertension and abnormal ambulatory blood BP monitoring (ABPM). Thus, the present study was conducted to examine 24 h ABPM parameters and renal dysfunction in children with SCD and compare them with healthy controls. METHODS: A cross-sectional study was conducted on 56 children (30 children having SCD and 26 controls). ABPM and evaluation of renal functions including serum creatinine, serum urea, urinary creatinine, urinary protein and specific gravity was performed. RESULTS: Spot urinary protein to creatinine ratio was found to be higher in patients with SCD (63.3%) as compared to controls (p < 0.001). Proteinuria was observed in 1/4th of the SCD patients less than ten years of age. Masked hypertension was present in 2 (6.6%) patients, ambulatory hypertension in 4 (13.3%), ambulatory pre-hypertension in 1 (3.3%) and abnormal dipping in 60%. A statistically significant correlation of BMI for age Z-score and standard deviation score (SDS/Z) of 24 h systolic BP (r = 0.56, p = 0.002); estimated glomerular filtration rate (eGFR) with 24 h diastolic BP SDS (r = -0.52; p = 0.038) and age with e GFR (r = 0.54; p = 0.025) was found in the present study. CONCLUSIONS: The present study corroborates that ABPM abnormalities (ambulatory hypertension, non-dipping pattern, ambulatory prehypertension) and early onset proteinuria are significant findings in patients with SCD. This underscores the importance of regular screening for proteinuria and ABPM in routine care, for early detection and prevention of progressive renal damage in SCD.

Takayasu arteritis presenting with malignant hypertension; a rare manifestation of a rare disease: a case report and review of the literature.

Takayasu arteritis (TA) is a chronic inflammatory and obliterative disease of large vessels, which mainly affects the aorta and its major branches. TA can lead to renal failure and renovascular hypertension in 60% of patients; it is rare in children aged <10 years and, more rarely, it presents with malignant hypertension in the paediatric age group. Here we present a case of 9-year-old boy with TA who presented with malignant hypertension and required surgical intervention to control the blood pressure. Subsequently, his medications were titrated using 24 h ambulatory blood pressure monitoring (ABPM) and is doing well on follow-up.