Gorham-Stout disease.

Gorham-Stout disease is a rare disease of unknown etiology. It is characterized by spontaneous excessive replacement of bone by proliferative non-neoplastic thin-walled lymphatic and/or blood vessels. Histology shows positive stain for the lymphatic endothelial marker LYVE-1 (lymphatic vascular endothelial hyaluronan receptor-1) and many lymphatic growth factors (PDGF-BB, VEGF-C, VEGFR-3). Patients may present with localized pain and/or weakness and radiographic evidence of massive osteolysis involving contiguous bone structures. The disease usually progresses and complications may occur with significant morbidity and mortality. Close monitoring of these patients is recommended. Treatment remains challenging. Surgical treatment has been combined with pre- and postoperative radiation therapy. Drug regimes including bisphosphonates and vitamin D have been used with various results. Currently, the most effective agent is INF-alpha due to its anti-angiogenic effect. The effect of the newer immunomodulatory agents such as the OK-432 remains to be proved.

Automated office versus home measurement of blood pressure in the assessment of morning hypertension.

OBJECTIVE: To compare the quality and accuracy of morning blood pressure (BP) readings as taken by automated office BP (AOBP) and morning home BP (mHBP) techniques using morning ambulatory BP (mABP) measurements as the gold standard. METHODS: A total of 139 individuals were included, 70 men and 69 women, mean age 53±13 years. The average AOBP readings as measured using a Microlife Watch BP office device taking triplicate automated simultaneous readings of both arms were compared with mHBP monitored on 6 routine days, using a validated automated electronic device. Both modalities were also compared with the ambulatory readings of the 3 h of waking (mABP3h). RESULTS: The AOBP values were slightly higher than the mABP3h (mean difference 8.2 mmHg, 95% limits of agreement, -18.8 to 35.2 mmHg for the systolic BP and mean difference 4.3 mmHg, 95% limits of agreement, -15.3 to 23.9 mmHg for the diastolic BP). Systolic and diastolic AOBP readings correlated with mABP3h (r=0.66, P=0.001 and r=0.64, P=0.001, respectively). Agreement was fair between AOBP and mHBP in the detection of morning hypertensive patients (agreement 70%, κ=0.32) as compared with AOBP and mABP3h (agreement 67%, κ=0.32) and mHBP and mABP3h (agreement 65%, κ=0.31). CONCLUSION: The AOBP technique could replace mHBP monitoring in the assessment of morning BP, as it provides comparable data in relation to the awake ambulatory BP. Given the simplicity of this method, it could be more readily applied in a larger number of individuals.

Automated office blood pressure is associated with urine albumin excretion in hypertensive subjects.

BACKGROUND: We aimed to investigate the association between automated office blood pressure (AOBP) readings and urine albumin excretion (UAE), and to assess if this association is as close as that between 24-h ambulatory blood pressure (ABP) and UAE. A strong association would suggest that AOBP may serve as an indicator of early renal impairment. METHODS: In a sample of 162 hypertensives, we compared AOBP with ABP measurements and their associations with UAE in two consecutive 24-h urine collections measured by an immunoturbidimetric assay. Microalbuminuria was defined as UAE of 30-300 mg/24 h. RESULTS: The age of the subjects was 53 ± 13 (mean ± s.d.) years. Twenty-two were microalbuminuric. In those, AOBP and 24-h ABP were higher than in the normoalbuminuric subjects: 152 ± 19 and 147 ± 20 vs. 138 ± 15 and 130 ± 11 mm Hg for systolic blood pressure (SBP), and 97 ± 15 and 92 ± 14 vs. 86 ± 10 and 82 ± 8 mm Hg for diastolic blood pressure (DBP) (P < 0.001). Correlations between AOBP and 24-h ABP with log-transformed urine albumin were 0.30 (P < 0.001) and 0.43 (P < 0.001) for SBP and 0.27 (P < 0.001) and 0.33 (P < 0.001) for DBP. Adjusting for age, sex, body mass index, and estimated glomerular filtration rate, both AOBP and 24-h ABP were independently associated with urine albumin (P < 0.001 for both associations). Receiver operating characteristics curve analysis showed a similar predictive ability for microalbuminuria for AOBP and for 24-h ABP (area under the curve: 0.819 (P < 0.001) for SBP, 0.836 (P < 0.001) for DBP vs. 0.830 (P < 0.001) for SBP and 0.845 (P < 0.001) for DBP). CONCLUSIONS: In this study, microalbuminuria correlated similarly with high-quality AOBP and ABP readings, further supporting the use of AOBP in the clinical setting.

Automated office blood pressure and 24-h ambulatory measurements are equally associated with left ventricular mass index.

BACKGROUND: To determine whether automated office blood pressure (AOBP) readings are associated with left ventricular mass (LVM) index as closely as those of 24-h ambulatory blood pressure (ABP) and also to confirm that the values of the two methods are comparable in the appraisal of blood pressure in a European population referred for suspected hypertension. METHODS: In a sample of 90 individuals with office hypertension, we compared AOBP to awake systolic ABP measurements (ABPM) values and their associations with LVM indices, expressed as LVM divided by body surface area (LVMI(BSA)) and by height(2.7) (LVMI(H)). RESULTS: Mean awake systolic ABP was 136 ± 16 mm Hg and mean systolic AOBP was 140 ± 15 mm Hg (P < 0.002). Mean awake diastolic ABP was 87 ± 11 mm Hg and mean diastolic AOBP was 88 ± 12 mm Hg (P = 0.08). AOBP readings were as closely associated with LVMI(BSA) (r = 0.37) as were those of awake systolic blood pressure (SBP) (r = 0.37). The correlation between LVMI(H) and both mean awake systolic ABP and mean systolic AOBP was r = 0.37 (P < 0.001) and r = 0.34 (P = 0.001), respectively. CONCLUSIONS: High-quality AOBP readings and ABP measurements correlate equally well with LVM indices, further supporting the use of AOBP in the clinical setting. Moreover, readings from both techniques are comparable in the assessment of blood pressure.

Why use automated office blood pressure measurements in clinical practice?

Automated office blood pressure (AOBP) measurement with the patient resting alone in a quiet examining room can eliminate the white-coat effect associated with conventional readings taken by manual sphygmomanometer. The key to reducing the white-coat response appears to be multiple blood pressure (BP) readings taken in a non-observer office setting, thus eliminating any interaction that could provoke an office-induced increase in BP. Furthermore, AOBP readings have shown a higher correlation with the mean awake ambulatory BP compared with BP readings recorded in routine clinical practice. Although there is a paucity of studies connecting AOBP with organ damage, AOBP values were recently found to be equally associated with left ventricular mass index as those of ambulatory BP. This concludes that in contrast to routine manual office BP, AOBP readings compare favourably with 24-hour ambulatory BP measurements in the appraisal of cardiac remodelling and, as such, could be complementary to ambulatory readings in a way similar to home BP measurements.