Ambulatory blood pressure monitoring in children with aortic coarctation and kidney transplantation.

BACKGROUND: Ambulatory blood pressure monitoring (ABPM) has been found to be of significant importance in clinical practice because numerous blood pressure (BP) measurements may be made throughout the 24-hour period. OBJECTIVE: To assess the clinical utility of ABPM in children with secondary hypertension. METHODS: We studied 37 patients (21 boys and 16 girls), with a mean age of 16.4 +/- 4.1 years, after kidney transplantation and 38 patients (27 boys and 11 girls), with a mean age of 10.2 +/- 2.1 years, after surgical correction of aortic coarctation. Data, expressed as mean +/- SD, were analyzed after dividing the patients into 4 groups. Group A consisted of 25 patients receiving antihypertensive therapy; group B included 12 patients not receiving antihypertensive therapy. Group C included 25 patients operated on before 3 years of age (8 +/- 7 months of age); group D included 13 patients operated on after 3 years of age (74 +/- 29 months of age). RESULTS: In groups A and B, casual BP measurement showed that 16 of 37 patients (43%) were hypertensive; 24-hour ABPM detected a larger number of patients who were hypertensive (23 of 37, 62%); there were 18 in group A and 5 in group B. In groups C and D, casual BP measurement identified 6 of 38 (15%) patients as hypertensive, whereas 24-hour ABPM again identified a higher number (13 of 38, 34%). CONCLUSIONS: Our findings confirm that 24-hour ABPM is more sensitive than casual BP in detecting abnormal BP in patients at high risk for secondary hypertension.

Autosomal dominant neuromuscular disease with cylindrical spirals.

Cylindrical spirals (CS) have been reported in muscle biopsies from five individual cases, as well as in two belonging to one family where there was another affected member, clinically associated with cramps, pain, stiffness and/or weakness. Here we studied muscle biopsies of a 70-yr-old mother and her 52-yr-old son, the latter with an associated neuropathy, both with late clinical onset in whose family at least 10 other members, spanning five generations, were diversely affected by muscular weakness, gait disorders, motor impairment and/or scoliosis, featuring an autosomal dominant trait with variable expression. CS as the main pathological findings were observed by light microscopy mostly in type 2 fibres, consisting of subsarcolemmal or intermyofibrillar granular and/or rod-like clusters, bluish with haematoxylin, bright red with Gomori's modified trichrome, non- or lightly reactive with PAS, faintly coloured with NADH-TR, non-reactive with SDH or ATPase, strongly stained with non-specific esterase and myoadenylate deaminase. Ultrastructurally, CS appeared as concentrically wrapped lamellae 1-2 microns in diameter. On occasion CS merged into tubular vesicular structures strongly resembling tubular aggregates (TA). Dilation of terminal cisternae (TC) in their proximity supports an origin from the sarcoplasmic reticulum (SR). Variable gene expression possibly explains both the highly diverse clinical compromise and time of onset.