Hormone resistance is defined as a reduced or absence of target tissues responsiveness to a hormone, where the presentation is related to either a relative lack or excess of hormones. Various disorders of hormone resistance were encountered including, Laron syndrome, nephrogenic diabetes insipidus, thyroid hormone resistance syndrome, pseudohypoparathyroidism, insulin resistance, familial glucocorticoid deficiency, pseudohypoaldosteronism, X linked hypophosphatemic rickets and androgen insensitivity syndrome. The article gives a summary that presents, in concentrated form, what the primary care physicians need to know about recognition, clinical presentation, diagnosis, and management of various hormone resistance in children.

Background: For transcatheter aortic valve implantation (TAVI), accurate determination of valve size is crucial.
Multidetector Computed Tomography (MDCT) is considered gold standard, however sometimes there are conflicting measurements or aortic annulus is ambiguous between 2 prosthesis sizes. In such cases balloon sizing can serve in selecting valve size.

Methods: 110 patients were prospectively enrolled. Aortic annular diameter was measured by 2D TEE. Balloon sizing was done with balloons equal or 1 mm smaller than TEE measurements. Supra-aortic angiography was performed during balloon inflation. Contrast regurgitation and balloon movement indicated annulus size underestimation, balloon wasting indicated annulus size overestimation. Valve size selection was based on balloon sizing. Agreement between 2D TEE and balloon measurements was calculated. Inhospital outcomes related to valve sizing and routine predilation were determined.
Results: TEE was correctly sizing the valve in 81% of patients, oversizing in 17% and undersizing in only 2% compared with balloon sizing. Agreement between 2D TEE and balloon sizing measurement yielded a K value of 0.71. Hemodynamic instability after balloon sizing was observed in 2 patients, valve embolization occurred in one patient, no case of aortic rupture or coronary occlusion was detected. Two patients needed a second valve. Stroke rate was 0.9%, inhospital mortality was 1.8%. At hospital discharge, rate of significant PVL (≥ grade II) was 3.4% and pacemaker implantation rate was 6.4%.
Conclusions: Balloon sizing based on 2D TEE annular measurements represents an appropriate approach for selecting valve size with favorable outcomes.

Objectives: To describe and to validate a new technique for crossing stenotic aortic valves (AV).

Background: Current techniques for crossing the AV may be time-consuming and hazardous.

Methods: One hundred consecutive patients with severe aortic stenosis treated by transfemoral TAVI were prospectively selected to have an initial attempt of 5 min to cross the AV with a novel pigtail/J-wire technique before switching to the conventional Amplatz ®/straight wire approach. For the pigtail/J-wire technique, the catheter is placed 3–4 cm above the AV and turned anteriorly in the 30° RAO view. A J-wire pushed out of the pigtail-catheter will reach the anterior wall of the ascending aorta, forming a u-shaped curve above the AV. The height of the pigtail catheter determines the width of the curve, rotation will help to find an orientation, where the vertex of the curved J-wire easily passes the AV. We analyzed the primary success rate within 5 min and the mean crossing time required.

Results: Patients were 83.5 ± 5.5 years of age and predominantly male (62%). Primary success rate was 86%, AV crossing took 48.2 ± 34.6 s without complications. Fourteen failed cases were successfully managed with AL1- (6) and both, AL1- and AL2-catheters (8), respectively

Conclusions: The pigtail/J-wire technique for AV crossing is safe, simple and fast. Primary placement of a pigtail catheter into the left ventricle at a success rate of 86% facilitates TAVI procedures.

Objective

High-grade gliomas (HGGs) carry dismal prognosis with survival typically reported as less than a year. We explored the predictive value of qualitative and quantitative evaluations of post-treatment 99m-technetium-labelled methoxyisobutylisonitrile (^99mmTc-MIBI) brain single-photon emission computed tomography-computed tomography (SPECT/CT) tumor uptake in relation to overall survival (OS) in patients with HGG.

Methods

Thirty patients with pathologically or radiologically documented high-grade glioma (HGG) were prospectively recruited for this study (24 male, 6 female; mean age 43 ± 14 years). All patients had a clinical or radiological suspicion of residual/recurrent tumor after initial therapy. ^99mTc-MIBI brain SPECT/CT scanning was performed, and the scans were evaluated qualitatively on a five-point probability score (1–5, scores ≥3 considered positive for residual/recurrent tumor); and quantitively via drawing volumes of interest (VOI) on the suspected lesions and normal contralateral brain tissue. All patients were followed up for 1 year or till death.

Results

Positive visual MIBI results were associated with poor survival. Among 10 patients with negative MIBI scores, only two patients died (OS = 75%), while 11/20 patients reported positive on MIBI died, with a median survival of 9 months (OS = 14.5%; P = 0.03). All patients with active isocontour volume ≤1.96 cm³ were alive at the end of the study, compared to a median survival of 9 months and OS of 12% for patients with an isocontour volume of >1.97% (P = 0.003).

Conclusion

In patients with HGG, post-therapy brain SPECT/CT with ^99mTc-MIBI can provide useful prognostic information.