Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature

<p>Abstract</p> <p>Background</p> <p>Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings.</p> <p>Case Presentation</p> <p>We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy.</p> <p>Conclusions</p> <p>Our results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH.</p

Subcutaneous fat patterning in athletes: selection of appropriate sites and standardisation of a novel ultrasound measurement technique: ad hoc working group on body composition, health and performance, under the auspices of the IOC Medical Commission.

Background: Precise and accurate field methods for body composition analyses in athletes are needed urgently. Aim: Standardisation of a novel ultrasound (US) technique for accurate and reliable measurement of subcutaneous adipose tissue (SAT). Methods: Three observers captured US images of uncompressed SAT in 12 athletes and applied a semiautomatic evaluation algorithm for multiple SAT measurements. Results: Eight new sites are recommended: upper abdomen, lower abdomen, erector spinae, distal triceps, brachioradialis, lateral thigh, front thigh, medial calf. Obtainable accuracy was 0.2 mm (18 MHz probe; speed of sound: 1450 m/s). Reliability of SAT thickness sums (N=36): R2=0.998, SEE=0.55 mm, ICC (95% CI) 0.998 (0.994 to 0.999); observer differences from their mean: 95% of the SAT thickness sums were within ±1 mm (sums of SAT thicknesses ranged from 10 to 50 mm). Embedded fibrous tissues were also measured. Conclusions: A minimum of eight sites is suggested to accommodate inter-individual differences in SAT patterning. All sites overlie muscle with a clearly visible fascia, which eases the acquisition of clear images and the marking of these sites takes only a few minutes. This US method reaches the fundamental accuracy and precision limits for SAT measurements given by tissue plasticity and furrowed borders, provided the measurers are trained appropriately