Organisational Performance and the Use of Multiple Performance Measures in an Emerging Market

Purpose This study is an empirical investigation of the relationship between the use of 41 multiple performance measures (MPMs), including financial performance measures (FPM), non-financial performance measures (NFPMs) and organisational performance (OP) in Libya. Design/methodology/approach The results are based on cross-sectional questionnaire survey data from 132 Libyan companies (response rate 61%), which were obtained just before the so-called Arab Spring. Findings MPMs are used by both manufacturing and non-manufacturing companies. Libyan business organisations are more likely to use FPMs than NFPMs. However, these companies still rely more heavily on FPMs. The relationships between the use of NFPMs and OP and the use of MPMs and OP are positive and highly significant. The relationship between the use of FPMs and OP is positive but not significant. Research limitations/implications The high power distance associated with the conservative, Libyan, Arab context will reinforce the tendency to use FPMs more than NFPMs. This may provide a performance advantage to those organisations which do adopt NFPMs. Practical implications Although there may be institutional barriers to the use of NFPMs in Libya, and other emerging markets, these are not insuperable and there is a payoff to their use. Originality/value No previous studies of emerging markets, such as the Middle East or North Africa, have looked at the relationship between OP and the adoption of such a large array of MPMs

A cohort of 17 patients with kyphoscoliotic Ehlers-Danlos syndrome caused by biallelic mutations in FKBP14: expansion of the clinical and mutational spectrum and description of the natural history.

PurposeIn 2012 we reported in six individuals a clinical condition almost indistinguishable from PLOD1-kyphoscoliotic Ehlers-Danlos syndrome (PLOD1-kEDS), caused by biallelic mutations in FKBP14, and characterized by progressive kyphoscoliosis, myopathy, and hearing loss in addition to connective tissue abnormalities such as joint hypermobility and hyperelastic skin. FKBP14 is an ER-resident protein belonging to the family of FK506-binding peptidyl-prolyl cis-trans isomerases (PPIases); it catalyzes the folding of type III collagen and interacts with type III, type VI, and type X collagens. Only nine affected individuals have been reported to date.MethodsWe report on a cohort of 17 individuals with FKBP14-kEDS and the follow-up of three previously reported patients, and provide an extensive overview of the disorder and its natural history based on clinical, biochemical, and molecular genetics data.ResultsBased on the frequency of the clinical features of 23 patients from the present and previous cohorts, we define major and minor features of FKBP14-kEDS. We show that myopathy is confirmed by histology and muscle imaging only in some patients, and that hearing impairment is predominantly sensorineural and may not be present in all individuals.ConclusionOur data further support the extensive clinical overlap with PLOD1-kEDS and show that vascular complications are rare manifestations of FKBP14-kEDS

Protein C levels in nephrotic syndrome: Use of a new enzyme‐linked immunoadsorbent assay for protein C antigen

Activated protein C is a potent, physiologic anticoagulant that inactivates the activated forms of factors V and VIII as well as facilitates in vivo fibrinolysis. We developed a competitive protein‐binding enzyme‐linked immunoadsorbent assay (ELISA) for protein C that was utilized to investigate if the hypercoagulability of the nephrotic syndrome is related to a deficiency of circulating plasma protein C. Protein C was measured in plasma of 11 patients with nephrotic syndrome (24 hr protein 8.4 ± 1.6 g, SEM; serum creatinine 4.2 ± .74 mg/dl, SEM). Ten azotemic nonnephrotic patients were employed as controls (serum creatinine 6.0 ± 1.25 mg/dl, SEM). No significant reduction of protein C values was observed (mean 108%, range 65–200%) in nephrotic patients when compared with the controls (mean 127%, range 100–200%) even though protein C antigen was present in all nephrotic urine samples tested. Also, no correlation existed between blood levels of urea nitrogen, creatinine, albumin, total protein, or 24‐hr urine protein excretion and the observed protein C values. These results suggest that in patients with the nephrotic syndrome, a hyper‐coagulable state may not be related to a deficiency of protein C and that the level of this zymogen in nephrotic syndrome reflects a dynamic balance between urinary losses and systemic production