A IHE-Like Approach Method for Quantitative Analysis of PACS Usage.

Today, many hospitals have a running enterprise picture archiving and communication system (PACS) and their administrators should have the tools to measure the system activity and, in particular, how much it is used. The information would be valuable for decision-makers to address asset management and the development of policies for its correct utilization and eventually start training initiatives to get the best in resource utilization and operators' satisfaction. On the economic side, a quantitative method to measure the usage of the workstations would be desirable to better redistribute existing resources and plan the purchase of new ones. The paper exploits in an unconventional way the potential of the IHE Audit Trail and Node Authentication (ATNA) profile: it uses the data generated in order to safeguard the security of patient data and to retrieve information about the workload of each PACS workstation. The method uses the traces recorded, according to the profile, for each access to image data and to calculate how much each station is used. The results, constituted by measures of the frequency of PACS station usage suitably classified and presented according to a convenient format for decision-makers, are encouraging. In the time of the spending review, the careful management of available resources is the top priority for a healthcare organization. Thanks to our work, a common medium such as the ATNA profile appears a very useful resource for purposes other than those for which it was born. This avoids additional investments in management tools and allows optimization of resources at no cost.

End-stage renal disease secondary to IgA nephropathy in recessive dystrophic epidermolysis bullosa: a case report.

Epidermolysis bullosa (EB) consists of a group of dominant or recessive autosomal diseases characterised by skin and mucosa fragility. The lesions leave erosions and scars that, in turn, can cause stenosis of tracheal, oesophageal, and genitourinary tract mucosae. The significantly increased survival of EB patients has determined the onset of complications never observed before, including genitourinary disorders such as hydroureteronephrosis, recurrent urinary tract infections, renal amyloidosis, IgA nephropathy and post-infectious glomerulonephritis. A 6-year-old boy diagnosed with recessive dystrophic EB Hallopeau-Siemens type (RDEB-HS) was referred to our clinic because of microhaematuria that evolved into intra-infectious macrohaematuria. Renal biopsy revealed an increase in both extracellular matrix and mesangial cells, with a focal segmental glomerulosclerosis with severe chronic tubulointerstitial damage. Immunofluorescence showed IgA mesangium deposits. Five years later, he was started on haemodialysis, because of worsening renal function. This is a rare case of a child with EB who was successfully treated with haemodialysis. The pertinent literature has been reviewed.

Patients with biallelic mutations in the chloride channel gene CLCNKB: long-term management and outcome.

BACKGROUND: Little information on the management and long-term follow-up of patients with biallelic mutations in the chloride channel gene CLCNKB is available. METHODS: Long-term follow-up was evaluated from 5.0 to 24 years (median, 14 years) after diagnosis in 13 patients with homozygous (n = 10) or compound heterozygous (n = 3) mutations. RESULTS: Medical treatment at last follow-up control included supplementation with potassium in 12 patients and sodium in 2 patients and medical treatment with indomethacin in 9 patients. At the end of follow-up, body height was 2.0 standard deviation score or less in 6 patients; 2 of these patients had growth hormone deficiency. Body weight (