This review confirms that the patient's position is a significant variable during urodynamics and that supine cystometry will fail to detect a significant percentage of patients with DO. We suggest that all patients should be filled sitting or standing, unless physically disabled. It seems desirable for the International Continence Society (ICS) to extend its "Good urodynamic practice guideline" [Schafer et al., Neurourol Urodyn 21:261-74, 2002] to cover this important issue.
We studied causes of mandibular fractures treated in oral and maxillofacial units in three countries in years 1990-2000 in Kuwait (n=596), 1995-2000 in Canada (n=228), and 1990-99 in Finland (n=268). Of the Finnish patients, 27% were women. Corresponding percentages in Kuwait and Canada were 13 and 17%, respectively. Traffic crashes were the cause of injury in 55% of the cases in Kuwait and 33% in Oulu, but only 7% in Toronto. In Kuwait, the victims were often young people, which is why more traffic education, more control of speed, and more control of the use of safety belts should be implemented. Assault was the cause in 54% in Toronto, 12% in Kuwait, and 37% in Oulu. Falling was the cause in 22% of the cases in Kuwait. Alcohol was implicated in 21% of cases in Canada and 15% in Finland.
A combination of noninvasive urodynamics and ultrasound derived measures provide promising methods of diagnosing bladder outlet obstruction. However, pressure flow studies still remain the gold standard for assessing bladder outlet obstruction.
Ultrasound derived measures such as bladder wall thickness and bladder weight offer a promising possibility of diagnosing bladder outlet obstruction noninvasively. However, further reproducibility and large accuracy studies with better methodological standards are required before they can replace pressure flow studies.
Background
Alström syndrome (AS) is a rare autosomal recessive ciliopathy with a wide spectrum of clinical features, including cone–rod retinal dystrophy, neuronal deafness, severe insulin resistance and major organ failure. The characteristics of renal disease in the syndrome have not been systematically described. The aim of this study is to define the onset and progression of renal disease in AS.
Method
Prospective observational cohort study.
Setting and Participants
Thirty-two adult subjects from a national specialist clinic in UK and 86 subjects from an international AS registry were studied.
Outcomes
First, an international registry cross-sectional study across all age groups to determine change in kidney function was performed. Secondly, a detailed assessment was carried out of adult AS patients with serial follow-up to determine incidence, aetiology and progression of renal disease.
Analytical approach
Generalized estimating equations were used to evaluate the relationship between age and estimated glomerular filtration rate (eGFR). Associations between patient factors and eGFR levels were then assessed in the adult AS cohort.
Results
The international registry study of the renal function of 118 subjects with AS (median age 21 years) showed a rapid decline with age, at an average of −16.7 and −10.9 mL/min/1.73 m2 per decade in males and females, respectively. In a UK national cohort of 32 patients with AS (median age 22 years), 20/32 (63%) had chronic kidney disease (CKD) Stage 3 or above based on eGFR <60 mL/min/1.73 m2 or evidence of albuminuria. Hyperuricaemia was noted in 25/32 (79%). Structural abnormalities such as nephrocalcinosis without hypercalcaemia and cysts were observed in 20/32 (63%) subjects. Lower urinary tract symptoms were frequent in 17/19 (70%) of AS patients. Histological evidence showed mixed tubulo-interstitial and glomerular disease.
Conclusions
This is the first study to demonstrate that renal disease is the hallmark of AS, which starts early and progresses with age, leading to a high prevalence of advanced CKD at young age. AS should be considered in the differential diagnosis of rare genetic renal diseases.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.