Polyarteritis nodosa in children: radiological aspects and diagnostic correlation

The radiological findings in 14 children with polyarteritis nodosa (PN) were evaluated in relation to clinical, laboratory, and histological findings. In this series, there were two major groups of radiological findings, one associated with renal insufficiency and the other with intestinal vascular insufficiency. No radiographic finding was pathognomonic. Two patients were suspected of having small bowel intussusception (SBI), and SBI was documented at laparotomy in a third patient. The diagnosis and management of PN in children is dependent upon the correlation of clinical and laboratory findings. Associated pertinent radiological features which may be useful in the management of children with PN are reviewed.     

A comparison of rates, risk factors, and outcomes of gestational diabetes between aboriginal and non-aboriginal women in the Saskatoon health district

OBJECTIVE: To determine possible differences in gestational diabetes mellitus (GDM) between aboriginal and non-aboriginal people in the Saskatoon Health District. RESEARCH DESIGN AND METHODS: This was a prospective survey of all women admitted for childbirth to the Saskatoon Royal University Hospital between January and July 1998. We compared prevalence rates, risk factors, and outcomes of GDM between aboriginal and non-aboriginal women. RESULTS: Information was obtained from 2,006 women, of whom 252 aboriginal and 1,360 non-aboriginal subjects had been tested for GDM. The overall rates of GDM were 3.5% for women in the general population and 11.5% for aboriginal women. For those living within the Saskatoon Health District, GDM rates were 3.7 and 6.4%, respectively. Multivariate analysis demonstrated that aboriginal ethnicity, most notably when combined with obesity, was an independent predictor for GDM. Pregravid BMI > or = 27 kg/m(2) and maternal age > or = 33 years were the most important risk factors for GDM in aboriginal women, whereas previous GDM, family history of diabetes, and maternal age > or = 38 years were the strongest predictors for GDM in non-aboriginal women. CONCLUSIONS: There may be fundamental differences in GDM between aboriginal and non-aboriginal people. Because GDM contributes to an increased risk for type 2 diabetes in aboriginal women and their offspring, the impact of prevention and optimal treatment of GDM on the type 2 diabetes epidemic in susceptible populations are important areas for further investigation.     

Increased frequency of HLA A2/DR4 and A2/DR8 haplotypes in young saskatchewan aboriginal people with diabetic end-stage renal disease

AIMS: To determine the association of HLA with diabetic end-stage renal disease (DESRD) in Saskatchewan aboriginal people. METHODS: This was a retrospective study of HLA profiles in four groups of Saskatchewan residents with ESRD diagnosed from 1980 to 1998: aboriginal people with and without DESRD, and non-aboriginal people with and without DESRD. The aboriginal DESRD group was also subdivided into those 50 years of age. Frequencies of individual and combinations of HLA antigens were compared between groups and subgroups. RESULTS: HLA data were available for 634 subjects. Young aboriginal people with DESRD had a higher frequency of HLA-A2 than older AB DESRD subjects (69 vs. 36%; p = 0.03), and of HLA-DR4 and/or DR8 compared to older AB DESRD subjects (91 vs. 68%; p = 0.07) and AB non-DESRD subjects (91 vs. 67%; p = 0.03). Over 65% of young AB DESRD subjects had either an A2/DR4 or A2/DR8 haplotype (odds ratio 5.09 [confidence intervals 1.35, 20.15] versus older AB DESRD subjects; odds ratio 3.32 [confidence intervals 1.20, 9.3] versus AB non-DESRD subjects). Forty percent of young AB DESRD subjects were homozygous for at least one of A2, DR4 or DR8. CONCLUSIONS: Our findings suggest that DESRD in young AB subjects with T2DM has a genetic basis related to HLA.     

The ubiquitously expressed MURR1 protein is absent in canine copper toxicosis

BACKGROUND/AIMS: Copper toxicosis (CT) in Bedlington terriers is an autosomal recessive disorder characterized by massive lysosomal copper accumulation in livers of affected dogs, and a defect in the biliary excretion of this metal. We propose that MURR1, the gene defective in canine CT, has a role in the regulation of copper excretion into bile during copper overload. METHODS: Polyclonal antibodies raised against full-length recombinant human MURR1 were used for immunoblot analysis and indirect immunofluorescence studies. RESULTS: Using Western blot analysis, these antibodies abundantly detected MURR1 as a 23 kDa protein in liver extracts of mice and dogs, but MURR1 was undetectable in the livers of affected Bedlington terriers. MURR1 was also detected in different tissues and cell lines; in cell lines the protein was found both in cytosol and membrane preparations. Consistent with this observation, indirect immunofluorescence staining revealed that in some cells MURR1 was associated with a vesicular compartment diffusely localized throughout the cell. CONCLUSIONS: The genomic deletion in MURR1 results in complete absence of MURR1 protein. Based on the unanticipated subcellular localization, our results suggest a role for MURR1 in the regulation of vesicular copper sequestration during copper overload.     

From "thrifty genotype" to "hefty fetal phenotype": the relationship between high birthweight and diabetes in Saskatchewan Registered Indians.

BACKGROUND: Intrauterine factors have been implicated in the pathogenesis of Type 2 diabetes mellitus (T2DM). METHODS: In a 1:1 matched pairs case-control study, high and low birthweight (HBW, LBW) rates in Saskatchewan Registered Indian (RI) diabetic cases were compared with corresponding rates in RI without diabetes, and non-RI people with and without diabetes. RESULTS: Birthweights were available for 73% of the 1,366 cases and 3 x 1,366 controls. A greater proportion of RI diabetics were born with HBW (> 4000 grams) compared to RI non-diabetics (16.2% vs 10.7%; p < 0.01). There was a significant association between HBW (but not LBW [< 2500 grams]) and diabetes for RI people (OR 1.63 [95% CI 1.20, 2.24]), which was stronger for RI females and strengthened progressively from mid to late 20th century. INTERPRETATION: Certain causes of HBW may predispose to subsequent development of T2DM in Canadian Aboriginal people ("hefty fetal phenotype" ["hefty fetal type"] hypothesis). Programs that optimize healthy pregnancies could reduce T2DM incidence in future generations.