Sonography in the diagnosis and management of pancreatic pseudocysts and effusions in childhood

Blunt trauma to the abdomen is the main cause of pancreatic injury in childhood. One of the most common causes of injury is child abuse. Pancreatitis, pancreatic pseudocyst, and loculated peripancreatic effusion are sequellae of this trauma. The diagnosis of these lesions is aided by ultrasound. Pancreatic injury in children may require different methods of management than it does in adults. Persistently elevated serum amylase levels and increasing cyst size are indications for surgical intervention. However, the natural history of a pseudocyst in an otherwise normal child may lead to spontaneous resolution. In this report, three cases of pancreatic pseudocyst caused by child abuse are presented. In two of the patients, the cysts underwent spontaneous resolution.     

Prosthetic arterial graft occlusion: a complication of tourniquet use during arteriography

Tourniquet-induced reactive hyperemia, generally considered a safe procedure for enhancing visualization of the arteries in the distal lower extremity, caused acute occlusion of a Dacron arterial graft in one patient. This rare complication can be avoided through use of an alternative such as a chemical vasodilator in patients with susceptible synthetic vascular grafts.     

Topology and order of formation of interchain disulfide bonds in von Willebrand factor

Interchain disulfide bonds between the subunits in von Willebrand factor (vWf) dimers and in vWf multimers have been studied using some unique features of the cultured human umbilical vein endothelial cell system. Ammonium chloride inhibition of multimerization of vWf allowed selective examination of vWf dimeric molecules, and monoclonal antibody against the vWf propolypeptide was used to separate pro-vWf dimers from mature dimers. After cleavage of dimers and multimers with Staphylococcus aureus V-8 protease, the location of interchain disulfide bonds in amino (N)-terminal or carboxyl (C)-terminal fragments was determined by gel electrophoresis under reduced and nonreduced conditions. The first interchain disulfide bonds formed during dimerization are in the C-terminal region of the subunits, whereas interdimer disulfide bonds are located in the N-terminal portion. These data confirm recent electron microscopic projections of disulfide bond locations and provide support to the hypothetical role of the propolypeptide in the multimerization process.     

Abdominal aortic aneurysm repair results in aortic stiffening

Long-term follow-up studies show that all-cause mortality remains unchanged after repair of abdominal aortic aneurysm (AAA), possibly because of an increased cardiovascular risk in this high risk group. Repair of the AAA introduces a semirigid conduit into the circulation with unknown effects on the central aortic haemodynamics, such as pulse-wave velocity (PWV). One recent study revealed that a 1 m/s increase in PWV confers a 15% increased risk of cardiovascular events. We investigated whether central aortic haemodynamic changes resulting from AAA repair could be contributing to this excess cardiovascular risk.In nine patients undergoing endovascular aneurysm repair of infrarenal AAA who were assessed for changes in carotid-femoral PWV (cfPWV), mean cfPWV (n=9) was 10·3 m/s (SD 1·0) preoperatively. 1 week and 6 weeks postoperatively, mean cfPWV was 10·2 m/s and 11·2m/s, respectively (mean difference at 6 weeks 0·9m/s [95% CI 0·1–1·8], p=0·03).AAA repair appears to result in a functional stiffening of the aorta. A larger powered study is in progress to confirm this finding and also investigate whether this phenomenon is sustained in the long term. Intensive cardiovascular risk monitoring and pharmacomodulation may be indicated in this high-risk population.FundingBritish Heart Foundation.     

An integrated strategy for the diagnosis of neuronal ceroid lipofuscinosis types 1 (CLN1) and 2 (CLN2) in eleven Latin American patients

The neuronal ceroid lipofuscinoses (NCLs) are a family of progressive neurodegenerative diseases that are characterized by the cellular accumulation of ceroid lipofuscin-like bodies. NCL type 1 (CLN1) and type 2 (CLN2) are caused by deficiencies of the lysosomal enzymes palmitoyl-protein thioesterase 1 (PPT-1) and tripeptidyl peptidase 1 (TPP-1), respectively. In this study, 118 Latin American patients were examined for NCL using an integrated multidisciplinary program. This revealed two patients affected by CLN1 and nine by CLN2. Both CLN1 patients had a juvenile-onset phenotype with mutation studies of one patient demonstrating the known mutation p.Arg151X and a novel mutation in intron 3, c.363-3T>G. Six of the CLN2 patients presented with the 'classical' late-infantile phenotype. The remaining three patients, who were siblings, presented with a 'protracted' phenotype and had a higher level of residual TPP-1 activity than the 'classical' CLN2 patients. Genotype analysis of the TPP1 gene in the 'classical' CLN2 patients showed the presence of the known mutation p.Arg208X and the novel mutations p.Leu104X, p.Asp276Val, and p.Ala453Val. The siblings with the 'protracted' phenotype were heterozygous for two known TPP1 mutations, p.Gln66X and c.887-10A>G. This multidisciplinary program is also being used to diagnose other NCL types.     

Safety of a fat-reduced diet: the Dietary Intervention Study in Children (DISC)

OBJECTIVE: To assess the relationship between energy intake from fat and anthropometric, biochemical, and dietary measures of nutritional adequacy and safety. DESIGN: Three-year longitudinal study of children participating in a randomized controlled trial; intervention and usual care group data pooled to assess effects of self-reported fat intake; longitudinal regression analyses of measurements at baseline, year 1, and year 3. PARTICIPANTS: Six hundred sixty-three children (362 boys and 301 girls), 8 to 10 years of age at baseline, with elevated low-density lipoprotein cholesterol, who are participants of the Dietary Intervention Study in Children. MEASURES: Energy intake from fat assessed from three 24-hour recalls at each time point was the independent variable. Outcomes were anthropometric measures (height, weight, body mass index, and sum of skinfolds), nutritional biochemical determinations (serum ferritin, zinc, retinol, albumin, beta-carotene, and vitamin E, red blood cell folate, and hemoglobin), and dietary micronutrients (vitamins A, C, E, thiamin, riboflavin, niacin, vitamins B-6, B-12, folate, calcium, iron, zinc, magnesium, and phosphorus). RESULTS: Lower fat intake was not related to anthropometric measures or serum zinc, retinol, albumin, beta-carotene, or vitamin E. Lower fat intake was related to: 1) higher levels of red blood cell folate and hemoglobin, with a trend toward higher serum ferritin; 2) higher intakes of folate, vitamin C, and vitamin A, with a trend toward higher iron intake; 3) lower intakes of calcium, zinc, magnesium, phosphorus, vitamin B-12, thiamin, niacin, and riboflavin; 4) increased risk of consuming less than two-thirds of the Recommended Dietary Allowances for calcium in girls at baseline, and zinc and vitamin E in boys and girls at all visits. CONCLUSIONS: Lower fat intakes during puberty are nutritionally adequate for growth and for maintenance of normal levels of nutritional biochemical measures, and are associated with beneficial effects on blood folate and hemoglobin. Although lower fat diets were related to lower self-reported intakes of several nutrients, no adverse effects were observed on blood biochemical measures of nutritional status. Current public health recommendations for moderately lower fat intakes in children during puberty may be followed safely.