Lipoprotein profiles in hypercholesterolemic children

Atherosclerosis is a process that begins in early life. Coronary heart disease is the result of complex interactions among a variety of risk factors of which hypercholesterolemia is but one. During routine screening, 500 children were identified with total cholesterol levels above the 95th percentile of 5.2 mmol/L (200 mg/dL). Lipoprotein profiles were then performed to confirm and delineate their lipid abnormalities. A definable lipid disorder was present in 85% of such children. Abnormal lipoprotein patterns included 292 children with type IIa, 99 with type IIb, and 25 with type IV phenotypes. An abnormally low high-density lipoprotein cholesterol level of less than 0.9 mmol/L (35 mg/dL) was observed in 20 children. Only 5% of patients were identified as being hypercholesterolemic because they had high-density lipoprotein cholesterol levels above the 95th percentile of 1.8 mmol/L (70 mg/dL). Thirty-two percent of children with total cholesterol levels above 5.2 mmol/L had a family member (sibling, parent, uncle, aunt, or grandparent) with a myocardial infarction prior to 55 years of age. Data from this study support universal cholesterol testing after 3 years of age and lipoprotein profiles for those with levels above 5.2 mmol/L.     

A new, highly sensitive assay for C-reactive protein can aid the differentiation of inflammatory bowel disorders from constipation- and diarrhoea-predominant functional bowel disorders

BACKGROUND: Patients presenting to gastroenterology clinics with symptoms suggestive of lower-bowel disorders often require extensive investigation to differentiate functional from organic disease. C-reactive protein (CRP) is a sensitive marker of systemic inflammation. Levels of CRP are frequently raised in cases of inflammatory bowel disease (IBD). However, using conventional assays, not all cases of IBD have a detectable level. OBJECTIVE: To determine whether a new highly sensitive CRP enzyme-linked immunosorbent assay (ELISA) can aid the differentiation between IBD and functional bowel disorders (FBDs) in gastroenterology outpatients presenting with lower-bowel symptoms. METHODS: Serum was taken from 224 subjects attending a gastroenterology outpatient clinic. Of these, 203 were new patients and 21 were follow-up patients with quiescent colitis. The serum was analysed using a sensitive in-house ELISA. All new patients had a rigid sigmoidoscopy and rectal biopsy. Patients were investigated as deemed appropriate by the attending physician. Notes were reviewed after at least 6 months to determine the final diagnosis. RESULTS: A cut-off value of 2.3 mg/l had a sensitivity of 100% and a specificity of 67% in differentiating FBD from new cases of IBD. The geometric mean CRP was 0.383 mg/l in the constipation-predominant FBD group, 1.435 mg/l in diarrhoea-predominant FBD, 1.455 mg/l in quiescent IBD, 8.892 mg/l in newly presenting cases of ulcerative colitis, and 13.123 mg/l in newly presenting cases of Crohn's disease. CONCLUSION: A new, highly sensitive assay for CRP may help to distinguish FBD from IBD.     

Primary osteosarcoma of the skull

Primary osteogenic sarcoma of the skull is an exceedingly rare condition. An adult male patient is described, who had a painless swelling in the right forehead that had rapidly enlarged in the previous 6 months. Radiological investigations showed a large destructive mass lesion involving the right side of the frontal bone with extension into the frontal sinus, causing marked extradural compression of brain parenchyma. Histopathological examination confirmed the lesion to be primary osteogenic sarcoma.     

Expression of differential nitric oxide synthase isoforms in human normal gastric mucosa and gastric cancer tissue

The present study investigated the expression and distribution of three isoforms of nitric oxide synthase (NOS) in different anatomical regions of the human stomach and in gastric neoplastic tissues by immunohistochemistry using specific antibodies. Intracellular localization of individual isoenzymes of NOS was detected in normal gastric mucosa. Gastric cancer tissues had a marked reduction of all three NOS isoforms expression. The expression of the endothelial NOS, neuronal NOS and inducible NOS in the tumor tissue was significantly lower than in normal gastric mucosa (P = 0.01, P = 0.02, P < 0.01, respectively). In the tumor tissue the expression of inducible NOS was significantly lower than the expression of both constitutive forms of NOS (P < 0.01). There was a tendency to higher expression of both constitutive forms of NOS in earlier stages T2 of the tumor compared to advanced T4 tumor. In contrast, the expression of inducible NOS was higher than in the advanced T4 tumor than in the earlier stages T2 of the tumor. The mapping of the expression of endothelial NOS, neuronal NOS and inducible NOS in human stomach showed higher expression of NOS isoforms in the distal third than in the proximal third of the stomach (P = 0.03, P = 0.04, P = 0.01, respectively). We conclude that there is greater expression of NOS in the stomach corpus and in antrum than in the proximal third of the normal human stomach mirroring the anatomical predilection of common pathological changes in this part of the human stomach. Furthermore, there was loss of the expression of individual isoenzymes in gastric neoplasms.      

Predictive validity of a procedure for pediatric hearing instrument fitting

In 1994, Moodie, Seewald, & Sinclair described the development of a clinical procedure for predicting real-ear hearing instrument performance in young children. The purpose of the present study was to determine the validity of this procedure for predicting the real-ear aided gain (REAG) and real-ear saturation response (RESR) of hearing instruments worn by children. To this end, both the REAG and RESR were measured, through probe-microphone measures, and predicted, using the Moodie et al. procedure. The findings confirmed that the 2-cc coupler-based procedure, with individualized acoustic transforms, described by Moodie et al., resulted in highly accurate predictions of real-ear hearing instrument performance for both REAG and RESR at five test frequencies. The implications of these findings for the clinical fitting of hearing instruments in infants and young children are discussed.