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Biomedical subjects

A Notarbartolo

Publications and source records attributed to A Notarbartolo.

At least 37 records · Page 2Linked to original sources

Eating behaviour, body mass index and lipids of children in a free-living rural Sicilian population.

BACKGROUND AND AIM: The preliminary findings of an epidemiological study conducted in 1989 in Ventimiglia di Sicilia (a rural village in the hilly hinterland 50 km from Palermo) described a population mainly made up of farmers, which had a very low incidence of early cardiovascular mortality, lower total cholesterol plasma concentration than the Italian average, and nutritional habits following the criteria of the Southern Italian Mediterranean diet. Since a high prevalence of body weight abnormalities was found in this population and an 11-year follow-up revealed that both body weight and fat distribution were significantly associated with total and cardiovascular mortality, we studied the eating behaviour, body weight and lipid levels of 249 school-children living in the village. The aim of the study was to verify whether the children showed similar body weight abnormalities and quantitative dietary excesses as the adults of the same families. METHODS AND RESULTS: The dietary habits of 97 children (about one third of the study population) had a higher total caloric, monounsaturated fatty acid and fibre intake, and a lower saturated fatty acid and cholesterol intake, than an all-Italy sample of school-children. The lipid profile of this group of very young subjects was fairly good, but 13% had LDL-cholesterol levels of more than 130 mg/dL and 11% had HDL-cholesterol levels of less than 35 mg/dL. About 28% were considered to be above the 95th percentile of body weight and this was associated with a higher daily total caloric intake. CONCLUSIONS: Because obesity is associated with a high total caloric intake and various other risk factors even in young children, it is possible that a successful prevention campaign including behavioural education in schools could reduce the incidence of cardiovascular disease at older ages.

Body Mass Index↗

Effects of gemfibrozil on insulin sensitivity and on haemostatic variables in hypertriglyceridemic patients.

In order to assess the efficacy of gemfibrozil on lipid and haemostatic parameters in patients with plurimetabolic syndrome, a multicenter double-blind placebo controlled, parallel study was carried out in 56 patients with primary hypertriglyceridemia and glucose intolerance. These patients had elevated PAI activity and antigen and t-PA antigen levels at rest and after venous occlusion. Gemfibrozil reduced plasma triglyceride levels (P<0.001), whereas it increased free fatty acids (P<0.05) and high density lipoprotein cholesterol levels (P<0.05). In those patients reaching normalization of plasma triglyceride levels (triglyceride reduction > or =50%) (n=15), insulin levels (P<0.05) as well as the insulin resistance index were reduced by gemfibrozil treatment, suggesting an improvement of the insulin resistance index in this patient subgroup. Gemfibrozil treatment did not affect plasma fibrinolysis or fibrinogen levels, despite marked reduction of plasma triglycerides and improvement of the insulin sensitivity associated with triglyceride normalization.

Adult↗

Evidence of very delayed clinical reactions to cow's milk in cow's milk-intolerant patients.

BACKGROUND: In patients with cow's milk protein intolerance (CMPI), delayed clinical reactions to cow's milk (CM) ingestion may be misdiagnosed if the clinical symptoms are not "classical" and there is a long time lapse between ingestion of CM and the clinical reaction. The aim was to evaluate the clinical outcome of CMPI in a cohort of CM-intolerant children, with particular attention to the occurrence of clinical manifestations beyond 72 h after CM challenge. METHODS: Eighty-six consecutive patients (44 boys, 42 girls) with new CMPI diagnoses were enrolled; median age at diagnosis was 4 months. Patients were followed up for a mean period of 40 months. In all patients, CMPI diagnosis was made on the observation of symptoms, their disappearance after elimination diet, and their reappearance on double-blind CM challenge. At CMPI diagnosis, immunologic tests to demonstrate IgE-mediated hypersensitivity were performed. After 12 months of CM-free diet, CM tolerance was re-evaluated with a CM challenge continued at home for up to 30 days, according to a double-blind, placebo-controlled method. Patients who did not achieve CM tolerance continued a CM-free diet and subsequently underwent yearly CM challenge. RESULTS: The percentages of CMPI patients who became CM-tolerant after 1, 2, and 3 years of CM-free diet were 30%, 54.5%, and 70%, respectively. At the end of the follow-up period, 26/86 subjects showed persistent CMPI; these patients had a higher percentage of positivity of total serum IgE (P<0.05), RAST (P<0.01), and cutaneous prick tests for CM antigens (P<0.001) than all the others. At CMPI diagnosis, all patients had a clinical reaction within 72 h from the beginning of the CM challenge; at the subsequent "cure" challenges, we observed patients who first reacted to CM more than 72 h after ingestion. In total, 10 out of 86 patients showed "very delayed reactions"; in these patients, the mean time between the beginning of CM challenge and the onset of a clinical symptom was 13.3 days (range 4-26 days). The number of "very late reactors" increased from the first to the third of the "cure" CM challenges, performed at yearly intervals. The "very delayed" CMPI manifestations in these subjects were constipation (five cases), wheezing (two cases), dermatitis plus constipation (two cases), and dermatitis alone (one case); in 6/10 patients, the symptoms observed at the "cure challenge" were different from those at CMPI onset. CONCLUSIONS: Very delayed clinical reactions to reintroduction of CM in the diet can occur in CMPI patients; thus, accurate follow-up and frequent outpatient observation in patients with a long history of CMPI are probably more useful and safer than prolonged CM challenge.

Animals↗

Carotid atherosclerosis in renal transplant recipients: relationships with cardiovascular risk factors and plasma lipoproteins.

BACKGROUND: Renal transplant recipients have an increased incidence of cardiovascular disease, but less data exist about cerebrovascular atherosclerosis. In this study, we assessed the prevalence of carotid lesions as evaluated by B-mode ultrasonography in a group of renal transplant recipients, and we evaluated univariate and multivariate relationships between common risk factors and plasma lipoproteins and carotid lesions. METHODS: Fifty-seven renal transplant recipients and 113 age- and gender-matched controls underwent a complete clinical visit for the evaluation of risk factors present. In all subjects, a blood sample was collected for lipoprotein determination, and an ultrasound high-resolution B-mode imaging examination of the common carotid arteries was performed. RESULTS: We found that among renal transplant recipients, there was a significantly increased prevalence of subjects with plaque in comparison with controls (24.6% vs. 6.2%, P<0.001). At multiple analysis, carotid lesions were independently associated with age, hypertension, diabetes, smoking habit, and the presence of cardiovascular disease in controls and with age and hypertension in renal transplant recipients. Neither the lipid profile nor the presence of dyslipidemias was related to carotid score in renal transplant recipients, whereas a nonsignificant trend was observed in controls. Finally, in transplant patients, we did not find any association between carotid lesions and high-density lipoprotein subfractions. CONCLUSIONS: Age and hypertension are the main predictors of extracranial cerebrovascular atherosclerosis after renal transplantation. Because carotid lesions may represent a useful predictive marker of clinical events in nontransplant subjects, carotid artery evaluation by B-mode ultrasound might be routinely included in the management of renal transplant patients.

Adult↗

Rapid screening of the LDL receptor point mutation FH-Genoa/Palermo. Mutation in brief no. 238. Online.

The LDL-receptor gene point mutation FH-Genoa/Palermo is the most frequent mutation responsible for Familial Hypercholesterolemia in Sicily. The mutation does not introduce or abolish any useful restriction site. We establish a GeneComb-based strategy to identify this mutation in a population of Sicilian unrelated clinically diagnosed FH probands. The method was very sensitive and specific; 12 out of 90 (13.3%) unrelated FH probands were found to carry the FH-Genoa/Palermo mutation. According to these results, the FH-Genoa/Palermo is the more frequent LDL-receptor gene mutation among the Sicilian FH patients. Moreover FH-Genoa/Palermo is the mutation cluster to date more represented in Southern Italy.

Genetic Testing↗

Lipoprotein(a) levels in relation to albumin concentration in childhood nephrotic syndrome.

BACKGROUND: Lipoprotein(a) [Lp(a)] is a lipoprotein consisting of a low-density lipoprotein (LDL) particle linked to a polymorphic glycoprotein, apoprotein(a) [apo(a)]. Prior studies have reported high Lp(a) levels in the nephrotic syndrome, but it is still controversial whether this is due to the degree of hypoalbuminemia or proteinuria. METHODS: To investigate a model of nephrotic syndrome in the absence of renal failure, we studied a group of 84 children in different clinical stages of the disease for a period of five years. We evaluated the direct relationships between lipoproteins, including Lp(a), and/or plasma albumin and proteinuria. RESULTS: Lp(a) levels were significantly higher in the subjects with the active disease compared with patients in remission, and were also significantly different when subjects were ranked by albumin quartiles. Multiple regression analysis revealed that Lp(a) levels were inversely correlated with apo(a) isoform size and plasma albumin levels but not with the proteinuria/creatinine clearance ratio. Among subjects in complete remission, Lp(a) levels were different in patients with albumin levels below or above the fifth percentile. After the improvement of the clinical stage of the disease, the Delta% variation of albumin levels was related to the Delta% of apoB and LDL cholesterol (LDL-C), but not with the Delta% variation of Lp(a), whereas the Delta% variation of LDL-C was, in turn, related to the Delta% of Lp(a) levels. CONCLUSIONS: These results suggest that in the childhood nephrotic syndrome, the increased Lp(a) levels are mainly related to hypoalbuminemia, probably through a mechanism involving apoB overproduction, which leads to an increased number of LDL particles to be converted into Lp(a).

Apolipoproteins B↗

Exocrine pancreatic function and fat malabsorption in human immunodeficiency virus-infected patients.

BACKGROUND: Nutrients malabsorption frequently occurs in human immunodeficiency virus (HIV)-infected patients, but very few studies have investigated exocrine pancreatic digestive capacity in these patients. We therefore evaluated the frequency of exocrine pancreatic impairment and its eventual relation with fat malabsorption in HIV-infected patients. METHODS: Thirty-five HIV-infected patients (30 male, 5 female: mean age +/- standard deviation, 33.6 +/- 7.2 years) and 51 sex- and age-matched controls without gastroenterologic diseases were studied. In all subjects fecal elastase 1 (EL-1) was assayed, and fecal fat excretion was evaluated with the steatocrit test. RESULTS: Nineteen of 35 (54%) HIV-infected patients showed subnormal EL-1 values, whereas all the controls had normal values; furthermore, EL-1 values were significantly lower in patients than in controls: mean (95% confidence intervals), 207 ( 164-251 ) microg/g versus 312 (291-332) microg/g (P < 0.0001). Increased fecal fat excretion was observed in almost all (25 of 35) HIV-infected patients, and an inverse but not significant correlation was found between fecal EL-1 and steatocrit values. No association was found between reduced fecal EL-1 and the severity of HIV disease or nutritional and immunologic status. Opportunistic infections and drug administration had no influence on EL-1 concentrations in stools. CONCLUSIONS: Reduced exopancreatic function is frequent in HIV-infected patients but does not seem to be a major factor contributing to fat malabsorption.

Adult↗

Effects of Mediterranean diet on lipid levels and cardiovascular risk in renal transplant recipients.

BACKGROUND: Renal transplant recipients have an increased incidence of cardiovascular disease. These patients present abnormalities of lipoprotein profile which are persistent and involve an increasing number of individuals, suggesting the opportunity of an early therapeutic intervention. METHODS: We evaluated the effects of a 10- to 12-week diet based on the American Heart Association step-one diet criteria, modified with an increased intake of monounsaturated fats and alimentary fibers, on lipid profile and lipid-related cardiovascular risk in 78 normolipidemic and hyperlipidemic renal transplant recipients. RESULTS: Diet led to a significant reduction in total cholesterol levels by 10%, triglycerides by 6.5%, low-density lipoprotein (LDL)-cholesterol by 10.4% and LDL-cholesterol/high-density lipoprotein (HDL)-cholesterol ratio by 10%, whereas HDL-cholesterol levels remained unchanged. Dividing renal transplant recipients into risk classes according to the National Cholesterol Expert Program guidelines and LDL-cholesterol levels, we observed a progressively increasing reduction in total cholesterol and LDL-cholesterol levels among 'desirable LDL-cholesterol', 'borderline high-risk LDL-cholesterol' and 'high-risk LDL-cholesterol' patients, while HDL-cholesterol levels did not change in any group and the LDL-cholesterol/HDL-cholesterol ratio significantly decreased in 'borderline high-risk LDL-cholesterol' and in 'high-risk LDL-cholesterol' patients (respectively by 6.8%, p < 0.05, and by 21.1%, p < 0.0001). Reduction in triglyceride levels was statistically significant only in subjects with 'desirable LDL-cholesterol' (by 12.3%, p < 0.01). Patients in the 'desirable LDL-cholesterol' class increased from 28 (35.9% of total patients) before diet to 45 (57.7% of total patients, p < 0.01), while subjects in the 'high-risk LDL-cholesterol' class reduced from 24 (30.8% of total patients) to 8 (10.2% of total patients, p < 0.005). CONCLUSION: These data suggest the possibility of a nutritional hypolipidemic approach in renal transplant recipients, even if normolipidemic. Dietetic treatment determined an inversion in the typical trend of renal transplant recipients, reducing instead of increasing the number of subjects with hypercholesterolemia, permitting the selection of individual candidates for further pharmacological treatment by carefully evaluating risk/benefit costs.

Adult↗

Intolerance of cow's milk and chronic constipation in children.

BACKGROUND: Chronic diarrhea is the most common gastrointestinal symptom of intolerance of cow's milk among children. On the basis of a prior open study, we hypothesized that intolerance of cow's milk can also cause severe perianal lesions with pain on defecation and consequent constipation in young children. METHODS: We performed a double-blind, crossover study comparing cow's milk with soy milk in 65 children (age range, 11 to 72 months) with chronic constipation (defined as having one bowel movement every 3 to 15 days). All had been referred to a pediatric gastroenterology clinic and had previously been treated with laxatives without success; 49 had anal fissures and perianal erythema or edema. After 15 days of observation, the patients received cow's milk or soy milk for two weeks. After a one-week washout period, the feedings were reversed. A response was defined as eight or more bowel movements during a treatment period. RESULTS: Forty-four of the 65 children (68 percent) had a response while receiving soy milk. Anal fissures and pain with defecation resolved. None of the children who received cow's milk had a response. In all 44 children with a response, the response was confirmed with a double-blind challenge with cow's milk. Children with a response had a higher frequency of coexistent rhinitis, dermatitis, or bronchospasm than those with no response (11 of 44 children vs. 1 of 21, P=0.05); they were also more likely to have anal fissures and erythema or edema at base line (40 of 44 vs. 9 of 21, P<0.001), evidence of inflammation of the rectal mucosa on biopsy (26 of 44 vs. 5 of 21, P=0.008), and signs of hypersensitivity, such as specific IgE antibodies to cow's-milk antigens (31 of 44 vs. 4 of 21, P<0.001). CONCLUSIONS: In young children, chronic constipation can be a manifestation of intolerance of cow's milk.

Animals↗

Ultrasound detection of abdominal lymphadenomegaly in subjects with hepatitis C virus infection and persistently normal transaminases: a predictive index of liver histology severity.

BACKGROUND/AIMS: The indications for liver biopsy in anti-HCV-positive patients with persistently normal alanine aminotransferase levels are not clearly established. Recent studies have correlated the presence of abdominal lymphoadenomegaly with disease severity in patients with chronic hepatitis C. Our study aimed to evaluate the frequency of abdominal lymphoadenomegaly in an anti-HCV positive blood donor population with persistently normal alanine aminotransferase and the relationship of abdominal lymphoadenomegaly with the severity of liver changes. METHODS: Eighty-six anti-HCV positive blood donors (58 M, 28 F) with normal alanine aminotransferase were followed up for a median of 31 months (range 12-50). To evaluate the frequency of abdominal lymphoadenomegaly, all patients underwent ultrasound scan. The common parameters of liver function as well as serum HCV RNA levels were determined. Histological changes were evaluated both in a conventional manner and using the numerical scoring systems of Knodell and Desmet. RESULTS: Of the 86 donors, 68 (79%) maintained persistently normal alanine aminotransferase levels during follow-up, and abdominal lymphoadenomegaly was present in 15 of them (22.0%). The remaining 18 donors (21%) showed rises in alanine aminotransferase above normal levels during the follow-up and seven of them (38%) had abdominal lymphoadenomegaly (p=n.s.). In the subjects with normal alanine aminotransferase, there were no significant differences in the common parameters of liver function and the serum presence of HCV RNA between those with or without abdominal lymphoadenomegaly. Normal liver was found in five patients without abdominal lymphoadenomegaly, but never in patients with abdominal lymphoadenomegaly. Analysis with the Mantel-Haenszel test showed a trend toward more serious changes in patients with abdominal lymphoadenomegaly (chi-square MH=9.5, p<0.003). Histological changes did not differ when Knodell's score was used; in contrast, staging, evaluated by Desmet's score, and periportal necrosis were significantly higher in subjects with abdominal lymphoadenomegaly (p<0.01 and p<0.004, respectively). Multiple logistic regression showed a significant relation between histological changes and the presence in serum of HCV RNA (p<0.004) and gamma-globulin (p<0.002), and abdominal lymphoadenomegaly p<0.003). CONCLUSION: Our study shows a prevalence of 22.0% of abdominal lymphoadenomegaly in anti-HCV positive subjects with normal alanine aminotransferase values and a relationship with the degree of liver histology change. Therefore, we suggest that the evidence of abdominal lymphoadenomegaly in these patients could be an indication to perform liver biopsy.

Abdomen↗

Sideropenic anemia and celiac disease: one study, two points of view.

Recent studies have pointed to the relationship between iron deficiency anemia and celiac disease, although data on the prevalence of celiac disease in anemic patients have been conflicting, and there is no agreement on the best screening procedure for CD in these patients. Our aims were to evaluate the relationship between anemia and celiac disease (CD) from two different points of view--the hematology clinic and the pediatric gastroenterology department--and to evaluate the utility of anti-endomysial antibody determination in screening anemic patients for CD using human umbilical cord as substrate. We studied 130 patients with CD (58 males, 72 females; median age 18 months) diagnosed at a department of Pediatric Gastroenterology, and 85 patients with iron deficiency anemia (38 males, 47 females; median age 48 years) observed at a hematology outpatient clinic. From the 85 adult patients with iron deficiency anemia, we selected a subgroup of 25 subjects with no improvement in Hb after two months of iron therapy (80 mg/day orally). Routine hematochemical tests were performed in all 215 patients. All pediatric and adult subjects underwent immunological screening for celiac disease (AGA and EmA assay); intestinal biopsy was also performed on patients testing positive. In the adult anemic patients a serum sample was stored at -20 degrees C on first observation, and after 6-18 months EmA on human umbilical cord were assayed. In the pediatric patients with CD, anemia was observed in 91/130 patients (70% of cases, the most frequent symptom after poor growth); however, this was the only presenting symptom of CD in 2/130 patients (1.5% of cases). Anemia was sideropenic in 41/91 patients (iron <45 microg/dl, ferritin <15 microg/liter). In the adult patients with iron deficiency anemia, immunological screening (AGA and EmA) showed suspected CD in 5/85 cases (5.8%), with diagnosis confirmed on intestinal biopsy. These five patients were in the subgroup of iron supplementation therapy nonresponders. CD prevalence in the refractory anemia subgroup was, therefore, 5/25 (20%). On diagnosis the hematological indices of the anemia + CD patients were not different than those of the refractory anemia patients without CD. The median age of the CD + anemia patients was significantly lower than that of the whole group of anemic subjects, and there was also a prevalence of females (4/5 cases). The results of the EmA determination on human umbilical cord in the adult anemic patients showed a perfect concordance with those using a traditional kit that uses monkey esophagus as substrate. In the pediatric age group many cases of CD with anemia as the only sign of the disease are probably not diagnosed. In our adult patients with sideropenic anemia, CD prevalence was 5-6%; however, the observation of anemic patients not responding to oral iron therapy makes a diagnosis of CD much more probable. EmA determination on human umbilical cord is the most logical approach to screen anemic patients for suspected CD.

Anemia, Iron-Deficiency↗

Persistent cow's milk protein intolerance in infants: the changing faces of the same disease.

BACKGROUND: Recent research has shown that cow's milk protein intolerance (CMPI) often persists beyond 4 years of age. AIMS: To evaluate the clinical and immunological characteristics of a group of infants with persistent CMPI. PATIENTS AND METHODS: Twelve infants (6 m, 6f) with persistent CMPI were followed up from birth until a median age of 5 years. The patients underwent CMP challenge each year to evaluate CMP-tolerance. As controls we followed 26 infants (12 m, 14 f) with CMPI that resolved within 1-2 years. RESULTS: A family history of atopic disease was found in 10/12 patients with persistent CMPI and in 10/26 controls (P<0.01). Clinical presentation changed over time: at onset symptoms were prevalently gastrointestinal, while at the end of the study there was an increased frequency of wheezing and constipation and a higher frequency of delayed reactions to CMP-challenge than at study commencement (9/12 vs 2/12; P<0.007). 11/12 infants with persistent CMPI and 3/26 controls (P<0.0001) presented multiple food intolerance. During the observation period 9/12 infants with persistent CMPI and 2/26 controls showed atopic disease: asthma, rhinitis, eczema (P < 0.0001). CONCLUSIONS: Persistent CMPI forms are characterized by: (a) considerable importance of familial atopic disease; (b) change in CMPI manifestations over time and more prolonged delay between CMP consumption and manifestation of symptoms; (c) very high frequency of multiple food intolerance and allergic diseases.

Animals↗

Lipoprotein profile and high-density lipoproteins: subfractions distribution in centenarians.

In order to assess the role of HDL on longevity, we studied HDL subfraction distribution in centenarian women compared with a group of weight- and gender-matched healthy normolipidemic controls. We did not find any significant difference in the mean plasma lipid, apolipoprotein, and Lp(a) levels. On the contrary, in spite of similar HDL-cholesterol concentrations (1.32 +/- 0.41 mmol/l in centenarians vs. 1.32 +/- 0.25 mmol/l in controls, p = not significant), HDL2b and HDL3a levels were, respectively, significantly increased and significantly reduced in centenarians in comparison with controls (HDL2b 32.4 +/- 9.2% in centenarians vs. 23.4 +/- 7.7% in controls, p < 0.002, and HDL3a 26.3 +/- 9.8% in centenarians vs. 34.1 +/- 7.3% in controls, p < 0.01). Moreover, HDL2b levels were significantly raised and HDL3a levels were significantly reduced in centenarians in comparison with both 'middle-aged' and 'elderly' subjects, whereas no difference for any HDL subfraction was found between the two groups of controls of different ages. Age was significantly correlated with HDL2b and HDL3a (respectively, +0.452, p < 0.001, and -0.370, p < 0.01) in all subjects, but not with all the other lipid, lipoprotein and apolipoprotein parameters, but we observed a large overlapping of individual values of HDL2b between centenarians and controls. Since HDL2b levels were found to be inversely correlated with coronary heart disease risk, we could speculate that, in some cases, this may probably favor a healthy ageing, but long-term longitudinal studies are necessary to define the relative importance of HDL subfractions distribution as a marker of longevity. Probably other factors or clinical characteristics play a major role in the ageing process.

Aged↗

[Evidence-based medicine. How to use biomedical literature to solve clinical problems. Italian Group on Evidence-Based Medicine-GIMBE].

Clinical practice is constantly changing, the rate of changing is accelerating and consequently it may even take years before the results of clinical research will be incorporated in day-to-day practice. So, there is a large gap between what the biomedical literature contains and the care that most of patients receive. The gap is widened by the extensive processing that results of clinical research require before they can be used. Evidence-based medicine is a new approach to health care promoting the collection, interpretation, and integration of valid, important and applicable research-derived evidence. The best available evidence, moderated by patient circumstances and preferences, is applied to improve the quality of clinical judgements. There are many information tools that facilitate the practice of evidence-based medicine. These include users' guides to the medical literature, strategies to improve the yield of MEDLINE searches, standardized formats for abstracts of journal articles and guidelines, new journals, systematic reviews and meta-analyses, resources on-line and software tools bringing high quality information to the point of clinical decision making. However, these tools are poorly spread and physicians lack of necessary skills for their effective utilization. In this article the authors describe guidelines for efficient and effective utilization of biomedical information tools to solve clinical problems and improve the quality and the cost/effectiveness of health care.

Evidence-Based Medicine↗

Usefulness of faecal elastase-1 assay in monitoring pancreatic function in childhood coeliac disease.

BACKGROUND: In coeliac disease it has been demonstrated that the indirect pancreatic function tests detect a greater percentage of subjects with exocrine pancreatic insufficiency than the secretin-caerulein test. AIMS: To evaluate faecal pancreatic elastase-1 assay in monitoring patients with coeliac disease. PATIENTS: Thirty patients with coeliac disease (11 m; age range 1-7 years) completed a 2-month follow-up. As controls, we studied two sex-, age-matched patient groups: a) 15 patients with cystic fibrosis, b) 40 surgical patients without gastroenterological disease. METHODS: In all coeliac subjects, stools were collected over 24 hours at diagnosis and then 30 and 60 days after commencement of the gluten-free diet; on a sample of the faeces we assayed elastase-1 activity. In the control patients, faeces were collected over 24 hours for elastase-1 assay only once. The coeliac patients only underwent the secretin-caerulein test, at diagnosis. RESULTS: Ten out of 30 coeliac patients (33%) had subnormal faecal elastase-1 values at diagnosis, while all the surgical controls had values within the normal range; median values in coeliac patients were significantly lower than those of the surgical controls (median 287 mcg/g, 95% CI 271-430, versus 487 mcg/g, 95% CI 426-538, p < 0.007). Cystic fibrosis patient values (median 10 mcg/g, 95% CI 7-155) were significantly lower than both those of coeliac patients and those of the surgical controls (p < 0.0001). The secretin-caerulein test showed that 7/30 coeliac patients (23%) had a deficiency in one or more pancreatic enzymes; all these subjects had below normal faecal elastase-1 values. During the follow-up, we observed a progressive reduction in the number of coeliacs with pancreatic impairment; however, after 2 months of gluten-free diet, faecal elastase-1 deficiency persisted in 2/30 coeliacs. CONCLUSIONS: Faecal elastase-1 determination in coeliac patients reveals a similar frequency and duration of pancreatic impairment to those observed in studies performed using the faecal chymotrypsin assay; a reduction in faecal elastase-1 values can be linked to "non-typical pancreatic diseases".

Celiac Disease↗

Liver is not the unique site of synthesis of beta 2-glycoprotein I (apolipoprotein H): evidence for an intestinal localization.

Apolipoprotein H is a protein of about 50 kilodaltons, structurally related to the regulators of the complement activation family. Its physiological function is poorly understood but it has been implicated in lipid metabolism and coagulative pathways. The major site of synthesis is thought to be the liver. Several reports indicate that apolipoprotein H is the antigen of the antiphospholipid antibodies and also behaves as an acute-phase reactant. Moreover, 40% of plasma apolipoprotein H is associated with very low-density lipoprotein, high-density lipoprotein, and postprandial chylomicrons. In this study we investigated other sites of synthesis by reverse transcription/polymerase chain reaction and we found apolipoprotein H mRNA expression in intestinal cell lines and tissues. Immunohistochemistry was performed on various fresh and paraffin-embedded tissues and apolipoprotein H was immunolocalized in the cytoplasm of hepatocytes and epithelial cells from colon and jejunum. This study indicates that apolipoprotein H is expressed at both mRNA and protein levels in enterocytes.

Apolipoproteins↗

Role of pancreatic impairment in growth recovery during gluten-free diet in childhood celiac disease.

BACKGROUND & AIMS: Clinical significance and duration of insufficient release of pancreatic enzymes in childhood celiac disease have not been clarified. The aim of this study was to evaluate the role that pancreatic impairment plays in growth recovery and the duration of this impairment. METHODS: Forty-six patients with celiac disease who had a median age of 2.5 years were enrolled. Fecal chymotrypsin level was determined at diagnosis and then every 15 days after the beginning of a gluten-free diet in all patients. RESULTS: At diagnosis, 17 of 46 patients with celiac disease had subnormal fecal chymotrypsin values. During the gluten-free diet, a progressive reduction in the percentage of patients with subnormal fecal chymotrypsin values was observed: 12 of 46 patients after 30 days and 2 of 46 patients after 60 days. Weight increase after 2 months of gluten-free diet was significantly greater in patients with normal fecal chymotrypsin values at diagnosis than in patients with subnormal values, and a positive correlation was found between fecal chymotrypsin at diagnosis and weight increase (r = 0.56). CONCLUSIONS: A small percentage of patients with celiac disease still had subnormal chymotrypsin concentrations after 60 days of gluten-free diet. Fecal chymotrypsin is a predictive index of weight recovery in the first months after diagnosis of celiac disease; it could be used to select patients for enzyme supplementation therapy.

Body Weight↗

LDL apheresis in a homozygous familial hypercholesterolemic child aged 4.5.

Preliminary experience with the efficacy and safety of dextran sulfate cellulose low-density lipoprotein (LDL) apheresis for the treatment of a 4.5-year-old girl with homozygous familial hypercholesterolemia and coronary artery disease is reported. The decrease of the most atherogenic apolipoprotein B-containing lipoproteins, low-density lipoprotein (LDL) and lipoprotein(a) (Lp [a]), were in the ranges of 63.1-68.7%, and 52.5-58.6%, respectively. The child tolerated LDL apheresis without any clinically significant complications. Therefore, she was submitted to a long-term program of treatment at intervals of 15 days. The experience suggests the possibility of an early beginning of extracorporeal treatment with LDL apheresis in children severely affected by homozygous or double heterozygous familial hypercholesterolemia.

Blood Component Removal↗