Dataset Information

Growth Patterns in the Irish Pyridoxine Nonresponsive Homocystinuria Population and the Influence of Metabolic Control and Protein Intake.

ABSTRACT: A low methionine diet is the mainstay of treatment for pyridoxine nonresponsive homocystinuria (HCU). There are various guidelines for recommended protein intakes for HCU and clinical practice varies. Poor growth has been associated with low cystine levels. This retrospective review of 48 Irish pyridoxine nonresponsive HCU patients assessed weight, height, body mass index (BMI), protein intake, and metabolic control up to 18 years at nine set time points. Patients diagnosed through newborn screening (NBS) were compared to late diagnosed (LD) patients. At 18 years the LD group (n = 12, mean age at diagnosis 5.09 years) were heavier (estimated effect +4.97?Kg, P = 0.0058) and taller (estimated effect +7.97?cm P = 0.0204) than the NBS group (n = 36). There was no difference in growth rate between the groups after 10 years of age. The HCU population were heavier and taller than the general population by one standard deviation with no difference in BMI. There was no association between intermittently low cystine levels and height. Three protein intake guidelines were compared; there was no difference in adult height between those who met the lowest of the guidelines (Genetic Metabolic Dietitians International) and those with a higher protein intake.

SUBMITTER: Purcell O

PROVIDER: S-EPMC5705888 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Growth Patterns in the Irish Pyridoxine Nonresponsive Homocystinuria Population and the Influence of Metabolic Control and Protein Intake.

Purcell Orla O Coughlan Aoife A Grant Tim T McNulty Jenny J Clark Anne A Deverell Deirdre D Mayne Philip P Hughes Joanne J Monavari Ahmad A Knerr Ina I Crushell Ellen E

Journal of nutrition and metabolism 20171115

A low methionine diet is the mainstay of treatment for pyridoxine nonresponsive homocystinuria (HCU). There are various guidelines for recommended protein intakes for HCU and clinical practice varies. Poor growth has been associated with low cystine levels. This retrospective review of 48 Irish pyridoxine nonresponsive HCU patients assessed weight, height, body mass index (BMI), protein intake, and metabolic control up to 18 years at nine set time points. Patients diagnosed through newborn scree ...[more]

PMID: 29270317

Similar Datasets

Project description:Background:Alcohol intake is a leading modifiable cause related to cancer-specific deaths. Various alcohol intake patterns have shown to impact cancer progression differently, however, many studies only evaluated simplified patterns (such as heavy vs. non-heavy drinking) of alcohol intake for cancer survivors. The objective of this study is to provide population-based prevalence of the complex alcohol drinking patterns for cancer survivors, and compare it with that of non-cancer individuals. Additionally, we evaluated the impact of cancer related factors (binary, alcohol-related cancer type, and length of cancer history) to the alcohol intake patterns adjusted for the selected factors. Methods:A total of 193,197 individuals, including 16,504 cancer survivors, with age ?18 years old in the 2012-2017 National Health Interview Survey (NHIS) were included in this study. The population-based prevalence of alcohol patterns was estimated. To evaluate cancer related factors associated with the alcohol intake patterns, we applied multinomial logistic models with the appropriate sampling weights and adjusted the selected demographic factors and smoking status. Results:There were 62.1% of cancer survivors and 66.0% of non-cancer individuals who were current alcohol drinkers in the past year. The prevalence of heavy drinking was identical for 5.2% of cancer and non-cancer individuals. For frequent binge drinking, cancer survivors tended to have less frequent binge than non-cancer individuals (2.8% vs. 4.9%). After adjusting for the selected demographic factors and smoking status, the cancer survivors were less likely to have the intermediate level of alcohol intake (light/moderate or occasional binge drinking) compared with non-cancer individuals, but no difference for the excessive alcohol intake (heavy or frequent binge drinking) was observed for those with and without cancer. As for cancer type, those with non-alcohol related cancer tended to be a current drinker compared with those with alcohol-related cancer. Compared with cancer survivors with a short cancer history (2-4 years), survivors with a cancer history of 5-9 years were more likely to be current drinkers after adjusting for the selected factors. Cancer status, alcohol-related cancer type and length of cancer history had no impact on excessive alcohol intake. Conclusions:In summary, cancer survivors have similar excessive alcohol drinking patterns but were less likely to have the intermediate level of alcohol intake compared to non-cancer individuals. Alcohol intake may enhance cancer progression, interfere with cancer treatments and increase cancer-related mortality. To improve cancer survivors' health, custom alcohol interventions and cessation programs should be conducted to minimize alcohol intake for cancer survivors, especially for excessive alcohol drinkers.

Project description:BACKGROUND:The association between milk and dairy intake and the incidence of cardiometabolic diseases, cancer and mortality has been evaluated in many studies, but these studies have had conflicting results with no clear conclusion on causal or confounding associations. The present study aims to further address this association by cross-sectional and longitudinal evaluation of the associations between exposure to various types of dairy products and metabolic risk markers among inhabitants in northern Sweden while taking other lifestyle factors into account. METHODS:Respondents in the Västerbotten Intervention Programme with complete and plausible diet data between 1991 and 2016 were included, yielding 124,934 observations from 90,512 unique subjects. For longitudinal analysis, 27,682 participants with a visit 8-12?years after the first visit were identified. All participants completed a validated Food Frequency Questionnaire. Metabolic risk markers, including body mass index (BMI), blood pressure, serum (S) cholesterol and triglycerides, and blood glucose, were measured. Participants were categorized into quintiles by intake of dairy products, and risk (odds ratios, OR) of undesirable levels of metabolic risk markers was assessed in multivariable logistic regression analyses. In longitudinal analyses, intake quintiles were related to desirable levels of metabolic risk markers at both visits or deterioration at follow-up using Cox regression analyses. RESULTS:The OR of being classified with an undesirable BMI decreased with increasing quintiles of total dairy, cheese and butter intake but increased with increasing non-fermented milk intake. The OR of being classified with an undesirable S-cholesterol level increased with increasing intake of total dairy, butter and high fat (3%) non-fermented milk, whereas an undesirable S-triglyceride level was inversely associated with cheese and butter intake in women. In longitudinal analyses, increasing butter intake was associated with deterioration of S-cholesterol and blood glucose levels, whereas increasing cheese intake was associated with a lower risk of deterioration of S-triglycerides. CONCLUSIONS:Confounding factors likely contribute to the demonstrated association between dairy intake and mortality, and other medical conditions and analyses should be stratified by dairy type.

Dataset Information

Growth Patterns in the Irish Pyridoxine Nonresponsive Homocystinuria Population and the Influence of Metabolic Control and Protein Intake.

Publications

Growth Patterns in the Irish Pyridoxine Nonresponsive Homocystinuria Population and the Influence of Metabolic Control and Protein Intake.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure