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Italian Journal of Pediatrics Jan 2023Nutrition practices for preterm infants include phases of parenteral nutrition, gradually interrupted parenteral nutrition (transition phase), and full enteral... (Review)
Review
Nutrition practices for preterm infants include phases of parenteral nutrition, gradually interrupted parenteral nutrition (transition phase), and full enteral nutrition. However, nutrition management during the transition phase is frequently overlooked. This review examined the relationship between nutrient intake during the transition phase and preterm infant growth. PubMed, Embase, Web of Science, Cochrane, Chinese National Knowledge Infrastructure Database, Wanfang Database, and Chinese Science and Technique Journals Database were searched for studies examining the relationship between nutrient intake during the transition phase and postnatal growth of preterm infants from each database's earliest inception through February 28, 2022. The quality of the studies was assessed using the Newcastle-Ottawa scale. A total of three studies conducted in the USA, Italy and China met the inclusion criteria. The growth indicators were extrauterine growth restriction (weight < 10th percentile for post-menstrual age) or inadequate weight growth velocity (growth velocity < 15 g/kg/d) at discharge or the end of the transition phase. The transition phase was divided into two periods in two studies: the early period (parenteral energy intake > 50% of total energy intake) and the late period (enteral energy intake > 50% of the total energy intake). The cumulative protein intake in the transition phase was generally lower in preterm infants with extrauterine growth restriction or inadequate weight growth velocity, especially in the early transition phase. The deficiency of energy and protein intake during the transition phase cannot be explicitly determined due to differences in growth indicators and definitions of the transition phase. However, enteral protein intake should be closely monitored in the early transition phase to ensure a better growth rate for preterm infants. To elucidate potential associations, further well-designed research will be required.
Topics: Infant; Infant, Newborn; Humans; Infant, Premature; Nutritional Status; Enteral Nutrition; Energy Intake; Eating; Infant, Very Low Birth Weight
PubMed: 36670430
DOI: 10.1186/s13052-022-01406-3 -
Andrology Sep 2023Although selective estrogen receptor modulators have been proposed as a treatment for men with central functional hypogonadism, only a few data have been produced in men... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Although selective estrogen receptor modulators have been proposed as a treatment for men with central functional hypogonadism, only a few data have been produced in men with obesity-related functional androgen deficiency.
OBJECTIVE
To determine whether and to what extent selective estrogen receptor modulators are an effective and safe therapy in men with obesity-related functional androgen deficiency.
MATERIALS AND METHODS
A thorough search of PubMed, Web of Science, Scopus, and Cochrane Library databases was performed to identify studies comparing testosterone levels before and after treatment. Mean differences with 95% coefficient intervals were combined using random effects models. Funnel plot, Egger's test, and trim-and-fill analysis were used to assess publication bias.
RESULTS
Seven studies met the inclusion criteria providing information on 292 men with obesity-related functional androgen deficiency treated with clomiphene citrate (12.5-50 mg daily) or enclomiphene citrate (12.5-25 mg daily) for 1.5-4 months. The pooled estimates indicated a significant increase in testosterone levels both with clomiphene (mean difference: 11.56 nmol/L; 95% coefficient interval: 9.68, 13.43; I = 69%, p = 0.01) and enclomiphene citrate (mean difference: 7.50 nmol/L; 95% coefficient interval: 6.52, 8.48; I = 4%, p = 0.37). After the exclusion of one study on severely obese men, who exhibited the highest response rate to clomiphene citrate, the heterogeneity disappeared (mean difference: 10.27 nmol/L; 95% coefficient interval: 9.39, 11.16; I = 0%, p = 0.66). No publication bias was revealed by Egger's test and trim-and-fill analysis. No treatment-related unexpected findings regarding safety profile were registered.
DISCUSSION AND CONCLUSION
Treatment with clomiphene citrate and enclomiphene citrate may be an effective and safe alternative to testosterone replacement therapy in men with obesity-related functional androgen deficiency. Further long-term studies are warranted to define clinical reflections of the selective estrogen receptor modulators-induced increase in testosterone levels and to better clarify the safety profile.
Topics: Humans; Male; Androgens; Clomiphene; Enclomiphene; Eunuchism; Hypogonadism; Obesity; Receptors, Estrogen; Selective Estrogen Receptor Modulators; Testosterone
PubMed: 36604313
DOI: 10.1111/andr.13373 -
International Journal of Health Policy... 2023This review was conducted to identify the impact of economic sanctions on household food and nutrition security and policies to cope with them in countries exposed to...
BACKGROUND
This review was conducted to identify the impact of economic sanctions on household food and nutrition security and policies to cope with them in countries exposed to sanctions.
METHODS
The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines 2020 were used to identify, select, appraise, and synthesize studies. Electronic databases in addition to Persian ones have been systematically searched for all related documents published until March 2022. Exclusion criteria were: lack of data related to food insecurity in countries subject to sanction and very low quality of the article. The quality of included studies was assessed using the Joanna Briggs Institute (JBI) Critical Appraisal checklists. The results were presented as qualitative and quantitative syntheses.
RESULTS
Of 1428 identified studies, 36 publications remained in the review, which belong to Iran (n=8), Cuba (n=8), Russia (n=7), Iraq (n=7), and Haiti (n=6), respectively. Declining gross domestic product (GDP), devaluation of the national currency, and the quality of food, increase in inflation, unemployment, and consumer prices, infant and under 5 years mortality, energy, and protein deficiency, and the poverty rate were reported as sanction consequences. The most important strategies to improve food security were the humanitarian assistance provided by the international community (Haiti), equity and priority for vulnerable groups mainly by expanding the healthcare system (Cuba), adopting a food ration system in the oil-for-food program, and fixing the price of food baskets (Iraq), import substitution and self-sufficiency (Russia), support domestic production, direct and indirect support and compensation packages for vulnerable households (the approach of resistance economy in Iran).
CONCLUSION
Due to the heterogeneity of studies, meta-analysis was not possible. Since inadequate physical and economic food access caused by sanctions affects especially disadvantaged and vulnerable groups, planning to improve food security and providing support packages for these groups seems necessary.
Topics: Infant; Humans; Gross Domestic Product; Policy; Academies and Institutes; Checklist; Cuba
PubMed: 38618825
DOI: 10.34172/ijhpm.2023.7362 -
The Cochrane Database of Systematic... Nov 2022Chronic arthropathy is a potentially debilitating complication for people with haemophilia - a genetic, X-linked, recessive bleeding disorder, characterised by the... (Review)
Review
BACKGROUND
Chronic arthropathy is a potentially debilitating complication for people with haemophilia - a genetic, X-linked, recessive bleeding disorder, characterised by the absence or deficiency of a clotting factor protein. Staging classifications, such as the Arnold-Hilgartner classification for haemophilic arthropathy of the knee, radiologically reflect the extent of knee joint destruction with underlying chronic synovitis. Management of this highly morbid disease process involves intensive prophylactic measures, and chemical or radioisotope synovectomy in its early stages. However, failure of non-surgical therapy in people with progression of chronic arthropathy often prompts surgical management, including synovectomy, joint debridement, arthrodesis, and arthroplasty, depending on the type of joint and extent of the damage. To date, management of people with mild to moderate chronic arthropathy from haemophilia remains controversial; there is no agreed standard treatment. Thus, the benefits and disadvantages of non-surgical and surgical management of mild to moderate chronic arthropathy in people with haemophilia needs to be systematically reviewed. OBJECTIVES: To assess the efficacy and safety of surgery for mild to moderate chronic arthropathy in people with haemophilia A or B.
SEARCH METHODS
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Coagulopathies Trials Register, CENTRAL, MEDLINE, Embase, CINAHL, and two trial registers to August 2022. We also handsearched relevant journals and conference abstract books.
SELECTION CRITERIA
Randomized controlled trials (RCTs) and quasi-RCTs comparing surgery and non-surgical interventions, for any joint with chronic arthropathy, in people with haemophilia, who were at least 12 years old.
DATA COLLECTION AND ANALYSIS
The review authors did not identify any trials to include in this review.
MAIN RESULTS
The review authors did not identify any trials to include in this review.
AUTHORS' CONCLUSIONS
The review authors did not identify any trials to include in this review. Due to a lack of research in this particular area, we plan to update the literature search every two years, and will update review if any new evidence is reported. There is a need for a well-designed RCT that assesses the safety and efficacy of surgical versus non-surgical interventions for chronic arthropathy in people with haemophilia.
Topics: Child; Humans; Hemophilia A; Joint Diseases; Knee Joint; MEDLINE; Randomized Controlled Trials as Topic
PubMed: 36448638
DOI: 10.1002/14651858.CD013634.pub2 -
World Journal of Surgical Oncology Nov 2022Many studies have reported the relationship between prognosis and Slug protein expression in breast cancer patients, but the results are discrepant. Therefore, there is... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Many studies have reported the relationship between prognosis and Slug protein expression in breast cancer patients, but the results are discrepant. Therefore, there is a need for meta-analyses with high statistical power to investigate and further explore their relationship.
METHODS
We used PubMed, Embase, the Cochrane Library, Scopus, MEDLINE, and the Web of Science to find studies on breast cancer and Slug. Overall survival (OS) and disease-free survival (DFS) were the study's primary endpoints. We pooled hazard ratios (HRs) and odds ratios (ORs) to assess the association between Slug protein expression and prognostic and clinicopathological parameters. This study was performed using STATA version 14.0 for data analysis. (Stata Corporation, TX, USA).
RESULTS
We conducted a literature search by searching six online databases. Ultimately, we obtained eight studies including 1458 patients through strict exclusion criteria. The results showed that increased Slug protein expression resulted in poorer OS (HR = 2.21; 95% CI = 1.47-3.33; P < 0.001) and DFS (HR = 2.03; 95% CI = 1.26-3.28; P = 0.004) in breast cancer patients. In addition, the results suggested that breast cancer patients with increased Slug protein expression had a higher TNM stage (I-II vs III-IV; OR = 0.42; 95% CI = 0.25-0.70; P = 0.001), a greater tendency to have axillary lymph node metastases (N+ vs N0; OR = 2.16; 95% CI = 1.31-3.56; P = 0.003) and were more prone to estrogen receptor deficiency (positive vs negative; OR = 0.67; 95% CI = 0.45-0.99; P = 0.042). However, Slug protein expression was not associated with age, histological grade, tumor size, progesterone receptor status, or human epidermal growth factor receptor 2 status in breast cancer patients.
CONCLUSION
This meta-analysis showed that elevated Slug protein expression may be related to poor outcomes in patients with breast cancer. Therefore, Slug is not only an indicator of patient survival but may also become a new target for breast cancer therapy.
Topics: Humans; Female; Prognosis; Breast Neoplasms; Disease-Free Survival; Lymphatic Metastasis; Receptors, Estrogen
PubMed: 36372891
DOI: 10.1186/s12957-022-02825-6 -
Clinical Endocrinology Mar 2023P450 side-chain cleavage deficiency (SCCD) patients present with primary adrenal insufficiency (PAI) with or without undervirilized external genitalia. The distinction...
OBJECTIVE
P450 side-chain cleavage deficiency (SCCD) patients present with primary adrenal insufficiency (PAI) with or without undervirilized external genitalia. The distinction between classic and nonclassic steroidogenic acute regulatory protein deficiency has been described, whereas in SCCD is unclear. The data on gonadal function and its correlation with SCCD genotype has not been studied. We describe our experience and perform a systematic review of genetically proven SCCD patients to determine the distinct phenotypic and genotypic characteristics of 46,XY SCCD patients with typical male external genitalia (SCCD-TMG) and atypical (SCCD-AG) external genitalia.
DESIGN, PATIENTS AND MEASUREMENTS
Retrospective review of three genetically proven SCCD patients from our centre and per-patient data analysis from a systematic review of 52 probands was performed. SCCD-TMG (n = 19) was defined as external genitalia of Sinnecker score 1 with 46,XY karyotype; the rest (Sinnecker 2-5) were classified as SCCD-AG (n = 15).
RESULTS
We report two new Indian cases of SCCD with three novel likely pathogenic variants and pubertal follow-up of a previously reported patient. In systematic review, age at diagnosis of PAI and elevated renin were not different between 46,XY SCCD-TMG (n = 19) and SCCD-AG (n = 15), whereas spontaneous puberty (9/9 vs. 0/3, p = .0045), normal prepubertal (5/5 vs. 6/6, p = .002), pubertal gonadotropins (2/9 vs. 0/3, p = 1) and normal pubertal testosterone (9/11 vs. 0/3, p = .027) were more common in SCCD-TMG. Testicular adrenal rest tumours were exclusive to SCCD-TMG (n = 4). SCCD-TMG was associated with four particular genotypes [monoallelic p.Glu314Lys with another deleterious variant on the second allele (p.Glu314Lys/X-CHS: X-compound heterozygous state), biallelic p.Arg451Trp, p.Phe215Ser/p.Arg232Ter and monoallelic p.Val79Ile]. 46,XX SCCD patients with p.Glu314Lys/X-CHS also had normal gonadotropins with spontaneous puberty.
CONCLUSION
SCCD-TMG is associated with four specific genotypes and distinct gonadal characteristics from SCCD-AG with overlapping features of PAI.
Topics: Humans; Male; Testosterone; Puberty; Testicular Neoplasms; Mutation
PubMed: 36357326
DOI: 10.1111/cen.14848 -
BMC Pregnancy and Childbirth Nov 2022Vitamin D deficiency, a common problem among pregnant women, is linked with maternal inflammation, oxidative stress and consequent adverse pregnancy outcomes. The aim of... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Vitamin D deficiency, a common problem among pregnant women, is linked with maternal inflammation, oxidative stress and consequent adverse pregnancy outcomes. The aim of this systematic review was to evaluate the effect of vitamin D supplementation on oxidative stress and inflammatory biomarkers in pregnant women according to the PRISMA guidance.
METHODS
Four databases including PubMed/MEDLINE, Scopus, Web of Science and Cochrane were used for searching papers published until 25 July 2022. Clinical trials that assessed 25-Hydroxyvitamin D (25(OH)D), inflammatory markers (including high sensitivity C-reactive protein (hs-CRP) and certain cytokines) and oxidative stress markers (including malondialdehyde (MDA), total antioxidant capacity (TAC) and glutathione (GSH)) in pregnant women, were included in this review. The primary search of three databases displayed 21571 records. After removing duplicates and irrelevant articles, 17 eligible RCTs included for more evaluation. Random effect model and Der Simonian-Laird method were used to pool the data of studies. Risk of bias assessed according to version 2 of the Cochrane risk-of-bias tool for randomized trials.
RESULTS
According to the meta-analysis result, vitamin D supplementation caused a significant increase in the maternal circulating concentrations of 25(OH)D (SMD 2.07; 95%, CI 1.51, 2.63; p < 0.001), TAC (SMD 2.13, 95% CI 1.04 to 3.23, p < 0.001) and GSH (SMD 4.37, 95% CI 2.9 to 5.74, p < 0.001) as well as a significant decrease in the levels of MDA (SMD -0.46, 95% CI -0.87 to -0.05, p = 0.02). However, it had no significant impact on hs-CRP concentrations (SMD 0.24; 95% CI, -0.55, 1.03; p = 0.50).
CONCLUSION
In the present study, vitamin D supplementation led to increased levels of 25(OH)D, TAC and GSH and also decreased concentration of MDA. Nevertheless, because of low certainty of evidence, these findings have to be declared conservatively.
TRIAL REGISTRATION
Registration code in PROSPERO website: CRD42020202600.
Topics: Female; Pregnancy; Humans; C-Reactive Protein; Dietary Supplements; Pregnant Women; Oxidative Stress; Biomarkers; Vitamin D; Antioxidants
PubMed: 36335311
DOI: 10.1186/s12884-022-05132-w -
Nutricion Hospitalaria Dec 2022Previous studies have pointed to a possible relationship between vitamin D deficiency and the severity of the disease promoted by SARS-CoV-2, reducing respiratory and...
Previous studies have pointed to a possible relationship between vitamin D deficiency and the severity of the disease promoted by SARS-CoV-2, reducing respiratory and cardiovascular complications caused by a hyperreaction of the immune system known as "cytokine storm". This vitamin exerts multiple functions that depend on the presence and levels of different proteins, such as the vitamin D receptor (VDR) and the vitamin D binding protein (DBP), and the existence of single nucleotide polymorphisms (SNPs) of the genes that encode these proteins. The objective of this review is to assess whether some VDR and GC SNPs are risk factors for the most severe forms of COVID-19 disease and whether they condition the response to vitamin D supplementation. A search was performed in PubMed, Google Scholar and Scielo, finding that genotypes in patients affected by COVID-19, were rarely performed, although some studies find a relationship between different alleles and the severity of the disease. The ApaI polymorphism of the VDR gene stands out, as the minor allele "a" increases the risk of mortality from COVID-19 (OR = 11.828, CI: 2,493-56,104, p = 0.002). Results divergency in the efficacy of vitamin D supplementation suggest the need for a larger number of studies. In conclusion, the study of VDR and GC polymorphisms seems essential to effectively treat vitamin D deficiency and particularly to protect against COVID-19. Well-designed studies are needed to elucidate whether plasma vitamin D levels play a role of casuality or causality.
Topics: Humans; COVID-19; Genotype; Polymorphism, Single Nucleotide; Receptors, Calcitriol; SARS-CoV-2; Vitamin D; Vitamin D Deficiency; Vitamin D-Binding Protein
PubMed: 36327123
DOI: 10.20960/nh.04299 -
Pediatric Pulmonology Feb 2023The lung is one of the target organs in the systemic involvement of autoinflammatory disease (AID), and interstitial lung disease (ILD) is the primary phenotype of lung...
BACKGROUND/OBJECTIVES
The lung is one of the target organs in the systemic involvement of autoinflammatory disease (AID), and interstitial lung disease (ILD) is the primary phenotype of lung involvement in AID. In this review, we aimed to conduct a systematic review of the available literature to highlight ILD in AID.
METHODS
We conducted a systematic literature search in PubMed/MEDLINE and Scopus from the inception of the databases to January 2022. References were first screened by title and then by abstract by two authors. Eighteen original papers were selected for full-text review.
RESULTS
During the literature search, we identified 18 relevant articles describing 52 cases of AID and ILD. Of those, 44 patients had stimulator of interferon genes-associated vasculopathy with onset in infancy (SAVI), six had coatomer protein complex (COPA) syndrome, one had haploinsufficiency of A20, and one had mevalonate kinase deficiency. Pulmonary fibrosis, cyst formation, and ground glass areas were the most common findings in chest tomography of patients with COPA syndrome and SAVI. Janus kinase inhibitors were used to treat most of the patients with SAVI, which stabilized ILD.
CONCLUSIONS
ILD should be considered carefully in children with AID, especially those with interferonopathy.
Topics: Humans; Hereditary Autoinflammatory Diseases; Lung; Lung Diseases, Interstitial; Syndrome; Vascular Diseases; Child
PubMed: 36314652
DOI: 10.1002/ppul.26220 -
Orphanet Journal of Rare Diseases Oct 2022Isolated sulfite oxidase deficiency (ISOD) caused by sulfite oxidase gene (SUOX) mutations is a rare neurometabolic disease associated with ectopia lentis (EL). However,...
BACKGROUND
Isolated sulfite oxidase deficiency (ISOD) caused by sulfite oxidase gene (SUOX) mutations is a rare neurometabolic disease associated with ectopia lentis (EL). However, few genotype-phenotype correlations have been established yet.
METHODS
Potentially pathogenic SUOX mutations were screened from a Chinese cohort of congenital EL using panel-based next-generation sequencing and analyzed with multiple bioinformatics tools. The genotype-phenotype correlations were evaluated via a systematic review of SUOX mutations within our data and from the literature.
RESULTS
A novel paternal missense mutation, c.205G > C (p.A69P), and a recurrent maternal nonsense mutation, c.1200 C > G (p.Y400*), of SUOX were identified in a 4-year-old boy from 312 probands. The biochemical assays manifested elevated urine sulfite and S-sulfocysteine accompanied by decreased homocysteine in the blood. The patient had bilateral EL and normal fundus, yet minimal neurological involvement and normal brain structure. Molecular modeling simulation revealed the p.A69P mutant had an unstable structure but an unchanged affinity for sulfite, while the truncated p.Y400* mutant showed decreased binding capacity. Genotype-phenotype analysis demonstrated patients with biallelic missense mutations had milder symptoms (P = 0.023), later age of onset (P < 0.001), and a higher incidence of regression (P = 0.017) than other genotypes. No correlations were found regarding EL and other neurological symptoms.
CONCLUSION
The data from this study not only enrich the known mutation spectrum of SUOX but also suggest that missense mutations are associated with mild and atypical symptoms.
Topics: Humans; Ectopia Lentis; Mutation; Genetic Association Studies; Sulfites; Oxidoreductases Acting on Sulfur Group Donors
PubMed: 36303223
DOI: 10.1186/s13023-022-02544-x