Infant consumption of 100% lactose-based and reduced lactose infant formula in the United States: Review of NHANES data from 1999 to 2020.

OBJECTIVES: An acceptable alternative to human milk is US Food and Drug Administration (US FDA)-registered infant formula, which must meet the requirements of the US FDA Infant Formula Act. Human milk contains lactose, but some infant formulas may contain alternative carbohydrate sources such as corn syrup solids, maltodextrin, and sucrose. Recent research shows that infant-formula made with corn syrup solids may be associated with increased obesity risk in the first 5 years of life. A previous study found that of all formulas purchased, 59.0% were lactose-reduced. More US infants consume infant formula with nonlactose carbohydrates more frequently than is medically necessary. The purpose of this study is to use National Health and Nutrition Examination Survey (NHANES) data to describe the type and prevalence of nonlactose carbohydrates consumed in infant formula. METHODS: NHANES data from 1999 to 2020 was used to perform cross-sectional analyses and analyses of comparison of prevalence over time on consumption of nonlactose carbohydrate sources in infant formulas. RESULTS: We identified 3709 unique infant IDs associated with 36,084 feeding sessions. More than half of the feeding sessions involved a formula with at least one nonlactose carbohydrate. Feeding sessions involving a formula with at least one nonlactose carbohydrate increased by 163% from 1999-2004 to 2017-2020; formulas containing single or multiple nonlactose carbohydrate types account for the increase in prevalence. CONCLUSIONS: This study highlights an increase in the consumption of infant formula containing a nonlactose carbohydrate. More studies are needed to understand the short- and long-term effects of early exposure to these carbohydrates.

Cross-Sectional Analysis of Infant Diet, Outcomes, Consumer Behavior and Parental Perspectives to Optimize Infant Feeding in Response to the 2022 U.S. Infant Formula Shortage.

In May of 2022, millions of U.S. parents encountered uncertainty in safely feeding their infants due to the infant formula shortage. METHODS: An anonymous, electronic, cross-sectional, retrospective survey was used. RESULTS: U.S. parents (n = 178) whose infants were ~10 weeks old during the shortage completed the survey. Of parents, 81% switched formulas during the shortage, 87% switched because they could not find the formula they typically used, 34% switched 3-5 times, 29% of parents visited ≥4 stores/24 h and 26% of parents traveled >20 miles/24 h to purchase formula. Use of infant formula increased (p < 0.01); in infants requiring specialty formula, use of intact cow's milk formula increased (p < 0.05) and use of premature infant formulas decreased (p < 0.05). Infants relying on specialty formulas experienced at least one undesirable outcome compared with non-specialty users. Parents used social media, relatives/friends and healthcare providers for support during the shortage, but their helpfulness scores were suboptimal. Parents reported the need for greater infant formula availability, free prenatal lactation education and postpartum lactation support. CONCLUSIONS: Government, regulatory and healthcare policy oversight are needed to protect the infant feeding system, including more commercially available products, access to banked donor milk and lactation support.

Nutrition Content of Young Child Formulas.

OBJECTIVE: European Society for Pediatric Gastroenterology, Hepatology and Nutrition and the American Academy of Pediatrics state that young child formula (YCF) is not considered necessary for healthy children. Despite these recommendations, YCF accounts for approximately 13% of overall formula sales. Five percent of infants less than 1 year of age in the United States are being fed YCF. The purpose of this study is to identify the most Internet recommended and encountered YCF in the United States and determine if they meet nutritional recommendations for use in children 0-3 years. STUDY DESIGN: We used the search terms "toddler formula," "toddler milk," "follow-up formula," or "young child formula" in Google and DuckDuckGo to identify the most like encountered or recommended YCF on the Internet. We compared their labeled nutrients to Food and Drug Administration (FDA) Infant Formula Act (IFA) and international nutrient recommendations, given the absence of US nutrient requirements for YCF recommendations, for children 12-36 months. RESULTS: Twenty-nine YCF were reviewed. On average, YCF did not meet nutrient recommendations for infants and toddlers with 2.17 and 4.6 ingredients not meeting formula recommendations for younger and older infants, respectively, and between 3 and 4 ingredients for 12-36 months. CONCLUSIONS: Nutrition content of YCF are variable and do not meet FDA IFA requirements or YCF international recommendations. Increased US regulation is needed for YCF. It is important for health care providers to ask patients what they are feeding their infants and toddlers so they can educate parents on potential nutritional safety concerns.

Nutritional and Safety Concerns of Infant Feeding Trends.

OBJECTIVE: The American Academy of Pediatrics (AAP) and North American Society for Pediatric Gastroenterology, Hepatology and Nutrition recommend either exclusively breastfeeding for at least 6 months or an u.S. Food and Drug Administration-reviewed infant formula or donor breast milk from an established milk bank as alternatives. The purpose of this study was to establish the prevalence of contemporary infant feeding practices such as informal human milk sharing, imported European infant formula, toddler formula and homemade formula and gain insight into the parental reasoning for their choices. STUDY DESIGN: An anonymous, cross-sectional, voluntary electronic survey was sent to active prescribers to a Yumi (a baby food subscription company) list server in April and May 2021. Basic demographic, utilization of infant feeding practices and general feeding practices were collected. RESULTS: Of 2315 respondents, at least 18% of the families were following at least one contemporary feeding practice. Thirty six percent of parents using donor breast milk obtained it from unregulated sources, 14% of the respondents were using European infant formula, 5% were using toddler formula for their infants, and 2% were making homemade infant formula. CONCLUSION: The AAP has clear guidelines on infant nutrition and breastfeeding and when not possible, FDA reviewed infant formula or donor breast milk from an established milk bank as alternatives. Yet, our study found that at least 18% of the families across the united States were following at least one contemporary feeding practice with possible nutritional and safety concerns. it is important for pediatric gastroenterologists and dieticians to ask their patients how they are feeding their infants and be aware of these feeding practices that may pose significant health risks.

Disparities in Telehealth Utilization Within Pediatric Gastroenterology: One Academic Center's Experience.

OBJECTIVES: While the use of telemedicine has accelerated significantly with the recent pandemic, it has also magnified disparities in access to telemedicine. This study aims to look at telemedicine utilization patterns within a large pediatric gastroenterology practice. METHODS: A retrospective study of ambulatory care visits within Yale-New Haven Hospital's pediatric gastroenterology practice during the peak expansion of the telemedicine program was conducted. Zip code-level socioeconomic data were obtained using the Distressed Communities Index. A multivariate logistic regression to evaluate disparities between the use of video versus telephone visits was computed, and unadjusted and adjusted odds ratios with 95% confidence intervals (CIs) were obtained. RESULTS: A total of 1273 clinic visits were included in analysis. The majority of the patients listed English as their preferred language, had private insurance, and identified as non-Hispanic White. When adjusting for co-variates, having public insurance/Medicaid was associated with decreased odds of having video over telephone visits (adjusted odds ratio [aOR] 0.60; 95% CI 0.44-0.80). Those whose primary language was not English continued to have a statistically significant decreased odds of using video visits (Spanish aOR 0.24; 95% CI 0.13-0.44; other aOR 0.29; 95% CI 0.12-0.72). Within the adjusted multivariate logistic regression, race/ethnicity and SES were, however, no longer found to have a statistically significant decreased odds of video visits. CONCLUSIONS: The accelerated implementation of telemedicine within pediatric gastroenterology has given rise to disparities in its use. Further studies are needed to understand these disparities and develop interventions to lessen this gap in usage.

Non-FDA-Reviewed Imported European Formula Use Among Parents in Urban Pediatric Private Practice.

In 2018, the American Academy of Pediatrics warned consumers over the increasing use of imported infant formulas. The purpose of this study was to assess the usage of imported European infant formula among parents in a large urban private practice. An anonymous survey was distributed at well-child appointments to a convenience sample of parents at an urban private pediatric practice from November 2017 to March 2018. Of the 750 eligible respondents, 552 (74%) completed the survey. Of the parents using formula, 20% were using imported European infant formulas. The most commonly used formula was Holle (33%), and 72% were acquired from web-based third-party vendors. Parents chose to use these formulas because they believed that European formulas contained better ingredients. Only 8% of parents received information about European infant formula from their pediatricians. Pediatricians need to be aware of these formulas and their risks to educate families on the use and safety of these formulas.

IgA-deficient humans exhibit gut microbiota dysbiosis despite secretion of compensatory IgM.

Immunoglobulin A is the dominant antibody isotype found in mucosal secretions and enforces host-microbiota symbiosis in mice, yet selective IgA-deficiency (sIgAd) in humans is often described as asymptomatic. Here, we determined the effects of IgA deficiency on human gut microbiota composition and evaluated the possibility that mucosal secretion of IgM can compensate for a lack of secretory IgA. We used 16S rRNA gene sequencing and bacterial cell sorting to evaluate gut microbiota composition and taxa-specific antibody coating of the gut microbiota in 15 sIgAd subjects and matched controls. Despite the secretion of compensatory IgM into the gut lumen, sIgAd subjects displayed an altered gut microbiota composition as compared to healthy controls. These alterations were characterized by a trend towards decreased overall microbial diversity as well as significant shifts in the relative abundances of specific microbial taxa. While secretory IgA in healthy controls targeted a defined subset of the microbiota via high-level coating, compensatory IgM in sIgAd subjects showed less specificity than IgA and bound a broader subset of the microbiota. We conclude that IgA plays a critical and non-redundant role in controlling gut microbiota composition in humans and that secretory IgA has evolved to maintain a diverse and stable gut microbial community.

Comparison of Imported European and US Infant Formulas: Labeling, Nutrient and Safety Concerns.

OBJECTIVE: Infant formula in the United States is highly regulated. The American Academy of Pediatrics (AAP) has reported concerns over the use of non-Food and Drug Administration (FDA)-registered imported infant formulas. The purpose of this study is to identify Internet purchased and recommended imported European infant formulas and compare them with FDA labeling and nutrient requirements. STUDY DESIGN: We searched "European infant formulas" in Google and DuckDuckGo to identify vendors of European formulas and blogs discussing these formulas to determine the most frequently purchased and recommended brands. We then compared the identified European formula's label and listed nutrients to FDA labeling and nutrient requirements. RESULTS: Thirteen of 18 vendors responded to our inquiry of their top selling formula and 17 blogs were reviewed. Sixteen formulas were identified. None met all FDA label requirements. Listed nutrients fell within FDA requirements in 15 of 16 formulas. CONCLUSIONS: Non-FDA-registered imported European formulas do not meet all FDA-labeling requirements. Although linoleic acid, which was not listed on all of the European formulas, could not be evaluated, all formulas except one met the remaining FDA nutrient requirements. These European infant formulas are being imported into the United States via third party vendors and are not FDA-regulated, limiting the notable consumer protections set by the FDA that ensure infant formula safety. Pediatric gastroenterologists and healthcare providers need to understand the composition, labelling and lack of FDA regulation and safety concerns of these formulas in order to better counsel parents.

Assessment of Community Pediatric Providers' Approach to a Child With Celiac Disease and Available Serological Testing Associated With a Large Tertiary Care Center.

An evidence-practice gap for use of celiac disease testing can lead to poor resource utilization. False positive tests may lead to unnecessary diet changes, gastroenterology consults, parental/patient concern, and additional testing and expenses complicated by varied available celiac bundles. An understanding of pediatric provider practices according to guideline recommendations further improves this gap.

Tissue Transglutaminase Levels Are Not Sufficient to Diagnose Celiac Disease in North American Practices Without Intestinal Biopsies.

BACKGROUND: Celiac serology is crucial for the diagnosis of celiac disease in children. The American guideline for celiac disease in children suggested that positive serology should be followed by confirmatory intestinal histology. The relationship between high tissue transglutaminase titers and celiac disease in children has not been well investigated in children from North America. AIMS: In the present study, we investigated whether different tissue transglutaminase titers in symptomatic children could predict celiac disease without the confirmation of intestinal histology. METHODS: Data from biopsy confirmed celiac children were collected from four different clinics in North America. Clinical, serological, histological, and follow-up data were collected. The accuracy rates of various tissue transglutaminase titers to predict celiac disease in children were calculated. RESULTS: The data from 240 children were calculated. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy rate of tissue transglutaminase titers at ≥10× upper limit of normal were 75.4, 48.8, 87.7, 29.0, and 70.8 %, respectively. Similar data were noted in the other tissue transglutaminase titers (≥3× upper limit of normal, >100 U/ml, or >100 U/ml and >10× upper limit of normal). CONCLUSIONS: The positive predictive value of tissue transglutaminase titers at ≥3× upper limit of normal or higher was too low to predict celiac disease in children. Our data suggested that in routine clinical practice, high titers of tissue transglutaminase are not sufficient to diagnose celiac disease in North American children without intestinal biopsies.

Lysosomal acid lipase deficiency: diagnosis and treatment of Wolman and Cholesteryl Ester Storage Diseases.

Lysosomal acid lipase (LAL) is responsible for the hydrolysis of cholesterol esters and triglycerides. LAL is coded by the LIPA gene on chromosome 10q23.31. Its deficiency leads to two autosomal recessive disorders, Wolman disease (WD) and Cholesteryl Ester Storage Disease (CESD). WD has an estimated incidence of 1 in 500,000 live births and is the result of a complete loss of LAL and presents in infancy with vomiting, diarrhea, poor weight gain and hepatomegaly subsequently leading to death. CESD is the result of partial loss of LAL and its presentation is more variable. Patients may be asymptomatic or present with nonspecific gastrointestinal symptoms, hepatomegaly, elevated transaminases and dystipidemia which may be confused with the diagnosis of Non-alcoholic Fatty Liver Disease. CESD is currently underdiagnosed and has an estimated prevalence as high as I in 40,000 individuals. Radiologic findings in WD is calcification of the adrenal glands. Hepatomegaly is noted on CT scan in both WD and CESD. MRI may demonstrate accumulation of cholesterol esters and may be useful to study effects of potential medical therapies. The diagnosis of WD and CESD is based on LIPA gene sequencing and the measurement of LAL levels in peripheral blood leukocytes. Treatment of LAL deficiency is currently limited to control of cholesterol levels and to prevent premature atherosclerosis. Use of enzyme replacement therapy with recombinant human LAL in short-term studies has shown to be safe and effective.

Cholesteryl ester storage disease: protean presentations of lysosomal acid lipase deficiency.

OBJECTIVE: LIPA gene mutations result in deficiency of lysosomal acid lipase and present phenotypically as Wolman disease or cholesteryl ester storage disease (CESD) depending on the level of deficiency. Patients with CESD may often be misdiagnosed because symptoms may be nonspecific. Symptoms may present in infancy if there is complete loss of lysosomal acid lipase or in early childhood or adulthood when there is partial loss. The purpose of the present study is to review the literature for pediatric cases of CESD to better understand the phenotype of CESD. METHODS: A PubMed search of all English-language publications from 1966 through June 2012 for pediatric CESD case reports using the following key words CESD, fatty liver, and NAFLD was performed. All of the cases were reviewed and information regarding age, sex, presenting symptoms, and pertinent laboratory tests were recorded. RESULTS: Seventy-one cases were culled from 39 published case reports. Nearly two-thirds of these patients presented with their first symptoms when they were younger than 5 years. Hepatomegaly and splenomegaly were common features. Serum transaminases and lipids were often elevated. Gastrointestinal symptoms were noted in approximately one-third of cases. Two-thirds of patients had liver fibrosis. CONCLUSIONS: CESD has an estimated incidence as high as 1 in 40,000, which means that it is presently underdiagnosed. Education about common symptoms of CESD as well as a higher level of suspicion for screening for CESD will lead to earlier diagnosis. New treatments for CESD including possible enzyme replacement therapy make early diagnosis especially important.

Conjugated Hyperbilirubinemia in a Child with Streptococcus pneumoniae-associated Hemolytic Uremic Syndrome.

Conjugated hyperbilirubinemia is a rare complication of hemolytic uremic syndrome (HUS). We report a case of a 2-year-old female with Streptococcus pneumonia-associated HUS (SP+ HUS) who developed severe cholestasis. It is important for pediatric gastroenterologists to be aware of manifestations of HUS, and that although rare, cholestasis can be one of the early findings in patients with SP+ HUS.

Management of chronic hepatitis C infection in children.

PURPOSE OF REVIEW: Chronic hepatitis C infection remains a global public health burden and has important clinical implications due to progressive liver fibrosis and development of cirrhosis and its complications. The role of antiviral therapy in infected children is an area of controversy due to an indolent clinical course in the majority of children, and a low likelihood of viral eradication in response to an intensive interferon-based treatment course that is associated with a wide spectrum of adverse effects. This review summarizes new concepts in the epidemiology, natural history, and management of chronic hepatitis C infection in children. RECENT FINDINGS: In the past 18 months, two large prospective studies demonstrated high rates of sustained virologic response in children with chronic hepatitis C infection, estimated at 53% in genotype 1 with peginterferon α-2b-ribavirin, and 47% in genotype 1 with peginterferon α-2a-ribavirin. On this basis, both combination regimens have been recently approved by the Food and Drug Administration (FDA) for use in children. SUMMARY: Children with hepatitis C infection may benefit from early treatment, and the decision to pursue antiviral therapy should be based on individual assessment of host and viral characteristics, and stage of liver fibrosis.

Niemann-Pick type C disease proteins: orphan transporters or membrane rheostats?

Niemann-Pick type C (NPC) disease is a panethnic lysosomal lipidosis, which results in severe cerebellar impairment and death, and is proposed to be a consequence of defective metabolite transport. Numerous models of this disorder have defined the phenotypic impact of misfunction of the NPC proteins, however, their mechanism of action and definition of substrate(s) remain vague and disputed. The proteins may be lipid chaperones, nonspecific transporters, orphan transporters or membrane-sensing regulators ('rheostats') of other transport reactions. These issues pertain to the nature or even existence of a toxic metabolite as causative to this disorder and thus ultimately to treatment of the disease. This review will present the issues that underpin NPC disease and current or future avenues of treatment.