1st Year Of Life Research Articles

Primitive Myxoid Mesenchymal Tumor of Infancy: A Lost Battle

ABSTRACT Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a low to intermediate-grade, poorly differentiated myofibroblastic tumor and is characterized by its tendency to recur locally. It commonly occurs in the 1st year of life and is predominantly seen in the axial skeleton and rarely in the retroperitoneum. We report one such case of PMMTI, which is the second case reported in English literature.

Demystifying Fetus-in-fetu: A Systematic Review of Its Clinical and Pathological Attributes.

Fetus-in-fetu (FIF) is an exceedingly rare condition, characterized by a fetal-like or fetiform mass with a calcified vertebral axis surrounded by other organs or limbs. This systematic review was conducted to comprehensively analyze the clinicopathological attributes, management strategies, and prognosis of FIF to consolidate existing knowledge on FIF. Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a comprehensive search was performed across various electronic databases, using the keywords "fetus-in-fetu," "fetus-ex-fetus," "homunculus," or "fetiform teratoma" to look for published articles until December 2023. Data extraction and analysis were carried out for cases meeting the defined criteria for FIF diagnosis, that is "presence of both/either the vertebral column and/or long bones of extremities" and "absence of immature elements." A total of 249 case reports/case series comprising 241 single FIF cases and 33 multiple FIF cases were included for analysis. The majority of single FIF cases presented within the 1st year of life, with a slight male predominance. Retroperitoneal location was most common, and imaging modalities played a crucial role in preoperative diagnosis. Complete surgical removal of the mass with detailed histopathological examination is the cornerstone of treatment for FIF, with favorable outcomes in the majority of cases. Both mature and immature teratoma can rarely be found in association with FIF. This comprehensive systematic review enhances understanding of FIF, emphasizing the importance of accurate diagnosis by diligent histopathological examination, appropriate management, and vigilant postoperative monitoring for favorable outcomes.

Events of children responsible for the development of epilepsy in patients with cerebral palsy

Background: Cerebral palsy (CP) is characterized by abnormal muscle tone, ‘posture, and movement’, thereby limiting the activity of the affected person. Epilepsy is said to occur in 15-90% of children with CP. The aim of this study was to evaluate the events of children responsible for the development of epilepsy considering natal, postnatal characteristics and associated impairment in patients with CP. Methods: This was a case-control study and was conducted in the Department of Pediatric Neurology at the National Institute of Neurosciences and Hospital, Dhaka, Bangladesh during the period from January 2020 to December 2020. In total 150 children with cerebral palsy were enrolled in this study who were divided into two groups. In the ‘CP with epilepsy’ group, there were 50 patients, and in the CP without epilepsy group taken as a case group, there were 100 age-sex matched CP patients without epilepsy patients were taken as a control group. Informed written consent was taken from all the parents. Along with baseline characteristics, data regarding natal, postnatal events, general clinical findings, psychological assessment, computed tomography (CT) scan findings of the head, and electroencephalogram (EEG) findings in epilepsy cases were recorded in a predesigned questionnaire and analyzed. Results: In distributing the CP patients’ comparison regarding postnatal complications neonatal seizures were a strong predictor for epilepsy (p≤0.001). 1st seizure during 1st year of life and family history of epilepsy was also a significant event of epilepsy (p≤0.001). In the case of microcephaly, no significant difference was observed between groups (p=0.278). A significant difference was observed when the distribution of severity of intellectual disability of CP patients was compared between groups (p<0.001). Abnormal CT scan findings of the head were significantly higher in CP with epilepsy 42 (84.0%) than in CP without epilepsy 46 (46.0%) (p≤0.001). The total mean age at the onset of epilepsy was 13.58±14.47 months. Epilepsy was most common in spastic quadriplegic CP (54%). 38.0% had focal epileptiform activity on EEG. Clinically focal epilepsy was found in 36.0% of cases. Conclusions: This study demonstrates the presence of a history of neonatal seizure, 1st seizure during 1st year of life, a family history of epilepsy, CT scan abnormalities, severe intellectual disability, and spastic quadriplegic CP were the events responsible for the development of epilepsy in children with CP.

A novel nutritional approach to infants and children with congenital diarrhea due to homozygous DGAT1 mutations.

Diacylglycerol acyltransferase (DGAT) catalyzes the final step in triglyceride synthesis. DGAT1 is expressed in human enterocytes and is essential for fat absorption. Homozygous DGAT1 deficiency often presents with severe diarrhea and protein-losing enteropathy (PLE) in the 1st weeks of life. Because severe restriction of fat intake controls diarrhea and decreases PLE, total parenteral nutrition (TPN) was the initial standard therapy in infants and children. We present tertiary center experience managing infants and children with DGAT1 deficiency resulting in the development of a nutritional approach that minimizes the use of TPN. From 2014 to 2020, 12 infants with DGAT1 deficiency were treated. Stool output, growth, and development, as well as essential fatty acid status, were monitored. This retrospective experience formed the basis for treatment recommendations, which include an ultralow fat formula with intermittent peripheral intravenous lipid infusions during the 1st year of life. All patients with prolonged intestinal fat exposure had PLE, which resolved when treated with the nutrition protocol. Essential fatty acid status as measured by triene:tetraene ratios normalized in all treated patients. Over time, early genetic diagnosis and prompt initiation of an ultralow fat diet with peripheral intravenous lipid infusions replaced the need for TPN. Children with DGAT1 deficiency respond to dietary restriction of lipids. Management with a novel nutritional approach provides effective treatment for infants with DGAT1 deficiency, treats diarrhea and PLE, promotes growth and development, avoids TPN dependency, and decreases the potential for essential fatty acid deficiency.

The developmental pattern of native and non-native speech perception during the 1st year of life in Japanese infants

Language development during the 1st year of life is characterized by perceptual attunement: following language-general perception, a decline in the perception of non-native phonemes and a parallel increase in or maintenance of the perception of native phonemes. While this general pattern is well established, there are still many gaps in the literature. First, most evidence documenting these patterns comes from “Minority world countries” with only a limited number of studies from “Majority world countries”, limiting the range of languages and contrasts assessed. Second, few studies test both the developmental patterns of native and non-native speech perception in the same group of infants, making it hard to draw conclusions on simultaneous decline in non-native and increase in native speech perception. Such limitations are in part due to the effort that goes into testing developing speech sound perception, where usually only discrimination of one contrast per infant can be tested at a time. The present study thus set out to assess the feasibility of assessing a given infant on their discrimination of two speech sound contrasts during the same lab visit. It leveraged the presence of documented patterns of the improvement of native and the decline of non-native phoneme discrimination abilities in Japanese, therefore assessing native and non-native speech perception in Japanese infants from 6 to 12 months of age. Results demonstrated that 76 % of infants contributed discrimination data for both contrasts. We found a decline in non-native speech perception evident in discrimination of the non-native /ɹ/-/l/ consonant contrast at 9–11, but not at 11–13 months of age. Additionally, a parallel increase in native speech perception was demonstrated evident in an absence of native phonemic vowel length discrimination at 6–7 and 9–11 months and a discrimination of this contrast at 11–13 months of age. These results, based on a simultaneous assessment of native and non-native speech perception in Japanese-learning infants, demonstrate the feasibility of assessing the discrimination of two contrasts in one testing session and corroborate theoretical proposals on two hallmarks of perceptual attunement: a decrease in non-native and a facilitation in native speech perception during the first year of life.

Type 1 Neurofibromatosis Sans Neurofibroma: A Case Report Emphasizing the Role of Ocular Examination

Neurofibromatosis type 1 (NF1) is one of the most common genetic disorders and is inherited in an autosomal dominant fashion. NF1 is a member of the RASopathies, a class of congenital disorders brought on by mutations in the Ras/mitogen-activated protein kinase pathway. Dermatologic manifestations are typically prevalent in NF1 individuals. Multiple café-au-lait macules (CALMs) may be present at birth or develop in the 1st year of life. Other cutaneous manifestations like freckles in the skinfolds appear in early childhood. Neurofibromas are present in almost all adult patients and may be cutaneous, subcutaneous, or along the peripheral nerves, but their appearance may be delayed till adulthood. CALMs and skinfold freckling are not specific for NF1, and we may need to consider other differential diagnoses like Legius syndrome. A recent modification of the diagnostic criteria includes the identification of the genetic abnormality to facilitate diagnosis in children who may not have cutaneous neurofibromas. This emphasizes the added value of genetic analysis in a condition which is generally a clinical diagnosis. Lisch nodules generally occur by about 7 years of age and are present in 90% of cases. Their presence can help differentiate from Legius syndrome with its more benign course without genetic analysis. We report the case of a 14-year-old male with NF1 who had no cutaneous neurofibromas to reiterate the importance of ophthalmic evaluation in all cases of NF1, especially in resource-poor settings where genetic studies may not be possible.

#2308 The effect of Dapagliflozin on albuminuria and biomarkers associated with renal function in patients with β-thalassemia and/or sickle-cell disease

Abstract Background and Aims Patients with β-thalassemia present already from the 1st year of life with serious anemia thus needing regular blood transfusions in order to attain normal hemoglobin levels. Chronic anemia, iron overload and the use of iron chelating agents have been associated with renal dysfunction. In sickle-cell syndromes, which include sickle-cell anemia and sickle-cell trait, patients present with typical kidney functional and structural defects, resulting in urinary concentrating defects, distal nephron dysfunction and albuminuria. Sodium–glucose cotransporter-2 inhibitors (SGLT-2i) have shown beneficial effects on renal outcomes in large clinical trials with the added benefit of a substantial decrease in albuminuria. The aim of this prospective, non randomized open label study is to investigate the effect of Dapagliflozin at a dose of 10 mg daily on albuminuria and the overall progression of CKD in patients with β-thalassemia and/or sickle-cell disease undergoing regular blood transfusions. Method In this study we included adult patients undergoing regular blood transfusions with a baseline albuminuria of ACR>30 mg/gCr (assessed with a spot urine sample) who received treatment with Dapagliflozin, a second group with no albuminuria that did not receive treatment and a group of adults with no kidney disease (Controls). In all patients renal function biomarkers were measured in urine and serum with commercial ELISA kits (NGAL and uPAR in serum, MMP9 and KIM-1 in urine), while in patients that received Dapagliflozin additional measurements of ACR and eGFR (CKD-EPI formula) were performed after 3 months of treatment initiation. Results In this study we included 16 patients (7 males) with β-thalassemia and/or sickle-cell disease with ACR>30 mg/gCr, 35 patients (17 males) with β-thalassemia and/or sickle-cell disease without albuminuria and 20 controls (13 males). Patients that received Dapagliflozin showed a significant reduction in albuminuria three months post-initiation of treatment (ACR: 0.52 ± 1.2 vs. 0.3 ± 0.68 mg/g, p=0.006) while kidney function showed a slight non-significant reduction (eGFR: 99.3 ± 21.7 vs. 94.7 ± 28.9 ml/min/1.73m2, p=0.36). Concerning the urine and serum biomarkers of tubular dysfunction, inflammation and kidney disease progression, all were significantly increased in patients with β-thalassemia and/or sickle-cell disease in comparison to controls (Table 1). In patients that received treatment with Dapagliflozin, NGAL, MMP-9 and suPAR at 3 months post-treatment initiation did not show any significant reduction (Table 2). Conclusion Patients with β-thalassemia and/or sickle-cell disease show increased urine and serum levels of kidney damage biomarkers in comparison to healthy controls. Treatment of these patients with Dapagliflozin for 3 months has a significant effect in albuminuria reduction while it does not alter urine and serum levels of these biomarkers.

Pattern of urinary tract infection in children with vesicoureteric reflux: Does breastfeeding reduce the occurrence of urinary tract infection?

The protective factors against urinary tract infections (UTIs) in the setting of vesicoureteric reflux (VUR) remain poorly defined. Breastfeeding was suggested as a protective factor against UTI, but its role remains undetermined in this highly susceptible population. The objective of the study was to identify the pattern and risk factors of UTI and investigate the effect of breastfeeding on UTI occurrence in VUR children. This was a mixed-method design, whereby the first part was a cross-sectional study that included children who were diagnosed with VUR and were assessed for their UTI pattern. The second part was a case-control study, which involved contacting the mothers of the children enrolled and questioning them about their breastfeeding pattern, and UTI development was assessed. Our study included 62 children with a median age of 4.4 (interquartile range = 21) months at diagnosis. Of those, 37 (60%) were male and 25 (40%) were female. Most UTIs occurred in the first 3 months of life, and the first episodes were more frequent in males. Constipation was significantly associated with the occurrence of UTI (relative risk [RR] =1.750 [95% confidence interval (CI): 1.231-2.489], P = 0.003). Children with breakthrough UTIs were more likely to have been breastfed for <9 months (odds ratio [OR] = 4.091 [95% CI: 1.287-13.002], P = 0.015) and to have been exclusively breastfed for <2 months (OR = 4.600 [95% CI: 1.337-15.823], P = 0.012). Children with VUR are more susceptible to UTIs in their 1st year of life. Constipation is a major risk factor for UTI occurrence in VUR children and should be aggressively managed. Breastfeeding for longer durations showed promising protective features against breakthrough UTIs.

Psycho-emotional state during pregnancy and one year after childbirth in mothers of children with functional gastrointestinal disorders

Aim. To determine the relationship between the incidence of functional gastrointestinal disorders in children up to 1 year of age and the psycho-emotional state of their mothers during pregnancy and after childbirth.&#x0D; Materials and methods. A survey of 1203 mothers of children under 4 years of age was conducted using a questionnaire of functional gastrointestinal disorders according to the Rome IV criteria (2016). The questionnaire contained domains on general issues (the age of the mother at the time of the child's birth, the data of obstetric and gynecological anamnesis, the type of child's nutrition at the time of the survey), psycho-emotional state (retrospectively, the psychological component of the gestational dominant – PСGD – during pregnancy, Beck's anxiety and Beck's depression questionnaires) and questions on functional disorders in infants and young children.&#x0D; Results. Questionnaires of 487 mothers of children of the 1st year of life were analyzed. Only in 34.4% of cases, mothers' responses lacked anxious and depressive PCGD. In mothers with anxious PСGD, the risk of regurgitation in their children was statistically significantly 2-fold higher compared to mothers with depressive PСGD. In mothers with optimal PСGD, the rate of colic in their children was statistically significantly 2-fold lower compared to mothers with anxious and depressive PСGD. After childbirth, anxiety was noted in 17% mothers and signs of depression in 59% mothers. Significant and severe depression was diagnosed in 10% mothers; the children of these mothers had persistent regurgitation.. In the absence of depression in mothers, the probability of no regurgitation and colic in their children was statistically significantly 2-fold higher compared to the children of mothers with depression.&#x0D; Conclusion. The incidence of functional gastrointestinal disorders in a child up to 1 year of age is associated with maternal anxiety during pregnancy and maternal depression after childbirth.

Utility and limitations of EEG in the diagnosis and management of ALDH7A1-related pyridoxine-dependent epilepsy. A retrospective observational study.

Pyridoxine-dependent epilepsy due to ALDH7A1 variants (PDE-ALDH7A1) is a rare disorder, presenting typically with severe neonatal, epileptic encephalopathy. Early diagnosis is imperative to prevent uncontrolled seizures. We have explored the role of EEG in the diagnosis and management of PDE. A total of 13 Norwegian patients with PDE-ALDH7A1 were identified, of whom five had reached adult age. Altogether 163 EEG recordings were assessed, 101 from the 1st year of life. Median age at seizure onset was 9 h (IQR 41), range 1 h-6 days. Median delay from first seizure to first pyridoxine injection was 2 days (IQR 5.5). An EEG burst suppression pattern was seen in eight patients (62%) during the first 5 days of life. Eleven patients had recordings during pyridoxine injections: in three, immediate EEG improvement correlated with seizure control, whereas in six, no change of epileptiform activity occurred. Of these six, one had prompt clinical effect, one had delayed effect (< 1 day), one had no effect, one had uncertain effect, and another had more seizures. A patient without seizures at time of pyridoxine trial remained seizure free for 6 days. Two patients with prompt clinical effect had increased paroxysmal activity, one as a conversion to burst suppression. Autonomic seizures in the form of apnoea appeared to promote respiratory distress and were documented by EEG in one patient. EEG follow-up in adult age did not show signs of progressing encephalopathy. A neonatal burst suppression EEG pattern should raise the suspicion of PDE-ALDH7A1. Respiratory distress is common; isolated apnoeic seizures may contribute. EEG responses during pyridoxine trials are diverse, often with poor correlation to immediate clinical effect. Reliance on single trials may lead to under-recognition of this treatable condition. Pyridoxine should be continued until results from biomarkers and genetic testing are available.

The effect of early skin-to-skin contact after cesarean section on breastfeeding duration and development of atopic-allergic diseases.

Breastfeeding to strengthen the immune system suggests allergy prevention as a possible option. The connection between breastfeeding and the development of atopic-allergic diseases is being discussed. The primary aim of this work was to investigate an association of the first early skin-to-skin contact following cesarean section with the development of atopic diseases within the 1st year of life. The present study was conducted as a bicentric prospective cohort study in central Germany with a 15-month recruitment period. Data collection was by telephone interviews with a follow-up of 12 months. The statistical evaluation procedure was based on a hierarchical test of the association of early skin-to-skin contact between mother and child with the two main outcome measures. The primary outcome is the duration of breastfeeding. The second outcome is the onset of atopic-allergic disease within the 1st year of life. Mothers breastfed longer if they had skin-to-skin contact within the first 30 minutes postpartum [χ²(df=5) = 19.020, p=0.002], if they breastfed their newborns early immediately after birth (p<0.001), and if the first skin-to-skin contact lasted more than one hour [χ²(df=4) = 19.617, p<0.001]. Regarding atopic-allergic diseases, no significant effects of skin-to-skin contact were found in relation to disease development. Regarding breastfeeding, no significant effects of atopic-allergic diseases could be detected either. The results of this study reflect the benefits of skin-to-skin contact in the context of breastfeeding and atopic disease. The current scientific knowledge regarding skin contact and the development of atopic-allergic diseases should be extended and deepened.

Variability in Receptive Language Development Following Bilateral Cochlear Implantation.

The primary aim was to investigate the variability in language development in children aged 5-7.5 years after bilateral cochlear implantation (CI) up to the age of 2 years, and any impact of the age at implantation and additional noncognitive or anatomical disorders at implantation. Data of 84 congenitally deaf children that had received simultaneous bilateral CI at the age of ≤ 24 months were included in this retrospective study. The results of language comprehension acquisition were evaluated using a standardized German language acquisition test for normal hearing preschoolers and first graders. Data on speech perception of monosyllables and sentences in quiet and noise were added. In a monosyllabic test, the children achieved a median performance of 75.0 ± 12.88%. In the sentence test in quiet, the median performance was 89 ± 12.69%, but dropped to 54 ± 18.92% in noise. A simple analysis showed a significant main effect of age at implantation on monosyllabic word comprehension (p < .001), but no significant effect of comorbidities that lacked cognitive effects (p = .24). Language acquisition values correspond to the normal range of children with normal hearing. Approximately 25% of the variability in the language acquisition tests is due to the outcome of the monosyllabic speech perception test. Congenitally deaf children who were fitted bilaterally in the 1st year of life can develop age-appropriate language skills by the time they start school. The high variability in the data is partly due to the age of implantation, but additional factors such as cognitive factors (e.g., working memory) are likely to influence the variability.

Multivitamins Supplement Uses and Reason Uses by Mother'S for Their Children Less than 24 Months Age Visiting King Abdulaziz Medical City

Background: This study aimed to assess mothers' knowledge regarding multivitamin use for their children in primary health care settings. Methods: It is a cross-sectional survey targeting mothers visiting Primary Health Care. A questionnaire with 14 questions was distributed among two major primary care clinics. Sampling was convenient, and randomness was assumed by visit sequence, on a first-come, first-served basis, and those who agreed to participate. Mothers visiting the Pediatric and Well Baby Clinic within the PHC centers for immunization of their children (under 2 years old) may have been disproportionately selected by this sampling method. The data were entered and analyzed using SPSS with univariate analysis on each question and use of chi-square, exploring mothers' reasons and practices of vitamin use. Results: In our survey, most mothers (62.3%) believed that the best age of giving multivitamins to their children is in the 1st year of life. Twenty-five percent (25%) believed giving at 2 years old is best. Forty-five percent (45%) believed that multivitamins are good for both boys and girls, whereas 33.5% believed that only girls needed it, and 21% believed that only boys need multivitamins. Mothers who believed that children need multivitamins in the first year of life were 2.3 times more likely to give multivitamins to their children than mothers who believed that multivitamins are important after the first year of life. Mothers who believed that their children benefit most from multivitamins in the first year of life were 1.85 times more likely to give them multivitamins to help increase their weight and physical development. Conclusion: Mothers who know foods containing vitamins are 3 times more likely to give multivitamins to their children. In this study, there is no significant difference between the opinions of mothers giving and not giving multivitamins to increase appetite for physical development beyond the first year of life or prevent diseases or for children with chronic illnesses. Others knowledgeable about foods containing multivitamins were similar to mothers who had no knowledge of buying and preparing foods at home.

Exposure of pregnant women and their children to pyrethroid insecticides in Rio de Janeiro, Brazil.

Pyrethroids are commonly used insecticides in Brazil. Gestational and early childhood exposure to pyrethroids has been linked to adverse health effects, including neurodevelopmental delays, behavioral issues, and endocrine disruption. This study evaluated the exposure of pregnant women and their children to pyrethroid insecticides in Rio de Janeiro, Brazil. Creatinine-adjusted levels of the pyrethroid metabolites 3-phenoxy benzoic acid (3-PBA) and 4-fluoro-3-phenoxybenzyl acid (4-FPBA) were measured in the urine of 142 pregnant women and their children at birth and in the first, third, and 6th months of life. The geometric mean (GM) and 95% confidence interval (95% CI) of 3-PBA and 4-FPBA urinary concentrations in pregnant women were 0.50 (0.37-0.67) and 0.37 (0.05-2.90) ng/mg, detected in 47.2 and 10.6%, respectively. Urinary concentrations of 3-PBA in the children were 0.18 (0.15-0.23) ng/mg at birth, 0.36 (0.08-1.56) ng/mg at 1-month-old, 0.68 (0.36-1.27) ng/mg at 3-month-old, and 1.36 (0.77-2.42) ng/mg at 6-month-old, and the detection rates were respectively 10.8, 9.4, 20.9, and 20.7%. This study is one of the few that has evaluated the urinary concentrations of pyrethroids in newborns and children in their 1st year of life. The results of this study show that children's exposure to pyrethroids significantly increases after birth.

The impact of the baby friendly hospital initiative on healthcare utilization among newborns insured by Medicaid in Delaware

BackgroundThe Baby Friendly Hospital Initiative was created to enhance breastfeeding, although its impact on infant healthcare utilization is unclear. Breast feeding infants are vulnerable to readmission soon after birth secondary to dehydration and hyperbilirubinemia. Breastfeeding can also protect infants from unnecessary health care utilization later in life by preventing infection. The objective of this study was to examine the impact of the Baby Friendly Hospital Initiative on readmissions and emergency department utilization among Medicaid births in Delaware.MethodsThe study was a quasi-experimental design. Medicaid claims files were used to study births at five hospitals in Delaware born between January 1, 2014, and December 31, 2018, and covered under Medicaid at time of birth. Three hospitals were designated Baby Friendly, two were not and served as controls. Outcomes included Emergency Department (ED) utilization and readmissions within 30 days and one-year of birth hospitalization. Exposure to the Baby Friendly Hospital Initiative was determined by year and hospital of birth. Logistic regression and interrupted time series segmented regression analysis with controls were used to assess the effect of Baby Friendly Hospital Initiative on healthcare utilization.ResultsIn total, 19,695 infants were born at five hospitals with 80% (15,939) born at hospitals that were designated Baby Friendly. ED utilization and readmissions over the 1st year of life for breastfeeding related diagnosis at the Baby Friendly hospitals occurred in 240 (1.5%) and 226 (1.4%) of infants, respectively. Exposure to the Baby Friendly Hospital Initiative was associated with increased odds of all cause 30-day readmission (AOR: 1.15; 95% CI: 1.03–1.28) but not readmissions over the 1st year of life. While 30-day ED visits did not change after BFHI, one-year ED visits were reduced (0.91, 95% CI 0.86–0.97). A significant negative trend was seen over time for ED utilization post BFHI compared to controls (B: -5.90, p < 0.01).ConclusionThere was a small observed increase in the odds of all cause 30-day readmissions with no change in one-year readmissions after BFHI in Delaware. Although there were no observed changes in 30-day ED utilization, there was a reduction in one-year ED utilization following the implementation of the Baby Friendly Hospital Initiative in Delaware birth hospitals. Our data help to inform policy and decision making for statewide systems of care that may be used to support breast feeding.

HLA Genotype and Probiotics Modify the Association Between Timing of Solid Food Introduction and Islet Autoimmunity in the TEDDY Study.

To study the interaction among HLA genotype, early probiotic exposure, and timing of complementary foods in relation to risk of islet autoimmunity (IA). The Environmental Determinants of Diabetes in the Young (TEDDY) study prospectively follows 8,676 children with increased genetic risk of type 1 diabetes. We used a Cox proportional hazards regression model adjusting for potential confounders to study early feeding and the risk of IA in a sample of 7,770 children. Any solid food introduced early (<6 months) was associated with increased risk of IA if the child had the HLA DR3/4 genotype and no probiotic exposure during the 1st year of life. Rice introduced at 4-5.9 months compared with later in the U.S. was associated with an increased risk of IA. Timing of solid food introduction, including rice, may be associated with IA in children with the HLA DR3/4 genotype not exposed to probiotics. The microbiome composition under these exposure combinations requires further study.

Natural history of mild trigonocephalic deformities.

Ridging along the metopic suture line can be a common cause of concern for parents and has been theorized to represent a mild form of trigonocephaly, a cranial deformity associated with risks of negative cosmetic outcomes, if not surgically corrected. Yet the literature contains sparse reports of long-term cosmetic results or expectations for infants with isolated metopic ridging compared with those with severe trigonocephaly, or even what objective metrics discriminate isolated metopic ridging from severe trigonocephaly. Therefore, the authors' goals for this study were to 1) quantify the degree of frontal deformity among patients with metopic ridge, metopic craniosynostosis, and normocephalic head shapes; and 2) document the natural history of frontal deformities in isolated metopic ridge patients in the 1st year of life. This was a retrospective cohort study of patients with normocephalic head shapes, metopic ridges, and metopic craniosynostoses who presented at < 1 year of age to the Connecticut Children's neurosurgery clinic from January 2019 to December 2021. Data were collected regarding demographics and photograph-based craniometrics. A total of 212 normocephalic, 34 metopic ridge, and 29 metopic craniosynostosis patients were included. Both the normocephalic and metopic ridge groups had a significantly higher anterior arc angle (AAA) value compared with the metopic craniosynostosis group (p < 0.0001). The AAA did not differ significantly among normocephalic patients and those with ridging. Over the course of 1 year of follow-up, patients with metopic ridging demonstrated a slight decrease in AAA values, but overall remained within the same range as normocephalic patients. Photograph-based craniometrics suggest that metopic ridge patients with frontal bone angulations > 2.2 radians have a mild degree of frontal constriction that does not significantly worsen over the 1st year of life.

Anomalous origin of the left coronary artery from the pulmonary artery: A rare congenital cardiac anomaly masquerading as dilated cardiomyopathy

ABSTRACT Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a cardiac anomaly which is quite complex and very rarely diagnosed. It can, however, have serious consequences for the patient. It has been divided into two types based on the age of presentation: the infant type and the adult type with different presentations and prognoses. The infant type usually presents with congestive heart failure or sometimes as myocardial infarction. Many of these patients do not make it beyond 1st year of life. The rare adult variant of ALCAPA syndrome may manifest as recurrent chest infections in the pediatric age group, dyspnea on exertion, or sometimes, even as sudden cardiac death. We report the case of a 7-year-old girl who presented with recurrent chest infections and echocardiography (ECG) was suggestive of dilated cardiomyopathy. The definitive diagnosis of ALCAPA was reached only by ECG-gated cardiac computed tomography.

Effects of early introduction of solid foods on nutrient intake in preterm infants during their 1st year of life: a secondary outcome analysis of a prospective, randomized intervention study.

ClinicalTrials.gov: NCT01809548.

The Relationship between the Stages of Formation and Development of the Child’s Voice and Archaic Tunes

The main purpose of the article is to identify the essential aspects of spontaneous vocalizations of children of the 1st year of life in comparison with the stages of formation of the sound and pitch systems of early song folklore. Based on the analysis of early folklore vocalizations, it becomes evident that their main acoustic characteristics fully coincide with the physiological features of the development of the auditory-motor function of the child in the first postnatal period of its life: from 2 to 7 months, which is characterized as a period of “humming”. Consideration of this interrelation is of the most important value for the vocal pedagogy, which can serve as a theoretical foundation of new approaches to the methods of teaching children singing, developing their hearing and musical thinking at the first stage of teaching them to sing. As a result of the analysis of the stages of formation of early folk intonation according to E.E. Alekseev’s research, it is possible to assume that they correspond to the physiological laws of development of the human auditory-motor function, which is so brightly reflected in the stages of its ontogenetic development. Apparently, the physiological regularities of the transformation of spontaneous vocalizations of children during the first months of their life should be taken into account when constructing methods for the initial stage of learning to sing.