Meconium Research Articles

Anti-obesity Potential of Lactobacillus spp. Isolated from Infant Feces

Anti-obesity Potential of Lactobacillus spp. Isolated from Infant Feces

Safety Evaluation of Bifidobacterium breve IDCC4401 Isolated from Infant Feces for Use as a Commercial Probiotic.

Previously, our research group isolated Bifidobacterium breve IDCC4401 from infant feces as a potential probiotic. For this study, we evaluated the safety of B. breve IDCC4401 using genomic and phenotypic analyses. Whole genome sequencing was performed to identify genomic characteristics and investigate the potential presence of genes encoding virulence, antibiotic resistance, and mobile genetic elements. Phenotypic analyses including antibiotic susceptibility, enzyme activity, production of biogenic amines (BAs), and proportion of D-/L-lactate were evaluated using E-test, API ZYM test, high-performance liquid chromatography (HPLC), and D-/L-lactic acid assay respectively. The genome of B. breve IDCC4401 consists of 2,426,499 bp with a GC content of 58.70% and 2,016 coding regions. Confirmation of the genome as B. breve was provided by its 98.93% similarity with B. breve DSM20213. Furthermore, B. breve IDCC4401 genes encoding virulence and antibiotic resistance were not identified. Although B. breve IDCC4401 showed antibiotic resistance against vancomycin, we confirmed that this was an intrinsic feature since the antibiotic resistance gene was not present. B. breve IDCC4401 showed leucine arylamidase, cystine arylamidase, α-galactosidase, β-galactosidase, and α-glucosidase activities, whereas it did not show production of harmful enzymes such as β-glucosidase and β-glucuronidase. In addition, B. breve IDCC4401 did not produce any tyramine, histamine, putrescine, cadaverine, or 2-phenethylamine, which are frequently detected BAs during fermentation. B. breve IDCC4401 produced 95.08% of L-lactate and 4.92% of Dlactate. Therefore, our findings demonstrate the safety of B. breve IDCC 4401 as a potential probiotic for use in the food industry.

A Novel Bacteriocin From Lactobacillus Pentosus ZFM94 and Its Antibacterial Mode of Action.

Bacteriocins are bioactive antimicrobial peptides synthesized in the ribosome of numerous bacteria and released extracellularly. Pentocin ZFM94 produced by Lactobacillus pentosus (L. pentosus) ZFM94, isolated from infant feces with strong antibacterial activity, was purified by ammonium sulfate precipitation, dextran gel chromatography, and reverse-phase high-performance liquid chromatography (RP-HPLC). The molecular mass of the purified bacteriocin was 3,547.74 Da determined by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). Pentocin ZFM94 exhibited broad-spectrum antimicrobial activity against tested Gram-positive and Gram-negative bacteria, and the minimal inhibitory concentrations (MICs) of Micrococcus luteus (M. luteus) 10,209, Staphylococcus aureus (S. aureus) D48, and Escherichia coli (E. coli) DH5α were 1.75, 2.00, and 2.50 μm, respectively. Pentocin ZFM94 was heat-stable (30 min at 80°C) and showed inhibitory activity over a wide pH range (5.00–7.00). It could be degraded by trypsin and pepsin, but not by amylase, lysozyme, lipase, and ribonuclease A. Fluorescence leakage assay showed that pentocin ZFM94 induced disruption of the cell membrane and caused leakage of cellular content. Furthermore, lipid II was not an antibacterial target of pentocin ZFM94. This study laid the foundation for further development and utilization of L. pentosus ZFM94 and its bacteriocin.

Outcomes of surgical treatment of neonates with intestinal stomas in a regional perinatal center

Introduction. Emergency intestinal surgeries in neonates can lead to stoma formation. Indications for stoming, associated complications and ways to prevent them are being actively discussed. The aim of this study was to analyze results of surgical treatment of neonates with intestinal stomas in a perinatal center for the last 10 years.Material and methods. 81 children with intestinal obstruction (32), necrotizing enterocolitis (27), meconium ileus (14) and others abdominal pathologies (8) were included into the study. Statistical processing was carried out using the SPSS v.26 package; differences were significant at p-value ≤0.05. Complications were assessed with the Clavien-Dindo Сlassification (CDC).Results. There were 59 premature infants (72.8%); 32 had body weight below 1000 grams (54.2%). Initially performed: 15 (18.5%) colostomies, 49 (60.5%) enterostomies, 17 (21%) T-anastomoses. A compression clip was put in six children with double-barreled ileostomies. Complications were the following: prolapse (12.3%), skin excoriation (43.2%), bleeding (19.8%), large losses of intestinal chyme (17.3%), liver failure (19.8%), sepsis (17.3%), wound dehiscence (6.2%), adhesive obstruction (16%), necrosis (9.9%) and stenosis (7.4%). 16 (19.7%) patients had no complications. 28 (43%) patients had complications by CDC of grade III, and 37 (57%) - by CDC of grade ≥III. Stomas were closed in 32 children (39.5%) after 35 days, on average (6-126 days). Mortality was 28.4%, mainly in children weighing less than 1000 gramm (p = 0.03).Conclusion. If a neonate patient has contraindications to primary anastomosing, double-barreled enterostomy with a compression clip is a safe alternative to it. The enterostomy technique in premature newborns does not increase the rate of complications and mortality. Skin excoriation, increased bleeding from the stoma, and liver failure are most common in neonates with enterostomy.

The Abundance of Human Milk Oligosaccharide (HMO)-Metabolizing Genes in Fecal Samples from Six-Month-Old Human Infants.

Herein, we report the abundance and prevalence of HMO-metabolizing genes, specifically those of Bifidobacterium infantis, in fecal samples from human infants. Forty dyads were enrolled, and each mother collected a fecal sample from her infant at six months of age. Genomic DNA was extracted, and quantitative real-time PCR was used to determine gene abundance. The mode of delivery was not associated with gene abundance. Several gene regions, Sia (a sialidase), B. inf (16S), and GH750 (a glycoside hydrolase), were more abundant in the feces of human milk-fed infants (p < 0.05). Others, Sia and HC bin (16S), tended to be less abundant when a larger percentage of an infant’s diet consisted of solids (p < 0.10). When accounting for solid food intake, human milk exposure was positively associated with Sia and B. inf (p < 0.05) and tended to be related to the abundance of the GH750 and HC bin (p < 0.10) gene regions. With further development and validation in additional populations of infants, these assays could be used to group samples by dietary exposure even where no record of dietary intake exists. Thus, these assays would provide a method by which infant human milk intake can be assessed quickly in any well-equipped molecular biology laboratory.

Maturation of the preterm gastrointestinal tract can be defined by host and microbial markers for digestion and barrier defense

Functionality of the gastrointestinal tract is essential for growth and development of newborns. Preterm infants have an immature gastrointestinal tract, which is a major challenge in neonatal care. This study aims to improve the understanding of gastrointestinal functionality and maturation during the early life of preterm infants by means of gastrointestinal enzyme activity assays and metaproteomics. In this single-center, observational study, preterm infants born between 24 and 33 weeks (n = 40) and term infants born between 37 and 42 weeks (n = 3), who were admitted to Isala (Zwolle, the Netherlands), were studied. Enzyme activity analyses identified active proteases in gastric aspirates of preterm infants. Metaproteomics revealed human milk, digestive and immunological proteins in gastric aspirates of preterm infants and feces of preterm and term infants. The fecal proteome of preterm infants was deprived of gastrointestinal barrier-related proteins during the first six postnatal weeks compared to term infants. In preterm infants, bacterial oxidative stress proteins were increased compared to term infants and higher birth weight correlated to higher relative abundance of bifidobacterial proteins in postnatal week 3 to 6. Our findings indicate that gastrointestinal and beneficial microbial proteins involved in gastrointestinal maturity are associated with gestational and postnatal age.

Neurodevelopmental outcome of very preterm infants with gastrointestinal tract perforations does not differ compared to controls.

SummaryPurposeTo evaluate gastrointestinal tract (GIT) perforations in very low birth weight infants and the effects on neurodevelopmental outcome.MethodsBetween 2000 and 2017 all cases with GIT perforation were analyzed regarding causes, associated morbidities and neurodevelopmental outcome and compared with matched (gestational age, birth weight, gender, year of birth) by 1:2 controls.ResultsThe incidence of GIT perforation was 2.0% (n = 38/1878). Diagnoses associated with GIT were meconium obstruction of prematurity (MOP,n = 19/50%), spontaneous intestinal perforation (SIP, n = 7/18%), necrotizing enterocolitis (NEC, n = 6/16%), iatrogenic perforation (n = 3/8%), volvulus (n = 2/5%) and meconium ileus (n = 1/3%). The NEC-associated perforations occurred later compared to those associated with MOP and SIP (median 8 days and 6 days vs. 17 days, p = 0.001 and 0.023, respectively) and main localization was the terminal ileum (84%). Cases had higher rates of late onset sepsis (55% vs. 24%, p = 0.003), longer duration of mechanical ventilation (median 30 days vs 18 days, p = 0.013) and longer stays at the hospital (median 122 days vs 83 days, p < 0.001); mortality rates did not differ. The 2‑year neurodevelopment follow-up revealed no differences between groups (normal development 49% vs. 40%).ConclusionDespite increased morbidities preterm infants with GIT perforation did not have a higher mortality rate and groups did not differ regarding neurodevelopmental outcome at the corrected for prematurity age of 2 years.

SLC26A6-selective inhibitor identified in a small-molecule screen blocks fluid absorption in small intestine.

SLC26A6 (also known as putative anion transporter 1 [PAT1]) is a Cl–/HCO3– exchanger expressed at the luminal membrane of enterocytes where it facilitates intestinal Cl– and fluid absorption. Here, high-throughput screening of 50,000 synthetic small molecules in cells expressing PAT1 and a halide-sensing fluorescent protein identified several classes of inhibitors. The most potent compound, the pyrazolo-pyrido-pyrimidinone PAT1inh-B01, fully inhibited PAT1-mediated anion exchange (IC50 ~350 nM), without inhibition of the related intestinal transporter SLC26A3 (also known as DRA). In closed midjejunal loops in mice, PAT1inh-B01 inhibited fluid absorption by 50%, which increased to >90% when coadministered with DRA inhibitor DRAinh-A270. In ileal loops, PAT1inh-B01 blocked fluid absorption by >80%, whereas DRAinh-A270 was without effect. In colonic loops, PAT1inh-B01 was without effect, whereas DRAinh-A270 completely blocked fluid absorption. In a loperamide constipation model, coadministration of PAT1inh-B01 with DRAinh-A270 increased stool output compared with DRAinh-A270 alone. These results provide functional evidence for complementary and region-specific roles of PAT1 and DRA in intestinal fluid absorption, with PAT1 as the predominant anion exchanger in mouse ileum. We believe that PAT1inh-B01 is a novel tool to study intestinal ion and fluid transport and perhaps a drug candidate for small intestinal hyposecretory disorders such as cystic fibrosis–related meconium ileus and distal intestinal obstruction syndrome.

Association and Occurrence of Bifidobacterial Phylotypes Between Breast Milk and Fecal Microbiomes in Mother-Infant Dyads During the First 2 Years of Life.

Breast milk acts as an intermediary for the transfer of functionally important commensal bacteria from mother to infant, especially for Bifidobacterium that can colonize the infant gut. However, the vast majority of rRNA amplicon-based studies reported the conspicuous intercohort and interindividual variation for the prevalence of Bifidobacterium in breast milk. In order to elucidate whether Bifidobacterium phylotypes persistently co-occured at the species or strain level in mother–breast milk–infant triads, we analyzed collectively the next-generation sequencing (NGS) datasets of bacterial 16S rRNA gene and the Bifidobacterium-specific groEL gene from maternal feces, breast milk, and infant feces in a small yet very homogeneous cohort of 25 healthy Uyghur mother–infant pairs (lactation for 7–720 days) in Kashgar, Xinjiang, China. Overall, 16S rRNA gene analysis showed that microbiome in the newborn gut was closer to that of breast milk in the first 4 months of lactation, and subsequently showed an obvious trend of adulthood at 6–12 months. Based on the BLAST accurate taxonomic result of the representative sequences of all ASVs (amplicon sequencing variants), only three sets of ASVs could be clearly assigned into Bifidobacterium species, whereas the remaining eight sets of ASVs corresponded to four indefinite Bifidobacterium species group. By contrast, the groEL gene dataset was partitioned into 376 ASVs, at least belonging to 13 well-known Bifidobacterium species or subspecies, of which 15 ASVs, annotated to seven well-known Bifidobacterium species or subspecies, showed triadic synchronism in most 23 mother–infant pairs tested. However, several other rare bifidobacterial phylotypes, which were frequently encountered in animals, were found to display no correspondence of the presence between the three ecosystems of mother–infant pairs. Our test results were obviously to support the hypothesis that breast milk acts as an intermediary for the transfer of probiotic commensal bacteria from mother to infant, especially for endosymbiotic Bifidobacterium that can colonize the infant gut. Some oxygen-insensitive exogenous Bifidobacterium phylotypes with a cosmopolitan lifestyle may be indirectly transferred to breast milk and the infant’s intestinal tract through environmental contamination. Thus, the groEL gene proved to be a very effective target for the depth resolution of Bifidobacterium community by high-throughput sequencing technologies.

Combining HPAEC-PAD, PGC-LC-MS, and 1D 1H NMR to Investigate Metabolic Fates of Human Milk Oligosaccharides in 1-Month-Old Infants: a Pilot Study.

A solid-phase extraction procedure was optimized to extract 3-fucosyllactose and other human milk oligosaccharides (HMOs) from human milk samples separately, followed by absolute quantitation using high-performance anion-exchange chromatography-pulsed amperometric detection and porous graphitized carbon-liquid chromatography–mass spectrometry, respectively. The approach developed was applied on a pilot sample set of 20 human milk samples and paired infant feces collected at around 1 month postpartum. One-dimensional 1H nuclear magnetic resonance spectroscopy was employed on the same samples to determine the relative levels of fucosylated epitopes and sialylated (Neu5Ac) structural elements. Based on different HMO consumption patterns in the gastrointestinal tract, the infants were assigned to three clusters as follows: complete consumption; specific consumption of non-fucosylated HMOs; and, considerable levels of HMOs still present with consumption showing no specific preference. The consumption of HMOs by infant microbiota also showed structure specificity, with HMO core structures and Neu5Ac(α2-3)-decorated HMOs being most prone to degradation. The degree and position of fucosylation impacted HMO metabolization differently.

Variation in Human Milk Composition Is Related to Differences in Milk and Infant Fecal Microbial Communities.

Previously published data from our group and others demonstrate that human milk oligosaccharide (HMOs), as well as milk and infant fecal microbial profiles, vary by geography. However, little is known about the geographical variation of other milk-borne factors, such as lactose and protein, as well as the associations among these factors and microbial community structures in milk and infant feces. Here, we characterized and contrasted concentrations of milk-borne lactose, protein, and HMOs, and examined their associations with milk and infant fecal microbiomes in samples collected in 11 geographically diverse sites. Although geographical site was strongly associated with milk and infant fecal microbiomes, both sample types assorted into a smaller number of community state types based on shared microbial profiles. Similar to HMOs, concentrations of lactose and protein also varied by geography. Concentrations of HMOs, lactose, and protein were associated with differences in the microbial community structures of milk and infant feces and in the abundance of specific taxa. Taken together, these data suggest that the composition of human milk, even when produced by relatively healthy women, differs based on geographical boundaries and that concentrations of HMOs, lactose, and protein in milk are related to variation in milk and infant fecal microbial communities.

Structure-Specific and Individual-Dependent Metabolization of Human Milk Oligosaccharides in Infants: A Longitudinal Birth Cohort Study

To follow human milk oligosaccharide (HMO) biosynthesis and in vivometabolization, mother milk and infant feces from 68 mother–infant dyads at 2, 6, and 12 weeks postpartum were analyzed, with 18 major HMOs quantitated. Fucosylated and neutral core HMO levels in milk were dependent on mothers’ Lewis/Secretor status, whereas most sialylated HMO levels were independent. Infant fecal excretion of HMOs gradually declined with age, especially for neutral core structures. Although decreasing in absolute concentrations in milk during lactation, the relative abundance of total fucosylated HMOs increased in both milk and feces. Mono-fucosylated HMOs were more consumed than those decorated with two fucose moieties. More (α2-3)-sialylated HMOs were degraded than (α2-6)-sialylated HMOs. The transition speed of HMO metabolization from nonspecific or structure-specific consumption stage to the complete consumption stage was individual-dependent. Variation was associated with mode and place of delivery, where caesarean section or early exposure to hospital environment delayed the transition.

Lactobacillus rhamnosus HDB1258 modulates gut microbiota-mediated immune response in mice with or without lipopolysaccharide-induced systemic inflammation

BackgroundGut microbiota closely communicate in the immune system to maintain a balanced immune homeostasis in the gastrointestinal tract of the host. Oral administration of probiotics modulates gut microbiota composition. In the present study, we isolated Lactobacillus rhamnosus HDB1258, which induced tumor necrosis factor (TNF)-α and interleukin (IL)-10 expression in macrophages, from the feces of breastfeeding infants and examined how HDB1258 could regulate the homeostatic immune response in mice with or without lipopolysaccharide (LPS)-induced systemic inflammation.ResultsOral administration of HDB1258 significantly increased splenic NK cell cytotoxicity, peritoneal macrophage phagocytosis, splenic and colonic TNF-α expression, TNF-α to IL-10 expression ratio, and fecal IgA level in control mice, while Th1 and Treg cell differentiation was not affected in the spleen. However, HDB1258 treatment significantly suppressed peritoneal macrophage phagocytosis and blood prostaglandin E2 level in mice with LPS-induced systemic inflammation. Its treatment increased LPS-suppressed ratios of Treg to Th1 cell population, Foxp3 to T-bet expression, and IL-10 to TNF-α expression. Oral administration of HDB1258 significantly decreased LPS-induced colon shortening, myeloperoxidase activity and NF-κB+/CD11c+ cell population in the colon, while the ratio of IL-10 to TNF-α expression increased. Moreover, HDB1258 treatment shifted gut microbiota composition in mice with and without LPS-induced systemic inflammation: it increased the Cyanobacteria and PAC000664_g (belonging to Bacteroidetes) populations and reduced Deferribacteres and EU622763_s group (belonging to Bacteroidetes) populations. In particular, PAC001066_g and PAC001072_s populations were negatively correlated with the ratio of IL-10 to TNF-α expression in the colon, while the PAC001070_s group population was positively correlated.ConclusionsOral administered HDB1258 may enhance the immune response by activating innate immunity including to macrophage phagocytosis and NK cell cytotoxicity in the healthy host and suppress systemic inflammation in the host with inflammation by the modulation of gut microbiota and IL-10 to TNF-α expression ratio in immune cells.

An Examination of Lactobacillus paracasei GKS6 and Bifidobacterium lactis GKK2 Isolated from Infant Feces in an Aged Mouse Model.

Supplementary which could maintain normal physiological mechanisms and functions while aging has drawn our attention due to the population aging in recent years. Probiotics have been believed with desirable properties such as antioxidation and anti-inflammatory for delaying the aging process. However, the age-related experiments conducted in the mammalian models with probiotics were few. In this study, we demonstrated the effects of administration of probiotics Lactobacillus paracasei GKS6 (GKS6) and Bifidobacterium lactis GKK2 (GKK2), respectively, at the dosage of 5.0 × 109 cfu/kg BW/day for fourteen weeks in senescence-accelerated mouse prone 8 (SAMP8) mice. The three-month-old SAMP8 mice were divided into three groups: control, mice fed with GKS6, and mice fed with GKK2. There were ten females and ten males in each group. The SAMP8 mice fed with probiotics GKS6 and GKK2 showed a significantly lower degree of aging followed by Takeda's grading method on the eleventh week of the experiment. The GKK2 group showed significantly increased forelimb grip strength in male SAMP8 mice and muscle fiber number in both genders. Compared to the control, both GKS6 and GKK2 presented a significant increase in liver superoxide dismutase and catalase activities. In addition, a significant decrease in the levels of liver thiobarbituric acid-reactive substances was observed in the probiotics group. These results suggested that probiotics GKS6 and GKK2 could act as antioxidants in delaying the process of aging and preventing age-related muscle loss.

Genetic mutation in Hirschsprungs/congenital central hypoventilation syndrome

Abstract Background Congenital central hypoventilation syndrome (CCHS) is a disorder of respiratory and autonomic regulation which presents with hypoventilation. In some patients, CCHS is associated with Hirschsprung disease and as such known as Haddad syndrome. Case presentation A preterm was referred to our intensive care unit due to suspicion of meconium ileus after loop-ileostomy at the age of 8 days. The patient presented with recurrent apnea, did not respond to caffeine citrate, and was dependent on ventilation. Furthermore, the patient was unable to pass stool spontaneously. In the course of clinical deterioration, fluoroscopy showed a perforation next to the ileostomy and resection of ileum was conducted. An ileostomy was performed, and several biopsies of the intestines were taken. Neither in the biopsies nor in the resected segment were ganglion cells identified histopathologically. Considering the poor prognosis, life support was withdrawn. Autopsy and histology verified total aganglionosis of the intestines. Genetic testing confirmed a rare pathogenic deletion in the alanine-repeat region in the PHOX2B (c.722-759del; p.Ala241Glyfs*106). Conclusion CCHS and intestinal aganglionosis are hints for Haddad syndrome. To confirm the diagnosis, biopsies of the intestines and genetic testing for mutations in the PHOX2B gene should be performed.

Screening of potential probiotics with anti-Helicobacter pylori activity from infant feces through principal component analysis

Helicobacter pylori is considered as the causative agent of gastrointestinal diseases. The large increase of drug-resistant strains caused by the treatment of H. pylori makes it very important to develop new treatment strategies. This study aimed to isolate and select Lactobacillus strains from infant feces and evaluate the inhibitory effects against H. pylori. Six strains were initially screened out of Forty-seven Lactobacillus strains obtained from healthy infants feces through evaluating acid (pH 3.0) and bile salt tolerance (0.3%). Based on the re-screening of the four indexes of transit tolerance, hydrophobicity, self-aggregation and anti-Helicobacter pylori, combined with the comprehensive evaluation of principal component analysis, these both strains of IF-3083 and IF-5014 were obtained. And these two strains were identified as Lactobacillus plantarum using 16S ribosomal RNA gene sequences. Also, the two identified strains showed good adhesion to human gastric adenocarcinoma (AGS) and human colorectal adenocarcinoma (HT-29) cells, γ-hemolytic activity and susceptibility to seven antibiotics. The viable count of H. pylori SS1 was significantly decreased when co-cultured with the live cells (LC) or cell-free supernatants (CFS) of these two strains. These preliminary results suggested that L. plantarum IF-3083 and L. plantarum IF-5014 had the potential to be used as probiotics to prevent H. pylori infection.

A Case of Idiopathic Diffuse Pneumoperitoneum Presented with Neonatal Abdominal Color Change

Background: Necrotizing enterocolitis is the leading cause of intestinal perforation and pneumoperitoneum in neonates. In addition, neonatal pneumoperitoneum includes several conditions requiring surgery, such as gastric and duodenal perforation, intestinal atresia, volvulus, meconium ileus, and Hirschsprungג��s disease. A type of pneumoperitoneum in which intra-abdominal free air is detected through direct X-ray; however, no cause is found clinically and radiologically during the surgery is named ג��benign pneumoperitoneumג��. Case report: This case report presents a 5-day-old male newborn who is presented with complaints of abdominal color change and vomiting and is diagnosed with Idiopathic Benign Pneumoperitoneum (IBP) during the operation. Neonatal pneumoperitoneum usually occurs as a result of perforation and requires emergency surgery. Rare cases of IBP should also be kept in mind. Unnecessary surgery can be prevented by establishing a differential diagnosis with clinical, laboratory, and imaging methods.  Conclusion: This case demonstrated that laparotomy is not a true routine in neonates with IBP if a timely diagnosis is established.

Isolation of Novel Strains of Lactobacillus gasseri EJL and Bifidobacterium breve JTL from Breast Milk and Infant Feces: A Longitudinal Study of a Mother-infant Pair

Human breast milk is a potential source of bacteria for the development of the intestinal microbiota of infants. Several species within the genera Lactobacillus and Bifidobacterium were demonstrated to shape the gut microbiota of infants. In this study, the bacterial diversity was investigated in the breast milk and feces of a mother-infant pair, and probiotic candidates were identified. Importantly, the novel L. gasseri EJL and B. breve JTL strains were isolated from breast milk and infant feces samples, respectively; their completed genome was resolved using de novo sequencing. In addition, the bacterial composition in the infant’s feces at 1 week revealed the prevalence of Bifidobacterium and Streptococcus; a higher diversity was observed after 3 weeks. In particular, the abundance of Akkermansia was sharply increased at 7 weeks, further increasing thereafter, up to 15 weeks. Our results suggest that human breast milk and infant’s feces are a source of probiotic candidates.

Phenotypic Presentations of Cystic Fibrosis in Children of African Descent.

The Robert Debre Pediatric Cystic Fibrosis (CF) centre, located in the North East of Paris, a multicultural area, is in charge of a cohort of around a hundred and sixty children diagnosed with CF. Between 2000 and 2019, the proportion of children of African descent in this centre increased from 2% to 10%. We report the clinical features of 17 children of African descent diagnosed with CF: 4 (23%) were diagnosed after a meconium ileus, 14 (83%) had exocrine pancreatic insufficiency, and 7 (41%) had early Pseudomonas aeruginosa infection before the age of two. Even though the majority of patients were diagnosed through NBS, the twenty-nine-mutation testing kit proved less effective in non-Caucasian populations, with a false negative rate of 25% in this series. CF is definitely not solely a Caucasian disease and the literature reveals similar phenotypes in Caucasian and African people provided that they present the same CFTR mutations. Clinicians have to keep in mind that the diagnosis of CF in patients of African descent must be evoked in the case of symptoms and a sweat test must be performed, despite a negative result for NBS.

The first report on CFTR mutations of meconium ileus in cystic fibrosis population in Saudi Arabia: A single center review.

IntroductionMeconium ileus (MI) is one of the most common causes of intestinal obstruction in newborns. It is the earliest clinical manifestation of cystic fibrosis (CF). MI is suspected if a baby fails to pass meconium shortly after birth and develops symptoms of bowel obstruction, such as distention of the abdomen or vomiting. MI can lead to bowel perforation, a twisting of the bowel, or inflammation and infection of the abdominal cavity.ObjectivesTo find the incidence and prevalence of meconium ileus in cystic fibrosis patients and to report on the most common gene mutation of MI in CF patients.MethodologyRetrospective review of the medical documentations of all MI patients during the period of 1989–2018.ResultsA total of 40 CF confirmed patients were presented with MI. Twenty-nine patients (71%) are alive and 11 patients (29%) died or lost to follow-up. The following CFTR mutations were found: Eight patients (20%) with c.2988+1G>A; Intron 18. Seven patients (17.5%) with c.1418delG; Exon 11. Five patients (12.5%) with c.579+1G>T; Intron 5. Four patients (10%) with c.1911delG; Exon 14. Four patients (10%) with c.1521_1523delCTT; Exon 11. Four patients (10%) with c.416A>T; Exon 13. Three patients (7.5%) with c.2421A>G; Exon 14. Two patients (5%) with c.3908A>C; Exon 21. One patient (2.5%) with c.3889dupT; Exon 24. One patient (2.5%) with c.1657C>T; Exon 12. One patient (2.5%) with c.2547C>A; Exon 14a. Eighteen patients (45%) were presented with vomiting, 38 patients (95%) had postnatal radiological findings, 7 patients (17.5%) had electrolytes imbalance. Five patients (12.5%) had cholestasis and 4 patients (10%) developed chronic liver disease. Thirty-five patients (79.5%) underwent surgical repair and 9 patients (20.5%) were treated medically. Mean age of operation was 2.25 (2) days. Of 9 patients, 6 (66.6%) were treated with gastrograffin enema, 2 patients (22.2%) with oral N-acetylcysteine and 1 patient (11.1%) with saline rectal wash. Thirteen patients (31.5%) required TPN. Five patients had recurrent operation.ConclusionCF and meconium ileus are commonly present in CF patients in Saudi Arabia. Prognosis is similar to other CFs without MI, if treated early. Thirty percent of our CF/MI patients have intronic mutations.