Infant Formula With a Specific Blend of Five Human Milk Oligosaccharides Drives the Gut Microbiota Development and Improves Gut Maturation Markers: A Randomized Controlled Trial.

Background: Human milk oligosaccharides (HMOs) have important biological functions for a healthy development in early life. Objective: This study aimed to investigate gut maturation effects of an infant formula containing five HMOs (2'-fucosyllactose, 2',3-di-fucosyllactose, lacto-N-tetraose, 3'-sialyllactose, and 6'-sialyllactose). Methods: In a multicenter study, healthy infants (7-21 days old) were randomly assigned to a standard cow's milk-based infant formula (control group, CG); the same formula with 1.5 g/L HMOs (test group 1, TG1); or with 2.5 g/L HMOs (test group 2, TG2). A human milk-fed group (HMG) was enrolled as a reference. Fecal samples collected at baseline (n∼150/formula group; HMG n = 60), age 3 (n∼140/formula group; HMG n = 65) and 6 (n∼115/formula group; HMG n = 60) months were analyzed for microbiome (shotgun metagenomics), metabolism, and biomarkers. Results: At both post-baseline visits, weighted UniFrac analysis indicated different microbiota compositions in the two test groups (TGs) compared to CG (P < 0.01) with coordinates closer to that of HMG. The relative abundance of Bifidobacterium longum subsp. infantis (B. infantis) was higher in TGs vs. CG (P < 0.05; except at 6 months: TG2 vs. CG P = 0.083). Bifidobacterium abundance was higher by ∼45% in TGs vs. CG at 6-month approaching HMG. At both post-baseline visits, toxigenic Clostridioides difficile abundance was 75-85% lower in TGs vs. CG (P < 0.05) and comparable with HMG. Fecal pH was significantly lower in TGs vs. CG, and the overall organic acid profile was different in TGs vs. CG, approaching HMG. At 3 months, TGs (vs. CG) had higher secretory immunoglobulin A (sIgA) and lower alpha-1-antitrypsin (P < 0.05). At 6 months, sIgA in TG2 vs. CG remained higher (P < 0.05), and calprotectin was lower in TG1 (P < 0.05) vs. CG. Conclusion: Infant formula with a specific blend of five HMOs supports the development of the intestinal immune system and gut barrier function and shifts the gut microbiome closer to that of breastfed infants with higher bifidobacteria, particularly B. infantis, and lower toxigenic Clostridioides difficile. Clinical Trial Registration: [https://clinicaltrials.gov/ct2/show/], identifier [NCT03722550].

[Bartter syndrome--case report]. / Zespól Barttera--opis przypadku.

The authors present the case of 4-month-old girl, who was admitted to our hospital with hypokalemia, metabolic alkalosis, hyperaldosteronism, hyperreninism with normal blood pressure and high urine concentration of PGE2. All the clinical and biochemical features have led to the diagnosis of Bartter syndrome. Treatment consisted of 15% KCI, spironolacton and indometacin.

[Gastroesophagopharyngeal reflux in infants and children with recurrent symptoms of the upper respiratory tract]. / Refluks zoladkowo-przelykowo-gardlowy u niemowlat i dzieci z nawracajacymi objawami z górnych dróg oddechowych.

Gastroesophageal reflux (GER) plays an important role in pathogenesis of recurrent/chronic disorders of the respiratory tract. Atypical symptoms of GER can be suggested to be cause of the otorhinolaryngological problems. For these last manifestations no cause-effect relationship has yet been proven. There are many therapeutic studies, in which treatment of GERD is examined for its impact on coexisting respiratory disorders. The aim of our study was to confirm the presence of acid reflux by using 24-hour intraesophageal pH monitoring. From the group of 29 patients with recurrent episodes of the pharyngitis, laryngitis and tracheitis, we evaluated 18 children aged 3 months to 8 years (mean, 4.23 +/- 2.85) with coexisting reflux symptoms. The protocol included a parenteral interview, physical examination, roentgenograms of the chest and larynx, laryngoscopy, as well as 24-hour simultaneous proximal and distal esophageal pH monitoring. The most significant differences between examined patients and control subjects was noted in terms of the lowest pH value, number of reflux episodes and index reflux while pH dropped below 4. Every significant drop under pH 6 recorded in proximal esophagus was simultaneous with reflux episode in distal esophagus. We found increased both sensitivity and specificity of the simultaneous pH monitoring in the distal and proximal part of the esophagus comparing to monitoring by the single probe. We confirmed the presence of gastroesophagopharyngeal reflux in patients with recurrent disorders of pharynx, larynx and/or trachea.

[Bartter's syndrome]. / Zespól Barttera.

Bartter syndrome is an uncommon tubular disorder inherited as an autosomal recessive entity. It is associated with hypokalemic metabolic alkalosis with high renin and aldosterone plasma concentration with low or normal blood pressure. Recent studies have demonstrated genetic heterogeneity in Bartter syndrome. Mutations of two genes encoding the Na/K/2Cl cotransporter and potassium channel ROMK are responsible for clinical features of neonatal Bartter syndrome. Mutations of gen encoding the chloride channel ClC-Kb is identified as being causative for the classic Bartter syndrome. And dysfunction of Na/Cl cotransporter in the distal convoluted renal tubule is described as Gitelman syndrome.

[Long-term clinical observation of infants with gastroesophageal reflux]. / Dlugotrwala obserwacja kliniczna niemowlat z rozpoznanym refluksem zoladkowo-przelykowym.

The aim of the study was long-term observation of patients with gastroesophageal reflux (GER), diagnosed in their infancy, in aspect of spontaneous resolution of this disorder. 290 symptomatic infants aged 5 weeks to 11 months (mean--5.8 months) underwent 24-hour esophageal pH monitoring. In 243 (83.8%) patients, abnormal reflux score was observed. After one year of conservative therapy, follow-up study by esophageal pH-monitoring has been performed in 136 (46.9%) children and still showed pathological GER in 95 (69.8%) subjects. After 2 years, 45 (15.5%) patients has been investigated--14/45 (31.0%) of them had normal reflux score. At the following five-year clinical observation was available group of 28/290 (9.6%) patients. In the 2-3 year of their life, 13 of them had resolved symptoms and had normal acid reflux parameters, whereas 9 patients still had pathological GER. In the following years, basing of results of 24-hour pH monitoring, pathological GER persisted: in 7 children in age 4, in 5 subjects in age 5 and in 3 children over fifth year of life. Long-term clinical follow-up of these children is necessary because of risk of GER complications.

[Dual simultaneous esophageal pH monitoring in infants with gastroesophageal reflux]. / Podwójne monitorowanie pH przelyku u niemowlat z objawami refluksu zoladkowo-przelykowego.

The aim of the current study was to analyse selected parameters of pH monitoring in the proximal and distal parts of esophagus. One hundred and twelve infants aged 1.25 to 18 months (mean = 5.6) with symptoms and signs suggesting gastroesophageal reflux (GER) were evaluated. The results are presented of the measurement of reflux index (RI), the number of reflux episodes and the duration of the longest reflux episode in patients classified into the following groups: group I--39 children with vomiting/excessive regurgitation, group II--29 infants with persistent distress/inconsolable crying, group III--16 children with Apparent Life Threatening Events (ALTE), group IV--28 infants with chronic/recurrent respiratory system diseases. No statistically significant difference was noted between the groups in pH parameters at the distal esophageal level, whereas at the proximal level the differences included only the number of reflux episodes. However, we found, that by using the ANOVA test, the incidence was higher in group IV than in group II. As determined by applying Mann-Whitney rank sum test, reflux episodes occurred most frequently in group IV, than in other groups of patients (including controls). None of the reflux parameters recorded at the proximal level among children presenting with ALTE was statistically significantly different than in other groups.

[Alkaptonuria: a rare metabolic disorder. A report of two cases in siblings]. / Alkaptonuria--rzadka choroba metaboliczna. Rozpoznanie rodzinne u dwojga dzieci.

Alkaptonuria is a rare metabolic condition caused by congenital homogentisate oxidase deficiency of recessive inheritance. Homogentisate polymers are accumulated and cause urine darkening, brown pigmentation of connective tissue, articular cartilage pathology. The authors present clinical picture, pathogenesis, diagnostic and therapeutic possibilities in patients with alkaptonuria. Two siblings with alkaptonuria are described.