Mesenchymal stem cells are multipotent progenitor cells capable of differentiating into several lineages including adipocytes, and osteocytes. While they are regarded as non-immunogenic, accumulating evidence suggests that MSC expresses several TLRs. We checked the contribution of TLR ligands to MSC differentiation. Rat bone marrow derived MSC surface marker and TLR transcript levels were checked by FACS and RT-PCR respectively. MSCs were CD29hi, CD71dim, CD90hi, and negative for CD45 & CD34. They expressed TLR1,2,3,4,6, and low levels of 7 and 9 (no TLR5 was detected) at passage zero. Cells were incubated in adipogenic and osteogenic differentiation media either alone or supplemented with, TLR2L:PGN, TLR3L:pI:C, TLR7L:R848, and TLR9L:CpGODN as well as control ODN and followed for three weeks. Oil Red O staining (to assess adipogenesis) suggested that, induction in adipogenic media (by one week) gave no adipose positive cells whereas treatment with R848 or PGN induced significantly high adipose positive cells (1-2% vs 30-35%, respectively). The degree of adipogenic differentiation at wk1, was; TLR7≥TLR2>TLR3>TLR9>Control group≥medium. During osteogenesis (checked by alizarin red staining) by one week the highest differentiation was seen with CpG ODN treatment (TLR9≥TLR7>TLR2>TLR3>Control≥medium). These results strongly support the view that TLR ligands differentially contribute to transition of MSCs into either adipogenic or osteogenic differentiation.

Introduction Herein, we aimed to discuss our experience in 16 newborn patients with Maple syrup urine disease (MSUD) who were treated with urgent renal replacement therapy (RRT). Methods The patients underwent continuous veno‐venous hemodiafiltration (CVVHDF) or peritoneal dialysis (PD) as renal replacement therapy. Results Eleven (68.75%) patients underwent CVVHDF and five (31.25%) underwent peritoneal dialysis. The median leucine reduction rate per hour was 2.56% (1.75–7.6) in the CVVHDF group, 0.78% (0.54–1.83) in the PD group, and was significantly higher in the CVVHDF group (p = 0.001). Posttreatment plasma leucine levels were found to be 198 (20–721) μmol/L in the CVVHDF group and 600 (250–967) μmol/L in the PD group, and CVVHDF was found to be significantly lower (p = 0.08). Complications such as hypotension, electrolyte imbalance, and filter obstruction occurred in the CVVHDF group. Conclusion This study showed that CVVHDF is more effective than PD for rapidly eliminating elevated leucine levels caused by MSUD in the newborn and it is not associated with increased complication rates.

Objective: Inborn errors of metabolisms (IEMs) are a heterogeneous group of disorders that can occur as a result of inherited or spontaneous mutations, are rare when considered alone, but have many diseases as a group. Although they are generally thought of as diseases of the neonatal period, 50% of these also occur outside the neonatal period, and some are not diagnosed until adulthood. The aim of this study is to examine the frequency, clinical and laboratory features of inborn errors of metabolism in the neonatal period. Methods: The results of 60 patients who were diagnosed with IEMs as a result of the study, out of 1400 patients who were followed up and treated in the Children's Hospital Neonatal Intensive Care Unit between January 2018 and December 2020, were evaluated. Results: In this study, it consisted of 60 cases, 30 (%50) of which were girls and 30 (%50) were boys (50%). The median age of the patients included in the study was 3 (1-25) days at admission, median weight was 3100 (1000-4000) g, and gestational week was found to be median 39 (27-40) weeks at admission. The most common complaints were feeding difficulty 43(72%), tachypnea 27 (45%) and vomiting 5 (8.3%). The most common laboratory findings were metabolic acidosis 39 (65%) and 36 (60%) hypoglycemia. There was a history of consanguinity in 46 (76.6%) cases, and a history of sibling death in 30 (50%). The mother had a history of abortion in 5 (8.3%) patients who did not have a history of sibling death. The most common diagnoses were Urea cycle disorder 13 (21.7%), organic acidemia 12 (20%), galactosemia 10 (16.7%) and amino acid metabolism disorders 9 (15%). Conclusion: Inborn errors of metabolisms, which are a group of diseases that can occur in different spectrums with clinical, biochemical and genetic heterogeneity, should be kept in mind especially in regions where consanguineous marriages are high. In cases such as metabolic acidosis, which presents with nonspecific symptoms such as malnutrition, tachypnea and jaundice in the neonatal period and cannot be explained in laboratory examinations, care should be taken and they should be referred to centers for further investigation.

Introduction: Continuous Renal Replacement Therapy (CRRT) is a well-known treatment modality for patients with acute renal failure and has been increasingly used for the treatment of metabolic disorders such as Maple Syrup Urine Disease (MSUD) in recent years. Herein, we aimed to discuss our experience in 16 newborn patients with MSUD who were treated with urgent renal replacement therapy (RRT).Materials and Method: The data of patients who presented with an acute metabolic crisis due to Maple syrup urine disease and who were treated with RRT at Neonatal intensive care unit(NICU) between November 2016 and March 2020 were retrospectively evaluated. The patients underwent continuous veno-venous hemodiafiltration (CVVHDF) or peritoneal dialysis (PD) as renal replacement therapy.Results: The study enrolled a total of 16 patients, of which 8 were male and 8 were female. Eleven (68.75%) patients underwent CVVHDF and five (31.25%) underwent peritoneal dialysis. The median post-treatment leucine level was 198(20-721) μmol/L in the CVVHDF group and 1050(303-1653) μmol/L in the PD group; the median leucine reduction rate per hour was 2.56% (1.75-7.6) in the CVVHDF group and 0.78% (0.54-1.83) in the PD group. There was a significant difference between both groups regarding both parameters (p= 0.08, p=0.001, respectively). Complications such as hypotension, electrolyte imbalance, and filter obstruction occurred in the CVVHDF group while catheter revision was needed due to catheter obstruction in one patient in the PD group.Conclusion: This study showed that CVVHDF is more effective than PD for rapidly eliminating elevated Leucine levels caused by MSUD in the newborn and it is not associated with increased complication rates.