Patients with PFIC present with a high risk of lipid disturbances. PEBD has a beneficial effect on lipid profile in the majority of cases.

PEBD is more effective than IB for the permanent improvement of symptoms of PFIC. Ileal bypass procedure, although initially effective, does not ensure good long-term results in more than 50 % of patients, probably due to intestinal re-absorption of bile acids increasing over time.

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The effects of the in ovo injection site of electrolytes on selected biochemical blood parameters and the quality of layer chicks were investigated. A total of 120 fertile eggs from Rhode Island Red breeders were randomly distributed into 4 groups, with each group including 30 birds. The groups were as follows: untreated control and groups with different injection sites/locations of 500 µL of 0.9% saline (NaCl) on day 18 of incubation, i.e., into the air cell (AC), through the air cell into the amniotic fluid (AFA), and directly into the amniotic fluid (AF). Measurement at 1 day of age showed that regardless of the injection site, embryos injected with 500 µL of saline had significantly higher Tona score (95/100 points) compared to the control group (90/100 points). Chick length was similar among the injected groups (mean 14.7 cm) and shorter in the control group (13.9 cm). There was no significant effect of in ovo injection on the biochemical blood parameters: total protein, cholesterol, high-density lipoprotein, low-density lipoprotein, glucose, urea, and uric acid. The highest concentration of sodium was noted in the control group (141.59 mmol/L). Regardless of the injection site/location, chicks treated with 500 µL of NaCl were characterized by a significantly lower blood sodium concentration (by 7.45% (AC), 7.90% (AFA), and 4.84% (AF) compared with birds from the control group (p ≤ 0.01)). The influence of saline solution administration in ovo on the blood potassium content of chicks was demonstrated. The concentration of potassium in the control group was significantly higher (by 11.36%) than in the AC group (p ≤ 0.01). In conclusion, the injection of 500 µL of saline solution into the developing chick embryo during the last days of incubation may have a positive effect on the quality of day-old chicks.

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Introduction Shwachman-Diamond syndrome (SDS) is a rare, autosomal recessive multisystemic disorder characterized by pancreatic insufficiency and bone marrow failure. Short stature is a recognized feature of SDS syndrome; however, systemic data concerning recombinant human growth hormone (rGH) treatment are limited. Aim of the study To assess the effect of rGH treatment in patients with SDS. Material and Methods Retrospective data were collected from patients with SDS and growth hormone deficiency (GHD) treated with rGH in the Children’s Memorial Health Institute in Warsaw. The annual growth velocity (GV) and height standard deviation score (SD) were compared for up to 2 years of rGH treatment. Results Six SDS patients (M : F = 1 : 5) treated with rGH were identified. The median age of starting rGH therapy was 7.5 years, with a mean baseline height SD of –4.06 (range: –6.3 to –2.3 SD). The height SD significantly improved to –3.3 ( p = 0.002) and then –3.03 ( p = 0.002), following 1 and 2 years of treatment, respectively. The average GV for the patients prior to starting treatment was 4.9 cm/year (range: 3.1–6.5 cm/year), which significantly improved to 7.6 cm/year (range: 5.7–9.6 cm/year) after 1 year of rGH treatment ( p = 0.020) and to 6.7 cm/year at the end of 2 years. Conclusions Our study has shown that rGH treatment significantly improves the height SDS and GV of patients with SDS and GHD without any side effects. Further research is required to analyse the long-term effect of rGH therapy in patients with SDS.

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