Effects of growth hormone therapeutic supplementation on hematopoietic stem/progenitor cells in children with growth hormone deficiency: focus on proliferation and differentiation capabilities.

We investigated the direct effects of growth hormone (GH) replacement therapy (GH-RT) on hematopoiesis in children with GH deficiency (GHD) with the special emphasis on proliferation and cell cycle regulation. Peripheral blood (PB) was collected from sixty control individuals and forty GHD children before GH-RT and in 3rd and 6th month of GH-RT to measure hematological parameters and isolate CD34(+)-enriched hematopoietic progenitor cells (HPCs). Selected parameters of PB were analyzed by hematological analyzer. Moreover, collected HPCs were used to analyze GH receptor (GHR) and IGF1 expression, clonogenicity, and cell cycle activity. Finally, global gene expression profile of collected HPCs was analyzed using genome-wide RNA microarrays. GHD resulted in a decrease in several hematological parameters related to RBCs and significantly diminished clonogenicity of erythroid progenies. In contrast, GH-RT stimulated increases in clonogenic growth of erythroid lineage and RBC counts as well as significant up-regulation of cell cycle-propagating genes, including MAP2K1, cyclins D1/E1, PCNA, and IGF1. Likewise, GH-RT significantly modified GHR expression in isolated HPCs and augmented systemic IGF1 levels. Global gene expression analysis revealed significantly higher expression of genes associated with cell cycle, proliferation, and differentiation in HPCs from GH-treated subjects. (i) GH-RT significantly augments cell cycle progression in HPCs and increases clonogenicity of erythroid progenitors; (ii) GHR expression in HPCs is modulated by GH status; (iii) molecular mechanisms by which GH influences hematopoiesis might provide a basis for designing therapeutic interventions for hematological complications related to GHD.

Tessier type 3 oblique facial cleft with a contralateral complete cleft lip and palate.

Oblique facial clefts are extremely rare congenital deformities with a reported incidence of 0.24% of all facial clefts. This report presents a patient with a right-sided oblique cleft extending through the upper lip, the alar groove and the lower palpebra accompanied by a left-sided complete cleft lip and palate. Hypertylorism and bilateral microphthalmia as well as flexion wrist contractures were also present. Primary straight-line closure of the oblique cleft was undertaken followed by primary closure of the contralateral cleft lip. The treatment modality and 2 year follow-up results are presented.

The influence of 3,3',5-triiodo-L-thyronine on human haematopoiesis.

OBJECTIVES: Thyroid hormones mediate many physiological and developmental functions in humans. The role of the 3,3',5-triiodo-L-thyronine (T3) in normal human haematopoiesis at the cellular and molecular levels has not been determined. In this study, it was revealed that the human haematopoietic system might be directly depended on T3 influence. MATERIALS AND METHODS: We detected the TRalpha1 and TRbeta1 gene expression at the mRNA level in human cord blood, peripheral blood and bone marrow CD34(+)-enriched progenitor cells, using the RT-PCR method. Furthermore, we performed Western blotting to prove TRalpha1 and TRbeta1 expression occurs at the protein level in human cord blood, peripheral blood and bone marrow CD34(+) cells. In addition, the examined populations of cells were exposed in serum-free conditions to increasing doses of T3 and were subsequently investigated for clonogenic growth of granulocyte-macrophage colony-forming unit and erythrocyte burst-forming unit in methylcellulose cultures, and for the level of apoptosis, by employing annexin V staining and the terminal deoxynucleotidyltransferase-mediated dUTP nick-end labelling method. We investigated expression levels of apoptosis-related Bax and antiapoptotic Bcl-2 and Bcl-x(L) genes in the examined cells. RESULTS: We found that exposure to higher and lower than normal concentration of thyroid hormone significantly influenced clonogenecity and induced apoptosis in human haematopoietic progenitor cells. CONCLUSIONS: This study expands the understanding of the role of thyroid disorders in normal human haematopoiesis and indicates a direct influence of T3 on this process.

Prevalence and forms of congenital anomalies in twins born in Pomeranian District during the period from 1.07.1997 to 31.12.1998. Polish Register of Congenital Anomalies.

The authors have analysed the frequency and structure of congenital anomalies in children born in the Pomeranian district in the period from 01.07.1997 to 31.12.1998. Among a total of 28.361 births in that area, 748 (2.64%) were affected by congenital anomalies. Among 28.361 births, 620 (2.18%) were from multiple pregnancies. 23 (3.71%) among births from multiple pregnancies were affected by congenital malformations. The prevalence rate of inborn anomalies in births from multiple pregnancy in our area were higher (3.71%) in comparison to births from singleton pregnancy (2.61%). It implies that children born from multiple pregnancy are at higher risk of developing congenital anomalies.

[Diagnosis and management of acute haematogenous osteomyelitis in newborns and infants]. / Rozpoznanie i leczenie ostrego zapalenia kosci i stawów u noworodków i niemowlat.

Many newborns in Poland still suffer from acute hematogenous osteomyelitis. The paper discusses the principles of early diagnosis and treatment.

[Use of cancellous bone and periosteal transplants in primary and secondary cleft palate during a change in dentition]. / Zastosowanie przeszczepu kosci gabczastej i okostnej w rozszczepie podniebienia pierwotnego i wtórnego w okresie wymiany uzebienia.

A group of patients that was selected jointly with orthodontists consisted of 25 subjects, aged 8-16 years, having undergone operation for the cleft of primary and secondary palate, with left jaw defect. The patients had correct bites with good cure prognosis. Cancellous bone and periosteal flap taken from the wing-like portion of ilium were grafted into their jaw defects. After 1 year the alveolar process continuity was obtained in 23 patients (92%) and in the remaining ones the resorption of the graft was recorded. Reconstruction of the alveolar process was visible in radiographs, and eruption of canine tooth through the graft was evidenced in 40% of cases. No satisfactory results were obtained as regards the closure of naso-palatine fistula and the total underpadding of the alar base.