| 1. |
- Fadeel, Bengt, et al.
(författare)
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Kostmann disease and other forms of severe congenital neutropenia
- 2021
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Ingår i: Acta Paediatrica. - : John Wiley & Sons. - 0803-5253 .- 1651-2227. ; 110:11, s. 2912-2920
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Tidskriftsartikel (refereegranskat)abstract
- Congenital neutropenia with autosomal recessive inheritance was first described by the Swedish paediatrician Rolf Kostmann who coined the term ‘infantile genetic agranulocytosis’. The condition is now commonly referred to as Kostmann disease. These patients display a maturation arrest of the myelopoiesis in the bone marrow and reduced neutrophil numbers and suffer from recurrent, often life-threatening infections. The molecular mechanism underlying congenital neutropenia has been intensively investigated, and mutations in genes that impinge on programmed cell death have been identified. The present review provides an overview of these studies.
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| 2. |
- Flemme, Inger, et al.
(författare)
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Life situation of patients with an implantable cardioverter defibrillator : a descriptive longitudinal study
- 2001
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Ingår i: Journal of Clinical Nursing. - : Wiley. - 0962-1067 .- 1365-2702. ; 10:4, s. 563-572
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Tidskriftsartikel (refereegranskat)abstract
- • The aim of this study was to describe changes in the life situation of patients with an implantable cardioverter defibrillator over a period of 1 year. A sample of 56 consecutive patients took part in the study.• Life situation was measured through uncertainty in illness, satisfaction, and fear of the life situation. Descriptive statistics were used to present results, and analytical statistics were used to map out changes over time.• Overall uncertainty showed a decrease over time. A statistically significant difference was found within the domain uncertainty related to information (P < 0.001).• Satisfaction increased within the domains health-functioning, socio-economic, psychological–spiritual, and family.• The ability to act within the domain health-functioning showed a statistical significance (P < 0.05).• The domain life changes within fear in the life situation decreased and showed a statistical significance (P < 0.05).• The overall life situation showed increased satisfaction as well as lower uncertainty and fear in the life situation.• The research indicates that patients need more information about changes in the life situation after the implantable cardioverter defibrillator-implantation. The study encourages more humanistic, holistic research about patients’ life situations as well as more education in teaching skills for health care personnel.
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| 3. |
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| 4. |
- Jamil, Seema, et al.
(författare)
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Tropism of the in situ growth from biopsies of childhood neuroectodermal tumors following transplantation into experimental teratoma
- 2014
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Ingår i: International Journal of Cancer. - : Wiley. - 0020-7136 .- 1097-0215. ; 134:7, s. 1630-1637
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Tidskriftsartikel (refereegranskat)abstract
- Experimental teratoma induced from human pluripotent stem cells with normal karyotype can be described as a failed embryonic process and includes besides advanced organoid development also large elements of tissue with a prolonged occurrence of immature neural components. Such immature components, although benign, exhibit strong morphological resemblance with tumors of embryonic neuroectodermal origin. Here, we demonstrate that biopsy material from childhood tumors of neural embryonic origin transplanted to mature experimental teratoma can show an exclusive preference for matching tissue. Tumor specimens from five children with; Supratentorial primitive neuroectodermal tumor (sPNET); Pilocytic astrocytoma of the brainstem; Classic medulloblastoma; peripheral primitive neuroectodermal tumor (pPNET) or neuroblastoma (NB), respectively, were transplanted. Analysis of up to 120 sections of each tumor revealed an engraftment for three of the transplanted tumors: pPNET, sPNET, and NB, with a protruding growth from the latter two that were selected for detailed examination. The histology revealed a strict tropism with a non-random integration into what morphologically appeared as matched embryonic microenvironment recuperating the patient tumor histology. The findings suggest specific advantages over xenotransplantation and lead us to propose that transplantation to the human embryonic microenvironment in experimental teratoma can be a well-needed complement for preclinical in vivo studies of childhood neuroectodermal tumors. What's new? The ability to better replicate the human neoplastic niche in vivo could help improve the predictive reliability of animal models. To that end, this study shows that biopsies from childhood neuroectodermal tumors are able to engraft into specific embryonic components of human experimental teratoma. Histological examination revealed a strict tropism of a neuroblastoma as well as a supratentorial primitive neuroectodermal tumor, showing nonrandom integration into morphologically identifiable tissues. The study opens new possibilities for the analysis of growth-promoting environmental factors and for investigating novel therapies targeted to the microenvironment of childhood neuroectodermal tumors.
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| 5. |
- Bylund, Bengt, et al.
(författare)
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Very low birth weight children at 9 years : School performance and behaviour in relation to risk factors
- 2000
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Ingår i: Prenatal and Neonatal Medicine. - 1359-8635 .- 1473-0774. ; 5:2, s. 124-133
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Tidskriftsartikel (refereegranskat)abstract
- Objectives To investigate the school performance and behavior of very low-birth-weight children in comparison with controls, and relate the findings to risk factors. Methods All children with birth weight below 1501 g (VLBW) and normal birth weight (NBW) controls, born in the south-east region of Sweden during a 15-month period in 1987-88, were enrolled in a prospective follow-up study; 81 % (n = 70) and 82% (n = 72), respectively, were re-examined at the age of 9 years regarding growth, neurological status, neurofunctional classification and academic achievement tests (Raven's matrices, mathematics, vocabulary, reading ability). The need for special education at school was assessed and four behavioral problem scores were also assessed (hyperactivity, fine motor skills, gross motor skills, social skills). Children with known handicaps were not re-examined. Results VLBW children were shorter and lighter than controls, and differed from them with regard to neurological functional classification. As a group, they produced poorer results in all academic achievement tests except vocabulary, and also in two out of four behavioral subscales (hyperactivity and fine motor skills). When the comparison was restricted to children with normal Raven scores, almost all the differences disappeared. VLBW children had more reading difficulties but were less often than expected defined as dyslexic compared to NEW children.
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| 6. |
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| 9. |
- Gertow, Karin, et al.
(författare)
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Early Events in Xenograft Development from the Human Embryonic Stem Cell Line HS181-Resemblance with an Initial Multiple Epiblast Formation
- 2011
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Ingår i: PLOS ONE. - : Public Library of Science (PLoS). - 1932-6203. ; 6:11, s. e27741-
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Tidskriftsartikel (refereegranskat)abstract
- Xenografting is widely used for assessing in vivo pluripotency of human stem cell populations. Here, we report on early to late events in the development of mature experimental teratoma from a well-characterized human embryonic stem cell (HESC) line, HS181. The results show an embryonic process, increasingly chaotic. Active proliferation of the stem cell derived cellular progeny was detected already at day 5, and characterized by the appearance of multiple sites of engraftment, with structures of single or pseudostratified columnar epithelium surrounding small cavities. The striking histological resemblance to developing embryonic ectoderm, and the formation of epiblast-like structures was supported by the expression of the markers OCT4, NANOG, SSEA-4 and KLF4, but a lack of REX1. The early neural marker NESTIN was uniformly expressed, while markers linked to gastrulation, such as BMP-4, NODAL or BRACHYURY were not detected. Thus, observations on day 5 indicated differentiation comparable to the most early transient cell populations in human post implantation development. Confirming and expanding on previous findings from HS181 xenografts, these early events were followed by an increasingly chaotic development, incorporated in the formation of a benign teratoma with complex embryonic components. In the mature HS181 teratomas not all types of organs/tissues were detected, indicating a restricted differentiation, and a lack of adequate spatial developmental cues during the further teratoma formation. Uniquely, a kinetic alignment of rare complex structures was made to human embryos at diagnosed gestation stages, showing minor kinetic deviations between HS181 teratoma and the human counterpart.
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| 10. |
- Gottfridsson, Christer, 1958, et al.
(författare)
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Spectral turbulence and late potentials in the signal-averaged electrocardiograms of patients with monomorphic ventricular tachycardia versus resuscitated ventricular fibrillation.
- 2000
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Ingår i: Scandinavian cardiovascular journal : SCJ. - 1401-7431. ; 34:3, s. 261-71
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Tidskriftsartikel (refereegranskat)abstract
- Signal-averaged electrocardiograms (SAECG) were analyzed for late potentials and spectral turbulence in 208 patients with ischemic heart disease with a history of sustained monomorphic ventricular tachycardia (MVT) (n = 62), resuscitation from ventricular fibrillation (VF) (n = 64) or no ventricular tachyarrhythmia (n = 82). Receiver operating characteristic curves were utilized to optimize cut-off values for prediction of MVT and VF. Patients with MVT had a lower ejection fraction (mean = 0.37) than patients with VF (0.44; p = 0.01) and controls (0.48; p < 0.0001). The mean FQRSD in MVT patients (126 ms) was longer than in VF and controls (113 ms; p = 0.005 and 102 ms; p < 0.0001, respectively). The RMS40 was lower in MVT (19 microV) than in VF and controls (29 microV; p = 0.0003 and 28 microV; p < 0.0001, respectively); 81% of the MVT patients were spectral turbulence-positive vs 47% of VF patients and 31% of control patients (p < 0.0001 for both differences). With optimized reference values, FQRSD, TQRSD and ISCSD contributed significantly to the identification of MVT patients and FQRSD to VF patients. The sensitivity of combined time-domain and spectral turbulence analysis was 90% for MVT and 58% for VF, with 63% specificity. MVT patients had a lower ejection fraction and were more often late potential and spectral turbulence positive than VF and control patients. These findings indicate that a large electroanatomic substrate is required in MVT. A long FQRSD was a risk marker for both MVT and VF. Spectral turbulence analysis added independent information, and the combination of time-domain and spectral turbulence analysis was superior to either method alone in identifying the MVT patients. Neither method of analysis, singly nor in combination, performed satisfactorily in identification of VF risk.
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