Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study

<p>Abstract</p> <p>Background</p> <p>Progressive encephalopathy (PE) in children is a heterogeneous group of diseases mainly composed of metabolic diseases, but it consists also of neurodegenerative disorders where neither metabolic nor other causes are found. We wanted to estimate the incidence rate and aetiology of PE, as well as the age of onset of the disease.</p> <p>Methods</p> <p>We included PE cases born between 1985 and 2003, living in Oslo, and registered the number presenting annually between 1985 and 2004. Person-years at risk between 0 and 15 years were based on the number of live births during the observation period which was divided into four 5-year intervals. We calculated incidence rates according to age at onset which was classified as neonatal (0–4 weeks), infantile (1–12 months), late infantile (1–5 years), and juvenile (6–12 years).</p> <p>Results</p> <p>We found 84 PE cases representing 28 diagnoses among 1,305,997 person years, giving an incidence rate of 6.43 per 100,000 person years. The age-specific incidence rates per 100,000 were: 79.89 (<1 year), 8.64 (1–2 years), 1.90 (2–5 years), and 0.65 (>5 years). 66% (55/84) of the cases were metabolic, 32% (27/54) were neurodegenerative, and 2% (2/84) had HIV encephalopathy. 71% (60/84) of the cases presented at < 1 year, 24% (20/84) were late infantile presentations, and 5% (4/84) were juvenile presentations. Neonatal onset was more common in the metabolic (46%) (25/55) compared to the neurodegenerative group (7%) (2/27). 20% (17/84) of all cases were classified as unspecified neurodegenerative disease.</p> <p>Conclusion</p> <p>The overall incidence rate of PE was 6.43 per 100,000 person years. There was a strong reduction in incidence rates with increasing age. Two-thirds of the cases were metabolic, of which almost half presented in the neonatal period.</p

STANDARD ENTHALPIES OF FORMATION OF TERBIUM ALLOYS, TB+ME (ME-NI, RU, RH, PD, IR, PT), BY HIGH-TEMPERATURE DIRECT SYNTHESIS CALORIMETRY

The standard enthalpies of formation of 12 terbium alloys with late transition metals have been determined by direct synthesis calorimetry at 1474+/-2 K. The following values of Delta H-f(0) (kJ g(-1) atom(-1)) are reported: TbNi5, -(27.4+/-0.9); TbRu2, -(23.6+/-1.7); Tb5Ru2, -(29.9+/-1.9); TbRh, -(72.3+/-1.1); TbRh2, -(64.4+/-1.5); TbPd, -(85.2+/-1.6); Tb3Pd4, -(85.5+/-1.4); TbPd3, -(78.8+/-1.5); TbIr2, -(70.6+/-2.6 ); TbPt, -(115.7+/-2.9); TbPt3, -(96.7+/-3.1); TbPt3, -(85.6+/-2.9 ). The results are compared with predicted values from the Miedema model and with available literature data for TbPd and TbPt

Standard enthalpies of formation of some thulium alloys, Tm+Me (Me=Ni, Rh, Pd, Pt), by high temperature direct synthesis calorimetry

The standard enthalpies of formation of nine thulium alloys with late transition metals have been determined by direct synthesis calorimetry at 1400+/-2 K. The following values of Delta H-f degrees (kJ(mol atom)(-1)) are reported: TmNi5, -29.1+/-0.7; TmRh, -88.9+/-2.0; TmRh2, -73.0+/-2.0; -92.6+/-2.0; Tm3Pd4, -93.0+/-1.7, TmPd3, -86.8+/-1.6, TmPt, -121.0+/-2.0; TmPt2, -112.4+/-3.1; TmPt3, -91.1+/-2.1. The results are compared with predicted values from the Miedema model and with available literature data for TmPd and TmPt

STANDARD ENTHALPIES OF FORMATION OF NI3TA, PD3TA, PT3NB, PT2V AND PT3V BY HIGH-TEMPERATURE DIRECT SYNTHESIS CALORIMETRY

The standard enthalpies of formation of Ni3Ta, Pd3Ta, Pt3Nb, Pt2V and Pt3V have been determined by direct synthesis calorimetry at 1473 +/- 2 K. The following values of DELTAH-f(0) (kJ g-1 atom-1) are reported: Ni3Ta, - (20.4 +/- 2.3); Pd3Ta, -(36.1 +/- 2.0); Pt3Nb, -(46.6 +/- 3.7); Pt2V, -(37.2 +/- 2.5); Pt3V, -(27.4 +/- 3.0). The results are compared with data available in the literature for Ni3Ta and Pt3V and with predicted values from the model of Miedema

Enthalpies of formation from the component oxides of MgWO4, CaWO4 (scheelite), SrWO4, and BaWO4, determined by high-temperature direct synthesis calorimetry

The enthalpies of formation, from the constituent oxides, of tungstates of alkaline earth metals, MeWO(4)(Me is Mg, Ca, Sr, Ba), have been determined by direct synthesis calorimetry at 1473 +/- 2 K. The following values of Delta H-f at 298 K (kJ mol(-1)) are reported: MgWO4, -73.9 +/- 4.8; CaWO4 (scheelite), -168.5 +/- 3.3; SrWO4, -2179 +/- 3.6; BaWO4, -256.2 +/- 3.9. The results are compared with data available in the literature

STANDARD ENTHALPIES OF FORMATION OF GADOLINIUM ALLOYS, GD+ME (ME-NI, RU, RH, PD, IR, PT), BY HIGH-TEMPERATURE DIRECT SYNTHESIS CALORIMETRY

The standard enthalpies of formation of eleven gadolinium alloys have been determined by direct synthesis calorimetry at 1477+/-2 K. The following values of Delta H-f(0) (kJ/g . atom) are reported: GdNi, -(30.3+/-2.1); GdNi5, -(27.5+/-1.2); GdRu2, -(21.7+/-1.9); GdRh, -(72.4+/-2.1); GdRh2, -(65.4+/-1.6 ); GdPd, -(82.6+/-1.1 ); Gd3Pd4, -(88.5+/-2.9); GdPd3, -(79.7+/-2.7); Gdlr(2), -(68.5+/-2.2); GdPt, -(109.3+/-2.8 ); GdPt2, -(100.0+/-2.6). The results are compared with the predicted values from the Miedema model and with available literature data for GdNi, GdNi5, GdPd, Gd3Pd4, GdPd3, GdPt and GdPt2

Standard enthalpies of formation of yttrium alloys, Y-Me (Me = Ru, Rh, Pd, Os, Ir, Pt), by high-temperature calorimetry

The standard enthalpies of formation of yttrium alloys, Y Me (Me = Ru, Rh, Pd, Os, Ir, Pt) were determined by direct synthesis calorimetry at 1473 ± 2 K. The following values of [math] are reported (J/mole of atoms) : Ru2Y5, - (27.3 ± 3.1), RhY, - (76.1 ± 3.4) ; Rh2Y, - (65.4 ± 1.3) ; PdY, - (94.9 ± 3.8) ; Pd4Y3, - (92.8 ± 3.7) ; Pd3Y, - (79.0 ± 6.5) ; Os2Y, - (24.8 ± 2.8) ;IrY, - (65.9 ± 3.8) ; Ir2Y, - (59.4 ± 2.8) ; PtY, - (104 ± 2.3) ; Pt3Y, - (86.9 ± 2.0). The results are compared with the predictions of the models, and with earlier data for some of the corresponding zirconium alloys

Standard enthalpies of formation of some holmium alloys, Ho+Me (Me=Ni, Ru, Rh, Pd, Ir, Pt), determined by high temperature direct synthesis calorimetry

The standard enthalpies of formation of 13 holmium alloys with late transition metals have been determined by direct synthesis calorimetry at 1474 +/- 2 K. The following values of Delta H-f(0) (kJ (mol atoms)(-1)) are reported: HoNi5, -(29.9 +/- 0.8); HoRu2, -(26.8 +/- 1.3); Ho3Rh2, -(73.4 +/- 2.0); HoRh, -(87.2 +/- 2.3); HoRh2, -(70.4 +/- 1.5); HoPd, -(91.5 +/- 2.2); Ho3Pd4, -(94.2 +/- 2.1); HoPd3, -(87.4 +/- 2.3); HoIr, - (80.7 +/- 2.3); HoIr,, - (74.4 +/- 2.0); HoPt, -(121.8 +/- 5.1); HoPt2, -(106.6 +/- 4.8); HoPt3, -(95.3 +/- 2.3). The results are compared with predicted values from the Miedema model and with available literature data for HoPd and HoPt