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...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2007-06-01
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| Series: | BMC Pediatrics |
| Online Access: | http://www.biomedcentral.com/1471-2431/7/25 |
| _version_ | 1818141501968875520 |
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| author | Diderichsen Jorgen Rootwelt Terje Woldseth Berit Abdelnoor Michael Kanavin Oivind Stromme Petter Bjurulf Bjorn Sommer Finn Magnus Per |
| author_facet | Diderichsen Jorgen Rootwelt Terje Woldseth Berit Abdelnoor Michael Kanavin Oivind Stromme Petter Bjurulf Bjorn Sommer Finn Magnus Per |
| author_sort | Diderichsen Jorgen |
| collection | DOAJ |
| description | <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> |
| first_indexed | 2024-12-11T11:00:54Z |
| format | Article |
| id | doaj.art-b289c9a820d042dcb9f735f717b43c50 |
| institution | Directory Open Access Journal |
| issn | 1471-2431 |
| language | English |
| last_indexed | 2024-12-11T11:00:54Z |
| publishDate | 2007-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pediatrics |
| spelling | doaj.art-b289c9a820d042dcb9f735f717b43c502022-12-22T01:09:53ZengBMCBMC Pediatrics1471-24312007-06-01712510.1186/1471-2431-7-25Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based studyDiderichsen JorgenRootwelt TerjeWoldseth BeritAbdelnoor MichaelKanavin OivindStromme PetterBjurulf BjornSommer FinnMagnus Per<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>http://www.biomedcentral.com/1471-2431/7/25 |
| spellingShingle | Diderichsen Jorgen Rootwelt Terje Woldseth Berit Abdelnoor Michael Kanavin Oivind Stromme Petter Bjurulf Bjorn Sommer Finn Magnus Per Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study BMC Pediatrics |
| title | Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study |
| title_full | Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study |
| title_fullStr | Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study |
| title_full_unstemmed | Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study |
| title_short | Incidence rates of progressive childhood encephalopathy in Oslo, Norway: a population based study |
| title_sort | incidence rates of progressive childhood encephalopathy in oslo norway a population based study |
| url | http://www.biomedcentral.com/1471-2431/7/25 |
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