Bone health and vitamin D status in children with motor disability and adults with intellectual disability

Osteoporosis is not only a disease of the elderly, but is increasingly diagnosed in chronically ill children. Children with severe motor disabilities, such as cerebral palsy (CP), have many risk factors for osteoporosis. Adults with intellectual disability (ID) are also prone to low bone mineral density (BMD) and increased fractures. This study was carried out to identify risk factors for low BMD and osteoporosis in children with severe motor disability and in adults with ID. In this study 59 children with severe motor disability, ranging in age from 5 to 16 years were evaluated. Lumbar spine BMD was measured with dual-energy x-ray absorptiometry. BMD values were corrected for bone size by calculating bone mineral apparent density (BMAD), and for bone age. The values were transformed into Z-scores by comparison with normative data. Spinal radiographs were assessed for vertebral morphology. Blood samples were obtained for biochemical parameters. Parents were requested to keep a food diary for three days. The median daily energy and nutrient intakes were calculated. Fractures were common; 17% of the children had sustained peripheral fractures and 25% had compression fractures. BMD was low in children; the median spinal BMAD Z-score was -1.0 (range -5.0 – +2.0) and the BMAD Z-score <-2.0 in 20% of the children. Low BMAD Z-score and hypercalciuria were significant risk factors for fractures. In children with motor disability, calcium intakes were sufficient, while total energy and vitamin D intakes were not. In the vitamin D intervention studies, 44 children and adolescents with severe motor disability and 138 adults with ID were studied. After baseline blood samples, the children were divided into two groups; those in the treatment group received 1000 IU peroral vitamin D3 five days a week for 10 weeks, and subjects in the control group continued with their normal diet. Adults with ID were allocated to receive either 800 IU peroral vitamin D3 daily for six months or a single intramuscular injection of 150 000 IU D3. Blood samples were obtained at baseline and after treatment. Serum concentrations of 25-OH-vitamin D (S-25-OHD) were low in all subgroups before vitamin D intervention: in almost 60% of children and in 77% of adults the S-25-OHD concentration was below 50 nmol/L, indicating vitamin D insufficiency. After vitamin D intervention, 19% of children and 42% adults who received vitamin D perorally and 12% of adults who received vitamin D intramuscularly had optimal S-25-OHD (>80 nmol/L). This study demonstrated that low BMD and peripheral and spinal fractures are common in children with severe motor disabilities. Vitamin D status was suboptimal in the majority of children with motor disability and adults with ID. Vitamin D insufficiency can be corrected with vitamin D supplements; the peroral dose should be at least 800 IU per day.Väitöstutkimuksen mukaan 60 %:a liikuntavammaisista lapsista ja 77 %:a kehitysvammaisista hoitokodeissa asuvista aikuisista kärsi D-vitamiinivajeesta. Tutkimuksen mukaan veren D-vitamiinitasoa voidaan turvallisesti nostaa antamalla suun kautta vähintään 800 KY (20 µg) D3-vitamiinia päivässä. Riittävän D-vitamiinin saanti on tärkeää osteoporoosin ehkäisyn kannalta. Osteoporoosi on luun haurastumiseen johtava sairaus, josta seuraa luun murtuma-alttius. Osteoporoosi on yhä enenevässä määrin myös lasten sairaus. Liikuntavammaisilla lapsilla luita kuormittavan liikunnan vähäisyys tai puute on riskitekijä osteoporoosille. Osasyynä luiden haurastumiselle voidaan pitää myös liian vähäistä D-vitamiinin saantia. Osana tutkimusta annettiin liikuntavammaisille lapsille D3-vitamiinilisää suun kautta 1000 IU (25 µg) koulupäivinä 10 vk:n ajan. D-vitamiiniannos osoittautui turvalliseksi, eikä sivuvaikutuksia havaittu. On mahdollista, että näillä lapsilla päivittäin tarvittava D-vitamiini annos on vieläkin suurempi. Osa tutkimukseen osallistuneista lapsista ei saanut D-vitamiinilisää ollenkaan. Näiden lasten veren D-vitamiininpitoisuudet laskivat alkukesällä huomattavan alhaisiksi osoittaen, että näillä lapsilla auringon valon tuottama D-vitamiini ei ole riittävää, vaan he tarvitsevat suun kautta otettavaa D-vitamiinilisää. Luuntiheysmittausten ja röntgenkuvausten avulla tutkimuksessa todettiin, että viidesosalla vaikeasti liikuntavammaisista lapsista luun tiheys oli selkeästi alentunut ja osteoporoosi voitiin todeta 17 % tutkituista lapsista. Neljäsosalla lapsista todettiin aiemmin täysin huomiotta jääneitä selkärangan murtumia, jotka voidaan katsoa aiheutuneen luuston haurastumisesta. Kehitysvammaisilla laitosasukkailla D-vitamiinin puute oli myös yleistä. Kehitysvammaisten hoitokodissa tehdyssä tutkimuksessa verrattiin lihakseen annettavan suuren D3-vitamiiniannoksen (150 000 KY) ja suun kautta päivittäin annosteltavan (800 KY) D3-vitamiiniannoksen tehoa ja turvallisuutta. Molemmat antotavat osoittautuivat yhtä turvallisiksi, mutta suun kautta annostelu oli tehokkaampi. Luuston terveydestä huolehtiminen ja sen tutkiminen ovat näillä potilasryhmillä erityisen tärkeää osteoporoosista johtuvien murtumien ehkäisemiseksi. Tutkimustulosten perusteella vaikeasti liikuntavammaisille lapsille ja kehitysvammaisille hoitokotiasukkaille suositellaan ympärivuotista suun kautta otettavaa D3-vitamiinilisää ainakin 800–1000 KY (20–25 µg) vuorokaudessa.

Barbituraattianestesian käyttö lasten vaikean epilepsian hoidossa.

Syventävä työ ei kirjastoss

Barbituraattianestesian käyttö lasten vaikean epilepsian hoidossa.

Syventävä työ ei kirjastoss

Idiopathic generalised epilepsies with 3 Hz and faster spike wave discharges: A population-based study with evaluation and long-term follow-up in 71 patients

For several years we have been following patients with intractable, childhood-onset idiopathic generalised epilepsies with ≥ 3 Hz spike-wave discharges. Our need to find explanations for their intractability was the starting point for this study. We were interested in identifying characteristics, which would predict intractability; evaluating how these patients were treated and whether polytherapy was useful. We identified patients with ≥ 3 Hz spike-wave discharges by reviewing EEG reports recorded between 1983 and 1992. Data were collected from medical records and through personal interviews. We identified 82 patients with tentative idiopathic generalised epilepsy. Eleven were excluded. Thirty-eight patients had childhood absence epilepsy, 18 had juvenile absence epilepsy, 13 had juvenile myoclonic epilepsy and two had eyelid myoclonia with absences: 89.5, 78, 38 and 0% of the patients in each group, respectively, had been seizure free for more than 2 years. Twenty percent of the patients had intractable seizures. All intractable patients with juvenile absence epilepsy had rhythmic, random eyelid blinking and generalised tonic-clonic seizures. A history of more than ten generalised tonic-clonic seizures was associated with intractability in juvenile myoclonic patients. Monotherapy with ethosuximide or valproate resulted in seizure control in 65% of patients. Seventeen patients (24%) were treated with polytherapy, six achieved remission. These six patients had childhood absence epilepsy and juvenile absence epilepsy. Positive outcome was found in childhood absence epilepsy and juvenile absence epilepsy. Intractable seizures were more frequent among patients with juvenile myoclonic epilepsy. None of them benefited from polytherapy with conventional anti-epileptic drugs.SCOPUS: ar.jinfo:eu-repo/semantics/publishe