Povezanost razširjene funkcijske lestvice Hammersmith z jakostjo prijemov in 6-minutnim testom hoje : diplomsko delo

Pukšič, Pija (avtor), Leonardis, Lea (mentor)

Uvod: Spinalna mišična atrofija (SMA) je najpogostejša bolezen spodnjega motoričnega nevrona, ki se pojavi pri 1/6-10,000 rojstev. Kaže se kot mišična atrofija in šibkost. SMA je avtosomna recesivna bolezen, do katere najpogosteje prihaja zaradi izgube 7. in 8. eksona gena Survival Motor Neuron 1 (SMN1). Obstaja pet podtipov s SMN1 povezane SMA, pri katerih se tip 0 razvije pred rojstvom, tipi 1, 2 in 3 se kažejo v otroštvu, tip 4 pa v odrasli dobi. Namen: Z diplomsko nalogo smo želeli ugotoviti, ali je motorična funkcija odraslih bolnikov SMA ocenjena z razširjeno Hammersmithsko lestvico motoričnih funkcij (ang. Hammersmith functional motor scale expanded, HFMSE) povezana z mišično jakostjo stiska in prijemom ključa ter s šest-minutnim testom hoje. Metode: V raziskavi je sodelovalo 31 odraslih bolnikov z genetsko potrjeno diagnozo SMA, ki jih vodijo na Kliničnem inštitutu za klinično nevrofiziologijo, UKC Ljubljana Testirali smo jih z lestvico HFMSE, jakostjo prijema roke in prijema ključa ter s šest-minutnim testom hoje. Za statistično obdelavo podatkov smo izračunali korelacijo po Spearmanu. Rezultati: Večje število točk na lestvici HFMSE in večja jakost prijema desne ter leve roke sta bila statistično pomembno povezana (desna roka r = 0,85, p < 0,0001; leva roka r = 0,82, p < 0,0001), statistično pomembno povezana sta bila tudi večje število točk na lestvici HFMSE in večja jakost prijema ključa desne in leve roke (desna roka r = 0,78, p < 0,0001; leva roka r = 0,89, p < 0,0001). Pozitivna korelacija obstaja tudi med lestvico HFMSE in šest- minutnim testom hoje (r = 0,80, p < 0,0001). Razprava in zaključek: Izsledki naše raziskave kažejo, da je motorična funkcija odraslih bolnikov s SMA ocenjena z lestvico HFMSE povezana z mišično silo prijema roke in prijema ključa med palcem in kazalcem ter šest-minutnim testom hoje. Dobljeni rezultati bodo lahko v pomoč raziskovalcem, ki bodo te bolnike testirali v prihodnje in tako spremljali napredovanje bolezni ali učinek zdravil, ter so pokazatelj trenutnega stanja motorike posameznega bolnika. Za nadaljnje raziskave predlagamo uporabo natančnejših (digitalnih) dinamometrov ter da se bolnike pred začetkom testiranja loči glede na tip SMA bolezni.

• Jezik: Slovenski jezik
• Ključne besede: dinamometrija prijemov roke, razširjena Hammersmithska lestvica motoričnih funkcij, spinalna mišična atrofija, šest-minutni test hoje
• Vrsta gradiva: Diplomsko delo/naloga
• Organizacija: ZF - Zdravstvena fakulteta
• Leto izida: 2019
• PID: 20.500.12556/RUL-109325
• COBISS.SI-ID: 5672811
• Datum objave v RUL: 30.08.2019

Sekundarni jezik

• Jezik: Angleški jezik
• Naslov: The correlation of Hammersmith Functional Motor Scale Expanded with dynamometry tests and 6-minute walk test : diploma work

Izvleček

Introduction: The Spinal muscular atrophy (SMA) is the most common disease of the lower motor neuron, which appears in the 1/6-10,000 births. It manifests as muscle atrophy and weakness. SMA is an autosomal recessive disease that is most commonly caused by a deletion of 7th and 8th exons of the gene Survival Motor Neuron 1 (SMN1). There are five subtypes of the SMN1 related SMA: type 0 develops before birth, types 1, 2, and 3 from a young age and the type 4 in adulthood. Purpose: The purpose of this diploma work was to assess whether the motor function in adult SMA patients, measured by the Hammersmith functional motor scale expanded (HFMSE) is associated with the muscular strength of the hand grip, the key grip and the 6-minute walk test. Methods: The study sample was comprised of 31 adult patients with a genetically confirmed diagnosis of SMA, who are followed in the Institute of clinical neurophysiology, UKC Ljubljana. They were tested with the HFMSE, hand held dynamometry test and the 6-minute walk test. For the statistical analysis of the data, we used Spearman correlation test. Results: There is statistically significant correlation between higher score on the HFMSE and the greater hand grip strength in the right and the left hand (right hand r = 0,85, p < 0,0001; left hand r = 0,82, p < 0,0001). Furthermore, there is also statistically significant correlation between higher score on the HFMSE and the greater key grip strength in the right-hand and left hand (right hand r = 0,78, p < 0,0001; left hand r = 0,89, p < 0,0001). There is also a positive correlation between the HFMSE, and the 6-minute walk test (r = 0,80; p < 0,0001). Discussion and conclusion: Results of our study show that motor function of adult SMA patients, measured by the HFMSE correlates with the muscular strength of the hand grip, the key grip between the thumb and the index finger, and the 6-minute walk test. The obtained results will be helpful to the researchers who will be monitoring the patients and the progression of the disease or the effectiveness of drugs, while being indicators of the current motor state of the patient. For future research, we suggest using more precise (digital) dynamometers tests and the separation of the SMA patients, based on the SMA type.

• Ključne besede: dynamometry, Hammersmith Functional Motor Scale Expanded, spinal muscular atrophy, six-minute walk test