Stanje prehranjenosti otrok s spinalno mišično atrofijo

Dolšak, Doroteja (author), Homan, Matjaž (mentor), Benedik, Evgen (comentor)

Spinalna mišična atrofija (SMA) je redka živčno-mišična bolezen (ŽMB), pri kateri zaradi degeneracije motoričnih nevronov prihaja do napredujoče mišične oslabelosti ske-letnih in dihalnih mišic. Bolezen vpliva na spremenjeno telesno sestavo in prehransko stanje bolnikov. Namen raziskave je bil opredeliti prehransko stanje otrok s SMA, ki se zdravijo na Pediatrični kliniki Univerzitetnega kliničnega centra Ljubljana. Etičnost raziskave je odobrila Komisija za medicinsko etiko (številka 0120-160/2016-2). Prehranjenost otrok smo ocenili na podlagi retrospektivno zbranih podatkov o meritvah tele-sne mase (TM), telesne višine (TV), indeksa telesne mase (ITM) ter meritvah z dvoe-nergijsko rentgensko absorpciometrijo (DXA), ki so bile opravljene do maja 2023. Ocenili smo tudi prehranski vnos z validiranim vprašalnikom o pogostosti uživanja živil. V raziskavo je bilo vključenih 21 otrok s SMA (tip I: n = 2, tip II: n = 13, tip III: n = 8, tip IV: n = 0), od tega 10 deklic in 11 dečkov. V času raziskave je bilo 19 otrok zdravljenih z zdravilom nusinersen. Meritve TM, TV in ITM smo primerjali v dveh časovnih točkah v obdobju od 0,5 do 2 let ter spremembe primerjali s spremembami kontrolne skupine. Delež otrok s prenizko TM se je s časom povečal (s 35 na 55 %). Tudi pri primerjavi sprememb telesne mase za starost in spol (z-TM), telesne višine za starost in spol (z-TV) in indeksa telesne mase za starost in spol (z-ITM) skozi čas sta se vrednosti me-diane z-TM in z-TV zmanjšali. V primerjavi s kontrolno skupino so bile razlike stati-stično značilne pri obeh meritvah za z-TM (prva meritev p = 0,003, druga meritev p < 0,01), z-TV (prva meritev p = 0,01, druga meritev p = 0,003) in z-ITM (prva meri-tev p = 0,05, druga meritev p = 0,008). Analiza telesne sestave je pokazala, da imajo otroci s SMA večji delež maščobne mase (p < 0,001) in manjši delež nemaščobne mase (p = 0,026) v primerjavi s kontrolno skupino. Iz analize prehranskega vnosa smo ugotovili, da otroci s SMA pogosto uživajo živila, ki so dober vir beljakovin (meso, ribe, stročnice, mesni izdelki), z izjemo mleka in mlečnih izdelkov. Prav tako večkrat dnevno uživajo sadje (frekvenca = 1,34) in zelenjavo (frekvenca = 2,41). Manj kot polovica (39 %) otrok redno dopolnjuje prehrano z oralnimi prehranskimi dodatki, vsi pa uživajo vitamin D (100 %) in skoraj vsi (83 %) kalcijev karbonat. Z raziskavo smo potrdili, da je delež neustrezno prehranjenih relativno visok, kar kaže na pomembnost vključitve dietetika pri obravnavi bolnikov s SMA.

• Language: Slovenian
• Keywords: prehrana, živčno-mišične bolezni, telesna sestava, telesna masa, telesna višina, indeks telesne mase, dvoenergijska rentgenska absorpciometrija
• Work type: Master's thesis/paper
• Typology: 2.09 - Master's Thesis
• Organization: BF - Biotechnical Faculty
• Publisher: [D. Dolšak]
• Year: 2024
• PID: 20.500.12556/RUL-161212-00dd945f-a187-0ff9-a7cd-537d9e98d753
• UDC: 613.2-053.2:616.8
• COBISS.SI-ID: 206749955
• Publication date in RUL: 08.09.2024

Secondary language

• Language: English
• Title: Nutritional status of children with spinal muscular atrophy

Abstract

Spinal muscular atrophy (SMA) is a rare neuromuscular disease characterized by the degeneration of motor neurons, leading to progressive weakness of skeletal and respi-ratory muscles. The disease affects the body composition and nutritional status of pati-ents. The aim of the study was to assess the nutritional status of children with SMA treated at the Pediatric Clinic of the University Medical Center Ljubljana. Nutritional status was evaluated based on retrospectively collected data on body weight (BW), he-ight (H), body mass index (BMI), and dual-energy X-ray absorptiometry (DXA) mea-surements conducted up to May 2023. Dietary intake was also assessed using a valida-ted food frequency questionnaire. The study included 21 children with SMA (type I: n = 2, type II: n = 13, type III: n = 8, type IV: n = 0), comprising 10 girls and 11 boys. During the study period, 19 children were treated with the drug nusinersen. Mea-surements of BW, H, and BMI were compared at two time points over a period from 0.5 to 2 years, and changes were compared with control group. The proportion of children with low BW increased over time (from 35 to 55 %). Also, when comparing changes in z- score weight for age (WAZ), z-score height for age (HAZ) and z-score body mass index (BMIZ) over time, the values WAZ and HAZ was decreased. Stati-stically significant differences was observed in both measurements for WAZ (first me-asurement p = 0,003, second measurement p < 0,001), HAZ (first measurement p = 0,01, second measurement p = 0,003), and BMIZ (first measurement p = 0,05, second measurement p = 0,008) compared to the control group. Body composition analysis re-vealed that children with SMA had a higher fat mass percentage (p < 0,001) and a lower lean mass (p = 0,026) compared to the control group. From the dietary intake analysis, it was found that children with SMA frequently consumed foods that are go-od sources of protein (meat, fish, legumes, meat products), except for milk and dairy products. They also consumed fruit (frequency = 1,34) and vegetables (frequency = 2,41) multiple times daily. Less than half (39 %) of the children regularly supplemented their diet with oral nutritional supplements, but all consumed vitamin D (100 %) and nearly all (83 %) consumed cal-cium carbonate. The study confirmed that a relatively high proportion of the children were inadequately nourished, highlighting the importance of including a dietitian in the treatment of patients with SMA.

• Keywords: nutrition, neuromuscular disorders, body composition, body weight, body height, body mass index, dual-energy X-ray absorptiometry