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Retrospective study links SMA type 1 to higher hip instability prevalence in childrenHip Instability in SMA: What New UAE Data Reveals

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Key Takeaway
Consider screening for hip instability in type 1 SMA patients based on this observational data.

This retrospective cross-sectional study included 54 children with a genetic diagnosis of spinal muscular atrophy. The setting was a single tertiary care center in the United Arab Emirates. Researchers examined associations between SMA type, motor status, SMN2 copies, and disease modifying therapies. Comparator groups included SMA types (1-3) and motor status groups.

Hip dislocation or subluxation was most prevalent in type 1 SMA patients, with p = 0.046. Patients with fewer SMN2 copies were more likely to have scoliosis, showing p = 0.023. However, no significant correlation existed between hip subluxation or dislocation and scoliosis. Absolute numbers for scoliosis were not reported.

Associations of hip abnormalities with gender, age, motor status, and scoliosis showed no statistically significant association. Adverse events, serious adverse events, and discontinuations were not reported. Tolerability information was not reported. No significant correlation was found between hip instability and scoliosis.

The retrospective cross-sectional design limits causal inference, noting that associations are reported rather than causation. Follow-up duration was not reported. Findings can aid the development of hip instability screening programs in SMA patients, enabling early intervention. Certainty is limited by the retrospective cross-sectional design.

A hidden challenge for families

Imagine a child with spinal muscular atrophy (SMA). Their muscles are weak, making it hard to sit, walk, or even breathe well. Now, add another problem: their hip joint is slipping out of place. This pain and instability can make daily care—like changing clothes or using a wheelchair—even harder.

This is the reality for many families. A new study from the United Arab Emirates is shedding light on how common hip problems are in children with SMA and what might make it worse.

SMA is a genetic disease that weakens muscles. It affects about 1 in 10,000 babies worldwide. There are different types, from Type 1 (most severe) to Type 3 (milder). Thanks to new medicines, many children are living longer and healthier lives.

But as they grow, new challenges appear. Hip instability—where the hip joint slips out of place—is one of them. It can cause pain, limit movement, and make daily tasks tough.

Right now, doctors don’t always know which children are at highest risk. This study tries to answer that.

The old way vs. the new way

Doctors have known that SMA can affect the hips. But most research comes from the United States or Europe. There’s less data from other parts of the world, like the Middle East.

This study is different. It looks at children in the UAE, where SMA care is evolving. It also checks how hip problems link to scoliosis (curved spine)—a common issue in SMA.

But here’s the twist: the study found that hip problems and scoliosis don’t always go together. That’s surprising, because doctors often watch for both.

How hip instability happens

Think of the hip joint like a ball and socket. The ball (top of the thigh bone) fits into the socket (part of the pelvis). Strong muscles hold it in place.

In SMA, weak muscles can’t keep the ball centered. Over time, the ball may slip partially (subluxation) or fully (dislocation). It’s like a loose lid on a jar—it doesn’t stay put.

This study suggests that children with the weakest muscles—those with Type 1 SMA—are most at risk. Their hips are more likely to slip out of place.

Researchers reviewed records of 54 children with SMA treated at a UAE hospital between 2018 and 2023. All had genetic confirmation of SMA and hip X-rays.

They grouped kids by SMA type and motor status: non-sitters, sitters, or walkers. They also noted scoliosis, SMN2 gene copies (a key factor in SMA severity), and whether kids got disease-modifying therapies (DMTs).

Hip dislocation or subluxation was most common in Type 1 SMA patients. This was statistically significant, meaning it’s unlikely due to chance.

But here’s what didn’t link up: hip problems and scoliosis. The study found no clear connection between the two. That’s important because it means doctors can’t assume one predicts the other.

Another finding: kids with fewer SMN2 copies were more likely to have scoliosis. SMN2 is a backup gene that makes some SMA protein. Fewer copies mean less protein, so more severe disease.

Gender, age, motor status, and scoliosis didn’t predict hip problems. That’s surprising—doctors might have expected walkers to have fewer hip issues, but that wasn’t clear here.

This is where things get interesting

The study also found that several factors—like age or motor status—didn’t strongly predict hip instability. That means doctors need better tools to spot kids at risk early.

This is the first study in the Middle East to look at hip instability and scoliosis in SMA kids. It fills a gap in global data. Experts say it could help build screening programs in the region, catching problems before they get worse.

If your child has SMA, talk to your doctor about hip checks. X-rays might be needed, especially for Type 1 SMA. But this study is just a starting point—it’s not a guide for all SMA care yet.

This doesn’t mean this treatment is available yet.

The study is small—only 54 kids—and from one hospital. It’s also retrospective, meaning it looked back at old records. That can miss details. Plus, it’s from one country, so results might not apply everywhere.

Researchers hope to study more children over time. They want to see if early hip screening changes outcomes. For now, this study adds a piece to the puzzle, helping doctors worldwide care for SMA kids better.

Study Details

Study typeCohort
EvidenceLevel 3
PublishedApr 2026
View Original Abstract ↓
IntroductionHip instability is an important complication of spinal muscular atrophy (SMA), which leads to various functional impairments, including mobility challenges and difficulties with daily care. The primary objective of this study is to determine the prevalence and severity of hip instability in a cohort of SMA patients managed at a tertiary care center. The prevalence of scoliosis and its association with hip instability in children with SMA were also assessed.MethodsThis is a retrospective cross-sectional study including children with a genetic diagnosis of SMA and available hip x-rays, conducted between 2018 and 2023. Patients were grouped based on SMA type and motor status (non-sitters, sitters, or walkers). Relevant parameters were collected including hip subluxation/dislocation, presence of scoliosis, number of SMN2 copies and disease modifying therapies (DMTs) received.ResultsFifty-four children with types 1–3 SMA were included in this study. Hip dislocation/subluxation in both hips was most prevalent in type 1 SMA patients [Fisher's Exact Test (FET): p = 0.046]. There was no significant correlation between the presence of hip subluxation or dislocation and scoliosis, but patients with fewer SMN2 copies were more likely to have scoliosis (p = 0.023). Several factors, including gender, age, motor status and presence of scoliosis were analysed, but none showed a statistically significant association with hip abnormalities.DiscussionTo the best of our knowledge, this is the first study in the Middle East on hip instability and scoliosis in a cohort of children with SMA. Hip dislocation and/or subluxation is most common and most severe in type 1 SMA. The study's findings can aid the development of hip instability screening programs in SMA patients, enabling early intervention.
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