Objectives: Evidence for nocturnal oximetry interpretation in patients with abnormal neuromuscular function is limited. We aimed to compare children with neuromuscular disease (NMD) or Prader-Willi syndrome (PWS) to otherwise healthy subjects with obstructive sleep-disordered breathing (SDB) or without respiratory disorder (controls) regarding nocturnal oximetry parameters.
Methods: We analyzed recordings from children with: (a) NMD; (b) PWS; (c) snoring and adenotonsillar hypertrophy and/or obesity (SDB); and (d) controls. Outcomes included: (a) basal SpO ; (b) proportions of subjects with McGill oximetry score (MOS) >1 (clusters of desaturations); and (c) desaturation index (SpO drops ≥3%/h-ODI3).
Results: Data of 12 subjects with NMD (median age, 5.2 years; IQR, 2.7, 8.2), 14 children with PWS (5 years; 2.3, 6.9), 21 children with SDB (5.8 years; 4.6, 9.6), and 20 controls (6.2 years; 5.4, 11.2) were analyzed. Children with NMD, PWS, and SDB had lower basal SpO than controls (95.6% [94.5%, 96.9%], 96.2% [95.1%, 97.4%], 96.1% [95.8%, 97.5%] vs 97.8% [97.2%, 97.9%], respectively; (P < .01). NMD and PWS showed the greatest negative effect on basal SpO (P < .05). Children with SDB or PWS had a higher risk of MOS >1 than patients with NMD (OR, 25.9 [95% CI, 3.4-200.4] and 9.5 [1.5-62.6]). NMD, PWS, and SDB were similar regarding ODI3, which was elevated compared to ODI3 in controls (P < .05). Frequent desaturations predominated in NMD, while periods of sustained desaturation were noted in NMD and PWS.
Conclusion: PWS and NMD have a negative effect on basal SpO , while clusters of desaturations are prevalent in patients with PWS or obstructive SDB.
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http://dx.doi.org/10.1002/ppul.24899 | DOI Listing |
J Clin Med
January 2025
Jackie and Gene Autry Children's Orthopedic Center, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA.
Postoperative ileus, the temporary cessation of gastrointestinal motility leading to accumulation of fluid and gas in the bowel, is a common complication following posterior spine fusion (PSF) in patients with neuromuscular scoliosis (NMS). Abdominal radiographs (KUBs) are often ordered to differentiate between ileus and mechanical obstruction but expose patients to radiation, add cost, and may lead to unnecessary work up. The aim of this study was to determine how often KUBs led to a change in treatment after PSF in patients with NMS.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Eye Clinic, Neuromuscular and Sense Organs Department, Careggi University Hospital, Largo Brambilla 3, 50134 Florence, Italy.
Background: To investigate functional and anatomical outcomes after pars plana vitrectomy (PPV) for lamellar macular hole (LMH) with a long-term follow-up.
Methods: An interventional study on 14 patients (16 eyes) with LMH was conducted. The inclusion criteria included a minimum 36-month follow-up after PPV.
Int J Pediatr Otorhinolaryngol
January 2025
Northwestern University Feinberg School of Medicine, Chicago, IL, USA; Ann & Robert H Lurie Children's Hospital of Chicago, Division of Otolaryngology, Chicago, IL, USA.
Background: Cerebral palsy (CP) is the most common neuromuscular disorder in children, and children with CP are at increased risk of respiratory distress potentially requiring tracheostomy placement. Previous studies have characterized indications for tracheostomy in neurologically compromised children, however no studies focus specifically on children with CP. The purpose of this study was to identify the indications for tracheostomy placement, sites of airway obstruction, and rate of decannulation in children with CP.
View Article and Find Full Text PDFNeurogastroenterol Motil
January 2025
Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Introduction: Colonic manometry (CM) is a diagnostic procedure used to evaluate pediatric patients with refractory constipation, fecal incontinence, Hirschsprung disease, and pediatric intestinal pseudo-obstruction. Pan-colonic high-amplitude propagated contractions (HAPCs), measured by CM, reflect an intact neuromuscular function of the colon. Current guidelines recommend starting CM with fasting recording for 1-2 h, but no prior evaluation has determined the diagnostic yield of the fasting phase.
View Article and Find Full Text PDFAm J Phys Med Rehabil
February 2025
From the Centre for Translational Medicine, Semmelweis University, Budapest, Hungary (OZG, MV, MAE, LS, ZM, PH, TT); Department of Orthopaedics, Semmelweis University, Budapest, Hungary (OZG, MV, TT); Department of Anesthesiology and Intensive Therapy, Semmelweis University, Budapest, Hungary (ZM); Department of Anesthesiology and Intensive Therapy, Poznan University for Medical Sciences, Poznan, Poland (ZM); and Institute of Pancreatic Diseases, Semmelweis University, Budapest, Hungary (PH).
Objectives: This systematic review and meta-analysis of randomized controlled trials aimed to summarize the reported functional effects of upper limb botulinum toxin treatment in children with spastic cerebral palsy.
Design: Six databases were searched in October 2022. Two independent authors screened and extracted data on upper limb function, body function, life quality, muscle tone, spasticity, goals, pain, adverse events, and client satisfaction.
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