Mary C Theroux1, Alicia Olivant, Robert E Akins. 1. Department of Anesthesiology and Critical Care, Alfred I. duPont Hospital for Children, Wilmington, DE 19899, USA. mtheroux@nemours.org
Abstract
BACKGROUND: Our objective was to better understand neuromuscular junction (NMJ) morphology in children with Duchenne muscular dystrophy (DMD). METHODS: We examined the NMJs of four children, age 6-13 years, who were diagnosed with DMD. Using our previously established staining technique, we examined the gross appearance of the NMJs in patients with DMD and evaluated the spread of acetylcholine receptors (AChRs) in relationship to the NMJs. We used a computerized algorithm to measure the area of staining corresponding to AChRs and NMJ. RESULTS: Abnormal shape and morphological appearance of some of the NMJs was clearly evident. The spread of AChRs in DMD patients is comparable with the spread of AChRs in nonDMD patients. CONCLUSIONS: The distribution of AChRs in relationship to the boundaries of NMJs in DMD children is similar to the distribution of NMJs in the erector spinae muscles of idiopathic scoliosis patients.
BACKGROUND: Our objective was to better understand neuromuscular junction (NMJ) morphology in children with Duchenne muscular dystrophy (DMD). METHODS: We examined the NMJs of four children, age 6-13 years, who were diagnosed with DMD. Using our previously established staining technique, we examined the gross appearance of the NMJs in patients with DMD and evaluated the spread of acetylcholine receptors (AChRs) in relationship to the NMJs. We used a computerized algorithm to measure the area of staining corresponding to AChRs and NMJ. RESULTS: Abnormal shape and morphological appearance of some of the NMJs was clearly evident. The spread of AChRs in DMDpatients is comparable with the spread of AChRs in nonDMD patients. CONCLUSIONS: The distribution of AChRs in relationship to the boundaries of NMJs in DMDchildren is similar to the distribution of NMJs in the erector spinae muscles of idiopathic scoliosispatients.
Authors: Karyn G Robinson; Matthew J Viereck; Megan V Margiotta; Karen W Gripp; Omar A Abdul-Rahman; Robert E Akins Journal: Am J Med Genet A Date: 2013-08-16 Impact factor: 2.802
Authors: Jia Li; Maureen Fredericks; Marishka Cannell; Kathryn Wang; Dianne Sako; Michelle C Maguire; Rosa Grenha; Katia Liharska; Lavanya Krishnan; Troy Bloom; Elitza P Belcheva; Pedro A Martinez; Roselyne Castonguay; Sarah Keates; Mark J Alexander; Hyunwoo Choi; Asya V Grinberg; R Scott Pearsall; Paul Oh; Ravindra Kumar; Rajasekhar Nvs Suragani Journal: J Clin Invest Date: 2021-02-15 Impact factor: 14.808