Carpal Tunnel Syndrome in the Setting of Mucopolysaccharidosis II (Hunter Syndrome)

Background:. Carpal tunnel syndrome (CTS) is a rare finding in children, but heavily represented in pediatric patients with mucopolysaccharidoses. Diagnosis is a challenge due to lack of the stereotypical symptomatic complaints and relies on examination and objective nerve conduction studies. Methods:. We present a case of delayed presentation of CTS in a 12-year-old boy with Hunter syndrome, followed by a review of the literature. Results:. Patient Z.D. presented with minimal reported CTS symptoms but advanced median nerve damage on electromyography. He underwent bilateral carpal tunnel release with median nerve neurolysis and flexor tenosynovectomies. Intraoperative examination demonstrated the presence of a “waist sign” of the median nerve and moderate flexor tenosynovial hypertrophy bilaterally. Parents reported mild subjective improvement of dexterity and fine motor skills postoperatively. Conclusion:. To optimize functional outcome, routine screening for CTS and intervention at an early age are emphasized in the mucopolysaccharidoses population

Local Application of Probiotic Bacteria Prophylaxes against Sepsis and Death Resulting from Burn Wound Infection.

To determine if local prophylactic application of probiotic bacteria to burn wounds will prevent death in a mouse model of burn wound sepsis.Infection remains the most common complication after burn injury and can result in sepsis and death, despite the use of topical and systemic antibiotics. Pseudomonas aeruginosa is a frequently implicated pathogen. Local application of probiotics directly to burn wounds is an attractive novel intervention that avoids the pitfalls of standard antibiotic therapies.A burn-sepsis model was established using a sub-eschar injection of bioluminescent P. aeruginosa; infection was tracked using a charge-coupled camera. Full-thickness burn injuries were placed on the dorsums of adult mice; the injured sites were then treated with vehicle (burn wound control), probiotics (Lactobacillus plantarum only), pathogenic bacteria (Pseudomonas aeruginosa only), or probiotics plus pathogen (Lactobacillus plus Pseudomonas). Animals were monitored until death/moribundity or for one week, then sacrificed. Harvested tissues were subjected to imaging and molecular assays.Control and probiotic-only animals showed no mortality (100% survival) at one week. Pseudomonas-only animals showed > 90% mortality within 40 hours of infection. In contrast, animals treated with probiotics plus Pseudomonas showed less than 10% mortality. Use of bioluminescent Pseudomonas bacteria demonstrated that probiotic therapy inhibited septicemic accumulation of the pathogen in remote organs. In addition, probiotic therapy successfully suppressed the infection-dependent induction of TNF-α and interleukins 6 and 10 in the liver.Local probiotic therapy shows great potential as a valuable adjunct in the management of complicated burn injury