Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients—four with MPS I and five with MPS II—underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from –4.09±0.83 to –3.68±0.43 after 1 year of hGH treatment, and to –3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.

Background: The costoclavicular brachial plexus block (CCB) is a recently established technique that uses the infraclavicular approach and is performed just below the clavicle. This meta-analysis aimed to determine whether CCB can reduce the incidence of hemidiaphragmatic paralysis (HDP), which is a major adverse event related to brachial plexus block (BPB), while yielding comparable block performance as other BPB techniques. Methods: We searched electronic databases to identify relevant studies that compared the incidence of HDP between CCB and other BPB techniques. The primary outcome was the incidence of HDP following CCB and other BPB techniques. The secondary outcomes were pulmonary function test results, other adverse events, and block performance parameters such as onset and performance time. Results: We included six randomized controlled trials that included 414 patients. Compared with the other BPB group, the CCB group had a significantly lower incidence of HDP (relative ratio: 0.21, 95% CI [0.12, 0.36], P < 0.001) and higher peak expiratory flow rate (mean difference: 0.68 L/s, 95% CI [0.13, 1.23], P = 0.015). There were no significant between-group differences with respect to other adverse events and block performance parameters. Conclusions Compared with other BPB techniques, CCB involves a lower incidence of HDP with comparable onset and performance time.

BACKGROUND: Aquafol, a microemulsion propofol, causes more severe and frequent pain on injection than propofol. The purpose of this study was to compare a combination of lidocaine and ketamine on aquafol-induced pain with lidocaine or ketamine alone during the induction of anesthesia. METHODS: In this prospective, randomized, double-blinded study, 130 healthy patients who were undergoing elective surgery under general anesthesia were enrolled. The patients received IV lidocaine 40 mg plus ketamine 25 mg (Group LK, n = 43), lidocaine 40 mg (Group L, n = 42), or ketamine 25 mg (Group K, n = 45) with a rubber tourniquet on the forearm 1 min before the injection of microemulsion propofol. The pain score was assessed by a 4-point verbal rating scale (VRS) at 10 seconds after injection of microemulsion propofol 30 mg and during the injection of the remaining total dose. RESULTS: The incidence and severity of pain was significantly lower in Group LK than Group L or Group K at 10 seconds after the injection of microemulsion propofol 30 mg (P < 0.05). And the incidence and severity of pain was significantly lower in Group LK and Group K than Group L during the injection of the remaining total dose (P < 0.05). CONCLUSIONS: Pretreatment with IV lidocaine 40 mg plus ketamine 25 mg with a rubber tourniquet on the forearm 1 min before the injection of microemulsion propofol is more effective than lidocaine 40 mg or ketamine 25 mg alone in preventing pain from the injection of microemulsion propofol.
Anesthesia ; Anesthesia, General ; Forearm ; Humans ; Incidence ; Ketamine ; Lidocaine ; Propofol ; Prospective Studies ; Rubber ; Tourniquets

BACKGROUND: Using analysis of air samples from the workplace, we report on one case of pneumoconiosis in an individual who has been working in a polytetrafluoroethylene (PTFE) spraying process for 28 years. CASE PRESENTATION: The patient was diagnosed with granulomatous lung disease caused by PTFE using computed tomography (CT), lung biopsy and electron microscopy. To assess the qualitative and quantitative exposure to PTFE in workplace, Fourier transform infrared spectroscopy (FT-IR), energy-dispersive X-ray spectroscopy (EDX) and thermogravimetric analysis (TGA) were performed on air samples from the workplace. The presence of PTFE particles was confirmed, and the airborne concentration of PTFE was estimated to be 0.75 mg/m3. CONCLUSIONS: This case demonstrates that long-term exposure to PTFE spraying can cause granulomatous lung lesions such as pneumoconiosis; such lesions appear to be caused not by the degradation products of PTFE from high temperatures but by spraying the particles of PTFE. Along with air-sampling analysis, we suggest monitoring the concentration of airborne PTFE particles related to chronic lung disease.
Biopsy ; Humans ; Hygiene ; Lung ; Lung Diseases ; Microscopy, Electron ; Occupational Diseases ; Pneumoconiosis ; Polytetrafluoroethylene ; Spectroscopy, Fourier Transform Infrared ; Spectrum Analysis