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Lysosomal Storage Disorders and Enzyme Replacement Therapy
Published in Peter Grunwald, Pharmaceutical Biocatalysis, 2020
Available treatment methods for different MPS types include ERT, substrate reduction therapy (SRT), HSCT, and gene therapy. A therapy based on pharmacological chaperones (PCT)—these small molecules improve the stability, intracellular localization, and function of mutated enzymes in LSDs (Hoshina et al., 2018)—alone or in combination with the previously mentioned methods may be also applied. In the recent past, advances in MPS treatment have rapidly developed; however, the efficacy of these therapies is limited because treatment starts in most cases when the physical symptoms are already pronounced (Beck, 2018), and, in addition, timely treatment is hampered by significant diagnostic delays (Laney et al., 2018) so that methods of newborn screening to provide early detection for specified disorders is an important public health action (Rajabi, 2018). Among the supportive measures are administering of nonsteroidal anti-inflammatory drugs (NSAID) to patients suffering from joint pain, and antibiotics for patients with upper respiratory infections; anti-inflammatory drugs, steroids, mechanical ventilation, and/or oxygen are employed in the case of pulmonary issues. Surgical removal of the adenoid (adenoidectomy), the removal of both palatine tonsils from the tonsillar fossa (tonsillectomy), anterior cervical decompression and fusion before irreversible spinal cord injury has occurred, or correction of hip dysplasia, e.g., via peri-acetabular osteotomy, are among the additional surgical procedures often required in MPS treatment (Tomatsu et al., 2015, 2015a). The focus of this section is on enzymes replacement therapy (Ries, 2017).
Response to High-Frequency Current Passing Through the Body
Published in Leslie A. Geddes, Handbook of Electrical Hazards and Accidents, 1995
“The surgeons performed the adenoidectomy and right tonsillectomy without incident then began to use a suction electrocautery to control the bleeding in the right tonsillar fossa. The electrocautery was set at 35-watts coagulation in the “spray” mode. After approximately 30 sec of cautery, a fire erupted in the pharynx that “blow-torched” toward the lips. Breath sounds were immediately lost and increased airway pressure was noted. The fire was extinguished with saline, the pharynx was suctioned, and the endotracheal tube was immediately removed. The tube was noted to be melted and charred externally for 2 cm, midway between the distal tip and the adaptor, and fused for 1 cm at that point with 100% occlusion. From the point of fusion distally, the tube was blackened internally. The trachea was immediately reintubated with a 5.0 mm ID PVC, uncuffed, endotracheal tube. With a fractional inspired 02 concentration (F,02) of°·99 and 1% halothane, pH, was 7.43, Paco2, 28 mmHg, and Pao2, 575 mmHg. On direct examination the mucosa of the posterior tongue, uvula, and hypopharynx were noted to be erythematous and charred. Rigid bronchoscopy using a 4.0 mmx30 cm bronchoscope was performed. The cords were not burned, but were edematous. The mucosa of the anterior trachea, carina, and left mainstem bronchus was erythematous and charred in some areas. This was probably caused by a “blow-torching” of the fire downward. The burns were mostly anterior and not circumferential. After bronchoscopy, the trachea was reintubated orally with a 5.0. mm ID PVC, uncuffed, endotracheal tube under direct vision. Breath sounds were again equal and clear bilaterally. The patient was given dexamethasone 2 mg iv.
A contemporary systematic review of the complications associated with SURGICEL
Published in Expert Review of Medical Devices, 2023
Matthew Masoudi, Jacob Wiseman, Sam M. Wiseman
Twelve studies reported hemorrhagic complications occurring despite the use of SURGICEL. A study by Carrion et al. (2017) that reported on the use of SURGICEL in 77 patients undergoing laparoscopic partial nephrectomy found that it did not prevent hemorrhagic complications [N = 77, P > 0.05]. Alternatively, when Myung et al., investigated the effectiveness of SURGICEL, they found that while its use significantly lowers mean hospitalization time [p < 0.001], and was an independent predictor of complications such as fever or delayed bleeding by multivariate analysis [OR 11.1114 p = 0.005]. Three of the included articles in this review reported infection and fever as a complication. Another reported complication was pain (n = 3), notably, how the use of SURGICEL affects pain experienced by patients undergoing tonsillectomy [50]. The authors found while SURGICEL significantly reduced bleeding, its use also significantly increased postoperative day-one pain scores that were reported by tonsillectomy patients (N = 760, 95% confidence interval, P < 0.05) [50]. Biliary complications were reported in three papers. Arisheh and colleagues conducted a retrospective cohort study over 4 years and found that when SURGICEL was used for liver bed hemostasis during laparoscopic cholecystectomy, patients were more likely to be re-hospitalized and diagnosed with a subhepatic abscess [P < 0.001] [48]. The same study also reported that there was a 6.7-fold increased risk for re-hospitalization for abdominal symptoms and/or fever when SURGICEL was used to control bleeding during laparoscopic cholecystectomy [48].