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Discussion Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane receptor (CFTR). It is found in the epithelium of the bronchi, intestine, pancreatic duct and biliary tree. It regulates chloride, bicarbonate and water secretion. The heterozygous state helps prevent against secretory diarrhea, but the homozygous state causes thickened secretions in the hollow tubes of the lungs and digestive tract. There are multiple mutations (> 2000) which have been currently classified into classes depending on their protein production and activity. CF patients generally are living longer with improved morbidity because of new treatments including lung transplantation but they also are at higher risk of having gastrointestinal problems over their lifespan. Gastrointestinal problems in patients with CF include: Pancreas Second most frequent organ affected after the lung Concentrated pancreatic juice and thickened secretions can increase obstruction leading to acute or chronic injury and inflammation Pancreatic insufficiency Most (~90%) patients have exocrine pancreatic insufficiency Greasy stools, abdominal bloating, flatulence, diarrhea and poor weight gain are symptoms Treatment is pancreatic enzyme replacement therapy (PERT), proton pump inhibitors are used sometimes also Fat soluble vitamins (A, D, E, and K) can occur and usually are also supplemented Poor nutrition Patients have increased nutrition needs because of the ex...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news

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Health ministers call on company to lower price of cystic fibrosis drug Orkambi so the NHS can afford it.
Source: BBC News | Health | UK Edition - Category: Consumer Health News Source Type: news
Adequately treating pain and nausea following uterine artery embolization (UAE) is a continuing challenge. Superior hypogastric nerve block (SHNB) is a successful adjunct technique for decreasing pain after embolization. This letter discusses safety measures for administration of SHNB during UAE using radial artery access, including avoiding inadvertent intravascular injection, choosing the optimal anesthetic, and treating local anesthetic systemic toxicity.
Source: Journal of Vascular and Interventional Radiology : JVIR - Category: Radiology Authors: Tags: Letter to the Editor Source Type: research
A 27-year-old competitive runner presented with deep right thigh pain for 1  year. A T2-weighted hyperintense 3-cm lesion was found in the right vastus lateralis muscle on magnetic resonance imaging, indicative of a ganglion cyst (Fig 1). Corresponding ultrasound showed a well-defined, hypoechoic lesion confirming the diagnosis of ganglion cyst. This location is rare for g anglion cyst, and such lesions have been treated only by surgical excision. Ultrasound-guided aspiration and sclerosis with sodium tetradecyl sulfate was performed.
Source: Journal of Vascular and Interventional Radiology : JVIR - Category: Radiology Authors: Tags: Images in IR Source Type: research
The complex nature of ankle fractures is magnified when seen in patients at high risk of soft tissue wound healing complications. The major categories include associated soft tissue injury, diabetes, tobacco use, peripheral vascular disease, malnutrition, alcoholism, and corticosteroid use. Because of the potential for wound dehiscence and infection with open reduction and internal fixation of ankle fractures in these patients, minimally invasive procedures have been described. The aims of the present study were to assess the possibility for, and evaluate the results and complications of, minimally invasive techniques for ...
Source: Journal of Foot and Ankle Surgery - Category: Orthopaedics Authors: Source Type: research
We report our experience with medial cuneiform decompressive exostectomy and superficial tendon debridement in 14 cases of recalcitrant tibilalis anterior insertional pain. We reviewed 13 patients (14 feet; 12 females, 1 male; mean age 67.9 ± 7.5; range 55 to 80 years) in whom conservative treatment had failed who had undergone debridement of the insertional tibialis anterior tendon and decompressive exostectomy of the medial cuneiform.
Source: Journal of Foot and Ankle Surgery - Category: Orthopaedics Authors: Source Type: research
Plantar heel pain is a common disabling condition in adults. Biomechanical factors are important in the development of plantar heel pain. Quantitative changes in rearfoot alignment in patients with plantar heel pain have not been previously investigated. From April 2016 to March 2017, 100 patients with plantar heel pain and 100 healthy individuals were recruited. The foot posture index was used for the measurement of foot alignment. The generalized joint hypermobility condition was assessed using the Beighton scale.
Source: Journal of Foot and Ankle Surgery - Category: Orthopaedics Authors: Source Type: research
PMID: 29672384 [PubMed - in process]
Source: Anesthesia and Analgesia - Category: Anesthesiology Authors: Tags: Anesth Analg Source Type: research
PMID: 29672377 [PubMed - in process]
Source: Anesthesia and Analgesia - Category: Anesthesiology Authors: Tags: Anesth Analg Source Type: research
Authors: Santonocito C, Noto A, Crimi C, Sanfilippo F Abstract The use of remifentanil in clinical practice offers several advantages and it is used for a wide range of procedures, ranging from day-surgery anesthesia to more complex procedures. Nonetheless, remifentanil has been consistently linked with development of opioid-induced hyperalgesia (OIH), which is described as a paradoxical increase in sensitivity to painful stimuli that develops after exposure to opioid treatment. The development of OIH may cause several issues, delaying recovery after surgery and preventing timely patient's discharge. Moreover, it c...
Source: Local and Regional Anesthesia - Category: Anesthesiology Tags: Local Reg Anesth Source Type: research
This may seem a little basic, but I’m a resident and am curious how most people dose their opioids. I generally don’t give any long acting opioids for cases without significant post op pain. For cases where they’ll need post op analgesia, I usually dose some long acting opioids at the start of GA because I think it lets me use less fentanyl and smoothes out the hemodynamics. So long as it’s a modest dose, I haven’t had anyone yet that fails to breath by the end of the surgery. Do others do... Opioid dosing under GA
Source: Student Doctor Network - Category: Universities & Medical Training Authors: Source Type: forums
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