The incidence of acute and chronic pancreatitis is increasing in the United States. Rates of acute pancreatitis (AP) are similar in both sexes, but chronic pancreatitis (CP) is more common in males. When stratified by etiology, women have higher rates of gallstone AP, while men have higher rates of alcohol- and tobacco-related AP and CP, hypercalcemic AP, hypertriglyceridemic AP, malignancy-related AP, and type 1 autoimmune pancreatitis (AIP). No significant sex-related differences have been reported in medication-induced AP or type 2 AIP. Whether post-endoscopic retrograde cholangiopancreatography pancreatitis is sex-associated remains controversial. Animal models have demonstrated sex-related differences in the rates of induction and severity of AP, CP, and AIP. Animal and human studies have suggested that a combination of risk factor profiles, as well as genes, may be responsible for the observed differences. More investigation into the sex-related differences of AP and CP is desired in order to improve clinical management by developing effective prevention strategies, diagnostics, and therapeutics.
Bone morphogenetic proteins (BMPs) comprise a major subgroup of the transforming growth factor (TGF)-β superfamily. They play pivotal roles in embryonic development and tissue homeostasis in adults. Deregulation of BMP and TGF-β signaling contributes to developmental anomalies and multiple diseases. In this mini-review, we focus on BMP signaling in inflammatory disorders of the pancreas, acute and chronic pancreatitis, in contrast to TGF-β signaling. We then discuss molecular mechanisms that interact with and connect between the BMP and TGF-β signaling pathways. Lastly, we review potential implications of these molecular mechanisms for therapeutic development. In summary, BMP signaling pathway plays different roles during pancreatitis disease development, and the antagonism between BMP and TGF-β signaling can be manipulated for therapeutic development against pancreatitis.
Objective: Spinal muscular atrophy (SMA) is a group of progressive and fatal neurodegenerative disorders that are characterized by destruction of the anterior horn cells of the spinal cord. In this case report we outline the medical and ethical issues involved in a 7-year-old boy with SMA type 2 who experienced acute respiratory failure.Methods: A review of the literature was conducted focusing particularly on the pathology, presentation, and outcomes of SMA and end-of-life decision-making in pediatrics. Results:In a world where 40%-60% of deaths in pediatric intensive care units are a result of withdrawal or limitation of life-sustaining treatment, end-of-life decision-making has become an integral and difficult part of pediatric practice. Conclusion:Limitation or withdrawal of life-sustaining treatment in a cognitively normal child with SMA poses a significant medical and ethical dilemma. This difficult decision is influenced by confluence of parental, doctor, social, cultural, moral, religious, legal, and economic factors and more recently the media. Neurology 1 The highest mortality rates continue to be in developing countries, where the etiology of child death remains predominantly due to poverty and lack of adequate medical care and education surrounding transmission of disease. This is in stark contrast to developed countries, where modern medicine has significantly improved survival. In fact, we have become so good at prolonging life, that up to 40%-60% of deaths in pediatric intensive care units are a result of the withdrawal of life-sustaining treatment.2 Limitation or withdrawal of life-sustaining treatment in a cognitively normal child with spinal muscular atrophy (SMA) and its acknowledged respiratory complications poses a significant medical and ethical dilemma. In this case report, we outline the medical and ethical issues involved in a 7-year-old boy who experienced acute respiratory failure.SMA II CASE A 7-year-old boy with SMA type II presented with a 10-day history of progressive cough, shortness of breath, lethargy, and feeding intolerance, complicated by desaturation and hypercarbia during sleep. This was on a background history of profound weakness of both upper and lower extremities and nocturnal hypoventilation requiring BiPAP (noninvasive ventilation). After admission, he became increasingly tachypneic, hypopneic, and bradycardic, then unresponsive. Following bag mask resuscitation, on BiPAP he continued to be in respiratory distress; however, he was alert, orientated, and conversing at an age-appropriate level. Progressive deterioration over a period of hours with increasing drowsiness required endotracheal intubation and mechanical ventilation. Intubation was extremely difficult due to a grade IV airway and multiple unsuccessful attempts at intubation were performed prior to securing the airway with a glidescope. It was recognized at this point that future emergency airway access would be a challenge and tracheostomy the only long-term solution. His parents saw his ability to e...
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