Proopiomelanocortin (POMC) disregulation and response to opiate blockers
Adult; Naltrexone; Autistic disorder; Pro-Opiomelanocortin; Self-Injurious behavior
Autism is a collection of disorders or subtypes with distinctive or prominent phenotypes and genotypes. The study of adults with autism offers a unique opportunity to examine its phenotypic diversity. Recent evidence has identified disturbances in specific neurochemical systems that are associated with primary autistic symptoms. Establishing biological markers, such as specific neurochemical disturbances, not only confers greater precision in phenotyping individuals but also provides the basis for rational intervention. Our initial studies in adult individuals exhibiting self-injurious behavior (SIB) generated evidence that the proopiomelanocortin disregulated in subgroups of autistic patients. These findings, corroborated in at least 15 other laboratories, indicated that treatment with an opiate blocker, naltrexone (NTX), reduced SIB in 30-70% of individuals observed. However, the effects of NTX on SIB were not simple. We and others have found that concentration of plasma POMC fragments, specifically opioid fragments, contributed to the symptoms of autism and to the response to treatment. Uncoupling of the release of POMC products predicted the efficacy of NTX treatment on the expression of SIB. Uncoupling of POMC fragments among autistic and SIB patients suggested a basic, underlying defect, perhaps in the POMC gene. The findings of a maternal influence on the C-terminal BE fragment among individuals with autism (Leboyer et al. [1999] Soc Biol Psychiatry 45:158-163) and our preliminary findings, reported here, of a mutation in the opioid region of the POMC gene in an autistic individual were consistent with the prospect that a subgroup of patients will be identified who share a POMC genetic defect.
Sandman CA; Spence MA; Smith M
Mental Retardation And Developmental Disabilities Research Reviews
1999
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/(sici)1098-2779(1999)5:4%3C314::aid-mrdd9%3E3.0.co" target="_blank" rel="noreferrer">10.1002/(sici)1098-2779(1999)5:4%3C314::aid-mrdd9%3E3.0.co</a>
Buprenorphine for chronic pain in a pediatric patient with sickle-cell disease
pediatric; palliative care; pain; buprenorphine; sickle-cell disease
Sickle-cell disease (SCD) is an inherited hematologic disorder characterized by the presence of sickle-shaped red blood cells (RBC). 1 Misshapen RBCs are rigid, which leads to occlusion of blood vessels resulting in tissue ischemia and pain. Pain can manifest as acute, intermittent episodes (vaso-occlusive crises [VOC]), chronic pain, or acute-on-chronic pain. 1 Buprenorphine is a semisynthetic opioid that has historically been used for opioid use disorder. Due to a unique receptor binding profile and favorable safety profile, including lower risk of tolerance and hyperalgesia, buprenorphine is increasingly recognized for its utility in chronic pain management, especially in complex cases. 2 ,3 Two small studies reported decreased healthcare utilization and daily opioid requirements in adults with chronic SCD pain transitioned from full opioid agonists to buprenorphine. 4 ,5 Another case series of two adolescents with chronic SCD pain described rotation to buprenorphine with improved functionality and decreased opioid requirements. 6 Literature on buprenorphine for pain in pediatric patients is sparse in general and practically nonexistent for pediatric chronic SCD pain. 7 Here, we report buprenorphine induction for chronic pain in a pediatric patient with SCD.
Irwin M; Gunther W; Keefer P; Saul D; Singh S; Wright J; Smith M
Journal of Pain and Symptom Management
2021
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/j.jpainsymman.2021.04.007" target="_blank" rel="noreferrer noopener">10.1016/j.jpainsymman.2021.04.007</a>
Consultation patterns before and after embedding pediatric palliative care into a pediatric hematology/oncology clinic
child; Palliative Care; article; controlled study; female; human; male; retrospective study; outpatient; palliative therapy; Referral and Consultation; Hematology; clinical article; consultation; medical record review; solid tumor; rank sum test; hematology; cancer model; health care delivery; embedding
Introduction: Palliative care is a critical component of pediatric oncology care. Embedded pediatric palliative care (PPC) is relatively new in pediatric hematology/oncology (PHO) and may improve access, utilization, and quality of PPC. In June 2020, the Mayo Clinic PPC service transitioned from an afternoon, physically independent clinic to an all-day clinic embedded within PHO. Method(s): Retrospective chart review was used to quantify consultation rates from PHO to PPC in 12-month study periods before and after establishment of an embedded clinic. Changes in descriptive statistics and consult patterns were calculated. Study periods were compared using either chi-square or Fisher's exact tests for categorical variables and Wilcox rank sum tests for continuous variables. Result(s): There was an 89% increase in consultations from PHO to PPC after initiation of an embedded clinic (n = 20 vs. n = 38 per 12 months). The absolute number of completed outpatient consults increased from three (15% of visits) pre-embedment to fourteen (37%) post-embedment (p =.082). The median number of days from first oncology visit to PPC assessment was unchanged after embedment (36 vs. 47 days, p =.98). Consults for solid tumors increased from 22% (n = 4) pre-embedment to 60% (n = 18) post-embedment (p <.05). Consults for symptom management increased from 60% (n = 12) to 87% (n = 33) (p <.05). Conclusion(s): Embedment of PPC into a PHO workspace was associated with an increased number of total consults, outpatient consults, solid tumor consults, and consults for symptom management. Our "partial-PPO" model allowed for provision of PPC in the outpatient oncology setting in a clinic where there is not enough volume to support a full-time oncology-focused clinician team.Copyright © 2023 Wiley Periodicals LLC.
Greenmyer JR; Ngo T; Smith M; Collura C; Schiltz B; McCarthy SR
Pediatric Blood and Cancer
2023
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/pbc.30663" target="_blank" rel="noreferrer noopener">10.1002/pbc.30663</a>