scholarly journals 063 A new case series of Olmsted syndrome subjects confirms EGFR activation and shows remarkable efficacy of targeted systemic EGFR inhibition with acceptable side effects

2021 ◽  
Vol 141 (10) ◽  
pp. S159
Author(s):  
J. Basset ◽  
Y. Diab ◽  
F. Santiago ◽  
L. Azulay ◽  
K. Cordoro ◽  
...  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Carlos Ramírez-Paesano ◽  
Albert Juanola Galceran ◽  
Claudia Rodiera Clarens ◽  
Vicenҫ Gilete García ◽  
Bartolomé Oliver Abadal ◽  
...  

Abstract Background Patients with Ehlers-Danlos Syndrome/Hypermobility Type (EDS-HT/JHS) and Craneo-Cervical Instability frequently suffer from severe widespread pain which is difficult to control. Chronic neuroinflammation, opioid-induced hyperalgesia, and central sensitization may explain this painful condition. The aim of this study was to determine if opioid-free anesthesia plus the postoperative administration of lidocaine, ketamine and dexmedetomidine can reduce postoperative pain and the need of methadone rescues in comparison with opioid-based management in these patients undergoing Craneo-Cervical Fixation (CCF). The secondary aim was to assess the needs of opioids at hospital-discharge, incidence of gastrointestinal complications and the requirement of anxiolytic. Methods A retrospective, consecutive case series study was designed. 42 patients with EDS-HT/JHS undergoing CCF were enrolled in two groups: an OFA-plus Group that received opioid-free anesthesia with propofol, lidocaine, ketamine and dexmedetomidine, and OP Group, opioid-based anesthesia-analgesia. The main variables: Preoperative Visual Analogue Score (VAS), postoperative VAS on the 1st, 2nd, 4th and 6th days, sufentanil or morphine requirements, need for methadone rescue, and VAS at hospital-discharge. Data was presented by mean ± SD, percentage, median or interquartile range. Chi-squared or Fisher’s test. 95% C.I and P values < 0.05. Results Nineteen patients in OFA-plus, and 23 patients in OP group. VAS was lower in OFA-plus on the postoperative days evaluated (p < 0.001).VAS at hospital-discharge was lower in OFA-plus: 4.96 (4.54–5.37) vs. OP 6.39 (6.07–6.71) (p < 0.001). Methadone requirement was lower in the OFA-plus (p < 0.001). 78% of patients in OFA-plus didn’t need methadone rescue. 95% in OP group needed methadone rescues at high doses(> 15 mg/day). No differences regarding equivalent doses of sufentanil or morphine consumption on the 2nd, 4th, and 6th postoperative days were found. OFA-plus decreased ileus, nausea and vomiting (p < 0.001). 60.9% in OFA-plus group decreased opioid requirements at hospital-discharge compared with preoperative values. A 77% reduction of anxiolytics requirements was shown. Conclusion OFA-plus management for patients undergoing CCF with EDS-HT/JHS shows significant reduction in postoperative pain and at hospital-discharge compared with opioid-based anesthesia. OFA-plus management decreases the total doses of methadone rescues, reduces anxiolytic requirements and gastrointestinal side-effects, except for constipation. OFA-plus management is a feasible option to improve postoperative pain control, reducing the opioids’ use and their postoperative side-effects in patients undergoing CCF with EDS-HT/JHS.


Processes ◽  
2021 ◽  
Vol 9 (7) ◽  
pp. 1081
Author(s):  
Ming-Yang Lee ◽  
Mei-Yi Lin ◽  
Yu-Ju Chang ◽  
Yu-Ting Tseng ◽  
I-An Huang ◽  
...  

(1) Background: The epidermal growth factor inhibitors (EGFRIs)/tyrosine kinase inhibitors (TKIs) are effective for cancer target therapy, but acneiform rashes or so-called inflammatory papulopustular exanthemas are common (50% to 90%). The conventional therapy for EGFRIs/TKIs-induced skin toxicity is steroids and antibacterial drugs, but it is still ineffective for some patients, and EGFRIs/TKIs dose reduction/interruption may be needed. In this study, a modified Chinese herbal medicine, Huang-Lian-Jie-Du decoction cream with Yin-Cold (YC) medicine characteristic, was investigated for the effect on patients suffering EGFRIs/TKIs-induced skin toxicity. (2) Methods: The modified Huang-Lian-Jie-Du (mHLJD) decoction cream was made from 10 herbal medicines, including 4 major medicines (Huanglian, Huangqin, Huangbo, and Zhizi) in traditional HLJD decoction. Patients with EGFRIs/TKIs-induced skin toxicity were enrolled. Patients were excluded if they also used other cream for skin toxicity. Skin conditions were monitored by follow up every 2 weeks. The patients’ characteristics, the skin toxicities, treatment response, and adverse events were recorded and analyzed until skin problems resolved or the study ended. (3) Results: The mHLJD decoction cream and its sub-packages were stored at 4 °C before use. Thirty-four patients who had grade 1–3 skin toxicities after receiving EGFRIs/TKIs were enrolled. Seven patients withdrew or were excluded. Finally, data from 27 patients were analyzed. The mean grade of rash acneiform was significantly decreased from 2.19 (ranged 1 to 3) to 0.88 (ranged 0 to 2) after mHLJD decoction cream treatment for 4 weeks and to 0.55 (ranged 0 to 2) after mHLJD decoction cream treatment for 8 weeks. Additionally, the mean grade of dry skin was also significantly decreased from 1.57 (ranged 1 to 2) to 0.71 (ranged 0 to 1) after mHLJD decoction cream treatment for 4 weeks. The changes of skin toxicity were significant, with no obvious adverse events. (4) Conclusions: In summary, the mHLJD decoction cream provides benefits for alleviation of EGFRIs/TKIs-induced skin rash acneiform and dry skin. Additionally, no obvious side effects were found in patients using mHLJD decoction cream.


2015 ◽  
Vol 61 (3) ◽  
pp. 241-244
Author(s):  
Lazar Alexandra ◽  
Szederjesi Janos ◽  
Copotoiu Sanda Maria ◽  
Simon Noemi Szidonia ◽  
Badea Iudita ◽  
...  

Abstract Postoperative pain management is of major importance and the existence of a device that ensures a good analgesia in the immediate postoperative period and also removes the side effects of the systemic drugs, is becoming a necessity. Objectives: The goal was to obtain a good quality anaesthesia and also a good postoperative analgesia by inserting a perineural catheter at the brachial plexus site. Material and method: This study included adult patients who underwent brachial plexus anaesthesia through a perineural catheter inserted at the brachial plexus site. The perineural catheter was introduced by ultrasound guidance with neurostimulation control. After insertion, a quantity of a an-aesthetic admixture of 0.4mg/kg is administered. The anaesthetic admixture contained Ropivacaine and Lidocaine, equimolar concentration of 0.5% In the postoperative period, the analgesia was ensured trough the already installed catheter. The analgesic mixture contained Ropivacaine and Lidocaine, equivalent concentrations of 0.25%. The administration rate was 5 ml every 4 hours, starting 6 hours postoperatively. Results: The anaesthesia, obtained through the perineural catheter, was a good quality anaesthesia ensuring both, good sensory and motor block. The feedback regarding postoperative analgesia was positive, this type of pain management being efficient and without the systemic drug side effects. This approach of brachial plexus block was accepted easily by the patients and was rated as a very satisfactory method. Conclusions: The insertion of a perineural catheter for anaesthesia and postoperative analgesia represents a safe and efficient method of achieving both analgesia and anaesthesia.


2018 ◽  
Vol 30 (1) ◽  
pp. 40-47 ◽  
Author(s):  
Islam SH Ahmed ◽  
Ahmed MA Hadi ◽  
Hassan H Hassan

Aim: To report the results of treatment of type 1 prethreshold retinopathy of prematurity using intravitreal injection of ultra-low dose of ranibizumab (0.1 mg in 0.01 mL). Design: A retrospective observational case series study. Methods: Review of files of eligible infants who received this form of treatment to determine the outcome of treatment and any associated ocular or systemic side effects. Results: The study included 24 eyes of 12 preterm infants with mean gestational age of 29.75 ± 1.54 weeks and mean birth weight of 1074.58 ± 320.59 g. A total of 22 eyes (91.67%) had zone II disease while 2 eyes of one infant (8.33%) had zone I disease. All cases showed regression of the signs of the active retinopathy of prematurity with complete retinal vascularization. None of the cases required retreatment. Three eyes developed ocular complications. Apart from mild feeding intolerance that lasted for 24 h after injection in one infant, none of the cases developed systemic side effects. Conclusion: Intravitreal injection of ultra-low-dose ranibizumab showed promising efficacy and good ocular safety. However, further large-scale studies are required to give stronger evidence about the efficacy and safety of ultra-low-dose ranibizumab.


2020 ◽  
Vol 7 (11) ◽  
pp. 1747
Author(s):  
Dnyanesh N. Morkar ◽  
Ankita Aneja ◽  
Rishabh Agarwal

The prognosis of HIV infection has considerably improved following the introduction of highly active anti-retroviral therapy by reducing AIDS related morbidity and mortality. At the same time, ART drugs are well known for their side effects. Gynaecomastia is a lesser known side effect of a commonly used anti-retroviral drug efavirenz. There are very few reports of HAART-induced gynaecomastia in resource-limited settings. The current study presents a series of three cases that developed ultrasound confirmed gynaecomastia following efavirenz containing HAART. Initial reports of gynaecomastia related to HAART were in HIV patients with lipodystrophy, they were termed as pseudogynaecomastia. Gradually, few reports of efavirenz related gynaecomastia were published wherein other causes of gynaecomastia were ruled out. Several hypothesis have been suggested for the pathophysiology of development of gynaecomastia related to efavirenz consumption. All other causes were ruled out in our patients too. The incidence of gynaecomastia is increasing in men with HIV on HAART therapy, proper identification and management will promote better drug adherence.


BJPsych Open ◽  
2021 ◽  
Vol 7 (S1) ◽  
pp. S116-S116
Author(s):  
Declan Hyland ◽  
Alex Walmsley ◽  
Victoria Simpson

ObjectiveThis retrospective case series followed emotionally unstable personality disorder (EUPD) patients following initiation of clozapine on an off-licence basis, aiming to examine tolerance by determining side effect prevalence and treatment cessation frequency, as well as examining efficacy, by investigating number of hospital re-admissions and symptom control.Case reportThis case series captured the experiences of 11 EUPD patients under the care of Mersey Care NHS Foundation Trust, all of whom had, at some time in the past five years, been initiated on clozapine. All patients were white British females, with a median age of 31. The median daily dose of clozapine was 300 mg. Most patients had significant psychiatric comorbidities, as well as illicit substance and / or alcohol misuse.Whilst prescribed clozapine, patients were only admitted to hospital once on average and this was commonly for clozapine re-titration. Whilst in hospital, rates of self-harm were low, but ligaturing and suicide attempts showed higher prevalence. Patients still demonstrated self-harming behaviour out of hospital leading to A and E presentations. In the community, contacts with the police were minimal, with only two patients undergoing Section 136 assessments or arrests.All patients reported side effects from clozapine - usually hypersalivation, over-sedation and constipation. All 11 patients experienced sinus tachycardia. Eight patients temporarily ceased taking clozapine at some point. In three patients, discontinuation of clozapine was as a result of intolerable side effects. Three patients experienced neutropenia, which subsequently resolved. Only two patients had a body mass index within healthy range.DiscussionDespite patients reporting clozapine to provide symptomatic benefit for their EUPD, and improved their engagement with mental health services, prevalence of self-harm and of A and E presentations remained high, indicating the importance of community support and concomitant psychotherapeutic treatment. Patients with more robust community support showed greater adherence to clozapine.High prevalence of side effects and obesity in these patients, in addition to risk of developing neutropenia, highlights the importance of rigorous monitoring after initiating clozapine. It is reassuring that, despite development of neutropenia in some patients, this recovered quickly, and clozapine treatment could resume.ConclusionClozapine may be an effective pharmacological treatment for enabling EUPD patients to engage more therapeutically with services. Clozapine may be of greater benefit to those with more stable, less chaotic lives. Although diminished, patients still show self-harming behaviour and need for A and E admissions and re-hospitalisation. Side effects of clozapine are common and regular monitoring is required.


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