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Introduction and Importance: PHACE syndrome is a rare neurocutaneous disorder characterized by large segmental hemangiomas on the face and is associated with multiple developmental defects. PHACE stands for posterior fossa malformations, hemangiomas, arterial abnormalities, cardiac defects, and eye anomalies, with the most common manifestation being hemangioma in the cervico-facial region in early childhood., Case Presentation: The authors report a case of a 15-year-female with complaints of facial hemangioma which on multisystemic imaging showed features of central nervous system (CNS) anomalies that led to the diagnosis of PHACE syndrome. The patient was started on propanolol which decreased the size of hemangioma in follow-up visits., Clinical Discussion: The hemangioma in the frontotemporal and frontonasal area of the face are associated more with CNS and cardiovascular anomalies needing a detailed multisystem approach. CNS anomalies include posterior cerebral fossa enlargement, cystic dilation of the fourth ventricle, arachnoid cyst, and cerebellar hypoplasia which were present in our case. Propanolol is considered the first-line drug for facial hemangioma with reported evidence of remarkable improvement and good tolerance. However, regular follow-up of the patient is needed to rule out any recurrence., Conclusion: PHACE syndrome, although being a rare occurrence, must be kept as a differential diagnosis in infants and children with facial hemangioma. Imaging modalities like MRI/magnetic resonance arteriography must be used to rule out possible associations related to PHACE syndrome and focus on early treatment to prevent possible complications., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

Cavernous sinus haemangiomas are neoplastic lesions notoriously difficult to diagnose and excise. We present a case of a lesion that was clinically, biochemically, and radiologically consistent to a giant pituitary macroadenoma and discuss the unexpected near intra-operative exsanguination which enabled a pathognomonic diagnosis of a much rarer lesion to be made. This highlights the sinister nature of such a lesion, and its potential impact on patient care if partially excised, and that despite our advances in neurosurgical diagnostics the unexpected is to be expected.

Infantile haemangiomas (IHs) are the most common benign soft tissue tumours in children. Usually, they evolve without clinical incurrences and regression of the lesion can occur spontaneously in the first years of life. The decision for treatment is dependent upon the intrinsic characteristics of the lesion such as location, extension, functional compromise and complications. We present the case of a newborn who was clinically accessed during ambulatory routine consultation when a lesion with 5x5 centimetres compatible with an IH was first observed. Inflammatory signs with no active bleeding were present and the newborn displayed signs of discomfort during a diaper change and manipulation of the anogenital area. For this reason, a referral was made for observation in a central hospital with specialised paediatrics, paediatric surgery and dermatology support. A 10-day antibiotic course with flucloxacillin and local topical care with silver sulfadiazine cream and barrier cream with zinc oxide were adopted, achieving a good clinical outcome. Laboratory workup, cardiovascular assessment, imagiological investigation with abdominopelvic and spinal cord ultrasonography as well as lumbosacral magnetic resonance imaging were all normal. Ulceration is the most prevalent complication of IHs and it is associated with pain, recurrent bleeding, infection and difficult scarring, thus early recognition and directed treatment are essential to achieve a good clinical outcome., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Carvalho et al.)

Harlequin syndrome (HS) is a rare entity derived from the dysfunction of the sympathetic nervous system. It is characterised by unilateral facial flushing and sweating induced by exercise, heat and emotion. Most cases are primary with an unknown pathogenic mechanism. In these cases, the prognosis is favourable. Medical or surgical treatments are not usually required for idiopathic HS. However, symptomatic treatment may be indicated when symptoms affect the quality of life of patients. We present the case of a patient with idiopathic HS successfully treated with oxybutynin and propranolol. In this patient, a marked improvement in both hyperhidrosis and facial erythema was noted with this combined therapy. We consider it of interest to highlight the response of our patient to the treatment employed, which may be advantageous in future cases of this rare disorder., (© 2021 Australasian College of Dermatologists.)

Long QT syndrome (LQTS) is one of the most common inherited cardiac channelopathies with a prevalence of 1:2000. The condition can be congenital or acquired with 15 recognized genotypes; the most common subtypes are LQTS 1, 2, and 3 making up to 85%-90% of the cases. LQTS is characterized by delayed ventricular cardiomyocyte repolarization manifesting on the surface electrocardiogram (EKG) by a prolonged corrected QT (QTc) interval. The mainstay of treatment for this condition involves in part or combination medical therapy via β-blockers as first-line (or other anti-arrhythmic), left cardiac sympathectomy, or implantable cardiac defibrillator placement. Given the high rate of adverse cardiac events (ACE) or sudden cardiac death (SCD) in this population of patients with this disease, this review seeks to highlight the genotype-specific treatment consensus in β-blocker therapy of the most common subtypes. A database search of PubMed, PMC, and Medline was conducted to ascertain the most recent data in the last five years on the management of LQTS types 1-3 and the role of β-blockers in reducing ACE in these types. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were adhered to in the study selection, and selected studies focused on humans, written in the English Language, and within the last five years of LQTS subtypes 1, 2, and 3. Eleven relevant studies were selected after considering inclusion criteria, exclusion criteria, and quality appraisal within the last five years, focusing on β-blocker selection directed based on the subtypes of LQTS. Two meta-analyses, one cohort study, and eight reviews provided significant data that non-selective β-blockers unequivocally are of benefit in these LQTS types. Summary of findings suggested nadolol followed by propranolol yields the best results in LQTS 1, while nadolol would yield the best effect in LQTS 2 and 3., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Went et al.)

Objective: Severe burn injuries are associated with a rapid escalating hypermetabolic state and catabolism of muscle mass. To ameliorate this process a standardized approach using pharmacological and non-pharmacological interventions was implemented within a single burns center. Whilst individual components of this standardized package are well documented in the literature, their collective or bundled effect has not as yet been assessed. The aim of this study was to evaluate the efficacy of this standardized bundle of metabolic modulators and assess the safety of including the anabolic steroid oxandrolone within it. Methods: This retrospective observational study constituted all patients in whom the metabolic bundle including oxandrolone therapy was applied. The other elements of the metabolic bundle consisted of early surgical burn excision within seven days to completion, early active mobilization, increased ambient room temperature, expediated carbohydrate and protein rich enteral feeding with glutamine and trace element supplements (such as copper and zinc). Finally, administration of propranolol as a non-selective beta-blocker. Data collection was through review of the patient data management system focusing on the outcome criteria and hepatic blood values. Results: The study looked at fifty consecutive patients meeting the inclusion criteria. Median patient age and burned total body surface area (TBSA) were 62 years [51.75; 73] and 33.75% [24.75; 51] respectively with an abbreviated burn severity index (ABSI) of 10 [9; 10.25]. Definitive surgical burn wound excision was completed in 44 patients [88%] within 7 days. 39 patients (78%) received propranolol over a therapeutic period of 29 days [19; 44]. Glutamine was supplemented in 45 patients (90%), while zinc and copper were applied to 42 (84%) and 31 (62%) respectively. Significant low zinc values were noted at therapeutic onset (6.5 mmol/l [4.7; 7.9]) requiring sustained substitution over 37.5 days [22; 46.75]). In respect of the inclusion criteria, all patients received oxandrolone at 20 mg/day [20; 20]. This was commenced on day 6.5 [4; 14] post burn injury and continued over 26 days [19; 31]. Despite a transitory elevation of hepatic enzyme values (ALT, GGT), these were only clinically relevant (>10 µmol/l*S) in 2.4% and 4.6% of all measurements respectively. None were sufficiently of concern to merit cessation of treatment. Conclusion: The application of a standardised bundle of metabolic treatment options of severe burns injured patients is reliable, repeatable and safe. Potential concerns of oxandrolone treatment regarding hepatic compromise remain unfounded., Competing Interests: The authors declare that they have no competing interests., (Copyright © 2019 Taha et al.)

In this issue of the Biomedical Journal we discover how alternative splicing might modulate the cell-type specificity and exact function of a ubiquitous purinergic receptor and how the beta blocker propanolol can contribute to breast cancer therapy. Moreover, we learn which culture conditions generate the best vascularisation of tissue engineered bone and which are the clinical features of acute necrotising encephalopathy in adults. Other studies reveal, how laser irradiation can fix fractured all-ceramic dental restorations in situ, and finally that nuclear magnetic resonance holds great potential for the rapid detection of pathogens., (Copyright © 2019 Chang Gung University. Published by Elsevier B.V. All rights reserved.)

Azadirachta indica A. Juss var. siamensis Valeton or commonly known as Siamese neem is one of the most well-known plant in traditional Ayurvedic medicine. The aim of the present study was to investigate the relaxant effects of A. indica on isolated rat ileum contractions and its potential underlying mechanisms involved. The isometric contractions of ileum segments were investigated in organ baths for spontaneous activity and response to aqueous extract of Siamese neem flower (SNF). The spasmolytic action of the extract was also assessed on contraction induced by acetylcholine and high potassium. Our findings indicate that cumulative concentrations of SNF aqueous extract induced relaxant effect on spontaneous rat ileum contractions. The extract has also suppressed the cumulative concentration response curve for acetylcholine and pottasium ions-induced contraction. The presence and absence of propranol (antagonist of β-adrenergic receptor) and l-Name (antagonist of nitric oxide synthase) in SNF aqeous extract co-treatment demonstrated no significant different in term of contraction activity when compared to SNF extract treatment alone. The treatment of SNF extract caused a significant inhibition in tissue contraction stimulated by accumulation of calcium ions. Our results showed the relaxant effect of SNF aqueous extract on the isolated rat ileum. In short, the SNF aqueous extract exhibited an inhibitory effect on the spontaneous ileum contactions particularly on the contraction stimulated by acetylcholine and high potassium. The observed effect might acted through the modulation of calcium channels. This findings provide a pharmacological basis for the traditional use of SNF for the treatment of gastrointestinal spasms.

Autobiographical memory is fundamental to the process of self-construction. Therefore, the possibility of modifying autobiographical memories, in particular with memory-modulation and memory-erasing, is a very important topic both from the theoretical and from the practical point of view. The aim of this paper is to illustrate the state of the art of some of the most promising areas of memory-modulation and memory-erasing, considering how they can affect the self and the overall balance of the "self and autobiographical memory" system. Indeed, different conceptualizations of the self and of personal identity in relation to autobiographical memory are what makes memory-modulation and memory-erasing more or less desirable. Because of the current limitations (both practical and ethical) to interventions on memory, I can only sketch some hypotheses. However, it can be argued that the choice to mitigate painful memories (or edit memories for other reasons) is somehow problematic, from an ethical point of view, according to some of the theories of the self and personal identity in relation to autobiographical memory, in particular for the so-called narrative theories of personal identity, chosen here as the main case of study. Other conceptualizations of the "self and autobiographical memory" system, namely the constructivist theories, do not have this sort of critical concerns. However, many theories rely on normative (and not empirical) conceptions of the self: for them, the actions aimed at mitigating or removing specific (negative) memories can be seen either as an improvement or as a depletion or impairment of the self.

Mammary pathology is infrequent during childhood. It is rare the probability of finding a breast mass in an infant. The most frequent possible diagnoses at this age are breast abscess, mastitis, breast engorgement due to maternal hormonal stimulation and hemangioma. Reaching the proper diagnosis is essential in order to apply a suitable treatment and avoid the potential disease complications. We present the case of a female infant having a mass in the right breast from birth. Initially the entity was treated as mastitis. Nevertheless, the bad evolution made necessary considering the differential diagnosis. It was concluded to be a hemangioma. Due to the lesion ulceration and the potential risk of developing breast hypoplasia, treatment with propranolol was started. The tumor was almost completely resolved., (Sociedad Argentina de Pediatría.)

A neonate presented to our clinic for evaluation of unusual congenital cleft lip. The clinical follow-up showed at first an ulceration of the lesion and then a stable result after propanolol systemic therapy. After 18 months of clinical follow-up, surgical treatment was performed, which consisted of double unilimb Z-plasty according to Mulliken's microform cleft lip repair. Knowing the existence of these strange vascular anomalies of the lip will allow us to improve the differential diagnosis and treatment plan.

Objectives: With the emergence of propranolol as the primary treatment for hemangiomas the indications for surgical intervention have been greatly reduced. There remains a role for surgical management in those patients who fail medical therapy, particularly for hemangiomas involving the airway. Detailed is our experience with subglottic hemangiomas, including three patients who failed propranolol treatment and were successfully treated with surgical excision and single stage laryngotracheoplasty (LTP) with thyroid ala graft., Methods: Retrospective case series (level of evidence: 4)., Results: Six patients were treated with propranolol for subglottic hemangiomas over a 6 year period (2008-2014). Three patients responded to propranolol therapy and required no adjunctive surgical procedures. Three patients failed propranolol treatment, and required open resection of their subglottic hemangiomas and thyroid ala graft placement. Indications for resection were complete lack of response to propranolol in one patient, and initial response to propranolol with subsequent regrowth in the other two patients. All three patients were treated with submucosal extirpation of their hemangioma and single stage LTP; hemangioma was confirmed in all cases by positive GLUT-1 staining. All three surgical patients were successfully extubated post-operatively and none had hemangioma regrowth., Conclusions: Fifty percent of patients in our series did not have long-term response to propranolol for subglottic hemangioma, highlighting the importance of close follow-up. When identified early, subglottic hemangiomas refractory to propranolol treatment can be successfully addressed with single stage LTP and tracheotomy can be avoided., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Primary hyperhidrosis commonly affects the axillae, palms, soles, scalp, face, and groin. Patients may have multiple areas involved making localized therapy challenging. Systemic therapy may be necessary and can be used as monotherapy or combined with other hyperhidrosis treatments for optimal outcomes. Systemic therapy can also be used to treat secondary hyperhidrosis and compensatory hyperhidrosis., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Infantile hemangiomas (IHs) are the most common proliferating embrional tumors of infancy, which are constituted by endothelial cell hyperproliferation. The authors want to report their observations of further 14 patients suffering from complicated IHs involving the facial district who were treated with propranolol. 14 patients, with ages between 3 and 12 months, completed a cycle of treatment with propanolol. The observational study aimed at focusing IHs involving the facial district. The treatment with propranolol showed good to very good results in the major part of the treated young patients. The authors want to report their experience and add more data in the confirmation of the use of β-blockers for IH (either in efficacy or in safety profile), focusing on the efficacy of propanolol when IHs involve the face., (© 2014 Wiley Periodicals, Inc.)

Both medication and non-medication based strategies are used in the management of problem behaviours in individuals with intellectual disabilities. Beta-adrenoceptor blocking medications are one group of drugs used for this purpose. However, despite its regular use, the evidence for the efficacy of these drugs for in this context is lacking. A systematic review was conducted to establish the research evidence for the efficacy of beta blockers in problem behaviours in adults and children with intellectual disabilities. Although the research identified supported the efficacy of beta blockers for this indication the overall quality of studies identified was poor and no randomised controlled trials were identified. There is a need for more robust research into the use of beta blockers for people with intellectual disabilities who show problem behaviours., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

The presence of pharmaceuticals and personal-care products (PPCPs) in aquatic environments is of concern. Although measured concentrations of individual substances are low, little consideration has been given to the likely chronic nature of the exposures or to the potential for mixture effects. The purpose of the present study was to use the RTG-2 rainbow trout cell line to analyse sub-lethal and cytotoxic effects of PPCPs present in a wastewater-treatment-plant (WWTP) effluents and their mixtures. Interactions with cytochrome P450 1A enzyme, oxidative stress, cellular senescence and cell viability were assessed using 7-ethoxyresorufin-o-deethylase (EROD), reactive oxygen species (ROS), ß-galactosidase (ß-gal) and neutral red (NR) uptake assays, respectively. Not all of the compounds that were tested exhibited significant effects. The lowest-observed-effect concentrations and half maximal effective concentrations (EC50 ) were within the range 0.15 to 784.47 µg l(-1) . Clear dose-response curves were found for cells exposed to different mixtures of PPCPs. The lowest-observed-effect concentrations and concentrations causing EC50 were within the range 0.05 to 54.61 µg l(-1) . Four out the seven tested mixtures induced EROD activity. ROS production was detected in two mixtures. The ß-gal inhibition response was observed in six out the seven tested mixtures and occurred at a higher concentration than was observed for EROD induction activity or ROS generation. The present study clearly shows that the stress response through which cells mount a homeostatic response to toxicants can be potentially used for an initial, rapid and cost-effective assessment of the complex mixtures of PPCP that present in WWTP effluents are difficult and expensive to analyse chemically., (Copyright © 2012 John Wiley & Sons, Ltd.)

Thyroidectomy is the most common endocrine surgical procedure being carried out throughout the world. Besides, many patients who have deranged thyroid physiology, namely hyperthyroidism and hypothyroidism, have to undergo various elective and emergency surgical procedures at some stage of their life. The attending anesthesiologist has to face numerous daunting tasks while administering anesthesia to such patients. The challenging scenarios can be encountered at any stage, be it preoperative, intra-op or postoperative period. Preoperatively, deranged thyroid physiology warrants optimal preparation, while anticipated difficult airway due to enlarged thyroid gland further adds to the anesthetic challenges. Cardiac complications are equally challenging as also the presence of various co-morbidities which make the task of anesthesiologist extremely difficult. Thyroid storm can occur during intra-op and post-op period in inadequately prepared surgical patients. Postoperatively, numerous complications can develop that include hemorrhage, laryngeal edema, nerve palsies, tracheomalacia, hypocalcemic tetany, pneumothorax, etc., The present review aims at an in-depth analysis of potential risk factors and challenges during administration of anesthesia and possible complications in patients with thyroid disease.

Introduction: Several therapeutic approaches have been developed to treat choroidal hemangioma. However, all these therapies are associated with a potential risk of damaging the overlying retina., Case Report: We report a case of circumscribed choroidal hemangioma (CCH) in a 59-year-old man refractory to laser treatment. Visual acuity was 20/200 and a serous macular detachment was present. The CCH was treated with oral propanolol, whereupon visual acuity improved to 20/20 and the macular detachment resolved without systemic or local adverse effects., Discussion: Propanolol is a β-blocker commonly used in cardiology that may induce endothelium vasoconstriction and inhibit endothelial proliferation. It has been shown to be effective in infantile facial hemangiomas, and proved safe and effective for the CCH in our patient. Further studies are needed to confirm our observation.