Factor replacement is currently the standard of care to prevent or treat bleeding episodes in haemophilia patients. This study examined current prescribing practices of factor therapy for patients at haemophilia treatment centres (HTCs) in the United States.
The aims were to evaluate the driving forces for prescribing factor products, to evaluate current attitudes and knowledge toward factor product and industry, and to discuss the implications for health care providers in practice.
An anonymous electronic survey was distributed to 744 HTC health care providers (HCPs); 118 responses were analysed.
The most common driving force for HCPs to change a patient's factor product was poor response to current therapy, while the most common perception of patients’ motivation to switch products was the potential for fewer infusions. HCPs with strong influence over the prescribed therapy identified inadequate pharmacokinetic (PK) studies as an important driving force; patients/caregivers perceived as having a strong influence over which therapy is prescribed selected less frequent dosing as an important motivator. HCPs who allow patients/caregivers to have a strong influence over which factor is prescribed were more likely to cite patient/caregiver request as a significant driving force for change in therapy.
The haemophilia treatment landscape continues to evolve and is becoming increasingly complex. The multitude of treatment options available now offer choices, presenting a need to focus on patient-centric prophylaxis.
Pain associated with bleeding disorders has been demonstrated to have an impact on patients’ and families’ quality of life. Both acute and chronic pain are common experiences and require attention by professionals working in haemophilia treatment centres (HTCs). The benefits of psychological pain management strategies such as cognitive behaviour therapy and self-management skills training are well documented; however, it is not well understood how Canadian social workers involved in haemophilia care perceive and provide pain management support to patients.
To explore the current understanding of pain management and practice as well as the education needs of members of Canadian Social Workers in Hemophilia Care (CSWHC).
Twelve semi-structured qualitative interviews were conducted with members of CSWHC. Transcribed interviews were coded with NVivo software and thematically analysed.
The four key themes reflecting the experiences of social workers are: 1) Limited comprehension of key issues related to pain; 2) Conditioning to push through pain; 3) Expanding pain knowledge to enhance practice; 4) How we practice social work and choose to step in.
The current practice of CSWHC members aligns with literature in three main areas including assessment, instrumental services, and counselling. Social workers support the development of pain education and practical resources for patients with haemophilia who experience pain. While formal education, advocacy, and policy development of pain assessment and management are recognised, these areas require further research and development.
X-linked adrenoleukodystrophy (X-ALD) is an inherited metabolic disease which causes demyelination of the white matter of the brain. The symptoms include mental impairment, progressive paresis, impaired motor coordination, and epileptic seizures. Diagnosis is established mainly by genetic testing. Currently, the recommended treatment is haematopoietic stem cell transplantation (HSCT).
The aim of the study was to present the case of a patient suffering from X-ALD, who developed symptoms of bipolar disorder in the initial phase of the disease prior to the onset of characteristic neurological symptoms.
In 2015, a 33-year-old patient was admitted to a psychiatric department due to aggressive behaviour he showed towards his wife and other family members. He had been treated for a depressive episode in 2005, and for a manic episode without psychotic symptoms earlier in 2015. During the successive psychiatric hospitalizations, in addition to psychopathological symptoms, the patient had been observed to have neurological symptoms, which included progressive paraparesis and ataxia. In 2018, based on imaging and genetic tests, the patient was diagnosed with X-ALD. The patient's condition gradually deteriorated; with time, he was unable to move on his own. During a hospital stay in 2019, he was transferred to an internal medicine department due to a progressive urinary tract infection, which, however, could not be controlled, and the patient died.
X-ALD is a rare metabolic illness. In the early stages of the disease, various psychopathological symptoms, including affective disorders, are observed.
Early initiation of adequate treatment increases the chances of extending the patient's life.
In the present case, the patient did not die due to the underlying disease, but due to causes typical of bed-bound patients, i.e. complications of progressing infection.
Introduction: Cotard's syndrome (CS) is a rare set of psychopathological symptoms, the main symptom of which is nihilistic delusions concerning the negation of the existence of internal organs or the entire body
Aim, material and methodology: The aim of the study is to present a case of a patient treated for postpartum depression who developed Cotard's syndrome. The patient's symptoms began immediately after her daughter. The clinical picture was dominated by anxiety and apathy, nihilistic delusions about the atrophy of the urethra and other lower abdominal organs, and olfactory hallucinations - she could smell rot.
Discussion: The available literature on Cotard’s Syndrome does not allow us to indicate a certain reason for its development. Perhaps the birth of the first child - the woman doubted herself as a mother, she was afraid that she would hurt the cause of the disorders observed and described by us was transient ischemia of the CNS during delivery.
Conclusions: Cotard's syndrome can develop in the course of many mental and somatoform disorders. The described case is, to our knowledge, the first description of Cotard’s Syndrome in the deprivation period. Difficulties in establishing the etiopathogenesis and pathophysiology of Cotard’s Syndrome translate into therapeutic problems. It has been suggested that the treatment of the underlying disorder on the basis of which CS is developed remains the most effective method of therapy.
Introduction: Deep Brain Stimulation can directly alter brain activity in a controlled manner and the effect is reversible. The mechanism is that the electrode acts locally on neural activity, which is transferred to monosynchronous and multisynaptic network connections.
Methods: We present studies conducted on a group of patients that show an improvement in mental state after Deep Brain Stimulation.
Material: The diseases we included in our work are: Obsessive-Compulsive Disorder, Eating Disorder, Depression and Bipolar Affective Disorder.
Discussion: The use of deep brain stimulation can inhibit development of acute state of patients and improve both psychiatric features and the time of remission. The results indicate the greatest effectiveness of Deep Brain Stimulation in Obsessive-Compulsive Disorders.
Conclusions: Brain stimulation may be a promising therapeutic target in mental illness. In a properly selected location, it can contribute to a significant clinical improvement however further research in this direction is necessary.
Introduction: Dyskinesia is a symptom complex in the form of involuntary, repetitive movements of lips, lower jaw, tongue, less often the trunk and limbs. Despite the use of newer drugs in treatment neuroleptics, dyskinesia has not ceased to be a clinical problem.
Method: The work is based on a research review for which the Google Scholar database was used as well PubMed. The search range was limited to 2008-2020. We have included descriptive publications tardive dyskinesia only as a consequence of antipsychotic medications.
Material: We present the use of tetrabenazine analogues, deep brain stimulation, neuroleptics, benzodiazepines and botulinum toxin in late-suffering patients drug-induced dyskinesias, which may indicate an improvement in your health.
Discussion: The first method of treating tardive dyskinesia are withdrawal antipsychotic medications, but for many patients this is impossible. Valbenazine and Deep Brain Stimulation are the most effective in treating Tardive Dyskinesia.
Conclusions: There are not enough studies with the highest reliability to create unequivocal recommendations in the treatment of drug-induced tardive dyskinesia.
Introduction: The study aims to present 1) a case report of an 18-year-old female patient with borderline personality disorder (BPD) 2) the diagnostic and treatment difficulties in BPD patients.
Materials and methods: The review of the literature from the years 1953-2020 searched from PubMed, Google Scholar, and Web of Science databases.
Discussion: Patient, 18-years old, hospitalized five times for psychiatric care, presenting self-injurious behaviours, hurting herself within forearms. A patient attempted suicide eight times. She is living with her divorced parents. The patient’s father has a new partner with whom he has children. The patient used marijuana and she was experiencing psychotic symptoms under its influence. She smokes 10 cigarettes per day and drinks alcohol once a week since she was 16 years old. Psychiatric problems appeared when the patient started learning in high school.
1. Borderline personality disorder (BPD) is a serious psychiatric condition of a difficult diagnosis that should be differentiated with many other psychiatric disorders such as an atypical or subclinical course of psychosis, affective disorders, or dissocial personality.
2. A therapeutic process of a person with BPD is based on psychotherapy and personalized treatment strategies, whereas, pharmacological treatment plays only a supporting role during BPD treatment.
Aim. Chemo-herbal combinations promise new clinical anticancer therapeutic modalities. The current study investigated and compared the in vitro effects of a bromelain-based chemo-herbal combination to/with cisplatin or 5-FU, with regard to the proliferation and apoptosis of human gastric AGS and breast MCF7 cell lines.
Material and methods. AGS and MCF7 cells were either treated with different concentrations of bromelain, cisplatin or 5-FU; or with bromelain-cisplatin and bromelain-5-FU combinations for 48h. Cell proliferative inhibition and inductive apoptosis were appraised using MTT assay and flowcytometry, respectively. Kruskal-Wallis and Dunn’s tests were used to analyze differences in cell groups’ means.
Results. AGS proliferation was adversely affected by single treatments of bromelain and cisplatin (p <0.003) or 5-FU (p <0.05). The anti-proliferative impact of single treatments was more pronounced on MCF7 cells. The bromelain-cisplatin combinations displayed synergistic effect on MCF7 cells (CIs ≤1), while being additive or antagonistic with cisplatin IC30 and IC40 to AGS cell proliferation, respectively. In addition, bromelian-5-FU combinations showed synergistic effect on AGS cells, while antagonistic to MCF7 cells. In terms of cell apoptosis induction, bromelain (IC30)-cisplatin (IC20) displayed additive effect on MCF7, compared to cisplatin single treatment (p <0.04), while bromelain (IC40)-5-FU (IC10) and bromelain (IC30)-5-FU (IC20) afforded additive apoptotic effects on AGS (p <0.04) and MCF7 (p <0.05), respectively, in comparison to 5-FU single treatment.
Conclusion. A bromelain-based combination using cisplatin showed concordant effects on cell proliferation impediment and apoptotic induction on MCF7, while the same results were noticed with a bromelain-5-FU combination to AGS cells. The bromelain-based chemo-herbal pathways should further be investigated in the frame of multi-chemotherapeutic drugs researches.
Introduction. When considering the array of biomedical problems associated with facial nerve palsy (FNP), it is apparent that the problem of choosing an effective type of facial nerve transfer is of paramount relevance. Hence, it is to the pursuit of a solution to the above stated problem that our study is devoted.
Materials and methods. We analyzed the data obtained as a result of assessing the outcome of 149 patients with trauma-caused FNP who had undergone surgical treatments in the period between 2001-2018. The FN nerve transfer techniques utilized were as such: for group 1 – the use as a donor nerve the branch of the accessory nerve innervating the sternocleidomastoid muscle (n=87); group 2 – the descending branch of the hypoglossal and the branch of the accessory nerve (n=62).
Results. In groups 1–2, the FN nerve transfer significantly improved FN function (p <0.01; Wilcoxon Matched Pairs Test), and most patients (n=135; 90.6%) noted an improvement in the clinical status up to degree II-III on the House-Brackmann scale (good result).
Conclusion. The use of the accessory nerve branches to the sternocleidomastoid muscle, as donated, ensures restoration of FN function to levels II-III on the House-Brackmann scale in 89.7% of all operated patients, and the results it achieves do not differ from those of the technically more complex nerve transfer of FN by descending branch of the hypoglossal nerve with combination of branches of the accessory nerve.
Varroa destructor is an external parasitic mite that is a serious pest of honeybees and has caused severe losses of colonies worldwide. One of the feasible alternative treatments being used for their control is the application of essential oils, which are generally inexpensive and most pose few health risks. The investigation was designed to determine the chemical composition, toxicity and acaricidal effects of Eucalyptus amygdalina leaf essential oil (EaEO) grown in Algeria and to compare its activity on Varroa destructor with that of Eucalyptus globulus from the same region. Fresh leaves of E. amygdalina (Ea) by steam distillation yielded 0.77% (v/w), and investigation of the oil on GC/MS resulted in the identification of 35 compounds, with 1.8-cineole (35.78%) as most abundant constituent. Other notable compounds include spathulenol (12.58%), camphene (7.73%), α-pinene (4.38%), valencene (2.64%), while 2-carene and ledol (1.45%) were also among the constituents identified. The acaricidal features of the essential oil was evaluated using bee hives infected by Varroa destructor, and a significant effect of oil application was observed (p < 0.05). Cytotoxic effect was assayed using the brine shrimp lethality assay, Probit’s analysis of the result revealed an LC50 value of 116.06 μg/mL. Essential oil of E. amygdalina (EaEO) has potential acaricidal effect on Varroa destructor, but this effect is less important than the one recorded by E. globulus. Further studies are needed to determine the active component responsible for this effect.