scholarly journals Prophylactic heme arginate therapy in acute intermittent hepatic porphyria - a case report

2021 ◽  
pp. 9-9
Author(s):  
Zarko Krnetic ◽  
Tatjana Pesic ◽  
Zeljka Savic ◽  
Tatiana Jocic ◽  
Olgica Latinovic-Bosnjak ◽  
...  

Introduction. Among the acute hepatic porphyrias, a small percentage of patients, predominantly female, present with recurrent cyclic attacks of acute intermittent porphyria (AIP) that occurs more than three times a year, and sometimes at intervals of less than a month. In women, the attacks are typically related to menstrual cycle, requiring several days of hospitalization and administration of heme arginate. For these patients, the prophylactic heme arginate therapy may be the optimal treatment modality. Case Report. We present a 40-year-old female patient who has been suffering from porphyria for seventeen years. The first attack occurred in 2003, presenting with severe neurological symptoms, requiring the use of heme arginate Normosang?, which resulted in a favorable therapeutic response. In 2004 and 2007, gonadorelin analogue Zoladex? (goserelin) was used, but without beneficial effects on the course of the disease. In 2008, a preventive administration of heme arginate was initiated. The patient received heme arginate in the early phase of symptoms, every month in the premenstrual phase of the cycle, which resulted in milder symptoms, full recovery within 24 hours, lower doses of Normosang? (1-2 ampoules) and fewer hospital days (1-2 days) per month. This regimen has significantly improved the patient's quality of life and reduced the risk of potential adverse effects. Conclusion. Preventive use of Normosang? is the optimal therapeutic modality in patients with frequent, recurrent severe attacks that are unresponsive to other therapeutic regimens. As a result, patients have a better quality of life due to an effective, short-term, targeted treatment regimen.

2020 ◽  
Vol 29 (4) ◽  
pp. 685-690
Author(s):  
C. S. Vanaja ◽  
Miriam Soni Abigail

Purpose Misophonia is a sound tolerance disorder condition in certain sounds that trigger intense emotional or physiological responses. While some persons may experience misophonia, a few patients suffer from misophonia. However, there is a dearth of literature on audiological assessment and management of persons with misophonia. The purpose of this report is to discuss the assessment of misophonia and highlight the management option that helped a patient with misophonia. Method A case study of a 26-year-old woman with the complaint of decreased tolerance to specific sounds affecting quality of life is reported. Audiological assessment differentiated misophonia from hyperacusis. Management included retraining counseling as well as desensitization and habituation therapy based on the principles described by P. J. Jastreboff and Jastreboff (2014). A misophonia questionnaire was administered at regular intervals to monitor the effectiveness of therapy. Results A detailed case history and audiological evaluations including pure-tone audiogram and Johnson Hyperacusis Index revealed the presence of misophonia. The patient benefitted from intervention, and the scores of the misophonia questionnaire indicated a decrease in the severity of the problem. Conclusions It is important to differentially diagnose misophonia and hyperacusis in persons with sound tolerance disorders. Retraining counseling as well as desensitization and habituation therapy can help patients who suffer from misophonia.


Author(s):  
Ganapathi Rao ◽  
Vijay Kumar ◽  
Ashok Naikar ◽  
Chandrakanth Halli

A standard Ksharasutra is practiced in treatment of Bhagandara (fistula-in-ano) with high success rate and minimum recurrence rate. In previous research it was noted that Pittaja Prakruti patients sometime might cause more perianal irritation due to Apamarga Ksharasutra. So in this study Palasha Ksharasutra prepared in Arkaksheera was prepared by Palasha Kshara (Ash of Butea monosperma), Arka Ksheera (Calotropis gigantic) and turmeric powder (Curcuma longa). This Ksharasutra was prepared as per the API guidelines and preserved in air tight tube. A patient of Pittaja predominant Prakruti with fistula-in ano having two external opening at 6 and 7 O’ clock position of anus was treated with application of Ksharasutra. The Palasha Ksharasutra prepared in Arkaksheera was applied in these two opening under spinal anesthesia. Then Ksharasutra was changed by weekly interval under local xylocaine jelly 2%. The length of thread was measured weekly and noted in the case to assess the unit cutting time (UCT). The unit cutting time (UCT) of first thread was 7.5 days/cm and second one had UCT 6.8 days/cm. During the treatment patient was doing his job regularly without hampering the quality of life. After 2 months patient was free from all symptoms of fistula with normal scar and without any complications. This case study demonstrated the utility of Palasha Ksharasutra prepared in Arkaksheera in multiple fistula-in ano.


Biomolecules ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 393
Author(s):  
Oliver Neuhaus ◽  
Wolfgang Köhler ◽  
Florian Then Bergh ◽  
Wolfgang Kristoferitsch ◽  
Jürgen Faiss ◽  
...  

Although fatigue is a common symptom in multiple sclerosis (MS), its pathomechanisms are incompletely understood. Glatiramer acetate (GA), an immunomodulatory agent approved for treatment of relapsing-remitting MS (RRMS), possesses unique mechanisms of action and has been shown to exhibit beneficial effects on MS fatigue. The objective of this study was to correlate clinical, neuropsychological, and immunological parameters in RRMS patients with fatigue before and during treatment with GA. In a prospective, open-label, multicenter trial, 30 patients with RRMS and fatigue were treated with GA for 12 months. Inclusion criterion was the presence of fatigue as one of the most frequent and disabling symptoms. Before and during treatment, fatigue was assessed using the Fatigue Severity Scale (FSS), the MS-FSS, and the Modified Fatigue Impact Scale (MFIS). In addition, fatigue and quality of life were assessed using the Visual Analog Scales (VAS). Laboratory assessments included screening of 188 parameters using real-time PCR microarrays followed by further analysis of several cytokines, chemokines, and neurotrophic factors. Fatigue self-assessments were completed in 25 patients. After 12 months of treatment with GA, 13 of these patients improved in all three scales (with the most prominent effects on the MFIS), whereas 5 patients had deteriorated. The remaining 7 patients exhibited inconsistent effects within the three scales. Fatigue and overall quality of life had improved, as assessed via VAS. Laboratory assessments revealed heterogeneous mRNA levels of cytokines, chemokines, and neurotrophic factors. In conclusion, we were not able to correlate clinical and molecular effects of GA in patients with RRMS and fatigue.


2013 ◽  
Vol 2013 ◽  
pp. 1-2 ◽  
Author(s):  
P. Lucas Ramirez ◽  
Kelly Barnhill ◽  
Alan Gutierrez ◽  
Claire Schutte ◽  
Laura Hewitson

This case report describes the benefits of antibiotic and antifungal therapy on behavior in a child with autism undergoing treatment for encopresis. Over the course of treatment, the child exhibited a reduction in aberrant behaviors, increased gastrointestinal function, and improved quality of life.


2021 ◽  
Vol 30 (3) ◽  
pp. 110-114
Author(s):  
Diana Maghiar ◽  
◽  
Nicoleta Anamaria Pascalau ◽  
Liviu Lazar ◽  
◽  
...  

Objectives. We want to present the evolution of a lot of patients, previously diagnosed with psoriatic arthritis, who last spring went through a difficult period due to infection with the new coronavirus. After healing from COVID-19, the patients had returned to the hospital after a period of 4-6 months, to follow a rehabilitation treatment, the majority of the accusations being those related to psoriatic arthritis, with close follow-up of these patients in connection with the treatment applied. Material and methods. The patients were evaluated at hospitalization (biological inflammatory markers like CRP and ESR), pain scale, DAPSA score, PASI and the quality of life score (DLQI and QOL scale), after which they followed different rehabilitation treatments for a period of 21 days. After 3 months of completing this treatment they were re-evaluated. Patients diagnosed with psoriatic arthritis who do not have documentation to suggest SARS-CoV-2 infection (antibodies/ previous RT-PCR positive tests) were not included in the study. Outcomes. There were some significant differences in terms of the initial score at hospitalization and that performed after rehabilitation treatment. Most of the indices performed had lower values at reassessment (pain scale score, DAPSA, PASI, DLQI and even lower values of CRP and ESR), thus resulting in an important step in terms of the beneficial effects of rehabilitation therapy, both for patients with psoriatic arthritis and for post-COVID-19 recovery. The most important change was observed in the score for quality of life. Conclusions. The inclusion of rehabilitation therapy in patients with psoriatic arthritis should be a step that each patient should take. Its effects are long-term, with periods of pain decreasing in frequency and intensity, thus changing the quality of life of these patients. The mental, social and emotional impact of COVID-19 on people who have gone through the disease can be changed in a good way, also following a rehabilitation therapy.


Children ◽  
2021 ◽  
Vol 8 (12) ◽  
pp. 1122
Author(s):  
Sarah Achterrath ◽  
Teresa Kruse ◽  
Julia Neuschulz ◽  
Isabelle Graf ◽  
Joachim Zöller ◽  
...  

The therapy of patients with Crouzon syndrome involves a multidisciplinary team. In most cases, this therapy is extensive, time-consuming, and exhausting for the patient. This case report illustrates a temporally coordinated therapy plan that succeeds in reducing the burden of care. Showing typical extraoral characteristics of Crouzon syndrome, the patient had a frontal and left-sided crossbite, and impaction of the maxillary canines. Multidisciplinary therapy included the extraction of multiple teeth, midface distraction at Le Fort III level, and alignment of the impacted teeth. Before starting, during, and after completion of the treatment, the patient’s oral health-related quality of life was assessed using COHIP-19. The combination of different treatment steps significantly reduced the duration of therapy. The therapy improved not only the patient’s oro- and craniofacial function, but also the patient’s facial appearance in a short treatment period. The patient’s quality of life improved considerably during this time. In the treatment of severe craniofacial anomalies, the highest priority should be given to keeping the burden of care low. All measures should encourage young patients’ appropriate psychosocial development despite extensive therapies, ensuring at the same time medically satisfactory treatment results.


Author(s):  
Gita Rani ◽  
Amarjeet Gambhir

ABSTRACT Successful prosthetic reconstruction of hemimaxillectomy defects is a challenging procedure that requires multidisciplinary expertize to achieve acceptable function, speech and esthetics. Rehabilitation of an acquired maxillary defect improves the quality of life for the patient as close to normal as possible. Obturation of the defect depends on its volume and position of remaining hard and soft tissues, to be utilized for retention, stability and support for the prosthesis. The prosthesis should be simple to handle, easy to maintain, biocompatible, light in weight and convenient for future adjustments. This case report describes a clinical case of subtotal maxillectomy, which was successfully rehabilitated with a hollow bulb obturator. How to cite this article Rani G, Gambhir A. Prosthodontic Rehabilitation of a Patient with Subtotal Maxillectomy using a Hollow Bulb Obturator. Int J Prosthodont Restor Dent 2012; 2(1):24-28.


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