KLHL40-related nemaline myopathy with a sustained, positive response to treatment with acetylcholinesterase inhibitors

2016 ◽  
Vol 263 (3) ◽  
pp. 517-523 ◽  
Author(s):  
D. Natera-de Benito ◽  
A. Nascimento ◽  
A. Abicht ◽  
C. Ortez ◽  
C. Jou ◽  
...  
Keyword(s):  
Positive Response ◽  
Acetylcholinesterase Inhibitors ◽  
Nemaline Myopathy ◽  
Response To Treatment

scholarly journals Unusual presentation of vitamin D3-dependent rickets type II in a kitten

2020 ◽  
Vol 6 (1) ◽  
pp. 205511692091027
Author(s):  
Florent Duplan ◽  
Christina Maunder
Keyword(s):  
Vitamin D ◽  
Positive Response ◽  
Abdominal Distension ◽  
Response To Treatment ◽  
Type I ◽  
Type Ii ◽  
Different Types ◽  
History Of ◽  
Radiographic Changes

Case summary A 15-week-old kitten presented with a 1-month history of intermittent generalised tremors and abdominal distension. Hypocalcaemia associated with increased 1,25-vitamin D3 was consistent with vitamin D3-dependent rickets type II. The bone appearance on CT scan was most consistent with the changes typically seen with nutritional secondary hyperparathyroidism and less typical of the changes seen with rickets. Our patient had a positive response to high vitamin D3 therapy as it remained normocalcaemic 16 months after diagnosis, supporting the diagnosis of rickets. Relevance and novel information This case report is an unusual and interesting presentation of rickets in a kitten. Despite the characteristic vitamin D3 disturbance for rickets type II, the atypical radiographic changes have not been previously reported. In the literature, a positive response to treatment is not commonly seen or follow-up is short. Our case responded well to treatment and was followed for 16 months from the time of diagnosis. This emphasises that the pathophysiology of the condition is not well understood, and that different types of vitamin D3-dependent rickets type II may exist. Although the genetic defects responsible for some cases of rickets type I have been identified, this has still not been determined for rickets type II.

When a Patient Presents With a Present: Quantitative and Qualitative Assessment of Gifts Given to Psychiatrists

2012 ◽  
Vol 13 (3) ◽  
pp. 209-228
Author(s):  
Richard M. Patel ◽  
Raquel Miller
Keyword(s):  
Positive Response ◽  
Low Cost ◽  
Qualitative Assessment ◽  
Response To Treatment ◽  
Mental Health Providers ◽  
Health Providers ◽  
Gift Giving ◽  
Important Data ◽  
Boundary Violation ◽  
Case Vignettes

Objective: This article reviews the issue of patients giving gifts to psychiatrists and mental health providers. Method: Anonymous survey of 100 academic psychiatrists measured prevalence of receiving gifts, type and estimated dollar value of gifts given, and psychiatrists’ reactions to gifts. Case vignettes illustrate clinical situations associated with gift giving and how failure to recognize motivation of gift giving may lead to situations requiring immediate intervention. Results: 71% of psychiatrists surveyed received (were offered & accepted) at least one gift in prior year (average 0.36 per month and 3.6 per year; $11.40 average [estimated] amount per gift). Group comparisons achieving at least a p < 0.05 significance: outpatient psychiatrists received gifts twice as often as inpatient, female and outpatient groups’ gifts were estimated as more expensive, a positive correlation was found between psychiatrists receiving gifts and psychiatrists giving a positive response to gifts, there was significantly more negative responses to high cost gifts (>$100) than to low cost (<$20), and outpatient psychiatrists reported interpreting gift’s meaning more often than inpatient. Conclusions: Psychiatrists are commonly offered and accept gifts from patients. Gifts communicate patient information and response to treatment. Although the act of gift giving sends important data to the receiving psychiatrist, including boundary violation issues, there are no agreed upon guidelines regarding how to respond. Future study should explore the meanings and appropriateness of a gift regarding type, cost, timing, frequency, intent, as well as how providers can respond to the gesture.

scholarly journals First Report of Psoriatic-Like Dermatitis and Arthritis in a 4-Year-Old Female Spayed Pug Mix

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Stephanie A. Regan ◽  
Rosanna Marsella ◽  
Ibrahim Ozmen
Keyword(s):  
Case Report ◽  
Positive Response ◽  
Treatment Options ◽  
Domestic Animals ◽  
Preliminary Evidence ◽  
Clobetasol Propionate ◽  
Response To Treatment ◽  
History Of ◽  
Control Pain ◽  
Tentative Diagnosis

Psoriasis manifests as chronic dermatitis and arthritis (PsA) in people. Psoriasis with concurrent PsA is characterized by erythematous, silvery, scaly plaques, especially on the extremities, and concurrent arthritis with enthesitis, tenosynovitis, and dactylitis. To date, no such disease has spontaneously occurred in domestic animals. This case report aims to describe the clinical, radiographic, and histologic appearance of a psoriasis-like dermatitis and psoriatic-like arthritis in a dog. A 4-year-old female spayed pug mix presented for the evaluation of chronic history of hyperkeratotic footpads and deforming arthritis. After ruling out other differential diagnoses and based on the similarity of clinical, radiographic, and histologic findings to human psoriasis and PsA, a tentative diagnosis of psoriasis-like disease was made. Treatment was begun to control pain (tramadol, gabapentin, and carprofen) and psoriatic dermatitis (clobetasol propionate 0.05%, calcipotriene 0.005%, and urea 40% ointment twice daily). Dramatic positive response to treatment was achieved confirming the tentative diagnosis. This case may provide preliminary evidence for the existence of a psoriasis-like condition in dogs and may elucidate treatment options in otherwise refractory cases of chronic dermatitis and polyarthropathy in dogs.

scholarly journals Clinical practice of managing corneal ulcer of unknown etiology with pro-tracted course in presence of toxic and allergic component

2021 ◽  
pp. 58-65
Author(s):  
D.Y. Maychuk ◽  
◽  
A. A. Tarkhanova ◽  
A.O. Loshkareva ◽  
I. A. Drozdkov ◽  
...  
Keyword(s):  
Allergic Reaction ◽  
Corneal Ulcer ◽  
Positive Response ◽  
Antifungal Drugs ◽  
Response To Treatment ◽  
Unknown Etiology ◽  
The Subject ◽  
Long Term Observation ◽  
Antibacterial And Antifungal

Purpose. The representation of the clinical case of corneal ulcer course with unknown etiology in presence of patient's acute toxic and allergic reaction. The patient was under long-term observation of the local ophthalmologist and didn't show positive response to treatment. Material and methods. Patient examination on the date of his encounter sug-gested bacterial etiology of the process. However, the etiological factor wasn't re-vealed by laboratory tests. The presence of toxic and allergic components in biomi-croscopy of both eyes, long-term prestudy antibacterial therapy, character of corneal ulcer gave rise to prescription of the therapy. Results. The main areas of the therapy are allergy medications, antibacterial and antifungal drugs. The positive response in the disease course allowed us to be-lieve that the treatment was accurately prescribed Conclusion. Protracted course of the corneal ulcer without positive response is the subject to estimate the presence of toxic and allergic components. Considering the duration of the disease the antifungal drugs were prescribed at the beginning of the therapy, in spite of the assumption that bacterial etiology of the process may take place. Keywords: corneal ulcer, toxic and allergic reaction.

scholarly journals Study the effect of Aqueous cold water and Alcoholic extracts of Ziziphus spina christi against bacteria isolated from Conjunctivitis In vitro and In vivo

2015 ◽  
Vol 12 (1) ◽  
pp. 14-19
Author(s):  
Baghdad Science Journal
Keyword(s):  
Streptococcus Pyogenes ◽  
Positive Response ◽  
Cold Water ◽  
Response To Treatment ◽  
Distilled Water ◽  
Alcoholic Extract ◽  
Laboratory Mice ◽  
Significant Superiority

This study aimed to study the effect of Ziziphus spina christi Aqueous cold and Alcoholic leaves and fruits extracts on the growth and activities of the following types of bacteria :( Escherichia coli, Pseudomonas aeruginosa, Staphylococcus aureus and Streptococcus pyogenes ). The results appeared outweigh the alcoholic extract of leaves and fruits of Sidr that prepared by saxholate extractor by addition of ethanol 95% significant superiority as compared with aqueous extract that prepared by using distilled water as was its influence inhibitor to the growth and effectiveness of bacteria , about the treatment of in-vivo to cause injury to these types of bacteria diagnosed laboratory mice and treated with alcoholic extract of the leaves of Sidr highest concentration of inhibitor Tests showed a positive response to treatment.

scholarly journals Efficacy of Zonisamide in Migraineurs with Nonresponse to Topiramate

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Jin-Young Chung ◽  
Min-Wook Kim ◽  
Manho Kim
Keyword(s):  
Adverse Effects ◽  
Migraine Without Aura ◽  
Positive Response ◽  
Migraine Prophylaxis ◽  
Response To Treatment ◽  
Single Center ◽  
Potential Effectiveness ◽  
Single Center Study ◽  
Headache Patients ◽  
Center Study

This study investigated another type of carbonic anhydrase inhibitor and antiepileptic drug, zonisamide, in order to evaluate its potential effectiveness for migraine prophylaxis refractory to topiramate, and to assess intolerability to adverse events, paresthesia in particular. This is an open-labeled retrospective single center study. We included headache patients who met the requirement of migraine without aura and were refractory to topiramate. Patients were treated only with zonisamide 100 mg/day, directly switching from topiramate. Patients were monitored every month for three months. A positive response to treatment (responders) was defined as a 50% or greater reduction in headache days at three months after study commencement, compared with baseline. One hundred and twenty migraineurs who were refractory to topiramate were recruited. Compared with baseline, headache days with zonisamide showed a decrease, compared with baseline (P<0.01). Two patients complained of adverse effects, such as paresthesia. These results suggest that zonisamide is effective for migraine prophylaxis refractory to topiramate, or intolerable patients due to topiramate-induced paresthesia.

scholarly journals Demographic and clinical predictors of response to internet-enabled cognitive–behavioural therapy for depression and anxiety

BJPsych Open ◽  
2018 ◽  
Vol 4 (5) ◽  
pp. 411-418 ◽  
Author(s):  
Ana Catarino ◽  
Sarah Bateup ◽  
Valentin Tablan ◽  
Katherine Innes ◽  
Stephen Freer ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Cognitive Behavioural Therapy ◽  
Mental Health Problems ◽  
Positive Response ◽  
Behavioural Therapy ◽  
Response To Treatment ◽  
The Internet ◽  
Clinical Predictors ◽  
Predictors Of Response ◽  
Cognitive Behavioural

BackgroundCommon mental health problems affect a quarter of the population. Online cognitive–behavioural therapy (CBT) is increasingly used, but the factors modulating response to this treatment modality remain unclear.AimsThis study aims to explore the demographic and clinical predictors of response to one-to-one CBT delivered via the internet.MethodReal-world clinical outcomes data were collected from 2211 NHS England patients completing a course of CBT delivered by a trained clinician via the internet. Logistic regression analyses were performed using patient and service variables to identify significant predictors of response to treatment.ResultsMultiple patient variables were significantly associated with positive response to treatment including older age, absence of long-term physical comorbidities and lower symptom severity at start of treatment. Service variables associated with positive response to treatment included shorter waiting times for initial assessment and longer treatment durations in terms of the number of sessions.ConclusionsKnowledge of which patient and service variables are associated with good clinical outcomes can be used to develop personalised treatment programmes, as part of a quality improvement cycle aiming to drive up standards in mental healthcare. This study exemplifies translational research put into practice and deployed at scale in the National Health Service, demonstrating the value of technology-enabled treatment delivery not only in facilitating access to care, but in enabling accelerated data capture for clinical research purposes.Declaration of interestA.C., S.B., V.T., K.I., S.F., A.R., A.H. and A.D.B. are employees or board members of the sponsor. S.R.C. consults for Cambridge Cognition and Shire. Keywords: Anxiety disorders; cognitive behavioural therapies; depressive disorders; individual psychotherapy

scholarly journals Prognostic value of serum erythropoietin in the correction of anemia using recombinant erythropoietin drugs in patients with lymphoproliferative diseases

2012 ◽  
Vol 93 (4) ◽  
pp. 584-590 ◽  
Author(s):  
N A Romanenko ◽  
M V Berkos ◽  
S S Bessmeltsev ◽  
N A Potikhonova ◽  
K M Abdulkadyrov
Keyword(s):  
Positive Response ◽  
Hemoglobin Level ◽  
Response To Treatment ◽  
Control Group ◽  
Recombinant Erythropoietin ◽  
Epoetin Alfa ◽  
Lymphoproliferative Diseases ◽  
Endogenous Erythropoietin ◽  
Serum Erythropoietin ◽  
Erythropoietin Level

Aim. To evaluate the prognostic significance of the baseline content of endogenous erythropoietin in the blood serum of patients with lymphoproliferative diseases while using recombinant erythropoietin drugs to correct the anemia. Methods. 69 patients with lymphoproliferative disorders (chronic lymphocytic leukemia, indolent forms of non-Hodgkins lymphoma and multiple myeloma) in combination with anemia, ages from 24 to 81 years, were under our observation. Treatment with recombinant erythropoietin was performed in the first group (48 patients), the second (control) group consisted of 21 patients without treatment with recombinant erythropoietin. In all patients the hemoglobin concentration was reduced to 37-100 g/l. Results. A positive response to treatment with recombinant erythropoietin for 6-16 weeks was noted in 30 out of 48 patients treated with epoetin alfa (62.5%, p 0.05). The monthly increase in hemoglobin level was 19.1±14.4 g/l, while in patients who were not responding to therapy - 2.1±4.3 g/l. In the control group of patients not receiving recombinant erythropoietin drugs the monthly increase in hemoglobin level was 2.0±4.4 g/l, which is the same as in the group who did not respond to recombinant erythropoietin and is significantly lower (p 0.01), than in patients with a positive response. In the control group, the increase in hemoglobin level 20 g/l during 20 weeks was observed only in 5 of 21 (23.4%) patients. A reduced level of endogenous erythropoietin was detected in 28 (58.3%) patients, while increased and correlating with the severity of anemia was detected in 20 (41.7%) patients. An inverse correlation was established between the positive response to treatment with recombinant erythropoietin and the baseline endogenous erythropoietin (r=-0.36, n=48, p 0,05). The positive effect of epoetin alfa was observed more frequently in patients with low serum erythropoietin level [22 of 28 (78.6%) patients responded, p 0.01], than in patients with high erythropoietin level [only 8 out of 20 (40%) patients responded to treatment, p 0.05]. Conclusion. Low concentration of endogenous erythropoietin suggests a positive response to recombinant erythropoietin products and can therefore be used as one of the predictors of response; in cases of high (above 500 mIU/ml) levels of endogenous erythropoietin the response occurs very rarely; an intermediate level of erythropoietin (130-500 mIU/ml) may also be justified for the administration of recombinant erythropoietin.

scholarly journals Laryngopharyngeal Reflux: Symptoms, Signs, and Presence of Pepsin in Saliva - A Reliable Diagnostic Triad

2020 ◽  
Author(s):  
Shilpa Divakaran ◽  
Sivaa Rajendran ◽  
Roshan Marie Thomas ◽  
Jaise Jacob ◽  
Mary Kurien
Keyword(s):  
Positive Response ◽  
Laryngopharyngeal Reflux ◽  
Ph Monitoring ◽  
Tertiary Care ◽  
Acid Reflux ◽  
Response To Treatment ◽  
Care Hospital ◽  
Reflux Symptom Index ◽  
Symptom Index ◽  
Chi Squared

Abstract Introduction Twenty-four-hour multichannel intraluminal impedance with double probe pH monitoring (MII-pH), though considered the most sensitive tool for the diagnosis of gastroesophageal reflux disease (GERD), is invasive, time consuming, not widely available, and unable to detect non-acid reflux. In contrast, the presence of pepsin in the saliva would act as a marker for reflux, considering that pepsin is only produced in the stomach. Objective To evaluate the predictive value of salivary pepsin in diagnosing laryngopharyngeal reflux (LPR) as suggested by the results of reflux symptom index (RSI > 13), reflux finding score (RFS > 7), and positive response to treatment with a 4-week course of proton-pump inhibitors. Methods This prospective study was done at a tertiary care hospital on 120 adult patients attending ENT OPD with clinical diagnosis of LPR. The presence of pepsin in their pharyngeal secretions and saliva using a lateral flow device, the Peptest, was compared with RSI, RFS, and with the response to medical treatment using the Chi-squared test. Results Salivary pepsin was found to be positive in 68% of the patients, with 87.5% of them showing positive response to treatment. Chi-squared analysis showed a significant association between positive salivary pepsin and RFS > 7, RSI >13, a combination of RFS > 7 and RSI > 13 as well as with response to treatment (p < 0.0001). Conclusion When considered along with the clinical indicators of RFS and RSI of more than 7 and 13, respectively, and/or with a response to treatment, a positive salivary pepsin test indicates statistically significant chance of presence of LPR.

scholarly journals CIDP Diagnostic Criteria and Response to Treatment

2020 ◽  
Vol 1 (2) ◽  
pp. 7-10
Author(s):  
Charles Roach ◽  
Mazen Dimachkie ◽  
Jeffrey Statland ◽  
Richard Barohn ◽  
Omar Jawdat ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Chart Review ◽  
Response Rate ◽  
Positive Response ◽  
Retrospective Chart Review ◽  
Response To Treatment ◽  
Ivig Treatment ◽  
University Of Kansas ◽  
Treatment Responses ◽  
The University

AbstractIntroduction: Diagnostic criteria for CIDP have been proven useful for clinical trials. However, use of these criteria in clinics has been limited by time constraints and unknown usefulness in predicting outcomes. Methods: A retrospective chart review of CIDP patients at the University of Kansas seen between 2008 and 2014 was performed. We determined the diagnostic criteria fulfilled by each patient and assessed treatment responses. A positive response was defined by improvement sensory or motor examination as determined by a neuromuscular physician.Results: There were 38 total patients included in the study. The response rate to IVIG in patients who fulfilled EFNS/PNS criteria was 20/22 (90.1%). Among patients who fulfilled AAN criteria, 8/9 (88.9%) responded positively to IVIG. Slightly lower response rates were seen in patients fulfilling INCAT criteria and Saperstein criteria at 10/15 (66.7%) and 12/17 (70.6%), respectively.Discussion: EFNS/PNS and AAN criteria can similarly predict IVIG treatment response.

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