scholarly journals Long-Term Hydromethylthionine Treatment Is Associated with Delayed Clinical Onset and Slowing of Cerebral Atrophy in a Pre-Symptomatic P301S MAPT Mutation Carrier

2021 ◽  
pp. 1-7
Author(s):  
Peter Bentham ◽  
Roger T. Staff ◽  
Bjoern O. Schelter ◽  
Helen Shiells ◽  
Charles R. Harrington ◽  
...  
Keyword(s):  
Age Of Onset ◽  
Asymptomatic Carrier ◽  
Cerebral Atrophy ◽  
Temporal Lobes ◽  
Clinical Onset ◽  
The Family ◽  
Mapt Mutation ◽  
Tau Aggregation ◽  
P301s Mutation

One of the genetic mutations in the microtubule-associated protein tau, P301S, is causative for dominantly inherited frontotemporal dementia characterized by extensive tau pathology for which no licensed treatment is available. Hydromethylthionine is a potent tau aggregation inhibitor. We report treatment of an asymptomatic carrier of the P301S mutation using hydromethylthionine over a 5-year period beginning at the mean age of onset of clinical decline in the family. During the period of treatment, the rates of progression of cerebral atrophy were reduced by 61%–66%in frontal and temporal lobes, and the patient remained clinically asymptomatic.

Evaluation of the Laxative activity of Saponin Enriched Hydroethanolic Pericarp extract of Sapindus emarginatus in Animal models

2020 ◽  
Vol 16 ◽  
Author(s):  
Lalitha Vivekanandan ◽  
Roxanne Gekonge Mandere ◽  
Sivakumar Thangavel
Keyword(s):  
Animal Models ◽  
Gravimetric Analysis ◽  
Triterpene Saponins ◽  
Laxative Effect ◽  
Saponin Content ◽  
The Family ◽  
Dose Dependent Increase ◽  
Dose Dependent ◽  
Efficacious Drug

Background: Constipation is a common, predominant, chronic gastrointestinal functional disorder. The drugs available to treat constipation are limited because of their side effects in long term use. So we need of efficacious drug to treat constipation. Sapindus emarginatus Vahl belongs to the family Sapindaceae, commonly known as soapnut. Traditionally used for the antipruritic, antifertility, constipation, and anti-inflammatory agents. Objective: The present study was undertaken to evaluate the laxative activity of hydroethanolic pericarp extract of Sapindus emarginatus (HESE) in animal models. Methods: The saponin content in extract was measured by gravimetric analysis. The laxative activity of hydroethanolic pericarp extract of Sapindus emarginatus is evaluated by the weight of feces matter, charcoal meal hyperperistalsis test, and loperamide induced constipation model. Results: The saponin content of the soapnut pericarp was 13.48 % and the extract was found to be 11.92 %. The results obtained from these models showed a significant dose-dependent increase in fecal weight, peristalsis index, and moisture content compared to control animals. Conclusion: The present study concluded that the oral administration of HESE showed a significant laxative activity by using different animal models. The presence of triterpene saponins is responsible for this activity. Further studies are needed to confirm their mechanism behind the laxative effect. The administration of extract was found to be a valid candidate in constipation therapy.

Redescription and relationships of Pseudothilmanus Pic (Coleoptera: Rhagophthalmidae)—a long-term neglected glow-worm beetle genus from the Himalayas

Zootaxa ◽  
2011 ◽  
Vol 2794 (1) ◽  
pp. 57 ◽  
Author(s):  
ROBIN KUNDRATA ◽  
LADISLAV BOCAK
Keyword(s):  
Morphological Divergence ◽  
Diagnostic Characters ◽  
The Family

Pseudothilmanus Pic, 1918 is redescribed and two species, P. alatus Pic, 1918 and P. marginatus Pic, 1918, are placed in the genus. The subgenus Drilothilmanus Pic, 1918 is synonymized with the nominotypical genus on the basis of low morphological divergence and redundancy in classification. Pseudothilmanus is newly classified in the family Rhagophthalmidae, in contrast to the original placement in Drilidae. Diagnostic characters for Pseudothilmanus and both species placed within the genus are illustrated.

scholarly journals Are Family Firms More Levered? An Analysis of Family and Non-Family Firms

SAGE Open ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 215824402110223
Author(s):  
Jahanzaib Haider ◽  
Abdul Qayyum ◽  
Zalina Zainudin
Keyword(s):  
Family Firms ◽  
East Asian ◽  
Ordinary Least Squares ◽  
Maturity Structure ◽  
The Family ◽  
Asian Economies ◽  
The Board Of Directors ◽  
East Asian Economies ◽  
Combined Data

This study analyzes the leverage policies of the family and non-family firms of eight East Asian Economies (Hong Kong, Indonesia, Japan, Korea, Malaysia, Philippines, Singapore, and Taiwan) by using combined data of 690 family and non-family firms with 3,224 firm–years over the period 2006–2010. This study has used an ordinary least squares (OLS) regression for analyzing the data for the first question, while for the second question, logit regression has been used as the dependent variable (a binary variable). Prior research on family and non-family firms has revealed that family firms issue less (high) debt than non-family firms. Our analysis on a sample of East Asian Economies discloses that family firms have significantly different leverage levels than non-family firms, but their signs are not consistent. On the contrary, when the owner works as CEO/Chairman or member of the Board of Directors, then the family firms issue less debt than the non-family firms. Besides that, this study adds a new question that has not been addressed in the prior studies. The new question has focused on the speed of leverage adjustment. It is found that family firms and non-family firms regarding their debt maturity structure (short-term debt and long-term debt), the speed of leverage adjustments, and their decision to issue securities (i.e., debt vs. equity) are not significantly different. This study concluded that though family firms have a strong influence on each economy, but in South-East Asian countries, leverage policies of the family firms are not much different than that of non-family firms.

scholarly journals Fibromuscular dysplasia with recurrence after “long-term” following percutaneous transcatheter renal angioplasty: two case reports with a review of 26 patients

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Shuntaro Oribe ◽  
Takafumi Toyohara ◽  
Eikan Mishima ◽  
Takehiro Suzuki ◽  
Koichi Kikuchi ◽  
...  
Keyword(s):  
Fibromuscular Dysplasia ◽  
Age Of Onset ◽  
Case Reports ◽  
Case Presentation ◽  
Renal Angioplasty ◽  
Long Term Follow Up ◽  
Percutaneous Transluminal Renal Angioplasty ◽  
The Difference

Abstract Background Fibromuscular dysplasia (FMD) often causes renal artery stenosis with renovascular hypertension. Recent clinical outcomes encourage percutaneous transluminal renal angioplasty (PTRA) to treat FMD; however, the necessary follow-up period remains unclear. Moreover, previous studies have not revealed the difference in the period until recurrence between two major types of FMD—multifocal and focal. Case presentation We describe two patients with multifocal FMD who developed hypertension during their teenage years and had recurrence of FMD > 10 years after PTRA. We further examined the types of FMD and age of onset in 26 patients who underwent PTRA. The period until recurrence of multifocal FMD was longer than that of focal FMD. Moreover, patients with early-onset multifocal FMD are likely to have a delayed recurrence after PTRA compared to other types. Conclusions Our report suggests that patients with multifocal FMD, especially those with onset at an early age, may need long-term follow-up for at least ≥ 10 years.

scholarly journals HGG-47. DECREASED GROWTH VELOCITY WITH LONG TERM USE OF BRAFV600e AND MEK INHIBITION IN A PATIENT WITH ANAPLASTIC GANGLIOGLIOMA

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii352-iii352
Author(s):  
Hung Tran ◽  
Robert Cooper
Keyword(s):  
Growth Velocity ◽  
Direct Result ◽  
Chromosomal Microarray ◽  
Mek Inhibition ◽  
Follow Up Study ◽  
The Family ◽  
The Relationship

Abstract PURPOSE To describe decreased growth velocity with long term use of BRAFV600e and MEK inhibition in a patient with anaplastic ganglioglioma. RESULTS 4-year-old patient was found to have a 6 x 4.6 x 5 cm mass in the hypothalamus. Pathology consistent with anaplastic ganglioglioma and chromosomal microarray revealed a BRAFV600e mutation. Patient started on dabrafenib and trametinib and tumor decreased 85% after 3 months. She is stable without significant toxicities 39 months on therapy, and is now 8 years old. Patient had been growing at the 25% for weight and 12% for height but is now 65% for weight and 0.5% for height. It is difficult to tease out the relationship between the tumor, the location of the tumor, and the BRAF and MEK inhibitors and their effect on growth. Discussions with the family and endocrinology are ongoing but being <1% for height will lead to decrease in quality of life. CONCLUSIONS Further follow-up study is needed to determine if this is truly a long-term toxicity, or if this may just be a direct result of the location of the tumor. Would supplementation with growth hormone in this patient lead to losing control of a high grade tumor, or would it simply replace a hormone that is not produced?

‘Clinically definite benign multiple sclerosis’, an unwarranted conceptual hodgepodge: evidence from a 30-year observational study

2012 ◽  
Vol 19 (4) ◽  
pp. 458-465 ◽  
Author(s):  
E Leray ◽  
M Coustans ◽  
E Le Page ◽  
J Yaouanq ◽  
J Oger ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Observational Study ◽  
Medical Treatments ◽  
Clinical Onset ◽  
Disability Status ◽  
Relapsing Remitting ◽  
Long Term Follow Up ◽  
Benign Multiple Sclerosis ◽  
New Biomarkers

Background: Benign multiple sclerosis (BMS) is a controversial concept which is still debated. However identification of this kind of patients is crucial to prevent them from unnecessary exposure to aggressive and/or long term medical treatments. Objectives: To assess two definitions of ‘clinically definite benign multiple sclerosis’ (CDBMS) using long-term follow-up data, and to look for prognostic factors of CDBMS. Methods: In 874 patients with definite relapsing–remitting MS, followed up for at least 10 years, disability was assessed using the Disability Status Scale (DSS). CDBMS was defined by either DSS score≤2 (CDBMS1 group) or DSS score≤ 3 (CDBMS2 group) at 10 years. We estimated the proportion of patients who were still benign at 20 and 30 years after clinical onset. Results: CDBMS frequency estimates were 57.7% and 73.9% when using CDBMS1 and CDBMS2 definitions, respectively. In the CDBMS1 group, only 41.7% (105/252) of cases were still benign 10 years later, and 41.1% (23/56) after an additional decade, while there were 53.8% (162/301) and 59.5% (44/74) respectively in the CDBMS2 group. Conclusions: This 30-year observational study, which is one of the largest published series, indicates that favourable 10-year disability scores of DSS 2 or 3 fail to ensure a long-term benign course of multiple sclerosis. After every decade almost half of the CDBMS were no longer benign. CDBMS, as currently defined, is an unwarranted conceptual hodgepodge. Other criteria using new biomarkers (genetic, biologic or MRI) should be found to detect benign cases of MS.

ANOREXIA NERVOSA

PEDIATRICS ◽  
1982 ◽  
Vol 70 (4) ◽  
pp. 525-525
Author(s):  
Robert J. Haggerty
Keyword(s):  
Anorexia Nervosa ◽  
Early Onset ◽  
Age Of Onset ◽  
Outcome Studies ◽  
Psychosomatic Disorder ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Follow Up Studies

Anorexia nervosa is a serious psychosomatic disorder which most typically begins between 16 and 18 years of age. Clinicians have often held that early onset (eg, ages 11 to 15) is associated with a better outcome. This paper reviews the long-term outcome studies on anorexia nervosa and concludes that this contention is not supported by available data. The methodologies of seven outcome studies that focus on an early onset population are critiqued, and it is concluded that two methodologies are strong. Because of the increasing prevalence of anorexia nervosa, this once rare disorder can now be more easily investigated, and consequently better follow-up studies, which examine potential prognostic factors including age of onset, should be forthcoming.

Behcet Disease: Long-term Follow-up of Three Children and Review of the Literature

PEDIATRICS ◽  
1989 ◽  
Vol 83 (6) ◽  
pp. 986-992
Author(s):  
Yardena Rakover ◽  
Hanna Adar ◽  
Itamar Tal ◽  
Yaron Lang ◽  
Amos Kedar
Keyword(s):  
Fever Of Unknown Origin ◽  
Age Of Onset ◽  
Unknown Origin ◽  
Behçet Disease ◽  
Behcet Disease ◽  
Cns Involvement ◽  
Course Of Illness ◽  
Long Term Follow Up

Behcet disease is rare in children. There are only two reports of Behcet disease in childhood, describing seven patients. Three pediatric patients are described, in whom the age of onset ranged from 6 to 11 years. Aphthous stomatitis and arthritis were present in all of the patients; genital ulcers, iridocylitis, erythema nodosum, and CNS involvement were present in two patients. Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil. In two patients, follow-up of more than 10 years was done, with complete cure in one patient and benign course of illness in the other. Because of the rarity of the disease in childhood and the difficulty in making the diagnosis, there is not enough awareness by pediatricians concerning this disease.

Counseling Families Who Have a Child with a Severe Congenital Anomaly

PEDIATRICS ◽  
1981 ◽  
Vol 67 (3) ◽  
pp. 321-324 ◽  
Author(s):  
Norman Fost
Keyword(s):  
Congenital Anomaly ◽  
Congenital Malformation ◽  
Conflicts Of Interest ◽  
Major Congenital Malformation ◽  
Legal Implications ◽  
The Family

The birth of an infant with a major congenital malformation is experienced by the family as a calamity. Parents have an urgent need for compassionate and skilled attention, and a long-term need for counseling to help them adapt to the crisis. The difficult ethical and legal implications of such cases further complicate the doctor's ability to care for the patient and family. Potential conflicts of interest have recently led to the use of voluntary consultation by hospital committees, or obligatory involvement by the courts.

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