scholarly journals LINC-17. SIROLIMUS AS AN ALTERNATIVE TO SURGICAL RESECTION OF PEDIATRIC TUBEROUS SCLEROSIS COMPLEX-ASSOCIATED BILATERAL SUBEPENDYMAL GIANT CELL ASTROCYTOMAS: AN AFFORDABLE OPTION FOR PATIENTS FROM LOW-MIDDLE INCOME COUNTRIES

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii381-iii381
Author(s):  
Patricia Orduña
Keyword(s):  
Intracranial Pressure ◽  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Surgical Resection ◽  
Tuberous Sclerosis Complex ◽  
Obstructive Hydrocephalus ◽  
Middle Income ◽  
Continuous Growth ◽  
Middle Income Countries ◽  
Low Middle Income Countries

Abstract Subependymal giant cell astrocytomas (SEGA) may lead to significant neurological morbidity in children diagnosed with tuberous sclerosis complex (TSC). Surgical resection is warranted for SEGAs demonstrating continuous growth, causing hydrocephalus and increased intracranial pressure. mTOR inhibitors (sirolimus and everolimus) are alternatives to surgery and have shown efficacy in stabilizing and shrinking SEGAs. Everolimus showed stronger evidence in efficacy, but its cost poses a limitation for this treatment among patients from low-middle income countries. We explored sirolimus as a potentially more cost-effective alternative in our setting. We present a 10-year-old Filipino child with TSC admitted due to headache, vomiting, and increased sleeping time. Neuroimaging revealed large bilateral SEGAs involving the frontal horns and foramina of Monro, causing moderate obstructive hydrocephalus. Surgical excision was offered, but parents opted for medical treatment. Bilateral posterior parietal ventriculoperitoneal shunts were inserted to decrease intracranial pressure. Due to the cost of everolimus, the patient was started on sirolimus at 1mg/m2/day. Imaging done 6 months after initiating therapy demonstrated significant decrease in size of both SEGAs (right: 82.5%, left: 64.1%). Sirolimus levels were maintained at 15.7ng/ml and minimal elevations on cholesterol and triglyceride levels were observed and treated with simvastatin. Results of this case and review of related data suggest that sirolimus can be used as a conservative approach in inducing regression of large bilateral SEGAs, and an affordable alternative to everolimus for pediatric TSC patients from low-middle income countries. Prospective studies and clinical trials are needed to further establish its efficacy, safety and cost-effectiveness in our setting.

The natural history of subependymal giant cell astrocytomas in tuberous sclerosis complex: a review

2018 ◽  
Vol 29 (3) ◽  
pp. 295-301 ◽  
Author(s):  
Denise L. Chan ◽  
Tessa Calder ◽  
John A. Lawson ◽  
David Mowat ◽  
Sean E. Kennedy
Keyword(s):  
Natural History ◽  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Tuberous Sclerosis Complex ◽  
Obstructive Hydrocephalus ◽  
Growth Patterns ◽  
Imaging Characteristics ◽  
Life Threatening ◽  
History Of ◽  
Mtor Activity

AbstractTuberous sclerosis complex (TSC) is an auto-somal-dominant inherited condition with an incidence of approximately 1:6000 births, characterised by deregulated mTOR activity with multi-site hamartomas. Subependymal giant cell astrocytomas (SEGA) are one such hamartoma, affecting up to 24% of patients with TSC. Their intraventricular location may lead to life-threatening obstructive hydrocephalus. Current management is hampered by a lack of understanding regarding the natural history, behaviour and growth patterns of SEGA. We review the current literature to summarise what is known about SEGA in the following areas: (1) diagnostic criteria, (2) prevalence, (3) origin, (4) imaging characteristics, (5) growth rate, (6) genotype-phenotype correlation, (7) congenital SEGA and (8) SEGA as a marker of severity of other TSC manifestations.

scholarly journals Experience using mTOR inhibitors for subependymal giant cell astrocytoma in tuberous sclerosis complex at a single facility

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Kyoichi Tomoto ◽  
Ayataka Fujimoto ◽  
Chikanori Inenaga ◽  
Tohru Okanishi ◽  
Shin Imai ◽  
...  
Keyword(s):  
Adverse Effects ◽  
Tuberous Sclerosis ◽  
Giant Cell ◽  
Malignant Transformation ◽  
Surgical Resection ◽  
Tuberous Sclerosis Complex ◽  
Mtor Inhibitors ◽  
Subependymal Giant Cell Astrocytoma ◽  
Acute Hydrocephalus ◽  
Giant Cell Astrocytoma

Abstract Background Subependymal giant cell astrocytoma (SEGA) is occasionally seen in tuberous sclerosis complex (TSC). Two main options are currently available for treating SEGA: surgical resection or pharmacotherapy using mammalian target of rapamycin inhibitors (mTORi). We hypothesized that opportunities for surgical resection of SEGA would have reduced with the advent of mTORi. Methods We retrospectively reviewed the charts of patients treated between August 1979 and July 2020, divided into a pre-mTORi era group (Pre-group) of patients treated before November 2012, and a post-mTORi era group (Post-group) comprising patients treated from November 2012, when mTORi became available in Japan for SEGA. We compared groups in terms of treatment with surgery or mTORi. We also reviewed SEGA size, rate of acute hydrocephalus, recurrence of SEGA, malignant transformation and adverse effects of mTORi. Results In total, 120 patients with TSC visited our facility, including 24 patients with SEGA. Surgical resection was significantly more frequent in the Pre-group (6 of 7 patients, 86 %) than in the Post-group (2 of 17 patients, 12 %; p = 0.001). Acute hydrocephalus was seen in 1 patient (4 %), and no patients showed malignant transformation of SEGA. The group treated using mTORi showed significantly smaller SEGA compared with the group treated under a wait-and-see policy (p = 0.012). Adverse effects of pharmacotherapy were identified in seven (64 %; 6 oral ulcers, 1 irregular menstruation) of the 11 patients receiving mTORi. Conclusions The Post-group underwent surgery significantly less often than the Pre-group. Since the treatment option to use mTORi in the treatment of SEGA in TSC became available, opportunities for surgical resection have decreased in our facility.

The Impact of Covid 19 on Pregnant Women and Child Health

2020 ◽  
Vol 11 (SPL1) ◽  
pp. 1367-1373
Author(s):  
Nikhil Sanjay Mujbaile ◽  
Smita Damke
Keyword(s):  
Social Issues ◽  
Middle Income ◽  
Web Based ◽  
Middle Income Countries ◽  
Low Middle Income Countries ◽  
The Social ◽  
Social Separation ◽  
The Impact ◽  
And Child Health ◽  
General Wellbeing

The Covid illness (COVID-19) pandemic has spread rapidly all through the world and has had a drawn-out impact. The Pandemic has done incredible damage to society and made genuine mental injury to numerous individuals. Mental emergencies frequently cause youngsters to deliver sentiments of relinquishment, despondency, insufficiency, and fatigue and even raise the danger of self-destruction. Youngsters with psychological instabilities are particularly powerless during the isolate and colonial removing period. Convenient and proper assurances are expected to forestall the event of mental and social issues. The rising advanced applications and wellbeing administrations, for example, telehealth, web-based media, versatile wellbeing, and far off intuitive online instruction can connect the social separation and backing mental and conduct wellbeing for youngsters. Because of the mental advancement qualities of youngsters, this investigation additionally outlines intercessions on the mental effect of the COVID-19 Pandemic. Further difficulties in Low Middle-Income Countries incorporate the failure to actualize successful general wellbeing estimates, for example, social separating, hand cleanliness, definitive distinguishing proof of contaminated individuals with self-disconnection and widespread utilization of covers The aberrant impacts of the Pandemic on youngster wellbeing are of extensive concern, including expanding neediness levels, upset tutoring, absence of admittance to the class taking care of plans, decreased admittance to wellbeing offices and breaks in inoculation and other kid wellbeing programs. Kept tutoring is critical for kids in Low Middle-Income Countries. Arrangement of safe situations is mainly testing in packed asset obliged schools. 

Risk Factors of Mortality Among Patients with Stroke in Low Middle-Income Countries:  A Series of 180 Patients

2020 ◽  
Author(s):  
Larrey Kamabu ◽  
Hervé Monka Lekuya ◽  
Bienvenu Muhindo Kasusula ◽  
Nicole Kavugho Mutimani ◽  
Louange Maha Kathaka ◽  
...  
Keyword(s):  
Risk Factors ◽  
Middle Income ◽  
Middle Income Countries ◽  
Low Middle Income Countries

scholarly journals Triaging and adaptations of surveillance of cancer services in the COVID pandemic

2020 ◽  
pp. 1-3
Author(s):  
Shirley Lewis ◽  
Lavanya Gurram ◽  
Umesh Velu ◽  
Krishna Sharan
Keyword(s):  
Cancer Patients ◽  
Clinical Examination ◽  
Cancer Care ◽  
Middle Income ◽  
Face To Face ◽  
Middle Income Countries ◽  
Cancer Services ◽  
Low Middle Income Countries

Abstract Introduction: Coronavirus disease (COVID-19) has significantly challenged the access to cancer care and follow-up for a patient with cancer. Methods: Based on published literature and our experiences, it is reasonable to presume that clinical examination and follow-up visits have been significantly curtailed worldwide in order to adhere to the new norms during the pandemic. Although telephonic and telemedicine consultations may help bridge a few gaps, completely dispensing with in-person consultation has its challenges, especially in low middle-income countries. Telephonic consultations could facilitate triaging of ambulatory cancer patients and allocation of face-to-face consultations for high priority patients. Conclusions: We propose a telephonic consultation-based triaging approach for ambulatory cancer patients in order to identify those needing in-hospital consultations.

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