scholarly journals Long-term outcomes of primarily metastatic juvenile pilocytic astrocytoma in children

2018 ◽  
Vol 21 (1) ◽  
pp. 49-53 ◽  
Author(s):  
Derek Yecies ◽  
Paul Graham Fisher ◽  
Samuel Cheshier ◽  
Michael Edwards ◽  
Gerald Grant

OBJECTIVEPrimarily metastatic juvenile pilocytic astrocytoma (JPA) is rare, likely representing 2%–3% of all cases of JPA. Due to the rarity of primarily metastatic JPA, there is currently no standard treatment paradigm and the long-term outcomes are not fully known. The goal of this case series was to add to the current understanding of this disease process.METHODSThe authors searched a comprehensive database of pediatric patients with brain and spinal cord tumors treated at Lucile Packard Children’s Hospital from 1997 to 2016 and identified 5 patients with primarily metastatic JPA. A retrospective chart review was performed and details of the patients’ treatment and clinical course were recorded for further analysis.RESULTSFor the 5 patients with primarily metastatic JPA, the mean follow-up period was 12.3 years. All patients in our series had biopsies or subtotal resections and upfront treatment. Three patients were treated with chemotherapy alone, one was treated with chemotherapy and radiotherapy, and one was treated with radiotherapy alone. Four patients had stable disease after initial treatment, and one patient had multiple episodes of progressive disease but underwent successful salvage therapy and has had stable disease for 19 years. One patient died of an intracerebral hemorrhage 10 years following initial radiation treatment believed to be secondary to radiation vasculopathy.CONCLUSIONSEvaluation of the entire neuraxis should be performed in all instances of initial JPA diagnosis to properly assess for primarily metastatic disease. Many patients with primarily metastatic JPA will have stable disease after upfront treatment, although the higher rate of stable disease found in this series relative to other reports is likely secondary to the small sample size.

Author(s):  
Marianna Rita Stancampiano ◽  
Kentaro Suzuki ◽  
Stuart O’Toole ◽  
Gianni Russo ◽  
Gen Yamada ◽  
...  

Abstract In the newborn, penile length is determined by a number of androgen dependent and independent factors. The current literature suggests that there are inter-racial differences in stretched penile length in the newborn and although congenital micropenis should be defined as a stretched penile length of less than 2.5SDS of the mean for the corresponding population and gestation, a pragmatic approach would be to evaluate all boys with a stretched penile length below 2 cm, as congenital micropenis can be a marker for a wide range of endocrine conditions. However, it remains unclear as to whether the state of micropenis, itself, is associated with any long-term consequences. There is a lack of systematic studies comparing the impact of different therapeutic options on long-term outcomes, in terms of genital appearance, quality of life and sexual satisfaction. To date, research has been hampered by a small sample size and inclusion of a wide range of heterogeneous diagnoses; for these reasons, condition specific outcomes have been difficult to compare between studies. Lastly, there is a need for a greater collaborative effort in collecting standardized data so that all real-world or experimental interventions performed at an early age can be studied systematically into adulthood.


2020 ◽  
Vol 11 (5) ◽  
pp. 611-618
Author(s):  
Maria Rodriguez ◽  
Anahita Malvea ◽  
Dayre McNally ◽  
Vid Bijelic ◽  
Ming Guo ◽  
...  

Background: Pediatric aortic root dilatation is a life-threatening condition that lacks guidelines for surgical management. We aimed to analyze the data on aortic valve interventions during root surgery to guide decision-making. Methods: A search was performed of MEDLINE, Embase, CENTRAL, ClinicalTrials.gov , and WHO ICTRP. Citations were screened in duplicate and independently to identify randomized controlled trials, cohorts, and case series involving populations aged 0 to 18 years, who received valve-sparing and valve-replacing aortic root surgeries between 1999 and 2019. Outcomes considered included mortality (perioperative, one year, five year), reintervention rates. Results: After duplicate removal, 689 citations were screened through abstract and full text review, identifying five eligible studies. All five were observational studies evaluating valve-sparing procedures. There were 81 patients with a mean study age range of 9.9 to 13.9 years. Both reimplantation (74%) and remodeling (26%) subtypes were done. Range of mean duration of follow-up was 1.2 to 4.4 years. There was no mortality reported until the one-year follow-up period. The long-term mortality rate was calculated as 0.02 per patient-year (95% CI: 0.01-0.05). The long-term reintervention rate was 0.08 per patient-year (95% CI: 0.05-0.13). Conclusions: There is limited experience on aortic valve intervention during aortic root surgery in children. Single-arm studies on valve-sparing surgeries show excellent survival up to one year. Mortality and reintervention rates increase in the longer term. The small sample size and lack of controlled studies do not allow for direct comparisons between procedure types.


2016 ◽  
Vol 27 (6) ◽  
pp. 775-780 ◽  
Author(s):  
Daniel Deluiz ◽  
Luciano Santos Oliveira ◽  
Paul Fletcher ◽  
Fábio Ramôa Pires ◽  
Justine Monnerat Tinoco ◽  
...  

Abstract The aim of this paper is to report histologic and tomographic findings of fresh frozen bone block allografts bearing dental implants in functional occlusion in a long-term follow-up. Four patients with implants functionally loaded for 4 years on augmented ridges requiring additional mucogingival surgery or implant placement were included in this case series. Cone-beam tomography scans were compared volumetrically between the baseline (first implant placement) and current images. Biopsies of the grafts were retrieved and sent to histological analysis. Volumetric reduction of the grafts varied from 2.1 to 7.7%. Histological evaluation demonstrated well-incorporated grafts with different degrees of remodeling. While data presented in this report are from a small sample size and do not allow definitive conclusions, the biopsies of the grafted sites were very similar to the host's native bone. Remodeling of the cortical portion of the allografts seems to take longer than the cancellous portion. The presence of unincorporated graft remains did not impair the implant success or the health of the surrounding tissues. This is the first time histologic and tomographic long term data of bone allograft have been made available in dentistry.


Author(s):  
Ana L. Creo ◽  
Aida N. Lteif

AbstractPituitary gigantism (PG) is a rare pediatric disease with poorly defined long-term outcomes. Our aim is to describe the longitudinal clinical course in PG patients using a single-center, retrospective cohort study.Patients younger than 19 years diagnosed with PG were identified. Thirteen cases were confirmed based on histopathology of a GH secreting adenoma or hyperplasia and a height >2 SD for age and gender. Laboratory studies, initial pathology, and imaging were abstracted.Average age at diagnosis was 13 years with an average initial tumor size of 7.4×3.8 mm. Initial transsphenoidal surgery was curative in 3/12 patients. Four of the nine patients who failed the initial surgery required a repeat procedure. Octreotide successfully normalized GH levels in 1/6 patients with disease refractory to surgery (1/6). Two out of five patients received pegvisomant after failing octreotide but only one patient responded to treatment. Five patients were ultimately treated with radiosurgery or radiation patients were followed for an average of 10 years.PG is difficult to treat. In most patients, the initial transsphenoidal surgery failed to normalize GH levels. If the initial surgery was unsuccessful, repeat surgery was unlikely to control GH secretion. Treatment with octreotide or pegvisomant was successful in less than half the patients failing surgery. Radiosurgery was curative, but is not an optimal treatment for pediatric patients. Despite the small sample, our study suggests that the treatment outcome of pediatric PG may be different than adults.


2021 ◽  
pp. 107110072199542
Author(s):  
Daniel Corr ◽  
Jared Raikin ◽  
Joseph O’Neil ◽  
Steven Raikin

Background: Microfracture is the most common reparative surgery for osteochondral lesions of the talus (OLTs). While shown to be effective in short- to midterm outcomes, the fibrocartilage that microfracture produces is both biomechanically and biologically inferior to that of native hyaline cartilage and is susceptible to possible deterioration over time following repair. With orthobiologics being proposed to augment repair, there exists a clear gap in the study of long-term clinical outcomes of microfracture to determine if this added expense is necessary. Methods: A retrospective review of patients undergoing microfracture of an OLT with a single fellowship-trained orthopedic surgeon from 2007 to 2009 was performed. Patients meeting the inclusion criteria were contacted to complete the Foot and Ankle Ability Measure (FAAM) Activities of Daily Living (ADL) and Sports subscales and visual analog scale (VAS) for pain, as well as surveyed regarding their satisfaction with the outcome of the procedure and their likelihood to recommend the procedure to a friend with the same problem using 5-point Likert scales. Patient demographics were reviewed and included for statistical analysis. Results: Of 45 respondents, 3 patients required additional surgery on their ankle for the osteochondral defect, yielding a 10-year survival rate of 93.3%. Of surviving cases, 90.4% (38/42) reported being “extremely satisfied” or “satisfied” with the outcome of the procedure. The VAS score at follow-up averaged 14 out of 100 (range, 0-75), while the FAAM-ADL and FAAM-Sports scores averaged 90.29 out of 100 and 82 out of 100, respectively. Thirty-six patients (85.7%) stated that their ankle did not prevent them from participating in the sports of their choice. Conclusion: The current study represents a minimum 10-year follow-up of patients undergoing isolated arthroscopic microfracture for talar osteochondral defects, with a 93.3% survival rate and 85.7% return to sport. While biological adjuvants may play a role in improving the long-term outcomes of microfracture procedures, larger and longer-term follow-up studies are required for procedures using orthobiologics before their cost can be justified for routine use. Level of Evidence: Level IV, retrospective cohort case series study.


2020 ◽  
Author(s):  
Sean Horoho ◽  
Stephen Musik ◽  
David Bryant ◽  
William Brooks ◽  
Ian M Porter

ABSTRACT It is well established that coronavirus disease 2019 is primarily transmitted through respiratory droplets, and there is mounting research speculation that it may also be transmitted via fomites. Several studies have shown that the virus can persist on both porous and nonporous surfaces for hours to days, depending upon the material. This article examines three cases of polymerase chain reaction–proven severe acute respiratory syndrome coronavirus 2 infection with several additional individuals meeting CDC close contact criteria. In 1 case, 195 downstream contacts were all tested to prevent a mass outbreak in a deployment posture. Analysis of these contacts yielded only a single positive test, which could be reasonably ascribed to respiratory droplet transmission. While these cases and their contacts ultimately represent a small sample size, we suggest fomite spread may not be a significant means of transmission for severe acute respiratory syndrome coronavirus 2 in real-world operational scenarios.


2015 ◽  
Vol 93 (5) ◽  
pp. 425-429 ◽  
Author(s):  
Heather J. Dean ◽  
Elizabeth A.C. Sellers

Prior to 1985, type 2 diabetes was a disease of adults. Simultaneously with the global epidemic of childhood obesity, type 2 diabetes has increased in children. Initially, the presentation of small case series of type 2 diabetes in children was met with skepticism. As the number and size of the case series grew and the first long-term outcomes of end-stage complications in young adults appeared in the literature, the international community took notice with guarded interest. Type 2 diabetes disproportionately affects the children of specific ethnic groups and from disadvantaged socioeconomic environments, especially Indigenous populations. The past decade has seen unprecedented intense global interest in the etiology, treatment, and prevention of type 2 diabetes in children.


2021 ◽  
pp. bjophthalmol-2019-315617
Author(s):  
Li-Qiang Wang ◽  
Teng-Yun Wu ◽  
Xiao-Niao Chen ◽  
Ze-Quan Xu ◽  
Min Yang ◽  
...  

PurposeTo report the long-term outcomes of Boston keratoprosthesis type I (B-KPro type I) implantation in the management of severe ocular surface disorders.MethodsRetrospective case series. Patients who underwent B-KPro type I implantation at the People’s Liberation Army General Hospital were enrolled between March 2011 and September 2019. Data regarding visual acuity (VA), B-KPro type I retention and postoperative complications were recorded and analysed.ResultsA total of 103 eyes of 100 patients who underwent B-KPro type I implantation were included. The main indications were chemical burn (59.2%), ocular trauma (25.2%), herpetic keratitis (11.7%) and autoimmune diseases (3.9%). The percentage of eyes with postoperative VA of 10/200 or better was 82.7% at 6 months, 82.8% at 12 months, 77.9% at 2 years, 72.4% at 3 years, 71.1% at 4 years, 69.4% at 5 years, 58.9% at 6 years, 56.8% at 7 years and 42.9% at 8 years. Preoperatively, 8.7% eyes were diagnosed with new-onset glaucoma. Retroprosthetic membrane formation occurred in 19.4% eye. Corneal melting occurred in 18.4% eyes. Sterile vitritis was diagnosed in 4.9% eyes and infectious endophthalmitis in 2.9% eyes. Retinal detachment occurred in 0.9% eyes.ConclusionsIn a Chinese patient group, B-KPro type I is a viable option for treating severe ocular surface disorders in eyes where conventional keratoplasty would have a poor prognosis, especially in patients with chemical and thermal burns. Improved visual outcomes and high retention rate can be achieved and maintained in most cases.


Author(s):  
Rujuta Katkar ◽  
Narasa Raju Madam

Objectives: This paper seeks to explore the hypothesis of the potential diabetogenic effect of SARS-COV-2 (Severe Acute respiratory syndrome coronavirus). Case series presentation: We present a case series of observation among 8 patients of age group ranging from 34 to 74 years with a BMI range of 26.61 to 53.21 Kilogram/square meters that developed new-onset diabetes after COVID-19 infection. Severe Acute Respiratory Syndrome Coronavirus (SARS-COV-2), commonly known as Coronavirus or COVID-19(Coronavirus infectious disease), gains entry into the cells by binding to the Angiotensin-converting enzyme-2(ACE-2) receptors located in essential metabolic tissues including the pancreas, adipose tissue, small intestine, and kidneys. The evidence reviewed from the scientific literature describes how ACE 2 receptors play a role in the pathogenesis of diabetes and the plausible interaction of SARS-COV-2 with ACE 2 receptors in metabolic organs and tissues. Conclusion: The 8 patients without a past medical history of diabetes admitted with COVID-19 infection developed new-onset diabetes mellitus due to plausible interaction of SARS-COV-2 with ACE 2 receptors. The resulting downregulation of ACE-2 and ACE-2 receptors expression caused islet-cell damage resulting into diabetes. The resulting observation has the potential to adversely impact significant number of the globally affected population. Screening patients with COVID-19 for diabetes routinely can help in early detection, significantly reducing morbidity and mortality associated with diabetes. Due to limitations of observational study with a small sample size will require further investigation in the form of Clinical trial.


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