POTENTIAL THERAPEUTIC PITFALLS IN REYE'S SYNDROME

PEDIATRICS ◽  
1973 ◽  
Vol 52 (4) ◽  
pp. 491-493
Author(s):  
R. E. Brown ◽  
F. G. Mullick ◽  
G. E. Madge
Keyword(s):  
Medical School ◽  
Rare Disease ◽  
Hepatic Dysfunction ◽  
Disease Process ◽  
Reye's Syndrome ◽  
Viral Type

Reye's syndrome1 is no longer considered to be a rare disease, even though it was not described in medical school to those who graduated more than 10 years ago. This is probably the result of several factors, including the education of the pediatrician and pathologist to recognize the cardinal manifestations of the syndrome and their better appreciation of the spectrum of this disease process. The essential features of this disease process which affects children are easily recognized. These include, in many instances, an antecedent, viral-type illness followed by protracted vomiting; the development of an encephalopathy without a concomitant encephalitis or meningitis; some evidence of hepatic dysfunction, usually in the absence of jaundice; and biopsy or autopsy evidence of fatty accumulation in the viscera, primarily the liver.

scholarly journals Valproic Acid Administration in Management of Status Epilepticus Causing Reye’s Syndrome

2021 ◽  
Vol 07 (01) ◽  
pp. 044-046
Author(s):  
Pooja Prabhakar Kamath
Keyword(s):  
Valproic Acid ◽  
Status Epilepticus ◽  
Hepatic Dysfunction ◽  
Rare Condition ◽  
Normal Serum ◽  
Reye's Syndrome ◽  
Serum Glutamic Oxaloacetic Transaminase ◽  
Viral Illness ◽  
Laboratory Investigations ◽  
Serum Ammonia

Abstract Introduction Valproic acid is commonly used to treat seizures in children. Regular use of valproic acid is known to cause hepatic dysfunction, and in extremely rare cases, it is known to have caused Reye’s syndrome. There are very few reports of Reye’s syndrome caused by valproic acid use. Methods A 2-year asymptomatic girl underwent modified Blalock–Taussig shunt surgery for correction of tetralogy of Fallot. Postoperatively the girl developed status epilepticus, which did not subside with initial use of intravenous midazolam and phenytoin sodium. She eventually responded to two doses of intravenous valproic acid administered 10 minutes apart. She developed depressed sensorium and was put on mechanical ventilation. The following day’s laboratory investigations revealed raised levels of serum ammonia, serum glutamic-oxaloacetic transaminase (SGOT), and serum glutamic-pyruvic transaminase (SGPT) with normal serum bilirubin. Thus, a diagnosis of Reye’s syndrome was established. The patient succumbed to disease 2 days later. Discussion Reye’s syndrome is a rare and a fulminant illness seen typically in children following a viral illness and/or use of salicylates or other medications. There are rare reports of Reye’s syndrome following use of medications like valproic acid. This patient had a noninflammatory encephalopathy with hepatic dysfunction following two doses of valproic acid. Conclusion There are very few reports on Reye’s syndrome in the literature as it is a rare condition and diagnosis is difficult. Knowledge of the presentation of Reye’s syndrome is essential for treatment and management. When using drugs like valproic acid in children, liver enzymes and serum ammonia levels should be monitored.

Diagnostic Criteria for Influenza B-Associated Reye's Syndrome: Clinical vs. Pathologic Criteria

PEDIATRICS ◽  
1977 ◽  
Vol 60 (5) ◽  
pp. 702-708
Author(s):  
Lawrence Corey ◽  
Robert J. Rubin ◽  
Dennis Bregman ◽  
Michael B. Gregg
Keyword(s):  
Disease Control ◽  
Diagnostic Criteria ◽  
Hepatic Dysfunction ◽  
Elevated Serum ◽  
Acute Onset ◽  
Demographic Characteristics ◽  
Influenza B ◽  
Elevated Blood ◽  
Blood Ammonia ◽  
Reye's Syndrome

Between December 15, 1973, and June 30, 1974, a total of 379 cases of Reye's syndrome was reported to the Center for Disease Control. One hundred forty-seven (40%) were confirmed by either autopsy or biopsy, while 232 were diagnosed by clinical and laboratory parameters. Comparisons of the epidemiologic and demographic characteristics, the hospital course, the outcome, and the laboratory abnormalities of the clinically diagnosed and the pathologically confirmed cases revealed no significant differences. In the epidemiologic setting of influenza B outbreaks, children who have the acute onset of noninflammatory encephalopathy associated with elevated serum transaminase levels, hypoprothrombinemia, and elevated blood ammonia levels should be considered to have Reye's syndrome. Further evaluation of diagnostic criteria is needed, however, for sporadically occurring, nonepidemic cases of noninflammatory encephalopathy associated with hepatic dysfunction.

Housestaff perspectives on end-of-life training during medical school and residency.

2015 ◽  
Vol 33 (29_suppl) ◽  
pp. 27-27 ◽  
Author(s):  
Jessica Marie Schmit ◽  
Lynne Meyer ◽  
Jennifer Michelle Duff ◽  
Julia Lee Close
Keyword(s):  
Medical School ◽  
End Of Life ◽  
Disease Process ◽  
Fellowship Training ◽  
Bad News ◽  
Training Experience ◽  
Classroom Training ◽  
Level Of Training ◽  
Oncology Practice ◽  
Delivering Bad News

27 Background: Participating in end-of-life discussions and delivering bad news is integral to oncology practice. Poor communication skills can negatively impact a patient’s understanding and acceptance of their disease process, increase their anxiety, and lead to anger and depression. Hematology/Oncology fellowship training (HOFT) programs may assume incoming fellows are capable of doing this well. A study by Hebert et al. revealed 37% of HOFT programs provide little to no formal training in this area. Our study sought to assess housestaff preparedness and comfort with end-of-life (EOL) discussions. Methods: An email survey was sent to 787 residents and fellows at a single institution in February 2015. Housestaff were queried as to extent of training, experience, supervision and comfort with EOL communication in both medical school and residency. Results: 176 surveys (22.4 %) were returned.During Medical School: Many housestaff (55.1%) reported “little” to “no” classroom training on EOL discussions. Most, 85.1%, reported having five or fewer EOL discussions. Most encounters (78.6%) were supervised by an attending or resident. Only 45.5% felt “somewhat” to “fully prepared” to have EOL discussions when graduating medical school. During Residency: Housestaff reported even less classroom training on EOL discussions: 88.2% reported “little” to “no training”. EOL discussions were more common: 51% reported more than 10 EOL conversations with patients. The level of supervision decreased: only 38.6% reported encounters were mostly supervised. Most housestaff (75%) feel comfortable with having EOL discussions at their current level of training. Conclusions: Despite increased awareness regarding the importance of EOL discussion education, it remains an area that a significant number of medical students and housestaff receive little to no training. EOL discussions by housestaff are frequently unsupervised, reducing opportunities for improvement via feedback. Competence in delivering bad news is an important skill for hematologists/oncologists. Our findings support the previously published perception of HOFT program directors that additional training and resources in delivering bad news is needed.

scholarly journals Xanthogranulomatous Pyelonephritis Associated with Hepatic Dysfunction in Pregnancy

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
L. Ferreira ◽  
C. Oliveira ◽  
C. Cruz ◽  
A. Pacheco
Keyword(s):  
Pregnant Woman ◽  
Rare Disease ◽  
Hepatic Dysfunction ◽  
Rare Event ◽  
Renal Parenchyma ◽  
Xanthogranulomatous Pyelonephritis ◽  
Normal Renal Parenchyma ◽  
Foamy Macrophages ◽  
In Pregnancy ◽  
Total Nephrectomy

Xanthogranulomatous pyelonephritis is a rare disease characterised by the replacement of normal renal parenchyma by foamy macrophages. The only treatment for this type of pyelonephritis is of a surgical nature with partial or total nephrectomy. The occurrence of xanthogranulomatous pyelonephritis during pregnancy is a rare event (with only 6 cases described in the literature). We report a case of xanthogranulomatous pyelonephritis in a 32-week pregnant woman associated with hepatic dysfunction.

scholarly journals Rare disease awareness and perspectives of physicians in China: a questionnaire-based study

2020 ◽  
Author(s):  
Xuefeng Li ◽  
Xiangyu Zhang ◽  
Shu Zhang ◽  
Zijuan Lu ◽  
Jianyong Zhang ◽  
...  
Keyword(s):  
Medical School ◽  
Rare Disease ◽  
Rare Diseases ◽  
Strong Support ◽  
Orphan Drugs ◽  
School Education ◽  
Sufficient Information ◽  
Medical School Education ◽  
Disease Awareness ◽  
Career Length

Abstract Background There are over 16.8 million rare disease patients in China, representing a significant challenge for the healthcare system and society. Rare disease patients often experience delayed diagnosis, misdiagnosis, or improper treatment, which may be due to the lack of rare disease awareness among physicians. Results A total of 224 physicians from different hospitals in China participated in the questionnaire, and 9 rare disease experts were interviewed with open-ended questions. Most physicians (83.5%) were from Tertiary hospitals, which have over 500 beds. Only 5.3% of physicians were moderately or well aware of rare diseases. Most physicians (80.1%) had suspected their patients to have rare diseases less than 3 times. There was a strong support for special legislations for rare diseases and orphan drugs. Further, multinomial logistic regression (MLR) was used to determine whether hospitals, gender, and career length has an impact on perspectives and awareness. It was shown that male physicians were more likely to think newborn screening is important (p<0.05). The longer the career length is, the more likely physicians believe that their previous education has not provided sufficient information about rare diseases and that their hospital has paid enough attention to rare diseases. Physicians from Tertiary A hospitals were more likely to rate the affordability of orphan drugs high. In addition, nine experts believed that rare disease awareness is essential for early diagnosis and timely treatment. These experts also made recommendations on how to improve rare disease awareness through medical school education and continuing training. Conclusions Our study highlighted the importance of improving rare disease awareness among physicians in China. Recommendations about how to improve rare disease awareness in medical school education and establish an online ‘information hub’ are made for considerations of policy-makers.

scholarly journals Streptococcus Intermedius Lung Abscess in a 17-year-old Male

2019 ◽  
Vol 6 ◽  
pp. 11-14
Author(s):  
Jason Andrew Levine ◽  
Karen Hovsepyan ◽  
Daniel Lubarsky ◽  
Deepa Mukundan ◽  
Jennifer Ruddy
Keyword(s):  
Back Pain ◽  
Rare Disease ◽  
Pediatric Population ◽  
Disease Process ◽  
Lung Abscess ◽  
Morbidity And Mortality ◽  
High Morbidity ◽  
Streptococcus Intermedius ◽  
Infectious Process ◽  
Rare Diagnosis

Streptococcus intermedius lung abscess and empyema is a chronic infectious process associated with a high morbidity and mortality.  It is typically seen in adults, and is considered a rare disease process in the pediatric population.  Our case describes a 17 year old male presenting with 3 months of cough associated with later development of chest and back pain.  He was later found to have a severe empyema secondary to Streptococcus intermedius.  This case underlines a rare diagnosis in the pediatric population, as well as a association with smoking and vaping. 

scholarly journals Gastrobronchial Fistula after Robotic Repair of Traumatic Diaphragmatic Hernia

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Jamie T. Tung ◽  
Leah M. Lucero ◽  
James W. Davis ◽  
Lawrence P. Sue
Keyword(s):  
Rare Disease ◽  
Diaphragmatic Hernia ◽  
Respiratory Symptoms ◽  
Disease Process ◽  
Lower Threshold ◽  
Rare Occurrence ◽  
Left Thoracotomy ◽  
Traumatic Diaphragmatic Hernia ◽  
Gastrobronchial Fistula

Gastrobronchial fistulas are a rare occurrence in the literature. We report a case of a gastrobronchial fistula after robotic repair of a chronic traumatic diaphragmatic hernia. The patient had severe respiratory symptoms with multiple studies that were inconclusive. The fistula was ultimately discovered after an esophagogastroduodenoscopy (EGD). The patient underwent a left thoracotomy for takedown of his fistula and eventually recovered. Earlier EGD and a lower threshold for differential that included this diagnosis would have led to an earlier identification and treatment of a rare disease process.

Genetic Diagnosis: Implications for Medical Practice

1989 ◽  
Vol 5 (4) ◽  
pp. 579-600 ◽  
Author(s):  
David Blumenthal ◽  
Richard Zeckhauser
Keyword(s):  
Health Care ◽  
Primary Care Physicians ◽  
Private Health Insurance ◽  
Health Care Organizations ◽  
Genetic Diagnosis ◽  
Disease Process ◽  
Diagnostic Techniques ◽  
Insurance Companies ◽  
Genetic Diagnostics ◽  
Reye's Syndrome

Genetic diagnostic techniques increasingly permit the detection of predisposition to illness long before the onset of the disease process itself. Medicine is on the verge of becoming a predictive science as well as a diagnostic and therapeutic one. Genetic diagnosis could have profound effects on many aspects of our health care system, including the prestige and effectiveness of preventive medicine, the competitive behavior of health care organizations and insurance companies, access to private health insurance, the ability of primary care physicians to serve as gatekeepers, and other matters. This article examines the range of potential effects of the new genetic diagnostics on the organization and financing of health care. For purposes of illustration the authors examine in detail the possible consequences of genetic tests for predisposition to two diseases: Reye's Syndrome and lung cancer in smokers.

scholarly journals Rare disease awareness and perspectives of physicians in China: a questionnaire-based study

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Xuefeng Li ◽  
Xiangyu Zhang ◽  
Shu Zhang ◽  
Zijuan Lu ◽  
Jianyong Zhang ◽  
...  
Keyword(s):  
Medical School ◽  
Rare Disease ◽  
Rare Diseases ◽  
Strong Support ◽  
Orphan Drugs ◽  
School Education ◽  
Sufficient Information ◽  
Medical School Education ◽  
Disease Awareness ◽  
Career Length

Abstract Background It is estimated that there are over 16.8 million rare disease patients in China, representing a significant challenge for the healthcare system and society. Rare disease patients often experience delayed diagnosis, misdiagnosis, or improper treatment, which may be due to the lack of rare disease awareness among physicians. Materials and methods A total of 224 physicians from different hospitals in China participated in the questionnaire, and 9 rare disease experts were interviewed with open-ended questions. Results Most physicians (83.5%) were from Tertiary hospitals, which have over 500 beds. Only 5.3% of physicians were moderately or well aware of rare diseases. Most physicians (80.1%) had suspected their patients to have rare diseases less than 3 times. There was a strong support for special legislations for rare diseases and orphan drugs. Further, multinomial logistic regression (MLR) was used to determine whether hospitals, gender, and career length has an impact on perspectives and awareness. It was shown that male physicians were more likely to think newborn screening is important (p < 0.05). The longer the career length is, the more likely physicians believe that their previous education has not provided sufficient information about rare diseases and that their hospital has paid enough attention to rare diseases. Physicians from Tertiary A hospitals were more likely to rate the affordability of orphan drugs high. In addition, nine experts believed that rare disease awareness is essential for early diagnosis and timely treatment. These experts also made recommendations on how to improve rare disease awareness through medical school education and continuing training. Conclusions Our study highlighted the importance of improving rare disease awareness among physicians in China. Recommendations about how to improve rare disease awareness in medical school education and establish an online ‘information hub’ are made for considerations of policy-makers.

Sign in / Sign up

Export Citation Format

Share Document