scholarly journals Symptom rates and profile clustering in tuberous sclerosis complex-associated neuropsychiatric disorders (TAND)

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Samuel Alperin ◽  
Darcy A. Krueger ◽  
David N. Franz ◽  
Karen D. Agricola ◽  
Gabrielle Stires ◽  
...  
Keyword(s):  
Cluster Analysis ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Medical Center ◽  
Symptom Burden ◽  
Neuropsychiatric Disorders ◽  
Children's Hospital ◽  
Center Of Excellence ◽  
Targeted Treatments ◽  
Children’S Hospital

Abstract Background Tuberous Sclerosis Complex (TSC) is associated with a range of neuropsychiatric difficulties, appropriately termed TSC-Associated Neuropsychiatric Disorders (TAND). The objectives of the study were to analyze the rates of TAND symptoms in a cohort of patients seen at the TSC Center of Excellence at Cincinnati Children’s Hospital and to identify clinically meaningful profiles based on TAND symptoms. Methods Data from the TAND Checklist was obtained from participants seen at the TSC Center of Excellence at Cincinnati Children’s Hospital Medical Center from June 2015 to August 2018. Cluster and factor analyses for each TAND symptom were performed. Factor scores were then calculated for participants, and a K-means cluster analysis of these scores was used to empirically identify distinct overall TAND symptom profiles occurring in TSC. Results A total of 1545 checklists was completed for 668 participants (37% adults and 63% children). Approximately 90% of participants reported at least one TAND symptom with an average of 12 symptoms (out of 29). Symptom rates ranged between 5 and 60%. The most common symptoms were neuropsychologic symptoms. A seven-cluster and seven-factor solution were found to be optimal. K-means cluster analysis resulted in a seven-profile solution, ranging from low to high symptom burden. Conclusion This study is the first to identify natural phenotypic profiles of TAND symptoms. Study of specific TAND subpopulations with shared profiles may facilitate better understanding of the underlying biology of TAND and better assessment of more targeted treatments.

scholarly journals Resective Epilepsy Surgery for Tuberous Sclerosis in Children

Neurosurgery ◽  
2015 ◽  
Vol 77 (4) ◽  
pp. 517-524 ◽  
Author(s):  
Aria Fallah ◽  
Shaun D. Rodgers ◽  
Alexander G. Weil ◽  
Sumeet Vadera ◽  
Alireza Mansouri ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Epilepsy Surgery ◽  
Medical Center ◽  
Cleveland Clinic ◽  
Intractable Epilepsy ◽  
Seizure Outcome ◽  
Seizure Freedom ◽  
Epileptogenic Zone ◽  
Children's Hospital ◽  
Children’S Hospital

Abstract BACKGROUND: There are no established variables that predict the success of curative resective epilepsy surgery in children with tuberous sclerosis complex (TSC). OBJECTIVE: We performed a multicenter observational study to identify preoperative factors associated with seizure outcome in children with TSC undergoing resective epilepsy surgery. METHODS: A retrospective chart review was performed in eligible children at New York Medical Center, Miami Children's Hospital, Cleveland Clinic Foundation, BC Children's Hospital, Hospital for Sick Children, and Sainte-Justine Hospital between January 2005 and December 2013. A time-to-event analysis was performed. The “event” was defined as seizures after resective epilepsy surgery. RESULTS: Seventy-four patients (41 male) were included. The median age of the patients at the time of surgery was 120 months (range, 3-216 months). The median time to seizure recurrence was 24.0 ± 12.7 months. Engel Class I outcome was achieved in 48 (65%) and 37 (50%) patients at 1- and 2-year follow-up, respectively. On univariate analyses, younger age at seizure onset (hazard ratio [HR]: 2.03, 95% confidence interval [CI]: 1.03-4.00, P = .04), larger size of predominant tuber (HR: 1.03, 95% CI: 0.99-1.06, P = .12), and resection larger than a tuberectomy (HR: 1.86, 95% CI: 0.92-3.74, P = .084) were associated with a longer duration of seizure freedom. In multivariate analyses, resection larger than a tuberectomy (HR: 2.90, 95% CI: 1.17-7.18, P = .022) was independently associated with a longer duration of seizure freedom. CONCLUSION: In this large consecutive cohort of children with TSC and medically intractable epilepsy, a greater extent of resection (more than just the tuber) is associated with a greater probability of seizure freedom. This suggests that the epileptogenic zone may include the cortex surrounding the presumed offending tuber.

An Association for Child Care in Hospitals

PEDIATRICS ◽  
1967 ◽  
Vol 40 (3) ◽  
pp. 471-472
Author(s):  
T. BERRY BRAZELTON
Keyword(s):  
Child Care ◽  
Patient Care ◽  
American Association ◽  
Medical Center ◽  
Total Patient ◽  
National Organization ◽  
Children's Hospital ◽  
The Past ◽  
Children’S Hospital ◽  
Initial Meeting

In the past 2 years a new national organization, called the American Association for Child Care in Hospitals, has evolved. This organization was initiated by the six "play ladies" who are in charge of the children's hospital programs in Baltimore, Boston, Cleveland, Montreal, Philadelphia, and Pittsburgh. Two years ago, the Children's Hospital Medical Center (CHMC) in Boston was host to 50 participants from these institutions to found the organization. This initial meeting was abetted by the CHMC's concern for total patient care and was made possible by the backing of the administration and the pediatric and psychiatric departments.

THE URINARY MANIFESTATIONS OF ANXIETY IN CHILD

PEDIATRICS ◽  
1973 ◽  
Vol 52 (6) ◽  
pp. 818-822
Author(s):  
Richard Galdston ◽  
Alan D. Perlmutter
Keyword(s):  
Urinary Tract Infection ◽  
Urinary Tract ◽  
Tract Infection ◽  
Medical Center ◽  
Children's Hospital ◽  
Hospital Medical ◽  
Intrapsychic Conflict ◽  
Children’S Hospital ◽  
Psychiatric Manifestations

This report comprises concurrent studies of the urologic and psychiatric manifestations of intrapsychic conflict among a group of children who had been admitted to the surgical wards of The Children's Hospital Medical Center, Boston, between 1965 to 1970 for complaints of disordered urination. Experience with these children indicates that anxiety can alter the frequency and disturb the adequacy of voiding to a degree sufficient to dispose the child to urinary tract infection. This effect of anxiety can occur both in the presence or absence of a demonstrable anatomic lesion. It suggests that an assessment of the degree and nature of the child's anxiety should be an integral part of the pediatric urologic examination.

Indications for Naloxone in Lomotil Poisoning

PEDIATRICS ◽  
1974 ◽  
Vol 54 (5) ◽  
pp. 658-658
Author(s):  
Frederick H. Lovejoy
Keyword(s):  
Medical Center ◽  
Respiratory Effort ◽  
Narcotic Antagonist ◽  
Children's Hospital ◽  
Narcotic Antagonists ◽  
Hospital Medical ◽  
Comatose State ◽  
Children’S Hospital

Rumack and Temple in their thoughtful analysis of Lomotil poisoning1 note that narcotic antagonists should be used "as soon as adequate indications exist." From our experience with Lomotil toxicity in the last three years at Children's Hospital Medical Center and with other drugs producing narcotic like effects,2.3 we would like briefly to comment on the indications for the use of the narcotic antagonist, naloxone (Narcan) hydrochloride. Four prominent signs of naloxone efficacy exist: (1) dilatation of constricted pupils; (2) increase in depth and rate of respiratory effort; (3) reversal of hypotension; and (4) correction of an obtunded or comatose state.4

Acute Phenothiazine Toxicity in Childhood: A Five-Year Survey

PEDIATRICS ◽  
1967 ◽  
Vol 39 (5) ◽  
pp. 771-774
Author(s):  
J. M. GUPTA ◽  
F. H. LOVEJOY
Keyword(s):  
Medical Center ◽  
Neurological Symptoms ◽  
Nausea And Vomiting ◽  
Diagnosis And Treatment ◽  
Acute Nausea ◽  
Children's Hospital ◽  
Hospital Medical ◽  
Children’S Hospital

Twenty patients with phenothiazine toxicity admitted to the Children's Hospital Medical Center have been reviewed. In any patient presenting with bizarre neurological symptoms, phenothiazine toxicity should be borne in mind. Diphenhydramine (Benadryl) was found to be useful in both diagnosis and treatment. The use of phenothiazines in the treatment of acute nausea and vomiting in childhood is questioned.

NEWS AND ANNOUNCEMENTS

PEDIATRICS ◽  
1972 ◽  
Vol 50 (3) ◽  
pp. 504-506
Keyword(s):  
Pediatric Cardiology ◽  
Medical Center ◽  
Newborn Infants ◽  
Heart Association ◽  
University Of Tennessee ◽  
Children's Hospital ◽  
Hospital Medical ◽  
Clinical Cardiology ◽  
Children’S Hospital ◽  
The University

Conference on Newborn Infants: The University of Tennessee College of Medicine will present the Fourth Memphis Conference on the Newborn at the Holiday Inn-Rivermont, Memphis, Tennessee, on September 21, 1972. Faculty will include Drs. Marshall Klaus, Leo Stern, and Paul Swyer. For further information write the Division of Continuing Education and Conferences, The University of Tennessee Medical Units, 800 Madison Avenue, Memphis, Tennessee 38103. Problems in Pediatric Cardiology: The American Heart Association Council on Clinical Cardiology, the Council on Rheumatic Fever and Congenital Heart Disease, and the Departments of Pediatrics, Surgery, and Pathology of Children's Hospital Medical Center and Harvard Medical School, in cooperation with the Massachusetts Heart Association, will cosponsor a course: Problems in Pediatric Cardiology, September 25-27, 1972, at Children's Hospital Medical Center, Boston, Massachusetts.

Utilization of Diagnostic Testing for Renal Anomalies and Congenital Heart Disease in Patients with Microtia

2020 ◽  
Vol 162 (4) ◽  
pp. 554-558
Author(s):  
Vaibhav H. Ramprasad ◽  
Amber D. Shaffer ◽  
Noel Jabbour
Keyword(s):  
Congenital Heart ◽  
Medical Center ◽  
Tertiary Care ◽  
Diagnostic Testing ◽  
Case Series ◽  
Standard Of Care ◽  
Renal Ultrasound ◽  
Renal Anomalies ◽  
Children's Hospital ◽  
Children’S Hospital

Objective Congenital ear anomalies are associated with congenital cardiac and renal defects. Renal ultrasounds, electrocardiogram, and echocardiogram can be utilized for diagnosis of these concurrent defects. No standard of care exists for the workup of patients with microtia. The goals of this study were to describe the utilization of diagnostic testing for cardiac and renal anomalies and to identify their prevalence in patients with microtia. Study Design Case series with chart review. Setting Children’s Hospital of Pittsburgh of the University of Pittsburgh Medical Center. Subjects and Methods This study is an Institutional Review Board–approved retrospective review of consecutive patients born between 2002 and 2016 who were diagnosed with microtia and seen in the otolaryngology clinic at a tertiary care children’s hospital. Demographics, sidedness and grade of microtia, comorbid diagnoses, and details of renal and cardiovascular evaluations were recorded. Factors associated with retroperitoneal ultrasound and cardiac testing were assessed with logistic regression. Results Microtia was present in 102 patients, and 98 patients were included as they received follow-up. Microtia was associated with craniofacial syndrome in 34.7% of patients. Renal ultrasound was performed in 64.3% of patients, and 12.9% of patients with ultrasounds had renal aplasia. Cardiac workup (electrocardiogram or echocardiogram) was completed in 60.2% of patients, and of this subset, 54.2% had a congenital heart defect. Conclusion Diagnostic testing revealed renal anomalies and cardiac defects in patients with isolated microtia at a higher rate than in the general population. This suggests the need for further evaluation of the role of routine screening in patients with microtia.

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