scholarly journals Long-term prognosis of patients with pediatric pheochromocytoma

2013 ◽  
Vol 21 (1) ◽  
pp. 17-25 ◽  
Author(s):  
Birke Bausch ◽  
Ulrich Wellner ◽  
Dirk Bausch ◽  
Francesca Schiavi ◽  
Marta Barontini ◽  
...  
Keyword(s):  
Life Expectancy ◽  
Malignant Tumors ◽  
Germline Mutations ◽  
Hereditary Disease ◽  
Initial Diagnosis ◽  
Second Primary ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Long Term Prognosis

A third of patients with paraganglial tumors, pheochromocytoma, and paraganglioma, carry germline mutations in one of the susceptibility genes, RET, VHL, NF1, SDHAF2, SDHA, SDHB, SDHC, SDHD, TMEM127, and MAX. Despite increasing importance, data for long-term prognosis are scarce in pediatric presentations. The European-American-Pheochromocytoma–Paraganglioma-Registry, with a total of 2001 patients with confirmed paraganglial tumors, was the platform for this study. Molecular genetic and phenotypic classification and assessment of gene-specific long-term outcome with second and/or malignant paraganglial tumors and life expectancy were performed in patients diagnosed at <18 years. Of 177 eligible registrants, 80% had mutations, 49% VHL, 15% SDHB, 10% SDHD, 4% NF1, and one patient each in RET, SDHA, and SDHC. A second primary paraganglial tumor developed in 38% with increasing frequency over time, reaching 50% at 30 years after initial diagnosis. Their prevalence was associated with hereditary disease (P=0.001), particularly in VHL and SDHD mutation carriers (VHL vs others, P=0.001 and SDHD vs others, P=0.042). A total of 16 (9%) patients with hereditary disease had malignant tumors, ten at initial diagnosis and another six during follow-up. The highest prevalence was associated with SDHB (SDHB vs others, P<0.001). Eight patients died (5%), all of whom had germline mutations. Mean life expectancy was 62 years with hereditary disease. Hereditary disease and the underlying germline mutation define the long-term prognosis of pediatric patients in terms of prevalence and time of second primaries, malignant transformation, and survival. Based on these data, gene-adjusted, specific surveillance guidelines can help effective preventive medicine.

scholarly journals Diagnosis, Treatment and Long-term Outcome in Fetal Hydrocephalus(Pediatric Neurosurgery and Long-term Prognosis)

2011 ◽  
Vol 20 (5) ◽  
pp. 322-329
Author(s):  
Mami Yamasaki ◽  
Masahiro Nonaka ◽  
Yohei Bamba ◽  
Chika Teramoto ◽  
Chiaki Ban ◽  
...  
Keyword(s):  
Pediatric Neurosurgery ◽  
Special Issue ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Fetal Hydrocephalus ◽  
Long Term Prognosis

The Ethics of Infant Dialysis

1996 ◽  
Vol 16 (1_suppl) ◽  
pp. 505-509 ◽  
Author(s):  
Timothy E. Bunchman
Keyword(s):  
Standard Of Care ◽  
Health Care Team ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Number Of Factors ◽  
Long Term Prognosis ◽  
Infant Dialysis ◽  
Stage Renal Disease ◽  
End Stage

The proper treatment of an infant with end-stage renal disease depends upon a number of factors including parental willingness to take on the task, experience of the health-care team, local and regional resources, and society's willingness to accept this support as a standard of care. Whereas the abilityto keep infants aliveon peritoneal dialysis (PD) is obtainable, it is not without physical, financial, as well as emotional cost. In order for a family to agree to take on such a task, an understanding of the risks and long-term prognosis should be offered. This “informed consent” is difficult to obtain in such a highly charged situation when emotions often dictate choice independently of logic. Long-term outcome of infants on PD has improved over time, yet is still fraught with complications. Options of treatment or nontreatment are explored.

Long‐term outcome of re‐irradiation for recurrent or second primary head and neck cancer: A multi‐institutional study of AIRO—Head and Neck working group

Head & Neck ◽  
2019 ◽  
Vol 41 (10) ◽  
pp. 3684-3692 ◽  
Author(s):  
Ester Orlandi ◽  
Pierluigi Bonomo ◽  
Letizia Ferella ◽  
Elisa D'Angelo ◽  
Marta Maddalo ◽  
...  
Keyword(s):  
Head And Neck Cancer ◽  
Head And Neck ◽  
Neck Cancer ◽  
Working Group ◽  
Second Primary ◽  
Term Outcome ◽  
Long Term Outcome

Neurobiologic Considerations in Early Surgery for Epilepsy

1994 ◽  
Vol 9 (2_suppl) ◽  
pp. 2S42-2S49 ◽  
Author(s):  
Michael Duchowny ◽  
Bonnie Levin ◽  
Prasanna Jayakar ◽  
Trevor J. Resnick
Keyword(s):  
Selection Process ◽  
Early Years ◽  
Early Surgery ◽  
Onset Seizure ◽  
Dynamic Changes ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Developmental Arrest ◽  
Long Term Prognosis

Children with well-localized medically resistant seizures are often referred for surgical therapy. In young children, at least three maturational issues playa significant role in the selection process and long-term outcome. First, the early years are a time of exceptionally rapid brain development leading to dynamic changes in the electroencephalogram and the clinical expression of seizures. Many early-onset seizure presentations are also associated with catastrophic outcomes, developmental arrest, or regression. Second, the immature limbic system may be vulnerable to stresses operating in early life, although the consequences may not become apparent for many years. Third, in comparison to the adult, the child's nervous system typically exhibits superior functional recovery after lesioning, but the process of sparing and recovery is often incomplete. An understanding of how these neurobiologic factors influence developmental outcome will ultimately lead to greater selectivity of candidates for early surgery and to improved long-term prognosis. (J Child Neurol 1994;9(Suppl):2S42-2S49).

scholarly journals Long-term outcome of Hurler syndrome patients after hematopoietic cell transplantation: an international multicenter study

Blood ◽  
2015 ◽  
Vol 125 (13) ◽  
pp. 2164-2172 ◽  
Author(s):  
Mieke Aldenhoven ◽  
Robert F. Wynn ◽  
Paul J. Orchard ◽  
Anne O’Meara ◽  
Paul Veys ◽  
...  
Keyword(s):  
Hematopoietic Cell Transplantation ◽  
Residual Disease ◽  
Hurler Syndrome ◽  
Term Outcome ◽  
Early Referral ◽  
Long Term Outcome ◽  
Key Points ◽  
Long Term Prognosis ◽  
International Multicenter Study

Key Points Patients with Hurler syndrome show significant residual disease burden despite HCT. Early referral for HCT, using noncarrier donors and regimens designed to achieve full-donor chimerism, offers the best long-term prognosis.

scholarly journals Long-term Outcome of Craniopharyngioma in Children: A Review(Pediatric Neurosurgery and Long-term Prognosis)

2011 ◽  
Vol 20 (5) ◽  
pp. 351-362
Author(s):  
Takashi Tamiya ◽  
Masaki Okada ◽  
Keisuke Miyake ◽  
Nobuyuki Kawai
Keyword(s):  
Pediatric Neurosurgery ◽  
Special Issue ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Long Term Prognosis

scholarly journals The clinical features and long-term prognosis of asymmetry childhood Guillain-Barré syndrome: a 5-year follow-up.

2020 ◽  
Author(s):  
Rui-Di Sun ◽  
jun Jiang
Keyword(s):  
Infectious Disease ◽  
Clinical Features ◽  
Guillain Barre Syndrome ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Guillain Barré Syndrome ◽  
Motor Nerves ◽  
Long Term Prognosis ◽  
Guillain Barré

Abstract Backgroud: The aim was to investigate clinical features and long-term prognosis of asymmetric childhood Guillain-Barré syndrome (GBS). Methods: In a retrospective cohort study, standardized data from all children with GBS seen at the Wuhan Children’s Hospital were collected regarding clinical presentation, auxiliary examinations and long-term outcome. We compared asymmetry GBS with symmetry GBS. Asymmetry GBS was defined by Medical Research Council (MRC) grade and motor nerves conduction in bilateral limbs. Recovery was defined as a return to normal life with a DSS of 0. Results: GBS was diagnosed in 72 children. 12（16.67%）were asymmetry GBS compared to 60 symmetry GBS . In asymmetry GBS, six children were transient asymmetry weakness and six children were persistent asymmetry weakness. Compared to symmetry weakness GBS, asymmetry weakness GBS had more preschool children (75% vs 25%, P=0.005), longer days on hospital(26.5(15-37) days vs 11(9-15) days, p =0.000), more mechanical ventilation(MV) (50% vs 8.33%, p=0.000), higher Disease severity score(DSS)at nadir of disease(4(3-5) vs 3(1-4), p=0.010), more axonal subtypes(50% vs 15%, p=0.013) and more complications(58.33% vs 8.33%, p=0.000). Eight children had sequelae and sixty-four children had recovery. Compared to recovery group, sequelae group had more axonal subtypes(62.5% vs 15.63%, p=0.002) and more persistent asymmetry weakness(62.5% vs 4.69%, p=0.000). In six persistent asymmetry GBS, 5(83.33%) had abnormal EEG (electroencephalogram) results, 3(50%) children had mild to marked pleocytosis in CSF and 5(83.33%) had sequelae. Conclusions: In conclusion, asymmetry GBS had two types, namely transient and persistent asymmetry weakness. Asymmetry GBS indicated a more complex condition during disease. Most of persistent asymmetry GBS had clinical or subclinical infectious disease and poor prognosis. Inflammatory in anterior horn cells or nerve root by infectious disease may be the possible function in persistent asymmetry GBS.

Spinal Arachnoid Cysts in 17 Dogs

2003 ◽  
Vol 39 (3) ◽  
pp. 271-282 ◽  
Author(s):  
Todd M. Skeen ◽  
Natasha J. Olby ◽  
Karen R. Muñana ◽  
Nicholas J. Sharp
Keyword(s):  
Cervical Vertebrae ◽  
Clinical Signs ◽  
Neurological Status ◽  
Arachnoid Cysts ◽  
Thoracic Vertebrae ◽  
Term Outcome ◽  
Long Term Outcome ◽  
North Carolina State University ◽  
Long Term Prognosis

The medical records of 17 dogs diagnosed with spinal arachnoid cysts at North Carolina State University Veterinary Teaching Hospital were retrospectively examined to identify trends in signalment, history, neurological status, treatment, and short- and long-term prognosis. The typical case was that of a nonpainful, progressive ataxia frequently characterized by hypermetria and incontinence. Cysts typically occurred in the dorsal subarachnoid space at the first to third cervical vertebrae of young, large-breed dogs or the caudal thoracic vertebrae of older, small-breed dogs. Although 14 of 15 dogs treated surgically did well in the short term, long-term successful outcomes were achieved in only eight of the 12 dogs that were followed for &gt;1 year. Significant predictors of good, long-term outcome were not identified; however, factors associated with a trend toward a good outcome included &lt;3 years of age, &lt;4 months’ duration of clinical signs, and marsupialization as the surgical technique.

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