Embryopathogenetic surgicoanatomical classification of dysraphism and surgical outcome of spinal lipoma: a nationwide multicenter cooperative study in Japan

2009 ◽  
Vol 3 (5) ◽  
pp. 412-419 ◽  
Author(s):  
Shizuo Oi ◽  
Sadahiro Nomura ◽  
Masato Nagasaka ◽  
Hajime Arai ◽  
Reizo Shirane ◽  
...  
Keyword(s):  
Natural History ◽  
Spina Bifida ◽  
Complete Resolution ◽  
Cooperative Study ◽  
Foot Deformity ◽  
Spinal Lipoma ◽  
Scale Scores ◽  
History Of ◽  
The Mean

Object The natural history of asymptomatic spinal lipoma in infancy remains unclear, and the indication for the prophylactic untethering operation is still debatable. To address this question, a multicenter cooperative study for the treatment of spinal lipoma was performed by the 7 most active institutions in neurosurgical care for spina bifida in Japan between 2001 and 2005. Methods Patients were classified using the embryopathogenetic surgicoanatomical classification. Their neurosurgical postoperative course was analyzed using the Spina Bifida Neurological Scale. Among 261 patients, 159 were asymptomatic and 102 were symptomatic. Results Of the 136 patients for whom prophylactic surgeries were performed, 135 remained asymptomatic and only 1 (0.4%) of the 261 patients presented with mild sensory disturbance. Mild foot deformity was identified in 1 (4.3%) of 23 conservatively observed patients. Of 100 symptomatic patients, deterioration after surgery was seen in 6%, and improvement in 44%. Complete resolution of symptoms was seen in only 14.2%. Filar types for patients > 3 years old improved in Spina Bifida Neurological Scale scores from 12.3 to 14.0. The mean age of symptomatic patients with lipomyelomeningocele was the youngest of all (1.3 years), which indicates lipomyelomeningocele may deteriorate in early infancy. Improvements from surgery were seen for all types of lipoma except the caudal type, presenting at an older mean age (15 years). Conclusions A low rate of postsurgical worsening indicates that surgeries for asymptomatic and symptomatic lipomas are safe. Surgeries done after the onset of symptoms seldom cure the patients. These two results support early untethering for any kind of lipoma; however, further study of the natural history is required.

La diagnosi e la gestione del prolasso rettale

2020 ◽  
Vol 39 (7) ◽  
pp. 453-457
Author(s):  
Silvia Ventresca ◽  
Micol Bacchini ◽  
Giulia Graziani ◽  
Federico Marchetti
Keyword(s):  
Cystic Fibrosis ◽  
Natural History ◽  
Spina Bifida ◽  
Rectal Prolapse ◽  
Newborn Screening ◽  
Complete Resolution ◽  
Toilet Training ◽  
Rare Occurrence ◽  
The Family ◽  
History Of

Rectal prolapse is an overall rare occurrence in children in the first 4 years of age. It typically tends to relapse. In the majority of cases it is not possible to highlight a single cause that determines prolapse, even if it is more frequent in children who suffer from constipation. In about 1 in 10 cases there is an underlying predisposing anatomical-neurological condition (in particular neurological: myelomenigocele, spina bifida occulta). A predisposing cause that must always be considered and excluded is cystic fibrosis, even in the age of newborn screening. Rectal prolapse management is conservative in most cases. Behavioural measures (correct toilet training, good hydration, diet rich in fibres) and the use of the macrogol laxative are fundamental. The prolapse that does not resolve spontaneously must be reduced manually by instructing the family on the technique to be used. In 90% of cases the natural history of rectal prolapse is favourable, with complete resolution within the first 4 years of age. After this age, it occurs more rarely. Surgery is rarely indicated. The current techniques that have a large consensus are sclerotherapy and laparoscopic rectopexy. The paper reports the management of recurrent rectal prolapse in a 3-year-old boy.

Revisiting the Natural History of Chronic Scaphoid Nonunions: A Retrospective Study of 20 Cases

2021 ◽  
Author(s):  
J. Terrence Jose Jerome
Keyword(s):  
Retrospective Study ◽  
Natural History ◽  
Functional Outcome ◽  
Grip Strength ◽  
Scaphoid Nonunion ◽  
Proximal Pole ◽  
Degenerative Arthritis ◽  
Level Of Evidence ◽  
History Of ◽  
The Mean

Abstract Background The natural history of scaphoid nonunion is the development of degenerative arthritis. A lot of information is still unclear about this progression. The purpose of this study is to analyze patients with scaphoid nonunions who had not received any kind of treatment and to assess the functional outcome. Materials and Methods This is a retrospective study that analyzed the patients with chronic scaphoid nonunions between 2009 and 2019. None of the patients received any treatment. The age at the time of injury, examination, pattern of fracture, types of scaphoid nonunion, symptoms, and duration of nonunion were noted. Diagnosis was confirmed by radiographs, computed tomography (CT) scan, and magnetic resonance imaging (MRI). Scapholunate and radiolunate angles were recorded. Pain score, modified mayo wrist score, grip strength, range of movement, and the functional outcome of these scaphoid nonunions were analyzed. A statistical correlation between the scaphoid nonunion presentations and the functional outcome was assessed. Results The mean age of the patients was 62 years (range: 35–82 years.). There were 17 male and 3 female patients. There were 9 waist and 11 proximal pole scaphoid nonunions. The mean duration of scaphoid nonunion was 34 years (range: 10–62 years). None of the patients had avascular necrosis (AVN) of the proximal scaphoid. The age at examination, gender, side of injury, fracture pattern (waist/proximal pole), fracture displacement ≤ 1 mm or > 1 mm, nonunion duration, and radiographic arthritic parameters had no significant impact on the functional outcome. Conclusions Untreated chronic scaphoid nonunion leads to the development of degenerative arthritis over a period of years, which is still unpredictable. Most of the patients become aware of the nonunion following a precedent injury or other reasons. Most of the patients have fair/good functional outcome despite reduced range of movements and grip strength. Many do not favor surgical intervention in the course of nonunion. Chronic nonunions open a lot of unanswered questions. Clinical relevance There have been numerous studies on the treatment aspects of scaphoid nonunion, with little knowledge about certain people with nonunion who did not have any kind of treatment. The demographics, clinical findings, and radiological parameters do confirm the progression of these nonunion to arthritis, but most of them had fair-to-good outcome throughout their life. It opens our thinking about the real need of treatment in such nonunions and raises numerous questions about the disease. Level of evidence This is a Level IV study.

scholarly journals SOD1 Mutation Spectrum and Natural History of ALS Patients in a 15-Year Cohort in Southeastern China

2021 ◽  
Vol 12 ◽  
Author(s):  
Lu-Xi Chen ◽  
Hai-Feng Xu ◽  
Pei-Shan Wang ◽  
Xin-Xia Yang ◽  
Zhi-Ying Wu ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Natural History ◽  
Diagnostic Delay ◽  
Mutation Spectrum ◽  
Survival Duration ◽  
Southeastern China ◽  
History Of ◽  
Male Patients ◽  
The Mean ◽  
Als Patients

Background: Mutations in superoxide dismutase 1 gene (SOD1) are the most frequent high penetrant genetic cause for amyotrophic lateral sclerosis (ALS) in the Chinese population. A detailed natural history of SOD1-mutated ALS patients will provide key information for ongoing genetic clinical trials.Methods: We screened for SOD1 mutations using whole exome sequencing (WES) in Chinese ALS cases from 2017 to 2021. Functional studies were then performed to confirm the pathogenicity of novel variants. In addition, we enrolled previously reported SOD1 mutations in our centers from 2007 to 2017. The SOD1 mutation spectrum, age at onset (AAO), diagnostic delay, and survival duration were analyzed.Results: We found two novel SOD1 variants (p.G17H and p.E134*) that exerted both gain-of-function and loss-of-function effects in vitro. Combined with our previous SOD1-mutated patients, 32 probands with 21 SOD1 mutations were included with the four most frequently occurring mutations of p.V48A, p.H47R, p.C112Y, and p.G148D. SOD1 mutations account for 58.9% of familial ALS (FALS) cases. The mean (SD) AAO was 46 ± 11.4 years with a significant difference between patients carrying mutations in exon 1 [n = 5, 34.6 (12.4) years] and exon 2 [n = 8, 51.4 (8.2) years] (p = 0.038). The mean of the diagnostic delay of FALS patients is significantly earlier than the sporadic ALS (SALS) patients [9.5 (4.8) vs. 20.3 (9.3) years, p = 0.0026]. In addition, male patients survived longer than female patients (40 vs. 16 months, p = 0.05).Conclusion: Our results expanded the spectrum of SOD1 mutations, highlighted the mutation distribution, and summarized the natural history of SOD1-mutated patients in southeastern China. Male patients were found to have better survival, and FALS patients received an earlier diagnosis. Our findings assist in providing a detailed clinical picture, which is important for ongoing genetic clinical trials.

scholarly journals Puelchesia gracilis, a new genus and species of Pachydemini endemic to the Monte biogeographic province in Argentina (Coleoptera: Scarabaeidae: Melolonthinae)

Zootaxa ◽  
2006 ◽  
Vol 1349 (1) ◽  
pp. 53 ◽  
Author(s):  
FEDERICO C. OCAMPO ◽  
ANDREW B.T. SMITH
Keyword(s):  
Natural History ◽  
New Genus ◽  
Scarab Beetle ◽  
New Genus And Species ◽  
Biogeographic Province ◽  
Central Argentina ◽  
West Central ◽  
Current Classification ◽  
History Of

A new scarab beetle genus and species, Puelchesia gracilis, is described based on specimens collected in the Monte biogeographic province of west central Argentina. This genus is placed in the tribe Pachydemini based on an evaluation of the characters within the context of the current classification of the subfamily Melolonthinae. The distribution and natural history of the taxon is also discussed.

NATURAL HISTORY OF SPINA BIFIDA

The Lancet ◽  
1969 ◽  
Vol 294 (7613) ◽  
pp. 213
Author(s):  
StephenP. Mark
Keyword(s):  
Natural History ◽  
Spina Bifida ◽  
History Of

The natural history of pineal cysts in children and young adults

2010 ◽  
Vol 5 (2) ◽  
pp. 162-166 ◽  
Author(s):  
Wajd N. Al-Holou ◽  
Cormac O. Maher ◽  
Karin M. Muraszko ◽  
Hugh J. L. Garton
Keyword(s):  
Natural History ◽  
Mr Imaging ◽  
Initial Study ◽  
Older Children ◽  
Imaging Evaluation ◽  
Imaging Appearance ◽  
Imaging Characteristics ◽  
History Of ◽  
The Mean

Object The authors reviewed their experience with pediatric pineal cysts to define the natural history and clinical relevance of this common intracranial finding. Methods The authors identified all patients with pineal cysts who had been clinically evaluated at their institution over an 11.5-year interval and were < 25 years of age at the time of diagnosis. All inclusion criteria were met in 106 patients, and included repeated MR imaging as well as repeated clinical evaluation over at least a 6-month interval. Results The mean age at diagnosis was 11.7 ± 7.2 years. Forty-two patients were male and 64 were female. On follow-up MR imaging evaluation at a mean interval of 3.0 years from the initial study, 98 pineal cysts had no increase in size and no change in imaging appearance. Six pineal cysts increased in size and 2 others had a change in imaging characteristics without associated growth. Younger age was associated with cyst change or growth on follow-up imaging (p = 0.02). The mean age of patients with cysts that changed or grew was 5.5 years, and the mean age of patients with stable pineal cysts was 12.2 years. Initial cyst size and appearance on MR imaging were not significant predictors of growth or change in imaging appearance at follow-up. Similarly, the patient's sex was not a significant predictor of growth or change in imaging characteristics. Conclusions Follow-up imaging and neurosurgical evaluation may be considered optional in older children with pineal cysts.

scholarly journals Natural History of Colorectal Cancer with New Classification of Cancer Infiltrating Proper Muscle.

1999 ◽  
Vol 52 (3) ◽  
pp. 214-221
Author(s):  
I. Takemasa ◽  
N. Kikkawa ◽  
I. Nishisho ◽  
H. Mishima ◽  
M. Takeda
Keyword(s):  
Colorectal Cancer ◽  
Natural History ◽  
New Classification ◽  
History Of

Watchful Waiting for Ventral Hernias: A Longitudinal Study

2014 ◽  
Vol 80 (3) ◽  
pp. 245-252 ◽  
Author(s):  
Charles F. Bellows ◽  
Celia Robinson ◽  
Robert J. Fitzgibbons ◽  
Larry S. Webber ◽  
David H. Berger
Keyword(s):  
Natural History ◽  
Repeated Measures ◽  
Watchful Waiting ◽  
Recurrence Rates ◽  
Safe Option ◽  
Sf 36 ◽  
History Of ◽  
The Mean ◽  
Ventral Hernias

Ventral hernias are a common clinical problem. Immediate repair is recommended for most ventral hernias despite significant recurrence rates. This practice may be related to a lack of understanding of the natural history of ventral hernias. The purpose of this study was to determine the natural history of ventral hernias and to determine if watchful waiting is an acceptable and safe option. Forty-one patients with ventral hernias were enrolled in a longitudinal cohort study of watchful waiting. Primary outcomes were functional impairment resulting from hernia disease as measured by the Activities Assessment Scale (AAS) and changes from baseline to two years in the physical and mental component score of the SF-36 Health Survey. Secondary outcomes included complications such as incarceration. Mixed-effects model for repeated measures and Student's t tests were used to evaluate scale performance. The mean age of enrollees was 64 years, and the mean hernia size was 239 cm2. Eleven patients were lost to follow-up, and seven patients died of other causes. All remaining patients were followed for two years. There was one incarceration during the follow-up period. There was no deterioration in the AAS score (baseline vs 24 months = 28 vs 25, P = 0.60). There was deterioration of the physical functioning dimension of the SF-36 (baseline vs 24 months = 40 vs 32, P < 0.01), but the mental functioning dimension was improved (45 vs 51; P = 0.01). Watchful waiting was a safe option for patients in this study with ventral hernias.

The effect of botulinum toxin type A injections on the natural history of equinus foot deformity in paediatric cerebral palsy patients

1999 ◽  
Vol 6 ◽  
pp. s19-s22 ◽  
Author(s):  
L. Andrew Koman ◽  
Beth Paterson Smith ◽  
Craig T. Tingey ◽  
James F. Mooney ◽  
Stacey Slone ◽  
...  
Keyword(s):  
Cerebral Palsy ◽  
Botulinum Toxin ◽  
Natural History ◽  
Botulinum Toxin Type A ◽  
Type A ◽  
Botulinum Toxin Type ◽  
Foot Deformity ◽  
History Of

scholarly journals Interferon-induced depression in patients with hepatitis C: an epidemiologic study

2014 ◽  
Vol 60 (1) ◽  
pp. 35-39 ◽  
Author(s):  
Lucas Pereira Jorge de Medeiros ◽  
Monica Kayo ◽  
Renata Barboza Vianna Medeiros ◽  
Mario Barreto Correa Lima ◽  
Carlos Eduardo Brandão Mello Mello
Keyword(s):  
Depressive Symptoms ◽  
Incidence Rate ◽  
Epidemiologic Study ◽  
Depression Scale ◽  
Epidemiologic Studies ◽  
Scale Scores ◽  
History Of ◽  
High Incidence Rate ◽  
The Mean ◽  
Highly Correlated

Objective To assess the incidence rate and severity of depressive symptoms in different time points (12, 24 and 48 weeks) in Brazilian patients with HCV treated with PEG IFN plus ribavirin. Methods We conducted an observational prospective study using the Beck Depression Inventory (BDI) and the Center for Epidemiologic Studies Depression Scale (CES-D). Results Fifty patients were included. The assessments with either scale showed the highest score of depressive symptoms in the 24th week of treatment; the mean BDI score before treatment was 6.5 ± 5.3 and the mean CES-D was 10.9 ± 7.8. After 24 weeks, the mean BDI was 16.1 ± 10.2 and mean CES-D was 18.6 ± 13.0; 46% were diagnosed with depression according to combined BDI and CES-D scores. The somatic/psychomotor subscales were highly correlated with overall scale scores . Subjects with history of substance and alcohol abuse had higher risk for IFN-induced depression. Conclusion Treatment with PEG IFN was associated with a high incidence rate of depressive symptoms in this sample of Brazilian patients, as measured by CES-D and BDI. Alcohol and substance abuse increase the risk of PEG IFN-induced depression.

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