The natural history of Dandy-Walker syndrome in the United States: A population-based analysis

ABSTRACT Background: Dandy-Walker syndrome (DWS) is a congenital disorder typically manifesting with hydrocephalus. The classic anatomic hallmarks of DWS are hypoplasia of the cerebellar vermis, anterior-posterior enlargement of the posterior fossa, upward displacement of the torcula and transverse sinuses, and cystic dilatation of the fourth ventricle. Aims: Although optimal treatment of DWS typically requires neurosurgical intervention to prevent intracranial pressure increases incompatible with life, the natural history of this disorder has yet to be evaluated on a nationwide level. Settings and Design/Materials and Methods: The Kids’ Inpatient Database covering 1997-2003 was used for analysis. Children younger than age 18 admitted for DWS (ICD-9-CM = 742.3) were analyzed with a matched control group. The primary procedure codes for operative CSF drainage were coded into the analysis. The incidence of DWS was 0.136%; 14,599 DWS patients were included. Statistical Analysis Used: Multiple logistic regression models were used. Odds ratios (OR) were reported with 95% confidence intervals. Results and Conclusions: Mortality (OR = 10.02; P < 0.0001) and adverse discharge disposition (OR = 4.59; P < 0.0001) were significantly greater in DWS patients compared with controls. 20.4% of DWS patients received operative cerebrospinal fluid (CSF) drainage, 81-times more than controls (P < 0.0001). CSF drainage reduced mortality by 44% among DWS patients (P < 0.0001). Although DWS is associated with a 10-fold increase in mortality, operative CSF drainage nearly halves the mortality rate. Based on these findings (Class IIB evidence), it is likely that the increased mortality associated with DWS is directly attributable to the nearly 80% of DWS patients who did not receive operative CSF drainage for hydrocephalus. Consequently, increased access to neurosurgical intervention could reduce the mortality rate of DWS towards that of the general population.

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76 The Prevalence and Natural History of Treatments of Hemorrhoid Disease: A Population-Based Survey of Adults in the United States

Gastroenterology ◽

10.1016/s0016-5085(12)60071-7 ◽

2012 ◽

Vol 142 (5) ◽

pp. S-18 ◽

Cited By ~ 1

Author(s):

Philip B. Miner ◽

Monil Shah ◽

Barry Tannenholz ◽

Cory Davis

Keyword(s):

United States ◽

Natural History ◽

The United States ◽

Population Based ◽

History Of ◽

Population Based Survey

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Mo1018 The Epidemiology and Natural History of Hemorrhoid Disease Among Adults: A Population-Based Survey in the United States

Gastroenterology ◽

10.1016/s0016-5085(12)62200-8 ◽

2012 ◽

Vol 142 (5) ◽

pp. S-573-S-574

Author(s):

Philip B. Miner ◽

Monil Shah ◽

Barry Tannenholz ◽

Cory Davis

Keyword(s):

United States ◽

Natural History ◽

The United States ◽

Population Based ◽

History Of ◽

Population Based Survey

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HIP (HPA-screening in pregnancy) study: protocol of a nationwide, prospective and observational study to assess incidence and natural history of fetal/neonatal alloimmune thrombocytopenia and identifying pregnancies at risk

BMJ Open ◽

10.1136/bmjopen-2019-034071 ◽

2020 ◽

Vol 10 (7) ◽

pp. e034071

Author(s):

Dian Winkelhorst ◽

Thijs W de Vos ◽

Marije M Kamphuis ◽

Leendert Porcelijn ◽

Enrico Lopriore ◽

...

Keyword(s):

High Risk ◽

Natural History ◽

Pregnant Women ◽

Perinatal Death ◽

Population Based ◽

Bleeding Complications ◽

Control Group ◽

History Of ◽

Alloimmune Thrombocytopenia ◽

In Pregnancy

IntroductionFetal and neonatal alloimmune thrombocytopenia (FNAIT) may lead to severe fetal or neonatal bleeding and/or perinatal death. Maternal alloantibodies, targeted against fetal human platelet antigens (HPAs), can result thrombocytopenia and bleeding complications. In pregnancies with known immunisation, fetal bleeding can be prevented by weekly maternal intravenous immunoglobulin infusions. Without population-based screening, immunisation is only detected after birth of an affected infant. Affected cases that might have been prevented, when timely identified through population-based screening. Implementation is hampered by the lack of knowledge on incidence, natural history and identification of pregnancies at high risk of bleeding. We designed a study aimed to obtain this missing knowledge.Methods and analysisThe HIP (HPA-screening in pregnancy) study is a nationwide, prospective and observational cohort study aimed to assess incidence and natural history of FNAIT as well as identifying pregnancies at high risk for developing bleeding complications. For logistic reasons, we invite rhesus D-negative or rhesus c-negative pregnant women, who take part in the Dutch population-based prenatal screening programme for erythrocyte immunisation, to participate in our study. Serological HPA-1a typing is performed and a luminex-based multiplex assay will be performed for the detection of anti-HPA-1a antibodies. Results will not be communicated to patients or caregivers. Clinical data of HPA-1a negative women and an HPA-1a positive control group will be collected after birth. Samples of HPA-1a immunised pregnancies with and without signs of bleeding will be compared with identify parameters for identification of pregnancies at high risk for bleeding complications.Ethics and disseminationEthical approval for this study has been obtained from the Medical Ethical Committee Leiden-The Hague-Delft (P16.002). Study enrolment began in March 2017. All pregnant women have to give informed consent for testing according to the protocol. Results of the study will be disseminated through congresses and publication in relevant peer-reviewed journals.Trial registration numberNCT04067375.

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Use of the Rochester Epidemiology Project for Clinical Research in Colon and Rectal Surgery

Clinics in Colon and Rectal Surgery ◽

10.1055/s-0038-1673349 ◽

2019 ◽

Vol 32 (01) ◽

pp. 008-015

Author(s):

Robert Cima ◽

Amy Lightner

Keyword(s):

United States ◽

Natural History ◽

Rectal Surgery ◽

The United States ◽

Population Based ◽

Colon And Rectal Surgery ◽

Unique Identifier ◽

Rochester Epidemiology Project ◽

History Of ◽

The Impact

AbstractThe Rochester Epidemiology Project (REP), a longitudinal population-based database, is the largest epidemiologic database in the world. Originally established at Mayo Clinic in Rochester, Minnesota, the REP has been instrumental in defining the natural history of disease states and the impact of treatment in a well-defined population. In the United States, the REP has made important contributions to the colon and rectal literature, largely because healthcare is fragmented with no unique identifier to longitudinally follow-up with a patient throughout the system over his or her lifespan. Investigation with the REP has provided insight to the economic burden associated with inflammatory bowel disease, the benefit of screening for colorectal cancer, and the natural history of Mekel's and diverticular disease. In addition to practice changing research, the REP can be used as a model for future linkage systems in the United States.

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