Steven H Erdman

Nationwide Children's Hospital, Columbus, Ohio, United States

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Publications (13)63.29 Total impact

  • Kate M Ellery · Steven H Erdman
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    ABSTRACT: Context: Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive syndrome characterized by dysmorphic nasal alae, ectodermal abnormalities, exocrine pancreatic insufficiency and early growth failure. Most patients are diagnosed by clinical criteria prenatally or in early infancy. Nonsense, frame shift and splice-site mutations of the ubiquitin ligase gene (UBR1) lead to early loss of acinar cells in individuals with JBS. Case report: We describe a previously asymptomatic patient with ectodermal dysplasia presenting with sudden onset exocrine pancreatic insufficiency in adolescence. The family reports an identical twin brother with similar symptoms. Conclusion: This case illustrates that the phenotypic variability of pancreatic involvement in JBS may be subtle and may not manifest until the second decade of life. We suspect that this mild phenotype results from mutations in UBR1 allowing for partial function.
    No preview · Article · Jul 2014 · JOP: Journal of the pancreas

  • No preview · Article · May 2014 · Gastroenterology
  • Sherry C Huang · Carol A Durno · Steven H Erdman
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    ABSTRACT: Colorectal cancer is a rare disease in the pediatric age group and when present suggests an underlying genetic predisposition. The most common hereditary colon cancer susceptibility condition, Lynch syndrome, previously known as Hereditary Non-Polyposis Colorectal Cancer is an autosomal dominant condition caused by a germline mutation in one of four DNA mismatch repair genes: MLH1, MSH2, MSH6 or PMS2. The mutation prone phenotype of this disorder is associated with gastrointestinal, endometrial and other cancers and is now being identified in both symptomatic adolescents with malignancy as well in asymptomatic mutation carriers who are at risk for a spectrum of gastrointestinal and other cancers later in life. We review the DNA mismatch repair system, our current understanding of Lynch Syndrome in the pediatric population and discuss the newly identified bi-allelic form of the disease known as Constitutional Mismatch Repair Deficiency Syndrome. Both family history and tumor characteristics can help to identify patients who should undergo genetic testing for these cancer predisposition syndromes. Patients who carry either single allele (Lynch Syndrome) or double allele (Constitutional Mismatch Repair Deficiency Syndrome) mutations in the mismatch repair genes benefit from cancer surveillance programs that target both the digestive and extra-intestinal cancer risk of these diseases. Since spontaneous mutation in any one of the mismatch repair genes is extremely rare, genetic counseling and testing is suggested for all at-risk family members.
    No preview · Article · Sep 2013 · Journal of pediatric gastroenterology and nutrition

  • No preview · Article · Apr 2013 · Journal of pediatric gastroenterology and nutrition
  • Christina M Laukaitis · Steven H Erdman · Eugene W Gerner
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    ABSTRACT: A number of genetic syndromes are known to convey a high risk of colorectal cancer. Current standards of medical practice for these patients involve genetic testing followed by screening and surgical procedures. Pharmaceutical therapies for any of these syndromes are limited in number and are generally not approved by any regulatory body for applications in these genetic groups. This review discusses advances in mechanistic understanding of the disease processes leading to the development of promising pharmaceutical therapies. Clinical trials of potential chemotherapeutic agents must focus on the reduction of disease-related events, including cancer and cancer-related mortality, in patients with genetic syndromes.
    No preview · Article · Jun 2012
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    ABSTRACT: In neonatal intensive care unit infants referred for home-tube feeding methods, we evaluated the effect of an innovative diagnostic and management approach on feeding outcomes at discharge and 1 year, by comparing data from historical controls; we hypothesized that clinical and aerodigestive motility characteristics at evaluation were predictive of feeding outcomes at discharge; we assessed the economic impact of feeding outcomes. Patients (N = 100) who were referred for development of long-term feeding management strategy at 46.4 ± 13.1 weeks' postmenstrual age were compared with 50 historical controls that received routine care. The focused approach included swallow-integrated pharyngoesophageal manometry, individualized feeding strategy, and prospective follow-up. Feeding success was defined as ability to achieve oral feedings at discharge and 1 year. Motility characteristics were evaluated in relation to feeding success or failure at discharge. Higher feeding success was achieved in the innovative feeding program (vs historical controls) at discharge (51% vs 10%, P < 0.0001) and at 1 year (84.3% vs 42.9%, P < 0.0001), at a reduced economic burden (P < 0.05). Contributing factors to the innovative program's feeding success (vs feeding failure) were earlier evaluation and discharge (both P < 0.05), greater peristaltic reflex-frequency to provocation (P < 0.05), normal pharyngeal manometry (P < 0.05), oral feeding challenge success (P < 0.05), and suck-swallow-breath-esophageal swallow sequence (P < 0.05). Probability of feeding success demonstrated a prediction rate of 79.6%. Short-term and long-term feeding outcomes in complex neonates can be significantly improved with innovative feeding strategies at a reduced cost. Clinical and aerodigestive motility characteristics were predictive of outcomes.
    No preview · Article · Jun 2011 · Journal of pediatric gastroenterology and nutrition
  • Alex Green · Edward G. Shepherd · Steven H. Erdman

    No preview · Article · Jan 2011 · Gastroenterology
  • Tom K Lin · Steven H Erdman
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    ABSTRACT: Double-balloon enteroscopy (DBE) is a newly developed endoscopic modality for diagnosis and treatment of small bowel disorders. Most publications on DBE are from adult medical centers. Publication related to the use and application of DBE in children and adolescents is limited. We present our experience with the use of DBE in the pediatric age group. We reviewed patient information on all of the DBE procedures performed in 2006 through 2008 at a single tertiary pediatric referral center in Columbus, Ohio. Compiled information included patient demographics, procedure indications, diagnostic and therapeutic results, and procedure-associated complications or adverse events. Thirteen DBE procedures were performed on eleven 8- to 20-year-old patients. Procedure indications were based on suspicion for organic small bowel pathology after an exhaustive diagnostic evaluation including upper and lower endoscopy failed to uncover an etiology. Clinically significant lesions were identified in 46% (6/13) of the procedures performed. No serious procedure-related complications occurred. Self-limited postprocedure abdominal pain and discomfort from gaseous distension was observed in several patients. DBE appears to be a safe endoscopic modality for the diagnosis and treatment of children and adolescents with suspected small bowel disease. However, performance should be selectively reserved for patients with a high suspicion for small bowel pathology, in which other less invasive techniques have failed to adequately diagnose and treat a patient's disease.
    No preview · Article · Oct 2010 · Journal of pediatric gastroenterology and nutrition
  • Julie Stoner · Yongyue Qi · Steven H Erdman · Thomas M Attard
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    ABSTRACT: Pediatricians need to competently care for children with hereditary gastrointestinal cancer-predisposing syndromes. Pediatrics resident education on this subject has hithertofore never been studied. Forty-five US pediatrics/internal medicine-pediatrics program directors allowed their residents to participate in an anonymous questionnaire. The survey-questionnaire was administered as a hyperlinked interactive Web page through e-mail to all consenting residents. Thirty-eight sites including 290 of 1327 residents (21.5%) and 33 internal medicine-pediatrics participated in the study. Knowledge on polyposis syndromes varied by syndrome (14% to 84% correct) but not by year of training. Internal medicine-pediatrics residents were more likely to inquire on family history including polyposis, early colorectal cancer, than pediatrics residents. This study suggests that familiarity with cancer syndromes does not accrue during resident training in pediatrics. The observations suggest that greater emphasis on resident education on these syndromes may improve outcomes in this vulnerable group.
    No preview · Article · Oct 2009 · Journal of Cancer Education
  • Sherry C Huang · Steven H Erdman
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    ABSTRACT: Colon polyps are a common finding in pediatrics and can present with rectal bleeding, abdominal pain, or polyp prolapse from the rectum. Histologically classified as hamartomas, these isolated pediatric polyps lack epithelial dysplasia and have no cancer risk. However, when polyps are present in greater numbers, or are associated with a family history of polyps or colon or other cancers, a polyposis or hereditary colorectal cancer syndrome should be considered. Using a case-based format, this article reviews the clinical features and provides updates on the three most common hamartomatous polyp syndromes of childhood: juvenile polyposis syndrome, Peutz-Jeghers syndrome, and the PTEN hamartoma tumor syndrome. Each syndrome has distinctive intestinal and extra-intestinal findings that, when present, can guide genetic counseling and testing. Lifelong cancer surveillance is crucial to disease prevention and the long-term health of these patients and their families.
    No preview · Article · Jul 2009 · Current Gastroenterology Reports

  • No preview · Article · May 2009 · Gastroenterology
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    ABSTRACT: Ataxia-Telangiectasia syndrome is characterized by progressive cerebellar dysfunction, conjuctival and cutaneous telangiectasias, severe immune deficiencies, premature aging and predisposition to cancer. Clinical and radiographic evaluation for malignancy in ataxia-telangiectasia patients is usually atypical, leading to delays in diagnosis. We report the case of a 20 year old ataxia-telangiectasia patient with gastric adenocarcinoma that presented as complete gastric outlet obstruction. A literature search of adenocarcinoma associated with ataxia-telangiectasia revealed 6 cases. All patients presented with non-specific gastrointestinal complaints suggestive of ulcer disease. Although there was no correlation between immunoglobulin levels and development of gastric adenocarcinoma, the presence of chronic gastritis and intestinal metaplasia seem to lead to the development of gastric adenocarcinoma. One should consider adenocarcinoma in any patient with ataxia-telangiectasia who presents with non-specific gastrointestinal complaints, since this can lead to earlier diagnosis.
    Preview · Article · Feb 2009 · World Journal of Surgical Oncology
  • Steven H Erdman
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    ABSTRACT: Juvenile polyps are a common finding in the pediatric population. In contrast, colon adenomas, which are viewed as dysplastic precancerous lesions, are found sporadically in late adulthood. Adenomas in children and young adults are highly unusual and suggest one of several forms of inherited colorectal cancer. These disorders show a predilection to early adenoma formation and can present in childhood. Familial adenomatous polyposis and Lynch syndrome are autosomal dominant, often with involvement of multiple family members, or can be seen in an individual arising from a de novo mutation. The most recently described adenomatous polyposis syndrome, MutYH-associated polyposis, is autosomal recessive, requiring an inherited mutation from each parent. All three adenomatous polyposis disorders can display tremendous variation in expression, even within the same family, and can have a common overlapping phenotype. These disorders require regular medical care to minimize cancer risk in the digestive tract and in other organ systems.
    No preview · Article · Jul 2007 · Current Gastroenterology Reports