Hirschsprung's disease presenting as intractable anemia: a report of two cases and review of the literature.

Abstract:

BACKGROUND:This report summarizes the clinical characteristics of intractable anemia as part of the clinical presentation of Hirschsprung's disease (HD) and aims to strengthen clinicians' ability to recognize early signs of HD. CASE PRESENTATION:An 11-year-old boy with a 6-year history of intractable anemia, low hemoglobin level (55 g/L), poor response to oral iron supplementation and blood transfusion, and difficulty with defecation was diagnosed with HD. A 19-month-old boy with a 3-month history of intractable anemia, low hemoglobin level (64 g/L), poor response to oral iron supplementation and blood transfusion, delayed meconium passage, and history of intestinal obstruction was also diagnosed with HD. Both patients underwent surgery, after which anemia was corrected effectively in both cases. Two more cases of intractable anemia as the chief complaint and diagnoses of HD over different durations since the onset of anemia (ranging from 1.7 years to 21 years) were identified in a literature search. Both patients underwent surgery, after which anemia was corrected. CONCLUSIONS:Intractable anemia as part of the clinical presentation of HD is extremely rare. Detailed inquiries of medical histories and physical examinations are key to early diagnoses and preventing misdiagnoses. Anemia in HD patients may primarily be caused by impaired iron absorption due to HD.

journal_name

BMC Pediatr

journal_title

BMC pediatrics

authors

Sun X,Chu J,Li C,Deng Z

doi

10.1186/s12887-020-02423-z

subject

Has Abstract

pub_date

2020-11-16 00:00:00

pages

525

issue

1

issn

1471-2431

pii

10.1186/s12887-020-02423-z

journal_volume

20

pub_type

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