Hirschsprung's disease presenting as intractable anemia: a report of two cases and review of the literature.
Study Goal
The researchers aimed to investigate the association between intractable anemia and Hirschsprung's disease (HD), focusing on the role of impaired iron absorption due to HD.
Results Summary
The study found that intractable anemia in HD patients was poorly responsive to oral iron supplementation and blood transfusions, but surgical intervention effectively corrected anemia in all cases. The results suggest that HD may impair iron absorption, leading to persistent anemia.
Population
Pediatric patients (ages 11 years and 19 months) with intractable anemia and Hirschsprung's disease.
Effective Dosage
Not specified
Duration
Varied (6-year and 3-month histories of anemia prior to intervention)
Interactions
None mentioned
| Intervention | Direction | Endpoint | Population | Dosage | Impact | Claim # |
|---|---|---|---|---|---|---|
oral iron supplementation | no change | intractable anemia | An 11-year-old boy with a 6-year history of intractable anemia | - | poor response | #1 |
blood transfusion | no change | intractable anemia | An 11-year-old boy with a 6-year history of intractable anemia | - | poor response | #2 |
surgery | decrease | anemia | An 11-year-old boy with a 6-year history of intractable anemia | - | corrected effectively | #3 |
oral iron supplementation | no change | intractable anemia | A 19-month-old boy with a 3-month history of intractable anemia | - | poor response | #4 |
blood transfusion | no change | intractable anemia | A 19-month-old boy with a 3-month history of intractable anemia | - | poor response | #5 |
surgery | decrease | anemia | A 19-month-old boy with a 3-month history of intractable anemia | - | corrected effectively | #6 |
surgery | decrease | anemia | Two more cases of intractable anemia as the chief complaint and diagnoses of HD | - | corrected | #7 |
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.