Correspondence

Case 11-2003: Ulcerative Colitis and Primary Sclerosing Cholangitis in a 14-Year-Old Boy

N Engl J Med 2003; 349:1482October 9, 2003

Article

To the Editor:

Case 11-2003 (April 10 issue)1 describes a 14-year-old boy with recurrent cholangitis complicating ulcerative colitis. The authors state that this presentation is unusual in such a young patient. We report a similar experience of a malignant duodenal tumor complicating the treatment of an 11-year-old boy with X-linked hyper-IgM combined immunodeficiency.2

The histologic diagnosis was duodenal neuroendocrine carcinoma, which makes this boy the youngest reported patient with this condition. The association of neuroendocrine tumors with the X-linked hyper-IgM syndrome is recognized and has been linked with hepatobiliary cryptosporidiosis and cholangitis, justifying early liver transplantation.3 In the case of our patient, lifelong precautions against cryptosporidiosis were pursued, including the sterilization of drinking water, surveillance microscopy of the stool, and liver biopsy. In Case 11-2003, the cause may be assumed to be chronic inflammation, but in our patient's case, neither infection nor cholangitis was identifiable.

These cases illustrate two points. First, the intensive management of previously life-threatening conditions permits complications to present at increasingly younger ages. Second, the early presentation of a tumor in a child with an inherited condition in a case in which the presumed mechanism of carcinogenesis is absent calls into question the role of the underlying defect (e.g., the role of CD40 ligand) in carcinogenesis.

Elizabeth Drewe, M.B., B.S.
Abed M. Zaitoun, M.D., Ph.D.
David A. Walker, M.B., B.S.
Queens Medical Centre, Nottingham NG7 2UH, United Kingdom
liz.drewe@nottingham.ac.uk

3 References

1. 1

   Case Records of the Massachusetts General Hospital (Case 11-2003). N Engl J Med 2003;348:1464-1476
   Full Text | Web of Science | Medline

2. 2

   Levy J, Espanol-Boren T, Thomas C, et al. Clinical spectrum of X-linked hyper-IgM syndrome. J Pediatr 1997;131:47-54
   CrossRef | Web of Science | Medline

3. 3

   Hayward AR, Levy J, Facchetti F, et al. Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM. J Immunol 1997;158:977-983
   Web of Science | Medline

To the Editor:

Table 3 in the Case Records is misleading. Immunodeficiency states and cryptosporidium infection are listed as conditions associated with primary sclerosing cholangitis, yet these conditions are only two of many diverse causes of secondary sclerosing cholangitis.1,2

At the risk of appearing pedantic, I would argue that it is prudent for clinicians to distinguish primary from secondary causes of sclerosing cholangitis, since such a distinction will influence patient care. Knowledge of the diseases associated with primary sclerosing cholangitis may hasten diagnosis by an astute clinician, but these diseases should not be confused with secondary causes of sclerosing cholangitis.3

Sandeep Mukherjee, M.D.
University of Nebraska Medical Center, Omaha, NE 68198-3285
smukherjee@surgery.unmc.edu

3 References

1. 1

   Lindor KD, Larusso NF. Primary sclerosing cholangitis. In: Schiff ER, Sorrell MF, Maddrey WC, eds. Schiff's diseases of the liver. 9th ed. Philadelphia: Lippincott Williams & Wilkins, 2003:674-8.
   

2. 2

   Lee Y-M, Kaplan MM. Primary sclerosing cholangitis. N Engl J Med 1995;332:924-933
   Full Text | Web of Science | Medline

3. 3

   Sclerosing cholangitis. In: Sherlock S, Dooley J. Diseases of the liver and biliary system. 11th ed. Oxford, England: Blackwell Science, 2002:255-65.
   

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