PMID: 8597835May 1, 1995Paper

Neonatal diabetes mellitus associated with severe diarrhea, hyperimmunoglobulin E syndrome, and absence of islets of Langerhans

Pediatric Pathology & Laboratory Medicine : Journal of the Society for Pediatric Pathology, Affiliated with the International Paediatric Pathology Association
J Roberts, J Searle

Abstract

Neonatal insulin-dependent diabetes mellitus (DM) is very rare and descriptions of the pancreatic pathology in affected infants vary considerably. Death after 10 months of a male child who suffered the onset of insulin-dependent DM as a neonate, together with severe diarrhea and features of the hyperimmunoglobulin E syndrome, was found to be associated with absence of islets of Langerhans. There was no evidence of any pancreatic exocrine abnormality or other endocrinopathy. Two male relatives with insulin-dependent DM have also died as infants, and after review of the literature it is suggested that this disease process may be part of the spectrum of an X-linked syndrome of diarrhea, polyendocrinopathy, and fatal infection in infancy. Evidence is presented to support the suggestion that this syndrome is an autoimmune disorder.

References

May 1, 1977·Archives of Disease in Childhood·J A Dodge, K M Laurence
Nov 1, 1991·Journal of Pediatric Gastroenterology and Nutrition·M M JonasG T Hensley
Feb 1, 1990·Journal of Pediatric Gastroenterology and Nutrition·M CoulthardR Shepherd
May 1, 1982·The Journal of Pediatrics·B R PowellP Stenzel
Nov 1, 1947·American Journal of Diseases of Children·J SCHWARTZMAND P BEIRNE

❮ Previous
Next ❯

Citations

Mar 12, 2004·Seminars in Neonatology : SN·Michel Polak, Julian Shield
Mar 14, 2007·Orphanet Journal of Rare Diseases·Michel Polak, Hélène Cavé
Dec 20, 2000·The Journal of Clinical Investigation·T A ChatilaA M Bowcock
Feb 19, 2004·Postgraduate Medical Journal·N PecacheJ M Papadimitriou
Sep 1, 2010·Surgical Pathology Clinics·Pierre Russo
Oct 27, 2005·Diabetes·Joseph C KosterColin G Nichols
Sep 30, 2003·Diabetes·Gabrielle S SellickRichard S Houlston
Mar 8, 2000·American Journal of Medical Genetics·P J FergusonT E Kelly
Jun 16, 2001·American Journal of Physiology. Gastrointestinal and Liver Physiology·N ChangK E Barrett
Jan 22, 2005·Immunological Reviews·Hans D OchsTroy R Torgerson

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Diabetes & Tolerance

Patients with type I diabetes lack insulin-producing beta cells due to the loss of immunological tolerance and autoimmune disease. Discover the latest research on targeting tolerance to prevent diabetes.

Autoimmune Polyendocrinopathies

Autoimmune polyendocrinopathies, also called polyglandular autoimmune syndromes (PGASs), or polyendocrine autoimmune syndromes(PASs), are a heterogeneous group of rare diseases characterized by autoimmune activity against more than one endocrine organ, although non-endocrine organs can be affected. Discover the latest research on autoimmune polyendocrinopathies here.

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.