Understanding pediatric intestinal pseudo-obstruction: implications for nurses.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Pseudo-obstruction is actually a group of disorders resulting in bowel dysmotility in the absence of anatomic obstruction . Because of varied pathologic abnormalities of the GI tract involved in the disorder , pseudo-obstruction has been reported in the literature under different names , including hypoganglionosis , chronic adynamic ileus , pseudo-Hirschprung 's disease , visceral neuropathy , visceral myopathy , and megacystis-microcolon -intestinal hypoperistalsis syndrome . Although a rare condition , its effects are severe , disabling , and life-threatening in pediatric clients . Prognosis is inversely correlated with age and is especially poor in those children with symptoms as newborns . Management involves promoting nutrition adequate for growth , treating symptoms , and preventing complications . Total parenteral nutrition is almost always required , and its long -term use is associated with chronic cholestasis and potential hepatic failure . Nurses may encounter pediatric clients with this disorder and their families in many different healthcare arenas , such as in radiology departments , ambulatory care , and acute and home care settings . The purpose of this article is to review current literature on this disease and summarize information useful to nurses .

Diseases
Validation

Diseases presenting "neuropathy" symptom

adrenomyeloneuropathy

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

fabry disease

focal myositis

gm1 gangliosidosis

hirschsprung disease

homocystinuria without methylmalonic aciduria

inclusion body myositis

krabbe disease

malignant atrophic papulosis

megacystis-microcolon-intestinal hypoperistalsis syndrome

neuralgic amyotrophy

oculocutaneous albinism

pendred syndrome

phenylketonuria

pyomyositis

pyruvate dehydrogenase deficiency

sneddon syndrome

thoracic outlet syndrome

triple a syndrome

von hippel-lindau disease

waldenström macroglobulinemia

wolf-hirschhorn syndrome

This symptom has already been validated