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Fever, intestinal symptoms may delay diagnosis of Kawasaki disease in children

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Abdominal-first presentation is similar to lymph node-first presentation of Kawasaki disease

Adding abdominal pain–first presentation to Kawasaki disease is not unprecedented considering lymph node–first presentation was first introduced as a concept in the Journal of Pediatrics in 2013, Sarah S. Long, MD, wrote in a related editorial.

“It should not be too surprising that intestinal vasculitis could be significant in some cases,” Dr. Long said. “Might it not suggest an intestinal portal of microbe or super antigen entry, as might cervical lymphadenitis a respiratory tract portal of entry?”

Dr. Long noted diagnostic and reporting bias was most likely the cause of the 43% rate of coronary artery aneurysms reported in the study by Colomba et al, but said that “it behooves us all to consider Kawasaki disease in the differential when a child has high fever and abdominal pain.”

Dr. Long is a professor of pediatrics at Drexel University, Philadelphia. She made her comments regarding the article by Colomba et al. in the Journal of Pediatrics (2018 Jul 17. doi: 10.1016/j.jpeds.2018.09.018 ). Dr. Long is on the editorial board of the journal, served as the chief editor on and receives royalties from “Principles and Practice of Pediatric Infectious Diseases,” and serves as the associate editor of the Red Book Report of the American Academy of Pediatrics Committee on Infectious Diseases.



Symptoms of gastrointestinal involvement such as abdominal pain and vomiting may delay diagnosis of Kawasaki disease in pediatric patients.

Child with IV in hospital bed NaiyanaDonraman/Thinkstock

“The clinical onset of Kawasaki disease with gastrointestinal involvement often leads to diagnostic and therapeutic delays – a risk factor for the development of coronary complications,” Claudia Colomba, MD, from the department of sciences for health promotion and mother and child care at the University of Palermo (Italy), and her colleagues wrote in the Journal of Pediatrics.

After caring for a boy aged 14 years at their center who presented with these symptoms, Dr. Colomba and her colleagues performed a search of the PubMed and Scopus databases and identified 33 articles with 48 total cases of Kawasaki disease with intestinal involvement between 1979 and 2017.

There were 40 cases of fever (82%), 34 cases of abdominal pain (69%), and 24 cases of vomiting (49%) at disease onset, with diarrhea occurring in 14 cases (29%) and jaundice in 1 case (2%), the researchers noted. Cardiac involvement occurred in 21 cases (43%). With regard to imaging, 38 cases of pseudo-obstruction (77%) were identified by plain radiograph, ultrasonography, and CT. Over half of the cases required surgery; of these 25 cases (51%), 8 cases involved a resection of the restricted loop and included a temporary colostomy (16%), 5 cases were exploratory laparotomy (10%), and there was 1 case with enterolysis (2%).

A total of 45 patients received medical treatment, with 12 patients (25%) receiving intravenous immunoglobulin and 18 (37%) receiving intravenous immunoglobulin plus aspirin. One patient had cyanosis and hand and foot gangrene. There were three patients who died, with massive liver necrosis identified during the autopsy of one patient. Of the other two who died, one did so 2 days after exploratory laparotomy and the other died because of Pseudomonas septic shock.

The researchers reported a good outcome in 28 patients (57%), which included 3 cases where there was no treatment.

“The diagnosis of Kawasaki disease should be considered in all children with fever, abdominal pain, and radiologic signs of pseudo-obstruction, even in the absence of typical symptoms and signs,” Dr. Colomba and her colleagues wrote. “A more comprehensive analysis including all clinical forms of Kawasaki disease would be useful to correlate intestinal involvement with worse outcomes for cardiac complications, as well as to clues to more rapid diagnosis and avoidance of unnecessary invasive procedures.”

The authors reported no relevant conflicts of interest.

SOURCE: Colomba C et al. J Pediatr. 2018 Jul 17. doi: 10.1016/j.jpeds.2018.06.034.

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