Delayed Recognition of Kawasaki Disease in Children with Presenting Intestinal Involvement

A recent study (C Columba et al. Full text: Intestinal Involvement in Kawasaki Disease J Pediatr 2018; 202: 186-93) indicates that when patients with Kawaski’s present with predominantly intestinal symptoms that this may lead to a delay in diagnosis and potential cardiac complications. In this systematic review, 33 articles reporting 48 cases of Kawasaki disease with intestinal involvement were considered.

Some key points:

  • In this study, abdominal pain and vomiting were the most frequently reported symptoms, followed by diarrhea. Fever was typically present but other features of Kawaski disease were not apparent at presentation.  Presentation can include dilated bowel loops suggestive of obstruction (pseudo-obstruction), pancreatitis, and intestinal vasculitis/bowel wall thickening.
  • “The prevalence of gastrointestinal involvement in Kawasaki disease is unknown as available data can only be derived from single reports and few case series. Miyake et al in their retrospective case series of 310 children with Kawasaki disease reported gastrointestinal involvement in only 7 cases (2.3%).”
  • “Abdominal symptoms in Kawasaki disease are more often due to hydrops of the gallbladder. In Taiwan, a routine abdominal ultrasound showed hydrops of the gallbladder in 21% of patients with Kawasaki disease.”

My take: Since early treatment of Kawasaki disease may improve outcomes, it is worthwhile to consider this in the differential diagnosis of patients presenting with possible obstruction and in those who have fever in addition to pain/emesis.

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Liver Briefs 2017

Briefly noted:

RJ Fontana et al. Hepatology 2016; 64: 1870-80.  In this study of 681 adults with acute liver failure in U.S., only 3 had detectable anti-HEV IgM and all three were negative for HEV-RNA.  In addition, other putative causes of acute liver failure were present in all three.  My take: Hepatitis E is very rare explanation for acute liver failure in the U.S.

RA Rosencrantz et al. Hepatology 2016; 64: 2253-6. Case report of 2.5 yr old with autoimmune sclerosing cholangitis with Kawasaki disease. This was a well-described case with MRCP and liver histology. My take: In patients with Kawasaki with protracted liver disease, another etiology to consider.

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Cholestatic Kawasaki Disease

Periodically, Kawasaki disease (KD) will present with fever and cholestasis (JPGN 2012; 55: 380-83).

The authors of this study which took place between 2003-2010 reviewed the presentation of children less than 16 years of age who presented with fever on admission (>38.5), total bilirubin >3 mg/dL and elevated ALT values.  In all 24 patients who met criteria for review; 5 (21%) had KD.  Patients with KD ranged in age between 1-10 years.

The other causes included viral hepatitis in 13 (EBV, HAV, CMV, Adenovirus, HSV), 4 had drug-induced cholestasis, 1 with cholelithiasis, and 1 with a choledochal cyst.

In almost 20% of KD patients, the presentation does not meet all of the diagnostic criteria: at least 5 days of fever and 4 of 5 following conditions: bilateral nonpurulent conjunctival injection, oral mucosal changes, peripheral extremity changes (edema/erythema of palms/soles, desquamation of fingers/toes), rash, cervical lymphadenopathy (unilateral, >1.5 cm).

Also, for those who did not receive yesterday’s blog on NASPGHAN postgraduate course, check out the following link:

NASPGHAN Postgraduate Course 2012 | gutsandgrowth

Additional references:

  • Kawasaki disease – Wikipedia, the free encyclopedia

  • -J Pediatr 2011; 158: 644.  Infliximab may work better than IVIG for Kawasaki.
  • -J Pediatr 2009; 155: 695.  Reviews recent experience & criteria for Kawasaki.
  • -Pediatrics 2004; 114: 1708. guidelines from AAP for Kawasaki.