Dr Theresa Raju,Dr C.Jayakumar,DrBhanu Vikraman Pillai Dr Praveena,DrAnupa Dr Navya George
6 year old female presented with high grade intermittent fever since 1 month associated with periumblical pain and recurrent oral ulcers . History is negative for conditions that affect other system The child was treated outside hospital on two occasion prior to this for fever and received antibiotics also
In the present admission child was febrile and pale .Child had hypopigmented lesions in the neck and multiple ulcers in the hard palate.Systemic examination was normal.
Differentials
EB virus infection
Periodic fever Aphthous stomatitis pharyngitis and adenitis (PFAPA)
Streptococcal pharyngitis
SLE
Stress
Drug induced
Bechets disease
Inflammatory bowel disease
Haematinic Deficiency
LAB
CRP:68.53mg/L,
Serum Ferritin:246ng/ml,
LDH:146U/L,
Fibrinogen:577.1mg/dl
Triglycerides of 79.2mg/dl.
USG abdomenshowed ?Mesentric Lymphadenitis,?Left mild prominence of pelvicalyceal system.
PUO workup
Salmonella negative
Brucella and EBV IgM were equivocal.
Stool culture and blood culture sterile.
Urine culture enterococcus species contaminant.
TB work up negative.
As fever persisited and brucella IgM was equivocal ,treatment for Brucella was initiated with Doxycycline and Rifampicin.
Echo done to rule out any features of Infective Endocarditis/vasculitis which was normal.
Rheumatology investigations like ANA IFA,ANA blot and DCT were done where ANA IFA was 4+(homogeneous) and ANA ds DNA and ANA profile were negative.
Fecal calprotectin was done which showed an elevated value of 306mcg/gm,hence pediatrics gastroenterology consultation was availed and advised for UGI Scopy and Colonoscopy.
Upper GI scopy was suggestive of Gastritis and colonoscopy revealed scattered aphthous ulcers over the colon and terminal ileum which was suggestive of Crohns.
MRI Enterogram Showed involvement of jejunum and duodenum and no evidence of active small bowel disease.
Biopsy done showed chronic active granulomatous inflammation of ileum and Granulomatous colitis with variable mild activity which was indicative of Crohns disease.
Diagnosis: Crohns disease
The child was started on Oral Azathioprine 25mg,Tapering doses of steroids,PPIs and Vitamin D
The child is on regular follow up
Discussion: Main feature of Crohns is patchy segmental transmural chronic inflammation of gut from oral cavity to anus.GI manifestation include abdominal pain and tenderness,anal fistulas,skin tags.Non specific symptoms include fever,anorexia
Extra-intestinal manifestation include aphthous stomatitis,arthralgia, necrotic skin lesions, Musculoskeletal involvement ,Growth failure and rarely renal problems
Investigations include CBC,ESR ,
Xray May reveal intestinal obstruction,bowel dilatation and upper GI studies(single and double contrast).
Double contrast Barium enema -shows skip lesions,loss of haustration,narrowing of colon,cobblestone appearance.
Endoscopy reveals shallow aphthous gastric ulcers or deep penetrating ulcers.Intestinal biopsy shows changes in terminal ileum.
Treatment :Treatment of malnutrition and induction of remission,Steroids,Anti inflammatory drugs,Immunosupressants,Antibiotics and Bile acid binding agents.
1) Induction of remission:Exclusive enteral nutrition(EEN)-for children with luminal CD
In moderate to severe luminal CD:Oral corticosteroids(Prednisolone 1-2mg/kg/day)
In mild luminal CD:Azithromycin and rifaximin can be used
In mild to moderate ileocaecal CD:Budesonide can be used orally
Steroids are given at full dose of 2-4 weeks followed gradual tapering over the next 8 weeks
For perianal CD-Metronidazole(10-20mg/kg/day)and ciprofloxacin(20mg/kg/day) are used
In steroid refractory CD:Anti TNF therapy with agents like Infliximab is used for induction of remission
2) Maintenance of remission:Thiopurines are the recommended agents for maintaining steroid free remission in children with CD
Methotrexate can be used as monotherapy for maintenance of remission.Can also be used as a second line drug in children with Thiopurine failure.Dosage:15mg/m2 weekly as subcutaneous injection.Oral folic acid is given 24 hours after methotrexate is given.
Anti TNF agents:for maintaining remission in children with chronic active luminal CD.Maintenance dose:5mg/kg every 8 weeks
INDICATIONS FOR SURGERY IN CD:
Failure of medical therapy
Growth failure despite maximal therapy
Extraintestinal involvement
Take home message
PUO is a night mare for paediatricians
Tempo of investigation depends on tempo of illness from non invasive to invasive
All investigations and interventions available in the literature is not justified on presentation
In most of the cases Broad spectrum antibiotics if we start it blindly will fail
Specialists help may be availed based up on the merit of the case