Whipple's disease ( intestinal lipodystrophy )

 



* It's form of malabsorption disease, caused by tropheryma whipplei organism. 

* Typically in white men of middle age 

* It's rare disease with unknown risk factors 


Symptoms 

 * The symptoms start slowly 
 
 * Joint pain is the most common early symptoms

 * GIT symptoms occure several years later 

 * Other symptoms like  
      
                " Abdominal pain    " Diarrhea   " Fever   " Darkening of skin in light exposed area of the skin

                " Joint pain [ ankle - knee - elbow - fingers - others ] 
  
                " Memory loss       " Weight loss     " mental changes 

Physical Exam 

 * Enlarged lymph nodes

 * Heart murmurs 

 * Oedema

Complications 

 * Brain damage 
 
 * Heart valve damage 

 * Weight loss

 * Symptoms return

Investigations

 * CBC      * PCR    * Fecal fat     * Albumin level       * D-xylose absorption 

*  Biopsy of small Bowel on periodic acid-schiff stain ( PAS ) positive macrophages  in lamina 

     properia contain Non-acid fast gram +ve bacilli

 * Upper GIT  endoscopy

Treatment

* Long term antibiotics to cure against CNS infection 👇
   
             " Ceftraixone IV then TM-SM up to 1 year

             " If symptoms back during antibiotics use → may change antibiotics

* Dietary supplements for malnutrition