Rare Diseases Symptoms Automatic Extraction

Operative management of splenic injury in a patient with proteus syndrome.

[proteus syndrome]

A 20-year-old female with Proteus syndrome sustained splenic injury after fall from a bike. She was initially managed non-operatively at a different hospital for three days and was then referred to our level I trauma center in view of increasing abdominal pain and distention. On admission in the Emergency Department (ED), her pulse rate was 120 per minute and blood pressure was 108/68 mm Hg. Clinical examination showed a distended abdomen with left hypochondrial pain. Ultrasonogram (USG) and Computed Tomography (CT) of the abdomen showed splenomegaly and grade III splenic injury with significant hemoperitoneum. Her hemoglobin was 2.9 g/dl with packed cell volume (PCV) of 12%. In view of low hemoglobin and possibility of pathologic spleen, splenectomy was done. Microscopic examination of the spleen showed hemangiolymphangioma. The patient was discharged on the 5(th) post-operative day and is doing well at 6 months of follow-up.

Diseases presenting "blood pressure" symptom

  • acute rheumatic fever
  • adrenal incidentaloma
  • alpha-thalassemia
  • cadasil
  • congenital adrenal hyperplasia
  • congenital diaphragmatic hernia
  • cushing syndrome
  • fabry disease
  • familial mediterranean fever
  • lymphangioleiomyomatosis
  • pendred syndrome
  • proteus syndrome
  • scrub typhus
  • systemic capillary leak syndrome
  • thoracic outlet syndrome
  • typhoid
  • von hippel-lindau disease
  • werner syndrome

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