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- Suneel Mundkur, DCH DNB*
- Sowmya Shashidhara, MD*
- Shrikiran Hebbar, MD*
- Shravan Kanaparthi, MD*
- *Department of Pediatrics, Women and Child Block, Kasturba Medical College, Manipal Academy of Higher Education, Madhav Nagar, Manipal, Karnataka, India
AUTHOR DISCLOSURE
Drs Mundkur, Shashidhara, Hebbar, and Kanaparthi have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.
Presentation
A 6-year-old girl of eastern Himalayan origin presents with a 1-year history of recurring fever and abdominal pain. The fever is intermittent and high grade, associated with chills and sweating, with recurrences every 2 to 3 weeks. She also reports left flank and abdominal pain, with some associated distention. She feels full easily during meals and often wakes up from sleep due to pain. There is no pedal edema, breathlessness, decreased urination, or facial puffiness. She feels easily fatigued and is described as getting more “white” by the parents. Her family and developmental histories are normal. Details of previous evaluation and care are not available.
On examination, she appears pale, quiet, and lethargic. Her pulse is 118 beats/min, her blood pressure 94/60 mm Hg, her respiratory rate is 22 beats/min, and her temperature is 101.2°F (38.4°C). Her weight and height are less than the third percentile. Diffuse hyperpigmentation is noted. There is no cyanosis, lymphadenopathy, clubbing, edema, or icterus. Abdominal examination reveals an increased girth of 46.5 cm, prominent veins, a firm liver edge 4.5 cm below the right costal margin with a liver span of 11 cm, and a massive spleen span of 11.5 cm. Results of the cardiovascular, respiratory, and central nervous system examinations are normal.
She has normal liver enzyme levels and a normal coagulation profile. Her renal function test results and electrolyte levels are normal. Her serum total protein level is elevated at 9.70 g/dL (97 g/L) (reference range, 6–8 …
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