Edema as the Presenting Symptom of Cystic Fibrosis: Difficulties in Diagnosis FREE

TANIA GUNN, MB, CHB; Mimi M. BELMONTE, MD; ELEANOR COLLE, MD; CLAIRE DUPONT, MD, PHD

Am J Dis Child. 1978;132(3):317-318. doi:10.1001/archpedi.1978.02120280101023.

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ABSTRACT

ABSTRACT | REFERENCES

Edema, hypoproteinemia, and anemia may be the presenting symptoms in young infants with cystic fibrosis (CF) of the pancreas. These symptoms are believed to be due to defective protein digestion and malabsorption secondary to trypsin and chymotrypsin deficiency, and are seen most frequently in infants fed either a soy bean formula¹ that contains a trypsin inhibitor,² or human milk that has a lower protein content than most commercial formulas.³ Other causes of this symptom complex include inadequate intake of protein, decreased protein synthesis in severe liver disease, increased protein degradation in transient dysproteinemia, pseudohypoproteinemia in states of plasma volume expansion such as congestive heart failure, or when there is protein loss in the urine as in the nephrotic syndrome or into the gut in exudative enteropathy. The finding of an elevated chloride concentration in the sweat is the cornerstone of the diagnosis of CF. There have been

REFERENCES

ABSTRACT | REFERENCES

Fleisher DS, DiGeorge AM, Barness LA, et al: Hypoproteinemia and edema in infants with cystic fibrosis of the pancreas . J Pediatr 64:341-348, 1964;.
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Mulne A, McClung HJ, Tokarski P: Anti-trypsin activity in soy formulas . Pediatr Res 10:358, 1976;.

Billee BS, Vahlquist B: Idiopathic hypoproteinemia versus hypoproteinemia due to pancreatic dysfunction . Acta Pediatr 44:435-443, 1955;.
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Goldman AS, Travis LB, Dodge WF, et al: Falsely negative skin tests in children with cystic fibrosis complicated by hypoproteinemic oedema . J Pediatr 59:301, 1961;.

Anderson C: Fibrocystic disease of the pancreas presenting with oedema and hypoproteinemia . Med J Aust 2:195-196, 1966;.

Masi M, Lazzari R: Forma anemico edematosa della mucoviscidosi . Clin Pediatr 53:55-72, 1971;.

MacLean WC Jr, Tripp RW: Cystic fibrosis with edema and falsely negative sweat tests . J Pediatr 83:86-88, 1973;.
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Lee PA, Roloff DW, Howatt MD: Hypoproteinemia and anemia in infants with cystic fibrosis: A presenting symptom complex often misdiagnosed . JAMA 228:585-588, 1974;.
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Vlachos P: Cystic fibrosis with edema and falsely negative sweat test . J Pediatr 84:926, 1974;.

Dolan TF, Rowe DS, Gibson LE: Edema and hypoproteinemia in infants with cystic fibrosis: The hypoalbuminemia sometimes seen is presumably due to malabsorption . Clin Pediatr 9:295-297, 1970;.
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Lock J: Breast milk-induced hypoproteinemic shock, coagulopathy, and siderocystic anemia in cystic fibrosis . J Pediatr 84:912, 1974;.

Paxson CL Jr: Idiopathic hypoproteinemia in twins . JAMA 230:1257-1258, 1974;.
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Bass HN, Miller AA: Cystic fibrosis presenting with anemia and hypoproteinemia in identical twins . Pediatrics 59:126-127, 1977;.

Sahidi NT, Diamond LK, Schwachman H: Anemia associated with protein deficiency: A study of two cases with cystic fibrosis . J Pediatr 59:533-541, 1961;.
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Roger R, Finegold MJ: Cholestasis in immature newborn infants: is parenteral nutrition responsible? J Pediatr 86:264-269, 1975;.
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