title: Hyperprostaglandin E syndrome: use of indomethacin and steroid, and death due to necrotizing enterocolitis and sepsis.
abstract: Hyperprostaglandin E syndrome (HPS) is the antenatal variant of Bartter syndrome and characterized by polyhydramnios and preterm delivery in the antenatal period and salt-wasting, isosthenuric or hyposthenuric polyuria, hypercalciuria and nephrocalcinosis in the postnatal period. We report a one-month-old infant with HPS with a 15-year-old sister with Bartter syndrome. The infant's birth weight was 2750 g and she had severe dehydration on the 2nd day of life. She had hypercalcemia, hyponatremia, hypokalemia, metabolic alkalosis and elevated plasma renin and aldosterone levels. We instituted indomethacin therapy accompanied by steroid therapy for hypercalcemia. However, the patient developed abdominal distention on the 30th day, which was due to diffuse pneumatosis in sigmoid colon revealed by a subsequent surgical intervention. Following surgery, the patient developed fever, electrolyte abnormalities and subsequently sepsis. The patient died due to sepsis 10 days after surgery. We conclude that indomethacin and steroid therapy must be used cautiously in infants with HPS.
authors.affiliation: Division of Neonatology, Department of Pediatrics, Uludağ University Faculty of Medicine, Bursa, Turkey.
authors.forename: Merih
authors.initials: M
authors.lastname: Cetinkaya
authors.forename: Nilgün
authors.initials: N
authors.lastname: Köksal
authors.forename: Hilal
authors.initials: H
authors.lastname: Ozkan
authors.forename: Osman
authors.initials: O
authors.lastname: Dönmez
authors.forename: Halil
authors.initials: H
authors.lastname: Sağlam
authors.forename: Irfan
authors.initials: I
authors.lastname: Kiriştioğlu
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001477:Bartter Syndrome / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology*; D020345:Enterocolitis, Necrotizing / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007213:Indomethacin / Q000627:therapeutic use; D007231:Infant, Newborn; D006831:Polyhydramnios; D011247:Pregnancy; D018805:Sepsis / Q000150:complications*; D013256:Steroids / Q000627:therapeutic use
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D013256:Steroids; D007213:Indomethacin