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Two Cases of Cystic Fibrosis in Japanese Children: Studies on the Essential Fatty Acid and Prostaglandin Metabolism
Author(s) -
Igarashi Noboru,
Hashimoto Hiroyuki,
Hiratani Michio,
Taniguchi Masashi,
Kasahara Yoshiro,
Sato Tamotsu,
Taniguchi Noboru,
Funabashi Takashi,
Okuda Norihiko,
Yamashiro Yuichiro
Publication year - 1989
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1989.tb01290.x
Subject(s) - medicine , cystic fibrosis , endocrinology , prostaglandin , fatty acid , lipid metabolism , respiratory system , fatty acid metabolism , metabolism , gastroenterology , physiology , biochemistry , biology
We describe the fatty acid (FA) and prostaglandin (PG) metabolism in two Japanese cases of cystic fibrosis (CF) with or without pancreatic insufficiency (PI). The diagnosis of CF was based on the elevated sweat chloride concentration by pilocarpine iontophoresis. A 1‐month‐old boy (case 1) showed poor weight gain, steatorrhea and scaly dermatitis, but no respiratory symptoms were noted. He had decreased levels of serum linoleate and arachido‐nate, and increased palmitoleate and oleate levels, indicating essential fatty acid (EFA) deficiency. Supplementation of fat‐emulsion improved his skin lesions and the altered FA pattern within a few months, associated with the definite reduction of the urinary PC F za levels. Until two years of age, he has been free from respiratory symptoms. A 12‐year‐old girl (case 2) had had recurrent respiratory tract infections due to Pseudomonas ueruginoso and Staphylococcus aureus for several years, and her pancreatic functions were preserved. The FA patterns of her serum lipid were almost within the normal range. These results indicate that 1) the altered FA composition appeared to be a secondary consequence of PI commonly complicating CF and 2) the correction of the altered FA and PC metabolism might have a beneficial effect on the respiratory function of CF patients with EFA deficiency.