Hexachlorobenzene episode in Turkey.
During the period 1955-1959, approximately 4000 people in southeast Anatolia developed porphyria due to the ingestion of hexachlorobenzene (HCB), a fungicide added to wheat seedlings. These HCB exposures subsequently led to the development of bullae on sun-exposed areas, hyperpigmentation, hypertrichosis, and porphyrinuria. The condition was called kara yara or "black sore." Many of the breast-fed children under the age of 2 years whose mothers had ingested HCB-treated grain died from a disease known as pembe yara or "pink sore." In this follow-up study of 252 patients, 20-30 years postexposure, there were 162 males and 90 females, with an average current age of 35.7 years, an average of onset of 7.6 years, and a duration of 2.2 years. Many patients had dermatologic, neurologic, and orthopedic symptoms and signs. The observed clinical findings include scarring of the face and hands (83.7%), hyperpigmentation (65%), hypertrichosis (44.8%), pinched facies (40.1%), painless arthritis (70.2%), small hands (66.6%), sensory shading (60.6%), myotonia (37.9%), cogwheeling (41.9%), enlarged thyroid (34.9%), and enlarged liver (4.8%). Urine and stool porphyrin levels were determined in all patients, and 17 have at least one of the porphyrins elevated. A total of 56 specimens of human milk obtained from mothers with porphyria were analyzed for HCB. The average value was 0.51 ppm in HCB-exposed patients compared to 0.07 ppm in unexposed controls. Offspring of mothers with three decades of HCB-induced porphyria appear normal.
Gocmen
A
A
Department of Pediatrics, School of Medicine, University of Hacettepe, Ankara, Turkey.
Peters
H A
HA
Cripps
D J
DJ
Bryan
G T
GT
Morris
C R
CR
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002722:Chlorobenzenes / Q000506:poisoning*; D004196:Disease Outbreaks; D005243:Feces / Q000032:analysis; D005260:Female; D005500:Follow-Up Studies; D006581:Hexachlorobenzene / Q000032:analysis / Q000506:poisoning*; D006801:Humans; D007223:Infant; D008297:Male; D008895:Milk, Human / Q000032:analysis; D011164:Porphyrias / Q000139:chemically induced* / Q000453:epidemiology / Q000378:metabolism; D011166:Porphyrins / Q000032:analysis / Q000652:urine; D012871:Skin Diseases / Q000139:chemically induced / Q000453:epidemiology / Q000378:metabolism; D014421:Turkey / Q000453:epidemiology
D002722:Chlorobenzenes; D011166:Porphyrins; D006581:Hexachlorobenzene