Sickle Cell Crisis Terminated by Use of Urea in Invert Sugar in Two Cases.
Abstract
The use of urea in invert sugar (UIS) for the treatment of SS crisis is related to the existence of pathologic hydrophobic bonds in sickled hemoglobin. Murayama's recently modified hypothesis for the molecular mechanism of sickling in SS hemoglobin clearly implicates the formation of hydrophobic bonds between interacting tetramers of S hemoglobin as the necessary condition for the sickling event. Hydrophobic bonds are now recognized by some biochemists to be responsible for the integrity of the steric configurations of protein molecules in aqueous systems. Urea, long known to attack hydrogen bonds and more recently recognized to attack hydrophobic bonds, has been used successfully by us as a chemical agent both to reverse and to block sickling in susceptible cells as shown elsewhere in optical and electron microscopy studies. Urea in invert sugar (UIS) has been established by a large body of literature and by Javid as a safe pharmaceutical agent; he has personally used this preparation in the treatment of over 2,000 cases of intracerebral edema. For these reasons, there was little hesitancy in using infusions of UIS in the treatment of our first two cases of SS crisis, a therapeutic strategy which has proved to be effective in these preliminary studies. Clinical histories and data are given. Precautions and principles concerning the use of UIS are discussed. A protocol including the use of controls for the evaluation of this therapeutic modality in the treatment of painful sickle cell crisis is recorded. (Author)
Document Details
- Document Type
- Technical Report
- Publication Date
- Sep 17, 1970
- Accession Number
- AD0721005
Entities
People
- Frank R. Camp Jr.
- Garth Schultz
- Paul L. Wolf
- Raymond L. Henry
- Robert M. Nalbandian
Organizations
- United States Army Medical Research Laboratory