Sickle cell disease clinical features

TABLE 65-1. Clinical Features of Sickle Cell Trait and Sickle Cell Disease... 

Mukisi-Mukaza M, Samuel-Leborge Y, Keclard L, et al. Prevalence, clinical features and risk factors of osteonecrosis of the femoral head among adults with sickle cell disease. Orthopedics 2000 23 357-363. 

Smith, E.W., Conley, C.L. Clinical features of the genetic variants of sickle cell disease. Johns Hopkins Hosp. Bull 94, 289-318 (1954)... 

Water Deficiency. This condition occurs when water output exceeds intake. Water is continually losl by way of the lungs, skin, and kidneys and dius a deficiency of body water will occur if a critical minimal supply is not maintained. Decreased intake when water is available is uncommon. Very rarely, a brain malfunction may interfere with one s sense of diirst. Increased output of water can result from many causes. For example, a person with diabetes insipidus who lacks ADH (antidiuretic hormone) or a person whose kidneys do not respond normally to ADH, as in instances of nephrogenic diabetes insipidus, will increase water output Other diseases which may cause excess excretion of water include osmotic diuresis, hypercalcemia, hypokalemia, chronic pyelonephritis, and sickle cell anemia, among others. Excessive water losses are also experienced in some cases with advanced age and in some burn cases. Two clinical features are good measures of dehydration—weight loss of the patient and an elevation of the serum sodium concentration. In situations of dehydration, the body initiates mechanisms which manipulate the transfer of water from one compartment to the next, retaining water in those cells and organs where it is most needed. 

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