Sclerema neonatorum......................... is a disorder of the subcutaneous fat in debilitated neonates and is considered best as a sign of a potentially fatal underlying disease process............Causes Associated underlying conditions include pneumonia, septicemia, septic shock, hypothermia, metabolic acidosis, transient hyperammonemia of the newborn, respiratory distress syndrome, congenital heart defects, gastroenteritis, intestinal obstruction, and severe malnutrition......................................... Physical findings of sclerema neonatorum appear suddenly, first on the thighs and buttocks and then, spreading rapidly, often affecting all parts of the body except the palms, soles, and genitalia. The involved skin is pale, waxy, and firm to palpation. The skin cannot be pitted or pinched up because it is bound to underlying subcutaneous tissue, muscle, and bone. The affected infant often displays flexion contractures at the elbows, knees, and hips; temperature instability; restricted respiration; difficulty in feeding; and decreased spontaneous movement. Newborns may also present with masklike facies or “pseudotrismus,” an inability to completely open the mouth, secondary to the thickening of the skin over the face, arms, and hands..........................................mortality rates range from 67-88%, with death occurring hours to days after onset...............Medical Care Recognition and the prompt institution of therapy specific for the underlying disease are mandatory. Careful monitoring, correction of electrolyte abnormalities, respiratory support, correction of hypovolemia, and control of hypothermia are important in sclerema neonatorum patients. Antibiotics: the prompt institution of prophylactic broad-spectrum antibiotic therapy for possible associated sepsis. Systemic steroids: Exchange transfusions:t. Exchange transfusion may enhance humoral and cellular immunity in these immunologically immature neonates, and may improve peripheral and pulmonary circulation, thereby also improving oxygen exchange
Posted on: Sun, 23 Nov 2014 08:57:50 +0000
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