Solution: First described in 1964, Shwachman-Diamond Syndrome is a rare disease which mainly involves the pancreas, bone marrow and skeleton, but other organs may also be affected. Next to Cystic Fibrosis, it is the most common cause of pancreatic insufficiency in children. Shwachman-Diamond syndrome is genetic, most likely an autosomal recessive condition. Infants often become ill with symptoms by the age of four or six months old. Early problems include failure to thrive, feeding problems and recurrent infections. Growth soon slows and remains below normal; only a few children show growth beyond the third percentile. Infections are present early in at least 85% of children. These occasionally lead to death. Diagnosis is generally made in the first few years of life, although occasionally diagnosis is delayed. Mortalities have been reported. Full growth may not be obtained despite aggressive therapy, particularly if hip disease is severe. The other features of the disease may persist into adulthood. If the pancreas is not working properly, enzyme replacement should be begun immediately. Guidelines for enzyme use are similar to those for Cystic Fibrosis. Enzyme replacement should decrease diarrhea and foul, greasy stools. This may not, however, improve growth. Multivitamins and supplemental fat-soluble vitamins (vitamins A,D,E,and K) should be given daily. The diet should provide protein and energy adequate for nutritional needs and may require supplementation with high calorie foods or prepared supplements. Infections should be treated vigorously with appropriate antibiotics. Bleeding problems and anemia may require blood and platelet transfusions or other intervention (such as DDAVP). Hip disease should be monitored closely and may require surgical intervention. Developmental delays may be helped by physical and speech therapy. This information is from the Shwachman Diamond Syndrome Foundation web-page.