Herman E. Branson

INHERITED PROTEIN C DEFICIENCY AND COUMARIN-RESPONSIVE CHRONIC RELAPSING PURPURA FULMINANS IN A NEWBORN INFANT

A coumarin-responsive chronic relapsing purpura fulminans syndrome is described in a protein-C-deficient newborn infant. Episodes of acute disseminated intravascular coagulation (DIC) and cutaneous gangrene, which first appeared at age 11 h, were effectively controlled for 28 months with transfusions of fresh-frozen plasma. Cryoprecipitate and cryoprecipitate-poor plasma induced remissions as long as those induced by fresh-frozen plasma (less than or equal to 72 h). Coumarins sustained a cryoprecipitate-induced remission for 19 days: they were then electively discontinued and 17 h later the patient had an acute exacerbation of DIC with haemorrhaging. Family studies showed protein C levels of 31-40% in the subjects symptom-free mother and full and half brothers. DIC, the coumarin effect, and the inherited protein C abnormality appear to have contributed to the extremely low plasma levels (less than or equal to 6%) of protein C in the patient. This experience suggests that protein C deficiency may greatly compromise the ability of newborn infants to control consumptive disorders.

Multiple myeloma complicated by myelomatous obstructive uropathy

Two patients with multiple myeloma with large retroperitoneal myelomatous masses are discussed. This extraosseous extension of disease caused obstructive uropathy in both patients. This complication has not been previously recognized in multiple myeloma and must be added to the potential causes of renal failure in this disease.

Normal Hematologic Values and Prevalence of Anemia in Children Living on Selected Pacific Atolls

The hematologic status of infants and children living on the small islands of the Pacific basin has been poorly documented. This report determines the normal ranges for hemoglobin (Hb) and mean corpuscular volume (MCV) for children residing on four of the small atolls of the Republic of the Marshall Islands in the archipelago of Micronesia. The difficulty in establishing normal hematologic values in pediatric populations is discussed and a methodology suggested that does not exclude any Hb value above the mean in determining the normal range for Hb. The study population was comprised of 563 Marshallese children representing approximately 3.4% of all children less than 16 years of age living in the Marshall Islands. The local prevalence of anemia and iron deficiency was also established.