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J M Lusher

Publications and source records attributed to J M Lusher.

131 records · Page 8Linked to original sources
PubMedDate not supplied

The incidence of development of irregular red cell antibodies in patients with sickle cell anemia.

Two hundred and forty-five patients with SS hemoglobinopathy were screened for alloimmunization; they had all been transfused previously. Their median age was 10 years. Nineteen patients (7.75%) were found to be sensitized to various red cell antigens. The median number of transfusions received by sensitized patients was 23 compared to three in the unsensitized group, indicating an increased risk with more transfusion exposures. Thirteen sensitized patients were re-exposed to transfusion and four (30%) developed new alloantibodies. Once immunized, the risk of developing more alloantibodies on re-exposure did not increase with increasing number of exposures. The most frequently observed alloantibody was anti-K, accounting for 38 percent of all instances. Anti-Lea and -Leb were next in frequency (24%); Rh antibodies were seen in 14 percent of cases. Children with sickle cell anemia who were multiply transfused had a low frequency of alloimmunization. Responders had an increased rate of further sensitization which did not increase with number of subsequent exposures. Phenotypically matched blood is probably not warranted in most patients receiving transfusions for sickle cell anemia.

Adolescent↗
PubMedDate not supplied

Factor IX inhibitors and anaphylaxis in hemophilia B.

PURPOSE: We present clinical and laboratory data on 18 children from 12 hemophilia treatment centers in the United States, Canada, and Europe with the purpose of disseminating information regarding a recently recognized, potentially life-threatening complication of treatment in very young children with hemophilia B. PATIENTS AND METHODS: Twelve hemophilia centers from the United States, Canada, and Europe provided clinical information and laboratory data concerning 18 children who had severe allergic reactions to infused factor (F) IX in close association with the development of an inhibitor to FIX. Laboratory testing for establishment of the diagnosis of hemophilia B and inhibitor to FIX was done locally at the centers treating these patients. FIX gene analysis was performed at one of six molecular genetics institutes. RESULTS: All 18 children had severe hemophilia B, and in each an inhibitor antibody to FIX developed. The median age at the time of anaphylaxis (or anaphylactoid reaction) was 16 months, and the median number of exposure days to FIX was 11. The FIX inhibitor was detected almost simultaneously with the first occurrence of anaphylaxis in 12 of 18 patients. Maximum inhibitor titers were 4.5-600 Bethesda units (BU), with a median titer of 48 BU. FIX gene analysis, performed in 17 of 18 patients, demonstrated complete deletion of the FIX gene in 10 and major derangements in seven. Immune tolerance induction (ITI) regimens have been attempted in 12 patients, with generally poor responses. Two of the 12 experienced nephrotic syndrome while on ITI. Recombinant FVIIa has been successfully used to treat bleeding episodes in 11 of these children. CONCLUSION: Physicians treating young children with hemophilia B should be aware of the potentially life-threatening complication of anaphylaxis. Children with complete gene deletions or major derangements of the FIX gene appear to be at greater risk. Those identified by genotype as being at greater risk may need to receive their first 10-20 treatments in a medical facility equipped for handling such emergencies. Recombinant FVIIa, although not licensed for use in the United States, appears to be the most suitable treatment option for bleeding episodes in such patients.

Anaphylaxis↗
PubMedDate not supplied

Intracranial and extracranial hemorrhages in newborns with hemophilia: a review of the literature.

PURPOSE: Intracranial hemorrhage (ICH) and extracranial hemorrhage (ECH) in newborns with hemophilia were reviewed with respect to incidence, anatomic location, clinical presentation, and relationship to the mode of delivery and type of hemophilia. MATERIALS AND METHODS: A MEDLINE search from 1964 to 1996 of all reports of neonatal hemophilia and head bleeds in children from birth to 1 month old was performed. ICH was defined as any bleed occurring within the cranial cavity, and ECH was defined as hemorrhage occurring outside of the cranial cavity, including subgaleal and cephalhematoma. The mode of delivery, type and severity of hemophilia, and clinical presentation were also noted. RESULTS: One hundred two newborns with hemophilia and cranial bleeds were described in 33 publications. The cumulative incidence of ICH and ECH was 3.58% in 5 studies that reported the total newborn population or that examined birth records. The type of hemophilia was reported for 40 of 102 newborns and was hemophilia A in 87%. The mode of delivery was recorded in 46% (47 of 102) of the patients. Of these, 13% had cesarean delivery, and 87% were delivered vaginally (40% had spontaneous vaginal delivery, and 47% had vaginal delivery with vacuum extraction or forceps). There were 109 episodes of ICH and ECH (65% were ICH, 35% were ECH, and 5.8% were a combination of both). Common clinical features of ICH and ECH included anemia, hypotension, shock, and lethargy. However, only patients with ICH were reported to have neurologic deficits (15%) and late neurologic sequelae (38%). CONCLUSION: In neonates with hemophilia and cranial bleeds, ICH occurs more often and is often associated with late neurologic deficits.

Cerebral Hemorrhage↗
PubMedDate not supplied

Response to 1-deamino-8-D-arginine vasopressin in von Willebrand disease.

The synthetic agent, 1-deamino-8-D-arginine vasopressin (DDAVP), is generally regarded to be the treatment of choice for persons with type I von Willebrand disease (vWD), and may be useful in type IIA vWD as well. Several formulations of the drug are available, including a highly concentrated intranasal spray formulation which is ideal for home use. The degree of F VIII and vWF response to DDAVP varies among individuals, but is generally consistent in individual patients over time. Some have tachyphylaxis while others do not. While side effects are uncommon, DDAVP is a potent antidiuretic agent. Thus one should be aware of the possibility of hyponatremia and water intoxication if certain precautions are not observed.

Administration, Intranasal↗