ABSTRACT
A ganglioneuroblastoma developed in a 35-month-old boy with both the fetal hydantoin and fetal alcohol syndromes. Our case, plus two recent reports in the literature, would very likely establish the relationship between fetal hydantoin syndrome and the development of neural crest tumors. Infants exposed in utero to hydantoins should be closely observed for the development of these tumors.
Subject(s)
Fetal Alcohol Spectrum Disorders/complications , Kidney Neoplasms/complications , Phenytoin/adverse effects , Abnormalities, Drug-Induced/etiology , Child, Preschool , Female , Humans , Male , PregnancyABSTRACT
During the 11 years between July, 1967 and June, 1978, 35 children with a major hemoglobinopathy (SS, SC, S-thalassemia) underwent 46 operations without any mortality or unusual morbidity. All of these children were prepared for surgery by preoperative transfusion of 15-20 cc/kg packed erythrocytes and a hematocrit of at least 36%. In urgent and emergent situations, the operation was performed as soon as the transfusion was completed. For elective patients, the operation was the following morning. The ease of application and the absence of morbidity and mortality suggest that this method of preoperative transfusion preparation of children with a major hemoglobinopathy is more desirable than a two-volume exchange or transfusions beginning 10-15 days prior to surgery.
Subject(s)
Anemia, Sickle Cell/surgery , Blood Transfusion/methods , Preoperative Care , Adolescent , Anemia, Sickle Cell/blood , Blood Transfusion/economics , Child , Child, Preschool , Female , Hematocrit , Hemoglobin, Sickle , Humans , Infant , MaleABSTRACT
Readers are invited to submit questions relating to problem cases. Inquiries will be answered by qualified consultants and replies forwarded by mail promptly. Selected problems and solutions are published every month in this section.
ABSTRACT
Acute splenic sequestration crisis occurs rarely as a complication of sickle hemoglobinopathy. It is a medical emergency requiring immediate restoration of intravascular volume with transfusions of fresh packed RBCs. If the blood of the patient under discussion had not contained strong irregular antibodies, the delay in blood transfusion and the subsequent fatal outcome might have been avoided. Whether young children with sickle hemoglobinopathies should be checked periodically for the presence of irregular antibodies is an unanswered question. Monitoring might be important in view of the small but definite risk during childhood of the development of acute splenic sequestration crisis.