Ruth Andrea Seeler

Deaths in Children with Sickle Cell Anemia A Clinical Analysis of 19 Fatal instances in Chicago

During a 5-year-period, 19 children died of complications of sickle cell anemia, giving a 5 year mortality rate of 8.4% in this Chicago clinic. The children, 13 girls, 6 boys, ranged in ages from 7 months to 13 years. A disproportionate number of deaths occurred amongst the younger chil dren. Infections caused 6 deaths, acute splenic sequestration crisis 4, cerebrovascular occlusion 3, cardiovascular pulmonary 3, profound un explained anemia 1, anemia secondary to epistaxis 1, and unexplained 1.

Perioperative management of children with sickle hemoglobinopathy

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.

Alpha fetoprotein in embryonal hepatoblastoma.

Summary Fetoprotein, normally present only in earlyfetal life, is reported here in the serum of a patient with hepatoblastoma. The serum concentration in this patient was similar to levels seen in early fetal life and is higher than that reported in association with most cases of hepatocellular carcinoma, regardless of age. The high fetoprotein level occurred in a hepatoblastoma with extensive growth and metastases and was, perhaps, related to the level of its embryonal dedifferentiation.

Nonsurgical Management of Thrombosis of Bilateral Renal Veins and Inferior Vena Cava in a Newborn Infant

From Cook County Hospital, 700 South Wood Street, Chicago, Ill. 60612. * Pediatric Hematologist. Address correspondence to Dr. Seeler. ** Chief Resident, Pediatrics. *** Director, Pediatric Radiology. Present address: Stritch School of Medicine, Loyola University, Chicago, Ill. ROPER management of renal vein throm1 ROP R, na emen bosis (RVT) in neonates remains controversial. Although the value of conservative nonsurgical treatment in these cases has been emphasized by Stark,’ Friolet et al.,2 Johnston,3 lBtIcFarland,4 and Belman et al.,19 recommendation for immediate nephrectomy is made in recently published authoritative pediatric texts.5, 8

Hemolysis During BAL Chelation Therapy For High Blood Lead Levels in Two G6PD Deficient Children

A 3 I I/1‘?-veGrr-c>Icl asymptomatic black male was admitted for chelation therapy because ot a whole Mood lead level of 95 mcg9(. He had had two prior admissions for the same problem, with whole blood lead levels of 101 1 my~~ and 85 ni(-g(,7( respectively. During these admissions, he received 4 mg/kg of’ BAL and t2.5 mg/kg of EDTA intramuscularlv every four hours for ‘’.~tl doses, in order tt~ remove lead from the body by chetation. The h<jspital courses were thought uneventful. but in retrospect there was a low fever on the fourth day of the

Ataxia‐telangiectasia and and Hodgkin's disease

This paper reports the occurrence of a mixed cellularity type Hodgkins disease in a 3 11/12‐year‐old Caucasian boy with ataxia telangiectasia. The patient is unusually young for the development of Hodgkins disease. Unfortunately the patient died shortly after the institution of therapy.

von Willebrand's disease: A comparative studyof diagnostic tests**

The comparative diagnostic value of the Duke, Ivy, and modified Ivy bleeding times, factor VIII assay, and in vivo and in vitro platelet adhesiveness was determined in 28 patients with von Willebrands disease. The combination of Duke and modified Ivy bleeding times plus factor VIII assay was adequate to detect an abnormality in all patients studied. Variability of the bleeding times and factor VIII level in some patients may make repeated testing necessary. Presently available tests of platelet adhesiveness may be abnormal in patients with von Willebrands disease, but are not necessary for the diagnosis and may give false positive results.

Acute splenic sequestration crisis in an adolescent with S-C hemoglobinopathy.

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.

Induction of Intravascular Coagulation and Renal Cortical Necrosis in Rabbits by Simultaneous Injection of Thorotrast and Endotoxin

Preparation of rabbits with 6 ml./kg. of Thorotrast, administered intravenously, permits the induction of intravascular clotting and the production of renal cortical necrosis with simultaneous injections of E. coli endotoxin. With this dose of Thorotrast, a dose‐response relationship was observed between the fall in fibrinogen after 4 hours and doses of endotoxin ranging from 5 to 50 μg./kg. body weight. With such preparation, renal cortical necrosis occurred in 46 per cent of animals receiving 100μg./kg. of endotoxin and increased to 80 per cent in animals receiving 100 μg./kg. Thorotrast preparation with 3 ml./kg. permits intravascular clotting to occur with a single injection of endotoxin administered simultaneously. However, no relationship between endotoxin dose and fibrinogen depletion was observed and renal cortical necrosis developed only with relatively high doses of endotoxin. An efficient and predictable experimental model for studying both intravascular clotting and renal cortical necrosis is described by these observations.

Comparison of anti-A and anti-B titers in factor VIII and IX concentrates

5. Blank H, Davis C, and Collins C: Electron microscopy for the diagnosis of cutaneous viral infections, Br J Dermatol 83:69, 1970. 6. Gardner SD, Field AM, Coleman DV, and Hulme B: New human papovavirus (B.K.) isolated from urine after renal transplantation, Lancet 1:1253, 1971. 7. Lecatsas G, Prozesky OW, Van Wyk J, and Els HJ: Papova virus in urine after renal transplantation, science 241:343, 1973. 8. Dougherty RM, and DiStefano HS: Isolation and characterization of a papovavirus from human urine, Proc Soc Exp Biol Med 146:481, 1974. 9. Takemoto KK, Rabson AS, Mullarkey MF, Blaese RM, Garon CF, and Nelson D: Isolation Of papovavirus from brain tumor and urine of a patient with Wiskott-Aldrich syndrome, J Natl Cancer Inst 53:1205, i974.