Jan Cejka

β2-Microglobulin: occurrence in fetal life and malignancy

Abstract The levels of β2-microglobulin in human fetal sera were found increased as compared with those in maternal and normal adult sera. Serum concentrations of β2-microglobulin in the course of intrauterine development parallel concentration changes of feto-specific proteins. Elevated serum levels of β2-microglobulin were also found in a number of pathological conditions, especially those of a neoplastic nature.

Serum β2-microglobulin levels in normal children and sex-linked agammaglobulinemia patients

Abstract β 2 -Microglobulin levels have been determined in cord blood sera and sera of healthy children of different ages, as well as in sera of patients with Brutons type of agammaglobulinemia. The results obtained showed the highest β 2 -microglobulin levels in cord blood sera and a gradual decrease in β 2 -microglobulin concentration with age; normal adult levels of β 2 -microglobulin are reached at the age of approx. 12 years. No correlation has been found between serum β 2 -microglobulin levels and immunoglobulin concentration. The results suggest that the production of β 2 -microglobulin does not depend solely on B-cell function. A simple and sensitive radioactive method for the quantitation of β 2 -microglobulin is described.

Immunoglobulins in Idiopathic Thrombocytopenic Purpura in Childhood

Levels of immunoglobulins G, A and M were determined in 55 children with idiopathic thrombocytopenic purpura (ITP) (32 acute, 18 chronic and 5 recurrent). Mean IgG levels tended to be low in acute ITP and returned to normal with recovery. In contrast, chronic and recurrent ITP had persistently low IgG and IgA levels. C3 complement levels in 10 cases of acute ITP were normal. Seven patients with chronic ITP had their spleens removed, and IgG and IgA levels tended to rise, while IgM levels decreased in 4 patients after the operation. Low IgA levels observed at the onset of purpura in a child might favor the diagnosis of chronic or recurrent ITP.

A simple method for the classification and typing of monoclonal immunoglobulins.

Abstract A convenient method for identifying heavy and light chains of monoclonal immunoglobulins is described. The technique, a modification of immunofixation electrophoresis, is most useful for the typing of monoclonal immunoglobulins present at very low concentrations relative to the population of normal heterogeneous immunoglobulins. It obviates the use of immunoelectrophoresis and shortens the analysis time of typical paraproteins to 2 hr. The identification of Bence Jones proteins and other urinary proteins using this method is also presented.

In vitro synthesis of β2-microglobulin by human fetal tissues☆

Abstract Human fetal tissues were cultured in the presence of 14C-labeled amino acids and the culture fluids analyzed by radioimmunoelectrophoresis for the presence of radioactive β2-microglobulin. Synthesis of the protein was demonstrated in all the tissues studied. Using the Ouchterlony method, β2-microglobulin was also detected in culture media from serially transferred cell cultures of fetal tissues and established fetal cell strains.

The Fetus as a Patient

A 19-WEEK PARTURIENT presented with a fetus with a lung mass. Magnetic resonance imaging (panel A) demonstrated a congenital cystic adenomatous malformation (CCAM) occupying the right chest causing mediastinal shift, cardiac compression (H heart), and displacement of the hemidiaphragm (arrow). Both lungs were compressed. Hydrops fetalis was present (A fetal ascites; B bowel; L liver). Echocardiography revealed a compressed but structurally normal heart. The hydrops improved after aspiration, but the macrocyst recurred and the solid component continued to enlarge. A thoracoamniotic shunt was placed for continuous drainage. Imaging at 36 weeks (panel B) demonstrates the right hemidiaphragm in the correct position and resolution of the fetal ascites. Lung hypoplasia and mediastinal shift necessitated mass resection during ex utero intrapartum therapy. A maternal laparotomy was performed, followed by hysterotomy allowing delivery of the fetal head, chest, and arm. High-dose volatile anesthetic (2 minimum alveolar concentration of desflurane) provided uterine relaxation and fetal anesthesia. Maternal blood pressure was maintained with phenylephrine. Intramuscular fetal injections included fentanyl (20 g/kg), vecuronium (200 g/kg), and atropine (20 g/kg). The fetus was intubated (not ventilated), and pulse oximeter and peripheral venous access were established. After pulmonary lobectomy, the fetus was ventilated and delivery and newborn resuscitation were completed. Congenital cystic adenomatous malformation results from overgrowth of terminal bronchial epithelium. Mass effect results in pulmonary hypoplasia. Cardiac compression with impaired venous return leads to lethal cardiac failure (hydrops). Maternal health is threatened, asa state similar topreeclampsia (maternalmirror syndrome)mayensue. Exutero intrapartumtherapyprocedure isa feasibleandpotentially a life-saving treatment for congenital cystic adenomatous malformation. It provides time on uteroplacental gas exchange for controlled resection of the large fetal lung mass. The anesthetic goals for ex utero intrapartum therapy procedure include achieving uterine hypotonia, usingdeepgeneralanesthesiaornitroglycerin, tomaintainuteroplacentalcirculation;avoidingpostpartumhemorrhage;maintainingnormal maternal blood pressure often requiring -adrenergic agonist support; and achieving surgical anesthesia for the fetus to avoid first breathing while avoiding fetal cardiac depression.

Developmental Changes of Adenocarcinoma-Associated Antigen

The occurrence and serum concentrations of adenocarcinoma-associated antigen (ACAA) were studied during the fetal period of life, in newborns and in their mothers. The mean concentrations were significantly higher in fetal, newborn and maternal sera when compared with the mean concentration of ACAA in healthy, nonpregnant adults. Thus, ACAA appears to show fetospecific features as is known for other oncofetal proteins. ACAA should be recognized not only as a potential tumor marker, but also as a normal protein constituent of human serum.

Ontogeny of Pancreatic Oncofetal Antigen

Serum concentration of pancreatic oncofetal antigen (POA) was determined in human fetuses, newborns and pregnant women. The mean fetal concentration of POA (mean = 5.27 micrograms/ml) changed very little with gestational age. Also, only a weak correlation was found between POA concentration of newborns (mean = 5.15 micrograms/ml) and their birth weight. It appears that between the 19th and 40th weeks of gestation POA exhibits no fetospecific features, i.e. POA concentration did not exceed significantly the concentration of nonpregnant adults (mean = 6.10 micrograms/ml). A number of pathophysiological variables was correlated with POA concentrations of newborns. The most striking statistical differences were found between American black and white newborns and adults; the mean concentration of POA in sera of black full-term newborns was 5.38 micrograms/ml as compared to white newborns, where the mean concentration was 3.58 micrograms/ml. Similarly, black mothers had a mean concentration (mean = 12.21 micrograms/ml) significantly greater than white mothers (mean = 5.62 micrograms/ml).