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Biomedical subjects

L Sirota

Publications and source records attributed to L Sirota.

99 records · Page 6Linked to original sources

Neonatal factor XIII deficiency.

We describe a patient diagnosed in the neonatal period as having factor XIII deficiency who presented with persistent umbilical bleeding. Factor XIII deficiency is the only coagulation factor deficiency that cannot be detected by classical hemostatic tests, and a rapid diagnosis is vital during the first decade of life. A newborn presenting with persistent umbilical stump bleeding should be screened for factor XIII deficiency when routine coagulation tests prove normal.

Blood Coagulation Tests↗

Neuroendocrine study of a male infant with septo-optic dysplasia.

Septo-optic dysplasia includes abnormalities of the optic nerves and tracts with absence of the septum pellucidum. Most of the recently reported patients were deficient in growth hormone. We describe a male infant with septo-optic dysplasia in whom extensive endocrine evaluation revealed central diabetes insipidus, hypothalamic hypothyroidism and combined (hypothalamic-pituitary) hypoadrenalism, along with normal pituitary growth hormone reserve. This is the first reported case of a patient with septo-optic dysplasia who underwent corticotropin-releasing factor and growth hormone-releasing hormone stimulation.

Adrenal Glands↗

Transient obstruction of the kidney and hypertension due to neonatal adrenal hemorrhage. Case report.

A case of neonatal adrenal hemorrhage associated with transient obstruction of the kidney and hypertension is reported. Sonography demonstrated a mass in the right suprarenal area, consistent with hemorrhage into the adrenal gland. DTPA renal scan showed prolonged retention of the injected material in the right kidney, consistent with obstructed outflow from the renal collecting system. Gradual decrease in the size of the suprarenal mass was associated with relief of the renal obstruction as evidenced by a normal repeat renal scan, and a return to normal of the blood pressure. It appears that the cause for the hypertension was the acute renal obstruction, which was due to either direct pressure on the proximal ureter by a large adrenal mass or secondary to displacement of the kidney by the mass resulting in kinking of the proximal ureter.

Adrenal Gland Diseases↗

New anomalies found in the 11q-syndrome.

The cytogenetic analysis of an infant with multiple congenital anomalies revealed a small deletion of the long arm of one No. 11 chromosome: 46XX del 11 (q23-q25). The main clinical manifestations included: trigonocephaly, flat broad nasal bridge, micrognathia, carp mouth, hypertelorism, low set ears, severe congenital heart disease, anomalies of limbs and external genitalia. In comparison to the previously reported cases of 11q-, the patient presented here had congenital anomalies not described before, including severe affected urogenital system, hypoplasia of the adrenal, accessory spleens and mild hydrocephaly.

Abnormalities, Multiple↗

49,XYYYY. A case report.

A 14-month-old boy with a 49,XYYYY karyotype is reported. The physical examination revealed unusual facial features, brachydactyly with clinodactyly, limitation of supination at the left elbow, and inguinal hernia. Radiological abnormalities of the skeleton and urinary tract were present, and the developmental examination showed a DQ of 70.

Abnormalities, Multiple↗

Placental transfer of maternal rubella antibodies to full-term and preterm infants.

Premature infants are vulnerable to infections, partly because of the low transplacental transfer of maternal antibodies. The present study investigated the placental transfer of maternal rubella-specific antibodies to full-term and preterm infants. The study group consisted of 133 healthy, native Israeli mothers and their 159 newborns. Of these, 69 were full-term infants (gestational age > 37 weeks) of 69 mothers, and 90 were preterm infants (gestational age < 35 weeks) of 64 mothers. Antibody titers against rubella were measured in maternal and umbilical cord blood samples by hemagglutination inhibition and microneutralization techniques. There was no significant difference in the level of protection and in geometrical mean titers by hemagglutination between the full-term and preterm groups. Conversely, significant differences in geometric mean titers of neutralizing antibodies were found between full-term and preterm infants, e.g., 65.9 and 39.8, respectively (P < 0.001). Very low birth weight preterm infants are at greater risk of rubella infection during the first year of life, due to the diminished transfer of neutralizing maternal antibodies. Therefore, earlier vaccination of this group may be beneficial.

Antibodies, Viral↗

Reported exposure to chickenpox: a predictor of positive anti-varicella-zoster antibodies in parturient women.

OBJECTIVE: To determine the positive and negative predictive values of recalled exposure to chickenpox for identifying anti-varicella-zoster virus (VZV) seropositive parturient women. METHODS: Blood samples were taken from laboring women during February 1998: All women completed questionnaires concerning a history of chickenpox in themselves and their children. Anti-VZV antibodies were determined by the immunofluorescent antibodies to membrane antigen (IFAMA) technique. RESULTS: Three hundred and twenty-seven women formed the study population; 239 women (73.1%) recalled chickenpox in themselves or their children, of which 229 (95.8%) were seropositive for anti-VZV antibodies. Of the 88 women who gave a negative/uncertain history of chickenpox 82 (93.2%) were seropositive and 6 (6.8%) were seronegative. All 87 mothers who were certain their children had had chickenpox were seropositive, including all 16 mothers who had a negative personal history. Thus, a woman with a history of chickenpox had a positive predictive value of 95.8%, and a woman with a lack of history had a negative predictive value of 6.8% (sensitivity 73.6%, specificity 37.5%), while a positive history of chickenpox in a child had a positive predictive value of 100%. CONCLUSIONS: Most women with no known history of VZV infection have evidence of prior exposure by serologic testing. Moreover, 100% of women with a negative history who were exposed to VZV in their children were protected from the disease. Therefore, mothers exposed to VZV during pregnancy can be reassured that most likely they are protected. However, the practice of testing all pregnant women exposed to the disease should be continued.

Adolescent↗