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G Pridjian

Publications and source records attributed to G Pridjian.

17 recordsLinked to original sources
PubMed1999

Fatal carnitine palmitoyltransferase II deficiency in a newborn: new phenotypic features.

We describe the term male infant of asymptomatic, healthy nonconsanguineous parents presenting on the first day of life with nonketotic hypoglycemia, seizures, hepatomegaly, cardiomegaly with biventricular hypertrophy, and ventricular arrhythmias. Cranial ultrasound revealed cystic dysplasia with several foci of hyperechogenicity within the right basal ganglia. Free carnitine was markedly decreased in the urine and plasma with a pronounced elevation of plasma long-chain acylcarnitines. Fibroblast carnitine palmitoyltransferase II activity was reduced to 26% and 38% in the father and mother, respectively. The infant expired on day 5 of life from malignant ventricular tachy-arrhythmias. Diffuse lipid accumulation was evident at autopsy, including in the liver, heart, kidney, adrenal cortex, skeletal muscle, and lungs. This new case of infantile CPT-II deficiency illustrates the severity of the early onset form of CPT-II deficiency.

Abnormalities, Multiple↗
PubMed1996

A complex five breakpoint intrachromosomal rearrangement ascertained through two recombinant offspring.

Intrachromosomal rearrangements usually result from three of fewer breaks. We report a complex intrachromosomal rearrangement resulting from five breaks in one chromosome 10 of a phenotypically normal father of two developmentally delayed children. GTG-banding analysis of the father's rearranged chromosome 10 suggested in initial pericentric inversion followed by an insertion from the short arm into the terminal band of the long arm [der(10) (pter-->p13::q21.2-->p12.2::q22.1::-->q26.3::q22.1-->q 21.2::p12.2-->p13::q26.3-->qter)]. To our knowledge, this rearrangement is the most complex ever reported in a single chromosome. Both children inherited a recombinant chromosome 10 with loss of the insertion and the segment distal to it [rec(10)der(pter-->p13: :q21.2-->p12.2::q22.1-->q26.3:)]. Mechanisms for both rearrangements are proposed.

Child↗
PubMed1995

Goldenhar sequence and mosaic trisomy 22.

We describe a term infant with facioauriculo-vertebral "dysplasia" (Goldenhar sequence), hypertelorism, and mosaic trisomy 22: peripheral blood, 46, XY/47, XY, + 22 (72%/28%); skin fibroblasts, 47, XY, + 22 (100%). This is the second report of Goldenhar anomaly with epibulbar dermoids in a liveborn infant with aneuploidy. Hypertelorism is rare in Goldenhar sequence, but typical of trisomy 22. We recommend chromosome analysis in all patients with Goldenhar sequence. Those with hypertelorism may be more likely to have aneuploidy as well.

Chromosomes, Human, Pair 22↗
PubMed1992

Labor after prior cesarean section.

1. All women with PCS should be considered candidates for vaginal delivery. Certain high-risk factors then should be used to recommend elective repeat cesarean section. Currently, a scar in the active segment of the uterus is considered an absolute contraindication to labor. 2. Women should be informed of the chances of success (which in most instances are excellent), course of action in labor, and the rare risk of uterine rupture. 3. Women in a very low risk category (one low-transverse PCS) should be managed like any laboring patient but including fetal monitoring. 4. The remaining laboring patients may benefit from more intensive intrapartum surveillance, including continuous electronic fetal monitoring, early rupture of the fetal membranes, and placement of an intrauterine pressure catheter. 5. The labor course in women with PCS will depend on the number of vaginal deliveries achieved previously and the stage of labor reached before the cesarean section was done. 6. Labor disorders in patients with PCS, as in all patients, should be diagnosed and managed promptly. 7. Neither oxytocin nor epidural use is contraindicated in these patients. As in any patient, care should be taken to avoid iatrogenic uterine hyperstimulation. 8. Uterine rupture may have many different presentations. However, the most common is abnormal fetal heart rate patterns that are especially variable or prolonged decelerations. 9. Most uterine ruptures can be repaired and do not require hysterectomy. Hysterectomy may be the appropriate choice in some situations. 10. A history of a prior uterine rupture is not a contraindication to future childbearing, but it may place the woman at greater risk for a repeat event.

Female↗
PubMed1992

Spontaneous resolution of nonimmune hydrops fetalis secondary to human parvovirus B19 infection.

Many instances of nonimmune hydrops fetalis ascribed to human parvovirus B19 have been reported. The leading proposed pathophysiologic mechanism of hydrops in affected fetuses is viral invasion of red blood cell progenitors, causing a profound reticulocytopenic fetal anemia. Although the natural history of fetal parvovirus infection remains to be elucidated fully, there have been recent reports of funipuncuture and intrauterine blood transfusions to diagnose and manage this problem. We report two pregnancies in which parvovirus-related hydrops fetalis was observed to resolve without intervention, followed by uncomplicated vaginal deliveries of healthy infants. These observations emphasize the need for further investigation before recommending routine fetal blood transfusion in affected cases.

Adolescent↗
PubMed1992

Parovarian cyst presenting as a groin hernia. A case report.

An inguinal hernia containing a parovarian cyst is extremely rare. This phenomenon occurred in a 77-year-old woman who presented with a right groin hernia. The hernia contained a cystic mass that arose between the leaves of the broad ligament and passed with the round ligament through the deep inguinal ring. Through a midline incision the hernial content was mobilized, reduced through the inguinal ring and removed from the abdomen with both ovaries, tubes and uterus. The mass was found to be a parovarian cyst of the mesothelial type.

Aged↗
PubMed1991

Left ventricular mechanics in preeclampsia.

Increased systemic vascular resistance and contracted blood volume are characteristic findings in preeclampsia. These alterations in cardiovascular hemodynamics can adversely affect ejection phase indices of left ventricular performance making it difficult to separate abnormalities resulting from changes in load from those caused by depressed myocardial contractility. To address this issue the contractility-sensitive, load-independent relationship between left ventricular end-systolic wall stress and rate-corrected velocity of fiber shortening was assessed in 10 nulliparous patients with preeclampsia. Comparisons were made with data obtained from 10 age-matched normotensive women with uncomplicated pregnancies (control subjects). Studies were performed by means of two-dimensionally targeted M-mode echocardiography and calibrated carotid pulse tracings during early labor, 1 day after delivery, and 4 weeks after delivery. During early labor and 1 day after delivery, patients with preeclampsia had elevated blood pressure and increased total systemic resistance. These parameters returned to normal by 4 weeks after delivery. Before delivery and 24 hours after delivery, the patients with preeclampsia had lower overall left ventricular performance (as measured by cardiac output and rate-corrected velocity of fiber shortening) and higher left ventricular afterload (as measured by left ventricular end-systolic wall stress) when compared with control subjects. These differences were no longer present 4 weeks after delivery. Despite the time-related intergroup differences in hemodynamics, left ventricular contractility was similar between normotensive and preeclamptic subjects at all stages of the study. Thus when load is eliminated as a confounding variable, the decrements in overall left ventricular performance measured in patients with preeclampsia reflect a mechanically appropriate response to increased afterload rather than an abnormality in the ventricular contractile state.

Adult↗
PubMed1991

Twin gestation: influence of placentation on fetal growth.

To study fetal growth in twin gestation, morphometric autopsy data of 52 midgestation twin pairs who were stillborn or who died less than or equal to 24 hours after birth were analyzed. Twins were divided into three groups: (1) monozygotic: diamniotic, monochorionic placenta (n = 18); (2) dizygotic: diamniotic, dichorionic placenta, unlike sex (n = 12); (3) like-sex: placenta diamniotic, dichorionic in 63.6%, unknown in 36.4% (n = 22). The monozygotic group had a significantly higher rate of growth discordance, which was defined as a greater than 20% difference in body weight (monozygotic 72.2%, dizygotic 16.7%, like-sex 0%), and polyhydramnios (monozygotic 50%, dizygotic 0%, like-sex 9.1%). Organ weight z scores for body weight and brain weight standards were calculated for the smaller and larger of each twin pair. In the monozygotic group highly significant z scores were obtained for brain weight in the smaller twin (z = 2.71, p = 0.003, body weight standards) and heart weight in the larger twin (body weight standards, z = 3.87, p less than 0.001; brain weight standards, z = 3.64, p less than 0.001). We conclude that monozygotic twins with diamniotic, monochorionic placentation have a high degree of brain-sparing growth restriction in the smaller twin and cardiac hyperplasia in the larger twin, most likely caused by hemodynamic inequalities.

Adolescent↗
PubMed1991

Cesarean: changing the trends.

A rising cesarean birth rate, both nationally and at our institution, led us to attempt to reverse the trend. Vaginal birth after cesarean (VBAC) was introduced as an alternative to repeat cesarean in 1982. The impact of VBAC on the cesarean rate for our institution was assessed from 1982-1988. Over the period studied, the rate of repeat cesarean rose from 5.0% in 1982 to 6.9% in 1985, then steadily fell to 5.3% in 1988. During this same period, the primary cesarean rate rose from 7.5% in 1982 to 11.9% in 1986, then declined to 10.6% in 1988. Changes in the rate for all cesareans paralleled those for primary procedures: 12.5% in 1982, 17.6% in 1986, and 15.9% in 1988. In patients with a history of previous cesarean, the relative risk of repeat cesarean dropped from 10.2 (95% confidence interval 8.8-11.8) in 1982 to 5.2 (95% confidence interval 4.5-6.0) in 1988. In the face of a rising rate of primary cesarean births, the introduction of VBAC has helped to stabilize the overall cesarean rate at our institution.

Adult↗
PubMed1990

Taurine transport in the in vitro perfused human placenta.

Taurine, required by the fetus for nutrition and neurological development, is inadequately synthesized by the fetus and substantial quantities of the amino acid are supplied by the mother. Maternal to fetal unidirectional taurine transport was studied in in vitro perfused human placentae from normal term deliveries. To determine whether the placenta can achieve and maintain a chemical gradient for taurine, recirculating equimolar maternal and fetal perfusions were performed. The ratio of fetal/maternal taurine concentration increased over 75 min and was maintained at 1.38 +/- 0.14 (SEM) through 2.25 h (n = 6). To determine the rate of taurine transport against a concentration gradient, flux was determined with maternal taurine constant at 50 microM whereas the fetal taurine concentration varied from 0 to 500 microM in a nonrecirculating system (n = 5). Despite increasing the chemical gradient tenfold, taurine was transported at a constant rate of 1.75 +/- 0.75 (SEM) nmol/min/g. Nonrecirculating perfusions were performed with B-alanine (n = 13) and hypotaurine (n = 10), both B-carrier competitors, and no inhibition of taurine transport could be detected, taurine flux being 104.1 +/- 6.0% (SEM) of baseline in the presence of B-alanine and 106.0 +/- 7.0% (SEM) with hypotaurine. Finally, ouabain inhibited transport (n = 3) by 58.1 +/- 5.4% (SEM). We conclude that taurine is transported in the human placenta by an active carrier mechanism.

Female↗
PubMed1989

Missed abortion: still appropriate terminology?

The term missed abortion has been in the obstetric literature more than 100 years. With ultrasonographic evaluation of early pregnancy, its textbook definition (death of a fetus without its expulsion for greater than or equal to 8 weeks) is rarely fulfilled. Yet the term missed abortion is still extensively used today with regard to early pregnancy losses. We recommend its abandonment and in its place the use of the following terms: first trimester intrauterine fetal death, second trimester intrauterine fetal death, and anembryonic gestation.

Abortion, Missed↗
PubMed1987

A stromal Leydig cell tumor of the ovary occurring in a pregnant 15-year-old girl. Ultrastructural findings.

The first case of stromal Leydig cell tumor of the ovary occurring during pregnancy is reported. A 15-year-old girl presented in labor at 36.5 weeks of gestation. The tumor mass was obstructing labor and the patient had to be delivered by cesarean section. Unique features of this case included the young age of the patient, the large size of the tumor, and the associated pregnancy. The tumor was encapsulated and showed the typical histologic and ultrastructural features of ovarian stromal Leydig cell tumor. The literature is reviewed, and the differential diagnosis is discussed.

Adolescent↗
PubMed1987

Medroxyprogesterone acetate: receptor binding and correlated effects on steroidogenesis in rat granulosa cells.

Medroxyprogesterone acetate (MPA), a widely used synthetic steroid, was studied to determine both its effects on steroid receptors and steroidogenesis in the well-characterized rat ovarian granulosa cell model. Initial receptor binding studies showed MPA was as potent as progesterone and 10-fold less potent than R-5020 (an active synthetic progestin) in binding to progesterone cytosolic receptors in rat ovarian granulosa cells. MPA was 20-fold less potent than testosterone, and 10-fold less potent than dexamethasone in binding to the androgen and glucocorticoid cytosolic receptors, respectively. The binding of MPA to progestrone, androgen and glucocorticoid receptors predicted direct effects of MPA on FSH-stimulated estrogen (E), progesterone (P), and 20 alpha-dihydroprogesterone (DHP) production by cultured rat ovarian granulosa cells. MPA at 10(-7) to 10(-6) M significantly augmented FSH-stimulated P and DHP production (a previously documented progestin, androgen and glucocorticoid effect). This augmentation was blocked by the concurrent addition to cell culture of 10-fold excess RU-486 (a potent anti-progestin and anti-glucocorticoid). At concentrations greater than 10(-6) M, MPA inhibited the production of P and DHP (a progestin effect), and the production of E (a progestin and glucocorticoid effect). MPA, structurally a progestin, has complex steroid hormone effects predicted by its interaction with progesterone, androgen and glucocorticoid receptors.

20-alpha-Dihydroprogesterone↗
PubMedDate not supplied

Significance of positive cervical cultures for Chlamydia trachomatis in patients with preterm premature rupture of membranes.

We tested the hypothesis that in patients with preterm premature rupture of membranes the presence of Chlamydia trachomatis in the cervix shortens the latent period (time from rupture of membranes to delivery) and increases the incidence of chorioamnionitis and early endometritis. A total of 178 conservatively managed patients with PROM between 22 and 35 weeks' gestation had cervical cultures for chlamydia, group B Streptococcus (GBS) and Neisseria gonorrhoeae performed at the time of rupture. Patients with GBS and gonorrhea were treated at the time the culture results were available and excluded from analysis. The remaining patients were divided into group 1: 26 patients (14.6%) positive for only chlamydia (and not treated until discharge from the hospital); group 2: 120 patients (67.4%) negative for all three organisms. The two groups did not differ in cesarean rate, duration of conservative management, hospital stay, or birthweight. Furthermore, the rates of chorioamnionitis (30.8% group 1; 38.3% group 2) or early endometritis (11.5% group 1; 20.8% group 2) were similar. We conclude that in patients with preterm premature rupture of membranes, the presence of chlamydia in the cervix appears to neither decrease the latent period nor increase the incidence of chorioamnionitis and early endometritis.

Adult↗