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Biomedical subjects

J A Kuller

Publications and source records attributed to J A Kuller.

At least 73 records · Page 4Linked to original sources
PubMed1994

A fetus with sirenomelia, omphalocele, and meningomyelocele, but normal kidneys.

Sirenomelia, which has been reported for more than 450 years, is the most severe form of caudal dysplasia. This disorder is classically associated with agenesis or severe dysplasia of the urinary tract. A 27-year-old gravida 3 para 0020 presented at 19 weeks' gestation with monoamniotic twins, one normal and one with sirenomelia. The pregnancy miscarried following invasive diagnostic testing. Autopsy confirmed the findings of sirenomelia, meningomyelocele, omphalocele, persistent cloaca, and two normal kidneys. The abnormalities accompanying sirenomelia appear as a spectrum. The pathogenic theories of sirenomelia are reviewed in light of this case with normal kidneys.

Abnormalities, Multiple↗
PubMed1994

Recurrent miscarriage.

The perinatologists are emerging as the physicians who evaluate and treat women with recurrent pregnancy loss. Recurrent miscarriage, previously referred to as habitual abortion, affects almost 1% of couples. The etiologies of recurrent miscarriage are diverse and may be divided into genetic defects, such as chromosomal anomalies; maternal reproductive anatomic disease, both developmental and acquired, such as septate uterus or cervical incompetence; and systemic maternal disease such as antiphospholipid antibody syndrome or maternal diabetes. A cause for recurrent miscarriage can be identified approximately 60% of the time. We emphasize the tremendous psychological impact of recurrent miscarriage. We contrast any proposed treatments with the empiric fact that with no treatment after recurrent losses, couples still have a 60 to 70% chance of delivering a viable infant.

Abortion, Habitual↗
PubMed1994

Nonimmune hydrops fetalis secondary to premature closure of the foramen ovale.

Premature closure of the foramen ovale is a rare cardiac anomaly. Severe right-sided heart failure accounts for the hepatomegaly, ascites, pleural and pericardial effusions, and generalized edema of these hydropic fetuses. Premature closure of the foramen ovale should be a part of the differential diagnosis when unexplained fetal hydrops is encountered.

Female↗
PubMed1994

Miscarriage: a historical perspective.

Ancient cultures have applied a variety of fascinating therapies to prevent the occurrence and reoccurrence of miscarriage. Ceremonies of ritual purification, special prayers, and a variety of medicinal therapies were employed throughout the ages to prevent this feared event. Rituals to memorialize and help mourn the lost pregnancy were developed in many cultures. These rituals reinforce how deeply men and women are affected by early pregnancy loss.

Abortion, Spontaneous↗
PubMed1994

The frequency of transplacental hemorrhage in patients with threatened abortions.

The frequency and volume of transplacental hemorrhage that occurs with threatened abortions is unknown. The use of Rh immune globulin for such patients is therefore undefined. We studied the incidence and quantity of transplacental hemorrhage in patients with threatened abortions and in a control population of patients presenting in the first 20 weeks of pregnancy without a history of bleeding. Using the Kleihauer-Betke test, the incidence of transplacental hemorrhage in our control and study population was not statistically different.

Abortion, Threatened↗
PubMed1994

Prenatal counseling in heterozygotes for ornithine transcarbamylase deficiency.

X-linked ornithine transcarbamylase deficiency (OTCD) often leads to fatal neonatal hyperammonemia in affected males (hemizygotes). In prenatal management of subsequent pregnancies, families carrying female fetuses are often reassured of the low risk of clinically overt disease. We suggest that such reassurance may be misleading. While OTCD heterozygotes may show no symptoms or only mild protein intolerance, the clinical course in a fraction of children can include manifestations similar to those in affected males. We present three cases of symptomatic and previously undiagnosed OTCD heterozygotes to illustrate the potential severity of this condition. Significant improvement in function and growth followed diagnosis and treatment; however, two of the three children remain significantly developmentally delayed. While a quantitative risk estimate cannot be derived from these data, the cases are indicative of an adverse outcome in manifesting heterozygotes. Accordingly, OTCD carrier families should be counseled regarding the possibility of significant hyperammonemia, neurologic deficit, and the need for pharmacologic and dietary intervention in their heterozygote daughters.

Adolescent↗
PubMed1994

Early postpartum endometritis. Randomized comparison of ampicillin/sulbactam vs. ampicillin, gentamicin and clindamycin.

Seventy-six parturients with a clinical diagnosis of early postpartum endometritis were randomized to be treated with either standard therapy--ampicillin, gentamicin and clindamycin--or a new regimen, ampicillin/sulbactam. We deliberately chose to administer 1.5 g of ampicillin/sulbactam rather than a 3-g dose every six hours in order to accentuate any differences that might occur between the regimens. Failure rates, days of therapy and cost of treatment were compared. There was no statistically significant difference (P > .9) in the failure or recovery rates: 4 of 42 (9.5%) patients failed standard therapy vs. 6 of 34 (17.6%) patients in the ampicillin/sulbactam group. The times to recovery were 3.6 +/- 1.8 SD and 3.3 +/- 1.3 days, respectively. There was no difference in side effects or drug toxicity between the two groups. The cost of standard therapy was $355.32 for 3.6 days, whereas ampicillin/sulbactam cost $139.49 for 3.3 days. Therapy with ampicillin/sulbactam may be an equally effective and efficient way to treat patients with early postpartum endometritis.

Adult↗
PubMed1994

Prenatal ultrasound findings of linear nevus sebaceous and its association with cystic adenomatoid malformation of the lung.

BACKGROUND: Nevus sebaceous is a congenital hamartomatous skin lesion that is commonly associated with other abnormalities and is most frequently located on the face and head. To our knowledge, there are no previous reports of the ultrasonographic appearance of nevus sebaceous or of an association with cystic adenomatoid malformation of the lung. CASE: A patient was referred at 25 weeks' gestation with multiple fetal anomalies, including echogenic soft-tissue structures external to the cranium and face, a large right-side intrathoracic mass, and abdominal ascites. After a preterm delivery, nevus sebaceous and a congenital cystic adenomatoid malformation of the lung were diagnosed. Neonatal death occurred secondary to pulmonary hypoplasia. CONCLUSION: Nevus sebaceous should be considered in the differential diagnosis when echogenic soft-tissue structures are seen on prenatal ultrasound. A detailed ultrasound examination is warranted to rule out other associated abnormalities.

Adult↗
PubMed1993

Neonatal complications after the administration of indomethacin for preterm labor.

BACKGROUND: The use of indomethacin as a tocolytic agent in pregnant women appears to be accompanied by a low incidence of neonatal complications. However, the neonatal effects of indomethacin have been studied primarily in infants born after 32 weeks' gestation. This study was designed to examine the incidence of neonatal complications in very premature infants. METHODS: We identified 57 infants delivered at or before 30 weeks' gestation whose mothers had been treated with indomethacin for preterm labor and matched them with 57 infants whose mothers had not received indomethacin. The infants in the two groups were matched for sex, gestational age at delivery (mean [+/- SD], 27.6 +/- 2.0 weeks), exposure to betamethasone for 24 hours or more before delivery, and rupture of membranes 24 hours or more before delivery. RESULTS: There were no significant differences between the two groups in birth weight, Apgar scores, cord-blood gas values, frequency of multiple gestation, or incidence of respiratory distress syndrome. The proportion of infants who required exogenous surfactant was similar, as were ventilator settings at 24 hours, the incidence of chronic lung disease, and the incidence of sepsis. The infants exposed to indomethacin had a lower urine output and higher serum creatinine concentrations during the first three days after delivery. More indomethacin-exposed infants had necrotizing enterocolitis (29 percent vs. 8 percent, P = 0.005), intracranial hemorrhage grade II to IV (28 percent vs. 9 percent, P = 0.02), and patent ductus arteriosus (62 percent, vs. 44 percent, P = 0.05). More indomethacin-exposed infants with a patent ductus arteriosus required surgical ligation because of either a lack of initial response or a reopening of the duct after postnatal indomethacin therapy (50 percent vs. 20 percent of the unexposed infants, P = 0.05). CONCLUSIONS: Antenatal indomethacin therapy for preterm labor appears to increase the risk of serious neonatal complications in infants born at or before 30 weeks' gestation.

Cerebral Hemorrhage↗
PubMed1993

Facial features of infants exposed prenatally to cocaine.

Thirty two infants referred for in-patient genetics evaluation at the University of California at San Francisco, 1987-1992, were found to have a history of maternal cocaine use. Genetics reports and medical records were reviewed on all these infants to identify features distinctive for cocaine exposure. Among these 32 cases, 14 infants were exposed only to cocaine; 18 were exposed to alcohol and cocaine. The infants evaluated displayed a distinctive phenotype, consisting of neurologic irritability, large fontanels, prominent glabella, marked periorbital and eyelid edema, low nasal bridge with transverse crease, short nose, lateral soft tissue nasal buildup, and small toenails. Features consistent with the fetal alcohol syndrome appeared distinct and coexistent with the other described facial findings. Other severe abnormalities included cleft lip/palate, atypical facial cleft, abnormal BSER, intraventricular hemorrhages, arthrogryposes, and genitourinary abnormalities. Forty percent of the infants were born prematurely; 28% were small for gestational age; 43% showed head circumference values less than the 10th percentile. We conclude that these findings may be distinctive for a diagnosis of fetal cocaine syndrome; such findings should be further established by a future blinded prospective study of mothers and neonates.

Alcoholism↗
PubMed1993

Fetal metatarsal length: an accurate predictor of gestational age and weight in the ovine fetus.

Accurate assessment of gestational age and fetal weight in the pregnant ewe is important in both acute and longitudinal perinatal studies. We describe two morphometric methods based on the length of the fetal metatarsal bone. These measurements can be made intraoperatively or at postmortem. Metatarsal bone lengths can be used to better predict gestational age and fetal weight than previously described methods.

Animals↗
PubMed1992

Pallister-Hall syndrome associated with an unbalanced chromosome translocation.

We report 3 cases of Pallister-Hall syndrome involving hypothalamic hamartoblastoma, hypopituitarism, cranial, and limb abnormalities. The first 2 cases represent the first apparent sibs reported with this syndrome. Patient 1 represents the first known patient with this syndrome with an abnormal karyotype.

Abnormalities, Multiple↗
PubMed1992

Outcome of antenatally diagnosed cystic adenomatoid malformations.

OBJECTIVE: Twenty-two cases of antenatally diagnosed congenital cystic adenomatoid malformations are reported. STUDY DESIGN: Case management is reviewed. RESULTS: Eighteen women continued pregnancy after diagnosis. In nine cases nonimmune hydrops fetalis did not develop and all infants survived. Nonimmune hydrops fetalis developed in the other nine; fetal intervention was performed in eight cases. In the single case of nonimmune hydrops fetalis without intervention, the neonate died. In four cases aspiration of macrocystic lesions was performed. In two cases cystoamniotic shunts were placed. Neither aspiration or shunting provided long-term benefit. In six cases fetal lobectomy was ultimately performed and four survived. Two fetuses did not undergo in utero surgery; one was delivered prematurely after cyst aspiration and lived, and the other previable fetus was delivered soon after shunting. CONCLUSIONS: Fetal survival is best related to development of nonimmune hydrops fetalis. Aspiration of cystic lesions and cystoamniotic shunts generally provide short-term benefit. Early experience with fetal surgery for congenital cystic adenomatoid malformations has been encouraging.

Cystic Adenomatoid Malformation of Lung, Congenita↗
PubMed1992

Pregnancy complicated by Goodpasture syndrome.

Goodpasture syndrome is characterized by pulmonary hemorrhage, glomerulonephritis, and an autoantibody to glomerular basement membrane antigens. Current management of this potentially lethal disease consists of glucocorticoids to control pulmonary hemorrhage, cytotoxic drugs to inhibit further renal damage, and plasma exchange to clear circulating anti-basement membrane antibodies. These treatment modalities pose difficult management issues during pregnancy. We describe a successful pregnancy outcome in a parturient with Goodpasture syndrome. Antepartum care included serial pulmonary and renal evaluations. In patients without disease progression, a favorable outcome may be possible.

Adult↗