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Biomedical subjects

B A Paes

Publications and source records attributed to B A Paes.

16 recordsLinked to original sources

Discordant fibular aplasia in twins.

A male infant, one of monozygotic twins, was born with absence of fibulae, ectrodactyly of the right hand and both feet, with accompanying deficiencies. This second case report of a discordant fibular aplasia developmental field defect occurring in monozygotic twins, although likely sporadic, is interesting because of the similar pattern of anomalies.

Abnormalities, Multiple

Group B streptococcus. Is it time for a screening program?

Group B streptococcal infection often causes perinatal sepsis. Early diagnosis is based on a high index of suspicion and laboratory tests. Proposed interventions targeted at the antepartum, intrapartum, and postpartum periods have met with limited success. Screening has not been widely adopted.

Adult

Changing physicians' behavior using combined strategies and an evidence-based protocol.

OBJECTIVES: To review the number of superficial cultures performed in the evaluation of neonatal sepsis and to validate physicians' compliance with an established protocol. DESIGN: Before-and-after-comparison using medical audit. SETTING: Intensive care nursery in a secondary level perinatal facility. PARTICIPANTS: One hundred seventy-five consecutive newborns admitted during a 6-month period for the assessment and potential treatment of sepsis. Eligible patients met the entrance criteria of having a superficial culture performed in conjunction with at least one deep culture, which included cerebrospinal fluid, blood, or urine. This cohort was compared with 205 patients before the introduction of the protocol. INTERVENTIONS: A second audit of physician practice 9 months following the introduction of an evidence-based hospital protocol to discontinue the use of superficial cultures in the diagnostic assessment of neonatal infection. Evidence-based medicine, an opinion leader, continuing medical education rounds, immediate feedback through direct encounters with physicians, and barriers in accessing microbiological tests were used to alter physician behavior. MEASUREMENTS/RESULTS: A significant reduction from 50.5% to 6.9% was achieved in the proportion of superficial cultures performed and a substantial cost savings of $4454.84 was realized without incurring patient morbidity. CONCLUSIONS: A methodologically rigorous reaudit process with planned, interventional strategies may be used as part of a continuous quality improvement program to affect change in physicians' practices. Existing hospital practice standards should be reevaluated against emerging scientific evidence.

Clinical Protocols

Cloacal exstrophy: prenatal diagnosis before rupture of the cloacal membrane.

Embryologically, cloacal exstrophy is thought to result from persistence and subsequent rupture of the infraumbilical cloacal membrane during the fifth embryonic week. We report a case of cloacal exstrophy in which a prenatal diagnosis was made prior to rupture of the cloacal membrane. A routine ultrasound at 17 weeks' gestation demonstrated monoamniotic twins. One twin was normal, but the other was found to have a sacral myelomeningocele, "rocker-bottom" feet, splaying of the pubic rami, and a large cystic mass protruding from the infraumbilical anterior abdominal wall. A repeat ultrasound was performed at 22 weeks, with the same findings. At 26 weeks, further examination showed disappearance of the abdominal cyst, a small omphalocele, no demonstrable bladder, and the suggestion of prolapsed bowel inferior to the umbilical cord insertion. After delivery at 34 weeks, the abnormal twin was found to have the typical findings of cloacal exstrophy, myelomeningocele, bilateral lower limb anomalies, and extremely foreshortened small bowel. Rupture of the presumed cloacal membrane after 22 weeks in this case is inconsistent with our current understanding of the embryology of this anomaly, and should stimulate a reexamination of the current concepts. If the characteristic features are recognized, cloacal exstrophy can be diagnosed by prenatal ultrasound, permitting prenatal counseling and appropriate perinatal management.

Abnormalities, Multiple

Management of prenatally diagnosed tracheal obstruction: access to the airway in utero prior to delivery.

A fetus of 23-weeks gestation presented with polyhydramnios, and was found on sonography to have a large anterior cervical teratoma. At 26 weeks the mother went into premature labor and the membranes ruptured; at this time the uterus was of 32-weeks size secondary to polyhydramnios. A cesarean section was performed through a low transverse uterine incision, and the fetal head and shoulders were delivered with the cord intact. An endotracheal tube was placed, and the cord was then divided. The baby was taken to the neonatal intensive care unit for stabilization prior to planned resection, but suffered pharyngeal hemorrhage followed by endotracheal tube dislodgement several hours after birth, and expired. Despite an unfortunate outcome, this case illustrates the feasibility and potential value of in utero airway access in cases of prenatally diagnosed tracheal obstruction.

Adult

Baller-Gerold syndrome associated with congenital hydrocephalus.

We present a new case of the Baller-Gerold syndrome (BGS) in an infant with prenatally apparent severe hydrocephalus, growth retardation, and cardiac and limb abnormalities detected by ultrasound at 26 weeks of gestational age. Subsequent survival to term and neonatal examination confirmed an unsuspected diagnosis of BGS.

Abnormalities, Multiple

Subcutaneous fat necrosis with hypercalcemia.

We report the case of an infant with diffuse subcutaneous fat necrosis following birth asphyxia who had hypercalcemia and the rare complication of venous calcification. The clinical and radiologic findings resolved over 5 months. The disease is characterized by the presence of painless subcutaneous nodules, mainly over bony prominences, possibly associated with hypercalcemia and calcification. Early identification of hypercalcemia will avert its serious sequelae.

Calcinosis

Birth asphyxia: does the Apgar score have diagnostic value?

The current literature was reviewed to evaluate the Apgar score as a diagnostic test for the presence of asphyxia. Several studies were examined and the sensitivity, specificity, and predictive values of the Apgar scores calculated. Using an umbilical cord arterial pH below 7.2 as evidence of asphyxia, the one-minute Apgar score showed poor sensitivity as a marker of asphyxia. Therefore, we discourage reference to the term "asphyxia" when Apgar scores alone are used as supportive evidence.

Apgar Score

Fatal malformations of the larynx and upper trachea.

Five infants with a spectrum of rare malformations of the larynx and trachea are presented. All infants died because of problems with intubation and ventilation. The series reflects the wide variety and complexity of malformations that may co-exist, and successful management may require open tracheotomy.

Abnormalities, Multiple

Intravenous infusion by superficial vein in the neonate.

State-of-the-art equipment, such as extremly-low-volume controlled infusion pumps and Teflon catheters, has simplified the management of intravenous therapy in the newborn. This article details the proper I.V. insertion procedure for neonates. It also reviews indications for neonatal infusion, preferred insertion sites, types of catheters currently in use, and potential complications relevant to neonatal intravenous care.

Catheterization, Peripheral