Biomedical subjects

M Gudavalli

Publications and source records attributed to M Gudavalli.

7 recordsLinked to original sources

PubMed2000

Ellis van Creveld syndrome (chondroectodermal dysplasia, MIM 22550) in three siblings from a non-consanguineous mating.

The major diagnostic features of Ellis van Creveld syndrome (EvC) includes disproportionate short stature, polydactyly, ectodermal anomalies, and structural heart defects. We describe three siblings with EvC of a non-consanguineous mating. The history of these siblings well illustrate the clinical manifestations and complications that children with EvC encounter. All three girls had short stature, narrow rib cage, polydactyly and nail hypoplasia. The first daughter died in early infancy in respiratory failure. The second daughter underwent open heart surgery for atrial septal defect repair. The third daughter, diagnosed in utero with fetal ultrasonography, currently is scheduled for surgical excision of extra-digits and extraction of neonatal teeth. Infant mortality rates among patients with EvC is strikingly high due primarily to cardiorespiratory failure. If they survive infancy morbidity is significant. The gene effected in individuals with EvC has recently been identified on the short arm of chromosome 4. Future testing for gene mutations may provide valuable information for premarital counseling and prenatal diagnosis. Three offspring with disproportionate short stature, polydactyly, and ectodermal dysplasia of a non-consanguineous mating, strongly indicate parental heterozygosity for Ellis van Creveld syndrome.

Adult↗

PubMed2000

Neonatal echocardiograms of macrosomic neonates.

OBJECTIVE: To compare echocardiograms of macrosomic and healthy full term neonates whose weight was appropriate for gestational age (AGA). METHODS: Echocardiography was performed on 9 healthy full term AGA neonates and 15 macrosomic neonates. A data base was generated from valid echocardiographic measurements on each infant. RESULTS: Macrosomic infants were heavier than control infants and had a greater body surface area, but their mean cardiac dimensions were similar. The mean LVES volume was smaller than that of the control group when expressed as a fraction of individual LVED values (0.61 +/- 0.04 vs 0.64 +/- 0.02; p = 0.02). When comparing IVS/PW, it was observed that the ratio was up to and including 1.33 in the control group, while the upper limit of the ratio of the nondiabetic macrosomic infants was 1.6. The shortening fraction (SF%) was increased in comparison to infants of normal weight (40.67% +/- 3.34 vs 36.00% +/- 1.89; p = 0.0009). The thickened IVS did not elevate SF% by decreasing LVES; the macrosomic infants had a smaller LVES mean volume. CONCLUSION: IVS/PW ratio of macrosomic infants fell outside of the normal range. Macrosomic neonates were found to have an increased SF% secondary to a proportionally smaller LVES volume, regardless of IVS thickness.

Aorta↗

PubMed2000

Adolescent pregnancy: positive perinatal outcome at a community hospital.

OBJECTIVE: The objective of the present study was to determine the association between early maternal age and pregnancy outcome at a community hospital. METHODS: A retrospective cohort of 188 nulliparous patients was divided into three study groups of adolescent patients and a control group based on maternal age at the time of delivery. RESULTS: Younger adolescents had an excellent prenatal visit record. Overall complication rates were low and similar among groups. The cesarean section rates were not higher for younger adolescents in comparison to the control group. Duration of second stage of labor was shorter in adolescents than in the older patients (34.3 +/- 39.5 vs 59.5 +/- 39.9 min; t = 3.24; p = 0.002). There were no differences in mean gestational ages at delivery. The mean birth weights were lower in all adolescent groups compared to the control (ANOVA: F = 3.729; p = 0.0124). There were few NICU admissions in adolescent groups. CONCLUSION: Early maternal age (13 to 19 years) was associated with shorter duration of second stage labor and lower birth weight in a cohort of patients delivering at a community hospital.

Adolescent↗

PubMed1999

Congenital diaphragmatic hernia: atypical presentation.

We report an unusual case of the left sided diaphragmatic hernia in which the left lobe of the liver blocked the herniation of the intestinal contents into the chest and the clinical symptoms were not apparent at birth. The patient was a full term infant born to a mother whose antenatal care included apparently normal sonographic evaluation. She developed increasing tachypnea within two hours period after birth. Auscultation of the chest revealed bilateral normal aeration. No cardiac murmurs were heard. An X-ray of the chest revealed a left sided haziness without any mediastinal shift. Progressive decrease in the breath sounds was noted over the left base. A CT examination demonstrated a left sided diaphragmatic hernia, with herniation of the left lobe of the liver causing compression of the lung. No loops of the bowel were observed in the thoracic cavity. She underwent laparotomy and repair of diaphragmatic hernia. This case history illustrates that large left sided diaphragmatic hernias may be missed on antenatal sonogram and may not present with clinical signs until several hours after birth. Both physical examination and routine chest X-ray may prove inconclusive and a CT scan is necessary to make a definitive diagnosis.

Adult↗

PubMed1999

Ventricular dominance patterns in preterm infants.

OBJECTIVE: The purpose of the study was to determine EKG and 2-D echocardiographic criteria of ventricular dominance in preterm infants and select those by which ventricular dominance could be established by EKG alone. METHODS: A database was constructed from EKG and 2-D echocardiographic measurements on preterm infants ranging in gestational ages from 23 to 34 weeks and birth weights from 555-2490 g, and fullterm controls. Twelve-lead EKGs were obtained in the first 4 days of life in 12 preterm infants and in the first 24 hours of 4 controls. 2-D echocardiograms were performed with sweeps from the subcostal, parasternal, apical and suprasternal views and M-mode measurements in the short axis parasternal view on 11 of the preterm infants and 9 fullterm controls. RESULTS: A definite leftward QRS axis for the preterm infants (+90 degrees, preterm; +133.75 degrees, term; t = -5.63; p < 0.001) indicated a left ventricular (LV) dominance. But R/S in favor of LV dominance for preterm infants was apparent in V6 only. A pooled amplitude index for each ventricle based on R and S wave from V1, V2, V5, and V6 leads, showed LV dominance for the preterm infants with a trend toward RV dominance with increasing gestational age (F = 20.82; p < 0.001). RVD/LVED M-mode echo ratios confirmed the LV dominance in preterm infants. CONCLUSION: A healthy full term infant is born with RV dominance. LV dominance with a trend toward RV dominance with increasing gestational age was found in preterm infants by EKG and echo criteria.

Child↗

PubMed1999

One day old infant with acyanotic congenital heart disease: critical aortic stenosis.

Congenital aortic stenosis accounts for about 5% of cardiac malformations recognized in childhood. It belongs to the category of acyanotic congenital heart disease. These lesions produce a load on the heart because of left ventricular outflow tract obstruction. Severe aortic stenosis in the newborn period (critical aortic stenosis) presents with signs of left sided heart failure (pulmonary edema, poor perfusion), right sided heart failure (hepatomegaly, peripheral edema) and may progress rapidly to total circulatory collapse. We present a case of an infant with critical aortic stenosis presenting with cyanosis, who was entirely dependent on ductal patency for systemic output. When oxygen was given, the ductus started to close, with a worsening of the left sided output and subsequent acidosis. With the right to left shunt across the ductus, the baby was cyanotic and dependent on prostaglandin to keep the ductus open. There was minimal flow across the aortic valve because of the stenosis and extremely poor left ventricular function prior to surgery. After relief of the aortic valvular obstruction, there was finally good antegrade flow across the aortic valve, terminating cyanosis.

Adult↗

PubMedDate not supplied

Perinatal hepatitis B virus transmission in the United States. Prevention by passive-active immunization.

Among infants born to women in whom sera are positive for both the hepatitis B surface antigen and the e antigen, 85% to 90% are infected with hepatitis B virus and become chronic hepatitis B surface antigen carriers. In a study to assess the effectiveness of passive-active prophylaxis (hepatitis B immune globulin and hepatitis B vaccine) of such infants, we screened 18,842 pregnant Asian-American women: 8.7% were positive for hepatitis B surface antigen and 3.0% were also positive for hepatitis B e antigen. Thus far, 113 infants have received hepatitis B immune globulin (0.5 mL at birth) and hepatitis B vaccine (three 20-micrograms doses beginning at birth or at 1 month) and have been followed up for nine to 18 months. Among these infants, 16 have become chronic carriers, an incidence of only 14.2%. All of the uninfected infants have retained high levels of antibody to surface antigen, suggesting that they have had an active immune response to the vaccine and should have long-term protection against hepatitis B virus.

Asian↗