Cardiac death and stored electrograms in patients with third-generation implantable cardioverter-defibrillators

OBJECTIVE: The study's aim was to analyze the type of cardiac involvement in fetuses or infants with twin-twin transfusion syndrome. STUDY DESIGN: Seventeen pairs of monochorionic diamniotic twin fetuses with twin-twin transfusion syndrome underwent serial Doppler echocardiographic studies. Repeated decompressive amniocenteses were performed in all pregnancies. RESULTS: No specific cardiac involvement was seen in donor twins in utero or after birth. All recipient twin fetuses showed variable degrees of biventricular hypertrophy and dilation with tricuspid regurgitation. These features were also evident in 45% of the recipient twin neonates. The fluid unbalance resolved with serial amniocenteses in 9 cases and in a further 2 after the death of the donor twin; in all 11 of these recipient twin fetuses there was some degree of improvement of the cardiac involvement, which became normal in all surviving recipient twin infants within 40 days to 6 months after birth. CONCLUSION: Features of cardiac hypertrophy with signs of a prehydropic or hydropic state develop during fetal life in recipient twins in twin-twin transfusion syndrome; this impairment is reversible with the resolution of the fluid imbalance or after delivery.     

Renal tubular dysgenesis

Renal tubular dysgenesis (RTD) is a very rare malformation, only 24 cases having been described. We present the case of twins born after 28 weeks gestation to a newly-arrived Ethiopian immigrant in whom oligohydramnios was diagnosed when she was admitted. 1 twin had developed polyhydramnios, died before birth and was born with hydrops fetalis. The other newborn suffered from pulmonary distress and anuria, and died 2 days after birth. At autopsy there were no apparent changes in the kidneys or urinary tract, but histological examination showed absence of proximal convoluted tubules. This was attributed to RTD and confirmed by histochemical examination.     

Marked twin-twin transfusion in the absence of nonimmune hydrops

The precise pathophysiology of development of nonimmune hydrops in either the recipient or donor twin associated with twin-twin transfusion syndrome is not entirely clear. At times the recipient twin may develop nonimmune hydrops, but at other times the donor twin, and infrequently both, may develop this ominous complication. We present an unusual cases of this syndrome in which discordant twins, despite neonatal hematocrit levels of 86 and 21% were both nonhydropic and discuss the possible underlying pathophysiology of this occurrence.     

Renal tubular dysgenesis with microcephaly

Renal tubular dysgenesis, a rare, lethal, autosomal recessive disorder, is characterized by short and poorly differentiated proximal convoluted tubules associated with oligohydramnios, Potter sequence, and neonatal death due to respiratory failure. Abnormalities of the skull may occur in some cases. We report an infant born of a consanguineous union who also had microcephaly, among other features. A history of oligohydramnios with or without skull abnormalities and a lethal outcome without obvious explanation should occasion renal histological study aimed at precise diagnosis and genetic counselling.     

Liveborn twin with intrauterine death of one twin: report of two cases

Twin pregnancies carry a greater mortality and morbidity rate than singleton pregnancies. In case of an intrauterine fetal death (IUFD), the risk of mortality and morbidity of the surviving twin is increased. The pathogenesis is usually due to twin to twin transfusion. The donor twin is hypovolemic, anemic and often shows a growth retardation or even severe enough to cause an IUFD. The recipient twin is hypervolemic, polycythemic and weighs more than its co-twin. In this paper we reported two cases of liveborn twin complicated by an IUFD of its co-twin. Both cases were monochorionic twins. The first case was born at 29 weeks and 6 days of gestation, a male infant weighed 1054 g. His co-twin was stillborn and weighed 722 g. At birth the surviving infant showed respiratory distress syndrome (RDS), anemia and bilateral periventricular echogenicity (PVE). The second case was a female infant with a gestational age of 26 weeks and 2 days and weighed 850 g. Her stillborn co-twin weighed 804 g. Both twins showed signs of hydrops, and the liveborn infant had RDS, marked anemia (Hb 6 g/dl) and hypoalbuminemia. We will discuss the possible pathogenesis in our cases and also review some literature.     

Vibrio spp., the dominant flora in shrimp hatchery against some fish pathogenic viruses

BACKGROUND: Single fetal demise in a twin pregnancy is a rare event, the common causes being twin-twin transfusion syndrome, chromosomal or congenital anomalies and abnormalities of the umbilical cord and placenta. Umbilical vein thrombosis is a very rare cause of single fetal demise in twins. CASE: Three days after a reassuring biophysical profile, a 40-year-old primigravida with twin pregnancy presented at 38 weeks' gestation in early labor when demise of 1 of the twins was recognized. She underwent a cesarean section for arrest of labor, delivering twin A, a stillborn female weighing 2, 360 g and twin B, a liveborn male weighing 2,200 g. Umbilical vein thrombus was noted in twin A. CONCLUSION: Umbilical vein thrombosis is a rare and sudden cause of fetal demise.     

Discordant Duane's retraction syndrome in monozygotic twins

PURPOSE: To evaluate whether differences in the intrauterine environment caused discordant Duane's retraction syndrome in monozygotic twins. METHODS: Pathology records were reviewed to determine monozygosity. RESULTS: Discordant Duane's retraction syndrome was present in twin boys with identical genetic foundations who had different intrauterine environments. The placenta indicated areas of anastomosis between the two umbilical cords suggestive of twin-to-twin transfusion that resulted in a visible growth discordance. CONCLUSIONS: Intrauterine environment influences ocular development, and twin-to-twin transfusion may cause Duane's retraction syndrome.     

Intrapartum amnioinfusion in twin gestation. A preliminary report of three cases

Amnioinfusion is an intrapartum technique that is not usually attempted in twin gestations. This report describes infusion of normal saline into the amniotic sacs of three twin gestations with oligohydramnios. All the twins were safely delivered vaginally. No untoward effects were noted. Ultrasound is advised following amnioinfusion to assess the increase in the amniotic fluid volume.     

Ultrasound in twin pregnancy

This review evaluates the role of ultrasound in twin gestation. Ultrasound is essential in the diagnosis and management of perinatal complications common in twins, including fetal anomalies, conjoined twins, acardiac twinning, fetal demise, prematurity, intrauterine growth retardation, and twin-to-twin transfusion. Indications for first-trimester scanning are reviewed, as well as its use in determining type of placentation, chorionicity, and amnionicity. The focus of this article will be the use of ultrasound and Doppler velocimetry in the evaluation of fetal growth and subsequent management of intrauterine growth retardation in twins, as well as the value of real-time ultrasound and Doppler evaluation in the diagnosis of discordant growth and twin-to-twin transfusion syndrome.     

HLA-DR4 and number of mutans streptococci in saliva among dental students and staff

OBJECTIVE: To evaluate the ratio of peak systolic to least diastolic flow velocity (S/D), pulsatile index (PI) and resistance index (RI) of the umbilical arterial doppler flow at 20-40 gestational weeks in twin pregnancy for the surveillance of twin transfusion syndrome. METHODS: The fetal hemodynamics in 36 cases of twin pregnancy were monitored by Combison A-ZIPE 4871 with spectral doppler ultrasound. RESULTS: The value of S/D ratio and PI in 4 patients with twin transfusion syndrome at 32-34 weeks were significantly higher than those of normal twin (P < 0.01). Two cases of twin reversed arterial pressure (TRAP), i.e. artery to artery transfusion in one of the twin with acardius, were found at 26 and 26 + 4 weeks respectively. CONCLUSION: The S/D ratio and PI of umbilical artery might be useful indices for the surveillance of twin transfusion syndrome.     

Selective feticide by embolization in twin-twin transfusion syndrome. A report of two cases

BACKGROUND: The early development of complications in twin-twin transfusion syndrome is a poor prognostic sign. For this reason, new techniques for intrauterine therapy are being developed: invasive options, such as selective feticide of one of the twins, have been reported. CASES: Two cases of twin-twin transfusion syndrome in the late second trimester were treated by selective feticide using vascular embolization to the more severely damaged fetus. In one case the embolized fetus was a hydropic recipient with a normal cotwin; in the other, the donor fetus was affected by bilateral hydrocephalus. These fetuses underwent ultrasound-guided embolization using a bolus of histoacryl injected into the umbilical vein and fetal heart. Both patients went on to deliver healthy singletons in the third trimester. CONCLUSION: In twin-twin transfusion syndrome of early onset, embolization may help one of the twins survive. This technique is neither time-consuming nor expensive and does not require a general anesthetic.     

Modulation of human epidermal cell response to 2,3,7,8-tetrachlorodibenzo-p-dioxin by epidermal growth factor

OBJECTIVE: To investigate cranial ultrasonographic findings in survivors of monochorionic pregnancies complicated by fetofetal transfusion syndrome. STUDY DESIGN: Case details of all monochorionic twin pregnancies complicated by fetofetal transfusion syndrome were obtained from the Centre for Fetal Care database for a 3-year period. Fetofetal transfusion syndrome was diagnosed according to ultrasonographic criteria. Eligible for entry were twin pregnancies resulting in live-born preterm infants and complicated by fetofetal transfusion syndrome severe enough to require amnioreduction. Cranial ultrasonographic scans performed within 48 hours of birth were reviewed for evidence of abnormality. RESULTS: Seventeen pregnancies were eligible for inclusion in the study. Median gestational age was 25 weeks (between 17 and 29 weeks) at diagnosis and 30 weeks (between 25 and 35 weeks) at delivery. Three infants died before ultrasonography could be performed. The remaining 31 twin infants received an early cranial ultrasonographic scan. One of the 31 had a major cerebral infarct; 10 others had evidence of other, more minor, antenatally acquired lesions. CONCLUSIONS: Both donor and recipient survivors from pregnancies complicated by fetofetal transfusion syndrome are at significant risk for antenatally acquired cerebral lesions. Long-term neurologic follow-up studies are indicated to determine the clinical significance of these lesions.     

Spine fusion with demineralized bone

This series describes the occurrence of unbalanced circulatory shunting in three consecutively treated pairs of conjoined twins. The impact of renal functional changes and compensatory cardiac physiology on the timing of surgery and perioperative management are illustrated. The craniopagus set of twins presented in renal failure (1A) and cardiac failure (1B) to our hospital at age 2.5 years. Renal transplantation for anuria was performed in 1A before separation of the twins. This did not improve urine output in the hypoperfused twin, and normal renal function was not restored until the twins were separated. Two sets of omphalopagus twins were delivered and transferred to our hospital and separated during the first week of life. In each case a preoperative oliguric state in the underperfused twin reversed after separation, and a postoliguric diuresis ensued. Serum creatinine levels were similar in each pair preoperatively despite the inequality in urine volumes. In conjoined twins, assessment of renal function is necessary and should include serial measurements of urine volumes and urine creatinine clearance. Serum creatinine is not a useful marker of individual twin renal function in this situation. When disparity in renal function exists, early separation is indicated to prevent renal failure and compensatory cardiac failure.     

Zygosity and intrauterine growth of twins

Six hundred seventy-eight sonar measurements of the fetal biparietal diameter (BPD) were used to assess the intrauterine growth of 182 twins between 18 and 40 weeks' gestation. These values, as well as the weight and body length at birth, were related to the zygosity, which was determined by sex of the infants, histologic examination of placenta, or blood grouping. The mean BPD of twin 1 was larger than that of twin 2 in both monozygotic and dizygotic pairs throughout the study period (P less than .001). The size of BPD of the dizygotic infants exceeded that of the monozygotic among both twin 1 (P less than .001) and twin 2 (P less than .001). The absolute intrapair differences of birth length and weight were larger among dizygotic than among monozygotic twins. These findings suggest that in late pregnancy a substantial one-way intertwin transfusion among monochorial twins does not occur frequently enough to mask the inherent genetically determined similarity between the twins. The appearance already in the second trimester of the difference in the longitudinally monitored BPD between monozygotic and dizygotic twins suggests that the discrepancy is determined early in gestation.     

Asplenia syndrome in conjoined twins: a case report

Incomplete fission of the primordial cell mass is generally believed to be the cause of conjoined twinning. It may also explain the tendency towards increased symmetry in these twins. We report a case of the rostral duplication type of conjoined twins with an absence of symmetry; one twin had the asplenia syndrome, whereas the other twin was normal. Local environmental factors acting upon the organ anlage may explain the asymmetric development in these conjoined twins.     

Severe renal dysgenesis produced by a dominant gene

A woman with the autosomal dominant syndrome of preauricular pits, cervical fistulae, and partial deafness gave birth to two children with preauricular pits and severe renal dysgenesis. The facies had some features of the Potter facies of renal agenesis. One child died soon after birth because of pneumothorax and immature development of the lungs. We suggest that all infants with renal agenesis or dysgenesis be examined for preauricular pits because of the high recurrence risk of renal anomalies in families with this syndrome.     

Physiatric research fellows'' perceptions of the quality of their training

After being poisoned by eating the mushroom species Cortinarius speciosissimus, a twin developed interstitial nephritis with acute renal failure. He received a renal transplant from his living twin brother, who was presumed dizygotic on phenotypic grounds. Fifteen years later, the twins were zygosity tested by DNA "fingerprint analysis" and found to be monozygotic, despite important phenotypic discordances. The recipient has discontinued immunosuppression therapy and remains well after 9 months. We suggest that, for medical and other reasons, zygosity should be determined at birth on all like-sexed twins.     

Prognosis and long-term follow-up of a twin after antenatal death of the co-twin

It is generally believed that among twin pregnancies with one fetal loss prior to delivery, the surviving twin has an increased rate of perinatal mortality and childhood morbidity (cerebral palsy and mental retardation). By using data from the National Medical Birth Registry of Sweden between 1973 and 1983, we identified 206 gestations with the death of at least one twin (in 36 pregnancies both twins died) prior to delivery. The original medical records were retrieved for study. The presence of childhood morbidity for 65 of 129 surviving (8 years of age or older) twins born between 1973 and 1980 was evaluated by a questionnaire sent to rehabilitation centers for disabled children, as well as to offices for the Provision of Care for the Mentally Retarded. Perinatal mortality for a twin after the antenatal death of the co-twin was considerable. Fifty percent of survivors died before 34 weeks' gestation, and 18.7% thereafter. At follow-up, 8 years or more after birth, three twins (4.6%) were handicapped. Our results indicate the need for careful monitoring of the surviving twin fetus after one twin has succumbed prenatally.     

Fetal alcohol syndrome in twins of alcoholic mothers: concordance of diagnosis and IQ

The effects of teratogens can be modified by genetic differences in fetal susceptibility and resistance. Twins of alcoholic mothers provide a unique opportunity to study this phenomenon with respect to alcohol teratogenesis. Sixteen pairs of twins, 5 MZ and 11 DZ, all heavily exposed to alcohol prenatally, were evaluated. They represented all available twins of alcohol-abusing mothers who were on the patient rolls of the authors. The rate of concordance for diagnosis was 5/5 for MZ and 7/11 for DZ twins. In two DZ pairs, one twin had fetal alcohol syndrome (FAS), while the other had fetal alcohol effects (FAE). In 2 other DZ pairs, one twin had no diagnosis while one had FAE. IQ scores were most similar within pairs of MZ twins and least similar within pairs of DZ twins discordant for diagnosis. Despite equivalent alcohol exposure within twin pairs, alcohol teratogenesis appears to be more uniformly expressed in MZ than in DZ twins. These data are interpreted as reflecting the modulating influence of genes in the expression of the teratogenic effects of alcohol.     

The hemodynamic and laboratory biochemical characteristics of the nephropathy in convalescent patients after hemorrhagic fever with renal syndrome

Laboratory and functional tests were made in 50 convalescents who had suffered hemorrhagic fever with renal syndrome (HFRS). It is shown that nephropathy in such patients runs with a decline in renal functional reserve indicative of intraglomerular hypertension, impaired ability of the kidneys for osmotic urine concentration, defective tubular reabsorption of beta 2-microglobulin, microcirculatory disorders, collagen disbolism, high levels of uric acid in the blood. The occurrence of hyperuricemia and intraglomerular hypertension in HFRS convalescents calls for special consideration as leading nonimmune factors of nephropathy progression.