Cases reported "Diseases in Twins"

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1/18. Severe hemolytic disease from rhesus anti-C antibodies in a surrogate pregnancy after oocyte donation. A case report.

    BACKGROUND: Maternal sensitization with rhesus anti-C antibodies is comparatively rare and usually benign. In pregnancies conceived using donor oocytes, the mother's blood group may differ from that of both the father and the oocyte donor, making blood group incompatibility more likely. CASE: twins, the result of a surrogate pregnancy using donor oocytes, were born with severe hemolytic disease due to rhesus anti-C antibodies. Both infants required exchange transfusion for profound anemia at birth. Isoimmunization in the surrogate mother was not detected antenatally. The twins were delivered by emergency cesarean section due to fetal compromise, detected fortuitously when the mother attended for routine fetal assessment at 35 weeks' gestation. CONCLUSION: Isoimmunization with anti-C antibodies is not always benign and may cause significant hemolytic disease. With the success of in vitro fertilization and oocyte donation, more infertile couples may use these methods to conceive, with or without surrogacy arrangements. In such cases, the provision of antenatal care may become a complex matter, involving several parties, and good communication between everyone involved is vital. In pregnancies conceived with donor oocytes, there may be a higher risk of blood group incompatibility, and special vigilance is warranted.
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2/18. Caring for the technology dependent child--a case study.

    Children who are dependent on medical technology present unique challenges for families and health professionals who attempt to care for them at home. The medical and nursing needs of these children challenge and stretch the financial, social and emotional capacities of parents in ways that service providers do not fully understand. case management is a collaborative parent/nurse model of community nursing practice that has been implemented to meet the needs of children and families with complex special needs where the care goes across primary and secondary care settings. The aim of case management is to facilitate communication and coordination of services between the multi-disciplinary team and the family to achieve desirable goals. This case study describes the special needs of a technology dependent child and the impact of chronic illness on both child and family. From a deeper understanding of the parent's perspective, implications for nursing practice are discussed.
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3/18. Absence of the ductus venosus with direct connection between the umbilical vein and right atrium.

    Abnormalities of the ductus venosus are rare. We present the case of a dichorionic, diamniotic twin pregnancy in which complete absence of the ductus venosus, with direct communication between the umbilical vein and right atrium, was detected in one infant on antenatal ultrasonography. autopsy confirmed the aberrant course of the umbilical vein, which also had an abnormal histological structure. Associated congenital anomalies included an ostium secundum type atrial septal defect, absent inferior vena cava, single left pulmonary vein, stenosed right pulmonary artery, proliferation of the hepatic arterioles with reduction of portal venules, and a duplex ureter. The literature is reviewed and pathogenesis relevant to this case is discussed.
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4/18. Unusual cardiac malformations in conjoined twins: thoracopagus twins with conjoined pentalogy of cantrell and an omphalopagus twin with atretic ventricles.

    Two unrelated cases of conjoined twins were found to have cardiac malformations that apparently have not been reported previously. In one case, thoracopagus twins had an extensive thoracoabdominal wall defect that resulted in ectopia cordis of a conjoined heart along with evisceration of the shared liver and intestine along with one spleen. These malformations, accompanied by defects in the sternum, diaphragm, and supraumbilical abdominal wall, constitute a conjoined pentalogy of cantrell. In the second case, the heart of one of omphalopagus twins consisted of a solid ventricular mass with only a minute aortic cavity but no atrioventricular communication-an ineffective heart that could develop only in a conjoined or chorioangiopagus twin. In both cases, a common atrium lay in the primitive (embryologic) position caudal to the ventricles.
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5/18. Congenital rubella infection: escape of one monozygotic twin with two amnions, one chorion, and single placenta.

    We report a 2 amnions, 1 chorion, single placenta, monozygotic identical twins, who were born by a mother with a subclinical rubella infection during the gestation; one twin had a congenital rubella infection, the other escaped from it. Both twins were premature and small for their gestational age at birth. They both had an intraventricular hemorrhage ascribed to prematurity. One had a strong positive rubella IgM antibody for more than 3 months and a persistent IgG antibody for more than 1 year. The other had a fading rubella IgG antibody within 3 months, and a negative IgM. The baby with the congenital infection of rubella did not have a defective appearance, but the auditory brain stem evoked potential test showed abnormal findings. In monozygotic identical twins, vessel communication usually exist and the escape of a twin from rubella is a rare condition; there have been only two such events reported in the English literature.
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6/18. A study on prognosis of surviving cotwin.

    It has been suggested that the surviving cotwin and the mother are seriously affected when intrautrine fetal death of one monozygotic twin occurs. Several authors have reported brain damage in the surviving twin coexisting in utero with dead monozygotic cotwin. Most of such instances are monochorionic and show vascular communication between paired twins in the placenta. In a total of 133 pairs of monochorionic twins, death of one twin during pregnancy occurred in 33 cases (24.8%). Of 33 surviving twins, 8 babies suffered from porencephaly, cerebral palsy and other abnormalities. More unfavorable prognosis was recognized when a cotwin died in the latter half of pregnancy, while when a monozygotic cotwin died in the early stage of pregnancy, the surviving twin uneventfully grew to term in most cases.
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7/18. When does death occur in an acardiac twin? Ultrasound diagnostic difficulties.

    Fetal acardia is a rare abnormality of multiple pregnancies, which is lethal for the affected fetus and can cause death in 50% of normal co-twins. Antenatal recognition with early ultrasound is essential to institute a prospective management to improve the outcome. Our communication outline the difficulties which may be encountered in ultrasound diagnosis. In particular the problem of distinguishing a fetal heart from large pulsating mediastinal vessels, which can be present in these fetuses, and the difficulty of diagnosing death in an acardiac fetus. Our report confirms that the co-twin remains at increased risk of sudden death, even without ultrasound evidence of cardiac failure or biochemical compromise. The finding in this fetus of intravascular fibrin deposits suggests the possibility of acute disseminated intravascular coagulation, not previously reported in association with an acardiac twin.
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8/18. dystonia musculorum deformans: three cases treated on a rehabilitation unit.

    dystonia musculorum deformans is a rare involuntary movement disorder of unknown etiology. We treated three brothers in one family on our rehabilitation unit, resulting in definite improvement in their ability to walk and control the dystonic movements, and in their speech and swallowing. All three benefited from orthoses to prevent equinovarus and maintain ankle stability. Intense concentration, such as needlepoint, seemed to reduce the amount of extra motion. Performing another activity during dystonic movements, such as pointing to a communication board, also seemed to give some relief. A special seating system using a collapsible wheelchair, a low center of gravity, and antitip wheels reduced one brother's frequency of falling. Swallowing finely cut foods was not a problem with any of our patients, although one had to be instructed in a special technique. An electronic communication board was very helpful. Our success in these cases should encourage others working in rehabilitation to consider such patients and to apply the principles of rehabilitation, particularly the total team approach, in their care.
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9/18. Enterolithiasis with imperforate anus. Report of two cases with sonographic demonstration and occurrence in a female.

    Enterolithiasis has been previously described as a plain film finding of rectovesical or rectourethral fistula in patients with anorectal malformation. Two cases of enterolithiasis with anorectal malformation are described in which the intraluminal nature of the calcifications was identified using ultrasound. The use of ultrasound for this purpose has not been previously reported to our knowledge. This simple, noninvasive technique permits antenatal differentiation between enterolithiasis and meconium peritonitis in some patients and can suggest the prenatal diagnosis of rectourinary communication when enterolithiasis is present. In addition, one of our cases occurred in a female. The entity of enterolithiasis with imperforate anus in a female has not been previously reported.
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10/18. twins discordant for vater association. Obstructed labor of the second twin due to ascites and persistent cloaca without communication to the exterior.

    The unusual delivery of a dead second twin with rare malformations is presented. The first twin, born live following a normal labor, had no malformations. The birth of the second twin was obstructed by massive ascites, and its abdomen had to be perforated before delivery. The sex could not be determined due to lack of the internal genitalia and the fetal appearance of the external genitals. The left kidney and ureter were hypoplastic. The right ureter and distal part of the colon were dilated and opened into a large primitive cystic cloaca without communication to the exterior. The ascites was probably caused by the urinary obstruction. These malformations probably represent one of the earliest arrested developments of the embryonic hindgut. The presence of a tracheo-esophageal fistula and a single umbilical artery, together with the anal atresia and the renal anomalies, could indicate that the anomalies formed part of the VATER association.
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