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1/87. pituitary gland gumma in congenital syphilis after failed maternal treatment: a case report.

    A preterm, very low birth weight infant was born to a mother with early latent syphilis who was treated 10 days and 3 days before delivery with 2.4 mU of benzathine penicillin. The infant had clinical, laboratory, and radiographic abnormalities consistent with congenital syphilis, ie, a Venereal disease research Laboratory test titer that was fourfold greater than was the maternal titer, hepatosplenomegaly, abnormal liver function tests, pneumonitis, osteochondritis of the long bones, and cerebrospinal fluid (CSF) examination showing a reactive Venereal disease research Laboratory test, pleocytosis, and elevated protein content. The infant died on the third day of life, and an autopsy revealed an evolving gumma of the anterior pituitary. immunoglobulin m immunoblotting of serum and CSF was positive, and polymerase chain reaction detected treponema pallidum dna in endotracheal aspirate and CSF. This case highlights the pathologic abnormalities observed in congenital syphilis and focuses on the rare finding of an evolving anterior pituitary gumma. Furthermore, it documents the failure of maternal syphilis treatment during the last 4 weeks of pregnancy to cure fetal infection and supports the recommendation that all infants born to mothers with syphilis treated during the last 4 weeks of pregnancy should receive penicillin therapy.
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2/87. Local low-dose urokinase treatment of acquired intracardiac thrombi in preterm infants.

    We report three cases of intracardiac thrombi in preterm infants of very low birth weight, in whom local low-dose urokinase treatment achieved complete thrombolysis without any signs of systemic fibrinolytic activity or side-effects. This new treatment strategy seems to be safe, requires minimal monitoring of fibrinolytic activity, and may be a new option for thrombolysis in high-risk patients such as premature infants, patients recently operated on, and patients presenting with other contra-indications for systemic fibrinolytic therapy.
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ranking = 6.1917679875234
keywords = cardiac
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3/87. Primary aldosteronism caused by aldosterone-producing adenoma in pregnancy--complicated by EPH gestosis.

    pregnancy in conjunction with primary aldosteronism is an unusual occurrence. We report a 28-year-old woman who presented with mild hypertension and hypokalemia as manifestations of primary aldosteronism caused by an aldosterone-producing adenoma in the left adrenal gland during pregnancy. Although the diagnosis was straightforward, the patient refused to undergo the proposed operation during the second trimester of her pregnancy. She was not admitted to hospital until she developed EPH gestosis in the 27th week of gestation, which had an unfavourable outcome for the infant who died nine days after delivery. The patient underwent a laparoscopic adrenalectomy which resulted in normalization of blood pressure and blood potassium levels. In cases of aldosterone-producing adenoma, surgery in the second trimester is the most appropriate option to avoid a poor obstetric outcome.
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keywords = gland
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4/87. A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.

    Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables. These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.
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ranking = 0.044900817381134
keywords = mucosa
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5/87. Right atrial tunnel to the left atrial appendage: a danger during balloon septostomy.

    Right atrial tunnel to the left atrial appendage is a very rare anomaly not previously described. Per se, it has no physiological significance but is a source of potential disaster during balloon atrial septostomy. The precise anatomy is demonstrated and ways are proposed to avoid tearing the atrial wall during therapeutic cardiac catheterization.
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ranking = 1.2383535975047
keywords = cardiac
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6/87. Balloon angioplasty of critical coarctation in a 970-gram premature infant.

    We describe a 970-gram infant born prematurely at 28 weeks gestation. On the third day of life, she presented in heart failure with Shone's syndrome and severe coarctation of the aorta. Despite initiation of intravenous prostaglandins, she developed progressive severe biventricular dysfunction and acidosis. Balloon angioplasty of the coarctation was successfully performed on the fifth day of life, with a dramatic improvement in ventricular function. At seven weeks of age, after improvement of ventricular function, she underwent surgical resection of the coarctation and arch advancement. At six months of age she is thriving and developing normally.
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keywords = gland
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7/87. Human cytomegalovirus as a direct pathogen: correlation of multiorgan involvement and cell distribution with clinical and pathological findings in a case of congenital inclusion disease.

    The human cytomegalovirus (HCMV), a member of the herpesviridae, is the most frequent cause of congenital virus infections and a major cause of morbidity and mortality in immunocompromised patients. Due to the lack of an appropriate animal model, insight into the pathogenesis of HCMV infections originates primarily from in situ examination of HCMV-infected tissues. Although in immunocompromised adults such tests are complicated frequently by the presence of additional misleading pathogens, the absence of additional pathogens renders congenital inclusion disease the most suitable access for investigation of pathogenetic aspects of HCMV infections. Immunohistochemical examination of tissue sections from a boy with fatal congenital inclusion disease was undertaken to detect the extent of multiorgan and cell involvement. Adrenal gland, bone marrow, diencephalon, heart, kidney, liver, lung, pancreas, placenta, small bowel and spleen were included in this study. Detection of virus antigens from different phases of viral replication revealed that all investigated organs were infected by HCMV. Simultaneous detection of cell type specific marker molecules showed that a variety of cell types stained positive for HCMV antigens including endothelial cells, epithelial cells, smooth muscle cells, mesenchymal cells, hepatocytes, monocytes/macrophages and granulocytes. The lung, the pancreas, the kidneys and the liver were the major target organs with a high number of HCMV infected cells. This correlated with multiorgan failure as the cause of death and strongly indicates direct pathogenetic effects of HCMV.
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keywords = gland
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8/87. Continuous insulin infusion: promoting growth in low birth weight infants.

    Development of improved technologies in neonatal care has yielded increasing numbers of surviving low birth weight (LBW) infants who have challenged methods of supportive care. As researchers and practitioners have focused on respiratory, cardiac, and other body system requirements, nutritional support has been low on the priority list. Support for growth and maximized neurologic development, rather than simply for survival, has been the challenge in care of small and sick newborns. Use of insulin to enhance glucose tolerance in LBW infants, and thereby maximize growth, is a management modality that can be initiated early in the infant's course and is facilitated by implementation of clear and consistent policies and procedures. Continuous insulin infusions have been demonstrated to enhance glucose uptake and utilization--facilitating neonatal growth and in turn enhancing brain growth and developmental outcome.
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ranking = 1.2383535975047
keywords = cardiac
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9/87. peritoneal dialysis for continuing renal support after cardiac ECMO and hemofiltration.

    Postoperative acute renal insufficiency after cardiac surgery in neonates is associated with increased mortality and is usually treated (while using ECMO, extracorporeal membrane oxygenation) with hemofiltration. Renal support has to be continued after weaning from ECMO when oliguria persists. When using hemofiltration, prolonged anticoagulation and a vascular access is needed, which, however, carries the risk of hemorrhagic as well as thromboembolic complications. Alternatively, peritoneal dialysis (PD) can be performed. We report data from 5 infants treated with ECMO after corrective cardiac surgery, who experienced oliguria after ECMO weaning and were consequently treated with PD. Arterial and central venous pressures, inotropic demand, urinary output, blood urea nitrogen, creatinine and survival were investigated. All patients survived. Installation of PD resulted in stable hemodynamics in all patients, despite continued oliguria. Normal renal function was established in four patients. One patient, suffering from persistent renal insufficiency, remained on PD. PD effectively supports insufficient renal functioning after ECMO weaning without the need for anticoagulation or a vascular access. Acute renal insufficiency may be reversible even after weeks and, if necessary, PD also enables prolonged treatment until renal transplantation.
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ranking = 7.430121585028
keywords = cardiac
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10/87. Successful use of tissue plasminogen activator in catheter-related intracardiac thrombus of a premature infant.

    The incidence of clinically apparent neonatal thrombosis is about 0.1 to 0.2% in the neonatal intensive care unit (NICU). The optimal treatment modalities for neonates with thromboses are not known. We report on our experience with recombinant tissue type plasminogen activator (rt-PA) in a premature infant with an intracardiac thrombus.
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ranking = 6.1917679875234
keywords = cardiac
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