chylothorax/seizures

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Successful factor XIII treatment of refractory chylothorax in tuberous sclerosis complex-associated lymphangioleiomyomatosis, multifocal multinodular pneumocyte hyperplasia and mediastinal lymphadenopathy.

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Tuberous sclerosis complex (TSC) or Bourneville disease is a rare autosomal dominant neurocutaneous disorder that affects various organs. Pulmonary involvement in TSC may consist of lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH), occurring together or alone.

LYMPHODYSPLASIA AND KRAS MUTATION: A CASE REPORT AND LITERATURE REVIEW.

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Cardio-facio-cutaneous (CFC) syndrome is a very rare and sporadic disease whose characteristics include dysmorphic facial appearance, ectodermal abnormalities, cardiac abnormalities, growth retardation and neurodevelopmental delay. This syndrome is classified as one of the RAS syndromes which are

Schimmelpenning syndrome: an association with vascular anomalies.

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OBJECTIVE Schimmelpenning syndrome is a rare neurocutaneous disorder characterized by craniofacial nevus sebaceus in association with seizures, developmental delay, and ocular or skeletal pathology. Vascular anomalies also have been described in this condition, and some authors have suggested that

Long-term complications in a premature infant with tracheoesophageal fistula.

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This is a report of the short- and long-term complications in a premature infant with tracheoesophageal fistula, including those related to central venous alimentation, seizures, chylothorax, bronchopulmonary dysplasia, dental erosions, gastroesophageal reflux, pulmonary problems, and gall stones.

The neural and vascular effects of killed Su-Streptococcus pyogenes (OK-432) in preterm fetal sheep.

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Fetal exposure to inflammatory mediators is associated with a greater risk of brain injury and may cause endothelial dysfunction; however, nearly all the evidence is derived from gram-negative bacteria. Intrapleural injections of OK-432, a killed Su-strain of Streptococcus pyogenes, has been used to

Serum lactate as mortality and morbidity marker in infants after Jatene's operation.

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OBJECTIVE To assess the morbidity and mortality after Jatene's operation using lactate as the main marker. METHODS We performed a historical cohort with infants admitted in a pediatric intensive care unit during 1995 to 2005 who underwent this surgery. We assessed the preoperative, immediate (IPD),

[Chylous effusions].

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This paper presents 4 patients with chylothorax, and one patient with bilateral chylothorax and chyloperitoneum. The chylous effusions were of benign etiology, developed as a complication of miliary tuberculosis (1 patient), after L-2 vertebral body fracture (1 patient), and idiopathic (2 patients).

Lymphangioleiomyomatosis: A review.

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Lymphangioleiomyomatosis (LAM) is a rare disease, of unknown etiology, affecting women almost exclusively. Microscopically, LAM consists of a diffuse proliferation of smooth muscle cells. LAM can occur without evidence of other disease (sporadic LAM) or in conjunction with tuberous sclerosis complex

Multivisceral transplantation for abdominal tumors in children: A single center experience and review of the literature.

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Standard management of intra-abdominal pediatric solid tumors requires complete resection. However, tumors with multiple organ and vascular involvement present a unique surgical challenge. We conducted a retrospective chart review of four patients, aged 2-14 years, undergoing MVT for intra-abdominal

Arch Augmentation via Median Sternotomy for Coarctation of Aorta With Proximal Arch Hypoplasia.

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BACKGROUND Coarctation of the aorta can be associated with hypoplasia of the proximal transverse aortic arch. One approach to manage this condition is via left thoracotomy and extended end-to-end anastomosis with the expectation that the proximal arch will grow over time. Our preferred approach is

Bilateral bidirectional Glenn: outcome of off-pump technique.

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Off-pump bilateral bidirectional Glenn (b-BDG) poses a surgical challenge and may add complexity to the postoperative outcome especially regarding uniformity of the anastomosis and central pulmonary artery growth. Herein, we report early- and mid-term outcomes after off-pump b-BDG without using

NTP workshop: animal models for the NTP rodent cancer bioassay: stocks and strains--should we switch?

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The National Toxicology Program (NTP) hosted a workshop, "Animal Models for the NTP Rodent Cancer Bioassay: Strains and Stocks--Should We Switch?" on June 16-17, 2005, at the National Institute of Environmental Health Sciences (NIEHS) in Research Triangle Park, North Carolina. The workshop's

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