Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of a female neonate with Beckwith-Wiedemann syndrome who manifested upper airway obstruction soon after birth and suffered from intractable hypoglycemia and abdominal distention caused by nephromegaly. She was delivered at 31 weeks of gestation with 2480 g and was diagnosed as Beckwith-Wiedeman syndrome, manifesting macroglossia, hepatomegaly, nephromegaly and omphalocele. Her trachea was intubated 30 minutes after birth due to upper airway obstruction. At 12 days of life, glossopexy was performed to relieve the airway obstruction. Although tracheal extubation was successfully accomplished 12 days later, 21 days after the glossopexy she manifested apnea and hypoxia and required tracheal intubation and mechanical ventilation again. We suspected hypoglycemia or central apnea to be the cause of apnea and started the administration of somatostatin analog as a treatment for hypoglycemia. In addition to the apnea, abdominal distention caused by nephromegaly exacerbated her respiratory condition. At 69 days of life she died of sepsis complicated with disseminated intravascular coagulation and renal failure. A needle biopsy at autopsy revealed nephroblastomatosis.
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PMID:[A neonate with Beckwith-Wiedemann syndrome who developed upper airway obstruction after glossopexy]. 1184 Jun 63

Placental mesenchymal dysplasia (PMD) is a rare disease that may be difficult to distinguish from molar pregnancy. The disease is associated with major fetal complications, including Beckwith-Wiedemann syndrome, fetal growth restriction and intrauterine fetal death. Rarely, fetal hematological disorders and liver tumors also may occur. Two patients were referred to our hospital during their second trimesters because of suspected molar pregnancies. Fetal karyotyping and maternal serum human chorionic gonadotropin level determinations led to the PMD diagnoses. In one case, the maternal clinical course was normal, but the neonate suffered from disseminated intravascular coagulation and needed a platelet transfusion. In the second case, the PMD decreased during pregnancy, but a gradually increasing fetal liver tumor appeared. The tumor was diagnosed as mesenchymal hamartoma, based on ultrasound and magnetic resonance imaging studies. The neonate was delivered without cardiovascular compromise. Due to the difficulty of immediate surgical treatment, expectant management, with close follow-up, was chosen.
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PMID:Rare fetal complications associated with placental mesenchymal dysplasia: a report of two cases. 2522 97