Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The radiographic findings of a series of infants of diabetic mothers and a review of the literature are presented to illustrate the wide spectrum of abnormalities that may be seen with this condition. Congenital anomalies of the spine and skeletal, genitourinary, and cardiovascular systems and visceral situs inversus are significantly more frequent among infants of diabetic mothers than normal. The most specific anomaly is sacral agenesis. Renal vein thrombosis and adrenal hemorrhage are also more common and may be diagnosed by sonography. Over one-half of the cases of the small left colon are associated with maternal diabetes and may be diagnosed and treated with a contrast enema. The incidence of the respiratory distress syndrome is higher in infants of diabetic mothers than other premature infants, and the disease may occur in the presence of reliable indicators of lung maturity. Other common causes of dyspnea include cardiomyopathy, congenital heart disease, wet lung syndrome, hyperviscosity syndrome, and persistence of fetal circulation. Echocardiography is the most valuable adjunct in differentiating cardiac from pulmonary problems.
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PMID:Infants of diabetic mothers: radiographic manifestations. 678 62

In 1981, the Food and Drug Administration approved prostaglandin E1 (PGE1) for use in the treatment of neonates with congenital heart disease. PGE1 is commonly used in neonatal and pediatric intensive care units to maintain patency of the ductus arteriosus in those cardiac lesions that depend on the ductus for either systemic or pulmonary blood flow. Early recognition of hemodynamic instability and prompt initiation of PGE1 therapy is vital to survival in neonates with ductal-dependent cardiac lesions. Administration of PGE1 allows delay of palliative or corrective surgery until stabilization or transfer of the neonate to a tertiary care facility is achieved. It is also used as a bridge to heart transplantation in neonates in whom this treatment is an option. Congenital anomalies requiring treatment with PGE1 are those that restrict pulmonary blood flow (cyanotic or right-sided outflow tract obstructions) and systemic blood flow (acyanotic or left-sided outflow tract obstructions). Prostaglandin E1 is excreted by the kidneys, and elimination is almost complete within 24 hours after administration; 80 percent of it is rapidly metabolized after one pass through the pulmonary bed. Therefore, a continuous infusion and adequate intravenous access are necessary. Nurses caring for these neonates must have knowledge of all its potential side effects. Some of the most common side effects include cutaneous vasodilation, bradycardia, tachycardia, hypotension, seizure-like activity, hyperthermia, and apnea.
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PMID:Nursing care of the neonate receiving prostaglandin E1 therapy. 851 Jun 22

MWS is a multiple congenital anomaly syndrome, first clinically delineated by Mowat et al in 1998. Over 45 cases have now been reported. All patients have typical dysmorphic features in association with severe intellectual disability, and nearly all have microcephaly and seizures. Congenital anomalies, including Hirschsprung disease (HSCR), congenital heart disease, hypospadias, genitourinary anomalies, agenesis of the corpus callosum, and short stature are common. The syndrome is the result of heterozygous deletions or truncating mutations of the ZFHX1B (SIP1) gene on chromosome 2q22.
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PMID:Mowat-Wilson syndrome. 1274 90

Solitary median maxillary central incisor (SMMCI) syndrome is a rare dental anomaly that affects 1:50,000 live births. SMMCI syndrome is characterized by the presence of a single central incisor located on the maxillary midline in both primary and permanent dentitions. It may occur as an isolated finding or in association with developmental defects and systemic involvement. Congenital anomalies associated with SMMCI syndrome can include short stature, mild forms of deviation in craniofacial morphology, mild to severe intellectual disability, congenital heart disease, and cleft lip and/or palate. This report describes a clinical case of a 7-year-old girl with SMMCI syndrome--in addition to bilateral residual cleft and associated nasal regurgitation--that was treated with a removable maxillary obturator.
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PMID:Solitary median maxillary central incisor syndrome associated with unique cleft palate: a rare case report. 2459 4