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)

Prostaglandin E1 intravenous infusion is used in infants with ductal-dependent congenital heart disease to maintain ductal patency and prolong life until palliative or corrective surgery is feasible. Complications of prostaglandin administration include fever, diarrhoea, hypotension, apnoea, bradycardia, pseudowidening of the cranial sutures, underossification of the calvarial bones, periostitis, and skin edema [1-3]. This paper presents dramatic plain radiographic features of prostaglandin-induced bone disease, including periosteal proliferation and the unusual bone-within-bone appearance, and provides the previously unpublished CT correlation.
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PMID:Plain film and CT observations in prostaglandin-induced bone changes. 152 47

A case of periosteal new bone in a newborn is presented. The periostitis resulted from long-term therapy with PGE1, which was administered to maintain patency of the ductus in a neonate with ductal-dependent cyanotic congenital heart disease. The features of PGE1 periostitis and the differential diagnosis are reviewed.
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PMID:Case report 701: Prostaglandin E1 (PGE1) periostitis. 177 31

Prostaglandin E1 (PGE1) is an essential drug for infants with ductal-dependent congenital heart disease. Cortical hyperostosis of the long bones is one of the complications during and after PGE1 therapy and should be considered in the differential diagnosis of periostitis in the infant.
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PMID:Cortical hyperostosis: a complication of prostaglandin E1 treatment in congenital heart disease. 260 53

The relationship of periostitis to disease duration in primary hypertrophic osteoarthropathy and the association of periostitis with cardiopulmonary disorders (secondary type) were studied in order to define distinguishing features between the two. Radiographic skeletal surveys were performed in 24 patients with hypertrophic osteoarthropathy to analyze pattern (single layer, multilayered, irregular) and site of involvement (diaphysis, metaphysis, epiphysis). The six patients with primary hypertrophic osteoarthropathy and the 11 patients with cyanotic congenital heart disease had thicker, more widespread periostitis involving the diaphysis, metaphysis, and epiphysis, in contrast to abnormalities in the seven patients with hypertrophic osteoarthropathy secondary to carcinoma of the lung. Average cortical bone widths as determined by radiogrammetric measurement of the second metacarpals were significantly greater for the patients with primary hypertrophic osteoarthropathy (8.9 +/- 6.0 mm) and cyanotic congenital heart disease (8.5 +/- 6.4 mm) as compared with the patients with bronchogenic carcinoma (6.0 +/- 3.9 mm). Correlation of radiographic patterns with duration of disease confirms that thicker, more extensive alterations are indicative of long-standing disease. The periostitis of hypertrophic osteoarthropathy is therefore not dependent on the primary or secondary nature of the disease but principally on its duration.
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PMID:Periostitis in hypertrophic osteoarthropathy: relationship to disease duration. 349 63

Prostaglandins are being commonly used to maintain the patency of the ductus arteriosus in infants with congenital ductal-dependent heart disease. A significant and unusual side effect of this drug treatment is the symmetrical development of periostitis of the long bones. A review of neonates with congenital heart disease requiring prostaglandin treatment at the Children's Hospital of Eastern Ontario revealed five infants who developed periostitis, the earliest onset being after 14 days of prostaglandin infusion. The drug dosage varied in these infants from 0.02 to 0.10 micrograms/kg/min. The periostitis was associated with limb pain and considerable swelling of the extremities in all children. The periostitis improved on cessation of the prostaglandin infusion, and by 6 weeks after the cessation of the drug, the periostitis had decreased significantly. Periostitis seemed more dependent on the duration of administration of the prostaglandin than on the dosage of prostaglandin administered. Awareness of this entity is essential not only for the treatment team caring for these infants but also for consultant pediatric orthopaedists to avoid excessive investigation for infection, metabolic disease, or vitamin deficiencies that resemble prostaglandin-induced periostitis.
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PMID:Prostaglandin-induced neonatal periostitis. 781

Hypertrophic osteoarthropathy (HOA), well known in adults, is rarely encountered in children. The clinical features include clubbing of the fingers and toes, arthritis, and a sometimes painful ossifying periostitis of the tubular bones. Apart from a hereditary form (primary HOA), most of the cases encountered in children are secondary and associated with conditions such as chronic suppurative lung processes (e.g., cystic fibrosis), congenital heart disease, biliary atresia, and polyposis coli. The association with malignant disorders, which is relatively common in adults, is very rare in children. In 1986 the authors published a case report of a patient with carcinoma of the nasopharynx who developed HOA. Another similar patient has been encountered. In both, the appearance of HOA was associated with a very poor prognosis. A meticulous research of the literature from 1890 to 1990 revealed only 24 children (19 boys, 5 girls) under the age of 18, with malignancy and associated HOA. Among them were 10 patients with a carcinoma of the nasopharynx, 8 with osteosarcoma, 3 with Hodgkin's lymphoma, 1 with a periosteal sarcoma, 1 with mesothelioma of the pleura, and 1 with carcinoma of the thymus. In five patients with HOA, there were no abnormalities of the lungs, mediastinum, or pleura, and none developed during the course of the disease. Many authors mention the predictive value of HOA, especially in association with malignant tumors. In contrast to suppurative processes in the lungs, in those with neoplastic disease involving the chest, HOA may precede pulmonary symptoms by 1-18 months. A striking feature of HOA in these instances is the reversibility of the complaints after successful treatment of the disorder of the chest, both in benign and malignant conditions. The present case is the second reported by the authors and the first description of a girl with carcinoma of the nasopharynx developing HOA.
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PMID:Hypertrophic osteoarthropathy in childhood malignancy. 841 3

Clubbing was first described by Hippocrates more than 2.500 years ago. It may be seen alone or as part of an entity called hypertrophic osteoarthropathy which include periostitis, arthritis and sometimes thickening and edema of the skin around the affected joints. Pulmonary diseases such as cancer, abscess, empyema, bronchiectasis and cystic fibrosis are the major diseases known to be associate with hypertrophic osteoarthropathy. Digestive tract cancer, cyanogenic congenital heart disease are well known association. Many theories have attempted to explain the appearance of this sign but few have persisted. In this article, we review characteristics, relation with etiology and the basis of the pathophysiology of hypertrophic osteoarthropathy and particularly of clubbing.
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PMID:[Review on hypertrophic osteoarthropathy and digital clubbing]. 1280 75

We report the development and spontaneous resolution of annular erythematous skin lesions consistent with sarcoid dermatitis in a child with DiGeorge syndrome (DGS) carrying the 22q11.2 microdeletion. The skin lesion developed after she was treated with isoniazid (INH) following exposure to active tuberculosis (TB). After resolution of the skin lesions, this child developed sterile hyperplastic osteomyelitis consistent with SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) osteomyelitis in her right mandible triggered by an odontogenic infection. This child had congenital heart disease, dysmorphic facies, recurrent sinopulmonary infection, gastroesophageal reflux disease, scoliosis, reactive periostitis, and developmental delay. She had a low CD4 and CD8 T cell count with a normal 4/8 ratio, but normal cell proliferation and T cell cytokine production in response to mitogens. When she was presented with sterile osteomyelitis of right mandible, she revealed polyclonal hypergammaglobulinemia with elevated erythrocyte sedimentation rate (ESR)/angiotensin converting enzyme (ACE) levels, but negative CRP. Autoimmune and sarcoidosis workup was negative. Inflammatory parameters gradually normalized following resolution of odontogenic infection and with the use of non-steroidal anti-inflammatory drugs (NSAIDs). The broad clinical spectrum of DGS is further expanded with the development of autoimmune and inflammatory complications later in life. This case suggests that patients with the DGS can present with unusual sterile inflammatory lesions triggered by environmental factors, further broadening the clinical spectrum of this syndrome.
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PMID:SAPHO osteomyelitis and sarcoid dermatitis in a patient with DiGeorge syndrome. 1649 84

Hypertrophic osteoarthropathy is an entity characterized by a triad of periostitis of long bones, clubbing and arthritis. Radiologically there are two patterns, one characterized by new bone formation which predominates in patients with pulmonary disease, and another by acro-osteolysis that is most frequently associated with congenital heart disease. We report the case of a 30-year-old man diagnosed with primary pulmonary hypertension for two years, developing hypertrophic osteoarthropathy with a mixed radiological pattern.
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PMID:Hypertrophic osteoarthropathy with acro-osteolysis in a patient with primary pulmonary hypertension. 2228 5