Herpes esophagitis in the immunocompetent child.
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Biomedical subjects
Publications and source records attributed to B B Dahms.
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A 3-month-old infant presented with cardiomegaly, mitral insufficiency, myocardial ischemia, and congestive heart failure suggestive of anomalous left coronary artery. A vestigial left coronary artery was demonstrated on angiography and was thought to represent the only abnormality. At autopsy, the infant had a vestigial left coronary artery arising from the aorta but in addition had a normal caliber anomalous left coronary artery arising from the pulmonary artery.
The incidence of fasting hyperglycemia and diabetes mellitus (DM) in older patients with cystic fibrosis (CF) is reported to be 8%, but few quantitative studies of the islets of Langerhans in this disease have been done. Three groups of patients were studied in this morphometric autopsy analysis: patients with CF and insulin-dependent DM (n = 7), normoglycemic patients with CF (n = 4), and age-matched adolescents and young-adult controls (n = 11). The islets of Langerhans were stained with immunoperoxidase for insulin, glucagon, and somatostatin. The percentage of the total islet surface area occupied by each immunoperoxidase positive cell type was determined by a point-counting method. The mean percent surface area occupied by insulin-producing cells (28.3%) in diabetics with CF was significantly less than normoglycemics with CF (46.7%) and controls (53.4%). The mean percent surface area occupied by glucagon-producing cells was similar in all three groups: 21.9% in CF diabetics, 25.4% in normoglycemics with CF, and 22.4% in controls. The mean percent surface area occupied by somatostatin was increased in both CF groups compared with controls: diabetics with CF, 29.3%; normoglycemics with CF, 26.2%; and controls, 15.5%. These findings correlate with published clinical endocrine studies of hyperglycemia in CF. Endocrine cell quantitation in diabetics with CF differs from that in both juvenile (type 1) and adult-onset (type 2) DM.
A 6-month-old infant presenting with pericardial effusion was found to have a malignant rhabdoid tumor of the heart. As visualized by two-dimensional echocardiography and subsequently by contrast-enhanced computerized axial tomographic scanning, the tumor arose from the left ventricular free wall and grew into the pericardial space. Despite two courses of single-agent chemotherapy with doxorubicin, the child died 3 months later. This is the first reported case of malignant rhabdoid tumor of the heart, and the fifth case of a primary cardiac malignancy in an infant.
Fresh isolated myocardial, elastic-arterial, and valvular tissues from seven canine hearts were irradiated by argon laser. Irradiation was transmitted through 300 and 400 micron flexible quartz fiberoptic elements. Minimal power densities for vaporization of the myocardial, arterial, and valvular tissues were 80, 90, and 110 W/cm2, respectively, with maximal vaporization distances (fiberoptic tip to tissue) of 4 mm, 1 mm, and 1 mm, respectively. Irradiation of the valves at power densities approaching perforation caused contraction of the tissue. When tissue vaporization occurred, histologic examination of irradiated tissues showed a central crater surrounded by sequential layers of char, vaporization, and coagulation necrosis. These findings were common to all cardiac tissues. Additional findings unique to myocardium were a normal-appearing myocardial layer (skip-area), circumferential halo, and tissue clefts. Elastic arteries showed concentration of necrosis around the collagen and elastic fibers. Valvular damage was the most extensive and also included contraction of adjacent valvular tissue and endocardial sloughing.
Systemic infection with Malassezia furfur was first reported in 1981 as a specific complication of Intralipid therapy in a neonate. Six additional patients, including three older than 16 years of age, were identified subsequently. All had received prolonged Intralipid infusion through central venous catheters. Pulmonary infection was documented in tissue in three cases, the clinical presentation was characterized by pulmonary infiltrates, fever, and, in the infants, thrombocytopenia. Two subgroups of patients appear to be at the greatest risk for Malassezia infection: neonates with cardiopulmonary disease and adults with severe gastrointestinal disease and immunosuppression. The documentation of pulmonary arterial lipid deposits in vessels that had been infiltrated by Malassezia organisms and the observation of organisms in small pulmonary thromboemboli suggest that these lipophilic and lipid-dependent organisms are introduced into the bloodstream from venous catheters and require high lipid concentrations to proliferate in tissue.
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Torulopsis glabrata causes an increasingly frequent infection in immunocompromised adults. In a 1,910-g, 36-week, small-for-gestational-age neonate, T glabrata caused necrotizing enterocolitis and endophthalmitis. The fungus was not grown from cultures obtained from four blood specimens but was seen microscopically in methenamine silver-stained sections of resected bowel.
Although hypervitaminosis A is not uncommon, fatal cases are rare. We describe a neonate who died after having ingested more than 60 times the suggested dose of vitamin A per day, for 11 days. His hospital course was marked by hypercalcemia, hyperphosphatemia, a bleeding disorder, and pulmonary insufficiency. An autopsy showed extensive calcifications of the alveolar septa and bronchioles. Metastatic calcifications were also present in the kidneys, stomach, soft tissue, and skin. The skeleton showed prominent alteration of the endochondral bone formation. There was also evidence of accelerated resorption of bone, which is presumably responsible for the development of hypercalcemia and metastatic calcification.
It is widely acknowledged that Barrett's esophagus in adults is an acquired condition resulting from prolonged gastroesophageal reflux. Barrett's esophagus is rare in childhood, even though gastroesophageal reflux occurs commonly in the pediatric age group. When a columnar-lined esophagus is present in children, it is often regarded as a congenital anomaly rather than as a consequence of chronic gastroesophageal reflux. Over a 5-yr period (1978-1982), we retrospectively studied Barrett's esophagus in children 19 yr of age or younger who were evaluated for gastroesophageal reflux and whose symptoms warranted esophagoscopy and esophageal biopsy. Esophageal biopsies were performed on 103 patients with gastroesophageal reflux. Thirteen children (age range, 8 mo-19 yr) had Barrett's esophagus, for a prevalence of 13%. Gastroesophageal reflux was documented in these children by upper gastrointestinal radiographs or pH monitoring. Radiographs demonstrated esophageal stricture in 5 of the 13 children; none had hiatal hernia. Children presented with symptoms suggestive of gastroesophageal reflux and esophagitis: vomiting, abdominal pain, odynophagia, dysphagia, and heartburn. All children had a past history of excessive regurgitation during infancy. Histologically, three types of columnar epithelium were present: gastric fundic type (11 patients), junctional-type columnar epithelium reminiscent of gastric cardia (7 patients), and specialized columnar (metaplastic intestinal) type (2 patients). We believe that Barrett's esophagus is more common in children than had previously been appreciated. In these children, we suggest that the distal columnar-lined esophagus resulted from chronic gastroesophageal reflux and is not a congenital anomaly.
Two female siblings died within three months after presenting with paravertebral tumors in the first year of life. The pathology of the two tumors was identical and characteristic of a malignant rhabdoid tumor. There were no identifiable tumor patterns within the kindred which have been associated with any hereditary cancer or precancer syndromes. Fibroblasts were cultured from skin biopsies obtained from the second patient and both parents. Assays of growth kinetics associated with cellular transformation revealed that fibroblasts from the affected sibling can be distinguished from those of the parents and age-matched controls by increased in vitro occurrence of tetraploidy. Such evidence suggests that increased in vitro tetraploidy occurring spontaneously in cultured fibroblasts is an expression of a cancer-prone gene. Increased in vitro tetraploidy has previously been demonstrated in some kindreds with heritable colon cancer syndromes, and may extend our understanding of the genetic etiology of some childhood cancers.
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Six children with chronic diarrhea and neutropenia, initially referred for evaluation of Shwachman-Diamond syndrome, were found to have no evidence of pancreatic insufficiency. All presented in the spring with a prodromal respiratory illness. Hematologic evaluation was normal except for iron deficiency anemia and neutropenia. Small intestinal biopsies of all children showed inflammation, consistent with chronic enteritis. The children were followed until growth returned to previous percentiles. Diarrhea and neutropenia resolved by 6-month follow-up, and there was no recurrence of the neutropenia at 1 year.
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