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Biomedical subjects

G S Zaatari

Publications and source records attributed to G S Zaatari.

At least 19 recordsLinked to original sources

Lateral cervical bronchogenic cyst: an unusual cause of a lump in the neck.

BACKGROUND: Congenital cysts of the neck in children are not uncommon. Most of these are thyroglossal, branchial cleft, or less commonly, thymic cysts. Bronchial cysts rarely are initially seen as a neck mass. METHODS: Use of an illustrative case of a bronchogenic cyst initially seen as an upper lateral neck mass. CONCLUSIONS: We emphasize that although ectopic bronchogenic cysts are rare lesions of the head and neck, especially in the upper lateral neck, they should be included in the differential diagnosis in the evaluation of congenital neck cysts.

Bronchogenic Cyst↗

Scrotal calcinosis: is it idiopathic?

Idiopathic calcinosis of the scrotum is a rare condition, and its pathogenesis remains unknown. We recently examined 3 patients in whom scrotal epidermoid cysts in varying stages of inflammation coexisted with scrotal calcinosis. Some cysts revealed calcification of their keratin contents with little evidence of active inflammation. Other cysts showed rupture of their epithelial walls associated with the presence of keratin fibers and calcium granules in the surrounding dermis. We found that this disorder is not idiopathic and that epidermal inclusion cysts constitute the basic abnormality.

Calcinosis↗

Dendritic cell tumors associated with low-grade B-cell malignancies. Report of three cases.

Indeterminate and interdigitating cell tumors are rare proliferations of immunoregulatory cells that demonstrate morphologic, immunologic, and ultrastructural features similar to their cells of origin. Although an association of lymphoproliferative disease with Langerhans' cell histiocytosis is well described, only sporadic cases of non-Langerhans' dendritic cell proliferations have been published. The authors describe three patients with low grade B-cell lymphoproliferative disease who developed subsequent indeterminate cell or interdigitating cell tumors. When the two cases of indeterminate cell tumor are added to those previously described in the literature, it appears that 4 of 13 cases (31%) are associated with a history of low grade B-cell malignancy. Possible explanations for the relationship between these two disorders are discussed.

Dendritic Cells↗

Malignant lymphoma of the skin in children.

The clinical and histopathologic findings in eight cases of malignant lymphoma of the skin in children are presented. All patients had skin lesions as a primary manifestation of the disorder. Three patients had simultaneous regional lymph node involvement documented by the findings of subsequent biopsies. The majority (five patients) had solitary nodules involving the skin of the head and neck region. Three of the skin biopsy specimens were classified as lymphoblastic lymphoma, two large cell lymphoma, two mixed small and large cell lymphoma, and one small cleaved cell lymphoma. Disseminated disease subsequently developed in four patients in an interval that ranged from 4 to 30 months after diagnosis. The follow-up period ranged from 8 to 56 months, and median survival was 56 months. A literature review of 33 previously reported patients and our eight patients indicate that: skin of the head and the neck region is the most common site of involvement (56%); the majority of lymphomas are diffuse (93%); lymphoblastic lymphoma is the predominant type (53%), with a high proportion showing a non-T-cell phenotype; Burkitt's lymphoma and Hodgkin's disease of the skin are extremely rare; and most patients presented in an early clinical stage (Stage IE 56%, Stage IIE 21%), and prolonged disease-free survival was seen mostly in Stage I patients. The cumulative probability of survival for Stage I patients at 24 months was 0.71; while for Stages II to IV patients combined, it was 0.33 at 26 months.

Adolescent↗

Coaxial laser energy delivery using a steerable catheter in canine coronary arteries.

Intracoronary delivery of argon laser energy was studied in eight anesthetized mongrel dogs. A No. 4.5 French single lumen catheter, with steerable guidewire and an optical fiber, was introduced through a Judkins-type femoral-coronary guiding catheter into three left anterior descending and eight left circumflex coronary arteries. A total of 65 laser energy exposures were made coaxially at 24 sites in the 11 arteries. At five sites, angiographically evident arterial perforation occurred with the first laser exposure, while at seven sites multiple laser exposures were made without angiographically evident perforation. All eight dogs remained hemodynamically stable, and were electively killed 5 +/- 1 hours following the procedure. Sections of myocardium from territories supplied by treated arteries demonstrated minimal or no pathology in 10 cases, while one territory had a small zone of early myocardial necrosis. This study suggests that standard coronary artery catheterization techniques can be used to introduce and position a steerable guidewire and an optical fiber in canine coronary arteries. Laser energy can repeatedly be delivered coaxially. Short-term deleterious effects may be reduced or eliminated, and exposure of blood elements to argon laser energy does not appear to create debris.

Animals↗

Immunohistochemical staining for apolipoprotein B in human sarcomas.

A study of immunocytochemical staining for apolipoprotein B (apo B) in 52 human sarcomas and eight benign soft-tissue tumors is described. A peroxidase-antiperoxidase technique was employed using a rabbit anti-apo B antiserum. All 27 liposarcomas examined were positive for apo B, and 23 of these tumors showed at least 2-plus staining on a scale of 0 to 3 plus. Of the ten myxoid liposarcomas within this group, seven showed 2-plus or 3-plus staining, while the other three stained 1 plus. By contrast, 11 of 13 malignant fibrous histiocytomas were negative for apo B; the remainder showed 1-plus staining. Myxoid areas present in three of these cases were negative for apo B. Five of seven rhabdomyosarcomas stained at least 2 plus for apo B. Both benign and malignant peripheral nerve sheath tumors showed no consistent pattern of staining. We conclude that apo B immunocytochemical staining tends to be high in tumors whose normal tissue counterparts exhibit relatively large numbers of low-density lipoprotein receptors. Furthermore, immunoperoxidase staining for apo B should be a useful adjunct in the evaluation of soft-tissue sarcomas, particularly in cases with myxoid differentiation.

Apolipoproteins B↗

Inhibition of serine palmitoyltransferase activity in rabbit aorta by L-cycloserine.

Serine palmitoyltransferase (EC 2.3.1.50) initiates the biosynthesis of sphingolipids. Its activity is induced in the aortas of rabbits fed a Purina lab chow supplemented with 2% cholesterol (Williams, R. D., D. S. Sgoutas, and G. S. Zaatari. 1986. J. Lipid Res. 27: 763-770). Induction occurs during atherogenesis in parallel with increased arterial sphingomyelin concentrations. In this study, L-cycloserine was shown to be a potent inhibitor of serine palmitoyltransferase in aortas from New Zealand White rabbits. Activity was reduced in vitro by 50% using 5 microM L-cycloserine with 50 micrograms of microsomal protein. To assess in vivo inhibition, L-cycloserine was administered by intraperitoneal injection to rabbits maintained on either a standard Purina laboratory chow or one supplemented with 2% cholesterol. Serine palmitoyltransferase activity was inhibited by 76% in the aortas of rabbits on the standard chow 4 hr after a single 25 mg/kg body weight dose and 52% after a 10 mg/kg dose. Activity was reduced by 36% in animals on the standard chow and by 37% in the cholesterol-fed group after 1 week of daily doses. These experiments demonstrate that L-cycloserine inhibits serine palmitoyltransferase in aorta, and thus may be used to reduce sphingomyelin concentrations during experimental atherogenesis.

Acyltransferases↗

Crohn's disease of the esophagus.

In this report we describe the case of a 50-year-old woman with Crohn's disease of the esophagus. She had severe distal stricture that necessitated distal esophagectomy, with esophagogastric anastomosis and Nissen fundoplication. Surgical treatment of esophageal Crohn's disease is associated with a number of complications; however, on the basis of our experience and a literature review, pyloroplasty and Nissen fundoplication in addition to surgical resection seems to be associated with fewer postoperative complications.

Crohn Disease↗

Steerable fiberoptic catheter delivery of laser energy in atherosclerotic rabbits.

Imprecision in guiding and positioning is a recurring problem with fiberoptic delivery of laser energy (E) in small arteries. Manipulation can produce mechanical perforation of the vessel, and noncoaxial alignment can result in thermal perforation at relatively low laser energy levels. A No. 4.5 French single-lumen catheter was designed to accommodate both a steerable guidewire and an optical fiber. It was passed, under fluoroscopic control, into the abdominal aorta in 18 atherosclerotic rabbits. Argon laser energy was delivered coaxially at three sites in each of 14 rabbits (total = 42 sites); four rabbits were controls. Laser power levels (1 to 6 W) and exposure times (20 to 60 seconds) were varied. Energy level in joules (J) was calculated for each exposure. Saline flush at 25 ml/min was delivered through the catheter during laser exposures. Angiographic or microscopic evidence of vessel perforation was observed at 10 sites (E = 174 +/- 108 J). Another six sites exhibited microscopic laser effect only, without evidence of vessel perforation (E = 155 +/- 91 J). The remaining 26 sites exhibited no effects of laser energy (E = 117 +/- 92 J). No angiographically visible perforation occurred with E less than 120 J. This study suggests that a fiberoptic catheter with steerable guidewire allows safer intravascular manipulation of optical fibers, improves coaxial alignment in the arterial lumen, and may permit substantial laser energy delivery into atherosclerotic arteries.

Animals↗

Oxyphilic granular cell adenoma (oncocytoma) of the esophagus.

An oncocytoma of the esophagus is presented. The patient's chief complaint was dysphagia, and barium swallow suggested cricopharyngeus muscle hypertrophy. Exploration revealed an intramural mass in the cervical esophagus. Enucleation resulted in relief of symptoms. Pathological and immunohistochemical analysis demonstrated an oncocytoma of thyroid origin.

Adenoma↗

Klippel-Trenaunay and Sturge-Weber syndromes with renal hemangioma and double inferior vena cava.

We describe a 3 1/2-year-old boy with the Klippel-Trenaunay and Sturge-Weber syndromes. The child had congenital superficial capillary hemangiomas, congenital glaucoma and mild hydrocephalus. During the first year of life he experienced intermittent hematuria. When he was 3 years old he presented with seizures and left hemihypertrophy first was noted. Several months later radiological examination of a large abdominal mass demonstrated its origin to be in the right kidney. Radical nephrectomy documented the presence of renal hemangioma with complicating perirenal hematoma. A double inferior vena cava was another unexpected surgical finding that complicated the course of this patient. All of these unusual features in these rare syndromes with their clinical, pathogenetic and therapeutic implications are discussed. The differential diagnosis of renal masses in these syndromes also is presented.

Angiomatosis↗

Cystic neuroblastoma in infants: radiographic and pathologic features.

Cystic neuroblastoma is a rare form of neuroblastoma. Three cases of cystic neuroblastoma in the infant are reported with emphasis on the sonographic findings. In two cases, the tumor was demonstrated in the fetus. The pathologic features of the tumors are described. Development of cysts may be related to a prominent microcystic arrangement of tumor nests.

Adrenal Gland Neoplasms↗

Enzymology of long-chain base synthesis by aorta: induction of serine palmitoyltransferase activity in rabbit aorta during atherogenesis.

Serine palmitoyltransferase [EC 2.3.1.50] initiates the biosynthesis of sphingolipids by catalyzing the condensation of a fatty acyl-CoA with serine to yield the committed intermediate 3-ketosphinganine or one of its homologues. The presence of serine palmitoyltransferase in aorta was established under optimal assay conditions using microsomes from New Zealand White rabbits. Its activity was dependent on microsomal protein, L-serine, pyridoxal 5'-phosphate, and palmitoyl-CoA. Although several different saturated and unsaturated fatty acyl-CoA thioesters were utilized as substrates, maximal activity was with palmitoyl-CoA, suggesting that this enzyme contributes to the predominance of 18-carbon long-chain bases in sphingolipids from aorta. Rabbits, fed a Purina lab chow supplemented with 2% cholesterol, were used to study serine palmitoyltransferase activity in aorta during experimental atherogenesis. An increase in activity from intimal-medial preparations was detectable prior to prominent lipid accumulation or cellular proliferation. Activity continued to elevate over the 12-week duration of feeding concurrent with the increase in serum cholesterol and in proportion to the development of plaques resulting in a 3.7-fold increase in activity (20.7 +/- 2.6 pmol per min per mg microsomal protein +/- SE in the cholesterol-fed group versus 5.6 +/- 1.9 in the pair-fed controls also matched for age and sex; P less than 0.005). Thus, the accumulation of sphingomyelin that occurs in aorta during experimental atherogenesis may be related to increased long-chain base synthesis by serine palmitoyltransferase.

Acyltransferases↗

Adenoid squamous cell carcinoma of the nasopharynx and neck region.

Adenoid squamous cell carcinoma (ASCC) is a rare variant of squamous cell carcinoma. This tumor most commonly occurs in the skin of the head and neck and rarely in the vermillion of the lip, vulva, and oral cavity. This report of two patients includes the first (to our knowledge) case of ASCC in the nasopharynx. It also describes the immunocytochemical and ultrastructural features of this tumor. Our findings and a review of the literature indicate that ASCC of the upper aerodigestive tract is associated with an aggressive behavior and poor prognosis, in contrast with ASCC of the skin.

Adenocarcinoma↗

Characteristics of children with endoscopically proved chronic bronchitis.

This study evaluated by chart review the clinical, allergic, pathologic, and immunologic characteristics of 20 children found to have chronic bronchitis (CB) by bronchoscopic evaluation, including 13 children in whom CB was documented by endobronchial biopsy. Two additional children likely to have had CB also are described. In this study group, all of the patients were believed to have asthma as well. Chronic bronchitis was predicted by chronicity of symptoms and incomplete response to bronchodilators and corticosteroids, but it was not predicted by a history of allergy or laboratory evidence of systemic infection or inflammation. In the children described herein, CB often was associated with an IgG subclass abnormality. Bronchoscopic evaluation documented CB and provided biopsy and secretion samples. Although the histomorphic findings were heterogeneous, patients with CB usually had white blood cells present on Gram stains of secretions in addition to mononuclear cell infiltrates on biopsy specimens. Chronic bronchitis in these children seems to be distinct from CB in adults. Further studies will be needed to define CB in children.

Adolescent↗

Recurrence of Wilms tumor after apparent cure.

The recurrence of Wilms tumor after a 5-year disease-free interval is rare. We present two patients who had recurrent disease after a disease-free interval of greater than 7 years. Three additional patients, registered with the National Wilms Tumor Study who had a recurrence after 5 years, are also described. Of these three patients, two had nephroblastomatosis. Because more patients are achieving long-term survival, careful surveillance after apparent "cure" is recommended, particularly if nephroblastomatosis is identified in the original nephrectomy specimen.

Child, Preschool↗

Bronchial extramedullary hematopoiesis preceding chronic myelogenous leukemia.

Extramedullary hematopoiesis of the bronchus is rare. The case of a 72-year-old man in whom the right lower lobe bronchus was obstructed by extramedullary hematopoiesis is presented. Ten months after the initial presentation, Philadelphia chromosome-negative chronic myelogenous leukemia was diagnosed. Such findings have not been reported previously. The various anatomic locations of extramedullary hematopoiesis are reviewed, with an emphasis on intrathoracic and pulmonary presentations. The clinical and pathologic features and the differential diagnosis in the present case are discussed.

Adenocarcinoma↗