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Biomedical subjects

R Drut

Publications and source records attributed to R Drut.

At least 91 records · Page 5Linked to original sources
PubMed1988

Ossifying fibrolipomatous hamartoma of the ulnar nerve.

An ossified fibrolipomatous epineural hamartoma limited to the right ulnar nerve and some of its branches and extending from the elbow distally is described in a 5-year-old boy who complained of pain in the right hand. Such extensive involvement of the ulnar nerve is unusual; moreover, metaplastic bone is rarely present in the more common fibrolipomatous hamartoma of the median nerve. Increased awareness of this entity is necessary to avoid sacrifice of large nerves that may be included in the lesion.

Bone and Bones↗
PubMed1988

Measles pneumonia in a newborn.

We report a 21-day-old preterm infant who had severe respiratory distress of 6 days' duration and whose lungs revealed a giant cell pneumonia at necropsy. Measles antigen was demonstrated in mononuclear and multinucleated epithelial cells of the lung by immunoperoxidase staining. We recommend the immunostaining procedure to differentiate measles from other viruses, such as parainfluenza 2 or 3, and respiratory syncytial virus, all of which may produce giant cell pneumonias.

Humans↗
PubMed1988

Rhabdomyomatous dysplasia of the lung.

This article deals with the presence of nontumoral striated muscle fibers in the lungs of 3 neonates. These cells were diffusely distributed in one lung (case 1) or in both (case 2), or focally localized to the lung parenchyma adjacent to the liver in a case with a large right diaphragmatic hernia (case 3). The striated muscle fibers were located in the walls of small bronchi and bronchioli or in the alveolar interstitium. Other major lung malformations found simultaneously were absence of lobation, hypoplastic lungs, and hypoplastic pulmonary vessels. The origin of striated muscle fibers in the neonatal lung has been attributed to anomalous differentiation of mesoblastic cells (as in cases 1 and 2). The presence of striated muscle cells in the lower margin of a hypoplastic lung associated with a right diaphragmatic hernia (case 3) suggests that intrapulmonary inclusion of diaphragmatic muscle fibers might be a source as well. Striated muscle fibers in the lung are commonly associated with major malformations involving heart and lungs, suggesting a much wider morphogenetic error.

Bronchopulmonary Dysplasia↗
PubMed1988

Angiocentric B-cell lymphoma of the lung in an immunocompromised boy.

An angiocentric IgA-kappa bilateral pulmonary lymphoma developed in a 9-year-old boy treated with chemotherapy for 3 1/2 years for acute lymphoblastic leukemia. The tumor was confined to the lungs and had extensive infarction secondary to vascular involvement. This and 2 similar reported cases suggest that compromise of the host's immune system may contribute to the development of pulmonary lymphoma in children, an uncommon site and probably labeled previously as lymphomatoid granulomatosis.

B-Lymphocytes↗
PubMed1988

Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association.

We present a male newborn (weight 4000 g) who died at age 12 days with a clinical history of persistent hypoglycemia and polycythemia. Clinical examination disclosed somatic hemihypertrophy (left side), a large umbilical hernia, macroglossia, and an intraabdominal tumor, consistent with the diagnosis of Beckwith-Wiedemann syndrome (EMG syndrome) and hemihypertrophy. Necropsy findings included visceromegaly (left kidney and adrenal), cytomegaly of the fetal cortex and nodular arrangement of both adrenals, diffuse nesidioblastosis and islet cell hyperplasia of the pancreas, and persistent glomerulogenesis. The tumor was a cystic pancreatoblastoma attached to the anterior surface of the pancreas. Three other examples of this association, congenital pancreatoblastoma and Beckwith-Wiedemann syndrome, all in males, are on record in the literature, indicating a strong relationship between both conditions.

Beckwith-Wiedemann Syndrome↗
PubMed1987

Neuron-specific enolase-positive rosettes in nephroblastoma: a possible diagnostic pitfall in aspiration cytology.

Neuron-specific enolase-positive cells, some arranged in rosettes, were identified in smears obtained in fine-needle aspiration of a left retroperitoneal tumor found in a 3-yr-old boy. Nephrectomy showed a typical nephroblastoma with a prominent blastemic component revealing a positive reaction for neuron-specific enolase in blastemic and tubular components. Neuron-specific, enolase-positive, small malignant cells are not diagnostic of neuroblastoma when coming from a tumor of the retroperitoneum even if rosettes are present.

Biopsy, Needle↗
PubMed1987

Focal giant-cell cardiomyopathy.

A newborn with supraventricular arrhythmia diagnosed during fetal life was found at autopsy to have a small nodule with hypertrophic and multinucleated myocardial cells in the atrial septum, near the atrioventricular node. There were no stigmata of Beckwith-Wiedemann syndrome or tuberous sclerosis. This focal giant-cell cardiomyopathy has been described only once, in an infant with Beckwith-Wiedemann syndrome. It is suggested that these dysplastic myocardial cells may be responsible for the atrial arrhythmia and that this hamartomatous lesion has the potential for malignant transformation.

Arrhythmias, Cardiac↗
PubMed1987

An immunohistochemical study of islet cells with macronuclei in infancy.

The presence of hypertrophic islet cells in infancy as evidenced by nuclear enlargement (2 to 6 times normal size) has been mentioned as a morphological accompaniment of hyperinsulinemic hypoglycemia of infancy. We report an immunohistochemical and semiquantitative study of hypertrophic islet cells in 14 infants with neonatal hypoglycemia (10 with documented persistent neonatal hypoglycemia and 4 with probable persistent neonatal hypoglycemia) and 6 infants born to diabetic mothers (IDM), using an indirect immunoperoxidase methods for the demonstration of insulin, somatostatin, and glucagon. Quantitation of immunoreactivity was performed in each case on 20 hypertrophic cells. Polyploid cells were positive for insulin and somatostatin but negative for glucagon; insulin-positive cells outnumbered somatostatin-positive cells in both groups. As nuclear hypertrophy is considered to be a sign of hyperfunction, our findings are in accordance with the concept that IDM involves reactive beta-cell hypertrophy and similar findings in the pancreases of infants with persistent neonatal hypoglycemia (PNH) suggest a primary dysfunction of their beta cells, too.

Diabetes Mellitus↗
PubMed1987

[Isolated macronodular sarcoidosis].

A particular case of macronodular isolated sarcoidosis with neurological and ocular involvement is presented, associated with pseudo-balding. A revision of the most important aspects of the diagnostic of this multifaceted disease is realized. The benefits of a surgical treatment for the cases of isolated nodes are pointed out.

Abscess↗
PubMed1987

[Antibodies against smooth muscle in the detection and follow-up of the celiac disease patient].

The presence of smooth muscle antibodies (SMA) as determined by indirect immunofluorescence was investigated in the serum of 40 patients, 36 out of which were children. Group I included patients having celiac disease (diagnosed by three biopsies). 7/7 (Group Ia) showed no SMA on gluten-free diet while antibodies were present after challenge. 4/6 (Group Ib) were positive also after challenge (these had not had previous determination). Group II was composed by patients presumed to have celiac disease for they had only one biopsy showing grade IV villous atrophy. 5 patients were of recent diagnosis (group IIa) and showed SMA. 4/5 (Group IIb) who admitted not to had been able to follow the gluten-free diet were also positive for SMA. 6/6 (Group IIc) patients on strict gluten-free diet and showing good clinical response had no detectable SMA. Group III included relatives of patients having celiac disease. 5/6 with SMA showed pathologic small bowel biopsies; only 1/4 with no SMA had mucosal alterations. It is concluded that the presence of serum SMA can be considered a useful marker in the follow-up of cases of celiac disease already diagnosed and to investigate presumed cases of such disease.

Autoantibodies↗
PubMed1986

Congenital fibrosarcoma: a congenital fibrous histiocytoma.

Using immunoperoxidase PAP technique in 2 cases of congenital fibrosarcoma, a great number of cells showed positive stain for alpha-1-antitrypsin (A1AT) and alpha-1-antichymotrypsin (A1ACT), both considered to be good histiocytic markers. The ultrastructure in 1 case also provides evidence of histiocytic differentiation. These findings suggest that congenital fibrosarcomas are actually congenital fibrous histiocytomas and may explain the presence of inflammatory infiltrates as in some malignant fibrous histiocytomas.

Arm↗
PubMed1986

Giant-cell myocarditis in a newborn with congenital herpes simplex virus (HSV) infection: an immunohistochemical study on the origin of the giant cells.

Giant-cell myocarditis is a rare inflammatory disorder characterized by degeneration and necrosis of myocardial fibers and presence of chronic inflammatory infiltrates associated with multinucleated giant cells forming a granulomatous inflammatory reaction. The etiology of giant-cell myocarditis is unknown. Many conditions have been reported as associated with this phenomenon such as fungi, virus, sarcoidosis, and hypersensitivity or autoimmune reactions. We are reporting a case of giant-cell myocarditis discovered in a newborn with congenital herpetic sepsis. The myogenic origin of the giant-cells of this case is supported by the positivity for desmin and myoglobin and negativity for muramidase and alpha-1-antichymotrypsin after immunoperoxidase procedure. The presence of Herpes simplex virus type II was confirmed by indirect immunoperoxidase reaction in most of the viscera including the heart, but is not considered a factor in the production of giant cells.

Female↗