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Biomedical subjects

H Isaacs

Publications and source records attributed to H Isaacs.

162 records · Page 9Linked to original sources
PubMedDate not supplied

Fetal and neonatal cardiac tumors.

Primary tumors of the heart are uncommon in the fetus and neonate. Nevertheless, the widespread use of new imaging techniques has contributed significantly to earlier diagnosis, treatment, and thus improved survival. The clinical findings, imaging studies, pathology, and outcome of 224 fetuses and neonates with cardiac tumors collected from the literature are evaluated and discussed. Most tumors are benign, and of these rhabdomyoma is the most common, followed by teratoma, fibroma, oncocytic cardiomyopathy, vascular tumors, and myxoma. Malignant and metastatic tumors are described but are rare. Murmurs, arrhythmias, cyanosis, respiratory distress, and cardiac failure are the main presenting signs of cardiac tumors in the perinatal period. Disturbances in hemodynamic function are correlated with the size and location of the tumor. Cardiac vascular tumors have the best outcome, whereas malignant tumors have the worst. The purpose of this review is to concentrate on the fetus and neonate in an attempt to determine the various ways cardiac tumors differ clinically and morphologically in this age group from those occurring in older children and adults and to show that certain types of tumors have a better prognosis than others.

Diagnostic Imaging↗
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Otolaryngological manifestations of arthrogryposis multiplex congenita.

The important otolaryngological manifestations of arthrogryposis multiplex congenita (AMC), which heretofore have not been described in otolaryngological and other specialty journals, are reviewed. Thirty-seven patients with AMC were studied. Nine of these patients with neurogenic AMC, had otolaryngological manifestations. Six of these had Pierre Robin-like syndromes. Seven patients had severe dysphagia and aspiration pneumonitis. Five patients had voice changes, and three of these required tracheotomy to relieve laryngeal obstruction and prevent aspiration. Two of these patients were aphonic and had laryngeal paralysis. Three of the nine patients who had laryngeal examination showed supraglottic narrowing similar to laryngomalacia. Six patients with otolaryngological problems were autopsied, and two had pharyngeal biopsies. The histological examination demonstrated normal laryngeal and pharyngeal musculature. The cricoarytenoid joints were found normal in all the larynges examined. Central nervous system dysfunction rather than simple muscle weakness, caused the dysphagia and respiratroy difficulties in patients with neurogenic AMC. Dysphagia with aspiration was the single most important cause of death in approximately 20% of patients with AMC. A tracheotomy and feeding gastrostomy appears essential for successful management of the otolaryngological complications of AMC.

Airway Obstruction↗
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Fibrous histiocytoma of the trachea.

Intratracheal tumors are rare in children. A case is reported of a two-year-old female child with a fibrous histiocytoma of the trachea which caused severe respiratory obstruction and persistent right middle lobe pneumonia. Fibrous histiocytomas are basically benign tumors of histiocytes and have been mistaken for other tumors such as fibroma, sclerosing hemangioma, neurilemoma, fibromatosis, hemangiopericytoma, fibrosarcoma, and some primitive sarcomas. Fibrous histiocytomas have not been reported in the trachea or bronchi. The patient was treated with endoscopic removal of the tumor, and has remained free of disease to this date.

Child, Preschool↗
PubMedDate not supplied

Lymphomatoid granulomatosis in a child with acute lymphatic leukemia in remission.

Lymphomatoid granulomatosis, a tumor-like process of unknown etiology, produced progressively destructive disease of the larynx, trachea and bronchi in an eight-year-old girl with acute lymphoblastic leukemia of five years duration. The leukemia had been in remission for 4 1/2 years at the clinical onset of the lymphomatoid granulomatosis. Whether this occurrence suggests that lymphomatoid granulomatosis is a type of neoplasm, or is associated with immunologic depression, cannot be stated. Fortunately rare, and of poor prognosis, the disorder may become more frequent with improved survival rate of patients with leukemia and other neoplasms.

Child, Preschool↗
PubMedDate not supplied

Solitary plasmacytoma of the larynx and upper trachea associated with systemic lupus erythematosus.

Solitary plasmacytoma rarely involves the larynx and trachea. This tumor caused severe laryngeal and tracheal obstruction in an adolescent female with systemic lupus erythematosus of nine years' duration. Her systemic disease had been in remission at the time she developed symptoms of laryngeal and tracheal obstruction by plasmacytoma invasion. A complete survey failed to reveal any other focus of tumor. Because steroid therapy failed to clear the plasma cell infiltrate, she was treated with irradiation after the tumor was surgically debulked endoscopically. Postirradiation biopsy showed no evidence of tumor, but the patient continued to experience moderate airway obstruction from fibrosis in the irradiated trachea. The patient has been followed, and is free of disease nine years after establishing the diagnosis.

Airway Obstruction↗
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Wegener's granulomatosis causing laryngeal and tracheobronchial obstruction in an adolescent girl.

A 12-year-old girl presenting with a painful neck mass and ulcerated gingival lesions was found to have Wegener's granulomatosis involving the gingivae, paranasal sinuses, ears, mastoids, larynx, trachea, bronchial tree, lungs, kidneys and joints. The initial illness and two subsequent exacerbations were treated with intravenous and oral cyclophosphamide and prednisone. Secondary infections have been managed with establishment of good drainage and use of appropriate antibiotics. The patient is well with a normal sedimentation rate 4 1/2 years after the onset of her disease.

Airway Obstruction↗
PubMedDate not supplied

Primary lymphosarcoma of the larynx in a child.

A 4 7/12-year-old Caucasian female with a history of "croup-like symptoms" and persistent airway obstruction, was found to have a primary lymphosarcoma by biopsy at the time of laryngoscopy and bronchoscopy. No metastatic disease was found. After an induction course of vincristine, prednisone and local irradiation, she received CNS prophylaxis with intrathecal methotrexate and cranial irradiation. Maintenance therapy, administered over a 2 3/4 year period, consisted of cyclophosphamide, methotrexate, and 6-mercaptopurine. Excluding the diagnostic evaluation, she was hospitalized only once for the management of suspected sepsis, gastrointestinal ulceration and severe bone marrow depression. Since discontinuing treatment 27 months ago, she has remained free of disease.

Child, Preschool↗
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Epidermolysis bullosa associated with laryngeal stenosis.

A boy with epidermolysis bullosa developed progressive laryngeal obstruction after age six months, with tracheotomy necessary at age three years. By eight years, the larynx was completely occluded by web and scar. Stenosis recurred repeatedly over the next seven years, three to six months after each corrective procedure, but laryngeal patency has been better-maintained since age 15. Laryngeal stenosis, presumably secondary to epithelial detachment, although a rare complication of epidermolysis bullosa, can be serious and life-threatening.

Child↗
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Neurofibroma of the larynx in a child.

Laryngeal obstruction due to neurofibromatosis in a girl with von Recklinghausen's disease first occurred in the neonatal period, and recurred by age seven years. She has done well for four years following laryngofissure surgery. Pharyngeal and laryngeal neurofibromatosis are rare manifestations of von Recklinghausen's disease. The dominant hereditary pattern of this disease requires that the condition be considered when respiratory distress occurs in the child of an affected parent, whether or not skin tumors or other features are present.

Child, Preschool↗
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Malignant transformation of tracheobronchial juvenile papillomatosis without prior radiotherapy.

Squamous cell carcinoma of the trachea and bronchi developed in a 19-year-old male with recurrent laryngeal papillomata since age four, who had received no prior radiotherapy, but who was tracheotomized for obstructive laryngeal papillomata. Treatment with intravenous cytosine arabinoside, "moderate dose" methotrexate, topical 5-fluorouracil and irradiation failed to alter tumor growth, and the patient succumbed to recurrent arterial tumor emboli.

Adult↗
PubMedDate not supplied

In vitro and in vivo preclinical chemotherapy studies of human neuroblastoma.

Two human neuroblastoma cell lines, LA-N-1 and SK-N-MC growing in vitro and as subcutaneous tumors in athymic nude mice, were evaluated for their sensitivity to cyclophosphamide, doxorubicin (Adriamycin), and vincristine. In vitro, cyclophosphamide, following liver S-9 metabolic activation, and vincristine were significantly more cytotoxic to SK-N-MC than to LA-N-1 cells; doxorubicin was equally cytotoxic to both cell types. Treatment of nude mice bearing LA-N-1 and SK-N-MC tumors with cyclophosphamide and vincristine produced significant reduction (> 50%) in SK-N-MC tumor weights but not in LA-N-1 tumor weights. Doxorubicin failed to produce significant reduction in the weight of either the LA-N-1 or the SK-N-MC tumor. These sensitivities were generally similar to the clinical response of the tumors to these same agents. Such an in vitro and in vivo system using these and other neuroblastoma cell lines may provide a preclinical model for evaluating the activity of chemotherapeutic agents against human neuroblastoma.

Animals↗