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Biomedical subjects

E N Faerber

Publications and source records attributed to E N Faerber.

At least 19 recordsLinked to original sources
PubMed1997

A newly recognized cause of wheezing: AIDS-related bronchial leiomyomas.

We present two human immunodeficiency virus-infected children who developed wheezing and radiological evidence of pulmonary air trapping due to intra- and peribronchial leiomyomas. At autopsy, leiomyomas were also found in their spleens, which to our knowledge, has never been reported. The smooth muscle tumors were strongly positive for the Epstein-Barr virus, as demonstrated by in situ hybridization to Epstein-Barr virus-encoded ribonucleic acid, confirming the findings of recent investigators and linking these tumors to the Epstein-Barr virus.

Acquired Immunodeficiency Syndrome↗
PubMed1997

Central nervous system tumors of childhood.

Primary tumors of the central nervous system are the most common solid tumors of childhood. Although numerous tumors of the brain and spine are common to both pediatric and adult population groups, there are many tumors unique to infancy and childhood. This article describes the characteristic locations, clinical presentations, and imaging features of these tumors.

Adult↗
PubMed1995

Coarctation of the aorta: diagnostic imaging after corrective surgery.

The clinical evaluation and management of the patient with coarctation of the aorta continues to evolve. Traditional imaging evaluation by plain film chest radiography, barium esophagography, and arteriography with pressure measurements across the coarctation has been largely supplanted by Doppler echocardiography and magnetic resonance imaging (MRI). The complications of surgery and balloon angioplasty, including residual or recurrent coarctation and aneurysm, can also be evaluated noninvasively by echocardiography and MRI. Chest radiography continues to play an important role in "first discovery" imaging in asymptomatic patients.

Aortic Coarctation↗
PubMed1995

Tetralogy of Fallot: diagnostic imaging after palliative and corrective surgery.

Tetralogy of Fallot was invariably fatal until the development of palliative and later corrective surgical procedures. The prognosis for children with tetralogy of Fallot continues to improve almost a half century after the earliest palliative surgical procedure was performed successfully. Imaging of the child and adult after surgery for tetralogy of Fallot remains an important challenge because surgical complications or limitations frequently require imaging for complete evaluation and further management of the patient. Traditional imaging by chest radiography and arteriography has been largely replaced by echocardiography and ultrafast and conventional CT, as well as magnetic resonance imaging. This article reviews those aspects of diagnostic imaging that are appropriate to study the postoperative chest in the child or adult with tetralogy of Fallot.

Diagnostic Imaging↗
PubMed1994

Cerebral syrinx with Chiari I malformation.

A 13-year-old female presented with acute left gaze paralysis. MRI revealed hydrosyringomyelia (HSM) with syringobulbia in the left pons extending cephalad into the centrum semiovale. A suboccipital craniotomy was performed and the foramen of Magendie was imperforate. Ocular movements normalized in 2 months and postoperative MRI confirmed resolution of the cerebral syrinx and syringobulbia and diminished HSM. This case represents an extreme example of the altered spinal fluid dynamics with Chiari I malformation. Theories on HSM are reviewed.

Adult↗
PubMed1994

Sedation of difficult-to-sedate children undergoing MR imaging: value of thioridazine as an adjunct to chloral hydrate.

OBJECTIVE: The purpose of this prospective study was to evaluate the safety and efficacy of thioridazine as an adjunct to chloral hydrate sedation when children undergoing MR imaging are difficult to sedate. SUBJECTS AND METHODS: All 87 children in the study either could not be sedated with chloral hydrate alone or were mentally retarded. Thioridazine (2-4 mg/kg) was administered orally 2 hr before and chloral hydrate (50-100 mg/kg) was administered orally 30 min before the 104 MR examinations. All children were monitored by continuous pulse oximetry. All images were individually evaluated by pediatric radiologists and were graded acceptable if they contained only minimal motion artifact or no motion artifact. Studies were considered successful only when 95% or more of the images were acceptable. RESULTS: MR imaging was successful in 93 (89%) of 104 examinations. The success rate for children entered into the study because of prior failure of chloral hydrate sedation was not significantly different from the success rate for children with mental retardation. A tendency for increasing failure rate with age was not significant. No serious complications occurred during the study. The most common adverse reaction, transient reduced oxygen saturation, was seen in five children. Other adverse effects encountered were vomiting in four children, hyperactivity in two children, transient tachycardia in one child, and prolonged sedation in one child. No child required hospitalization because of an adverse reaction to sedation. CONCLUSION: The study indicates that thioridazine is a safe and effective adjunct to chloral hydrate when a child undergoing MR imaging is difficult to sedate.

Administration, Oral↗
PubMed1994

Undifferentiated (embryonal) sarcoma arising in conjunction with mesenchymal hamartoma of the liver.

The histogenesis of the undifferentiated (embryonal) sarcoma of the liver has been much debated. Originally, some investigators suggested that the tumor could originate from a mesenchymal hamartoma. Others doubted this hypothesis. The demonstration of alpha-1-antitrypsin in some tumors and of cytokeratins in others gave rise to new theories suggesting that the tumor was either a fibrous histiocytoma or a sarcomatoid hepatocellular carcinoma. As a new development in this search for the genesis of the undifferentiated (embryonal) sarcoma of the liver, this report describes a case that seems to substantiate, for the first time, the original hypothesis histogenetically linking the undifferentiated (embryonal) sarcoma and the mesenchymal hamartoma. The case is that of a 12-yr-old girl who developed an undifferentiated (embryonal) sarcoma of the liver in conjunction with a mesenchymal hamartoma. Furthermore, in places, the tumor exhibited striking epithelial differentiation as well as immunoreactivity for cytokeratins, alpha-1-antitrypsin and vimentin. Immunoreactivity for alpha-fetoprotein was limited to the areas of epithelial differentiation.

Child↗
PubMed1993

Technetium 99mTc-HMPAO SPECT in children and adolescents with neurologic disorders.

We evaluated regional cerebral blood flow with technetium 99mTc hexamethylpropyleneamineoxime single photon emission computed tomography (SPECT) in 20 children and adolescents with neurologic dysfunction of varied etiology and abnormal electroencephalograms (EEGs). All patients were also examined with computed tomography (CT) and magnetic resonance imaging (MRI). Abnormal perfusion was found in 17 (85%) of 20 SPECT scans. Abnormal CT or MRI scans were noted in nine (45%) and in 10 (50%) of 20 cases, respectively. In eight (73%) of 11 cases with normal CT scans and in seven (70%) of 10 with normal MRI scans, the SPECT scan was abnormal. Abnormal regional cerebral blood flow on SPECT scans correlated better with EEG abnormalities than with neurologic examination or CT or MRI scan findings. We conclude that in children and adolescents with a spectrum of neurologic diseases and abnormal EEGs, abnormalities of brain structure or function are more likely to be documented by SPECT than by CT or MRI scans. SPECT findings correlate well with the location and type of EEG abnormality.

Adolescent↗
PubMed1993

High-dose chloral hydrate sedation for children undergoing MR imaging: safety and efficacy in relation to age.

OBJECTIVE: Sedation is frequently essential for successful MR imaging, and chloral hydrate is the most commonly used drug for this purpose in infants and children. Our experience with these patients suggested that this sedative is less effective in older children, even when administered in high doses. However, no prospective study comparing the efficacy of chloral hydrate sedation for children of different ages undergoing MR imaging has been reported. Accordingly, we performed a study to evaluate the effectiveness and safety of chloral hydrate sedation in children of various ages. SUBJECTS AND METHODS: The study included 300 infants and children, 1 month to 11 years old (mean, 3 years), who were given oral chloral hydrate, 100 mg/kg, for sedation before MR imaging. The maximum total dose administered was 2.5 g, which limited the study to children who weighed 25 kg or less. Sedation was considered successful when MR studies were completed and at least 95% of the images had little or no motion artifact. RESULTS: Sedation was successful in 273 (91%) of 300 children. It was unsuccessful in nine of the 203 children who were 48 months old or younger (96% success rate) and in 18 of the 97 children who were more than 48 months old (81% success rate). A single-tailed t-test showed that the children in whom sedation was unsuccessful were significantly older than those in whom it was successful to the .0005 level of significance. The failure rate increased steadily for children more than 48 months old. Several failures may also have resulted from lengthy examination times. Adverse reactions to chloral hydrate sedation included hyperactivity (6%), vomiting (4%), and mild respiratory depression (4%). No adverse reaction was severe enough to require hospitalization. CONCLUSION: The higher failure rate for chloral hydrate sedation in children more than 48 months old suggests that the patient's age is an important limitation to the usefulness of chloral hydrate sedation for children undergoing MR imaging. However, the low rate of adverse reactions makes chloral hydrate a safe drug for sedation of children undergoing MR imaging.

Administration, Oral↗
PubMed1991

Benign hemorrhagic adrenocortical macrocysts in Beckwith-Wiedemann syndrome.

A new pseudotumorous lesion found in the adrenal cortex of six infants with Beckwith-Wiedemann syndrome is described. These cystic masses were discovered either prenatally by using sonography or early in the neonatal period as palpable flank masses. Imaging studies, including sonography and CT, could not confidently exclude malignancy. After the masses were removed surgically, histologic examination showed them all to be benign hemorrhagic macrocysts within the capsule or permanent cortex (in contrast to neonatal adrenal hemorrhage, which usually occurs more centrally in the fetal cortex). The cysts were as large as 8 cm in diameter, and in one case a solitary cyst was predominant. Hemihypertrophy was present in all cases. Four of the six lesions were right-sided, and there was a male-female ratio of 5:1. Benign hemorrhagic adrenocortical macrocysts are a cause of abdominal mass in the fetus and neonate with Beckwith-Wiedemann syndrome.

Adrenal Cortex Diseases↗
PubMed1990

Desmoplastic cerebral astrocytoma of infancy. Light microscopy, immunocytochemistry, and ultrastructure.

The first detailed ultrastructural study of a superficial desmoplastic cerebral astrocytoma of infancy is reported. This is a neoplasm which mimics a mesenchymal tumor, originally described in 1984 under the name of "superficial cerebral astrocytoma attached to dura." This tumor, which is believed to have a good prognosis, was resected from the frontoparietal region of a 6.5-month-old girl, in whom it had presented as a large densely enhancing vascular and cystic mass. The ultrastructure of the tumor appeared to be distinctive, characterized by the absence of neuronal elements, and the presence of large amounts of redundant and sometimes extensively duplicated basal laminal material and collagen between nonpleomorphic and nonlipidized astrocytes, corresponding to the reticulin fibers seen by light microscopic analysis between the S-100 protein and glial fibrillary acidic protein (GFAP)-positive cells. The features of the tumor, its differential diagnosis, and its relationship to other pediatric supratentorial tumors are discussed.

Astrocytoma↗
PubMed1990

Subacute necrotizing encephalomyelopathy (Leigh disease): CT and MRI appearances.

Subacute necrotizing encephalomyelopathy (SNE) is a genetic disorder of pyruvate metabolism. Until recently the diagnosis of SNE could only be made at autopsy. However, an antemortem diagnosis can now be suggested by the correlation of clinical and laboratory data with computed tomography (CT) and/or magnetic resonance imaging (MRI). Five children with clinical and laboratory data suggesting the diagnosis of Leigh disease were evaluated by CT and MR. MR was found to be more sensitive than CT in the detection of areas of necrosis in the brain of the five children we studied. The absence of focal lesions detected by either modality in one of our patients did not exclude the diagnosis of SNE since focal lesions were present at autopsy one month following CT and MR.

Caudate Nucleus↗
PubMed1990

Thymolipoma: computed tomographic appearances.

The computed tomographic (CT) appearances of a thymolipoma are described in a 5 1/2-year-old girl. The location and CT appearances of the tumor appear to be specific for the diagnosis of thymolipoma.

Child, Preschool↗