Susceptibility of B-cell lymphoma to human antibodies encoded by the V4-34 gene.

Our previous studies have shown that mAbs derived from the human V4-34 gene bind and kill human B-lymphocytes via membrane disruption. This study demonstrates the cytotoxicity of two V4-34 encoded mAbs, 216 and Z2D2, towards human B-cell lymphoma. In vitro, 216 and Z2D2 are cytotoxic to a variety of B-cell lymphomas obtained from patient biopsies. In vivo, increased survival was observed with both mAbs in a lymphoma model developed in scid mice with human B-cell line Nalm-6. Studies in mice show that these mAbs are well tolerated with minimum side effects. Since 216 and Z2D2 show increased toxicity towards cycling cells, V4-34 mAb-based therapy can be additive with drugs that block cell-cycle progression. Stem cells that are V4-34 mAb ligand negative would not be depleted. Together, these studies recommend an evaluation of the two completely human mAbs in a phase I trial for B-cell lymphoma.

Naumoff short-rib polydactyly syndrome compounded with Mohr oral-facial-digital syndrome.

A stillborn baby boy had findings of severe constitutional dwarfism with short limbs, short ribs, and polydactyly that were consistent with Naumoff (type III) short-rib polydactyly syndrome. He also had additional congenital anomalies, including cleft palate, notching of the upper lip, small tongue with accessory sublingual tissue. These oral and pharyngeal anomalies were consistent with Mohr (type II) oral-facial-digital syndrome. We suggest the stillborn infant represented a compound of Naumoff short-rib polydactyly syndrome (SRPS-III) and Mohr oral-facial-digital syndrome (OFDS-II).

Asynchronous asymmetric form of heterogeneous osteopetrosis: initial case expanded and a new case.

We have discovered additional serial radiographs and clinical information on the initial case of "regional osteopetrosis tarda" that has been included in several editions of Caffey's Pediatric X-Ray Diagnosis. A definite second case was found after a search of radiology teaching files of other selected medical centers and the International Skeletal Dysplasia Registry. Analysis of the sequential unusual radiographic findings of the initial case and the equivalent compelling findings of the second case justifies renewed attention to an asynchronous asymmetric form of heterogeneous osteopetrosis.

Brucellosis: unusual presentations in two adolescent boys.

Two boys presented with variable signs and symptoms of infectious disease that challenged diagnosis. One of the two patients had aortic valve vegetations and lower extremity aneurysms, and the other had calvarial osteomyelitis, epidural abscess, pleural effusions, and pulmonary nodules. Only after a battery of bacterial and fungal agglutination tests was the unsuspected diagnosis made in each of brucellosis from Brucella canis.

Scoliosis circa 2000: radiologic imaging perspective. I. Diagnosis and pretreatment evaluation.

Plain film imaging remains important for the diagnosis and surveillance of scoliosis, as well as for the detection of complications after surgery. Advances in CT and MR imaging have greatly improved the ability to detect or confirming non-idiopathic causes of scoliosis, including abnormalities within the spinal canal. Three-dimensional thinking has become more important in evaluating and understanding scoliosis.

Diffuse neonatal gastrointestinal hemangiomatosis: CT findings.

A 3-week-old boy presenting with a cutaneous hemangioma and gastrointestinal bleeding was found to have gastrointestinal hemangiomatosis involving the entire small bowel diagnosed by exploratory laparotomy. We present the striking, diffuse enhancement of the small bowel wall in this unusual disorder as demonstrated by dynamic contrast-enhanced computed tomography after the bowel was distended with non-radiopaque material.

Magnetic resonance imaging of a left circumflex aortic arch and aberrant right subclavian artery: the other vascular ring.

We present a case of a rare congenital cardiac anomaly. Magnetic resonance imaging accurately demonstrated a left circumflex aortic arch. This finding was not apparent on a prior conventional angiogram or echocardiography. Magnetic resonance imaging documentation of this anomaly is uncommon. Review of the embryonic development, clinical presentation of complete and incomplete vascular rings, and additional associated cardiac anomalies are discussed. This is one of only a few reported cases of a left circumflex aortic arch.

Diagnostic imaging of ventriculoperitoneal shunt malfunctions and complications.

Most pediatric patients with hydrocephalus are treated with ventriculoperitoneal (VP) shunt placement. However, shunt malfunction is common and is usually caused by mechanical failure. Shunt obstructions may be confirmed with radioisotope examination or with fluoroscopically guided injection of iodinated contrast material into the shunt reservoir. Disconnections or breaks are more readily detected at radiography in cases in which barium-impregnated shunt tubing was used. Migration and leakage may also occur. Cerebrospinal pseudocysts may be demonstrated with plain radiography and further evaluated with computed tomography (CT) and sonography. In increasing hydrocephalus, plain radiography may reveal sutural diastasis and increased cranial cavity size, and CT can be used to evaluate ventricle size. In cases of enlarging intracranial cysts, injection of iodinated contrast material followed by CT can help document a connection between the cyst and the ventricles. Ventriculitis and meningitis can be visualized at CT and magnetic resonance imaging as enhancement of the ventricular ependymal lining or cerebral cortical sulci. Other complications associated with VP shunts include surgery-related complications, shunt overdrainage and slit-ventricle syndrome, neoplastic metastasis, pleural effusion, and complications related to shunt variants. Imaging analysis is an essential adjunct to the clinical evaluation of patients with suspected VP shunt malfunctions or complications. Radiologists should be familiar with these potential problems and the diagnostic utility of various imaging modalities.

Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases.

OBJECTIVE: Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material. DESIGN AND PATIENTS: Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion. RESULTS: Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial 'button' sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet. CONCLUSIONS: Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis.

Scoliosis circa 2000: radiologic imaging perspective. II. Treatment and follow-up.

Plain film imaging remains important for the diagnosis and surveillance of scoliosis, as well as for the detection of complications after surgery. New means of treating scoliosis have become established and should be understood by the radiologist. To the well-known postoperative complications, including pneumothorax, pneumonia, and gastrointestinal obstruction, are added new specific potential problems with the new surgical methodology.

Detection of infectious bursal disease virus by reverse transcription-polymerase chain reaction amplification of the virus segment A gene.

A reverse transcription-polymerase chain reaction (RT-PCR) amplification assay was developed to detect infectious bursal disease virus (IBDV) gene sequences in clinical samples, infected cell cultures and chicken embryos. Two pairs of primers were designed to amplify the 5'- and 3'-termini of segment A genes that partially code for the IBDV proteins VP2 and VP3, respectively. One primer pair specifies a 309-bp fragment, the other a 520-bp fragment. Direct RT-PCR analysis of 5 bursal samples of chickens derived from a suspected first outbreak of infectious bursal disease in New Zealand yielded the 309-bp and 520-bp by fragments. The identity of both amplified fragments was confirmed by restriction endonuclease analysis, chemiluminescence Southern blot hybridization and direct cycle sequencing. RT-PCR amplification of RNAs extracted from 4 out of 5 IBDV isolates propagated in Vero cells, chicken embryo fibroblasts and specific pathogen-free chicken embryos yielded IBDV-specific fragments of unpredicted small sizes.

Polymerase chain reaction amplification of wildebeest-associated and cervine-derived malignant catarrhal fever virus DNA.

A polymerase chain reaction (PCR) assay was developed for the detection of alcelaphine herpesvirus 1 (AHV1), a causative agent of malignant catarrhal fever (MCF) of ruminants. A pair of 20-base primers was constructed based on the published nucleotide sequence of gene A of the WC11 isolate of AHV1 and was used to amplify a DNA fragment of 413 base pairs. The optimised PCR assay was highly sensitive, i.e. it detected 10 fg of genomic DNA of AHV1 (WC11 isolate). The amplified fragment was shown to be specific for AHV1 DNA by (i) cleavage with XbaI which yielded 2 subfragments of approximately 140 and 280 base pairs and (ii) chemiluminescence Southern blot hybridisation with a digoxigenin-labelled 25-base internal probe. The PCR assay also amplified AHV1 gene sequences in tissue samples from deer and rabbits experimentally infected with materials derived from deer with clinical sheep-associated MCF.

Pulmonary edema complicating upper airway obstruction in infants and children.

The association of pulmonary edema with upper airway obstruction occurs in three clinical settings: acute and chronic upper airway obstruction and immediately after the relief of acute upper airway obstruction. Iatrogenic causes, such as adenotonsillectomy and tracheal intubation, were the most frequently encountered in the authors' series of 21 pediatric patients with such an association. The pathogenesis of this kind of pulmonary edema is multifactorial. The application of moderate continuous positive airway pressure in conjunction with the administration of diuretics rapidly clears pulmonary edema in all three clinical settings, usually within 24 hours, and can probably prevent pulmonary edema immediately after acute obstruction is relieved.

A computerized test battery for the assessment of cardiovascular reactivity.

Recent evidence has suggested a possible relationship between the tendency to exhibit excessive cardiovascular reactions during psychological challenge and the risk of cardiovascular disease. Valid techniques for reliably assessing such reactions are, however, minimally available. A test battery for the assessment of cardiovascular reactivity to experimental challenge is currently being developed at the University of Pittsburgh in conjunction with the University of Miami and Duke University. An IBM-AT compatible microcomputer is being used for the concurrent collection of physiological data and presentation of the laboratory stressors. Digitized cardiovascular data being collected include EKG, Impedance Cardiogram (ICG), phonocardiogram, and a peripheral pulse wave measure. Blood pressure readings are also being collected and stored on disk. The computer presents three challenging video games, each designed to elicit cardiovascular reactions. Processing programs are being used and developed for the standardized scoring of the digitized signals. To assist in epidemiological research a mobile testing unit has been assembled for the easy administration of the test battery in varying geographical locations. The test battery being developed will increase the feasibility of epidemiological and clinical assessment of stress-induced cardiovascular responses which may substantiate a link between reactivity and cardiovascular disease.

Increased renal parenchymal echogenicity: causes in pediatric patients.

The authors discuss some of the diseases that cause increased echogenicity of the renal parenchyma on sonograms in children. The illustrated cases include patients with more common diseases, such as nephrotic syndrome and glomerulonephritis, and those with rarer diseases, such as oculocerebrorenal syndrome. Hyperechogenicity is a nonspecific finding but a significant one in that it suggests the presence of renal abnormalities. When it is demonstrated, further investigation is usually warranted.

Effectiveness of abdominal radiographs in visualizing chewable iron supplements following overdose.

The purpose of this study was to determine the clinical effectiveness of visualizing chewable and nonchewable iron supplements remaining in the gastrointestinal tract following an overdose. A 5-year retrospective review of 93 pediatric patients who had ingested potentially toxic amounts of an iron supplement found that 58% had abdominal radiographs taken. Fifty-five percent of these patients had ingested chewable multiple vitamins with iron and 40% had ingested nonchewable tablets. After radiographic review, radiopaque densities could be marginally visualized in only one case of ingestion of a chewable iron supplement. The mean serum iron level of patients who had ingested a chewable form of iron was 270 micrograms/dL. An in vitro study was performed to determine the radiopacity of various chewable multiple vitamins with iron. It was concluded that although chewable multiple vitamins with iron are radiopaque in vitro, clinical radiographic visualization is unlikely.