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Biomedical subjects

R L Wesenberg

Publications and source records attributed to R L Wesenberg.

At least 19 recordsLinked to original sources

Syndrome of multiple epiphyseal dysplasia (ribbing type) with rhizomelic shortness, cleft palate, and micrognathia in two unrelated patients.

We report on two unrelated patients with an apparently new syndrome. In each family they are the only affected members, their parents are not consanguineous, and paternal and maternal ages are not advanced. At birth each patient was noted to have a marked Robin phenotype (cleft of the secondary palate and micrognathia) plus rhizomelic shortness. Delay in the appearance of long bone epiphyses was noted and followed by small fragmented and later very flat epiphyses of all long bones. The fibulae are short and radial heads dislocated. Scoliosis and marked genu valgum developed in both. Both patients have normal intelligence, vision, and hearing. Both have mildly upward slanting palpebral fissures, broad nasal tip, and apparent hypertelorism.

Adult↗

Acute scrotal abnormalities in children: diagnosis by combined sonography and scintigraphy.

Both scintigraphy and real-time sonography have been used to assess acute symptoms involving the scrotum. However, because of its high sensitivity and ability to document physiologic abnormalities, scintigraphy has been the procedure of choice. Scintigraphy, however, lacks specificity; its value lies mainly in serving to distinguish torsion from nontorsion. The purpose of this study was to supplement scrotal scintigraphy with sonography to determine if the combination improves diagnosis and management compared with scintigraphy alone. Forty-three scrotal scintigrams and sonograms were performed on 40 consecutive patients with acute scrotal symptoms. The interpretation of the scintigram was altered by sonography in six (14%) of the combined scans, directly affecting clinical management. In three patients with acute hydroceles diagnosed by sonography, exploratory surgery was avoided despite scintigraphic findings suggesting testicular torsion. Scintigraphy was normal in two patients with spontaneous testicular detorsion, whereas sonography showed recent spermatic-cord torsion that required subsequent orchiopexy. In a patient with epididymitis and orchitis, sonography showed a complicating scrotal abscess, which was not apparent on scintigraphy and which required antibiotic treatment. The addition of sonography to the scintigraphic evaluations of children with acute scrotal abnormalities changed the diagnosis and clinical management in 14% of the patients studied.

Acute Disease↗

Neuroblastoma: a specific sonographic tissue pattern.

Previous attempts to determine a sonographic appearance characteristic of neuroblastoma have had diverse results. Sonograms of 53 abdominal tumors, including 10 neuroblastomas, imaged during 1982-1986 were reviewed. Four of the patients with neuroblastoma had a distinctive sonographic "lobule" of increased echogenicity in a part of the larger tumor mass. This sonographic appearance was secondary to the growth pattern of the tumor and was not cell specific. Correlative CT scans in two of the four patients did not differentiate this lobule. Histologically, the lobule was an aggregate of uniform neuroblastoma cells (marginated by reticulin and collagen) without hemorrhage, necrosis, or calcification. This tissue pattern was not seen in any of the other 43 neoplasms, including 12 Wilms' tumors. When identified sonographically, the lobule identified in this study seems specific for neuroblastoma and is a valuable diagnostic sign in children with an abdominal mass.

Abdominal Neoplasms↗

Ultra-low-dose routine pediatric radiography utilizing a rare-earth filter.

Clinical evaluation of the usefulness of primary beam filtration with erbium to achieve marked reductions in radiation exposure and dosage was undertaken. We evaluated 250 routine pediatric radiographic and 100 contrast examinations. An additional 52,727 radiographic and 4745 fluoroscopic examinations were analyzed prospectively by evaluation of subjective film quality. Radiation exposure and dosage reductions ranged from 57% at 50 kVp to 36% at 80 kVp and 39% at 120 kVp. These reductions were obtained in addition to those previously attained by using a 600-speed gadolinium oxysulphide screen/film system (Trimax 12, XDL). The erbium filter requires that the tube current be increased to approximately double that without the filter (in the most commonly used range of 60-100 kVp). However, tube loading is already so low with the 600 system that this effect on wear is considered inconsequential. This ultra-low-dose rare-earth beam filter and screen/film combination results in films with no demonstrable loss of overall quality, plus a subjective slight improvement in contrast definition for iodine and barium contrast studies. The addition of erbium foil beam filtration to existing rare-earth screen/film technology results in what is currently the ultimate in low-dose conventional radiographic imaging.

Child↗

Ultrasonography and the impalpable testis.

The accuracy of inguinal ultrasonography in the pre-operative localization of the maldescended testis was assessed in 51 patients with 57 maldescended testes. Pre-operative sonographic results were compared with the clinical assessment and the operative findings. Testes located in the inguinal canal or at the internal ring, which were occasionally impalpable, were readily demonstrated by sonography. The management of children with an impalpable maldescended testis should include a sonographic examination as the investigative procedure of choice.

Adolescent↗

CAMPS: computer-automated metacarpophalangeal profile system.

The metacarpophalangeal profile (MCP) pattern has been proven useful in describing individuals with genetic and nongenetic syndromes. However, the measurement of the 19 bone lengths is a tedious procedure requiring use of hand vernier calipers, detailed normative data to be looked up in extensive tables, hand calculator, and manual graphing techniques. Presently there are no reports of microcomputer-automated systems for the accurate measurement, recording, analysis, and graphing of MCP profiles. We describe a computer-automated metacarpophalangeal profile system (CAMPS) that will assist in the derivation of the MCP profile. This program allows the user to select different program routines that perform the functions necessary for MCP profile construction. The "data acquisition module" (DAM) assists in bone length measurement from contact prints of hand radiographs and stores the 19 measurements on a floppy disk. The "standardization analysis module" (SAM) then compares the 19 measurements to age- and sex-matched normal data and converts the raw data to z-score values. The "Pearson product-moment correlation module" (PPM) generates a correlation coefficient describing the degree of similarity between the two hands measured and graphically illustrates the resulting scatterplot. The "MCP plotting module" (MCPM) provides a graphic plot of the 19 bones in either transverse rows or phalangeal rays on a dot-matrix printer or X-Y plotter.

Bone and Bones↗

Morquio syndrome (MPS IVA) and hypophosphatasia in a Hutterite kindred.

A patient is described who has Morquio syndrome (MPS IVA). He is a member of the Hutterite Brethren and genealogic analysis discloses a high inbreeding coefficient for the proband. The proband's sibship is segregating two autosomal recessive disorders, ie, MPS IVA and infantile hypophosphatasia. Two other families each have one or the other of these diseases but not both. The three families are distantly related.

Cartilage↗

A computerized X-ray dose-monitoring system.

An x-ray dose-monitoring system using a small digital computer is described. Initially, and for every 6 months afterward, the system is calibrated using an exposure meter. For each exposure, the computer receives values of x-ray technique and beam geometry from the x-ray generator through a specially designed electronic interface. Then, by means of calibration data, entrance exposure, area exposure product, and integral dose are obtained and printed for each patient examined. The overall accuracy of the system is better than +/- 20%. Operation is semiautomatic, requiring minimum operator intervention. Over 2,000 patients have been monitored with the device. Because the system is computer-based, it offers the opportunity for statistical analysis of the data base created, as the results for each patient are stored on computer disk.

Calibration↗

Fluoroscopy in children: low-exposure technology.

A fluoroscopic system was modified to achieve a 95-98% reduction in radiation exposure and dosage to patients compared with other systems that reduce fluoroscopic radiation dosage. This reduction was accomplished by custom selection of a high conversion-factor, triplemode image intensifier; custom design of a variable-dose rheostat, allowing maximum operator control of video camera gain; installation of an erbium rare-earth beam filter on the x-ray tube; and addition of a digital noise reducer (recursive filter). A total of 1,577 fluoroscopic examinations has been performed on this system, with excellent results. Contrast resolution was increased, while spatial resolution was maintained. Noise (quantum mottle) has been reduced by the addition of a digital image processor. Advantages of the ultra-low-dose system include: all fluoroscopic work is performed in a smooth, continuous real-time mode; the radiation exposure and dose saving is significantly greater than with pulsed and other proposed low-dose fluoroscopic systems; and the system automatically adapts for the wide variation in patient size routinely encountered in pediatric and adult radiology. The image quality is now such that this system could be used routinely for both adults and children.

Adult↗

Pediatric digital subtraction angiography.

Experience with intravenous digital subtraction angiography (DSA) in infants and children is limited, although its relative ease of performance, low complication rate, and diagnostic accuracy indicate great potential. We performed 87 DSA examinations (74 patients) and obtained sufficient detail to facilitate diagnosis in most cases. The major problems of patient movement and overlapping vessels can be minimized by judicious use of sedation and strict attention to technique. Exposure of patients to radiation has not been a limiting factor since our system uses low exposure factors (2-38 mR [.51-9.76 mu C/kg]/frame). Our results demonstrate that DSA has wide applicability to many organ systems and is especially useful in intracranial disease and for preoperative evaluation of neoplasms. More specific indications for its use in children must await further correlative studies.

Adolescent↗

Localized rigidity and narrowing of the antrum: a cause of gastric outlet obstruction in infancy.

In infants with persistent vomiting without bile staining, in whom congenital hypertrophic pyloric stenosis has been excluded, an upper gastrointestinal roentgenogram may show antral obstruction. Of four infants with partial gastric outlet obstruction described by the authors, an antral membrane was demonstrated radiologically in two. Other causes of antral obstruction, such as granulomatous disease, hour-glass deformity of the antrum, cholecystogastrocolic band and antral dysmotility, were considered and excluded as the cause of the vomiting. After medical management failed, gastroscopy revealed a rigid stenotic circumferential area in the antrum in all four infants. An antral membrane was not found. Laparotomy confirmed this finding and a pyloroplasty successfully resolved the symptoms. Localized rigidity and narrowing of the pyloric antrum, masquerading as an antral membrane radiologically, should be considered among the causes of gastric outlet obstruction in infancy. Treatment is determined by the severity of the symptoms. Pyloroplasty is successful when medical management fails.

Body Weight↗

Unusual osteopathy in a newborn.

A newborn baby presented with hyaline membrane disease, interstitial pneumonia, jaundice, hepatosplenomegaly, and unusual bone manifestations with lytic and sclerotic bone lesions and virtually absent periosteal reaction. He subsequently developed intracranial calcifications and mental retardation. The pneumonia and hepatosplenomegaly resolved. At the time of the delivery, a sibling was suffering from a severe undetermined viral infection. The clinical evolution of the disease and the radiologic findings led us to believe that this patient had a prenatal viral infection. The laboratory tests and the histologic picture of the bone biopsy supported the diagnosis.

Humans↗

Endotracheal foreign bodies. Difficulties in diagnosis.

The diagnosis of foreign body aspiration into the lower airway depends primarily on the radiographic demonstration of partial bronchial obstruction causing localized air trapping or atelectasis, present in 95 per cent of the cases. Endotracheal foreign bodies may often be visualized directly on high kilovoltage radiographs of the airway of the airway or by fluoroscopy. In three of the four cases presented in this report, radiologic evaluation was normal, but endotracheal foreign bodies were subsequently demonstrated endoscopically. Foreign objects may remain in the trachea for prolonged periods of time, causing persistent coughing, wheezing, or stridor. When there is a clear history or strong suspicion of foreign body aspiration in a patient with persistent symptoms, bronchoscopy should be used for diagnosis and treatment.

Bronchoscopy↗

Low positioning of umbilical-artery catheters increases associated complications in newborn infants.

We performed a randomized prospective study of the effect of placement position of umbilical-artery catheters on complication rates in high-risk newborn infants. A higher complication rate (31 of 40 vs. 13 of 33) (P less than 0.005) occurred in the group with the catheter tip at the third to fourth lumbar segment, as compared to those with the tip at the seventh to eighth thoracic segment, owing to more episodes of blanching and cyanosis of the extremities. There was no difference between groups in the rate of complications requiring catheter removal. Aortography revealed thrombosis in 21 of 23 patients studied, but there was no clinical evidence of impaired circulation. In retrospect, we found that, independently of catheter position, administration of antibiotics through the catheter was associated with an increased rate of complications (63 vs. 20 per cent). Umbilical-artery catheterization entails potential risks regardless of the position of the catheter; placement of the catheter with its tip at the seventh to eighth thoracic segment may be associated with fewer complications than at lower positions.

Amino Acids↗

Neonatal 'thick blood' syndrome.

It has long been known that neonatal hyperviscosity can produce serious central nervous system consequences, including paresis. In recent years, it has been shown that his condition occurs in approximately 5% of newborns and that partial plasma exchange transfusion can lower the hematocrit and may help prevent sequelae. Simple screening of all neonates four hours after birth is recommended so that treatment can be given early.

Blood Viscosity↗