Radiological case of the month. Congenital microgastria.
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Biomedical subjects
Publications and source records attributed to A Bowen.
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Endogenous serum thrombopoietin (TPO) levels were measured in 31 patients with aplastic anaemia (AA) using an enzyme immunoassay with a sensitivity of 20 pg/ ml. The median platelet count for all AA patients was 30 +/- 29 x 10(9)/l (range 5-102) compared with a median of 284 +/- 59 x 10(9)/l (range 148-538) for normal controls. Serum TPO levels were significantly elevated in all patients compared with normals (1706 +/- 1114.2, range 375-5000 v 78 +/- 54, range 16.5-312.9, P < 0.0001). There was no correlation between serum TPO levels and the degree of thrombocytopenia in AA patients, but TPO levels were significantly higher in patients who were platelet transfusion dependent than in patients who were transfusion independent (P < 0.01). There was a trend for higher TPO levels in patients with severe AA compared with non-severe AA patients. Clinical trials of TPO and a related truncated, pegylated molecule, megakaryocyte growth and development factor (PEG-rHuMGDF), are awaited to determine whether treatment with these drugs will result in increased platelet counts in patients with AA.
The present study was undertaken to determine whether the addition of an androgen to estrogen therapy in postmenopausal women would alter the skeletal response as determined by measurements of markers of bone formation and resorption. Postmenopausal women were treated for 9 weeks with either a combination of 1.25 mg esterified estrogen and 2.5 mg methyltestosterone (E+A) or 1.25 mg conjugated equine estrogen (CEE). Both groups showed a similar decrease in urinary excretion of the bone resorption markers, deoxypyridinoline, pyridinoline, and hydroxyproline. Patients treated with CEE showed decreases in the serum markers of bone formation, bone-specific alkaline phosphatase, osteocalcin, and C-terminal procollagen peptide. In contrast, subjects treated with E+A showed increases in these markers of bone formation. CEE increased, and E+A decreased serum levels of sex hormone-binding globulin as well as triglycerides and high density lipoprotein levels. Only CEE significantly reduced low density lipoproteins. Both regimens were effective in reducing postmenopausal somatic symptoms, but only E+A had a significant effect on psychological symptoms. We conclude that short term administration of androgen with estrogen may reverse the inhibitory effects of estrogen on bone formation. Long term studies are needed to determine the relative benefits and risks of the combination of estrogen and androgen and whether this results in greater increases in bone mass and strength.
Thoughtfully planned and skillfully executed diagnostic and interventional imaging is indispensible in preoperative assessment of the pediatric liver transplant candidate and for the detection and management of the myriad postoperative complications that may affect the liver recipient.
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Thirteen children initially suspected to have an intrathoracic or intraabdominal mass had malposition of the liver and/or spleen discovered by ultrasound. This group consisted of five children with diaphragmatic eventration or hernia and eight with wandering spleen or liver. Careful ultrasonographic examinations were diagnostic in all patients; seven had surgical confirmation. CT, MRI, nuclear scan and fluoroscopic imaging were useful in select instances. The liver and spleen have a characteristic anatomic configuration and sonographic appearance that should enable them to be recognized, and pathological alterations appreciated, even when these organs are ectopic in location or malformed.
The clinical features and current management of cystic fibrosis are reviewed, with emphasis on pulmonary manifestations. Pulmonary radiographic findings in cystic fibrosis are illustrated. The role of transplantation as a therapeutic avenue is discussed. Recent discoveries--the specific genetic mutation in cystic fibrosis, the elucidation of the cellular defect, and the development of gene-transfer techniques to correct the cellular defect in vitro--have generated fresh excitement in the search for an ultimate cure for cystic fibrosis.
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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.
Cf the 87 survivors of extracorporeal membrane oxygenation over a 10-year period, 67 participated in a follow-up study which included neurologic examination (n = 67), cognitive testing (n = 67), and audiologic assessment (n = 33). Matched control subjects for those older than 5 years were also evaluated. Outcome was defined as normal for cognitive scores greater than or equal to 85 and normal neurologic examination results, suspect for cognitive scores 70 through 84 or nonfocal neurologic findings such as hypertonia/hypotonia, and abnormal for cognitive scores less than 70 or abnormal neurologic examination results. Of the 10 school-aged children studied, 9 were normal and there were no differences in mean cognitive scores between subjects and controls (IQ subjects = 109 +/- 12 [SD], IQ controls = 107 +/- 13). For preschoolers aged 2.7 through 4.11 years, the mean cognitive score was 91 +/- 11 and 7 (70%) were normal. For infants 6 through 30 months, the mean cognitive score was 101 +/- 22 and 27 (57%) were normal. A total of 7 children (21% of those studied) had abnormal audiologic assessments. Three children demonstrated mild high-frequency and 4 moderately severe high-frequency sensorineural hearing loss which was bilateral in 3 and of undetermined laterality in 1. Abnormal neurodevelopmental outcome was significantly associated with cerebral infarction and chronic lung disease. Outcome was not related to demographic or perinatal variables, illness severity prior to extracorporeal membrane oxygenation, or underlying diagnosis. Neurodevelopmental outcome among survivors of extracorporeal membrane oxygenation in this series is consistent with previous reports of morbidity among neonates with severe respiratory failure treated conventionally.
Bronchopulmonary foregut malformations encompass a great variety of anomalies that may arise from abnormal differentiation of the respiratory and alimentary tracts, abnormal separation of the two systems, or abnormal development of blood supply, perhaps singly or in combination, during early embryogenesis. The pulmonary and neurenteric forms share the common features of a pulmonary parenchymal opacification and/or intrathoracic mass, with the addition of vertebral anomalies in the instance of the neurenteric malformations. Plain radiographs serve as the starting point for diagnostic evaluation and sometimes are all that is needed; more often, though, the plain film findings suggest which road to follow in further imaging. No single imaging approach can be advocated for all patients. The lesion may be found initially, for example, on prenatal sonography. Ultrasonography also can establish the cystic nature of a mass and may help define vascular supply, although it cannot yet be said to have supplanted angiography's role in evaluating suspected sequestrations. For most intramediastinal or intrapulmonary masses without associated vertebral anomalies, CT will satisfactorily establish the cystic nature and should afford a reasonably confident preoperative diagnosis of bronchogenic cysts or type 1 or 2 cystic adenomatoid malformations. Type 3 cystic adenomatoid malformation, because of its relatively "solid" imaging characteristics, may not be diagnosed confidently by preoperative imaging. The finding of vertebral anomalies associated with a mediastinal mass especially warrants MR imaging to define intraspinal involvement. However, the association of vertebral or rib anomalies with apparent unilateral pulmonary agenesis or hypoplasia arouses suspicion of a bronchopulmonary foregut malformation that communicates with the gastrointestinal tract, and this uncommon situation calls for a barium examination. Radionuclide scintigraphy has an ancillary role in assessing foregut anomalies, but many findings from scintigraphy are relatively nonspecific and anatomic definition is poor. Scintigraphy may yield supportive information in cases of sequestration that have inconclusive findings as determined by plain radiographs and CT.
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The consistency among a number of measures of self-report of alcohol consumption in imprisoned male young offenders was studied by asking 56 young offenders to report, based on a 'typical week', how many days they had consumed alcohol, how many days they were drunk and how much alcohol they had consumed. They were asked to rate their confidence in the accuracy of their self-report and to complete the short EPQ-R, which contains a Lie Scale. Results show that self-report in this population is reliable over time and that different indices of alcohol consumption correlate significantly with each other. Young offenders report a moderate degree of confidence in their accuracy of self-report and the mean EPQ-R Lie Scale score is within 1 standard deviation of the norm for a comparable sample.
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The authors describe radiologic findings in five patients with spontaneous perforation of the common bile duct (a rare disorder). The patients were 5 weeks, 9 weeks, 3 months, 11 months, and 2 1/2 years old at presentation. The most common presenting complaints were jaundice and abdominal distention (due to ascites). Sonographic findings included ascites in three patients, a loculated fluid collection around the gallbladder in two patients, and both in one patient. The biliary tree was undilated in all patients. Results of hepatobiliary scintigraphy definitely demonstrated that intraperitoneal fluid originated from the biliary tract. Intraoperative cholangiography was used to confirm the diagnosis at surgery. All children were successfully treated with surgery. Recognition of these findings on sonograms and subsequent confirmation with scintigraphy are important to prevent delays in diagnosis of a potentially fatal condition.
In a retrospective study of proved pseudoaneurysms (PAs) in 15 patients with transplanted organs (11 liver, three kidney, one pancreas), the results of computed tomography (CT), duplex sonography, and angiography were reviewed. Of the 15 cases of PA, eight occurred at the arterial anastomosis and seven were nonanastomotic. Three of the eight anastomotic PAs were caused by infection. Of the seven nonanastomotic PAs, four were caused by percutaneous biopsy, two were caused by infection, and one was of undetermined cause. In nine (60%) of the 15 patients the PAs were incidentally detected at imaging studies performed for other reasons. Diagnosis requires a high degree of suspicion. CT was performed in nine cases and duplex sonography in ten. The diagnosis of PA was made with CT in six (67%) patients and with duplex sonography in five (50%). CT and duplex sonography could not enable diagnosis when the PA was small, when the arterial anastomosis was not included in the field of study, or when enhancement with intravenously administered contract material was suboptimal. Angiography depicted the PAs in all 15 patients. In three liver transplant recipients with gastrointestinal tract bleeding, the causative PAs were detected only with angiography.