Computed tomography scan assessment of lung disease in primary immunodeficiencies

A wide spectrum of lung disease can complicate primary immunodeficiencies and early recognition influences management and prognosis. Computed tomography (CT) especially high resolution computed tomography (HRCT) has been shown to detect lung disease in adult immunodeficient patients often when the chest radiograph (CXR) is normal, but this has not been studied in children. Twenty-five CT scans [10 HRCT] and CXRs were reviewed in 23 children [14 male, 9 female] with primary immunodeficiency. Eighteen [72%] of the CT scans were abnormal, bronchiectasis being the commonest finding present in eight CT scans in patients with antibody deficiency. In eight cases CT scan revealed changes not seen on CXR (bronchiectasis;interstitial changes; small parenchymal nodules; air trapping;and a small upper lobe cyst) which influenced treatment in six cases. Conclusion CT scans have a valuable role in assessing lung disease in children with primary immunodeficiencies and will detect important changes not visible on CXR. Received: 9 December 1997 / Accepted in revised form: 3 June 1998     

Infantile hemiplegia: clinical,electroencephalographic and computerised tomography study

Thirty-one patients with post-neonatally acquired infantile hemiplegia were assessed clinically, electroencephalographically and by CT scan. The mean age of onset of illness and when first seen in the neurology clinic were 20·2±20·1 months and 16·8±8·4 yr, respectively. Epilepsy was seen in 74 per cent of cases and mental retardation in 70 percent. Among patients with epilepsy, the EEG and CT scan were abnormal in 83 per cent and 87 per cent respectively. There was a positive correlation between an abnormal EEG and CT scan in 84 per cent of cases. The cause of hemiplegia could be clinically determined in only eight cases (26%). However, the CT scan showed abnormalities like cortical atrophy and porencephalic cyst, suggestive of a vascular or inflammatory aetiology in 68 per cent. The usefulness of CT in determining the aetiology in acquired infantile hemiplegia, especially when seen many years after the onset of illness is, emphasized.     

Therapeutic efficacy and adverse effects of adrenocorticotropic hormone therapy in west syndrome: differences in dosage of adrenocorticotropic hormone, onset of age, and cause

OBJECTIVES: To determine the dosage and factors influencing efficacy of adrenocorticotropic hormone (ACTH) for West syndrome. STUDY DESIGN: A retrospective study of 135 patients receiving ACTH therapy with a synthetic analogue for initial effect, seizure outcome 1 year after therapy, and adverse effects. Efficacy and adverse effects were compared among the groups divided by clinical factors: dosage, treatment lag, onset age, and cause. RESULTS: One hundred thirteen patients had seizure control with ACTH. For more than 1 year after ACTH, 59 remained seizure free. Adverse effects were observed in 57, and ACTH therapy was discontinued in 23. The lowest dosage group (0.0125 mg/kg/d) had fewer episodes of discontinuation (P<.05), whereas differences in efficacy between different dosages were insignificant. None of the clinical factors correlated with initial effect. The earlier-onset group (<4 months) showed unfavorable seizure outcome 1 year after ACTH (P<.01). The cryptogenic patients showed better seizure outcome (P<.05) compared with the symptomatic. CONCLUSION: Synthetic ACTH therapy at a lower dosage is as effective as natural ACTH therapy at a higher dosage. Considering the adverse effects and the benefits for seizure control, the ACTH dosage of 0.0125 mg/kg/d (synthetic analogue) is more favorable than larger dosage.     

Early diagnosis of herpes simplex encephalitis in childhood

In the initial phase of HSE the clinical symptomatology is more variable and insidious in babies and young children than in older children and adults. Combined clinical, neurophysiological and neuroradiological studies have been carried out in 12 children with proven HSE.Ten patients had the first EEGs taken during the acute phase of the illness and all showed large amplitude irregular slow activity, sharp waves and often spikes with variable distribution; in 7 cases periodic phenomena were recognisable. At a later stage localised low amplitude EEG activities were found in children with focal neurological symptoms. Areas of low attenuation were seen in the CT scans of the 7 children who had this investigation done at an early stage of their illness. Such low density regions persisted at follow-up and eventually cerebral atrophy with irregular features became obvious.Prompt EEG investigations combined with CT scans provide an early diagnostic clue for treatment. Follow-up EEG studies (including VEP) and CT scans may help assess the severity of residual cerebral damage in the survivors.     

Chest computed tomorgraphy in infants and children An analysis of 50 patients

Chest computed tomography (CT) scans were performed in 50 pediatric patients. The techniques and inherent problems are presented. CT provides detailed anatomic information about the chest wall, mediastinum, and lungs. Pediatric chest CT is particularly valuable in the evaluation of extrapleural masses, parenchymal metastatic disease, and posterior mediastinal tumors.Presented at the Annual Meeting of the Society for Pediatric Radiology, Salt Lake City, Utah, USA, April 1980     

Long-term follow-up study of West syndrome: Differences of outcome among symptomatic etiologies

OBJECTIVES: To evaluate the outcome of West syndrome and to elucidate the differences in the outcome related to the timing of brain injury. STUDY DESIGN: Medical records of 60 patients who were followed regularly for more than seven years were reviewed. The following clinical features were assessed: onset, seizure evolution, electroencephalography and intelligence. Those variables were compared among five groups: cryptogenic, prenatal, preterm, term, and postnatal groups. RESULTS: The onset ages of the postnatal group were later than those of the others (P<.05). The relapse after adrenocorticotropic hormone therapy of the preterm group was the earliest among the groups (P<.05). Regarding encelphalography, the ratio of patients with focal discharges was higher in the postnatal group than in the prenatal group (P<.05). The ratios of patients in whom focal epilepsy developed were higher in the term and postnatal group than in the cryptogenic and prenatal group (P<.05). The term group showed similar characteristics to those of the postnatal group. Seven of the 60 had normal intelligence, including three girls with tuberous sclerosis. CONCLUSION: The diverse outcomes of West syndrome depending on etiology seemed to be related to the timing of brain injury and brain development.     

Nephrocalcinosis in radiographs,computed tomography,sonography and histology

In 12 patients with nephrocalcinosis, radiographs were compared with computed tomography, sonography and histology. Computed tomography detects nephrocalcinosis at a very early stage of the disease, gives a better picture of the density and exten of nephrocalcinosis and may detect other findings (e. g. cysts). Radiographs may show many more details than computed tomography; they seem to be more suitable for observing the course of the disease and have a lower radiation dose. Sonography has not been helpful in the diagnosis of nephrocalcinosis. Histology correlates only moderately with the degree of nephrocalcinosis demonstrated in radiographs and computed tomography.     

Osteopetrosis: Brain ultrasound and computed tomography findings

A case of osteopetrosis presenting with rare features of dysmorphism with proptosis due to hypoplasia of the orbits and the temporal bone is described. The case also had calcifications in the periventricular regions, the falx cerebri and the corpora colliculi. These features and sonographic findings of osteopetrosis have not been reported previously. The sensitivity and specificity of imaging modalities in the diagnosis of intracranial calcifications is discussed.     

Some rare causes of intracranial calcification in childhood: computed tomographic findings

The CT findings in 36 cases of childhood intracranial calcification were analysed. Tuberous sclerosis was the commonest disease, predisposing to calcifications that were essentially nodular in type and bilaterally periventricular in distribution. Calcifications were also noted in other common diseases such as tuberculoma and osteopetrosis, and after meningitis. There were three cases of osteopetrosis with intracranial calcifications: one had calcifications in the peritentorial region and two in the junction regions between the grey and white matters with basal ganglia. These observations were previously unreported. The presence of basal ganglia calcification in Down syndrome is also reported. Most calcifications were not visualized on plain radiographs. Our study shows that if the CT attenuation values are less than 200 Hounsfield units (HU), the calcifications are not visualized on the plain radiographs.     

Childhood exposure to ionizing radiation from computed tomography imaging in Nova Scotia

BACKGROUND:

Examining radiation dose in the paediatric population is particularly important due to the vulnerability of paediatric patients (increased radiosensitive tissues and postexposure life-years) and risk for future radiogenic malignancy.

OBJECTIVES:

To evaluate trends in paediatric computed tomography (CT) use and ionizing radiation exposure using population-based data from Nova Scotia.

METHODS:

A retrospective, population-based cohort study of CT use in patients <20 years of age, from January 1, 2004 to December 31, 2011, was performed in Nova Scotia. CT examination data were retrieved from a provincial imaging repository. Trends in CT use were described, and both annual and cumulative effective dose exposures were calculated.

RESULTS:

In total, 29,452 CT events, involving up to 22,867 individuals were retrieved. Overall annual paediatric CT examination rates remained static (range 17.4 to 18.8 per 1000 per year). However, use in children <10 years of age decreased by >50% (P<0.001); this was counterbalanced by a steady increase among 15- to 19-year-olds (P<0.0001). Overall, 15.4% of scanned patients underwent ≥2 examinations, of which 58 patients (1.6%) exceeded 50 mSv of exposure.

CONCLUSIONS:

Despite a static rate in CT imaging among the entire cohort, children <15 years of age and, particularly, those <10 years of age displayed marked reductions in CT use. This may reflect increased awareness of campaigns emphasizing judicious CT use, revised clinical practice guidelines and increased availability of alternative modalities. A small subgroup demonstrated high-dose exposure (>50 mSv), and rates in individuals >15 years of age steadily increased, suggesting further exposure reduction efforts are necessary.     

Polyarteritis nodosa: successful diagnostic imaging utilizing pulsed and color Doppler ultrasonography and computed tomography angiography

The diagnosis of polyarteritis nodosa (PAN) is often delayed because patients present with diverse clinical symptoms, but with less disease-specific signs. However, early diagnosis and treatment of PAN is necessary to prevent serious organ damage. Herein, we present a boy in whom the diagnosis of classical PAN was established rapidly with ultrasound and computed tomography (CT) angiography. Moreover, complete disappearance of the aneurysms after one month of therapy was documented. This case is presented in order to underline the role of new imaging techniques in the diagnosis and follow-up of patients with PAN.     

Complex congenital cardiovascular anomalies evaluated by continuous-rotation computed tomography in children

Summary The slip ring and nutate/rotate configuration of the new, fourth-generation, computed tomography (CT) (TCT-900S, Toshiba Medical Company) makes it possible to scan consecutively 25 slices every 75 s. This scanner also rapidly provides hemodynamic and multiplanar reconstruction images. Studies were performed on 13 children with various forms of complex congenital cardiovascular anomalies. The findings correlated very well with those by angiocardiography and echocardiography. But the cardiac valves were difficult to scan, and combined use with echocardiography was considered to be indispensable. The rapid multiplanar reconstruction imaging is very effective in depicting coarctation of the aortic isthmus, ductus arteriosus, and hypoplasia of the great arteries in newborns and infants who have small target regions. Cine-displays of such images can clearly show ventricular and atrial septal defects. This new CT system proved effective in diagnosing complex congenital cardiovascular anomalies even in young infants.     

Cerebral infarction in infants and children: Clinical features, CT and EEG findings

In a retrospective study, medical records of twenty one infants and children who were diagnosed for cerebrovascular disease, were analysed. Patients with history of head injury, brain tumor and intracranial infections were excluded from the study. Age ranged between six weeks and 12 years. Fourteen were boys and 7 girls. Ten patients (47%) had known underlying risk factor. Hemiparesis and seizures were commonest presenting features as well as long term neurological sequelae in survivors. CT scan was abnormal in all patients whereas, EEG was abnormal in 90% with 76% lateralizing effect.