FIG 3. Computed tomography of the thorax reveals bronchiectasis, with centrilobular nodules in a tree-in-bud pattern at the right middle and lower lobes and the lingular segment, suggesting active endobronchial sepsis

Anomalous systemic arterial supply to the normal basal segments of the lower lobe of the lung without sequestration is a rare congenital abnormality. Whether the condition belongs to the broad spectrum of sequestration disorders remains controversial. This report is of a patient with anomalous systemic arterial supply to the normal basal segments of the left lower lobe, together with an attenuated pulmonary artery and normal tracheobronchial tree.

Chronic graft-versus-host disease is a cellular immune-mediated donor bone marrow versus patient rejection reaction, which is a major cause of morbidity and mortality following allogeneic bone marrow transplantation or stem cell transplantation. Chronic graft-versus-host-disease–related myositis and fasciitis, which have been described as rare complications in patients who develop chronic graft-versus-host disease after allogeneic bone marrow transplantation or stem cell transplantation, can severely impair a patient’s quality of life. This report describes a patient with chronic graft-versus-host-disease–related myositis and fasciitis, and presented with progressive contracture. This report illustrates the imaging features and the role of radiology in the diagnostic process.

Congenital muscular dystrophy (CMD) comprises a heterogeneous group of disorders present at birth with muscle weakness, hypotonia and contractures. Congenital muscular dystrophy (CMD) comprises a heterogeneous group of disorders with muscle weakness, hypotonia and contractures present at birth. A particular subset of classic CMD is characterized by a complete absence of merosin. Merosin-deficient congenital muscular dystrophy (MDCMD) is a rare genetic disease involving the central and peripheral nervous system in the childhood. High signal intensities are often observed throughout the centrum semiovale, periventricular, and sub-cortical white matters on T2-weighted images in MRI brain in children with MDCMD. Apparent diffusion coefficient (ADC) map may reveal increased signal intensity and apparent diffusion coefficient values in the periventricular and deep white matters. These white matter findings, observed in late infancy, decrease in severity with age. The pathogenesis of these changes remains uncertain at present. In this article, we outline the specific MR imaging findings seen in a patient with documented MDCMD and also suggest the causes.

Chiari I malformation may not be congenital, but may be acquired as a consequence of skull deformities and other associated intracranial factors in patients with craniosynostosis. Pfeiffer syndrome is one of the many conditions associated with Chiari I malformation. Premature fusion of multiple cranial sutures and cloverleaf skull (kleeblattschadel deformity) are often observed in the calvaria of patients with Pfeiffer syndrome. This report is of a male infant, with Pfeiffer syndrome who was noted to have progressive Chiari I malformation, with classical imaging features illustrated. Important aspects of preoperative imaging will be discussed, with a brief review of literature.

Osteogenesis imperfecta type V is a rare subtype of osteogenesis imperfecta with unique clinical, radiological, and histological features. Herein, we report two paediatric patients with osteogenesis imperfecta type V, both presenting with recurrent bone fractures since early childhood and the classical radiological features of osteogenesis imperfecta type V (calcified radio-ulnar interosseous membrane). One of them also developed hypertrophic callus formation. Knowledge and recognition of these specific radiographic findings can facilitate a timely diagnosis of osteogenesis imperfecta type V, which may have significant implications on management. Such consideration includes bisphosphonate use (a well-established treatment for certain disease subtypes of osteogenesis imperfecta), but may lead to progression of hypertrophic callus in osteogenesis imperfecta type V.

Congenital umbilical anomalies are common presentations in paediatric patients, and are usually classified as persistent umbilical cord structures or failure of umbilical ring closures. Urachal anomalies, umbilical herniations, and vitelline duct anomalies are the main classifications for these disorders. Among these disorders, only congenital urachal cyst is common. All anomalies associated with the vitelline duct are rare, and umbilico-enteric fistula is the rarest of all. This report is of a rare umbilical fistula in a neonate with persistent vitelline duct, with a brief review of the embryology, anatomy, and classification of vitelline duct malformations. The differential diagnosis and management options are also discussed.

Vascular channel of the frontal bone is a rare anatomical variant. This report describes a child who suffered from head injury with right parietal skull fracture and another suspicious fracture at the right frontal bone. Follow-up computed tomography showed persistent frontal "fracture" while the parietal fracture had resolved. Post-processing with 3D volume rendering showed that it was actually not a fracture. While there is no accessory frontal suture reported other than the metopic suture, the most likely cause for the radiolucency here is vascular channel of the frontal bone. Image post-processing is very helpful in doubtful situation, and should be considered in addition to axial images in our daily practice.

A patient with redundant nerve roots of the cauda equina associated with the presence of an intra-spinal arachnoid cyst is described. She presented with sensory, motor and sphincter dysfunction. Magnetic resonance imaging revealed redundant nerve roots manifesting as serpiginous structures within the thecal sac. Marked improvement of symptoms was noted after laminectomy. The serpiginous appearance of the nerve roots persisted postoperatively despite good decompression of the spine. The clinical and radiological presentation, aetiology, and treatment will be discussed.

Osteogenesis imperfecta type V was first described in 2000. It is a distinct clinical entity with unique clinical, radiological, and histological features. Clinically, it is only moderately deforming. Patients have normal scleraand teeth. Radiological diagnostic criteria include a triad of calcification of the radioulnar interosseous membrane, presence of hypertrophic callus at fractures or post-operative sites, and radiodense metaphysealbandadjacenttogrowthplates(1). Histologically, it is distinguished by a mesh-like pattern of lamellation under polarized light microscopy for iliac bone samples. Ossification of the interosseous membrane of the forearm is a constant feature, although it may vary in its extent. The presence of ossification can severely limit movements of the forearm, and is associated with secondary dislocation of the radial head (1, 2). The frequency of radial head dislocation/subluxation is significantly higher in type V osteogenesis imperfecta (86%) than in the other types (0% to 29%) (3). The presence of interosseous membrane ossification in a pediatric patient, with or without radial head dislocation, should prompt radiologists to consider OI type Vas a diagnosis. The formationof hypertrophic callus, if present, isthe most conspicuous clinical symptom in OI type V (1). It is reported that while not all OI type V patients have hypertrophic callus formation, all patients with hypertrophic callus formation are OI type V in the proper clinical context (1, 4). Lesions form during the growth years at sites of rapid periosteal apposition. The long bones are most often affected, particularly in the lower extremities (4). Hypertrophic callus can be precipitated by fracture or surgery, or arise spontaneously. It can become very large or even mimic osteosarcoma (2, 5). In unclear cases, MRI and CT can be helpful in distinguishing hypertrophic callus from sarcoma (6, 7). Evolution of the lesions is variable, ranging from complete resolution to significant persisting morbidity.

Aims Epidermal fatty-acid-binding protein (E-FABP) is highly homologous to adipocyte FABP (A-FABP), which mediates obesity-related metabolic syndrome (MetS), diabetes and atherosclerosis in animals. Combined deficiency of E-FABP and A-FABP protects against the MetS and atherosclerosis in mice. This study investigated the association of serum E-FABP with cardio-metabolic risk factors and carotid atherosclerosis in humans.Methods and results The presence of E-FABP in human plasma was detected by tandem mass spectrometry. Serum E-FABP levels, determined by an enzyme-linked immunosorbent assay in 479 Chinese subjects (age: 55.4 +/- 13.5 years; M/F: 232/247), correlated positively (P < 0.05 to < 0.001, age-adjusted) with parameters of adiposity, adverse lipid profiles, serum insulin, A-FABP, and C-reactive protein levels and were higher in subjects with the MetS (P < 0.001 vs. no MetS). The association of E-FABP with the MetS was independent of A-FABP. Furthermore, serum E-FABP correlated with carotid intima-media thickness (IMT; P < 0.001) and was independently associated with carotid IMT in men (adjusted P = 0.03).Conclusion E-FABP is a new circulating biomarker associated with increased cardio-metabolic risk. It may contribute to the development of the MetS and carotid atherosclerosis in humans, independent of the effect of A-FABP.

Objective-Adipocyte fatty acid-binding protein (A-FABP) has been shown to be an important player in atherosclerosis in animal models. However, the clinical relevance of these findings is still unknown. This study aims to examine the relationship between serum A-FABP level and carotid intima-media thickness (IMT), an indicator of atherosclerosis in humans.Methods and Results-The study cohort included 479 Chinese subjects who underwent carotid IMT measurement. Serum A-FABP levels were determined by enzyme-linked immunosorbent assays. Serum A-FABP levels positively correlated with carotid IMT in both men ( r = 0.211, P = 0.001) and women ( r = 0.435, P = 0.001). In women, but not in men, the presence of plaques was associated with significantly higher serum A-FABP levels ( P = 0.001 versus women without plaques). Stepwise multiple regression analysis showed that serum A-FABP level was independently associated with carotid IMT in women ( P = 0.034), together with age and hypertension ( both P = 0.001).Conclusions -A-FABP is an independent determinant of carotid atherosclerosis in Chinese women, but not in men. This gender difference may be attributed to the lower serum A-FABP levels in men, and the effect of other risk factors, such as smoking, among our male participants. Our results have provided clinical evidence supporting the role of A-FABP in the development of atherosclerosis.

PURPOSE:The aim of this study was to analyze the early and late results of pediatric liver transplantation, with particular reference to complications that required surgical or radiologic intervention.METHODS:The records and code sheets of children who underwent liver transplantation in the authors' institution between September 1993 and December 2001 were reviewed.RESULTS:Twenty-nine children (16 boys and 13 girls) underwent 31 liver transplantations (23 living donor, 8 cadaveric donor) during the study period. The ages of the children ranged from 4 months to 132 months (median, 16 months). Eighteen children had complications that required surgical or radiologic interventional procedures. Complications included, among others, hepatic vein thrombosis (n = 1, 3%), hepatic vein stenosis (n = 2, 7%), portal vein thrombosis (n = 2, 7%), biliary stricture (n = 3, 10%), bile leakage (n = 2, 7%), hepatic artery pseudoaneurysm (n = 1, 3%), jejuno-jejunostomy leakage (n = 1, 3%), graft hepatitis (n = 1, 3%), and posttransplant lymphoproliferative disorder (n = 2, 7%). In addition, 6 children (21%) suffered from intraabdominal bleeding from a variety of causes. After appropriate interventions, at a median follow-up of 38 months (range, 1 to 96 months), patient and graft survival rates were 79% and 74%, respectively. The retransplantation rate was only 7%. There was no incidence of hepatic artery thrombosis. All living donors remain alive and well.CONCLUSIONS:Complications are inevitable in pediatric liver transplantation. However, with timely recognition and active intervention, a good outcome can be achieved.

OBJECTIVES:To review evidence of iodine deficiency and clinical thyroid disorders in Hong Kong.DATA SOURCES:Publications on local dietary iodine intake, the iodine content of local food items, and clinical thyroid problems in the Hong Kong population.DATA EXTRACTION:Data was extracted and evaluated independently by the authors.DATA SYNTHESIS:Iodine is an essential nutrient. Iodine deficiency can lead to goitre, hypothyroidism, mental deficiency, and impaired growth. It is now appreciated that determination of goitre incidence in children alone may grossly underestimate the problem of iodine deficiency in a population. In total, the evidence indicates that iodine deficiency exists in Hong Kong, leading to clinical problems of transient neonatal hypothyroidism, goitrogenesis, and thyroid disorders in pregnant women and neonates, as well as thyroid dysfunction in the elderly.CONCLUSION:A supplementation programme aimed at a relatively uniform iodine intake is recommended to avoid deficient or excessive iodine intake in subpopulations.