GIST presenting as refractory iron-deficiency anaemia in paediatric patient.

Gastrointestinal stromal tumours (GISTs) are very rare gastrointestinal (GI) mesenchymal tumours affecting only 0.02 children/million/year below the age of 14 years. We reported a 9-year-old girl presented to emergency department with pallor and haemoglobin of 50 g/L. Extensive workup for anaemia suggested iron-deficiency anaemia secondary to GI loss. Ultimately after blood transfusion of packed cells, she was discharged with a haemoglobin of 92 g/L with iron supplementation. Upper endoscopy showed incidental antral nodularity with biopsy proven helicobacter gastritis and an isolate 3-4 cm suspicious mass in the lesser curvature. Abdomen imaging confirmed the gastric mass in addition to two lesions, one retroperitoneal and one paraspinal. She undergone open laparotomy with complete surgical resection of the gastric and retroperitoneal masses with histological confirmation of GIST and paraganglioma. This case emphasises the importance of proper examination of the stomach at endoscopy and to illustrate that although anaemia is common in paediatric age group it may be reflect serious medical condition even in normal looking child.

Adenovirus as a rare cause of acute necrotising encephalitis.

Adenovirus is a common cause of upper and lower respiratory tract infections. Rarely, neurological manifestations may occur, ranging from mild aseptic meningitis to potentially fatal acute necrotising encephalopathy (ANE). Very little is known in regards to the exact pathogenesis of ANE in association with adenovirus. This report describes the presentation of a previously healthy 14-month-old girl diagnosed with adenovirus-induced ANE. Herein, we highlight the clinicoradiological manifestation of this uncommon association with adenovirus in order to maintain a high index of suspicion for early diagnosis and a better outcome.

Intracranial volume (ICV) in isolated sagittal craniosynostosis: a retrospective case-matched-control study.

PURPOSE: Children with sagittal craniosynostosis (SC) are at risk of developing raised intracranial pressure (ICP). This is thought to result from cephalocranial disproportion-the restriction of normal cerebral development by a small cranial vault. It remains unclear whether intracranial volume (ICV) is altered in SC. This study offers a novel volumetric analysis of the scaphocephalic skull, comparing supratentorial (ST) volume, infratentorial (IT) volume, and total ICV of patients with sagittal synostosis to normal controls. METHODS: ICVs of 32 consecutive patients undergoing total calvarial vault remodelling (TCVR) for isolated SC were compared to 32 age- and sex-matched normal controls. ICV was measured with manual techniques on head computerised tomographic (CT) scans using OsiriX software. A paired t test was used to compare data between cases and controls. RESULTS: Mean total ICV, ST volume and IT volume were larger in SC than in controls, except in females > 6 months of age. There was no statistical significance. Regression analysis demonstrated larger ICVs in diseased children than in controls younger than 10 months, at which age trend lines intersected and the reverse became true for older children. This likely represents an evolving risk of cephalocranial disproportion beyond 10 months of age. The IT/ST volume ratio was conserved in scaphocephaly, and very closely approximated that of controls. CONCLUSIONS: Sagittal craniosynostosis appears to be associated with a larger cranial vault at less than 10 months and a smaller vault at greater than 10 months, although statistical significance was not achieved.

A Case of Primary Intra-abdominal Synovial Sarcoma.

Synovial sarcomas are relatively common intermediate-to-high-grade malignant soft tissue tumors, often with an initial indolent course. And among the sarcomas primary intra-abdominal synovial sarcoma is a relatively rare entity that may present with an abdominal mass and diagnosis is usually confirmed by immunohistochemistry. The authors report a case of a 46-year-old man who presented with a large palpable abdominal mass. Contrast-enhanced computed tomography (CT) scan showed a large well-defined heterogeneous intra-abdominal mass filling the entire pelvis and extending upwards till the subhepatic region causing displacement of intra-abdominal organs. However, no other focal lesions were seen in the solid organs. The same findings were confirmed on surgery. Histopathology was suggestive of desmoid tumor/hemangiopericytoma. Immunohistochemistry showed positive markers corresponding to synovial sarcoma. The patient was advised to undergo chemotherapy which was refused and follow-up was lost. After 10 months, patient presented for follow-up CT, which showed marked increase in size of the lesion.

Pediatric whole-body magnetic resonance imaging: Intra-individual comparison of technical quality, artifacts, and fixed structure visibility at 1.5 and 3 T.

PURPOSE: To compare whole-body magnetic resonance imaging (WBMRI) performed at 1.5 and 3 T for technical quality, artifacts, and visibility of selected fixed structures. PATIENTS AND METHODS: 21 children who had WBMRI at both 1.5 and 3 T scanners within a relatively short interval (3-13 months; average-8.6 months) were included. The images were objectively compared with scores from 4 to 1 for five parameters including severity of artifacts; visibility of liver, vertebral column, and marrow in legs; and overall image quality. Inter-observer agreement was calculated using Kendall's coefficient of Concordance (W) and scores were compared using Signed Rank test. RESULTS: There was substantial inter-observer agreement for all five categories at both field strengths. The difference between averages of mean scores of all five parameters for two field strengths was statistically significant (P < 0.05), indicating less artifact, better fixed structure visibility, and overall image quality at 1.5 T as compared to 3 T. However, scores at 3 T were also rated within a good range (around 3) indicating its feasibility for WBMRI in children. CONCLUSION: WBMRI at 1.5 T has significantly better image quality, fixed structure visibility, and fewer artifacts, as compared to WBMRI at 3 T in children. This difference is unlikely to significantly affect detection of pathology on 3 T WBMRI as the image quality score at 3 T was also within good range.

NetPath: a public resource of curated signal transduction pathways.

We have developed NetPath as a resource of curated human signaling pathways. As an initial step, NetPath provides detailed maps of a number of immune signaling pathways, which include approximately 1,600 reactions annotated from the literature and more than 2,800 instances of transcriptionally regulated genes - all linked to over 5,500 published articles. We anticipate NetPath to become a consolidated resource for human signaling pathways that should enable systems biology approaches.

Large low-frequency fluctuations in the velocity of a driven vortex lattice in a single crystal of 2H-NbSe2 superconductor.

The driven state of a well-ordered flux line lattice in a single crystal of 2H-NbSe2 in the time domain has revealed the presence of substantial fluctuations in velocity, with large and distinct time periods ( approximately seconds). A superposition of a periodic drive in the driven vortex lattice causes distinct changes in these fluctuations. We propose that prior to the onset of the peak effect there exists a heretofore unexplored regime of coherent dynamics, with unexpected behavior in velocity fluctuations.

Mapping giant magnetic fields around dense solid plasmas by high-resolution magneto-optical microscopy.

We investigate the distribution of magnetic fields around dense solid plasmas generated by intense p-polarized laser approximately 10(16) W cm(-2), 100 fs) irradiation of magnetic tapes, using high sensitivity magneto-optical microscopy. By investigating the effect of irradiation on the magnetic tape, we present evidence for axial magnetic fields and map out the spatial distribution of these fields around the laser generated plasma. By using the axial magnetic field distribution as a diagnostic tool we uncover evidence for angular momentum associated with the plasma.

Instabilities in the vortex matter and the peak effect phenomenon.

In single crystals of 2H-NbSe2, we identify for the first time a crossover from a weak collective to a strong pinning regime in the vortex state which is not associated with the peak effect phenomenon. Instead, we find the crossover is associated with an anomalous history dependent magnetization response. In the dc magnetic field (Bdc)-temperature (T) vortex matter phase diagram we demarcate this pinning crossover boundary. We also delineate another boundary which separates the strong pinning region from a thermal fluctuation dominated regime, and find that a peak effect appears on this boundary.