PP39. 5-AMINOLEVULINIC ACID AIDED RESECTION OF PAEDIATRIC BRAIN TUMOURS: THE UK’S FIRST CASE SERIES

Abstract Long term survival following paediatric brain tumours has vastly improved in recent decades. Consequently there is a drive towards improved quality of survivorship. Brain tumours, surgical resection and adjuvant therapies represent mechanisms for brain injury and can therefore negatively impact a child’s neuropsychological trajectory; affecting cognition, behaviour, emotional and adaptive functioning and educational/occupational outcomes. A biopsychosocial approach to rehabilitation should target each of these domains through supported remediation, environmental modification and psychoeducation for young people and the key systems around them (e.g. families, education). There is a growing evidence base for the role of concordant psychopharmacologies to improve neuropsychological outcome. Since 2015 children treated at RHSC Edinburgh for brain tumours have been offered pharmacotherapy alongside usual rehabilitation approaches if they demonstrate significant difficulties with Attention, Processing Speed and/or Executive Function on formal neuropsychological assessment. Patients are referred to a Consultant Psychiatrist or Paediatrician (as per local protocol) for medication selection, titration and monitoring. A short case series (N=14) is presented outlining brain tumour pathologies, treatment modalities, neuropsychological profile and rationale for recommending pharmacotherapy. Approximately 50% of patients took up the offer. The treatment/s offered and self or parents reported outcomes is summarised. Pharmacotherapy was broadly effective; “it’s been like night and day”, although for one case (N=1) the side effects outweighed any benefit; “she became even more emotional”. Findings indicate that pharmacotherapy should be considered alongside conventional neurorehabilitation techniques for CYP with specific cognitive difficulties following treatment for paediatric brain tumours.

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Clinical value of amino acid imaging in paediatric brain tumours

Nuklearmedizin ◽

10.1055/s-0038-1625755 ◽

2005 ◽

Vol 44 (04) ◽

pp. 131-136 ◽

Cited By ~ 3

Author(s):

K. Lang ◽

S. Kloska ◽

R. Straeter ◽

C. H. Rickert ◽

G. Goder ◽

...

Keyword(s):

Amino Acid ◽

Brain Tumours ◽

Photon Emission ◽

Diagnostic Tools ◽

Emission Computed Tomography ◽

Gadolinium Enhancement ◽

Tumour Grading ◽

Enhanced Mri ◽

Contrast Enhanced ◽

Paediatric Brain Tumours

Summary Purpose: To evaluate single photon emission computed tomography (SPECT) using the amino acid l-3-[123I]-α-methyl tyrosine (IMT) and contrast enhanced magnetic resonance imaging (MRI) as diagnostic tools in primary paediatric brain tumours in respect of non-invasive tumour grading. Patients, materials, methods: 45 children with primary brain tumours were retrospectively evaluated. IMT uptake was quantified as tumour/nontumour- ratio, a 4-value-scale was used to measure gadolinium enhancement on contrast enhanced MRI. Statistical analyses were performed to evaluate IMT uptake and gadolinium enhancement in low (WHO I/II) and high (WHO III/ IV) grade tumours and to disclose a potential relationship of IMT uptake to disruption of blood brain barrier as measured in corresponding MRI scans. Results: IMT uptake above background level was observed in 35 of 45 patients. IMT uptake was slightly higher in high grade tumours but the difference failed to attain statistical significance. Grading of individual tumours was neither possible by IMT SPECT nor by gadolinium enhanced MRI. Conclusion: IMT is accumulated in most brain tumours in children. Tumour grading was not possible using IMT or contrast enhancement as determined by MRI. Neither morphological nor functional imaging can replace histology in paediatric brain tumours.

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Cystoisospora belli infection: the first case series in Thailand

10.26226/morressier.56d5ba2fd462b80296c953a9 ◽

2016 ◽

Author(s):

Benjawan Skulsujirapa

Keyword(s):

Case Series ◽

First Case

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Sodium-Glucose Cotransporter-2 Inhibitors in Patients with Hereditary Podocytopathies, Alport Syndrome, and FSGS: A Case Series to Better Plan a Large-Scale Study

Cells ◽

10.3390/cells10071815 ◽

2021 ◽

Vol 10 (7) ◽

pp. 1815

Author(s):

Jan Boeckhaus ◽

Oliver Gross

Keyword(s):

Glomerular Filtration ◽

Alport Syndrome ◽

Large Scale ◽

Case Series ◽

Hereditary Diseases ◽

Glomerular Filtration Barrier ◽

Early Effect ◽

Filtration Barrier ◽

First Case ◽

Sodium Glucose Cotransporter

Hereditary diseases of the glomerular filtration barrier are characterized by a more vulnerable glomerular basement membrane and dysfunctional podocytes. Recent clinical trials have demonstrated the nephroprotective effect of sodium-glucose cotransporter-2 inhibitors (SGLT2i) in chronic kidney disease (CKD). SGLT2-mediated afferent arteriole vasoconstriction is hypothesized to correct the hemodynamic overload of the glomerular filtration barrier in hereditary podocytopathies. To test this hypothesis, we report data in a case series of patients with Alport syndrome and focal segmental glomerulosclerosis (FSGS) with respect of the early effect of SGLT2i on the kidney function. Mean duration of treatment was 4.5 (±2.9) months. Mean serum creatinine before and after SGLT-2i initiation was 1.46 (±0.42) and 1.58 (±0.55) mg/dL, respectively, with a median estimated glomerular filtration rate of 64 (±27) before and 64 (±32) mL/min/1.73 m2 after initiation of SGLT2i. Mean urinary albumin-creatinine ratio in mg/g creatinine before SGLT-2i initiation was 1827 (±1560) and decreased by almost 40% to 1127 (±854) after SGLT2i initiation. To our knowledge, this is the first case series on the effect and safety of SGLT2i in patients with hereditary podocytopathies. Specific large-scale trials in podocytopathies are needed to confirm our findings in this population with a tremendous unmet medical need for more effective, early on, and safe nephroprotective therapies.

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Clinical, Laboratory and Histological Features of Dipeptidyl Peptidase-4 Inhibitor Related Noninflammatory Bullous Pemphigoid

Journal of Clinical Medicine ◽

10.3390/jcm10091916 ◽

2021 ◽

Vol 10 (9) ◽

pp. 1916

Author(s):

Ágnes Kinyó ◽

Anita Hanyecz ◽

Zsuzsanna Lengyel ◽

Dalma Várszegi ◽

Péter Oláh ◽

...

Keyword(s):

Bullous Pemphigoid ◽

Clinical Laboratory ◽

Case Series ◽

Dipeptidyl Peptidase ◽

Area Index ◽

Histological Features ◽

Dipeptidyl Peptidase 4 ◽

First Case ◽

Dipeptidyl Peptidase 4 Inhibitor ◽

The Mean

Bullous pemphigoid (BP) is an autoimmune blistering disease of elderly patients that has shown increasing incidence in the last decades. Higher prevalence of BP may be due to more frequent use of provoking agents, such as antidiabetic dipeptidyl peptidase-4 inhibitor (DPP4i) drugs. Our aim was to assess DPP4i-induced bullous pemphigoid among our BP patients and characterize the clinical, laboratory and histological features of this drug-induced disease form. In our patient cohort, out of 127 BP patients (79 females (62.2%), 48 males (37.7%)), 14 (9 females and 5 males) were treated with DPP4i at the time of BP diagnosis. The Bullous Pemphigoid Disease Area Index (BPDAI) urticaria/erythema score was significantly lower, and the BPDAI damage score was significantly higher in DPP4i-BP patients compared to the nonDPP4i group. Both the mean absolute eosinophil number and the mean periblister eosinophil number was significantly lower in DPP4i-BP patients than in nonDPP4i cases (317.7 ± 0.204 vs. 894.0 ± 1.171 cells/μL, p < 0.0001; 6.75 ± 1.72 vs. 19.09 ± 3.1, p = 0.0012, respectively). Our results provide further evidence that DPP4i-associated BP differs significantly from classical BP, and presents with less distributed skin symptoms, mild erythema, normal or slightly elevated peripheral eosinophil count, and lower titers of BP180 autoantibodies. To our knowledge, this is the first case series of DPP4i-related BP with a non-inflammatory phenotype in European patients.

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Classification of paediatric brain tumours by diffusion weighted imaging and machine learning

Scientific Reports ◽

10.1038/s41598-021-82214-3 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Jan Novak ◽

Niloufar Zarinabad ◽

Heather Rose ◽

Theodoros Arvanitis ◽

Lesley MacPherson ◽

...

Keyword(s):

Diffusion Coefficient ◽

Apparent Diffusion Coefficient ◽

Posterior Fossa ◽

Brain Tumours ◽

Diffusion Weighted Imaging ◽

Regions Of Interest ◽

Apparent Diffusion Coefficients ◽

Diffusion Weighted ◽

Apparent Diffusion ◽

Paediatric Brain Tumours

AbstractTo determine if apparent diffusion coefficients (ADC) can discriminate between posterior fossa brain tumours on a multicentre basis. A total of 124 paediatric patients with posterior fossa tumours (including 55 Medulloblastomas, 36 Pilocytic Astrocytomas and 26 Ependymomas) were scanned using diffusion weighted imaging across 12 different hospitals using a total of 18 different scanners. Apparent diffusion coefficient maps were produced and histogram data was extracted from tumour regions of interest. Total histograms and histogram metrics (mean, variance, skew, kurtosis and 10th, 20th and 50th quantiles) were used as data input for classifiers with accuracy determined by tenfold cross validation. Mean ADC values from the tumour regions of interest differed between tumour types, (ANOVA P < 0.001). A cut off value for mean ADC between Ependymomas and Medulloblastomas was found to be of 0.984 × 10−3 mm2 s−1 with sensitivity 80.8% and specificity 80.0%. Overall classification for the ADC histogram metrics were 85% using Naïve Bayes and 84% for Random Forest classifiers. The most commonly occurring posterior fossa paediatric brain tumours can be classified using Apparent Diffusion Coefficient histogram values to a high accuracy on a multicentre basis.

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Challenging Presentations of Seven Cases of Gastrointestinal Basidiobolomycosis in Sudan: Clinical Features, Histology, Imaging, and Recommendations

Journal of Laboratory Physicians ◽

10.1055/s-0040-1721149 ◽

2020 ◽

Vol 12 (04) ◽

pp. 281-284

Author(s):

Sawsan A. Mohammed ◽

Azza A. Abdelsatir ◽

Mohamed Abdellatif ◽

Suliman Hussein Suliman ◽

Omer Mohammed Ibrahim Elbasheer ◽

...

Keyword(s):

Gastrointestinal Tract ◽

Fungal Infection ◽

Clinical Features ◽

Subcutaneous Tissue ◽

Case Series ◽

First Case

AbstractsBasidiobolomycosis is a fungal infection caused by Basidiobolus ranarum which affects the skin and subcutaneous tissue and rarely the gastrointestinal tract. We report seven cases of gastrointestinal basidiobolomycosis with interesting clinical, radiological, and histological presentations. To our knowledge, this is the first case series of abdominal basidiobolomycosis to be reported from Sudan.

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FIRST CASE SERIES OF VENOUS SINUS STENTING IN IIH PATIENTS IN WALES

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2015-312379.25 ◽

2015 ◽

Vol 86 (11) ◽

pp. e4.113-e4

Author(s):

Gauhar Abbas Malik ◽

Yogish Joshi

Keyword(s):

Intracranial Hypertension ◽

Case Series ◽

Headache Disorder ◽

Venous Sinus ◽

First Line ◽

Venous Stenting ◽

First Case ◽

Sinus Stenosis

BackgroundIdiopathic Intracranial Hypertension (IIH), is defined by increased cerebral spinal fluid (CSF) pressure in the absence of other causes of intracranial hypertension. There has been recent interest in the role of intracranial venous sinus stenosis in IIH. The raised pressures in IIH are argued to worsen by the secondary appearance of the venous sinus stenosis.Objective5 patients have undergone endovascular pressure measurement in Wales and their clinical details including history, examination, initial management, neuroimaging pre- and post venous stenting, and follow-up (6–24 months) to provide the first case study of patients undergoing Venous sinus stenting in Wales.Methods5 patients with IIH refractory to first line treatments underwent venography and manometry and 4 patients underwent stenting of the venous sinuses after this procedure had shown a pressure gradient proximal to stenosis in the lateral sinuses.ResultsThree patients were rendered asymptomatic, two were improved including one patient unmasking a different headache disorder following treatment.ConclusionsStenting in venous stenosis provides a further treatment option to patients refractory to first line treatments with IIH. This case series highlights in selected cases treatment is promising with good outcomes.

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Calcaneal Reconstruction With Free Deep Circumflex Iliac Artery Osseocutaneous Flap Following Aggressive Benign Bone Tumor Resection

Foot & Ankle International ◽

10.1177/10711007211025280 ◽

2021 ◽

pp. 107110072110252

Author(s):

Mohamad Aizat Rosli ◽

Wan Faisham Wan Ismail ◽

Wan Azman Wan Sulaiman ◽

Nor Azman Mat Zin ◽

Syurahbil Abdul Halim ◽

...

Keyword(s):

Iliac Artery ◽

Donor Site ◽

Tumor Resection ◽

Case Series ◽

Benign Tumors ◽

Reconstruction Method ◽

Short Term ◽

Deep Circumflex Iliac Artery ◽

First Case ◽

Donor Site Pain

Background: Advances in limb-salvage techniques have made total calcanectomy and primary reconstruction possible in managing calcaneal aggressive benign tumors and selected cases of intraosseous malignant tumors. However, there is still no consensus on the operative approach, oncologic margin, and the best reconstruction method to date. These 2 cases describe our experience in calcaneal reconstruction with the free deep circumflex iliac artery (DCIA) osseocutaneous flap in benign aggressive calcaneal tumors. Methods: We reported 2 consecutive male and female patients, with an average age of 25 years (age 19 and 31, respectively), who underwent total calcanectomy and primary calcaneal reconstruction with the free DCIA osseocutaneous flaps for calcaneal chondroblastoma and giant cell tumor. A marginal resection of the entire calcaneus through the subtalar and calcaneocuboid joints (intra-articular approach) was performed in the first case and a wide local resection leaving 1 cm normal calcaneal bone margin anterosuperiorly (intraosseous approach) was performed in the second case. Results: The follow-up period averaged 48 months. Negative oncologic margins were achieved in both cases. The first case was complicated with venous thrombosis; however, the graft remained viable after emergency reexploration. Normal foot function was restored with good solid osseous union and bony hypertrophy observed. Both patients achieved good short-term functional and aesthetic outcomes with no donor site pain or disability. No local recurrence was reported either. Conclusion: Primary calcaneal reconstruction with the free DCIA osseocutaneous flap can lead to good short-term functional and aesthetic outcomes. Level of Evidence: Level IV, case series.

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Medication-Related Osteonecrosis of the Jaw (MRONJ): Are Antiresorptive Drugs the Main Culprits or Only Accomplices? The Triggering Role of Vitamin D Deficiency

Nutrients ◽

10.3390/nu13020561 ◽

2021 ◽

Vol 13 (2) ◽

pp. 561

Author(s):

Luca Dalle Carbonare ◽

Monica Mottes ◽

Maria Teresa Valenti

Keyword(s):

Vitamin D ◽

Clinical Condition ◽

Case Series ◽

Osteonecrosis Of The Jaw ◽

First Case ◽

Vitamin D Levels ◽

Exposed Bone ◽

Antiresorptive Drugs ◽

Almost All

Osteonecrosis of the jaw (ONJ) is a severe clinical condition characterized mostly but not exclusively by an area of exposed bone in the mandible and/or maxilla that typically does not heal over a period of 6–8 weeks. The diagnosis is first of all clinical, but an imaging feedback such as Magnetic Resonance is essential to confirm clinical suspicions. In the last few decades, medication-related osteonecrosis of the jaw (MRONJ) has been widely discussed. From the first case reported in 2003, many case series and reviews have appeared in the scientific literature. Almost all papers concerning this topic conclude that bisphosphonates (BPs) can induce this severe clinical condition, particularly in cancer patients. Nevertheless, the exact mechanism by which amino-BPs would be responsible for ONJ is still debatable. Recent findings suggest a possible alternative explanation for BPs role in this pattern. In the present work we discuss how a condition of osteomalacia and low vitamin D levels might be determinant factors.

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