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Biomedical subjects

Ajay Gupta

Publications and source records attributed to Ajay Gupta.

At least 19 recordsLinked to original sources
PubMed2007

Fluctuating Kluver-Bucy syndrome in a child with epilepsy due to bilateral anterior temporal congenital malformations.

An 11-year-old boy with epilepsy due to congenital bilateral anterior temporal lobe malformations presented with fluctuating Kluver-Bucy syndrome (KBS). Since the age of 2, he had experienced clusters of three or four daily complex partial seizures over 2-3 days in a month, followed by a seizure-free interval of 3-4 weeks. During the seizure-free period, the patient exhibited hyperorality, sniffing, irritability alternating with placidity, anxiety, unsolicited sexual gestures, and unusual calmness after eating. KBS features escalated up to the onset of the seizure cluster, and remitted after the seizures. Brain MRI revealed bilateral anterior temporal cortical dysplasia with enlarged and dysmorphic amygdalar-hippocampal complex. Brain [(18)F]fluorodeoxyglucose positron emission tomography PET showed bilateral anterior and mesial temporal hypometabolism. Video/EEG monitoring revealed independent right and left temporal lobe seizures. This is the first reported case of KBS due to congenital bitemporal malformations. Also, KBS behavior phenotype in this patient fluctuated, with escalation during the seizure-free period and remission induced by the monthly seizure cluster. This fluctuating pattern could represent forced normalization.

Brain↗
PubMed2006

Subperiosteal hemangioendothelioma of the femur.

Primary neoplastic vascular lesions of bone are rare, and include haemangiomas, haemangioendothelioma, epithelioid haemangioendothelioma and angiosarcoma. These lesions may be multicentric, and when they involve bone are typically intraosseous and lytic. Radiological findings are not always specific. We report a case of haemangioendothelioma localised to the subperiosteum of the femur, a site not previously described for this lesion. The nomenclature for vascular neoplasms in bone is discussed.

Adult↗
PubMed2006

Acute myeloid leukemia with severe aplastic anemia following immunosuppressive therapy.

Severe aplastic anemia (AA) is a life-threatening condition wherein bone marrow transplantation (BMT) is the therapy of choice in a young patient who has a matched sibling donor. Here, we report an 11-year-old boy with severe AA who was referred for BMT late in its course when he had developed acute myeloid leukemia following two courses of immunosuppressive therapy with antithymocyte globulin and cyclosporin. He was then treated with induction therapy using cytosine arabinoside and daunomycin for acute myeloid leukemia, but he succumbed due to infection and refractory leukemia. We discuss the relevance of early referral for BMT in severe AA.

Anemia, Aplastic↗
PubMed2006

Computerized tomography guided percutaneous renal cryoablation with the patient under conscious sedation: initial clinical experience.

PURPOSE: We report on our initial clinical experience with CT guided percutaneous renal cryoablation. MATERIALS AND METHODS: CT guided percutaneous renal cryoablation was performed on 27 tumors using conscious sedation in 20 patients. Eligible patients had tumors of 5 cm or less and were poor surgical candidates or otherwise warranted nephron sparing treatment. Tumors were classified as central or noncentral depending on their relationship to the renal sinus fat. During cryoablation intraoperative active ice ball formation was monitored with real-time CT imaging to ensure adequate tumor coverage. Postoperative followup imaging was obtained at regular intervals. RESULTS: Our method appears technically feasible as of the 27 cryoablations performed, only 1 complication occurred requiring blood transfusion in a patient with a large, centrally located tumor. To date we have 16 tumors in 12 patients with imaging followup of 1 month or more (mean followup 5.9 months). Mean baseline tumor size in this group was 2.5 cm with 11 small (3 cm or less) and 5 large (more than 3 cm) tumors. Of the tumors 5 were centrally located and 11 were noncentrally located. Preliminary data suggest that of the 16 cryoablated tumors 15 showed no signs of enhancement on followup. CONCLUSIONS: CT guided percutaneous renal cryoablation appears to be a feasible treatment option for small, noncentrally located renal tumors. While early results appear promising, longer followup is needed to more clearly define the role of this treatment method.

Adult↗
PubMed2006

Cost analysis of open, laparoscopic, and percutaneous treatment options for nephron-sparing surgery.

BACKGROUND AND PURPOSES: A variety of nephron-sparing options exist for the management of small renal masses. The perioperative cost of open (OPN) and laparoscopic (LPN) partial nephrectomy, laparoscopic (LCA), and CT-guided percutaneous (PCA) cryoablation was compared using a detailed computer model. PATIENTS AND METHODS: The model incorporates operative time, consumables, anesthesia, CT usage, percutaneous biopsy, hospitalization, and transfusion expenses. Starting values were derived from a retrospective review of 317 patients treated at the Johns Hopkins Medical Institutions within the past 7 years. Hypothesis testing was performed with sensitivity analysis. RESULTS: The PCA was 2.2 to 2.7 times less costly than the other options and resulted in a cost savings of $3625 to $5155 per case. For OPN, LPN, and LCA, the operative time and hospitalization accounted for 69% to 91% of the cost. In contrast, cryoprobe consumables were responsible for >70% of total cost of PCA. An OPN was 1.2x as costly as LPN and could achieve cost equivalence only with operative times of <2.8 hours or hospitalization of <3 days. An LCA was more costly than all forms of extirpative surgery if more than two cryoprobes were used. Reusing cryoprobes during LCA was always a less-costly option than adding a second cryoprobe to the procedure. The LCA was no longer cost advantageous over OPN if more than four CT scans were obtained during the first postoperative year or if local recurrence rates exceeded 23%. CONCLUSIONS: This model defines and simplifies a series of complex cost relations between the options for nephron-sparing surgery.

Costs and Cost Analysis↗
PubMed2006

A case of auditory auras: application of general principles to define and localize the epileptogenic zone.

An illustrative case of auditory aura and complex partial seizures is presented to highlight challenges in the accurate localization of the epileptogenic zone. Application and interpretation of various diagnostic tools is discussed in this case using the North American approach to the localization of the epileptogenic zone. Whenever possible, the differences and similarities between the North American and the French/Italian approach for the localization of the epileptogenic zone are discussed.

Adult↗
PubMed2005

A fatal fungus.

We report the case of a 66-year-old man who presented with a prodromal type illness and pre-sternal swelling after having coronary artery bypass grafts 4 years earlier. Computed tomography showed that the mass had a retrosternal extension, to join a collection anterior to the right ventricle. Candida famata was isolated from operative specimens and he was treated with aggressive antifungal therapy. We believe that this is the first reported case of mediastinal Candida famata.

Abscess↗
PubMed2005

How much can carpus rotate axially? An in vivo study.

BACKGROUND: Supination and pronation movements occur primarily at the forearm though are possible at the wrist joint too. The axial rotation of the wrist also called the radiometacarpal rotation has been quantified but for its passive range which may never occur during the day-to-day routine activities. It is normally not possible for the wrist joint to rotate axially in an active manner. However, voluntary effort to rotate the forearm while keeping the hand fixed on a custom designed device is able to provide active rotation of the wrist which occurs in a manner similar to that occurring during the daily routine activities. METHODS: The present study measured axial rotation of the wrist in 20 asymptomatic volunteers who had axial CT done of their wrist with elbow in 10-30 degrees flexion and forearm positioned parallel to the long axis of the table with thumb pointing up towards the roof. The examination was repeated twice while the subject actively tried to supinate and pronate the forearm against the fixed hand and the metacarpals using maximum voluntary effort on a custom designed positioning device. FINDINGS: The mean radiometacarpal supination and pronation were 17.15 degrees (SD 7.9) and 17.0 degrees (SD 10) respectively. The movement was found to occur predominantly at midcarpal joint with radiocarpal joint contributing only 18% to supination and 31% to pronation. INTERPRETATION: The radiometacarpal rotation has a crucial bearing in the development of the wrist prostheses. The design of the prostheses should consider accommodating axial movements that occur in the carpus during the activities of daily living.

Activities of Daily Living↗
PubMed2005

The effect of inadequate in situ perfusion in the non heart-beating donor.

In situ aortic perfusion in the nonheart-beating donors (NHBD) is an important procedure to reduce primary warm ischaemic injury prior to formal donor organ retrieval. It allows an interim period to obtain donor family consent and theatre preparation. This study describes our experience of inadequate aortic perfusions resulting from difficult aortic cannulations and associated adverse outcome despite reasonable viability tests. Since 1998, all NHBD in our institution are perfused in situ using a double balloon triple lumen (DBTL) catheter inserted through a femoral artery cut-down procedure. The DBTL catheter is positioned with distal occlusive balloon at the aortic bifurcation using the "pull-back" technique, the proximal occlusive balloon lies above the renal arteries. This provides selective aortic perfusion in particular the kidneys. Venous decompression using a femoral vein catheter enables a "two-way infusion system". Pre-transplant viability status of retrieved kidneys is determined by measuring pressure/resistance characteristics to the flow and biochemical markers for ischaemic injury. There were 90 NHBD renal transplants performed from 72 donors. Three renal transplants were carried out from three donors of ineffective in situ perfusion secondary to cannulation difficulties. Femoral cannulation was difficult as a result of extensive atherosclerosis of donor vessels. The comparison of allograft outcome from effective and ineffective in situ perfusion of donors showed high rate of primary nonfunction (PNF) from ineffective perfusion (chi-squared, P < 0.0001). The cases demonstrated poor outcome from ineffective perfusion related to the cannulation difficulties. Therefore a strict policy should be taken in cases where aortic cannulation and perfusion is inadequate, despite pretransplant assessment. In these circumstances, the primary warm ischaemia time should be extended to include this period of ineffective perfusion.

Adult↗
PubMed2005

Serum fetuin-A in nondialyzed patients with diabetic nephropathy: relationship with coronary artery calcification.

BACKGROUND: Fetuin-A is the most potent circulating inhibitor of calcium phosphorus precipitation and, possibly, an important mediator of insulin resistance. METHODS: In order to determine the role of fetuin-A in the high coronary artery calcification (CAC) burden seen in nondialyzed individuals with diabetic nephropathy (DN), post-hoc analyses of data collected from a cross-sectional study of 88 patients with type 2 diabetes mellitus was done [age, 40-65 years; normoalbuminuria, N= 30 (Latinos); DN, N= 58 (Latinos and African Americans)]. RESULTS: The serum levels of fetuin-A were significantly higher among Latinos with DN when compared to either African Americans with DN or Latino diabetics with normoalbuminuria. Upon adjusting the data for race/ethnicity, there was a strong, direct relationship between serum fetuin-A levels and the CAC score (r= 0.22, P= 0.038) in the study cohort; however, a strong interaction between the nephropathy status and relationship of serum fetuin-A levels with CAC score was present (DN: r= 0.36, P= 0.006; diabetic controls, r= 0.0, P= 0.98). Among individuals with DN, the significance of the association persisted even after controlling the data for other predictors of CAC (partial r= 0.33, P= 0.018). Furthermore, there was a significant direct relationship between serum fetuin-A and serum triglycerides (partial r= 0.27, P= 0.01) and albumin (partial r= 0.30, P= 0.005), and an inverse relationship with glomerular filtration rate (r=-0.24, P= 0.03). CONCLUSION: This first study in early stages of diabetic chronic kidney disease shows that the role of serum fetuin-A may be far more complex than previously described. During predialysis stage of DN, there is a direct relationship between serum fetuin-A levels and CAC score. The reasons for this association in the presence of nephropathy are unclear, but may be secondary to proatherogenic insulin resistance.

Adult↗
PubMed2005

Hemispherectomy for catastrophic epilepsy in infants.

PURPOSE: To report our experience with hemispherectomy in the treatment of catastrophic epilepsy in children younger than 2 years. METHODS: In a single-surgeon series, we performed a retrospective analysis of 18 patients with refractory epilepsy undergoing hemispherectomy (22 procedures). Three different surgical techniques were performed: anatomic hemispherectomy, functional hemispherectomy, and modified anatomic hemispherectomy. Pre- and postoperative evaluations included extensive video-EEG monitoring, magnetic resonance imaging, and positron emission tomography scanning. Seizure outcome was correlated with possible variables associated with persistent postoperative seizures. The Generalized Estimation Equation (GEE) and the Barnard's exact test were used as statistical methods. RESULTS: The follow-up was 12-74 months (mean, 34.8 months). Mean weight was 9.3 kg (6-12.3 kg). The population age was 3-22 months (mean, 11.7 months). Thirteen (66%) patients were seizure free, and four patients had >90% reduction of the seizure frequency and intensity. The overall complication rate was 16.7%. No deaths occurred. Twelve (54.5%) of 22 procedures resulted in incomplete disconnection, evidenced on postoperative images. Type of surgical procedure, diagnosis categories, persistence of insular cortex, and bilateral interictal epileptiform activity were not associated with persistent seizures after surgery. Incomplete disconnection was the only variable statistically associated with persistent seizures after surgery (p<0.05). CONCLUSIONS: Hemispherectomy for seizure control provides excellent and dramatic results with a satisfactory complication rate. Our results support the concept that early surgery should be indicated in highly selected patients with catastrophic epilepsy. Safety factors such as an expert team in the pediatric intensive care unit, neuroanesthesia, and a pediatric epilepsy surgeon familiar with the procedure are mandatory.

Cerebral Cortex↗
PubMed2005

Osteoblast-derived PTHrP is a potent endogenous bone anabolic agent that modifies the therapeutic efficacy of administered PTH 1-34.

Mice heterozygous for targeted disruption of Pthrp exhibit, by 3 months of age, diminished bone volume and skeletal microarchitectural changes indicative of advanced osteoporosis. Impaired bone formation arising from decreased BM precursor cell recruitment and increased apoptotic death of osteoblastic cells was identified as the underlying mechanism for low bone mass. The osteoporotic phenotype was recapitulated in mice with osteoblast-specific targeted disruption of Pthrp, generated using Cre-LoxP technology, and defective bone formation was reaffirmed as the underlying etiology. Daily administration of the 1-34 amino-terminal fragment of parathyroid hormone (PTH 1-34) to Pthrp+/- mice resulted in profound improvement in all parameters of skeletal microarchitecture, surpassing the improvement observed in treated WT littermates. These findings establish a pivotal role for osteoblast-derived PTH-related protein (PTHrP) as a potent endogenous bone anabolic factor that potentiates bone formation by altering osteoblast recruitment and survival and whose level of expression in the bone microenvironment influences the therapeutic efficacy of exogenous PTH 1-34.

Animals↗
PubMed2005

A proposal for a five-dimensional patient-oriented epilepsy classification.

The recent proposal by the ILAE Task Force for Epilepsy Classification consists of a multi-axial syndrome-oriented approach. Epilepsy syndromes, as defined by the ILAE, group patients according to various, poorly defined parameters. The resulting syndromes have frequently no biological significance, with overlap among different syndromes and syndromes changing with age. Additionally, only a minority of patients can be classified syndromatically, and the axes of this classification system convey redundant information. We propose a five-dimensional, patient-oriented approach to classifying epilepsies. This approach shifts from the syndrome-oriented approach to a standard, neurological, methodological, patient-oriented approach, using independent criteria in each of the five dimensions. Similar to general neurology, the first step in each patient-physician encounter in epileptology is to take a history of the presenting symptoms and generate a hypothesis regarding the localization and etiology of the symptom within the nervous system. Therefore, the main dimensions of this classification consist of: 1) localization of the epileptogenic zone, 2) seizure semiology classified according to the semiological seizure classification, 3) etiology, 4) seizure frequency, and 5) related medical conditions. These dimensions characterize all of the information necessary for patient management, are independent parameters, and include more pertinent information with regards to patient management than the ILAE axes. All patients can be classified according to this five-dimensional system even at the initial patient encounter when no detailed test results are available. Information from clinical tests, such as MRI and EEG, are translated into the best possible working hypothesis at the time of classification, allowing for increasing precision of the classification as additional information becomes available. This patient-oriented classification envisions an epileptic seizure as an independent symptom of a central nervous system dysfunction due to different causes, with various cortical localizations, occurring at various frequencies, and in conjunction with other diseases and clinical symptoms.

Adult↗
PubMed2004

Chromatin remodeling factors and BRM/BRG1 expression as prognostic indicators in non-small cell lung cancer.

We immunohistochemically examined 12 core proteins involved in the chromatin remodeling machinery using a tissue microarray composed of 150 lung adenocarcinoma (AD) and 150 squamous cell carcinoma (SCC) cases. Most of the proteins showed nuclear staining, whereas some also showed cytoplasmic or membranous staining. When the expression patterns of all tested antigens were considered, proteins with nuclear staining clustered into two major groups. Nuclear signals of BRM, Ini-1, retinoblastoma, mSin3A, HDAC1, and HAT1 clustered together, whereas nuclear signals of BRG1, BAF155, HDAC2, BAF170, and RbAP48 formed a second cluster. Additionally, two thirds of the cases on the lung tissue array had follow-up information, and survival analysis was performed for each of the tested proteins. Positive nuclear BRM (N-BRM) staining correlated with a favorable prognosis in SCC and AD patients with a 5 year-survival of 53.5% compared with 32.3% for those whose tumors were negative for N-BRM (P = 0.015). Furthermore, patients whose tumors stained positive for both N-BRM and nuclear BRG1 had a 5 year-survival of 72% compared with 33.6% (P = 0.013) for those whose tumors were positive for either or negative for both markers. In contrast, membranous BRM (M-BRM) staining correlated with a poorer prognosis in AD patients with a 5 year-survival of 16.7% compared with those without M-BRM staining (38.1%; P = 0.016). These results support the notion that BRM and BRG1 participate in two distinct chromosome remodeling complexes that are functionally complementary and that the nuclear presence of BRM, its coexpression with nuclear BRG1, and the altered cellular localization of BRM (M-BRM) are useful markers for non-small cell lung cancer prognosis.

Adenocarcinoma↗
PubMed2004

Hemispheric malformations of cortical development.

The term hemispheric malformations of cortical development (MCDs) is used in this article to represent congenital malformations that predominantly or exclusively involve complete or substantial portion of one cerebral hemisphere. Hemispheric MCDs usually present during early childhood with intractable epilepsy, cognitive delay, and contralateral hemiparesis. Early identification and selection of children who may be candidates for epilepsy surgery are important during diagnostic evaluation of hemispheric MCD. Surgical intervention at an appropriate time, whenever possible, offers the best chance for seizure freedom and improved cognitive outcome. In addition, making an accurate genetic diagnosis is imperative to offer genetic testing and counseling to the family. In this article, the authors discuss the developmental and genetic mechanisms, pathologic features, diagnosis, and treatment of hemispheric MCD that are substrates of catastrophic childhood epilepsy amenable to surgical treatment.

Brain Diseases↗
PubMed2004

Ictal SPECT in children with partial epilepsy due to focal cortical dysplasia.

We studied the usefulness of ictal single-photon emission computed tomography in the presurgical evaluation of children with partial epilepsy resulting from focal cortical dysplasia. Fifteen children, age 1-18 years, were identified with partial epilepsy caused by focal cortical dysplasia (confirmed by histology) who underwent subtraction ictal single-photon emission computed tomography during presurgical evaluation. All children later underwent surgery at the Cleveland Clinic Epilepsy Center between 1996 and 2000. The findings of ictal single-photon emission computed tomography and brain positron emission tomography were classified as localized when "localizing and concordant" with the surgical resection site, nonconcordant when "localizing but not concordant" with the surgical resection, or nonlocalized when "no well-localized region of ictal hyperperfusion was observed on the difference image". In 15 patients, age 1.5-18 years (median age 8 years), epilepsy was classified as frontal in 7, posterior temporal/occipital in 3, temporal in 2, multilobar in 2, and parietal in 1. Of 15 patients, preoperative magnetic resonance imaging revealed focal cortical dysplasia in 11, positron emission tomography was localized in 9, and ictal single-photon emission computed tomography was localized in 8 patients. In 4 patients with normal magnetic resonance imaging but scalp electroencephalographic findings of partial epilepsy, ictal single-photon emission computed tomography and positron emission tomography were localized in 3 each. Fourteen patients were monitored for 6-39 months (mean 20 months). Six of 7 patients (85%) with localized ictal single-photon emission computed tomography compared with 4 of 7 (57%) with nonconcordant/nonlocalized ictal single-photon emission computed tomography had no seizures at follow-up. In 4 patients with normal magnetic resonance imaging, 3 patients with localized ictal single-photon emission computed tomography were free of seizures compared with 1 with nonconcordant ictal single-photon emission computed tomography who continued to have seizures. Ictal single-photon emission computed tomography is a useful adjunctive test in presurgical evaluation of children with refractory partial epilepsy due to focal cortical dysplasia, especially when brain magnetic resonance imaging is normal.

Adolescent↗