Publications by authors named "Amit Soni"

19 Publications

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Lower Gastrointestinal Bleeding: Liver Rams into Gut!

GE Port J Gastroenterol 2019 May 27;26(3):218-220. Epub 2018 Apr 27.

Department of Gastroenterology and Liver Disease, SPS Hospital, Ludhiana, India.

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http://dx.doi.org/10.1159/000488605DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6528081PMC
May 2019

Baffling Cause of Upper Gastrointestinal Bleeding!

GE Port J Gastroenterol 2019 Mar 27;26(2):142-144. Epub 2018 Apr 27.

Department of Gastroenterology and Liver Disease, SPS Hospital, Ludhiana, India.

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http://dx.doi.org/10.1159/000488604DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454382PMC
March 2019

Central retinal vein occlusion revealing celiac disease: The first report of two cases from India.

Indian J Ophthalmol 2018 09;66(9):1315-1317

Department of Cataract and Refractive Surgery, Dhami Eye Care Hospital, Ludhiana, Punjab, India.

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http://dx.doi.org/10.4103/ijo.IJO_351_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6113809PMC
September 2018

Reliability of patient-reported outcome instruments in US adults with hemophilia: the Pain, Functional Impairment and Quality of life (P-FiQ) study.

Patient Prefer Adherence 2017 19;11:1603-1612. Epub 2017 Sep 19.

Clinical, Medical and Regulatory Affairs, Novo Nordisk Inc., Plainsboro, NJ, USA.

Background: Hemophilia is marked by frequent joint bleeding, resulting in pain and functional impairment.

Objective: This study aimed to assess the reliability of five patient-reported outcome (PRO) instruments in people with hemophilia (PWH) in a non-bleeding state.

Methods: Adult male PWH of any severity and inhibitor status, with a history of joint pain or bleeding, completed a pain history and five PRO instruments (EQ-5D-5L, Brief Pain Inventory v2 [BPI], International Physical Activity Questionnaire [IPAQ], Short Form 36 Health Survey v2 [SF-36v2], and Hemophilia Activities List [HAL]) during their routine comprehensive care visit. Patients were approached to complete the PRO instruments again at the end of their visit while in a similar non-bleeding state. Concordance of individual questionnaire items and correlation between domain scores were assessed using intra-class correlation coefficient (ICC).

Results: Participants completing the retest (n=164) had a median age of 33.9 years. Median time for completion of the initial survey with PRO instruments was 36.0 minutes and for the five PRO instruments, median retest time was 21.0 minutes. The majority of participants had hemophilia A (74.4%), were white and non-Hispanic (72.6%), and self-reported arthritis/bone/joint problems (61%). Median/mean test-retest concordance was EQ-5D-5L 80.0%/79.1%, BPI 54.5%/58.9%, IPAQ 100%/100%, SF-36v2 77.8%/76.4%, and HAL 77.4%/75.9%. ICCs for test-retest reliability were EQ-5D-5L index 0.890; BPI - severity 0.950; BPI - interference 0.920; IPAQ total activity 0.940; SF-36v2 overall health 0.910; HAL total score 0.970.

Conclusion: All five PRO scales showed acceptable test-retest reliability in adult PWH. Therefore, the choice of instrument to be used for research or clinical care should be driven by instrument characteristics other than reliability.
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http://dx.doi.org/10.2147/PPA.S141389DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5614789PMC
September 2017

Cognitive and Functional Outcomes following Inpatient Rehabilitation in Patients with Acquired Brain Injury: A Prospective Follow-up Study.

J Neurosci Rural Pract 2017 Jul-Sep;8(3):357-363

Department of Biostatistics, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.

Objectives: To study the effects of cognitive retraining and inpatient rehabilitation to study the effects of cognitive retraining and inpatient rehabilitation in patients with acquired brain injury (ABI).

Design And Setting: This was a prospective follow-up study in a neurological rehabilitation department of quaternary research hospital.

Patients And Methods: Thirty patients with ABI, mean age 36.43 years (standard deviation [SD] 12.6, range 18-60), mean duration of illness 77.87 days (SD 91.78, range 21-300 days) with cognitive, physical, and motor-sensory deficits underwent inpatient rehabilitation for minimum of 14 sessions over a period of 3 weeks. Nineteen patients (63%) reported in the follow-up of minimum 3 months after discharge. Type of ABI, cognitive status (using Montreal Cognitive assessment scale [MoCA] and cognitive Functional Independence Measure [Cog FIM]), and functional status (motor FIM) were noted at admission, discharge, and follow-up and scores were compared.

Results: Patients received inpatient rehabilitation addressing cognitive and functional impairments. Baseline MoCA, motor FIM, and Cog FIM scores were 15.27 (SD = 7.2, range 3-30), 31.57 (SD = 15.6, range 12-63), and 23.47 (SD = 9.7, range 5-35), respectively. All the parameters improved significantly at the time of discharge (MoCA = 19.6 ± 7.4 range 3-30, motor FIM = 61.33 ± 18.7 range 12-89, Cog FIM =27.23 ± 8.10 range 9-35). Patients were discharged with home-based programs. Nineteen patients reported in follow-up and observed to have maintained cognition on MoCA (18.8 ± 6.8 range 6-27), significantly improved ( < 0.01) on Cog FIM (28.0 ± 7.7 range 14-35) and motor FIM =72.89 ± 16.2 range 40-96) as compare to discharge scores.

Conclusions: Cognitive and functional outcomes improve significantly with dedicated and specialized inpatient rehabilitation in ABI patients, which is sustainable over a period.
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http://dx.doi.org/10.4103/jnrp.jnrp_53_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5488554PMC
July 2017

Unrelated Hematopoietic Cell Transplantation in a Patient with Combined Immunodeficiency with Granulomatous Disease and Autoimmunity Secondary to RAG Deficiency.

J Clin Immunol 2016 10 18;36(7):725-32. Epub 2016 Aug 18.

Division of Hematology, CHOC Children's Hospital, 1201 W. La Veta Avenue, Orange, CA, 92868, USA.

The use of HLA-identical hematopoietic stem cell transplantation (HSCT) demonstrates overall survival rates greater than 75 % for T-B-NK+ severe combined immunodeficiency secondary to pathogenic mutation of recombinase activating genes 1 and 2 (RAG1/2). Limited data exist regarding the use of HSCT in patients with hypomorphic RAG variants marked by greater preservation of RAG activity and associated phenotypes such as granulomatous disease in combination with autoimmunity. We describe a 17-year-old with combined immunodeficiency and immune dysregulation characterized by granulomatous lung disease and autoimmunity secondary to compound heterozygous RAG mutations. A myeloablative reduced toxicity HSCT was completed using an unrelated bone marrow donor. With the increasing cases of immune dysregulation being discovered with hypomorphic RAG variants, the use of HSCT may advance to the forefront of treatment. This case serves to discuss indications of HSCT, approaches to preparative therapy, and the potential complications in this growing cohort of patients with immune dysregulation and RAG deficiency.
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http://dx.doi.org/10.1007/s10875-016-0326-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5286911PMC
October 2016

Successful treatment of secondary graft failure following unrelated cord blood transplant with hematopoietic growth factors in a pediatric patient with Fanconi anemia.

Pediatr Transplant 2015 Nov 30;19(7):E181-4. Epub 2015 Jul 30.

Division of Hematology, CHOC Children's Hospital, Orange, CA, USA.

Graft failure following allogeneic HCT in Fanconi anemia is associated with significant mortality. Retransplantation may be considered; however, the limited toxicity profile of HGFs also makes them an option for the treatment of graft failure. We describe a five-yr-old female diagnosed with Fanconi anemia and marrow failure treated with HCT. The course was complicated by secondary graft failure treated successfully with HGFs including G-CSF, EPO, and romiplostim. The outcome could be related to the intervention, but could also be the natural course of recovery, including recovering from a recent CMV infection treated with ganciclovir. We found the use of HGFs to be an effective and safe alternative to the potential complications as well as morbidity and mortality associated with the use of retransplantation.
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http://dx.doi.org/10.1111/petr.12564DOI Listing
November 2015

Chronic liver disease in alcohol-related chronic pancreatitis patients: Does lightning strike twice?

Indian J Gastroenterol 2015 Jul;34(4):345-6

Department of Gastroenterology, Sawai Man Singh Medical College, Jawaharlal Nehru Marg, Gangawal Park, Jaipur, 302 004, India.

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http://dx.doi.org/10.1007/s12664-015-0566-3DOI Listing
July 2015

A randomized study comparing the use of a pelvic lead shield during trans-radial interventions: Threefold decrease in radiation to the operator but double exposure to the patient.

Catheter Cardiovasc Interv 2015 Jun 3;85(7):1164-70. Epub 2015 Feb 3.

Department of Cardiology, Rambam Medical Center, Haifa, Israel.

Objectives: To determine the efficacy of a 0.5-mm lead apron across the patient's abdomen in addition to standard operator protection for the reduction of scatter radiation on operator and patient radiation exposure

Background: Cardiac angiography using the radial access compared to the femoral approach is associated with reduced complication rate and improved patient comfort but has significantly increased radiation dose. Improvements in radiation protection are needed

Methods: We randomly assigned 332 patients undergoing coronary angiography to a group with pelvic lead shielding and a group with standard protection. In each procedure, eight digital dosimeters were used to measure operator radiation dose [under the lead apron, outside the thyroid shield, and at the left side of the head], patient dose at the level of the umbilicus [above and beneath the lead apron], and two on the acrylic shielding and one on the image receptor to measure scattered radiation

Results: Both groups were similar in BMI, procedures performed, and number of sequences. Usage of lead shielding statistically significantly reduced the radiation dose of the operator at all three sites measured: under lead apron [all in µSv]: 0.53 ± 1.4 vs. 0.17 ± 0.6, on thyroid collar 5.9 ± 7.7 vs. 2.9 ± 3.4, and left side of head 3.3 ± 3.4 vs. 2.1 ± 2.2, P<0.001. However the radiation to the patient was doubled from 15.4 ± 24.1 to 28.9 ± 81.1, P=0.04 CONCLUSIONS: The use of a pelvic lead shield during radial angiography reduced the operator radiation exposure at multiple measurement sites. However there was an increased exposure to the patient. This balance should be further investigated before the widespread adoption of this method. .
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http://dx.doi.org/10.1002/ccd.25777DOI Listing
June 2015

Spectrum of chronic liver disease admitted to a medical college hospital in northern India: is there cause for concern?

Indian J Gastroenterol 2014 Sep;33(5):480-1

Department of Gastroenterology, Sawai Man Singh Medical College, Gangawal Park, Ashok Nagar, Jaipur, 302 004, India.

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http://dx.doi.org/10.1007/s12664-014-0465-zDOI Listing
September 2014

Global Emerging HEmophilia Panel (GEHEP): A Multinational Collaboration for Advancing Hemophilia Research and Treatment.

Transfus Med Hemother 2013 Oct 26;40(5):352-5. Epub 2013 Aug 26.

Queen's University, Kingston, ON, Canada.

GEHEP, established in 2009, is an independent, multi-institutional, international consortium of early career hematology specialists in the field of hemophilia and other inherited bleeding disorders. The main objective of the group, whose members practice at institutions in North America, Europe, and South Africa, is to advance hemophilia care by providing a forum for mentored collaborative research, developing programs for improving clinical care, and promoting academic career development of junior faculty. GEHEP members collect and document anonymized data on intra- and interinstitutional differences in patient populations, diagnosis, and treatment in the field of hemophilia and other bleeding disorders. To facilitate sharing of aggregated data among GEHEP members, a global protocol was developed and approved by most members' local institutional review board. Current GEHEP research initiatives are varied, encompassing work in pediatric and adult patients. GEHEP members have presented research at international meetings on the initiation of prophylaxis in children, use of immune tolerance induction in adults, and prevalence of acute coronary syndromes in older patients with hemophilia. The main goal of the continuing work of GEHEP is to advance the care of patients with hemophilia worldwide.
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http://dx.doi.org/10.1159/000354843DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3822276PMC
October 2013

Should proximal LAD be treated differently? Insights from a large DES stent registry.

Cardiovasc Revasc Med 2013 Nov-Dec;14(6):325-32. Epub 2013 Sep 23.

Department of Cardiology, Rambam Medical Center, Haifa, Israel.

Background: The current revascularization treatment recommendation is different according to lesion location and a higher recommendation is given to surgery for proximal LAD (pLAD) lesions over PCI. This is based on previous studies and expert opinion. We aimed to investigate whether indeed there is a difference in outcome with respect to LAD lesion location while using a drug eluting stent (DES).

Methods: The NOBORI-2 trial, enrolled 3067 consecutive patients in 125 centers who were treated with DES for single and multivessel disease. We compared 834 [27.2%] patients who underwent PCI of the pLAD as part of their revascularization, to 2203 [71.8%] patients in which stenting to other lesion(s) but not the pLAD was performed.

Results: The pLAD group had lower incidence of hypertension, peripheral vascular disease, prior PCI and CABG, but had more lesions treated [1.55 ± 0.8 vs. 1.35 ± 0.6], more stents implanted [1.98 ± 1.2 vs. 1.66 ± 1.0] and longer overall stent length [31.8 ± 20.2 vs. 28.2 ± 17.8 mm]. There was no difference in the occurrence of the primary endpoint [cardiac death, myocardial infarction and target lesion revascularization] at 1 or 2 years of follow up between the pLAD and non pLAD [6.0% vs. 4.6%, p=0.14 and 7.7% vs. 6.6%; p=0.22, respectively]. The relief from anginal symptoms was similar. Multivariate analysis showed that pLAD location was not a variable that predicted MACE or TLF. Stent thrombosis rate was similar.

Conclusion: When considering PCI with DES, there is no difference in outcome between patients with and without proximal LAD lesions.
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http://dx.doi.org/10.1016/j.carrev.2013.08.003DOI Listing
August 2014

Successful autologous cord blood transplantation in a child with acquired severe aplastic anemia.

Pediatr Transplant 2013 May 7;17(3):E104-7. Epub 2013 Mar 7.

Division of Hematology, CHOC Children's Hospital, Orange, CA 92868, USA.

Over 400 cases of pediatric SAA occur annually in the United States. A growing number of children with SAA may have had their stem cells harvested through cord blood collection. We describe a nine-yr-old male with SAA treated successfully with an autologous cord blood transplant following immunoablative chemotherapy. With the increasing number of people cryopreserving autologous cord blood, the use of autologous cord blood in the treatment of SAA might be considered as initial therapy. This case serves to discuss approaches to preparative therapy as well as the potential complications in this growing cohort of patients.
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http://dx.doi.org/10.1111/petr.12068DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4174564PMC
May 2013

An unusual case of Munchausen syndrome by proxy.

Indian J Psychiatry 2012 Oct;54(4):389-90

Department of Psychiatry, PGIMS, Rohtak, Haryana, India. E-mail:

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http://dx.doi.org/10.4103/0019-5545.104843DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3554979PMC
October 2012

Successful cord blood transplantation in a patient with malignant infantile osteopetrosis and hemophilia.

Pediatr Transplant 2013 Feb 23;17(1):E20-4. Epub 2012 Aug 23.

Division of Hematology, CHOC Children's Hospital, Orange, CA 92868, USA.

MIOP is a congenital disorder of osteoclast differentiation or dysfunction. Inadequate bone resorption by osteoclasts results in a spectrum of complications including hypocalcemia, osteosclerosis, marrow failure, extramedullary hematopoiesis, hydrocephalus, visual deficits, and eventual mortality. Early diagnosis and timely HCT is a recommended treatment approach for select patients prior to the development of end-organ damage. A comorbid bleeding disorder presents a unique challenge in the setting of MIOP and cord blood HCT given the additional risk factors for bleeding including delayed engraftment, a high risk of developing sinusoidal obstruction syndrome, and potential need for emergent invasive procedures. To our knowledge, this is the first report of a patient with an autosomal recessive form of MIOP who successfully underwent a cord blood HCT complicated by the presence of mild hemophilia A and HCT-related complications including delayed engraftment, sinusoidal obstruction syndrome, and need for multiple invasive procedures (e.g., ventriculostomy, tracheostomy) without clinically significant bleeding. Given the underlying diagnosis of MIOP and need for HCT, the challenge of mitigating the significant risk of bleeding in a patient with a comorbid bleeding disorder is discussed.
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http://dx.doi.org/10.1111/j.1399-3046.2012.01758.xDOI Listing
February 2013

Inflammatory polyps following successful HLA-matched cord blood transplantation in a patient with X-linked lymphoproliferative syndrome.

Pediatr Transplant 2012 Aug 8;16(5):E188-91. Epub 2011 Jul 8.

Divisions of Hematology, CHOC Children's Hospital, Orange, CA 92868, USA.

Gastrointestinal complications following HSCT are numerous and include a variety of issues resulting in hepatic, biliary, pancreatic, and intestinal compromise. In the context of an underlying state of immune dysregulation, novel complications may arise including autoimmunity. To our knowledge, this is the first report of a patient with XLP who was successfully treated with HSCT using an HLA-matched unrelated cord blood unit that was complicated by the development of inflammatory polyps of the colon. Given the underlying diagnosis of XLP and its associated immune dysregulation, the challenge of understanding unique gastrointestinal manifestations of autoimmunity following HSCT is discussed.
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http://dx.doi.org/10.1111/j.1399-3046.2011.01520.xDOI Listing
August 2012

Unrelated hematopoietic stem cell transplantation in a patient with congenital dyserythropoietic anemia and iron overload.

Pediatr Transplant 2012 May 26;16(3):E69-73. Epub 2010 Nov 26.

Division of Hematology, CHOC Children's Hospital and UC Irvine Medical Center, Orange, CA 92868, USA.

CDA is a heterogeneous group of disorders that result in morphologically abnormal erythroid maturation and ineffective erythropoiesis. Curative therapy for CDA focuses on the use of HSCT using fully matched sibling donors. This is the first report of a Type II CDA patient with severe iron overload who was successfully treated with HSCT using a HLA-matched unrelated donor after aggressive chelation therapy. Given the challenges of HSCT in any patient with CDA and severe iron overload, the role of novel approaches to iron chelation and HSCT is discussed.
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http://dx.doi.org/10.1111/j.1399-3046.2010.01414.xDOI Listing
May 2012

Living Profiles: design of a health media platform for teens with special healthcare needs.

J Biomed Inform 2010 Oct;43(5 Suppl):S9-S12

Stanford University, Department of Pediatrics, Division of Rheumatology, 700 Welch Road, Suite 301, Palo Alto, CA 94304, USA. Electronic address:

Living Profiles is a health media platform in development that aggregates multiple data flows to help teens with special healthcare needs (SHCN), particularly with regard to self-management and independence. A teen-oriented personal health record (PHR) incorporates typical teen behaviors and attitudes about health and wellness, encompasses how teens perceive and convey quality of life, and aligns with data related to their chronic medical condition. We have conceived a secure personalized user interface called the Quality of Life Timeline, which will assist with the transition from pediatric care to an adult provider through modules that include a mood meter, reminder device, and teleport medicine. With this personalized PHR, teens with SHCN can better understand their condition and its effects on daily activities and life goals and vice versa; additionally, use of this PHR allows for better information sharing and communication between providers and patients. The use of a teen-oriented tool such as Living Profiles can impact teens' overall quality of life and disease self-management, important attributes for a successful transition program.
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http://dx.doi.org/10.1016/j.jbi.2010.05.008DOI Listing
October 2010
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