Dr. Namjoshi specializes in the care and support of children with intestinal failure on & off home parenteral nutrition (PN). Her research interests include long term outcomes for patients on home PN, iron in gastrointestinal diseases, clinical pathologic correlates in intestinal failure, quality of life & nutrition for patients with intestinal pseudoobstruction, and the assessment & treatment of congenital enteropathies.
- Congenital onset diarrhea and enteropathy (CODEs)
- Nutrition support and intestinal rehabilitation
- PLE and lymphatic diseases
- GI Long Covid
- Pediatric Gastroenterology
Clinical Assistant Professor, Pediatrics - Gastroenterology
Medical Director Intestinal Rehabilitation & Nutrition Support, Lucile Packard Children's Hospital (2020 - Present)
Medical Director Children's Home Pharmacy, Lucile Packard Children's Hospital (2020 - Present)
Associate Director of Health Policy, Office of Child Health Equity, Stanford University School of Medicine (2021 - Present)
Honors & Awards
Alwin C. Rambar-James B.D. Mark nominee, Department of Pediatrics (2022)
Learning Health System Registry Proposal Award Winner, LPCH (2022)
Auxillary Foundation Award, Children’s Books for Disability Advocacy, LPCH (2020)
Mission Zero award, Lucile Packard Children's Hospital (2020)
High Value Innovation Challenge award for Intestinal Rehabilitation Rounds, Lucile Packard Children's Hospital (2019)
UCLA Children’s Discovery and Innovation Institute Fellow’s Research Support Award, UCLA CDI (2018-2019)
NIH Fellowship Training Grant T32DK07180, NIH (2017-2019)
Housestaff Quality Improvement Award, UCLA (2014)
Resident Research Award, UCLA (2014)
Dean's Merit Scholarship, University of Southern California (2002-2006)
Boards, Advisory Committees, Professional Organizations
Committee Member, NASPGHAN Advocacy Committee (2021 - Present)
Committee Member, NASPGHAN Intestinal Rehabilitation Special Interest Group (2019 - Present)
Invited Reviewer, NASPGHAN Nutrition Section & Research Committee, Grants Review (2020 - Present)
Committee Member, Intestinal Rehabilitation & Transplantation Association Intestinal Failure Registry Committee (2021 - Present)
Committee Member, NASPGHAN Nutrition Committee (2019 - Present)
Residency: UCLA Pediatric Residency (2014) CA
Board Certification, Pediatric Gastroenterology, Hepatology, & Nutrition, American Board of Pediatrics (2019)
Fellowship: UCLA Pediatric Gastroenterology Fellowship (2019) CA
Board Certification: American Board of Pediatrics, Pediatrics (2014)
Medical Education: Penn State College of Medicine Registrar (2011) PA
Current Research and Scholarly Interests
1. The mission of the International Intestinal Failure Registry (IIFR) is to provide the international intestinal rehabilitation and transplant community with accurate data on the outcomes and course of intestinal failure to support research, quality improvement, and policy development. https://tts.org/irta-registries/irta-ifr
2. NCT05241444 is the first-in-human, Phase 1 clinical trial will test the feasibility of the manufacturing and the safety of the administration of CD4^LVFOXP3 in up to 36 evaluable human participants with IPEX and evaluate the impact of the CD4^LVFOXP3 infusion on the disease.
3. Stanford's local Intestinal Failure Registry (SIFR) ensures ongoing assessment and improvement of intestinal failure outcomes and care provided at Stanford in collaboratiton with the Division of Pediatric Surgery. This registry focuses on clinical outcomes and social developmental outcomes for patients with short bowel syndrome, pediatric CODEs, and pseudoobstruction.
4. Multiple, multicenter projects with NASPGHAN (https://naspghan.org/) Nutrition Committee's and the NASPGHAN Intestinal Rehabilitation Special Interest Group ensure assessment of blenderized tube feeding, iron in intestinal failure, and other multicenter research.
5. Through the Office of Child Health Equity, I collaborate with the subgroup focusing on equity analytics & community based participatory research as assistant director of health policy to advocate for communities and a world where healthcare and life outcomes are equitable and just. My interests include civic health & disability justice.
CD4^LVFOXP3 in Participants With IPEX
This first-in-human, Phase 1 clinical trial will test the feasibility of the manufacturing and the safety of the administration of CD4^LVFOXP3 in up to 36 evaluable human participants with IPEX and evaluate the impact of the CD4^LVFOXP3 infusion on the disease.
Evaluation and management of iron deficiency in children undergoing Intestinal Rehabilitation - A Position Paper from the NASPGHAN Intestinal Rehabilitation Special Interest Group.
Journal of pediatric gastroenterology and nutrition
Iron deficiency is the most common nutritional deficiency affecting children undergoing intestinal rehabilitation. Patients may be asymptomatic or present with non-specific symptoms including fatigue, irritability, and dizziness. The diagnosis of iron deficiency in this population can be complicated by the coexistence of systemic inflammation or other nutritional deficiencies which may mimic iron deficiency. Many routinely available laboratory tests lack specificity and no consensus on screening is available. Success in oral and enteral treatment is impeded by poor tolerance of iron formulations in a population already challenged with intolerance. Newer parenteral iron formulations exhibit excellent safety profiles, but their role in repletion in this population remains unclear. The following report, compiled by a multidisciplinary group of providers caring for children undergoing intestinal rehabilitation and representing the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Special Interest Group for Intestinal Rehabilitation, seeks to address these challenges. After discussing iron physiology and population-specific pathophysiology, we make recommendations on iron intake, iron status assessment, and evaluation for alternative causes of anemia. We then provide recommendations on iron supplementation and treatment of iron deficiency anemia specific to this nutritionally vulnerable population.
View details for DOI 10.1097/MPG.0000000000003736
View details for PubMedID 36800275
Importance of Ileum and Colon in Children with Short Bowel Syndrome.
Journal of pediatric surgery
BACKGROUND: It is well known that small bowel length is a dominant prognostic indicator in patients with short bowel syndrome (SBS). The relative importance of jejunum, ileum, and colon is less well defined in children with SBS. Here we review the outcome of children with SBS with respect to the type of remnant intestine.METHODS: A retrospective review of 51 children with SBS was conducted at a single institution. The duration of parenteral nutrition use was the main outcome variable. The length of the remaining intestine as well as the type of intestine were recorded for each patient. Kaplan-Meier analyses were conducted to compare the subgroups.RESULTS: Children with greater than 10% expected small bowel length or more than 30cm of small bowel achieved enteral autonomy faster than those with less. The presence of ileocecal valve enhanced the ability to wean from parenteral nutrition. The presence of ileum significantly enhanced the ability to wean from parenteral nutrition. Patients with the entire colon also achieved enteral autonomy sooner than those with partial colon.CONCLUSIONS: The preservation of ileum and colon is important in patients with SBS. Approaches to preserve or lengthen ileum and colon may be beneficial for these patients.LEVEL OF EVIDENCE: IV.
View details for DOI 10.1016/j.jpedsurg.2023.01.053
View details for PubMedID 36894441
Pitfalls of Iron Supplementation in Parenteral Nutrition Admixtures for Children with Intestinal Failure.
JPEN. Journal of parenteral and enteral nutrition
BACKGROUND: Pediatric patients with intestinal failure are at increased risk for iron deficiency. Supplementation is not routinely included in parenteral nutrition solutions. There is currently limited research related to the safety of iron supplementation in parenteral nutrition and for intravenous forms used in patients with intestinal failure. Current ASPEN and ESPGHAN guidelines promote the use of enteral iron, acknowledging the risks of using iron supplementation within parenteral nutrition admixtures.METHODS: We review a patient case and the current available literature related to iron in parenteral nutrition.RESULTS: Five major concerns are identified: peroxidation reactions, incompatibility, hypersensitivity, infection risk, and iron overload.CONCLUSION: We propose an argument against the preferential use of iron supplementation within parenteral nutrition in children with intestinal failure when enteral supplementation or intermittent parenteral infusion may be sufficient.CLINICAL RELEVANCY STATEMENT: Pediatric patients with intestinal failure are at risk for iron deficiency anemia, anemia due to folate or B12 deficiency, vitamin B6 deficiency, copper deficiency, non-anemic iron deficiency, and anemia of inflammation. Iron status assessment and supplementation for these patients is variable over time. There are several biochemical and physiologic concerns about the safety of adding iron supplementation to parenteral nutrition admixtures. This paper briefly reviews the current available literature. Further research is needed to evaluate best practices for iron supplementation for pediatric patients with intestinal failure. This article is protected by copyright. All rights reserved.
View details for DOI 10.1002/jpen.2428
View details for PubMedID 35730416
Enteral ferric citrate absorption is dependent on the iron transport protein ferroportin
2022; 101 (4): 711-719
Ferric citrate is approved as an iron replacement product in patients with non-dialysis chronic kidney disease and iron deficiency anemia. Ferric citrate-delivered iron is enterally absorbed, but the specific mechanisms involved have not been evaluated, including the possibilities of conventional, transcellular ferroportin-mediated absorption and/or citrate-mediated paracellular absorption. Here, we first demonstrate the efficacy of ferric citrate in high hepcidin models, including Tmprss6 knockout mice (characterized by iron-refractory iron deficiency anemia) with and without adenine diet-induced chronic kidney disease. Next, to assess whether or not enteral ferric citrate absorption is dependent on ferroportin, we evaluated the effects of ferric citrate in a tamoxifen-inducible, enterocyte-specific ferroportin knockout murine model (Villin-Cre-ERT2, Fpnflox/flox). In this model, ferroportin deletion was efficient, as tamoxifen injection induced a 4000-fold decrease in duodenum ferroportin mRNA expression, with undetectable ferroportin protein on Western blot of duodenal enterocytes, resulting in a severe iron deficiency anemia phenotype. In ferroportin-deficient mice, three weeks of 1% ferric citrate dietary supplementation, a dose that prevented iron deficiency in control mice, did not improve iron status or rescue the iron deficiency anemia phenotype. We repeated the conditional ferroportin knockout experiment in the setting of uremia, using an adenine nephropathy model, where three weeks of 1% ferric citrate dietary supplementation again failed to improve iron status or rescue the iron deficiency anemia phenotype. Thus, our data suggest that enteral ferric citrate absorption is dependent on conventional enterocyte iron transport by ferroportin and that, in these models, significant paracellular absorption does not occur.
View details for DOI 10.1016/j.kint.2021.10.036
View details for PubMedCentralID PMC8940695
Bacterial overgrowth assessment and treatment among pediatric intestinal rehabilitation & nutrition support providers: an international survey of clinical practice patterns.
JPEN. Journal of parenteral and enteral nutrition
BACKGROUND: Small bowel bacterial overgrowth (SBBO) is a common, but difficult to diagnose and treat problem in pediatric short bowel syndrome (SBS). Lack of clinical consensus criteria and unknown sensitivity and specificity of bedside diagnosis makes research on this potential SBS disease modifier challenging. The objective of this research was to describe clinical care of SBBO among international intestinal rehabilitation and nutrition support (IR&NS) providers treating patients with SBS.METHODS & MATERIALS: A secure, confidential, international, electronic survey of IR&NS practitioners was conducted between March 2021 and May 2021. All analyses were conducted in the R statistical computing framework , version 4.0 RESULTS: 60% of respondents agreed and 0% strongly disagreed that abdominal pain, distension, emesis, diarrhea, and malodorous stool, were attributable to SBBO. No more than 20% of respondents strongly agreed and no more than 40% agreed that any sign or symptom was specific for SBBO. For a first-time diagnosis, 31 practitioners agreed with use of a 7-day course of a single antibiotic, with a majority citing Grade 5 evidence to inform their decisions (case series, uncontrolled studies, or expert opinion). The most common first antibiotic used to treat a new onset SBBO was metronidazole, and rifaximin was the 2nd most commonly used. 100% of respondents reported they would consider a consensus algorithm for SBBO, even if the algorithm may be divergent from their current practice.CONCLUSION: SBBO practice varies widely among experienced IR&NS providers. Development of a clinical consensus algorithm may help standardize care to improve research and care of this complex problem and to identify risks and benefits of chronic antibiotic use in SBS. This article is protected by copyright. All rights reserved.
View details for DOI 10.1002/jpen.2369
View details for PubMedID 35274342
Intestinal Failure in Junctional Epidermolysis Bullosa: Mild Skin Disease, Severe Diarrhea.
Digestive diseases and sciences
View details for DOI 10.1007/s10620-022-07410-1
View details for PubMedID 35147818
THE IMPACT OF SARS-COV2 INFECTION IN CHILDREN WITH LIVER DISEASE: AN INTERNATIONAL OBSERVATIONAL REGISTRY STUDY
WILEY. 2021: 1180A-1181A
View details for Web of Science ID 000707188005343
THE IMPACT OF SARS-CoV2 INFECTION IN PEDIATRIC LIVER TRANSPLANT RECIPIENTS: AN INTERNATIONAL OBSERVATIONAL REGISTRY STUDY
WILEY. 2021: 136A-137A
View details for Web of Science ID 000707188000205
Initial Presentation of a Pediatric Intestinal Pseudo-Obstruction Episode After SARS-CoV-2 Virus (COVID-19) Infection.
2021; 2 (2): e059
View details for DOI 10.1097/PG9.0000000000000059
View details for PubMedID 34192292
View details for PubMedCentralID PMC8043327
Early Intervention and Resolution of Pediatric Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus: A Pediatric Case Report.
2021; 2 (1): e041
View details for DOI 10.1097/PG9.0000000000000041
View details for PubMedID 37206925
View details for PubMedCentralID PMC10191485
Long-Term Dietary Changes in Subjects with Glucose Galactose Malabsorption Secondary to Biallelic Mutations of SLC5A1.
Digestive diseases and sciences
BACKGROUND: Glucose galactose malabsorption (GGM) is a congenital diarrheal disorder of intestinal Na+/glucose cotransport (SGLT1/SLC5A1). The required glucose and galactose-restricted diet has been well described in infancy, but long-term nutrition follow-up is limited.AIM: To perform a comprehensive nutritional assessment on a cohort of patients with GGM to gain insights into the consumption patterns within the population.METHODS: A cross-sectional study examining dietary intake of a GGM cohort using prospective food records. The calories and nutrients of all foods, beverages, and condiments were analyzed with descriptive statistics and compared to intake patterns of age- and sex-matched NHANES groups.RESULTS: The six patients were 0.7-26years old. Whole foods and vegetable fats were major parts of the diet, while dairy and added sweeteners were restricted. Compared to typical US intakes, mean macronutrient distribution was 88th percentile from fat, 18th percentile from carbohydrates, and 78th percentile from protein. Fructose consumption, as a proportion of total sugar intake, decreased with age, from 86.1 to 50.4%. Meanwhile, glucose consumption increased with age, from 13.8 to 48.6% of sugar intake. However, the actual amount of glucose consumed remained low, equivalent to 4th percentile of US consumption level. Galactose intake was marginal throughout life.CONCLUSIONS: A GGM diet is a high-fat and high-protein/low-carbohydrate diet that is rich in fruits and vegetables but limited in dairy and added sugar. Relatively less fructose but more glucose is incorporated into the diet with age. Future studies should investigate the effects of the GGM diet on gut microbiome and long-term health.
View details for DOI 10.1007/s10620-020-06792-4
View details for PubMedID 33433815
SARS-CoV2 Infection in Children with Liver Transplant and Native Liver Disease: An International Observational Registry Study.
Journal of pediatric gastroenterology and nutrition
Increased mortality risk due to severe acute respiratory syndrome coronavirus-2 (SARS-CoV2) infection in adults with native liver disease (LD) and liver transplant (LT) is associated with advanced age and comorbid conditions. We aim to report outcomes for children with LD and LT enrolled in the NASPGHAN/SPLIT SARS-CoV2 registry.In this multicenter observational cohort study, we collected data from 91 patients <21 years (LD 44, LT 47) with laboratory-confirmed SARS-CoV2 infection between April 21 and September 17, 2020.Patients with LD were more likely to require admission (70% vs 43% LT, p = 0.007) and pediatric intensive care unit (PICU) management (32% vs 4% LT, p = 0.001). Seven LD patients required mechanical ventilation (MV) and 2 patients died; no patients in the LT cohort died or required MV. Four LD patients presented in pediatric acute liver failure (PALF), 2 with concurrent multisystem inflammatory syndrome in children (MIS-C); all recovered without LT. Two LD patients had MIS-C alone and one patient died. Bivariable logistic-regression analysis found that patients with non-alcoholic fatty liver disease (NAFLD) (OR 5.6, p = 0.02) and LD (OR 6.1, p = 0.01, vs LT) had higher odds of severe disease (PICU, vasopressor support, MV, renal replacement therapy or death).Although not directly comparable, LT recipients had lower odds of severe SARS-CoV2 infection (vs LD), despite immunosuppression burden. NAFLD patients reported to the registry had higher odds of severe SARS-CoV2 disease. Future controlled studies are needed to evaluate effective treatments and further stratify LD and LT patients with SARS-CoV2 infection.
View details for DOI 10.1097/MPG.0000000000003077
View details for PubMedID 33605666
- Early Intervention and Resolution of Pediatric Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus: A Pediatric Case Report JPGN Reports 2021; 2 (1)
Visual Diagnosis: Anal Mass in a 3-year-old Boy.
Pediatrics in review
2020; 41 (2): e4–e7
View details for DOI 10.1542/pir.2018-0144
View details for PubMedID 32005692
Anemia of Inflammation in Patients with Intestinal Failure on Home Parenteral Nutrition
SN Compr. Clin. Med.
View details for DOI 10.1007/s42399-020-00404-y
A Novel Case of Carcinoid Tumor in a Pediatric Patient With Short Bowel Syndrome Secondary to Gastroschisis
2020; 1 (2)
View details for DOI 10.1097/PG9.0000000000000023
- Development of gastrointestinal function. Pediatric Nutrition edited by Kleinman, R. E., Greer, F. R. American Academy of Pediatrics. 2019; 8th: 17–56
Nutrition Deficiencies in Children With Intestinal Failure Receiving Chronic Parenteral Nutrition
JOURNAL OF PARENTERAL AND ENTERAL NUTRITION
2018; 42 (2): 427–35
View details for DOI 10.1177/0148607117690528
View details for Web of Science ID 000425094500020
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