Shweta S. Namjoshi MD MPH

All Publications

• The Development of the International Intestinal Failure Registry and an Overview of its Results. European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie Avitzur, Y., Pahl, E., Venick, R. 2024; 34 (2): 172-181

  Abstract

  Pediatric intestinal failure (IF) is a rare disease that represents an evolving field in pediatric gastroenterology and surgery. With only a limited number of multicenter collaborations, much of the research in pediatric IF is often confined to single-center reports with small sample sizes. This has resulted in challenges in data interpretation and left many knowledge gaps unanswered. Over the past two decades, five large multicenter collaborations, primarily from North America and Europe, have published their findings. Apart from one ongoing European adult and pediatric registry, these relatively large-scale efforts have been concluded.In 2018, the International Intestinal Failure Registry (IIFR) was initiated by the International Intestinal Rehabilitation and Transplant Association to continue these efforts and answer some of the knowledge gaps in pediatric IF. The IIFR goals are to prospectively assess the natural history of children diagnosed with IF and creating a worldwide platform to facilitate benchmarking and evidence-based interventions in pediatric IF. A pilot phase involving 204 enrolled patients was initiated in 2018 to assess the feasibility of an international IF registry and refine the study protocol and data collection forms. Following the successful completion of this phase, the current phase of the IIFR was launched in 2021. As of May 2023, the registry includes 362 prospectively followed children from 26 centers worldwide. This review provides an overview of the development, structure, and challenges of the IIFR, as well as the main findings from both the pilot and current phase.

  View details for DOI 10.1055/a-2212-6874

  View details for PubMedID 37973155

  View details for PubMedCentralID PMC10920018

• Review of long COVID in pediatric gastroenterology, hepatology, & nutrition. Journal of pediatric gastroenterology and nutrition Namjoshi, S. S., Mast, A., Patel, A. S. 2024

  View details for DOI 10.1002/jpn3.12199

  View details for PubMedID 38557961

• Vertebral compression fractures in pediatric patients with intestinal failure: A prospective observational case series. Intestinal Failure Smith, A., Namjoshi, S., Bachrach, L., Grover, M., Hoyer, C., Dunn, J. C., Hanudel, M., Kinberg, S. 2024; 1

  View details for DOI 10.1016/j.intf.2024.100006

• Digital Phenotype Generation and Time to Encounter Closure. JAMA pediatrics Mezoff, E. A., Gil, L. A., Lee, J. A., Gumer, L., Namjoshi, S. S., Hoffman, J. M., Wendel, D. 2023

  View details for DOI 10.1001/jamapediatrics.2023.3651

  View details for PubMedID 37747729

• Push and pull: the art of intestinal rehabilitation. JPEN. Journal of parenteral and enteral nutrition Raghu, V., Abdelhadi, R., Garcia, M. A., McDonnell, E., Mezoff, E., Namjoshi, S. S. 2023

  Abstract

  From the time of their initial surgery, children with short bowel syndrome (SBS) begin rehabilitating their remaining bowel in a quest towards reaching enteral autonomy. Even in the earliest experiences from 1987, nearly 98% of children with intestinal failure (IF) attend school during this process and 50% of children were shown to adapt defined as achieving independence from parenteral nutrition (PN) This article is protected by copyright. All rights reserved.

  View details for DOI 10.1002/jpen.2559

  View details for PubMedID 37689981

• Clinicopathologic Features of IDEDNIK (MEDNIK) Syndrome in a Term Infant: Histopathologic Features of the Gastrointestinal Tract and Report of a Novel AP1S1 Variant. Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Lu, J. G., Namjoshi, S. S., Niehaus, A. D., Tahata, S., Lee, C. U., Wang, L., McDonnell, E., Seely, M., Martin, M. G., Hazard, F. K. 2023: 10935266231177402

  Abstract

  Inherited syndromes of congenital enteropathy are rare, with many genetic causes described. Mutations of the AP1S1 gene results in the syndrome of intellectual disability, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma (IDEDNIK, formerly in the medical literature as MEDNIK). The clinicopathologic features of the enteropathy in IDEDNIK syndrome have not been fully explored. We describe a female infant who presented with metabolic acidosis, lethargy, and 14 watery stools per day. In the intensive care unit she required parenteral nutrition. She was found to have a novel homozygous pathogenic variant in the AP1S1 gene c.186T>G (p.Y62*). Esophagogastroduodenoscopy and colonoscopy at 6months of age were grossly normal. However, histologic sections of the duodenum showed mild villous blunting and enterocytes with cytoplasmic vacuoles. CD10 immunostaining highlighted the disrupted brush border. MOC31 immunostaining was wild-type with a membranous pattern of expression. Electron microscopy of the duodenum showed scattered enterocytes cells with shortened and disrupted apical microvilli. Although there is a mixed gap diarrhea and disrupted brush border, there are no significant inclusions typical of microvillus inclusion disease, nor tufted enterocytes typical of tufting enteropathy, making the clinical and histopathologic features for this syndrome unique.

  View details for DOI 10.1177/10935266231177402

  View details for PubMedID 37278357

• 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 Talathi, S., Namjoshi, S., Raghu, V., Wendel, D., Oliveira, S., Reed, K., Yanchis, D., Mezoff, E. A. 2023

  Abstract

  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 Smith, A., Namjoshi, S., Kerner, J. A., Dunn, J. C. 2023

  Abstract

  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

• Retrospective Observational Analysis of Free Serum Retinol in a Cohort of Pediatric Patients with Short Bowel Syndrome and Intestinal Failure SN Comprehensive Clinical Medicine Smith, A., Dahlen, A., Diyaolu, M., McDonnell, E., Kerner, J., Dunn, J. C., Namjoshi, S. S. 2023; 5

  View details for DOI 10.1007/s42399-023-01626-6

• Pitfalls of Iron Supplementation in Parenteral Nutrition Admixtures for Children with Intestinal Failure. JPEN. Journal of parenteral and enteral nutrition Herdes, R. E., Oliveira, S. B., Kocoshis, S. A., Bernieh, A., Namjoshi, S. S. 2022

  Abstract

  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 Kidney International Hanudel, M. R., Czaya, B., Wong, S., Rappaport, M., Namjoshi, S. S., Chua, K., Jung, G., Gabayan, V., Qiao, B., Nemeth, E., Ganz, T. 2022; 101 (4): 711-719

  Abstract

  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 Namjoshi, S. S., Galloway, D., Herdes, R. E., Talathi, S., Ding, V. Y., Mezoff, E. A. 2022

  Abstract

  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 DeMaria, K. A., Fink, C., Pepper, M., Rieger, K., Tan, S. Y., Namjoshi, S. S. 2022

  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 Kehar, M., Ebel, N. H., Ng, V., Sehgal, A., Slowik, V., Leung, D. H., Shah, A. A., Ovchinsky, N., Kogan-Liberman, D., Arnon, R., Vitola, B., Waheed, N., Lebel, S., Mohammad, S., Squires, J. E., Shteyer, E., Miloh, T. A., Sanchez, M., Hildreth, A., Yerushalmi, B. Y., Chu, C., Kader, H., Book, L., Alrabadi, L., Zheng, M., Namjoshi, S. S., Cagil, Y., Fuchs, Y., Lobritto, S. J., Martinez, M. 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 Ebel, N. H., Kehar, M., Ng, V., Sehgal, A., Leung, D. H., Shah, A. A., Gupta, N., Baqueros, J., Botha, J., Slowik, V., Lebel, S., Miloh, T. A., Shteyer, E., Arnon, R., Azzam, R. K., Ovchinsky, N., Mohammad, S., Kogan-Liberman, D., Squires, J. E., Sanchez, M., Hildreth, A., Book, L., Chu, C., Alrabadi, L., Chepuri, B., D'Agostino, D., Elisofon, S., Falik, R., Gallagher, L., Kader, H., Lam, S., Mogul, D., Mujawar, Q., Namjoshi, S. S., Valentino, P. L., Vitola, B., Waheed, N., Zheng, M., Blondet, N., Lobritto, S. J., Martinez, M. 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. JPGN reports Herdes, R. E., Cagil, Y., Namjoshi, S., Hassan, M. 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. JPGN reports Hsu, D., Khalsa, U. K., Hassan, M., Sandborg, C. I., Namjoshi, S. S. 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 Chan, A. P., Namjoshi, S. S., Jardack, P. M., Maloney, L., Ardjmand, A., Jackson, N. N., Martin, M. G. 2021

  Abstract

  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 Kehar, M. n., Ebel, N. H., Ng, V. L., Baquero, J. E., Leung, D. H., Slowik, V. n., Ovchinsky, N. n., Shah, A. A., Arnon, R. n., Miloh, T. n., Gupta, N. n., Mohammad, S. n., Kogan-Liberman, D. n., Squires, J. E., Sanchez, M. C., Hildreth, A. n., Book, L. n., Chu, C. n., Alrabadi, L. n., Azzam, R. n., Chepuri, B. n., Falik, R. n., Gallagher, L. n., Kader, H. n., Mogul, D. n., Mujawar, Q. n., Namjoshi, S. S., Valentino, P. L., Vitola, B. n., Waheed, N. n., Zheng, M. H., Lobritto, S. n., Martinez, M. n. 2021

  Abstract

  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 Hsu, D., Khalsa, U. J., Hassan, M., Sandborg, C., Namjoshi, S. S. 2021; 2 (1)
• Visual Diagnosis: Anal Mass in a 3-year-old Boy. Pediatrics in review Ogbonnaya, S. A., Namjoshi, S. S. 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. Namjoshi, S. S., Farkas, C., Jackson, N., Reyen, L. E., Baldivia, P. S., Vargas, J. H., Venick, R. S., Weng, P. L., Hanudel, M. R., Ganz, T., Wozniak, L. 2020

  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 JPGN Reports Huang, A., Montiel-Esparza, R., Scott, G., Martin, B., Bruzoni, M., Kadapakkam, M., Namjoshi, S. S. 2020; 1 (2)

  View details for DOI 10.1097/PG9.0000000000000023

• Development of gastrointestinal function. Pediatric Nutrition Namjoshi, S. S., Martin, M. G. 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 Namjoshi, S. S., Muradian, S., Bechtold, H., Reyen, L., Venick, R. S., Marcus, E. A., Vargas, J. H., Wozniak, L. J. 2018; 42 (2): 427–35

  View details for DOI 10.1177/0148607117690528

  View details for Web of Science ID 000425094500020

• Flu vaccinations among general population inmates in a large urban jail, Los Angeles, 2007-08 Journal of the American Correctional Association Namjoshi, S. S., Nelson, A., Kodagoda, D., Tadrous, M., Miranda, A., Kamara, F., Arslanian, H., Malek, M. 2008: 21-28
• Off-label marketing: a primer for physicians & policy makers. Namjoshi, S. S., Wen, L. S. AMSA Online Publication. Reston, VA. 2005