Doctors’ Jobs Obsolete

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Thanks to Jose Garza for sharing this important information:

Full link The Science Post: Hospital to replace doctors with parents who have done their research

Other important article links available at this website include the following:

Is It OK To Swim With A Central Line?

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D Wendel et al. JPGN 2021; 72: 474-486. Free full text: Management of Central Venous Access in Children With Intestinal Failure: A Position Paper From the NASPGHAN Intestinal Rehabilitation Special Interest Group

This is a very useful article with recommendations for central venous access in children. The main recommendations are summarized in Table 3 & listed below; however, there is a lot of detailed information in the article on frequent issues like schools, travel (including dealing with TSA), sports, and even swimming. In addition, the article delineates recommendations for management and prevention of line complications.

1. Recommendations for venous access:

  • Tunneled, single lumen, cuffed silicone catheters should be used for children with IF.
  • Upper extremity access is the preferred location when available.

2. Recommendations pertaining to routine CVC care:

  • Proper technique and hygiene surrounding CVC care are of paramount importance in preventing CVC-associated complications. Caregivers should receive directed education regarding CVC care before initial discharge, with subsequent reinforcement education as needed.
  • CHG impregnated supplies (disk, sponge, or dressing) should be considered for central line dressing in pediatric IF patients.
  • Routine surveillance of central venous access should be performed by US. MR, CT, or traditional venography should be reserved for when further delineation of access is required.

3. Recommendations regarding general considerations—sports, travel, and emergencies:

  • All children with IF should be provided with an emergency letter that details the specific needs of the individual child in case of an emergency. (See at bottom for example -Figure 1)
  • Discuss with families the risks of swimming and sports participation with strategies to protect the dressing and central line.
  • All travel plans should be discussed with the intestinal rehabilitation team well in advance of travel to facilitate discussion of a plan of care in case of emergency.

4. Recommendations regarding central line-associated bloodstream infections:

  • All children with IF and CVC who develop a fever (≥38.0°C) should be admitted to the hospital and assessed for bacteremia with central and peripheral blood cultures while receiving broad-spectrum empiric antibiotics through the CVC for at least 48 h, awaiting culture results regardless of other infectious sources.
  • If clinically stable, discuss with the patient’s IRP before line removal for CLABSI.
  • Prophylactic lock therapy with ethanol or other nonantibiotic locks should be strongly considered in all children with IF who have had at least one central line-associated bloodstream infection or are at high risk for infection.

5. Recommendations pertaining to central line mechanical complications:

  • In children with IF, CVC should be repaired whenever possible to preserve central venous access.
  • Children with IF and a newly identified CRT should be treated with low molecular weight heparin for at least 6 weeks with guidance from a hematologist.
  • Children with IF who have persistence of at least one chronic thrombus should be maintained on prophylactic anticoagulation with low molecular weight heparin.
  • Children who have lost multiple sites of central venous access should be considered for referral to an intestinal transplant center for evaluation and management

6. Recommendations for central venous access program management:

  • All centers following children with IF should, at a minimum, track the number of outpatient CLABSI per 1000 catheter days.

With regard to swimming: “Swimming introduces an incompletely defined but potentially severe risk to those requiring chronic central venous access. Contamination of various chlorine-treated (swimming pools), stagnant (lakes and ponds), and flowing (oceans and rivers) bodies of water with human pathogens has been well documented, though proper maintenance may minimize outbreaks. The potentially fatal risk of such contaminants gaining access to central circulation via the CVC is unclear…Parents seeking guidance are confronted by mixed messaging from support programs, online resources and blogs, and even IRP. These conflicting recommendations and practices reflect the paucity of data to guide a safe and clear approach for swimming with a central line…[in one study of 16 home PN programs] swimming in low-risk situations [was permitted but] recommended immediate site cleaning and dressing change following water exposure and avoidance of submersion for 4–6 weeks after CVC placement. Ultimately, the decision to permit children with IF to swim lies with the parent or guardian.”

For pets (like Charlie), this article notes that “steps should be taken to promote line integrity in the presence of pets. Particularly in the setting of pets that may attempt to chew or play with tubing, adequate physical protection of the insertion site and catheter itself is recommended. Any line or tubing puncture by an animal should prompt immediate evaluation. Family awareness of zoonotic disease risk and advocacy of handwashing before and after animal care should be made clear.”
Figure 1 -Emergency Letter Template

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Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

Short Bowel Syndrome and Long Duration of Feeding Problems

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VJ Christian et al. JPGN 2021; 72: 442-445. Pediatric Feeding Disorder in Children With Short Bowel Syndrome

This small (n=28) retrospective study provides useful information on the persistence of feeding problems in children with short bowel syndrome (SBS). The authors defined a pediatric feeding disorder (PFD) as “reliance on enteral feeds to sustain nutrition, reliance on high-calorie oral supplements to sustain nutrition, or feeding skill dysfunction resulting in not consuming an age-appropriate diet.” Patients who remained on PN were considered to have a PFD as well.

Key findings:

  • Of the 21 patients (75% of total cohort) who were weaned off parenteral nutrition, 57.1%, 81.0%, 90.5%, and 100.0% achieved this by 12, 24, 36, and 48 months of age, respectively. Median age at time of weaning PN was 10.8 months.
  • Of the 13 patients who were weaned off enteral nutrition (EN), 30.8%, 69.2%, 76.9%, and 100.0% achieved this by 12, 24, 36, and 48 months, respectively. Median age of weaning EN was 15.7 months. Overall, about a third of patients required EN beyond 2 years of life.
  • The prevalence of PFD (of entire cohort) was 100.0%, 76.5%, 68.8%, and 70.0% at 1, 2, 3, and 4 years of age, respectively

My take: When parents ask how long it will be before my child is off PN and eating by mouth, this study’s results could be useful.

Chicago at Sunrise

Related blog posts for Short Bowel Syndrome:

The Best Time To Treat Children with Hepatitis C And Cost Considerations

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E Greenaway et al. J Pediatr 2021; 230: 38-45. Treatment of Chronic Hepatitis C in Young Children Reduces Adverse Outcomes and Is Cost-Effective Compared with Deferring Treatment to Adulthood

Related editorial: N Rodriguez-Baez. J Pediatr 2021; 230: 9-10. Full text: Hepatitis C in Young Children: To Treat or Not to Treat – Is It Cost-Effective?

In this study, the authors used a state-transition model to assess cost-effectiveness of hepatitis C virus (HCV) infection in children; the model treated a hypothetical cohort of 10,000 children with chronic HCV at age 6 years with combination therapy of sofosbuvir/ledipasvir for 12 weeks vs deferring treatment until 18 years of age.

Key findings:

  • The incremental cost effectiveness of early treatment of young children was $12 690 per QALY gained after 20 years, which is considered cost effective compared with deferred treatment.
  • The authors note that if the cost of DAA medications dropped by 60%, then early treatment would not be more cost effective.
  • However, early treatment of 10,000 children would prevent 330 cases of cirrhosis, 18 cases of hepatocellular carcinoma, and 48 liver-related deaths.
  • The investigators presented an additional scenario treating children as young as 3 years of age and using alternative treatment with the pan-genotypic combination of glecaprevir/pibrentasvir for 8 weeks; using glecaprevir/pibrentasvir resulted in an incremental cost effectiveness of $12 563 per QALY compared with deferring treatment to age 18 years.

All cost effective models have built in assumptions. This model, for example, presumes each patient is offered treatment only once and does not get reinfected before age 18 years.

Other aspects about early treatment that are difficult to quantitate:

  • Improved adherence at younger age which improves cost effectiveness
  • Reduction in transmission of HCV as a consequence of successful treatment
  • Detrimental effects of untreated/deferred treatment HCV on quality of life, psychosocial health, and cognitive functioning

My take: This study (& editorial) demonstrate that early treatment of HCV is a good value and delivers non-economic benefits as well. Every child (>3 years) with HCV should be treated and cured of HCV infection.

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From Journal of Pediatrics twitter feed

Telomere Biology Disorders and GI Bleeding

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RW Himes et al. J Pediatr 2021; 230: 55-61. Gastrointestinal Hemorrhage: A Manifestation of the Telomere Biology Disorders

Background: Telomere biology disorders are a complex set of illnesses defined by the presence of very short telomeres; these individuals are at very high risk of bone marrow failure, cancer, and pulmonary fibrosis. There are 15 known genes which can experience damaging mutations, or other abnormalities, that can cause very short telomeres (for example the telomerase genes TERC and TERT). The most widely recognized telomere biology disorder is known as dyskeratosis congenita (DC); others include Hoyeraal-Hreidarsson syndrome, Revesz syndrome, and Coats plus.

Key findings:

  • Sixteen patients who experienced GI hemorrhage were identified at 11 centers. Ten patients had a history of hematopoietic cell transplantation.
  • Initial GI bleeding occurred at a median of 12.5 years.
  • Angiodysplasia of the stomach and/or small bowel was described in 8 of the 12 patients who underwent endoscopy; 4 had esophageal varices. The lesions were often diffuse and widespread (see Figures 1 & 2).
  • GI bleeding appeared to be more prevalent in those with TINF2, CTC1 or STN1 mutations (12 of 14 with genetic testing).
  • Recurrence was common, and the overall long-term outcome for affected patients was poor. 12 of 16 were deceased at time of data collection (median age of 16.5 years at time of death), though the proximate cause of death was not reported.
  • No single intervention was uniformly associated with cessation of bleeding, although 1 patient had a sustained response to treatment with bevacizumab. Other treatments that were tried included endoscopic treatments, thalidomide, octreotide, proton pump inhibitors, sirolimus and hormonal treatments.

My take: GI bleeding in these rare disorders is a difficult clinical problem.

Related blog post: Patterns and Puzzles with Very Early Onset IBD

Liver Shorts: Delisting Transplant Candidates, Albumin Infusions for Cirrhosis, Terlipresin & Liver Learning System

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KL Karunungan et al. Liver Transplantation 20021: 27: 200-208. Impact of Payer Status on Delisting Among Liver Transplant Candidates in the United States

This was a retrospective study which relied on large national databases.

  • The 1‐year cumulative incidence of delisting was 9.0% (95% confidence interval [CI], 8.3%‐9.8%) for patients with private insurance, 10.7% (95% CI, 9.9%‐11.6%) for Medicare, and 10.7% (95% CI, 9.8%‐11.6%) for Medicaid
  • Medicare (HR, 1.20; 95% CI, 1.17‐1.24; P < 0.001) and Medicaid (HR, 1.20; 95% CI, 1.16‐1.24; P < 0.001) were independently associated with an increased hazard of death or deterioration compared with private insurance.
  • The article highlights regional variation in payor coverage and change in watilist death or deterioration from 2002-2018 (Figure 1)
  • Higher levels of education and employment were protective against waitlist mortality and deterioration
  • Female sex was a risk factor for delisting which may be in part to body size as women are more likely to have an organ declined as a result of small stature

L China et al. NEJM 2021; 384: 808-17. A Randomized Trial of Albumin Infusions in Hospitalized Patients with Cirrhosis This was the ATTIRE trial; somehow ATTIRE is an acronym to allude to “Albumin to Prevent Infection in Chronic Liver Failure.” This trial was a multicenter, randomized controlled study.

“In patients hospitalized with decompensated cirrhosis, [daily] albumin infusions to increase the albumin level to a target of 30 g per liter or more was not more beneficial than the current standard care.” The standard of care included giving albumin under specific circumstances: large volume paracentesis, spontaneous bacterial peritonitis, or hepatorenal syndrome. Infusions (20% albumin) were infused at a rate of 100 mL/hr. In addition, the albumin group, which received 10 times as much albumin as the standard group, had more severe or life-threatening adverse events, especially pulmonary edema or fluid overload.

F Wong et al. NEJM 2021; 384: 818-828. Terlipressin plus Albumin for the Treatment of Type 1 Hepatorenal Syndrome In this multicenter, randomized controlled study, terlipressin was associated with improved renal function -reversal of HRS occurred in 32% compared to 17% in placebo group; however, it was associated with increased serious adverse events (eg. respiratory failure) and increased death (51% vs 45% in placebo group).

ER Perito et al. JPGN 2021; 72: 417-424. A Learning Health System for Pediatric Liver Transplant: The Starzl Network for Excellence in Pediatric Transplantation The Starzl Network for Excellence in Pediatric Transplantation (SNEPT) is the first multicenter effort by pediatric liver transplant teams. Its goal is to establish and share evidence-based care to improve liver transplantation outcomes. If successful, SNEPT should be to liver transplantation as ImproveCareNow network is for pediatric inflammatory bowel disease.

When To Perform Genetic Testing In The Setting Of Inflammatory Bowel Disease

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HH Uhilg et al. JPGN 2021; 72: 45-473. Free Full Text: Clinical Genomics for the Diagnosis of Monogenic Forms of Inflammatory Bowel Disease: A Position Paper From the Paediatric IBD Porto Group of European Society of Paediatric Gastroenterology, Hepatology and Nutrition

  • This is a very useful article. Table 3 lists many of the features of some monogenic inflammatory bowel disease (IBD). Table 4 details potential immune workup tests. Table 5 lists 75 genes that should be included when testing for monogenic IBD.
  • Box 2 (see below) provides a list of conditions that should prompt consideration of genetic testing. Figure 1 provides an algorithm for testing.
  • Table 6 provides a summary of statements
    • #3:”Genetic screening for monogenic IBD is recommended in all patients with infantile-onset IBD (<2 years) and should be considered in patients with very early-onset IBD (<6 years), in particular, in those patients with relevant comorbidity, extraintestinal manifestations, and/or family history”
    • #5: “Routine genetic screening for all IBD patients is not recommended since a monogenic cause of IBD in patients with IBD onset over 6 year of age, especially those with adolescent or adult age onset of IBD is exceptional in the absence of relevant comorbidity”
  • There is also some advice on variants of unknown significance: “Databases, such as Clinvar, ClinGen, or The Human Gene Mutation Database can help to assess variant phenotype relations”

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From The Onion:

From The Onion

Is Fatty Liver Increased in Pediatric Population with Type 1 Diabetes Mellitus?

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J Sae-wong et al. J Pediatr 2021; 230: 32-37. The Prevalence of Nonalcoholic Fatty Liver Disease and Its Risk Factors in Children and Young Adults with Type 1 Diabetes Mellitus

In this cross-sectional study with 50 children with Type 1 Diabetes Mellitus (T1DM), MRE and MRI-PDFF studies were undertaken to determine whether the participants had nonalcoholic fatty liver disease (NAFLD). Key findings:

  • The median age and duration of T1D were 16.9 years (IQR, 13.6-20 years) and 6.5 years (IQR, 4-11 years), respectively. 26% of the cohort were overweight or obese.
  • The prevalence of NAFLD was 10% (more than half had normal ALT values). Four out of 5 patients with NAFLD were overweight/obese, and 2 had an and elevated alanine aminotransferase (ALT) level. None had liver fibrosis (defined as MRE >2.9 kPa).
  • High BMI-SDS (body mass index standard deviation score) was the sole independent risk factor associated with NAFLD (OR, 5.79; 95% CI, 1.04-32.18).

My take: This study is reassuring regarding the prevalence of NAFLD in children and young adults with T1D which was comparable to that in the general population. Routine screening for NAFLD in patients with T1D does not appear to be useful.

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Why It Is Still A Bad Idea To Test Asymptomatic Patients For Clostridium Difficile

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JM Parnell et al. JPGN 2021; 72: 378-383. Two-step Testing for Clostridioides Difficile is Inadequate in Differentiating Infection From Colonization in Children

In this prospective study, the authors enrolled asymptomatic pediatric patients (n=225) and compared C diff testing results with symptomatic patients (n=41) with positive nucleic-acid amplification-based testing (NAAT).

Key findings:

  • Of the 225 asymptomatic children enrolled in the study, 47 (21%) were colonized with C. difficile including 9/59 (15.5%) with cancer, 30/92 (32.6%) with CF, and 8/74 (10.8%) with IBD. 
  • Overall, “use of a multistep testing algorithm with NAAT followed by EIA failed to differentiate symptomatic CDI from asymptomatic colonization in our pediatric cohort.” When symptomatic and colonized children were compared, neither EIA (enzyme immunoassay) positivity (44% vs 26%, P = 0.07) nor CCNA (functional cell cytotoxicity neutralization assay) positivity (49% vs 45%, P = 0.70) differed significantly

My take: Don’t test children who are asymptomatic for Clostridium difficile. Even in children with symptoms, C diff positivity could reflect colonization and symptoms could be due to other etiologies.

Related blog posts:

Parenteral Nutrition: “The Scar Remains”

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A recent study (KM Gura et al. J Pediatr 2021; 230: 46-54. Fish Oil Emulsion Reduces Liver Injury and Liver Transplantation in Children with Intestinal Failure-Associated Liver Disease: A Multicenter Integrated Study) provides multicenter data comparing fish oil emulsion (FOLE) (Omegaven) with a historical control of soybean emulsion (SOLE) (Intralipid). The FOLE group was enrolled between 2004-2018; the SOLE group had data from 1999-2012.

Key points:

  • Among FOLE recipients (n=189), 65% experienced cholestasis resolution vs 16% of SOLE recipients (n=73) (P < .0001).
  • The aspartate aminotransferase to platelet ratio index scores improved in FOLE recipients (1.235 vs 0.810 and 0.758, P < .02) but worsened in SOLE recipients (0.540 vs 2.564 and 2.098; P ≤ .0003) 
  • Liver transplantation was reduced in FOLE vs SOLE (4% vs 12%; P = .0245).

My main criticisms of the study:

  1. While the methods explain that FOLE received 1 gm/kg/d, compared with 3 gm/kg/d for SOLE, this was NOT reviewed in the discussion. This is quite important in terms of proving that one product is preferred over the other. With lipid toxicity, it would be expected that delivering 3 times as much would be more damaging on the liver.
  2. The discussion does not discuss the potential neurological consequences of lipid minimization/lower doses of lipids. In the same Journal of Pediatrics issue, Bell et al report that 77% of SBS in their cohort of extremely premature infants with short bowel syndrome had moderate-to-severe neurodevelopmental impairment (related blog post: Neurodevelopmental Impairment in the Majority of Extremely Premature Infants with Short Bowel Syndrome)
  3. The discussion has only a single sentence regarding the change in care between the eras of SOLE and FOLE: “Additional limitations include a relatively small sample size and changes in surgical, medical, and nutritional practice between the 2 eras that could not be controlled for this study.”
  4. Also, the discussion omits the development of other FOLE alternatives (eg. SMOFlipid) which has been a very important advance in the management of patients with SBS.

The commentary by Samuel Kocoshis (J Pediatr 2021; 230: 11-12) provides a good deal of insight. The title and first paragraph provides some interesting historical context: (full text) “Even When the Would Is Healed, the Scar Remains” “The above maxim was coined by the Roman author Publilius Syrus when referring to wounds of most tissues or body parts.1 Because hepatic regeneration was recognized (as evidenced by the story of Prometheus’s liver being eaten daily by an eagle only to regenerate the next day) in Syrus’s time, his dictum was too far too simplistic when applied to the liver. One must delve more deeply into the mechanism of liver injury to ascertain just when hepatic scaring persists or when it disappears.”

My take: This study illustrates harm reduction with the change in lipid administration. The development of new lipid products has made a huge difference in the outcomes of children with short bowel syndrome.

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