Importance of early detection of infantile inflammatory bowel disease with defective IL-10 pathway: A case report.

RATIONALE: Infantile inflammatory bowel disease (IBD) is an extremely rare subgroup of IBD that includes patients whose age of onset is younger than 2 years old. These patients can have more surgical interventions, and a severe and refractory disease course with higher rates of conventional treatment failure. Monogenic defects play an important role in this subgroup of IBD, and identification of the underlying defect can guide the therapeutic approach. PATIENT CONCERNS: In 2007, a 4-month-old girl from a nonconsanguineous family presenting with anal fistula, chronic diarrhea, and failure to thrive. She underwent multiple surgical repairs but continued to have persistent colitis and perianal fistulas. DIAGNOSIS: Crohn's disease was confirmed by endoscopic and histologic finding. INTERVENTION: Conventional pediatric IBD therapy including multiple surgical interventions and antitumor necrosis factor alpha agents were applied. OUTCOMES: The patient did not respond to conventional pediatric IBD therapy. Interleukin-10 (IL-10) receptor mutation was discovered by whole-exome sequencing and defective IL-10 signaling was proved by functional test of IL-10 signaling pathway by the age of 12. The patient is currently awaiting hematopoietic stem cell transplantation. LESSONS: Early detection of underlying genetic causes of patients with infantile-IBD is crucial, since it may prevent patients from undergoing unnecessary surgeries and adverse effects from ineffective medical therapies. Moreover, infantile-IBD patients with complex perianal disease, intractable early onset enterocolitis and extraintestinal manifestations including oral ulcers and skin folliculitis, should undergo genetic and functional testing for IL-10 pathway defect.

Congenital heart defects in Noonan syndrome: Diagnosis, management, and treatment.

Noonan syndrome is a pleomorphic genetic disorder, in which a high percentage of affected individuals have cardiovascular involvement, most prevalently various forms of congenital heart disease (i.e., pulmonary valve stenosis, septal defects, left-sided lesions, and complex forms with multiple anomalies). Care includes attentiveness to several comorbidities, some directly impacting cardiac management (bleeding diatheses and lymphatic anomalies). More than 50% of patients with Noonan syndrome harbor PTPN11 pathogenic variation, which results in hyperactivation of RAS/mitogen-activated protein kinase signaling. Several other disease genes with similar biological effects have been uncovered for NS and phenotypically related disorders, collectively called the RASopathies. Molecular diagnosis with gene resequencing panels is now widely available, but phenotype variability and in some cases, subtlety, continues to make identification of Noonan syndrome difficult. Until genetic testing becomes universal for patients with congenital heart disease, alertness to Noonan syndrome's broad clinical presentations remains crucial. Genotype-phenotype associations for Noonan syndrome enable better prognostication for affected patients when a molecular diagnosis is established. We still lack Noonan syndrome-specific treatment; however, newly developed anticancer RAS pathway inhibitors could fill that gap if safety and efficacy can be established for indications such as pulmonary valve stenosis.

Advancing RAS/RASopathy therapies: An NCI-sponsored intramural and extramural collaboration for the study of RASopathies.

RASopathies caused by germline pathogenic variants in genes that encode RAS pathway proteins. These disorders include neurofibromatosis type 1 (NF1), Noonan syndrome (NS), cardiofaciocutaneous syndrome (CFC), and Costello syndrome (CS), and others. RASopathies are characterized by heterogenous manifestations, including congenital heart disease, failure to thrive, and increased risk of cancers. Previous work led by the NCI Pediatric Oncology Branch has altered the natural course of one of the key manifestations of the RASopathy NF1. Through the conduct of a longitudinal cohort study and early phase clinical trials, the MEK inhibitor selumetinib was identified as the first active therapy for the NF1-related peripheral nerve sheath tumors called plexiform neurofibromas (PNs). As a result, selumetinib was granted breakthrough therapy designation by the FDA for the treatment of PN. Other RASopathy manifestations may also benefit from RAS targeted therapies. The overall goal of Advancing RAS/RASopathy Therapies (ART), a new NCI initiative, is to develop effective therapies and prevention strategies for the clinical manifestations of the non-NF1 RASopathies and for tumors characterized by somatic RAS mutations. This report reflects discussions from a February 2019 initiation meeting for this project, which had broad international collaboration from basic and clinical researchers and patient advocates.

The "Silk Road": A novel method for inserting transpyloric feeding tubes in low resource settings.

INTRODUCTION: Transpyloric tube (TPT) feeding is used in a multitude of conditions including gastroesophageal reflux disease. We here describe a new simple method to insert TPTs. METHODS: 6 French feeding tube is premeasured nose to xiphisternum, and then another 7cm of length is added and 3-5 silk (4-0) ties are applied to the end of the tube spaced 0.5-1cm apart. The knots are placed in different radial directions, and multiple throws are placed on each knot so as to add bulkiness. The tube is then inserted transnasally to the premeasured length and secured. The child is given a single dose of metoclopramide and placed on his right side for 4h. A plain abdominal x-ray is then performed to confirm adequate TP placement. Following correct placement the patient is tube fed with small volumes every 15-20min. Descriptive data was prospectively collected. RESULTS: 34 patients were recruited, median age 3.5months. All presented with vomiting, and 26 had failure to thrive. 24had successful TP tube placement from the first attempt, 6 from the second attempt, 2 on third attempt, and in 2 placement was unsuccessful. In 28 patients vomiting almost stopped completely. 9 patients had fundoplication, and 1 had gastrostomy placement. 3 patients died during the study because of unknown reasons. CONCLUSION: The silk tie technique is a safe and simple way to treat persistent vomiting and may prove useful in low resourced environments. LEVEL OF EVIDENCE: IV.

Surgery for ulcerative colitis in geriatric patients is safe with similar risk to younger patients.

OBJECTIVE: A prior study indicated that postoperative mortality and complications were higher in geriatrics with inflammatory bowel disease (IBD). We sought to assess the rates of surgical complications and mortality in patients aged ≥65 years after colectomy for ulcerative colitis (UC). METHODS: This is a single center retrospective study at a tertiary care center. We reviewed all hospital discharges with ICD-9 code 556.X between January 2002 and January 2014. Patients were included if they underwent a colectomy for UC. All records were manually reviewed for demographics, complications and mortality within 90 days postoperatively. RESULTS: A total of 259 patients underwent surgery for UC during the study period and 34 patients were ≥65 years old (range 65-82) at the time of their surgery. There was no difference in overall length of stay (10.5 days vs. 9.6 days; P = 0.645) or complication rates (44% vs. 47%; P = 0.854) in the ≥65 cohort compared with the under 65 cohort. Mortality was higher in the geriatric cohort but this included only two deaths within 90 days, one of which was unrelated to the surgery, compared with one death related to surgery within 90 days in the younger cohort. Readmissions occurred in 24% of both cohorts within 90 days. CONCLUSION: Geriatric patients undergoing surgery for UC are not at increased risk of surgery-related morbidity or mortality compared with a younger cohort.

Complications in children with percutaneous endoscopic gastrostomy (PEG) placement.

INTRODUCTION: The aim of this study was to analyze the complication rates and mortality in association with different operative techniques of percutaneous endoscopic gastrostomy (PEG), age, underlying diseases and other risk factors. Moreover, analysis of the indications of PEG insertion and the underlying comorbidities was also performed. METHODS: This study performs a literature analysis of PEG-related complications in children. Literature was searched on PubMed® (1994-2017) using terms "percutaneous endoscopic gastrostomy", "complications", "mortality" and "children". RESULTS: Eighteen articles with 4631 patients were analyzed. The mean age was 3 years (0-26 years). Operative techniques were: pull technique in 3507 (75.7%), 1 stage PEG insertion in 449 (9.7%), introducer technique in 435 (9.4%), image-guided technique in 195 (4.2%) and laparoscopic-assisted PEG in 45 (1.6%). Most frequent indications for PEG insertion were dysphagia (n = 859, 32.6%), failure to thrive (n = 723, 27.5%) and feeding difficulties (n = 459,17.4%). Minor complications developed in n1518 patients (33%), including granulation  (n = 478, 10.3%), local infection (n = 384, 8.3%) and leakage (n = 279, 6%). In 464 (10%) patients, major complications occurred; the most common were systemic infection (n = 163, 3.5%) and cellulitis (n = 47, 1%). Severe complication like perforation occurred in less than 0.3%. Patients with lethal outcomes (n = 7, 0.15%) had severe comorbidities; and the cause of mortality was sepsis in all cases. Prematurity or young age did not affect complication rate. Patients with ventriculoperitoneal (VP) shunt had higher risk of major complications. In high-risk patients, laparoscopic-assisted PEG insertion had less major and severe complication than traditional pull technique. CONCLUSIONS: PEG is a safe operative technique; although minor complications are relatively common and occur in up to 1/3 of patients, there is a fairly low rate of severe complications. Two-thirds of PEG patients have at least one comorbidity. Patients with VP shunt have higher risk of major complications. In high-risk patients, laparoscopic-assisted PEG is recommended.

Noncardiac Challenges in the Cardiac ICU: Feeding, Growth and Gastrointestinal Complications, Anticoagulation, and Analgesia.

Outcomes following cardiac intensive care unit (CICU) admission are influenced by many factors including initial cardiac diagnosis, surgical complexity, and burden of critical illness. Additionally, the presence of noncardiac issues may have a significant impact on outcomes and the patient experience during and following an intensive care unit stay. This review focuses on three common noncardiac areas which impact outcomes and patient experience in and beyond the CICU: feeding and growth, pain and analgesia, and anticoagulation. Growth failure and feeding dysfunction are commonly encountered in infants requiring cardiac surgery and have been associated with worse surgical and developmental outcomes. Recent studies most notably in the single ventricle population have demonstrated improved weight gain and outcomes when feeding protocols are implemented. Children undergoing cardiac surgery may experience both acute and chronic pain. Emerging research is investigating the impact of sedatives and analgesics on neurodevelopmental outcomes and quality of life. Improved pain scores and standardized management of pain and withdrawal may improve the patient experience and outcomes. Effective anticoagulation is a critical component of perioperative care but may be complicated by inflammation, multiorgan dysfunction, and patient factors. Advances in monitoring of anticoagulation and emerging therapies are reviewed.

Treino materno para estimulação domiciliar sugere melhora no desenvolvimento motor de prematuros / Maternal training for home stimulation suggests an improvement in the motor development of preterm infants

Objetivo: Avaliar o desenvolvimento motor de lactentes pré-termo no primeiro mês de idade cronológica e comparar com o desempenho no primeiro e no terceiro mês de idades corrigidas, após treinamento materno para estimulação domiciliar. Métodos: Selecionaram-se seis lactentes, com idade gestacional média de 33,6 semanas, peso ao nascimento médio de 1853 g e APGAR ≥ 7 no quinto minuto. Realizou-se treinamento em cinco encontros, sendo utilizada uma cartilha para auxiliar orientações. Para avaliação, usou-se a Escala Motora Infantil de Alberta. Resultados: Os lactentes obtiveram pontuações abaixo do esperado com um mês cronológico, evidenciando a necessidade de correção da idade. Houve aumento significativo na pontuação da amostra no primeiro mês de idade corrigida, e manutenção no terceiro mês de idade corrigida. Conclusão: Verificou-se que há uma tendência à melhora do desempenho motor dos lactentes prematuros cujas mães realizam a estimulação precoce domiciliar e essa pode ser mais uma estratégia terapêutica utilizada pelos profissionais.

Objective: To evaluate the motor development of newborn babies with one month of chronological age and compare with the development in the first and third months of corrected age, after maternal training of home stimulation. Methods: We selected six infants born with 33,6 weeks in average, average weight of 1853 g and APGAR ≥ 7 in the fifth minute. The training was performed in five meetings and a book was used to help with home stimulation. The Alberta Infant Motor Scale was used to evaluate development. Results: Lower scores in the scale were observed at the chronological age, evidencing the necessity of age correction. An improvement in the score was observed at the first month of corrected age and it was maintained at the third month. Conclusion: The proposed methodology suggests a tendency to improve motor development of premature infants whose mother performs the premature home stimulation and this may be another therapeutic strategy used by professionals.

Emergence of a Genetic Diagnosis: Case Presentation of a Preterm Infant With Cardiofaciocutaneous Syndrome.

BACKGROUND: Advanced prenatal screening and diagnostic testing have increased the number of newborns born with a confirmed diagnosis. Not all infants, however, are born with a known diagnosis. In fact, for some conditions, physical findings evolve over time and diagnosis can be further delayed because of premature birth. PURPOSE: This article shares a case report of a dysmorphic preterm infant admitted to the intensive care nursery for routine care. The emergence of physical findings as the baby matured during the first weeks of life and the stepwise, diagnostic approach used to confirm a rare genetic condition, cardiofaciocutaneous (CFC) syndrome, is provided. CASE FINDINGS/RESULTS: Key physical differences apparent at birth prompted screening for several genetic syndromes and a number of inborn errors of metabolism. As the phenotype emerged, a type of RASopathy entered the differential, the most likely of which was CFC syndrome. IMPLICATIONS FOR PRACTICE: Although CFC syndrome is rare, the combined incidence rate of RASopathies is greater, and as such, providers should be familiar with such conditions. Classic features may not be apparent in preterm infants so providers must remain astute to physical changes and communicate them with genetic consultants. IMPLICATIONS FOR RESEARCH: Gaining a better understanding of how providers can best support parents through the lengthy, diagnostic odyssey of genetic testing is important. In addition, ongoing research is needed to try to identify a genotype-phenotype correlation for CFC syndrome to guide patient surveillance and provide prognostic information to parents.

Predictors of gastrostomy placement in children with inherited metabolic diseases treated by umbilical cord blood transplantation.

BACKGROUND/PURPOSE: Children with inherited metabolic diseases (IMDs) undergoing umbilical cord blood transplantation (UCBT) who are at risk for post-transplant failure to thrive may benefit from pretransplant gastrostomy tube (GT) placement. Here we sought to determine predictors of posttransplant failure to thrive. METHODS: A retrospective analysis was performed for IMD patients who underwent UCBT at a single center from 2001 to 2011. Patients who received GTs were compared with controls. Multivariable logistic regression was used to determine significant predictors for GT placement. Recursive partitioning was performed to determine appropriate cut-offs for significant continuous variables. RESULTS: Two hundred and seventeen patients met inclusion criteria of which twenty-three were excluded due to death within one hundred days of transplant. Forty (20.6%) of the remaining patients underwent a surgical GT placement. Multivariable logistic regression demonstrated that weight percentile at time of transplant was significantly associated with GT placement (Adjusted odds ratio (AOR): 0.87 per 10th percentile, p=0.022). Recursive partitioning demonstrated that the 40th weight percentile at time of transplant was an optimal cut-off for predicting GT placement. CONCLUSIONS: Patients preparing for umbilical cord transplantation who are below the 40th percentile for weight may benefit from pre-emptive GT placement prior to transplant.

A practical approach to classifying and managing feeding difficulties.

Many young children are thought by their parents to eat poorly. Although the majority of these children are mildly affected, a small percentage have a serious feeding disorder. Nevertheless, even mildly affected children whose anxious parents adopt inappropriate feeding practices may experience consequences. Therefore, pediatricians must take all parental concerns seriously and offer appropriate guidance. This requires a workable classification of feeding problems and a systematic approach. The classification and approach we describe incorporate more recent considerations by specialists, both medical and psychological. In our model, children are categorized under the 3 principal eating behaviors that concern parents: limited appetite, selective intake, and fear of feeding. Each category includes a range from normal (misperceived) to severe (behavioral and organic). The feeding styles of caregivers (responsive, controlling, indulgent, and neglectful) are also incorporated. The objective is to allow the physician to efficiently sort out the wide variety of conditions, categorize them for therapy, and where necessary refer to specialists in the field.

Emergency ECT in an incapacitated, medically compromised patient with Huntington's disease.

Electroconvulsive therapy (ECT) is infrequently considered an "emergency" medical procedure; however, there are certain conditions in which there is considerable urgency to initiate ECT. For example, prompt administration of ECT to treat neuroleptic malignant syndrome and malignant catatonia is necessary to improve a patient's overall prognosis and potentially save the patient's life. In this case, a 57-year-old woman with Huntington's disease was admitted to our medical intensive care unit for failure to thrive due to severe psychotic symptoms. Prior to her admission, the patient had become increasingly psychotic and agitated, resulting in her refusal and/or inability to eat. Efforts to treat her severe psychiatric and behavioral symptoms with various psychopharmacological strategies were largely unsuccessful. As the patient's physical health continued to decline, with loss of approximately 35 pounds over 2 months, her family began making arrangements to transfer her to a hospice facility. The day before she was to be transferred, the psychiatry consultation-liaison service recommended ECT. Unfortunately, this recommendation was complicated because the patient was unable to provide consent. This case report describes the legal and administrative process used to ethically and legally administer ECT without consent from the patient or a court-appointed guardian in order to treat a life-threatening condition. To the best of our knowledge, this report documents the first time ECT has been granted "medical emergency" status in Texas.

Cardio-facio-cutaneous syndrome: clinical features, diagnosis, and management guidelines.

Cardio-facio-cutaneous syndrome (CFC) is one of the RASopathies that bears many clinical features in common with the other syndromes in this group, most notably Noonan syndrome and Costello syndrome. CFC is genetically heterogeneous and caused by gene mutations in the Ras/mitogen-activated protein kinase pathway. The major features of CFC include characteristic craniofacial dysmorphology, congenital heart disease, dermatologic abnormalities, growth retardation, and intellectual disability. It is essential that this condition be differentiated from other RASopathies, as a correct diagnosis is important for appropriate medical management and determining recurrence risk. Children and adults with CFC require multidisciplinary care from specialists, and the need for comprehensive management has been apparent to families and health care professionals caring for affected individuals. To address this need, CFC International, a nonprofit family support organization that provides a forum for information, support, and facilitation of research in basic medical and social issues affecting individuals with CFC, organized a consensus conference. Experts in multiple medical specialties provided clinical management guidelines for pediatricians and other care providers. These guidelines will assist in an accurate diagnosis of individuals with CFC, provide best practice recommendations, and facilitate long-term medical care.

Growth in children with congenital diaphragmatic hernia during the first year of life.

PURPOSE: Infants with congenital diaphragmatic hernia (CDH) have high rates of mortality and long-term morbidity, including poor growth and failure to thrive. The aim of this study was to describe growth patterns during the first year of life in infants with congenital diaphragmatic hernia in a non-ECMO cohort. METHODS: Medical records of infants with CDH admitted to our center between January 2005 and December 2011 were reviewed. Infants with anthropometric measurements at 3, 6 and 12months were included. Anthropometric measurements were obtained for the first year of life. Logistic regression analyses were performed to find predictive associations with failure to thrive (FTT). RESULT: Of the 45 survivors, 38 were seen twice (84%) and 24 (53%) were seen on three occasions to age 12months. Poor growth was observed with weight being most affected. FTT was present in 63% during the first six months of life. Days of mechanical ventilation were the only predictor of FTT. Besides poor weight gain, height and head circumference were also reduced. However, catch-up growth occurred during the second half of infancy and at age 12months failure to thrive had reduced by two thirds to 21%. CONCLUSIONS: Poor growth is a common early finding in CDH patients, which improves during infancy. This emphasizes the importance of close follow-up and aggressive nutritional management in CDH patients.

Atención temprana comunitaria en niños con retardo en el neurodesarrollo / Community-based early care of children with neurodevelopment retardation

Objetivo: determinar la evolución de los pacientes con retardo en el neurodesarrollo, atendidos en el Programa Comunitario de Atención Temprana, del municipio Habana Vieja. Métodos: se realizó una investigación prospectiva de evaluación del Programa Comunitario de Atención Temprana en la Habana Vieja entre 1998 y 2008. El universo fue de 170 pacientes con retardo del neurodesarrollo, remitidos por su médico de familia, y evaluados por un equipo interdisciplinario mediante examen clínico y por escalas estandarizadas. Posterior a cada evaluación se aplicó un programa de estimulación individual ejecutada por rehabilitadores y defectólogos de cada policlínico y en el hogar. Se utilizó como estadígrafo el resumen frecuencial, porcentajes y el test de chi cuadrado. Resultados: al inicio los pacientes obtuvieron fundamentalmente evaluación de deficiente, tanto en la área mental (55,29 por ciento) como en la área motora (57,64 por ciento). En la evaluación final, se comprobó el ascenso significativo de los porcentajes en la evaluación normal (mental: X²= 58,50; p= 0,00001; motor: X²= 73,06; p= 0,00001). Los pacientes afectados asociados a afecciones perinatales tuvieron una evolución satisfactoria asociados a las categorías superadas (24,75 por ciento) y favorables (43,78 por ciento). Resultó significativo (X²= 18,90; p= 0,00001) que los pacientes con antecedente de sepsis neonatal evolucionaron desfavorablemente. La tercera parte de los pacientes con bajo peso al nacer evolucionó de manera estática y agravada (19,23/16,00 por ciento). Conclusiones: los resultados del programa comunitario de atención temprana demuestran que se alcanzó mejoría en el neurodesarrollo en la mayoría de los pacientes

Objective: to determine the progress of patients with neurodevelopment retardation, who are attended to in the community-based early care program of Habana Vieja municipality. Methods: a prospective assessment research study of the community-based early care program in Habana Vieja municipality was conducted from 1998 to 2008. The universe of study was 170 patients with neurodevelopment retardation, who were referred to the multidisciplinary medical team by their family physicians, in order to be evaluated through clinical exams and standardized scales. After each evaluation, an individual stimulation program was applied to them by rehabilitation specialists and phoniatricians of their polyclinics and at home. Frequency summary, percentages and chi-square test were used for statistical purposes. Results: the patients were initially evaluated as deficient both in the mental and motor areas (55.29 percent and 57.64 percent , respectively). The final evaluation showed a significant rise of normal evaluation percentages (mental: X²= 58.50; p= 0.00001; motor: X²= 73.06; p= 0.00001). The affected patients associated to perinatal illnesses had satisfactory recovery as to overcome (24.75 percent) and favorable (43.78 percent) categories. It was significant that patients with a history of neonatal sepsis progressed unfavorably ((X²= 18.90; p= 0.00001). One third of patients with low birthweight progressed in a static and severe way (19.23/16 percent). Conclusions: the results of the community-based early care program proved that there was some improvement in the neurodevelopment of most of the patients after the treatment

The perinatal presentation of cardiofaciocutaneous syndrome.

There is limited information available related to the perinatal course of cardiofaciocutaneous syndrome (CFC) compared to other syndromes within the Ras-MAP kinase pathway (rasopathies) such as Noonan and Costello syndrome. Retrospective chart review revealed four cases of CFC with molecular confirmation between 2005 and 2012 at Hawaii's largest obstetric and pediatric referral center. We report on details of the prenatal, neonatal, and infancy course and long-term follow-up beyond infancy in two patients. This report includes novel features including systemic hypertension, hyponatremia, and chronic respiratory insufficiency, not previously reported in CFC. We provide pathologic diagnosis of loose anagen hair in one patient. Some of these findings have been reported in the other rasopathies, documenting further clinical overlap among these conditions. Molecular testing can be useful to differentiate CFC from other rasopathies and in counseling families about potential complications and prognosis. We recommend a full phenotypic evaluation including echocardiogram, renal ultrasound, brain imaging, and ophthalmology examination. We additionally recommend close follow-up of blood pressure, pulmonary function, and monitoring for electrolyte disturbance and extra-vascular fluid shifts.

The role of lay health workers in pediatric chronic disease: a systematic review.

BACKGROUND: Children with chronic diseases represent a high-cost and resource-intensive population of children. With continued gaps in chronic disease management and persistent fragmentation in the health care system, stakeholders are seeking new strategies to address the needs of these children. OBJECTIVE: We sought to systematically assess the effectiveness of lay health worker interventions in improving health care utilization, symptom management, and family psychosocial outcomes for children with chronic conditions. DATA SOURCE: PubMed, PsycINFO, and Web of Science (January 1961 to February 2013). STUDY ELIGIBILITY CRITERIA, PARTICIPANTS, AND INTERVENTIONS: We developed a strategy to search citations to identify relevant articles. Search terms included randomized controlled trial (RCT), lay worker, parent mentor, peer mentor, peer educator, community health workers, community health aids, patient advocate, patient facilitator, patient liaison, promotoras(es), care ambassadors, patient navigator, and nonprofessional. Additional studies were identified by searching the reference lists of retrieved articles and contacting clinical experts. RCTs of lay health worker interventions for children with chronic conditions were included. Studies were restricted to those concentrated on children 0-18 years of age with chronic illnesses. STUDY APPRAISAL AND SYNTHESIS METHODS: Abstracts were independently screened by 2 reviewers. Articles with relevant abstracts underwent full text review and were evaluated for inclusion criteria. A structured tool was used to abstract data from selected articles. Because of the heterogeneous interventions and outcomes, we did not conduct a meta-analysis. RESULTS: The search yielded 736 unique articles, of which 17 met inclusion criteria. All interventions focused on specific conditions: asthma, type I diabetes, obesity, and failure to thrive. Interventions were heterogeneous in frequency, mode, and duration of interactions between lay health workers and subjects. Several interventions were multifaceted, including both one-on-one and group interactions. Improved outcomes most commonly reported were reduced urgent care use, decreases in symptoms, fewer missed work and school days, and increased parental quality of life. One study demonstrated that lay health worker interventions were cost-effective. CONCLUSIONS: Lay health workers interventions in children with chronic conditions may lead to modest improvements in urgent care use, symptoms, and parental psychosocial outcomes. Such interventions may also be cost-effective. Future research should focus on interventions targeted toward other chronic conditions such as sickle cell disease or cystic fibrosis and medically complex children whose conditions are noncategorical.