Disease In Infants Research Articles

Farber disease in infancy resembling juvenile idiopathic arthritis: identification of two new mutations and a good early response to allogeneic haematopoietic stem cell transplantation

Farber disease in infancy resembling juvenile idiopathic arthritis: identification of two new mutations and a good early response to allogeneic haematopoietic stem cell transplantation

Evaluation of quality of life by young adult survivors of severe chronic kidney disease in infancy

The health related quality of life (HRQoL) of young adults treated for chronic kidney disease (CKD) stage 4/5 from infancy is unknown. A HRQoL questionnaire was sent to all 41 patients aged >16 years from a previously characterised cohort of infants with CKD stage 4/5 born between 1986 and 1997. Patient scores were compared with a previously reported cohort of patients who needed renal replacement therapy (RRT) in mid childhood and in the normal population. All patients (11 women) completed the questionnaire at a median (range) age of 19.2 (16.3-23.4) years. At the time of the survey, 5 (12.5 %) were on dialysis, 35 (85.5 %) had a functioning kidney transplant, one (2 %) was still conservatively treated and 22 (54 %) had comorbidities; 68 % were either studying or in paid employment, with 17 % actively seeking employment. Although patients described a lower HRQoL than a healthy, age-matched UK group, in some aspects, scores were comparable with patients needing RRT in later childhood. Lower scores were associated with comorbidities, dialysis at last follow-up, more than one treatment modality change and short stature. Our survey demonstrates very encouraging results for long-term HRQoL of infants with severe CKD and highlights the negative impact of comorbidities. These data will help clinicians to counsel and inform families.

O035 Current Victorian antenatal detection rates of congenital heart disease in infancy

O035 Current Victorian antenatal detection rates of congenital heart disease in infancy

Omega-6 and Omega-3 Polyunsaturated Fatty Acids and Allergic Diseases in Infancy and Childhood

There may be a causal relationship between intake of n-6 polyunsaturated fatty acids (PUFAs) and childhood allergic diseases. This can be explained by plausible biological mechanisms involving eicosanoid mediators produced from the n-6 PUFA arachidonic acid. Long chain n-3 PUFAs are found in fish and fish oils. These fatty acids act to oppose the actions of n-6 PUFAs. Thus, it is considered that n-3 PUFAs will lower the risk of developing allergic diseases. In support of this, protective associations have been reported between maternal fish intake during pregnancy and allergic outcomes in infants and children from those pregnancies. However, studies of fish intake during infancy and childhood and allergic outcomes in those infants or children are inconsistent, although some reported a protective association. Supplementing pregnant women with fish oil can induce immunologic changes in cord blood. This supplementation has been reported in some studies to decrease sensitisation to common food allergens and to lower the prevalence and severity of atopic dermatitis in the first year of life. The protective effect of maternal n-3 PUFAs may last until adolescence of the offspring. Fish oil supplementation in infancy may decrease the risk of developing some manifestations of allergic disease, although this benefit may not persist. Whether fish oil is a useful therapy in children with asthma receiving standard therapy is not clear from studies performed to date and this requires further exploration.

Novel vaccine regimen elicits strong airway immune responses and control of respiratory syncytial virus in nonhuman primates.

Induction of long-lasting immunity against viral respiratory tract infections remains an elusive goal. Using a nonhuman primate model of human respiratory syncytial virus (hRSV) infection, we compared mucosal and systemic immune responses induced by different DNA delivery approaches to a novel parenteral DNA prime-tonsillar adenoviral vector booster immunization regimen. Intramuscular (i.m.) electroporation (EP) of a DNA vaccine encoding the fusion protein of hRSV induced stronger systemic immune responses than intradermal EP, tattoo immunization, and conventional i.m. DNA injection. A single EP i.m., followed by two atraumatic tonsillar immunizations with the adenoviral vector, elicited strong systemic immune responses, an unique persistent CD4(+) and CD8(+) T cell response in the lower respiratory tract and protection from intranasal hRSV challenge. Thus, parenteral DNA priming followed by booster immunization targeted to a mucosal inductive site constitutes an effective vaccine regimen for eliciting protective immune responses at mucosal effector sites. The human respiratory syncytial virus (hRSV) is the most common cause of severe respiratory tract disease in infancy and leads to substantial morbidity and morality in the elderly. In this study, we compared the immunogenicity and efficacy of several gene-based immunization protocols in rhesus macaques. Thereby, we found that the combination of an initially parenterally delivered DNA vaccine with a subsequent atraumatic tonsillar adenoviral vector immunization results in a strong systemic immune response accompanied by an exceptional high T-cell response in the mucosa. Strikingly, these animals were protected against a RSV challenge infection controlling the viral replication indicated by a 1,000-fold-lower viral load in the lower respiratory tract. Since mucosal cellular responses of this strength had not been described in earlier RSV vaccine studies, this heterologous DNA prime-tonsillar boost vaccine strategy is very promising and should be pursued for further preclinical and clinical testing.

Cystic Fibrosis in the Genomic Era: CFTR Genotyping as a Diagnostic Test

Our insight into cystic fibrosis (CF) disease and diseases associated with CF gene mutations has significantly increased in recent years, particularly after the discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene over two decades ago. This has resulted in a widened spectrum of phenotypic manifestations of CF ranging from the classic multisystem disease in infancy and early childhood to adults with single-organ manifestations of CF such as chronic sinopulmonary disease, pancreatitis and obstructive azoospermia. As a consequence, the diagnosis of CF can be difficult to establish or exclude. Extensive CFTR genotyping has been limited by the inability to interpret the functional and clinical significance of a large number of identified CFTR mutations. As identified CFTR mutations can either be “disease-causing”, “of varying clinical consequences”, “benign” or have unknown consequences, the identification of mutations on both alleles may be insufficient alone to confirm the diagnosis of CF. More recently though, the number of mutations recognized to be disease-causing was greatly expanded by the Clinical and Functional Translation of CFTR (CFTR2) project. This review article discusses the role and yield of CFTR mutation analysis in the diagnosis of CF.

Children’s Interstitial Lung Diseases in Early Infancy

Children’s interstitial lung disease is a group of relatively rare pulmonary disorders that can present in the neonatal or early infancy periods. A classification scheme was developed in 2007 to aid clinicians and researchers in more accurate recognition and diagnosis. Infants who present with a prolonged oxygen requirement and/or persistent tachypnea may suffer from one of these conditions. Although more widely described afflictions, such as pulmonary hypoplasia or genetic errors of surfactant metabolism, may be considered in the differential diagnosis of these symptoms, a few of the remaining disorders have been only recently described, making them important entities to become familiar with in contemporary medical practice. This article describes the lesser-known conditions that have the potential to cause significant pulmonary disease in infancy.

Diffuse lung disease in infancy and childhood: expanding the chILD classification

Diffuse parenchymal lung diseases (DPLD) in children comprise a wide spectrum of rare disorders. In 2007 the Children's Interstitial Lung Disease (chILD) Research Cooperative proposed a classification system for DPLD in children <2 years of age. The aims of our study were to determine the utility and reproducibility of this system in children <2 years of age, and test its extension to 18 years of age. Of 211 cases, 93 were <2 years of age at presentation and 58% were included in the chILD classification. In 118 cases aged between 2 and 18 years there was a wider distribution of disorders, overlapping with those seen in adults, necessitating expansion of the chILD classification types to encompass all reviewed cases, in particular patients with 'adult' diffuse lung diseases. Many cases showed mixed histological patterns, overlap often being between groups of disorders more prevalent in infancy. Concordance between reporting pathologists was 90%. The chILD scheme allows classification of conditions more common in children <2 years of age. It can be applied to children of any age, although additional entities need to be included. We propose a more histologically based system for use when assessing biopsies in this context.

A birth cohort study of viral infections in Vietnamese infants and children: study design, methods and characteristics of the cohort

BackgroundIn Ho Chi Minh City, Vietnam, more than one-third of admissions to the two paediatric hospitals are attributable to four infectious syndromes: dengue, diarrhoeal disease, acute respiratory infection, and hand, foot and mouth disease. We have established a large prospective birth cohort study to investigate individual, environmental, virological, and immunological determinants of infection and disease in infants. Specific research questions are focused on the role of maternal antibody in protection against infection in infancy, and the adaptive immune response to vaccination and natural infection. This paper presents the cohort design, methods, and baseline characteristics of the participants enrolled in the first two years.Methods/designWomen are enrolled prior to delivery at one hospital in each of two catchment areas: an urban district in central HCMC, and a mixed urban/rural district in the Mekong Delta 150 km southwest of HCMC. Infants are enrolled within 3 days of birth, and maternal and cord blood samples are collected. Routine blood samples and data on growth, health status and vaccinations are collected from infants at scheduled visits at 4, 9 and 12 months. Clinical data and specimens are collected from infants presenting at a study clinic, or admitted to hospital, with any of the the four infectious syndromes of interest.DiscussionIn four years since since the study began in July 2009, >6400 infants have been enrolled, and enrolment is ongoing. Attrition is low: 84% of participants have completed the full 12-month follow-up period. Baseline characteristics of the first 4300 enrollees are presented here. We have demonstrated the feasibility of establishing a large prospective study of infectious diseases in infancy in a resource-limited setting, with minimal loss to follow-up. Our linked socio-demographic, clinical and laboratory data will help elucidate the viral aetiology and epidemiology of common infectious diseases of infancy, and can inform the implemention of existing and future vaccines. This study furthermore provides a platform to which additional endpoints could be added in the future.

Chronic pruritus associated with dermatologic disease in infancy and childhood: Update from an interdisciplinary group of dermatologists and pediatricians

An effective treatment strategy for chronic pruritus in children with dermatologic disorders should consider the multidimensional aspects of pruritus, the unique challenges associated with treating pruritic skin disorders in the pediatric population, and evidence-based therapies with demonstrated antipruritic benefits and clinically relevant effects on patient/family quality of life (QoL). The Course of Advanced Learning for the Management of ITch (CALM-IT) Task Force is an interdisciplinary group of experts specializing in core aspects of pruritus treatment, integrating pediatrics, dermatology, psychotherapy, pruritus management, and sleep. CALM-IT recently convened to provide updated guidance on managing chronic pruritus associated with dermatologic diseases in pediatric patients, with a special focus on atopic dermatitis (AD) and chronic spontaneous urticaria (csU). This review highlights the updated concepts and best practices, which were built upon international PRACTALL consensus and modified for children and infants with AD and csU. CALM-IT supports the routine use of basic skin therapy and the escalation of topical medications, according to severity and focused on rapid itch control. Anti-inflammatory agents should be appropriate for infants and children (i.e., with an optimized therapeutic index) and have proven antipruritic properties, such as those demonstrated by methylprednisolone aceponate. New experimental findings do not support the use of non-sedating oral antihistamines as adjuvant antipruritic therapy for AD. In csU, oral H1 -antihistamine use is justified, consistent with the distinct pathophysiologic mechanisms of itch underlying AD and csU. All encompassing QoL assessments should consider the burden of both patient and caregiver and should address outstanding unmet clinical needs of pediatric patients. Future research areas include integrated QoL assessments and multidisciplinary treatment programs with pediatric-targeted pruritic therapies providing rapid itch control.

An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy

There is growing recognition and understanding of the entities that cause interstitial lung disease (ILD) in infants. These entities are distinct from those that cause ILD in older children and adults. A multidisciplinary panel was convened to develop evidence-based guidelines on the classification, diagnosis, and management of ILD in children, focusing on neonates and infants under 2 years of age. Recommendations were formulated using a systematic approach. Outcomes considered important included the accuracy of the diagnostic evaluation, complications of delayed or incorrect diagnosis, psychosocial complications affecting the patient's or family's quality of life, and death. No controlled clinical trials were identified. Therefore, observational evidence and clinical experience informed judgments. These guidelines: (1) describe the clinical characteristics of neonates and infants (<2 yr of age) with diffuse lung disease (DLD); (2) list the common causes of DLD that should be eliminated during the evaluation of neonates and infants with DLD; (3) recommend methods for further clinical investigation of the remaining infants, who are regarded as having "childhood ILD syndrome"; (4) describe a new pathologic classification scheme of DLD in infants; (5) outline supportive and continuing care; and (6) suggest areas for future research. After common causes of DLD are excluded, neonates and infants with childhood ILD syndrome should be evaluated by a knowledgeable subspecialist. The evaluation may include echocardiography, controlled ventilation high-resolution computed tomography, infant pulmonary function testing, bronchoscopy with bronchoalveolar lavage, genetic testing, and/or lung biopsy. Preventive care, family education, and support are essential.

No effect of probiotics on respiratory allergies: a seven‐year follow‐up of a randomized controlled trial in infancy

Supplementation with the probiotic Lactobacillus reuteri reduced the incidence of IgE-associated allergic disease in infancy. This treatment might therefore also reduce the risk of asthma and allergic rhinoconjunctivitis in school age. To evaluate whether perinatal and infant supplementation with L. reuteri reduced the prevalence of respiratory allergic disease in school age and to explore whether this supplementation was associated with any long-term side effects. A randomized, placebo-controlled trial with oral supplementation with L. reuteri ATCC 55730 (1 × 10(8) CFU) during the last month of gestation and through the first year of life comprising 232 families with allergic disease, of whom 184 completed a 7-yr follow-up. The primary outcomes at 7 yr of age were allergic disease and skin prick test reactivity (ClinicalTrials.gov ID NCT01285830). The prevalence of asthma (15% in the probiotic vs. 16% in placebo group), allergic rhinoconjunctivitis (27% vs. 20%), eczema (21% vs. 19%) and skin prick test reactivity (29% vs. 26%) was similar in the probiotic and placebo group. Growth indices and gastrointestinal symptoms were similar in the two groups. No severe adverse events were reported. The effect of L. reuteri on sensitization and IgE-associated eczema in infancy did not lead to a lower prevalence of respiratory allergic disease in school age. Thus, the effect of L. reuteri on the immune system seems to be transient. Administration of L. reuteri during the last weeks of gestation and in infancy was not associated with any long-term side effects.

Variable clinical outcome of ABCA3 deficiency in two siblings

This case report describes an unusual outcome of ABCA3 deficiency with resolution of symptoms, normalization of chest imaging and lung function in a 9-year-old child whose sibling died of the same disease in infancy.

Functional residual capacity (FRC) and lung clearance index (LCI) in mechanically ventilated infants: Application in the newborn with congenital diaphragmatic hernia (CDH)

IntroductionFunctional residual capacity (FRC) and lung clearance index (LCI) are sensitive parameters for early detection of airway disease in infancy. The closed helium dilution method has been applied to assess lung volume and ventilation inhomogeneity (VI) in spontaneously breathing infants. AimsThe aims of this study were as follows: (1) to assess applicability of the helium gas dilution technique in mechanically ventilated infants with high-risk congenital diaphragmatic hernia (CDH) and to evaluate changes in breathing patterns, lung volume, and VI during the first days of life before and after surgery, and (2) to analyze the possible correlation between changes in lung volume, cerebral hemodynamics, and oxygenation before and after surgical correction of CDH through near-infrared spectroscopy (NIRS) monitoring. MethodsLung function tests were performed by multibreath washout traces with an ultrasonic flowmeter and helium gas dilution technique. For all babies, three acceptable FRC and LCI measurements were collected for each test (mean and SD of three measurements were calculated) before surgery (T0), 24h after surgery (T1) during mechanical ventilation, and within 24h after extubation in spontaneous breathing (T2). Cerebral and splanchnic hemodynamics were continuously monitored by NIRS during mechanical ventilation to evaluate relationships between changes in lung volume and capillary–venous oxyhemoglobin saturation in tissues. Fraction of inspired oxygen delivered was adjusted to keep oxygen saturation between 90% and 95%. ResultsThirteen CDH infants were studied; median GA=38weeks (range 35–41) and median BW=3000g (range 1850–3670). FRC and LCI significantly improved after extubation when compared with pre-surgical values. No differences were found in tidal volume (Vt) and NIRS monitoring before and after surgery and after extubation. Neither LCI nor FRC was correlated with NIRS values. ConclusionsHelium gas dilution technique is an applicable and reliable technique to measure lung volumes and ventilation inhomogeneity also in ventilated infants. NIRS is a non-invasive technique to monitor tissue oxygenation during surgery and mechanical ventilation. In CDH newborns these preliminary data show an improvement in both FRC and LCI after extubation.

Implications of Bicuspid Aortic Valves for Transcatheter Aortic Valve Implantation

Bicuspid aortic valve (BAV) occurs in 0.5% to 2% of the general population, with strong male predilection.1 Valvular complications include aortic stenosis, aortic regurgitation (AR), and infective endocarditis. Vascular complications comprise aortic dilatation, aneurysm formation, and aortic dissection. Coarctation of the aorta and left coronary artery dominance are also associated with BAV.1 BAV consists of 2 cusps, usually of unequal size.2 The larger cusp may contain 1 or 2 raphes—fibrous ridges extending from the malformed commissure to the free edge of the 2 conjoint cusps.2 An anatomic classification distinguishes BAV types principally by the number of raphes (Figure).2 Because a raphe can be missed on echocardiography, Schaefer et al3 proposed 3 types based instead on the pattern of leaflet fusion. Clinical presentation of BAV is variable. Some patients present with severe disease in infancy, whereas others present with asymptomatic disease in old age.1 In a recent series of 642 asymptomatic adults with BAV referred to a tertiary center, 25% had a primary cardiac event at mean follow-up of 9 years.4 Aortic valve or ascending aorta intervention comprised most (22%) events.4 In another series of 212 asymptomatic adults with BAV recruited from the community, primary cardiac outcomes occurred in 42% at 20-year follow-up.5 Overall survival rates were similar to the general population.5 Figure. Anatomic classification of bicuspid aortic valves (BAV) based on a study of 304 surgical specimens.2 Prominent black lines denote the raphe (s). Type 0=no raphe, type 1=1 raphe, and type 2=2 raphes. Each type is subcategorized according to the spatial position of the cusps (type 0) or raphes (types 1 and 2). Percentages represent the approximate prevalence of each BAV type observed in the Sievers study.2 ap indicates anterior-posterior; L, left coronary sinus; lat, lateral; …

Association of lung function, chest radiographs and clinical features in infants with cystic fibrosis

The optimal strategy for monitoring cystic fibrosis lung disease in infancy remains unclear. Our objective was to describe longitudinal associations between infant pulmonary function tests, chest radiograph scores and other characteristics. Cystic fibrosis patients aged ≤24 months were enrolled in a 10-centre study evaluating infant pulmonary function tests four times over a year. Chest radiographs ∼1 year apart were scored using the Wisconsin and Brasfield systems. Associations of infant pulmonary function tests with clinical characteristics were evaluated with mixed effects models. The 100 participants contributed 246 acceptable flow/volume (forced expiratory volume in 0.5 s (FEV0.5) and forced expiratory flow at 75% of the forced vital capacity (FEF75%)), 303 functional residual capacity measurements and 171 chest radiographs. Both Brasfield and Wisconsin chest radiograph scores worsened significantly over the 1-year interval. Worse Wisconsin chest radiograph scores and Staphylococcus aureus were both associated with hyperinflation (significantly increased functional residual capacity), but not with diminished FEV0.5 or FEF75%. Parent-reported cough was associated with significantly diminished forced expiratory flow at 75% but not with hyperinflation. In this infant cohort in whom we previously reported worsening in average lung function, chest radiograph scores also worsened over a year. The significant associations detected between both Wisconsin chest radiograph score and S. aureus and hyperinflation, as well as between cough and diminished flows, reinforce the ability of infant pulmonary function tests and chest radiographs to detect early cystic fibrosis lung disease.

Relationship between breast milk sCD14, TGF‐β1 and total IgA in the first month and development of eczema during infancy

The overall beneficial effects of breastfeeding for infants have been well documented, but its role in allergy prevention is controversial. We investigated the relationship between breast milk immunomodulatory factors and subsequent development of eczema and atopic sensitization in the first year of life. Day 7 and 28 breast milk samples were collected from mothers carrying infants at high risk of allergic disease. Aqueous-phase breast milk samples were assayed for TGF-β1, sCD14 and total IgA. Infants were assessed for the presence of eczema and atopic sensitization at 12months of age. The levels of breast milk TGF-β1, sCD14 and total IgA were compared in infants who subsequently developed eczema and sensitization in the first year and those who did not. The levels of breast milk sCD14, total IgA, and TGF-β1 at either day 7 or 28 were not associated with subsequent development of eczema or atopic sensitization during the first year of life. Levels of breast milk immune parameters were not associated with eczema outcomes or sensitization in infants at 12months. This suggests that apparent immunological effects on breast milk immunomodulatory factors may not necessarily lead to clinical benefits, and these immune markers may not be critical determinants of allergic disease in infancy.

Antibodies to Deamidated Gliadin Peptides: An Accurate Predictor of Coeliac Disease in Infancy

Immunoglobulin G antibodies against deamidated gliadin peptides are now known to have diagnostic accuracy comparable to tissue transglutaminase and endomysium autoantibodies in patients with coeliac disease. However, little is known about their predictive value in infants with a suspected gluten enteropathy. We tested whether deamidated gliadin immunoglobulin G antibodies are more reliable than traditional tests for coeliac disease screening in infancy. Sixty-five children under 2 years of age (42 with malabsorption, 23 controls) were tested for deamidated gliadin immunoglobulin G, tissue transglutaminase and endomysium immunoglobulin A, and gliadin immunoglobulins A and G . Thirty-seven of the 42 children with malabsorption had deamidated gliadin antibodies, associated with tissue transglutaminase and endomysial antibodies in 33, and with gliadin immunoglobulins A and G in 21 and 29, respectively. Intestinal biopsy was performed in 34 of the 37 children positive for deamidated gliadin antibodies. Thirty-two/34 showed villous atrophy consistent with coeliac disease, while the remaining two had a Marsh 1 and a normal mucosa, respectively. Only gliadin immunoglobulins A (4.3%) and G (39.1%) were found in controls. The sensitivity of deamidated gliadin, tissue transglutaminase and endomysial antibodies for coeliac disease was significantly higher than that of gliadin immunoglobulins G and A. High titre deamidated gliadin antibodies correlated with severe intestinal damage. Deamidated gliadin antibodies showed a higher diagnostic accuracy for coeliac disease than gliadin antibodies in infancy. High titre deamidated gliadin antibodies predict a severe gluten-dependent duodenal damage.

Recurrence of Infantile Cortical Hyperostosis

Also named Caffey disease, infantile cortical hyperostosis is a rare disease that usually affects children of a few weeks of age. The clinical picture is that of irritability, soft tissue swelling at various sites (mandible, clavicle, limbs) with local warmth, and pain on palpation. Radiographs demonstrate an important cortical thickening of the affected bony structures. There is generally spontaneous resolution of the inflammatory signs within the first few months or years of life, without future recurrence. We report the case of a recurrence in the adolescent period and conducted a thorough review of the literature to confirm this rare possibility. We report the case of a 12-year-old female patient, presenting symptoms of pain and swelling of both forearms and legs, similar to her initial clinical picture at 1 month of age when the diagnosis of Caffey disease was made. Genetic analysis confirmed the COL1A1 mutation, the classic mutation described in the familial form of this disease. Radiologic investigation revealed new periosteal bone formation of the right and left ulnae and the left fibula, suggesting a recurrence of cortical hyperostosis. A thorough review of the literature was conducted, using the Medline database between 1948 and 2011, to confirm this hypothesis. The literature confirms the possibility but also the rarity of a recurrence of cortical hyperostosis in an adolescent who was diagnosed with Caffey disease in infancy. We have identified less than a dozen cases in the literature describing such a recurrence and in the majority there are no medical photographs or radiographs to support the clinical diagnosis. Our case is well documented both clinically and radiologically regarding the initial presentation and the recurrence in adolescence. Although very rare and poorly recognized, the diagnosis of recurrent cortical hyperostosis must be evoked in a patient who suffered from Caffey disease in infancy, and presents similar clinical characteristics in the adolescent period. Level V--case report.

An Evaluation on the Effectiveness of Structured Teaching Programme Regarding Prevention and Management of Napkin Dermatitis in Infants

The neonates are unique in several ways in comparison with older children and adults which render them highly susceptible to severe dermatological disorders. Skin diseases in infancy and childhood often-present patterns, which may diverge from the same diseases in adults. Infants can get a variety of skin diseases, and some are more common than others. Infants are particularly at risk for developing diaper dermatitis and its potential consequences. It is one of the most common skin problems in infants and children, affecting between 7 and 35 per cent of infants at some point. Diaper rash occurs most commonly in infants between ages nine and 12 months, but can occur at any time the child wears a diaper. The present study was conducted to assess the effectiveness of structured teaching programme on prevention and management of napkin dermatitis in infants’ on mothers residing at Rural Naini, Allahabad. The conceptual framework set up for the study is Conceptual framework based on modified general system theory (Bertalamffy & JW Kenny). The model which has four phases i.e. input, throughput, output and feedback, context, It provide comprehension, systematic and continuous ongoing frame work for program evaluation and hence helped the investigator to assess the effectiveness of structured teaching programme on prevention and management of napkin dermatitis in infants and assess the level of knowledge gained by the mothers regarding prevention and management of napkin dermatitis in infants. In this study various literature was reviewed which includes, the research design selected for the study was pre experimental research design of one group pre-test post-test design. The independent variable was structured teaching programme and dependent variables were level of knowledge of mothers in pre-test and post-test.