Current Pediatric Reviews - Volume 15, Issue 3, 2019

Congenital heart disease is present in almost 1% of live births and despite current progress in prenatal screening a significant percentage has delayed diagnosis or remain undiagnosed. A cardiac murmur may be the first or unique clinical sign of congenital heart disease in childhood, however, less than 1% of auscultated murmurs are of an organic cause. Distinguishing between an innocent and a pathologic murmur can be challenging and the experience of the examiner is crucial for identifying the distinctive properties of an innocent murmur. Timely diagnosis of underlying cardiovascular pathology is of great significance so that prompt management is provided and morbidity or mortality are restricted. Of similar importance is the avoidance of unnecessary anxiety for the parents and unreasonable referrals to Paediatric Cardiologists. Indications for referral include a medical history suggestive of a cardiac abnormality, such as the presence of relevant symptoms, the identification of abnormal findings on clinical examination, auscultatory findings suggestive of an organic murmur, and very young patient age. ECG and a chest X-ray are not usually part of the diagnostic approach of a child with a cardiac murmur, as they do not increase the success rate of diagnosing heart disease, as compared to a detailed medical history accompanied by a thorough physical examination. In conclusion, the recognition of suspicious distinctive features of cardiac murmurs is crucial and requires skills based on sufficient training and experience.

Congenital Central Hypoventilation Syndrome (CCHS), also referred with the expression “Ondine’s Curse”, is a rare genetic life-long disease resulting from the mutation of PHOX2B gene on chromosome 4p12.3. CCHS represents an autonomic nervous system disorder; its more fearsome manifestation is central hypoventilation, due to a deficient response of chemoreceptors to hypercapnia and hypoxia. Several associated symptoms can occur, such as pupillary anomalies, arrhythmias, reduced heart rate variability, esophageal dysmotility, and structural comorbidities (Hirschsprung’s Disease or neural crest tumours). CCHS typical onset is during the neonatal period, but cases of delayed diagnosis have been reported; moreover, both sporadic or familial cases can occur. In preterm newborns, asphyxia and typical prematurity-related findings may overlap CCHS clinical manifestations and make it harder to formulate a correct diagnosis. The early recognition of CCHS allows appropriate management, useful to reduce immediate and long- term consequences.

Background: If not timely diagnosed and adequately treated, skeletal system infections in children may result in severe and permanent disability. Prompt identification of the etiology of the disease and determination of its antibiotic susceptibility are crucial for the successful management of septic arthritis, osteomyelitis, and spondylodiscitis. However, the bacteriological diagnosis of these infections has been traditionally limited by the low yield of conventional cultures and, on average, one-third of cases of pediatric joint and bone infections remained unconfirmed. Objective: To review the medical literature to summarize the current approach diagnosing the pediatric skeletal system infections. Methods: The relevant publications for the last three decades were reviewed. Results: In recent years, the detection of skeletal system pathogens has been revolutionized by the use of improved laboratory methods, including seeding of synovial fluid and bone exudates into blood culture vials, and the development and implementation of sensitive nucleic acid amplification assays. These advances have resulted in the recognition of Kingella kingae as the predominant etiology of hematogenous infections of bones, joints, intervertebral discs and tendon sheaths in children aged 6-48 months, and reduced the fraction of culture-negative osteoarthritis. Conclusion: As the exudate and tissue samples obtained from young children with skeletal system infections are frequently insufficient for a comprehensive laboratory workup, physicians should take in consideration the patient’s age, predisposing medical conditions and possible exposure to zoonotic organisms, and use a judicious combination of Gram’s stain, culture on blood culture vials, and molecular tests to maximize the microbiological diagnosis of these diseases.

Background: Pica is a common condition in childhood that is commonly missed. Objective: To familiarize physicians with the clinical evaluation and management of children with pica. Methods: A PubMed search was completed in Clinical Queries using the key term "pica" OR “dirteating”. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. Only papers published in English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article. Results: Pica refers to the persistent, compulsive craving for and the ingestion of substances usually considered inedible and the behavior is discordant with cultural practices and continues beyond the normal developmental phase of occasional indiscriminate and experimental mouthing and swallowing over a period of at least one month. The condition is more common among children in lower socioeconomic classes and those who are mentally handicapped or emotionally deprived. Pica is a significant cause of anemia and lead poisoning. Pica generally resolves in children of normal intelligence after they have been trained to discriminate between edible and inedible items and proper supervision is provided. While relief of family economic and housing difficulties is an adjunct, attention to the individual’s emotional needs and stresses is of paramount importance. Children with iron deficiency anemia should be treated with iron replacement therapy. Complications such as gastrointestinal obstruction and lead poisoning should be promptly recognized and treated. Conclusion: Pica is often an overlooked phenomenon and its association with iron deficiency and lead poisoning has been known for centuries. The underlying cause and complications should be treated if possible. Primary care physicians should be aware of pica and proactively seek information about pica in patients that belong to the high-risk groups.

Background: Tinea imbricata is a chronic superficial mycosis caused mainly by Trichophyton concentricum. The condition mainly affects individuals living in primitive and isolated environment in developing countries and is rarely seen in developed countries. Physicians in nonendemic areas might not be aware of this fungal infection. Objective: To familiarize physicians with the clinical manifestations, diagnosis, and treatment of tinea imbricata. Methods: A PubMed search was completed in Clinical Queries using the key terms "Tinea imbricata" and "Trichophyton concentricum". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, reviews, and case reports. The information retrieved from the above search was used in the compilation of the present article. Results: The typical initial lesions of tinea imbricata consist of multiple, brownish red, scaly, pruritic papules. The papules then spread centrifugally to form annular and/or concentric rings that can extend to form serpinginous or polycyclic plaques with or without erythema. With time, multiple overlapping lesions develop, and the plaques become lamellar with abundant thick scales adhering to the interior of the lesion, giving rise to the appearance of overlapping roof tiles, lace, or fish scales. Lamellar detachment of the scales is common. The diagnosis is mainly clinical, based on the characteristic skin lesions. If necessary, the diagnosis can be confirmed by potassium hydroxide wet-mount examination of skin scrapings of the active border of the lesion which typically shows short septate hyphae, numerous chlamydoconidia, and no arthroconidia. Currently, oral terbinafine is the drug of choice for the treatment of tinea imbricata. Combined therapy of an oral antifungal agent with a topical antifungal and keratolytic agent may increase the cure rate. Conclusion: In most cases, a spot diagnosis of tinea imbricata can be made based on the characteristic skin lesions consisting of scaly, concentric annular rings and overlapping plaques that are pruritic. Due to popularity of international travel, physicians involved in patient care should be aware of this fungal infection previously restricted to limited geographical areas.

Background: Various complementary or alternative medicines (including breathing exercises and yoga/pranayama) have been tried as an attractive option to pharmacotherapy in childhood asthma. Objective: To evaluate the role of breathing exercise and yoga/pranayama as add on therapy to the “pharmacologically recommended treatment” of childhood asthma. Methods: We searched the published literature in the major databases: Medline via Ovid, PubMed, CENTRAL, Embase, and Google Scholar till June 2018. Randomized trials comparing breathing exercises and yoga/ pranayama versus control or as part of a composite intervention versus control were included. The primary outcome measures were quality of life and change in asthma symptoms. Secondary outcomes were: decrease in medication use, number of exacerbations, change in lung function and immunological parameters, school absenteeism and adverse events. Results: A total of 10 trials (466 children, 6-14 years age) were included. The severity of asthma varied among the trials. The data for primary outcome measures could not be pooled, there were mixed results for both primary and secondary outcomes. No significant benefit was obtained in acute asthma and the lung function tests [except PEFR % at 4-6 weeks, PEF absolute at 3 months, and FVC absolute at 3 months] in chronic asthma. One trial compared breathing exercise versus yoga and found no difference. Adverse events were not significant. Conclusions: Breathing exercise and yoga/ pranayama may have some additive role in the treatment of childhood asthma. However, at present, it cannot be recommended as a standard of care due to insufficient data.

Background: Increased oxidative stress has been reported in autistic patients besides, evidence linking oxidative stress to enhancement of advanced glycation and lipoxidation end products (AGEs and ALEs) and their precursors. Objective: This study aimed to compare the plasma levels of the AGEs and precursors of ALEs in autistic and healthy children and to evaluate their relationship with autism comorbidities. Methods: In this descriptive study, 54 children, 36 autistic and 18 healthy participated. Plasma levels of AGEs and precursors of ALEs were measured by ELISA method. Severity of autism and Gastrointestinal (GI) disorders were measured by GARSII questionnaire and QPGS-ROME III questionnaire, respectively. Results: Plasma levels of AGEs and precursors of ALEs in autistic children were comparable with healthy children. Plasma levels of AGEs and precursor of ALEs were correlated with physical activity and GI disorders in autistic children. A strong association was also found between AGEs and precursors of ALEs. Conclusion: The results indicate that AGEs and ALEs have a strong correlation together but the AGEs and precursor of ALEs in autistic children are not different from healthy children.

Aim: To describe the methodology in studying patient’s acceptability and efficacy of an ectoin containing emollient for atopic dermatitis (AD). Methods: We described the methodology that we used in studying emollients and moisturisers, and patient acceptability of a group of AD patients before and following usage of an ectoin-containing proprietary emollient. These data were also compared with other brand emollients that we previously reported, namely Restoradom®, Ezerra® and Ezerra plus®. Results: 30 subjects (50% Male, Mean (SD) age: 9.8 (3.6) years with AD used the trial emollient W for four weeks. AD severity of subjects (by objective SCORAD) was moderate (n=22) and severe (n=8). Compliance was good and patients generally managed to use the moisturisers daily, with individual reports of a ‘tingly’ sensation by some subjects when applied to inflamed wounds. 63% reported “very good” or “good”, whereas 37% reported “fair” or “poor” acceptability of the moisturisers. Following use of the trial emollient, area affected, disease intensity and severity significantly improved, as demonstrated in objective SCORAD (p=0.002). There were also significant improvements in POEM (p=0.035), and PADQLQ scores (p=0.017). For skin measurements, only transepidermal water loss had improved (p=0.035) after the treatment. There was no significant improvement of itch or sleep scores, skin hydration, pH, S. aureus colonization status, or need for use of topical medications. When compared with historical data of other emollients, the mean age of patients on emollient W was younger; efficacy and acceptability among these emollients were similar. Conclusion: Methodology of emollient research is described. Doctors should provide evidencebased information about the efficacy of emollients. The ectoin-containing proprietary emollient improves disease and quality of life following its use in 4 weeks. Efficacy and acceptability are similar among 4 proprietary emollients.