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  1. Leung AKC, Lam JM, Barankin B, Wong AHC, Leong KF, Hon KL
    Curr Pediatr Rev, 2024 Jan 28.
    PMID: 38288810 DOI: 10.2174/0115733963283507240115112552
    BACKGROUND: Pinworm infestation is an important public health problem worldwide, especially among children 5 to 10 years of age in developing countries with temperate climates. The problem is often overlooked because of its mild or asymptomatic clinical manifestations.

    OBJECTIVE: The purpose of this article was to familiarize pediatricians with the diagnosis and management of pinworm infestation.

    METHODS: A search was conducted in August 2023 in PubMed Clinical Queries using the key terms "Enterobius vermicularis," OR "enterobiasis," OR "pinworm." The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article.

    RESULTS: Enterobiasis is a cosmopolitan parasitosis caused by Enterobius vermicularis. It affects approximately 30% of children worldwide and up to 60% of children in some developing countries. Predisposing factors include poor socioeconomic conditions, inadequate sanitation, poor personal hygiene, and overcrowding. Children aged 5 to 14 years have shown the highest prevalence of enterobiasis.. Egg transmission is mainly by the fecal-oral route. Approximately 30 to 40% of infested patients do not show any clinical symptoms of the disease. For symptomatic patients, the most common presenting symptom is nocturnal pruritus ani. The diagnosis of E. vermicularis infection is best established by the cellophane tape test. The sensitivity of one single test is around 50%; however, the sensitivity increases to approximately 90% with tests performed on three different mornings. If a worm is visualized in the perianal area or the stool, a pathological examination of the worm will yield a definitive diagnosis. As pinworms and eggs are not usually passed in the stool, examination of the stool is not recommended. The drugs of choice for the treatment of pinworm infestation are mebendazole (100 mg), pyrantel pamoate (11 mg/kg, maximum 1 g), and albendazole (400 mg), all of the above-mentioned drugs are given in a single dose and repeated in two weeks. Mebendazole and albendazole are both adulticidal and ovicidal, whereas pyrantel pamoate is only adulticidal. Given their safety and effectiveness, mebendazole and albendazole are currently the best available drugs for the treatment of pinworm infestation. For pregnant women, pyrantel is preferred to mebendazole and albendazole. Treatment of all household members should be considered, especially if there are multiple or repeated symptomatic infections because reinfection is common even when effective medication is given.

    CONCLUSION: In spite of effective treatment of pinworm infestation, recurrences are common. Recurrences are likely due to repeated cycles of reinfection (particularly, autoinfection) because of the short life span of adult pinworms. Good personal hygiene, such as frequent handwashing, especially after bowel movements and before meals, clipping of fingernails, avoidance of finger-sucking, nail-biting, and scratching in the anogenital area, are important preventive measures. Treatment of all household members should be considered, especially if there are multiple or repeated symptomatic infections.

  2. Leung AKC, Lam JM, Barankin B, Leong KF
    Curr Pediatr Rev, 2024 Jan 25.
    PMID: 38279714 DOI: 10.2174/0115733963273945240101042423
    BACKGROUND: Lichen striatus is a benign dermatosis that affects mainly children. This condition mimics many other dermatoses.

    OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations of lichen striatus to avoid misdiagnosis, unnecessary investigations, unnecessary referrals, and mismanagement of lichen striatus.

    METHODS: A search was conducted in June 2023 in PubMed Clinical Queries using the key term "Lichen striatus". The search strategy included all observational studies, clinical trials, and reviews published within the past ten years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of this article.

    RESULTS: Lichen striatus is a benign self-limited T-cell mediated dermatosis characterized by a linear inflammatory papular eruption seen primarily in children. The onset is usually sudden with minimal or absent symptomatology. The eruption in typical lichen striatus consists of discrete, skin- colored, pink, erythematous, or violaceous, flat-topped, slightly elevated, smooth or scaly papules that coalesce to form a dull red, potentially scaly, interrupted or continuous band over days to weeks. Although any part of the body may be involved, the extremities are the sites of predilection. Typically, the rash is solitary, unilateral, and follows Blaschko lines. In dark-skinned individuals, the skin lesions may be hypopigmented at onset. Nails may be affected alone or, more commonly, along with the skin lesions of lichen striatus. The differential diagnoses of lichen striatus are many and the salient features of other conditions are highlighted in the text.

    CONCLUSION: Lichen striatus is a self-limited condition that often resolves within one year without residual scarring but may have transient post-inflammatory hypopigmentation or hyperpigmentation. As such, treatment may not be necessary. For patients who desire treatment for cosmesis or for the symptomatic treatment of pruritus, a low- to mid-potency topical corticosteroid or a topical immunomodulator can be used. A fading cream can be used for post-inflammatory hyperpigmentation.

  3. Leung AKC, Lam J, Barankin B, Leong KF, Hon KL
    Curr Pediatr Rev, 2023 Jul 26.
    PMID: 37493159 DOI: 10.2174/1573396320666230726145436
    BACKGROUND: Group A ß-hemolytic streptococcus (GABHS) is the leading bacterial cause of acute pharyngitis in children and adolescents worldwide.

    OBJECTIVE: This article aims to familiarize clinicians with the clinical manifestations, evaluation, diagnosis, and management of GABHS pharyngitis.

    METHODS: A search was conducted in December 2022 in PubMed Clinical Queries using the key term "group A β-hemolytic streptococcal pharyngitis". This review covers mainly literature published in the previous ten years.

    RESULTS: Children with GABHS pharyngitis typically present with an abrupt onset of fever, intense pain in the throat, pain on swallowing, an inflamed pharynx, enlarged and erythematous tonsils, a red and swollen uvula, enlarged tender anterior cervical lymph nodes. As clinical manifestations may not be specific, even experienced clinicians may have difficulties diagnosing GABHS pharyngitis solely based on epidemiologic or clinical grounds alone. Patients suspected of having GABHS pharyngitis should be confirmed by microbiologic testing (e.g., culture, rapid antigen detection test, molecular point-of-care test) of a throat swab specimen prior to the initiation of antimicrobial therapy. Microbiologic testing is generally unnecessary in patients with pharyngitis whose clinical and epidemiologic findings do not suggest GABHS. Clinical score systems such as the Centor score and McIssac score have been developed to help clinicians decide which patients should undergo diagnostic testing and reduce the unnecessary use of antimicrobials. Antimicrobial therapy should be initiated without delay once the diagnosis is confirmed. Oral penicillin V and amoxicillin remain the drugs of choice. For patients who have a non-anaphylactic allergy to penicillin, oral cephalosporin is an acceptable alternative. For patients with a history of immediate, anaphylactic-type hypersensitivity to penicillin, oral clindamycin, clarithromycin, and azithromycin are acceptable alternatives.

    CONCLUSION: Early diagnosis and antimicrobial treatment are recommended to prevent suppurative complications (e.g., cervical lymphadenitis, peritonsillar abscess) and non-suppurative complications (particularly rheumatic fever) as well as to reduce the severity of symptoms, to shorten the duration of the illness and to reduce disease transmission.

  4. Leung AKC, Lam JM, Barankin B, Leong KF, Hon KL
    Curr Pediatr Rev, 2023 Apr 06.
    PMID: 37038684 DOI: 10.2174/1573396320666230406084801
    In the past decade, there has been a global resurgence of bed bug infestations, especially in developed countries. Proper awareness and identification of bed bug infestations are essential to guide treatment and eradication. The purpose of this article is to familiarize physicians with bed bug bites so that they can effectively diagnose, treat, and address questions about bed bug bites and infestations. Bed bug bites are often painless. Typical reactions include pruritic, erythematous maculopapules occurring in clusters or in a linear or curvilinear distribution in exposed areas of the body. A small red punctum may be visualized at the center of the bite mark. Lesions that appear three in a row and papules on the upper eyelid associated with erythema and edema are highly suggestive of bites from bed bugs. Exaggerated local reactions such as vesicles, urticarial wheals, urticarial perilesional plaques, diffuse urticaria, bullae, and nodules may occur in previously sensitized individuals. Reactions to bed bug bites are self-limited. As such, treatment is mainly symptomatic. Topical pramoxine and oral antihistamines can be used to alleviate pruritus. Topical corticosteroids can be used for significant eruptions to control inflammation and pruritus, and to hasten resolution of the lesions. Integrated pest management, an approach for the eradication of bed bugs, includes monitoring devices (active monitors include the use of heat or carbon dioxide attractants and passive monitors include the use of sticky pads for trapping), and judicious use of nonchemical and chemical treatments known to be effective. Nonchemical interventions include keeping affected areas clean and free of clutter, vacuuming, washing linens with hot water, caulking wall holes and cracks where bugs can hide, proper disposal of highly infested items, and placement of bed bug traps/interceptors at the base of beds and furniture. Chemical interventions involve the use of insecticides such as synthetic pyrethroids, silicates, insect growth disruptors, carbamates, organophosphates, neonicotinoids, diethyl-meta-toluamide, chlorfenapyr, fipronil and plant essential oils. Insecticides should be used with caution to prevent over-exposure and toxicity (in particular, cardiovascular and neurologic toxicity), especially if there are young children around. It is important to note that multiple mechanisms of insecticide resistance exist and as such, chemical treatment should only be undertaken by trained professionals who understand the current literature on resistance. Both nonchemical and chemical technologies should be combined for optimal results. Bed bug infestations may cause diverse dermal reactions, stigmatization, poor self-esteem, emotional stress, anxiety, significant adverse effect on quality of life, and substantial socioeconomic burden to society. As such, their rapid detection and eradication are of paramount importance. Consultation with a professional exterminator is recommended to fully eradicate an infestation.
  5. Leung AKC, Lam J, Barankin B, Leong KF, Hon KL
    Curr Pediatr Rev, 2022 Nov 18.
    PMID: 36411550 DOI: 10.2174/1573396319666221118123844
    BACKGROUND: Roseola infantum is a common viral disease that occurs during childhood worldwide.

    OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations, evaluation, diagnosis, and management of roseola infantum.

    METHODS: A search was conducted in April, 2022, in PubMed Clinical Queries using the key terms "roseola infantum" OR "exanthem subitum" OR "sixth disease". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article.

    RESULTS: Roseola infantum is a viral illness characterized by high fever that lasts 3 to 4 days, followed by the sudden appearance of rash at defervescence. The disease occurs most frequently in children between 6 months and 2 years of age. Human herpesvirus-6 (HHV-6) is the major cause of roseola infantum, followed by HHV-7. Transmission of the infection most likely results from the asymptomatic shedding of the virus in the saliva of the caregivers or other close contacts. Characteristically, the rash is discrete, rose-pink in color, circular or elliptical, macular or maculopapular, measuring 2 to 3 mm in diameter. The eruption is first seen on the trunk. It then spreads to the neck and proximal extremities. Typically, the rash blanches on pressure and subsides in 2 to 4 days without sequelae. Most children look well otherwise and appear to be happy, active, alert, and playful. The diagnosis is mainly clinical. Febrile seizures occur in 10 to 15 % of children with roseola infantum during the febrile period. In general, serious complications are rare and occur more often in individuals who are immunocompromised. There is no specific treatment. An antipyretic may be used to reduce fever and discomfort.

    CONCLUSION: Roseola infantum is generally a benign and self-limited disease. Failure to recognize this condition may result in undue parental fear, unnecessary investigations, delay in treatment for conditions that mimic roseola infantum and complications from roseola infantum, unnecessary treatment of roseola infantum per se, and misuse of healthcare expenditure.

  6. Leung AKC, Lam JM, Barankin B, Leong KF, Hon KL
    Curr Pediatr Rev, 2022;19(1):68-82.
    PMID: 36698243 DOI: 10.2174/1573396318666220429085231
    BACKGROUND: Early recognition of acanthosis nigricans is important because acanthosis nigricans can be a cutaneous manifestation of a variety of systemic disorders and, rarely, as a sign of internal malignancy.

    OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations, evaluation, diagnosis, and management of acanthosis nigricans.

    METHODS: A search was conducted in November 2021in PubMed Clinical Queries using the key term "acanthosis nigricans". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article.

    RESULTS: Acanthosis nigricans is characterized by symmetric, hyperpigmented, and velvety plaques with ill-defined borders, typically involving intertriginous areas. Obesity is the most common cause of acanthosis nigricans which is increasingly observed in obese children and adolescents and can serve as a cutaneous marker of insulin resistance. Early recognition of acanthosis nigricans is important because acanthosis nigricans can also be a cutaneous manifestation of a variety of systemic disorders and, rarely, as a sign of internal malignancy. This may consist of weight reduction, discontinuation of causative drugs, treatment of underlying endocrinopathy, or treatment of an underlying malignancy. For patients with isolated acanthosis nigricans and for those whose underlying cause is not amenable to treatment, treatment of the lesion may be considered for cosmetic reasons. Topical retinoids, vitamin D analogs, chemical peels, and other keratolytics are often used for the treatment of localized lesions. Seldom, systemic therapy such as oral retinoids may be considered for extensive or generalized acanthosis nigricans and acanthosis nigricans unresponsive to topical therapy. Other uncommon treatment modalities include dermabrasion, laser therapy, and surgical removal.

    CONCLUSION: Although acanthosis nigricans is treatable, a complete cure is difficult to achieve. The underlying cause should be treated, if possible, to resolve and prevent the recurrence of acanthosis nigricans. The diagnosis is mainly clinical, based on the characteristic appearance (symmetrically distributed, hyperpigmented, velvety, papillomatous, hyperkeratotic plaques with ill-defined borders) and the typical sites (intertriginous areas, flexural area, and skin folds) of the lesions. The diagnosis might be difficult for lesions that have atypical morphology or are in an unusual location. Clinicians should be familiar with the clinical signs, evaluation, diagnosis, and therapy of acanthosis nigricans because of the link between it and underlying diseases.

  7. Leung AKC, Lam JM, Barankin B, Leong KF, Hon KL
    PMID: 36284392 DOI: 10.2174/1570180820666221024095837
    BACKGROUND: Hand, foot, and mouth disease is a common viral disease in childhood. Because the disease has the potential to reach epidemic levels and mortality is high in some countries, early recognition of this disease is of paramount importance.

    OBJECTIVE: This purpose of this article is to familiarize pediatricians with the clinical manifestations and management of hand, foot, and mouth disease.

    METHODS: A search was conducted in February 2022 in PubMed Clinical Queries using the key term "hand, foot, and mouth disease". The search strategy included all clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in English were included in this review.

    RESULTS: Hand, foot, and mouth disease is characterized by a painful oral enanthem and asymptomatic exanthem on the palms and soles. Children younger than 5 years are most commonly affected. Hand, foot, and mouth disease caused by enterovirus A71 is more severe and has a higher rate of complications than that attributed to other viruses such as coxsackievirus A16. Circulatory failure secondary to myocardial impairment and neurogenic pulmonary edema secondary to brainstem damage are the main causes of death. Fortunately, the disease is usually benign and resolves in 7 to10 days without sequelae. Given the self-limited nature of most cases, treatment is mainly symptomatic and supportive. Intravenous immunoglobulin should be considered for the treatment of severe/complicated hand, foot, and mouth disease and has been recommended by several national and international guideline committees. Currently, there are no specific antiviral agents approved for the treatment of the disease. Drugs such as ribavirin, suramin, mulberroside C, aminothiazole analogs, and sertraline have emerged as potential candidates for the treatment of hand, foot, and mouth disease. Vaccination of susceptible individuals in high-risk areas and good personal hygiene are important preventative measures to combat the disease.

    CONCLUSION: Familiarity of the disease including its atypical manifestations is crucial so that a correct diagnosis can be made, and appropriate treatment initiated. A timely diagnosis can help avoid contact with the affected individual and decrease the risk of an outbreak.

  8. Leung AKC, Leong KF, Lam JM
    CMAJ, 2021 Feb 16;193(7):E243.
    PMID: 33593949 DOI: 10.1503/cmaj.201181
  9. Leung AKC, Leong KF, Lam JM
    Case Rep Pediatr, 2021;2021:9994067.
    PMID: 34194861 DOI: 10.1155/2021/9994067
    Granuloma gluteale infantum is a rare complication of irritant contact dermatitis. For the treatment of granuloma gluteale infantum, the diaper area should be kept clean and dry and the source of skin irritation should be removed or mitigated. For those infants who fail to respond to the conservative management, the use of topical calcineurin inhibitors should be considered. We report the successful treatment of a 12-month-old infant with recalcitrant granuloma gluteale infantum with the use of topical tacrolimus 0.03% ointment. To our knowledge, this is the second case reporting the use of topical calcineurin inhibitors in the treatment of recalcitrant granuloma gluteale infantum.
  10. Leung AKC, Lam JM, Leong KF, Hon KL
    Curr Pediatr Rev, 2021;17(1):55-69.
    PMID: 32384034 DOI: 10.2174/1573396316666200508100038
    BACKGROUND: Infantile hemangiomas are the most common vascular tumors of infancy, affecting up to 12% of infants by the first year of life.

    OBJECTIVE: To familiarize physicians with the natural history, clinical manifestations, diagnosis, and management of infantile hemangiomas.

    METHODS: A Pubmed search was conducted in November 2019 in Clinical Queries using the key term "infantile hemangioma". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 20 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article.

    RESULTS: The majority of infantile hemangiomas are not present at birth. They often appear in the first few weeks of life as areas of pallor, followed by telangiectatic or faint red patches. Then, they grow rapidly in the first 3 to 6 months of life. Superficial lesions are bright red, protuberant, bosselated, or with a smooth surface, and sharply demarcated. Deep lesions are bluish and dome-shaped. Infantile hemangiomas continue to grow until 9 to 12 months of age, at which time the growth rate slows down to parallel the growth of the child. Involution typically begins by the time the child is a year old. Approximately 50% of infantile hemangiomas will show complete involution by the time a child reaches age 5; 70% will have disappeared by age 7; and 95% will have regressed by 10 to 12 years of age. The majority of infantile hemangiomas require no treatment. Treatment options include oral propranolol, topical timolol, and oral corticosteroids. Indications for active intervention include hemorrhage unresponsive to treatment, impending ulceration in areas where serious complications might ensue, interference with vital structures, life- or function-threatening complications, and significant disfigurement.

    CONCLUSION: Treatment should be individualized, depending upon the size, rate of growth, morphology, number, and location of the lesion (s), existing or potential complications, benefits and adverse events associated with the treatment, age of the patient, level of parental concern, and the physician's comfort level with the various treatment options. Currently, oral propranolol is the treatment of choice for high-risk and complicated infantile hemangiomas. Topical timolol may be considered for superficial infantile hemangiomas that need to be treated and for complicated infantile hemangiomas in patients at risk for severe adverse events from oral administration of propranolol.

  11. Leung AKC, Lam JM, Leong KF, Hon KL
    Curr Pediatr Rev, 2021;17(2):76-91.
    PMID: 33302860 DOI: 10.2174/1573396316666201210125858
    BACKGROUND: Vitiligo is a relatively common acquired pigmentation disorder that can cause significant psychological stress and stigmatism.

    OBJECTIVE: This article aims to familiarize physicians with the clinical manifestations, evaluation, diagnosis, and management of vitiligo.

    METHODS: A Pubmed search was conducted in Clinical Queries using the key term "vitiligo". The search included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English language. The information retrieved from the above search was used in the compilation of the present article.

    RESULTS: Approximately one quarter of patients with vitiligo have the onset before 10 years of age. Genetic, immunological, neurogenic and environmental factors may have a role to play in the pathogenesis. Vitiligo typically presents as acquired depigmented, well-demarcated macules/patches that appear milk- or chalk-white in color. Lesions tend to increase in number and enlarge centrifugally in size with time. Sites of predilection include the face, followed by the neck, lower limbs, trunk, and upper limbs. The clinical course is generally unpredictable. In children with fair skin, no active treatment is usually necessary other than the use of sunscreens and camouflage cosmetics. If treatment is preferred for cosmesis, topical corticosteroids, topical calcineurin inhibitors, and narrowband ultraviolet B phototherapy are the mainstays of treatment.

    CONCLUSION: The therapeutic effect of all the treatment modalities varies considerably from individual to individual. As such, treatment must be individualized. In general, the best treatment response is seen in younger patients, recent disease onset, darker skin types, and head and neck lesions. Topical corticosteroids and calcineurin inhibitors are the treatment choice for those with localized disease. Topical calcineurin inhibitors are generally preferred for lesions on genitalia, intertriginous areas, face, and neck. Narrowband ultraviolet B phototherapy should be considered in patients who have widespread vitiligo or those with localized vitiligo associated with a significant impact on the quality of life who do not respond to treatment with topical corticosteroids and calcineurin inhibitors.

  12. Leung AKC, Lam JM, Leong KF, Hon KL
    Curr Pediatr Rev, 2021;17(3):201-211.
    PMID: 32964824 DOI: 10.2174/1573396316666200923161330
    BACKGROUND: Pityriasis rosea is a common acute, self-limited papulosquamous dermatosis that primarily affects children and young adults. The condition and its clinical variants may pose a diagnostic challenge, especially in the absence of the herald patch.

    OBJECTIVE: This article aimed to familiarize pediatricians with clinical manifestations, evaluation, diagnosis, and management of pityriasis rosea.

    METHODS: A search was conducted in March 2020 in Pubmed Clinical Queries using the key term " pityriasis rosea". The search strategy included all clinical trials (including open trials, non-randomized controlled trials, and randomized controlled trials), observational studies, and reviews (including narrative reviews and meta-analyses) published within the past 10 years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of the present article.

    RESULTS: Pityriasis rosea occurs mainly in individuals between 10 and 35 years of age with a peak during adolescence. Human herpesvirus (HHV)-7 and HHV-6 have been implicated as the causative agents in some patients with pityriasis rosea. A mild prodrome consisting of headaches, fever, malaise, fatigue, anorexia, sore throat, enlarged lymph nodes and arthralgia is present in about 5% of patients. The most common presenting sign, found in approximately 80% of patients, is a "herald" or "mother" patch which is larger and more noticeable than the lesions of the later eruption. A generalized, bilateral, symmetrical eruption develops in approximately 4 to 14 days and continues to erupt in crops over the next 12 to 21 days. Typical lesions are 0.5 to 1 cm, oval or elliptical, dull pink or salmon-colored macules with a delicate collarette of scales at the periphery. The long axes tend to be oriented along the skin lines of cleavage (Langer lines). Lesions on the back may have a characteristic "Christmas tree", whereas lesions on the upper chest may have a V-shaped pattern. There are many conditions that may mimic pityriasis rosea. Pityriasis rosea in the absence of the herald patch and its variants may pose a diagnostic challenge. The typical course is 6 to 8 weeks. In the vast majority of cases, reassurance and symptomatic treatment should suffice. Active intervention may be considered for individuals with severe or recurrent pityriasis rosea and pregnant women with the disease. Treatment options include acyclovir, macrolides (in particular, erythromycin), and ultraviolet phototherapy. If active intervention is needed, there is evidence supporting the use of oral acyclovir to shorten the duration of illness.

    CONCLUSION: Pityriasis rosea is a common, acute, self-limiting exanthematous skin disease that primarily affects children and young adults. The condition is characterized by a "herald patch" after which oval erythematous squamous lesions appear along Langer's lines of cleavage on the trunk and proximal extremities, giving it a "Christmas tree" appearance. The disease presenting in its classical form can easily be diagnosed. Clinical variants of the disease may pose a diagnostic challenge for the general pediatrician. Knowledge of the disease is essential to allow prompt diagnosis and to avoid unnecessary investigations.

  13. Leung AKC, Lam JM, Alobaida S, Leong KF, Wong AHC
    Curr Pediatr Rev, 2021;17(4):273-287.
    PMID: 33902423 DOI: 10.2174/1573396317666210426105045
    BACKGROUND: Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood rheumatic diseases. Mounting evidence suggests that early diagnosis and timely aggressive treatment are associated with better outcomes.

    OBJECTIVE: The purpose of this article is to provide readers with an update on the evaluation, diagnosis, and the treatment of juvenile dermatomyositis.

    METHODS: A PubMed search was performed in Clinical Queries using the key term "juvenile dermatomyositis" in the search engine. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to English literature. The information retrieved from the above search was used in the compilation of the present article.

    RESULTS: Juvenile dermatomyositis is a chronic autoimmune inflammatory condition characterized by systemic capillary vasculopathy that primarily affects the skin and muscles with possible involvement of other organs. In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) developed diagnostic criteria for juvenile idiopathic inflammatory myopathies and juvenile dermatomyositis. In the absence of muscle biopsies which are infrequently performed in children, scores (in brackets) are assigned to four variables related to muscle weakness, three variables related to skin manifestations, one variable related to other clinical manifestations, and two variables related to laboratory measurements to discriminate idiopathic inflammatory myopathies from non-idiopathic inflammatory myopathies as follows: objective symmetric weakness, usually progressive, of the proximal upper extremities (0.7); objective symmetric weakness, usually progressive, of the proximal lower extremities (0.8); neck flexors relatively weaker than neck extensors (1.9); leg proximal muscles relatively weaker than distal muscles (0.9); heliotrope rash (3.1); Gottron papules (2.1); Gottron sign (3.3); dysphagia or esophageal dysmotility (0.7); the presence of anti-Jo-1 autoantibody (3.9); and elevated serum levels of muscle enzymes (1.3). In the absence of muscle biopsy, a definite diagnosis of idiopathic inflammatory myopathy can be made if the total score is ≥7.5. Patients whose age at onset of symptoms is less than 18 years and who meet the above criteria for idiopathic inflammatory myopathy and have a heliotrope rash, Gottron papules or Gottron sign are deemed to have juvenile dermatomyositis. The mainstay of therapy at the time of diagnosis is a high-dose corticosteroid (oral or intravenous) in combination with methotrexate.

    CONCLUSION: For mild to moderate active muscle disease, early aggressive treatment with high-dose oral prednisone alone or in combination with methotrexate is the cornerstone of management. Pulse intravenous methylprednisolone is often preferred to oral prednisone in more severely affected patients, patients who respond poorly to oral prednisone, and those with gastrointestinal vasculopathy. Other steroid-sparing immunosuppressive agents such as cyclosporine and cyclophosphamide are reserved for patients with contraindications or intolerance to methotrexate and for refractory cases, as the use of these agents is associated with more adverse events. Various biological agents have been used in the treatment of juvenile dermatomyositis. Data on their efficacy are limited, and their use in the treatment of juvenile dermatomyositis is considered investigational.

  14. Leung AKC, Leong KF, Lam JM
    Paediatr Child Health, 2020 04 24;26(2):e70-e72.
    PMID: 33747313 DOI: 10.1093/pch/pxaa050
    The majority of infantile hemangiomas are benign and will resolve on their own. We report a 4-month-old infant with an ulcerated giant segmental infantile hemangioma involving the left upper limb who developed a contracture of the left elbow despite treatment with oral propranolol, proper wound care, and regular intense physiotherapy. To our knowledge, contracture resulting from an infantile hemangioma has not been reported previously.
  15. Leung AKC, Leong KF, Barankin B
    J Pediatr, 2020 01;216:239-239.e1.
    PMID: 31542154 DOI: 10.1016/j.jpeds.2019.08.034
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