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Paediatrica Indonesiana

paediatrica-indonesiana
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Published by Ikatan Dokter Anak Indonesia

ISSN : 00309311 EISSN : 2338476X DOI : -

Core Subject : Health,

Health Professions Medicine & Pharmacology

Paediatrica Indonesiana is a medical journal devoted to the health, in a broad sense, affecting fetuses, infants, children, and adolescents, belonged to the Indonesian Pediatric Society. Its publications are directed to pediatricians and other medical practitioners or researchers at all levels of health practice throughout the world.

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Articles 9 Documents

Search results for , issue "Vol 58 No 6 (2018): November 2018" : 9 Documents clear

Hunter syndrome with hyperthyroidism: a 16 month follow-up reprt Din Alfina; Endy Paryanto Prawirohartono; Roni Naning; Neti Nurani
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Mucopolysaccharidosis (MPS) is a rare genetic disorder caused by a deficiency in the activity of lysosomal enzymes required for glycosaminoglycan (GAG) degradation. An accumulation of GAG in many organs results in progressive cellular damage, and clinically results in joint stiffness, airway and cardiac as well as, mental and hearing impairments. Incidence of MPS was reportedly 2.04 per 100,000 live births, but varies depending on type and region. In Taiwan, MPS type II was the most prevalent MPS, with an incidence of 1.07 per 100,000 live births.1 MPS is generally inherited in an autosomal recessive pattern, with the exception of MPS II, which is X-linked recessive.2 There are seven types of MPS (MPS I, II, III, IV, VI, VII, and IX), based on enzyme deficits.3 The types of MPS with their enzyme deficiencies are listed in Table 1. Mucopolysaccharidosis shows wide clinical heterogenity, and is, therefore, difficult to diagnose. Skeletal involvement in MPS include coarse face, loss of joint range of motion, restricted mobility, and slowed growth leading to short stature. Other signs and symptoms include vision and hearing loss, recurrent respiratory infections, obstructive sleep apnea, hepatosplenomegaly, umbilical and inguinal hernia, hydrocephalus, spinal cord compression, and cognitive impairment.2,4 Patients with suspected MPS should have urinary GAG laboratory testing and enzyme activity assays in tissue (blood or fibroblasts). Urinary elevation of GAG, as compared with GAG levels expected in age-matched normal subjects, is the first diagnostic approach. The definitive specific diagnosis for MPS is based on enzyme activity assays from cultured fibroblasts, leukocytes, plasma, or serum.2,5,6 The MPS patients require multidiciplinary subspeciality management, including ENT, orthopedics, cardiology, pulmonary, growth and development, and physiotherapy. Specific treatments for MPS are hematopoietic stem cell transplantation (HSCT) and enzyme-replacement therapy (ERT) with recombinant human enzymes for MPS I, II, and VI.3,6,7,8 Life expectancies in MPS may vary among types, but generally are markedly reduced. Patients with MPS III and VII and severe forms of MPS I and MPS II have mental retardation. Patients with MPS II usually survive until only the second decade of life, with respiratory failure as the leading cause of death (56%), followed by cardiac failure (18%).9,10

Oxidative stress in neonates with hyperbilirubinemia before and after phototherapy: malondialdehyde and catalase activity Putu Junara Putra; Rinawati Rohsiswatmo; Pustika Amalia Wahidiyat
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background Phototherapy is used to treat neonatal hyperbilirubinemia, but is currently thought to cause photodynamic stress and can induce lipid peroxidation. There is increasing evidence that many severe diseases of the neonates are caused by oxidative injury and lipid peroxidation. In the present communique, we review the oxidative succeptibility of the neonate and the evidence now available that phototherapy induces oxidative stress. Malondialdehyde (MDA) is a metabolic product of free radicals. Catalase is a antioxidant that binds free radicals. Objective To compare the levels of oxidants and antioxidants before and after phototherapy in neonates with hyperbilirubinemia. Methods This pretest-posttest control group study was conducted in Sanglah Hospital, Bali from November 2016 to April 2017. Thirty babies with gestational age â‰¥35 weeks and hyperbilirubinemia with total bilirubin levels requiring phototherapy were included in this study. The MDA levels and catalase activity were measured before and after 24 hours of phototherapy. Results Comparative analysis using paired T-test showed a significant increase of malondialdehyde level, with mean MDA 23.73 (SD 8.20) nmol/mL before and 53.05 (SD 10.18) nmol/mL after phototherapy (P<0.001). However, catalase activity significantly decreased from of 72.33 (SD 10.63) kU/L before phototherapy to 44.85 (SD 14.79) kU/L after phototherapy (P<0.001). The MDA level had a significant, negative association with catalase activity after phototherapy (r =-0.4; P=0.028). Conclusion Neonates with hyperbilirubinemia are found to have increased oxidative stress after phototherapy, as indicated by increased MDA levels and decreased CAT activity after 24 hours of phototherapy.

Polymorphisms associated with type 1 diabetes mellitus Rachman Indra Jaya; Yenni Riska Zettyana; Achirul Bakri; Yuwono Yuwono; Aditiawati Aditiawati
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background Type 1 diabetes mellitus (T1DM) is an organ-specific autoimmune disease characterized by T cell-mediated destruction of pancreatic islets. The genetic factors involved consist of at least five vulnerability genes: HLA, INS, CTLA-4, PTPN22, and IL2RA/CD25. Objective To investigate for associations of PTPN22-1123 G>C SNP and CTLA-4 +49A/G polymorphisms with T1DM. Methods Case and control groups underwent CTLA-4 +49A/G gene examination from June to December 2017, using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) analysis. Results The study population consisted of 30 T1DM patients and 30 healthy subjects with no family history of diabetes or autoimmune diseases. With regards to the PTPN22-1123 G>C SNP, significantly more subjects with T1DM had the GC genotype than the GG genotype (OR 7.64; 95%CI 1.48 to 39.29; P=0.007). For the CTLA-4 +49A/G polymorphism, although the total number of G alleles in the case group was more than that of the control group (OR 2.286; 95%CI 0.804 to 6.945; P=0.118), there were no significant relationships between the frequency of G alleles (P=0.248) and genotypes GG or AG (P=0.293) with the incidence of T1DM. However, the PTPN22-1123 G>C SNP had a significantly positive association with T1DM, and may be considered as a risk factor for T1DM. In contrast, the CTLA-4 +49A/G polymorphism was not recognized as a risk susceptibility factor for T1DM. Conclusion These study confirms an association between PTPN22-1123 G>C SNP and T1DM, but no significant association between CTLA-4 +49A/G polymorphism and T1DM.

Immunotherapy and probiotic treatment for allergic rhinitis in children Sumadiono Sumadiono; Cahya Dewi Satria; Nurul Mardhiah; Grace Iva Susanti
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background Allergic rhinitis is a global health problem that is increasing in prevalence. Many kinds of therapy have been tried, such as antihistamines, probiotics, and immunotherapy. Immunotherapy may restore the patientâ€™s normal immunity against the specific allergen, while probiotics may modify the natural course of allergy. Objective To evaluate probiotics and immunotherapy for improving clinical symptoms of allergic rhinitis. Methods This randomized controlled trial (RCT) involved 64 patients, aged 3-18 years, and diagnosed with persistent allergic rhinitis in the Department of Child Health, Sardjito General Hospital from April 2016 until May 2017. Patients were randomly allocated into three therapy groups: group A (standard therapy/cetirizine only), group B (standard and probiotic therapy), and group C (standard therapy and immunotherapy). Clinical symptoms of allergic rhinitis including sneezing, rhinorrhea, and itchy nose, were evaluated for 7 weeks and classified as improved or not improved. The significance of the data was analyzed using proportion test. Results Sixty-four patients completed 7 weeks of therapy, 15 subjects in group A, 26 in group B, and 23 in group C. Group C showed significantly more improvement of sneezing and rhinorrhea compared to both group A (Z=5.71; Z=7.57, respectively) and group B (Z=2.82; Z=6.90, respectively). However, itchy nose was not significantly improved in group C compared to group B (Z=0.50), but was significantly improved in group C compared to group A (Z=10.91). Group B had significant improvement of sneezing, rhinorrhea, and itchy nose compared to group A (Z=3.81, Z=2.86, and Z=10.91, respectively). Conclusion The combined standard-immunotherapy group has significantly superior improvement compared to the combined standard-probiotic group and the standard therapy group, in terms of sneezing and rhinorrhea in children with persistent allergic rhinitis.

Depression in children with thalassemia major: prevalence and contributing factors Venty Venty; Rismarini Rismarini; Dian Puspitasari; Yudianita Kesuma; Raden Muhammad Indra
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background Thalassemia major is a chronic disease requiring lifetime treatment. A recent study showed that 11-62% of thalassemia patients developed depression, which is associated with high morbidity and mortality. Understanding the extent of the problem related to depression and its contributing factors is important for early management. Objective To determine the prevalence and contributing factors for depression in children with thalassemia major. Methods This cross-sectional observational analytic study included thalassemia major patients aged 7 to <18 years in the Department of Child Health, Dr. Moh. Hoesin General Hospital (RSMH) in Palembang from June to July 2018 and had received blood transfusions at least 3 times. Subjects completed the Children's Depression Inventory (CDI) questionnaire. Depression was defined as a total score > 13. Data were analyzed using SPSS for Windows ver. 22.0. Results There were 64 patients included in this study, with mean age 12 (SD 3) years and 82.8% female. Most subjects came from families with low socio-economic status and low parental education. Deferiprone was the most commonly used type of iron-chelating agent. Depression was detected in 34.4% of respondents. Multivariate analysis revealed that factors affecting depression in children with thalassemia major were low maternal education (OR 4.014; 95%CI 1.066 to 15.112) and use of deferasirox (OR 4.129; 95%CI 1.168 to 14.601). Conclusion Prevalence of depression in children with thalassemia major is 34.4%. Low maternal education and deferasirox use as an iron-chelating agent are associated with depression in children with thalassemia major.

Impact of malnutrition on febrile neutropenia in children with acute lymphoblastic leukemia during induction phase chemotherapy Marshalla Agnes; Pudjo Hagung Widjajanto; Wahyu Damayanti
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background Acute lymphoblastic leukemia (ALL) is the most common malignancy in children and adolescents. Febrile Neutropenia (FN) is a medical emergency on ALL that often leads to death. Nutrition status assessment on ALL patient is important because malnutrition can reduce the tolerance of chemotherapy, increase incidence of infection and decrease survival rate. Objectives To assess malnutrition as a risk factor for FN in children with ALL. Methods This case-control study was performed at Sardjito Hospital, Yogyakarta on patients aged 1 month to 18 years diagnosed with ALL and undergoing induction phase chemotherapy between January 2013 and December 2015. The case and control subjects were children with and without FN, respectively. Febrile neutropenia was confirmed by patients temperature above 38ÂºC at one measurement and a peripheral neutrophil count of less than 1,000/mm3. Malnutrition was defined as body weight-for-height was between -2 and <-3 standard deviation. Subjects were included using simple random sampling. Result Bivariate analysis showed a significant correlation between malnutrition and FN (OR 2.62; 95%CI 1.07 to 6.45; P=0.03). However, there was no inverse correlation between socioeconomic status and FN (OR 1.1; 95%CI 0.42 to 2.41; P=0.83). There was no correlation between nutritional status and duration of FN (P= 0.48). Conclusion Malnutrition is a risk factor for FN in children with acute lymphoblastic leukemia.

Serum creatinine levels to estimate kidney function in small-for-gestational age and appropriate-for-gestational age newborns Indra Sandinirwan; Aris Primadi; Dany Hilmanto
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background The main parameter used to determine renal function in newborns is serum creatinine. Fetal growth restriction during pregnancy can cause the baby to be born small-for-gestational age. Serum creatinine levels in newborns are affected by muscle mass, gestational age, as well as the number of nephrons and kidney development. Objective To determine the usefulness of serum creatinine levels as an estimate of glomerular filtration rate in small-for-gestational age and appropriate-for-gestational age newborns. Methods This cross-sectional study was conducted in May-June 2018. The subjects were full term newborn infants consisting of small-for-gestational age and appropriate-for-gestational age groups (16 subjects each), born in Bandung City Regional Public Hospital. Serum creatinine level was tested by the Jaffe method. The estimated glomerular filtration rate was calculated based on serum creatinine, infant height, and a proportionality constant using the original Schwartz method, eGFR = [k * height]/SCr. Results Of 32 subjects, there were 17 spontaneous deliveries, 14 males, and 18 females. Mean serum creatinine levels in the small-for-gestational age and appropriate-for-gestational age groups were 0.94 (SD 0.36; 95%CI 0.75 to 1.14) mg/dL and 0.69 (SD 0.18; 95%CI 0.60 to 0.79) mg/dL (mean difference 0.25; 95%CI 0.05 to 0.46; P=0.009), respectively. The median estimated glomerular filtration rates (eGFR) in the small-for-gestational age and appropriate-for-gestational age groups were 25.69 mL/min/1.73m2 and 30.10 mL/min/1.73m2 (median difference 4.42; 95%CI 2.04 to 15.8; P=0.008), respectively. There was a weak negative correlation between serum creatinine and birth weight (r=-0.344; P=0.027). Conclusion Serum creatinine levels in small-for-gestational age newborns are significantly higher than in appropriate-for-gestational age newborns.

Sepsis calculator to support antibiotic stewardship in early-onset neonatal sepsis: a meta-analysis Rinawati Rohsiswatmo; Hardya Gustada Hikmahrachim; Dinarda Ulf Nadobudskaya; Sonia Miyajima Anjani; Albert You
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background Establishing a diagnosis of neonatal sepsis is difficult. As such, appropriate timing of antibiotic therapy remains the biggest challenge. As a consequence of non-definitive diagnoses, inappropriate antibiotic administration is common. Recently, a sepsis calculator to estimate risk of early-onset sepsis (EOS) based on both maternal risk factors and infants' clinical presentation was established. Objective To determine the impact of the sepsis calculator in daily clinical settings, especially with regards to antibiotic usage. Methods A literature search of Pubmed, EBSCO, Embase, and Scopus database from January 2011 (after sepsis calculator was established) to June 2018 was performed. We included observational studies that compared the sepsis calculator to recent neonatal sepsis guidelines in terms of antibiotic administration, blood culture, and admission to the neonatal intensive care unit (NICU). The literature search, validation study, and assessment risk of bias were done independently by our four authors, while the first author did the statistical analysis. Results Of the 35 studies identified, 5 cohort studies met the criteria, with a total sample size of 18,352 infants from various countries. We developed a fixed-effect meta analysis of the data. The use of the sepsis calculator significantly reduced inappropriate use of antibiotics [RR 0.46; 95%CI 0.41 to 0.51; z=13.57; P<0.001], blood culture sampling [RR 0.46; 95%CI 0.40 to 0.52; z=12.11; P<0.001), and higher neonatal care level admissions [RR 0.68; 95%CI 0.59 to 0.78); z=5.47; P<0.001). No safety issues were reported from studies using the sepsis calculator. Conclusion The new EOS risk estimation using a neonatal sepsis calculator is an easy, effective, and safe tool to improve appropriate antibiotic use and outcomes. This calculator is ready to be implemented in all levels of neonatal care units.

Congenital heart disease in children with Down syndrome in Afghanistan Abdul Muhib Sharifi; Abdul Rashid Mansoor; M. Akbar Ibrahimi; Abdul Wali; Wali Wali; Khesrow Ekram
Paediatrica Indonesiana Vol 58 No 6 (2018): November 2018
Publisher : Indonesian Pediatric Society

Background Congenital heart disease (CHD) is frequently cited as the main cause of death in the pediatric Down syndrome (DS) population. The prevalence and spectrum of CHD patterns in DS varies widely worldwide; this variation could be due to sociodemographic, genetic, and/or geographic factors. Objective To verify the prevalence, pattern, and frequency distribution of CHD in children with Down syndrome. Methods A three-year retrospective study was conducted in children aged 0-14 years with Down syndrome who underwent echocardiography for possible CHD from January 2014 to December 2016, based on the Pediatric Unit CHD Registry of the Cardiac Research Institute, Kabul Medical University. Clinical, echocardiographic, and outcome data were collected and sorted according to confirmation of the syndrome and echocardiography result. Results During the three-year study period, 420 DS patients were identified, 286 (68%) of whom had CHDs. The prevalence of isolated and multiple CHD in the 420 children with DS were 38% (160 patients) and 30% (126 patients), respectively. Ventricular septal defect (23%) and atrial septal defect (16.4%) were the most common isolated defects. The combination of VSD and ASD (19.9%) were the most frequent multiple CHDs. The most common associations of CHD were VSD + ASD (19.9%) and VSD + PDA (9%). Conclusion A high prevalence of CHDs was noted in children with Down syndrome. VSD and ASD are the most commonly diagnosed isolated CHDs in our study. ASD + VSD is the most common multiple CHD pairing. To our knowledge, this is the first extensive study in Afghanistan to demonstrate the pattern and prevalence of CHD associated with Down syndrome.

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