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Systemic Lupus Erythematosus (SLE) is a chromic autoimmune disease with multisystemic involvement. The estimated prevalence of SLE among children is 1-6/100000. Here we report a child who presented with generalized lymphadeopathy as the first manifestation of SLE. A 13 year old previously healthy girl presented with cervical lymphadenopathy and low grade remitting fever for 1 month duration. On examination she was found to have generalized lymphadenopathy and unremarkable rest of the clinical examination. Her basic haematological and biochemical parameters were normal. ESR was 110mm/hr and CRP was 6mg/dL. Ultrasound scan and Contrast CT of the abdomen and pelvis revealed extensive mediastinal, mesenteric, para-aortic, splenic hilar, bilateral axillary and inguinal lymphadenopathy suggestive of lymphoproliferative malignancy. However, her bone marrow and lymph node biopsies revealed reactive pattern. Meantime she developed a malar rash, oral ulcers and 2 episodes of generalized tonic-clonic convulsions. Her urinalysis revealed microscopic haematuria and sub nephrotic range proteinuria. Her ANA and dsDNA levels were positive. Her subsequent full blood count revealed severe bicytopenia, predominetly lymphopenia.The diagnosis of SLE was made and she was started on Hydroxychloroquine, high dose prednisolone and cyclophosphamide pulse therapy. The most common presenting symptoms of SLE in children include, constituitional symptoms, joint symptoms and haematological abnormalities. It is estimated 23-34% children with SLE are having lymphadenopathy. Though, generalized lymphadenopathy as the presenting symptom of SLE is uncommon, it should not be forgotten in order to avoid diagnostic delay.

Background Birth defects (BD) are considered a leading cause of childhood morbidity and mortality. Personal, cultural, and health care system barriers may increase the incidence of BD in low and middle income countries. In this study we assessed the knowledge of antenatal mothers on BD, associated factors, and prevention and management. Methods Three hundred and fifty (350) antenatal mothers were surveyed using a pretested, self-administered questionnaire. The knowledge on BD was evaluated under 3 categories; knowledge on BD, knowledge on associated factors, and knowledge on prevention and management. The total scores were calculated for each category and converted into percentages. A higher percentage score indicates a high level of knowledge. Descriptive statistics and regression models were used for data analysis. Level of significance was considered as p

Morquio syndrome (Type IV mucopolysaccharidosis) is an autosomal recessive condition with a prevalence of 1:40000 to 1:200000.1 The deficiencies of N-acetyl-galactosamine-6-sulfatase and beta-galactosidase leads to accumulation of glycosaminoglycan resulting Morquio syndrome type A and B respectively. Here, we report a case of two siblings with Morquio syndrome. Parents brought two male siblings aged 5 years and 30 months due to abnormal physical appearance. They were born to non-consanguineous healthy parents who had uneventful antenatal periods. Parents were worried about short stature and abnormal chest shape. On examination, both of them had similar physical characteristics such as a coarse face, short stature, pectus carinatum, scoliosis, short neck, coxa vulga, and multiple bony deformities. They had normal basic biochemistry, haematological indices, and bone profile. Thoracolumbar spine and pelvis X rays showed platyspondyly with anterior beaking and flaring of iliac wings. Ophthalmology assessment of both siblings revealed amblyopia. Both siblings had elevated urine glycosaminoglycans. Qualitative urine analysis showed moderate excretion of chondroitin sulphate and mildly elevated Heparan sulphate, which favoured MPS type IV. Lysosomal enzyme assay performed on elder sibling showed a low level of beta-galactosidase-6-Sulphate-Sulphatase and normal level of beta-galactosidase, and the diagnosis was confirmed as MPS type IV-A. Since no curative therapy was available yet, parents were explained about the disease condition, and follow-up was arranged with a multidisciplinary approach. Unfortunately, there is a significant amount of financial constraint in diagnosing and managing Morquio syndrome. Developing countries such as Sri Lanka cannot afford enzyme replacement therapy, and HSCT is not developed for children with inborn errors of metabolism. However, it is essential to have surveillance for anticipated complications of the condition with a multidisciplinary team approach until a cheaper disease-modifying agent is available.

The COVID-19 pandemic has presented educational institutions and educationists with unprecedented challenges with regard to the delivery of high-quality education. The challenges have been greater for the medical education institutions as the students engaging in clinical attachments work in a high-risk environment for COVID-19 transmission and place themselves and their associates at personal risk. The paper outlines the robust and pragmatic framework adopted by the Faculty of Medicine, University of Ruhuna to meet its twin goals of continuing to provide high-quality medical education whilst ensuring health and wellbeing of students. Measures were adopted to minimize exposure outside the educational environs as well as within the hospital and halls of residence. Guidelines were adopted, discussed and disseminated. Suitable personal protective equipment was defined and all students’ inward settings requested to wear them. Unanticipated COVID-19 exposures took place on two occasions and the faculty risk assessment committee quarantined a few students with high-risk exposure within the halls of residence. The ‘protected exposure’ to minimize the risk of COVID-19 provided by the personal protective equipment they wore, prevented many students from being quarantined. All who were quarantined were COVID-RT-qPCR negative on day 14 of exposure. A community-acquired COVID-19 outbreak with 7 students testing positive for COVID-19 in the halls of residence required the Faculty of Medicine to institute the first civilian administered quarantine facility in Sri Lanka quarantining 200 students as first-line contacts and isolating a further 264 as second-line contacts. The administration of the quarantine facility was an unprecedented logistical challenge to the university administration and the academic staff. Meticulous attention to detail and commitment helped the faculty quarantine centre to meet up with all challenges and the 464 students all tested negative on the exit RT-qPCR. This was a zero cross-transmission rate for the infection and was a remarkable achievement for a quarantine facility. Leadership principles specifically aimed at managing crisis situations such as transparent communication, the urgency of action, commitment to core values and empowering all stakeholders formed the crux of the successful measures adopted. It is hoped that the wider lessons emanating from the experiences outlined in this paper will be of value to academic colleagues from similar medical educational settings to create bio-secure, COVID-19 protective environments in their respective institutions.

ObjectivesTo determine aetiology of illness among children and adults presenting during outbreak of severe respiratory illness in Southern Province, Sri Lanka, in 2018.DesignProspective, cross-sectional study.Setting1600-bed, public, tertiary care hospital in Southern Province, Sri Lanka.Participants410 consecutive patients, including 371 children and 39 adults, who were admitted with suspected viral pneumonia (passive surveillance) or who met case definition for acute respiratory illness (active surveillance) in May to June 2018.ResultsWe found that cocirculation of influenza A (22.6% of cases), respiratory syncytial virus (27.8%) and adenovirus (AdV) (30.7%; type B3) was responsible for the outbreak. Mortality was noted in 4.5% of paediatric cases identified during active surveillance. Virus type and viral coinfection were not significantly associated with mortality.ConclusionsThis is the first report of intense cocirculation of multiple respiratory viruses as a cause of an outbreak of severe acute respiratory illness in Sri Lanka, and the first time that AdV has been documented as a cause of a respiratory outbreak in the country. Our results emphasise the need for continued vigilance in surveying for known and emerging respiratory viruses in the tropics.

Questionnaire to assess the knowledge of the antenatal mothers on birth defects, associated factors, prevention and management. This is a self-administered questionnaire used to collect data from the antenatal mothers. In the development of the questionnaire for the present study, the questionnaire developed by Bello et al., (2013) was used as a reference [3]. Additional questions were added to address the aims of the study. The questionnaire consisted of two parts; part A and part B. Part A of the questionnaire was used to gather socio-demographic data including age, ethnicity, highest educational qualification, monthly income, parity, previous children with birth defects, and the number of clinics attended. Data was obtained on prior awareness on birth defects and the sources of such information. An added question was included to inquire on the preconceptional FA intake. If FA was taken preconceptionally, the reason for them to take preconceptional FA supplementation was inquired. Part B of the questionnaire consisted of three sections; knowledge on BD, knowledge on associated factors of BD and knowledge on prevention and management of BD. Respectively, 10, 21 and 7 statements were included in each section. In all 3 sections mothers were asked to mark their responses stating whether the given statement is true, false or do not know. (DOCX 22 kb)

The WHO recommends that exclusive breastfeeding should last up to 6 mo. However, human milk intake of Sri Lankan infants has not been quantified scientifically.The objectives of this study were to measure the human milk intake of Sri Lankan infants during the first 6 mo of age and to document the breastfeeding practices of their mothers.Forty-eight healthy mother-infant dyads were randomly recruited for this cross-sectional study at well-baby clinics. Milk intake was measured using the deuterium oxide-to-the-mother technique over a period of 2 wk. Information on breastfeeding practice and living standards of the participants were gathered using an interviewer-administered questionnaire.Human milk intake was 672 ± 123 g ⋅ d(-1) (mean ± SD), 776 ± 212 g ⋅ d(-1), and 801 ± 51 g ⋅ d(-1) for infants2 mo, 2 to4 mo, and 4-6 mo of age, respectively. The milk intakes were not different among the age groups. Maternal body composition, age, or parity had no effects on milk intake of the infants. However, mother's education : P0.05, r = 0.35), infant's age (P0.05, r = 0.30), and body mass index (P0.05, r = 0.41 : positively correlated with the milk intake. Over 63% of mothers had commenced breastfeeding within 30 min of delivery. About 60% of the mothers were feeding the baby 6-10 times during the daytime and81% intended to continue exclusive breastfeeding until 6 mo of age.This study, for the first time, documented the adequacy of breast-milk intake among Sri Lankan infants and the nutrition status of the mothers.

BACKGROUND: Sri Lanka has made remarkable improvements in health social and educational indices. However child malnutrition exists as a significant health problem. Infant feeding indicators have not reached expected levels and improvements are partly constrained by a lack of data. The present study aimed to determine current infant feeding practices and their impact on growth among 6-12-month-old infants. METHODS: The study comprised a descriptive cross-sectional investigation conducted in randomly selected (n = 7) Public Health Midwife areas in Galle Sri Lanka. An interviewer-administered questionnaire was used to obtain data on sociodemographics and infant feeding. Mothers (n = 515) attending well-baby clinics were recruited on voluntary and consecutive basis. Infants body weights and lengths were measured using standard procedures. RESULTS: Exclusive breastfeeding rate for first 6 months was 49.0%. In total 42.6% infants (219 out of 515) were given rice as first weaning food followed by salt (58.6%) and sugar (42.3%). Oil had been introduced to 84.9% of infants by the end of 12 months. Most infants (over 71%) were given dairy products whereas 62.3% were being fed various liquid foods using bottles. The introduction of commercial infant cereals chocolates plain tea ice cream and deep fried snacks was noted. Age-specific body weight and length were not achieved by 30.5% and 29.5% of infants respectively. Weight for length was not achieved by 25.5% of the infants. Delayed achievements of motor milestones were observed. Mothers knowledge scores on basic nutrients were low. CONCLUSIONS: Complementary feeding indices of the study group were not satisfactory. Maternal and child healthcare personnel need to identify causative factors for inappropriate feeding with a view to improve the complementary feeding patterns. (c) 2014 The British Dietetic Association Ltd.

Introduction: Glucose-6-phosphate dehydrogenase (G6PD) deficiency affects over 400 million individuals worldwide and commonly found in Asia and in the Mediterranean region. This study was aimed to assess the feasibility of incorporating G6PD screening to the existing newborn screening panel in Sri Lanka. Methods: Dried blood spot samples (n=8795) received during a period of two months consecutively were subjected to the initial analysis and the G6PD activity was calculated by a quantitative analysis of comparing the sample to the control with a known activity using the haemoglobin normalization method. All babies with G6PD residual activity Results: Analysis of second samples revealed that 58 babies (36 males and 22 females) continued to have Conclusions: The incidence of G6PD deficiency is reported for the first time among neonates in Sri Lanka. However, the cut-offs we have defined at 6 months may be at a disadvantage with high attrition, but it saves the cost of additional genetic analysis.

Introduction Galactosemia is a rare autosomal recessive metabolic disorder with the prevalence of 1;60000. Classical Galactosemia (CG) is the most common variant of Galactosemia and which is due to deficiency of Galactose-1-phosphate uridyltransferace enzyme. Fanconi Syndrome (FS) is a rare presentation of CG. Here we present a baby girl with CG presenting with FS. Case Report A 5-month old baby girl presents with recurrent non bilious vomiting since early neonatal period. She was the first baby born to second degree consanguineous parents. The baby was exclusively breast fed since birth and there was a history of feeding intolerance. Her weight gain was suboptimal. On admission, she looks ill, tachypneic and dehydrated and her anthropometric parameters were

Introduction: Exclusive breastfeeding (EBF) is recommended up to six months of age and is usually estimated by the 24-recall method which actually overestimates the real rate. EBF rate in Sri Lanka up to 4 months of age is 75% and up to 6 months of age is 51% according to data of the Sri Lanka Demographic & Health Survey 2006/07. Deuterium oxide dose-to-mother technique could be utilised for assessment of intake by babies of breastmilk as well as intake of water from sources other than breastmilk. Objective: To determine the actual EBF rates at 3 and 6 months of age of infants in Sri Lanka using deuterium oxide dose-to-mother technique. Method: Thirty healthy mother-infant dyads were followed up for a six month period from birth and breast milk intake was measured at 3 and 6 months using deuterium isotope analysis. Further, an interviewer administered questionnaire asked about the feeding history. Results: The average milk intake at 3 months was 772±134 g.d -1 (range 587-1057) and 800±174 g.d -1 (range 500-1113) at 6 months ( p =0.30). The non-milk oral intake at 3 months was 91±45 g.d -1 (range -2-166) and 128±63 g.d -1 (range 25-304) at 6 months (p=0.01). Breastfeeding practices revealed that only 40% (n=12) of mothers at 3 months and 30% (n=9) of mothers at 6 months were practising EBF. It confirmed that the EBF rate among study subjects was 50% at 3 months and 27% at 6 months, with the cut-off of 82.5 g.d -1 of non-milk oral intake. Conclusions : A decrease in EBF was noted between 3 and 6 months using deuterium oxide dose-to-mother technique. Further, the EBF rates in this study sample are much lower than the presently reported value for Sri Lanka using recall method. Sri Lanka Journal of Child Health , 2018; 47 (4): 311-315

The South Asian Association for Regional Cooperation (SAARC) is an organization of eight countries--Bangladesh, Bhutan, India, the Maldives, Nepal, Pakistan, Sri Lanka and Afghanistan. The major objectives of this review are to examine trends and progress in newborn and neonatal health care in the region. A landscape analysis of the current state of neonatal mortality, stillbirths and trends over the years for each country and the effective interventions to reduce neonatal mortality and stillbirths was undertaken. A modelling exercise using the Lives Saved Tool (LiST) was also undertaken to determine the impact of scaling up a set of essential interventions on neonatal mortality and stillbirths. The findings demonstrate that there is an unacceptably high and uneven burden of neonatal mortality and stillbirths in the region which together account for 39% of global neonatal deaths and 41% of global stillbirths. Progress is uneven across countries in the region, with five of the eight SAARC countries having reduced their neonatal mortality rate by more than 50% since 1990, while India (43%), Afghanistan (29%) and Pakistan (25%) have made slower progress and will not reach their MDG4 targets. The major causes of neonatal mortality are intrapartum-related deaths, preterm birth complications and sepsis which account for nearly 80% of all deaths. The LiST analysis shows that a gradual increase in coverage of proven available interventions until 2020 followed by a uniform scale-up to 90% of all interventions until 2030 could avert 52% of neonatal deaths (0.71 million), 29% of stillbirths (0.31 million) and achieve a 31% reduction in maternal deaths (0.25 million). The analysis demonstrates that the Maldives and Sri Lanka have done remarkably well while other countries need greater attention and specific focus on strategies to improve neonatal health.

Turner syndrome (TS) (approximately 1:5,000 births) and craniopharyngioma (CP) (1:50,000 children) are both rare conditions. We present three cases of TS with CP, an association not previously described. Visual failure, poor growth or headache led to MRI diagnosis of CP. Whilst two had evidence of hypopituitarism at diagnosis of CP, they all developed hypopituitarism following surgical debulking. Two required radiotherapy due to regrowth. Whether CP and TS share a similar aetiology is unknown. Clinicians need to be aware of this association, and should perform urgent MRI scanning in TS patients with headache, visual impairment or clinical/biochemical evidence of hypopituitarism. Pediatr Blood Cancer 2013; 60: E7–E9. © 2012 Wiley Periodicals, Inc.

Background Body composition indicators provide a better guidance for growth and nutritional status of the infants. This study was designed to (1) measure the body composition of the Sri Lankan infants using a reference method, the 18O dilution method; (2) calculate the body fat content of the infants using published skinfold prediction equations; and (3) evaluate the applicability of the skinfold equations to predict body fat among Sri Lankan infants against the 18O dilution method. Methods Twenty five healthy, exclusively breast-fed infants were randomly recruited at well-baby clinics, for this cross-sectional study. Body composition was measured using 18O dilution. Infant body weight, length, skinfold thicknesses and mid upper-arm circumference were measured using standard procedures. The Bland and Atlman pair-wise comparison method was used to evaluate the agreement of body fat generated using the anthropometric prediction equations against the 18O dilution values as the reference. Results Mean (SD) body weight and length of the infants were 6.5 kg (0.9) and 64.7 cm (2.8) respectively. Mean total body water, fat free mass, fat mass and % fat mass as measured by 18O dilution method were 58.8% (5.0), 4.6 kg (0.8), 1.9 (0.5) and 29.5% (6.1). Total body water and fat free mass were significantly higher in boys when compared to girls. With the exception of three prediction equations (Bandana et al., Goran et al. and Durnin and Wormsley), most of the other commonly used anthropometry-based prediction equations yielded a bias which was not constant but a function of the % fat mass. Conclusions Body composition of Sri Lankan infants is comparable to the normative data available from the industrialized countries. Most of the commonly used anthropometric prediction equations generated a bias which varies with the size of the body fat. Only three prediction equations (Bandana, Goran, Durnin & Wormsley) yield a constant bias. The Durnin & Wormsely equation showed the smallest bias when compared to the 18O dilution values with the narrowest limits of agreement. Accuracy of some of the prediction equations is a function of gender. Electronic supplementary material The online version of this article (doi:10.1186/s12887-015-0371-2) contains supplementary material, which is available to authorized users.

Sri Lanka Journal of Child Health, 2009; 38: 72-73 (Key Words: acrodysostosis, short stature, exostoses) Normal 0 false false false EN-US X-NONE X-NONE MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:"Times New Roman"; mso-bidi-theme-font:minor-bidi;} doi: 10.4038/sljch.v38i2.684

Key words: Quadruple cephalhaematomata, neonate, anaemia, heart failure DOI: http://dx.doi.org/10.4038/sljch.v37i4.305 Sri Lanka Journal of Child Health , 2008; 37 : 127-128

Sri Lanka Journal of Child Health , 2008; 37: 26-29 Key words: neurofibromatosis, Chiari malformation, Syringobulbia, duplex kidney DOI = 10.4038/sljch.v37i1.89

Objective To determine the incidence of breast and nipple abnormalities among primigravid women and their effects on lactation in Sri Lanka. Design Prospective descriptive survey. Method All primigravid women attending antenatal clinics of the university obstetric unit, giving informed consent, were recruited into the study. Their breasts were examined clinically and breast abnormalities noted. They were followed up until successful lactation was established. Results 956 mothers were examined. 768 had normal breasts and 188 abnormalities were noted. 725 completed the study. 72.5% established successful lactation. 80 had flat nipples of whom 52 completed the study. 44 flat nipples were corrected antenatally with exercises. All established lactation successfully. Other abnormalities included breast surgery, hypoplastic breasts or breast asymmetry. 9.8% of women with breast/nipple abnormalities failed to establish lactation. Conclusion Large majority (84.6%) of flat non protractile nipples are correctable with antenatal exercises.

Body composition indicators provide a better guidance for growth and nutritional status of the infants. This study was designed to (1) measure the body composition of the Sri Lankan infants using a reference method, the 18O dilution method; (2) calculate the body fat content of the infants using published skinfold prediction equations; and (3) evaluate the applicability of the skinfold equations to predict body fat among Sri Lankan infants against the 18O dilution method. Twenty five healthy, exclusively breast-fed infants were randomly recruited at well-baby clinics, for this cross-sectional study. Body composition was measured using 18O dilution. Infant body weight, length, skinfold thicknesses and mid upper-arm circumference were measured using standard procedures. The Bland and Atlman pair-wise comparison method was used to evaluate the agreement of body fat generated using the anthropometric prediction equations against the 18O dilution values as the reference. Mean (SD) body weight and length of the infants were 6.5 kg (0.9) and 64.7 cm (2.8) respectively. Mean total body water, fat free mass, fat mass and % fat mass as measured by 18O dilution method were 58.8% (5.0), 4.6 kg (0.8), 1.9 (0.5) and 29.5% (6.1). Total body water and fat free mass were significantly higher in boys when compared to girls. With the exception of three prediction equations (Bandana et al., Goran et al. and Durnin and Wormsley), most of the other commonly used anthropometry-based prediction equations yielded a bias which was not constant but a function of the % fat mass. Body composition of Sri Lankan infants is comparable to the normative data available from the industrialized countries. Most of the commonly used anthropometric prediction equations generated a bias which varies with the size of the body fat. Only three prediction equations (Bandana, Goran, Durnin & Wormsley) yield a constant bias. The Durnin & Wormsely equation showed the smallest bias when compared to the 18O dilution values with the narrowest limits of agreement. Accuracy of some of the prediction equations is a function of gender.

Background Many of the countries in the Asia Pacific Region are just initiating newborn screening programs for selected metabolic and other congenital disorders. The present study is aimed at evaluating the congenital hypothyroidism screening program in the Southern region of Sri Lanka in terms of coverage, effectiveness of detecting and managing the cases. Methods The Newborn Screening System Database of Sri Lanka was reviewed from January 2011 to December 2012. The data of 47 babies who tested positive for hypothyroidism were analyzed. Results Total of 78,167 babies (99.0% of live births) were screened. Of them, 5.8% (n = 4,472) were discharged within 12 hrs of delivery where as 58.1% (n = 44969) were discharged afterwards but within next 12 hrs (i.e., day 1). The positive predictive value for congenital hypothyroidism (CH) was 9.0%. The incidences of primary CH among screened infants were 1:1682. False positive rate among screened infants was maintained below 0.70%. Mean age of serum confirmation was 23.8 (±8) days. Conclusions In the light of the present findings, we would suggest direct communication systems, linking newborn screening program to the family unit. This would enhance timely follow-up for screen-positive infants and facilitate information sharing. Establishing a program with, public-private sector partnership should be considered. Costs could be contained if the specimen collection, its transportation and communication are carried out by this partnership and the laboratory tests are conducted by a non-profit organization such as a University in order to achieve the goal of universal coverage. Electronic supplementary material The online version of this article (doi:10.1186/1472-6963-14-385) contains supplementary material, which is available to authorized users.