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https://hdl.handle.net/20.500.14572/6

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Caffey’s Disease (Infantile Cortical Hyperostosis)
(Nepal Paediatric Society (JNPS), 2015) Paria, Pradipprava; Halder, Ramesh Chandra; Ghosh, Sibarjun
Abstract: Caffey's disease is a rare, self limiting condition of infancy. Most commonly mandible, clavicle and ulna are affected leading to inflammation of periosteum and overlying soft tissue. We here present an 1 year 3 month old girl with features of Caffey's disease.
Multi Organ Dysfunction in Term Neonates with Perinatal Asphyxia
(Nepal Paediatric Society (JNPS), 2015) Vemuri, Aditya; Lalwani, Sanjay
Abstract: Introduction: Multiorgan dysfunction (MOD) is one of the four consensus based criteria for the diagnosis of intrapartum asphyxia. The theoretical concept behind MOD is the diving reflex -conservation of blood flow to vital organs at the cost of non-vital organs. The objective of this study was to assess the patterns of involvement of each major organ/system in term asphyxiated neonates. Material and Methods: This was a hospital based prospective study. Sixty term neonates who had suffered perinatal asphyxia were assessed for central nervous system, kidney, cardiovascular system, gastrointestinal system and liver dysfunction. Results: Out of 60 eligible neonates, 57 (95%) had evidence of at least one organ dysfunction. Cardiovascular system involvement (95%) was most common, followed by renal system (37%), hepatic system (22%), central nervous system and hematological system (20% each) and finally, gastrointestinal tract (8%). Conclusion: MOD is frequently associated with perinatal asphyxia with cardiovascular system being the most commonly affected.
Thanatophoric Dysplasia
(Nepal Paediatric Society (JNPS), 2015) Badal, Sachendra; Roy, Shuvendu; Singh, Daljit
Abstract: Thanatophoric Dysplasia (TD) is a severe skeletal dysplasia that is lethal in the neonatal period. There are two defined TD subtypes which have been classified clinically. The incidence is approximately 1/20,000 to 1/50,000. Type I TD being more frequent than Type II. Most individuals with TD die within the first few hours.This condition has characteristic sonographic features detected antenatally by midgestation, although distinction from other short-limbed dysplasia syndromes may be difficult. We report a case of type I TD with typical clinicoradiological features who succumbed within one hour of life.
Spontaneous Perforation of Stomach in a Neonate
(Nepal Paediatric Society (JNPS), 2015) Sethi, Harish; Simalti, Ashish; Chauhan, SS; Chandra, Mukesh
Abstract: Spontaneous gastric perforation is not a very common entity. If not suspected and managed in time, this is uniformly fatal. Although documented as early as in1825 exact pathology remains unclear even to date. A case of spontaneous rupture in a term neonate is being presented as a case report.
Splenic Infarction in Plasmodium Falciparum Malaria: Case Report and Review of Literature
(Nepal Paediatric Society (JNPS), 2015) Dwivedi, Deepak; Singh, Jyoti
Abstract: Splenic involvement is frequently observed during malarial episode. This involvement may vary from simple asymptomatic splenic enlargement to serious complication such as hematoma, rupture, or infarction. Very few cases of splenic infarction in paediatric patients are reported in literature. This case report suggests that if a patient with malaria is complaining of left upper quadrant abdominal pain, and/or enlarging tender splenomegaly is observed during treatment, splenic infarct should be suspected and managed accordingly to avoid further life-threatening complications.
Rasmussen's Encephalitis
(Nepal Paediatric Society (JNPS), 2015) Mitra, Nibedita; Kavitha, G; Nithya, M; Bakhne, Atish
Abstract: Rasmussen’s encephalitis is a chronic inflammatory disease of unknown origin affecting one cerebral hemisphere. We report a case of a seven year old boy who presented with unilateral seizures and progressive hemiparesis.
Rare Presentation of Epstein Barr Virus
(Nepal Paediatric Society (JNPS), 2015) Ari, Abhijit; Ghosh, Taraknath; Bose, Sayan; Shetty, Prashat Kumar; Chaudhary, Ruchi
Abstract: Two rare complication of infection with the Epstein-Barr virus is the development of hemophagocytic Lymphohistiocytosis and opsoclonus myoclonus syndrome. Here we are describing two cases which are describing these complications of Epstein-Barr virus infection. As they mimic several other condition but a strong suspicion is needed to diagnose these rare complications early as it will help us to reduce morbidity and mortality of these complications.
Johanson-Blizzard Syndrome with Short Stature
(Nepal Paediatric Society (JNPS), 2015) Khorasani, Enayatollah Nemat; Mansouri, Fariba
Abstract: Johanson–Blizzard syndrome (JBS) is a rare, sometimes fatal autosomal recessive multisystem congenital disorder featuring abnormal development of the pancreas, nose and scalp, with mental retardation, hearing loss and growth failure. It is sometimes described as a form of ectodermal dysplasia. The disorder is especially noted for causing profound developmental errors and exocrine dysfunction of the pancreas, and it is considered to be an inherited pancreatic disease. We report a ten years Irannian child with signs and symptoms suggestive of this syndrome (JBS).
Familial Hemophagocytic Lymphohistiocytosis (FHL), Report of two Unique Cases
(Nepal Paediatric Society (JNPS), 2015) Mishra, Shubhankar; Agarwalla, Sunil Kumar; Behura, Sushree Smita; Pattnaik, Gouranga Charan
Abstract: Familial hemophagocytic lymphohistiocytosis (FHLH) is a rare genetic disorder associated with early onset in life with overwhelming activation of T lymphocytes and macrophages invariably leading to death. We present two cases of FHLH admitted to our hospital at different points of time. First child presented with multiple episodes of GTCS and high grade fever. There was a history of sibling death before. He was having hepatosplenomegaly with leucopenia, hyper-triglyceridemia, hyper-ferritinemia and bone marrow revealed abundant hemophagocytes in smear. Second case was a 6 month male with complaint of (Generalised Tonic Clonic Seizure (GTCS) with past history of repeated attacks of acute Respiratory Infection and neuroinfection. Previous sibling died in similar presentation. He was having hepatosplenomegaly, leucopenia, hyper triglyceridemia, hyper ferritinemia with abundant hemophagocytes in bone marrow smear. Both the cases were diagnosed as FHLH and treated according to protocol.
Doss Porphyria (δ-Aminolevulinic Acid Dehydratase Porphyria) Presenting with Acute Onset Flaccid Paralysis
(Nepal Paediatric Society (JNPS), 2015) Singh, Sandip Kumar; Gauchan, Eva; Koirala, Deepak Prasad; Rao, KS
Abstract: δ–Aminolevulinic acid dehydratase porphyria is an autosomal recessive disorder of heme synthesis resulting from deficiency of δ-aminolevulinic acid dehydratase (ALAD). Patients present with fatal neurovisceral manifestations and motor neuropathy. Here we report a patient with rapidly progressive flaccid tetraplegia with respiratory and bulbar paralysis. The importance of early diagnosis, prompt treatment and screening of relatives is stressed.
Complex Spinal Dysraphism Complicated by Intramedullary Dermoid
(Nepal Paediatric Society (JNPS), 2015) Gopal Sedain,; Mohan Raj Sharma,; Sushil Krishna Shilpakar
Abstract: Spinal dysraphism presents in myriad ways. Meningomyeloceles presenting with Split cord malformations are termed as complex dysraphisms. We encountered a child with myelomeningocele and a split cord with intramedullary lesion making it a more complex dysraphism.
The Association Between Biliary Atresia and Cytomegalovirus Hepatitis
(Nepal Paediatric Society (JNPS), 2015) Lazim, Hussam Hussain; Arif, Hala Sameh; Kadhim, Haider Sabah; Al-Khafaji, Khitam Razak; Abdulghafour, Kifah Hamdan
Abstract: Introduction: Biliary atresia (BA) is a disease characterized by a biliary obstruction of unknown origin. Viral agents have been proposed in the aetiology of BA such as cytomegalovirus (CMV). This virus also considered as a one of agents that can infect the liver and cause hepatitis. The aim of this study was to determine the role of CMV in children with both chronic hepatitis (negative for hepatitis B and C) and have biliary atresia in the same time. Material and Methods: A retrospective study done on 13 liver tissue paraffin blocks of children with chronic hepatitis (negative for hepatitis B and C) and biliary atresia (extra and intra). The diagnosis was based on the presence of HCMV protein (pp65) by using immunohistochemistry. Results: Immunohistochemistry for pp65 showed the liver tissue blocks were positive for 10 cases (76.9%).The mild inflammation and moderate fibrosis were the highest among the cases. Conclusion: CMV is one of the important viruses that can causes hepatitis in infants (whom are negative for hepatitis B and C), also this virus has significant role in pathogenesis of biliary atresia.
Nucleated Red Blood Cell in Cord Blood as a Marker of Perinatal Asphyxia
(Nepal Paediatric Society (JNPS), 2015) Kanodia, Piush; Bhatta, Nisha Keshary; Singh, Rupa Rajbhandari; Shah, Gauri Shankar; Yadav, Shankar Prasad; Yadav, Sunil Kumar
Abstract: Introduction: Perinatal asphyxia is a common problem with the incidence varying from 0.5 –2% of live births. According to World Health Organization, approximately 4 million babies die each year before they reach the age of one month. The number of NRBC/100 WBC is variable but is rarely greater than 10 in normal neonates. This simple test can be helpful in the rapid assessment of perinatal asphyxia. Material and Methods: This prospective case-control study and there were 82 newborns in Case and 82 newborns in Controls comprising of asphyxiated and nonasphyxiated neonates, respectively, over a period of 12 months. Results: Out of the 82 neonates in case group, fifty nine (59) neonates were found to have NRBC level ≥10/100WBC, out of which 58 (70.7%) were cases and 1(1.2%) was a control. NRBCs count of ≥10/100WBC were seen more in the newborn who had low 5 min Apgar score and in the newborn with severe HIE, these association were statistically significant (P value <0.001). The cut-off NRBC value of ≥10/100WBC also found to have a sensitivity of 70.30% with a specificity of 98.78%. NRBC has a positive predictive value of 98.31% with a negative predictive value of 77.14%. Significance and sensitive area for ROC curve was 0.875. The ROC curve was calculated with cut-off NRBC value of ≥10/100WBC. Conclusions: NRBC counts can be very useful to differentiate HIE newborns from non-HIE newborns which will help in appropriate management and better outcome of these newborns.
Morbidities and Mortalities among Infant of Diabetic Mother in a Newly Established Scabu of a Tertiary Care Hospital, Bangladesh
(Nepal Paediatric Society (JNPS), 2015) Islam, Mohammad Nazrul; Tazmin, Tanha; Siddika, Mohosina; Bhuiyan, Mohammod Kamruj Jaman
Abstract: Introduction: Diabetes mellitus has long been associated with perinatal morbidity and mortality. It complicates 2% to 3% of all pregnancies; 90% of these cases present with gestational diabetes mellitus. In our population Gestational diabetes mellitus develops among 6.7% of all pregnancies. Macrosomia (28%), hypocalcaemia (22%), hyperbillirubinaemia (19%), polycythemia (34%), perinatal asphyxia, birth injury and congenital anomaly (6-9%) are the complications of infant of diabetic mother (IDM). Materials and Methods: This prospective observational study was done in the newly established Special Care Neonatal Unit (SCANU) to determine the morbidities and mortalities among IDM babies admitted in to Mymensingh Medical College Hospital (MMCH), Bangladesh from January to March 2015. A total 50 IDM patients who admitted during this period were recruited in the study irrespective of their gestational age, birth weight, pattern and duration of maternal diabetes. Results: Male and female patients were 29 (58%) and 21 (42%). Caesarian and vaginal delivery were 43 and seven cases. Gestational and pre-gestational diabetes mothers were 35 (70%) and 15 (30%) respectively. The important morbidities in order of frequency were found perinatal asphyxia (50%), macrosomia (48%), neonatal jaundice (44%), hypoglycaemia (40%), hypocalcaemia (36%), polycythemia (28%), CHD (20%), neonatal sepsis (20%), birth trauma (12%), TTN (6%), RDS (6%), GIT problem (2%) respectively. Mortality was recorded in three patients. Conclusion: Our observations show the high prevalence of IDM (24/1000 live birth) and their various complications. Mortality and morbidity is a bit higher in IDM. Our health policy maker should give adequate emphasis on management of IDM babies.
Incidence of Retinopathy of Prematurity and Its Association with Oxygen Therapy in Preterm Low Birth Weight Babies
(Nepal Paediatric Society (JNPS), 2015) Pal, AK; Sur, Somen; Gupta, AK
Abstract: Introduction: The objectives of this study were to study the incidence and risk factors of retinopathy of prematurity (ROP) among preterm low birth weight babies and its association with oxygen therapy. Material and Methods: During one year study period, 50 neonates, who had fulfilled the inclusion criteria (born at gestational age ≤ 35 wks), were included in the present study. They were examined by indirect ophthalmoscopy by an ophthalmologist in NICU or ophthalmology outdoor. Staging of ROP was done according to the International classification. Data obtained from this study were entered in Microsoft Excel and subsequently analysed in SPSS version 20.0. Results: Among study population, 12 neonates (24%) showed evidence of retinopathy of prematurity, and Stage 2 ROP was most common (41.7%) within this group. Those neonates who had born ≤28 wks gestational age, showed more incidence (75%) of ROP. There was significant association between ROP and prematurity, low birth weight, mechanical ventilation, duration and concentration of oxygen therapy. Conclusion: Apart from prematurity and low birth weight, oxygen therapy also carries significant risk for ROP. Thus, judicious use of oxygen in premature babies can reduce incidence of ROP significantly.
Home Management of Childhood Diarrhoea by Mothers Presenting at a Tertiary Hospital in Bangladesh
(Nepal Paediatric Society (JNPS), 2015) Akhtaruzzaman, Muhammad; Hossain, Md Anwar; Choudhury, Ahmed Murtaza; Islam, Md Nazul; Dhar, Sonjib Kumar
Abstract: Introduction: Diarrhoea is a leading cause of under-five morbidity and mortality. Most of the diarrhoeal episodes are treated initially at home by mothers. The objective of the study was to assess mothers’ knowledge and practice of home management of diarrhoea. Material and Methods: This hospital based descriptive cross sectional study was carried out in Mymensingh Medical College Hospital. Three hundred and sixty children under five years of age having acute diarrhoea were included in the study by systematic random sampling. A structured pretested interview schedule was used to collect data. There were 11 knowledge and 11 practice questions; each was scored a point if correctly answered. The level of knowledge and the level of practice both were classified as poor for score 0-5 and good for score 6-11. Results: Of the 360 mothers, 45.55% had a good level of knowledge, while 33.89% had good level of practice. Both higher knowledge and practice score was significantly (p<0.001) associated with mother’s age, educational level and occupation and father’s educational level occupation and income. After adjusting for other factors, mother’s age was significantly associated with level of knowledge (p=0.003) and mother’s educational level was significantly associated with both level of knowledge (p=0.001) and practice (p=0.002) in home management of diarrhoea. Conclusion: The study has shown a low level of knowledge and practice in mothers regarding management of diarrhoea. The educational programs for mothers must be an essential part of the health facility programs.
Cord Serum Bilirubin Level in Predicting the Development of Significant Hyperbilirubinemia in Newborns with ABO Incompatibility
(Nepal Paediatric Society (JNPS), 2015) Arora, Sunita; Shifali
Abstract: Introduction: Neonatal hyperbilirubinaemia is common problem which is benign in majority of neonates. Rh iso immune hemolytic disease as a cause of hyperbilirubinemia is becoming nearly nonexistent due to the use of prophylactic anti D. Hence Isoimmune hemolytic disease due to ABO incompatibility assumes significance as a cause of significant hyperbilirubinaemia. This study was conducted to determine the incidence of ABO incompatibility, ABO iso immune disease in new born, to determine critical cord serum bilirubin level to predict subsequent significant hyperbilirubinemia. Material and Methods: The study was done in neonatal ICU of a tertiary care hospital where 100 full term healthy newborns with B.W≥2500gm and gestational age ≥37 wk with blood group A, B, AB, born to mothers with O blood group without simultaneous Rh incompatibility at SGRDIMSR were included. Serum bilirubin was measured approximately at 12-24hrs, 36-48hrs, 60-72hrs. Results: Out 100 ABO incompatible newborns 33(33%) developed ABO isoimmune disease manifesting as significant hyperbilirubinaemia with any of the four total serum bilirubin levels exceeding threshold levels defined for phototherapy. TSB of ≥ 2.16mg/d1 from cord blood has a sensitivity of 100% specificity of 89.55%, NPV 100% and PPV of 82.50% to predict significant hyperbilirubinaemia. Conclusion: A critical cord S.bilirubin between 2.16 mg/d1 and 4.09mg/d1 will predict all newborns who will have significant hyperbilirubinaemia and can be used as a safe demarcator to decide time of discharge. Any therapeutic intervention if necessary can be started as early as possible.
Clinical Profile of Children with Acute Febrile Encephalopathy in a Tertiary Health Care Center of Nepal
(Nepal Paediatric Society (JNPS), 2015) Sharma, Poonam; Sarmah, BK; Kayastha, Pawana; Shrestha, Asim; Tiwari, Damodar
Abstract: Background: Acute febrile encephalopathy is a common and severe neurological syndrome that is associated with significant morbidity and mortality in children. The etiologies differ according to geographical regions and appropriate and efficient protocols for investigations and management requires proper understanding of various potential etiologies. Methods: In this prospective observational study, 54 children of one month to 14 years presenting to the hospital with acute onset of fever with altered sensorium were clinically evaluated and investigated. Clinical examinations included temperature, pulse rate, heart rate, blood pressure, coma severity by GCS, respiratory pattern and detail systemic and CNS examination. Etiology of acute febrile encephalopathy was based on clinical history, examination and relevant laboratory investigations. Results: The incidence of acute febrile encephalopathy was 5.5% of the total hospital admissions. There were 35(64.8%) boys and 19(35.2%) girls. The maximum number of children 24(44.4%) were between one to five years of age. The most common presenting complains besides fever and altered sensorium were seizures and vomiting which was present in 59.3% and 46.3% of children respectively. Signs of meningeal irritation, hypertonia, brisk deep tendon reflexes and extensor plantar response was present in 50%, 24%, 29.6%, and 44.4% respectively. The diagnosis based on clinical findings and laboratory investigations were viral encephalitis 34(63%), bacterial meningitis 12(22.2%), cerebral malaria 4(7.4), enteric encephalopathy 3(5.6%) and hepatic encephalopathy 1(1.9%). Conclusion: Viral encephalitis and bacterial meningitis are the most common cause of acute febrile encephalopathy. Preventive strategies can be done to decrease the incidence.
Clinical Profile and Outcome of Mechanically Ventilated Neonates in a Tertiary Level Hospital Authors
(Nepal Paediatric Society (JNPS), 2015) Shrestha, Prabina; Basnet, Srijana; Shrestha, Laxman
Abstract: Introduction: Many sick neonates admitted to neonatal intensive care unit (NICU) require mechanical ventilation but it is associated with various complications and the outcome of neonates is unpredictable. This study aims to identify the indications for mechanical ventilation, complications, co-morbid conditions and outcome of those neonates in terms of survival. Materials and Methods: Retrospective observational study of all neonates who underwent mechanical ventilation from 1 January 2014 to 31 December 2014 in NICU, Tribhuvan University Teaching Hospital. Medical records of the patients were retrieved from hospital record section to collect the relevant data. Results: One-third of admitted neonates in NICU required mechanical ventilation (MV). Commonest indication was severe respiratory distress (70%) followed by perinatal asphyxia (12%) and recurrent apnea (8%). Disease pattern were sepsis (37.2%), RDS of prematurity (17.6%), perinatal asphyxia (11.7%), meconium aspiration syndrome (9.8%), apnea of prematurity (7.8%) and congenital pneumonia (4%). Hospital acquired sepsis was a major complication occurring in 47% patients on mechanical ventilation. Survival rate among neonates on MV was 33%. Survival was better with increasing birth weight and gestational age. Survival was 100% in congenital pneumonia, 50% in perinatal asphyxia, 50% in recurrent apnea, 26% in sepsis, 20% in MAS and 0% in RDS of prematurity. Conclusion: Survival rate of neonates on mechanical ventilation in NICU was 33%. Sepsis was a major problem in NICU, which must be addressed to improve outcome.
A Study on Pattern of Growth in Height Among Children of Dhulikhel Municipality
(Nepal Paediatric Society (JNPS), 2015) Mansur, Dil Islam; Shrestha, Anupama; Sharma, Kalpana; Mehta, Dilip Kumar; Shakya, Rojina; Timalsina, Binod
Abstract: Introduction: Growth reference charts are essential to assess appropriate growth of individual children. The height of an individual is the most widely accepted method for the evaluation of growth of the children. The child's height is primarily determined by the length of his/her bones, and thus the children become tall because their bones grow in length. The purpose of the present study was to develop the mean height reference charts for school going children residing in Dhulikhel and to evolve an easily applied formula to assess the height of the children at different age groups. Material and Methods: The present study consisted of 1726 healthy school going children (945 boys and 781 girls) aged 3-16 years, from different Schools of Dhulikhel Municipality during July – December 2015. Age was recorded in year and height of each child was measured cross-sectionally in centimeter and statistical analysis was done. Results: It has been observed that there was a progressively increasing trend in the mean values of the height with advancement of age in both sexes. The study revealed that the adolescent growth spurt or highest peak velocity of girls (11-12 years) was attained earlier by one year than boys (12-13 years). Conclusions: If a child’s height is consistently or substantially different from the height of other children of the same age and gender, it indicates that the child may have a medical problem and requires monitoring or treatment. The trend of physical growth as observed in height may be expected to serve as the growth standards of Dhulikhel’s children.

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