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Publication Study on Clinico-Laboratory Profile of Children with Scrub Typhus(Nepal Paediatric Society (JNPS), 2012) Jain, N; Jain, VAbstract: Scrub typhus is an acute, febrile, infectious illness caused by Orientia (formerly Rickettsia) tsutsugamushi,an obligate intracellular gram-negative bacterium. Humans are accidental hosts in this zoonotic disease.The present retrospective study describes clinical profile of the children hospitalized in hospital with Scrub Typhus infection during period 2010-2011. 19 hospitalized children with clinical features suggestive of Rickettsial disease (fever, generalized rash) who tested ELISA positive for IgM against Scrub Typhus were included in the present study between the age of 1 years and 15 years. All children presented with fever (100%). 42.10% had lymphadenopathy, 47.36% had eschar and 57.89% patients had cough. 26.31, 21.05%, 10.52%, had hepatomegaly, pain abdomen, and respiratory distress and gastrointestinal bleed respectively. All of the children were investigated and treated with as per protocol. None of them died. We think that Rickettsial infection is not quite common in this region but study showed that it is not an uncommon entity and thus, high degree of suspicion, knowledge of geographical distribution and clinical features of Rickettsial disease helps in its early diagnosis and treatment.Publication Unilateral lower motor neuron-type of facial palsy following snake bite presumably due to Krait (Bungarus Caeruleus)(Nepal Paediatric Society (JNPS), 2012) Verma, S; Kumar, K; Arvind, S; Khadwal, AAbstract: We describe an unusual case of snake bite presumably due to Krait (Bungarus Caeruleus) in a 12-year-old child from Mohali, Punjab. He presented with a history of bite behind his left ear, while he was sleeping at night on floor. He had bilateral ptosis, dysphonia initially, which progressed gradually to cause respiratory paralysis. Child was managed with antisnake venom, ventilation and other supportive measures. He recovered gradually, but persisted to have lower motor neuron paralysis of left facial nerve, which was noted post extubation. This uncommon presentation could be because of exposure of the facial muscles to the venom, spreading directly from the injection site and destroying the nerve terminals of facial nerve in the muscle tissue. At three months follow-up, child showed complete recovery. Facial nerve involvement following snake bite, which usually has a good prognosis, remain an uncommon presentation in paediatric age group.Publication Tuberous Sclerosis in a Child(Nepal Paediatric Society (JNPS), 2012) Baghel, BAbstract: A female child presented with the complaints of seizure and learning difficulties. On examination various types of skin lesion were found, including adenoma sebacum, hypomelanic macules ("ash leaf spots"), Shagreen patches. On investigation, CT scan of brain showed subepedymal nodule in walls of ventricle. B scan of eyes revealed multiple tiny nodular lesions in posterior chamber of eyes called astrocytic hamartomas (or "phakomas"). Antiepileptic drugs started, now patient is seizure free.Publication Staphylococcal Scalded Skin Syndrome in an Infant(Nepal Paediatric Society (JNPS), 2012) Taksande, A; Vilhekar, KAbstract: Staphylococcal scalded skin syndrome is a bullous disease of the skin caused by toxins (exfoliatins) secreted by certain types of Staphylococcus aureus. These toxins may act as superantigens, stimulating the proliferation of T lymphocytes, with a massive release of cytokines and consequent epidermal damage. In neonates, the lesions are mostly found on the perineum or periumbilically, while the extremities are more commonly affected in older children. The disease begins with erythema and fever, followed by formation of large fluid filled bullae which quickly rupture on slightest pressure to leave extensive areas of denuded skin. We present a case of staphylococcal scalded skin syndrome in infant.Publication Progeroid Syndrome of De Barsy With Hypocalcemic Seizures(Nepal Paediatric Society (JNPS), 2012) Cheriathu, J; D'souza, IE; John, LJ; Bahtimi, R ElAbstract: De Barsy et al first reported a rare cutaneo-oculo-cerebral malformation-syndrome now commonly referred as ‘progerioid syndrome of de Barsy’. It is the constellation of progeria-like appearance, cutis laxa, intrauterine growth retardation, corneal clouding and hypotonia. We report a case of Debarsy syndrome in a neonate presented at birth with typical clinical features with hypocalcemic seizures. There are no previous reports among Afghani origin and also first case reported from United Arab Emirates, there have been no reported cases of hypocalcemic seizures.Publication Lamotrigine Induced Severe Cutaneous Reaction(Nepal Paediatric Society (JNPS), 2012) Roka, YB; Roka, N; Adhikari, HBAbstract: Antiepileptic drugs are used commonly by physicians, neurologist and neurosurgeons in Nepal. Lamotrigine is an effective antiepileptic drug used as an add-on and monotherapy for a variety of seizure types in both adults and children. Rashes due to AED including SJS and hypersensitivity syndrome leading to hospitalization occur in approximately 0.33% of adults and 0.8% in children. Reactions due to LTG alone occur in around 5.7% of cases. We report probably the first case of this reaction in a 12-year old girl with a brief discussion on its etiology and management.Publication Holocord Syrinx, Tethered Cord and Diastematomyelia: Case Report and Review of Literature(Nepal Paediatric Society (JNPS), 2012) Maheshwari, A; Yadav, D; Aneja, S; Kaur, S; Patra, B; Seth, AAbstract: Syringomyelia refers to the presence of cavities within the spinal cord or a dilatation of the central spinal cord canal. In 90% of cases, syringomyelia is associated with a Chiari I malformation. The association of syringomyelia with tethered cord is well known but syrinxes associated with these defects are usually below vertebral level T6. Holocord syrinx associated with tethered cord is rare and is almost always associated with Chiari 1 malformation. To the best of our knowledge, only a single case report of holocord syrinx with tethered cord has been reported, but this patient had multiple overt lumbosacral defects (tethered cord, meningocele and diastematomyelia). We are reporting a three year old child with holocord syrinx with tethered cord and diastematomyelia and no evidence of Chiari malformation, meningocele or any overt spinal malformation and minimal neurological abnormalities.Publication Cystic Hygroma of Wrist(Nepal Paediatric Society (JNPS), 2012) Wani, I; Bhat, AR; Amin, A; Wani, S; Malik, RAbstract: Cystic hygroma is the rare congenital lesion. This is a cystic variety of lymphangioma. Cystic hygroma arising outside of the cervicofacial, thoracic, and abdominal areas are extremely rare. Wrist is a very rare site for occurrence of cystic hygroma. Presentation depends on site of location of cystic hygroma. A case of cystic hygroma of wrist in a child is reported. This child presented with painless swelling of left wrist. Excision of cyst was done. This is first case report of literature reporting occurrence of cystic hygroma on wrist.Publication A Rare Case of Idiopathic Congenital Complete Heart Block in a Neonate(Nepal Paediatric Society (JNPS), 2012) Sinha, R; Negi, V; Dala, SSAbstract: Congenital complete heart block in utero has become diagnosed more frequently with the clinical use of fetal echocardiography. Autoimmune-associated congenital complete heart block (CHB) is a rare but important disease that can now be diagnosed in utero using fetal echocardiography1,2,3,4. The overall incidence of isolated CHB has been reported to be approximately 1:15,000 to 20,000 live births5. Several reviews have shown that children diagnosed prenatally have an increased mortality compared to those diagnosed immediately after birth or later in childhood1,2,3,4. At birth, some neonate with complete heart block may remain asymptomatic and may not require a pacemaker but majority require pacemaker to increase the heart rate. We report a rare case of idiopathic congenital complete heart block in a newborn male.Publication IGF-1 and IGFBP 3 in Growth Hormone Deficiency Role of Insulin Like Growth Factor-1 (IGF-1) and IGF Binding Protein 3 in the Diagnosis of Growth Hormone Deficiency: Changing Paradigm(Nepal Paediatric Society (JNPS), 2012) Kota, SK; Jammula, S; Gayatri, K; Kota, SK; Tripathy, PR; Modi, KDAbstract: GH stimulation tests are widely used in the diagnosis of GH deficiency (GHD), although they are associated with a high false positive rate. Serum IGF-I levels are monitored during GH replacement treatment in subjects with GH deficiency (GHD) to guide GH dose adjustment and to minimize occurrence of GHrelated side-effects. The need for reliance on provocative testing is based on evidence that the evaluation of spontaneous growth hormone (GH) secretion over 24 hours and the measurement of IGF-I and IGFBP-3 levels do not distinguish between normal and GHD subjects. Regarding IGF-I, it has been demonstrated that very low levels in patients highly suspected for GHD (i.e., patients with childhood-onset, severe GHD, or with multiple hypopituitarism acquired in adulthood) may be considered definitive evidence for severe GHD obviating the need for provocative tests. However, normal IGF-I levels do not rule out severe GHD and therefore adults suspected for GHD and with normal IGF-I levels must undergo a provocative test of GH secretion. We hereby review the various literatures at disposal justifying the use of IGF-1 and IGBP3 for diagnosis of growth hormone deficiency. Data Source: We searched PUBMED and MEDLINE database for relevant articles including key words. References of each article were further reviewed for final synthesis of the manuscript.Publication Trends in Perinatal Mortality in Tribhuvan University Teaching Hospital: 13 Years Review(Nepal Paediatric Society (JNPS), 2012) Shrestha, M; Shrestha, L; Basnet, S; Shrestha, PSAbstract: Introduction: The perinatal mortality rate (PMR) in Nepal is still very high. In major hospitals of Nepal, it is still ranging from 20-30 per thousand births. This study was carried out with the objective to review PMR and classifying it according to Wigglesworth classification to identify the causes of perinatal deaths at Tribhuvan University Teaching Hospital (TUTH), Kathmandu, Nepal over the past 13 years and assess need for improvement in care. Material and Methods: It was a retrospective study carried out in TUTH. Data of all stillbirths from 28 weeks of pregnancy and neonatal deaths within first seven days of life in the hospital was taken from monthly perinatal audit and annual mortality review. All the perinatal deaths were then classified according to Wigglesworth classification. Results: Over a 13 year period, there were total 42,746 births and 921 perinatal deaths giving a perinatal mortality of 21.5 per thousand births. Over this period PMR has decreased from 31 to 18 per thousand births. Still births contributed almost 50% of the perinatal deaths; deaths related to prematurity show an increasing trend and have increased by almost 70% in past 5 years. Deaths due to perinatal asphyxia were static. Conclusion: PMR over the years has shown declining trend at TUTH. There is need to improve antenatal, obstetric as well as intrapartum services to further reduce the still birth as well as deaths due to prematurity and perinatal asphyxia.Publication Transcranial Doppler Study Among Children with Sickle Cell Anaemia Vs Normal Children(2012) Lakhkar, BB; Lakhkar, BN; Vaswani, PAbstract: Introduction: Role of transcranial Doppler in prevention of stroke in sickle cell children has been well appreciated. Studies are being done to develop the protocol in children. Since we don’t find stroke very commonly in this part of the world, this study was done in order to see the prevalence of abnormal flow velocity in sickle children attending sickle cell clinic. The aims of this study were to measure mean flow velocity in different vessels in homozygous sickle cell patients using transcranial Doppler study, to compare the mean velocity in sickle children with age and sex matched controls and to correlate mean velocity with headache or stroke if any and also to correlate mean velocity with number of transfusions. Materials and Methods: The study was done in Paediatric wards. It was a prospective crosssectional comparative study. Twenty six children below 14yrs of age with homozygous sickle cell disease attending the Sickle cell clinic were selected as the cases. Forty cases of similar age and sex were recruited as normal control group. Transcranial Doppler was done in six different vessels in both the groups and mean flow velocity was measured. Mean flow velocity was correlated with symptoms and number of transfusions. Velocity was classified as normal (<170cm/sec), conditional (170-199cm/sec) or abnormal (>200cm/sec). Statistical analysis was done using SPSS 10 software. Results: In normal age and sex matched controls mean blood flow velocity was 50cm/sec where as in the cases of sickle cell disease was 180cm/sec. Maximum mean velocity was observed in middle and posterior cerebral artery. In two Sickle cell cases (8%) blood flow velocity was abnormal, these children had headache though received 5-10 transfusions/year. In only 4% sickle cell children flow velocity was normal and rest had conditional velocity. Among these children 39% received less than 5 and rest received 5-9 transfusions /yr and had no symptoms of stroke. Conclusions: Flow velocity measured by Transcranial Doppler is highest in middle cerebral artery and Posterior cerebral artery which appear to be the best arteries for this test in this region. Flow velocity was significantly high in children with sickle cell disease as compared to normal children. Prevalence of abnormal flow velocity in our children was 8% and children with abnormal mean flow velocity presented with headache.Publication Pattern of Ear Diseases among Paediatric ENT Patient: An Experience from Tertiary Care Centre, Pokhara, Nepal(Nepal Paediatric Society (JNPS), 2012) Sigdel, B; Nepali, RAbstract: Introduction: Ear diseases are common in children mainly due to altered anatomy of Eustachian tube which is straighter in children as compared to that in adults. However, the cause of hearing loss in children is more varied, including the etiologies. This study was done to find out the pattern of ear diseases in paediatric age group attending ear, nose and throat OPD in a tertiary care centre in Pokhara, Nepal. Materials and Methods: This is a prospective study done in paediatric patients attending ENT OPD over a period of one year from January 2010 to January 2011. The diagnoses were made on the basis of history and clinical examination. Results were expressed in numbers and percentages. Results: Out of 1632 Pediatric ENT patients, 944 had ear diseases, 59.2% were males and 40.8% females. Wax (33.4%) was the commonest diagnosis followed by Chronic suppurative otitis media (24.3%) and Acute ottitis media (13%). Conclusion: Ear diseases are most common condition in ENT OPD among paediatric age group. Wax, CSOM and ASOM were the three most common ear diseases.Publication Ocular Manifestations of Meningitis in Children(Nepal Paediatric Society (JNPS), 2012) Chaudhary, M; Shah, DN; Sharma, PRAbstract: Introduction: Meningitis is the most common central nervous system disease affecting children leading to focal neurological deficits and various oculovisual anomalies including blindness in children. The objective of this study was to evaluate the oculovisual anomalies occurring in Nepalese children suffering from different types of bacterial meningitis. Materials and Methods: A Prospective, study was undertaken for 18 months at B.P.Koirala Lion’s Center for ophthalmic studies, TU Teaching Hospital to study the children suffering from bacterial meningitis admitted at Kanti Children’s Hospital for ocular involvement. A through history, anterior and posterior segment ocular examination and investigations like blood, CSF and CT scan were done. Results: A total of 182 cases of bacterial meningitis were screened. Tubercular meningitis cases were 40 (21.97%) and Pyogenic were 142 (78.02%). Oculovisual anomalies were seen in 70 (38.46%) cases. The ocular abnormalities included pupillary changes (34.28%), Cranial Nerve Palsy (22.86%), Fundus changes (35.72%), Cortical Blindness (4.28%), Panophthalmitis and Proptosis (1.43%). Third nerve involvement was seen in 17.14% cases, sixth nerve in 4.29% cases, Papilledema in 11.43 % and Optic atrophy in 22.86 %. Risk factors included late presentation; hydrocephalous and increased CSF cell count and protein level. Conclusion: Oculovisual anomalies formed an important group of clinical manifestations of bacterial meningitis. Incidence of oculovisual anomalies was more frequently seen in Tubercular meningitis (55%).Children with early presentation and intervention had better prognosis. Hence, timely intervention and health education is important.Publication Molecular Characterization of Citrobacter freundii Isolated from Neonates in Neonatal Intensive Care Unit of Nepal(Nepal Paediatric Society (JNPS), 2012) Thapa, B; Tribuddharat, CAbstract: Introduction: Nosocomial Citrobacter spp. is emerging as a successful nosocomial pathogen in neonates in Nepal. The important risk factor being poor infection prevention and control practices. The objective of this study was to investigate the clonal relatedness of Citrobacter freundii isolated from clinical and nonclinical sources in Neonatal Intensive Care Unit (NICU) and to determine the presence of Extended Spectrum Beta-Lactamase (ESBL) genes and class 1 integron element. Materials and Methods: Polymerase chain Reaction (PCR) and PCR-Randomly Amplified Polymorphic DNA typing of the isolates were performed in three isolates to amplify class 1 integron element integrase gene, ESBL genes, and to study the clonal relatedness, respectively. Results: Two isolates harbored class 1 integron element. The blaCTX-M was present in all isolates and blaTEM-1 was present in one isolate. An isolate carried blaCTX-M and blaTEM-1 genes. All of these isolates were not clonally related. Conclusion: The study for the first time documented the emergence and spread of ESBL genes and class 1 integron element in multidrug resistant C. freundii in Nepal and urge for monitoring and surveillance of these strains.Publication Impact of Family Conflict on the Psychosocial Behaviour in Male Adolescents(Nepal Paediatric Society (JNPS), 2012) Chhabra, GS; Sodhi, MKAbstract: Introduction: Accepting and supportive family relationships during childhood and adolescence may have long-term associations with psychosocial functioning into adulthood. Cross-sectional studies provide evidence by which positive family relationships promote adolescent health by increasing the likelihood of positive outcomes including higher educational and occupational attainment and reducing the risk for negative outcomes including suicidal behaviour and poor health. Materials and Methods: The sample consisted of 500 male adolescents from the urban and rural field practice areas in the vicinity of Sri Guru Ram Das Medical College Amritsar. Cross sectional study was conducted to examine the relation between familial conflict problems and psychosocial/behavioural disorders in adolescents. Results: Almost one third (33%) adolescents were having parental and family dispute problems. These problems were significantly higher in middle adolescence (14-16 years), adolescents of large extended families (>8 members) and in lower socioeconomic status. Residence had no significant relation to family problems in the adolescents. On correlation, poor and unhealthy family atmosphere had more chances in adolescents of having greater academic problems, depression, suicidal thoughts, substance abuse and more sexual activity. Conclusion: Family involvement, family structure, parental values, parental monitoring and parent-child communication are important factors influencing critical life choices in teens. The family support and communication should be improved through these education programs to develop healthy psychosocial and sexual attitudes and counteract unsafe behaviours in the adolescents. Future research is needed to identify additional pathways underlying this association, and the extent to which these factors are modifiable.Publication Evaluation of physical growth and sexual maturation rating of Adolescent boys(Nepal Paediatric Society (JNPS), 2012) Gedam, DS; Waseem, M; Chansoria, MAbstract: Introduction: The present study is an attempt to evaluate the incidence of malnutrition as well as obesity. We have evaluated physical growth and sexual maturity rating of 10-18 years adolescent boys–overweight Vs Normal Vs Underweight. Material and Methods: A random sample of 530 adolescent boys (one private English Medium and one Govt. Hindi Medium School) between the ages of 10-18 years during the period of August 2008 to August 2009 constituted the material for this study. Weight, Height and BMI and Sexual maturation rating (SMR) were calculated. Results: Nearly 39% of the adolescent boys studied were either underweight (31%) or overweight (8%). All overweight adolescent boys have their mean weight more as compared to WHO 50th centile. Normal weight and underweight adolescent boys have lesser mean weight as compared to WHO 50th centile. All overweight and normal weight adolescent boys have their mean height lesser as compared to WHO 50th centile. All underweight adolescent boys except for age group of 12 yr have their mean height lesser as compared to WHO 50th centile. Underweight boys achieved different stages of genital growth prior to normal weight and overweight boys but lag behind as compared to Tanner. It was obvious that underweight boys achieved different stages of pubic hair growth prior to normal weight and overweight boys. Conclusion: Present study indicates that incidence of overweight children is also increasing significantly. Adolescent psychological need should be assessed and they should be given knowledge regarding their normal sexual growth pattern to decrease their anxiety.Publication Evaluation of Nutritional Status by Mid-Upper Arm Circumference Amongst Affluent Children(Nepal Paediatric Society (JNPS), 2012) Maiti, S; De, D; Ali, KM; Ghosh, A; Ghosh, Debidas; Paul, SAbstract: Introduction: Anthropometry is widely accepted as low-cost technique for defining the nutritional status of children. The mid upper arm circumference (MUAC) is a fast screening method in detecting acute malnutrition and it is also a predictor of childhood mortality. Materials and Methods: This is the retrospective study to evaluate the nutritional status based on mid upper arm circumference (MUAC) among urban, pre-school age children. A total of 1060 children, aged 1 to 3 years, attending the vaccination clinic of the Indian Red Cross Society Paschim Medinipur Hospital over a two years period, were enrolled in the study. The measurement of MUAC (in centimetres) was taken by the standard technique. Undernutrition was defined based on age and sex-specific MUAC cut-off values as recommended by the World Health Organization (WHO). Results: Boys were higher level of mean MUAC than girls at all ages and there was no significant sex differences. The age-combined rate of overall undernutrition was 18.96% and it was slightly higher among the boys (19.38%) than girls (18.46%). The age-combined moderate undernutrition was higher among the boys (16.08% vs 14.11%) but girls’ value was higher in case of severe undernutrition (3.28% vs 4.35%). Conclusion: The present study showed that a remarkable number of pre-schooler was undernourished based on MUAC.Publication Congenital Anomalies at Birth: A Hospital Based Study in UAE(Nepal Paediatric Society (JNPS), 2012) Aryasinghe, L; Moezzi, D; Ansari, TA; Mathew, E; Sharbatti, SA; Shaikh, RBAbstract: Introduction: It is widely acknowledged that congenital anomalies heavily contribute to infant morbidity and mortality worldwide, with an estimated 9 million infants (7% of all births) born annually having a serious congenital anomaly that result in death or lifelong disability. The objective of our study was to estimate the frequency of congenital anomalies at the Gulf Medical College Hospital and Research Center, Ajman and to estimate the proportion of births with congenital anomalies per 1,000 live births. Materials and Methods: It was a Hospital-Record based descriptive study of the 1,222 consecutive live births from December 2007 to June 2008. Results: There were 84 cases of congenital anomalies among the 1,222 live births; the rate of anomalous births was 68.7 per 1,000 live births or 6.9%. Anomalies of the genitourinary system (40.5%) were the most common, followed by musculoskeletal (28.6%) and cardiovascular (10.7%). Gastrointestinal, chromosomal and multiple system anomalies accounted for 3.6% each and miscellaneous anomalies were seen in 7.1%. Central Nervous system (2.4%) anomalies were the least. Congenital hydrocele (19.0%) was seen to have the highest frequency amongst all recorded anomalies, followed by Talipes or Clubfoot (14.3%). 9.3% of the male and 4.3% of the female population of live births had a congenital anomaly. Birth Weight (p=0.005) and Gender (p=0.001) were found to have significant association with the frequency of birth defects. Maternal Age, gravidity, parity, previous abortions, VDRL and Hepatitis B status, Gestational Diabetes, Pregnancy Induced Hypertension, gestational age, maternal and infant blood group, nationality and consanguinity showed no statistically significant association with congenital anomalies. Conclusion: The proportion of congenital anomalies found in our study is alarmingly high and emphasizes the need for more accessible nationwide screening, counseling and surveillance systems.Publication Comparison of Pain Response to Venepuncture Versus Heel Lance Blood Sampling in Term Neonates(Nepal Paediatric Society (JNPS), 2012) Shrestha, M; Adhikari, RKAbstract: Introduction: Pain in neonates is largely underestimated and neglected. Pain experience can alter clinical outcome, brain development and subsequent behavior in newborns. Numerous newborns undergo blood sampling routinely in nurseries/NICUs and these procedures are often done without pain relieving measures. Heel lancing and venepuncture are two common procedures for blood sampling in neonates. The objective of this study was to compare pain response to venepuncture versus heel lance in full term neonates. Materials and Methods: A comparative observational study was conducted among 200 term neonates who were undergoing blood sampling for bilirubin or glucose estimation. Neonates were randomly assigned to heel lance (HL) and venepuncture (VP) groups with 100 babies in each group. During the procedure, pain was assessed by Neonatal/Infant pain Scale (NIPS). Heart rate (HR) and oxygen saturation (SpO2) were continuously monitored 5 minutes prior to procedure and upto 5 minutes after the procedure. Results: The median NIPS score in HL and VP were 7 and 3.5 respectively which showed statistically significant (p= 0.0001) higher level of pain experience in HL than in VP. During the procedure, both the groups (HL and VP) showed significant changes in heart rate and oxygen saturation i.e., increase in HR (p= 0.0001) and decrease in SpO2 (p= 0.0001), however the increase in heart rate and decrease in oxygen saturation were significantly more in HL than in VP (p= 0.0001). Conclusion: Neonates perceive pain as demonstrated by Neonatal Pain Scale and venepuncture is the less painful procedure than heel lancing for blood sampling in neonates.