Painless Loss of Vision as The First Presentation of Undiagnosed Neurofibromatosis 1 in A Child

How to Cite This Article: Paul SP, Ahmed S, Painless Loss of Vision as The First Presentation of Undiagnosed Neurofibromatosis 1 in A Child. Iran J Child Neurol. Autumn 2015;9(4):58-60.AbstractObjectiveA 10 year old presented with painless loss of vision as the first manifestation of neurofibromatosis 1 (NF1). Clinical assessment detected diagnostic features of NF1 and Magnetic Resonance Imaging (MRI) scan confirmed presence of plexiform neurofibroma and bilateral optic pathway glioma (OPG). The child was managed with chemotherapy which helped in improvement of vision. Review of current literature recommends vision testing in diagnosed cases of NP1 till 7 years of age; this is aimed at detecting visual impairments resulting from a symptomatic OPG

Chronic Renal Failure Secondary to Unrecognized Neurogenic Bladder in A Child with Myelodysplasia

How to Cite This Article: Ahmed S, Paul SP. Chronic Renal Failure Secondary to Unrecognized Neurogenic Bladder in A Child with Myelodysplasia. Iran J Child Neurol. Spring 2017; 11(2):78-81.AbstractMyelodysplasia includes a group of developmental anomalies resulting from defects that occur during neural tube closure. Urological morbidity in patients with myelodysplasia is significant and if not treated appropriately in a timely manner can potentially lead to progressive renal failure, requiring dialysis or transplantation. We report the case of a 13-year old girl with neurogenic bladder who presented chronic renal failure secondary to lipomyelomeningocele with retethering of cord. She was managed with urinary indwelling catheterization until optimization of renal function and then underwent detethering of cord with excision and repair of residual lipomeningomyelocele. Her renal parameters improved gradually over weeks and then were managed on self clean intermittent catheterization. The case emphasizes the need for considering rethering of spinal cord in children with myelodysplasia where symptoms of neurogenic bladder and recurrent urinary tract infections occur. References 1. Favazza TF. Myelodysplasia and Neurogenic Bladder Dysfunction. 2014. Available at http://emedicine. medscape.com/article/1015695-overview (accessed 30th August 2016)2. Larijani FJ, Moghtaderi M, Hajizadeh N, Assadi F. Preventing Kidney Injury in Children with Neurogenic Bladder Dysfunction. Int J Prev Med. 2013;4(12):1359- 64.3. de Azevedo RV, Oliveira EA, Vasconcelos MM, et al. Impact of an interdisciplinary approach in children and adolescents with lower urinary tract dysfunction (LUTD). J Bras Nefrol. 2014;36(4):451-9.4. Elliott SP, Villar R, Duncan B. Bacteriuria management and urological evaluation of patients with spina bifida and neurogenic bladder: a multicenter survey. J Urol. 2005;173(1):217-20.5. Danforth TL, Ginsberg DA. Neurogenic lower urinary tract dysfunction: how, when, and with which patients do we use urodynamics? Urol Clin North Am. 2014;41(3): 445-52.6. Seki N, Masuda K, Kinukawa N, Senoh K, Naito S. Risk factors for febrile urinary tract infection in children with myelodysplasia treated by clean intermittent catheterization. Int J Urol. 2004;11(11):973-7.7. Kochakarn W, Ratana-Olarn K, Lertsithichai P, Roongreungsilp U. Follow-up of long-term treatment with clean intermittent catheterization for neurogenic bladder in children. Asian J Surg. 2004; 27(2):134-6.8. Obara K, Mizusawa T, Isahaya E, et al. Efficacy of Clean Intermittent Catheterization for Urinary Incontinence in Children with Neurogenic Bladder Dysfunction Secondary to Myelodysplasia. Low Urin Tract Symptoms. 2010;2(2):100-5

Congenital spinal dysraphism with infected sacrococcygeal sinus tract: need for improved awareness amongst clinicians

Spinal dysraphism (SD) includes a group of developmental anomalies resulting from failure of fusion of parts along dorsal aspect of midline structures lying along spinal axis from skin to vertebrae and spinal cord. There are two types of SD, open and closed. Close SD, also known as spina bifida occulta, can present with diagnostic challenges in resource limited settings where awareness regarding the condition and specialist radiological investigations, including Magnetic Resonance Imaging (MRI), may not be easily available. Undiagnosed cases can potentially lead to long term morbidities. We report the case of a 13-year old boy with closed SD presenting with recurrent infections of the sacrococcygeal sinus tract which were treated with oral antibiotics for what was considered to be localized infection. Following neurosurgical assessment and spinal MRI a diagnosis of SD was made. He underwent surgical excision of the sinus tract and closure of the defect with good outcome. The case emphasizes the need for awareness regarding SD in children who have sinus tracts in the intergluteal fold with symptoms of recurrent discharge and infection

Antibiotic resistant Shigella is a major cause of diarrhoea in the Highlands of Papua New Guinea

Introduction: Diarrhoea remains a major cause of illness in Papua New Guinea (PNG); however, little is known about its aetiology. As a result of the cholera outbreak that spread throughout PNG in 2009-2011, we conducted diarrhoeal surveillance in Eastern Highlands Province. Methodology: Following informed consent and a brief questionnaire, participants provided a stool sample or duplicate rectal swabs. Samples were tested for common bacterial pathogens Salmonella spp., Shigella spp., Vibrio spp., Campylobacter spp. and Yersinia enterocolitica using established culture methods. Enteric parasites were detected using microscopy. Results: A total of 216 participants were enrolled; where age was recorded, 42% were under 5 years of age, 6.7% were 5 to 17 years of age and 51.3% ≥18 years of age. One or more pathogens were detected in 68 (31.5%) participants, with Shigella (primarily S. flexneri) being the most commonly isolated (47 of 216 participants). Enteric parasites were detected in 23 of the 216 participants, occurring as a co-infection with another pathogen in 12 of 23 cases. No Vibrio cholerae was detected. Shigella isolates were commonly resistant to ampicillin, tetracycline, co-trimoxazole and chloramphenicol. Conclusions: Shigellae, specifically S. flexneri, are important pathogens in the highlands of PNG. While most studies in low-income settings focus on childhood aetiology, we have demonstrated the importance of Shigella in both children and adults. Enteric parasites remain present and presumably contribute to the burden of gastrointestinal illness. While improvements in sanitation and hygiene would help lower the burden of all aetiologies of infectious diarrhoea, additional control strategies targeting Shigella may also be warranted

Diarrhoeal disease surveillance in Papua New Guinea : findings and challenges

Diarrhoeal diseases are among the leading causes of morbidity and mortality in the Western Pacific Region. However, data on the major causes of infectious diarrhoea are limited in many countries within the Region, including Papua New Guinea. In 2013-2014, we conducted surveillance for acute diarrhoeal illness in four provinces in Papua New Guinea. One rural health clinic from each province participated in the surveillance activity. Samples were sent to central laboratories and batch analysed for bacterial and viral gastrointestinal pathogens that are commonly associated with diarrhoea. Across the four sites, the most commonly detected pathogens were Shigella spp., Campylobacter spp. and rotavirus. In this paper, we report the results of the surveillance activity and the challenges that we faced. The lessons learnt may be applicable to other parts of the Region with a similar socioeconomic status

Detection of enteric viral and bacterial pathogens associated with paediatric diarrhoea in Goroka, Papua New Guinea

Objectives: The aim of this study was to investigate the viral and bacterial causes of acute watery diarrhoea in hospitalized children in Papua New Guinea. Methods: A retrospective analysis was conducted on stool samples collected from 199 children (age < 5 years) admitted to the paediatric ward of Goroka General Hospital from August 2009 through November 2010. A large range of viral and bacterial enteric pathogens were targeted using real-time PCR/RT-PCR assays. Results: Young children were much more likely to be admitted with acute gastroenteritis, with 62.8% of patients aged <1 year and 88.4% aged <2 years. An enteric pathogen was detected in 69.8% (n = 138) of patients. The most commonly detected pathogens were Shigella spp (26.6%), rotavirus (25.6%), adenovirus types 40/41 (11.6%), enterotoxigenic Escherichia coli (11.1%), enteropathogenic E. coli (8.5%), norovirus G2 (6.0%), and Campylobacter spp (4.0%). Norovirus G1, sapovirus, and Salmonella spp were also detected, but below our statistical limit of detection. Vibrio cholerae and astrovirus were not detected in any patients. Mixed infections were detected in 22.1% of patients, with Shigella and rotavirus most commonly detected in co-infections with other pathogens. Conclusions: This study demonstrates that Shigella and rotavirus are the major pathogens associated with acute paediatric gastroenteritis in this setting

Quantifying the Importance of MSP1-19 as a Target of Growth-Inhibitory and Protective Antibodies against Plasmodium falciparum in Humans

BACKGROUND: Antibodies targeting blood stage antigens are important in protection against malaria, but the key targets and mechanisms of immunity are not well understood. Merozoite surface protein 1 (MSP1) is an abundant and essential protein. The C-terminal 19 kDa region (MSP1-19) is regarded as a promising vaccine candidate and may also be an important target of immunity. METHODOLOGY/FINDINGS: Growth inhibitory antibodies against asexual-stage parasites and IgG to recombinant MSP1-19 were measured in plasma samples from a longitudinal cohort of 206 children in Papua New Guinea. Differential inhibition by samples of mutant P. falciparum lines that expressed either the P. falciparum or P. chabaudi form of MSP1-19 were used to quantify MSP1-19 specific growth-inhibitory antibodies. The great majority of children had detectable IgG to MSP1-19, and high levels of IgG were significantly associated with a reduced risk of symptomatic P. falciparum malaria during the 6-month follow-up period. However, there was little evidence of PfMSP1-19 specific growth inhibition by plasma samples from children. Similar results were found when testing non-dialysed or dialysed plasma, or purified antibodies, or when measuring growth inhibition in flow cytometry or microscopy-based assays. Rabbit antisera generated by immunization with recombinant MSP1-19 demonstrated strong MSP1-19 specific growth-inhibitory activity, which appeared to be due to much higher antibody levels than human samples; antibody avidity was similar between rabbit antisera and human plasma. CONCLUSIONS/SIGNIFICANCE: These data suggest that MSP1-19 is not a major target of growth inhibitory antibodies and that the protective effects of antibodies to MSP1-19 are not due to growth inhibitory activity, but may instead be mediated by other mechanisms. Alternatively, antibodies to MSP1-19 may act as a marker of protective immunity

Spatio-temporal epidemiology of the cholera outbreak in Papua New Guinea, 2009-2011

Background: Cholera continues to be a devastating disease in many developing countries where inadequate safe water supply and poor sanitation facilitate spread. From July 2009 until late 2011 Papua New Guinea experienced the first outbreak of cholera recorded in the country, resulting in > 15,500 cases and > 500 deaths. Methods: Using the national cholera database, we analysed the spatio-temporal distribution and clustering of the Papua New Guinea cholera outbreak. The Kulldorff space-time permutation scan statistic, contained in the software package SatScan v9.2 was used to describe the first 8 weeks of the outbreak in Morobe Province before cholera cases spread throughout other regions of the country. Data were aggregated at the provincial level to describe the spread of the disease to other affected provinces. Results: Spatio-temporal and cluster analyses revealed that the outbreak was characterized by three distinct phases punctuated by explosive propagation of cases when the outbreak spread to a new region. The lack of road networks across most of Papua New Guinea is likely to have had a major influence on the slow spread of the disease during this outbreak. Conclusions: Identification of high risk areas and the likely mode of spread can guide government health authorities to formulate public health strategies to mitigate the spread of the disease through education campaigns, vaccination, increased surveillance in targeted areas and interventions to improve water, sanitation and hygiene