Charcot-Marie-Tooth Disease in a Child with Acute Lymphoblastic Leukaemia: A Case Report and Review of Literature

Background: Charcot-Marie-Tooth disease (CMT) is a common inherited neurologic disorder with various modes of inheritance. Existing peripheral neuropathy is a generally accepted risk factor for increased susceptibility to neurotoxic agents and there is a general acceptance of the concept of medication-induced worsening of CMT. Several authors have reported vincristine neurotoxicity in CMT and vincristine treatment triggering the expression of asymptomatic CMT disease. We report the case of a 10 year old male patient who developed severe neuropathy following treatment with vincristine for his Acute Lymphoblastic Leukaemia.Methods: The case records of the patient and a review of the relevant literature using available books, journals and online literature search was utilized.Results: Facial nerve palsy, increasing lower extremities muscle weakness and abnormal gait were noticed 4 weeks into vincristine therapy in a ten year old male on treatment for acute lymphoblastic leukaemia (ALL). On a suspicion of vincristine neurotoxicity, vincristine was excluded from his chemotherapy regimen. Although remission of ALL was achieved within 6 weeks of treatment, the patient's neurological symptoms did not improve even with the withdrawal of vincristine. The patient was diagnosed with Charcot-Marie-Tooth disease resulting from vincristine toxicity.Conclusion: Patients with Charcot-Marie-Tooth disease may show severe toxicities with vincristine. It is therefore recommended that an extensive neurologic examination should be conducted on any paediatric patient with a diagnosis of malignancy to identify any undiagnosed neurologic deficits and screen for suitability or otherwise of a vincristine containing cytotoxic therapy regimen. This case stresses the need for an urgent health sector response to provide adequate facilities for electrophysiological screening and genetic studies in resource limited centres like ours in order to identify possible cases of undiagnosed CMT and prevent the occurrence of exacerbation by vincristine administration

Discharge against medical advice amongst patients admitted into the Paediatric wards of the University of Port Harcourt Teaching Hospital

Objectives: To identify the characteristics of patients and factorscontributing to DAMA in paediatric patients admitted into the University of Port Harcourt Teaching Hospital and to determine waysto reduce its prevalence.Method: This was a retrospective study of children admitted into thepaediatric wards of the Hospital. Case files of affected patients over atwo year period were reviewed and relevant information obtained andanalyzed.Results: There were 150 of 3981 patients admitted over the periodgiving a prevalence rate of 3.8%. There were 86 (57.3%) males and64 females (42.7%) with a male: female ratio of 1.3:1. Neonatal sepsis,birth asphyxia and prematurity were the commonest diagnosis inneonates whilst severe malaria, meningitis and bronchopneumoniawere the commonest in other children. Most discharges (54%) occurredwithin the first week of admission. Majority of patients were from low (35.3%) and middle (41.3%) income classes. The commonestreasons for DAMA were lack of funds (26.6%) and no improvement(26.6%), necessitating need to seek alternative therapy. The discharge document was signed by the father in 61.3% of cases.Conclusion: Children who were DAMA suffered from commoncauses of childhood mortality. Lack of funds, no improvement and lack/low decision making power of mothers were contributory factors.There is need for more universal implementation of the NationalHealth Insurance Scheme; effective communication between medicalteam and parents and female empowerment to reduce rates ofDAMA in our environment

Nutritional status of day care attendees in Port Harcourt metropolis

Objective: To determine the nutritional status of children aged 0-35 months attending day care centres in Port Harcourt metropolis.Methodology: A cross-sectional descriptive survey approved by the University of Port Harcourt Research Ethics Committee, the parents, Authorities of Day care centres and National Association of Proprietors of Private Schools, was carried out between November 2011 and July 2012 in 200 day care centres in Port Harcourt metropolis. The centres were selected using stratified multistage random sampling technique. At each day care 10 children (5 males and 5 females) selected randomly from those who met the inclusion criteria were recruited. With parental cooperation, each child's socio-demographic data, nutritional history and anthropometric measurements were obtained. Data were analysed using SPSS version 20. Statistical significance was set at p<0.05.Results: Complete data for analysis were available for 1541 children aged 5-34months (mean 23.78 ± 7.04 months, median 25 months and modal 24 months) with 110(7.2%) children aged less than 12 months and 283(18%) less than 18 months. They consisted of 766 (49.7%) males and 775 (50.3%) females. Most children had normal nutritional status using the various indices but 46 (3%) children were underweight, 85 (5.5%) stunted, 109 (7.1%) wasted and 328 (21.3%) overweight. The mean Mid Upper Arm Circumference (MUAC) was 15.95 ± 1.698 cm with 29 (2%) children having MUAC below 115mm.Conclusion: Majority of the day care attendees were well nourished. However, the high prevalence of overweight malnutrition is of serious concern in view of its associated long term morbidity and the need for effective interventions to reduce this risk.Key Words: day care centres, under-fives, malnutrition, overweight, children

Mothers Perception of Sexuality Education for Children

Sexuality education is the process of acquiring information and forming attitudes and beliefs about sex, sexual identity, relationships and intimacy. It develops young people's skills so that they make informed choices about their behaviour, and feel confident and competent about acting on these choices. It also equips children to face developmental challenges and empowers them against the ills of abuse, exploitation, unwanted pregnancies amongst others. Mothers who are the primary caregivers should be well informed about sexuality issues. The objective of the study is to determine mothers' perception of sexuality education in children, in Port Harcourt.A structured, anonymous and selfadministered questionnaire, used as instrument for data collection, was distributed amongst a convenient sample of women attending a Christian women's convention in Port Harcourt.One hundred and fifty eight women participated in the study. Most of them were married (80.4%), and belonged to the 30-49 years age bracket. Seventy one (44.9%) of the respondents had tertiary education. Over 80% agreed that children needed sexuality education but only 15 women (9.5%) had a good knowledge of the concept of sexuality education. One hundred and eleven (70.2%) believed it was the responsibility of both parents to educate their children and over 70% acknowledged that the home was the best place for such education. 64 (40.5%) believed that 6-10 years was the ideal age for starting sex education while 49% thought that the ideal age was 11-15 years. 65% of respondents discussed sexuality issues with their children at least occasionally,the content mostly involved description of body parts and reproductive organs. The average age of menarche amongst respondents was 14.0. One hundred (63%) of the women had prior knowledge of menstruation before menarche. About half of them had received information from their mothers.  The study highlights the need for enlightenment of women on sexuality education to enable them empower their children against the ills of child abuse.

Perception of Rape Amongst Secondary School Students In Port Harcourt

Background: Rape in children is a significant public health problem and represents the least reported but most devastating major form of child abuse all over the world. It occurs when one person does not fully and freely consent to sexual intercourse. This study seeks to evaluate the perception of rape among secondary school students in Port Harcourt.Methods: This study was conducted among secondary school students during a series of debates organised by the Medical Women Association of Nigeria in Port Harcourt. Using a structured, anonymous and self-administered questionnaire, data collected included bio data, awareness information, response and practices.Results: One thousand and fifty students, 486 (46.3 %) males and 564 (53.7 %) females participated in the study. Their ages varied between 10 and 20 years with a median age of 15. 872 (83%) knew the general definition of rape but only 4% knew that intercourse between an adult and a minor is also rape. 412 (39.3%) believed that only persons between 16 and 19 years could be raped. 442 (42.1%) believed that anyone, both relatives and strangers could be the perpetrator. Only 138 (13.2%) knew that rape should be reported to the police. 48(4.6%) of them had been raped, of which 62.5% were by close acquaintances.Conclusion: The study shows that respondents had a fair general knowledge about rape but the perception of age at risk and actions to take in case of rape is poor. There is therefore need for  awareness/enlightenment campaigns about rape targeted at secondary  school students.Key words: rape, secondary schools, student

Feeding pattern of children in day care centres in Port Harcourt metropolis

Objective: To determine the feeding pattern of day care attendees aged 0-35 months in Port Harcourt metropolis.Methodology : Following appropriate approvals, a pilot-tested questionnaire was used to obtain the socio-demographic data and information on the feeding pattern of 10 day care attendees aged 0- 35 months who met the study criteria selected from each of 200 schools using a stratified multistage random sampling technique. Data entry and analyses utilised SPSS version 20 and statistical significance was set at p<0.05.Results: Between November, 2011 and July, 2012, 1541 children comprised of 766 (49.7%) males and 775 (50.3%) females were studied. They were aged 5- 34months (mean 23.78 ± 7.04 months, median 25 months and modal 24 months) with 110(7.2%) children aged less than 12 months and 283(18%) aged less than 18 months. Although 102(6.6%) children were still breastfeeding, none of the 21 (1.4%) children aged less than 6 months was being exclusively breastfed. Complementary feeding commenced at age 6- 8 months in 747(48.5%) children, before 6 months in 613(39.8%) and after 9 months in 181(11.7%) children. The ages at starting day care attendance which ranged from 1-25 months with 56.3% children starting before 11 months of age statistically significantly influenced the ages at which complementary feeding commenced (χ2= 53.431, df = 8, p = 0.000). The main foods fed to day care attendees while at day care centres were adult diet for 1152 (74.8%) children and breast milk and/or cereals for 389 (25.2%). Daily feeding frequencies while at the centre which ranged between 1-6 times and decreased with the child’s age an the age was statistically significantly affected by the child’s age, the feeding frequency (p = 0.000).Conclusion: The feeding pattern of these day care attendees did not comply with the recommendations of the National Infant and Young Child Feeding Guidelines. This situation at a national level may contribute to the high prevalence of under-five malnutrition and calls for strategies to correct these deficiencies.Key words: Feeding pattern, Day care attendees, Day care centres

Dipstick Urinalysis as a Screening Tool in the Diagnosis of Urinary Tract Infection in Children.

Urinary tract infection (UTI), a significant but preventable cause of morbidity in children, is an important risk factor for development of renal insufficiency. Having simple and reliable means of screening children facilitate its prompt treatment.To evaluate the use of dipstick test for leukocyte esterase (LE) and nitrite as screening tool in the diagnosis of UTI in children.This cross-sectional study was done in the department of Paediatrics, University of Port Harcourt Teaching Hospital (UPTH) from February to October 2005. Children 2 to 15 years with symptoms suggestive of UTI were studied. Midstream urine specimen was collected from each patient forLEtest, nitrite test and culture.Out of 139 children, 72(51.8 percent) males and 67(48.2 percent) females studied, 33(23.7 percent) had positive urine culture. The prevalence of UTI was significantly higher in females than in males (p=0.001). The LE dipstick test correctly identified 17 of the 33 patients with culture proven UTI, with 51.5 percent sensitivity and 79.2 percent specificity. The nitrite dipstick test showed 42.4 percent sensitivity and 93.3 percent specificity. The combination ofLEand nitrite correctly identified 24, giving 73 percent sensitivity and 75.5 percent specificity. The LE seemed to have a higher sensitivity as a screening tool in the diagnosis of UTI than nitrite, but the difference was not statistically significant (p=0.46). However, the combination of LE and nitrite provided a higher sensitivity than either of them with a statistically significant difference (p=0.04).Key words: Dipstick urinalysis, Screening too

Wilms tumour: Experience at a tertiary centre in the Niger Delta region of Nigeria

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Teaching of essential maternal and newborn interventions: how skilled are the trainers from health service provider training institutions in Port Harcourt, Rivers State?

Background: Maternal and neonatal indices in Nigeria and other developing countries have remained poor, with poor access to health care and most deaths occurring at home. This necessitated the development of several low-cost, evidence-based essential interventions such as the Home Based Care for Mothers and Newborns (HBCMN) package by United Nations Children's Fund, of which 90% coverage could prevent up to 90% of these deaths. Therefore, achieving a meaningful reduction of maternal and perinatal mortality would require capacity building of health workers and their trainers.Aim: To present the impact of the short course on HBCMN on the skills of health worker's trainers.Methods: Teachers from five health institutions attended the course.  Pre- and post-tests were administered to assess the knowledge they acquired from the training. Results were analysed using percentages and the level of significance was set at P<0.05. Results: Thirty-seven participants registered and took the pre-test, 26 (70.3%) completed the training. In the pre-test, 96% of participants could name at least 2 danger signs in a pregnant woman and 84.6% in the newborn. Few participants knew the importance and timing of early postnatal visits. Twenty-four (92.3%) knew about early initiation, frequency and duration of exclusive breastfeeding; but none knew the management of sore nipples. Four (15.3%) participants knew about the Kangaroo Mother Care. These deficiencies significantly improved during the post-test.Conclusion: Trainers of health workers require regular updating of their knowledge to improve the quality of their training. Keywords: Essential interventions, Maternal, Newborn, Skills, Trainers, HBCMN packag