Priapism in paediatric patients with sickle cell disease - a report of three cases at the University of Port Harcourt Teaching Hospital

2007 ◽  
Vol 2 (1) ◽  
Author(s):  
E B Otaigbe ◽  
L Yagoe-Ide
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Cell Disease ◽  
Paediatric Patients ◽  
Port Harcourt ◽  
The University

scholarly journals Prenatal Diagnosis in Sickle Cell Disease: In the Eyes of the Couple at Risk

2020 ◽  
pp. 65-71
Author(s):  
Okechukwu Chioma
Keyword(s):  
At Risk ◽  
Prenatal Diagnosis ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Health Care Professionals ◽  
Cell Disease ◽  
Hospital Data ◽  
Port Harcourt ◽  
Knowledge And Attitude ◽  
The University

Background: Prenatal diagnosis of sickle cell disorders provides a couple at risk the opportunity to make informed decisions whether or not to birth a child with Sickle Cell Disease (SCD). Aim: To explore the knowledge of prenatal diagnosis and its acceptability among parents at risk of having another child with SCD Methods: A self-administered structured questionnaire was used to obtain information on socio-demographics, knowledge and attitude towards prenatal diagnosis. The respondents were parents of SCD patients seen at the haematology consultant paediatric clinic of the University of Port Harcourt Teaching hospital. Data were analysed using SPSS version 20.0. Results: Forty-six parents were interviewed and they were all Christians. Thirty-two (69.6%) were females and fourteen (30.4%) were males. All the respondents were from the south-south region of Nigeria. Four (8.7%) had lost children from complications of SCD. Twenty-four (52.2%), had heard about prenatal diagnosis of sickle cell disease while twelve (50%) of them heard about it from health care professionals. Four (8.7%) had done prenatal diagnosis in previous pregnancies while 32 (69.6%) were willing to do it in their next pregnancy. The most common reasons given for not doing prenatal diagnosis were religious beliefs, personal beliefs and fear of the procedure. Conclusion: There is still a gap in knowledge and utilization of prenatal diagnosis by at risk couples. Appropriate information and regular counselling should be given to at risk parents as a key method of preventing SCD.

scholarly journals Sonographic Diagnosis and Clinical Correlates of Gallbladder Stones in Patients with Sickle Cell Disease in Calabar, Nigeria

2019 ◽  
Vol 7 (1) ◽  
pp. 68-72 ◽  
Author(s):  
Grace B. Inah ◽  
Emmanuel E. Ekanem
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Abdominal Ultrasound ◽  
Cell Disease ◽  
Clinical Correlates ◽  
Gallbladder Stones ◽  
Study Population ◽  
A Prospective Study ◽  
The University

BACKGROUND: Sickle Cell Disease (SCD) causes chronic haemolysis which is a risk factor for cholelithiasis. AIM: To determine the prevalence and clinical correlates of cholelithiasis in SCD patients in steady state treated at the University of Calabar Teaching Hospital. METHODS: This was a prospective study that took place at the Haematology and sickle cell disease clinics, University of Calabar Teaching Hospital, Calabar, Nigeria between January and June 2018. The study population were aged between 1.5-5.5 years and confirmed to have SCD through haemoglobin electrophoresis. A brief history was obtained, and all the patients had a physical examination. Ultrasound examination was performed using a B-mode mind-ray ultrasound machine using a 3.5-5.0 MHz probe after an overnight fast. A Calculus is diagnosed when a highly echogenic structure casting a concrete shadow is detected in the lumen of the gallbladder. RESULTS: One hundred and twenty confirmed SCD patients aged between 1.5-55 years were recruited in the study, 69 (57.5%) were males, while 51 (42.5%) were females. The overall prevalence of cholelithiasis was 10%, and it increased with age. The youngest patient with cholelithiasis was 13 years old. All the patients were asymptomatic at the time of examination. At the multivariate level, age, gender, weight and gallbladder volume were associated with gallbladder stones. CONCLUSION: The prevalence of cholelithiasis in patients treated at the Sickle Cell Clinic at the University of Calabar Teaching Hospital, Calabar is fairly high. The patients were largely asymptomatic, and cholelithiasis is more common in females than males. This study showed a weak association between blood transfusion and gallbladder stone. It is recommended that routine abdominal ultrasound scan for gallbladder be done for SCD patients from the second decade of life in our environment.

scholarly journals Pneumococcal Vaccination Status of Children with Sickle Cell Disease in Port Harcourt, Nigeria

2017 ◽  
Vol 2 (1) ◽  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pneumococcal Vaccination ◽  
Immunization Coverage ◽  
Vaccination Rate ◽  
Cell Disease ◽  
Pneumococcal Infections ◽  
Cross Sectional ◽  
Port Harcourt ◽  
The University

Background: The sickle gene confers an increased vulnerability to infection, particularly to certain bacterial pathogens, and this has significantly contributed to morbidity and mortality in individuals with sickle cell disease. Immunization plays an important role in the prevention of infection in children with sickle cell disease; we sought to assess the pneumococcal vaccination rate of children with this disease in Port Harcourt, Nigeria. Materials and Methods: This was a cross-sectional questionnaire based study of children with sickle cell disease presenting in the Sickle Cell Clinic of the University of Port Harcourt Teaching Hospital, Nigeria from January 2013 to December 2013. Results: There were 181 children with sickle cell disease. One hundred and seventy nine (98.9%) of them were SS genotype while 2(1.1%) children had SC genotype. Immunization coverage according to the National Programme on Immunization was 172 (95.1 %). The rates for other specific vaccines, range from 102(66.2%) for the antiHaemophilus influenza vaccine in the form of pentavalent vaccine to 10(5.5%) for the pneumococci. The major reasons for non-vaccination of pneumococcal were unavailable in Government Health Facility, ignorance, lack of proper education on the need of the vaccine and the outrageous cost of these vaccines for the families who knew about them. Conclusion: The immunization coverage for pneumococcal infections among children with sickle cell disease is poor. These results propose the need for a national program against sickle cell disease, which should license health facilities to include pneumococcal vaccine in their routine vaccination program.

scholarly journals Predictors of musculoskeletal manifestations in paediatric patients presenting with sickle cell disease at a tertiary teaching hospital in Lusaka, Zambia

2020 ◽  
Vol 1 (6) ◽  
pp. 175-181
Author(s):  
Raymond Mpanjilwa Musowoya ◽  
Patrick Kaonga ◽  
Alick Bwanga ◽  
Catherine Chunda-Lyoka ◽  
Christopher Lavy ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Clinical Manifestations ◽  
Classification Systems ◽  
University Teaching ◽  
Cell Disease ◽  
Tertiary Teaching ◽  
Musculoskeletal Manifestations

Aims Sickle cell disease (SCD) is an autosomal recessive inherited condition that presents with a number of clinical manifestations that include musculoskeletal manifestations (MM). MM may present differently in different individuals and settings and the predictors are not well known. Herein, we aimed at determining the predictors of MM in patients with SCD at the University Teaching Hospital, Lusaka, Zambia. Methods An unmatched case-control study was conducted between January and May 2019 in children below the age of 16 years. In all, 57 cases and 114 controls were obtained by systematic sampling method. A structured questionnaire was used to collect data. The different MM were identified, staged, and classified according to the Standard Orthopaedic Classification Systems using radiological and laboratory investigations. The data was entered in Epidata version 3.1 and exported to STATA 15 for analysis. Multiple logistic regression was used to determine predictors and predictive margins were used to determine the probability of MM. Results The cases were older median age 9.5 (interquartile range (IQR) 7 to 12) years compared to controls 7 (IQR 4 to 11) years; p = 0.003. After multivariate logistic regression, increase in age (adjusted odds ratio (AOR) = 1.2, 95% confidence interval (CI) 1.04 to 1.45; p = 0.043), increase in the frequency of vaso-occlusive crisis (VOC) (AOR = 1.3, 95% CI 1.09 to 1.52; p = 0.009) and increase in percentage of haemoglobin S (HbS) (AOR = 1.18, 95% CI 1.09 to 1.29; p < 0.001) were significant predictors of MM. Predictive margins showed that for a 16-year-old the average probability of having MM would be 51 percentage points higher than that of a two-year-old. Conclusion Increase in age, frequency of VOC, and an increase in the percentage of HbS were significant predictors of MM. These predictors maybe useful to clinicians in determining children who are at risk. Cite this article: Bone Joint Open 2020;1-6:175–181.

Gross hematuria in sickle cell disease at Tokoin teaching hospital in Lomé (Togo)

2015 ◽  
Vol 25 (4) ◽  
pp. 432-433
Author(s):  
K.M. Guedenon ◽  
A.D. Gbadoe ◽  
N.B. Nouwakpo
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Teaching Hospital ◽  
Cell Disease ◽  
Gross Hematuria

scholarly journals P10 An audit to assess the prescribing of analgesia in children who present with pain crisis due to Sickle Cell Disease (SCD)

2020 ◽  
Vol 105 (9) ◽  
pp. e11.1-e11
Author(s):  
Masuma Dhanji
Keyword(s):  
Sickle Cell Disease ◽  
Integrated Care ◽  
Sickle Cell ◽  
Care Pathway ◽  
Electronic Prescribing ◽  
List Type ◽  
Cell Disease ◽  
Paediatric Patients ◽  
Pain Crisis ◽  
Integrated Care Pathway

AimTo assess the prescribing of analgesia to manage pain crises in children with SCD. This was to establish whether the Trust was meeting national and local standards. Prompt pain control is essential to reduce length of stay and further complications.1Standards100% of admissions will be prescribed regular paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs) at the recommended frequency unless contraindicated in accordance with national guidance.2 3100% of admissions will be prescribed appropriate doses of analgesia with consideration to weight and age in accordance with local policy.4MethodThe audit was registered with the Trust’s audit committee. A list of paediatric patients with the diagnosis of SCD was sought from paediatricians with an interest in haematology. A data collection form was created. Data was collected retrospectively over a one-year period. A total of 60 admissions were reviewed to check whether analgesia was prescribed regularly at the recommended frequency, and at the correct dose. Results were analysed using descriptive statistical analysis. Exclusion criteria included patients with hospital admissions under 24 hours.ResultsA total of 55 admissions were included in the final sample. The audit showed the Trust was non-adherent to both standards assessed. A total of 45% (95% CI [31.9%, 58.1%]) of admissions were prescribed regular analgesia. A total of 78% (95% CI [67.9%, 88.9%] of admissions were prescribed appropriate doses of analgesia. Two main reasons were found as to why analgesia was prescribed at the incorrect dose. This was due to incorrect weights recorded on the electronic system (n=4) and doses based on age only (n=8).ConclusionThe results show prescribers are familiar with the correct doses of analgesia but fail to prescribe analgesia regularly. This highlights an opportunity for education and training in the management of pain crisis in SCD. One recommendation includes development of an integrated care pathway booklet for paediatric patients presenting with pain crisis due to SCD. Integrated care pathway booklets have been implemented for other conditions such as cystic fibrosis yielding positive outcomes. The results have highlighted key issues surrounding the electronic prescribing system such as out-of-date weights remaining on the system unless updated, and default treatment protocols. The electronic prescribing system requires refinement for use within paediatrics. One suggestion includes compulsory weight field on admission. Limitations of this audit included small sample size. There was a lack of data to make suggestions based on different ages.ReferencesRees D, Olujohungbe A, Parker N, et al. Guidelines for the management of the acute painful crises in sickle cell disease. Br J Haemato 2003;120:744–752.National Institute for Health and Care Excellence (2012) Sickle cell disease: managing acute painful episodes in hospital. NICE Guideline (CG143).Paediatric Formulary Committee. BNF for Children (2018–2019). London: BMJ Group, Pharmaceutical Press, and RCPCH Publications; (2018).General Hospital (2015) Management of sickle cell disease in paediatric patients (CG377).

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