Congenital heart disease in the Niger Delta region of Nigeria: a four-year prospective echocardiographic analysis.

INTRODUCTION: Echocardiographic evaluation remains the gold standard for the diagnosis of structural cardiac disease. No previous prospective studies have been done on the prevalence of congenital heart disease (CHD) in the Niger Delta area. This study was done to determine the frequency and pattern of congenital heart disease, using echocardiography as a diagnostic tool. METHODS: All patients presenting to the Paediatric Cardiology clinics of two centres, the University of Port Harcourt Teaching Hospital and the Paediatric Care Hospital between April 2009 and March 2013, were recruited and all had echocardiography performed. RESULTS: Prevalence of CHD in this study was 14.4 per 1 000 children; 277 (83.4%) of the patients had acyanotic CHD and 55 (16.6%) had cyanotic CHD. Ventricular septal defect and tetralogy of Fallot were the commonest acyanotic and cyanotic heart defects, respectively. CONCLUSION: The high prevalence of CHD in this study is the highest in the country and Africa, and may be attributable to the increased oil spillage and gas flaring from petroleum exploitation in this region.

Congenital Heart Disease in the Niger Delta region of Nigeria : a Four-Year Prospective Echocardiographic Analysis : Cardiovascular Topic

Introduction : Echocardiographic evaluation remains the gold standard for the diagnosis of structural cardiac disease. No previous prospective studies have been done on the prevalence of congenital heart disease (CHD) in the Niger Delta area. This study was done to determine the frequency and pattern of congenital heart disease; using echocardiography as a diagnostic tool. Methods : All patients presenting to the Paediatric Cardiology clinics of two centres; the University of Port Harcourt Teaching Hospital and the Paediatric Care Hospital between April 2009 and March 2013; were recruited and all had echocardiography performed.Results : Prevalence of CHD in this study was 14.4 per 1 000 children; 277 (83.4) of the patients had acyanotic CHD and 55 (16.6) had cyanotic CHD. Ventricular septal defect and tetralogy of Fallot were the commonest acyanotic and cyanotic heart defects; respectively.Conclusion : The high prevalence of CHD in this study is the highest in the country and Africa; and may be attributable to the increased oil spillage and gas flaring from petroleum exploitation in this region

Echocardiography findings in clinically confirmed congenital rubella syndrome cases seen at the University of Port Harcourt Teaching Hospital, Nigeria.

BACKGROUND: Congenital rubella syndrome (CRS) is known to affect thousands of children in the developing world because rubella vaccination is not available routinely in most of these countries. Among its many manifestations only congenital heart disease is life threatening. This study was undertaken to ascertain the cases of echocardiographic determined congenital heart disease in clinically confirmed CRS cases. METHODS: Data of patients with clinically confirmed CRS seen over a period of 5 years in the Paediatric cardiology clinic of the University of Port Harcourt Teaching Hospital was retrieved and analysed. RESULTS: Seven cases (2.8 % of total cardiac cases) were seen. 6 (85.7%) cases had at least one murmur on auscultation. Patent ductus arteriosus was the commonest cardiac defect seen either in isolation or incombination with a VSD or ASD. Only one child had no cardiac defect. 4 (57.1%) of them had been admitted for heart failure at least once and 2 (28.6 %) were on anti-failure regimen, one of whom had cardiac surgery one month ago. CONCLUSION: In view of the fact that 6 (85.7%) of the patients with CRS had at least one congenital heart defect, we advocate routine echocardiography on patients with CRS to ensure early treatment and reduce mortality and morbidity.We also advocate that rubella vaccination be given routinely in developing countries.

Clinical profile of patients with tetralogy of Fallot admitted for surgery at a cardiac surgical centre.

INTRODUCTION: Tetralogy of Fallot (TOF), a conotruncal defect, has been documented to be associated with chromosome abnormalities, single gene syndrome (22q11 microdeletion), known teratogens, with the rest associations being multifactorial. This study was carried out to determine the clinical profile and associated risk factors in patients with TOF admitted for surgical repairs. METHODS: Case files of all patients admitted for Tetralogy of Fallot over a period of one year were retrieved from the Medical Records Department and reviewed. Data on the patients' and their family history and associated cardiac anomalies were noted. RESULTS: There were 54 patients, 37 males and 17 females, with a mean age of 6.8 years +/- 7.1. Sixty percent were born between July and December, 81.5% as full term and 44% as first born. Twenty-six percent were born into consanguineous marriages. Five patients had dysmorphic features. Associated cardiac anomalies included right aortic arch, pulmonary atresia, dextrocardia and left superior vena cavae. CONCLUSION: The associated risk factors noted in this study were male sex, birthdates between July and December, first born and increased paternal age. Other risk factors were consanguinity and specific patterns of cardiovascular diseases associated with 22q 11 deletions. This suggests a multifactorial etiology for TOF.

Type 2 diabetes mellitus in a Nigerian child: a case report.

BACKGROUND: Type 2 diabetes mellitus initially said to be an adult disease is now reported in children and adolescents in the developed countries because of increased incidence of obesity and sedentary habits associated with westernization and lifestyle changes. There is a paucity of reports from Africa. METHOD: A 9 year old overweight female with a BMI of 28 kg/m(2) and a strong family history of DM in at least two generations presented with polyuria and weight loss. The mother had gestational diabetes and is on oral hypoglycaemics. Fasting blood sugar was 11.9 mmol/l. Urinalysis had +1 of glucose, no ketones. She was managed with diet control and exercise. RESULT: The patient has remained euglycaemic in the past two months without drugs and is losing weight. CONCLUSION: Type 2 diabetes mellitus is being reported in an obese Nigerian child with a family history of DM and high socio-economic class. Routine screening of overweight children with a family history of DM is recommended.

Growth pattern of exclusively breastfed infants in the first six months of life: a study of babies delivered at the University of Port Harcourt Teaching Hospital, Rivers State, Nigeria.

BACKGROUND: Some authors demonstrate that exclusively breastfed infants have growth patterns comparable with the NCHS standards while others conclude that exclusively breastfed infants grow slower. These conflicting results informed the need for this study to aid the paediatricians and health workers to justify or condemn the recommendation of exclusive breastfeeding for six months. This was a prospective longitudinal study carried out to determine the growth pattern of exclusively breastfed infants in the first 6 months of life and compare them with the International, National Centre for Health Statistics (NCHS/WHO) reference and the Local, Janes' Elite Standards. METHOD: Using weight, length and occipito-frontal circumference as indices, 530 infants were recruited consecutively by convenience sampling over 17 months. Measurements were taken at birth, 2 weeks, 6 weeks, 2 months and subsequently monthly until the end of the 6th month. RESULTS: The exclusively breastfed infants doubled their birth weights at 3 months. The mean weight, OFC and length gains were maximal at 2 months, 2 weeks and 2 months respectively The growth of exclusively breastfed infants compared favourably with the NCHS/WHO reference group and also compared favourably with the Janes' 'Elite' reference group. CONCLUSION: It is concluded that the growth in weight, length and OFC of exclusively breastfed infants appears adequate for the first 6 months of life.

Growth pattern of exclusively breastfed infants in the first six months of life: a study of babies delivered at the University of Port Harcourt Teaching Hospital, Rivers State, Nigeria

BACKGROUND: Some authors demonstrate that exclusively breastfed infants have growth patterns comparable with the NCHS standards while others conclude that exclusively breastfed infants grow slower. These conflicting results informed the need for this study to aid the paediatricians and health workers to justify or condemn the recommendation of exclusive breastfeeding for six months. This was a prospective longitudinal study carried out to determine the growth pattern of exclusively breastfed infants in the first 6 months of life and compare them with the International, National Centre for Health Statistics (NCHS/WHO) reference and the Local, Janes' Elite Standards. METHODS:Using weight, length and occipito-frontal circumference as indices, 530 infants were recruited consecutively by convenience sampling over 17 months. Measurements were taken at birth, 2 weeks, 6 weeks, 2 months and subsequently monthly until the end of the 6th month.RESULTS: The exclusively breastfed infants doubled their birth weights at 3 months. The mean weight, OFC and length gains were maximal at 2 months, 2 weeks and 2 months respectively. The growth of exclusively breastfed infants compared favourably with the NCHS/WHO reference group and also compared favourably with the Janes' 'Elite' reference group. CONCLUSION:It is concluded that the growth in weight, length and OFC of exclusively breastfed infants appears adequate for the first 6 months of life

The Profile of Non-Communicable Disease in Patients Admitted into the Children's Medical Ward of the University of Port Harcourt Teaching Hospital

Background: The prevalence of non-communicable diseases (NCD) is increasing in recent years in low income countries in sub-Saharan Africa because of changing disease patterns following socioeconomic development. Nevertheless; communicable diseases (CD) still remain the predominant health problem. At present; non-communicable diseases are not a high priority in sub-Saharan Africa but the probability of death from a NCD is higher in sub-Saharan Africa than in the developed world. It is therefore important to know the existing disease burden due to NCD with a view to alerting policy makers and health workers of the trend of disease in our environment. Objectives: To determine the pattern of admission of paediatric patients seen at the children's ward of the University of Port Harcourt Teaching Hospital (UPTH) and ascertain the pattern of non communi- cable diseases. Methods: The study was retrospective and involved analysis of data from the admission records of the children's wards of the UPTH from February 2004-November 2005. Patients with non-communicable diseases were analysed for this study. Results: A total of two thousand four hundred and fourteen (2414) children were admitted during the period of study. Out of these; 479 (19.8) had non-communicable diseases consisting of 279 (58.2) males and 200 (41.8) females with a male: female ratio of 1.4:1. The top five non-communicable diseases were sickle cell disease (SCD) (17.1); malignancies (14.8); renal diseases (12.9); tetanus (10.2) and malnutrition (10.0). Conclusion: This study suggests a concomitant rise in NCD with four of them being among the top 10 disease burden when combined with communicable diseases. This poses a risk of a 'double burden' of disease which we cannot afford in our country which is fraught with poor government policies; poverty and poor funding of the health sector. Effective strategies are needed to control the risk factors for NCD

Down syndrome in one of non-identical Nigerian twins: a case report.

BACKGROUND: Down syndrome (DS) or Trisomy 21 is the most frequent and best known Trisomy in humans. Mothers under 25 years of age are known to have the average risk of a DS pregnancy of 1:1600, rising to 1:350 at 35 and 1:40 at age 43. Twining in DS occurs at a rate of 1.2% of pregnancies with only 1/6th of both of the pairs having Trisomy 21. METHOD: A 4-week-old male, second twin, was admitted in the University of Port Harcourt Teaching Hospital (UPTH) due to fast breathing from birth, cough and poor weight gain. He was very pale, (haematocrit 18%), dyspnoeic and had mongoloid features. There were coarse crepitations in lung fields, a systolic murmur and an enlarged liver The diagnoses were Down syndrome, bronchopneumonia and congenital heart disease with failure. The mother was aged 22 years and the twin sister was normal. RESULT: He was promptly treated with oxygen, diuretics, and antibiotics but died within three hours of admission before blood transfusion could be offered. CONCLUSION: Down syndrome in twin pregnancy is very rare and, to our knowledge none has been reported in Nigeria. That this was a product of first pregnancy in a lady as young as 22 years makes an interesting reading.

Priapism in Paediatric Patients with Sickle Cell Disease - A Report of Three Cases at University of Port Harcourt Teaching Hospital

Background: Priapism is an abnormal persistent erection of the penis which may be painful or not painful. Approximately two-thirds of all paediatric patients who have priapism also have sickle cell disease (SCD). The aim of this study is to review all the cases of priapism admitted into the paediatric emergency room of the University of Port Harcourt Teaching Hospital (UPTH) over a 5-year period and to determine the prevalence; presentation; precipitating factors and management of cases seen.Method: The admission register in the Children's emergency ward was reviewed from January 2001 to December 2005. All the cases of sickle cell disease were extracted. The folders of the patients with priapism were studied and data analysed.Results: Of the 185 SCD cases; three (1.6) had priapism. They were adolescents aged 17years; 11years and 10 years 9months respectively. Two patients had never attended a sickle cell clinic; never been on routine drugs nor received advice on oral liberal fluids intake. One patient had stuttering priapism; 24hours before the event. Two of them had glanulo-cavernous shunts; with early detumescence on Day 3 and 4 respectively; while the patient who was managed conservatively had detumescence after 12 days.Conclusion: Priapism is an uncommon presentation of SCD in our environment. With prolonged duration; priapism has severe sequelae of impotence and erectile dysfunction; thus; its presentation and management should be known by patients and parents or care givers. Surgical intervention is recommended as it is more effective and results in shorter hospital stay

Kartagener syndrome: an unusual cause of respiratory distress in the newborn.

BACKGROUND: Kartagener Syndrome (KS) a rare genetic disorder belongs to a group of disorders referred to as primary ciliary dyskinesia (PCD) where the cilia covering the respiratory epithelium is either immotile or beat in an uncoordinated fashion. It is characterized by a triad of dextrocardia (with or without situs inversus), chronic sinusitis and bronchiectasis as a result of poor mucociliary clearance of mucus and bacteria. This may lead to respiratory distress in the newborn period. METHOD: This is a case report of a 14-day old male who presented with respiratory distress (which was noticed soon afterbirth) and features suggestive of KS. CONCLUSION: KS should be suspected in neonates presenting with respiratory distress, pneumonia and no risk factors for infection.

Confirmed congenital rubella syndrome--A case report.

BACKGROUND: Congenital Rubella Syndrome (CRS) is defined clinically as an illness usually manifesting in infancy, resulting from rubella infection in utero with certain specific signs and symptoms. Confirmed CRS is a clinically consistent case which is laboratory confirmed. A report of a 3 month old male diagnosed with confirmed congenital rubella is here presented on account of its rarity and easy prevention with vaccination. METHOD: The case notes of 3 month old male admitted with features consistent with CRS and managed for overwhelming septicaemia with heart failure and encephalitis and review of literature on the subject using manual library and Medline search. RESULT: The infant's Rubella-specific immunoglobulin M (IgM) antibody demonstrated positive and the rubella antibody level was high at 11.6 IU/ml. CONCLUSION: This is the first report of confirmed CRS in our centre. Though few cases are seen, the effects and defects on the child are severe and irreversible thus, vaccination with Measles-mump-rubella (MMR) vaccine is recommended as part of the National Programme on Immunization and for all females of child bearing age, who did not receive it in childhood.

Suspected epidermolysis bullosa simplex in a 5-week-old Nigerian: a case report.

BACKGROUND: Epidermolysis bullosa is a rare non scaring autosomal dominant disorder characterized by recurrent blistering of the skin and mucous membrane. The skin is fragile and minor rubbing may cause blistering. It's epidemiology in our environment is unknown probably because of paucity of information on the clinical presentation and management with resultant mortality within the first few months of life. METHOD: A case report of a 5-week-old female who presented with generalized blistering and denudation of the skin first noticed on the left foot at birth and a review of the literature on the subject using Medline and online search was used. She was treated at various traditional medicine homes and clinics before referral to the teaching hospital. She was managed initially for bullous pemphigus with antibiotics for proven septicaemia, and the wound infection ignorantly managed with daily sofratulle dressing alternating with closed dressings before a definitive clinical diagnosis of was made. RESULT: The patient was referred late to the tertiary centre. She was initially treated for bullous pemphigus and sepsis with antibiotics and wound dressings with poor response before the diagnosis of epidermlysis bullosa was made. The patient died from severe foot bleeding before blood could be transfused. CONCLUSION: Delay in making the right diagnosis hence the appropriate treatment even in a tertiary health is being highlighted.

Missed diagnosis of amoebic liver abscess: a case for forward clinical reasoning.

BACKGROUND: Increasingly, a wrong diagnosis is made and wrong investigations and treatment ordered, because the doctor uses one or two symptoms to jump into a premature conclusion, without consideration of the totality of a patient's presentation. This is not forward clinical reasoning. Forward clinical reasoning is based on a systematic approach to patient's problems. The objective of this paper is to use a real case to illustrate the pitfall in ignoring forward clinical reasoning and how forward clinical reasoning serves the physician's and patient's interest better. METHOD/RESULT: The case of a 3 year old who had amoebic liver abscess but was wrongly diagnosed and treated for lobar Pneumonia with pleural effusion is highlighted. The sequence for forward clinical reasoning is used to show how the right diagnosis could have been arrived at. CONCLUSION: The correct diagnosis following sequential forward clinical reasoning saves time, money and life.

Fatal relapse of hyperreactive malarial splenomegaly (HMS) in a 10-year old Nigerian female--a case report.

BACKGROUND: The Hyperreactive Malarial Splenomegaly Syndrome (HMS) originally called the tropical splenomegaly syndrome (TSS) or Big spleen disease refers to cases of splenomegaly in the tropics for which no cause was found despite thorough investigation. It is restricted to the malarial belt, yet there are few reports on HMS in Nigeria, probably due to a low index of suspicion and non-availability of laboratory facilities to determine titres of malarial antibodies. The objective of this paper is to highlight the features, management, risk of relapse and prognosis of HMS. METHOD/RESULT: We present a 10-year old female with recurrent massive splenomegaly with previous clinical response to antimalarials and evidence of hypersplenism. CONCLUSION: HMS should be suspected in any child with moderate to massive splenomegaly with evidence of hypersplenism and clinical response to antimalarials.

Growth pattern of exclusively breastfed infants in the first six months of life: a study of babies delivered at the University of Port Harcourt Teaching Hospital, Rivers State, Nigeria.

BACKGROUND: Some authors demonstrate that exclusively breastfed infants have growth patterns comparable with the NCHS standards while others conclude that exclusively breastfed infants grow slower. These conflicting results informed the need for this study to aid the paediatricians and health workers to justify or condemn the recommendation of exclusive breastfeeding for six months. This was a prospective longitudinal study carried out to determine the growth pattern of exclusively breastfed infants in the first 6 months of life and compare them with the International, National Centre for Health Statistics (NCHS/WHO) reference and the Local, Janes' Elite Standards. METHODS: Using weight, length and occipito-frontal circumference as indices, 530 infants were recruited consecutively by convenience sampling over 17 months. Measurements were taken at birth, 2 weeks, 6 weeks, 2 months and subsequently monthly until the end of the 6th month. RESULTS: The exclusively breastfed infants doubled their birth weights at 3 months. The mean weight, OFC and length gains were maximal at 2 months, 2 weeks and 2 months respectively. The growth of exclusively breastfed infants compared favourably with the NCHS/WHO reference group and also compared favourably with the Janes' 'Elite' reference group. CONCLUSION: It is concluded that the growth in weight, length and OFC of exclusively breastfed infants appears adequate for the first 6 months of life.

Hookworm infestation in a 3-month old female.

BACKGROUND: Intestinal helminthiasis is a major cause of morbidity and mortality in infants and children particularly in the tropics and subtropics. This report highlights the possibility of hookworm infestation in infancy. METHOD: A case report of hookworm infestation in a three-month old infant who was managed in the University of Port Harcourt Teaching Hospital, Port Harcourt in May 2001 for failure to thrive and recurrent severe anaemia. RESULT: The patient was admitted in the children's emergency ward with passage of dark watery stools, fever, excessive crying and severe anemia and was transfused twice. Stool microscopy revealed numerous ova of hookworm and she was treated with albendazole. Three days after administration of anti-helminthic, stools became formed with normal colour and temperature was normal. She gained weight before discharge home. CONCLUSION: Hookworm infestation should be suspected as a cause of severe anaemia in infants in communities with a high risk of infestation such as fishing port communities. To the best of my knowledge, symptomatic hookworm infestation in the first year of life has not been previously documented in Nigeria.