Pattern of congenital heart defects in children with Down syndrome at the University of Port Harcourt Teaching Hospital, Port Harcourt

Down syndrome (DS) is the most common chromosomal abnormality in man and congenital heart defects (CHD) the most life threatening of its manifestations. The cardiac anomalies and early pulmonary hypertension are associated with high morbidity and mortality. It is thus important to diagnose and intervene early to improve the quality of life. Few studies have been done on Down syndrome patients in Nigeria. Objective: The objective was to find out the incidence and types of congenital heart defects in Down syndrome patients seen in the Paediatric cardiology unit of this tertiary institution. Method: A prospective study of Down Syndrome patients seen at the Paediatric Cardiology clinic of the University of Port Harcourt Teaching Hospital over a three year period was carried out. Data of age, sex, birth order, mother’s age, indication for surgery, clinical and echocardiographic findings and outcome was analysed. Result: Of the 31 patients who had echocardiography done, all had cardiac defects (100%). Patent ductus arteriosus, occurring solitarily or in combinations was the commonest cardiac defect seen followed by ventricular septal defects. Interestingly, over 60 % of the patients had multiple cardiac defects with VSD/PDA, VSD/ASD accounting for 50%. Conclusion: The incidence of multiple cardiac defects in more than 60% of DS patients seen underscores the need for early diagnosis and intervention. Routine extensive cardiac evaluation in  the first few weeks of life is advocated.Key word: Down syndrome, congenital heart defects, Port Harcourt, Nigeria

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 22years 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 22years makes an interesting reading. Nigerian Journal of Medicine Vol. 16 (1) 2007: pp. 74-7

Epidemiology of childhood heart failure in Port Harcourt, Nigeria

Background: Heart failure (HF) is a clinical syndrome that complicates a variety of childhood illnesses. Recent studies in various parts of Nigeria show a prevalence rate of 5.8-15.5%. There is relatively scant data on heart failure in children in the south-south geo-political region of the country, necessitating this study.Aim: To determine the epidemiology of HF in the University of Port Harcourt Teaching HospitalMethods: A prospective study of consecutive children presenting with three of the four cardinal signs of HF (tachycardia, tachypnoea, tender hepatomegaly and cardiomegaly), over one year. Data sought included age, sex, underlying cause of HF and outcome.Results: There were 1152 children admitted into the Children Emergency Ward (CHEW) during the period; 75 met the criteria for HF giving a prevalence rate of 6.5%. There were 39(52.0%) males and 36(48.0%) females. Their ages ranged from 1month to 11years. Common acute causes of HF were pneumonias 32(42.7%) and severe anaemia secondary to severe malaria 27(36.0%). Thirteen patients had underlying chronic diseases such as congenital heart diseases in 11(14.7%), sickle cell anaemia in 1(1.3%), and renal disease in 1(1.3%). Hospital stay ranged from 1 to 15 days. Six mortalities were recorded.Conclusion: HF is a common complication of the emerging double burden of communicable and non-communicable childhood diseases in Nigeria and a potential contributor to mortality in these diseases. An up-scaling and sustenance of effective infectious disease control measures and screening and early detection of chronic diseases is necessary to avoid complications and preventable death in Nigerian children. Keywords: Heart failure, Childhood, Epidemiology, Port Harcour

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 9year old overweight female with a BMI of 28kg/m2 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 11.9mmol/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

Acute rheumatic fever in a child with Ebstein anomaly

Background: Ebstein anomaly (atrialisation of the right ventricle ) alone constitutes 0.5 -1% of congenital heart disease and usually presents as an isolated lesion. Association with mitral valve stenosis has been reported previously. We however present a case of Ebstein's anomaly with recurrent acute rheumatic fever (ARF) and mitral and aortic valve disease.Case report: An 8-year-old girl with Ebstein anomaly presented to a Cardiac hospital in  India with fever, polyarthritis and a recent history of sore throat. General examination showed subcutaneous nodules and investigations revealed leucocytosis, elevated erythrocyte sedimentation rate (ESR) and antistreptolysin O (ASO) titre. Echocardiography confirmed Ebstein anomaly with mitral and aortic regurgitation. A diagnosis of recurrent ARF was made based on the carditis, arthritis, subcutaneous nodules and evidence of recent streptococcal infection and a history of recurrence of symptoms in the past year. She was promptly commenced on oral penicillin, steroids, secondary prophylaxis with benzathine penicillin and aspirin. She responded to complete bed rest and these therapy; symptoms abated before discharge 7 days later. She was doing well on monthly follow-up in the past 4 months.Conclusion: This report supports the need to investigate non cardiac symptoms such as arthritis in children with existing congenital heart disease because co-morbidity with ARF can exacerbate the symptoms, signs and prognosis of a congenital heart disease, if left untreated.  Keywords: Ebstein  anomaly, Acute rheumatic fever, Recurrenc

Fatal relapse of Hyperreactive Malarial Splenomegaly (HMS) in a 10-year old Nigerian female: A case report

No Abstract. Nigerian Journal of Medicine Vol. 14(4) October-December 2005: 447-44

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

No Abstract. Nigerian Journal of Medicine Vol 15 (3) July-September: 344-34

Confirmed congenital rubella syndrome: A case report

No Abstract. Nigerian Journal of Medicine Vol. 15 (4) October-December 2006: 448-45

Hookworm infestation in a 3-month old female

No Abstract. Nigerian Journal of Medicine Vol. 14(2) 2005: 227-23

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 of hospital, River State, Nigeria

No Abstract. Nigerian Journal of Medicine Vol. 14(2) 2005: 137-14