Background: Pulmonary artery hypertension (PAH) is a known complication of patients with sickle cell disease (SCD). The prevalence of PAH has been reported to be high in children with SCD in developed countries. The mortality rate of patients with SCD who develop PAH is said to be 40% at 24 to 40 months after diagnosis. In Sub-Saharan Africa, particularly Nigeria, where the prevalence of SCD is high, there is a dearth of data on the prevalence of PAH in children with SCD. PAH may be a likely contributor to the unacceptably high mortality rate of children with SCD in Africa. The present study aimed to determine the pattern of pulmonary artery pressure in children with sickle cell anaemia (SCA) aged 1 to 12 years in their steady state using Doppler echocardiography in our environment. Methods: Analytical, comparative and cross-sectional study. It was carried out at Lagos State University Teaching Hospital (LASUTH) over a period of seven months (31st August 2015 through 31st March 2016). A total of 400 children, aged 1 to 12 years were recruited following parental consent and assent. Two hundred children with SCA in steady state and 200 age and sex matched children with haemoglobin genotype AA who served as controls were studied. All subjects and controls had Doppler echocardiography performed on them by the author. PAH was defined as a pulmonary artery systolic pressure (PASP) of ≥30 mmHg at rest derived from the tricuspid regurgitant velocity (TRV) measured from Doppler echocardiography. This corresponds with TRV value of ≥2.5 m/s. Results: The prevalence of PAH in children with SCA was 8% (n=16). None of the controls had PAH. The youngest subject with PAH was aged 2 years. The overall mean pulmonary artery pressure of children with SCA was significantly higher than that of controls (18.54±5.81 vs. 13.76±5.71 mmHg, P=0.000). The prevalence of PAH in children with SCA demonstrated an increase in trend with age. Conclusions: The prevalence of PAH in children with SCA in steady state is high. This complication was noticed as early as the second year of life. It is recommended that all children with SCA should have periodic Doppler echocardiography for early detection of PAH.

Background The word scleroderma means ‘hard skin’ that develops due to excessive accumulation of collagen. It is the third most frequent rheumatic disease in paediatric rheumatology after juvenile idiopathic arthritis and systemic lupus erythematosus. When it occurs in individual <16 years, it is called juvenile scleroderma. It is a rare disease that occurs in one per million children, documented to be more common in African adults with poorer survival states compared with Caucasians. Objectives To describe the clinical and laboratory characteristics of children with juvenile scleroderma seen in our clinic, thus, increasing its awareness. Methods Retrospective review of records of three children diagnosed with juvenile scleroderma at the paediatric Rheumatology Clinic of Lagos State University Teaching Hospital(LASUTH) between May 2018 to April 2022. Results Case 1 A 12-year-old girl presented with skin tightness of the left hand and thigh of one year, contracture of the 3rd, 4th and 5th proximal interphalangeal joints, arthritis of the left wrist, skin induration of the dorsum of the left hand involving the 4th and 5th finger and extending to the wrist and skin and induration of the anterolateral aspect of the left thigh. Her blood tests showed an erythrocyte sedimentation rate (ESR) at 20 mm/h, an ANA titre of 1:2560, a negative anti-Scl 70/anti-centromere antibodies, a normal complete blood count and serum electrolytes/urea/creatinine. A diagnosis of linear scleroderma was made, the patient had prednisolone, methotrexate and folic acid, in addition to topical emollients. She improved clinically as observed during follow up visits six weeks after initiation of treatment but later defaulted from the clinic due to unknown reason. Case 2 A 4-year-old girl presented with constitutional symptoms, swollen hands and feet, sclerodactyly, narrowing of oral aperture, ulcers at tips of the fingers, inflammatory pain of the large joints, hypopigmented macules on the face, trunk, abdomen and back and also abnormal capillaroscopy. Erythrocyte sedimentation rate was 22 mm/h with normal levels of electrolytes/urea/creatinine, thrombocytosis, ANA titre of 1:640 and negative anti-centromere and anti-U1RNP antibodies. A diagnosis of diffuse systemic sclerosis was made. She started prednisolone, methotrexate, nifedipine and omeprazole and was asked to do an ECG, an Echocardiogram, a spirometry and a chest HRCT but she couldn’t afford to do these investigations due to severe financial constraints. She was clinically stable for four months until she presented at the emergency room with sudden loss of consciousness and congestive cardiac failure. She died during resuscitation attempt. Case 3 An 11-year-old girl known patient of haematology unit with sickle cell anaemia, presented with inflammatory arthritis of the small joints of the hands, elbows and knees of nine-month duration, sclerodactyly, contractures of the PIP of the fingers, narrowing of oral aperture, generalized hypopigmented macules and abnormal nailfold capillaroscopy. Investigation showed an ANA titre of 1:640, positive anti-Scl 70 antibodies, negative anti-centromere antibodies, ESR of 130 mm/h and thrombocytosis. The echocardiogram showed a normally structured heart with severe restriction on spirometry and features of interstitial lung disease on HRCT of the chest. She started mycophenolate mofetil, prednisolone and nifedipine. She later received 2 doses of rituximab due to slow clinical improvement. She is being followed up. Conclusion Most data on scleroderma are from adult studies, we reported these three cases due to the rare occurrence of scleroderma in children, thus increasing its awareness.