Abnormal Lower Extremity Hemodynamics at Doppler US in Children with Sickle Cell Anemia.

Background Children with sickle cell anemia (SCA) are predisposed to developing leg ulcers in early adolescence; however, the underlying physiologic mechanisms are not well understood, which hinders the development of prophylactic antiulcer interventions. Purpose To determine if occlusive arterial disease or abnormal hemodynamics exist in the lower limb peripheral arteries of children with SCA. Materials and Methods This was a prospective cross-sectional study conducted between July 2020 and May 2021. Two groups of participants were enrolled: children with SCA and healthy controls. Each child had their anthropometric measurements taken, followed by B-mode, color Doppler, and spectral Doppler US evaluation of the distal anterior and posterior tibial arteries. Ankle-brachial index evaluation was also performed. Hemodynamic values were compared between children with SCA and healthy controls, with statistical significance set at the P < .05 level. Results Forty-five children with SCA (mean age, 9.5 years ± 3.7 [SD]; 26 boys) and 45 healthy controls (mean age, 9.3 years ± 3.8; 25 boys) were evaluated. Children with SCA had higher peak systolic velocities, end-diastolic velocities, and blood volume flow in their distal anterior and posterior tibial arteries compared with the healthy participants (P < .001 across all 12 measurements). Children with SCA also had lower resistive index and pulsatility index values compared with healthy controls in at least one of the anterior and posterior tibial arteries bilaterally. Monophasic spectral patterns were exclusively observed in participants with SCA. The ankle-brachial index values for the participants with SCA (mean, 1.09 ± 0.08) and healthy participants (mean, 1.06 ± 0.02) were within normal range. Conclusion Abnormal peripheral hemodynamic characteristics were found in the lower limbs of children with sickle cell anemia. No evidence of occlusive peripheral artery disease was present. © RSNA, 2022 Online supplemental material is available for this article. See also the editorial by Paltiel in this issue.

Early experience with laparoscopic surgery in children in Ile-Ife, Nigeria.

BACKGROUND: Laparoscopy is not yet routinely employed in many Paediatric Surgical Units in Nigeria despite the advantages it offers. This study describes the preliminary experience with laparoscopic procedures in a single centre. PATIENTS AND METHODS: A retrospective analysis of all children who had laparoscopic surgery between January 2009 and December 2013 at the Paediatric Surgical Unit of Obafemi Awolowo University Teaching Hospitals Complex Ile-Ife was carried out. Their sociodemographic, preoperative and intraoperative data along with postoperative records were subjected to descriptive analysis. RESULTS: Eleven (44%) diagnostic and 14 (56%) therapeutic procedures were performed on 25 children whose age ranged from 5 months to 15 years (Median: 84 months, Mean: 103 ± 64.1 months), including eight (32%) females and 17 (68%) males. Indications included acute appendicitis in 12 (48%), intra-abdominal masses in six (24%), three (12%) disorders of sexual differentiation, two (8%) ventriculoperitoneal shunt malfunctions and impalpable undescended testes in two (8%) children. The procedures lasted 15-90 minutes (Mean = 54 (±21.6) minutes). Conversion rate was 17% for two patients who had ruptured retrocaecal appendices. No intra operative complications were recorded while three (12%) patients had superficial port site infections post-operatively. All diagnostic (11) and two therapeutic procedures were done as day case surgery. The mean duration of hospital stay was 3.1 (±3.3) days for those who had appendectomies. CONCLUSION: Laparoscopic surgery in children is safe and feasible in our hospital. We advocate increased use of laparoscopy in paediatric surgical practice in Nigeria and similar developing settings.

Early experience with laparoscopic surgery in children in Ile-Ife, Nigeria

Background: Laparoscopy is not yet routinely employed in many Paediatric Surgical Units in Nigeria despite the advantages it offers. This study describes the preliminary experience with laparoscopic procedures in a single centre. Patients and Methods: A retrospective analysis of all children who had laparoscopic surgery between January 2009 and December 2013 at the Paediatric Surgical Unit of Obafemi Awolowo University Teaching Hospitals Complex Ile-Ife was carried out. Their sociodemographic, preoperative and intraoperative data along with postoperative records were subjected to descriptive analysis. Results: Eleven (44%) diagnostic and 14 (56%) therapeutic procedures were performed on 25 children whose age ranged from 5 months to 15 years (Median: 84 months, Mean: 103 ± 64.1 months), including eight (32%) females and 17 (68%) males. Indications included acute appendicitis in 12 (48%), intra-abdominal masses in six (24%), three (12%) disorders of sexual differentiation, two (8%) ventriculoperitoneal shunt malfunctions and impalpable undescended testes in two (8%) children. The procedures lasted 15-90 minutes (Mean = 54 (±21.6) minutes). Conversion rate was 17% for two patients who had ruptured retrocaecal appendices. No intra operative complications were recorded while three (12%) patients had superficial port site infections post-operatively. All diagnostic (11) and two therapeutic procedures were done as day case surgery. The mean duration of hospital stay was 3.1 (±3.3) days for those who had appendectomies. Conclusion: Laparoscopic surgery in children is safe and feasible in our hospital. We advocate increased use of laparoscopy in paediatric surgical practice in Nigeria and similar developing settings

Epidemiology and clinicopathologic outcome of pediatric chronic kidney disease in Nigeria, a single cenetr study.

INTRODUCTION: Due to dearth of data, chronic kidney disease (CKD) outcome in African children has been dismal owing to poor understanding of its etiology, manifestations and management. METHODS: We retrospectively analyzed the records of 154 CKD children and adolescents who were managed at Obafemi Awolowo University Teaching Hospitals Complex between 2000 and 2009 to evaluate the epidemiology and clinicopathologic outcome of pediatric CKD in Nigeria. RESULTS: Overall mean incidence was 11 (6-20) per million children population (pmcp)/year while prevalence averaged 48 (8-101) pmcp. There were 86 males (55.8%). Median age was 10.0 (0.2-15.5) years with 83.8%≥5 years old. Etiologies were glomerular disease (GMD, 90.26%), congenital and acquired urinary tract (7.79%) and hereditary disorders (1.95%). CKD stages at diagnosis were 45.5% CKD-1, 22.7% CKD-2, 10.4% CKD-3, 2.6% CKD-4 and 18.8% CKD-5. Median progression time through the CKD stages was 24.0 (3-108) months. Mean dialysis incidence and prevalence were 1 (0-4) pmcp/year and 4 (1-12) pmcp, respectively. Hypertension, heart failure (HF), malnutrition, anemia, acute-on-CKD, need for dialysis, azotemia, hypercreatininemia, and high calcium-phosphorous product (≥55 mg2/dL2) were mortality risk factors. CKD-1 survived significantly better than CKD stages 3-5 (p<0.05) but not CKD-2 (p=0.1). Hypertensive CKDs without HF survived better (73.0%) than hypertensive CKDs with HF (16.0%) [Hazard ratio (HR): 0.34, 95% CI: 0.14-0.83]. GMD survived better (68.5%) than non-GMD patients (33.0%) [HR: 2.87, 95% CI: 1.16-7.06]. CONCLUSION: CKD was commoner among school than pre-school age children. GMD was the predominant etiology with better outcome than non-GMD. Comorbidity prevalence increased significantly with increasing severity of CKD stage.

Hospital-acquired acute kidney injury in critically ill children and adolescents.

This study determined the (1) hospital incidence, prevalence and etiology; (2) frequency of each of the acute kidney injury (AKI) stages and (3) the 60-day outcome. Retrospective analysis of clinico-laboratory data of Nigerian children/adolescents with hospital-acquired acute kidney injury (hAKI) was performed. AKI occurred in 103 (3.13%) of 3,286 childhood and adolescent admissions. Twenty-eight (27.2%) were hAKI while 72.8% were community-acquired AKI (cAKI). Annual hAKI incidence and prevalence rates were 0.17% (or 3.7 per million children population [pmcp]/year) and 0.84% (or 18.3 pmcp), respectively. Male (20):female (8) ratio was 2.5:1. In the hAKI group, median age was 5 (0.063-15.0) years. AKI stages 1, 2 and 3 accounted for 14.3%, 25.0% and 60.7%, respectively. AKI stage 3 was most anuric, with high dialysis requirement (P = 0.0329). Nephrotoxics (42.87%) were a leading cause of hAKI. Seventy-five percent of the recorded deaths were in the first 28 hAKI days. Median survival time was 23.5 admission (11-52) days. The means values of maximum serum creatinine (Scr) for survivors (486.0 ± 382.0 µmol/L or 5.5 ± 4.3 mg/dL) and for non-survivors (353.0 ± 160.0 µmol/L or 4.0 ± 1.8 mg/dL) were similar (P > 0.20). The 60-day cumulative mortality was 36.7%. Scr severity may not be a reliable mortality determinant among AKI patients. The maximal mortality in the first 28 days of hAKI onset and overall high mortality rate indicate that high level of clinical vigilance and informed therapeutic intervention will be critical to survival during this period. Cause of death was multi-factorial.

Aminophylline improves urine flow rates but not survival in childhood oliguric/anuric acute kidney injury.

INTRODUCTION: Acute kidney injury (AKI) morbidity and mortality rates remain high. Variable AKI outcomes have been reported in association with aminophylline treatment. This study evaluated AKI outcome in a group of Nigerian children treated with aminophylline. METHODS: This is a retrospective study of AKI in children treated with (N=9) and without (N=8) aminophylline. Studied outcome indices comprised urine flow rate (UFR), duration of oliguria/anuria, progression through AKI stages, number of patients requiring dialysis and mortality. RESULTS: Mean ages for the control and aminophylline arms were 4.6±2.7 and 4.9±2.1 years (P=0.7), respectively. All patients progressed to stage-3 AKI. Baseline median UFRs in the aminophylline and control arms were similar (0.13 Vs 0.04 ml/kg/hour respectively, P=0.5). The median UFR was significantly higher on day-5 (0.8 Vs 0.1; P=0.03), day-6 (1.0 Vs 0.2; P=0.02), and day-7 (1.2 Vs 0.2; P=0.03) in the aminophylline than the control arm, respectively. Short duration of oliguria/anuria (≤ 6 days) was more frequently observed in aminophylline- treated patients compared to controls (77.8% Vs 25.0%; odds ratio 0.09; 95% CI: 0.01-0.89; P=0.04). Only the aminophylline group maintained steady serum creatinine levels. Four out of five patients in the control group were dialyzed compared to only one out of eight patients in the aminophylline group (odds ratio 0.16; 95% CI: 0.04-0.71; P=0.03). Mortality rates were similar in aminophylline- treated and control patients (33% Vs 25%; hazard ratio 0.8; 95% CI: 0.1-5.5; P=0.8). CONCLUSION: Aminophylline therapy was beneficial for patients with AKI in terms of improved UFR and reduced need for dialysis, but failed to impact positively on survival.

Prolonged febrile illness due to CTX-M-15 extended-spectrum ß-lactamase-producing Klebsiella pneumoniae infection in Nigeria.

We report on an 8-year-old patient with septicaemia unresponsive to therapy for five weeks. Undetected, extended-spectrum ß-lactamase (ESBL) production by the infecting Klebsiella strain was regarded as responsible for treatment failure. Intravenously administered imipenem during the sixth week led to sustained resolution of fever. Resource-limited hospitals can incur prohibitive costs from ESBL-producer infections because of diagnostic limitations and consequent treatment failure involving prolonged supportive therapy.

Childhood idiopathic steroid resistant nephrotic syndrome in Southwestern Nigeria.

Clinical charts of 23 Nigerian children diagnosed with idiopathic steroid resistant nephrotic syndrome (iSRNS) between January 2001 and December 2007 were retrospectively reviewed to determine their clinicopathologic characteristics and outcome. iSRNS (54.8%) was primary in 19 patients (83%) and secondary in four (17%). The mean age at diagnosis was 8.3 ± 3.5 years (2.1-13 years). Histopathology revealed membranoproliferative glomerulonephritis (MPGN) in 43.5%, focal and segmental glomerulosclerosis (FSGS) in 39.1% and mesangial proliferative glomerulonephritis in 8.7% of the patients while minimal change disease (MCD) and membranous nephropathy accounted for 4.35% each. Routine treatment protocol comprised pulse intravenous (i.v.) cylophosphamide infusion and i.v. dexamethasone lisinopril or spironolactone. Cumulative Complete Remission (CR) rate was 57.12%. The overall median time to CR from start of steroid sparing agents in 12/21 treated patients was 4.5 weeks. CR was better achieved in MPGN than FSGS (P = 0.0186). Five patients had eight relapses with the overall median relapse-free duration being four months. Cumulative renal survival at 36 months was 41.8%. The median follow-up duration was eight months. Our study revealed that there was a high prevalence of iSRNS and preponderance of non-MCD lesions, with MPGN and FSGS being the major morphologic lesions. The outcome with steroid and cyclophosphamide-based treatment for iSRNS was further enhanced with addition of either lisinopril or spironolactone.

Quartan malaria-associated childhood nephrotic syndrome: now a rare clinical entity in malaria endemic Nigeria.

BACKGROUND: The study determined (i) whether or not quartan malaria nephropathy (QMN) is still a major cause of childhood nephrotic syndrome (CNS) in Nigeria, (ii) secondary causes other than QMN and their associated glomerular pathology and (iii) renal and patient outcome. METHODS: The study was a prospective non-randomized study of consecutive cases of secondary CNS. Patients with idiopathic CNS were excluded. RESULTS: Twenty-four of 78 (30.8%) CNS cases were of secondary aetiology. Overall mean ages at onset of secondary CNS aetiology and CNS onset were 8.97 +/- 3.59 (1-15.3) and 9.95 +/- 3.15 (5-15.3) years, respectively. Male (14)/female (10) ratio was 1.4. Secondary causes comprised systemic lupus erythematosus (SLE, 37.5%), sickle cell anaemia (SCA, 16.7%), hepatitis B virus (HBV, 16.7%) infection, Churg-Strauss syndrome (12.6%), SLE/human immunodeficiency virus infection (4.2%), rhabdomyosarcoma (4.2%), bee stings (4.2%) and Addison's disease (4.2%). The overall cumulative complete remission (CR) rate was 88.0%. Remission was sustained in 11 of 16 (68.8%) CR patients, while one patient (6.25%) relapsed; the remaining four patients (24.95%) were yet to attain sustained remission. Median relapse-free period was 10.5 (0.75-25) months. Cumulative renal survival was 75.2% at 3 years. Three patients were lost to follow-up, while two died. Overall cumulative patient survival probability at 36 months was 90.8%. All patients were followed for a median period of 12.5 (0.11-36.0) months. CONCLUSION: Overall outcome of CNS has improved significantly compared to the 1960s and 1970s when the poor outcome of QMN was the predominant glomerular lesion in Nigeria. While quartan malaria-associated nephrotic syndrome has become a rare clinical entity, SLE, SCA and HBV infection have become the major secondary aetiologies of CNS in Nigeria.

Focal segmental glomerulosclerosis and nephrotic syndrome in a child with embryonal rhabdomyosarcoma.

A case of nephrotic syndrome (NS) and acute renal failure (ARF) associated with embryonal rhabdomyosarcoma (RMS) in a 10-year-old boy is reported. Ultrasound revealed irregular, echogenic, circumferential urinary bladder base mass, bilateral hydroureter and hydronephrosis. Histopathology of percutaneous renal and urethrocystoscopic biopsy specimens, respectively, revealed focal segmental glomerulosclerosis (FSGS) and embryonal RMS. Tumour remission was induced with pulse doses of intravenous vincristine, cyclophosphamide, methotrexate and actinomycin D over a 15-month period. He has been followed-up for 28 months and has maintained a drug-free tumour and proteinuria remission for 1 year. While some malignancies have been reported in association with NS, its occurrence in association with RMS is quite exceptional. We conclude that RMS may be associated with FSGS and NS. Effective treatment of the RMS was associated with sustained remission of the nephrotic proteinuria.