Relationship between rickets and incomplete distal renal tubular acidosis in children

<p>Abstract</p> <p>Background</p> <p>In the Sub Saharan Africa Rickets has now been established to be due primarily to calcium deficiency and sometimes in combination with vitamin D deficiency. The main thrust of management is calcium supplementation with or without vitamin D. An observation was made that some children with nutritional rickets do not respond to this management modality. The recently reported high prevalence of Incomplete Distal Renal Tubular Acidosis (idRTA) in adults with osteoporosis as brought to fore the possibility of this being a possible cause of calcium wastage and therefore the poor response in these group of children with rickets.</p> <p>Aim</p> <p>To determine the prevalence of idRTA amongst a cohort of subjects with rickets</p> <p>To show a relationship between rickets and incomplete distal renal acidosis</p> <p>To determine the response of children with rickets and idRTA to addition of Shohl's solution to therapy</p> <p>Methodology</p> <p>Two separate cohorts of children with rickets performed the ammonium chloride loading test to detect those with incomplete renal tubular acidosis. Following identification for idRTA, Shohl's solution was added to therapy of calcium and vitamin D supplementation and their response compared to those without idRTA on calcium and vitamin D supplementation solely.</p> <p>Results</p> <p>50 children with rickets aged from two to six years of age and composed of 29 females and 21males were investigated. Incomplete renal tubular acidosis was found in 38% of them. Prevalence of idRTA was highest amongst those aged 3-6 years of age. Those with idRTA had worse limb deformities, biochemical and radiological parameters than those who hadn't. Rate of response on those with idRTA treated with Shohl's solution was at par with those without idRTA.</p> <p>Conclusion</p> <p>Incomplete idRTA exist amongst children with rickets and should be looked out for in severe rickets and older children. Treatment of idRTA will lead to optimal response and healing of rickets.</p

A National Survey of Resources to Address Sepsis in Children in Tertiary Care Centers in Nigeria.

Background: Infections leading to sepsis are major contributors to mortality and morbidity in children world-wide. Determining the capacity of pediatric hospitals in Nigeria to manage sepsis establishes an important baseline for quality-improvement interventions and resource allocations. Objectives: To assess the availability and functionality of resources and manpower for early detection and prompt management of sepsis in children at tertiary pediatric centers in Nigeria. Methods: This was an online survey of tertiary pediatric hospitals in Nigeria using a modified survey tool designed by the World Federation of Pediatric Intensive and Critical Care Societies (WFPICCS). The survey addressed all aspects of pediatric sepsis identification, management, barriers and readiness. Results: While majority of the hospitals 97% (28/29) reported having adequate triage systems, only 60% (16/27) follow some form of guideline for sepsis management. There was no consensus national guideline for management of pediatric sepsis. Over 50% of the respondents identified deficit in parental education, poor access to healthcare services, failure to diagnose sepsis at referring institutions, lack of medical equipment and lack of a definitive protocol for managing pediatric sepsis, as significant barriers. Conclusions: Certain sepsis-related interventions were reportedly widespread, however, there is no standardized sepsis protocol, and majority of the hospitals do not have pediatric intensive care units (PICU). These findings could guide quality improvement measures at institutional level, and healthcare policy/spending at the national level

The Scourge of Generalized Post-neonatal Tetanus in Children in Lagos: A Review of the Prevalence and Case Fatality Rate

Background: While global efforts are focused on eradicating neonatal tetanus, reports from various regions in Nigeria suggest that post-neonatal tetanus remains ab major health problem in children.Objective: In this study the cases and outcome of postneonatal tetanus in a tertiary hospital in Lagos, Nigeria were reported.Methods: The clinical records of children older than one month with generalized tetanus admitted between July 2010 and June 2013 were reviewed. Further analysisincluded determination of factors associated with inhospital mortality.Results: Of 6898 children older than one month admitted during the period, 46 (0.67%) had generalized tetanus equalling 6.7 cases per 1000 children. The records of three children could not be retrieved and were excluded. The mean (SD) age of the children with tetanus was 8.9 (3.3) years; 6 (14%) were =5 years while 21 (48.8%) children were aged 8-10 years. Females made up 51.2% of the cohort. The portal of entry was the lower limb and ear in 24 and 5 children respectively. At least one dose of diphtheriapertussis-tetanus (DPT) vaccine was received in infancy by 23 (53.5%) children. Twenty-three (53.5%) children had severe tetanus. Thirteen (30.2%) children died. The children who survived spent 15 (7.8-20.0) days in hospital. Factors associated with death were onset interval =24 hours, temperature =380C and severe tetanus.Conclusion: Post-neonatal tetanus remains a common childhood problem in Nigeria. A third of children with tetanus die. Severe tetanus, fever or a short onset interval increases the likelihood of death. There is a need to scale up routine childhood immunization in Nigeria as well as revise DPT vaccine schedule to include booster doses in older children.Key words: booster doses of DPT vaccine, routine childhood immunization, DPT vaccine, post-neonatal tetanu

Upper gastrointestinal endoscopy in children: The Lagos University Teaching Hospital experience

Background. Paediatric endoscopy is now standard care in the developed world for the management of gastrointestinal (GI) disorders. However, in developing countries endoscopy remains an underutilised tool. Objective. To determine the indications and the spectrum of endoscopic findings in children seen at the Lagos University Teaching Hospital, Nigeria. Methods. The indications for upper GI endoscopy and endoscopic findings in children ≤16 years old, referred for the procedure from June 2013 to June 2016, were documented. The endoscopic yield in these children was also determined. Results. In total 71 children were referred for upper GI endoscopy during the study period. There were 35 boys and 36 girls aged 3 months to 16 years. The indications for upper endoscopy were recurrent abdominal pain in 37 (52.1%), upper GI bleeding in 17 (23.9%), recurrent vomiting in 7 (9.9%), dyspepsia in 5 (7.0), heartburn in 2 (2.8%), dysphagia in 1 (1.4), portal hypertension in 1 (1.4) and ingestion of corrosives in 1 (1.4%) of the subjects. Endoscopic findings were as follows: gastritis 19 (26.8%), hiatus hernia in 13 (18.3%), gastric erosions in 12 (16.9%), oesophageal varices 6 (8.4%), duodenitis in 4 (5.6%), gastric ulcer in 3 (4.2%), gastric polyp in 2 (2.8%). The overall endoscopic yield was 60.2%. Conclusion. There is a need to increase the awareness of the role of paediatric endoscopy in the diagnosis and treatment of GI disorders in developing countries. Recurrent abdominal pain still remains a relevant indication for the procedure. The need to develop training programmes for paediatric endoscopy and paediatric gastroenterology in general in developing countries cannot be overemphasised

Antioxidant supplementation for sickle cell disease.

Background Sickle cell disease (SCD) refers to a group of genetic disorders characterized by the presence of an abnormal haemoglobin molecule called haemoglobin S (HbS). When subjected to oxidative stress from low oxygen concentrations, HbS molecules form rigid polymers, giving the red cell the typical sickle shape. Antioxidants have been shown to reduce oxidative stress and improve outcomes in other diseases associated with oxidative stress. Therefore, it is important to review and synthesize the available evidence on the effect of antioxidants on the clinical outcomes of people with SCD. Objectives To assess the effectiveness and safety of antioxidant supplementation for improving health outcomes in people with SCD. Search methods We used standard, extensive Cochrane search methods. The latest search date was 15 August 2023. Selection criteria We included randomized and quasi‐randomized controlled trials comparing antioxidant supplementation to placebo, other antioxidants, or different doses of antioxidants, in people with SCD. Data collection and analysis Two authors independently extracted data, assessed the risk of bias and certainty of the evidence, and reported according to Cochrane methodological procedures. Main results The review included 1609 participants in 26 studies, with 17 comparisons. We rated 13 studies as having a high risk of bias overall, and 13 studies as having an unclear risk of bias overall due to study limitations. We used GRADE to rate the certainty of evidence. Only eight studies reported on our important outcomes at six months. Vitamin C (1400 mg) plus vitamin E (800 mg) versus placebo Based on evidence from one study in 83 participants, vitamin C (1400 mg) plus vitamin E (800 mg) may not be better than placebo at reducing the frequency of crisis (risk ratio (RR) 1.18, 95% confidence interval (CI) 0.64 to 2.18), the severity of pain (RR 1.33, 95% CI 0.40 to 4.37), or adverse effects (AE), of which the most common were headache, nausea, fatigue, diarrhoea, and epigastric pain (RR 0.56, 95% CI 0.31 to 1.00). Vitamin C plus vitamin E may increase the risk of SCD‐related complications (acute chest syndrome: RR 2.66, 95% CI 0.77 to 9.13; 1 study, 83 participants), and increase haemoglobin level (median (interquartile range) 90 (81 to 96) g/L versus 93.5 (84 to 105) g/L) (1 study, 83 participants) compared to placebo. However, the evidence for all the above effects is very uncertain. The study did not report on quality of life (QoL) of participants and their caregivers, nor on frequency of hospitalization. Zinc versus placebo Zinc may not be better than placebo at reducing the frequency of crisis at six months (rate ratio 0.62, 95% CI 0.17 to 2.29; 1 study, 36 participants; low‐certainty evidence). We are uncertain whether zinc is better than placebo at improving sickle cell‐related complications (complete healing of leg ulcers at six months: RR 2.00, 95% CI 0.60 to 6.72; 1 study, 34 participants; very low‐certainty evidence). Zinc may be better than placebo at increasing haemoglobin level (g/dL) (MD 1.26, 95% CI 0.44 to 1.26; 1 study, 36 participants; low‐certainty evidence). The study did not report on severity of pain, QoL, AE, and frequency of hospitalization. N‐acetylcysteine versus placebo N‐acetylcysteine (NAC) 1200 mg may not be better than placebo at reducing the frequency of crisis in SCD, reported as pain days (rate ratio 0.99 days, 95% CI 0.53 to 1.84; 1 study, 96 participants; low‐certainty evidence). Low‐certainty evidence from one study (96 participants) suggests NAC (1200 mg) may not be better than placebo at reducing the severity of pain (MD 0.17, 95% CI ‐0.53 to 0.87). Compared to placebo, NAC (1200 mg) may not be better at improving physical QoL (MD ‐1.80, 95% CI ‐5.01 to 1.41) and mental QoL (MD 2.00, 95% CI ‐1.45 to 5.45; very low‐certainty evidence), reducing the risk of adverse effects (gastrointestinal complaints, pruritus, or rash) (RR 0.92, 95% CI 0.75 to 1.14; low‐certainty evidence), reducing the frequency of hospitalizations (rate ratio 0.98, 95% CI 0.41 to 2.38; low‐certainty evidence), and sickle cell‐related complications (RR 5.00, 95% CI 0.25 to 101.48; very low‐certainty evidence), or increasing haemoglobin level (MD ‐0.18 g/dL, 95% CI ‐0.40 to 0.04; low‐certainty evidence). L‐arginine versus placebo L‐arginine may not be better than placebo at reducing the frequency of crisis (monthly pain) (RR 0.71, 95% CI 0.26 to 1.95; 1 study, 50 participants; low‐certainty evidence). However, L‐arginine may be better than placebo at reducing the severity of pain (MD ‐1.41, 95% CI ‐1.65 to ‐1.18; 2 studies, 125 participants; low‐certainty evidence). One participant allocated to L‐arginine developed hives during infusion of L‐arginine, another experienced acute clinical deterioration, and a participant in the placebo group had clinically relevant increases in liver function enzymes. The evidence is very uncertain whether L‐arginine is better at reducing the mean number of days in hospital compared to placebo (MD ‐0.85 days, 95% CI ‐1.87 to 0.17; 2 studies, 125 participants; very low‐certainty evidence). Also, L‐arginine may not be better than placebo at increasing haemoglobin level (MD 0.4 g/dL, 95% CI ‐0.50 to 1.3; 2 studies, 106 participants; low‐certainty evidence). No study in this comparison reported on QoL and sickle cell‐related complications. Omega‐3 versus placebo Very low‐certainty evidence shows no evidence of a difference in the risk of adverse effects of omega‐3 compared to placebo (RR 1.05, 95% CI 0.74 to 1.48; 1 study, 67 participants). Very low‐certainty evidence suggests that omega‐3 may not be better than placebo at increasing haemoglobin level (MD 0.36 g/L, 95% CI ‐0.21 to 0.93; 1 study, 67 participants). The study did not report on frequency of crisis, severity of pain, QoL, frequency of hospitalization, and sickle cell‐related complications. Authors' conclusions There was inconsistent evidence on all outcomes to draw conclusions on the beneficial and harmful effects of antioxidants. However, L‐arginine may be better than placebo at reducing the severity of pain at six months, and zinc may be better than placebo at increasing haemoglobin level. We are uncertain whether other antioxidants are beneficial for SCD. Larger studies conducted on each comparison would reduce the current uncertainties

Case Report: Iatrogenic cushing’s syndrome in children following nasal steroid

Cushing syndrome is a hormonal disorder caused by prolonged exposure of body tissue to cortisol. We report two cases of iatrogenic Cushing's syndrome in two Nigerian children following intranasal administration of aristobed-N (Betamethasone+Neomycin) given at a private hospital where the children presented with feature of adenoidal hypertrophy. Two months into treatment children were noticed to have developed clinical and laboratory features of iatrogenic Cushing's syndrome with critical adrenal suppression. Serum cortisol (at presentation): 1st patient: 12nmol/L (reference range 240-618), 2nd Patient: 1.69nmol/L. Serum cortisol (3 months after weaning off steroid): 343.27 nmol/L (within normal range for the first patient; second patient newly presented and has just begun steroid weaning off process. The serum cortisol level one month into weaninig off process was 128 nmol/L). Unsupervised topical steroid administration in children can cause adrenal suppression with clinical features of Cushing's syndrome.Key words: Iatrogenic, cushing’s syndrome, nasal steroi

Global consensus recommendations on prevention and management of nutritional rickets

Background: Vitamin D and calcium deficiencies are common worldwide, causing nutritional rickets and osteomalacia, which have a major impact on health, growth, and development of infants, children, and adolescents; the consequences can be lethal or can last into adulthood. The goals of this evidence-based consensus document are to provide health care professionals with guidance for prevention, diagnosis, and management of nutritional rickets and to provide policy makers with a framework to work toward its eradication. Evidence: A systematic literature search examining the definition, diagnosis, treatment, and prevention of nutritional rickets in children was conducted. Evidence-based recommendations were developed using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system that describes the strength of the recommendation and the quality of supporting evidence. Process: Thirty-three nominated experts in pediatric endocrinology, pediatrics, nutrition, epidemiology, public health, and health economics evaluated the evidence on specific questions within five working groups. The consensus group, representing 11 international scientific organizations, participated in a multiday conference in May 2014 to reach a global evidence-based consensus. Results: This consensus document defines nutritional rickets and its diagnostic criteria and describes the clinical management of rickets and osteomalacia. Risk factors, particularly in mothers and infants, are ranked, and specific prevention recommendations including food fortification and supplementation are offered for both the clinical and public health contexts. Conclusion: Rickets, osteomalacia, and vitamin D and calcium deficiencies are preventable global public health problems in infants, children, and adolescents. Implementation of international rickets prevention programs, including supplementation and food fortification, is urgently required

Global consensus recommendations on prevention and management of nutritional rickets

BACKGROUND: Vitamin D and calcium deficiencies are common worldwide, causing nutritional rickets and osteomalacia, which have a major impact on health, growth, and development of infants, children, and adolescents; the consequences can be lethal or can last into adulthood. The goals of this evidence-based consensus document are to provide health care professionals with guidance for prevention, diagnosis, and management of nutritional rickets and to provide policy makers with a framework to work toward its eradication. EVIDENCE: A systematic literature search examining the definition, diagnosis, treatment, and prevention of nutritional rickets in children was conducted. Evidence-based recommendations were developed using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system that describe the strength of the recommendation and the quality of supporting evidence. PROCESS: Thirty-three nominated experts in pediatric endocrinology, pediatrics, nutrition, epidemiology, public health, and health economics evaluated the evidence on specific questions within five working groups. The consensus group, representing 11 international scientific organizations, participated in a multiday conference in May 2014 to reach a global evidence-based consensus. RESULTS: This consensus document defines nutritional rickets and its diagnostic criteria and describes the clinical management of rickets and osteomalacia. Risk factors, particularly in mothers and infants, are ranked, and specific prevention recommendations including food fortification and supplementation are offered for both the clinical and public health contexts. CONCLUSION: Rickets, osteomalacia, and vitamin D and calcium deficiencies are preventable global public health problems in infants, children, and adolescents. Implementation of international rickets prevention programs, including supplementation and food fortification, is urgently required