Elizabeth Eberechi Oyenusi

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 Cushings 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 Cushings 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 Cushings syndrome.

Hyperglycemia in Acutely Ill Non-diabetic Children in the Emergency Rooms of 2 Tertiary Hospitals in Lagos, Nigeria.

Objectives The study aimed to determine the prevalence of hyperglycemia in sick children admitted into the emergency rooms and to investigate its relationship with adverse outcomes. Methods A prospective study involving 2 tertiary hospitals in Lagos. Study subjects included all children aged beyond 1 month. An Accu-Chek Active glucometer was used for the bedside blood glucose determination. Hyperglycemia was defined as blood glucose greater than 7.8 mmol/L. Results A total of 1045 patients were recruited with hyperglycemia being recorded in 135 patients (prevalence rate of 12.9%). Mean age of the hyperglycemic patients was 29.0 ± 31.23 months. Prevalence rates of hyperglycemia among the leading diagnoses were 17.4% in acute respiratory tract infections, 11% in malaria, 15.3% in septicemia, 14.9% in gastroenteritis, and 18.2% in burns. Other conditions include sickle cell anemia, meningitis, and malnutrition. Mortality rate was significantly higher overall in hyperglycemic compared with the normoglycemic patients (15.4% vs 8.0%, P = 0.011). With regard to specific diagnoses, significantly higher mortality rates were recorded in hyperglycemic patients with acute respiratory tract infections (28% vs 8%, P = 0.011) and malaria (21.4% vs 5.0%, P = 0.006) than in their normoglycemic counterparts. Conclusions Hyperglycemia is common in ill children admitted to the emergency rooms and is associated with 2 to 4 times higher mortality in common childhood diseases encountered. Blood glucose determination is important in all acutely ill children at presentation. The practice of empirical administration of intravenous glucose in some resource-constrained facilities where blood glucose testing facilities are not readily available should be discouraged.

Congenital adrenal hyperplasia at the Lagos University Teaching Hospital: A 10-year review

Introduction: Congenital adrenal hyperplasia (CAH) encompasses a group of autosomal recessive disorders, each of which involves a deficiency of an enzyme involved in the synthesis of cortisol, aldosterone, or both. More than 90% of CAH are caused by 21-hydroxylase deficiency (21HD), found in 1:10,000 to 1:15,000 live births. Early diagnosis of CAH is important because newborn babies with salt-losing forms of CAH die within days or weeks if not treated. In a resource-constrained setting, lack of awareness, poor health facilities, inadequate laboratory support, and expensive and erratic availability of drugs make most children with CAH vulnerable. Objectives: To describe characteristics of the children with CAH attending the Pediatric Endocrinology Clinic of Lagos University Teaching Hospital over a 10 years period. Subjects and Methods: Case records of patients with CAH attending the clinic from February 2005 to January 2015 were reviewed and data extracted from them for further statistical analysis using Microsoft Excel 2010. Results: Twenty eight patients (14 males and 14 females) were seen constituting 34% of the patients with ambiguous genitalia. The median (range) age of the patients was 7 (0.33-  21) years, and the median (range) age at presentation was 3 (0.01-15) years. These include within the neonatal period (25.0%), before 1 year (17.9%), before 5 years (25.0%), and beyond 5 years (32.1%). Modes of presentation included ambiguous genitalia, precocious puberty, salt loss, and discovery on screening because of an affected sibling. Presumed enzyme deficiencies are 21 hydroxylase (78.5% of which 18.2% salt-losing), 11-beta-hydroxylase (14.3%), 17α-hydroxylase (3.6%), and P450 oxidoreductase (3.6%), respectively. Six patients (21.4%) had associated hypertension. Other comorbidities include Blount′s disease and pelvi-ureteric junction obstruction. Three male patients were also managed for subsequent associated central precocious puberty. Two siblings were affected in 3 nonconsanguineous families. One patient had contracted HIV/Acquired Immune Deficiency Syndrome from blood transfusion after clitoridectomy. Challenges in management include unavailability and unaffordability of investigations and drugs for treatment. Conclusions: CAH is an important cause of ambiguous genitalia and precocious puberty. Careful examination of the newborn genitalia is important, and a high index of suspicion maintained for salt-losing forms. Increased awareness should be created nationwide for prompt diagnosis and referral when applicable. Treatment is life-long and expensive.

Primary congenital hypothyroidism complicated by persistent severe anaemia in early infancy: a case report with a literature review

Although anaemia is a common finding in adults with hypothyroidism, there is a general paucity of studies on anaemia in infants with congenital hypothyroidism. 1 The degree of anaemia is usually mild to moderate, with a haemoglobin concentration that is rarely less than 8-9 g/dl. 2 The associated anaemia in hypothyroidism is generally normochromic and normocytic, and occasionally mildly macrocytic. 1 This heterogeneity has been attributed to coexisting deficiencies of iron, vitamin B12 and folic acid, largely due to impaired absorption. 2,3 Anaemia in hypothyroidism has been linked to impaired haemoglobin synthesis due to a deficiency of thyroxine (T4). 3 There is evidence of a direct effect of the thyroid hormone on erythropoiesis. The pathogenesis of anaemia in hypothyroidism has been linked to either a lack of erythropoietin production, or a physiological adaptation to the decreased tissue oxygen requirements resulting from a decrease in the basal metabolic rate. 4 The red cell life span is normal and the results of ferrokinetic studies are compatible with hypoproliferative erythropoiesis in hypothyroidism. 5,6 Thyroid hormones affect oxygen needs at cellular level. Therefore, the responses are compatible with an appropriate physiological adjustment. 2 In vitro studies have shown that thyroid hormones potentiate the effect of erythropoietin on erythroid colony formation. 7 Conversely, iron deficiency impairs T4 synthesis by reducing the activity of haeme-dependent thyroid peroxidase. 8 White blood cell and platelet counts are usually unaffected in hypothyroidism. 2 Indeed, it has been stated that the presence of pancytopenia suggests that hypothyroidism is not primary, but instead relates to hypopituitarism. 9 In their report, Antonijevic Nesovic, Trbojevic and Milosevic 10 state that the presence of acanthocytosis in a peripheral blood smear suggests hypothyroidism in approximately 90% of cases. The response to thyroid hormone therapy is gradual. A slow improvement in haemoglobin concentration is seen over several months. 11 Children with a haematocrit value less than 21% and 15%, are considered to have severe anaemia and very severe anaemia, respectively. 12 We report on a case of a six-month old infant who presented with persistent severe anaemia, and who was found to have primary congenital hypothyroidism after a prolonged stay in hospital.

Recurrent hypoglycaemia in type 1 diabetes mellitus resulting from intensive academic activity: an illustrative case report

Abstract We report a common diabetes management problem illustrated by an adolescent female university student with recurrent episodes of hypoglycaemia on Tuesdays when she has intensive academic activity lasting most of the day. Steps taken to reduce the risk of hypoglycaemia were patient education and empowerment, frequent self monitoring of blood glucose, reduction in insulin dose on Tuesdays and emphasizing availability of ongoing professional guidance and support anytime she may need it. One of the challenges encountered in the management of this patient was her family’s inability to afford the cost of basal-bolus regimen or continuous subcutaneous insulin infusion via insulin pump; the two insulin regimens that best fit into university lifestyle. Conclusion: Adolescents with diabetes mellitus attending tertiary educational institutions may be at increased risk of hypoglycaemia, particularly on days when they have intensive academic activities.