Medium-chain acyl-Coenzyme A dehydrogenase deficiency (MCADD) is a disorder of fatty acid β oxidation inherited in an autosomal recessive manner. The enzyme is useful in hepatic ketogenesis, a major source of energy once hepatic glycogen stores become depleted during prolonged fasting. It is a cause of hypoketotic hypoglycaemia in a previously well child. MCADD is not part of newborn screening in Ireland; children are likely to be missed if routine hypoglycaemic screen is not instituted when blood glucose level is below 2.6 mmol/L. This is a case of an otherwise healthy 23-month-old baby girl who presented with severe hypoglycaemia with some initial diagnostic dilemma.

Background: Urinary tract infection (UTI) is a common reason for referral to the emergency department (ED) especially in unwell infants. Upper UTIs are particularly at risk of significant complications later in life. Rapid dipstick urinalysis and microscopy are often used in unwell children as a screening tool to guide early diagnosis and treatment. This study aims to evaluate the sensitivity of dipstick urinalysis and microscopy in the diagnosis of UTI. Methods: A retrospective review of children aged 16 years and below with positive urine culture (UC) over a 3-year period was done. The results of urine dipstick and microscopy were compared with the positive UC and sensitivities calculated. Results: Dipstick urinalysis and microscopy of 262 children were studied. Female-to -male ratio of 1.8:1. Median age was 0.79 (range: 0.02–15.95) years. The sensitivity of nitrite, blood, and leukocyte esterase (LE) were 0.54, 0.74, and 0.86 (95% confidence interval [CI] = 0.46–0.62, 0.68–0.80, and 0.82–0.91), respectively. The sensitivity of pyuria of ≥100 cells/mm 3 was 0.92 (95% CI = 0.89–0.95). The presence of any of the 3 dipstick parameters increased the sensitivity to 0.97 (95% CI = 0.95–0.99). The lowest sensitivity 0.49 (95% CI = 0.40–0.58) was found with combined positive LE and nitrite. There was a significant comparison between positive LE dipstick test and pyuria ( P = 0.000004). Conclusions: Dipstick urinalysis may not be reliable in ruling out UTI in children. However, considering both positive dipstick and pyuria will be more useful in making the diagnosis.

This is a case of hyperglycaemic hyperosmolar state (HHS) as first presentation of type 1 diabetes mellitus in a 14-year-old girl with background complex medical needs. She presented with marked hyperglycaemia (56 mmol/L) without significant ketonaemia (2.6 mmol/L) and serum hyperosmolality (426 mOsm/kg). Managing her profound hypernatraemic (>180 mmol/L) dehydration was challenging but resulted in good outcome. Paediatric patients with HHS will likely be treated with the diabetes ketoacidosis (DKA) protocol because of perceived rarity of HHS leading to inadequate rehydration and risk of vascular collapse. Hence, emphasis on the differences in the management protocols of DKA and HHS is paramount. Prompt recognition and adequate management are crucial to avert complications. The undesirable rate of decline of hypernatraemia due to the use of hypotonic fluid was captured in this case. We describe the pivotal role of liberal fluid therapy with non-hypotonic fluids.

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Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown aetiology, which can affect any organ system. Tuberculosis (TB) is a common infection in SLE because of immune dysregulation associated with the latter. We report a case of an adolescent female who presented with a year's history of polyarticular arthralgia and fever. Physical examination revealed a large left effusion that needed drainage. Investigations revealed a combined diagnosis of SLE and TB. Management comprised quadruple anti-TB therapy and SLE treatment. She made a steady recovery and has maintained a stable state from the lupus perspective.

Background: The use of oral rehydration therapy (ORT) remains limited despite international guidelines for diarrhoeal disease management. This study was done to assess the perception and practice of ORT among caregivers in the management of diarrhoeal disease. Methods: A descriptive cross-sectional study was conducted in the Paediatric Department of Cork University Hospital, Ireland. The study population consisted of parents/guardians of children attending the Paediatric Day Ward or Paediatric Outpatient Department. A questionnaire consisting of 30 questions was originally designed by the first author to include all the study variables, and was given to the participants. Results: Out of 402 respondents, 76.6% (n=308) could describe diarrhoea correctly and perceived it as a serious illness. 81.3% (n=327) of participants knew about ORT but only 27.2% (n=89) agreed it was an ideal first line of management of diarrhoeal disease. Diarrhoeal episodes in the preceding 12 months were reported predominantly in children under the age of 5 years (p<0.001). Only 8.7% (n=17) of caregivers used ORT for diarrhoeal management. Conclusions: Poor uptake of ORT appears to be due to caregivers’ negative perceptions of ORT. This emphasizes the need for correct and adequate education.

International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations.IJCRI publishes Review Articles, Case Series, Case Reports, Case in Images, Clinical Images and Letters to Editor. Website: www.ijcasereportsandimages.comSevere hypernatremia in an adolescent male with anorexia nervosa Kene Ebuka Maduemem, Comfort O. Adedokun ABSTRACT Introduction: Anorexia nervosa is a commonly encountered cause of severe underweight in pediatric settings. It is an eating disorder characterized by the inability to maintain a minimally normal weight, a devastating fear of weight gain, relentless dietary habits that prevent weight gain, and a disturbance in the way in which the body image is perceived. Hypernatremia is an unusual electrolyte imbalance in anorexia nervosa. Case Report: This is a case of a 12-year-old male admitted with progressive weight loss following restrictive food intake for the preceding one year. He presented with severe hypernatremia. Correction of severe hypernatremia was successful after four to five days. This correction was slow but gradually achieved. There was no altered sensorium throughout admission which remains the worst nightmare of any managing team. Feeding protocol as per Junior MARSIPAN guidelines was adhered to. He made an excellent recovery and was discharged after 24 days of admission with a weight gain of 6.1 kg. Conclusion: Anorexia nervosa is commonly encountered in pediatric settings and can cause potentially life-threatening physical and psychological complications. Hypernatremia is an uncommon metabolic abnormality; slow correction averts untoward side effects.

International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations.