Frequency, Clinical Characteristics and Predictors of Ketoacidosis at Diagnosis of Type One Diabetes Mellitus in Children and Adolescents from Jordan

Objective: Data regarding diabetic ketoacidosis (DKA) at diagnosis of type one diabetes (T1D) in developing countries are scarce. The aim of this study was to describe the frequency of DKA at the onset of T1D in children and adolescents in Jordan and to compare the clinical and biochemical characteristics between the group that presented with DKA and the group that did not. Methods: The records of 341 children and adolescents, less than sixteen years of age, who were diagnosed with T1D between 2015 and 2019 were evaluated retrospectively. Results: Of all the children diagnosed with T1D, 108 (31.7%) presented with DKA. The majority had mild or moderate DKA (38% and 33.3% respectively). Higher paternal education levels were associated with a lower probability of presenting with DKA (p=0.043). A family history of T1D had a protective effect on the occurrence of DKA (Odds ratio=2.138; 95% confidence interval=1.167-3.917, p=0.014). Patients with celiac disease and higher HbA1c levels were more likely to experience recurrent episodes of DKA, (p=0.004 and 0.011, respectively). Conclusion: In Jordan, the rate of DKA at presentation of T1D remains high. Prevention campaigns are needed to increase diabetes awareness among the public and healthcare providers.

Effect of COVID-19 pandemic on presentation and referral patterns of newly diagnosed children with type 1 diabetes in a developing country.

OBJECTIVES: The global spread of coronavirus disease 2019 (COVID-19), had a great impact on patients worldwide, including those with chronic diseases. We aim to study the effect of COVID-19 pandemic on presentation patterns of patients with type 1 diabetes (T1D) in Jordan, as an example a developing country with limited resources. METHODS: Medical charts were reviewed for patients presented with new-onset T1D to Jordan University hospital during the first year of pandemic and the preceding year. Categorical data were compared using Pearson Chi-Square and Fisher's exact test. Continuous data were compared using the Independent Sample t-Test. RESULTS: A total of 137 children were diagnosed with T1D during the study period, with 60.6% of those children were diagnosed in the pre-pandemic year compared to 39.4% during the first year of pandemic, p-value=0.013. Percentage of patients diagnosed with DKA as first presentation of T1D during the pre-pandemic year was 34.9% compared to 51.9% during the pandemic year, p-value=0.049. Significant differences in family monthly income (p-value=0.006) and paternal education level (p-value=0.036) were found between children with DKA and those without DKA in the pre-pandemic year, but they were not significant during the pandemic year. CONCLUSIONS: The unprecedented COVID-19 pandemic had affected presentation pattern of newly diagnosed T1D patients, manifested by lower number of children diagnosed with T1D and higher percentage of DKA as first presentation compared to the preceding year. Health care services should be at utmost preparedness for possible future waves and other pandemics.

Recessive mutation in GALNT3 causes hyperphosphatemic familial tumoral calcinosis associated with chronic recurrent multifocal osteomyelitis.

Albaramki J, Dmour H, Shboul M, Bonnard C, Venkatesh B, Odeh R. Recessive mutation in GALNT3 causes hyperphosphatemic familial tumoral calcinosis associated with chronic recurrent multifocal osteomyelitis. Turk J Pediatr 2019; 61: 130-133. Hyperphosphatemic familial tumoral calcinosis is a rare autosomal recessive disorder that is characterized by persistent hyperphosphatemia and extra-articular calcifications. Three cases were previously reported with hyperphosphatemic familial tumoral calcinosis that were associated with chronic recurrent multifocal osteomyelitis, an autoinflammatory disorder that is characterized by recurrent episodes of bone pain. We describe here an 11-year-old child who was diagnosed with these two conditions and was found to carry a splice site mutation c.1524+1G > A in the GALNT3 gene.

Urinary tract infection caused by extended-spectrum ß-lactamase-producing bacteria: Risk factors and antibiotic resistance.

BACKGROUND: The aim of this study was to investigate the risk factors for acquisition of extended spectrum ß-lactamase (ESBL)-producing bacteria in community-acquired urinary tract infection (UTI) and to evaluate their antimicrobial resistance. METHODS: The medical records of hospitalized children were retrospectively evaluated. Children with ESBL-producing bacteria UTI were matched with controls with non-ESBL-producing bacteria UTI of the same age and gender. RESULTS: A total of 243 patients with community-acquired UTI in a 5 year period were evaluated, of whom 46% had UTI caused by ESBL bacteria. Seventy-seven cases were matched with 77 controls. There were no significant differences in the clinical presentation between the two groups apart from a longer hospital stay in the ESBL group (9.1 ± 5.5 days vs 8.0 ± 4.4 days, P = 0.013). Significant potential risk factors for ESBL-UTI were previous use of antibiotics in the last 3 months, previous hospitalization in the last 3 months, history of recurrent UTI, and presence of renal anomalies. On logistic regression analysis, history of previous hospitalization in the last 3 months (OR, 3.83; 95%CI: 1.49-9.84) was identified as an independent significant risk factor for ESBL-UTI. There was a significantly higher resistance to amoxicillin-clavulanate, amikacin, gentamycin and quinolones in the ESBL group compared with the control group. CONCLUSION: Recognizing the risk factors for ESBL-UTI helps to identify the high-risk cases and enables proper management.

Congenital glucose-galactose malabsorption: A case report with a novel SLC5A1 mutation.

A three-day-old newborn girl presented with decreased feeding and dehydration. She was sick and in shock. She had renal impairment and hypernatremia. With the resumption of breast feeding, she developed watery stools and hypernatremia. Glucose-Galactose Malabsorption was suspected and confirmed by the presence of a likely pathogenic homozygous variant in SLC5A1.

Pediatric focal segmental glomerulosclerosis in Jordan: A tertiary hospital experience.

Our objective is to study the demographical data, clinical course and outcome of children with primary focal segmental glomerulosclerosis (FSGS) in Jordan. A retrospective chart review of patients with a diagnosis of FSGS at a tertiary care hospital from the period July 2010 to July 2016 was conducted. A total of 99 patients were analyzed. The mean age of presentation was 3.71 ± 2.59 years, 66% were male. At presentation, 66.6% of patients were steroid-resistant, 10% had a steroid dependant course and 20.2% had familial FSGS. Cyclosporine was used in 66.6% of children with a response rate of 46.9%. Long-term follow-up showed complete remission in 29.3%, partial remission in 31.3%, end-stage renal disease in 22.2%, and death in 11.1%. There is a high prevalence of familial FSGS in our Jordanian cohort with a high rate of progression to end-stage kidney disease.

HENOCH-SCHONLEIN PURPURA IN CHILDHOOD A FIFTEEN-YEAR EXPERIENCE AT A TERTIARY HOSPITAL.

OBJECTIVE: To examine the epidemiological and clinical characteristics of children diagnosed with Henoch Sch6nlein purpura (HSP) and to compare them with other areas in the world. METHODS: The medical records of children with HSP were retrospectively reviewed at the Jordan University Hospital between the years 1998 and 2012. The clinical and demographical features, laboratory tests, management and outcome were assessed. RESULTS: There were 55 children with HSP, with a mean age of 7 years (60% were males); 85.4% of patients were less than 10 years; 72.7% of cases presented during the winter and autumn. There was a history of antecedent upper respiratory tract infection in 49.1% of cases; 32.7% of children had more than one hospitalization. Purpuric skin rash was seen in 100%, abdominal pain in 74.5%, arthritis in 58.2%, renal involvement in 30.9% of patients. In four patients HSP was the presenting feature of familial Mediterranean fever (FMF). CONCLUSION: HSP is a benign and self-limiting disease with an excellent prognosis. There are no significant differences in the epidemiological and clinical profile than reported elsewhere.

Neurological and cardiac complications in a cohort of children with end-stage renal disease.

Adult patients with chronic kidney disease are at risk of major neurologic and cardiac complications. The purpose of this study is to review the neurological and cardiac complications in children with end-stage renal disease (ESRD). A retrospective review of medical records of children with ESRD at Jordan University Hospital was performed. All neurological and cardiac events were recorded and analyzed. Data of a total of 68 children with ESRD presenting between 2002 and 2013 were reviewed. Neurological complications occurred in 32.4%; seizures were the most common event. Uncontrolled hypertension was the leading cause of neurological events. Cardiac complications occurred in 39.7%, the most common being pericardial effusion. Mortality from neurological complications was 45%. Neurological and cardiac complications occurred in around a third of children with ESRD with a high mortality rate. More effective control of hypertension, anemia, and intensive and gentle dialysis are needed.

A family with five siblings affected with nephronophthisis.

Nephronophthisis is an autosomal recessive disease that leads to end-stage renal disease. These days, molecular genetic analysis is used pre-emptively for making a definitive diagnosis in patients who have clinical and radiological data suggestive of the disease. Herein, we are reporting a 12-year-old girl who was genetically diagnosed to have juvenile nephronophthisis, which explained the mystery of the chronic kidney disease in her four affected siblings.

Parenteral versus oral iron therapy for adults and children with chronic kidney disease.

BACKGROUND: The anaemia seen in chronic kidney disease (CKD) may be exacerbated by iron deficiency. Iron can be provided through different routes, with advantages and drawbacks of each route. It remains unclear whether the potential harms and additional costs of intravenous (IV) compared with oral iron are justified. OBJECTIVES: To determine the benefits and harms of IV iron supplementation compared with oral iron for anaemia in adults and children with CKD. SEARCH METHODS: In March 2010 we searched the Cochrane Renal Group's specialised register, the Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library, MEDLINE and EMBASE without language restriction. SELECTION CRITERIA: We included randomised controlled trials (RCTs) and quasi-RCTs in which oral and IV routes of iron administration were compared in adults and children with CKD. DATA COLLECTION AND ANALYSIS: Two authors independently assessed study eligibility, risk of bias, and extracted data. Results were reported as risk ratios (RR) or risk differences (RD) with 95% confidence intervals (CI) for dichotomous outcomes and for continuous outcomes the mean difference (MD) was used or standardised mean difference (SMD) if different scales had been used. Statistical analyses were performed using the random-effects model. Subgroup analysis and univariate meta-regression were performed to investigate between study differences. MAIN RESULTS: Twenty eight studies (2098 participants) were included. Risk of bias attributes were poorly performed and/or reported with low risk of bias reported in 12 (43%) studies for sequence generation, incomplete outcome reporting and selective outcome reporting and in 6 (16%) studies for allocation concealment. No study was blinded for participants, investigators and outcome assessors but all were considered at low risk of bias because the primary outcome of haemoglobin was a laboratory outcome and unlikely to be influenced by lack of blinding. Haemoglobin (22 studies, 1862 patients: MD 0.90 g/dL, 95% CI 0.44 to 1.37); ferritin (24 studies, 1751 patients: MD 243.25 µg/L, 95% CI 188.74 to 297.75); and transferrin saturation (18 studies, 1457 patients: MD 10.20%, 95% CI 5.56 to 14.83) were significantly increased by IV iron compared with oral iron. There was a significant reduction in erythropoiesis-stimulating agent (ESA) dose in patients receiving dialysis who were treated with IV iron (9 studies, 487 patients: SMD -0.76, 95% CI -1.22 to -0.30). There was a high level of heterogeneity in all analyses. Mortality and cardiovascular morbidity did not differ significantly, but were reported in few studies. Gastrointestinal side effects were more common with oral iron, but hypotensive and allergic reactions were more common with IV iron. AUTHORS' CONCLUSIONS: The included studies provide strong evidence for increased ferritin and transferrin saturation levels, together with a small increase in haemoglobin, in patients with CKD who were treated with IV iron compared with oral iron. From a limited body of evidence, we identified a significant reduction in ESA requirements in patients treated with IV iron, and found no significant difference in mortality. Adverse effects were reported in only 50% of included studies. We therefore suggest that further studies that focus on patient-centred outcomes are needed to determine if the use of IV iron is justified on the basis of reductions in ESA dose and cost, improvements in patient quality of life, and with few serious adverse effects.

Rituximab therapy in two children with autoimmune thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is a rare disease among pediatric patients, in whom it may be mistaken for hemolytic uremic syndrome (HUS) and idiopathic thrombocytopenic purpura (ITP). Familial forms are caused by mutations in the ADAMTS13 gene, whereas acquired forms may result from an inhibitory antibody directed against ADAMTS13, a metalloprotease that cleaves very large multimers of Von Willebrand factor (VWF), thereby preventing platelet aggregation in blood vessels. We report two cases of TTP. The first was a 15-year-old girl with her first episode of TTP that failed to respond after 10 days of plasmapheresis and was treated with rituximab; she has remained in remission at 12 months of follow-up. The second was a 6-year-old boy with acquired relapsing TTP previously managed with plasmapheresis and prednisolone, who presented with a third relapse that was treated with plasmapheresis and rituximab; he remains in remission 17 months after treatment. Rituximab has been used by pediatricians for treating B cell malignancy, autoimmune diseases and antibody-mediated diseases, such as the Factor VIII inhibitors in hemophilia A, and may also have a promising role in children with acute refractory or chronic relapsing TTP.