Primary hyperoxaluria type 1 (PH1) is an inborn error of glyoxylate metabolism. It results from genetic mutation of the AGXT gene. The study objective was to verify the clinical and epidemiological patterns of PH1 in Libyan children at Tripoli Children Hospital confirmed by AGXT gene mutation. A descriptive case series study of 53 children with PH1 diagnosed between 1994 and 2015 was carried out in the Nephrology Unit at Tripoli Children Hospital. Diagnosis of PH1 was based on the clinical presentation (renal stones or nephrocalcinosis), positive family history of PH1, and high 24 h urinary oxalate. Sampling for AGXT gene mutation was collected from April 2012 to December. 2015. Among the 53 children included, males composed of 62.3% of patients. Their age at presentation ranged between two months and 20 years with a mean age of 55.4 ± 48 months. The parents of 81.1% of these patients had positive consanguinity. Forty (75.5%) patients were from South West (mountain area), and 16 (40%) of them were from Yefrin. The most common mutation found in this study was c.731T>C (p.lle244thr) seen in 32 (71%) of children, and interestingly, among these patients, 87.1% were homozygous in gene typing, 86.2% had positive history of consanguinity, 71.4% were from South West (mountain area), 96.6% had family history of PH1, and 20% presented with impaired renal function. The patients with this mutation were younger at presentation than that with other genes, and it was more prevalent among boys (61.3%). Thus, the most common gene mutation found in Libyan children with PH1 was c.731T>C (p.lle244thr) and this is more likely due to the strong genetic pooling caused by the high consanguinity rate which requires an extensive genetic counseling.

Background: In patients with type 2 diabetes mellitus (T2DM), failure to control of blood glucose with lifestyle modifications and oral hypoglycemic agents (OHA) leads to insulin therapy. Objectives: We aimed to 1) find out the prevalence of psychological insulin resistance among patients with T2DM and 2) explore the factors affecting and reasons behind their attitude towards insulin use. Patients and methods: In a cross sectional study, 1703 Libyan patients with T2DM on oral hypoglycemic agents (OHA) were studied. They were recruited from outpatient clinics of two hospitals and 5 primary health care centers in Tripoli, Libya over a period of six months. They were asked to complete a self-administered questionnaire. Results: From the total number of 1703 participants, 1611 (94.6%) reported unwillingness to accept insulin therapy should it be prescribed to them. Hesitant patients reported more concerns about possible side effects that may develop from errors in insulin dose than acceptors (73.1% vs 46.7% respectively). 25.6% of the reluctant patients perceived that insulin may cause blindness. Concerns about painful injection of insulin and occurrence of hypoglycemia were expressed by 48.4% and 66.2% of the unwilling group respectively. Conclusion: Psychological insulin resistance is a common obstacle to initiation of insulin therapy in Libyan patients with T2DM. There is an urgent need for enhanced patient education to change the attitude of the patients towards insulin therapy.

Introduction: Nephrotic syndrome is a clinical picture characterized by severe proteinuria, hypoalbuminemia, edema and hypercholesterolemia. A retrospective study was carried out in order to describe disease pattern in newly diagnosed nephrotic syndrome of children admitted to Tripoli children hospital during the year 2014.Methods: The medical data of 56 patients aged between 1 year and 11 years diagnosed with idiopathic nephrotic syndrome were analysed using SPSS software. The data included gender differences, sensitivity to steroid therapy, relapses during six months of follow up and the effect of variable factors such as family history, hypertension, hematuria, serum urea on the degree of relapse.Results: Out of 56 patients with newly diagnosed nephrotic syndrome (NS), 60.7% were boys and 39.3% were girls, with a mean age 4.2±2.2 years. Age  was related significantly to the response to steroid therapy, where 79.5% of patients aged between 2-8 years (group 1) had steroid sensitive nephrotic syndrome (SSNS) compared with only 41.7% of patients aged less than 2 years or more than 8 years (group 2)  (P<0.001).  Although girls relapsed more than boys (70.5% versus 57.1%) during six months of therapy, this difference was not statistically significant. Similarly, no other factors measured such as family history of NS, hypertension, hematuria, serum complement and urea had any effect on the percentage of relapse in patients with newly diagnosed NS.  Conclusion: NS is one of the commonest reasons for admission to nephrology ward. It is more common in boys than girls. The age at presentation related significantly to the response to steroidal therapy. Regarding relapses, girls seems to relapse more frequent than boys and relapses was seen more in age group 1 than group 2, however, these differences were not significant. Other factors studied seems to have no effect on the relapse rate of children with newly diagnosed NS.Key-words: Idiopathic nephrotic syndrome, Steroid sensitive nephrotic syndrome, Steroid resistant nephrotic syndrome, Proteinuria.

Introduction: Nephrotic syndrome (NS) is a clinical picture characterized by severe proteinuria, hypoalbuminemia, edema and hypercholesterolemia. A retrospective study was carried out in order to describe disease pattern in newly diagnosed NS of children admitted to Tripoli children hospital during the year 2014. Methods: The medical data of 56 patients aged between 1 year and 11 years diagnosed with idiopathic nephrotic syndrome were analysed using SPSS software. The data included gender differences, sensitivity to steroid therapy, relapses withen six months of follow up and the effect of variable factors such as family history, hypertension, hematuria, serum urea on the degree of relapse. Results: Out of 56 patients with newly diagnosed NS, 60.7% were boys and 39.3% were girls, with a mean age 4.2±2.2 years. Age was related significantly to the response to steroid therapy, where 79.5% of patients aged between 2-8 years (group 1) had steroid sensitive NS (SSNS) compared with only 41.7% of patients aged less than 2 years or more than 8 years (group 2) (P<0.001). Although girls relapsed more than boys (70.5% versus 57.1%) during six months of therapy, this difference was not statistically significant. Similarly, no other factors measured such as family history of NS, hypertension, hematuria, serum complement and urea had any effect on the percentage of relapse in patients with newly diagnosed NS. Conclusion: NS is one of the commonest reasons for admission to nephrology ward. It is more common in boys than girls. The age at presentation related significantly to the response to steroidal therapy. Regarding relapses, girls seems to relapse more frequent than boys and relapses was seen more in age group 1 than group 2, however, these differences were not significant. Other factors studied seems to have no effect on the relapse rate of children with newly diagnosed NS.

Background: Pyoderma gangrenousm (PG) is an idiopathic ulcerative, non-infective chronic inflammatory skin disorder of unknown etiology. The most common reported underlying diseases in pediatrics are inflammatory bowel disease, followed by hematologic disorders, vasculitis, immune deficiencies and pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome. More than half of the cases occur with no underlying disease. The most frequently treatment should be tailored according to the underlying etiology. It includes systemic steroids, corticosteroid sparing agents such as dapsone, cyclosporine, azathioprine and currently TNF -alpha inhibitors such as etanercept, adalimumab and infliximab is a promising treatment of refractory PG. Response to treatment is high with cure rates reaching 90%. A high index suspicion and a through workup are mandatory in the management of pediatrics PG.Objective: To describe the clinical presentation, laboratory tests and challenges with treatment trials in two pediatric cases.Methods: Clinical information was gathered from the history, which was taken from their parents after an informed consent. Result:For the first time we report two pediatric cases in Libya who diagnosed with refractory pyoderma gangrenousm with no associated systemic diseases except PAPA which can't be excluded since no genetic tests available in all public hospitals in Libya, we report PG in two Libyan children of 2 year-old and 5 year-old, they presented with skin lesions beginning as a small pustule that progressed to very painful large ulcer with irregular borders. There were healed ulcers with scaring present on chest, both upper and lower extremities and abdominal wall. Laboratory tests for both cases showed an increase in white blood cell counts mainly neutrophils, anaemia, increased platelets count, elevated erythrocyte sedimentation rate, and elevated C-reactive protein. No haematological abnormalities were seen in peripheral blood smears except neutrophilic response, the red cell indices were suggestive of iron deficiency anaemia. Cultures for bacteria and fungi from skin lesions, and blood cultures were repeatedly negative. Immunoglobulin assay (IgG, IgM, IgA, IgE) was normal. The antinuclear antibody, cytoplasmic antibody, and antiphospholipid antibodies, tests for hepatitis B, C and HIV were all negative. They didn't have symptoms suggest ulcerative colitis, their colonoscopy was normal. Histopathology from their skin was consistent with pyoderma gangrenousm. Both cases were moderate response to high dose of oral prednisolone 2mg\kg per day complicated, however by growth retardation and cushingoid habitus and minimal response to corticosteroid sparing agent azathioprine. Anti TNF-alpha inhibitors etanercept, which has been available at hospital since March 2018, we used it in case 2 because he was inadequate response to azathioprine. Both cases showed briefly remitted on azathioprine with relapsed at least 2-3 episodes per year. The ulcer lesions healed with cribriform scaring within 6-8 weeks in...