Correlation between antiphospholipid antibodies and renal involvement in children with Henoch-Schönlein purpura: A cross-sectional study.

Background: Renal involvement is the most damaging long-term complication of Immunoglobulin-A (IgA) vasculitis. In the lack of a definite predictive biomarker for renal involvement, antiphospholipid antibodies (aPL) have been proposed in recent years. Methods: In this prospective cohort of 48 pediatric patients who were admitted with IgA vasculitis from September 2015 to June 2017, two serum samples were taken 12 weeks apart to detect Anti-Phospholipid antibodies. All patients were followed-up for renal involvement for six months. Results: Renal involvement occurred in 14 out of 48 patients with IgA vasculitis (29.16%). APLs were positive in nine out of 14 patients with IgA vasculitis and renal involvement (64.28%), in contrast to only six out of 34 patients with IgA vasculitis without renal involvement (17.64%). The presence of aPL antibodies was statistically associated with renal involvement (P=0.002). Although, the relationship between both sex (P=0.025) and age (P=0.046) with aPL positivity was statistically significant, performing a modified logistic regression test, the odds ratio was significant between the groups with and without renal involvement only in term of age and aPL positivity). Conclusion: The presence of aPL antibodies was statistically associated with renal involvement. We found a significant relationship between the age and aPL positivity. Hence, we need multicenter, more extensive cohort studies to reach a better and more accurate conclusion on the relationship between serum aPLs and renal involvement in IgA vasculitis patients.

Post SARS-CoV-2 infection reactive arthritis: a brief report of two pediatric cases.

BACKGROUND: Although, preliminary reports of Severe Acute Respiratory Syndrome (SARS)-CoV-2 infection suggest that the infection causes a less severe illness in children, there is now growing evidence of other rare or even serious complications of disease. CASE PRESENTATION: During the recent COVID-19 pandemic in Kerman, Iran, two children (an 8 year-old boy and a 6 year-old girl) were referred to outpatient Clinic of Pediatric Rheumatology with complaints of limping. Both children had experienced fever and mild respiratory tract infection. At the beginning of the second week of infection, they developed joint effusion. They both tested positive for coronavirus infection and were therefore diagnosed with post Coronavirus reactive arthritis. Both children were treated successfully with rest and Non-Steroidal Anti-Inflammatory Drugs (NSAID). They did not have any medical problems in the two months fallow up. CONCLUSIONS: These two cases suggest that COVID-19 may be rheumatogenic. Highlighting the need for awareness of physicians, especially pediatricians, regarding the pathogenesis margins of this virus, as late presentations are of great importance.

Determination of the Relationship Between Kobayashi, Sano, and Egami Criteria and Prevalence of Intravenous Immunoglobulin Resistance and Coronary Artery Aneurysm in Iranian Children with Kawasaki Disease.

INTRODUCTION: Kawasaki disease (KD) is a systemic vasculitis that occurs mostly in children under five years old. Kawasaki affects the middle-size arteries, especially the coronary arteries. Therefore, without adequate treatment, it may cause coronary artery aneurysm in 25% of patients. The purpose of this study was to investigate the relationship between Kobayashi, Sano, and Egami criterions with coronary artery aneurysm in KD patients during the last ten years and to identify risk factors in patients with intravenous immunoglobulin (IVIG)-resistant and coronary artery aneurysms. METHODOLOGY: Medical records of 363 Kawasaki patients referred during 2008-2017 were reviewed. Patients' demographic data and Kobayashi, Sano, and Egami scores of each patient were calculated. Based on echocardiographic findings, cases of coronary artery aneurysm were determined. Sensitivity, specificity, positive and negative predictive value, and the accuracy of each criterion were determined to predicting IVIG resistance and detect coronary artery aneurysm. RESULTS: There was a slight relationship between IVIG-resistance in Kawasaki children and its prediction based on the Kobayashi risk score, but no relationship was found between the Egami and Sano criteria. Sixty-three patients (17.4%) had coronary artery lesions (CALs) on time of diagnosis. There were no statistically significant differences between gender and mean age of children with and without CALs. Also, there was no significant relationship between coronary artery aneurysm in Kawasaki children and its prediction based on the above three risk factors. The area under the ROC-curve of all three risk measures of Kobayashi, Egami, and Sano indicated that all three criteria were not useful in predicting CALs. CONCLUSION: Despite the low accuracy of the three above criteria to predictive of patients with IVIG resistance, it seems that the variables of age, duration of fever, and C-reactive protein (CRP) are more useful than other variables and may be utilized to evaluate patients by establishing a more appropriate cut-off point.

A dermatopathic Juvenile Dermatomyositis; An Unexpected Case in Childhood.

Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory disease, which usually presents with skin rashes along with muscle weakness. We report a case of JDM in a 10- year-old girl with no skin manifestations presenting with progressive muscle weakness and fatigue. Further laboratory investigations, along with a muscle biopsy, confirmed the diagnosis of adermatopathic JDM. The patient was treated with intravenous immunoglobulin, corticosteroids, methotrexate, hydroxychloroquine, pamidronate, and rituximab. Following treatment, patients' symptoms subsided, and she gained normal muscular strength over a year.

Determination of diagnostic value (validity) leukocyte esterase (urine dipstick strip) in differentiating inflammatory arthritis from bacterial arthritis.

BACKGROUND: The current diagnostic cornerstone for septic arthritis contains gram stains, bacterial culture, and cell count with a differential of aspirated synovial fluid. Recently, a synovial leukocyte esterase (LE) test has been used for diagnosing septic arthritis. Since this test measures the esterase activity of leukocytes, there is always a dilemma for using this test in patients with inflammatory arthritis. METHODS: We collected the synovial fluid specimens as part of the general diagnostic protocol for patients suspected of Juvenile Idiopathic Arthritis (JIA) or Septic Arthritis (SA). Each group included 34 patients. We compared the result of the synovial LE test with the result of the culture of each patient. RESULTS: The mean ages of patients were 64.14 ± 31.27 and 50.88 ± 23.19 months in the JIA group and septic arthritis group, respectively. The LE test results were positive in 30 specimens, trace in 3 and negative in one in the first-time test and were positive in 31 specimens and trace in 3 in the second-time test, while it was negative in all patients with JIA. Hence, the sensitivity of the synovial LE test was 80.8%, the specificity, PPV, and NPV were 78.6, 70.0, 86.8% respectively based on a positive culture. CONCLUSION: The leukocyte esterase strip test can be used as a rapid, bedside method for diagnosing or excluding bacterial infections in different body fluids. The synovial LE test can be used as an accurate test to rapidly rule in or out an acute articular bacterial infection, even in patients with concurrent inflammatory arthritis.

Another Limping Child: An Interesting Diagnosis Journey.

Limp is described as any deviation from a normal gait pattern for the child's age. Limping takes many forms and is one of the most enigmatic complaints in pediatric medicine. It is never normal, and both benign and life-threatening illnesses can present with limp. The provisional diagnosis can be a challenge to establish even after history, physical, and laboratory examinations.

Distal renal tubular acidosis and its relationship with hearing loss in children: preliminary report.

INTRODUCTION: In autosomal recessive distal renal tubular acidosis (DRTA), a substantial fraction of the patients have progressive bilateral sensorineural hearing loss. This coexistence is due to the mutations of a gene expressed both in the kidney and in the cochlea. The aim of this study was to assess the correlation between hearing loss and DRTA. MATERIALS AND METHODS: In this study, 51 children diagnosed with renal tubular acidosis were evaluated. Diagnosis of DRTA was based on clinical manifestations and detection of normal anion gap metabolic acidosis, urine pH higher than 5.5, and positive urinary anion gap. Audiometry was performed in children with DRTA and sequencing of the ATP6V1B1 gene was done for those with sensorineural hearing loss. RESULTS: Twenty-seven patients (52.9%) had DRTA, of whom 51.9% were younger than 1 year old, 55.6% were boys, and 44.4% were girls. Eleven patients (40.7%) had bilateral sensorineural hearing loss, consisting of 5 of 15 boys (33.3%) and 6 of 12 girls (50.0%). There was no correlation between hearing loss and gender. Three patients with hearing loss had mutation in the ATP6V1B1 gene (11.1% of patients with DRTA and 27.3% of patients with DRTA and hearing loss). CONCLUSIONS: This study indicated that a significant percentage of the children with DRTA had sensorineural hearing loss and mutation in ATP6V1B1 gene. It is recommended to investigate hearing impairment in all children with DRTA.

Modified ilioinguinal approach to the acetabulum and pelvis from beneath the inguinal ligament: a subinguinal approach.

The ilioinguinal approach is favoured for fractures of the anterior part of the pelvis, but it is associated with some technical difficulties and it is not suitable for some complex fractures. In 2008, a modified ilioinguinal approach was introduced by Farid Yr which afforded visualisation of the inside and outside of the pelvis. The approach involves retroperitoneal access below the inguinal ligament to preserve the integrity of the inguinal canal and allows exposure of anterior and medial wall fractures as well as the anterior hip capsule. Despite the dimensions of the procedure, closure is anatomical because repair of the inguinal canal floor is not required. We report the use of this approach in 17 patients with T-type fractures of the acetabulum, transverse fractures of acetabulum, Tile C1 pelvic fractures, malunion of the pelvis and acetabular dysplasia. The approach is recommended in complex cases of acetabular surgery (including obese or muscular patients) and complicated or old fractures in which a better exposure is required. It is useful in visualisation of the anterior wall and labrum and intra articular structures, and in viewing the internal anatomy of the pelvis and acetabulum. It may be a suitable approach for periacetabular osteotomy.

Sudden peroneal nerve palsy in a varus arthritic knee.

Peroneal nerve palsy has been reported in association with traumatic and nontraumatic causes. We encountered a 75-year-old man whose peroneal nerve palsy developed suddenly following varus deformity of the arthritic knee. A review of the literature found 1 other report describing a progressive peroneal nerve palsy associated with a varus deformity of the knee due to arthritis. Our patient had progressive intractable knee pain; 3-compartment, severe degenerative changes in the knees; varus knee malalignment and laxity; right peroneal nerve palsy; and decreased sensation to light touch and pinprick on the dorsum of the right foot. The preoperative WOMAC score was 36. Nerve conduction studies confirmed acute peroneal neuropathy with conduction block at the fibular neck and secondary axonal degeneration. Magnetic resonance imaging of the knee showed osteophytes and cysts surrounding the fibular neck. Although their compression could be responsible for the nerve palsy, the sudden process made this less possible. Thus, the patient underwent total knee arthroplasty of both knees without exploration of the nerve. At 5-month follow-up, the WOMAC score was 78. The patient walked with a cane with no varus thrust, and his right knee had no varus laxity in full extension. The peroneal nerve did not retain its function. Sensory examination and postoperative nerve conduction studies showed no improvement.