Twenty-Year Experience of a Single Referral Center on Pediatric Familial Mediterranean Fever: What Has Changed Over the Last Decade?

BACKGROUND/OBJECTIVE: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease and is characterized by recurrent fever and serositis episodes. We aimed to share our 20-year FMF experience, clarify a phenotype-genotype correlation, and compare the characteristics and outcomes of pediatric FMF patients over the last 2 decades in this study. METHODS: This medical record review study included 714 pediatric FMF patients (340 females, 374 males), diagnosed by Tel Hashomer diagnostic criteria between January 2009 and January 2019 and followed up in our department. Demographic and disease characteristics, obtained from medical records of the patients, were compared between patients with M694V homozygosity and other genotypes and showed whether they were diagnosed before (n = 137) or after January 2010 (n = 577). χ2, Student t, and Mann-Whitney U tests were used to compare categorical and continuous variables between these groups. RESULTS: The most common symptoms were abdominal pain (92%), fever (89.5%), and arthralgia (64.5%). Mean ages at symptom onset and diagnosis were 5.16 ± 3.73 and 7.71 ± 3.87 years, respectively. M694V homozygosity was recorded in 111 patients (15.5%). Fever, arthralgia, arthritis, myalgia, erysipela-like erythema, colchicine resistance, and subclinical inflammation were more frequent, and mean disease severity score was higher in patients with M694V homozygosity. Fever, chest pain, and proteinuria were statistically more frequent in patients diagnosed before January 2010. Although M694V homozygosity rate was similar, patients diagnosed in the last decade had lower mean disease severity score. CONCLUSIONS: With this study, we speculate that although genotype and delay in diagnosis were similar, patients diagnosed in the last decade have a milder disease severity.

Efficacy of Magnesium Sulfate Treatment in Children with Acute Asthma.

OBJECTIVE: Systemic administration of magnesium sulfate (MgSO4) has been proposed as a treatment for pediatric patients with acute asthma. However, previous trials show mixed results and uncertain evidence of benefit. The aim of the study was to ascertain whether intravenous (IV) MgSO4 improves lung function parameters in children with acute asthma. METHODS: This was a prospective clinical trial. All patients with acute asthma received 40-50 mg/kg or maximum 1,500 mg (>30 kg) of single dose IV MgSO4, administered over 60 min. Spirometry was conducted before and 15 min after MgSO4 infusion. RESULTS: One hundred and fifteen children aged 6 to 17 years presenting with acute asthma and FEV1 between 40% and 75% of predicted were included. Then, the patients were classified into 2 groups; mild asthma attack (FEV1 ranged from 60% to 75%; n = 50) or moderate asthma attack (FEV1 ranged from 40% to 59%; n = 65). The baseline characteristics were similar in both groups. The mean percent predicted pre and post values for FEV1/FVC ratio (mild group: 82.59 ± 9.46 vs. 85.06 ± 8.95; moderate group: 77.31 ± 11.17 vs. 79.99 ± 11.77), FEV1 (mild group: 67.14 ± 4.99 vs. 72.29 ± 8.05; moderate group: 48.50 ± 6.81 vs. 53.78 ± 9.81), PEF (mild group: 65.49 ± 12.32 vs. 71.37 ± 12.96; moderate group: 47.56 ± 11.78 vs. 51.97 ± 13.98), and FEF25-75 (mild group: 58.20 ± 12.24 vs. 66.57 ± 16.95; moderate group: 37.77 ± 11.37 vs. 43.41 ± 14.19) showed a statistically significant (p < 0.05 for all) bronchodilator effect after MgSO4 infusion in both groups with few side effects. CONCLUSION: Administration of IV MgSO4 was associated with improved pulmonary function in children with acute asthma.

Clinical manifestations and outcomes of 420 children with Henoch Schönlein Purpura from a single referral center from Turkey: A three-year experience.

Objectives: Henoch Schönlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients.Methods: We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses.Results: The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 ± 3.15 years. Clinical manifestations were arthralgia and/or arthritis (n = 244, 58.1%), abdominal pain (n = 235, 56%), subcutaneous edema (n = 163, 38.8%), and renal involvement (n = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration.Conclusion: We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease.

Retrospective analyzes of adverse events during biologic agents in children with juvenile idiopathic arthritis from a single center in Turkey.

OBJECTIVES: Juvenile idiopathic arthritis is the most common rheumatic disease in childhood. Biologic agents have changed the course of juvenile idiopathic arthritis. However, there are concerns regarding the occurrence of serious adverse events in patients receiving biologic agents. The aim of this study was to evaluate adverse events in children with juvenile idiopathic arthritis receiving biologic agents. MATERIAL AND METHODS: This retrospective study includes juvenile idiopathic arthritis patients receiving biologic agents. Demographic features and adverse events during biologic agents were collected from medical files. Adverse events that either resulted in death, were life-threatening, required inpatient hospitalization, or resulted in persistent or significant disability/incapacity were considered as serious adverse events. RESULTS: In total, 162 juvenile idiopathic arthritis patients (55.6% female) receiving biologic agents were enrolled: 101 (62.3%) patients treated with etanercept, 27 (16.7) with tocilizumab, 14 (8.6%) with adalimumab, 15 (9.2%) with anti-interleukin 1 agents (13 canakinumab, 2 anakinra), and 5 (3.1%) with infliximab. 75.9% of the patients received concomitantly disease-modifying anti-rheumatic drugs, and 20.4% received disease-modifying anti-rheumatic drugs plus corticosteroid. The mean age at initiation of the biologic agent was 10.5 ±4.3 years. The mean age at the study enrolment was 12.1 ±4.5 years. The mean follow-up duration was 19.7 ±2.1 months. The most frequent adverse event was upper respiratory tract infections (54.3%) followed by urinary tract infections (21%). Anaphylaxis occurred in 3 patients (1.9%): 2 with tocilizumab and one with infliximab. Macrophage activation syndrome occurred in 1 patient (0.6%) receiving tocilizumab. Lung tuberculosis developed in 2 patients (1.2%) receiving canakinumab. The frequency of serious adverse events in total was 6.7%. CONCLUSIONS: While the most frequent adverse events during biologic agents was upper respiratory tract infections, the frequency of serious adverse events was 6.7%; therefore, juvenile idiopathic arthritis patients receiving biologic agents should be carefully evaluated for these adverse events in clinical practice.

Is Henoch-Schönlein purpura a susceptibility factor for functional gastrointestinal disorders in children?

Henoch-Schönlein purpura (HSP), the most common childhood vasculitis is characterized by non-thrombocytopenic palpable purpura, arthritis/arthralgia, abdominal pain and renal involvement. Functional gastrointestinal disorders (FGIDs) are heterogeneous disease spectrum with unclear etiology and include the most common subtypes: functional dyspepsia, irritable bowel syndrome (IBS), functional abdominal pain and functional constipation. Formerly, FGIDs were known as non-organic disorders; however, recent advances revealed that low-grade inflammation may also play a role. We aimed to clarify whether HSP predisposes to FGIDs in pediatric population. Seventy-four children with HSP, diagnosed at least 6 months before the study and 78 healthy controls were enrolled to the study. Patients with red flag signs for organic GI disorders were excluded. Rome IV criteria were utilized for FGIDs diagnosis. We compared the frequencies of FGIDs between HSP patients and healthy subjects. We also examined the parameters including age, abdominal pain, arthralgia, bloody stool, renal involvement and treatment with corticosteroids and laboratory results at HSP diagnosis such as erythrocyte sedimentation rate, C-reactive protein, hemoglobin, leukocytes and platelet counts among patients with and without FGIDs. Overall FGIDs and IBS frequency were 35.1% (n = 26) and 10.8% (n = 8) in HSP patients, 19.2% (n = 15) and 2.6% (n = 2) in healthy controls, respectively. Disease characteristics and laboratory parameters at disease onset were similar between HSP patients with and without FGIDs. Overall FGIDs rate, particularly IBS were statistically higher in HSP patients. We speculate that children with preceding HSP may be predisposed to FGIDs. Since the FGIDs pathogenesis is still remains unclear, further studies are needed to confirm this hypothesis and clarify the etiology. Physicians also should pay attention to FGIDs in HSP patients with ongoing abdominal pain and thus prevent this comorbidity with dietary and psychologic measures.

Hyperimmunoglobulinemia D syndrome with recurrent perianal abscess successfully treated with canakinumab.

Hyperimmunoglobulinaemia D syndrome is an autoinflammatory disease usually representing recurrent episodes of fever, arthralgia/arthritis, cervical lymphadenopathy, vomiting, diarrhoea, abdominal pain and skin rashes lasting 3-7 days every 4-8 weeks since their infancy. Recent reports suggested a link between perianal fistulae/abscess and severe colitis with hyperimmunoglobulinaemia D syndrome resembling an inflammatory bowel disease phenotype. Herein, we report an 18-month-old patient with recurrent attacks of fever and pharyngitis lasting 2-3 days every 10-15 days since the first two weeks of life. Inflammatory attacks were accompanied by diarrhoea, oral aphthous ulcers, cervical lymphadenopathy, maculopapular rash, severe leukocytosis and perianal fistulae/abscess. After the initiation of canakinumab, the patient was clinically improved with complete healing of perianal fistulas/abscesses. In conclusion, hyperimmunoglobulinaemia D syndrome should be considered in differential diagnosis of inflammatory bowel disease and recurrent perianal abscess/fistula in a patient with inflammatory attacks.

Recurrent Henoch Schönlein purpura without renal involvement successfully treated with methotrexate.

INTRODUCTION: Henoch Schönlein purpura is characterised by palpable purpura, abdominal pain, arthritis/arthralgia, often with a self-limiting course. Herein, we report a patient with recurrent Henoch Schönlein purpura and severe gastrointestinal involvement, successfully treated with methotrexate. CASE PRESENTATION: A 12-year-old boy was admitted to our department with palpable purpura, abdominal pain and arthralgia. Since gastrointestinal complaints were severe, systemic steroids were administered, with tapering of dosage. Henoch Schönlein purpura recurred several times with severe abdominal pain, maelena and purpura during next two months. Colchicine and hydroxychloroquine were initiated. After four months, we also introduced methotrexate, which enabled discontinuation of previous medications including corticosteroids. Methotrexate was ceased four months later, and remission was sustained without any medications for 24 months. CONCLUSION: Besides the conflicting data regarding the use of methotrexate in recurrent Henoch Schönlein purpura, our case introduces successful methotrexate experience in a child with Henoch Schönlein purpura and recurrent severe gastrointestinal involvement.

Symptomatic multifocal avascular necrosis in an adolescent with neuropsychiatric systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by malar rash, oral ulcers, arthralgia, photosensitivity and nephritis. Herein, we report a rare comorbidity, multiple avascular necrosis (AVN), in an adolescent SLE patient and also highlight the importance of risk factors for this comorbidity with a brief literature review. A 13-year-old female patient was admitted with severe headache, visual plus auditory hallucinations, polyarthritis and a history of recurrent oral ulcers. Acneiform malar rash, arthritis, cytopenia, low complement levels and autoantibody positivity yielded SLE diagnosis. We diagnosed her as having multifocal AVN after the 4th dose of cyclophosphamide, with bilateral knee pain and swelling and typical geographical lesions on magnetic resonance imaging. Avascular necrosis is a rare comorbidity of SLE and neuropsychiatric involvement, cyclophosphamide administration and severe disease may be the possible risk factors in addition to corticosteroid use. Further multicenter studies investigating the possible risk factors of AVN with a large number of patients are needed.

Squamous Cell Carcinoma With Hyper-IgE Syndrome: A Case Report.

BACKGROUND: Hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disease characterized by recurrent infections and elevated levels of serum immunoglobulin E, usually over 2000 IU/mL. Recurrent and chronic infection of the epidermis and squamous epithelium may also be a cause of squamous cell carcinoma (SCC). SCC is rare with HIES. CASE REPORT: A 17-year-old male patient who was diagnosed as HIES was admitted with purulent right ear discharge. The patient had a history of eczema starting from the age of 7 months and a history of recurrent middle ear infection starting from the age of 5. Biopsy specimens were taken from the lesion in the external auditory canal, and the lesion was reported as SCC. CONSLUSION: Patients with autosomal recessive HIES are at an increased risk for infections and malignancies. SCC should be considered in the differential diagnosis of the patients presenting with recurrent middle ear infections and immunodeficiency.

Decreased serum vitamin B12 and vitamin D levels affect sleep quality in children with familial Mediterranean fever.

Familial Mediterranean fever is an autoinflammatory disorder characterized by recurrent fever and serositis. We aimed to describe serum vitamin B12 and vitamin D levels and their correlation with self-reported sleep quality of pediatric FMF patients. Sixty-three children with FMF were enrolled to the study. Information on sleep quality was obtained using self-administration of Pittsburg Sleep Quality Index (PSQI). The patients were divided into subgroups depending on vitamin D serum concentrations: ≥ 20 and < 20 ng/ml. We also grouped patients according to their serum B12 concentration: ≥ 200, < 200 pg/ml. B12 levels were not correlated with PSQI scores, whereas significant correlation was found between vitamin D and total PSQI scores and daytime sleepiness. Total PSQI score, sleep disorders and daytime sleepiness sub-scores were statistically high in patients with serum D vitamin levels below 20 ng/ml. Sleep efficiency was found lower in patients with serum B12 levels below 200 pg/ml. B12 may have a positive role on effective sleep. More importantly, we suggest that vitamin D is protective against sleep disorders and poor sleep, it may also improve daytime activities.

The Trend of Change of Allergic Diseases over the Years: Three Repeated Surveys from 1994 to 2014.

BACKGROUND: Although it is known that there has been an increase in asthma and allergic diseases among school-aged children, results vary between countries. The aim of this study was to examine trends in the prevalence of these diseases in the city of Adana (south Turkey) over the last 20 years based on the results of 3 cross-sectional studies. METHODS: Three cross-sectional surveys were performed 10 years apart in south Turkey. Here, we compare the surveys conducted among children between the ages of 6 and 14 years from 1994, 2004, and 2014. The participants were randomly chosen among children attending participating primary schools in Adana. The questionnaire in the first survey contained almost the same questions as the ISAAC. In the 2 latter surveys, we used the ISAAC questionnaire to investigate the prevalence of asthma and other allergic diseases. RESULTS: A total of 2,334 children in 1994, 3,728 children in 2004, and 3,209 children in 2014 were included in the final analysis. The results showed a statistically significant increase in the prevalence of physician-diagnosed allergic diseases across the years, respectively: asthma (5, 4.6, and 8.9%), allergic rhinitis (8.8, 11.4, and 15.6%), and atopic dermatitis (5, 9.9, and 7%). In contrast, the prevalence of parent-reported current wheeze (16.2, 13.2%) decreased in the last 10 years. CONCLUSIONS: Our findings were consistent with the increased prevalence of childhood asthma and other allergic diseases. However, this pattern did not occur in respiratory symptoms.

A Case of Idiopathic Pulmonary Hemosiderosis Presenting With Signs and Symptoms Mimicking Hemolytic Anemia.

Idiopathic pulmonary hemosiderosis is primarily a disorder of childhood, which is characterized by hemoptysis, iron deficiency anemia, and diffuse parenchymal infiltrates on chest x-ray secondary to recurrent attacks of alveolar hemorrhage. It can be diagnosed by showing hemosiderin laden macrophages in bronchoalveolar lavage fluid after other specific causes of diffuse alveolar hemorrhage are definitely excluded. A 5-year-old male patient was admitted to our clinic with sudden-onset pallor during iron therapy given for anemia. While he was being investigated for clinical and laboratory signs mimicking hemolytic anemia, he developed cough and dyspnea. He had infiltrates on chest x-ray and scattered patchy infiltrates in both lungs on high-resolution computed tomography. Hemosiderin laden macrophages were identified in fasting gastric juice and bronchoalveolar lavage fluid. The patient was diagnosed with idiopathic pulmonary hemosiderosis and started corticosteroid therapy.

Oxygen saturation/minute heart rate index: Simple lung function test for children.

BACKGROUND: The severity of airway obstruction can be accurately determined on spirometry in children with asthma. Other assessments may include peak expiratory flow and pulse oximetry. In the present study, we evaluated the validity and reliability of oxygen saturation/minute heart rate (SpO2 /MHR) index in the prediction of degree of severe airway obstruction in children with asthma. METHODS: This was a retrospective study of children aged 7-17 followed for asthma at Mersin Women and Children's Hospital. The study compared SpO2 /MHR ratio with forced expiratory volume in 1 s (FEV1 ) measured on spirometry, an important indicator of small airway obstruction. A total of 296 patients were included in the study, and classified either as having normal FEV1 (FEV1 > 80% of predicted, n = 178) or severely reduced FEV1 (FEV1 < 60% of predicted, n = 118). Positive and negative predictive values (PPV and NPV), sensitivity and specificity of SpO2 /MHR index in predicting low FEV1 were calculated on receiver operating characteristics analysis. RESULTS: An SpO2 /MHR ratio cut-off <0.90 was associated with a PPV of 83.14%, NPV of 71.77%, sensitivity of 80.34% and specificity of 75.42% in predicting low FEV1 . CONCLUSIONS: SpO2 /MHR ratio appears to be a highly useful index to assess airway obstruction in older children with asthma. Thus, it can be used as a marker of airway obstruction severity when spirometry is not available.

Allergic sensitization in kidney transplanted patients - Is it a result of immunosuppressive agents or the sensitization of living donor?

BACKGROUND: Allergic sensitization has been reported increasingly in organ transplant recipients. However, the pathogenesis of this sensitization has still not been clearly understood. OBJECTIVE: The aim of this study was to evaluate allergic sensitization in kidney transplanted children and adolescents under immunosuppressive treatment. METHODS: Twenty seven kidney-transplanted subjects were studied by standardized interviews from the International Study of Asthma and Allergies in Childhood criteria, skin prick test (SPT) and measurement of specific immunoglobulin E (s-IgE). Patients were considered to have allergic sensitization when presenting a positive SPT and/or s-IgE >0.35 kUA/l to at least one of the tested allergens. Patients with a history of allergic diseases accompanied by sensitization were accepted as allergic. We also performed SPT on the living donors of the allergic groups. RESULTS: Seven patients (25.9%) were found to be sensitized to ≥1 common inhalant and 3 subjects (11.1%) additionally reported a corresponding present history of allergic diseases. All of the living donors' sensitized patients were allergic. New-onset post-transplantation food allergy was not documented in any patients. CONCLUSIONS: This study supports the concept that not only immunosuppressant agents but also sensitization of living donors could be a significant contributor to allergic sensitization in kidney recipients.

The ADAPAR Birth Cohort Study: Food Allergy Results at Five Years and New Insights.

BACKGROUND: Although food allergy (FA) is often a transient condition during childhood, when and in whom FA will resolve can be affected by many factors. In this study, we analyzed the data at 5 years on 33 children diagnosed with FA in the ADAPAR (Adana Pediatric Allergy Research) birth cohort study in southern Turkey. METHODS: Thirty-three infants detected as having FA at the end of their first year in the ADAPAR study were assessed every 6 months until the age of 5 years. Each follow-up included a clinical examination, questionnaire, blood sampling and a skin-prick test. RESULTS: Culprit allergens were cow's milk (n = 20), eggs (n = 17), chicken meat (n = 1) and bananas (n = 1). Of the 17 patients with egg allergy, 14 developed complete tolerance and 1 developed partial tolerance (i.e. tolerance to baked food). Of the 20 patients with milk allergy, complete tolerance was observed in 16 and partial tolerance in 1. The mean age of tolerance to egg was 22.4 ± 7.5 months and to cow's milk, it was 20.9 ± 1.1 months. Complete tolerance developed in 1 case allergic to chicken meat and in 1 case allergic to banana. Other allergic conditions were also determined: allergic rhinitis in 27.2%, atopic dermatitis in 21.2%, asthma in 9%, urticaria in 9% and drugs in 9%. CONCLUSIONS: Our results confirm early and high tolerance rates before school age in children with food allergies that started in infancy. This will help pediatricians to give more informed advice to parents of infants with cow's milk or hen's egg allergy.

Clinical Features of Food Allergy during the 1st Year of Life: The ADAPAR Birth Cohort Study.

BACKGROUND: Although food allergies (FAs) occur most commonly during the 1st year of life, there is limited information available regarding the epidemiology of FAs. In this study, we investigated the incidence of FA and the associated risk factors during the 1st year of life in southeast Turkey. METHODS: This study is a prospective evaluation of 1,377 infants born at the Balcali Hospital (Çukurova University) and includes four routine follow-up assessments until the age of 1 year. At birth, a physical examination was performed, cord blood samples were taken, and parents completed a baseline questionnaire. Follow-up visits were scheduled at 3, 6 and 12 months and included the infants' physical examination and a follow-up questionnaire. A skin prick test (SPT) was performed and food-specific IgE levels were measured at 6 and 12 months. Telephone interviews were conducted when the infants were 9 months of age, and the questionnaire was administered. The diagnosis of FA was based on food-specific IgE levels, positive SPT results, associated clinical findings and an oral food challenge (OFC) test. RESULTS: Patient histories, physical examinations and laboratory results indicated a possible FA in 90 infants (6.5%) during the 1st year of life. All of them underwent OFC testing with the suspected foods, and FA was confirmed in 33 cases (2.4%). Cow's milk allergy was the major cause of FA. Skin reactions were major clinical findings in FA. A family history of atopy was identified as the major risk factor for FA. CONCLUSIONS: The prevalence and risk factors of FA in our region are consistent with those reported in the literature.

Newborn Screening for Cystic Fibrosis in Mersin Province: Yearly Assessment of the National Program.

OBJECTIVES: A national newborn screening program for cystic fibrosis (CF) was started using immunoreactive trypsinogen (IRT) test on January 1, 2015, in Turkey. We aimed to analyze the characteristics of newborn screen-positive (NBSP) infants in Mersin province. MATERIALS AND METHODS: The data on NBSP infants were retrospectively analyzed between 2015 and 2017 from records of Mersin Women & Children's Hospital and Mersin City Training and Research Hospital. RESULTS: A total of 82,273 newborns were screened for CF by IRT test between January 2015 and December 2017 in Mersin. Among those, 512 infants were defined as NBSP after two repeated IRT tests (IRT/IRT) (138 infants in 2015, 217 in 2016, and 157 in 2017). Sweat test was normal in the majority of infants (115 infants [83.3%] in 2015, 189 [87.1%] in 2016, and 129 [82.2%] in 2017). Overall, between 2015 and 2017, after two repeated sweat tests, 4 infants had sweat test results in the intermediate range and 9 infants had positive sweat tests. The incidence of CF for a 3-year period was approximately 1/9300 in our region. The positive predictive value of IRT test for defining CF was 1.8%, with a sensitivity of 90.0% and specificity of 99.4%. CONCLUSION: IRT/IRT test as a newborn screening strategy provides the opportunity for earlier diagnosis and treatment of CF patients. More data are needed to understand the frequency of CF on a national level.

Growth Parameters of Turkish Children With an Autoinflammatory Disease Before and After Canakinumab Treatment.

OBJECTIVE: To evaluate the effect of canakinumab on growth parameters of patients with autoinflammatory diseases. METHODS: This retrospective study included Colchicine resistant familial Mediterranean fever (FMF), Mevalonate kinase deficiency (MKD), Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS), Deficiency of adenosine deaminase 2 (DADA2) patients treated with canakinumab for at least six consecutive months. RESULTS: Eleven patients with FMF, 9 with MKD, 3 with TRAPS, and 1 with DADA2 were included. The median age (range) at diagnosis and drug initiation was 6.06 (1.45-16.06) years and 9.72 (1.82-19.11) years, respectively. The mean weight, height, and BMI SD scores significantly increased after canakinumab. There were significant improvements in laboratory parameters and disease activities. However, growth parameters after the drug did not differ according to gender, the duration of diagnostic delay, and age at the diagnosis. CONCLUSIONS: Canakinumab seems to have a positive effect on growth in patients with autoinflammatory diseases by controlling disease activity and inflammation.

Retrospective Analysis of the Factors Affecting Growth Parameters in Turkish Children With Systemic Lupus Erythematosus.

OBJECTIVES: This study aims to investigate the growth parameters in Turkish children with systemic lupus erythematosus (SLE) and to compare these growth parameters according to the presence or absence of cyclophosphamide, rituximab treatment, cumulative corticosteroid dose, proliferative nephritis, and the last visit disease activity and damage index. PATIENTS AND METHODS: Medical data, growth parameters including z-scores for weight, height, and body mass index and parent-adjusted height z-scores of 45 juvenile SLE (jSLE) patients (5 males, 40 females; mean age 12.3±3.2 years; range 7.1 to 18 years) were retrospectively evaluated. Growth parameters were calculated by anthropometric references in Turkish children. The disease activity was assessed by the SLE Disease Activity Index 2000 (SLEDAI-2K). The disease outcome was measured by the Pediatric version of Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. RESULTS: The median diagnostic delay was two months (range, 0-36 months). The median follow-up duration was 3.21 years (range, 0.63-9.48 years). Mean height z-score was significantly lower at last visit than at the time of diagnosis. The growth parameters did not differ according to age at disease onset, diagnostic delay, presence of proliferative nephritis, having cyclophosphamide and rituximab treatment and the last visit (SLEDAI-2K) scores. The median duration of corticosteroid treatment was 3.2 years (range, 0.6-9.4 years) and the median cumulative corticosteroid dosage was 13.9 g (range, 1.9-58 g) of methylprednisolone. The mean height z-score at last visit was significantly lower in those who received at least 10 g of cumulative dose of corticosteroid. The last visit mean parent-adjusted height z-scores did not differ significantly regarding the cumulative corticosteroid dose. CONCLUSION: The last visit height and parent-adjusted height z-scores of jSLE patients were significantly lower. The patients treated with at least 10 g of cumulative dose of corticosteroids had lower mean height z-score.