Complications of single growing rod constructs in the treatment of severe early-onset scoliosis: a lesson relearned.

PURPOSE: Early-onset scoliosis (EOS) is one of the most challenging areas of orthopedic management. Previous studies have reported that EOS patients were associated with high risk of complications following growth-friendly surgery. This study was performed to evaluate the complications of single traditional growing rods (TGRs) in the treatment of EOS. METHODS: In a retrospective chart review study, medical records of all EOS patients treated with single TGRs between 2006 and 2018 were analyzed. Patients under the age of 10 at the time of surgery who had at least 24 months of follow-up were included. Intra- and post-operative complications included both device-related and disease-related complications. Statistical analysis was performed with SPSS. RESULTS: A total of 35 patients with a mean age of 5.7 ± 2.1 years with the mean follow-up duration of 33.3 ± 9.1 months were included in the final analysis. Of the 35 participants, 27 children (77.1%) experienced at least one complication. A total of 61 complications were observed, giving rise to 1.7 complications per patient. An unplanned surgical procedure was needed to manage 42 of the 61 complications (68.8%). Thirty-five cases of implant failure, 11 cases of deep infection, and 2 cases of junctional kyphosis were identified. CONCLUSION: It seems that even in the setting that the use of dual TGRs is not possible, the use of single TGRs as the only therapeutic modality should be minimized due to high rate of complications even as a bridge treatment. LEVEL OF EVIDENCE: Therapeutic level III.

Morbidity and mortality of Iranian patients with hyper IgM syndrome: a clinical analysis.

BACKGROUND: Defects in B cell class switch recombination (CSR) are a heterogeneous and yet very uncommon group of disorders which all have a genetic basis uniformly leading to hyper IgM (HIgM) syndrome. Due to the rare frequency of these conditions, a very small number of case series have been conducted on the affected patients. OBJECTIVE: To shed some light on the morbidity and mortality regarding a relatively large cohort of diagnosed CSR defective Iranian patients. METHODS: This study was performed using demographic information, laboratory findings and clinical data obtained from an observation of 33 Iranian patients of different ethnicities referred from all medical centers of Iran to the Children's Medical Center Hospital, pediatrics center of excellence, Tehran, Iran; of which 28 were males and 5 were females. RESULTS: Our patients mean age at the onset of symptoms was 1.8 ± 0.2 years; they were diagnosed with a mean delay of 4.4 ± 3.3 years and followed for a mean time of 5.7 ± 4.8 years. The most prominent clinical features observed were multi-organ infections, affecting mostly the respiratory system, followed by lymphoproliferative and autoimmune disorders, the latter being of much higher frequency (44%) in our study than the reported frequency in previous reports. The three year survival rate for our enrolled patients was 67.9%. CONCLUSIONS: Based on our findings, the most common cause of death in HIgM patients is respiratory failure. The molecular mechanism behind the nature of the CSR defective patients in Iran is more compatible with autosomal recessive mutations rather than X-linked HIgM syndrome which is in contrast with other large cohorts of patients with CSR defect.

Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry.

BACKGROUND: Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase the awareness of medical personnel as well as health care providers. METHODS: This study presents the demographic data and clinical manifestations of Iranian PID patients who were diagnosed from March 2006 till the March of 2013 and were registered in Iranian PID Registry (IPIDR) after its second report of 2006. RESULTS: A total number of 731 new PID patients (455 male and 276 female) from 14 medical centers were enrolled in the current study. Predominantly antibody deficiencies were the most common subcategory of PID (32.3 %) and were followed by combined immunodeficiencies (22.3 %), congenital defects of phagocyte number, function, or both (17.4 %), well-defined syndromes with immunodeficiency (17.2 %), autoinflammatory disorders (5.2 %), diseases of immune dysregulation (2.6 %), defects in innate immunity (1.6 %), and complement deficiencies (1.4 %). Severe combined immunodeficiency was the most common disorder (21.1 %). Other prevalent disorders were common variable immunodeficiency (14.9 %), hyper IgE syndrome (7.7 %), and selective IgA deficiency (7.5 %). CONCLUSIONS: Registration of Iranian PID patients increased the awareness of medical community of Iran and developed diagnostic and therapeutic techniques across more parts of the country. Further efforts must be taken by increasing the coverage of IPIDR via electronically registration and gradual referral system in order to provide better estimation of PID in Iran and reduce the number of undiagnosed cases.