Visual Perception in Children with a History of Hypoglycemia due to Hyperinsulinism.

Objectives: Hyperinsulinism refers to improper insulin secretion in the presence of low plasma glucose, causing severe and persistent hypoglycemia in infants and children. The brain's occipital lobe, which includes the visual and plays an essential role in visual perception is specifically sensitive to hypoglycemia-induced damage. The present study aims to investigate the visual perception in children suffering from hyperinsulinism and to compare it with the control group. Materials & Methods: This cross-sectional control study, conducted in 2020 in Isfahan, Iran, involved 20 children aged 4-13 years with hyperinsulinism and 20 healthy children of the same age and gender for comparison. In both groups, the measuring instrument was the Test of Visual Perceptual Skills (non-motor) Third Edition. Results: The mean visual perceptual quotient in the case and control groups was 80.50±26.74 and 116.50±7.56 (p-value<0.001), respectively. The results overall indicated that children suffering from hyperinsulinism were weaker than healthy children in all areas of visual perception. Conclusion: Based on the obtained results, it is recommended that children suffering from hyperinsulinism be screened regarding visual perceptual disorders since this screening may be helpful in initiating different rehabilitation programs among these patients.

Prediction of breath-holding spells based on electrocardiographic parameters using machine-learning model.

BACKGROUND: Breath-holding spells (BHS) are common in infancy and early childhood and may appear like seizures. Factors such as autonomic dysfunction and iron deficiency anemia are thought to contribute to the incidence of BHS. In this study, electrocardiographic (ECG) parameters of patients with BHS were compared to those of healthy, normal children. Logistic regression and machine-learning (ML) models were then created to predict these spells based on ECG characteristics. METHODS: In this case-control study, 52 BHS children have included as the case and 150 healthy children as the control group. ECG was taken from all children along with clinical examinations. Multivariate logistic regression model was used to predict BHS occurrence based on ECG parameters. ML model was trained and validated using the Gradient-Boosting algorithm, in the R programming language. RESULTS: In BHS and control groups, the average age was 11.90 ± 6.63 and 11.33 ± 6.17 months, respectively (p = .58). Mean heart rate, PR interval, and QRS interval on ECGs did not differ significantly between the two groups. BHS patients had significantly higher QTc, QTd, TpTe, and TpTe/QT (all p-values < .001). Evaluation of the ML model for prediction of BHS, fitting on the testing data showed AUC, specificity, and sensitivity of 0.94, 0.90, and 0.94 respectively. CONCLUSION: There are repolarization changes in patients with BHS, as the QTc, QTd, TpTe, and TpTe/QT ratio were significantly higher in these patients, which might be noticeable for future arrhythmia occurrence. In this regard, we developed a successful ML model to predict the possibility of BHS in suspected subjects.

Assessing Leg Length Discrepancy Is Necessary Before Arthroplasty in Patients With Unilateral Crowe Type IV Hip Dislocation.

BACKGROUND: THA for high-riding developmental dysplasia of the hip (DDH) is challenging in terms of length equalization. Although previous studies suggested preoperative templating on AP pelvic radiographs is insufficient in patients with unilateral high-riding DDH because of hypoplasia of the hemipelvis on the affected side and unequal femoral and tibial length on scanograms, the results were controversial. The EOS™ (EOS™ Imaging) is a biplane X-ray imaging system using slot-scanning technology. Length and alignment measurements have been shown to be accurate. We used the EOS to compare the lower limb length and alignment in patients with unilateral high-riding DDH. QUESTIONS/PURPOSES: (1) Is there an overall leg length difference in patients with unilateral Crowe Type IV hip dysplasia? (2) In patients with unilateral Crowe Type IV hip dysplasia with an overall leg length difference, is there a consistent pattern of abnormalities in the femur or tibia that account for observed differences? (3) What is the impact of unilateral high-riding Crowe Type IV dysplasia on femoral neck offset and knee coronal alignment? METHODS: Between March 2018 and April 2021, we treated 61 patients with THA for Crowe Type IV DDH (high-riding dislocation). EOS imaging was performed preoperatively in all patients. Eighteen percent (11 of 61) of the patients were excluded because of involvement of the opposite hip, 3% (two of 61) were excluded for neuromuscular involvement, and 13% (eight of 61) had previous surgery or fracture, leaving 40 patients for analysis in this prospective, cross-sectional study. Each patient's demographic, clinical, and radiographic information was collected with a checklist using charts, Picture Archiving and Communication System, and an EOS database. EOS-related measurements that were related to the proximal femur, limb length, and knee-related angles were recorded for both sides by two examiners. The findings of the two sides were statistically compared. RESULTS: The overall limb length was not different between the dislocated and nondislocated sides (mean 725 ± 40 mm versus 722 ± 45 mm, mean difference 3 mm [95% CI -3 to 9 mm); p = 0.08). Apparent leg length was shorter on the dislocated side (mean 742 ± 44 mm versus 767 ± 52 mm, mean difference -25 mm [95% CI -32 to 3 mm]; p < 0.001). We observed that a longer tibia on the dislocated side was the only consistent pattern (mean 338 ± 19 mm versus 335 ± 20 mm, mean difference 4 [95% CI 2 to 6 mm]; p = 0.002), but there was no difference between the femur length (mean 346 ± 21 mm versus 343 ± 19 mm, mean difference 3 mm [95% CI -1 to 7]; p = 0.10). The femur of the dislocated side was longer by greater than 5 mm in 40% (16 of 40) of patients and shorter in 20% (eight of 40). The mean femoral neck offset of the involved side was shorter than that of the normal side (mean 28 ± 8 mm versus 39 ± 8 mm, mean difference -11 mm [95% CI -14 to -8 mm]; p < 0.001). There was a higher valgus alignment of the knee on the dislocated side with a decreased lateral distal femoral angle (mean 84° ± 3° versus 89° ± 3°, mean difference - 5° [95% CI -6° to -4°]; p < 0.001) and increased medial proximal tibia angle (mean 89° ± 3° versus 87° ± 3°, mean difference 1° [95% CI 0° to 2°]; p = 0.04). CONCLUSION: A consistent pattern of anatomic alteration on the contralateral side does not exist in Crowe Type IV hips except for the length of the tibia. All parameters of the limb length could be shorter, equal to, or longer on the dislocated side. Given this unpredictability, AP pelvis radiographs are not sufficient for preoperative planning, and individualized preoperative planning using full-length images of the lower limbs should be performed before arthroplasty in Crowe Type IV hips. LEVEL OF EVIDENCE: Level I, prognostic study.

Hip dysfunction-related urinary incontinence and total hip arthroplasty with the direct lateral approach.

OBJECTIVES: There is a functional relationship between the hip joint and the pelvic floor muscles. In patients with secondary osteoarthritis of the hip, urinary incontinence is also seen. Research has shown that total hip arthroplasty (THA) surgery improves the symptoms of urinary incontinence. This prospective cross-sectional study without a control group was performed on THA candidates with urinary incontinence and secondary osteoarthritis with the aim of investigating the effect of THA with a direct lateral approach and subsequent routine physiotherapy on the symptoms of urinary incontinence. METHODS: Sampling was performed using a simple method among those referred to orthopedic clinics in the private sector. Data were collected in all patients before and 3 months after THA using demographic, International Consultation on Incontinence Questionnaire-Short Form (ICIQ-SF), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), and hip range of motion record tables. The results are presented as mean with standard deviation. RESULTS: In this study, 16 men with a mean age of 76.75 years (±4.65) and 8 women with a mean age of 72.75 years (±7.32) participated. Before THA, stress urinary incontinence (54.16%) and urgency urinary incontinence (20.83%) had the highest frequency. However, after THA, the frequency of stress and urgency urinary incontinence decreased (16.66% and 8.33%, respectively). According to the results of the ICIQ, complete improvement of urinary incontinence symptoms was observed in up to 62.5% of the subjects. After THA, there was a significant difference between the mean total score obtained from the WOMAC questionnaire compared to before surgery. There was a significant improvement in the range of motion of the hip joint in all directions. CONCLUSIONS: THA and routine hip physiotherapy in patients with urinary incontinence and secondary hip osteoarthritis have a significant positive effect on improving symptoms of urinary incontinence and hip function. In addition, it significantly improves the ICIQ and WOMAC questionnaire scores.

A Comparative Study on the Efficacy of Coenzyme Q10 and Amitriptyline in the Prophylactic Treatment of Migraine Headaches in Children: A Randomized Controlled Trial.

Background: Migraine, one of the most common headaches in children, has a significant impact on children and their family's quality of life (QoL). There are two approaches for controlling migraine headaches preventative and controlling acute attacks. Several drugs have been used for this purpose, and tricyclic antidepressants were at the top. Amitriptyline has shown not only a desirable effect on controlling the headaches but also some adverse side effects. Recently, finding effective drugs with fewer side effects, become more critical. Among them, nutraceuticals were one of the promising ones. Materials and Methods: In this randomized clinical trial on 72 patients aged 5-15 years old with diagnosis of migraine based on the International Headache Society criteria, we compare the effectiveness of coenzyme Qten on frequency, duration, and severity of childhood migraine. For comparing the QoL, we used the International PedMIDAS questionnaire. Results: Coenzyme Qten showed good therapeutic effects in children, especially in long-term use; however, amitriptyline showed more rapid response. After 3 months of treatment, clinical outcomes in the two groups did not significantly differ from each other. Similarly, Children's QoL increased in the same way. There are more reported side effects in children using amitriptyline compared to coenzyme Qten. Conclusions: According to results, Co-enzyme Q10, with fewer side effects and comparable therapeutic effects, especially in the long term, could be a good drug for prophylactic treatment of migraine headaches.

Impaction Grafting Is Sufficient to Address Acetabular Deficiency During Total Hip Arthroplasty of Most Dysplastic Hips With Over 30% Bone Defect.

BACKGROUND: Addressing acetabular deficiency during arthroplasty of dysplastic hips is challenging. We assessed outcomes of a protocol for choosing either impaction or structural graft for this purpose. METHODS: This retrospective study included 59 patients (71 hips) with a dysplastic hip and over 30% uncoverage that underwent cementless total hip arthroplasty. Morselized impaction grafting was performed for hips where initial stability of the acetabular cup was achieved. In others, a shelf graft was inserted before implantation of the acetabular cup. Outcomes were assessed at a minimum follow-up of 4 years. RESULTS: Fifty-seven (80.3%) hips underwent impaction grafting and 14 (19.7%) received a structural graft. Mean age at surgery was 48.1 ± 13.5 (18-68) years for impaction and 48.6 ± 14 (24-70) years for shelf grafts. Mean increase in Harris Hip Score was 51.5 ± 9.3 and 50 ± 11.2 for the impaction and structural groups, respectively, at a mean follow-up of 92 (49-136) months (P = .6). Heterotopic ossification occurred in 16 patients in the impaction group vs none in the structural group (P = .004). Radiologically, mean percentages of cup coverage provided by the graft were 47.8 ± 10.9% and 48.9 ± 13.3% in the impaction and structural groups, respectively (P = .75). All but one of shelf grafts united to host bone and all impaction grafts incorporated. There was one case of cup loosening in the structural graft group. CONCLUSION: Most dysplastic acetabula with over 30% defect can be addressed using a cementless cup and impaction grafting, with good results in the midterm. In about 20% of cases, initial press-fit is not attainable and structural support-like shelf graft becomes necessary. LEVEL OF EVIDENCE: IV.

Efficacy of Lacosamide Add-on Therapy on Refractory Focal Epilepsies in Children and Adolescents: An Open-Label Clinical Trial.

Objective: Epilepsy is a chronic neurological disorder that affects 0.5%-1% of children. 30%-40% of patients are resistant to current anti-epileptic drugs. Lacosamide (LCM) appeared to be effective, safe, and well tolerated in children and adolescents. This study was aimed to evaluate whether LCM could be an effective add-on therapy in children with refractory focal epilepsies. Methods: This study was conducted from April 2020 to April 2021 in Imam Hossein Children Hospital, Isfahan, Iran. We included 44 children aged 6 months to 16 years with refractory focal epilepsy (based on International League Against Epilepsy criteria). LCM was given in divided doses of 2 mg/kg/day, increasing by 2 mg/kg every week. The first follow-up visit was 6 weeks later, when all patients had reached the therapeutic dose. Findings: The average age of the patients was 89.9 months. 72.5% of children had focal motor seizures. Evaluation of percent change in seizure frequency and duration before and after treatment showed a 53.22% reduction in seizure frequency and 43.72% reduction in seizure duration after treatment. Our study group tolerated LCM well, with few side effects. Headache, dizziness, and nausea were common side effects. In line with other studies, none of the suspected risk factors could predict response to LCM treatment. Conclusion: LCM appears to be an effective, safe, and well-tolerated medication in children with uncontrolled drug-resistant focal epilepsy.

The Comparison of Levetiracetam and Piracetam Effectiveness on Breath-Holding Spells in Children: A Randomized Controlled Clinical Trial.

BACKGROUND: We aimed to compare the effectiveness of Levetiracetam and Piracetam on the severity and frequency of spells in children with severe breath-holding spells (BHS), i.e. bening, paroxysmal, and nonepileptic events that are common in early childhood. MATERIALS AND METHODS: This study is a randomized controlled clinical trial in 71 children from 6 months to 6 years of age with BHS. They were randomly assigned to the two study groups (Levetiracetam and Piracetam group). The frequency and severity of BHS and the response to treatment were recorded on monthly visits during our 3 months follow-up. RESULTS: There was a significant decline in the average number of frequency of spells before and after 3 months of treatment in each group in this study. Levetiracetam had significant effects on the average incidence of the loss of consciousness and seizure-like movements in our study, while Piracetam had no significant effect on the loss of consciousness. Our result showed better response in the Levetiracetam group (88.9% partial or complete response after treatment) compared with the Piracetam group (77.1% partial or complete response after treatment); however, it was not significant. It seems that Levetiracetam had better effect than Piracetam in some aspects in the treatment of BHS. CONCLUSIONS: Both Piracetam and Levetiracetam are safe and had significant effects on the frequency of BHS in our study, however, levetiracetam showed superior effects on the severity of BHS.

A Comparative Study on the Efficacy of Levetiracetam and Carbamazepine in the Treatment of Rolandic Seizures in Children: An Open-Label Randomized Controlled Trial.

OBJECTIVE: This study was performed to investigate whether levetiracetam should be preferred to carbamazepine as a treatment choice for benign childhood epilepsy with centro Temporal spikes (BCECTS), the most common partial epilepsy of childhood. METHODS: This randomized clinical trial study included 92 children with rolandic epilepsy aged 4-12 years referred to the Pediatric Neurology Clinic at Imam Hossein Hospital, Isfahan, Iran, from April 2019 to January 2020. Patients were selected consecutively and randomly assigned to two study groups (levetiracetam and carbamazepine groups). Patients were followed and revisited every 2 months for 6 months after starting the medication. The frequency and duration of seizure attacks and drug side effects were recorded before treatment and in bi-monthly visits. Data were analyzed by SPSS software Version 24 using Mann-Whitney U- test and Friedman test. FINDINGS: In our study, the seizure frequency decrease was not significantly different between the two groups; however, patients in both groups showed significantly lower seizure frequency in 2, 4, and 6 months of follow-up compared to starting time. After a follow-up for 6 months, one out of 47 (2.1%) patients using levetiracetam showed intolerance, resulting in changing the medication. In addition, two out of 48 (4.1%) patients in the carbamazepine group had skin rashes. No significant changes had been reported regarding the duration of seizure attacks in both groups after treatment. CONCLUSION: This study showed encouraging results for using levetiracetam, with acceptable results and fewer side effects for the treatment of children with BCECTS in Iran.

Hemi-pelvic slope is correlated with the acetabular depth in adults-a radiological study.

OBJECTIVE: The purpose of this study was to determine if there is an anatomic relationship between pelvic geometry and acetabular depth. MATERIALS AND METHODS: One hundred and fifty-one anteroposterior pelvic radiographs (157 hip joints) were selected and analyzed in a retrospective fashion. Six parameters, including iliac offset, ischial offset, acetabular offset, pelvic height, center-edge (CE) angle of Wiberg, and acetabular index (AI) angle of Tönnis were measured for each of the hip joints. Based on the defined variables, three formulas (formulas 1, 2, and 3) were calculated to represent the hemi-pelvis slope. RESULTS: There was a strong relationship between the hemi-pelvis slope and probability of acetabular dysplasia. Wider upper pelvis was associated with deeper acetabulum and wider lower pelvis was associated with dysplastic acetabulum. CONCLUSIONS: Pelvic anatomic dimensions and the relationship between them are strongly correlated with hip dysplasia indices. Dysplastic hips tend to be found in pelvises with lower slope (low width of the upper pelvis at the level of iliac crest and high width of the lower part at the level of ischium).

Adalimumab for Treating Moderate-to-Severe Hidradenitis Suppurativa: An Evidence Review Group Perspective of a NICE Single Technology Appraisal.

As part of its single technology appraisal (STA) process, the UK National Institute for Health and Care Excellence (NICE) invited the manufacturer of adalimumab (AbbVie) to submit evidence on the clinical effectiveness and cost effectiveness of adalimumab for the treatment of moderate-to-severe hidradenitis suppurativa (HS). The appraisal assessed adalimumab as monotherapy in adult patients with an inadequate response to conventional systemic HS therapy. The School of Health and Related Research Technology Appraisal Group was commissioned to act as the independent Evidence Review Group (ERG). The ERG produced a critical review of the evidence for the clinical effectiveness and cost effectiveness of the technology based on the company's submission to NICE. The evidence was mainly derived from three randomised controlled trials comparing adalimumab with placebo in adults with moderate-to-severe HS. The clinical-effectiveness review found that significantly more patients achieved a clinical response in the adalimumab groups than in the control groups but that the treatment effect varied between trials and there was uncertainty regarding its impact on a range of other relevant outcomes as well as long-term efficacy. The company's submitted Markov model assessed the incremental cost effectiveness of adalimumab versus standard care for the treatment of HS from the perspective of the UK NHS and Personal Social Services (PSS) over a lifetime horizon. The original submitted model, including a patient access scheme (PAS), suggested that the incremental cost-effectiveness ratio (ICER) for adalimumab versus standard care was expected to be £16,162 per quality-adjusted life-year (QALY) gained. Following a critique of the model, the ERG's preferred base case, which corrected programming errors and structural problems surrounding discontinuation rules and incorporated a lower unit cost for HS surgery, resulted in a probabilistic ICER of £29,725 per QALY gained. Based on additional analyses undertaken by the company and the ERG following the publication of the appraisal consultation document (ACD), the Appraisal Committee concluded that the maximum possible ICER for adalimumab compared with supportive care was between £28,500 and £33,200 per QALY gained but was likely to be lower. The Appraisal Committee recommended adalimumab (with the PAS) for the treatment of active moderate-to-severe HS in adults whose disease has not responded to conventional systemic therapy.

Neurological outcome after arterial ischemic stroke in children.

BACKGROUND: Stroke is an important cause of disability in children. Pediatric stroke may be due to significant permanent cognitive and motor handicap in children. In this study, we evaluated long-term outcomes of stroke in pediatric patients who have been discharged with definite diagnosis of stroke in Tehran Mofid children's Hospital and Imam Hossein children's Hospital located in Isfahan, Iran, from 2005 to 2012. MATERIALS AND METHODS: A total of 53 children with stroke were included in the study. Stroke outcomes as motor disability, seizures, and cognitive dysfunctions were assessed. RESULTS: After a median follow-up of 4 years, 15 (29%) patients experienced full recovery. Thirty-eight (71%) patients had some degree of neurological handicap. CONCLUSION: Approximately 70% of children with arterial ischemic stroke suffer from long-term neurological disabilities including motor deficits, cognitive impairment, and late seizures. Stroke recurrence is the most important risk factor responsible for severe adverse neurological outcomes in pediatric stroke.

Iranian Joint Registry (Iranian National Hip and Knee Arthroplasty Registry).

Periodic evaluation and monitoring the health and economic outcome of joint replacement surgery is a common and popular process under the territory of joint registries in many countries. In this article we introduce the methodology used for the foundation of the National Iranian Joint Registry (IJR) with a joint collaboration of the Social Security Organization (SSO) and academic research departments considering the requirements of the Iran's Ministry of Health and Education.

Bilateral Staged Total Hip Replacement and the Natural Progress of an Untreated Case of Developmental Dysplasia (Dislocation) of the Hip: A Clinical Case Report by the Surgeon and the Patient.

The natural history of an untreated case of a Developmental Dysplasia (Dislocation) of the Hip (DDH) associated with multiple congenital abnormalities is reported in a 55-years-old man. The patient's complaints and the varieties of the typical manifestations emerged in other parts of the body throughout the life are reviewed and discussed as comorbidities of a dysplastic condition. Two-stage bilateral total hip replacement (THR) operations were performed at the age of 55. In addition, to relieve the pain, the walking disabilities were overcome, hence gaining normal walking in swing and stances. The leg length discrepancy was corrected by anatomically positioned prostheses, examined by the knee bending test and characterized and evidenced by radiological features and indices.

Habitual dislocation of the hip: a rare case report.

Habitual dislocation of the hip (HDH) in children is a rare entity and can be a causative factor for popping or snapping hip which is a common problem in children with good prognosis. We report a case of HDH in a 9 year old girl who was suffering from frequent snapping hip at night, its course and treatment process.

Evaluation of multiplex ligation-dependent probe amplification analysis versus multiplex polymerase chain reaction assays in the detection of dystrophin gene rearrangements in an Iranian population subset.

BACKGROUND: The Duchenne muscular dystrophy (DMD) gene is located in the short arm of the X chromosome (Xp21). It spans 2.4 Mb of the human genomic DNA and is composed of 79 exons. Mutations in the Dystrophin gene result in DMD and Becker muscular dystrophy. In this study, the efficiency of multiplex ligation-dependent probe amplification (MLPA) over multiplex polymerase chain reaction (PCR) assays in an Iranian population was investigated. MATERIALS AND METHODS: Multiplex PCR assays and MLPA analysis were carried out in 74 patients affected with DMD. RESULTS: Multiplex PCR detected deletions in 51% of the patients with DMD. MLPA analysis could determine all the deletions detected by the multiplex PCR. Additionally, MLPA was able to identify one more deletion and duplication in patients without detectable mutations by multiplex PCR. Moreover, MLPA precisely determined the exact size of the deletions. CONCLUSION: Although MLPA analysis is more sensitive for detection of deletions and duplications in the dystrophin gene, multiplex PCR might be used for the initial analysis of the boys affected with DMD in the Iranian population as it was able to detect 95% of the rearrangements in patients with DMD.