Duchess of Kent Children's Hospital

Evidence-based guidelines, or recommendations, for the management of infants with seizures are lacking. A Task Force of the Commission of Pediatrics developed a consensus document addressing diagnostic markers, management interventions, and outcome measures for infants with seizures. Levels of evidence to support recommendations and statements were assessed using the American Academy of Neurology Guidelines and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. The report contains recommendations for different levels of care, noting which would be regarded as standard care, compared to optimal care, or "state of the art" interventions. The incidence of epilepsy in the infantile period is the highest of all age groups (strong evidence), with epileptic spasms the largest single subgroup and, in the first 2 years of life, febrile seizures are the most commonly occurring seizures. Acute intervention at the time of a febrile seizure does not alter the risk for subsequent epilepsy (class 1 evidence). The use of antipyretic agents does not alter the recurrence rate (class 1 evidence), and there is no evidence to support initiation of regular antiepileptic drugs for simple febrile seizures (class 1 evidence). Infants with abnormal movements whose routine electroencephalography (EEG) study is not diagnostic, would benefit from video-EEG analysis, or home video to capture events (expert opinion, level U recommendation). Neuroimaging is recommended at all levels of care for infants presenting with epilepsy, with magnetic resonance imaging (MRI) recommended as the standard investigation at tertiary level (level A recommendation). Genetic screening should not be undertaken at primary or secondary level care (expert opinion). Standard care should permit genetic counseling by trained personal at all levels of care (expert opinion). Genetic evaluation for Dravet syndrome, and other infantile-onset epileptic encephalopathies, should be available in tertiary care (weak evidence, level C recommendation). Patients should be referred from primary or secondary to tertiary level care after failure of one antiepileptic drug (standard care) and optimal care equates to referral of all infants after presentation with a seizure (expert opinion, level U evidence). Infants with recurrent seizures warrant urgent assessment for initiation of antiepileptic drugs (expert opinion, level U recommendation). Infantile encephalopathies should have rapid introduction and increment of antiepileptic drug dosage (expert opinion, level U recommendation). There is no high level evidence to support any particular current agents for use in infants with seizures. For focal seizures, levetiracetam is effective (strong evidence); for generalized seizures, weak evidence supports levetiracetam, valproate, lamotrigine, topiramate, and clobazam; for Dravet syndrome, strong evidence supports that stiripentol is effective (in combination with valproate and clobazam), whereas weak evidence supports that topiramate, zonisamide, valproate, bromide, and the ketogenic diet are possibly effective; and for Ohtahara syndrome, there is weak evidence that most antiepileptic drugs are poorly effective. For epileptic spasms, clinical suspicion remains central to the diagnosis and is supported by EEG, which ideally is prolonged (level C recommendation). Adrenocorticotropic hormone (ACTH) is preferred for short-term control of epileptic spasms (level B recommendation), oral steroids are probably effective in short-term control of spasms (level C recommendation), and a shorter interval from the onset of spasms to treatment initiation may improve long-term neurodevelopmental outcome (level C recommendation). The ketogenic diet is the treatment of choice for epilepsy related to glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency (expert opinion, level U recommendation). The identification of patients as potential candidates for epilepsy surgery should be part of standard practice at primary and secondary level care. Tertiary care facilities with experience in epilepsy surgery should undertake the screening for epilepsy surgical candidates (level U recommendation). There is insufficient evidence to conclude if there is benefit from vagus nerve stimulation (level U recommendation). The key recommendations are summarized into an executive summary. The full report is available as Supporting Information. This report provides a comprehensive foundation of an approach to infants with seizures, while identifying where there are inadequate data to support recommended practice, and where further data collection is needed to address these deficits.

The authors declare no conflict of interest.

Importance: Early onset of myopia is associated with high myopia later in life, and myopia is irreversible once developed. Objective: To evaluate the efficacy of low-concentration atropine eyedrops at 0.05% and 0.01% concentration for delaying the onset of myopia. Design, Setting, and Participants: This randomized, placebo-controlled, double-masked trial conducted at the Chinese University of Hong Kong Eye Centre enrolled 474 nonmyopic children aged 4 through 9 years with cycloplegic spherical equivalent between +1.00 D to 0.00 D and astigmatism less than -1.00 D. The first recruited participant started treatment on July 11, 2017, and the last participant was enrolled on June 4, 2020; the date of the final follow-up session was June 4, 2022. Interventions: Participants were assigned at random to the 0.05% atropine (n = 160), 0.01% atropine (n = 159), and placebo (n = 155) groups and had eyedrops applied once nightly in both eyes over 2 years. Main Outcomes and Measures: The primary outcomes were the 2-year cumulative incidence rate of myopia (cycloplegic spherical equivalent of at least -0.50 D in either eye) and the percentage of participants with fast myopic shift (spherical equivalent myopic shift of at least 1.00 D). Results: Of the 474 randomized patients (mean age, 6.8 years; 50% female), 353 (74.5%) completed the trial. The 2-year cumulative incidence of myopia in the 0.05% atropine, 0.01% atropine, and placebo groups were 28.4% (33/116), 45.9% (56/122), and 53.0% (61/115), respectively, and the percentages of participants with fast myopic shift at 2 years were 25.0%, 45.1%, and 53.9%. Compared with the placebo group, the 0.05% atropine group had significantly lower 2-year cumulative myopia incidence (difference, 24.6% [95% CI, 12.0%-36.4%]) and percentage of patients with fast myopic shift (difference, 28.9% [95% CI, 16.5%-40.5%]). Compared with the 0.01% atropine group, the 0.05% atropine group had significantly lower 2-year cumulative myopia incidence (difference, 17.5% [95% CI, 5.2%-29.2%]) and percentage of patients with fast myopic shift (difference, 20.1% [95% CI, 8.0%-31.6%]). The 0.01% atropine and placebo groups were not significantly different in 2-year cumulative myopia incidence or percentage of patients with fast myopic shift. Photophobia was the most common adverse event and was reported by 12.9% of participants in the 0.05% atropine group, 18.9% in the 0.01% atropine group, and 12.2% in the placebo group in the second year. Conclusions and Relevance: Among children aged 4 to 9 years without myopia, nightly use of 0.05% atropine eyedrops compared with placebo resulted in a significantly lower incidence of myopia and lower percentage of participants with fast myopic shift at 2 years. There was no significant difference between 0.01% atropine and placebo. Further research is needed to replicate the findings, to understand whether this represents a delay or prevention of myopia, and to assess longer-term safety. Trial Registration: Chinese Clinical Trial Registry: ChiCTR-IPR-15006883.

We used a new method to assess spinal flexibility in thirty patients who were to be managed operatively for adolescent idiopathic scoliosis. The method involves placing the patient in the lateral decubitus position and bent over a fulcrum (a radiolucent padded cylinder) so that the spine is passively hinged open. For thoracic curves the fulcrum is centered under the rib corresponding to the apex of the curve, and for lumbar curves the fulcrum is placed directly under the apex. The preoperative workup for the thirty patients included an anteroposterior radiograph made with the patient standing, a lateral-bending radiograph made with the patient supine, and the new fulcrum bending radiograph. All patients were treated with posterior spinal arthrodesis with segmental spinal instrumentation. The degree of flexibility obtained with the traditional and new methods was compared with the degree of correction observed on the radiograph made, with the patient standing, one week after the operation. Preoperatively, the mean Cobb angle was 58 degrees on the anteroposterior radiograph made with the patient standing, 31 degrees on the lateral-bending radiograph made with the patient supine, and 24 degrees on the fulcrum bending radiograph. The mean angle was 25 degrees on the anteroposterior radiograph made one week postoperatively, so the mean correction was 57 per cent. The difference between the mean angle on the lateral-bending radiograph and that on the postoperative radiograph was significant (p < 0.001); however, the mean angle measured on the preoperative fulcrum bending radiograph and the postoperative angle were almost identical. We found the fulcrum bending radiograph to be more predictive of the degree of flexibility and correctability than the lateral-bending radiograph in this group of patients who had segmental spinal instrumentation for correction of idiopathic scoliosis.

BACKGROUND: Traditional growing rods have a reported wound and implant complication rate as high as 58%. It is unclear whether the use of magnetically controlled growing rods (MCGR) will affect this rate. This study was performed to characterize surgical complications following MCGR in early onset scoliosis. METHODS: A multicenter retrospective review of MCGR cases was performed. Inclusion criteria were: (1) diagnosis of early onset scoliosis of any etiology; (2) 10 years and younger at time of index surgery; (3) preoperative major curve size >30 degrees; (4) preoperative thoracic spine height <22 cm. Complications were categorized as wound related and instrumentation related. Complications were also classified as early (<6 mo from index surgery) versus late (>6 mo). Distraction technique and interval of distraction was surgeon preference without standardization across sites. RESULTS: Fifty-four MCGR patients met inclusion criteria. There were 30 primary and 24 conversion procedures. Mean age at initial surgery was 7.3 years (range, 2.4 to 11 y), and mean duration of follow-up 19.4 months. Twenty-one (38.8%) of 54 patients had at least 1 complication. Fifteen (27.8%) had at least 1 revision surgery. Six (11.1%) had broken rods (2 to 4.5 and 4 to 5.5 mm rods); two 5.5 mm rods failed early (4 mo) and 4 late (mean=14.5 mo). Six (11.1%) patients experienced 1 episode of lack or loss of lengthening, of which 4 patients subsequently lengthened. Seven patients (13.0%) had either proximal or distal fixation-related complication at average of 8.4 months. Two patients (3.7%) had infections requiring incision and drainage; 1 early (2 wk) with wound drainage and 1 late (8 mo). The late case required removal of one of the dual rods. CONCLUSIONS: This study shows that compared with traditional growing rods, MCGR has a lower infection rate (3.7% vs. 11.1%). MCGR does not appear to prevent common implant-related complications such as rod or foundation failure. The long-term implication remains to be determined. LEVEL OF EVIDENCE: Level IV.

We compared the long-term changes in spinal deformity after a radical operation for tuberculosis of the spine in thirty-three children who were ten years old or younger at the time of the operation with those of seventy-one adult patients who were at least eighteen years old at the time of the operation. The spinal deformity was measured with use of the angles of kyphosis and deformity as assessed on lateral spinal radiographs made preoperatively and postoperatively at six months, one year, and five years and at the most recent follow-up evaluation (at a mean of fifteen years). We detected no significant difference in the mean angles of kyphosis and deformity between the children and the adults postoperatively at any follow-up evaluation; thus, we found that growth of the posterior portion of the spine does not contribute to the progression of deformity after a radical anterior procedure. The children who had tuberculosis of the thoracic spine had much better correction than the adults at the six-month follow-up examination. This correction was maintained. However, there were no such differences in the correction of the deformity between the adults and the children who had tuberculosis of the thoracolumbar or the lumbar spine. Our findings clearly show that a short anterior spinal arthrodesis done at an early age was not associated with progression of deformity during growth and development in our patients. The longitudinal pattern of changes in deformity was similar in the children and the adults, and there was no evidence of disproportionate posterior spinal growth contributing to the progression of deformity after anterior spinal arthrodesis.(ABSTRACT TRUNCATED AT 250 WORDS)

BACKGROUND: Age-specific incidence of acute myocarditis/pericarditis in adolescents following Comirnaty vaccination in Asia is lacking. This study aimed to study the clinical characteristics and incidence of acute myocarditis/pericarditis among Hong Kong adolescents following Comirnaty vaccination. METHODS: This is a population cohort study in Hong Kong that monitored adverse events following immunization through a pharmacovigilance system for coronavirus disease 2019 (COVID-19) vaccines. All adolescents aged between 12 and 17 years following Comirnaty vaccination were monitored under the COVID-19 vaccine adverse event response and evaluation program. The clinical characteristics and overall incidence of acute myocarditis/pericarditis in adolescents following Comirnaty vaccination were analyzed. RESULTS: Between 14 June 2021 and 4 September 2021, 33 Chinese adolescents who developed acute myocarditis/pericarditis following Comirnaty vaccination were identified. In total, 29 (87.88%) were male and 4 (12.12%) were female, with a median age of 15.25 years. And 27 (81.82%) and 6 (18.18%) cases developed acute myocarditis/pericarditis after receiving the second and first dose, respectively. All cases are mild and required only conservative management. The overall incidence of acute myocarditis/pericarditis was 18.52 (95% confidence interval [CI], 11.67-29.01) per 100 000 persons vaccinated. The incidence after the first and second doses were 3.37 (95% CI, 1.12-9.51) and 21.22 (95% CI, 13.78-32.28 per 100 000 persons vaccinated, respectively. Among male adolescents, the incidence after the first and second doses were 5.57 (95% CI, 2.38-12.53) and 37.32 (95% CI, 26.98-51.25) per 100 000 persons vaccinated. CONCLUSIONS: There is a significant increase in the risk of acute myocarditis/pericarditis following Comirnaty vaccination among Chinese male adolescents, especially after the second dose.

Introduction: The aim of this study was to investigate the accelerated emergence of telepractice in speech language pathology during the coronavirus disease 2019 (COVID-19) pandemic in Hong Kong—a small city with limited accessibility concern that telepractice has not been widely implemented. Method: An online survey of speech therapists in Hong Kong was conducted between February and March in 2020. The survey comprised up to 15 questions to assess participants' demographics, existing service delivery in telepractice, perception, and their training and knowledge on telepractice. Results: One hundred thirty-five speech (n = 135) speech language pathologists responded to the survey. About one-third (34.8%; n = 47) of participants reported having provided services through telepractice, whereas 72.3% of them started in &lt;3 months and half of them considered it was less effective than face-to-face service. Among the other participants (n = 88), 83% of them indicated that unsuitable patient type and age as the main reason for not providing telepractice. The majority of participants had no prior training for delivering telepractice, and focused on technology when asked about their desired training. Knowledge of participants on telepractice was found only to align fairly with international guidelines. Discussions: The survey findings suggested that telepractice provision in Hong Kong was different from that of other countries where telepractice has been well established, due to the accelerated emergence by the COVID-19 pandemic. Conclusions: The study showed how the development of telepractice would be like in a short time frame, and findings on perception and desired training could be a reference for a better establishment of this model of service.

Thirty unpremedicated ASA physical status 1-3 patients aged between 18 and 69 years, scheduled for upper limb surgery, received a conventional supraclavicular brachial plexus block using a nerve stimulator and bupivacaine 0.375% 0.5 ml.kg-1. Spirometric measurements of pulmonary function and ultrasonographic assessments of diaphragmatic function were made before the block and at 10-min intervals after injection until full motor block of the brachial plexus had developed. Complete paralysis of the ipsilateral hemidiaphragm occurred in 50% of patients. Seventeen per cent of patients had reduced diaphragmatic movement and the rest (33%) had no change in diaphragmatic movement. Those with complete paralysis all showed significant decreases in pulmonary function, whereas those with reduced or normal movement had minimal change. All patients remained asymptomatic throughout, with normal oxygen saturation on room air.

Background: is a well-known gene identified in patients with this syndromic presentation, other genes in the PI3K-AKT-mTOR signalling pathway have also recently been suggested to have important roles. The aim of this study is to characterise the mutation spectrum of this group of patients. Methods: We performed whole-exome sequencing of 21 patients with macrocephaly and developmental delay/autism spectrum disorder. Sources of genomic DNA included blood, buccal mucosa and saliva. Germline mutations were validated by Sanger sequencing, whereas somatic mutations were validated by droplet digital PCR. Results: mutations, and the level of somatic mosaicism in blood DNA was low. Patients who tested positive for mutations in the PI3K-AKT-mTOR pathway had a lower developmental quotient than the rest of the cohort (DQ = 62.8 vs. 76.1, p = 0.021). Their dysmorphic features were non-specific, except for macrocephaly. Among the ten patients with identified mutations, brain magnetic resonance imaging was performed in nine, all of whom showed megalencephaly. Conclusion: We identified mutations in the PI3K-AKT-mTOR signalling pathway in nearly half of our patients with macrocephaly and developmental delay/autism spectrum disorder. These patients have subtle dysmorphic features and mild developmental issues. Clinically, patients with germline mutations are difficult to distinguish from patients with somatic mutations, and therefore, sequencing of buccal or saliva DNA is important to identify somatic mosaicism. Given the high diagnostic yield and the management implications, we suggest implementing comprehensive genetic testing in the PI3K-AKT-mTOR pathway in the clinical evaluation of patients with macrocephaly and developmental delay and/or autism spectrum disorder.

OBJECTIVE: The need for radiologic surveillance of spinal tumors in children with neurofibromatosis 1 is controversial and unresolved. We aimed to determine the incidence of spinal tumors in asymptomatic patients, describe the imaging findings, and evaluate the clinical usefulness of a prospective MR imaging surveillance program of the spine in children with neurofibromatosis 1. SUBJECTS AND METHODS: Of 62 children consecutively seen in a neurofibromatosis 1 clinic, 53 (85.5%) were recruited for MR imaging of the whole spine. All children satisfied the clinical criteria for diagnosis of neurofibromatosis 1. Imaging findings, clinical signs and symptoms, and subsequent clinical management were reviewed. RESULTS: The patients were 35 boys and 18 girls (age range, 11 months-18 years; mean age, 9.6 years), all of whom were asymptomatic, with no remarkable neurologic signs. Seven children (13.2%) had spinal neurofibromas: four had solitary neurofibromas (two dumbbell, one intradural, and one paraspinal tumor) and three had plexiform neurofibromas of the sacral plexus and thoracic and lumbar nerve sheaths. The incidences of scoliosis, localized cutaneous neurofibromas, and massive soft-tissue neurofibromas were 71.4%, 71.4%, and 28.6%, respectively, in the group with spinal neurofibromas (n = 7), and 30.4%, 39.1%, and 8.7%, respectively, in the group without spinal neurofibromas (n = 46). Patient clinical outcome was affected in only one patient (1.9%) in whom a solitary neurofibroma was resected. Follow-up imaging in 10 patients (mean period, 29 months) showed no evidence of tumor occurrence, progression, or recurrence. CONCLUSION: Although benign spinal neurofibromas are not uncommon in asymptomatic children with neurofibromatosis 1, the clinical usefulness of spine surveillance with MR imaging is limited in these children, making its effectiveness questionable.

STUDY DESIGN: A prospective study on adolescent idiopathic scoliosis to assess a new method of describing frontal plane correction. OBJECTIVE: To assess the difference between using the correction rate and using the new fulcrum bending correction index when describing scoliosis correction. SUMMARY OF BACKGROUND DATA: A description of the outcome of scoliosis surgery based on the correction rate does not take into account the curve's flexibility. METHODS: The fulcrum bending correction index is calculated by dividing the correction rate by the fulcrum flexibility (based on the fulcrum bending radiograph) and is expressed as a percentage. The fulcrum bending correction index was compared with the correction rate in 35 patients with adolescent idiopathic scoliosis undergoing surgical correction. These patients were divided into a stiff and a flexible group based on a fulcrum flexibility of 50% to show the advantage of the fulcrum bending correction index over the correction rate. RESULTS: The difference in correction rate between the flexible (66%) and the stiff group (45%) was significant (P < 0.05). The difference in the fulcrum bending correction index between the flexible (96%) and the stiff group (107%) was not significant (P = 0.2). CONCLUSIONS: The fulcrum bending correction index takes into account the curve flexibility and is therefore better than the correction rate for comparing curve correction and use of instrumentation systems between different series of patients. A fulcrum bending correction index of close to 100% suggests that the instrumentation has taken up all the flexibility revealed by the fulcrum bending radiograph. It is recommended that the fulcrum bending correction index be used in the future as part of the postoperative assessment of frontal plane correction in cases of thoracic scoliosis.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has put tremendous pressure on the healthcare system worldwide. Diagnostic testing remained one of the limiting factors for early identification and isolation of infected patients. This study aimed to evaluate posterior oropharyngeal saliva (POPS) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) detection among patients with confirmed or suspected COVID-19. METHODS: The laboratory information system was searched retrospectively for all respiratory specimens and POPS requested for SARS-CoV-2 RNA detection between 1 February 2020 and 15 April 2020. The agreement and diagnostic performance of POPS against NPsp were evaluated. RESULTS: A total of 13772 specimens were identified during the study period, including 2130 POPS and 8438 nasopharyngeal specimens (NPsp). Two hundred and twenty-nine same-day POPS-NPsp paired were identified with POPS and NPsp positivity of 61.5% (95% confidence interval [CI] 55.1-67.6%) and 53.3% (95% CI 46.8-59.6%). The overall, negative and positive percent agreement were 76.0% (95% CI 70.2-80.9%), 65.4% (95% CI 55.5-74.2%), 85.2% (95% CI 77.4-90.8%). Better positive percent agreement was observed in POPS-NPsp obtained within 7 days (96.6%, 95% CI 87.3-99.4%) compared with after 7 days of symptom onset (75.0%, 95% CI 61.4-85.2%). Among the 104 positive pairs, the mean difference in Cp value was 0.26 (range: 12.63 to -14.74), with an overall higher Cp value in NPsp (Pearson coefficient 0.579). No significant temporal variation was noted between the 2 specimen types. CONCLUSIONS: POPS is an acceptable alternative specimen to nasopharyngeal specimen for the detection of SARS-CoV-2.

STUDY DESIGN: Eighty-five patients with adolescent idiopathic scoliosis treated with Milwaukee or thoracolumbosacral orthoses at The Duchess of Kent Children's Hospital were studied longitudinally at 4-6-month intervals until maturity for spinal curvature and vertebral rotation, or until termination of brace treatment for persons who experienced brace failure who went on to have surgery. OBJECTIVES: To identify radiologic features so that it may be possible to predict outcome of brace treatment early on. SUMMARY OF BACKGROUND DATA: The structural curve with poor flexibility and large rotational prominence have been found to be associated with poor prognosis for brace treatment. However, early response to bracing for spinal deformity and its relationship to final outcome of brace treatment in a longitudinal study is not available in the literature. METHODS: Vertebral rotation and Cobb angles measured from anteroposterior radiographs of the spine obtained before bracing and 1-2 months after bracing were found valuable for prediction. Changes in post-brace Cobb angle and vertebral rotation were considered as an increase or reduction only when there was an increase or reduction of minimum 5 degrees or more from their prebrace measurements. RESULTS: Those patients who showed increase in vertebral rotation and/or in Cobb angle after brace application were shown to have progression of curves leading to brace failure in 93% of patients, and 79% of these required surgery. The patients with no change in both vertebral rotation and Cobb angle after bracing often experienced brace failure (69%). Two patients (15%) required surgery. The results show that reduction of both Cobb angle and vertebral rotation after application of a brace is a prognostic indicator for a good outcome (97%), and no patients required surgery. Most of the patients with lumbar scoliosis (91%) showed such reductions. CONCLUSION: The findings show a strong association between changes in vertebral rotation and the Cobb angle after application of a brace and the final outcome. Reduction in both is indicative of a good outcome, whereas increase in one or both indicates brace failure.

Vertebral anomalies were studied in 144 patients. The specific anatomic defects were defined by serial roentgenograms: anterior approaches were used in 42 operations and posterior approaches in 74 operations. Growth features of the spine were examined histologically in 15 embryos and fetuses ranging in length from 6 to 120 mm. After an extensive review of the literature on normal human spine embryology and fetal development, and after defining the specific vertebral anatomic defects and their pathogenesis, some new concepts of congenital vertebral anomalies formation are proposed. Old concepts are found to be inadequate in light of our current knowledge of embryogenesis. The authors' new and all-inclusive classification of congenital vertebral anomalies is based on specific defects, pathogenesis, and time for origin in embryonic or fetal development.

A retrospective review of 1542 Charnley low-friction arthroplasties was carried out to compare the incidence of deep infection arising after plain and gentamicin-containing acrylic cement. There was no significant difference between the two series in primary operations, the infection rate being 1.72% and 1.65% respectively. However, in secondary operations (conversions and revisions) the gentamicin-containing cement gave significantly better results--a 0.81% infection rate as compared with 3.46%. All operations were performed in a clean air enclosure using total-body exhaust suits. Radiological diagnosis of deep infection was possible within one year of operation in all cases. Late haematogenous infection occurred in one case and was related to a skin lesion.

Significance Statement Children with frequently relapsing, steroid-dependent nephrotic syndrome (FRSDNS) often require multiple courses of rituximab. However, long-term effects from repeated treatments remain unknown. In this international, multicenter study of 346 children receiving 1149 courses of rituximab, the risk of relapse decreased and relapse-free survival significantly improved with repeated treatments. Important side effects, including hypogammaglobulinemia, neutropenia, and infections, were mostly mild, but significant adverse events could occur. The incidence of side effects did not increase with more treatment courses nor a higher cumulative dose of rituximab. These findings suggest that repeating rituximab therapy is an effective and reasonably safe approach for most children with FRSDNS. Background Long-term outcomes after multiple courses of rituximab among children with frequently relapsing, steroid-dependent nephrotic syndrome (FRSDNS) are unknown. Methods A retrospective cohort study at 16 pediatric nephrology centers from ten countries in Asia, Europe, and North America included children with FRSDNS who received two or more courses of rituximab. Primary outcomes were relapse-free survival and adverse events. Results A total of 346 children (age, 9.8 years; IQR, 6.6–13.5 years; 73% boys) received 1149 courses of rituximab. A total of 145, 83, 50, 28, 22, and 18 children received two, three, four, five, six, and seven or more courses, respectively. Median (IQR) follow-up was 5.9 (4.3–7.7) years. Relapse-free survival differed by treatment courses (clustered log-rank test P &lt;0.001). Compared with the first course (10.0 months; 95% CI, 9.0 to 10.7 months), relapse-free period and relapse risk progressively improved after subsequent courses (12.0–16.0 months; HR adj , 0.03–0.13; 95% CI, 0.01 to 0.18; P &lt;0.001). The duration of B-cell depletion remained similar with repeated treatments (6.1 months; 95% CI, 6.0 to 6.3 months). Adverse events were mostly mild; the most common adverse events were hypogammaglobulinemia (50.9%), infection (4.5%), and neutropenia (3.7%). Side effects did not increase with more treatment courses nor a higher cumulative dose. Only 78 of the 353 episodes of hypogammaglobulinemia were clinically significant. Younger age at presentation (2.8 versus 3.3 years; P =0.05), age at first rituximab treatment (8.0 versus 10.0 years; P= 0.01), and history of steroid resistance (28% versus 18%; P =0.01) were associated with significant hypogammaglobulinemia. All 53 infective episodes resolved, except for one patient with hepatitis B infection and another with EBV infection. There were 42 episodes of neutropenia, associated with history of steroid resistance (30% versus 20%; P =0.04). Upon last follow-up, 332 children (96%) had normal kidney function. Conclusions Children receiving repeated courses of rituximab for FRSDNS experience an improving clinical response. Side effects appear acceptable, but significant complications can occur. These findings support repeated rituximab use in FRSDNS.

The contributory roles of vitamin D in ocular and visual health have long been discussed, with numerous studies pointing to the adverse effects of vitamin D deficiency. In this paper, we provide a systematic review of recent findings on the association between vitamin D and different ocular diseases, including myopia, age-related macular degeneration (AMD), glaucoma, diabetic retinopathy (DR), dry eye syndrome (DES), thyroid eye disease (TED), uveitis, retinoblastoma (RB), cataract, and others, from epidemiological, clinical and basic studies, and briefly discuss vitamin D metabolism in the eye. We searched two research databases for articles examining the association between vitamin D deficiency and different ocular diseases. One hundred and sixty-two studies were found. There is evidence on the association between vitamin D and myopia, AMD, DR, and DES. Overall, 17 out of 27 studies reported an association between vitamin D and AMD, while 48 out of 54 studies reported that vitamin D was associated with DR, and 25 out of 27 studies reported an association between vitamin D and DES. However, the available evidence for the association with other ocular diseases, such as glaucoma, TED, and RB, remains limited.

BACKGROUND: Worldwide, iron deficiency anemia is the most prevalent nutritional deficiency disorder and the leading cause of anemia in children, especially in developing countries. When present in early childhood, especially if severe and prolonged, iron deficiency anemia can result in neurodevelopmental and cognitive deficits, which may not always be fully reversible even following the correction of iron deficiency anemia. OBJECTIVE: This article aimed to familiarize physicians with the clinical manifestations, diagnosis, evaluation, prevention, and management of children with iron deficiency anemia. METHODS: A PubMed search was conducted in February 2023 in Clinical Queries using the key term "iron deficiency anemia". The search strategy included all clinical trials (including open trials, non-randomized controlled trials, and randomized controlled trials), observational studies (including case reports and case series), and reviews (including narrative reviews, clinical guidelines, and meta-analyses) published within the past 10 years. Google, UpToDate, and Wikipedia were also searched to enrich the review. Only papers published in the English literature were included in this review. The information retrieved from the search was used in the compilation of the present article. RESULTS: Iron deficiency anemia is most common among children aged nine months to three years and during adolescence. Iron deficiency anemia can result from increased demand for iron, inadequate iron intake, decreased iron absorption (malabsorption), increased blood loss, and rarely, defective plasma iron transport. Most children with mild iron deficiency anemia are asymptomatic. Pallor is the most frequent presenting feature. In mild to moderate iron deficiency anemia, poor appetite, fatigability, lassitude, lethargy, exercise intolerance, irritability, and dizziness may be seen. In severe iron deficiency anemia, tachycardia, shortness of breath, diaphoresis, and poor capillary refilling may occur. When present in early childhood, especially if severe and prolonged, iron deficiency anemia can result in neurodevelopmental and cognitive deficits, which may not always be fully reversible even with the correction of iron deficiency anemia. A low hemoglobin and a peripheral blood film showing hypochromia, microcytosis, and marked anisocytosis, should arouse suspicion of iron deficiency anemia. A low serum ferritin level may confirm the diagnosis. Oral iron therapy is the first-line treatment for iron deficiency anemia. This can be achieved by oral administration of one of the ferrous preparations, which is the most cost-effective medication for the treatment of iron deficiency anemia. The optimal response can be achieved with a dosage of 3 to 6 mg/kg of elemental iron per day. Parenteral iron therapy or red blood cell transfusion is usually not necessary. CONCLUSION: In spite of a decline in prevalence, iron deficiency anemia remains a common cause of anemia in young children and adolescents, especially in developing countries; hence, its prevention is important. Primary prevention can be achieved by supplementary iron or iron fortification of staple foods. The importance of dietary counseling and nutritional education cannot be overemphasized. Secondary prevention involves screening for, diagnosing, and treating iron deficiency anemia. The American Academy of Pediatrics recommends universal laboratory screening for iron deficiency anemia at approximately one year of age for healthy children. Assessment of risk factors associated with iron deficiency anemia should be performed at this time. Selective laboratory screening should be performed at any age when risk factors for iron deficiency anemia have been identified.

STUDY DESIGN: Retrospective study. OBJECTIVE: To report 2-year clinical and radiographic results of patients treated with magnetically controlled growing rods (MCGR). SUMMARY OF BACKGROUND DATA: MCGR for early-onset scoliosis has been reported to provide adequate spinal growth and curve correction by eliminating surgical lengthening procedures. This study was designed to report the results of MCGR patients with 2-year follow-up. METHODS: A retrospective study of MCGR patients with the following inclusion criteria: (i) major curve size ≥30°, (ii) T1-T12 height <22 cm, (iii) <11-years old; all at the time of index surgery was performed. Of 54 patients enrolled, 23 had 2-year follow-up. Both primary and conversion patients were evaluated at baseline 6, 12, and 24 months. RESULTS: Mean preoperative age in the primary group was 6.6 ± 2.6 years versus 8.3 ± 2.2 years for the conversion group. A total of 41 adverse events occurred in 11 patients, of which 14 events were implant related. Major coronal curve magnitude improved from 61.3° to 34.3° from baseline to postoperation in primary cases and from 49.4° to 43.8° in conversion cases. Curve correction was maintained for 2 years in both groups. T1-S1 height improved from 252.7 to 288.9 mm in primary cases and was maintained for 2 years. However, conversion cases had some decline in T1- S1 height (270.3 at baseline to 294.4 mm post-MCGR and 290.2 mm at 2-year follow-up; mean loss of 4.2 mm (1.5%) from postoperation to 2 years, P > 0.05). CONCLUSION: This study showed satisfactory curve correction and growth is achieved among primary cases. T1-S1 height in conversion cases had a slight decline in 2 years. However, this decline was not statistically significant. LEVEL OF EVIDENCE: 3.