Peripheral Neuropathy In Children Research Articles

Vincristine‑induced peripheral neuropathy in children with acute lymphoblastic leukemia: Clinical and instrumental characteristics, search for new solutions

Objective. To evaluate clinical and electroneuromyographic characteristics of vincristine polyneuropathy in children with acute lymphoblastic leukemia.Materials and methods. A single-centre prospective cohort study involved 106 children with acute lymphoblastic leukemia, aged 3 to 17 years, with vincristine polyneuropathy; clinical and electroneuromyographic data were analyzed.Results. In the majority of cases, 84.9 % (n = 90) of patients, vincristine polyneuropathy debuted during the induction phase of chemotherapy. The clinical picture was dominated by combined neurological disorders in 67.9 % (n = 72) cases, with sensory and motor disorders dominating. Sensory dysfunction was manifested predominantly by pain in the lower extremities, changes in the motor sphere were characterized by the development of distal paresis of the lower extremities. In 66.0 % (n = 70) of patients, neurological disorders corresponded to the 2nd degree of severity according to the toxicity scale (NCI-STAE). According to the ENMG study, all children had motor axonal neuropathy of the peroneal nerves.Conclusions. The results of the study showed that the clinical picture of vincristine polyneuropathy is accompanied by sensory and motor symptoms. Currently, a complex of clinical and instrumental methods is used to verify the neurotoxic complication. The main objective method of diagnosis is an electroneuromyographic study. However, its capabilities are limited for determining the clinical manifestations associated with sensory disorders.

Vincristine-Induced Peripheral Neuropathy in Children With Malignancy and the Effect of Missed Doses on Treatment Success.

Recognizing the symptoms of vincristine-induced peripheral neuropathy (VIPN) earlier is crucial to preventing the persistent neurological sequelae. The treatment of neuropathy is to discontinue the drug, and the effect of a missed dose of vincristine on treatment success is unclear. This study aims to evaluate VIPN in children with malignancy and the effect of skipping vincristine doses on the treatment success of patients at a single center, retrospectively. Medical records of the children with cancer who received vincristine in our institution between 2013 and 2020 were analyzed retrospectively. Vincristine neuropathy was found in 42 (7%) of 598 pediatric patients who received at least one dose of vincristine during the study period. Neuropathy developed at a statistically significantly lower cumulative dose in patients younger than seven years of age (p=0.04). The mean neuropathy duration of the cases was 8.5 months, and the findings of 40 (95.2%) cases improved. The mean cumulative dose was higher in patients with diffuse nerve involvement. The missed dose of vincristine was lower in the cases in complete remission compared to the other cases and higher doses of vincristine were missed in the stable disease group than in the remission group (p=0.03). VIPN can be encountered in less cumulative doses, mainly in the younger age group. Missed doses of vincristine may affect treatment success, and more comprehensive studies are needed to show this effect more clearly.

Evaluation of sympathetic skin response for early diagnosis and follow-up of diabetic peripheral neuropathy in children

BackgroundThe morbidity of type 1 diabetes mellitus (T1DM) in children is increasing and diabetic peripheral neuropathy (DPN) is one of the main microvascular complications of T1DM. The aim of this study was to explore sympathetic skin response (SSR) characteristics in children with T1DM and analyze the value of early diagnosis and follow-up in T1DM complicated with DPN.MethodsOur prospective study enrolling 85 participants diagnosed with T1DM and 30 healthy controls (HCs) in the Children’s Hospital of Hebei Province from 2017 to 2020. Compared the outcomes of SSR and nerve conduction study (NCS) in T1DM, and evaluated the variations in SSR and NCS of different durations, as well as changes after six months of therapy.ResultsSSR latency of T1DM group showed statistical difference as compared to HCs (p < 0.05). The SSR test was more sensitive than the NCS test in the early diagnosis of T1DM with DPN (p < 0.05). The abnormal rates of SSR and NCS in long duration of disease were higher than those in short duration of disease (p < 0.05). Among 65 participants with diabetic neuropathy, the onset latencies of SSR were shortened and the NCS were improved after treatment (p < 0.05).ConclusionsSSR could provide the accurate early diagnosis and follow-up of pediatric diabetic peripheral neuropathy.

Chemotherapy-Induced Peripheral Neuropathy in Children Treated for Acute Lymphoblastic Leukemia: A Role for Oxidative Stress and Brain-Derived Neurotrophic Factor

Chemotherapy-Induced Peripheral Neuropathy in Children Treated for Acute Lymphoblastic Leukemia: A Role for Oxidative Stress and Brain-Derived Neurotrophic Factor

Assessment of proxy-reported responses as predictors of motor and sensory peripheral neuropathy in children with B-lymphoblastic leukemia.

Chemotherapy-induced peripheral neuropathy (CIPN), a common condition in children with acute lymphoblastic leukemia, can be challenging to diagnose. Using data from Children's Oncology Group AALL0932 physical function study, we sought to determine if parent/guardian proxy-reported responses from the Pediatric Outcomes Data Collection Instrument could identify children with motor or sensory CIPN diagnosed by physical/occupational therapists (PT/OT). Four variables moderately discriminated between children with and without motor CIPN (c-index 0.76, 95% confidence interval [CI]: 0.64-0.84), but sensory and optimism-corrected models had weak discrimination (c-index sensory models 0.65, 95% CI: 0.54-0.74). New proxy-report measures are needed to identify children with PT/OT diagnosed CIPN.

A cohort study of vincristine-induced peripheral neuropathy in children

To study the characteristics of vincristine-induced peripheral neuropathy (VIPN) in children with acute lymphoblastic leukemia (ALL) and the factors influencing the development of VIPN. The children with ALL, aged 1-18 years, who were treated with CCCG-ALL2015 or CCCG-ALL2020 regimen in the Affiliated Hospital of Guizhou Medical University from January 2018 to February 2022 were enrolled as subjects. According to the influence of age on risk, the children were divided into 1-10 years group with 91 children and >10 years group with 29 children. VIPN was graded according to the National Cancer Institute Common Terminology Criteria for Adverse Events (5th edition), and the incidence rate, severity, and type of VIPN were compared between different groups. A total of 120 children were enrolled in this study, among whom 56 (46.7%) developed VIPN. The >10 years group had a significantly higher incidence rate of VIPN than the 1-10 years group (69% vs 40%, P<0.05). Among the 56 children with VIPN, 12 (21%) had grade 3 VIPN or above, and 44 (79%) had grade 2 VIPN. There were 77 cases of autonomic nerve symptoms (59.7%), 42 cases of peripheral nerve injury (32.5%), and 10 cases of cranial nerve injury (7.8%). There were no significant differences in the severity and type of VIPN between the groups with different ages, sexes, degrees of risk, or treatment regimens (P>0.05). The results of binary logistic regression analysis showed that age is the influencing factor for the occurrence of VIPN (P>0.05). There is a relatively high incidence rate of VIPN in children with ALL, with the highest incidence rate of autonomic nervous symptoms. The incidence of VIP in children over 10 years old is relatively high.

Vincristine induced peripheral neuropathy in children undergoing chemotherapy for acute lymphoblastic leukaemia during induction

Background: Vincristine is an anticancer agent administered to all children with acute lymphoblastic leukemia (ALL), and peripheral neuropathy is the major dose-limiting toxicity of this therapy. As cure rates of childhood ALL exceeds 80%, therefore treatment-related toxicities need to be reduced. Thus, the aim of this study was to determine the prevalence and risk factors of vincristine-induced peripheral neuropathy (VIPN) in children with ALL undergoing induction chemotherapy. Methods: A case-control study was conducted from September 2017 to August 2018 in the Department of Paediatric Haematology and Oncology at Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh. Eighty newly diagnosed ALL and 35 acute myeloid leukemia (AML) cases aged 5 to 17 years with no pre-existing neurological abnormality were recruited. To assess the peripheral neuropathy, we used pediatric-modified total neuropathy score and National Cancer Institute- Common Terminology Criteria for Adverse Events (NCI-CTCAE), version-04 grade. Results: Among ALL patients, 29.2% developed peripheral neuropathy compared to 10% in AML control group (P=0.04). Higher proportion (57.1%) of peripheral neuropathy was found in age below 10 years (P&lt;0.001). There was no significant association of peripheral neuropathy with sex and body mass index of the patients. Conclusion: Almost 3 in 10 patients developed VIPN during the induction therapy which is significantly higher in age below 10 years compared to ≥ 10 years. Bangabandhu Sheikh Mujib Medical University Journal 2023;16(1): 02-07

Evaluation of vincristine-induced peripheral neuropathy in children with cancer: Turkish validity and reliability study

BackgroundThe evaluation of peripheral neuropathy in children receiving Vincristine treatment is challenging. This study examined the Turkish validity and reliability of the Total Neuropathy Score-Pediatric Vincristine (TNS-PV) measurement tool, which can measure Vincristine-induced peripheral neuropathy symptoms in children with cancer. MethodsA total of 53 children aged 5–17 years who received Vincristine treatment in two pediatric hematology-oncology centers participated in the study. Data was collected using the Total Neuropathy Score-Pediatric Vincristine (TNS-PV), the Common Terminology Criteria for Adverse Events (CTCAE), the Wong-Baker FACES Pain Scale, and the Adolescent Pediatric Pain Tool (APPT). The correlation between the TNS-PV total score and other scales and the inter-rater reliability coefficient was evaluated. FindingsOf the children, 81.1% were diagnosed with ALL and 13.2% with Ewing Sarcoma. Cronbach's alpha values of form A and B of the TNS-PV scale were 0.628 and 0.639, respectively. As the cumulative Vincristine dose increased, the children's scores on TNS-PV were higher. A moderate and significant positive correlation was found between the TNS-PV form A total score and the worst subjective symptoms a, b (A), strength, tendon reflexes, and autonomic / constipation (r = 0.441, r = 0.545, r = 0.472, r = 0.536, p < 0.01). DiscussionThe TNS-PV form B total score was found to have a moderate level, significant correlation with CTCAE sensory neuropathy score and Wong-Baker FACES Pain Scale, and a high level, significant positive correlation with CTCAE motor neuropathy score. Application to practiceThe TNS-PV is valid and reliable for measuring Vincristine-induced peripheral neuropathy in practice in Turkish children 5 years and older.

Electrophysiologic Patterns of Symptomatic Vincristine-Induced Peripheral Neuropathy in Children with Acute Lymphocytic Leukemia.

Acute lymphocytic leukemia is one of the most common cancers in children. Multi-drug chemotherapy is used for treatment, and the representative drug is vincristine. Although various side effects may occur due to vincristine, the association with peripheral neuropathy is high compared to that of other drugs. This study focused on children under the age of 18 years of age with ALL who received chemotherapy containing vincristine. We retrospectively analyzed the results of a nerve conduction study and a cumulative dose of vincristine in 30 children diagnosed with peripheral neuropathy. The average cumulative dose until diagnosis of vincristine-induced peripheral neuropathy was 14.99 ± 1.21 mg/m2, and motor nerves were predominantly involved. Additionally, a marked decrease in average amplitude was also observed in motor nerves. In addition, when the relationship between the incidence of peripheral neuropathy and the cumulative dose was analyzed through the survival curve, about 50% of children developed peripheral neuropathy at a dose of 15.5 ± 1.77 mg/m2. Based on the electrophysiological characteristics of pediatric vincristine-induced peripheral neuropathy, as well as the relationship between the incidence rate and the cumulative dose, it is possible to observe more closely the vincristine-induced peripheral neuropathy occurrence in children with ALL at an appropriate time.

Total Neuropathy Scale Pediatric Vincristine to detect vincristine induced peripheral neuropathy in children with Acute Lymphoblastic Leukemia

Objective: To analyze the Total Neuropathy Scale Pediatric Vincristine (TNS-PV) score as a vincristine induced peripheral neuropathy (VIPN) diagnostic tool. Methodology: This observational analytic study was conducted in Outpatient Pediatric Hematology Oncology Dr. Soetomo Hospital. The inclusion criteria were Acute Lymphoblastic Leukemia (ALL) children aged 4-18 years that had undergone chemotherapy treatment, received a cumulative dose of vincristine &gt;12 mg/m2, had no muscle weakness and signed informed consent. The TNS-PV instrument were used to assess VIPN and nerve conduction studies (NCS) was used as the gold standard. Results: A total of 54 children were enrolled, two refused to sign the informed consent. About 60% were male children, 76.9% aged &lt;10 years old, and 94% were ALL-L1. The TNS-PV had an area under the curve (AUC) of 0.638 (95% CI 0.531-0.834) with cut off value of 3.5. It had a sensitivity 87.9%; specificity 42.1%, positive predictive value 72.5%; negative predictive value (NPV) 66.6% and OR (Odds ratio) 5,273. Conclusion: TNS-PV score cannot replace NCS as the gold standard, but TNS-PV can be an alternative tool for diagnosing VIPN.

Peripheral Neuropathy in Children with High Risk Hodgkin Lymphoma (HL): The Role of Protocol-Stipulated Dose Modification in the Children's Oncology Group (COG) AHOD1331 Study

Background Peripheral neuropathy (PN) is commonly associated with several classes of chemotherapy, including vinca alkaloids and the novel anti-CD30-directed antibody-drug conjugate, Brentuximab vedotin (Bv). The drug moiety in Bv, monomethyl auristatin E, a potent microtubule tubulin inhibitor, is known to cause PN. In the 2018 Echelon-1 study, rates of PN among adults with advanced stage HL were 67% in the Bv arm as compared to 43% in the control arm (Connors, 2018) with dose modifications at the discretion of the treating physician of 66% for Bv and 57% for vinblastine (VBL). We examined rates of PN and use of protocol-stipulated dose modification in the COG AHOD1331, a Phase 3 randomized comparison of standard multi-agent chemotherapy (ABVE-PC) vs. Bv-AVE-PC among children and adolescents with high risk HL (Castellino, 2022). The combination of vincristine (VCR) with Bv warrants the formal study of PN in this trial. Methods Eligible patients (pts) ages 2-21 years with high risk HL were enrolled from 2015-2019 (NCT02166463). After randomization, pts received 5 cycles of chemotherapy. Pts in the standard (std) arm received VCR on Day 1 and 8 of each 21-day treatment cycle; pts in the experimental (exp) arm received Bv (1.8 mg/kg) on Day 1 and VCR on Day 8. Clinician grading of PN was required at each cycle, using the Balis PN Scale, with protocol-stipulated dose modifications of VCR preceding modification of Bv in cases of PN > grade 2. Rates of PN were calculated by treatment cycle by study arm. Dose modifications were summarized for each drug by treatment cycle. Median time to first dose modification was estimated by treatment arm. A Cox model estimated hazard ratios (HR) and 95% confidence intervals (CI) for dose modifications by treatment arm and pt and clinical characteristics. Progression-free survival (PFS) was compared with Log-rank test by study arms and within arm by presence or absence of PN. Results 587 pts were enrolled on the study. While the proportion of pts overall developing PN did not differ by study arm (19%, p=0.86), dose modification of Bv, VCR, or both occurred more frequently on the exp arm (50/298, 16.8%) than on the std arm (12/289, 4.2%). Among pts on the exp arm, Bv was dose modified in 8% of pts; VCR was dose modified in 13.4% of pts. The median time to first dose modification was 40.8 days (31.5-73.5) for the exp arm vs. 75.5 days (59.0-94.8) for the std arm (p=.0043). During cycle 5, the rate of any PN was 3.1% in the exp arm vs. 7.8% in the std arm. Compared to the std arm, the HR for dose modification was 5.4 in the exp arm (95% CI, 2.3-12.6, p<0.0001); none of the other pt or disease characteristics were significantly associated with dose modification of the tubulin inhibitors. PFS was significantly better in the exp arm and did not differ by PN status (Figure). Conclusions The rate of dose modifications for PN was consistent with protocol-specified dose modification among children and adolescents treated with known tubulin inhibitors as part of a multi-drug regimen for high risk HL. The strategy to preserve Bv dose in the experimental arm was successful. Overall rates of PN were substantially lower than previously reported in adults treated with Bv. While there were no significant differences in the rate of PN between study arms, we found shorter median time to dose modification in the exp arm, corresponding to the second treatment cycle, as compared to the 3rd cycle for the std arm. Despite dose modifications of vincristine and Bv as management strategies for PN in the experimental arm, patients treated with Bv-AVEPC had superior disease control in children with high-risk HL. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Chemotherapy-induced peripheral neuropathy in children and adolescent cancer patients.

Brain cancer and leukemia are the most common cancers diagnosed in the pediatric population and are often treated with lifesaving chemotherapy. However, chemotherapy causes severe adverse effects and chemotherapy-induced peripheral neuropathy (CIPN) is a major dose-limiting and debilitating side effect. CIPN can greatly impair quality of life and increases morbidity of pediatric patients with cancer, with the accompanying symptoms frequently remaining underdiagnosed. Little is known about the incidence of CIPN, its impact on the pediatric population, and the underlying pathophysiological mechanisms, as most existing information stems from studies in animal models or adult cancer patients. Herein, we aim to provide an understanding of CIPN in the pediatric population and focus on the 6 main substance groups that frequently cause CIPN, namely the vinca alkaloids (vincristine), platinum-based antineoplastics (cisplatin, carboplatin and oxaliplatin), taxanes (paclitaxel and docetaxel), epothilones (ixabepilone), proteasome inhibitors (bortezomib) and immunomodulatory drugs (thalidomide). We discuss the clinical manifestations, assessments and diagnostic tools, as well as risk factors, pathophysiological processes and current pharmacological and non-pharmacological approaches for the prevention and treatment of CIPN.

Childhood focal compressive mononeuropathies during the COVID-19 pandemic in Buenos Aires, Argentina.

Introduction/AimsFocal peripheral neuropathies are infrequently seen in pediatric patients. The COVID‐19 pandemic has disrupted normal life for many people, including complete lockdowns and school closing for long periods of time in many countries, which prompted children to stay at home. Our aim is to assess whether there has been an increased incidence of focal compressive peripheral neuropathies in the pediatric population during COVID‐19–associated lockdown.MethodsClinical, electrophysiological, and imaging characteristics were reviewed for patients referred to the electrodiagnostic (EDx) laboratory with suspicion of a focal neuropathy. The incidence of focal compressive peripheral neuropathies seen during the period of March to September 2020 was compared with the same time period in 2019.ResultsAn increased incidence of focal neuropathies was seen in 2020 (31%) compared with 2019 (6.8%). During 2020, 7 fibular (peroneal) mononeuropathies and 2 ulnar neuropathies were diagnosed. Most patients with focal neuropathies were underweight and acknowledged prolonged screen time periods. Electrophysiological findings consisted of mostly demyelinating lesions with an overall good clinical outcome.DiscussionIn this study we raise awareness about a possible increased incidence of focal compressive peripheral neuropathies in children during COVID‐19–associated lockdown, which may be prevented with changing positions during sedentary activities.

A systematic review of the prevalence, risk factors and screening tools for autonomic and diabetic peripheral neuropathy in children, adolescents and young adults with type 1 diabetes.

We aimed to estimate the prevalence of Diabetic peripheral neuropathy (DPN) and Cardiac autonomic neuropathy (CAN) in youth with type 1 diabetes; identify key risk factors; identify the most useful tests for the diagnostic evaluation of DPN and CAN; identify key treatment options for DPN and CAN. A systematic search was performed including studies published in the last 15years. PICO framework was used in the selection process and evidence was assessed using the GRADE system. A total of 758 studies were identified and a final number of 49 studies were included in this systematic review. According to moderate-high level quality studies, the prevalence of probable DPN, ranged between 13.5 and 62%; subclinical DPN between 22 and 88%; confirmed DPN between 2.6 and 11%. The Michigan Neuropathy Screening Instrument was the tool with higher sensitivity and specificity for detecting DPN, which needs to be confirmed by nerve conduction velocity. The prevalence of CAN was 4-39%. Specific treatment options for DPN or CAN in patients younger than 25years are not available. Key risk factors for DPN and CAN are hyperglycemia/HbA1c, age, diabetes duration, the presence of other microvascular complications, waist/height ratio, lipid profile and blood pressure. For CAN, additional risk factors were cigarette smoking, BMI and total daily insulin. Prevalence of neuropathy in youth with type 1 diabetes varies depending on different screening methods and characteristics of the study populations. However, the assessed studies confirmed a relatively high prevalence of subclinical neuropathy, reiterating the importance of early identification of risk factors to prevent this complication.

Pharmacogenomics of Vincristine-Induced Peripheral Neuropathy in Children with Cancer: A Systematic Review and Meta-Analysis.

Simple SummaryVincristine is a drug that is part of the treatment for many children with cancer. Its main side-effect is vincristine-induced peripheral neuropathy (VIPN), which often presents as tingling, pain, and lack of strength in the hands and feet. It is not yet possible to predict which children will suffer from VIPN. In this review, we report on all genetic variations that are associated with VIPN. We found that variations in genes related to vincristine transport, cell structure, hereditary nerve disease, and genes without a previously known connection to vincristine or VIPN are related to VIPN. Variations in genes involved in vincristine breakdown are not significantly associated with VIPN. In conclusion, genetic variations affect a child’s tendency to develop VIPN. In the future, this information might be used to predict the risk of VIPN and adapt treatment on this.Vincristine-induced peripheral neuropathy (VIPN) is a debilitating side-effect of vincristine. It remains a challenge to predict which patients will suffer from VIPN. Pharmacogenomics may explain an individuals’ susceptibility to side-effects. In this systematic review and meta-analysis, we describe the influence of pharmacogenomic parameters on the development of VIPN in children with cancer. PubMed, Embase and Web of Science were searched. In total, 1597 records were identified and 21 studies were included. A random-effects meta-analysis was performed for the influence of CYP3A5 expression on the development of VIPN. Single-nucleotide polymorphisms (SNPs) in transporter-, metabolism-, cytoskeleton-, and hereditary neuropathy-associated genes and SNPs in genes previously unrelated to vincristine or neuropathy were associated with VIPN. CYP3A5 expression status was not significantly associated with VIPN. The comparison and interpretation of the results of the included studies was limited due to heterogeneity in the study population, treatment protocol and assessment methods and definitions of VIPN. Independent replication is essential to validate the clinical significance of the reported associations. Future research should aim for prospective VIPN assessment in both a discovery and a replication cohort. Ultimately, the goal would be to screen patients upfront to determine optimal vincristine dosage with regards to efficacy and risk of VIPN.

Peripheral Neuropathy in Children With Chronic Kidney Disease: Are We Looking Enough?

Background:Peripheral neuropathy in chronic kidney disease (CKD) is the most common neurological complication. We aimed to look at the prevalence and patterns of neuropathy in children with CKD.Methods:This cross-sectional study was conducted over 1 year in children with CKD, stage III and above. Nerve conduction studies (NCS) were performed as per standard protocols using surface electrodes on the muscles and by supramaximal stimulation of the corresponding nerves. Presence of electrophysiological abnormalities in the absence of clinical symptoms or signs was considered as subclinical neuropathy.Results:Nearly 45 children were evaluated. The majority were males (n = 39, 86.7%). The mean age was 7.9 ± 3 years (range 2–14). The mean estimated glomerular filtration rate (GFR) at enrolment was 23.3 ± 14.6 mL/min/1.73 m2 (range 5–67). The majority of children were in stage III (n = 19, 42%), followed by stages V (n = 15, 33%) and IV (n = 11, 25%). There was no evidence of clinical neuropathy; 13 children (29%) showed subclinical neuropathy. All the nerves had an axonal pattern of involvement. Motor polyneuropathy was most common type of peripheral neuropathy. The commonest nerves involved were tibial and common peroneal nerves. There were no biochemical or clinical predictors of neuropathy in our cohort.Conclusion:The prevalence of subclinical neuropathy is high in children with CKD, stage III and above. Axonal motor polyneuropathy is the predominant pattern. Electrophysiological assessment of nerve function should be routinely done in children with advanced stages of CKD to prevent chronic complications.

Prevalence of peripheral neuropathy in children with type 1 diabetes mellitus

Prevalence of peripheral neuropathy in children with type 1 diabetes mellitus

Prevalence and predictors of peripheral neuropathy in children with end stage renal disease on regular hemodialysis

Prevalence and predictors of peripheral neuropathy in children with end stage renal disease on regular hemodialysis

Relationship between Vitamin B9 (Folic Acid), Vitamin B12 (Cobalamin), and Peripheral Neuropathy in Children with Beta-Thalassemia Major

Vitamin B9 (folic acid) and B12 (cobalamin) are essential vitamins that play roles in the process of hematopoiesis and maintaining the function of peripheral nerves. Therefore, these deficiencies may create a risk for peripheral neuropathy in beta-thalassemia major patients. The purpose of this study is to determine the relationship between vitamin B9 level, vitamin B12 level, and peripheral neuropathy in beta-thalassemia major children. It was an observational analytical study with a case-control design has been conducted at Dr. Hasan Sadikin General Hospital Bandung, Indonesia, in May–July 2019. There were 47 beta-thalassemia major children with peripheral neuropathy (case) and 41 healthy children (control). All subjects completed a general demographic questionnaire, underwent neurological examination, and were tested for vitamin B9 and B12 serum levels. Data were then analyzed using the unpaired t test to compare the vitamin levels between both groups and Spearman’s rank correlation test to investigate the correlation between vitamin levels and the number of affected nerves in the case group. Comparison of folic acid levels in the case group (21.52±6.22 ng/mL) and the control group (23.81±7.51 ng/mL) showed no significant difference (p=0.19). In contrast, cobalamin in the case group (288.57±168.61 ng/mL) and the control group (385.95±197.48 ng/mL) showed a significant difference (p=0.01). In addition, there was a moderate correlation (p=0.004, r=0.41) between folic acid level and the number of motoric nerves affected in the case group. In conclusion, cobalamin level correlates with peripheral neuropathy in beta-thalassemia major patients, and folic acid level correlates with the number of affected nerves, especially motoric nerves. HUBUNGAN ANTARA VITAMIN B9 (ASAM FOLAT), VITAMIN B12 (KOBALAMIN), DAN NEUROPATI PERIFER PADA ANAK DENGAN TALASEMIA BETA MAYORVitamin B9 (asam folat) dan B12 (kobalamin) merupakan vitamin esensial yang berperan dalam proses hematopoiesis dan menjaga fungsi saraf tepi. Defisiensi vitamin ini dapat menimbulkan risiko neuropati perifer pada pasien talasemia beta mayor. Tujuan penelitian ini mengetahui hubungan antara kadar vitamin B9, vitamin B12, dan neuropati perifer pada anak talasemia beta mayor. Metode penelitian ini adalah analitik observasional dengan rancangan studi kasus kontrol yang dilakukan di RSUP Dr. Hasan Sadikin Bandung, Indonesia pada Mei–Juli 2019. Terdapat 47 anak talasemia beta mayor dengan neuropati perifer (kelompok kasus) dan 41 anak sehat (kelompok kontrol). Seluruh subjek penelitian mengisi kuesioner demografi umum, menjalani pemeriksaan fisis neurologis, serta dilakukan tes kadar vitamin B9 dan B12 serum. Uji t test tidak berpasangan digunakan untuk membandingkan kadar vitamin pada kedua kelompok dan uji korelasi Spearman untuk membandingkan kadar kedua vitamin tersebut dengan jumlah saraf yang terkena pada kelompok kasus. Perbandingan kadar asam folat kelompok kasus (21,52±6,22 ng/mL) dan kelompok kontrol (23,81±7,51 ng/mL) menunjukkan perbedaan yang tidak bermakna (p=0,19), sedangkan perbandingan kadar kobalamin kelompok kasus (288,57±168,61 ng/mL) dan kelompok kontrol (385,95±197,48 ng/mL) menunjukkan perbedaan yang bermakna (p=0,01). Selain itu, terdapat korelasi sedang (p=0,004; r=0,41) antara kadar asam folat dam jumlah saraf motorik yang terkena pada kelompok kasus. Kesimpulan, kadar kobalamin berhubungan dengan neuropati perifer pada penderita talasemia beta mayor dan kadar asam folat berhubungan dengan jumlah saraf yang terkena, terutama saraf motorik.

Point Prevalence of Peripheral Neuropathy in Children and Adolescents with Type 1 Diabetes Mellitus.

To assess the point prevalence of peripheral neuropathy (PN) in children with type 1 diabetes mellitus (T1DM) and to determine their predictors. In this cross-sectional study, children aged 8-18 y with T1DM on insulin therapy for > 2 y and free from acute complications were enrolled. All participants were evaluated for symptoms of PN with diabetic neuropathy symptom (DNS) score and underwent a detailed neurological examination. Assessment of nerve dysfunction was done using nerve conduction studies (NCS). The disease-related factors that increase the risk of PN were determined. Fifty children (52% boys) were enrolled with mean age of 12.2 ± 2.8 y and duration of diabetes 5.1 ± 2.1 y. No subject had clinical evidence or DNS score suggestive of PN. Twenty-eight (56%) children demonstrated subclinical neuropathy on NCS. Proportion of children with pure motor, pure sensory, and mixed motor-sensory neuropathy was 40%, 2%, and 14%, respectively. The peroneal nerve was the most common motor nerve affected. Poor glycemic control (HbA1c > 9%) and longer duration of diabetes (> 5 y) were significantly associated with the risk of PN (p value < 0.05). A large proportion of children with T1DM have subclinical PN. Poor glycemic control and longer duration of diabetes are risk factors for nerve dysfunction. Neurophysiological studies should be performed in these children to facilitate early detection.