Objective: This study aimed to evaluate the effectiveness of soft-tissue release on hip subluxation and dislocation in cerebral palsy as well as activities of daily living after surgery.

Patients and Methods: Soft-tissue release was performed in 13 patients (19 hips) with cerebral palsy. Of them, 10 had spastic quadriplegia and three had spastic diplegia. Mean ages were 8.6 years at surgery and 13.8 years at the last investigation. The mean follow-up period was 5.2 years. Hip subluxation and dislocation severities were analyzed before and after surgery and at the final investigation as migration percentage on radiographs. Postoperative activities of daily living were also evaluated in 12 patients.

Results: Seven hips classified as mild and moderate preoperatively were classified as good, mild, and moderate at the last investigation. Nine of 12 hips classified as severe preoperatively continued to be severe at the last investigation. However, three of 12 hips classified as severe preoperatively improved at the last investigation. There was a positive correlation between preoperative migration percentage and that at the last investigation. Daily activities improved postoperatively in 12 patients.

Conclusions: Early treatment is necessary to prevent hip dislocation and improve hip subluxation. However, several patients with severe subluxation might experience improvement with soft-tissue release alone. Soft-tissue release is effective for treating hip dislocation and subluxation in cerebral palsy and improving daily activities.

We investigated whether polymorphisms of the endothelial nitric oxide synthase (eNOS), neuronal NOS (nNOS), and GTP cyclohydrolase I (GTPCH) genes are involved in the aggravation of migraine induced by overuse of medications. We studied 47 patients with migraine (six males and 41 females; 36.4 ± 10.3 years of age) and 22 patients with migraine exhibiting medication overuse headache (MOH, one male and 21 females; 39.6 ± 9.9 years of age). The genotypes of polymorphisms of the eNOS (rs1799983), nNOS (rs2682826), and GTPCH (rs841) genes were analyzed using the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method. The genotypic distributions of rs2682826 (T/T plus T/C vs. C/C, P = 0.254), rs1799983 (G/G vs. G/T plus T/T, P = 1.000), and rs841 (T/T plus T/C vs. C/C, P = 0.149) were not significantly different between patients with migraine and patients with MOH. The results of this study showed an absence of association between the polymorphisms of eNOS, nNOS, and GTPCH genes and the complication of MOH in patients with migraine.

Objectives: To investigate the details of patients’ status on admission and at discharge at our hospital, to compare the ambulatory group and non-ambulatory group at discharge, and to assess the factors associated with ambulatory ability at discharge in patients aged ≥ 90 years with proximal femoral fractures (PFFs).

Patients/Materials and Methods: Twenty patients admitted to our hospital for rehabilitation after surgery for a PFF were evaluated retrospectively. The rate of regaining ambulatory ability, presence of dementia, body mass index, serum albumin level, hemoglobin level, lymphocyte count, and functional independence measure (FIM) were assessed on admission and at discharge. Relationships between patients’ ambulatory ability and ambulatory parameters were compared between the ambulatory and non-ambulatory groups.

Results: The rate of regaining ambulatory ability was 55% at discharge. The serum albumin level at discharge was significantly higher in the ambulatory group than that in the non-ambulatory group. More patients had dementia on admission in the non-ambulatory group than in the ambulatory group. On admission, scores for the cognitive items of the FIM (“expression” and “memory”) were significantly higher in the ambulatory group than those in the non-ambulatory group.

Conclusions: The rate of ambulatory ability at discharge was 55% in those with a PFF, who were aged ≥ 90 years. The presence of dementia on admission and serum albumin level at discharge were factors related to ambulatory ability. It is very important to use a general rehabilitation protocol that takes cognitive function and nourishment into account, in addition to the physical aspect.

Objectives: This study was performed to elucidate the characteristics of amputees in our hospital. We also evaluated whether the causes and characteristics of the amputations influenced the patients’ prosthetic walking ability.

Materials and Methods: We retrospectively examined 47 amputees in our hospital from December 1996 to April 2016 with respect to the causes and levels of amputation. Of 28 lower limb amputees from April 2008 to April 2016, 22 received prostheses and were divided into 2 groups according to the cause of the amputation, as follows: the internal cause group (e.g., vascular deficiency and infection) and the external cause group (e.g., trauma, burn injury, and crush syndrome). The characteristics and process of achieving prosthetic ambulation were compared between these groups.

Results: Trauma was the most common cause of both upper (70.0%) and lower limb amputations (40.5%). Unilateral amputation was performed in 93.2% of patients (upper limb amputation, 100.0%; lower limb amputation, 91.9%). Patients were older in the internal than in the external cause group (P = 0.026). The serum albumin (P = 0.003) and total cholesterol concentrations (P = 0.046) on admission were significantly lower in the internal than in the external cause group. All patients in the internal cause group had comorbidities. The proportions of patients with diabetes mellitus (P = 0.011) and cerebrovascular disease (P=0.036) were significantly higher in the internal than in the external cause group. No significant difference in walking ability was found between the internal and external cause groups at the time of discharge.

Conclusion: Most amputees in our hospital underwent unilateral lower limb amputation due to trauma. Although the patients with internal causes of amputation were older, more frequently had malnutrition, and had more comorbidities than those with external causes, they achieved prosthetic walking with statistically insignificant difference at the end of hospitalization, excluding six patients who had no prosthetic prescription.

Purpose: Golimumab (GLM) is an anti-tumor necrosis factor (TNF)-α antibody preparation known to be less immunogenic than infliximab (IFX) or adalimumab. Few reports on GLM in pediatric patients with ulcerative colitis (UC) are available. This study aimed to review the long-term durability and safety of GLM in a pediatric center. Methods: The medical records of 17 pediatric patients (eight boys and nine girls) who received GLM at the National Center for Child Health and Development were retrospectively reviewed. Results: The median age at GLM initiation was 13.9 (interquartile range 12.0–16.3) years.Fourteen patients had pancolitis, and 11 had severe disease (pediatric ulcerative colitis activity index ≥65). Ten patients were biologic-naïve, and 50% achieved corticosteroid-free remission at week 54. Two patients discontinued prior anti-TNF-α agents because of adverse events during remission. Both showed responses to GLM without unfavorable events through week 54. However, the efficacy of GLM in patients who showed primary nonresponse or loss of response to IFX was limited. Four of the five patients showed non-response at week 54. Patients with severe disease had significantly lower corticosteroid-free remission rate at week 54 than those without severe disease. No severe adverse events were observed during the study period. Conclusion GLM appears to be safe and useful for pediatric patients with UC. Patients with mild to moderate disease who responded to but had some adverse events with prior biologics may be good candidates for GLM. Its safety and low immunogenicity profile serve as favorable options for selected children with UC.

Purpose: The development of assistive devices has allowed for the performance of capsule endoscopy in children. Anticipating the capsule’s transit time could affect the efficacy of the investigation and potentially minimize the fasting period. This study determined the predictors of small bowel transit time for small-bowel capsule endoscopy in children and adolescents with inflammatory bowel disease. Methods: We retrospectively examined children and adolescents with inflammatory bowel disease who underwent capsule endoscopy by the age 18 at a Japanese tertiary care children’s hospital. Small bowel transit time predictors were analyzed using multiple regression with explanatory variables. Results: Overall, 92 patients, aged 1–17 years, with inflammatory bowel disease (63 Crohn’s disease and 29 ulcerative colitis cases) were examined for factors affecting small bowel transit time. In the simple regression analysis, diagnosis, age, height, weight, serum albumin, general anesthesia, and small intestine lesions were significantly associated with small bowel transit time. In the multiple regression analyses, serum albumin (partial regression coefficient: −58.9, p=0.008), general anesthesia (partial regression coefficient: 127, p<0.001), and small intestine lesions (partial regression coefficient: 30.1, p=0.037) showed significant associations with small bowel transit time. Conclusion Hypoalbuminemia, the use of general anesthesia for endoscopic delivery of the capsule, and small intestine lesions appeared to be predictors of prolonged small bowel transit time in children and adolescents with inflammatory bowel disease. Expecting the finishing time may improve examination with a fasting period reduction, which benefits both patients and caregivers.

BACKGROUND AND PURPOSE: Migraine patients are particularly prone to the complication of medication-overuse headache (MOH). Although it has been shown that A allele carriers for the tumor necrosis factor (TNF)-beta gene G252A polymorphism are at high risk of the development of migraine without aura, the relationship between the TNF-beta gene G252A polymorphism and MOH is unknown. We investigated whether the TNF-beta gene G252A polymorphism is involved in the aggravation of migraine by overuse of medications. METHODS: Forty-seven migraine patients (6 males and 41 females; age 36.4+/-10.3 years, mean+/-SD) and 22 MOH patients (1 male and 21 females; age 39.6+/-9.9 years) who had migraine were included in this study. The genotype for the TNF-beta gene G252A polymorphism was determined by polymerase-chain-reaction restriction-fragment-length polymorphism analysis. RESULTS: The distribution of TNF-beta gene G252A genotype frequency differed significantly between migraine and MOH patients (p=0.013). The G/G genotype was carried by 23% of the migraine patients but it was absent in MOH patients. CONCLUSIONS: G/G genotype carriers appear to be less susceptible to the aggravation of migraine by overuse of medications. The G252A TNF-beta gene polymorphism may be one of the factors contributing to the complications of MOH in patients with migraine.
Alleles ; Genotype ; Headache ; Humans ; Lymphotoxin-alpha ; Male ; Migraine Disorders ; Migraine without Aura ; Tumor Necrosis Factor-alpha

Purpose: The parents of adolescents with inflammatory bowel disease may experience impaired mental health and quality of life. This longitudinal study aimed to verify whether the mental health and quality of life of the parents of adolescents with inflammatory bowel disease declined when their children had active disease. Methods: Sociodemographic data, parental anxiety, depression, and quality of life were analyzed using validated questionnaires for each variable. After the baseline survey, the second and follow-up surveys were conducted at 3 and 12 months, respectively. The active disease group comprised eight parents whose children had active disease during the baseline and second surveys. The remission group comprised 14 parents whose children remained in remission during both surveys. The improved group comprised nine parents whose children experienced active disease at baseline and remission during the second survey. Parental mental health and quality of life were compared among the groups. Results: Significantly higher levels of anxiety were observed in the active disease group in all surveys (p<0.050). Although depression levels and quality of life did not differ significantly among the three groups, pairing the active disease group with other groups showed some large effect sizes. Conclusion Parents tended to experience decreased mental health and quality of life when their adolescents experienced active inflammatory bowel disease. Consequently, our hypothesis was partially verified. Therefore, parents need support when their children have active disease; this finding highlights the need for parental support systems.

Purpose: The long-term efficacy and safety of infliximab (IFX) in children with ulcerative colitis (UC) have not been well-evaluated. Here, we reviewed the long-term durability and safety of IFX in our single center pediatric cohort with UC. Methods: This retrospective study included 20 children with UC who were administered IFX. Results: For induction, 5 mg/kg IFX was administered at weeks 0, 2, and 6, followed by every 8 weeks for maintenance. The dose and interval of IFX were adjusted depending on clinical decisions. Corticosteroid (CS)-free remission without dose escalation (DE) occurred in 30% and 25% of patients at weeks 30 and 54, respectively. Patients who achieved CS-free remission without DE at week 30 sustained long-term IFX treatment without colectomy. However, one-third of the patients discontinued IFX treatment because of a primary nonresponse, and one-third experienced secondary loss of response (sLOR). IFX durability was higher in patients administered IFX plus azathioprine for >6 months. Four of five patients with very early onset UC had a primary nonresponse. Infusion reactions (IRs) occurred in 10 patients, resulting in discontinuation of IFX in four of these patients. No severe opportunistic infections occurred, except in one patient who developed acute focal bacterial nephritis. Three patients developed psoriasis-like lesions. Conclusion IFX is relatively safe and effective for children with UC. Clinical remission at week 30 was associated with long-term durability of colectomy-free IFX treatment. However, approximately two-thirds of the patients were unable to continue IFX therapy because of primary nonresponse, sLOR, IRs, and other side effects.

Purpose: A change in diagnosis from ulcerative colitis (UC) to Crohn's disease (CD) has been reported in pediatric inflammatory bowel disease; however, only a few clinical characteristics and predictors of this diagnostic change have been reported. We aimed to describe the clinical characteristics of patients with UC who underwent a change in diagnosis to CD and identify variables associated with the change. Methods: The medical records of pediatric patients with UC who were followed up at the National Center for Child Health and Development between 2006 and 2019 were retrospectively reviewed. Clinical data on disease phenotype, laboratory parameters, endoscopic findings, and treatment of patients whose diagnosis changed to CD (cCD) were compared to those of patients whose diagnosis remained UC (rUC). Results: Among the 111 patients initially diagnosed with UC, 11 (9.9%) patients were subsequently diagnosed with CD during follow-up. There was no significant difference between the cCD and rUC groups in terms of sex, age at initial diagnosis, and the extent and severity of disease at initial diagnosis. Albumin and hemoglobin levels were significantly lower in the cCD group than in the rUC group. The proportion of patients who required biologics was significantly higher in the cCD group than in the rUC group (p<0.05). Conclusion Approximately 10% children initially diagnosed with UC were subsequently diagnosed with CD. Hypoalbuminemia and anemia at initial diagnosis and use of biologics could be predictors of this diagnostic change.