Kawasaki disease (KD) is an immune-mediated disease which is a leading cause of acquired cardiovascular disease in developed country. Recently, tumor necrosis factor-alpha (TNF-alpha) blocker, infliximab has been considered a promising option for patients with refractory KD. Although chronic use of a TNF-alpha blocker could increase risk of opportunistic infections, a few studies have documented that use of infliximab was safe without serious adverse effects in patients with KD. We observed serious bacterial infection after infliximab treatment in an infant with refractory KD. Our patient was a 5-month-old male infant diagnosed with KD who did not respond to repeated doses of intravenous immunoglobulin. We effectively treated him with a single infusion of infliximab (5 mg/kg), but gram-negative (Acinetobacter lwoffii) septicemia developed after infliximab infusion. Therefore, we report a case of serious septicemia after treatment with infliximab, and suggest considering the risk of severe infection when deciding whether to prescribe infliximab to an infant with refractory KD.
Bacterial Infections ; Cardiovascular Diseases ; Developed Countries ; Humans ; Immunoglobulins ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Opportunistic Infections ; Sepsis* ; Tumor Necrosis Factor-alpha ; Infliximab

A 21-year-old woman with gargoyloid face and short trunkal stature showed progressive quadriparesis. Cervical spine MRI showed circumferential compression of cervical spinal cord by thickened dura mater. Elevated urinary dermatan sulfate and decreased enzyme activity of -iduronidase revealed that she has mild form of mucopolysaccharidosis type I. Her weakness was improved with cervical laminectomy and duraplasty. In this case, progressing quadriparesis resulted from cervical spinal cord compression by thickened dura mater. Earlier surgical decompression could have been better for the patient.
Decompression, Surgical ; Dermatan Sulfate ; Dura Mater ; Female ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Mucopolysaccharidoses* ; Mucopolysaccharidosis I* ; Quadriplegia ; Spinal Cord Compression* ; Spinal Cord* ; Spine ; Young Adult

Hemiparkinsonism with hemiatrophy (HP-HA) syndrome is characterized by a predominant unilateral parkisonism associated with ipsilateral body and/or contralateral brain abnormality. We report three cases of Hemiparkinsonism- hemiatrophy, which is a rare form of secondary parkinsonism. One patient revealed motor complication in the involved limb suggesting that the degeneration of nigrostriatal pathway as the underlying mechanism in this disorder. The functional neuro-imaging can play an important role to verify the cortical abnormalities when structural changes are not evident in brain MRI.
Brain ; Extremities ; Humans ; Magnetic Resonance Imaging ; Neuroimaging ; Parkinson Disease, Secondary ; Parkinsonian Disorders

No abstract available.
Carcinoid Tumor

Tetrodotoxin (TTX) cause neurologic dysfunction by blocking the voltage-gated sodium channels located in all of the peripheral nerves and muscles. We experienced two patients presenting with generalized motor weakness after ingestion of pufferfish. The nerve conduction study showed diffuse slowing of motor and sensory nerve conduction velocity, prolonged motor terminal latency and decreased sensory nerve action potentials without temporal dispersion or conduction block. Abnormal findings of nerve conduction study improved rapidly without any deterioration. Clinical symptoms and signs ameliorated in accordance with findings of nerve conduction study without any neurologic sequelae. These imply that tetrodotoxication is reversible and functional neurologic disorder. We suggest that nerve conduction studies can be available in serial monitoring of tetrodotoxication as an objective means.
Action Potentials ; Eating ; Humans ; Muscles ; Nervous System Diseases ; Neural Conduction ; Neurologic Manifestations ; Peripheral Nerves* ; Tetraodontiformes ; Tetrodotoxin ; Voltage-Gated Sodium Channels

A considerable number of patients with inflammatory bowel disease (IBD) experience extraintestinal manifestations (EIMs), which can present either before or after IBD diagnosis. Unraveling the pathogenic pathways of EIMs in IBD is challenging because of the lack of reliable criteria for diagnosis and difficulty in distinguishing EIMs from external pathologies caused by drugs or other etiologies. Optimizing treatment can also be difficult. Early diagnosis and management of EIM revolve around multidisciplinary teams, and they should have the resources necessary to make and implement appropriate decisions. In addition, specialists of the affected organs should be trained in IBD treatment. Furthermore, patient awareness regarding the extraintestinal symptoms of IBD is of paramount importance for improving patient understanding of disease and health outcomes. Herein, we review the pathogenesis and clinical perspectives of EIMs in IBD.

In patients with inflammatory bowel disease (IBD), ocular extraintestinal manifestations (EIM) are less common than EIM of other systems, but they are clinically important because they can lead to complications that can cause catastrophic damage to the visual acuity and ocular structure. Anterior uveitis and episcleritis are the most common ocular EIM. Involvement of the orbit, posterior segment, and optic nerve can also occur. A variety of treatments are available ranging from topical steroids to systemic immunosuppressive therapies. The treatment of IBD is also essential if the activity of inflammatory bowel disease affects the ocular symptoms.
Colitis, Ulcerative ; Crohn Disease ; Humans ; Inflammatory Bowel Diseases ; Ophthalmology ; Optic Nerve ; Orbit ; Scleritis ; Steroids ; Uveitis ; Uveitis, Anterior ; Visual Acuity

In patients with inflammatory bowel disease (IBD), ocular extraintestinal manifestations (EIM) are less common than EIM of other systems, but they are clinically important because they can lead to complications that can cause catastrophic damage to the visual acuity and ocular structure. Anterior uveitis and episcleritis are the most common ocular EIM. Involvement of the orbit, posterior segment, and optic nerve can also occur. A variety of treatments are available ranging from topical steroids to systemic immunosuppressive therapies. The treatment of IBD is also essential if the activity of inflammatory bowel disease affects the ocular symptoms.
Colitis, Ulcerative ; Crohn Disease ; Humans ; Inflammatory Bowel Diseases ; Ophthalmology ; Optic Nerve ; Orbit ; Scleritis ; Steroids ; Uveitis ; Uveitis, Anterior ; Visual Acuity

Purpose: In this study, we propose a deep learning (DL)–based voxel-based dosimetry method in which dose maps acquired using the multiple voxel S-value (VSV) approach were used for residual learning. Methods: Twenty-two SPECT/CT datasets from seven patients who underwent 177 Lu-DOTATATE treatment were used in this study. The dose maps generated from Monte Carlo (MC) simulations were used as the reference approach and target images for network training. The multiple VSV approach was used for residual learning and compared with dose maps generated from deep learning. The conventional 3D U-Net network was modified for residual learning. The absorbed doses in the organs were calculated as the mass-weighted average of the volume of interest (VOI). Results: The DL approach provided a slightly more accurate estimation than the multiple-VSV approach, but the results were not statistically significant. The single-VSV approach yielded a relatively inaccurate estimation. No significant difference was noted between the multiple VSV and DL approach on the dose maps. However, this difference was prominent in the error maps. The multiple VSV and DL approach showed a similar correlation. In contrast, the multiple VSV approach underestimated doses in the low-dose range, but it accounted for the underestimation when the DL approach was applied. Conclusion Dose estimation using the deep learning–based approach was approximately equal to that in the MC simulation. Accordingly, the proposed deep learning network is useful for accurate and fast dosimetry after radiation therapy using 177 Lu-labeled radiopharmaceuticals.

Background/Aims: Although pharmacist intervention for patients with chronic diseases has been shown to improve medication adherence, few studies have evaluated its effects on the objective clinical outcomes. We investigated the impact of pharmacist intervention on medication adherence and clinical outcomes in patients with ulcerative colitis (UC). Methods: Patients with UC and low medication adherence were divided into two groups, based on pharmacist intervention. Their medication possession ratio and nonadherence rate for 6 months before and after the baseline were investigated. The partial Mayo score, flare-up incidence, and factors influencing flare-up events for 1 year after the baseline were analyzed. Results: Of 99 patients, 33 and 66 were included in the intervention and control groups, respectively. The nonadherence rate significantly declined in the intervention group 6 months after the baseline (60.6% before vs 30.3% after; p=0.013). The groups showed a significant difference regarding time-related partial Mayo scores (p=0.002). Intervention was significantly negatively correlated with time and the partial Mayo score (r2 =0.035, p=0.013). A significant difference was observed in the flare-up incidence (33.3% in the intervention group vs 54.6% in the control group; p=0.046). Multivariate logistic regression indicated that pharmacist intervention (adjusted odds ratio, 0.370; 95% confidence interval, 0.145 to 0.945; p=0.038) independently reduced the flareup risk. Conclusions Pharmacist intervention significantly decreased the nonadherence rate, improved the partial Mayo score, and reduced the flare-up incidence compared with the control group in a cohort of UC patients identified to have low medication adherence.