A case of ARC syndrome was found in a newborn male with persistent cholestasis. He showed renal dysfunction, failure to thrive, and ichthyosis as well as arthrogryposis. The patient who had a dead sibling with similar symptoms has not been described previously in our country.
Arthrogryposis ; Cholestasis ; Failure to Thrive ; Humans ; Ichthyosis ; Infant, Newborn ; Jaundice, Obstructive* ; Male ; Siblings*

PURPOSE: Kawasaki disease is the most common cause of systemic vasculitis in children less than 5 years of age. Recent immunohistochemistry findings suggest that many vascular growth factors play a role in the formation of the coronary artery lesions. Active remodeling of the coronary artery lesions in Kawasaki disease continues in the form of intimal proliferation and neoangiogenesis for several years after the onset of the disease. Intravenous immunoglobulin (IVIG) and corticosteroid have been used in the treatment of Kawasaki disease but the exact mechanism is not clear. We have investigated that IVIG and corticosteroid inhibited vascular endothelial growth factor (VEGF)- induced tube formation of endothelial cells in vitro on Matrigel assay. METHODS: Human umbilical vein endothelial cells (HUVECs) were cultured and seeded on Matrigel coated 24 well plates in medium with or without the following agents: VEGF, VEGF plus IVIG, VEGF plus VEGF antibody, VEGF plus methylprednisolone, VEGF, IVIG plus methylprednisolone for 18 hours. The total length of tube structures in each photograph was quantified. RESULTS: IVIG significantly inhibited the proliferation of HUVECs. The inhibitory effect of IVIG was also reversible. In the meantime, VEGF induced the differentiation of HUVECs into capillary like structures on Matrigel, which was inhibited by VEGF antibody in a dose-dependent manner. Interestingly, IVIG and methylprednisolone inhibited VEGF-induced tube formation of HUVECs. IVIG was more effective in inhibition than methylprednisolone alone. CONCLUSION: We revealed that VEGF induced the differentiation of HUVECs and this effect was inhibited by IVIG and methylprednisolone.
Capillaries ; Child ; Coronary Vessels ; Endothelial Cells* ; Human Umbilical Vein Endothelial Cells ; Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Immunohistochemistry ; Intercellular Signaling Peptides and Proteins ; Methylprednisolone* ; Mucocutaneous Lymph Node Syndrome ; Systemic Vasculitis ; Vascular Endothelial Growth Factor A

OBJECTIVE: To investigate neural correlates associated with recovery of motor function over 6 months in patients with basal ganglia (BG) stroke using acetazolamide (ACZ) stress brain-perfusion single-photon emission computed tomography (SPECT). METHODS: Medical records of 22 patients presenting first-ever BG stroke were retrospectively reviewed. Regional cerebral blood flow (CBF) and cerebrovascular reserve (CVR) were measured for 9 regions in each cerebral hemisphere (primary motor cortex, supplementary motor area, premotor cortex, prefrontal cortex, temporal lobe, parietal lobe, occipital lobe, BG, and thalamus). The Fugl-Meyer Assessment (FMA) motor score was used to assess motor function. RESULTS: After ACZ injection, CBF of all regions of interest (ROIs) increased compared with baseline. Baseline CBF of all ROIs was not significantly correlated with changes in FMA upper or lower motor score. However, multivariate analysis revealed CVR was significantly associated with change in FMA upper score in the ipsilateral primary motor cortex (R2=0.216, p=0.017), the ipsilateral parietal lobe (R2=0.135, p=0.029), and the contralateral primary motor cortex (R2=0.210, p=0.041). CONCLUSION: CVR in the bilateral primary motor cortex and ipsilateral parietal lobe was associated with restoration of upper motor function 6 months after BG stroke. SPECT is a readily available imaging modality useful in studying brain residual function in patients with BG stroke.
Acetazolamide ; Basal Ganglia* ; Brain ; Cerebrovascular Circulation ; Cerebrum ; Humans ; Medical Records ; Motor Cortex ; Multivariate Analysis ; Occipital Lobe ; Parietal Lobe ; Prefrontal Cortex ; Recovery of Function ; Retrospective Studies ; Stroke* ; Temporal Lobe ; Tomography, Emission-Computed ; Tomography, Emission-Computed, Single-Photon*

OBJECTIVE: To investigate neural correlates associated with recovery of motor function over 6 months in patients with basal ganglia (BG) stroke using acetazolamide (ACZ) stress brain-perfusion single-photon emission computed tomography (SPECT). METHODS: Medical records of 22 patients presenting first-ever BG stroke were retrospectively reviewed. Regional cerebral blood flow (CBF) and cerebrovascular reserve (CVR) were measured for 9 regions in each cerebral hemisphere (primary motor cortex, supplementary motor area, premotor cortex, prefrontal cortex, temporal lobe, parietal lobe, occipital lobe, BG, and thalamus). The Fugl-Meyer Assessment (FMA) motor score was used to assess motor function. RESULTS: After ACZ injection, CBF of all regions of interest (ROIs) increased compared with baseline. Baseline CBF of all ROIs was not significantly correlated with changes in FMA upper or lower motor score. However, multivariate analysis revealed CVR was significantly associated with change in FMA upper score in the ipsilateral primary motor cortex (R2=0.216, p=0.017), the ipsilateral parietal lobe (R2=0.135, p=0.029), and the contralateral primary motor cortex (R2=0.210, p=0.041). CONCLUSION: CVR in the bilateral primary motor cortex and ipsilateral parietal lobe was associated with restoration of upper motor function 6 months after BG stroke. SPECT is a readily available imaging modality useful in studying brain residual function in patients with BG stroke.
Acetazolamide ; Basal Ganglia* ; Brain ; Cerebrovascular Circulation ; Cerebrum ; Humans ; Medical Records ; Motor Cortex ; Multivariate Analysis ; Occipital Lobe ; Parietal Lobe ; Prefrontal Cortex ; Recovery of Function ; Retrospective Studies ; Stroke* ; Temporal Lobe ; Tomography, Emission-Computed ; Tomography, Emission-Computed, Single-Photon*

Objective: The objective of this study was to evaluate the safety and efficacy of light-emitting diode therapy (LEDT) in the management of pain and stiffness in patients with refractory hand tenosynovitis to non-steroidal anti-inflammatory drugs. Methods: A total of 12 patients were enrolled in the study and received LEDT twice a week for four weeks. Sociodemographic, clinical, and laboratory data were collected, and the visual analog scale (VAS) pain and stiffness scores of each hand were assessed every two weeks. The thickness of the flexor tendon in the patients’ hand was evaluated using ultrasonography. To investigate the molecular effects of LEDT, we measured the expression levels of type III collagen in tendon cells, with and without LEDT treatment. Results: After undergoing LEDT, participants showed clinically significant improvements in VAS pain scores at weeks 2, 4, and 8 compared to their baseline, and in VAS stiffness scores at weeks 4 and 8. According to the ultrasonography results, there was a decreasing tendency in tendon thickness for each finger in week 8 compared to the baseline, but the difference was not statistically significant. No adverse events were reported. Additionally, our results indicated a significant increase in type III collagen levels in the LEDT group compared to the control group (1.48±0.18 vs. 0.99±0.02, p=0.031), indicating a potential molecular mechanism for the observed clinical improvements. Conclusion LEDT may provide a viable alternative to pharmacological treatments in the future, due to its simple and easy method of administration.

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder characterized by fever, pancytopenia, hyperferritinemia, and phagocytosis of hematopoietic cells in bone marrow, liver, or lymph nodes. HLH can occur during the course of systemic lupus erythematosus (SLE), but can also be a presenting manifestation. Because development of pancytopenia occurs in less than 10 percent of SLE cases, investigation for HLH is necessary when otherwise unexplained pancytopenia persists despite adequate treatment. We experienced three cases of secondary HLH associated with SLE. Among the three patients, two patients developed HLH during the clinical course of SLE. The other patient who presented with pancytopenia was first diagnosed with HLH, and later with SLE. In her case, HLH turned out to be a presenting manifestation of SLE. We report on three successfully treated cases, and discuss the prevalence, characteristics, treatments, and prognosis of secondary HLH associated with SLE.
Bone Marrow ; Fever ; Humans ; Liver ; Lupus Erythematosus, Systemic ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia ; Phagocytosis ; Prevalence ; Prognosis

The spondyloarthropathies are a group of inflammatory rheumatic diseases including ankylosing spondylitis, reactive arthritis, psoriatic arthritis, arthritis associated with inflammatory bowel disease, and undifferentiated spondyloarthropathy. It usually begins in young or middle aged adults, but sometimes presents with atypical symptoms in the older patients. Late onset spondyloarthropathies are characterized by severe disease, marked elevation of laboratory parameters of inflammation, oligoarthritis involving the lower limbs with edema of the extremities. We described a 66-year-old patient who presented with asymmetric polyarthritis mainly involving lower extremities with fever, marked elevation of laboratory inflammatory markers, and was successfully treated with mini-pulse corticosteroids with disease modifying antirheumatic drugs.
Adrenal Cortex Hormones ; Adult ; Aged ; Antirheumatic Agents ; Arthritis ; Arthritis, Psoriatic ; Arthritis, Reactive ; Edema ; Extremities ; Fever ; Humans ; Inflammation ; Inflammatory Bowel Diseases ; Lower Extremity ; Middle Aged ; Rheumatic Diseases ; Spondylarthropathies* ; Spondylitis, Ankylosing

Suppurative sialadenitis is rare in the neonate and usually involves the parotid glands. Isolated suppurative submandibular sialadenitis in the neonatal population is extremely rare. There are only 11 cases found in the literature of suppurative submandibular sialadenitis occurring as an isolated lesion. We describe a case of isolated submandibular sialadenitis progressing to submandibular abscess requiring incision and drainage in a term neonate. Pus culture yielded methicillin-resistant Staphylococcus aureus (MRSA). A brief review of literature is included.
Abscess* ; Drainage ; Humans ; Infant, Newborn ; Methicillin-Resistant Staphylococcus aureus ; Parotid Gland ; Sialadenitis ; Suppuration

Rat bite fever is a rare, systemic illness caused by Streptobacillus moniliformis or Spirillum minus following a rat or other rodent bite. Characteristically, fever develops abruptly with maculopapular skin rash after an incubation period of two to ten days, and asymmetric migrating polyarthritis starts later in up to 50% of patients. The arthritis involves the knees, shoulders, elbows, wrists and hands, which may either be suppurative or non-suppurative. Although most cases seem to resolve spontaneously within two weeks, the mortality in untreated cases is around 10~15%. The response to antibiotic treatment is good and early diagnosis is the most important prognostic factor. We report a patient who developed arthritis with fever after biting by rat.
Animals ; Arthritis* ; Early Diagnosis ; Elbow ; Exanthema ; Fever ; Hand ; Humans ; Knee ; Moniliformis ; Mortality ; Rat-Bite Fever* ; Rats* ; Rodentia ; Shoulder ; Spirillum ; Streptobacillus ; Wrist

Primary non-Hodgkin's lymphoma of the extrahepatic bile duct presenting as obstructive jaundice is extremely rare. A 60-year-old man was admitted due to suddenly developed jaundice. Computerized tomography and endoscopic retrograde cholangiopancreatography showed a tumor at the proximal common hepatic duct. These clinical and radiologic findings resembled those of Klatskin tumor. The resection of the common hepatic duct tumor, lymph node dissection, and Roux-en-Y hepaticojejunostomy were carried out. There was no regional lymph node metastasis and no residual tumor at the resection margins. Histology and immunohistochemistry of the resected specimen confirmed a diffuse large B-cell malignant lymphoma involving the common hepatic duct. The patient is scheduled to receive adjuvant chemotherapy. In summary, primary non-Hodgkin's lymphoma of the extrahepatic bile duct, despite its rarity, should be considered in the differential diagnosis of causes for obstructive jaundice. An accurate histopathologic diagnosis and surgical resection combined with chemotherapy may be the approach to offer a chance for cure.
Antigens, CD20/metabolism ; Bile Duct Neoplasms/*diagnosis/pathology/surgery ; Cholangiopancreatography, Magnetic Resonance ; Diagnosis, Differential ; Humans ; Klatskin's Tumor/diagnosis ; Lymphoma, Large B-Cell, Diffuse/*diagnosis/pathology/surgery ; Male ; Middle Aged ; Tomography, X-Ray Computed