Child-onset systemic sclerosis positive for anticentromere antibodies: two Japanese cases.

Systemic sclerosis (SSc) is an uncommon connective tissue disease of childhood. Moreover, pediatric SSc positive for anticentromere antibodies (ACA) is extremely rare. We describe two cases of ACA-positive SSc in Japanese girls with clinical findings.

Successful combination treatment of a patient with progressive juvenile localized scleroderma (morphea) using imatinib, corticosteroids, and methotrexate.

We report a case of progressive juvenile localized scleroderma (JLS or morphea) treated with a combination of imatinib, corticosteroids, and methotrexate. This therapy halted the progressive skin thickening and the hand and finger joint deformity in the early stages of the disease. We conclude that imatinib used in addition to standard treatment with systemic corticosteroids and methotrexate may be of therapeutic benefit for individuals with JLS.

Does warfarin help prevent ischemic stroke in patients presenting with post coronary bypass paroxysmal atrial fibrillation?

Purpose: This study examines the efficacy of warfarin in preventing ischemic stroke due to paroxysmal atrial fibrillation (PAF) after coronary artery bypass grafting (CABG).

Methods: Postoperative PAF occurred in 151(33.5%) of 447 patients undergoing conventional CABG.

Subacute thyroiditis in a patient with juvenile idiopathic arthritis undergoing etanercept treatment: a case report and review of the literature.

We report on a 24-year-old woman with juvenile idiopathic arthritis (JIA) who developed subacute thyroiditis (SAT) while being treated with etanercept. She had suffered from JIA for 12 years, and her arthritis proved refractory to treatment with ibuprofen, prednisolone, and methotrexate. For the past 5 years, the patient had been treated successfully with etanercept at 25 mg/week.

A 4-year-old girl with clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection.

Rotavirus is a common cause of severe gastroenteritis in children. It is known that rotavirus gastroenteritis may be accompanied by neurological manifestations, including encephalitis/encephalopathy and seizures. We report a case of a 4-year-old girl with clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection.

Successful childbearing in two women with rheumatoid arthritis and a history of miscarriage after etanercept treatment.

Two women with rheumatoid arthritis who had experienced miscarriages became pregnant while they were under etanercept treatment. One stopped etanercept after 3 weeks with increased doses of prednisolone, and the other restarted etanercept at a half doses 3 months later. They delivered a healthy baby at full term, and no problems in both expecting mothers and babies were observed.

A 7-year-old girl with subcutaneous emphysema, pneumomediastinum, pneumothorax, and pneumoretroperitoneum caused by Mycoplasma pneumoniae pneumonia.

A 7-year-old girl presented with subcutaneous emphysema, pneumomediastinum (PM), pneumoretroperitoneum, and pneumothorax caused by Mycoplasma pneumoniae (MP). The patient had been treated with clarithromycin for pneumonia at another hospital; however, her condition deteriorated and complications developed. Soon after admission to our hospital, we started the patient on minocycline and prednisolone, and the complications improved promptly.

A surgical case of mitral regurgitation due to active infective endocarditis with idiopathic thrombocytopenic purpura.

A 71-year-old woman with idiopathic thrombocytopenic purpura (ITP), who had been treated with steroid and cyclosporine, was admitted in an emergency with fever and dyspnea. The diagnosis was mitral regurgitation due ton infective endocarditis. Although she received treatments for infection and cardiac failure, the cardiac failure could not be controlled.

[Tuberculosis infection in the nervous system].

Most cases of Mycobacterium tuberculosis infection in the nervous system involve tuberculous meningitis (TBM), which is a severe form of extrapulmonary tuberculosis. Patients who demonstrate a subacute clinical course with headache, vomiting, pyrexia and anorexia should be suspected of having TBM. Diagnosis is based on the clinical symptoms, and cerebrospinal fluid changes (increased protein, low glucose and mononuclear cell pleocytosis).

Serum vitamin D concentrations and associated severity of acute lower respiratory tract infections in Japanese hospitalized children.

Background: Vitamin D is an immunomodulatory molecule related to innate immunity that may contribute to the increased occurrence of acute lower respiratory infection (ALRI) in children, one of the most common reasons for hospitalization and intensive care unit admission. In the present study, the association between vitamin D deficiency and the severity of respiratory infection was evaluated by determining serum concentrations of 25-hydroxyvitamin D (25(OH)D) in a group of hospitalized children with ALRI.

Methods: Of the 28 children admitted to Nihon University Nerima-Hikarigaoka Hospital with ALRI over the period November 2008–May 2009, 26 were diagnosed as having bronchiolitis and two were found to have pneumonia.

Aortic balloon used in a cardiac reoperation of a patient with patent coronary grafts.

Reoperation in patients with patent coronary artery bypass grafts behind the sternum is associated with a high risk of graft injury that may be life-threatening. We recently performed mitral valve replacement in a patient with patent coronary artery bypass grafts and grade IV mitral regurgitation. Surgery was safely performed with minimal adhesion dissection and modified Port-Access Technique using an aortic balloon.

Henoch-Schönlein purpura in a child with Guillain-Barré syndrome.

A case of Henoch-Schönlein purpura with Guillain-Barré syndrome in a 3-year-old-girl is presented. This association is extremely rare. During the course of Guillain-Barré syndrome, a decrease in plasma factor XIII activity was noted.

A pediatric patient with acute suppurative thyroiditis caused by Eikenella corrodens.

A previously healthy 6-year-old boy had continuous fever for 6 days before admission to our hospital. His general condition was good except for pyrexia. The left lobe of the thyroid gland was swollen, red, hot, and tender, and neck movement was limited.

Protein-losing enteropathy associated with Henoch-Schönlein purpura.

The gastrointestinal manifestations of Henoch-Schönlein purpura include abdominal pain, gastrointestinal bleeding, intussusception, and perforation. Protein-losing enteropathy is rarely associated with Henoch-Schönlein purpura. Two pediatric patients with Henoch-Schönlein purpura who developed protein-losing enteropathy are reported.

Spontaneous pneumomediastinum complicating pneumonia in children infected with the 2009 pandemic influenza A (H1N1) virus.

We report two occurrences of spontaneous pneumomediastinum (SPM) complicating pneumonia in Japanese children infected with the novel influenza A (H1N1) virus (IV). General practitioners especially should suspect possible SPM when examining and treating children with the novel influenza accompanied by status asthmaticus or wheezing. The presented patients illustrate the specific clinical and radiological signs associated with SPM complicating pneumonia in children infected with A(H1N1)v.

Effect of L-asparaginase combined with vincristine and prednisolone on acute myeloblastic leukemia (M0) associated with non-Hodgkin lymphoma.

A 66-year-old Japanese woman was referred to us because of severe anemia and fever and presented at our hospital. She was eventually diagnosed as having acute myeloblastic leukemia (AML; M0) with non-Hodgkin lymphoma (NHL). We investigated the therapeutic efficacy of L-asparaginase (L-Asp), vincristine and prednisolone for both her AML and NHL.

Transaortic stent grafting for distal arch aneurysm with antegrade and retrograde extracorporeal circulation.

Purpose: The purpose of this study was to determine whether cerebrospinal cord injury can be avoided with transaortic stent grafting for distal aortic aneurysm, and whether this technique can be widely used.

Methods: Seventeen patients underwent distal aortic aneurysm repair with the transaortic stent grafting technique. All patients had a median sternotomy with combined antegrade and retrograde perfusion and selective cerebral perfusion.

A boy with growth disturbance caused by hypothalamic damage associated with intracranial hypotension syndrome following a motor vehicle accident: case report.

We examined the endocrine profile of a boy aged 10 years and 4 months with intracranial hypotension syndrome (IHS) following a motor vehicle accident. His complaint was growth disturbance. GH secretion gradually decreased and finally was lost in spite of an epidural blood patch procedure.

[Three cases with liver metastasis from gastric or colon cancer successfully treated with S-1 combined with CPT- 11].

We report three cases with liver metastasis from gastric or colon cancer successfully treated with S-1 with CPT-11. Case 1: A total gastrectomy was performed for a gastric cancer located in the lower to upper body of the stomach (T3 (SE), N2, H0, P0, por 2, stage III B). Abdominal computed-tomography (CT) revealed a solitary liver metastasis in the S8 subsegment of the liver.

[Case of pontine infarction causing alternating hemiplegia with ipsilateral abducens nerve palsy and contralateral supranuclear facial nerve palsy].

We report a 73-year-old man with alternating abducent hemiplegia (Raymond syndrome) and contralateral supranuclear facial nerve palsy. On admission, he showed lateral gaze palsy of the right eye, left supranuclear facial nerve palsy, dysarthria and left hemiparesis. Brain MRI showed an infarct that was located in the paramedian and lateral area in the base of the caudal pons on the right side.

Mitochondrial encephalomyopathy associated with diabetes mellitus, cataract, and corpus callosum atrophy.

A 44-year-old woman with mitochondrial encephalomyopathy noticed weakness of the lower extremities at the age of 30 years. She also has type 2 diabetes mellitus, posterior subcapsular cataracts in both eyes, and corpus callosum atrophy. Family history showed that a maternal cousin had a myopathy, 3 maternal aunts had diabetes mellitus, and her mother and 2 maternal aunts had cataracts.

Enteral versus parenteral nutrition after gastrointestinal surgery: a systematic review and meta-analysis of randomized controlled trials in the English literature.

Background: Although previous studies recommend the use of enteral nutrition (EN), the benefit of EN after elective gastrointestinal surgery has not been comprehensively demonstrated as through a meta-analysis. Our aim is to determine whether enteral nutrition is more beneficial than parenteral nutrition.

Methods: A search was conducted on Medline, Web of Science, the Cochrane Library electronic databases, and bibliographic reviews.

Chondrosarcoma with myxoid change: a study using a quick-freezing and deep-etching method.

A middle-aged Japanese woman visited the Orthopedics Department of Nihon University Nerima Hikarigaoka Hospital complaining of pain in the left hip joint that had started approximately 8 months earlier. Following several examinations, including imaging diagnoses, an incisional biopsy demonstrated a malignant acetabular bone tumor, which was removed and examined by a quick-freezing and deep-etching (QF-DE) method, conventional electron microscopy, and light microscopy. Histologically, the tumor was a chondrosarcoma with marked myxoid changes.

SLAM-associated protein solves a mystery of autoimmunity.

SLAM-associated protein (SAP) is essential for viral protection, lifelong immune memory (vaccination), and lifelong autoantibody production. We discuss how SAP is a key player in the development of autoimmune disease.