Modeling on social spread from immunity

We are now planning to make a transmission model of infectious diseases in the scale of a city. People live in the city contacting other persons with daily life. The model regards a contact as a source of infection. A person will be simulated as a simple system of differential equations. As a candidate of differential equations, we are now investigating Marchuk's simple model. We adopt Marchuk's simple model because it has formation time, i.e., latent time. As Dr. Takeuchi showed, latent time is very important. There remain problems of choosing parameters for special diseases. We are now planning to use Marquardt method to minimize residuals form clinical data to estimate parameters. As for contacts, there are many approaches. The approach of the MIDAS project is very intensive. Our approach is simple. There are about 30,000 Japanese every 15 minutes daily life data, sleeping, eating, work, study, house keeping, etc. Our approach is to make virtual families, husband, wife, children in a city and assign actions from the every 15 minutes data statistically and estimate their contacts in the companies or schools, etc.     

A father and son with Turcot's syndrome: Evidence for autosomal dominant inheritance

Typical Turcot's syndrome is characterized by the association of a brain glioma together with multiple colonic polyposis, in which the number of polypoid lesions is small and the association of colonic cancer occurs at a younger age than in familial adenomatous polyposis. We describe a family in which both the father and his son presented with typical Turcot's syndrome without parental consanguinity. This is the first report of a family that is considered to follow an autosomal dominant inheritance. After reviewing 25 documented cases in which the average age of death was 20.3 years old, it was learned that the major cause of death was brain tumor (76 percent) and the minor cause was colon cancer (16 percent). Patients were very young and, therefore, unlikely to have produced a child before their death. These facts seem to support the theory that Turcot's syndrome is an autosomal dominant disorder.     

Fatal eosinophilic heart disease in a child with neurofibromatosis-1 complicated by acute lymphoblastic leukemia

We present a pediatric case of neurofibromatosis-1 (NF-1) complicated by acute lymphoblastic leukemia and hypereosinophilia, which caused multiple end-organ damage. Although clinical symptoms such as fever and coughing were noted only 1 week before admission, the condition deteriorated rapidly with a fatal outcome prior to antileukemic therapy. A postmortem examination demonstrated extensive endomyocardial fibrosis with thrombotic occlusion and recanalization of the coronary arteries. Leukemic cell infiltration was not seen in the cardiac tissue. When eosinophilia is diagnosed in patients with NF-1, eosinophilic end-organ damage, particularly cardiac involvement, in addition to hematological malignancies, should be screened for in order to start medical treatment at the early stage of the disease.     

Intravascular large B cell lymphoma with migratory local high density shadow by chest CT and diagnosed by transbronchial lung biopsy

The intravascular large B cell lymphoma (IVL) is a rare subtype characterized by the presence of lymphoma cells in the lumina of small vessels. Reported here is the case of a 68-year-old woman with a high-grade fever uncontrolled by antibiotics or antipyretic drugs, and elevation of the serum LDH and sIL-2R levels. After she was admitted, dyspnea, hypoxia, and severe body weight gain with leg edema gradually developed. Chest computed tomography (CT) revealed a characteristic migratory local high density area typical of atelectasis. A diagnosis of IVL was made with a transbronchial lung biopsy (TBLB) and immunohistochemical analysis. The patient was treated with combination chemotherapy (modified CHOP), and her symptoms of dyspnea, hypoxia, pyrexia and leg edema were quickly resolved. The level of LDH and sIL-2R returned to normal, and a complete response was obtained. Although diagnosis of IVL is difficult, an early and appropriate diagnostic procedure (biopsy of tissue with vessels, such as lung and skin, is required) will improve the prognosis of IVL.     

Epitope mapping of anti-alpha-fodrin autoantibody in juvenile Sjögren's syndrome: difference in major epitopes between primary and secondary cases

OBJECTIVE: Juvenile Sj?gren's syndrome (SS) is an early-onset type of SS. Autoantibody against the N-terminal 120 kDa form of a-fodrin is a specific and sensitive disease marker for both juvenile and adult SS. We investigated the initial and major determinants of a-fodrin in SS. METHODS: Sera were obtained from patients with juvenile SS, 10 with primary SS and 10 with secondary SS. Epitope specificities of IgG antibodies were examined by dot-blot analyses using overlapping fusion proteins of the N-terminal part (561 amino acid residues) of a-fodrin as antigens. RESULTS: All sera from patients with primary SS reacted with amino acid residues 1 to 98 and 36 to 150, but not with 91 to 199. Epitope mapping using fusion proteins with subfragments, each consisting of about 50 amino acid residues, showed reactivity with amino acid residues 27-80 and 79-132, suggesting that at least 2 epitopes are contained in the first 150 amino acid residues. All 3 cases with neurological complications had additional epitope specificities. Sera from patients with secondary SS showed more diversified specificities and strongly reacted with amino acid residues 1-98 and 334-432, whereas the reactivities to 36-150, a major epitope in primary SS, were minimal. CONCLUSION: Major and initial B cell epitopes specifically reside in N-terminal amino acids 36-132 and could be used as a diagnostic tool for primary SS. The epitope subsequently expands to other regions of a-fodrin in association with the development of neurological complications or disease progression. Secondary SS has distinct epitope specificities.     

Parent artery occlusion for ruptured “true” posterior communicating artery aneurysm

A case of a patient with a ruptured true posterior communicating artery (PCoA) aneurysm is reported, who had been managed by early endovascular parent artery occlusion with coils. The small blister aneurysm was located at the proximal PCoA itself and directed superiorly. Postoperative course was uneventful. During 1-month follow-up, the patient recovered well and could care for herself. Aneurysms of the PCoA itself are very rare. As reported to date, surgical procedures would favor microsurgical clipping over endovascular coil embolization. Endovascular treatment may be a good alternative to surgical trapping for true PCoA blister aneurysm.