SAP discovery: the sword edges--beneficial and harmful.

We cloned the SLAM associated protein (SAP) gene in 1995. In 1998, it was discovered that the SAP gene was defective in patients with X-linked lymphoproliferative disease. Subsequently, details on the key role of life-long immune memory (vaccination) and of life-long autoantibody production in patients suffering from autoimmune disease have been revealed.

Quantitative nested real-time PCR assay for assessing the clinical course of tuberculous meningitis.

Although the "gold standard" for diagnosis of tuberculous meningitis (TBM) is bacterial isolation of Mycobacterium tuberculosis (M. Tb), there are still several complex issues. Recently, in the diagnosis of TBM, the detection of M.

What is after cytokine-blocking therapy, a novel therapeutic target--synovial Epstein-Barr virus for rheumatoid arthritis.

There has been significant progress in cytokine-blocking therapy for treatment of rheumatoid arthritis (RA) However, inhibition of cytokines involved in immune defense raises severe side effects. The cost of cytokine-blocking treatment is another major issue. Why are levels of inflammatory cytokines increased in RA patients? We have a large amount of circumstantial and direct evidence for the presence of Epstein-Barr virus (EBV) in RA synovial cells.

Placement of two types of spiral Z-stents at the bronchial carina for the treatment of terminal lung cancer--a new method.

Dumon Y-stents and Dynamic stents are used to treat carinal stenosis, but their placement severely impairs the expectoration of secretions, making frequent bronchoscopic aspiration necessary. We report here five patients with terminal lung cancer who had stenosis of the lower trachea and main bronchi treated using spiral Z-stents. A long tapered spiral Z-stent was placed in the lower trachea and one main bronchus, and a short straight spiral Z-stent in the contralateral main bronchus.

Application of a quick-freezing and deep-etching method to pathological diagnosis: a case of elastofibroma.

A case of elastofibroma in a middle-aged Japanese woman was examined by the quick-freezing and deep-etching (QF-DE) method, as well as by immunohistochemistry and conventional electron microscopy. The slowly growing tumor developed at the right scapular region and was composed of fibrous connective tissue with unique elastic materials called elastofibroma fibers. A normal elastic fiber consists of a central core and peripheral zone, in which the latter has small aggregates of 10 nm microfibrils.

Limited availability of nutritional vitamin D causing inappropriate treatment of vitamin D deficiency rickets with a response resembling pseudohypoparathyroidism type II in a Japanese patient.

Vitamin D deficiency rickets occasionally resembles pseudohypoparathyroidism type II (PHP type II) with respect to the response to exogenous PTH in the presence of hypocalcemia. We encountered a Japanese patient with stage 2 vitamin D deficiency rickets, who had increased urinary cAMP excretion and no response of urinary phosphate or N-acetyl-beta-D-glucosaminidase excretion to exogenous PTH under normocalcemic and normophosphatemic conditions, after treatment with 1,25(OH)2 vitamin D3. This case shows that it is possible for a response mimicking that of PHP type II to occur when the serum calcidiol level is low due to causes other than hypocalcemia and secondary hyperparathyroidism.

Recording of surgery with two crane-type tripods and video cameras.

At medical meetings, a video-recorded surgical procedure can be more persuasive than an oral or poster presentation. Because operating rooms generally have limited space, video setups must be efficient and effective. Patients undergoing hepatectomy and rectal resection were recorded using two sectional crane-type tripods and video cameras placed in different positions.

[A case of acute autonomic, sensory and motor neuropathy (AASMN)--less favorable response of the autonomic dysfunctions to IVIg treatment].

We report a rare case of acute autonomic, sensory and motor neuropathy (AASMN). The patient, a 26-year-old woman, developed fever and common cold around January 20, 2001 and was admitted because of abdominal pain due to ileus on January 30. After admission, the patient complained of muscle weakness and numbness in the extremities, difficulty in seeing with the right eye, and dysuria.

Squamous cell carcinoma with sarcomatous feature (so-called carcinosarcoma) of stomach probably metastasized from esophageal tumor: a case report with quick-freezing and deep-etching method.

A squamous cell carcinoma (SCC) with sarcomatous features (so-called carcinosarcoma) of stomach is reported in a 72-year-old man. The gastric submucosal tumor (12 x 11 x 6 cm) consisted of carcinoma cells and sarcomatous spindle cells, which were immunohistochemically recognized to contain high molecular weight cytokeratin. These histological and immunohistochemical results indicated that carcinoma cells and spindle tumor cells had cytokeratin similar to that of stratified squamous epithelium.

[A case of long survival with UFT and lentinan treatment in a patient with peritoneal metastasis of gastric carcinoma].

The patient was a 50-year-old female with peritoneal metastasis of Type 4 gastric cancer. She underwent a relative curative resection with total gastrectomy and peritonectomy. Postoperative chemotherapy with 5'-DFUR following 5-FU and CDDP was performed.

Examination of expandable metallic stent removed at autopsy.

Objective: We evaluated the damage to expandable metallic stents (EMS) based upon analysis of EMS removed at autopsy.

Methodology: Seventeen EMS were obtained during autopsy from the main bronchi of nine patients with lung cancer. Each EMS was categorized into one of the following three groups, according to the degree of EMS damage: no damage at all (ND), damage to part of the EMS (PD), and marked damage (MD) that caused loss of function of the airway stent.

[A central nervous system lupus showing peculiar findings on cranial magnetic resonance imaging (MRI)].

We report a case of central nervous system (CNS) lupus showing peculiar findings on cranial magnetic resonance imaging (MRI) with remarkable improvement after corticosteriod therapy. The patient was a 28-year-old woman, admitted to our hospital with severe fever, general malaise, and facial edema on June 4, 2001. After admission, she was diagnosed with systemic lupus erythematosus (SLE).

[A case of anaphylactoid shock occurring immediately after the initiation of second intravenous administration of high-dose immunoglobulin (IVIg) in a patient with Crow-Fukase syndrome].

We report a case of anaphylactoid shock occurring immediately after the initiation of second intravenous administration of high-dose immunoglobulin (IVIg) in a patient with Crow-Fukase syndrome. The patient was a 57-year-old woman, who was admitted to our hospital because of numbness and muscle weakness in the four extremities, difficulty in walking, and foot edema. On admission, her skin was dry and rough, and also showing scattered pigmentation, small hemangiomas, and hypertrichosis in both legs.

Agenesis of the internal carotid artery and congenital pituitary hypoplasia: proposal of a cause of congenital hypopituitarism.

Unlabelled: We describe a patient with microphallus without pigmentation and multiple pituitary hormone deficiencies. The left internal carotid artery and carotid canal were absent and the pituitary gland and sella turcica showed hypoplasia on MRI and magnetic resonance angiography. The internal carotid artery develops in the 4th embryonic week, while the pituitary primordium develops in the 3rd to 4th week.

[A case of refractory tuberculous meningitis markedly improved by intrathecal administration of isoniazid (INH)].

We report a case of refractory tuberculous meningitis which was markedly improved by intrathecal administration of isoniazid (INH). The patient was a 35-year-old woman diagnosed with systemic lupus erythematosus (SLE) at age 25, who was being managed with steroid therapy. She was admitted to another hospital due to miliary tuberculosis at age 34, and after discharge continued with a regimen of 2 anti-tuberculosis drugs (INH.

A case of growth failure caused by 13-CIS-retinoic acid administration after bone marrow transplantation for neuroblasoma.

We report an 11-year-old girl with growth failure caused by long-term administration of 13-cis-retinoic acid after bone marrow transplantation for neuroblastoma. Her growth velocity was 1-2 cm/year after 13-cis-retinoic acid administration. Her endocrinological findings were normal except for peak growth hormone levels of 6.

Intravenous ulinastatin therapy for Stevens-Johnson syndrome and toxic epidermal necrolysis in pediatric patients. Three case reports.

Background: More effective therapy is needed for the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The clinical efficacy of intravenous ulinastatin therapy was investigated in 3 Japanese pediatric patients with SJS or TEN.

Methods: Ulinastatin was given to 1 pediatric SJS patient and 2 pediatric TEN patients within 7 days (patient 1; SJS), 6 days (patient 2; TEN), or 4 days (patient 3; TEN) after the onset of the skin rash.

A case report: a pediatric patient with acute lupus hemophagocytic syndrome; differences from reactive hemophagocytosis caused by hypercytokinemia.

Abstract We report the youngest known girl with acute lupus hemophagocytic syndrome (ALHS) at the onset of her illness. We investigated the pathogenesis of ALHS by assessment of factors thought to influence the onset, such as cytokines, Th1/Th2 balance, immune complexes, and autoantibodies. A girl 8 years and 10 months old with systemic lupus erythematosus (SLE) had high fever, pancytopenia, and hemophagocytosis in the bone marrow.

[A case of Folliculitis barbae Candidomycetica].

A 71-year-old man was referred to our department on January 30, 1998 with hard red papules that had developed on the philtrum in mid-January. On January 2, the patient had received high-dose steroid therapy (pulse therapy) for cluster asthma attacks and antibiotics at the Department of Internal Medicine of our hospital. Infiltrative, protruding reddish plaques were observed on the philtrum, which contained a number of small pustules at sites corresponding to hair follicles.

[A new sedation technique with propofol during spinal anesthesia].

We compared our new sedation technique with propofol during spinal anesthesia (Group B, n = 50) with a previously described method by Mackenzie et al. (Group A, n = 20). In Group A, propofol was started at a rate of 6 mg.

[A case of multiple cranial neuropathy with positive antinuclear antibody responded to steroid].

A 56-year-old woman was admitted to our hospital because of bilateral ptosis, total ophthalmoplegia, bilateral facial palsy and left hypoglossal nerve palsy. Antinuclear antibody (ANA) showed high titer of 1280 but other data were normal. With oral prednisolone therapy (40 mg/day), the symptoms improved gradually but ANA titer did not show any significant change.