Two cases of limited cutaneous nodular amyloidosis with primary Sj?gren's syndrome]

We described two female patients with primary Sj?gren's syndrome associated with localized cutaneous nodular amyloidosis (LCNA), in which amyloid protein was derived from immunoglobulin light chain. Case 1; a 70-year-old female had complained with polyarthralgia, low-grade fever and parotid gland swelling. She was diagnosed as primary Sj?gren's syndrome. Three years later she noticed brown color small tumor on the thigh and yellow to brown nodules on the bilateral calves of legs. Skin biopsy from the left thigh revealed amyloid L protein deposition, which was positive for anti-lambda light chain staining, in almost entire dermis. Infiltration of lymphocytes and plasma cells around the amyloid deposit were prominent. Case 2; a 51-year-old female had noticed increasing eruption on the hip. Skin biopsy revealed amyloid L protein deposition in the dermis, which was negative for anti-lambda nor kappa light chain staining. When she was refereed to our hospital, she complained of xerostomia and xerophthalmia. She was diagnosed as primary Sj?gren's syndrome. In both cases, histological examination of a minor salivary gland biopsy revealed infiltration of lymphocytes and plasma cells but not amyloid deposit. Serum M protein and urine Bence-Jones protein were not detected. These cases represent localized amyloidosis without systemic involvement. It is widely recognized that Sj?gren's syndrome is frequently accompanied by B cell lymphoproliferative disorders. In LCNA, infiltration of plasma cells around the amyloid deposits was frequently prominent. The relation between these two disorders is discussed.     

International trends in clinical laboratory testing

During the 20th century, at least until the 1980s, clinical laboratory practice had been rapidly expanded, mainly because of a significant advancement in medicine as a whole and also in laboratory technology. However, recent economic changes in health care environment worldwide have been influencing greatly future trends in clinical laboratory practice. In 1983, the DRG/PPS was introduced in the Medicare hospital care in the United States, and it will be introduced also in out-patient care after the January of 2000. This payment system has been expanded to the HMO, and a drastic change has occurred in clinical laboratory practice, particularly since 1988 when the CLIA'88 was implemented. In Japan, the DRG/PPS is now in the process of preparing for the future introduction into the National Health Insurance System. The clinical laboratory must pursue both quality management and adequate utilization of laboratory tests. In near future, the ISO standards in clinical laboratory testing will prompt to the implementation of quality management in the clinical laboratory. Evidence-based laboratory medicine or systematic review in diagnostic testing will be introduced in order to improve the utilization of clinical laboratory tests.     

ASO Author Reflections: Perspectives of Laparoscopic Hepatectomy with Venous Tumor Thrombectomy for Hepatocellular Carcinoma

Annals of Surgical Oncology -     

Wnt signaling plays an essential role in neuronal specification of the dorsal spinal cord

In the developing spinal cord, signals from the roof plate are required for the development of three classes of dorsal interneuron: D1, D2, and D3, listed from dorsal to ventral. Here, we demonstrate that absence of Wnt1 and Wnt3a, normally expressed in the roof plate, leads to diminished development of D1 and D2 neurons and a compensatory increase in D3 neuron populations. This occurs without significantly altered expression of BMP and related genes in the roof plate. Moreover, Wnt3a protein induces expression of D1 and D2 markers in the isolated medial region of the chick neural plate, and Noggin does not interfere with this induction. Thus, Wnt signaling plays a critical role in the specification of cell types for dorsal interneurons.     

Brain uptake of iodinated L-meta-tyrosine, a metabolically stable amino acid derivative

The possibility of using L-meta-tyrosine (L-mTyr) with high metabolic stability and amino acid transport affinity was evaluated. mTyr was first separated into D- and L-isomers with high-performance liquid chromatography and both were labelled with non-carrier-mediated 125I. Biodistribution and pharmacological studies of radioiodinated mTyr in mice and rats were then performed. 125I-L-mTyr showed greater accumulation in the brain and the pancreas. It accumulated in the brain stereospecifically in the in vivo studies and by the L-tyrosine competitive energy dependent transport system in the in vitro studies. It was resistant to deiodination, appeared to have no retention mechanism and was rapidly excreted. 123I-L-mTyr has the potential of an amino acid transport marker, especially in the brain and the pancreas.     

Insertion of the patella tendon after prosthetic replacement of the proximal tibia

We describe the technique and the results of a method to reconstruct the patellar tendon insertion to a tumor prosthesis by wrapping an artificial mesh around the prosthesis, followed by suturing the patellar tendon and a gastrocnemius flap to the mesh.     

Epileptic cases of late onset with the diffuse slow spike-wave

Twenty-two cases with the diffuse slow spike-wave, whose onset of clinical symptoms was at the age of seven or later, were examined. Half of them presented symptoms pertinent to the Lennox-Gastaut syndrome. The other half were cases of absence, psychomotor seizure or grand mal. The former group was distinguished from the latter in that it had worse prognoses concerning the seizure and intelligence, and discussions were held to see if it corresponds to the "Lennox-Gastaut syndrome of late onset" (Lipinski). Atypical absence and atonic seizure were the commonest symptoms in this group, and they, preceded by other types of seizures such as grand mal, developed mostly in adolescence, and their appearance was closely followed by that of the slow spike-wave on the EEG.     

Membrane structures of human oligodendroglioma

Summary Two cases of McArdle's syndrome are reported. One is a classical exaple; the other is unusual because of the in vitro presence of muscle phosphorylase activity. In the latter case. the electronmicroscopic investigation confirmed the diagnosis.The fine structural changes characteristic of this disease are summarized and it is concluded that histochemical studies alone are insufficient to exclude the diagnosis of McArdle's myopathy.