Plasma cell dyscrasia with kappa light-chain crystals in proximal tubular cells: a histological, immunofluorescent, and ultrastructural study

This is a case report of a 56-year-old man with plasma cell dyscrasia who presented with proximal tubulopathy manifested as kappa light-chain crystal deposition in the proximal convoluted tubular cells. This was associated with mild cellular damage. The crystals were seen as negative images with the hematoxylin-eosin and periodic acid-Schiff (PAS) stains. They were identified as kappa light-chains by immunofluorescent imaging and confirmed by immunoelectron microscopy. Ultrastructurally, the crystals appear to be located within lysosomes. No deposits of light-chains were seen elsewhere in the kidney biopsy.     

Basilar artery angulation and vertigo due to the hemodynamic effect of dominant vertebral artery

Vertebral arteries form the basilar artery at the pontobulbar junction. The vertebral artery may have dominancy in one of them. The branches of basilar arteries supply blood for the vestibular nuclei and its connections. Vertigo is seen generally in the upper middle aged patients. Vertigo can be observed in dolichoectasia of basilar artery such as angulation and elongation, because of the diminished blood supply and changed hemodynamic factors of vestibular nuclei and its connections. We hypothesized that angulation or elongation of basilar artery can be estimated according to the unilateral vertebral artery dominant hypertensive patients. The basilar artery can angulate from the dominant side of vertebral artery to the recessive side. These angulation and elongation can effect the hemodynamic factors in absence of growing collateral arteries. So, the vertigo attacks may occur in these patients.     

High cystine in platelets from patients with nephropathic cystinosis: a chemical, ultrastructural, and functional evaluation

AIM: To investigate the morphology and function of platelets in nephropathic cystinosis (NC).METHODS: Seven patients (mean age, 6.5 years; SD, 20 months) with NC were investigated. Their platelets were examined by transmission electron microscopy (TEM) and the characteristics of the dense granules (DGs) were determined by mepacrine labelling and the uranaffin reaction. Bleeding time, turbidometric aggregation, and luminescence aggregation were studied and intraplatelet cystine was measured. RESULTS: Increased intraplatelet cystine, primary and secondary aggregation defects, and the absence of ATP release were demonstrated. TEM revealed DGs of various shapes and sizes and lamellary or amorphous cytoplasmic inclusions. Viscous material had been released into the vacuolar spaces and enlarged open canalicular system. Mepacrine labelling revealed that the numbers of DGs/platelet were comparable between the patients and the controls (mean, 2.9 (SD, 0.22) v 3.32 (0.18); p = 0.34). The uranaffin reaction revealed that the numbers of type 1, 3, and 4 DGs were comparable between the patients and the controls, but that there were fewer type 2 DGs in the patients (mean, 8.5 (SD, 1.95) v 17.22 (1.58); p = 0.01). TEM for platelet aggregation revealed a lack of induction and/or defective execution and/or delayed transmission. The patients' intraplatelet cystine concentrations were higher than the controls (mean, 1.56 (SD, 0.84) v 0.08 (0.01) nmol/mg protein; p = 0.009). CONCLUSIONS: This is the first report to demonstrate raised intraplatelet cystine, abnormal platelet ultrastructural findings, and defective aggregation in NC.     

The effect of extracorporeal shock wave treatment (ESWT) on bone defects. An experimental study.

The aim of this study was to investigate the effects of extracorporeal shock wave therapy (ESWT) on the formation of callus in bone defects created in rabbit radii. this study searches for an answer to whether ESWT may have a therapeutic effect on bone defects. A bone defect with a radius of 1 cm was created in both forelimbs of 20 rabbits. At the 7th, 14th, and 21st days ESWT treatment was applied to the forming callus in the right radius under fluoroscopic control. At the 6th and 12th weeks, the animals were sacrificed and callus analysis was performed by computerized scan, dual energy x-ray absorptiometer. Histological analyses were also performed. The results revealed that the average callus area in the right (ESWT applied) radial defect was greater in both groups and statistically significant at the 12th week (p < 0.05). There was no difference in bone density between defects. Histologically the callus area was greater on the right side (ESWT applied side) in both groups. However in the first group trabeculae were occupying less space on the right side. Granulation tissue areas and chondroid areas were greater on the right side. We conclude that ESWT has a disorganizing and dispersing rather than a direct osteoinductive effect on forming callus. This effect may play a therapeutic role in bone defects and in situations like callus lengthening where a greater amount of callus is necessary.     

Cerebral infarct associated with factor V Leiden mutation in a boy with hemophilia A

An 11-year-old boy with mild hemophilia A was admitted to our hospital because of focal convulsions. Magnetic resonance imaging showed an old occipital infarct. Protein C, S, antithrombin III, anticardiolipin antibodies and fibrinogen were normal. Heterozygosity for factor V Leiden mutation was detected. We suggest that factor V Leiden mutation should be studied in hemophiliacs with thrombosis. Am. J. Hematol. 56:189–190, 1997. © 1997 Wiley-Liss, Inc.     

Factor V Q 506 mutation in children with thrombosis

The factor V Leiden mutation in 12 children with thrombosis and in 20 controls was investigated. Five heterozygous individuals and 1 homozygous individual among the cases with thrombosis and 1 heterozygous individual among controls were found. Central nervous system thromboses were increased in children with the factor V mutation, associated with protein S deficiency. © 1996 Wiley-Liss, Inc.