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Biomedical subjects

J Janda

Publications and source records attributed to J Janda.

At least 91 records · Page 5Linked to original sources

Freeman-Sheldon syndrome: a disorder of congenital myopathic origin?

Freeman-Sheldon syndrome was diagnosed in an unrelated adult man and woman, with severe abnormalities of the extremities but only slight anomalies of the face. Electromyography and muscle biopsy showed a myopathy which was classified as a congenital disproportion of fibre type and seemed to be the primary cause of the deformities. This allowed classification of the syndrome as a separate type of myopathic arthrogryposis.

Adult↗

[Secondary hyperparathyroidism in patients on long-term hemodialysis].

Evidence of secondary hyperparathyroidism was evaluated in 233 autopsied patients with uremia (of whom 115 patients had been treated with long-term haemodialysis). Morphology of parathyroid glands correlates with serum level of immunoreactive parathormone. Extraskeletal soft tissue calcification was observed in three different forms: 1st calcifying arteriopathy, 2nd paraarticular soft-tissue calcification and 3rd classic metastatic calcification. Hypocalcemia may lead to any of these 3 forms of tissue calcification and only local factors play a decisive role in determining which type actually occurs. Arteriopathy and paraarticular calcification of soft tissues belong to calciphylaxis and different challenger factors are presumed.

Adrenal Glands↗

[Calcifying uremic arteriopathy].

28 cases of calcifying arteriopathy were found among 217 autopsies of uremic patients (96 of them treated by hemodialysis). It was confined to one organ (10 cases) or generalized (18 cases). Morphology and relation of arteriopathy to secondary hyperparathyroidism were presented. Calcifying uremic arteriopathy ranks among calcifylactic syndromes and is taken for a dystrophic calcification from the formal pathogenetic point of view.

Adult↗

[Skeletal muscle in uremia].

A post mortem histochemical study of girdle muscles was performed in 54 persons having died of uremia (42 of them treated by long-term dialysis). The most important findings was an atrophy of 2B-type fibres which was combined with osteomalacia in 36% dialysed patients. An etiopathogenetical discussion included toxic effects of parathormone and aluminium ions, disturbance of glycolysis and uremic carnitine deficiency.

Adult↗

[Thrombosis of the inferior vena cava with successive lung embolization in a 15-year-old boy with the nephrotic syndrome].

The authors report on a boy with typical course of recent nephrotic syndrome, who developed massive thrombosis involving the iliac inferior holden veins during the standard therapy with corticosteroid. The first sign of this complication was pulmonary embolism. Thrombosis was treated by surgery, during which 3 large thrombi from veins were removed. The patient developed recurrent thrombosis occurring at the previous sites in the later stage and he died 23 days after the first sign of thromboembolic complication. Autopsy showed only minimal glomerular changes. The cause of the death was massive pulmonary embolisation. The aethiopathogenesis of the complication and preventive approach in children with nephrotic syndrome are discussed.

Adolescent↗

[Thrombosis of the inferior vena cava with successive lung embolization in a 15-year-old boy with nephrotic syndrome].

The authors report on a boy with typical course of recent nephrotic syndrome, who developed massive thrombosis involving the iliac inferior holden veins during the standard therapy with corticosteroid. The first sign of this complication was pulmonary embolism. Thrombosis was treated by surgery, during which 3 large thrombi from veins were removed. The patient developed recurrent thrombosis occurring at the previous sites in the later stage and he died 23 days after the first sign of thromboembolic complication. Autopsy showed only minimal glomerular changes. The cause of the death was massive pulmonary embolisation. The aethiopathogenesis of the complication and preventive approach in children with nephrotic syndrome are discussed.

Adolescent↗

[Significance of immunohistochemistry in neuro-oncology. I. Demonstration of glial fibrillary acid protein (GFAP) in extracranial metastases from primary brain tumors].

8 cases were studied to determine whether immunohistochemical investigation with anti-GFAP could contribute to confirming a primary brain tumor origin for an extracranial metastasis. The materials studied consisted of 3 glioblastomas, 3 anaplastic astrocytomas, and 2 medulloblastomas, along with their extracranial metastases. GFAP could be immunohistochemically demonstrated in all 6 primary glial tumors as well as in the metastases of the 3 astrocytomas and of 2 glioblastomas. The medulloblastomas and their metastases were immunohistochemically GFAP-negative. GFAP is thus a marker for extracranial metastases of astrocytomas and glioblastomas. A negative result however does not exclude the possibility that a metastasis is of glial origin as shown by the GFAP-negative metastasis of the one glioblastoma.

Adult↗

[Parathyroid glands in long-term dialysis].

Parathyroid glands from 100 deceased persons treated by hemodialysis were compared with 84 bone lesions and with immunoreactive parathormon in serum of 59 cases. Parathyroid findings were in correlation with the type of bone lesion and parathormon rates. Increase of parathormon in hemodialysed patients reflected the hyperplasia of parathyroid glands.

Bone Diseases↗

[Isolated atrial amyloid].

Isolated atrial amyloid was found in 44 cases among 67 patients deceased at the age of 10-87 years. There was an increase in frequency from 11% in young up to 100% in the old ones. An independent position of this type of amyloid heart dystrophy was discussed according to literature.

Adolescent↗