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Biomedical subjects

R Lachman

Publications and source records attributed to R Lachman.

67 records · Page 4Linked to original sources

Pseudohypohyperparathyroidism: a case study of differential resistance to parathyroid hormone.

OBJECTIVE: To present a rare case of a combination of hyperparathyroidism and hypoparathyroidism in a 30-year-old woman. METHODS: We review the laboratory, radiographic, and pathologic findings in a healthy-appearing woman who sustained a patellar fracture after a simple fall at home. RESULTS: Our patient had features of hypoparathyroidism--that is, tetanic crises and hypocalcemia--and also hyperparathyroidism--fracture of the patella, multiple bone cysts, and confirmed osteitis fibrosa cystica on bone biopsy specimens. These features were associated with a high serum level of intact parathyroid hormone (PTH) and parathyroid hyperplasia. A lack of response of nephrogenic cyclic adenosine monophosphate (cAMP) to PTH stimulation was observed along with subnormal serum cAMP responses. In contrast, urine phosphate excretion increased after administration of PTH. CONCLUSION: These results demonstrate a state of renal PTH resistance in a patient with osteitis fibrosa cystica.

Journal Article↗

CT of the temporal bone in achondroplasia.

In an attempt to better define the changes affecting the temporal bone that might predispose achondroplastic dwarfs to otitis media, nine achondroplastic subjects who were evaluated for hearing loss underwent high-resolution CT scanning of the temporal bone. Comparisons were made with 10 nonachondroplastic subjects. A number of morphologic changes were seen, including (1) poor development of mastoid air cells, (2) foreshortening of the carotid canals, (3) narrowing of the skull base, (4) "towering" petrous ridges, and (5) relative "rotation" of the cochlea and other temporal bone structures. The most significant change was the rotational effect, which was more pronounced medially, resulting in an abnormal orientation of inner ear structures relative to middle ear structures and of middle ear structures relative to the external auditory canal. There was a notable lack of evidence for otitis media or its sequelae in any of the achondroplastic subjects. Audiograms were obtained in six of the nine achondroplastic subjects (two adults and four children). There was evidence of mixed hearing loss in the four children, but only of sensorineural hearing loss in the adults. We believe that the persistent hearing loss in achondroplasia is not due to sequelae of otitis media as some authors have suggested. Intrinsic vestibulocochlear changes below the limits of resolution of high-resolution CT scanning may be responsible.

Achondroplasia↗

In vitro effects of iodinated contrast media on the growth of staphylococci.

Seven commonly used arthrographic contrast media were examined in vitro for bactericidal effects against Staphylococcus aureus under various conditions simulating clinical settings, using a wide range of bacterial populations (10(1) to 10(8) colony-forming units per ml). The media tested were: sodium diatrizoate and megulumine diatrizoate (E.R. Squibb and Sons, Renogragin-60 and Renografin-76), megulumine diatrizoate (E. R. Squibb and Sons, Renografin-M-60), sodium iothalamate and megulumine iothalamate (Mallinckrodt Chemical Works, Vascoray), sodium iothalamate (Mallinckrodt Chemical Works, Conray 400), meglumine iothalamate (Mallinckrodt Chemical Works, Conray 60), and sodium diatrizoate (Winthrop Laboratories, Hypaque sodium 50%). Effects secondary to the contrast medium used, dilution ratios of the specimens, and time elapsed between the collection and cultivation of the specimens were examined. The contrast material used was found to affect bacterial growth: the Renografin materials showed a bactericidal or bacteriostatic effect, while Vascoray, the Conray materials, and Hypaque sodium 50% failed to inhibit bacterial growth. Bacterial inoculum size exhibited a direct bearing on the bactericidal or bacteriostatic effects of the Renografin but had no effect with the other media. The results indicate that Vascoray, Conray and Hypaque sodium 50% are superior to the Renografin materials for use in arthrography and other procedures in which the aspirate is submitted for microbial culture, because they interfere less with the recovery of these microorganisms.

Contrast Media↗

Kasabach-Merritt syndrome-associated kaposiform hemangioendothelioma successfully treated with cyclophosphamide, vincristine, and actinomycin D.

PURPOSE: The authors introduced a successful trial of a chemotherapy regimen to treat refractory thrombocytopenia and pain caused by a rare kaposiform hemangioendothelioma that had been refractory to corticosteroids. PATIENT AND METHOD: A 6-year-old Latina girl with diffuse petechiae, constant pain and edema in her right lower extremity, and severe thrombocytopenia was brought for treatment. Physical examination revealed a large mass in the right lower extremity. The surgical specimen resected earlier in Mexico showed kaposiform hemangioendothelioma, an extremely rare vascular tumor that is frequently associated with consumptive coagulopathy and lymphangiomatosis. Corticosteroid therapy in Mexico had been ineffective in controlling the tumor size, pain, or thrombocytopenia. The patient was brought to the United States because of a rapidly enlarging tumor and intractable leg pain, causing inability to ambulate. Laboratory examinations revealed profound thrombocytopenia and evidence of consumptive coagulopathy. Because of the rapidly enlarging lesion, it was decided to give six cycles of a chemotherapy regimen consisting of vincristine, cyclophosphamide, and actinomycin D (VAC). RESULT: The patient responded to the first cycle of chemotherapy with partial reversal of the thrombocytopenia. The additional five cycles of the same chemotherapy regimen resulted in complete resolution of thrombocytopenia and significant improvement of the pain and edema in the right lower extremity. A follow-up imaging study 9 months later revealed a marked decrease in size of the vascular tumor. CONCLUSION: Inoperable kaposiform hemangioendothelioma unresponsive to corticosteroids can be successfully treated with chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗