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Biomedical subjects

P Spiegel

Publications and source records attributed to P Spiegel.

17 recordsLinked to original sources

[Osteomalacia as an apparently rare side effect of oral fumaric acid therapy. Secondary DeToni-Debré Fanconi syndrome in the adult].

A case of fully reversible tubular toxicity with consecutive metabolic osteopathy following systemic fumaric acid therapy is reported. This secondary effect of oral fumaric acid therapy obviously occurs very rarely, never having been described before. A 46-year-old female patient with a long history of recurrent palmoplantar psoriasis underwent oral treatment with fumaric acid and its esters in accordance with the Schäfer method, preceding attempts at curative treatment with conventional antipsoriatic agents having proved unsatisfactory. Two months later, the patient started to experience arthralgia, back pain in the early hours of the morning and myalgia with increasing frequency, progressing to disablement in moving and walking and, finally, to total immobility. Not until 9 months later was the reason for these severe disabilities found: they stemmed from hypophosphataemic osteomalacia as a result of a complex disturbance of the renal tubular system. The clinical symptoms and the results of laboratory chemistry tests returned to normal as soon as fumaric acid medication was discontinued. Two attempts at reexposure confirmed the causal relationship. Oral fumaric acid medication should never be administered without clinical and chemical controls.

Administration, Oral

Acquired pendular nystagmus in toluene addiction.

We studied the ocular motor abnormalities in 4 patients chronically addicted to sniffing glue containing toluene. They showed acquired pendular nystagmus with horizontal and vertical components. One patient also showed saccadic oscillations. The pendular nystagmus may be a manifestation of a disturbance in brainstem-cerebellar connections secondary to the toxic effect of toluene on white matter.

Adult

[Treatment of obliterating arteriopathies of the lower limbs using angioplasty. Comparison of 2 techniques of recanalization: Nd-YAG laser and radiofrequency thermal probe].

The Authors report their experience about 20 cases of femoro-popliteal revascularization: in 15 cases a Nd-YAG Laser was used; in 5 cases a unity which employs radiofrequency energy to heat the tip (Thermoprobe) was utilized. We obtained an immediate recanalization in 19 out of 20 superficial femoral arteries with two early thrombosis. In 12 patients of the first group a significant increase of the ankle-brachial index (45%) was seen; it remained unchanged during the follow-up. We had successful revascularization in all the 5 patients of the second group (Thermoplaster). In selected cases, when the lesion is shorter than 10 cm, not completely calcified, and there is a valid run-off, Laser angioplasty could be a valid alternative to surgery or medical therapy.

Aged

Ca sensitivity and acetylcholine receptor currents of twitch and tonic snake muscle fibers.

Myofibrillar Ca sensitivity and single-channel acetylcholine receptor (AChR) currents were studied in garter snake (Thamnophis sirtalis sirtalis) costocutaneous muscle fibers. Nomarski optics were used to identify tonic and fast- and slow-twitch fibers. Measurements of tension generation were made using chemically skinned fibers. The maximum tensions of the three types of fibers were similar, and the fast- and slow-twitch fibers had similar Ca sensitivities. Compared with twitch fibers, tonic fibers had lower threshold Ca concentrations for tension generation and a larger range of Ca concentrations between threshold and maximum tension. The AChR channels were studied by enzymatically removing the nerve terminals and performing patch-clamp recordings on the exposed postsynaptic membrane. Twitch fibers had only one class of end-plate channel with a conductance of approximately 51 pS. Tonic fibers had two types of synaptic channels. One AChR channel in the tonic fibers resembled the type seen in twitch fibers. The other channel in tonic fibers had a smaller conductance of approximately 33 pS and resembled extrajunctional AChRs on denervated twitch fibers.

Acetylcholine

Unusual presentation of paraproteinemic corneal infiltrates.

A 76-year-old man with bilateral corneal infiltrates secondary to a monoclonal gammopathy of undetermined significance (MGUS) is described. The corneal changes simulated a remote lattice dystrophy. Although the clinical presentation of corneal opacities secondary to paraproteinemic conditions is somewhat variable, this patient's corneas lacked the common crystalline appearance of immunoglobulin deposits. This patient demonstrates an atypical clinical presentation of corneal infiltration secondary to a paraproteinemia.

Aged

Audiologic and other clinical findings in a case of basilar artery aneurysm.

A patient with a large, right-sided basilar artery aneurysm was evaluated. Major symptoms included progressive hearing loss, facial numbness, occipital headaches, dizziness, and diplopia of less than a year's duration. Audiologic results indicated a low-frequency sensorineural hearing loss with marked discrepancies between ascending and descending pure-tone thresholds for the ear ipsilateral to the lesion. Auditory brain-stem response demonstrated bilateral abnormalities, but the early waves were normal. Contralateral acoustic reflexes were absent on the right and elevated on the left. Electronystagmography results showed bilaterally absent caloric responses as well as gaze nystagmus and abnormal pursuit movements. Additional audiologic results as well as radiologic and medical findings are also presented.

Aneurysm

A cluster of Pallister-Hall syndrome cases, (congenital hypothalamic hamartoblastoma syndrome).

We report on three infants with hand anomalies and congenital hypopituitarism. In two of the cases, a hypothalamic tumor was found; the third infant died without postmortem brain studies. Family history in the first case suggested possible familial recurrence; the mother's sister had died at 17 hr of age with polydactyly, microglossia, and flat nasal bridge (no autopsy done). Our second case was born by cesarean section after a pregnancy complicated by extremely low maternal estriols. At birth, hypopituitarism was diagnosed, a cranial CT scan was read as normal, and hormonal replacement was begun with thyroxine, hydrocortisone, and growth hormone. At 11.5 mo of age she developed seizures; and a repeat CT scan showed a mass extending beneath the hypothalamus. This tumor was removed surgically at 12 mo, the first successful treatment of this disorder. Our third possible case had a bifid epiglottis, hypopituitarism, and hand anomalies. A CT scan at birth failed to reveal a mass in the hypothalamus. This child died from complications of untreated hypopituitarism, and no neuropathology studies were done. These three cases were conceived between March 10th and April 17th in three different years in three geographically contiguous counties of Vermont. Clustering in time and space and possible familial recurrence, in one of these cases, suggest a possible gene/environment interaction.

Abnormalities, Multiple

[Dialysis encephalopathy and aluminum poisoning? Correlated clinical, pathologic-anatomic and spectrometric findings].

A case of severe dialysis encephalopathy is reported, in which the autopsy finding of an extensive spongiform change in areas of the cortex directly corresponded to high aluminium-concentration in these areas, supporting the theory of an aetiological role for aluminium. A therapeutic approach combining low aluminium intake and removal of aluminium by a chelating agent (desferrioxamine) is promising, but more precise guidelines are needed.

Aluminum

Femoral neuropathy as a complication of heparin anticoagulation therapy.

Femoral nerve compression by retroperitoneal hematoma is an uncommon but significant complication of Heparin anticoagulation. The orthopedic surgeon today, utilizing anticoagulation more frequently than ever, must be ever mindful of the early warning signs and symptoms of this syndrome.

Adult

[Flowmeter test].

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Anesthesia, Inhalation