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Biomedical subjects

B Pastakia

Publications and source records attributed to B Pastakia.

At least 19 recordsLinked to original sources
PubMed1992

Developmental defects in Gorlin syndrome related to a putative tumor suppressor gene on chromosome 9.

Gorlin syndrome is an autosomal dominant disorder that predisposes to basal cell carcinomas of the skin, ovarian fibromas, and medulloblastomas. Unlike other hereditary disorders associated with cancer, it features widespread developmental defects. To investigate the possibility that the syndrome is caused by mutation in a tumor suppressor gene, we searched for loss of heterozygosity in 16 sporadic basal cell carcinomas, 2 hereditary basal cell carcinomas, and 1 hereditary ovarian fibroma and performed genetic linkage studies in five Gorlin syndrome kindreds. Eleven sporadic basal cell carcinomas and all 3 hereditary tumors had allelic loss of chromosome 9q31, and all informative kindreds showed tight linkage between the Gorlin syndrome gene and a genetic marker in this region. Loss of heterozygosity at this chromosomal location, particularly in hereditary tumors, implies that the gene is homozygously inactivated and normally functions as a tumor suppressor. In contrast, hemizygous germline mutations lead to multiple congenital anomalies.

Basal Cell Nevus Syndrome↗
PubMed1990

Immediate postexcision mammography for occult noncalcified breast lesions.

To evaluate completeness of surgical excision, we studied the use of film screen mammography done immediately after excision of occult noncalcified breast lesions in 11 patients. The postoperative mammograms were well tolerated, and allowed high quality films to be obtained. Complete excision was documented in ten patients, and one patient had small artifacts that resolved on follow-up studies. Roentgenograms of the surgical specimen from ten patients showed a discrete lesion in only three. Histologic analysis revealed malignancy in two patients and benign changes in nine. These findings indicate that mammography done immediately after excision of occult noncalcified lesions is more reliable than roentgenograms of the specimen and may be a useful method for confirming extent of excision. This method may also eliminate the present delay of four to six weeks before adequate postoperative follow-up films can be obtained to evaluate excision.

Adult↗
PubMed1988

Arthropathy in acromegalic patients before and after treatment: a long-term follow-up study.

The medical records of 90 patients with acromegaly were reviewed. Arthralgias were noted in 76% of the patients with 17% having the onset of joint pain concomitant with the clinical onset of acromegaly. Of 47 patients followed prospectively for 5 or more years after pituitary irradiation, six (12.8%) were unaffected by arthralgias. A statistically higher mean baseline growth hormone level was found for the 19 (40.4%) radiotherapy patients who had severe and disabling arthropathy. Mean intervals between clinical onset of acromegaly and the development of arthropathic symptoms were shorter (4.1 years) for patients over 40 years of age and longer (9.7 years) for those under 31 years of age. Severely affected patients tended to have increased joint spaces in both weight-bearing and non-weight-bearing joints followed by a progressive decrease in joint spaces. Arthropathy is a common complication of acromegaly and may progress independently of a fall in growth hormone, induced by any form of treatment, once significant cartilage overgrowth develops. Cartilage overgrowth is a predisposing factor in the development of an arthropathy associated with the wide range of growth hormone levels characteristic of acromegaly.

Acromegaly↗
PubMed1988

Hepatosplenic candidiasis: wheels within wheels.

Eight immunocompromised cancer patients with tissue-proved candidiasis underwent serial abdominal ultrasound (US) and computed tomography (CT). At US, four patterns of hepatic and splenic candidiasis were recognized, one of which the authors call a "wheels-within-wheels" pattern. In addition, periportal linear areas of increased attenuation, possibly calcified, were identified at follow-up, nonenhanced CT. Some abscesses were better seen on nonenhanced CT scans, while others became visible only with enhancement. Although lesions not seen at US were often seen at CT, the opposite was also true. In two cases, pathologic proof of candidiasis was established even when all imaging studies were normal. For maximum imaging sensitivity, patients should be studied with US and nonenhanced and enhanced CT. Even when both US and CT scans are negative, if there is a strong clinical suggestion of candidiasis, open biopsy is recommended.

Abscess↗
PubMed1988

Hepatic candidiasis in cancer patients: the evolving picture of the syndrome.

Focal hepatosplenic candidiasis has been recognized with increasing frequency in recent years. We reviewed the cases of eight patients seen between 1982 and 1985, and information on 60 patients whose cases have been reported in the world literature. The characteristics of focal hepatosplenic candidiasis include persistent fever in a neutropenic patient whose leukocyte count is returning to normal, often coupled with abdominal pain; an elevated alkaline phosphatase level; and less commonly, rebound leukocytosis. The characteristic "bull's eye" lesions seen with hepatic ultrasound examination or computed tomography generally are not detectable until neutrophil recovery has occurred. Diagnosis can be established only by biopsy evidence of yeasts or pseudohyphae in the granulomatous lesions. Cultures are frequently negative, however, especially in patients who have been pretreated with antifungal agents. We review the evolving nature of hepatosplenic candidiasis, focusing on diagnosis and treatment.

Adolescent↗
PubMed1987

Seventeen year follow-up and autopsy findings in a case of massive osteolysis.

Clinical, radiologic, and pathologic findings in an unusual case of massive osteolysis are described. Peculiar clinical episodes of a foul body odor with fever and erythematous skin lesions, presence of radiographic findings such as acro-osteolysis and periarticular erosions, and the absence of angiomatous or lymphangiomatous dysplasias at autopsy, indicate that this is a new form of massive osteolysis rather than a variant of Gorham disease.

Bone and Bones↗
PubMed1987

Sinus histiocytosis with massive lymphadenopathy.

Gallium uptake corresponding to the extent of the disease in a patient with histologically proven sinus histiocytosis with massive lymphadenopathy (SHML) is reported. Computerized tomography confirmed the presence of bilateral retrobulbar masses, involvement of both lateral recti, erosion of the bony orbital floor with encroachment of tumor into the right maxillary antrum, and retropharyngeal involvement.

Adult↗
PubMed1987

MRI in autonomic failure.

A significant rank correlation between rigidity and putaminal signal dropout on magnetic resonance imaging (MRI) in patients with multiple system atrophy suggests that putaminal degeneration may cause this clinical finding. Absence of putaminal abnormalities on MRI in patients with pure autonomic failure may prove useful in differentiating these two autonomic disorders.

Aged↗
PubMed1986

Multiple system atrophy (Shy-Drager syndrome): MR imaging.

The Shy-Drager syndrome (SDS) is a form of progressive autonomic nervous system failure (PAF) with orthostatic hypotension and associated extrapyramidal involvement that is often mistaken for Parkinson disease. SDS includes olivopontocerebellar atrophy and striatonigral degeneration which is attended by PAF. Eight patients with SDS were studied on a 0.5-T superconducting system utilizing T1-weighted inversion recovery (IR) and T2-weighted spin-echo pulse sequences and also on a 1.5-T system using spin-echo sequences. With IR sequences, atrophy of the putamina was demonstrated in patients with SDS that is consistent with findings of neuronal loss in these nuclei reported on postmortem examinations. An abnormal decrease in signal intensity of the putamina, particularly along their lateral and posterior portions, was also detected, predominantly on T2-weighted sequences, and in three cases on T1-weighted spin-echo sequences. Abnormalities were detected on both imagers but were shown with greater clarity on the 1.5-T device. SDS is the first disease in which convincing basal ganglia changes have been shown in vivo exclusively by MR imaging.

Adult↗
PubMed1979

Radionuclide scintiphotography in defining postoperative pulmonary vasoconstriction. Succesful results after tolazoline administration in a four-month-old infant with congenital heart disease.

99mTechnetium macroaggregated albumin has successfully been used to define severe postoperative pulmonary vasoconstriction in a four-month-old boy with D-transposition of the great vessels who had undergone a Blalock-Hanlon surgical atrial septectomy. Radionuclide imaging documented clinically suspected pulmonary vasoconstriction and led to the successful use of tolazoline (Priscoline) to reverse the vasoconstriction with improved pulmonary blood flow patterns.

Heart Septum↗
PubMed1979

99mTechnetium pyridoxylidene glutamate imaging in visceral heterotaxy (Ivemark's syndrome).

A two and one-half year old boy with complex congenital heart disease had Howell-Jolly bodies in his blood raising the possibility of the asplenia syndrome. A 99mTc-sulfur colloid liver-spleen scan was reported normal, but the right lobe of the abnormally reversed liver was mistaken for the spleen. The diagnosis of visceral heterotaxy (Ivemark's syndrome) was established after scanning the patient with a new radiopharmaceutical, 99mTechnetium pyridoxylidene glutamate. The agent clearly demonstrated a left-sided gall bladder, and on comparison with the sulfur colloid scan it was established that asplenia was present. Radionuclide imaging with the new generation of hepatobiliary agents is a reliable method to document asplenia and is useful in studying patients with visceral heterotaxy.

Child, Preschool↗