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Biomedical subjects

S B Turkel

Publications and source records attributed to S B Turkel.

34 records · Page 2Linked to original sources

Ultrastructure of the dermal microvasculature in leprosy.

Infection with M. leprae may lead to the presence of the organism within the dermal vascular endothelium, a phenomenon most pronounced in lepromatous leprosy. In order to study the ultrastructural features of the dermal microvasculature in leprosy, biopsies from 18 patients with lepromatous (14), borderline lepromatous (1) and borderline tuberculoid (3) leprosy were examined. Four patients with Lucio's phenomenon and four with erythema nodosum leprosum were included. The ultrastructural changes in the dermal microvasculature included endothelial swelling and hypertrophy, increased endothelial and pericytic cytoplasmic processes, and pronounced basal lamina reduplication. Occasional large, pale, endothelial cells with widely dispersed organelles were encountered. Phagocytized, membrane-bound intraendothelial organisms were found, similar in appearance to those within dermal macrophages. The predominantly perivascular dermal inflammatory infiltrate consisted of lymphocytes, macrophages and mast cells. The observed ultrastructural changes in the dermal microvasculature are similar to those previously described in the endoneurial vessels. While reflecting nonspecific responses of the dermal microvasculature in chronic inflammation, the findings support a possible role of the small dermal vessels in the chronic nature of the host's response to infection with M. leprae.

Adolescent↗

A clinical pathologic reappraisal of kernicterus.

Kernicterus at autopsy, traditionally associated with hyperbilirubinemia, is now often observed in the absence of markedly elevated levels of serum bilirubin. Attempts to document clinically predictive risk factors for kernicterus have been largely unsuccessful in the current population of sick neonates. In a study of 32 pairs of newborns with and without kernicterus at autopsy and matched for gestational age, weight, length of survival, and year of birth, no differences were found when multiple clinical factors thought to potentiate risk for kernicterus were compared. Microscopic sections of brains from these matched pairs were then evaluated without knowledge of the clinical or gross findings according to prearranged criteria. Statistic analysis of the microscopic findings was done, comparing each observed change in each anatomic site, all changes in each site, and each change in all sites. Although the gross pattern of staining followed that of classic kernicterus, the expected, specific histologic changes characteristic of kernicterus were found in only three patients. Spongy change, or edema, was significantly more common in the grossly stained brains (P less than .0005), but other findings were similar in both groups. Gross bilirubin staining of the neonatal brain with neither hyperbilirubinemia nor specific microscopic changes is probably not the same clinicopathologic entity as classic kernicterus.

Birth Weight↗

Ultrastructure of muscle in fetal Duchenne's dystrophy.

Skeletal muscle was studied from two male fetuses with Duchenne's muscular dystrophy (DMD) that were at 17 and 23 weeks' gestational age, respectively. In comparison with the ultrastructure of normal fetal muscle, a spectrum of changes could be seen, from normal-appearing to very degenerated fibers. The changes followed an apparent progression from early actin filament disruption and fraying of the Z bands to progressive disorganization of myofibrillar alignment, increasing vacuolization, and condensation of fibers. Significant plasma membrane defects were not found. These findings are similar to those described in older persons with DMD and confirm that DMD is a congenital disorder associated with consistent skeletal muscle changes in utero.

Female↗

Malformation complex. Spondylohypoplasia, arthrogryposis, and popliteal pterygium.

Two unrelated children were born with severe congenital malformations: a girl now seven years old and a boy who died in the newborn period. Both had severe cervical hyperextension, severe flexion deformities of the limbs, and bilateral popliteal pterygia. Postmortem examination of the second case showed small, well-formed thoracic vertebrae with mild epidural fibrosis and hemorrhage. These births defects are rare and usually occur separately. We postulate that they are related in these two cases; that the arthrogryposis and popliteal pterygia were probably the result of the spondylohypoplasia and associated changes; and that together, these related defects may constitute a new malformation complex.

Abnormalities, Multiple↗

Lack of identifiable risk factors for kernicterus.

In recent years kernicterus at autopsy has been observed in sick premature infants in the absence of markedly elevated levels of serum bilirubin. Potentiating factors have been suggested to explain kernicterus in such a setting. In order to establish which factors are associated with increased risk for kernicterus in these small babies, this retrospective matched control study was undertaken. Thirty-two infants with kernicterus at autopsy were matched for gestational age, birth weight, length of survival, and year of birth to 32 control infants without kernicterus. Multiple historical, clinical, and laboratory factors were compared, including therapy, sepsis, hypothermia, asphyxia as reflected by Apgar score, hematocrit, acidosis, hypercarbia, hypoxia, hypoglycemia, and hyperbilirubinemia. No statistically significant differences between the kernicteric and nonkernicteric infants were demonstrated for any of these factors, including peak total serum bilirubin levels. Multivariant analysis also failed to determine a group of factors associated with increased risk for kernicterus. It was not possible to separate those infants with and without kernicterus at autopsy on the basis of the clinical factors evaluated.

Birth Weight↗

Antenatal detection of fetal A-V dissociation utilizing real-time B-mode ultrasound.

Congenital heart block is an unusual cause of fetal bradycardia. Until recently, the diagnosis could be confirmed only by evaluation of the fetal cardiac electrical activity. Two cases are reported in which the diagnosis of atrio-ventricular (A-V) dissociation was confirmed in the antepartum period by real-time ultrasound evaluation of cardiac mechanical activity.

Adult↗

Neuroblastoma and adrenal morphologic features in anencephalic infants.

At autopsy, an incidental congenital neuroblastoma was found in the adrenal gland of an 8-day-old full-term anencephalic baby. This previously unreported coincidence of neuroblastoma and anencephaly prompted a retrospective study of adrenal morphologic features in 20 anencephalic patients and 20 matched controls. Anencephalic adrenal glands were smaller and the fasciculoreticular zone was thinner (average 32%) than in the normal controls (average 76%). The neural crest-derived cells represented an average of 2% of the total gland area in anencephalic patients and 0.5% in the controls. These findings, statistically significant and compatible with previous studies, verify the deficient development of the fetal cortex in anencephalic patients. Furthermore, the neural component in anencephalic patients may be delayed in its maturation by the abnormal cortex, and thus may be prone to undergo malignant transformation and become a neuroblastoma.

Adrenal Cortex↗

A fetus with recombinant of chromosome 8 inherited from her carrier father.

A pericentric inversion of chromosome 8, inv(8)(p23q22), in a male carrier resulted in an unbalanced recombinant, rec(8)dup q, inv(8)(p23q22), which was diagnosed prenatally. The features seen in the aborted fetus resembled the features seen in a previously affected child who received the identical recombinant from her carrier mother. In this particular inversion involving chromosome 8, both male and female carriers risk producing an unbalanced progeny. Different familial pericentric inversions are reviewed for the presence or absence of unbalanced recombinants.

Abortion, Spontaneous↗

The cerebral malformation of the amnionic band syndrome.

The Amnionic Band Syndrome is a not uncommon disorder of the fetus which may result in cerebral malformations that mimic anencephaly. It is important to distinguish these two entities because of differences in pathogenesis and risk of recurrence. A preliminary stillborn female of 29 weeks gestation with multiple anomalies, including cerebral-placental fusion, cranial agenesis, cleft lip, syndactyly and ectopic liver is presented. These malformations are typical of the Amnionic Band Syndrome, an entity which has not been described in the neurologic literature.

Abnormalities, Multiple↗

Vaccinia necrosum complicating immunoblastic sarcoma.

A 59-year-old man is presented who had immunoblastic lymphadenopathy which evolved over a three-year period into immunoblastic sarcoma. His course was complicated by vaccinia necrosum, which necessitated prolonged therapy with Marboran and vaccinia-immune globulin. The persistence of virus was documented at autopsy by positive viral culture and ultra-structural examination. This case illustrates the potential hazards of administration of live viral vaccines to an immune compromised host presumed to be in remission and suggests that the continued activity of viral infection may signal the unsuspected persistence of underlying disease.

Adult↗

Hematogenous candida osteomyelitis. Report of three cases and review of the literature.

Candida osteomyelitis of the spine and intervertebral disc developed in three patients without evidence of back trauma of overlying cutaneous infection. Two patients were prone to the development of disseminated candidiasis by the use of multiple antibiotics and other predisposing modalities following abdominal surgery. One patient had no identifiable cause for development of the infection. The diagnosis was established in all three cases by x-ray evidence of osteomyelitis and culture from needle aspirate. Two patients had bone scans consistent with infection. Each patient received different therapy. One was treated with amphotericin B, one with spinal fusion and 5-fluorocytosine, and one with no antifungal therapy. All patients had complete healing of the involved vertebrae. Candida organisms have the potential to cause destructive bone infection following hematogenous dissemination. The presence of Candida osteomyelitis may be helpful in diagnosing disseminated candidiasis.

Aged↗

The natural history of neuroblastic cells in the fetal adrenal gland.

A study was made of neuroblastic nodules of the human adrenal gland in fetal life to determine their embryonic morphology and derivation. One hundred sixty-nine glands from 92 fetuses selected from 106 consecutively submitted fetuses formed the basis of this study. All adrenal glands examined contained neuroblastic nodules. They increased in number and maximum size with increasing age, peaked at 17 to 20 weeks gestational age, and regressed in the oldest fetuses. It is concluded that such neuroblastic nodules are an integral part of the normal morphogenesis of the adrenal gland. Their presence at birth and early infancy may represent a normal variation, and they do not necessarily constitute microscopic incipient malignancy.

Adrenal Glands↗

Possible serotonin syndrome in association with 5-HT(3) antagonist agents.

The serotonin syndrome results when serotonergic activity increases to abnormally high levels. It occurs with selective serotonin reuptake inhibitors (SSRIs), opioids, and other serotonergic agents when the serotonin system has been modulated by another serotonergic agent or compromised by illness. Although the symptoms are quite variable, the syndrome is characterized by a triad of altered mental status, neuromuscular abnormalities, and autonomic dysfunction. The authors report the probable occurrence of the serotonin syndrome with serotonin receptor subtype 3 (5-HT(3)) antagonist therapy when used to control nausea associated with chemotherapy in two seriously ill children. The first case involves combined use with mirtazapine and the second with fentanyl. These agents may pose a potential risk when used in such combination in seriously ill patients.

5-Hydroxytryptophan↗