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Biomedical subjects

A Emami

Publications and source records attributed to A Emami.

At least 19 recordsLinked to original sources

Treatment of closed tibial shaft fractures with unilateral external fixation.

Sixty-eight closed tibial shaft fractures were treated with an anterior unilateral external fixator over a 5 year period (1986-1991). Pin tract drainage and/or infection was seen in 71/380 pins. The total number of secondary operations, excluding planned pin extraction, during fracture healing was 61 (including 22 due to pin tract problems and 25 secondary corrections of alignment). Delayed union was seen in 14 fractures and non-union in three. Healing disturbances were more frequent following high-energy trauma. Bone grafting was done in 11 fractures. Eventually, all fractures healed within an average of 22 weeks. There were three refractures. At follow-up, on average 3 years after injury, functional results were excellent in 41 per cent, good in 46 and acceptable in 13 per cent. Due to the high number of unplanned secondary operations and prolonged healing times we do not consider the use of unilateral external fixation to be an adequate method for the treatment of closed tibial shaft fractures. The poor results are probably due to weight-bearing being too high in these patients relative to the mechanical stability provided by the external fixator system.

Adolescent

Infected tibial nonunion. Good results after open cancellous bone grafting in 37 cases.

We treated 37 infected tibial shaft nonunions by debridement followed by open autogenous cancellous bone grafting in a 2-stage procedure. Additional surgery was done in 21 fractures including second debridement before bone grafting and/or a second limited bone grafting and/or a split-thickness skin grafting. All fractures healed after an average of 11 (8-16) months. During 2 years follow-up there were no recurrences of the infection. Two cases of early refracture occurred, both healed following new bone grafting.

Adult

Heat stress protein-associated cytoprotection of inner medullary collecting duct cells from rat kidney.

Although heat stress proteins (HSPs) mediate thermotolerance, the cellular targets of thermal injury and mechanisms of acquired cytoprotection are unknown. To describe the metabolic effects of hyperthermia and the potential mechanisms of thermotolerance, the following were measured in inner medullary collecting duct cells after a 43 degrees C and/or a 50 degrees C thermal insult: 1) state III mitochondrial respiration (SIII MR), 2) glycolytic rate, 3) lactate dehydrogenase activity, 4) membrane permeability, and 5) HSP 72 content. Compared with controls incubated at 37 degrees C, cells heated to 50 degrees C showed a 30 and 50% reduction in glycolysis and SIII MR, respectively. After heating to 50 degrees C, the cell membrane remained intact and immunoreactive HSP 72 was not detected. In contrast, heating to 43 degrees C induced accumulation of HSP 72 and transiently increased both SIII MR and glycolysis. In addition, prior exposure to 43 degrees C completely prevented the fall in SIII MR and glycolysis anticipated with a subsequent 50 degrees C insult. Cytoprotection gradually diminished over several days and correlated with the disappearance of HSP 72. Preservation of oxidative and anaerobic metabolism associated with HSPs may be important in developing resistance to thermal injury.

Animals

Acute megakaryoblastic leukemia in infants with t(1;22)(p13;q13) abnormality.

Six infants with acute megakaryoblastic leukemia and a translocation (1;22)(p13;q13) were studied. There were five female infants and one male infant, and the age at initial examination varied from 0.8 to 6.5 months (median, 2.3 months). All the patients had hepatosplenomegaly and anemia (6 to 8.3 g/dL), and four patients had thrombocytopenia (9,000 to 63,000/mm3). The bone marrow showed prominent fibrosis in five cases and reticulin fibrosis in one patient at presentation. Crush artifact often made the histologic sections difficult to interpret, but typical megakaryoblasts could be identified in the smears. Biopsy specimens of the liver and lymph node were suggestive of a nonhematopoietic malignant condition because of the cohesiveness of the tumor cells, stromal fibrosis, and the prominent sinusoidal and vascular pattern of infiltration. Immunophenotyping of peripheral blood mononuclear cells was helpful in identifying the blasts as belonging to the megakaryoblastic lineage. Using a panel of mononclonal antibodies, it was also possible to confirm the nature of the infiltration in paraffin sections and to differentiate it from other childhood small round cell tumors, especially neuroblastoma in paraffin sections (typical staining pattern: CD45-, CD43+, vW Factor, Ulex europeus I+, CD20-, CD45RO-, synaptophysin-, chromogranin-, cytokeratin-, desmin-). This special type of infantile acute leukemia can be recognized with confidence if one is aware of its clinical features, peculiar pathologic characteristics, the morphologic features and immunophenotype of the megakaryoblasts, and the unique cytogenetic abnormality.

Biopsy

The t(1;22) (p13;q13) is nonrandom and restricted to infants with acute megakaryoblastic leukemia: a Pediatric Oncology Group Study.

We report the nonrandom occurrence and frequency of the t(1;22)(p13;q13) in acute myeloid leukemia (AML) and its close association with the French-American-British M7 subtype of AML in infants (less than 1 year). This chromosomal abnormality occurred in 6 of 252 (2.4%) children and adolescents with AML (6 of 28 infants, 22%; 6 of 18 M7 AML cases overall, 33%; and 6 of 6 M7 cases in infants). Infants with AML of M7 subtype and the t(1;22) often presented with prominent abdominal masses. Two of these infants were not treated and died early. Three of four treated infants entered complete remission with therapy for AML; the remaining infant died of hemorrhage on day 8. Of the three infants who entered remission, only one remains alive and disease free at 5+ months. The other two infants relapsed in the bone marrow at 5 and 2 months from the start of therapy, respectively. We conclude that M7 AML with the t(1;22) usually presents in infants with extensive infiltration of abdominal organs by leukemic cells and may confer a poor prognosis despite intensive AML-directed treatment. Identification of this nonrandom translocation exclusively in infants with acute megakaryoblastic leukemia (AMkL) implies that it may serve as an additional diagnostic marker for this disease and links it to the pathogenesis of AMkL in infants.

Antigens, CD

Transient ischemia or heat stress induces a cytoprotectant protein in rat kidney.

Sublethal heat exposure induces the production of heat stress protein (HSP) 72 kDa, a reported cytoprotectant, in several tissues including the rat kidney. However, the localization and time course of HSP 72 accumulation in the kidney in response to heat or other cell stresses such as transient ischemia have not been described. In anesthetized rats exposed to either 42 +/- 0.5 degrees C (heat stress) or 37 degrees C (sham) for 15 min, accumulation of HSP 72 in kidney homogenates, detected by immunoblot analysis using a specific monoclonal anti-HSP 72 antibody, peaked 4-6 h after heat stress and persisted for 10 days. HSP 72 appeared rapidly in renal papilla within 1 h and in medulla and cortex within 4 h after heat stress. No HSP 72 was detected in tissues from sham heat stress. HSP 72 was also detected within 3 h of at least 15 min of renal ischemia in situ. Accumulation was maximal after 60 min of ischemia and persisted for 5 days, whereas no HSP 72 was detected after 90 min of ischemia or in the contralateral nonischemic kidney at any time point. This study demonstrates that transient ischemia, like heat stress, results in the rapid cytosolic accumulation of HSP 72, a known cytoprotectant that may be important in mediating cell repair or increasing resistance to subsequent injury.

Animals

Secondary biliary cirrhosis as a consequence of graft-versus-host disease.

A 9-yr-old white girl with acute monoblastic leukemia received an HLA-identical, mixed lymphocyte culture-nonreactive bone marrow transplant from her sister. Twelve days after the transplant, a diffuse, pruritic, maculopapular rash involving the entire body surface (including the palms and soles) developed. Subsequent skin biopsy was consistent with cutaneous graft-versus-host disease, and biopsy-proven hepatic involvement manifested by severe, unremitting cholestatic jaundice soon followed. The patient's biliary status as monitored by serial liver biopsies demonstrated progression from chronic graft-versus-host disease to cirrhosis, culminating in death secondary to liver failure 25 mo after transplant.

Bone Marrow Transplantation

Lower limb reconstruction in children using expanded free flaps.

Controlled expansion is a technique that increases the area of local tissue available for reconstruction. An extension of this is to expand free flaps prior to elevation, thereby increasing their area. This has been particularly useful in children where there may be insufficient tissue available at free flap donor sites. Four children have had extensive cutaneous defects of the lower limb reconstructed with expanded parascapular free flaps. Measurements indicate an approximate doubling in skin area. There has been normal growth of the affected limbs and there has been no donor site morbidity. Apart from small areas of narrow marginal necrosis at the tip of the flaps in the first three cases, which were of no consequence, healing at the recipient site was complete.

Child

Failure of systemic thrombolytic and heparin therapy in the treatment of neonatal aortic thrombosis.

An unsuccessful attempt was made to lyse a large aortic thrombus in a newborn using systemic high-dose streptokinase and urokinase therapy and subsequently the use of heparin failed to prevent the propagation of thrombus. The patient was a seven-day old premature, sick neonate in whom an aortic thrombosis developed following umbilical artery catheterization. Surgical thrombectomy could not be performed in this patient, and local thrombolytic therapy was not technically feasible. Systemic thrombolytic therapy failed to induce any noteable clinical or laboratory response, and the use of heparin failed to prevent thrombus extension. Experience with the use of fibrinolytic agents in neonates is limited. Local therapy has been variably effective, and systemic therapy has not been adequately investigated. The thrombotic phenomenon in neonates and the role of umbilical vessel catheterization as a cause are discussed in reference to this patient and suggestions are made regarding the management of similar cases.

Aortic Diseases

Juvenile onset pernicious anemia, partial intestinal villous atrophy, ulcerative colitis, and squamous metaplasia of the stomach.

We report a case of a 13-yr-old white boy with juvenile onset pernicious anemia in association with IgG deficiency. He had marked gastric atrophy, intestinal metaplasia of the stomach, and an intractable antral ulcer that required surgery. In addition, his gastric mucosa showed evidence of a progressive squamous metaplasia. Diffuse squamous metaplasia of the stomach, a very rare gastric lesion, has not previously been described either in association with pernicious anemia, atrophic gastritis, or hypogammaglobulinemia. This patient also has ulcerative colitis involving the entire colon and partial villous atrophy noted on small intestinal biopsy.

Anemia, Pernicious

Idiopathic thrombocytopenic purpura in children. The case for management without corticosteroids.

Acute ITP in children under 13 years of age is generally a benign, self-limited condition with spontaneous recovery occurring within a matter of days or weeks. Our analysis of platelet data indicate no advantage in terms of rate of recovery when steroids are used. In fact, the median of 3 weeks and mean of 3 1/2 weeks from onset to recovery in the nonsteroid-treated children were significantly better than the corresponding figures in the steroid-treated group. In addition, while reducing the risk of intracranial hemorrhage is generally given as the chief therapeutic rationale for using steroids, we have not seen a single case of ICH among 465 consecutive cases of acute ITP in children, the majority (93%) of whom did not receive steroids. On the other hand, adolescents, as adults, with ITP often have the autoimmune (chronic) form of the disease. In this group, corticosteroids may be of at least transient benefit and should be used.

Acute Disease

Vincristine neurotoxicity with residual equinocavus deformity in children with acute leukemia.

Vincristine has been demonstrated to be a neurotoxic agent with distal axonal degeneration progressing proximally. Five children with acute lymphoblastic leukemia developed bilateral peroneal nerve palsies with equinocavus deformities. Three developed fixed contractures requiring surgical correction. One patient was braced prior to development of fixed deformity and the other had physical therapy preventing fixed deformities and did not require surgery. All of the children obtained complete return of peroneal nerve function. Proper bracing and/or physical therapy at the time of diagnosis of neurologic deficit will prevent fixed contractures and the necessity for surgery.

Braces

Phenotypic change of acute monocytic leukemia to acute lymphoblastic leukemia on therapy.

Acute nonlymphocytic leukemias comprise most of the therapy-linked leukemias in cancer patients. We report here the unusual occurrence of acute lymphoblastic leukemia in a patient while on therapy for acute monocytic leukemia. The morphologic and histochemical studies were distinctly different between the initial presentation and the subsequent "relapse". At "relapse," the Philadelphia chromosome was not present, ruling out chronic myelogenous leukemia presenting in two morphologically different blastic phases. Cytogenetic and histochemical studies both at original presentation and at the time of relapse would be helpful in establishing the occurrence of a new leukemia.

Child