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Biomedical subjects

A Emami

Publications and source records attributed to A Emami.

At least 37 records · Page 2Linked to original sources

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↗

Attempts to define the minimal serum level of vitamin A required for normal visual function in a patient with severe fat malabsorption.

A case with severe malabsorption of fat soluble vitamins is described. The malabsorption developed after an intestinal bypass operation due to morbid obesity. Night blindness occurred as the first symptom of vitamin A deficiency. The cone visual sensory threshold was elevated about one log unit and the rod threshold abot two and a half log units. No changes of the a- and b-waves of the electroretinogram (ERG) was observed. However, during the initial phase of very low serum reninol level (0.21 mumol/l) the summed amplitudes of the oscillatory potentials (OPs) were lower. After parenteral therapy with vitamin A the night blindness disappeared and the dark-adapted rod and cone threshold sensitivity recovered to normal. However, the time-course of rod adaptation first reached normal levels after 5 months. The amplitudes of the OPs of the ERG response returned to normal when the serum retinol level had increased close to normal. Serum retinol levels of 0.7 mumol/l or higher were always associated with normal or close to normal dark-adapted rod sensitivity. However, a normal serum retinol level (> 0.95 mumol/l) and a normal dark-adapted rod threshold sensitivity were not always associated with a normal time-course of the rod adaptation. It is concluded, that the maintenance dosage of vitamin A must be individualized and that patients who have undergone jejuno-ilea bypass surgery must be carefully monitored for vitamin A deficiency by both serum levels and dark adaptation measurements.

Dark Adaptation↗

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↗

Incidence of cholelithiasis in sickle cell anemia using the ultrasonic gray-scale technique.

Gray-scale ultrasound examination of the gallbladder was successfully performed in 226 patients with sickle cell hemoglobinopathy from 2 to 18 years of age. Sixty-three (27%) demonstrated the presence of gallstones; 14 additional patients had "sludge." The incidence of gallstones increased significantly with age, from 12% in the 2- to 4-year age group to 42% in the 15- to 18-year-old group. The proportion of females with gallstones increased significantly in adolescence. Patients with gallstones had higher mean bilirubin levels, but their mean hemoglobin values and reticulocyte counts were not significantly different from those in patients without gallstones. Ultrasound examination of the gallbladder is a simple, noninvasive technique for evaluating the biliary system. The incidence of gallstones in patients below the age of 10 is higher than expected and raises management questions that must be resolved.

Adolescent↗

[Contamination of cotton seeds and of cotton seed cake by aflatoxin].

Following the discovery of aflatoxin M in cow's milk in the previous study, the contamination with aflatoxin of cotton-seed and cotton-seed cake which constitute the principal feed of local animals was measured. The samples were taken from 2 factories. The grain from the 1st factory was grown in the centre of the country in dry climate, and those from the 2nd source originated from the humid region of the north of Iran. The grain and cotton-seed cake from the 1st factory did not contain aflatoxin before storage. During storage the sample contamination amount and percent increased with time in both regions, with the dry less than the humid. In conclusion, Iranian cotton-seed and cotton-seed cake appear to be contaminated with aflatoxin B in 2 processes. The 1st is aflatoxin contamination before harvest, in the humid region of Iran; the 2nd is general storage aflatoxin contamination in both regions in which humidity and length of storage appear to be the principal factors.

Aflatoxins↗

Goiter in Iran.

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Adolescent↗