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Biomedical subjects

H al-Mondhiry

Publications and source records attributed to H al-Mondhiry.

15 recordsLinked to original sources

Sweet's syndrome without granulocytosis.

BACKGROUND: Sweet's syndrome (SS), acute febrile neutrophilic dermatosis, has been linked to hematologic malignancies and presents with characteristic edematous dermal plaques. Peripheral blood neutrophilia is frequently seen in association with SS and is one of the diagnostic criteria. OBJECTIVE: To report the clinical, laboratory, and hematologic data of four patients with myeloid leukemia who developed SS after chemotherapy. Three of these patients were neutropenic. METHODS: A retrospective study of four patients with SS and hematologic malignancies was undertaken. Three patients had de novo acute myelogenous leukemia and one was in the acute blast crisis of chronic myelogenous leukemia. RESULTS: Sweet's syndrome was not originally suspected in these patients because of the low peripheral white blood cell counts caused by chemotherapy. All of the patients presented with fevers, arthralgias, and an eruption. They had been treated with antibiotics because of a presumed infection. Once the correct diagnosis was made and oral prednisolone was started, a rapid response followed. CONCLUSIONS: Sweet's syndrome should be considered in the differential diagnosis when acute myeloid leukemic patients develop skin lesions and unexplained fevers regardless of the peripheral blood counts.

Acute Disease↗

Congenital afibrinogenemia.

Congenital afibrinogenemia is a rare disorder with unusual clinical manifestations. The disease is inherited as an autosomal recessive trait and consanguinity is common among affected families. Clinical manifestations range from minimal bleeding to catastrophic hemorrhage. Congenitally afibrinogenemic patients seem to be peculiarly susceptible to spontaneous rupture of the spleen. Coagulation tests which depend on clot formation as an end point may be infinitely prolonged and abnormalities of platelet function are usually present. The diagnosis is established by demonstrating trace or no immunoreactive fibrinogen. The disease is caused by markedly reduced or absent synthesis of fibrinogen by liver cells, but the genetic defect remains unknown. Bleeding episodes can be effectively treated with cryoprecipitate. Purified virally inactivated fibrinogen concentrates have been used in Europe and may soon be widely available.

Afibrinogenemia↗

Platelet and fibrinogen survival in calves implanted with artificial heart and ventricular assist device--correlation with autopsy findings.

The two main ingredients of blood clots formed on artificial surface are platelets and fibrinogen. In this study, we measured platelet and fibrinogen survival in calves implanted with total artificial heart (TAH) and left ventricular assist device (LVAD), and correlate these data with autopsy findings. Platelet survival with autologous 111In-labeled platelets was performed on nine calves implanted with TAH and five with LVAD. Fibrinogen survival with 131I-labeled homologous fibrinogen was performed on six calves with TAH and three with LVAD. Platelet survival was significantly shortened in both groups of animals: 5.89 +/- 0.52 days, control 6.46 +/- 0.31 days, p = 0.0013; fibrinogen survival was normal: 8.79 +/- 1.20 days, control 8.64 +/- 1.16 days. At autopsy two calves with TAH had multiorgan thromboembolism. Two other animals with TAH and four with LVAD had focal renal infarcts. Most animals had minor clot formation within the prosthetic device. Major septic complications occurred in four calves with TAH and one with LVAD. Continuous platelet activation by artificial surface probably explains the shortened platelet survival and thromboembolic complications.

Animals↗

Hematologic and hemostatic complications associated with long-term use of total artificial heart: clinical and experimental observations.

The two most serious complications associated with long-term use of a total artificial heart (TAH) are thromboembolism and infection. In this article, we review our experience in one patient implanted with a pneumatic TAH for 396 days, and in 24 calves implanted with the same type of device for 70 to 353 (median 150) days. During his survival with the TAH, our patient suffered several thromboembolic episodes despite adequate anti-coagulation with warfarin. Autopsy showed widespread thrombotic lesions, mostly in the brain. Throughout his survival, the patient had markedly elevated plasma beta-thromboglobulin and fibrinopeptide A levels, indicating sustained activation of platelets and the coagulation system secondary to blood contact with the artificial surface. Long-term use of TAH in calves causes significant mechanical hemolytic anemia and a small reduction in the total leukocyte and neutrophil counts. The platelet count normalized to preimplantation levels by 25 to 35 weeks. At autopsy, thrombotic lesions and organ infarction were noted in 13 calves, and major septic complications were documented in 10 animals. Although impressive gains in the clinical and experimental use of TAH were achieved during the last 15 years, thromboembolism and infection remain challenging problems.

Anemia, Hemolytic↗

Synthesis of vitamin K-dependent proteins by cultured human tumor cells.

The observation that warfarin inhibits the growth and metastasis of certain types of clinical and experimental tumors suggests a role for vitamin K in tumor biology. We have investigated synthesis of vitamin K-dependent proteins in four malignant (lung epidermoid carcinoma, melanoma, colon adenocarcinoma, and breast adenocarcinoma) and three normal (colon epithelium, breast epithelium, and fibroblasts) cell lines of human origin grown in tissue cultures. Our results show the following: 1) Vitamin K-dependent carboxylase activity is present in all of the malignant and normal cell lines studied. 2) The malignant as well as normal cell lines synthesize a family of vitamin K-dependent proteins. Microsomal precursors of these proteins with apparent molecular mass of 74, 62, and 34 kDa are common to all malignant and normal cell lines whereas precursors of higher and lower molecular mass seem to be synthesized by some but not all tumor cell lines. 3) The 74 kDa precursor synthesized by colon carcinoma and breast carcinoma was positively identified as a precursor of protein S.

Carbon-Carbon Ligases↗

Hemostatic abnormalities in two patients implanted with total artificial hearts.

Patients implanted with total artificial hearts (TAH) suffer frequent thromboembolic complications. The authors report results of continuous monitoring of multiple hemostatic parameters and autopsy findings in two patients implanted with TAH. Case 1 who used a TAH for 10 days and then underwent orthotopic transplantation died of fungal infection 7 days later. At autopsy, mycotic and nonmycotic thrombi were found in the brain, kidneys, spleen, and large bowel. Case 2, who survived 396 days with a TAH, suffered multiple thrombotic episodes despite adequate anticoagulation with warfarin. Autopsy revealed widespread thrombotic lesions mostly in the brain and lungs. Both patients had markedly elevated plasma beta-thromboglobulin and fibrinopeptide A levels throughout their survival, indicating sustained activation of platelets and coagulation secondary to blood contact with artificial surface. Other factors such as infection, liver damage, and acute pancreatitis might have also contributed to activation of hemostasis. As used in the authors two patients, antiplatelet drugs and warfarin seem to be ineffective in preventing artificial heart-related thromboembolic complications.

Adult↗

Hematologic abnormalities and thromboembolic complications in calves implanted with pneumatic artificial hearts. Long-term studies and autopsy findings.

The purpose of this study was to assess hematologic abnormalities and thromboembolic complications related to long-term use of the pneumatic artificial heart (PAH). Laboratory data and autopsy findings were analyzed from 24 calves that survived 70-353 (150 +/- 82) days after implantation of a PAH. Compared with baseline levels, the hemoglobin was significantly lower, and the plasma hemoglobin and serum LDH significantly higher, throughout the follow-up period. The platelet count decreased during the first 10-30 weeks, but returned to preoperative levels by week 35. Platelet survival in 4 calves in stable condition was normal, and the leukocyte count and absolute neutrophil count were within normal limits. At autopsy, thrombotic complications and organ infarction were noted in 13 calves, and major septic complications were documented in 10 animals. The authors' observations indicate that thromboembolism and infection are major complications of long-term use of the PAH.

Animals↗

Evaluation of the fibrinolytic system in calves implanted with an artificial heart and ventricular assist device.

Thromboembolism is a serious complication of prolonged use of intracirculatory devices such as total artificial hearts (TAH) and ventricular assist devices. The authors have evaluated alteration in the hemostatic system associated with long-term use of TAH and ventricular assist devices. This article reports results of a prospective evaluation of the fibrinolytic system in four calves implanted with TAHs and four with ventricular assist devices. Blood fibrinolytic activity measured with a solid phase radiometric assay was elevated in two of four TAH calves; plasma plasminogen activity was increased in three. Plasma plasminogen activator activity was undetectable (normal) in all animals. Slight to moderate hypofibrinogenemia was noted in all TAH calves. Calves implanted with a left ventricular assist device had mostly normal blood fibrinolytic activity, fibrinogen, and plasminogen levels. Fibrinogen survival was measured in two calves with ventricular assist devices and three with TAH and was in the normal range in all of these animals. No significant thrombotic lesions were noted at autopsy in five calves that died or were electively killed. These observations suggest enhanced activation of the fibrinolytic system in some calves implanted with a TAH. This may offer a measure of protection against thrombosis in some animals.

Animals↗