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C S Kitchens

Publications and source records attributed to C S Kitchens.

At least 37 records · Page 2Linked to original sources

Approach to the bleeding patient.

A broad, open, inquisitive, and semiskeptical mind must be used when approaching the bleeding patient. As in most endeavors in medicine, the history and physical examination provide an important baseline. Key laboratory tests must be quickly ordered and interpreted. Using this data base, one can quickly determine whether the hemorrhagic disorder is congenital or acquired, severe or mild, and progressive or stable. Hemostasis may fail owing to deficiencies of platelets, the plasma coagulation protein system, or endothelial disturbances. A precise diagnosis and appreciation of the tempo of the disorder will guide specific therapy.

Blood Coagulation Factors↗

Small bowel hematoma in a hemophiliac as a cause of pseudoappendicitis: diagnosis by CT imaging.

Acute abdominal pain in a hemophiliac should be approached as hemorrhage until proven otherwise. With factor replacement available, a conservative approach can be taken as opposed to immediate exploratory surgery. Diagnostic imaging can define areas of hemorrhage. We report a case of "pseudoappendicitis" in a hemophiliac with terminal ileum circumferential bowel wall hematoma diagnosed by CT with the initial diagnostic scan. Factor replacement resulted in resolution of the signs and symptoms of the pseudoappendicitis and follow-up CT scan revealed resolution of the hematoma. A review of the literature reveals few CT documented circumferential bowel wall hematomas with limited clinical histories and no cases with follow-up scans after conservative management.

Abdominal Pain↗

Factor XI: a review of its biochemistry and deficiency.

The biochemistry and physiology of Factor XI have been reviewed. The clinical history of our 25 patients was reviewed and compared with others' experiences. Factor XI deficiency remains enigmatic in that there is no correlation between Factor XI levels and clinical manifestations. Approximately 40 to 50% of all persons lacking Factor XI are of Ashkenazi Jewish extraction, and the remainder are represented by nearly all populations. Persons may be found to lack Factor XI because of evaluation for hemorrhage, evaluation of a prolonged PTT, or through family or other genetic studies. Hemorrhage is not as severe as in patients with hemophilia A or B. Data are presented suggesting that hemorrhage may, in part, be associated with aspirin use.

Adolescent↗

Prolonged activated partial thromboplastin time of unknown etiology: a prospective study of 100 consecutive cases referred for consultation.

The activated partial thromboplastin time (aPTT) is frequently used to assess overall competency of the intrinsic pathway of coagulation. An abnormal value may be caused by any of several abnormalities along this pathway or by many other variables including the presence of inhibitors, poor collection of the sample, or variables in the laboratory. When the cause for the prolongation is unknown to the requesting physician, the hematologist may be consulted. In this prospective study, the cause and perceived hemostatic risk to 100 consecutive patients referred to use for consultation regarding a prolonged aPTT of previous unknown cause were evaluated. We found that these abnormal aPTTs may be either indicative of a hemostatic defect, in 50% of the cases, or of no particular risk, in 36% of the cases. In 14%, the aPTT was artifactually prolonged. Most (81%) patients with a prolonged aPTT due to a hemostatic defect had an abnormal hemostatic history but some (19%) did not. Even among true abnormal tests, the degree of abnormality indicated little or nothing about hemostatic competency. We conclude that the cause of an abnormal aPTT is more important than the result itself. These data may be of use to those who consult on such matters.

Adult↗

Envenomation by the Eastern coral snake (Micrurus fulvius fulvius). A study of 39 victims.

We gathered data on 39 victims of Eastern coral snake bite over a 12-year period. The most common situation resulting in snakebite was erroneous identification of the snake as the nonpoisonous scarlet king snake. While no patient died, several experienced severe envenomation, including bulbospinal respiratory paralysis. We found that neurologic symptoms may be delayed for 12 hours, and then may be precipitous. Envenomation occurs in 75% of the persons bitten by a coral snake. Antivenin is effective and should be intravenously administered early to patients who have been bitten by a positively identified coral snake, depending on the clinical presentation.

Adolescent↗

Acquired hemophilia. A natural history study of 16 patients with factor VIII inhibitors receiving little or no therapy.

Rarely, a patient develops an antibody against factor VIII coagulant activity. The resultant hemorrhagic diathesis is clinically distinct from inherited hemophilia, being characterized by few hemarthroses but frequent skin and other soft-tissue hematomas. Hematuria may be troublesome. These patients represent therapeutic challenges. This study is one institution's results with 16 such patients followed up over an average of 31 months (range, four to 120 months; median, 19 months). It describes the largest group from a single institution receiving essentially no immunosuppressive agents, yet has one of the better overall results. Two patients experienced fatal hemorrhage and five patients underwent spontaneous remission. Long-term survival is not incompatible with persistence of the inhibitor. We conclude that this hemorrhagic diathesis is clinically distinct, less fatal than usually perceived, and may undergo spontaneous remission. Clearly, there is no mandate for any particular therapeutic regimen, such as immunosuppression, in the attempt to rid the patient of the antibody.

Adult↗

Clinical efficacy of desmopressin acetate for hemostatic control in patients with primary platelet disorders undergoing surgery.

Desmopressin acetate (DDAVP) is efficacious in patients with von Willebrand's disease. It additionally appears to have value in patients with uremic or aspirin-induced platelet dysfunction. We report here three patients with primary platelet defects who had previously experienced grossly inadequate hemostasis to whom we administered DDAVP. Each successfully underwent surgical procedures with DDAVP as the only hemostatic agent. Although the mechanism of these salutary effects is unclear, DDAVP may exert an influence directly on the endothelium independent of correcting abnormalities of the factor VIII:von Willebrand complex associated with von Willebrand's disease.

Adolescent↗

Discordance in a pair of identical twin carriers of factor IX deficiency.

Female twin daughters of a man with hemophilia B were determined to be identical but widely discordant with respect to their degree of factor IX deficiency. The proposita had a marked deficiency (3%) of factor IX with clinical bleeding. She became pregnant. Delivery was managed with infusion of factor IX concentrates. These twins represent the second reported case of identical twins with discordant factor IX levels.

Adult↗

Physiology of hemostasis and its relationship to cardiovascular disease.

Our original understanding of coagulation was too simple. The waterfall or cascade system made sense but never gave clues either to points for control of the reaction or to areas to inhibit or reverse clotting. We now know critical points for control and reversal of hemostasis. By understanding more modern physiology, one is better able to design rational treatment programs to negate unwanted thrombosis. This chapter gives the reader a physiologic foundation to underscore the subsequent chapters.

Blood Coagulation↗

A unique 7p/12q chromosomal abnormality associated with recurrent abortion and hypofibrinogenemia.

Recurrent first trimester abortions led to evaluation of a 25-year-old woman. Studies revealed she had hypofibrinogenemia (68 mg/dL) without evidence of dysfibrinogenemia or increased fibrinogen turnover. She was also found to have a unique 46,XX, t(7;12) (p 15.2;q24.31) karyotype. Hypofibrinogenemia and identical chromosomal abnormalities were found in other members of her kindred. Southern blots of genomic DNA from the patient, her mother, and her daughter hybridized to human fibrinogen probes showed alpha, beta, and gamma fibrinogen genes to be present and without structural alterations when compared to normal controls. We conclude that the chromosomal abnormality and the hypofibrinogenemia are related but in an unclear manner. Because fibrinogen infusion in the proposita was associated with successful gestation, we also concluded that the chromosomal abnormality itself was not responsible for the repeated abortions but that fibrinogen concentration may be critical in securing implantation.

Abortion, Habitual↗

Surgery in hemophilia and related disorders. A prospective study of 100 consecutive procedures.

Surgery is safe in hemophiliacs. The current problem of blood product-transmitted AIDS has recently dampened an unqualified stance, but the dangers of not performing indicated nonelective surgery outweigh the possibility of inflicting AIDS. Successful surgery in hemophiliacs first requires determination of the type of hemophilia involved. If an inhibitor is present, the procedure needs careful re-evaluation, but in general, surgical indications should be those of the general population and routine procedures should be followed. We find it helpful to have nursing personnel aware from the onset that these patients and their medications require certain precautions, especially with respect to pain medication. Unfortunately, too few medical personnel realize how many pain compounds contain aspirin. In addition, we usually prescribe intramuscular injection of medication. Factor levels must be monitored. A less-than-expected yield of factor following infusion is an excellent screen for clinically significant inhibitors. Patients also vary with respect to factor replacement and the half-life of infused factors. Kasper et al found no difference in half-life of infused factor with respect to whether the patient was in a nonoperative or postoperative status; they did not find evidence that factor was consumed during the hemostatic stress of the surgery and that a given patient's response in a nonoperative setting was predictive of his response in the postoperative period. The internist should be available so that any hemostatic problems experienced by the surgeon can be promptly addressed.

Adolescent↗

Human thrombocytopenia is associated with structural abnormalities of the endothelium that are ameliorated by glucocorticosteroid administration.

Capillary fragility is characteristic of severe thrombocytopenia. This mechanical weakness may not be solely accounted for by decreased ability of platelets to repair endothelial breaks. Platelets may have a role in maintaining endothelial hemostasis. This laboratory has demonstrated thinning of capillary endothelium in experimental thrombocytopenia. We now report similar findings in human thrombocytopenia. Capillary endothelium supplying either skin or skeletal muscle was found to have a mean thickness only half that of normal as well as frequent very thinned areas, including some fenestrations. All findings reverted toward normal after four days of prednisone administration at a time the degree of thrombocytopenia was equally severe. These findings are consistent with the hypothesis that platelets are necessary for normal structure and function of endothelial cells and that glucocorticosteroid administration may ameliorate the pathophysiology of thrombocytopenia.

Adolescent↗

Anaplastic neoplasm in a patient with hairy cell leukemia.

A 63-year-old white man had a history of recurrent pneumonia, pancytopenia, and splenomegaly when the diagnosis of hairy cell leukemia was made on bone marrow biopsy examination. Splenectomy confirmed that diagnosis and his pancytopenia moderately improved. Three years following the diagnosis, the patient developed an upper abdominal mass involving the stomach wall that was found to be an anaplastic "large cell" neoplasm. Palliative radiotherapy was started, but the patient died 2 months later. Cytochemical studies of the anaplastic gastric neoplasm revealed cytoplasmic tartrate resistant acid phosphatase activity. Electron microscopy showed no epithelial differentiation. These observations suggest that the gastric neoplasm represented an evolution of hairy cell leukemia into a more aggressive tumor analogous to the transformation that occurs in other B-cell neoplasms.

Bone Marrow↗

The effects of low-dose oral contraceptives on coagulation and fibrinolysis in two high-risk populations: young female smokers and older premenopausal women.

A study was undertaken to determine the effect of a low-dose oral contraceptive on the coagulation and inhibitory system of coagulation in 22 young healthy women who smoke and in 15 nonsmoking healthy women between the ages of 34 and 41. Smokers showed statistically significant oral contraceptive-related procoagulant alterations in prothrombin time, thrombin time, and fibrinogen antigen. Antithrombin III antigen and activity were significantly reduced, whereas plasminogen antigen and activity were increased. Inhibitor and fibrinolytic activity was either unaffected or enhanced by oral contraceptives in women over the age of 34: antithrombin III activity was unchanged, plasminogen antigen and activity increased (p less than 0.0007), and alpha 2-antiplasmin was significantly reduced (p less than 0.07). Whereas usage of oral contraceptives in young smokers may initiate biochemical changes in favor of thrombogenesis, their usage in nonsmoking older women enhanced fibrinolysis and had a neutral effect on inhibition and a minimal procoagulant effect.

Adult↗