PubMed Health⌕ Search

Biomedical subjects

M W Hilgartner

Publications and source records attributed to M W Hilgartner.

At least 109 records · Page 6Linked to original sources
PubMed1980

Recognition and management of central nervous system hemorrhage in hemophilia.

18 central nervous system bleeding episodes in 8 patients with hemophilia A, B, or von Willebrand's disease are reviewed. Survival was seen in 17 of the 18 episodes. There was no mortality from uncontrolled bleeding and permanent neurological sequelae were seen in 8 instances. Management consisted of the use of neurological procedures to define the site of bleeding, therapeutic neurological or neurosurgical treatment where indicated, and vigorous factor replacement under laboratory control. The optimal duration of replacement therapy appears to be 14 days. Trauma as a precipitant was found in only 40% of the episodes with no other common etiologic factor.

Cerebral Hemorrhage↗
PubMed1979

Disseminated intravascular coagulation in the newborn.

We have reviewed 53 cases of disseminated intravascular coagulation (DIC) in the newborn, including 29 cases that were confirmed at autopsy. Factors predisposing to DIC included maternal complications (60%), low Apgar scores (30%), hyaline membrane disease (62%), and sepsis (26%). Diagnostic criteria common to autopsy-proved cases included presence of fibrin degradation products, low factor V activity, a prolonged prothrombin time, and a prolonged partial thromboplastin time and/or thrombocytopenia. There appeared to be no difference in coagulation response or in mortality among patients treated with different therapeutic regimens. Survivors were older gestationally, had higher birth weights, and higher Apgar scores.

Disseminated Intravascular Coagulation↗
PubMed1978

Prothrombin complex concentrates. Use in treatment of hemophiliacs with factor VIII inhibitors.

The development of factor VIII inhibitors has been a major problem in the management of hemophiliacs. The use of prothrombin complex concentrates (PCC) has been advocated as a method of overcoming this problem. We utilized PCC in the treatment of nine hemophiliac patients who had significant levels of inhibitor. Acute bleeding episodes were successfully treated in all nine patients. Single infusions of PCC were usually adequate for control of joint and soft tissue bleeding episodes, while multiple infusions were usually required for more serious hemorrhages. In addition, programs of repeated infusions were used to heal chronically inflamed joints in three patients. The only side effect of treatment was one episode of asymptomatic elevation of liver function values that resolved spontaneously. There were no thrombotic events associated with the PCC infusions. Therapy with PCC is recommended for the management of hemophiliacs with factor VIII inhibitors.

Adolescent↗
PubMed1977

Surgery in patients with congenital disorders of blood coagulation.

Surgical procedures on patients with congenital disorders of blood coagulation can be performed with a high degree of confidence and an acceptable incidence of complications. During the period 1960-1975, 42 patients with congenital disorders of blood coagulation underwent 94 operative procedures at the New York Hopital-Cornell Medical Center. The coagulation defect was diagnosed preoperatively, in nearly all patients. Careful hematologic management, including specific factor replacement, is essential. The importance of meticulous hemostasis at surgery and careful monitoring of blood coagulation in the postoperative period is strongly emphasized.

Adolescent↗
PubMed1977

Liver dysfunction in patients with hemophilia.

The incidence of post-transfusion hepatitis and liver dysfunction is presented in fifty-four patients with classical hemophilia who received episodic and/or prophylactic Factor VIII concentrate. 42.5% had persistent biochemical evidence of liver dysfunction with elevated SGOT and SGPT; 3.8% have persistent (HBs) antigenicity and 90% have (HBsAb) antibodies. The results are the same for those who were treated episodically and received an average of 753 units Factor VIII per week as those treated prophylactically who received an average of 686 Factor VIII units per week. The incidence of clinical and/or subclinical disease is unaffected by the transfusion regimen or the amount of concentrate used. The necessity for close follow is emphasized for determination of chronic liver disease and its further therapy.

Adolescent↗
PubMed1977

Home care for hemophilia: current state of the art.

While home care treatment for hemophilia is not a panacea and does not supplant good comprehensive care, it does seem to offer improved medical care for those hemophiliacs enrolled in such a program. The results have been very encouraging in the programs of this type throughout the United States. Since most programs have been in existence for 5 years or less, long-term data have not accumulated. However, the evidence seems to suggest that crippling is less in children and that orthopedic deformities do not progress at the same rate in adults. Children have improved school attendance; young men may attend college and graduate schools without difficulty. Adults may hold a job without fear of absenteeism. There are fewer dollar costs of the disease for the patient and the psychiatric cost of this chronic illness is markedly less. The quality of life is improved and the patient may develop a new self-confidence which allows him to function as a productive member of society.

Blood Transfusion↗
PubMed1977

Anticonvulsivant-induced depression of clotting factors in children.

A few neonates born to mothers receiving anticonvulsant drugs during pregnancy have shown defects in vitamin K dependent clotting factors with or without clinical bleeding. Experimentally, phenytoin (diphenyl hydantoin, DPH) has induced clotting defects in cats and inhibited production of clotting factors in rat liver slices. Phenobarbital has produced similar but milder defects. Anticonvulsants have been observed to produce clotting defects in 9 children, 2 weeks to 8 years in age. Elevated levels of phenytoin or other anticonvulsants, or a combination of anticonvulsants were measured in the children. Six patients were on drug combination including two or more of the following: phenytoin, phenobarbital, primidone, carbamazepine, diazepam, ethosuximide. Clotting defects included: elevated prothrombin time, elevated partial thromboplastin time, diminished factors V, VII or X. All children had neurologic symptoms of anticonvulsant toxicity, but the only hematologic problems were oozing from venipuncture sites and increased bruising in 3. All patients were on normal diets and had normal liver function tests. By lowering the level of anticonvulsants, clotting factors returned toward normal. Elevated levels of anticonvulsants can potentially produce clotting defects in neonates and young children.

Anticonvulsants↗
PubMed1976

Platelet dysfunction in vincristine treated patients.

Recent revival of interest in the use of vincristine (VCR) for the treatment of idiopathic thrombocytopenic purpura prompted us to evaluate the platelet function of our patients on VCR. Eighteen patients with acute lymphoblastic leukaemia (ALL) in remission, and nine children with solid tumours were studied on 80 occasions at different time intervals after their last VCR dose. A mildly elevated threshold for epinephrine-induced second phase aggregation and a delay in the onset of collagen-induced aggregation was found in patients with ALL not on VCR. Vincristine induced unobtainable second phase aggregation to epinephrine in 67%, 38%, 30% and to ADP in 53%, 13%, 33% of the patients 1 week, 2-3 weeks and 4 weeks respectively after administration. The thrombocytopathy was relative, not absolute, since collagen induced aggregation at all times. Platelet counts, uptake and release of serotonin, bleeding times, clot retractions and release of platelet factor 3 were normal. Platelet adhesion was abnormal in five of 12 patients tested. In vitro platelets are a hundred-fold less sensitive to VCR than in vivo. Cyclic adenosine monophosphate, cyclic guanosine monophosphate and dimethylsulfoxide do not protect platelets from VCR. The exact mechanism by which VCR abolishes second phase aggregation in patients is uncertain. Because of VCR's narrow therapeutic index between thrombocytopenia and thrombocythaemia, the use of VCR should be reserved for life-threatening haematologic disorders when treating non-malignant conditions.

Adenosine Diphosphate↗
PubMed1975

The effect of cyanate on the clotting proteins and platelet function.

Cyanate reacts with unchanged amino groups of various proteins in a specific irreversible carbamylation reaction. The effect of this molecule on the clotting process and the effects of carbamylation on the clotting proteins and platelet functions were investigated in vitro. An immediate effect on the clotting proteins, not related to pH, was seen in the screening tests prothrombin time, partial thromboplastin time and thrombin time at the highest concentration (100 mM), to a lesser degree at the lower concentration (10 mM). These changes reflected decreases of 19 and 36% respectively in Factor V and X activity, an inhibition of 63-75% of Factors VII, IX, X and XI activity, and 80% inhibition of thrombin activity. The inhibitory changes of carbamylation increased with time. No changes were seen in the activity of Factors I and VIII. Platelet function studies revealed no inhibition of Factor III release; aggregation was abnormal only at high concentrations with epinephrine and collagen induction and partially reversible by resuspension in normal plasma.

Adenosine Diphosphate↗