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Biomedical subjects

J H Lewis

Publications and source records attributed to J H Lewis.

At least 181 records · Page 10Linked to original sources
PubMed1981

Factor VII deficiency.

This report describes three patients with factor (F) VII deficiency: two adult siblings and an unrelated 5 1/2-month-old child who succumbed after several central nervous system (CNS) hemorrhages. This event prompted a review of the literature concerning the incidence and characteristics of intracranial hemorrhage in congenital F VII deficiency. Of 138 patients reported to have F VII deficiency, only 75 were considered to have a true deficiency. There was a 1:1 sex distribution with a 19% incidence of consanguinity in the 63 families which these 75 patients represented. CNS hemorrhage occurred in 12 of the 75 proven factor-deficient patients -- an incidence of 16.0%. There was a 1.4:1 female predominance in this group with a 44.4% incidence of consanguinity in their nine families. Except for one patient with hypertension, there was no history of preceding trauma or previous underlying CNS abnormality, though head trauma with a difficult vaginal delivery may have occurred in five infants. Diagnostic lumbar puncture or ventricular tap revealed bloody, xanthochromic cerebrospinal fluid in five. Five patients with F VII deficiency developed a CNS hemorrhage prior to 1 week of age, and none survived. Seven patients older than 1 week of age suffered such an event, and four of these survived. It is concluded that the greatest risk factor for development of CNS hemorrhage is trauma related to the birth process.

Adult↗
PubMed1981

Corticosteroid therapy for acquired F VIII:C inhibitors.

The appearance of an acquired inhibitor to factor VIII clotting activity (F VIII:C) in a non-haemophiliac is an uncommon phenomenon; but, when it occurs, represents a difficult therapeutic problem. Cyclophosphamide has been the most frequently used form of therapy while corticosteroids, when used alone, have been felt to be ineffective. During the past 25 years, 18 of these patients have been evaluated in Pittsburgh. Their mean age was 63.7 years. Nine were female and nine male. Sixteen of the 18 received corticosteroids as their primary form of therapy. Eleven of the 16 (69%) showed clinical and laboratory improvement. Seven of the 11 had a complete response and the remaining four a partial response. Five patients demonstrated no improvement with corticoid therapy. The time to response varied from 4 to 41 d (mean 15.8). It was concluded that corticosteroids when used alone provide therapeutic results comparable to immunosuppressive therapy in the treatment of acquired inhibitors to F VIII:C and therefore represent a therapeutic alternative to immunosuppressive agents.

Adrenal Cortex Hormones↗
PubMed1980

Disseminated intravascular coagulation. Findings in 346 patients.

An analysis was made of 346 cases of disseminated intravascular coagulation (DIC) diagnosed by utilizing a combination of laboratory tests which reflect the pathophysiology of the syndrome. The goals of the study were three fold: 1) to compare our clinical disease categories with those of other investigators, 2) to re-evaluate the diagnostic tests and, 3) most importantly, to report the results of tests infrequently performed when evaluating DIC. The patients fell into the following groups: 1) infection -- 26%, 2) malignancy -- 24%, 3) surgery and trauma -- 19%, 4) liver disease -- 8%, 5) miscellaneous -- 23%. Of the diagnostic tests, those for fibrin split products (FSP), fibrin monomer and antithrombin III were the most valuable. Of the clotting proteins, factors II, V, VII and X were the most frequently decreased. The factor VIII: C levels were in conflict with the prevailing dogma. Factor VIII:C levels were decreased in only 9% of patients studied and, in fact, were increased in the majority of cases. Factor VIIIR:Ag and F VIIIR:vW were elevated in 80% of the patients evaluated. An overall mortality of 68% further confirms the dismal prognosis previously associated with DIC.

Adolescent↗
PubMed1980

The Pennsylvania hemophilia program 1973-1978.

In Pennsylvania, the prevalence of hemophilia is one per 10,000 males. Factor VIII deficiency is five times more frequent than Factor IX deficiency, and 34% of the patients have no relatives affected with the disease. The mean age is 23 years old, and 50% of the patients are less than 20 years old. Approximately one-third of the patients with Factor VIII deficiency and one fourth of the patients with Factor IX deficiency have levels of less than 0.01 mu/ml. By clinical criteria, 55% of those with Factor VIII deficiency are severe compared to 45% of those with Factor IX deficiency. Factor VIII-deficient patients are treated an average of 18 times per year compared to ten times per year for patients with Factor IX deficiency. Hemarthroses account for 70% of the hemorrhages treated and for 40% of the concentrate usage. Home therapy patients use an average of 45,950 Factor VIII units per year at a cost of ø170 per patient and their use accounts for 60% of the total Factor VIII usage of 1.7 million units. Less than five days per patient per year are lost from school or work because of bleeding, and patients are hospitalized for bleeding an average of only two to three days per patient year. Adverse immediate reactions to therapy are infrequent. Five percent of patients have persistence of the hepatitis B virus, and 7.5% have inhibitors. The mortality rate is 0.04% per year, with half of the deaths being hemophilia-related.

Adult↗
PubMed1980

Liver dysfunction in Pennsylvania's multitransfused hemophiliacs.

Transaminase values [alanine amino transferase (ALT) and aspartate amino transferase (AST)] and markers for hepatitis B were serially determined in 558 hemophiliacs exposed to blood products. Hepatitis B surface antigen (HBsAg) persistent for over 12 months was present in 6% of the patients. Antibody to hepatitis B surface antigen (anti-HBs) was noted in 90% of the 259 patients treated with factor VIII or IX concentrates but in only 49% of the 43 patients treated with fresh frozen plasma (FFP) or cryoprecipitate. Persistently abnormal transaminase values were noted in 31% of the patients treated with commercial concentrates but in only one (2%) of the patients exposed to cryoprecipitate or FFP. This difference continued even when the two groups of patients were matched for the amount of blood products, up to 50,000 units, which they had received in the study period. In the concentrate-treated patients, no correlation could be found between transaminase values and the number of units of factor VIII or IX they had received during the six years of the study (1973-1978).

Adolescent↗
PubMed1980

Efficacy and safety of ibuprofen for hemophilic arthropathy.

The safety and usefulness of ibuprofen (Motrin) were evaluated in 15 patients suffering from hemophilic arthropathy via a double-blind protocol with individual crossover. No significant increase in hemorrhagic episodes or alteration in platelet function was observed. A decrease in morning stiffness was noted in nine patients, and three decreased markedly their use of other pain medications. We conclude that ibuprofen represents a relatively safe agent for the management of discomfort caused by hemophilic arthropathy in a select group of hemophilic patients.

Ankle Joint↗
PubMed1979

A routine screening method for the major metabolite of methyl phenidate in the urine of greyhounds.

A qualitative urine screening procedure for the determination of the major urinary metabolite of methyl phenidate, alpha-phenyl-2-piperidine acetic acid (ritalinic acid), has been devised. The method involves a salting-out extraction, conversion of the metabolite to the parent drug using methanol-hydrogen chloride, and simultaneous two-column gas-chromatographic detection. Final confirmation of the presence of the drug is by two-system thin-layer chromatography. The method is suitable for the detection of illicitly administered methyl phenidate in greyhounds.

Animals↗
PubMed1979

The high risk of chronic liver disease in multitransfused juvenile hemophiliac patients.

Eighty-seven asymptomatic children with either hemophilia A or B were treated before they were 21 years of age. Seventy-two received factor concentrates and 15 cryoprecipitate or fresh-frozen plasma only. Thirty-two of the 72 in the former group have persistently elevated alanine aminotransferase values compared to one of 15 in the latter group. In a subset of children treated with factor concentrates before five years of age, four of seven are chronically HBsAg positive. Liver biopsies were performed in 13 of the 32 asymptomatic patients with abnormal ALT values in the fraction group. It is recommended that until further data become available, children with mild hemophilia and all less than 5 years of age should receive only cryo or FFP.

Adolescent↗
PubMed1979

Temporal facilitation and stroboscopic motion.

Luminance of extended-duration adapting field was varied from zero to a level sufficient to reduce brightness of a 5 ms test stimulus to a level just above increment threshold. Increasing the luminance of surrounds or solid adapting fields resulted in increased reaction time. With the perceived-order method, reduction of latency with increasing surround luminance (temporal facilitation) was obtained with a visual comparison stimulus but not with an auditory comparison. It was concluded that perceived motion is a necessary condition for temporal facilitation. This study and earlier studies using moving test stimuli may be explained by a shift from sustained to transient units with increasing adapting luminance.

Adaptation, Ocular↗