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Hereditary factor VII deficiency in a Chinese family.

Hereditary deficiency of factor VII is demonstrated in a Chinese family. The proposita was a 32-year-old female with bleeding diathesis consisting of spontaneous ecchymosis, menorrhagia and recurrent haemarthrosis. The prothrombin time was prolonged and the prothrombin and proconvertin test was 10% of normal. The activated partial thromboplastin time and the Stypven-cephalin clotting time were normal. The prolonged prothrombin time could be corrected by the addition of normal serum, but not by adsorbed normal or coumadin plasma. The factor VII level was 3.6% of normal. One of her brothers had bleeding symptoms and died at age 25, suggesting that factor VII deficiency might have been present. 13 of her family members had partial deficiency of factor VII with plasma levels ranging from 24 to 50%. These results suggest an autosomal recessive inheritance with a homozygous state occurring in the proposita and possibly in her brother, and a heterozygous state occurring in 13 of her family members. Our study marks an extensive survey of factor VII deficiency in an Oriental family.

Adult↗

Myocarditis and generalised vasculitis associated with leishmaniosis in a dog.

A three-year-old, female bulldog was presented with bilateral uveitis, apathy, listlessness, generalised lymphadenopathy and perivulvar haematoma. The initial laboratory studies showed non-regenerative anaemia, polyclonal gammopathy and a high urine protein:creatinine ratio. Serology for leishmaniosis was positive and treatment with allopurinol and meglumine antimoniate was started. Despite treatment, the dog's clinical condition deteriorated. Signs included cutaneous ecchymosis, respiratory distress and finally cardiorespiratory arrest. Histopathological studies of postmortem tissue samples revealed a generalised vasculitis of several internal organs and severe myocarditis. Leishmania species organisms were identified in affected tissues using immunoperoxidase labelling and PCR techniques.

Animals↗

Performing a breast biopsy with a directional, vacuum-assisted biopsy instrument.

A recently developed method of minimally invasive breast biopsy involves use of a directional, vacuum-assisted instrument. Use of this instrument requires some changes in techniques and applications of breast biopsy, but it enables confident biopsy of breast lesions under both ultrasound (US) and stereotactic guidance. The device uses vacuum to pull tissue into the probe and to remove the specimen without withdrawing the probe each time. For stereotactic biopsy, to target the lesion, the probe is placed anterior or posterior to the lesion and stereotactic positioning views are obtained; for a US-guided procedure, the probe is advanced posterior to the lesion. Next, the direction that the probe aperture must be rotated to face the lesion is determined. Tissue samples are obtained at consecutive clock positions of 1 1/2-hour intervals to achieve contiguous sampling. At least 15 samples are obtained with an 11-gauge probe to acquire a minimum of 1,500 mg of tissue. If postbiopsy images reveal that the lesion has been removed, a percutaneous clip is placed to mark the biopsy site for follow-up examination and possible further treatment. Patients are examined the next day and given the biopsy results and treatment considerations, if needed; they are followed up approximately 1 week later to detect any complications (eg, discomfort, ecchymosis). By learning how to perform a directional, vacuum-assisted biopsy with either stereotactic or US guidance, the radiologist has an additional, valuable tool for bringing accurate breast biopsy to his or her community.

Biopsy, Needle↗

Complications of outpatient transbrachial intraarterial digital subtraction angiography. Work in progress.

To assess the safety of nonselective intraarterial digital subtraction angiography (DSA) performed from a brachial artery approach, the complications of 660 consecutive examinations, most of which (greater than 95%) were performed on outpatients, were studied. Contrast material injections into the aorta were made through a 4-F multiple side-hole pigtail catheter inserted percutaneously from the brachial artery. Two brachial artery complications severe enough to require surgery occurred (one hematoma and one arterial laceration/thrombosis), for a rate of 0.3%. No cerebrovascular complications were encountered. Delayed and minor complications were retrospectively studied in 137 patients; they included local arm pain in 24 patients (17.5%), transient paresthesia in ten (7.3%), ecchymosis in 69 (50.4%), and hematoma in 13 (9.5%). The transbrachial approach to nonselective intraarterial DSA is a safe alternative to the femoral artery approach.

Adolescent↗

Bleeding and subsequent anemia: a precipitant for cerebral infarction.

BACKGROUND AND OBJECTIVES: The relationship between bleeding and subsequent anemia (BSA) and the occurrence of stroke has not been sufficiently studied. The purpose of the present study was to elucidate the characteristics of stroke associated with BSA. METHODS: We studied 16 consecutive patients with acute stroke associated with anemia (hemoglobin level on admission < or =9.0 g/dl) and compared their stroke subtypes with those of 32 control subjects. RESULTS: The cause of anemia was upper gastrointestinal bleeding in 11 patients (ulcers in 8; carcinomas in 2, and hemorrhagic gastritis in 1), bleeding from a hemorrhoid in 2, uterine cervical bleeding in 1, ecchymosis probably related to medication in 1, and chronic blood drainage in 1. At least 10 patients had a history of recent (<1 week), active bleeding. Clinical and imaging studies showed that all the patients had infarcts and none had intracerebral hemorrhages. Thirteen patients had infarcts in the region of the middle cerebral artery (MCA) (total MCA region in 2; partial, cortical area in 5; subcortical area in 5, and lenticulostriate artery region in 1), 2 had anterior cerebral artery (ACA) region infarction, and 1 had cerebellar infarction. All 11 patients who underwent vascular imaging studies showed significant stenosis and/or occlusion of the internal carotid artery (ICA) (n = 5), the MCA (n = 4), both the ICA and MCA (n = 1), or the ACA (n = 1). Of the different stroke subtypes (large vessel infarction (LVI), small vessel infarction, cardiogenic embolic infarction, intracerebral hemorrhage), LVI was significantly (p<0.05) more frequent in patients with stroke associated with BSA than in the controls, even though the demographics and risk factors were similar in each group. CONCLUSIONS: The close temporal relationship between the bleeding and the onset of stroke, as well as the predominance of the LVI subtype in the BSA-associated group as compared to controls, suggest that BSA may precipitate atherothrombotic cerebral infarction. A hemodynamic alteration, enhanced thrombosis or a combination of these appears to be the pathogenic mechanism.

Aged↗

Sphincterolysis: a novel approach towards chronic anal fissure.

BACKGROUND AND AIMS: The surgical approach in chronic anal fissure is often found associated with disturbed anal continence as well as recurrence. This report describes the author's approach of 'sphincterolysis' or fragmentation of the fibers of the internal sphincter on the left lateral anal wall. PATIENTS AND METHODS: 132 patients with chronic anal fissures were treated with this technique. Pre- and postoperative anal manometry was recorded. The postoperative course and early and 1-year follow-up results were recorded. RESULTS: Early complications included ecchymosis, hematoma, and pain. Fissure healing and relief of symptoms observed in 97% of patients. A transient, variable degree of incontinence occurred in 23 patients and persistent incontinence to flatus and soiling in 5. CONCLUSION: Internal anal sphincterolysis is a safe, effective procedure for the treatment of chronic anal fissure.

Adolescent↗

Spontaneous retroperitoneal hemorrhage in chronically hemodialyzed patients.

Patients maintained on chronic hemodialysis have an increased incidence of spontaneous bleeding from various parts of the body. In this paper we describe 5 patients who developed spontaneous retroperitoneal hemorrhage. All patients were receiving anticoagulants (Coumadin and/or heparin) at the time of bleeding. Clinically, retroperitoneal hemorrhage was evident in the interdialytic period. Presenting symptoms and signs included sudden and progressive onset of pain in the abdomen, flank, back, thigh or hip associated with a drop in blood pressure and hematocrit in the absence of obvious blood loss. Subsequently ecchymosis, swelling and/or mass were observed. Abdominal X-rays showed absent psoas shadows. Treatment included blood transfusions, discontinuation of Coumadin and/or heparin, the use of regional heparinization for hemodialysis and occasionally peritoneal dialysis. Surgical exploration was not required in any case and all 5 patients recovered. Anticoagulation therapy and platelet dysfunction may be contributory causes.

Aged↗

Platelet aggregation defect in megakaryoblastic leukemia.

Platelet aggregation induced in vitro with ADP, adrenalin and ristocetin was tested in 7 patients with megakaryoblastic leukemia (MKL). All patients had normal or high platelet counts and presented with hemorrhagic diathesis including purpura ecchymosis and epistaxis. Platelet morphology was grossly abnormal and electron microscopy revealed few, or absence of, alpha-granules. Platelet aggregation was reduced in all the cases with at least one aggregating agent. Our studies confirm that MKL is often accompanied by a thrombocytopathy which should be taken into account in the management of these patients.

Aged↗

Vasopressin-induced amber-like skin necrosis.

Case reports about vasopressin-induced cutaneous necrosis are not frequent. Here we report a further case, of which skin manifestations included not only mottling, cyanosis, ecchymosis, bullae and gangrene, but also amber-like change in focal areas. Besides, intermittent paling of the skin with or without deep pain sensation of the limbs over non-injection sites was observed that might be a warning sign of impending skin necrosis. Based on the literature about vasopressin-induced skin necrosis we discuss the possible role of coagulation enhancement of this molecule.

Esophageal and Gastric Varices↗

Spontaneous orbital hemorrhage in adult females. A report of three cases.

Three cases of spontaneous orbital hemorrhages in 3 adult females are reported. All of the patients had acute radiating pain in the orbit, vomiting and proptosis with a limitation of motility and ecchymosis of the eyelids. One was due to a large orbital varix with a preceding history of intermittent exophthalmos; the causes of the other cases could not be determined from their backgrounds. Within a few weeks, all of them had recovered from hematoma and had good prognoses without surgery. Orbital venous bleeding with 40 mm Hg pressure will cause more than 500 g tension on the four rectus muscles. To treat this clinical emergency, hemostasis with compression in the early phase and waiting for its spontaneous absorption are recommended.

Adult↗

A new, noninvasive technique for inducing post-extrasystolic potentiation during echocardiography.

Left ventricular function was evaluated in 34 patients with the echocardiogram, and an external mechanical cardiac stimulator was used to induce a ventricular premature contraction (VPC) noninvasively. Extent of post-extrasystolic potentiation (PESP) was determined by comparing systolic dimensional shortening and ejection fraction of the sinus beat preceding the VPC to that of the potentiated beat which followed it. Using this technique, a VPC could be introduced into the cardiac cycle of 30 of the 34 patients, six of whom were free of obvious cardiac disease and 24 of whom had valvular, coronary or myopathic heart disease. The only complication observed was mild breast ecchymosis in a female patient. Systolic dimensional shortening and ejection fraction increased from control values by an average of 21% and 17% respectively, with a range of 0-100%. The degree of PESP was very reproducible in repeat studies and when the same patients were subsequently evaluated during a spontaneously occurring or catheter-induced VPC. The technique can safely and reliably induce post-extrasystolic potentiation during echocardiography and is a potentially important adjunct to the noninvasive evaluation of left ventricular function.

Adult↗

A pilot study of a new thrombolytic agent for acute ischemic stroke in Taiwan within a five-hour window.

BACKGROUND AND PURPOSE: This study was the first clinical trial in Taiwan of a new thrombolytic agent human tissue urokinase type plasminogen activator (HTUPA) in patients with acute ischemic stroke. METHODS: Patients were treated with a single bolus intravenous HTUPA under an open-label dose escalation design within 5 hours after symptom onset. Safety outcomes were assessed by symptomatic and asymptomatic intracerebral hemorrhage (ICH) as well as other bleeding episodes. Preliminary efficacy was measured by National Institutes of Health Stroke Scale (NIHSS). RESULTS: Three doses of HTUPA (0.3 mg/kg, 0.35 mg/kg, and 0.4 mg/kg) were administered to 33 patients, with the majority of patients (n=29) receiving 0.3 mg/kg. Two cases of fatal ICH occurred: 1 in the patient who received 0.4 mg/kg and the other in the 0.3 mg/kg group. Asymptomatic ICH occurred in 6 patients. Other treatment-related serious adverse events were ecchymosis, hematuria, and upper gastrointestinal bleeding, which were completely recovered. At day 90, in patients treated with 0.3 mg/kg within a 0- to 5-hour window, 34% reached NIHSS scores 0 to 1, whereas of those treated within 0 to 3 hours, 86% reached this score. CONCLUSIONS: Intravenous HTUPA, given at 0.3 mg/kg as a bolus injection within 5 hours after symptom onset, had an acceptable safety and efficacious profile in patients with acute ischemic stroke.

Aged↗

Similarity of insulin detemir pharmacokinetics, safety, and tolerability profiles in healthy caucasian and Japanese american subjects.

The objective of this study was to compare the pharmacokinetics of insulin detemir in three ascending doses in healthy Japanese and Caucasian subjects. This was an open-label, single-center, parallel-group design evaluating 30 subjects (15 Japanese and 15 Caucasians). Subjects received a total of three subcutaneous injections (one injection per visit) of insulin detemir (0.19, 0.38, 0.75 U/kg [1 U = 24 nmol]) in ascending order. Following drug administration, subjects received intravenous glucose in 0.5-mg/kg/min increments every 30 minutes, followed by a constant rate of 2.0 mg/kg/min for up to 12 hours. For pharmacokinetic evaluations, serial blood sampling was performed over a period of 30 hours after dosing. Of the subjects, 36 were enrolled, and 30 completed the study. There was a linear dose-response relationship between the three ascending insulin detemir doses and serum insulin detemir AUC values for both the Japanese and Caucasian subjects. The two dose-response regression lines had equivalent slopes but slightly different intercepts (although not statistically significant). This difference may be due to variation in AUC, body weight differences, or chance. Six subjects discontinued the study, 2 as a result of adverse events (blood draw-related ecchymosis and hypoglycemia). The most frequent treatment-emergent adverse events (TEAE) were headache, dizziness, and reactions related to blood draws/infusion sites. All TEAEs were mild to moderate in severity. The results show that an increase in insulin detemir dose will result in a similar increase in insulin detemir concentration in the two ethnic groups. Therefore, therapeutic dosing of insulin detemir is expected to be similar in both ethnic groups, with no special dose adjustment or algorithm based on race. Insulin detemir at 0.19, 0.38, and 0.75 U/kg was generally well tolerated in both Japanese and Caucasian subjects.

Adolescent↗

Immediate surgical repair of the medial patellar stabilizers for acute patellar dislocation. A review of eight cases.

An open surgical repair of the injured medial patellar stabilizers, including the vastus medialis obliquus muscle and the medial patellofemoral ligament, after acute patellar dislocation was studied in eight patients. At initial examination, all patients had tenderness over the adductor tubercle and a positive patellar apprehension sign. Four of eight patients had obvious ecchymosis over the adductor tubercle. Magnetic resonance imaging, diagnostic arthroscopy, and open surgical exploration documented injury to both the medial patellofemoral ligament and the origin of the vastus medialis obliquus muscle. In all patients, the torn muscle was retracted in an anterior and superior direction and an arthroscopic lateral release was performed followed by open primary repair of the medial patellofemoral ligament to the adductor tubercle and repair of the vastus medialis obliquus muscle to the adductor magnus tendon. Patients were evaluated postoperatively with the Kujala scoring questionnaire. The average follow-up was 3.0 years, with a minimum of 1.5 years. No patients experienced a recurrent dislocation. The average Kujala score was 91.9. Patients rated their return to athletic activity at an average 86% of their pre-injury level. The average subjective satisfaction was 96%. In appropriate cases of acute patellar dislocation, we recommend primary repair of the medial patellofemoral ligament and the vastus medialis obliquus muscle to avoid recurrent dislocation, chronic subluxation, pain, and disability.

Adolescent↗

Traumatic dislocations of the peroneal tendons.

Traumatic dislocation of the peroneal tendons is an often unrecognized injury which has been reported to occur most commonly during snow skiing. The strength of the peroneal retinaculum is exceeded during resistance to violent passive dorsiflexion or to inversion stress. Pain, swelling, and ecchymosis may hinder early diagnosis; however, intense retromalleolar pain on active eversion is a specific, highly suggestive finding. Fracture of a thin shell of the lateral malleolar cortex is diagnostic. In chronic cases, marked dislocation of the tendons is frequently demonstrable, with more than the usual degree of snapping. Surgical repair is advocated, using one of several procedures available. Most acute cases can be treated by simple repair of the torn or fractured structures. In chronic cases, or in acute cases with deficient structures predisposing to dislocation, it is necessary to reconstruct the peroneal retinaculum and/or deepen the peroneal groove. Longitudinal splitting of the peroneus brevis tendon was a new finding in this series.

Ankle Injuries↗

Spontaneous rupture of the plantar fascia.

In this study, rupture of the plantar fascia was seen in five feet, of which four had had plantar fasciitis. At the time of the injury, which is an acceleration type of motion, there is severe pain in the heel followed by the development of ecchymosis in the sole and toward the heel of the foot. With conservative symptomatic care, the acute symptoms as well as the plantar fasciitis symptoms subside, generally allowing full activity in 3 to 4 weeks.

Adult↗

Traumatic focal posterior tibialis muscle denervation.

The purpose of this case presentation is to demonstrate posterior tibialis muscle (PTM) denervation as a cause of traumatic plantarflexion inversion weakness. In a 42-year-old woman, severe pain, swelling, and ecchymosis over the medial aspect of her left ankle developed after she twisted it while playing tennis. Plantarflexion inversion weakness developed (grade 3/5). The strength of all other muscle groups of the lower extremity was normal. Her pin and light touch sensation were normal in the left lower extremity. Deep tendon reflexes were equal and active at both knees and ankles. A magnetic resonance image of the left leg, ankle, and foot performed 1 month after injury demonstrated an intact posterior tibialis tendon behind the medial malleolus and edema-like increased signal intensity in the PTM on the T1-weighted image consistent with denervation. On electromyographic testing, there were continuous fibrillation and positive sharp wave potentials in every site tested in the PTM without any voluntary motor unit activity. The left extensor hallucis, left gastrocnemius, and lumbar paraspinal muscles were normal. In conclusion, combined magnetic resonance imaging and electromyographic studies supported denervation of the PTM as the cause of plantarflexion inversion weakness, rather than posterior tibialis tendon rupture in this patient.

Adult↗

Acute infantile haemorrhagic oedema associated with hepatitis A.

Acute infantile haemorrhagic oedema (AIHO) is characterised by purpura, ecchymosis and inflammatory oedema of the face and extremities. It is seen in children aged 4-24 months. The atiology is not known. We report a case of AIHO diagnosed by skin biopsy demonstrating leukocyte-elastic vasculitis. Laboratory studies showed positive hepatitis A IgM and IgG antibodies. The liver function tests were normal, indicating subclinical hepatitis. Cryoglobulinaemia was detected, suggesting that the disease was related to hepatitis A.

Acute Disease↗