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Late onset of clinical symptoms and recurrent ecchymotic skin lesions in a 12-year-old girl with a severe double heterozygous protein C deficiency.

The authors describe a 12-year-old girl with late-onset clinical symptoms due to severe protein C deficiency. Protein C gene analysis showed double heterozygosity for two distinct mutations, associated with type I protein C deficiency. Her parents and only brother were also evaluated. Coumarin-induced skin necrosis was a recurrent feature during oral anticoagulation therapy, forcing her physicians to treat her with nadroparin (Fraxiparin) for only a few months.

Age of Onset↗

Severe bleeding symptoms in refractory idiopathic thrombocytopenic purpura: a case successfully treated with melatonin.

The prognosis of patients with idiopathic thrombocytopenic purpura refractory to corticosteroids and splenectomy (refractory ITP) is poor; these patients present high morbidity from disease and its treatment and have a mortality rate of approximately 16%. A patient is reported with severe bleeding symptoms related to refractory ITP successfully treated with melatonin.

Adjuvants, Immunologic↗

Bilateral eyelid ecchymoses and corneal crystals: an unusual presentation of multiple myeloma.

PURPOSE: This report describes a case of multiple myeloma that presented as bilateral eyelid ecchymoses and corneal crystals in the absence of widespread signs of systemic disease. METHODS: A 55-year-old man was found to have the sudden appearance of bilateral eyelid ecchymoses after he flexed 90 degrees at the waist. On examination, amyloid deposition was found in the area of the lid ecchymoses, and corneal crystals were dispersed centrally and peripherally throughout all levels of the cornea on slit lamp examination. Urinalysis revealed Bence-Jones proteins and free kappa light chains. Bone marrow plasmacytosis suggested multiple myeloma. RESULT: Ophthalmic and systemic findings led to a workup, which resulted in a diagnosis of multiple myeloma. CONCLUSION: Our case demonstrates that multiple myeloma may present clinically with ophthalmic findings such as bilateral eyelid ecchymoses and corneal crystals in the absence of widespread signs of disease. The ocular findings may be the first manifestations of disease. Therefore, a systemic workup should be performed on patients with this presentation, including urine and bone marrow analyses.

Amyloidosis↗

Envenomation by the Northern Blacktail Rattlesnake (Crotalus molossus molossus): case report.

The clinical course after a human envenomation by the Northern Blacktail rattlesnake (Crotalus molossus molossus) is not well described in the literature. The present report discusses a 12-year-old girl who was envenomated by C. molossus molossus and treated with antivenom (Crotalidae polyvalent immune Fab). Her recovery was uncomplicated, and she was discharged after 48 hours of hospitalization. Hematologic and coagulation studies were within normal limits at follow up. The clinical effects of C. molossus molossus envenomation are reviewed, and previous reports of bites by C. molossus molossus are discussed and compared with our patient.

Animals↗

Avoiding and treating dermal filler complications.

All fillers are associated with the risk of both early and late complications. Early side effects such as swelling, redness, and bruising occur after intradermal or subdermal injections. The patient has to be aware of and accept these risks. Adverse events that last longer than 2 weeks can be attributable to technical shortcomings (e.g., too superficial an implantation of a long-lasting filler substance). Such adverse events can be treated with intradermal 5-fluorouracil, steroid injections, vascular lasers, or intense pulsed light, and later with dermabrasion or shaving. Late adverse events also include immunologic phenomena such as late-onset allergy and nonallergic foreign body granuloma. Both react well to intralesional steroid injections, which often have to be repeated to establish the right dose. Surgical excisions shall remain the last option and are indicated for hard lumps in the lips and visible hard nodules or hard granuloma in the subcutaneous fat.

Adipose Tissue↗

Management of penile fracture.

BACKGROUND: Penile fracture is not a frequent event. It consists of rupture of the tunica albuginea of the corpora cavernosa. Fracture occurs when the penis is erect, as the tunica is very thin and not flexible. METHODS: This prospective study was carried out over a period of 1 year and included 12 patients presenting with penile fracture. RESULTS: Diagnosis was made clinically, and there was no need to perform cavernosography in any case. The most common cause of fracture was trauma to the erect penis during intercourse. Mean age of patients was 29.5 (+8.96) years, and mean time of presentation was 15.5 (+8.04) hours. Subcoronal circumferential degloving incision was done in all cases. Nine patients were operated on, and three patients refused surgery and were treated conservatively. Repair consisted of evacuation of hematoma and repair of the tunical defect with absorbable sutures. The mean operative time was 33.9 (+8.2) minutes. Preoperative and postoperative antibiotics were used, and all operated cases were discharged on the second postoperative day. All operated cases were able to achieve full erection with straight penis except one, in whom mild curvature and pain during erection was observed. CONCLUSION: Penis fracture is a true urologic emergency. It should be treated surgically as early as possible to ensure a better outcome.

Adolescent↗

The utility of clinical and laboratory data for predicting intraabdominal injury among children.

BACKGROUND: The initial assessment of the child with blunt injury should lead ideally to a low rate of missed intraabdominal injury (IAI) while avoiding unnecessary imaging among children without IAI. The purpose of this study was to determine the utility of clinical and laboratory data for predicting the risk for IAI. METHODS: Among 351 children evaluated for possible blunt abdominal trauma, 23 variables potentially associated with IAI were determined retrospectively. Logistic regression and recursive partitioning were used to identify variables and develop predictive models. RESULTS: Logistic regression identified four positive predictors (abdominal tenderness, abrasion, ecchymoses, and alanine aminotransferase) and two negative predictors (injury caused by a motor vehicle crash and hematocrit) for IAI. The recursive partitioning model predicted the absence of IAI with a sensitivity of 100% (95% CI confidence interval, 86-100%) and a specificity of 87% (95% CI confidence interval, 81-91%) using abdominal examination and aspartate aminotransferase as discriminating variables. CONCLUSIONS: Physical examination combined with selected laboratory studies can be used to predict the risk of IAI accurately among children who sustain blunt trauma. Application of these findings may be useful in reducing costs and improving the accuracy of diagnosing IAI among children.

Abdominal Injuries↗

Autosensitization to DNA: evidence for an immunologic basis.

A 59-year-old female with spontaneous painful ecchymoses developed ecchymoses after intracutaneous injection of washed autologous whole blood cells and calf thymus DNA. Immunofluorescent studies of the spontaneous lesions revealed granular deposits of IgM, C3, factor B and properdin at the dermal-epidermal junction but no deposits in her normal skin. T cells were decreased in number but responded normally to polyclonal mitogens and did not transform in response to DNA containing antigens. Repair of UV-damaged DNA by her lymphocytes appeared to be depressed. The findings presented here are the first immunologic abnormalities uncovered in this disorder and may help in understanding the pathogenesis of the inflammatory lesions seen in autosensitization to DNA.

Autoantibodies↗

Venlafaxine-induced ecchymoses and impaired platelet aggregation.

OBJECTIVE: To describe a case of venlafaxine-induced ecchymoses. METHODS: A patient with a history of ecchymoses coincident with venlafaxine therapy was rechallenged with the drug. Her platelet function was assessed with aggregation and ATP release studies before the rechallenge and after she developed ecchymoses. In addition, the effect of venlafaxine on platelet aggregation and ATP release was studied in vitro by adding the drug to platelet-rich plasma from normal donors. RESULTS: After 4 wk of treatment with venlafaxine our patient developed extensive ecchymoses. At that time her platelet aggregation and release responses to epinephrine, ADP, collagen, and arachidonic acid were markedly suppressed. Adding venlafaxine to normal platelet-rich plasma also dramatically reduced the aggregation and release responses to the same agonists as well as to serotonin, but the concentrations of venlafaxine required were 1000-fold greater than those normally achieved clinically. CONCLUSIONS: Our patient demonstrated an idiosyncratic hypersensitivity to the platelet inhibitory effects of venlafaxine. Because venlafaxine is an inhibitor of serotonin uptake by platelets and neurons, this mechanism may contribute to the impact of this drug on platelet function. However, our in vitro studies suggest that this hypothesis is inadequate to explain the observations completely.

Adenosine Triphosphate↗