Diagnosis and treatment of disseminated intravascular coagulation

Disseminated intravascular coagulation (DIC) is a complex clinical syndrome with activation of the procoagulant and fibrinolytic systems along with inhibitor consumption. We discuss regarding the controversies in diagnosis and management of DIC. Bleeding is a more common manifestation of DIC but most of the morbidity and mortality of DIC is due to microvascular thrombosis. Routinely performed tests for DIC such as platelet count and prothrombin time may be normal in chronic DIC. There is no single test that would diagnose DIC, however, estimation of D-dimer appears to be the most sensitive and specific test. Therapy of DIC aims at treating the primary cause. Fresh frozen plasma and platelet concentrates are recommended only in bleeding patients and have the potential risk of adding procoagulant material to the already activated procoagulant system. Role of heparin and antithrombin in patients with sepsis and DIC is discussed.     

Adult type granulosa cell tumor causing precocious pseudopuberty in a 6 year-old girl

Malignant ovarian tumors are responsible for 2-3% of all cases of precocious pseudopuberty (PP) in girls. The most common forms of ovarian tumors presenting as PP are the granulosa cell tumors (GCT). The clinical and pathological features of granulosa cell tumors that occur frequently in young girls, so-called juvenile granulosa cell tumors (JGCT), differ histologically from those occurring in adults. As a cause of PP in young girls, adult type granulosa cell tumors (AGCT) are extremely rare. We report a 6 year-old girl presenting with early breast development and vaginal bleeding due to a well encapsulated ovarian tumor. Microscopic features of the resected tumor were characteristic of AGCT.     

Three-dimensional ultrasonographic ovarian measurements and in vitro fertilization outcome are related to age

OBJECTIVE: To evaluate whether the number of ovarian antral follicles, ovarian volume, and ovarian stromal blood flow change with age and to prospectively analyze whether three-dimensional ultrasonographic measurements predict ovarian response and IVF outcome. DESIGN: Prospective analysis. SETTING: Assisted reproductive unit. PATIENT(S): Fifty-six consecutive women 22 to 43 years of age with normal basal serum FSH concentrations who were undergoing their first IVF cycle. MAIN OUTCOME MEASURE(S): Number of ovarian antral follicles, ovarian volume, and ovarian stromal flow index were determined by three-dimensional and power Doppler ultrasonography. Pretreatment measurements were compared with number of recovered oocytes, fertilization rates, and pregnancy rates. RESULT(S): As patient age increased, significant trends in ovarian volume, number of follicles, and stromal vascularity decreased. Three-dimensional ovarian measurements and fertilization rates differed significantly among age groups. For each age group, a higher number of antral follicles, greater ovarian volume, and favorable ovarian stromal vascularity was associated with higher number of retrieved oocytes and increased pregnancy rates. CONCLUSION(S): Increasing patient age is associated with poor ovarian response, as represented by smaller ovarian volume, lower antral follicle count, and poor stromal vascularity. Three-dimensional power Doppler ultrasonography can help to individualize IVF in patients regardless of age.     

Prenatal diagnosis: what does four-dimensional ultrasound add?

INTRODUCTION: The key benefit of 4D ultrasound lies in providing real-time 3D images of embryonic or fetal movements, previously limited by technological advancement. AIM: To classify types of first trimester embryonic and fetal movements in normal pregnancies as seen by 4D sonography. RESULTS: Three types of movements can be visualized in the first trimester: gross body between seven and eight weeks, limb movements after ten weeks and complex limb movements after 11 weeks of gestation. DISCUSSION: An alteration from the given pattern of motoric development should be considered as an indication for further investigation. CONCLUSION: 4D ultrasound enables visualization of more details of the dynamics of small anatomical structures. Therefore, body and limb movements can be visualized a week earlier than with 2D.     

Predicting death from HIV/AIDS: a case-control study from Florida public HIV/AIDS clinics

BACKGROUND: After markedly decreasing for 3 years, HIV/AIDS mortality declined only slightly in 1999. METHODS: The authors conducted a case-control study in four Florida urban public health HIV clinics to evaluate modifiable factors associated with HIV/AIDS mortality in a non-research setting. Structured chart review was conducted for 120 case-patients who died in 1999 and for 240 randomly selected control-patients. Risk factors associated with death in univariate analysis were entered into three conceptually related, matched logistic regression models. RESULTS: In the final multivariate model, homelessness (adjusted odds ratio [AOR], 9.98; 95% confidence interval [CI], 2.34-42.5), Medicaid insurance (AOR, 3.10; 95% CI, 1.43-6.74), having a documented adherence problem (AOR, 3.50; 95% CI, 1.64-7.47), injection drug use (AOR, 2.46; 95% CI, 1.11-5.43), non-specific liver failure (AOR, 76.9; 95% CI, 6.79-870.9), interrupted highly active antiretroviral therapy (HAART) secondary to side effects (AOR, 4.00; 95% CI, 1.46-10.9), and not receiving HAART (AOR, 2.62; 95% CI, 1.03-6.68) were independent predictors of mortality. CONCLUSIONS: In addition to medical and clinical indicators, several sociobehavioral-demographic factors remained important throughout the multivariate analysis. Improvement in care should include a focus on social circumstances of infected people. Special attention to the homeless, those with adherence problems, and those with liver disease is clearly indicated.     

Cutaneous melanoma in childhood and adolescence

Using population-based data from the Surveillance Epidemiology and End Results Program of the National Cancer Institute, melanoma occurring during childhood was evaluated. Compared to adult cases of melanoma, childhood cases had a higher proportion of females (61%) and non-Caucasians (6.5%). The incidence of melanoma increased 85% among 15- to 19-year-olds from 1973 to 1996. Incidence for 15- to 19-year-olds was higher in southern (23.9/million) than northern registries (14.5/million). Non-Caucasians had 3-30% of the cases expected compared to Caucasians. Overall survival of children/adolescents with melanoma was 89% and 79% at 5 and 20 years post diagnosis, respectively. The majority of deaths were directly attributed to melanoma (72%).     

Concealed penis

A small phallus causes great concern regarding genital adequacy. A concealed penis, although of normal size, appears small either because it is buried in prepubic tissues, enclosed in scrotal tissue penis palmatus (PP), or trapped due to phimosis or a scar following circumcision or trauma. From July 1978 to January 2001 we operated upon 92 boys with concealed penises; 49 had buried penises (BP), while PP of varying degrees was noted in 14. Of 29 patients with a trapped penis, phimosis was noted in 9, post-circumcision cicatrix (PCC) in 17, radical circumcision in 2, and posttraumatic scarring in 1. The BP was corrected at 2-3 years of age by incising the inner prepuce circumferentially, degloving the penis to the penopubic junction, dividing dysgenetic bands, and suturing the dermis of the penopubic skin to Buck's fascia with nonabsorbable sutures. Patients with PP required displacement of the scrotum in addition to correction of the BP. Phimosis was treated by circumcision. Patients with a PCC were recircumcised carefully, preserving normal skin, but Z-plasties and Byars flaps were often required for skin coverage. After radical circumcision and trauma, vascularized flaps were raised to cover the defect. Satisfactory results were obtained in all cases although 2 patients with BP required a second operation. The operation required to correct a concealed penis has to be tailored to its etiology.     

Infantile fibrosarcoma associated with urticaria pigmentosa

The authors report the case of an infant with urticaria pigmentosa who developed infantile fibrosarcoma. The tumor was successfully resected but the skin lesions have persisted. This is the first report of a fibrosarcoma in association with urticaria pigmentosa. A brief review is presented of the available medical literature on associations of solid tumors with mastocytosis.     

Update on spondyloarthropathies

Spondyloarthropathies are a cluster of interrelated and overlapping chronic inflammatory rheumatic diseases that primarily include ankylosing spondylitis, reactive arthritis, and the arthritis associated with psoriasis and inflammatory bowel diseases. The primary pathologic sites are the entheses (the sites of bony insertion of ligaments and tendons); the axial skeleton, including the sacroiliac joints; the limb joints; and some nonarticular structures, such as the gut, skin, eye, and aortic valve. Although spondyloarthropathies are not associated with rheumatoid factor, they show a strong association with HLA-B27; however, this association varies markedly among various spondyloarthropathies and among ethnic groups. The most widely used classification criterion, from the European Spondyloarthropathy Study Group, encompasses the currently recognized wider disease spectrum, with a sensitivity and specificity that generally exceed 85%. Spondyloarthropathies occur in genetically predisposed persons and are triggered by environmental factors, but the cellular and molecular mechanisms of inflammation are not yet fully understood. Chlamydial and many enterobacterial infections can trigger reactive arthritis, but an infectious trigger for ankylosing spondylitis has not yet been established. HLA-B27 itself is involved in enhancing genetic susceptibility, but the underlying molecular basis is still unknown; additional genes include the putative susceptibility genes for Crohn disease, ulcerative colitis, and psoriasis. A specific susceptibility gene for Crohn disease, NOD2, is located on chromosome 16q12, and one of the candidate genes for psoriasis, PSORS1, has been mapped to a 60-kb fragment on chromosome 6p, which is telomeric to the HLA-C locus. This paper reviews the efficacy of anti-tumor necrosis factor-alpha therapy and other therapeutic advances.