Oral hairy leukoplakia: clinicopathologic features, pathogenesis, diagnosis, and clinical significance

Oral hairy leukoplakia (OHL) is a lesion frequently, although not exclusively, observed in patients infected by human immunodeficiency viruses (HIV). OHL is clinically characterized by bilateral, often elevated, white patches of the lateral borders and dorsum of the tongue. Histologically, there is profound acanthosis, sometimes with koilocytic changes, and a lack of a notable inflammatory infiltrate. The koilocytic changes are due to intense replication of Epstein-Barr virus (EBV), while epithelial hyperplasia and acanthosis are likely to result from the combined action of the EBV-encoded proteins, latent membrane protein-1, and antiapoptotic BHRF1. How OHL is initiated and whether it develops after EBV reactivation from latency or superinfection remain unresolved; nevertheless, definitive diagnosis requires the demonstration of EBV replicating vegetatively in histological or cytological specimens. In patients with HIV infection, the development of OHL may herald severe HIV disease and the rapid onset of AIDS, but despite its title, OHL is not regarded as premalignant and is unlikely to give rise to oral squamous cell carcinoma.     

Autoimmune hyperthyroidism in prepubertal children and adolescents: comparison of clinical and biochemical features at diagnosis and responses to medical therapy

The purpose of this study was: (i) to examine the impact of the clinical severity, anatomical location and treatment of psoriasis on patients' quality of life, and (ii) to investigate the effects of perceptions of psoriasis-related stress on patients' physical and mental health and on areas of disability in everyday life. All patients (n = 204) attending a psoriasis specialty clinic were invited to complete a multidimensional quality of life assessment comprising the Psoriasis Disability Index (PDI), the SF-36 Health Survey and the Psoriasis Life Stress Inventory (PLSI). Results (n = 150) indicated that overall clinical severity of psoriasis as assessed by the Psoriasis Area and Severity Index, and duration of psoriasis, were unrelated to impairment in any areas of quality of life. Anatomical location (social visibility) of psoriasis was associated with self-report of poor physical health (P = 0.01), and there was a modest association with patients' mental health (P = 0.04); however, anatomical location of psoriasis was not significantly associated with self-reported disability in everyday life, or stress scores. Patients who were classified as more reactive to the stress associated with psoriasis (78% of the sample) were functioning less well in terms of their mental health (P = 0.001) and also experienced significantly more disability in all areas of everyday life (P = 0.001). Differences in method of treatment for psoriasis did not significantly affect scores on the psoriasis-specific (PDI; PLSI) or generic (SF-36) quality of life measures. A multiple regression analysis demonstrated that stress resulting from anticipating other people's reactions to their psoriasis contributed more to the variance in patients' disability in everyday life than any other medical or health status variable. The results support the importance of assessing the effects of stress in patients' adjustment to their condition and may indicate a role for adjunctive psychological stress management training for a significant number of patients with psoriasis.     

Subclavian steal syndrome: existence, clinical features, diagnosis and management

Cytogenetic studies of ovarian sex cord stromal cell tumors, although limited in number, have found trisomy 12 to be a recurring abnormality, especially in fibromas and granulosa cell tumors (GCTs). However, recent fluorescence in situ hybridization (FISH) studies have failed to confirm a high prevalence of trisomy 12 in GCTs. We describe the karyotypic findings in one adult and one juvenile GCT. Only the juvenile GCT had an extra, abnormal chromosome 12, but both the adult and juvenile GCT had monosomy 22. In light of these findings and the data in the literature, we suggest that monosomy 22 may be important in the genesis of these relatively rare tumors.     

Gastric motor dysfunction in diabetic patients: pathogenesis, clinical manifestations, diagnosis and treatment

Recent reports provide evidence, that the disordered gastric motoric function in diabetic patients depends on glycaemia and that it can influence metabolic control. The etiopathogenesis of disordered gastric motoric function is poorly understood and is mainly hypothetical. The diagnosis of this dysfunction is difficult. It is recommended to evaluate of gastric motility, especially by scintigraphy, manometry and electrogastrography. In the treatment, it is important to remember about the good metabolic control of diabetes and use the gastrokinetic drugs in the combination or alternating therapy. In especially severe cases, surgical therapy is indicated. Lack of effective method of gastric motoric dysfunction treatment in diabetic patients suggest the necessity of further studies on new drugs, which would normalise the motoric abnormality and eliminate the symptoms.     

Dengue virus infection: epidemiology, pathogenesis, clinical presentation, diagnosis, and prevention

OBJECTIVES: To investigate whether the isolated urinary bladder spontaneously releases substance P (SP) or bradykinin (BK), which can act as potent mediators of pain and inflammation of the urinary bladder, and whether peptidase inhibitors enhance peptide release. MATERIALS AND METHODS: Urinary bladder segments (2 x 10 x 0.8-1 mm) were isolated from guinea pigs and studied in vitro; tissue contraction was assessed using force-displacement transducers and the release of peptides by specific enzyme immunoassays. RESULTS: In the absence of any exogenous agonists, the inhibition of neutral endopeptidase and angiotensin-converting enzyme by phosphoramidon and captopril, respectively, increased the frequency and magnitude of spontaneous motility of isolated bladder strips. Phosphoramidon increased the net release of SP-like immunoreactivity (SP-LI) and captopril increased the net release of SP-LI and BK-LI, concomitant with contraction. Peptide-LI was recovered primarily from bladder mucosa and to a lesser degree from detrusor smooth muscle. Similarly, peptidase inhibitors primarily affected the bladder mucosa; phosphoramidon induced a fourfold increase in SP-LI and captopril induced a significant increase of SP-LI and BK-LI from the mucosa. Tissues contracted in response to peptidase inhibitors in the presence of atropine and indomethacin, but contraction was reduced significantly by in vitro capsaicin desensitization or removal of bladder mucosa. BK stimulated SP-LI release from mucosa but not detrusor. SP stimulated increased BK-LI release from mucosa and detrusor. CONCLUSIONS: These findings indicate the basal release of peptide-like immunoreactivity by isolated bladder and further support the concept that peptidases located in the bladder mucosa are important in terminating the effects of endogenous peptides.     

Prospective research on fetal cholelithiasis: incidence, predisposing conditions, echographic diagnosis, and clinical features

The Authors report a prospective study on fetal cholelithiasis, analyzing its differences with the more widely known cholelithiasis of paediatric age. The study shows that the number of cases diagnosed by ultrasonography is higher than expected (0.39%, 3 cases on 764 pregnancies). The Authors could find no correlation between fetal cholelithiasis and any maternal, obstetrical and fetal factor. They have focused attention on sonographic imaging showing clinical-instrumental correlation between echogenic material, clinical features and their evolution. The study confirms that the most common evolution results in spontaneous resolution of fetal endocholecystic pathological images. Finally, wide review of the international literature is reported on the rare, but possible clinical manifestations and their complications.     

Sudanese mucosal leishmaniasis: epidemiology, clinical features, diagnosis, immune responses and treatment

The epidemiology, clinical features, pathology, immune responses, diagnosis and treatment of 14 patients with mucosal leishmaniasis in the Sudan are described. The condition occurred mainly in adult males, particularly in certain closely related tribes from the western Sudan. It affected the mucosa of the upper respiratory tract and/or the oral mucosa and sometimes followed treated kala azar. The parasites were sometimes confined to the mucosa, sometimes spread to the lymph nodes, and rarely infected the bone marrow and spleen. One of the 2 patients with both visceral and mucosal leishmaniasis differed from classical kala azar cases; his infection was longer lasting, he was leishmanin positive, and his peripheral mononuclear cells proliferated in response to leishmanial antigens. Mucosal leishmaniasis following treated kala azar is a similar phenomenon to post-kala azar dermal leishmaniasis and post-kala azar uveitis. Post-kala azar mucosal leishmaniasis can therefore be added to the other post-kala azar leishmanial infections. Using the polymerase chain reaction, Southern blot analysis with specific probes, and isoenzyme characterization, the causative parasite was identified as Leishmania donovani in 4 patients and as L. major in one. Unlike American mucocutaneous leishmaniasis, mucosal leishmaniasis in the Sudan was not preceded or accompanied by cutaneous lesions and the response to pentavalent antimony or ketoconazole was good.     

Osteoporosis: pathogenesis, diagnosis, and treatment in older adults

Prevention of osteoporosis is a major health concern. Bone loss occurs throughout life in both women and men due to calcium deficiency, hormonal deficiency, and changes in bone formation. The diagnosis of osteoporosis can now be made prior to fragility fracture, allowing for prevention as well as treatment. Criteria for diagnosis of osteoporosis are reviewed, and a plan for the evaluation of secondary causes of osteoporosis is discussed. Also reviewed are prevention and treatment options such as exercise, calcium supplementation, hormone replacement, and new and investigational drugs.     

Investigations on clinical diagnosis and pathogenesis of otitis media et interna in swine

A total of 25 pigs with a head tilt as clinical sign of otitis media et interna were examined. The majority were weaner-pigs with respiratory tract disorders. In lateral and ventrodorsal radiographic views of the bulla tympanica, there was an increased opacity, often accompanied by marginal destruction or thickening of the bulla wall. The findings confirmed the clinical diagnosis in each affected pig. In the case of leptomeningitis an examination of the cerebrospinal fluid revealed a drastic increase in the cell count. In 20 of 23 microbiologically examined empyemic bullae a polyinfection was seen. The results indicate that the route of infection of the middle ear is by the auditory tube. Mange on the other hand plays only a minor role in the pathogenesis of otitis media.     

Autonomic paroxysms: their pathogenesis, diagnosis and treatment

In the article the rules about etiology and pathogenesis of vegetative paroxysms are stated based on careful analysis of the publications of researches as Russian, so foreign authors, and also own experimental and clinical supervision. During experimental and clinical researches the modern methods were used, enabling to estimate from positions of the system analysis different parts of pathogenesis of vegetative paroxysms, and also to offer ways of differential diagnostics of the various forms of disease. The application of some new preparations and direction of therapy of vegetative paroxysms are substantiated, and also the various circuits of treatment of the patients with distinguishing forms of given pathology are motivated.     

Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis

This paper reviews current knowledge of childhood sarcoidosis with regard to the epidemiology in Danes, clinical presentation, diagnostic procedures, treatment and prognosis. Sarcoidosis is a granulomatous disease of unknown aetiology, with multiorgan involvement. The diagnosis is confirmed by the demonstration of epitheloid cell granulomas in tissue biopsy specimens. During the period 1980-92, three cases of childhood sarcoidosis were recorded in Copenhagen County, which has a total population of 610,000. The approximate incidence of clinically recognized sarcoidosis in Danish children younger than 15 y of age was 0.22-0.27/100,000 children per year, corresponding to approximately three new cases in Denmark each year. The true incidence is unknown, since the disease is often asymptomatic and resolves without a clinical diagnosis being made. In children younger than 5 y of age, the disease is characterized by involvement of skin, eyes and joints, whereas in older children involvement of lungs, lymph nodes and eyes predominate. The mainstay of treatment consists of oral corticosteroids. The risk/benefit ratio of using long-term corticosteroids needs to be evaluated in each individual patient. Some patients may benefit from additional therapy with methotrexate. The long-term prognosis is not well established, but it seems to be poorer in children younger than 5 y. Older children appear to have as favourable a prognosis as young adults.     

A reliable endocrine test with human menopausal gonadotropins for diagnosis of true hermaphroditism in early infancy

In true hermaphroditism diverse phenotypes and karyotypes are found; there are no distinctive laboratory features that can distinguish it from other intersex disorders, thus the diagnosis is made by the histological findings. Existence of Leydig cells is demonstrated by testosterone levels above the female range; however, presence of ovarian tissue cannot be ascertained because of the absence of a reliable functional test. Unless appropriate biopsies are performed or the whole gonad is removed, there is a risk of not diagnosing true hermaphroditism. To find a reliable test that can differentiate patients with true hermaphroditism from those with other intersex disorders, we investigated the estradiol (E2) response to human menopausal gonadotropins (hMG) in infants with genital ambiguity. These results were correlated with the histological findings. Eleven infants with genital ambiguity and four with a high scrotal testis were stimulated every 12 h with 2 IU/kg hMG. If E2 rose above 80 pg/mL (cut-off point), the test was discontinued; if after 7 days E2 remained below 80 pg/mL, the hMG dose was doubled and stimulation extended for 7 additional days. In five patients in whom true hermaphroditism was later histologically demonstrated, E2 rose above 80 pg/mL. In two of them, ovarian tissue was removed and hMG stimulation repeated; no response above our cut-off point was observed during the second test. The maximal E2 response to hMG in the remaining 10 individuals was 43 pg/mL; after laparotomy or gonadal biopsies no ovarian tissue was found. The hMG stimulation test can be considered a reliable and safe dynamic procedure for demonstrating the presence or absence of ovarian tissue in infants with genital ambiguity.     

Female idiopathic urethra tumor: clinical features diagnosis and treatment]

Female idiopathic urethra tumors are reported rarely. We treated fourteen patients patients, and described the clinical manifestations and local features of the tumors, urethra liomyoma, and fibropolypus. We found that there are close relations among tumor stage, prognosis and pathological classification. The main diagnostic methods include vaginal touch, urethroscopy, B-ultrasound exam and biopsy. The diagnostic procedure recommended is tumor location-quality-stage or a three-step method. The main treatment is operation, however, the operative mode should depend on tumor stage and patient's life quality after operation.     

Familial lipoprotein lipase deficiency in infancy: clinical, biochemical, and molecular study

Although almost all of the energy contained within the ultraviolet (UV) wavelengths of solar radiation is absorbed within the epidermis and upper layers of the dermis, UV irradiation can suppress the immune response to antigens introduced at distant, non-irradiated body sites. The production of immune modulatory cytokines, such as interleukin-10 (IL-10), by UV-irradiated keratinocytes and its effect on T helper type 1 (Th1)/Th2-cell balance are thought to play a major role in the induction of systemic immune suppression. Because it is suggested that costimulatory molecules, such as CD80 and CD86, differentially stimulate Th1 and Th2 cells we wished to investigate the role of these costimulatory molecules in the activation of immune suppression. We injected UV-irradiated mice with monoclonal antibodies to CD80 and CD86 and asked what effect, if any, this would have on UV-induced immune suppression. Anti-CD86, but not anti-CD80 or control rat IgG, blocked UV-induced immune suppression. Moreover, monoclonal anti-CD86 blocked the induction of suppressor T cells normally found in the spleens of the UV-irradiated mice. Monoclonal anti-CD86 also reversed the UV-induced impairment of systemic antigen-presenting cell function. IL-10 was detectable in the serum of UV-irradiated mice as compared with normal controls, and injecting UV-irradiated mice with anti-CD86, but not anti-CD80 or control rat IgG, blocked the secretion of IL-10 into the serum. We propose that UV exposure favours costimulation by CD86, which enhances the production of serum IL-10, thus suppressing Th1-cell-mediated immune reactions.     

Early-onset hyperthyroidism with muscle involvement: a report on a patient

A case of Graves' disease in a 2 years child old is reported. He presented tachycardia, loss of weight, anxiety and gait abnormality. The serum free T3 and T4 and thyrotropin receptor antibodies values were elevated, TSH was not measurable. The EMG was abnormal with short duration motor unit potentials.