Infective endocarditis due to Leptotrichia buccalis: a case report.

A patient with Down''s syndrome presented with infective endocarditis due to Leptotrichia buccalis. The source of the infection was not detected, but the predisposing factor was a complex cardiac malformation. The disease followed a subacute course, had a number of immunologic manifestations and was successfully treated with a 28-day course of penicillin G, given intravenously. L. buccalis has never been reported before as a cause of endocarditis.     

The immunological hazard of Cushing's syndrome.

A 24-year-old woman was found to have cryptococcal meningitis and Cushing''s syndrome due to an adrenal adenoma. Her meningitis was successfully arrested with fluorouracil. Treatment with metyrapone decreased her cortisol production and produced clinical remission of Cushing''s syndrome. On admission her peripheral T lymphocytes were few and hyporeactive. When the overproduction of cortisol ceased the numbers of T lymphocytes and their reactivity returned to normal and she developed in-vitro lymphocyte responsiveness to the cryptococci.     

免疫诱导对胰岛素抵抗患者干燥综合征的影响

目的:分析免疫诱导治疗胰岛素抵抗合并干燥综合症的临床效果。方法:选取2013年1月到2014年12月内在我院接受治疗的156例胰岛素抵抗合并干燥综合症患者并随机分为对照组和研究组,对照组患者采用药物治疗,研究组患者在对照组患者基础上诱导其发生自身免疫反应治疗,分别对两组患者治疗后检测两组患者的FT3、FT4、TSH、血清IL-21水平并统计干燥综合症的治愈例数。结果:研究组患者的FT3、FT4以及血清IL-21水平明显高于对照组,TSH显著低于对照组,差异均具有统计学意义(P0.05);研究组干燥综合症的治愈率为53.85%,显著高于对照组的34.62%,差异具有统计学意义(P0.05)。结论:免疫诱导治疗胰岛素抵抗合并干燥综合症能够获得较好的治疗效果。     

Physicians as 'double agents.

The opportunities and stresses faced by today''s adolescent women have the potential to affect their health, either directly or through interaction with biologic and psychosocial events. Recently achieved entry into the world of endurance and competitive sports; smoking, drug, and alcohol use; the toxic shock syndrome; and the acquired immunodeficiency syndrome threaten the health of young women today. Physicians who care for adolescent women in the 1980s and in the future will require special skills to do so successfully.     

Toxic Epidermal Necrolysis: The Scalded Skin Syndrome

Toxic epidermal necrolysis is a condition that may occur in all age groups and resembles skin scalding. It presents as a generalized, markedly tender erythema and rapidly progresses to bullous desquamation of the superficial epidermal layers and systemic toxicity.The condition was first described in 1956. A review of the recent literature suggests that the incidence of the syndrome is increasing or it is being recognized more frequently. Its etiology has not been established, but it may represent unusual hypersensitivity to drug, bacterial, viral or immunization factors. The mortality rate in reported cases is approximately 30% and young children and debilitated adults are at greatest risk.Recently, two children with this condition were successfully treated at the Montreal Children''s Hospital, using protective isolation, intensive nursing care, intravenous antibiotics and hydrocortisone hemisuccinate. Early use of corticosteroids may inhibit the progression of epidermal necrolysis and improve the prognosis.     

Nephrotic Syndrome in the Elderly

Though the nephrotic syndrome is generally believed to be uncommon in the elderly, patients aged 60 years or more accounted for 25 out of 100 consecutive adult cases. Six (24%) of these had the minimal change lesion, compared with 16% of the younger adults. The incidence of membranous glomerulonephritis was similar in the two age groups, but proliferative glomerulonephritis was more common in the younger (29%) than in the older group (16%). Amyloidosis did not have a higher incidence in the higher age group. Five of the elderly patients with minimal change lesion were treated with prednisone—in four a complete remission from the nephrotic syndrome followed, while the fifth patient''s course is unknown.These results suggest that, when the patient''s other circumstances allow, the nephrotic syndrome in an elderly patient should be investigated and managed as in younger age groups.     

Transient myeloproliferative disorder in a newborn with down syndrome treated with rasburicase for the risk of development of tumor lysis syndrome: A case report

Introduction

Transient myeloproliferative disorder is a hematologic abnormality characterized by an uncontrolled proliferation of myeloblasts in peripheral blood and bone marrow that primarily affects newborns and babies with Down syndrome. Tumor lysis syndrome is rarely associated with transient myeloproliferative disorder.

Case presentation

Transient myeloproliferative disorder was diagnosed in a seven-day-old baby girl with Down syndrome, who was referred to our department due to hyperleukocytosis. Our patient developed tumor lysis syndrome, successfully treated with rasburicase, as a complication of transient myeloproliferative disorder resulting from rapid degradation of myeloid blasts after initiation of effective chemotherapy.

Conclusions

Tumor lysis syndrome is rarely reported as a complication of transient myeloproliferative disorder. To the best of our knowledge, this is the first case of a newborn with Down syndrome and transient myeloproliferative disorder treated with rasburicase for developing tumor lysis syndrome.

     

Cutaneous phaeohyphomycosis of the sole caused by Exophiala jeanselmei and its susceptibility to amphotericin B, 5-FC and ketaoconazole

Cutaneous phaeohyphomycosis due to E. jeanselmei developed on the sole of a 61 years old Japanese female who was receiving the corticoids therapy for nephrotic syndrome. Although the causative fungus was resistant to 5-fluorocytosine, amphotericin B and ketoconazole, the lesion was successfully treated by surgical excision.     

Pregnancy,prolactin, and pituitary tumours

Nine pregnancies are described in patients with pituitary tumours. All patients had definite radiological evidence of a pituitary tumour and no evidence of acromegaly or Cushing''s disease. In seven patients serum prolactin levels were estimated before pregnancy and found to be raised.Seven patients had been treated with pituitary implantation of yttrium-90. The remaining two developed complications of the tumour during pregnancy. One developed a bitemporal visual field defect in the second trimester which was successfully treated by emergency yttrium-90 implantation. The other developed diabetes insipidus in the third trimester which resolved spontaneously after delivery.Six patients were treated with drugs to achieve pregnancy. Four took bromocriptine to suppress raised prolactin levels, one was treated with human menopausal gonadotrophin, and one was treated with clomiphene.     

Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis

Sweet's syndrome (the eponym for acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis. Several hundreds cases of Sweet's syndrome have been published. Sweet's syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced. Classical Sweet's syndrome (CSS) usually presents in women between the age of 30 to 50 years, it is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. Approximately one-third of patients with CSS experience recurrence of the dermatosis. The malignancy-associated Sweet's syndrome (MASS) can occur as a paraneoplastic syndrome in patients with an established cancer or individuals whose Sweet's syndrome-related hematologic dyscrasia or solid tumor was previously undiscovered; MASS is most commonly related to acute myelogenous leukemia. The dermatosis can precede, follow, or appear concurrent with the diagnosis of the patient's cancer. Hence, MASS can be the cutaneous harbinger of either an undiagnosed visceral malignancy in a previously cancer-free individual or an unsuspected cancer recurrence in an oncology patient. Drug-induced Sweet's syndrome (DISS) most commonly occurs in patients who have been treated with granulocyte-colony stimulating factor, however, other medications may also be associated with DISS. The pathogenesis of Sweet's syndrome may be multifactorial and still remains to be definitively established. Clinical and laboratory evidence suggests that cytokines have an etiologic role. Systemic corticosteroids are the therapeutic gold standard for Sweet's syndrome. After initiation of treatment with systemic corticosteroids, there is a prompt response consisting of dramatic improvement of both the dermatosis-related symptoms and skin lesions. Topical application of high potency corticosteroids or intralesional corticosteroids may be efficacious for treating localized lesions. Other first-line oral systemic agents are potassium iodide and colchicine. Second-line oral systemic agents include indomethacin, clofazimine, cyclosporine, and dapsone. The symptoms and lesions of Sweet's syndrome may resolved spontaneously, without any therapeutic intervention; however, recurrence may follow either spontaneous remission or therapy-induced clinical resolution.     

氟西汀联合六味地黄丸对更年期综合征患者血清孕激素、叶酸及5-羟色 胺水平的影响

目的:探讨氟西汀联合六味地黄丸对更年期综合征患者抑郁症状及血清孕激素、叶酸及5-羟色胺水平的影响。方法:收集在我院就诊或住院治疗的80例更年期综合征患者,随机分为实验组和对照组,每组40例。对照组患者给予盐酸氟西汀治疗,实验组患者在对照组基础上给予六味地黄丸治疗。观察并比较两组患者治疗前后血清孕激素、叶酸及5-羟色胺水平的变化以及汉密尔顿抑郁量表(HAMD)评分。结果:与治疗前相比,两组患者治疗后的孕激素、叶酸以及5-羟色胺水平均显著升高(P0.05),HAMD评分水平明显下降(P0.05);与对照组相比,实验组患者的孕激素、叶酸以及5-羟色胺水平较高(P0.05),HAMD评分水平较低(P0.05)。结论:氟西汀联合六味地黄丸能够显著升高更年期综合征患者血清孕激素、叶酸及5-羟色胺水平,改善更年期综合征症状。     

Anaphylaxis to husband's seminal plasma and treatment by local desensitization

Hypersensitivity to human seminal fluid is rare but can be life threatening. We report a case of IgE-mediated anaphylaxis to seminal plasma that was diagnosed by skin prick tests and successfully treated by local desensitization. A 32-year-old woman suffering from angioedema and hypotension after exposure to semen was treated with epinephrine upon admission. Skin prick tests and immunoblotting for IgE binding components showed that she was sensitized to her husband's seminal plasma. Local desensitization, which persisted for six months, was achieved by intravaginal administration of serial dilutions of her husband's seminal plasma.     

A little help from my friends: caring for premature babies in a war zone

Background

Too much or too little milk production are common problems in a lactation consultant's practice. Whereas underproduction is widely discussed in the lactation literature, overabundant milk supply is not. In my practice I work with women who experience moderate to severe oversupply syndrome. In most cases the syndrome can be successfully treated with full removal of milk followed by unilateral breastfeeding ad lib with the same breast offered at every breastfeed in a certain time block ("block feeding").

Case presentations

Four cases of over-supply of breast milk are presented. The management and outcome of each case is described.

Conclusion

Overabundant milk supply is an often under-diagnosed condition in otherwise healthy lactating women. Full drainage and "block feeding" offer an adequate and userfriendly way to normalize milk production and treat symptoms in both mother and child.     

Prenatal and postnatal prevalence of Turner's syndrome: a registry study.

OBJECTIVE--To study prevalence of Turner''s syndrome in Denmark and to assess validity of prenatal diagnosis. DESIGN--Study of data on prenatal and postnatal Turner''s syndrome in Danish Cytogenetic Central Register. SUBJECTS--All registered Turner''s syndrome karyotypes (100 prenatal cases and 215 postnatal cases) during 1970-93. MAIN OUTCOME MEASURES--Prevalence of Turner''s syndrome karyotypes among prenatally tested fetuses and Turner''s syndrome among liveborn infants. RESULTS--Among infant girls, prevalence of Turner''s syndrome was 32/100,000. Among female fetuses tested by amniocentesis, prevalence of Turner''s syndrome karyotypes was 176/100,000 (relative risk of syndrome, 6.74 compared with prevalence among untested pregnancies). Among female fetuses tested by chorion villus sampling, prevalence of syndrome karyotypes was 392/100,000 (relative risk, 16.8). We excluded prenatal tests referred because of results of ultrasound scanning: among fetuses tested by amniocentesis revised relative risk was 5.68, while revised relative risk among fetuses tested by chorion villus sampling was 13.3. For 29 fetuses with prenatal diagnosis of possible Turner''s syndrome, pregnancy was allowed to continue and 24 children were live born. Thirteen of these children were karyotyped postnatally, and diagnosis of Turner''s syndrome had to be revised for eight, seven being normal girls and one boy. This gives tentative predictive value of amniocentesis in diagnosing Turner''s syndrome of between 21% and 67%. There was no significant relation between mother''s age and risk of Turner''s syndrome. CONCLUSIONS--Discrepancy between prenatal and postnatal prevalence of Turner''s syndrome challenges specificity of prenatal examination in diagnosing Turner''s syndrome.     

Plasma exchange in the long-term management of Waldenstr?m's macroglobulinemia.

Two patients with Waldenström''s macroglobulinemia (WM), which had become resistant to cytotoxic drugs, were treated for features of the hyperviscosity syndrome by repeated plasma exchange with the continuous-flow blood-cell separator over periods of 36 and 28 months, respectively. After four initial weekly plasma exchanges the procedure was carried out every 4 to 6 weeks and both patients tolerated it well. Relative viscosity of the serum was maintained within the normal range in one patient, and both patients remained free of symptoms of the hyperviscosity syndrome. The results suggest that treatment of WM by long-term "maintenance" plasma exchange alone should be considered in any patient with complications due to chemotherapy or whose disease fails to respond to chemotherapy.     

Perineal repair of a full‐thickness rectal prolapse in a wild Sumatran orangutan (Pongo abelii)

A Delorme's procedure perineal surgical repair was performed in a wild adult male Sumatran orangutan (Pongo abelii) with a chronic persistent rectal prolapse that had been unsuccessfully treated by 6 previous surgeries. The rectal prolapse did not recur, and the orangutan was successfully released to the wild, 6 weeks later.     

The Restless Leg Syndrome

The author reviews and provides examples of the restless leg syndrome (Ekbom''s syndrome), emphasizing the frequency of the disorder and its distressing symptoms. The syndrome is characterized by a creeping, crawling sensation in the lower legs, usually at night. It is accompanied by an irresistible urge to move the legs, and this movement eventually relieves the symptoms. It is found in 5% of the general population, with a higher incidence in pregnant women and people with anemia, but no vascular, neurological or other abnormalities are found on examination. Patients are treated empirically with vasodilators or intravenous iron. The etiology is unknown.     

Subclavian-axillary vein thrombosis: successful treatment with streptokinase.

Proximal vein thrombosis has been associated with residual disability from the postphlebetic syndrome in patients receiving symptomatic therapy or heparin and sodium warfarin. This paper describes a man with painless swelling of the right arm secondary to subclavian-axillary vein thrombosis that was successfully treated with streptokinase and conventional anticoagulant therapy.     

Quinidine: an “Endangered Species” Drug Appropriate for Management of Electrical Storm in Brugada Syndrome

Brugada syndrome is an inherited channelopathy associated with an increased risk of syncope and sudden cardiac death. In rare cases it can be manifested with electrical storm. We report two cases of Brugada syndrome that presented with electrical storm and were treated successfully with oral quinidine, an "endangered species" drug.     

ACUTE SUPERIOR VENA CAVA OBSTRUCTION WITH INTRACAVITARY THROMBOSIS: A COMPLICATION OF CENTRAL VENOUS CATHETERIZATION

This report describes a patient who developed acute superior vena cava syndrome, intracavitary right atrial thrombus, and recurrent pulmonary emboli 10 days after central venous catheterization. The patient was treated successfully by emergency thoracotomy.