Peculiar Clinical Course Research Articles

Obsessive-compulsive Disorder with Comorbid Bipolar Disorders: Clinical Features and Treatment Implications.

Obsessive-compulsive disorder (OCD) symptoms within the context of a bipolar disorder (BD) have been described since the 19th century. Interestingly, the existence of a relevant overlap between the aforementioned psychiatric syndromes has been confirmed by a number of recent epidemiological and family studies. The aim of the present paper is to review the clinical features and the therapeutic implications of the OCD-BD comorbidity. In the last two decades, the frequent association between OCD and BD has been earning a growing interest given its relevant nosological and therapeutic implications. Usually patients suffering from OCD-BD comorbidity show a peculiar clinical course, characterized by a larger number of concomitant depressive episodes and episodic course. In these cases, the treatment with antidepressants is more likely to elicit hypomanic or manic switches, while mood stabilizers significantly improve the overall clinical picture. Moreover, OCD-BD patients are frequently comorbid with a number of other psychiatric disorders, in particular anxiety disorders, social phobia, and different substance abuses, such as alcohol, nicotine, caffeine and sedatives. BD-OCD comorbidity needs further investigations in order to provide more solid evidences to give patients a more precise clinical diagnosis and a more targeted therapeutic approach.

Recurrent glomerular disease after kidney transplantation: an update of selected areas and the impact of protocol biopsy.

Recurrence of native kidney disease following kidney transplantation affects between 10% and 20% of patients, and accounts for up to 8% of graft failures. In a considerable number of recipients with transplant glomerulopathy, it is impossible to distinguish between recurrent and de novo types. An accurate estimate of the incidence of recurrence is difficult due to limitations in the diagnosis of recurrent glomerulonephritis. De novo glomerular lesions may be misclassified if histological confirmation of the patient's native kidney disease is lacking. Asymptomatic histological recurrence in renal allografts may be missed if protocol biopsies are not available. Studies based on protocol biopsy are pivotal to accurately estimate the incidence of recurrence. Many factors are known to influence recurrence of kidney disease after transplantation, including the type and severity of the original disease, age at onset, interval from onset to end-stage renal disease, and clinical course of the previous transplantation. Early recognition of recurrence is possible in several glomerular diseases. Factors such as the existence of circulating permeability factors, circulating urokinase receptor and anti-phospholipase A2 receptor antibody, as well as disorders of complement regulatory proteins like factor I mutation and factor H mutation factors are expected to be useful predictors of recurrence. Peculiar clinical course of atypical haemolytic uremic syndrome after kidney transplantation is an informative sign of recurrent glomerular disease. These factors play pivotal roles in the development of recurrence of certain types of glomerulopathies. Understanding the pathogenesis of recurrent glomerulonephritis is critical to optimize prevention as well as treat individual cases of recurrent glomerulonephritis. Subclinical recurrence of IgA nephropathy after kidney transplantation is well recognized. Only protocol biopsies of clinically silent recipient can provide the accurate prevalence of recurrent IgA nephropathy. The study of recurrent glomerulonephritis will contribute not only to improving long-term graft survival, but also to clarifying the pathogenesis of glomerulonephritis. Protocol biopsy is one the most effective methods for elucidating the pathogenesis of recurrent glomerulonephritis.

Unilateral aquagenic wrinkling of the palms with a peculiar clinical course

ejd.2012.1792 Auteur(s) : Chieko Ibusuki, Masahiro Oka oka@med.kobe-u.ac.jp, Atsushi Fukunaga, Makoto Kunisada, Chikako Nishigori Kobe University School of Medicine, Department of Dermatology, 7-5-1 Kusunoki-cho Chuo-ku, Kobe 650-0017, Japan Aquagenic wrinkling of the palms (AWP) is a rare condition characterized by the rapid development of transient, edematous, white to translucent papules and plaques on the palms after brief exposure to water [1, 2]. To date, more than 30 cases have [...]

Janus kinase (JAK) 2 V617F mutation as the cause of primary thrombocythemia in acromegaly with severe visceromegaly and divergence between growth hormone and insulin-like growth factor-1 concentrations during the follow-up: causal or casual association?

ObjectiveAn increased prevalence of hematological abnormalities is reported in acromegaly, but to date no reports about the presence of the Janus Kinase (JAK) 2 mutation in acromegalic patients have been described. DesignWe report the complex clinical presentation of the unique case, never described, of acromegaly due to GH-secreting pituitary adenoma associated with JAK2 V617F mutation. ResultsThe patient shows primary thrombocythemia and myelofibrosis, due to JAK2 V617F mutation, severe visceromegaly and a peculiar clinical course of the disease characterized by discrepant values of GH and IGF-1 during somatostatin analog (SA) treatment despite a significant reduction in pituitary adenoma size and therapeutic resistance both to SA and pegvisomant. ConclusionsThe presence of JAK2 V617F mutation is a cause of primary thrombocythemia and myelofibrosis in acromegaly. In this patient, a peculiar clinical course of acromegaly was observed, with the difficulty in controlling the disease. More data, on a larger cohort of patients, could clarify whether JAK2 V617F mutation has a serious impact on the clinical features and course of acromegaly.

Hyperinsulinemic hypoglycemia associated with ectopic Cushing’s syndrome due to a pancreatic endocrine tumor in a Type 2 diabetes mellitus patient: Clinical implications of a rare association

The coexistence of insulin and ACTH hypersecretion in the same patient is extremely rare. A diabetic patient with a pancreatic endocrine tumor (PET) co-secreting insulin and ACTH is even rarer and has never been described. The combination of these two endocrine syndromes results in a peculiar clinical picture. To determine the cause of glycemic variations in a patient with previously stable diabetes mellitus. This is a clinical case report from the Endocrinology Unit of Aosta Hospital and Internal Medicine and Surgical Unit of Verona University. A 69-yr-old diabetic patient was hospitalized for recurrent severe hypoglycemic events persistent after withdrawal of anti-diabetic drugs. The causes of hypoglycemia and subsequent resumption of hyperglycemia were investigated. An insulin-secreting PET was diagnosed. Diazoxide and octreotide therapy initially was able to control hypoglycemic symptoms, then, a Cushing's syndrome occurred resulting in worsening of diabetes control. ACTH was found to be released by the PET previously diagnosed as an insulin-secreting tumor. The tumor was removed and the histology was consistent with a well differentiated endocrine carcinoma. After surgery, adrenal function was normal and insulin therapy was again necessary to control diabetes. A single PET may be responsible for both a hyperinsulinemic and a Cushing's syndrome. When this rare association occurs, each of the two syndromes may affect the other resulting in a peculiar clinical course. Finally, an insulin-secreting PET has to be kept in mind as a rare cause of hypoglycemia in diabetic patients.

自己誘発発作を主症状とし特異な経過を示した頭部外傷後てんかんの男児例

自己誘発発作を主症状とし特異な経過を示した頭部外傷後てんかんの男児例

Fallo hepático agudo por el virus de la hepatitis C en el puerperio

Hepatitis C virus (HCV) infection is a global problem in our health systems. The prevalence of infected patients in general population is about 2%. In the subgroup of pregnant woman the percentage of infected patients ranges from 1-3%. Several studies have described the relationship between pregnancy and chronic HCV infection, and in most cases serum transaminases levels return to normal values while HCV-RNA levels increase during the second and third trimester. The influence of maternal hormones after delivery in the natural history of HCV infection has not been sufficiently studied and consequently remains controversial. This case report shows the peculiar clinical course of a patient with chronic HCV infection in the puerperium.

Erythema annulare centrifugum: report of a case with neonatal onset

Darier's erythema annulare centrifugum (EAC) is often associated with infectious, autoimmune or neoplastic disease, nevertheless, most cases of EAC remain unexplained. We report a case of EAC with neonatal onset and a clinical course of over 20 years. The patient presented from the 6th day of life with erythematous papules that enlarged centrifugally to form figurate, annular or policyclic plaques involving the entire cutaneous surface. The clinical picture had a chronic course, disappearing only during unrelated febrile episodes. Histologic examination revealed a normal epidermis and a dense perivascular lymphohistiocytic infiltrate and numerous eosinophils in the superficial and reticular dermis. Today the patient is 24 years old and his lesions are still unchanged. Recently, clinical evaluation revealed a diffuse sderodermic induration. A second biopsy showed a diffuse thickening of collagen fibers in the dermis. To our knowledge, this is the second report of EAC with neonatal onset and such a long and peculiar clinical course.

A CASE OF ABDOMINAL AORTIC ANEURYSM COMPLICATED BY PERFORATED SIGMOID COLON DIVERTICULITIS

A 64-year-old man was admitted to another hospital because of weight loss and constipation. He was diagnosed as perforated sigmoid colon diverticulitis with abscess formation and an incidental abdominal aortic aneurysm (AAA). After four-month medical treatment of the abscess, he was referred to our institution for the treatment of AAA. Since the patient was asymptomatic and the abscess was apparently contained and inactive, routine aneurysm replacement was performed. After the operation, however, the patient developed a septic fever and the abscess was found to be enlarged, which required operative drainage. Thereafter the patient was with transverse colostomy, fistulectomy with partial resection of the sigmoid colon and the urinary bladder, and repair of the colostomy. This case presented a peculiar clinical course that routine aneurysm replacement for AAA performed under our judgment that the abscess was inactivated with medical treatment came out subsequent formation of colovesical fistula.

Adenoid cystic carcinoma of the head and neck.

Adenoid cystic carcinoma of the head and neck is relatively rare and is characterized by slow evolution, multiple recurrences, protracted clinical course, and late distant metastasis. This article presents its peculiar clinical course, response to therapy, and long-term treatment results by analyzing 67 cases treated from 1979 to 1991 at the Seoul (Korea) National University Hospital. The most common primary site was the parotid gland in the major salivary glands and the nose and paranasal sinuses in the minor salivary glands. The local control rate was 71.1% at 5 years and 44.3% at 8 years. Late-occurring distant metastases did not allow a plateau in survival curves, even after 8 years. Our study also revealed that surgery combined with postoperative radiotherapy could yield better local control.

CD8+ Cutaneous T-cell Lymphoma With Pagetoid Epidermotropism and Angiocentric and Angiodestructive Infiltration

A 68-year-old woman had a peculiar clinical course of cutaneous lymphoma. She first developed nonpuritic erythematous macules with fine scales followed by development of erythematous infiltrated plaques. The clinical course could be interpreted as that of mycosis fungoides. Histologically, the lesions showed pagetoid infiltration of atypical lymphoid cells. Suddenly, with high fever, numerous purpuric, ulcerated, or crusted plaques with underlying infiltration or nodules began to appear on most areas of the patient's body. Biopsy specimens of the lesions revealed angiocentric and angiodestructive infiltration by neoplastic T cells and marked epidermotropism of these cells. These atypical cells immunohistochemically had CD8+ surface phenotype. The patient died of respiratory insufficiency due to acute pulmonary infiltration. Autopsy demonstrated angiocentric and angiodestructive lymphomatous infiltration in the lung.

A new approach to development of live vaccine against tick-borne encephalitis.

SUMMARYInvestigations of the biological properties of tick-borne encephalitis viruses immediately after isolation from naturally infected Ixodes persulcatus from Western Siberia has revealed their different pathogenicity for monkeys and mice. Strains highly pathogenic for mice on extraneural inoculation and for rhesus monkeys on intracerebral inoculation have been isolated, but also strains with a low extraneural pathogenicity for mice and a complete non-pathogenicity or a mild pathogenicity for monkeys on intracerebral inoculation.The viruses have been classified into three main groups by their virulence for mice and monkeys, and their ability to grow in tissue culture at 40° C. and the findings are summarized in Table 6. Group I included viruses highly virulent for mice by any route of inoculation, which caused fatal paralytic disease in monkeys, and which grew well in chick embryo culture at 40° C.Group II strains also had a high virulence for mice, but in monkeys caused milder disease with a peculiar clinical course indicative of primary damage to the cerebellum. About half of the infected monkeys recovered completely or with mild sequelae (ataxia, pareses of the limbs). This group grew moderately well in chick embryo cultures at 40° C. Groups I and III differed essentially in their virulence for mice by subcutaneous inoculation, and virulence for monkeys decreased distinctly with transition from group I to group III. Group III strains showed a lower reproduction rate at 40° C. In chick embryo culture than group I. Groups I and II showed good correlation between extraneural pathogenicity for mice, pathogenicity for monkeys by IC inoculation, and growth rate in chick embryo cultures at 40° C.Group I strains were isolated chiefly from foci with a high TBE morbidity and the strains of groups II and III in foci with sporadic cases. It is, however, highly probable that in any tick-borne encephalitis focus several variant strains of differing virulence exist.Recent experience of vaccinating volunteers with naturally attenuated group III strains suggests that safe and effective live vaccines can be developed against tick-borne encephalitis. Vaccines against many other arbovirus diseases can probably also be derived from naturally occurring strains of virus isolated from arthropods in areas of low disease but high infection rate. This approach looks more promising than the usual approach of attempting to develop attenuated strains from highly virulent strains.

ATYPICAL PYOGENIC GRANULOMA; A CASE OF BENIGN HEMANGIOENDOTHELIOSIS.

A case is described of atypical vascular lesions occurring successively on the face. The differential diagnostic possibilities are discussed in connection with its peculiar clinical course and unusual histology. The presence of large endothelial cells in histologic sections supports the idea that pyogenic granuloma may be primarily of a vascular origin rather than a reactive granuloma.

PLASMACYTOMA OF THE NOSE AND NASOPHARYNX

Plasmacytomas of the nose and nasopharynx are not frequently encountered. Ewing¹said that considering the comparative frequency and peculiar clinical course of plasma cell tumors of the mucous membranes and lymph nodes, they have received inadequate attention. The literature does not contain many reports of cases of plasmacytoma of the nasal mucosa or nasopharynx. Claiborn and Ferris,²in 1931, found only twelve cases in a review of the literature, to which they added two cases of their own. In the last few years, reports of several more cases have been added. Geschickter and Copeland,³in a review of the literature on multiple myeloma, reported rare metastases to the tonsil, but none to the nose and nasopharynx. My purpose in this paper is to report two cases of plasmacytoma that have occurred recently in the routine pathologic work at the Massachusetts Eye and Ear Infirmary; one was a

PLASMA CELL TUMORS OF THE NASAL AND NASOPHARYNGEAL MUCOSA

Most of the textbooks on pathology make little if any mention of plasma cell tumors except those occurring in the bone marrow, the plasma cell myelomas. Ewing, 1 however, in his work on neoplastic diseases, discussed extramedullary plasma cell tumors, and stated that in a rather numerous group of tumors of the mucous membranes and lymph nodes the growth is composed exclusively of plasma cells. He said: Considering their comparative frequency and rather peculiar clinical course, they have received inadequate attention. The growths occur chiefly in the nasopharynx, alveolar borders, tongue, lips, and cervical lymph nodes, but they have been observed in many other regions. The course is relatively slow, but they may recur after extirpation and become associated with a chronic cachexia. They are commonly classed as lymphosarcoma, but being, as a rule, benign processes with very indistinct neoplastic properties, it is highly important that they should be separated