Early development of multiple epithelial neoplasms in Netherton syndrome

We report a case of Netherton syndrome manifested as congenital ichthyosiform erythroderma, trichorrhexis invaginata and atopy, who in early adulthood developed multiple, aggressive epithelial neoplasms in sun-exposed areas of the skin, in areas with papillomatous skin hyperplasia and at the left parotid region. The occurrence of cutaneous neoplasia has been reported in syndromes with congenital ichthyosis and suggests that the underlying genetic defects may cause the development of cancer in prone patients.     

High frequencies of antibody responses to CD4 induced epitopes in HIV infected patients started on HAART during acute infection

For many years only two human monoclonal antibodies (HMAbs) recognizing the CD4 induced (CD4i) epitopes of HIV-1 gp120 existed. Although a number of new CD4i HMAbs have been published recently, we have noted that in most attempts to produce HMAbs using EBV transformation a majority of antibodies produced in culture are lost within a few weeks. To determine what kinds of antibodies are made in these cultures we devised a semiquantitative culture to assess the frequency of B cells capable of producing antibodies and a microcompetition assay to determine what kinds of antibodies were made. Our results show that in three patients started on HAART during acute infection the most frequently produced antibodies binding to gp120 were directed against the CD4i epitopes. Our observations suggest that CD4i epitopes are much more immunogenic than had been previously appreciated. It is possible that envelope glycoproteins shed from virions and perhaps complexed with CD4 are responsible for eliciting these antibodies. The preservation of well regulated immune responses in these patients, together with repeated exposure to viral antigens (i.e. env), may explain the presence of larger than usual numbers of env-specific B cells that could be detected in EBV transformed cultures.     

Epidemiological differences for cutaneous melanoma in a relatively dark-skinned Caucasian population with chronic sun exposure

The aim of this study was to reveal differences in the epidemiology and to identify significant risk factors for cutaneous melanoma (CM) in a relatively dark-skinned, chronically sun-exposed Caucasian population. This group is considered to have a low risk for this tumour. One hundred and ten newly diagnosed patients with primary CM and 110 age- and gender-matched controls, all of Cretan origin, were interviewed and underwent a complete skin examination. Solar keratoses odds ratio (OR) 6.2 and lentigines (OR 2.2), common and atypical naevi (OR 5.4 and 3.0, respectively), blonde or red hair colour (OR 3.1), skin phototypes I/II (OR 1.8), as well as total sun exposure (weeks per year) (OR 1.03), were all significantly associated with CM risk in a multivariate logistic regression analysis. In the relatively dark-skinned Cretan population, sun exposure indices represent the most important risk markers for CM which contrasts with data from fair-skinned Caucasian populations where melanocytic naevi are the main risk factors.     

Prevalence and pathogenesis of hypokalemia in patients on chronic peritoneal dialysis: One center's experience and review of the literature

International Urology and Nephrology -     

A type 2A protein phosphatase gene from Aspergillus nidulans is involved in hyphal morphogenesis

A PCR-based approach, using degenerate oligonucleotide primers, was used to isolate fragments of two genes encoding type 2A protein phosphatases from the filamentous fungus, Aspergillus nidulans. The complete genomic sequence of one of these genes, pphA, was isolated and characterised. The pphA gene was predicted to encode a 329-residue protein which is about 85% identical to mammalian protein phosphatase 2A. Ectopic expression of the wild-type pphA ⁺ gene slightly inhibited growth in some transformants; but a mutant form of pphA, in which R259 was mutated to Q, led to slow growth, delayed germ tube emergence and mitotic defects at low temperature. These results indicate that the pphA ⁺ gene plays an important role in hyphal growth. Received: 18 October 2000 / Accepted: 25 October 2000     

Cutaneous alternariosis in a patient with idiopathic pulmonary fibrosis

A 78-year-old farmer presented with symptomless skin lesions for evaluation. Two years prior, he had developed idiopathic pulmonary fibrosis (IPF) and had been treated thereafter with oral prednisolone 20 mg/day and occasionally with colchicine 1 mg/day. On examination, erythematoviolaceous, slightly infiltrated plaques, measuring approximately 5 x 9 cm, rubbery in consistency, intermingled with pustules, sometimes eroded, with distinctive borders, were noted on the dorsum of both hands and on the extensor surface of both forearms. The lesions had developed over a 20-day period. The skin of these areas was atrophic or eroded with multiple ecchymoses (Fig. 1). The abnormal laboratory findings included an elevated white blood cell count of 17,100/mm3, with 79% neutrophils, 16% lymphocytes, and 5% monocytes, C-reactive protein of 33.15 mg/dL (normal, <0.8 mg/dL), and immunoglobulin G of 598 mg/dL (normal, 701-1545 mg/dL). Other blood and urine tests performed were within normal limits. The diagnosis of IPF was reconfirmed through radiology, high-resolution computed tomography, and spirometry, as well as bronchoscopy and bronchoalveolar lavage fluid analysis. Coexistence of presumptive pulmonary alternariosis was excluded. Hematoxylin and eosin stained sections of the excised cutaneous specimen showed focal ulceration of the epidermis adjacent to a mainly intradermal abscess cavity. Within the latter, remnants of a partly destroyed hair follicle were seen amongst degenerating polymorphonuclear leukocytes, as well as many histiocytes and a few Langhans-type multinucleated giant cells. Minute collections of polymorphonuclear leukocytes were seen in the adjacent epidermis. Periodic acid-Schiff (PAS) and Gomori's silver methenamine stains showed a multitude of broad branching fungal hyphae and large spores within the aforementioned cavity, both free and within the cytoplasm of giant cells (Fig. 2). Immunohistochemistry was performed by means of the alkaline phosphatase anti-alkaline phosphatase (APAAP) method. Sections showed that the infiltrate consisted of an almost equal number of B and T lymphocytes, whereas histiocytes and the few giant cells were labeled with anti-CD68 antibodies. Skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid Skin smears and biopsy specimens taken twice from all lesions were used for mycologic examination. Wet mounts revealed numerous, brownish, septate hyphae and ovoid structures. Biopsy material was plated on Sabourand's dextrose agar with cloramphenicol (0.05 mg/mL). After 7 days at 27 degrees C, dark, gray-white colonies with a dark brown underside appeared. Microscopic examination of the colonies revealed hyphae with typical conidia having transverse and longitudinal septa. Based on macroscopic and microscopic examination, the isolates were identified as Alternaria alternata (Fig. 3). Treatment with prednisolone was reduced to 10 mg/day and the patient received oral itraconazole (200 mg/day). This resulted in progressive improvement of alternariosis, and the lesions healed completely within 3 months, when treatment was interrupted. Two years later, there is no evidence of recurrence.     

Low molecular weight heparin; a novel alternative therapeutic approach for lichen planus

Heparin analogues in low doses have antiproliferative and immunomodulatory properties. The aim of this study was to evaluate the effect of low-dose enoxaparin administered subcutaneously in lichen planus (LP). Eighteen patients with various types of LP were treated in an open study for 6-13 weeks. Efficacy and safety data were recorded. Complete remission was observed in 11 of 18 patients (61%) and marked improvement in two (11%). Widespread cutaneous involvement and reticulated oral LP had the best response, while in LP of the scalp the response was poor. Enoxaparin is a promising alternative therapy for various types of LP.     

Quantitative description of overjet and overbite and their relationship with the craniofacial morphology.

OBJECTIVES: 1) To define the sagittal and vertical characteristics of anterior teeth in adults with normal occlusions; 2) to explore a relationship between the overbite and overjet; and 3) to relate overbite and overjet to the skeletal pattern. DESIGN: Prospective data collection. SETTING AND SAMPLE POPULATION: Ninety-two adult dental students from the Aristotle University of Thessaloniki (49 females and 43 males) with naturally occurring Class I occlusions. EXPERIMENTAL VARIABLE AND OUTCOME MEASURES: Cephalometric data were collected for overbite, overjet, and skeletal relationships. These were then correlated for potential association between front teeth and vertical and horizontal skeletal relationships. RESULTS: The overjet measures were equally distributed among men and women, but overbite was higher in women. Facial proportions were also bigger in men, but the Mediterranean face was bigger than Northern American Caucasian. The mandibular plane angle could be associated with either increased or decreased overjet and overbite. CONCLUSION: The overbite and overjet features of an occlusion cannot be predictably associated with any particular craniofacial pattern.     

The Need for Graduate Education in Community Health Nursing: A Survey

Home health care agency administrators and staff members were surveyed to determine areas of interest for a proposed expansion of the graduate program in community health nursing at Boston University School of Nursing. Two interrelated questionnaires were designed, one for administrators, the other for staff, and were mailed to agency directors. Forty-six percent of the administrators and 29 percent of staff members responded. Roughly half (49%) of the staff members were graduates of baccalaureate programs in nursing and interested in graduate education in nursing. Clinical specialization within home health care was the area of greatest interest for both administrators and staff members, followed by home health care administration. Many agencies provided released time for the part-time evening study preferred by staff. It was concluded that there is a great deal of interest in graduate education in home health care, including both clinical specialization and administration. Agency directors stated they were willing to hire graduates of such programs, and the overwhelming majority of staff members who were eligible for and interested in graduate study identified an interest in the subject.