bluuebunny/crossref_metadata_2025_split huggingface dataset:
Perforation of large and small bowel in Henoch‐Schonlein purpura
Authors: Bissonnette Robert , Dansereau Alain , D'Amico Patrick , Pateneaude Jean‐Victor , Paradis Jean 
Published: August 2010

A 43‐year‐old woman with an unremarkable medical history was admitted with severe polyarthralgia, purpuric papules on her lower legs (Fig. 1), and recent pain with blue discoloration of her third left finger. She was taking no medication. At the time of presentation the abdominal examination was normal. Laboratory evaluation showed normal or negative values of BUN, creatinine, AST, WBC, Hb, VDRL, rheumatoid factor, HBSAg, ANA, ASO, cryoglobulins, cryofibrinogen, anticardiolipins, anti‐Sm, anti‐RNP, anti‐Ro, anti‐La, anti‐SCl70, and anti‐centromere. Hepatitis C titer was not obtained. Sedimentation rate was 61 mm/h and immune circulating complexes were present (14.2 <1.5μg/ml); normal, <1.5 μg/ml). Urine sediment analysis revealed the presence of cellular, hyalin, granular, and erythrocyteic casts. IgA level was 1.98 g/l (normal, 0.5–3.0 g/l). Skin biopsy showed extensive leucocytoclastic vasculitis. Deposits of IgA, IgM, and C3 were observed in the dermal vessels under direct immunofluorescence microscopy.The patient was put on prednisone 60 mg/day. Three days after admission she complained of acute abdominal pain. Increased abdominal tenderness was noted on examination. An abdominal angiogram was normal. Because of the deteriorating intestinal condition, she received methylprednisolone 1 g IV per day. Despite this treatment she developed acute peritonitis 2 days later. Laparotomy disclosed three perforations on the caecum and ascending colon, and one on the ileum. She underwent sub‐total colectomy with terminal ileostomy. Following surgery she was kept on parenteral methylprednisolone 1 g daily; cyclophosphamide 300 mg daily was started with progressive improvement of her condition.The histopathologic examination of the colonic segment disclosed vasculitis of the medium‐sized vessels (Fig. 2). Direct immunofluorescence microscopy revealed strong deposits of IgA and C3 in the vessel walls of the large intestine (Fig, 3).
Palmoplantar keratoderma – Mal de Meleda syndrome
Authors: Rajashekhar Nadiga , Moideen Rafeeq 
Published: August 2010

One sister and one brother (of four), aged 18 and 12 years, respectively, born of a consanguineous marriage between first cousins, presented with thickening of the palms and soles after 1 year of life, which was slowly increasing, and the simultaneous appearance of thickened skin: colored plaques on the dorsal aspect of the hands, feet, knees, and ankles. There was hyperhidrosis of the palms and soles, but no history of similar complaints in the maternal/paternal family. The pedigree was as shown in Fig. 1.On examination, the sister showed diffuse palmoplantar keratoderma extending to the dorsal surface with sharp margins. The fingers were short and mutilated, and the nails showed subungual hyperkeratosis, onycholysis, and Beau's lines (Figs 2 and 3). Circular hyperkeratotic knuckle‐pad‐like thickenings were present on all proximal interphalangeal joints and some knuckles. Irregular‐shaped hyperkeratotic plaques and linear keratosis were present on the flexor aspect of the wrists and on the dorsal aspect of the feet, ankles, and knees.The brother (12 years old) showed the same type of clinical features.The histopathology revealed hyperkeratosis, hypergranulosis, acanthosis, and chronic inflammatory dermal infiltrate.General and systemic examination of all the sisters and brothers was normal. Ophthalmologic examination was normal. Oto‐rhino‐laryngologic examination showed a high arched palate. Intelligence testing of all sisters and brothers was normal.The patients were given oral vitamin A with vitamin E and topical keratolytes. They are under follow‐up at present.
Pyoderma gangrenosum associated with anticardiolipin antibodies in a pregnant patient
Authors: Freedman Alan M. , Phelps Robert G. , Lebwohl Mark 
Published: August 2010

A 40‐year‐old pregnant woman at 17 weeks of gestation presented with a 10‐day history of pain and erythema in the right leg. She had experienced a similar episode in the postpartum period (1 week) 14 months earlier, lasting 2 months. This was diagnosed as cellulitis and resolved following antimicrobial therapy. The patient had no fever or chills. Pertinent past medical history involved a possible history of ampicillin allergy.The initial differential diagnosis included cellulitis and thrombophlebitis. Antibiotic therapy with intravenous (IV) cefazolin was started at the same time as anticoagulation with heparin. Over the next few days the ulceration enlarged. No microbial growth was detected on wound cultures and Doppler studies carried out prior to admission revealed normal venous flow. The patient did not respond to therapy.A dermatology consult was requested. When the patient was first examined by us, the ulceration had been progressively increasing in size. There was an oozing ulcerated patch 7 cm in diameter on the right distal medial thigh (Fig. 1). The ulcer contained crater‐like holes and fistulous tracts from which pus could be expressed upon application of pressure. The surrounding border was undermined and violaceous. A clinical diagnosis of pyoderma gangrenosum (PG) was made.Antinuclear antibodies and antibodies to Smith antigen were negative; anticardiolipin antibody was positive. A 3‐mm punch biopsy showed an intense neutrophilic response with fibrinoid change in some vessels (Fig. 2), which was felt to be consistent with PG with a concomitant small vessel vasculitis. No fibrin thrombi were noted. The heparin was replaced with aspirin and the IV antibiotics were changed to oral cephalexin. Intralesional triamcinolone (40 mg/cc) was started and a 3‐day course (80 mg/40 mg/20 mg) of oral prednisone was initiated. Within 2 days of starting steroid therapy, the inflammation and discharge were greatly reduced.Approximately 2 weeks after admission the therapy was noted to halt the progression. The lesion became more well demarcated with healthy granulation tissue and decreased tenderness and warmth.The patient was discharged on Burow's solution, saline soaks, and bacitracin and was seen by us on two occasions after discharge for intralesional steroid administration. One flare‐up since discharge was controlled by increasing her prednisone from 10 mg to 20 mg daily. The patient had premature rupture of membranes at 37 weeks, had a spontaneous vaginal delivery, and delivered a healthy baby boy weighing 5 Ib 14 oz. Since then, the ulcer had re‐epithelialized with tapering doses of prednisone.
Large B‐cell lymphoma of the leg
Authors: Vasquez‐del‐Mercado Elsa , Toussaint Sonia , De La Barreda Fernando , Ortiz‐Hidalgo Carlos 
Published: August 2010

A 74‐year‐old Mexican man presented with an 18‐month history of multiple, violaceous, coalescing, firm, tender nodules with an ulcer in the anterior aspect of the right leg (Fig. 1) and slightly infiltrated, ill‐defined erythematous plaques affecting the left leg and both forearms. He had not received any treatment for his condition. Past medical history was relevant for noninsulin‐dependent diabetes mellitus and hypertension without formal treatment and a history of heavy alcohol intake in his youth. A biopsy specimen of both plaque‐type lesions of the forearm and tumorous lesions of the leg showed a diffuse, nonepidermotropic mononuclear infiltrate throughout the dermis and extending to the subcutis. The infiltrate was composed of pleomorphic, atypical, large mononuclear cells (Fig. 2). Immunostaining with CD20 was positive for the atypical cells while CD3 was positive for normal appearing lymphocytes, characterized as reactive T cells. Additional laboratory and image studies ruled out extracutaneous involvement. The diagnosis of primary cutaneous large B cell lymphoma of the leg (LBCLL) was made. The patient was initiated on radiotherapy localized to the right leg with a very good initial response, nevertheless resolution was not achieved and the plaques in the rest of the limbs remained unchanged. Thus, the patient started chemotherapy with CHOP (Cyclophosphamide, Vincristine, Doxorubicin, Prednisone). He has currently finished his fourth cycle with this chemotherapy regimen. The tumorous lesions involuted leaving only residual hyperpigmentation (Fig. 3) and the plaques in the rest of the limbs disappeared, the area of the ulcer diminished considerably. There is still no evidence of extracutaneous involvement.Nodules and ulcer in the anterior aspect of the right legimageAtypical lymphocytes, with large, pleomorphic nuclei and multiple nucleoles. Positivity for CD20 antigen was demonstrated by immunohistochemical analysis (hematoxylin and eosin; X 600)imageResidual hyperpigmentation and granulation tissue after chemotherapyimage
Caspase‐9 expression is increased in endothelial cells of active Behçet's disease patients
Authors: Öztaş Pınar , Lortlar Neşe , Polat Muhterem , Allı Nuran , Ömeroğlu Suna , Basman Adil 
Published: August 2010

AbstractBackground  Behçet's disease is a multisystem disease of unknown etiology. Caspase‐9 is responsible for initiating the caspase activation cascade during apoptosis. The aim of this study was to examine caspase‐9 expression in both endothelial and perivascular infiltrates of patients with active Behçet's disease.Methods  Fifteen patients with active Behçet's disease, attending the First Dermatology Department, Ankara Numune Hospital, Ankara, Turkey between June 2003 and December 2005, were included in the study. Oral biopsy specimens from nine healthy volunteers were taken as the healthy control group, and skin biopsies from 18 psoriasis patients were used as the inflammatory control group. The specimens were examined with caspase‐9 primary antibody. Statistical analyses were performed using SPSS 11.5.Results  The mean caspase‐9‐positive endothelial cell counts were 7.17 ± 2.45 in active Behçet's disease, 4.81 ± 0.76 in healthy controls, and 4.35 ± 1.34 in inflammatory controls. The difference between Behçet's disease and healthy controls was statistically significant, with increased endothelial staining in active Behçet's disease (P = 0.049). The difference between Behçet's disease and inflammatory controls was also statistically significant; the rate of staining was higher in Behçet's disease (P = 0.006). The mean caspase‐9‐positive dermal perivascular cell counts were 5.15 ± 2.32 in Behçet's disease, 3.32 ± 0.82 in healthy controls, and 5.54 ± 4.95 in inflammatory controls. These values did not show any statistically significant difference (P = 0.407).Conclusion  Endothelial cells are one of the key cells in Behçet's disease, and our findings support the role of endothelial cells in the etiopathogenesis of Behçet's disease.
A case of linear IgA disease in a child with IgA and IgG circulating antibodies directed to BPAg2
Authors: Viglizzo Gianmaria , Cozzani Emanuele , Nozza Paolo , Occella Corrado , Parodi Aurora 
Published: August 2010

A 7‐year‐old Caucasian girl had multiple bullae on the trunk, upper and lower limbs (Fig. 1a,b) for 10 days. The lesions were large, tense, and asymptomatic, and mucosae were not involved. Laboratory findings were all normal. Histopathology revealed a subepidermal blister containing numerous neutrophils, eosinophils, and fibrin. Direct immunofluorescence of perilesional skin disclosed linear deposition of IgA and slight linear deposits of IgM at the basement membrane zone (Fig. 2). Indirect immunofluorescence on monkey esophagus and on human salt‐split skin was negative. Immunoblot assay (IB), performed with an epidermal extract, revealed IgA and IgG antibodies directed to BPAg2 antigen (Fig. 3), while it was negative when performed on dermal extract. Enzyme‐linked immunosorbent serologic assay using a commercial kit (MBL, Naka‐Ku Nagoya, Japan) with the noncollagenous domain (NC16A) of the BPAg2 antigen was negative for both IgA and IgG. A diagnosis of linear IgA disease (LAD) was made and a treatment with dapsone (50 mg/day) and prednisolone (30 mg/day) was initiated. One month later, lesions had cleared.Multiple tense bullae on the limbsimageDirect immunofluorescence shows a continuous linear IgA deposit at the basement membrane zone (BMZ) (magnification ×300)imageImmunoblot assay (IB) of the patient's serum on an epidermal extract using an anti‐IgG antiserum (lane 2) and an anti‐IgA antiserum (lane 3) reveals a 180‐kDa medium wave band, identical to that revealed by a BPAg2 IgG positive control serum (lane1)image
Polish dermatology in the 19th and the first half of the 20th centuries
Authors: Grzybowski Andrzej 
Published: August 2010

AbstractThe beginnings of Polish dermatology date back to the first half of the 19th century in Kraków. The first textbook of dermatology was written by Ludwik Bierkowski. Later the progress in the development of this field of medicine was due to Franciszek Krzyształowicz, Marian Grzybowski, Franciszek Walter, and Jan Alkiewicz. Krzyształowicz's most remarkable achievements were related to his studies of the Treponema pallidum of syphilis. Grzybowski's main contribution to international dermatology was the first description in the medical literature of a specific variant of keratoacanthoma, which has since then been called Grzybowski's eruptive keratoacanthoma or generalized eruptive keratoacanthoma – Grzybowski's variant. Alkiewicz described trachyonychia, or twenty‐nail dystrophy, a disease that became well established in the dermatological literature; he also described the so‐called transverse net in onychomycosis. Walter identified the syphilitic skin and bone lesions in some figures carved in the Veit Stoss's altar in Kraków, thus presenting the famous thesis of the non‐American origin of syphilis in Europe. Considering all these achievements, it is the goal of this paper to review Polish contributions to international dermatology.
Buschke–Ollendorff syndrome
Authors: Schena Donatella , Germi Lerica , Zamperetti Maria Rosa , Colato Chiara , Girolomoni Giampiero 
Published: August 2010

AbstractBackground  Buschke–Ollendorff syndrome is a rare autosomal dominant disease featuring osteopoikilosis and skin lesions. It is caused by genetic mutations in a protein deeply involved in bone and connective tissue morphogenesis.Methods  We describe a 39‐year‐old woman with Buschke–Ollendorff syndrome.Results  After a minor trauma, radiologic examination of the left ankle of a 39‐year‐old woman revealed features of osteopoikilosis. Physical examination of the patient showed multiple asymptomatic nodules on both thighs, present since the age of 20 years, which had increased in size and number. Recently, a linear, string‐like lesion had appeared on the right thigh.Conclusion  The correct diagnosis of Buschke–Ollendorff syndrome may require a high index of suspicion.
The frequency and seasonal variations in juvenile outpatients treated in a dermatology polyclinic
Authors: Cicek Demet , Kandi Basak , Oguz Sevilay , Ucak Haydar , Saral Yunus , Kurt Abdullah 
Published: August 2010

AbstractObjective  This study was carried out to evaluate the frequency and seasonal characteristics of dermatological problems of juvenile outpatients in the dermatology polyclinic.Material and method  Children under the age of 17 treated in an outpatient setting in our polyclinic within a 10‐year period were retrospectively evaluated.Results  The study registered a total of 3244 patients, of whom 1651 (50.90%) were females, and 1593 (49.10%) were males. The largest group was infectious diseases, with 1031 patients (31.78%). This was followed by the dermatitis group with 635 patients (19.57%). The months when the number of presentations at the hospital was the highest were August and November, and the months with the lowest number of presentations were January and July.Conclusion  In the present study we found that the most frequent cause of hospital visits of juvenile outpatients at our polyclinic was infectious diseases and dermatitis, and that there was significant correlation between diseases and seasons.
Mycobacterium tuberculosis chaperonin 10 and N‐truncated fragments Their synthesis and purification by the isoelectric focusing technique carried out in solution
Authors: LUCIETTO PIERLUIGI , FOSSATI GIANLUCA , BALL HAYDN L. , GIULIANI PAOLA , MASCAGNI PAOLO 
Published: August 2010

The Mycobacterium tuberculosis chaperonin 10 protein and fragments corresponding to sequences 59‐99, 51‐99 and 26‐99 were synthesised by the solid‐phase methodology using a double coupling protocol and without the aid of capping agents. After the final acid cleavage using the low TFMSA‐high HF protocol the polypeptides were purified by either the ion exchange chromatography/RP‐HPLC combination or the isoelectric separation carried out in solution and followed by semi‐preparative RP‐HPLC. Comparison of the results obtained through the two approaches indicated that in general the isoeletricfocusing/HPLC combination was superior both in terms of recovery of final material and its purity. The advantages found were as follows: (i) Unlike ion exchange chromatography, no tailoring of the separation conditions is required. (ii) Several consecutive focusings can be carried out in progressively narrower pH gradients. This increases the separation resolution without the need of changing other separation parameters, (iii) Very little manipulation is needed, and each focusing requires 3‐5 h. (iv) Full compatibility with non‐ionic denaturants such as 8 M urea. This increases solubility so that using the ROTOFOR instrument described here 50‐100 mg crude polypeptide can be processed daily.Thus the isoelectric focusing technique carried out in solution is a valid and inexpensive alternative to ion exchange chromatography. © Munksgaard 1997.
Effect of oxidation of β‐amyloid precursor protein on its β‐secretase cleavage. A model study with synthetic peptides and candidate β‐secretases
Authors: Rpaea‐Kozik M. , Kozik A. , Travis J. 
Published: August 2010

AbstractAs the amyloidogenic processing of β‐amyloid precursor protein (βAPP) proceeds under conditions of oxidative stress, the methionine‐596 residue at the β‐secretase cleavage point is likely in an oxidized state. In the present work, possible consequences of the oxidation of Met‐596 for the generation of the N‐terminus of amyloid p protein were modeled using synthetic peptide substrates, matching 587‐606 sequence fragment of βAPP and containing either intact methionine or methionine sulfoxide. Patterns and rates for the cleavage of these substrates by purified mast cell chymase, cathepsin G, cathepsin D, matrix metallopro‐teinase‐3 and neutrophil elastase, were compared. Only the three first proteases, all previously suggested as candidate β‐secretases, preferentially cleaved the “intact” substrate after Met‐596. For chymase and cathepsin G, the specificity of this cleavage increased upon a shift from optimal alkaline pH to acidic pH, which is also more compatible with the plausible intracellular localization of amyloidogenic βAPP processing. The substitution of methionine sulfoxide for methionine in the substrate slowed down the cleavage rate for all the enzymes tested, by a factor of 6‐15. This was associated with shifts of cleavage preferences to points of minor importance for the “intact” peptide, suggesting a specific resistance of the peptide bond after MetSO‐596 against proteolysis.
Sequence‐assisted peptide synthesis (SAPS)
Authors: Larsen B. Due , Holm A. 
Published: August 2010

Abstract:In solid‐phase peptide synthesis (SPPS) the growing peptide chain may undergo chain aggregation which can cause serious synthetic problems. A number of investigations concerning this problem have been reported in the chemical literature. During a study of such “difficult sequences” using the Fmoc‐protection strategy, we have observed that peptide‐chain aggregation may be significantly reduced when certain amino acid sequences are incorporated C‐terminally (1). Thus, synthesis of the difficult poly‐alanine, (Ala)n, sequence (n ≦ 20) has been investigated with (Lys(Boc))m (m lE; 6) and (Glu(tBu))m (m ≧ 6) as pre‐sequences. With m ≧ 3, peptides are obtained as single, homogeneous products while a complex mixture of deletion peptides and corresponding Fmoc‐protected peptides is formed (n ≧ 6) without the pre‐sequence. A mixed pre‐sequence, (Lys(Boc)Glu(tBu))3, has a similar favorable effect on the synthetic results, but the positive effect seems confined to a rather narrow framework of amino acids and side‐chain protecting groups in the pre‐sequence as discussed in the article.Among other reputedly difficult sequences the synthesis of H‐(Thr‐Val)5‐OH, H‐Val‐Asn‐Val‐Asn‐Val‐Gln‐Val‐Gln‐Val‐Asp‐OH, the Acyl Carrier Protein (65–74) and the human insulin B‐chain has been investigated. In all cases introduction of a pre‐sequence gives rise to satisfactory synthetic results. In the latter case, the lysine pre‐sequence may be cleaved enzymatically to give the desB30 insulin B‐chain.NIR‐FT Raman studies of the synthesis of the poly‐alanine, (Ala)n, sequences have shown that the pre‐sequence (Lys(Boc))6 shifts the conformation of the growing peptide chain from a β‐structure (n ≧ 6) to a random coil conformation (1c). This result is in agreement with the general observation that SPPS proceeds optimally under random coil conditions.
Solid‐phase synthesis, conformational analysis, and biological activity of AVRp elicitor peptides of the fungal tomato pathogen Cladosporium falvum
Authors: Mahé E. , Vossen P. , Van den Hooven H. W. , Le‐Nguyen D. , Vervoort J. , De Wit P. J. G. M. 
Published: August 2010

Abstract:The race‐specific peptide elicitor AVR9 of the fungal pathogen Cladosporium fulvum specifically induces a hypersensitive response in tomato genotypes carrying the complementary resistance gene Cf‐9. The total chemical syntheses of this 28‐residue AVR9 peptide containing three disulfide bonds, and of three mutant peptides [R8K]AVR9, [F10A]AVR9 and [F21A]AVR9, have been accomplished. The syntheses were carried out using a stepwise solid‐phase approach based on tBoc chemistry. The disulfide bridges were formed by air oxidation. The correctness of the chemical structure of all folded synthetic peptides was confirmed by combined NMR and MS analyses. The biological activity and a number of physicochemical properties of folded synthetic AVR9 are identical to those of native fungal 28‐residue AVR9. The overall conformations of the folded synthetic mutant peptides were comparable to that of synthetic wild‐type AVR9 as demonstrated by NMR spectroscopy. Mutant [R8K]AVR9 showed a threefold higher, and mutant [F10A]AVR9 a threefold lower necrosis‐inducing activity when compared to synthetic wild‐type AVR9. However, mutant [F21A]AVR9 showed hardly any necrosis‐inducing activity. Affinity for polyclonal antibodies raised against native fungal AVR9 is positively correlated with the necrosis‐inducing activity of the synthetic AVR9 peptides ([R8K]AVR9 > wild‐type AVR9 > [F10A]AVR9 > [F21A]AVR9).