Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.     

A patient with cystinosis presenting transient features of Bartter syndrome

A 16-month-old boy was admitted to the clinic because of vomiting and growth failure. His weight and height measurements were under the fifth percentile. He had fair hair and skin, enlarged wrists and rachitic rosaries. The presence of metabolic alkalosis, hypokalemia, hypochloremia, and high renin and aldosterone levels were suggestive of Bartter syndrome. However, in view of the growth failure, fair hair and skin, proteinuria, polyuria and active rickets, cystinosis was considered. Bone marrow smear examination was normal, despite the existence of suspicious crystals in the cornea. Cystine crystals were seen in the conjunctiva biopsy and increased leukocyte cystine level was measured; therefore, definitive cystinosis diagnosis was made. Renal Fanconi syndrome with metabolic acidosis is prominent in cystinosis; however, in rare instances, if sodium-dependent trans-tubular transport defect is present, patients could have Bartter syndrome findings such as hypochloremic metabolic alkalosis. Our case is a good example demonstrating that metabolic alkalosis should not exclude cystinosis and the other signs and symptoms of the patient should be thoroughly evaluated.     

Clinical and epidemiological characteristics of retinoblastoma: correlation with prognosis in a Turkish pediatric oncology center

Advanced intraocular tumors and metastatic disease in retinoblastoma patients still occur frequently in developing countries. The aim of this retrospective study was to describe the clinical and epidemiological characteristics of patients with retinoblastoma and the effects of these features on disease prognosis in the authors' pediatric oncology unit as a developing country profile to define the problem. A retrospective chart review of 91 patients who presented to the unit between May 1996 and December 2003 was conducted in this study. Patients with unilateral disease presented at a median age of 24 months and those with bilateral disease at a median age of 9.5 months (p < .01). Most of the eyes with retinoblastoma (68.6%) had Reese-Ellsworth stage V disease. Metastatic disease was diagnosed in 19 (20.9%) patients. Cases with metastatic disease presented at a median age of 24 months and those without metastatic disease at a median age of 12.5 months (p < .05). In 31 patients (34.1%) there was a delay in diagnosis. The enucleation ratio in eyes with advanced intraocular stage was significantly higher than in eyes with early intraocular stage (57.9 vs. 3.8%) (p < .001). In patients with metastatic disease, tumor recurrence was more frequent than in the nonmetastatic patients (36.8 vs. 4.2%) (p < .01). Seven children (7.7%) died due to central nervous system (CNS) metastasis (p < .01). Advanced intraocular disease and distant metastases occur more frequently in Turkish children with retinoblastoma than in children in developed countries, causing a higher rate of enucleation and mortality. Late referral might account for the delayed diagnosis.     

Sonoelastography of the knee joint

Magnetic resonance imaging (MRI) is generally the preferred method for assessing lesions of the knee cartilage and subchondral bone. There have been a few cartilage imaging studies using real‐time elastosonography (RTE), which has increased in importance and range of use in recent years. The aim of this cadaveric study was to assess the efficacy of a new diagnostic method combining USG and RTE and also to perform intra‐articular examinations together with arthroscopy. A total of 12 fresh unpaired human knees were examined. The laparoscopic ultrasound transducer was deployed using standard anteromedial and anterolateral arthroscopic portals. Iatrogenic defects were examined using mosaicplasty tools in healthy‐looking areas of cartilage, and strain in those areas was measured using RTE. The median strain value of the pathological femoral cartilage region was significantly higher than that of the normal cartilage region (1.23 [0.71–2.24] vs. 0.01 [0.01–0.01], P = 0.002, respectively). Arthroscopic study of cartilage using RTE can be a guide for orthopedic surgeons and use of intra‐articular probes could be universalized. Clin. Anat. 32:99–104, 2019. © 2018 Wiley Periodicals, Inc.     

Combined occurence of primary cerebral lymphoma and meningioma

A unique combination of primary cerebral lymphoma and meningioma was encountered in a 38-year-old woman who recently presented clinically with headache and papilledema. The both tumors were identified by CT scan and diagnosed histopathologically after the surgery. The association of a highly malignant primary cerebral lymphoma and a slowly growing meningioma is extremely rare. The possibility of an unknown underlying pathologic mechanism predisposing to multiple tumors should be considered.     

RIGHT ATRIAL CATHETER-RELATED COMPLICATIONS IN PEDIATRIC ONCOLOGY PATIENTS: The Situation in a Developing Country

The complications of right atrial catheters (RACs) in pediatric oncology patients are unknown for centers in developing countries. This study examined the complications of RACs at Ankara University Medical School, Turkey. A total of 90 RACs were placed in 61 children for long-term chemotherapy with a total experience of 15,536 catheter days. The rate of catheter-related sepsis was 4.9 episodes per 1000 catheter days. Coagulase-negative staphylococci and Candida species were the most common organisms, accounting for 25.0 and 13.1% of all organisms, respectively. The most common reasons for the removal of the RACs were infection (42.4%) and dislodgement (32.2%). The rates of complications were significantly higher in this study than in western studies. This increase could be explained by the differences in catheter care practices in the Turkish center. In conclusion, the use of RACs in a developing country necessitates an appraisal of the benefits and risks for each patient and improvement of catheter care procedures.     

Esophageal candidiasis and Candida colonization in asthma patients on inhaled steroids.

The aim of this study was to evaluate the risk of esophageal candidiasis in asthma patients who are on inhaled steroids without any other risk factors for esophageal candidiasis by comparing the treatment group with the control group. Moreover, the oropharyngeal and esophageal Candida colonizations were evaluated in the subgroups of both control and treatment groups. Upper gastrointestinal system endoscopic evaluation was performed in 40 asthma patients who were on inhaled steroids for at least 1 month. The control group consisted of 40 steroid na?ve patients without asthma. Oral and esophageal samples were obtained for performing quantitative culture. Candida growth in cultures without any clinical signs and symptoms was described as colonization. Candida growth accompanied by clinical signs and symptoms was described as infection. None of the patients in the control group had either esophageal or oropharyngeal candidiasis; however, one (2.5%) asthma patient had esophageal candidiasis and two (5%) asthma patients had oropharyngeal candidiasis. Esophageal and oropharyngeal Candida colonization was determined in 5 (22.7%) and 11 (50%) of the asthma patients and 7 (31%) and 9 (41%) of the control group, respectively. Although the mean numbers of Candida colonies were higher in the asthma group in both localizations, there were no statistically significant differences between the two groups regarding esophageal or oropharyngeal Candida colonization. The risk of esophageal candidiasis due to inhaled steroids is low and inhaled steroids may be used safely in terms of esophageal candidiasis. Future prospective studies are needed to draw more definitive conclusions.     

Serum Prolidase and IGF-1 as Non-invasive Markers of Hepatic Fibrosis During Four Different Periods After Bile-duct Ligation in Rats

AIM: Our aim was to study the correlation of serum prolidase and insulin like growth factor-1 to liver collagen and assess their utility as markers of fibrosis during four different periods of hepatic injury and fibrosis after bile-duct ligation in rats. METHODS: Forty-eight Wistar albino rats were included in the study and divided into six groups. Seven rats served as the control group (Control), while seven rats had a sham operation (Sham group). Thirty-four rats underwent bile-duct ligation. Bile-duct ligated (BDL) animals were sacrificed at the end of the first week (Group 1; n = 8), second week (Group 2; n = 8), third week (Group 3; n = 9), or fourth week (Group 4; n = 9) after BDL. Liver collagen, liver prolidase, and serum prolidase and IGF-I, were determined. RESULTS: There was a positive correlation between liver collagen and serum prolidase (r(s): 0.843, P < 0.001) levels and a negative correlation among liver collagen and serum IGF-1 levels (r(s): -0.667, P < 0.001). The peak levels of liver collagen and serum prolidase were reached in the third week while the lowest levels of IGF-1 were found at the end of the third week. CONCLUSION: Serum prolidase and IGF-1 either independently or in combination correlate with liver collagen content in hepatic fibrosis.     

Hyperbaric Oxygen Therapy Is as Effective as Dexamethasone in the Treatment of TNBS-E-Induced Experimental Colitis

Introduction Hyperbaric oxygen (HBO) has been demonstrated to be useful as an adjunctive therapy for Crohn’s disease. In the present study, HBO was tested as a treatment for trinitrobenzenesulfonic acid–ethanol (TNBS-E)-induced distal colitis, and its effects were compared with dexamethasone therapy. Methods A total of 48 Sprague-Dawley rats were separated into six groups: the control, and those treated with vehicle, TNBS-E, HBO, dexamethasone, or combined HBO + dexamethasone. The HBO treatment group was exposed to 100% HBO at 2 ATM for 75 min twice daily at 6-h intervals in a HBO chamber, both on the day of colitis induction and 3 days thereafter. Treatment with intraperitoneal dexamethasone twice daily was started 1 h before the induction of colitis and was continued for 7 days in the dexamethasone group. The rats were decapitated 8 days after the induction of colitis, and the colonic tissue wet weight, macroscopic and microscopic lesion score, and tissue myeloperoxidase (MPO) activity were determined. Results HBO therapy decreased the activity of experimental colitis measured by the tissue wet weight, macroscopic score, microscopic score, and MPO activity. The dexamethasone treatment significantly reduced the colitis activity as determined by the tissue MPO activity and wet weight. There were also decreases in the macroscopic and microscopic activity scores with the dexamethasone therapy; however, these changes were not statistically significant. The combined therapy with HBO and dexamethasone provided no additional benefit over HBO therapy alone. Conclusion HBO therapy can be a valuable therapeutic option in treatment of patients with inflammatory bowel disease. HBO therapy in the refractory patients deserves further, larger clinical studies.