Cardiovascular responses of exercises performed within the extreme exercise domain.

Stroke volume (SV), heart rate (HR) and arterio-venous O2 difference (a-vO2diff) responses to heavy and severe-intensity exercise have been well documented; however, there is a lack of information on the SV, HR and a v-O2diff responses of work rates within extreme exercise domain. The aim of this study was, therefore, to focus on central and peripheral components of VO2 responses to exercises performed within the heavy, severe and extreme exercise domain. Eight well-trained male cyclists participated in this study. Maximal O2 consumption (VO2max) and corresponding work rate (P@VO2max) were determined by multisession constant work rate exercises. Cardiovascular responses to exercises were evaluated by nitrous-oxide rebreathing method with work rates from 40 % to 160 % of P@VO2max, VO2max corresponded to 324+/-39.4 W; however, maximal SV responses occurred at 205+/-54.3 W (p<0.01). Maximal cardiac output (Q), HR, and a vO2diff responses were revealed by the P@VO2max. VO2 response to exercise significantly decreased from severe-intense exercises to the first work rate of extreme exercise domain due to significant decreases in Q, SV, and HR responses (p<0.05), except a v-O2diff (p>0.05). Moreover, non-significant decreases in Q, SV, and a v-O2diff were evaluated as response to increase in work rate belonging to extreme work rates (p>0.05), except the HR (p<0.05). Work rates within the lower district of the extreme exercise domain have an important potential to improve peripheral component of VO2, while the P@VO2max seems the most appropriate intensity for aerobic endurance development as it maximizes the central component of VO2max.

A single-center kidney transplantation experience in children with low weight: is low weight a contraindication?

OBJECTIVE: Kidney transplantation (KT) might be difficult for underweight kids (under 15 kg). Our goal was to convey information on KT in underweight children. PATIENTS AND METHODS: The study's target population consisted of children (age 18) weighing 15 kg or fewer who received KT at our facility between January 2018 and June 2021. A retrospective analysis was performed on demographic and clinical data, including age, gender, primary disease, pretransplant dialysis status, recipient weight, recipient body mass index (BMI), surgical approach type (intraperitoneal/extraperitoneal), complications, graft status (functioning/failed), patient survival, and immunological data. RESULTS: There were 94 pediatric KTs completed. Thirty-three patients were included when the selection criteria were applied. The mean recipient weight was 11.45 [6.7-15] kg, and the average patient age was 3.36 [1-7]. Three (9.9%) patients had kidney transplants from dead adult donors, whereas thirty (90.1%) patients underwent live donor kidney transplantation (LDKT). While the intraperitoneal (IPA) technique was used in 19 cases, the extraperitoneal (KT) strategy was used on 14 patients (EPA). The donor BMI was 28.24 [19.6-42] kg/m2, and the mean donor weight was 78.13 [55-109] kg. Bridectomy was necessary because five individuals experienced ileus. IPA was performed in each of these patients during LDKT. Following IPA, a 2-year-old patient with a BMI of 16 kg/m2 had renal allograft compartment syndrome and required graft nephrectomy. CONCLUSIONS: Pediatric patients weighing 15 kg or fewer can get kidney transplants successfully. Gastrointestinal problems are relatively uncommon with EPA, even though there is no agreement on the best surgical strategy.

Brooke-Spiegler syndrome: focus on reflectance confocal microscopy findings of trichoepithelioma and flat cylindroma.

Brooke-Spiegler syndrome (BSS) is a rare, autosomal dominant disorder characterized by multiple adnexal tumours, especially trichoepitheliomas, cylindromas and occasionally spiradenomas. These lesions usually begin to appear in the second or third decade of life. Malignant transformation of pre-existing tumours may occur. In vivo reflectance-mode confocal microscopy (RCM) is a noninvasive method that can be used to visualize the epidermis and the upper dermis at almost histological resolution. It has been used to evaluate several skin conditions, especially malignant lesions, and has been reported to be useful for differentiating between malignant and benign skin tumours. Only a few studies have reported on the use of confocal microscopy to characterize the features of benign adnexal neoplasms. We present a patient with BSS who presented to our clinic with multiple skin tumours. The possible utility of RCM for identifying adnexal neoplasms is emphasized in this report, which also describes the observed microscopic features.

Frequency of juvenile fibromyalgia syndrome in children with familial Mediterranean fever: effects on depression and quality of life.

OBJECTIVES: To determine the prevalence of juvenile fibromyalgia syndrome (JFMS) in children with familial Mediterranean fever (FMF) and to evaluate quality of life (QoL) and depression. METHODS: Ninety-one FMF patients (M/F: 44/47) who fulfilled the Livneh criteria and 60 healthy children (M/F: 27/33) were enrolled in the study. Yunus and Masi's criteria were used for diagnosis of JFMS. Depression was assessed with Children's Depression Inventory (CDI) and QoL was evaluated with child and parent reports of Paediatric Quality of Life Inventory 4.0 (PedsQL™). RESULTS: While 20 (21.9%) of 91 FMF patients fulfilled JFMS criteria, 2 (3.3%) of the control group met the diagnostic criteria of JFMS (p=0.002). PedsQL™ scores (child self-report and parent-report) of the FMF patients were significantly lower and the depression scores were significantly higher than the healthy controls (p<0.001 for all). When the FMF patients were assigned to two groups as FMF with or without JFMS, patients with JFMS were found to have a higher depression score (p=0.007) and child and parent reports of PedsQL™ 4.0 were lower in the children with JFMS than in the patients without JFMS (p=0.001, p=0.003, respectively). CONCLUSIONS: We have determined that JFMS frequency was higher in children with FMF and patients with FMF and JFMS had a poor QoL and were more susceptible to depression. FMF patients with widespread and persistent pain should be evaluated for JFMS in order to avoid unnecessary investigations and inappropriate treatment.

Plasma cell-free DNA levels in children on peritoneal dialysis.

BACKGROUND/AIMS: Plasma levels of cell-free DNA (cfDNA) are elevated in various clinical conditions including cancer, stroke, trauma, myocardial infarction, autoimmune disorders, and pregnancy-associated complications. Previously, increased cfDNA levels were reported during hemodialysis. However, there is limited data regarding cfDNA levels in peritoneal dialysis (PD) patients. The aim of this study was to investigate the levels of cfDNA in children on PD. METHODS: Twenty-one children on PD (median age: 12; range: 4-18 years) and 21 healthy children (median age: 10; range: 6-16 years) were enrolled into the study. Plasma cfDNA was measured using a real-time quantitative PCR for the beta-globin gene. RESULTS: The median concentrations of cfDNA in the plasma of PD patients and healthy controls were 2,205 genome-equivalents/ml of plasma (range: 39-5,845) and 1,033 genome-equivalents/ml of plasma (range: 254-5,116), respectively (p = 0.026). A significant positive correlation was observed between C-reactive protein levels and plasma cfDNA levels (r: 0.52, p < 0.0001). CONCLUSION: Our data have demonstrated for the first time that cfDNA is increased in children on PD treatment. However, the mechanism by which the levels of cfDNA is increased and the clinical significance of this finding in PD patients is unclear. Further studies are warranted to clarify the precise mechanism and clinical significance of elevated cfDNA in children on PD.

Henoch-Schönlein nephritis: a nationwide study.

BACKGROUND/AIM: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schönlein purpura (HSP) nephritis in children. METHODS: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). RESULTS: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. CONCLUSION: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely.

Fluid dynamics of gingiva and gingival health in children with end stage renal failure.

OBJECTIVES: Impaired homeostasis and fluid balance are important physiopathological alterations in patients with chronic renal failure which may adversely affect the fluid dynamics and health status of tissues and organs. There are insufficient data about this phenomenon in periodontal tissues. The aim of this study was to evaluate the fluid dynamics of gingiva in children with end stage renal failure (ESRF), correlating this entity with gingival health in the same patient group. DESIGN: Fifteen paediatric ESRF patients undergoing peritoneal dialysis (test group) and 15 systemically healthy children (control group) who were without periodontitis participated in the study. Fluid dynamics of gingiva were assessed via the gingival crevicular fluid (GCF) volume and tissue osmotic pressure (GOP) levels in the groups. GCF volume was measured using a Periotron 8000, whereas GOP was measured using a digital osmometer. Silness and Löe Plaque index (PI) and, Löe and Silness gingival index (GI) scores were utilized to determine the gingival health status in the study population. RESULTS: There were increases in the GCF volume and GOP of the test group compared to those of the control group (p<0.01). The PI and GI scores were higher in the test group than in the control group (p<0.01). Strong and positive correlations were found between GI and GCF volume, GI and GOP and, GCF volume and GOP in both groups (p<0.01). CONCLUSIONS: Our findings suggest that the fluid dynamics of gingiva may alter in children with ESRF, and this phenomenon may consequently affect the gingival health of these patients.

MRI of a benign chondroid syringoma.

Chondroid syringoma, also known as mixed tumour of the skin, is a relatively rare, usually benign tumour. A few malignant cases, especially in the lower extremities, have been published, but most of them behave in a benign fashion. A case of a middle-aged woman with surgically proved chondroid syringoma in the pretibial region is presented in this report with MRI and histological findings. Despite rapid growth over a short period of time and a location reportedly associated with malignancy, the histological features were benign.

Lipid peroxidation at various estradiol concentrations in human circulation during ovarian stimulation with exogenous gonadotropins.

The aim of our study was to investigate the correlation between serum malondialdehyde levels and serum estradiol concentrations in healthy human female subjects. Nine hundred and fifty-five blood samples, from infertile women undergoing controlled ovarian hyperstimulation treatment with recombinant follicle-stimulating hormone, were collected for estradiol and malondialdehyde measurements. Five groups were formed according to serum estradiol levels: Group I (< 50 pg/ml), group II (50 - 299 pg/ml), group III (300-999 pg/ml), group IV (1000-1999 pg/ml) and group V (> or = 2000 pg/ml). One-way analysis of variance was used for comparisons. Mean malondialdehyde concentrations were 1.74 +/- 0.24 mmol/ml (group I), 1.53 +/- 0.20 mmol/ml (group II), 1.69 +/- 0.24 mmol/ml (group III), 1.77 +/- 0.21 mmol/ml (group IV) and 1.86 +/- 0.20 mmol/ml (group V), respectively. Mean serum malondialdehyde level at physiological estradiol concentrations (50-199 pg/ml, group II) was significantly (p < 0.01) lower than the mean malondialdehyde levels in other groups. Mean malondialdehyde concentrations among the remaining groups did not significantly differ. Our findings suggest that in vivo lipid peroxidation might be increased when circulating estradiol concentrations are below (< 50 pg/ml) or above (> 300 pg/ml) the physiological limits. High blood estradiol levels in human female subjects during ovarian stimulation with exogenous gonadotropins could be associated with increased serum malondialdehyde concentrations.

Synchronous squamous cell carcinoma of the stomach, the lung and the skin.

BACKGROUND: The incidence of multiple primary cancers is reported to be 0.3-4.3%. CASE REPORT: A 70-year-old man was admitted to the hospital because of fatigue, weight loss, epigastric pain with melena. A biopsy taken from a tumoral lesion in the gastric corpus revealed squamous cell carcinoma. A nodular lesion was also detected on the nasal columella and biopsy from this lesion also showed squamous cell carcinoma. Computed tomography performed as a part of metastatic work-up revealed a lesion in the left pulmonary lower lobe. Bronchoscopic biopsy from this lesion also demonstrated squamous cell carcinoma. RESULTS: We report a case with synchronous squamous cell carcinoma of the lung, skin and stomach and discuss the multiple synchronous occurrence of carcinoma with the same histopathology in different sites with a review of the literature.

An unusual presentation of familial Mediterranean fever with prolonged hip pain and amyloidosis.

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limiting attacks of joint, chest and abdominal associated with fever. We present an unusual case of FMF with prolonged arthritis and amyloidosis. Familial Mediterranean fever should be considered in the differential diagnosis of prolonged hip pain, even in the absence of symptoms or signs of FMF.

Acute generalized exanthematous pustulosis induced by bleomycin and confirmed by patch testing.

Acute generalized exanthematous pustulosis is a potentially severe disease caused by drugs in about 90% of cases. To the best of our knowledge, acute generalized exanthematous pustulosis following use of bleomycin has not been previously reported. Here we report a case of acute generalized exanthematous pustulosis induced by bleomycin, which was confirmed by patch testing.

Gastric emptying time in renal transplant recipients treated with cyclosporine.

Gastric emptying time (GET) appears to be a rate-limiting factor in the absorption of cyclosporine-A (CsA) and may be responsible for intra- and interpatient variability of CsA bioavailability. Few studies have assessed gastric motility after renal transplantation. The purpose of this study was to evaluate gastric emptying of semi-solid material in stable renal transplant patients with reference to blood CsA levels. The GET of semi-solids (GET t(1/2), half emptying time) was measured in 16 transplant recipients who were taking CsA (Neoral), prednisolone and azathioprine (or mycophenolate mofetil). The GET (t(1/2)) measured by radionuclide methods, was analyzed with reference to the daily CsA doses, levels of CsA (C(0)), and serum creatinine concentrations. The mean GET (t(1/2)) was 89.1 +/- 26.4 minutes. Twelve patients exhibited delayed gastric emptying with a mean CsA level of 171.8 +/- 56 ng/mL and a mean dose of 4.1 +/- 1.1 mg/kg/d. The GET (t(1/2)) was not significantly correlated with the serum creatinine levels, the time since transplantation, or the CsA concentration. In addition, the correlation between the mean daily CsA dose and the GET (t(1/2)) was only weakly positive, (r =.33, P =.2) and therefore, statistically insignificant. In conclusion, it could not be ascertained whether a higher dose of CsA delays gastric emptying or whether patients with delayed emptying require higher doses of CsA. However, it is believed that determining the GET after transplantation helps in the adjustment of immunosuppressant doses.

An unusual form of generalized granuloma annulare in a patient with insulin-dependent diabetes mellitus.

The generalized form of granuloma annulare may be associated with systemic disorders, including diabetes mellitus. We describe here an unusual form of generalized granuloma annulare in a patient with complicated insulin-dependent diabetes mellitus. The cutaneous eruption had been present for decades as non-pruritic, persistent, violet-brown patches with raised edges. There were flexion deformities of the small joints of the hands and feet associated with thickening of the skin over dorsa of the fingers. The patient is currently on isotretinoin therapy, with partial resolution of lesions at 3 months follow-up.

Safety of inhaled corticosteroid therapy in young children with asthma.

BACKGROUND: Physicians have had some reluctance to use inhaled corticosteroids in very young children with asthma because of the possible risks of adverse systemic effects. OBJECTIVE: The purpose of this study was to evaluate the effects of fluticasone propionate on growth and adrenocortical function in young children with asthma. METHODS: We performed an open, prospective study for 24 weeks of 20 children with asthma, 2.5 to 5.0 years of age, who had received fluticasone by a large volume spacer at dosages ranging from 190.50 to 565.40 microg/m2 daily. Growth was evaluated by height standard deviation scores measured by a stadiometer. Adrenocortical function was evaluated twice in each child, before and after the study, by determining fasting serum cortisol concentrations at 8 AM and also at 30 and 60 minutes after adrenocorticotropic hormone stimulation. Posttreatment values of height standard deviation scores and fasting morning serum cortisol concentrations were compared with those of 18 age-matched children, who constituted the control group. RESULTS: The evaluation of mean +/- SEM (and range) of height standard deviation scores revealed a significant decrease from 0.44 +/- 0.27 (-1.46 to 2.22) to 0.28 +/- 0.26 (-1.51 to 2.07; P = 0.01) at week 18 and to 0.25 +/- 0.24 (-1.90 to 2.13; P = 0.04) at the week 24 in fluticasone-treated children. At the end of the treatment, however, height standard deviation scores of these children did not differ significantly (P = 0.35) from those of the control group. Delayed growth with medium-duration treatment was not associated with alterations in serum cortisol measurements, either at baseline or after stimulation. The mean fasting morning serum cortisol concentrations did not differ significantly between the fluticasone-treated patients and the control group. CONCLUSIONS: Some concern prevails about the safety of medium- or long-term treatment with regularly inhaled corticosteroids in young children with asthma. The prepubertal growth may be delayed, but the effect on ultimate height remains uncertain in such children. Growth should be regularly monitored in children who begin inhaled corticosteroid therapy for mild persistent asthma at an age <5 years old.

Hitherto undescribed venous vacuolar myopathy without mucoid degeneration in the varicose saphenous vein of a child.

Varicose vein abnormalities involving the lesser saphenous vein of a 7-year-old boy are presented. The histopathology of the vein wall revealed a vacuolar degeneration without mucoid deposits of the muscle cells, which has not been described previously in the literature in congenital varices.

Gianotti-Crosti syndrome preceded by oral polio vaccine and followed by varicella infection.

Gianotti-Crosti syndrome (papular acrodermatitis of childhood) is considered a nonspecific cutaneous host response to a variety of infectious agents, particularly viruses. Herein we report a case of Gianotti- Crosti syndrome preceded by oral polio vaccine and followed by varicella infection. The present case may support the hypothesis of initial immunization and following viral infection as a possible mechanism for Gianotti-Crosti syndrome.