Successful treatment of fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions

Background

Ecthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of the innate immunity, is an autosomal recessive disorder characterized in its severe phenotype by a complete defect in CD18 expression on neutrophils, delayed cord separation, chronic skin ulcers mainly due to recurrent bacterial and fungal infections, leucocytosis with high numbers of circulating neutrophils and an accumulation of abnormally low number of neutrophils at sites of infection.

Case Presentation

We report at our knowledge the first case of a child affected by LAD-1, who experienced during her disease course a multi-bacterial and fungal EG lesion caused by fusarium solani. Despite targeted antibiotics and anti-fungi therapy, the lesion extended for as long as 18 months and only massive granulocytes pockets transfusions in association with G-CSF had the capacity to cure this lesion.

Conclusion

We propose that granulocytes pockets transfusions will be beneficial to heal EG especially in severely immunocompromised patients.     

Optimizing venetoclax dose in combination with low intensive therapies in elderly patients with newly diagnosed acute myeloid leukemia: An exposure‐response analysis

The objective of this research was to characterize the venetoclax exposure‐efficacy and exposure‐safety relationships and determine its optimal dose in elderly patients with newly diagnosed acute myeloid leukemia (AML) receiving venetoclax in combination with low intensity therapies (hypomethylating agent [HMA; azacitidine or decitabine] or low‐dose cytarabine [LDAC]). A total of 212 patients from the HMA study and 92 patients from the LDAC study were included in the exposure‐safety analyses. Those who received at least one dose of venetoclax and had at least one measurable response (201 and 83 in the HMA and LDAC studies, respectively) were included in the exposure‐efficacy analyses. The probability of response based on International Working Group (IWG) for AML response criteria, adverse events of grade 3 or worse neutropenia or infection or a serious adverse event was modeled using logistic regression analyses to characterize the venetoclax exposure‐response relationships. In combination with an HMA, increasing concentrations of venetoclax, up to those associated with a less than or equal to 400‐mg once daily (QD) dose, were associated with a higher probability of response, with a trend for flat or decreasing probabilities of response thereafter. In combination with LDAC, increasing concentrations of venetoclax were associated with higher probabilities of response, with no plateau observed. Increasing concentrations of venetoclax were not associated with increasing probability of any safety event except for a slight increase in grade 3 or worse infections with HMAs; however, tolerability issues were observed at doses of greater than or equal to 800 mg QD in each study. Exposure‐response analyses support the use of venetoclax 400 mg QD in combination with an HMA and 600 mg QD in combination with LDAC (ie, the next highest dose evaluated below 800 mg in each combination) to safely maximize the probability of response in elderly patients with newly diagnosed AML.     

Forced vital capacity and cross-domain late-onset Pompe disease outcomes: an individual patient-level data meta-analysis

Journal of Neurology - Late-onset Pompe disease (LOPD) is a rare, metabolic disease primarily affecting the musculoskeletal and respiratory systems. Forced vital capacity (FVC) is commonly used to...     

Medium containing different concentrations of catalase as a strategy for optimising sperm parameters and chromatin in normospermic persons

The aim of this study was to comprise the effect of catalase on sperm parameters and chromatin in normospermic persons. Semen samples were obtained from fertile men. A certain amount of different concentrations of catalase (0.1, 1, 10, 50, 100, 150 and 200 IU.ml) was added to each vial containing semen. Control group had similar condition to treated groups without treatment. Treatment was done for one hour in incubator and 4 and 24 hr in room temperature. Sperm parameters (motility, viability and morphology ) and chromatin were evaluated after incubation. The results show that percentage of motility was insignificantly increased at concentration of 100 IU.ml catalase. This increase was higher than other examined concentration in all incubation time. The increase in sperm motility had significant difference in concentrations of 100 IU.ml with other concentrations. Other parameters showed no significant difference in all concentrations. Regarding the health of sperm chromatin, low concentrations of catalase had significant effect on this variable. This effect was more in low concentrations than high concentrations. This study showed the use of lower concentrations of antioxidant can improve the sperm parameters and chromatin quality. The low concentrations of catalase led to protection of chromatin and optimisation of sperm parameters.     

Prevalence of premature ejaculation and its impact on the quality of life: Results from a sample of Egyptian patients

Premature ejaculation is the most common sexual dysfunction affecting 25%–40% of males of different age groups, imposing psychological and physical burden and causing relationship problems. Because there is no standard definition of PE, there has been the variability in its prevalence data. Aims of this study were to detect the prevalence of premature ejaculation in a sample of Egyptian population and to assess the effect of premature ejaculation on the patients' quality of life and on the sexual function of their partners. Our results revealed that the prevalence of PE was 26.67%. Also, PE‐affected patients and their wives negatively causing dysfunctions in physiological, psychological, cultural and relationship dimensions.     

Comparison of local and intra venous dexamethasone on post operative pain and recovery after caeseream section. A randomized controlled trial

Objective

To compare the effect of local infiltration and intravenous dexamethasone on postoperative pain and recovery after Cesarean Section (CS).

Prognostic value of cortisol and thyroid function tests in poisoned patients admitted to toxicology ICU

BACKGROUND: Prognostic value of cortisol and thyroid function tests (TFTs) has previously been evaluated in medical ICUs. We aimed to evaluate prognostic efficacy of cortisol and TFTs in critically ill poisoned patients admitted to toxicology intensive care unit (ICU).METHODS: In a prospective study of consecutively enrolled subjects admitted to the toxicology ICU, lab analyses included TFTs (total T3 and T4 as well as TSH) and cortisol levels drawn between 8 am-10 am during period of the first 24 hours post-ingestion/exposure. Simplified Acute Physiology Score II (SAPS II) and Acute Physiology and Chronic Health Evaluation II (APACHE II) were recorded. All scores were compared to detect the best prognostic factor. Type of poisoning was also included.RESULTS: In 200 patients evaluated, 129 were male and mean age was 31 years. In general, SAPS II, T4, and cortisol could prognosticate death. After regression analysis, only cortisol had such efficacy (P=0.04; OR=1.06; 95%CI=1.05-1.08; cut-off=42 μg/dL; sensitivity=70%; specificity=82%). Between aluminium phosphide (ALP)- and non ALP-poisoned patients, level of consciousness, mean arterial pressure, and cortisol level could prognosticate death in ALP poisoning (all Ps<0.001 in both uni and multivariate analyses). Median (interquartile range; IQR) GCS was 7 (6, 10) and 15 (8, 15) in non-ALP and ALP-poisoned patients (P<0.003). SAPS II and APACHE II could not prognosticate death at all.CONCLUSION: Cortisol best prognosticated outcomes for subjects admitted to the toxicology ICU. Its level is higher in ALP-poisoned patients probably due to the higher stress while they remain conscious till the final stages of toxicity and are aware of deterioration of their clinical condition or may be due to their significantly lower blood pressures.     

Low iris and anterior chamber volume is associated with deepening after laser peripheral iridotomy in primary angle closure suspects

Graefe's Archive for Clinical and Experimental Ophthalmology - To evaluate the association between baseline ocular variables and the widening of the anterior chamber angle by laser peripheral...     

An investigation on optic nerve head involvement in Fuchs uveitis syndrome using optical coherence tomography and fluorescein angiography

Graefe's Archive for Clinical and Experimental Ophthalmology - To investigate optic nerve head involvement in patients with Fuchs uveitis syndrome (FUS). Optic nerve head of 43 FUS eyes without...