Seronegative celiac disease with transient protein-losing enteropathy and vitamin B12 deficiency in a pediatric patient: Case report.

Key Clinical Message: Early recognition and management of seronegative celiac disease, even in the absence of typical serological markers, can prevent complications and ensure better health outcomes in pediatric patients. Consideration of a gluten-free diet in similar cases can lead to significant clinical improvement. Abstract: Celiac disease, characterized by its diverse clinical manifestations, often necessitates adherence to a gluten-free diet, particularly in pediatric patients for optimal growth and development. This report presents the case of an 11-year-old male who exhibited recurrent symptoms of fever and diarrhea progressing to edema and pallor, with a history dating back to age 3. Laboratory findings revealed pancytopenia, hypoalbuminemia, and proteinuria. Despite negative serological markers, noninvasive tests, along with clinical improvement on a gluten-free diet and supportive measures within a month, suggested celiac disease complicated by transient protein-losing enteropathy and vitamin B12 deficiency. It is important to note that other malabsorption disorders can also show clinical improvement following a gluten-free diet. Additionally, the antibiotic treatment received by the patient could have addressed other possible causes of malabsorption, complicating the differential diagnosis. This case highlights the importance of early recognition and management of celiac disease, especially in pediatric patients, to prevent complications and promote optimal health outcomes.

Computed Tomography-Derived Extracellular Volume Fraction and Splenic Size for Liver Fibrosis Staging.

OBJECTIVE: Extracellular volume fraction (fECV) and liver and spleen size have been correlated with liver fibrosis stages and cirrhosis. The purpose of the current study was to determine the predictive value of fECV alone and in conjunction with measurement of liver and spleen size for severity of liver fibrosis. METHODS: This was a retrospective study of 95 subjects (65 with liver biopsy and 30 controls). Spearman rank correlation coefficient was used to assess correlation between radiological markers and fibrosis stage. Receiver operating characteristic analysis was performed to assess the discriminative ability of radiological markers for significant (F2+) and advanced (F3+) fibrosis and cirrhosis (F4), by reporting the area under the curve (AUC). RESULTS: The cohort had a mean age of 51.4 ± 14.4 years, and 52 were female (55%). There were 36, 5, 6, 9, and 39 in fibrosis stages F0, F1, F2, F3, and F4, respectively. Spleen volume alone showed the highest correlation (r = 0.552, P < 0.001) and AUCs of 0.823, 0.807, and 0.785 for identification of significant and advanced fibrosis and cirrhosis, respectively. Adding fECV to spleen length improved AUCs (0.764, 0.745, and 0.717 to 0.812, 0.781, and 0.738, respectively) compared with splenic length alone. However, adding fECV to spleen volume did not improve the AUCs for significant or advanced fibrosis or cirrhosis. CONCLUSIONS: Spleen size (measured in length or volume) showed better correlation with liver fibrosis stages compared with fECV. The combination of fECV and spleen length had higher accuracy compared with fECV alone or spleen length alone.

Evaluating the role of ivabradine in acute decompensated heart failure: A systematic review and meta-analysis.

BACKGROUND: Acute decompensated heart failure (ADHF) presents a significant global health challenge, with high morbidity, mortality, and healthcare costs. The current therapeutic options for ADHF are limited. Ivabradine, a selective inhibitor of hyperpolarization-activated cyclic nucleotide-gated (HCN) channels, has emerged as a potential therapy for ADHF by reducing the heart rate (HR) without negatively affecting myocardial contractility. However, the evidence regarding the efficacy and safety of ivabradine in patients with ADHF is limited and inconsistent. This meta-analysis aimed to evaluate the efficacy and safety of ivabradine for ADHF based on observational studies. METHODS: A systematic literature search was conducted following PRISMA guidelines to identify relevant observational studies comparing ivabradine with placebo in adult patients with ADHF. Data were pooled using a random-effects model, and heterogeneity was assessed. The risk of bias was evaluated using the Newcastle-Ottawa Scale. RESULTS: Four observational studies comprising a total of 12034 patients. Meta-analysis revealed that ivabradine significantly reduced all-cause mortality (RR: 0.66, 95 % CI: 0.49-0.89, p < 0.01) and resting HR (MD: -12.54, 95 % CI: -21.66-3.42, p < 0.01) compared to placebo. However, no significant differences were observed in cardiovascular mortality, hospital readmission for all causes, changes in LVEF, or changes in LVEDD. Sensitivity and publication bias assessments were conducted for each outcome. CONCLUSION: Ivabradine may be beneficial for reducing mortality and HR in patients with ADHF. However, its impact on other clinical outcomes such as cardiovascular mortality, hospital readmission, and cardiac function remains inconclusive. Further research, particularly well-designed RCTs with larger sample sizes and longer follow-up durations, are warranted.

Revolutionary breakthrough: FDA approves Vyjuvek, the first topical gene therapy for dystrophic epidermolysis bullosa.

This article provides an updated overview of Vyjuvek, a Food and Drug Administration (FDA) approved medication and its potential in managing dystrophic epidermolysis bullosa (DEB). DEB is a rare genetic disorder characterized by skin fragility, blistering, wounds, and scarring. The underlying cause of DEB is the impaired production of type VII collagen (COL7), leading to weakened anchoring fibrils in the skin. Vyjuvek is the first topical gene therapy for DEB, utilizing a genetically modified HSV-1 (herpes simplex virus 1) vector to express human COL7 and promote wound healing. Clinical trials have shown that Vyjuvek increases the probability of complete wound healing compared to placebo. Although further research is needed, Vyjuvek represents a significant advancement in addressing the unmet medical needs of patients with DEB, offering hope for improved quality of life and long-term complication reduction.

Herpes Simplex Virus Meningoencephalitis Following Pulse-Dose Methylprednisolone: A Case Report and Literature Review.

BACKGROUND Several cases of herpes simplex virus type 1 meningoencephalitis (HSVE) have been reported in patients receiving steroids, but the exact contribution of steroids to the disorder remains unclear because other risk factors, such as chemotherapy, brain radiation, or surgery, were present in almost all cases. CASE REPORT We report the case of a 76-year-old man who developed HSVE following the administration of pulse-dose steroids. The patient had occupational asbestos exposure and a chronic interstitial lung disease of unclear etiology (sarcoidosis versus hypersensitivity pneumonitis) and was admitted for acute-on-chronic respiratory failure requiring mechanical ventilation. After a negative infectious workup and several days of antibiotics without improvement, pulse-dose steroids were administered. In the following days, the patient developed a fever and worsening encephalopathy. A lumbar puncture showed elevated nucleated cells and positive polymerase chain reaction for herpes simplex virus 1 in the cerebrospinal fluid, confirming the diagnosis of HSVE. Acyclovir treatment was initiated, but the patient later died as a result of persistent severe encephalopathy and respiratory failure with an inability to wean mechanical ventilation. CONCLUSIONS Clinicians should keep in mind that HSVE is a potential complication of steroids and carefully consider the benefit/risk ratio of pulse-dose steroids, taking into account associated factors of immunosuppression. A high level of awareness should be especially maintained in critically ill patients because of associated risk factors (critical illness immune paralysis) and because neurological signs of HSVE may be missed in mechanically ventilated, sedated patients.

Staged repair of proximal hypospadias: Reporting outcome of staged tubularized autograft repair (STAG).

INTRODUCTION: Proximal hypospadias (PPH) repair is a challenge. Dilemma exists whether to do it in single or staged repair. Staged repair is our adopted procedure which was recently modified by Snodgrass into staged tubularized autograft repair (STAG), in which attention was given to ventral straightening of the penis together with some other technical details. Herein, we report our experience with STAG in a cohort of primary posterior hypospadias. PATIENTS AND METHODS: In the period from 2011 to 2018 we operated 43 primary posterior hypospadias. Two principal surgeons (HB, MY) and multiple assistants operate children the same way, and data are recorded in a prospectively designed data base. In all children, inner prepuce graft was utilized, when curvature is more than 30 degrees, plate transection with or without ventral corporotomies were adopted. RESULTS: Forty-three children with PPH and ventral curvature more than 30 degrees underwent first stage with median age 12 months (6-132 IQR16). Penile curvature was corrected by plate transection in 27 children (62.8%), ventral corporotomies in 16 children (37.2%). Graft take was successful in 90.7%, 4 children needed revision of fibrotic graft. Second stage was completed in 37 children, success was 56.8%, 21.6% fistula, 24.3% glanular dehiscence. Overall success after third surgery to correct complications was 78.4%. In a mean follow up of 3.2 years, we had recurrence of curvature in 2 children taking success rate to 72.9%. No meatal stenosis, no diverticulum, no stricture, no urethral dehiscence was encountered. Cosmetic appearance was excellent in follow up. CONCLUSION: STAG achieves proper straightening of the penis and allows for reconstruction of a good urethra, yet urethrocutaneous fistula and glanular dehiscence remain the main complications. Follow up is important to address results of ventral corporotomies. TYPE OF STUDY: Therapeutic. LEVEL OF EVIDENCE: Level IV case series with no comparison group.

First experience of the Egyptian National Cancer Institute using the robot-assisted laparoscopic approach in radical hysterectomies for cervical cancer.

AIM OF WORK: Robotics in surgery led to an improvement of visualization, a better handling of tissues and better suturing. This study aimed to document the first experience of the Egyptian National Cancer Institute (NCI) using the robot-assisted laparoscopic approach in radical hysterectomies for cervical cancer and to highlight observed advantages, disadvantages, morbidity and oncological outcomes. PATIENTS AND METHODS: Data of cases that had either early cervical cancer (stage IB-IIA1 with a tumor ≥2 cm) or locally advanced cervical cancer (Stage IIA2-IIB after chemo-radiotherapy) were collected prospectively. Study patients underwent robotic radical hysterectomies for their cervical cancers at the NCI, Cairo University, between January 1, 2015 and December 31, 2016. For each patient, duration of surgery, amount of blood loss, and intra-operative complications were recorded. Similarly, the duration of postoperative hospital-stay, analgesia used and post-operative gastrointestinal recovery were documented. Pathological assessment of safety margins and the lymph nodes number yield were also assessed. RESULTS: Twenty patients underwent robotic radical hysterectomy during the study period. Twelve cases had early cervical cancer while 8 suffered locally advanced disease. The mean procedure time was 319 (range 240-560) minutes; the mean blood loss was 309 (range 150-600) ml. Three cases had bladder injuries during their procedures. The median hospital stay was 6 (range 4-10) days. One case had a positive margin. The median of lymph nodes yield number was 15 (range 10-25). Follow-up ranged 9-31 months, with only one case developing local recurrence. CONCLUSION: Robotic radical hysterectomy is a feasible approach with a tolerable rate of complications.

A search for new otomycotic species and their sensitivity to different antifungals.

BACKGROUND: Otomycosis is a common ear problem in countries with hot and humid climate. Emergence of new fungal species is a possibility particularly in patients with chronic illness or who receives antibiotics either systemically or topically. AIM: To identify the otomycotic species, which are responsible for developing the otomycosis. METHODS: A descriptive study was carried out in 63 patients who were clinically diagnosed with otomycosis. Swabs were taken from the fungal debris to identify the causative agent and to determine the effective antifungals against it. RESULTS: Aspergillus species were the most common agents and were found in 47 patients (74.6%), represented by A. fumigatus (36.5%), A. niger (27%), A. flavus (6.5%), and A. terreus (4.8%). Ketoconazole (90%) and miconazole (76%) were the most effective antifungals against the species obtained from cultures, whereas fluconazole was the least effective. CONCLUSIONS: Although there is no change in the mycology of otomycosis, there is still a significant variability in the isolated species and in their sensitivity to antifungal drugs. Ketoconazole exhibited broad spectrum effect against fungal isolates in this study, whereas fluconazole was the least efficacious.

Studies on Behaviors of Interactions Between New Polymer-based ZIC-HILIC Stationary Phases and Carboxylic Acids.

Zwitterionic stationary phases with nearly identical capacities were prepared by graft polymerization of a series of sulfobetaine precursors onto the surface of porous PS/DVB particles. The different spacer lengths are used as an investigative tool for the retention behavior of carboxylic acids; namely malonic, succinic, glutaric and maleic acid. In zwitterionic ion chromatography-hydrophilic interaction liquid chromatography (ZIC-HILIC) separation mode, the retention characteristic of carboxylic acids was examined using sodium acetate/acetonitrile eluents and UV detection. The retention is based on partitioning in reversed as well as in HILIC mode and zwitterionic ion exchange resulting in a mixed separation mode for the carboxylic acids. This ion exchange behavior has never been observed before for sulfobetaine-based zwitterionic stationary phases.

Preliminary results of robotic colorectal surgery at the National Cancer Institute, Cairo University.

BACKGROUND: The available literature on minimally invasive colorectal cancer demonstrates that laparoscopic approach is feasible and associated with better short term outcomes than open surgery while maintaining equivalent oncologic safety. Reports have shown that robotic surgery may overcome some of the pitfalls of laparoscopic intervention. OBJECTIVE OF THE WORK: To evaluate early results of robotic colorectal surgery, in a cohort of Egyptian patients, regarding operative time, operative and early post-operative complications, hospital stay and pathological results. PATIENTS AND METHODS: A case series study which was carried out in surgical department at National Cancer Institute, Cairo University. Ten Egyptian cases of colorectal cancer (age ranged from 30 to 67, 5 males and 5 females) were recruited from the period of April 2013 to April 2014. Robotic surgery was performed to all cases. RESULTS: Three patients had low anterior resection, three anterior resection, one total proctectomy, one abdominoperineal resection, one left hemicolectomy and one colostomy. The study reported no mortalities and two morbidities. The mean operative time was 333min. The conversion to open was done in only one patient. A total mesorectal excision with negative circumferential margin was accomplished in all patients, distal margin was positive in one patient. Mean lymph nodes removed was 10.7. Mean hospital stay was 7.4days. CONCLUSION: To the best of our knowledge, this is the first study reporting the outcomes of robotic colorectal cancer intervention in Egyptian patients. Our preliminary results suggest that robotic-assisted surgery for colorectal cancer can be carried out safely and according to oncological principles.

Color image segmentation based on different color space models using automatic GrabCut.

This paper presents a comparative study using different color spaces to evaluate the performance of color image segmentation using the automatic GrabCut technique. GrabCut is considered as one of the semiautomatic image segmentation techniques, since it requires user interaction for the initialization of the segmentation process. The automation of the GrabCut technique is proposed as a modification of the original semiautomatic one in order to eliminate the user interaction. The automatic GrabCut utilizes the unsupervised Orchard and Bouman clustering technique for the initialization phase. Comparisons with the original GrabCut show the efficiency of the proposed automatic technique in terms of segmentation, quality, and accuracy. As no explicit color space is recommended for every segmentation problem, automatic GrabCut is applied with RGB, HSV, CMY, XYZ, and YUV color spaces. The comparative study and experimental results using different color images show that RGB color space is the best color space representation for the set of the images used.

The diagnostic efficacy of urinary survivin and hyaluronidase mRNA as urine markers in patients with bladder cancer.

BACKGROUND: A new, sensitive, noninvasive method for the detection of urothelial carcinomas of the bladder would open new possibilities in both the diagnosis and follow up of patients. METHODS: Voided urine specimens were collected from patients with histologically confirmed bladder urothelial carcinoma (Group 1: n = 60), urological patients without urothelial carcinoma (Group 2: n = 20), and healthy volunteers (Group 3: n = 20). All underwent serological assessment of schistosomiasis antibody, quantitative measurement of survivin by ELISA in urine supernatant, urine cytology, and detection of hyaluronidase (HYAL-1) by RT-PCR in urothelial cells of voided urine samples. RESULTS: Urinary survivin mean rank was higher in malignant and benign groups than in the healthy group (p < 0.001). Urinary survivin best-cutoff was determined using receiver operating characteristic curve to discriminate between malignant and nonmalignant groups (2537.25 pg/mg protein) at 78.33% sensitivity and 82.5% specificity. HAase mRNA showed superior sensitivity (86.67%) over cytology (38.33%) and urinary survivin (78.33%) with specificity of 97.5%, 100%, and 82.5%, respectively. The sensitivity of urine cytology was increased on combination with either survivin (83.33%) or HAase (90%). Also, the combination of both markers increased overall sensitivity (95%). CONCLUSIONS: Survivin can be reliably and quantitatively measured in urine of bladder cancer patients, improving the sensitivity and specificity of urine cytology for the diagnosis of bladder cancer. Combined use of cytology with survivin and HAase was the best recommended combination for bladder cancer detection.

The role of streptokinase in induction of posterior vitreous detachment: a scanning and transmission electron microscopic study of the retina in rabbits.

PURPOSE: To evaluate the possibility of inducing a posterior vitreous detachment (PVD) by intravitreal injection of streptokinase using electron microscopy and electrophysiological study. METHODS: The current study was performed on 30 eyes of 15 male white rabbits. The rabbits were divided into three equal groups. The right eye of the three groups received an intravitreal injection of streptokinase at three different concentrations (150, 1,500 and 15,000 IU in 0.1 mL balanced salt solution) in the mid vitreous cavity. The left eye in all animals received an intravitreal injection of balanced salt solution and was considered the control group. Electroretinography was performed 1 day and 1 week after injection. The rabbits were killed after 10 days, and the enucleated eyes were processed for transmission and scanning electron microscopic examination. RESULTS: In Group 1, scanning electron microscopy showed the retinal surface covered with thin collagen fibers, whereas in Group 2, a complete PVD with bare retinal surface was seen. Group 3 showed a bare retinal surface with hemorrhagic reaction and toxic effects on the retina by transmission electron microscopy. CONCLUSION: An intravitreal injection of 1,500 IU of streptokinase can lead to a PVD without major toxic effects on the retina.

Efficacy of increased gentamicin concentration for intratympanic injection therapy in Ménière's disease.

OBJECTIVES: Update of ongoing case series of intratympanic gentamicin use in intractable Ménière's disease. Comparison of the treatment results of two gentamicin concentrations: 30 mg/ml and 40 mg/ml. STUDY DESIGN: Retrospective case-matched review of an ongoing protocol. SETTING: Ambulatory visits in an office setting. PATIENTS: Eighty-seven patients were included in this study according to the 1995 guidelines of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) for reporting treatment results of Ménière's disease. INTERVENTION: Intratympanic injection of a buffered gentamicin solution (30 mg/ml) was used in 44 patients (Group 1) in years 1992 to 1995. Stock gentamicin solution (40 mg/ml) was used in 43 patients (Group 2) in years 1996 to 1999. Treatment was titrated for each patient, and the hearing was strictly monitored. The endpoint of treatment was the complete cessation of vertigo spells. MAIN OUTCOME MEASURES: Hearing results, vertigo control scores, and ice water caloric testing responses were analyzed after 24 months of follow-up. Thirty-two patients from each group were selected for case-matched statistical analysis. RESULTS: Four or fewer gentamicin injections were used in 70% of Group 1 patients and 96% of Group 2 patients. In Group 1, vertigo control was achieved in 81% of patients, and the hearing remained the same or was improved in 68% of patients. In Group 2, vertigo control was achieved in 72% of patients, and the hearing remained the same or was improved in 81% of patients. Treatment was aborted in four patients of Group 2 for early reversible hearing loss, and 31 of 39 patients (79%) who completed the protocol experienced lasting vertigo control. There was a trend for longer-lasting vertigo control in the Group 2 patients. CONCLUSION: It was concluded that increasing the gentamicin concentration to 40 mg/ml probably produces similar rates of vertigo control as those of the lower dose, at least initially, but requires fewer injections. The higher dose did not increase the risk of hearing loss if treatment was stopped at the first indication of injury. A larger study is needed to confirm the trend of improved long-term hearing results in patients treated with the 40 mg/ml solution.