Visceral basidiobolomycosis: An overlooked infection in immunocompetent children

Visceral basidiobolomycosis is an unusual fungal infection of viscera caused by saprophyte Basidiobolus ranarum. It is very rare in healthy children and poses a diagnostic challenge due to the non-specific clinical presentation and the absence of predisposing factors. We report a case of gastrointestinal basidiobolomycosis in a 4-year-old healthy girl who presented with a short history of abdominal pain, bleeding per rectum, fever, and weight loss. The diagnosis was based on high eosinophilic count, classical histopathology findings of fungal hyphae (the Splendore-Hoeppli phenomenon), and positive fungal culture from a tissue biopsy. Fungal infection was successfully eradicated with a combined approach of surgical resection of the infected tissue and a well-monitored course of antifungal therapy. The atypical clinical presentation, diagnostic techniques, and the role of surgery in the management of a rare and lethal fungal disease in an immunocompetent child are discussed.Key words: Basidiobolomycosis, child, fungal infection, gastrointestinal, immunocompetent     

Vascular remodeling and plaque composition between focal and diffuse coronary lesions assessed by intravascular ultrasound

Coronary remodeling and plaque composition were compared between focal and diffuse coronary lesions. Negative remodeling and fibrous and calcified plaque compositions contribute to stenosis development in diffuse lesions more frequently than in focal lesions.     

The association of primary hyperparathyroidism and primary ovarian failure: a de novo t(X; 2) (q22p13) reciprocal translocation

CASE: A 40-year-old female presented with primary amenorrhoea at 17 years of age. She was tall at 98th centile for height with eunuchoidal body habitus. Her breast development was Tanner stage 3, pubic and axillary hair Tanner stage 4 with normal external genitalia. Her bone age was 13.4 years at a chronological age of 17.8 years. Gonadotrophins were elevated indicating primary ovarian failure. A diagnostic laparotomy revealed hypoplastic, infantile uterus with bilateral streak gonads. Chromosomal analysis showed a balanced reciprocal translocation 46X, t(X; 2) (q22 p13). She became pregnant by in vitro fertilization with egg donation at the age of 36 years. At 13 weeks of gestation, she presented with intractable vomiting. She had raised corrected serum calcium and parathyroid hormone concentrations consistent with the diagnosis of primary hyperparathyroidism (PHPT). She underwent parathyroidectomy at 24 weeks of gestation with removal of a large left inferior parathyroid adenoma which normalized her serum calcium. Multipoint linkage from a genome-wide screen has identified a region of suggestive linkage on chromosome 2p13.3-14 in some cases of familial isolated hyperparathyroidism (FIHP). CONCLUSION: To our knowledge, this is the first case of primary amenorrhoea due to reciprocal translocation involving chromosome 2 and the X chromosome associated with PHPT. PHPT in this case is most likely to be as a result of chromosome 2 involvement where a locus for FIHP has been identified. Identification of the gene involved on chromosome 2p13.3-14 will be of considerable interest.     

Prevalence of Pulmonary Hypertension and its Influence on Survival in Patients With Advanced Chronic Obstructive Pulmonary Disease Prior to Lung Transplantation

Introduction: Prevalence of pulmonary hypertension (PH) and its influence on survival in chronic obstructive pulmonary disease (COPD) are not well studied in the lung allocation score (LAS) era.

Methods: The UNOS database was queried from 2005 to 2013 to identify first-time adult lung transplant candidates with COPD who were tracked from wait list entry date until death or censoring to determine both prevalence and influence of PH. Using right heart catheterization measurements, mild PH was defined as mean pulmonary artery pressure (mPAP) ≥ 25 mmHg and severe ≥ 35 mmHg.

Results: Of 1315 COPD candidates not transplanted, 1243 were used for survival analysis using Cox proportional hazards models, and 1010 (mild PH) and 244 (severe PH) were used for propensity score matching, respectively. A total of 52% (652) of subjects had PH mPAP ≥ 25 mmHg. Univariate analysis revealed significant differences in survival for mild PH (HR = 1.769; 95% CI: 1.331, 2.351; p < 0.001) and severe PH (HR = 3.271; 95% CI: 2.311, 4.630; p < 0.001). Kaplan–Meier survival function demonstrated significant disparities for mild PH (Log-rank test: Chi-square₁: 15.87, p < 0.0001) and severe PH (Log-rank test: Chi-square₁: 50.13, p < 0.0001). Multivariate Cox models identified significant risk for death for mild PH (HR = 1.987; 95% CI: 1.484, 2.662; p < 0.001) and severe PH (HR = 3.432; 95% CI: 2.410, 4.888; p < 0.001). Propensity score matching confirmed increased mortality hazard associated with mild PH (HR = 2.280; 95% CI: 1.425, 3.649; p = 0.001) and severe PH (HR = 7.000; 95% CI: 2.455, 19.957; p < 0.001).

Conclusions: PH is highly prevalent in advanced COPD and associated with a significantly higher risk for mortality.     

Prevalence and causes of blindness and diabetic retinopathy in Southern Saudi Arabia

Objectives:

To determine the prevalence and causes of blindness and diabetic retinopathy (DR) in Jazan district, Southern Saudi Arabia.

Methods:

Using the standardized Rapid Assessment for Avoidable Blindness (RAAB) and DR cross-sectional methodology, 3800 subjects were randomly selected from the population of ≥50 years of age in Jazan, Saudi Arabia between November 2011 and January 2012. Participants underwent screening comprised of interview, random blood glucose test, and ophthalmic assessment including visual acuity (VA) and fundus examination. Among participants with VA <6/18 in either eye, the cause(s) of visual impairment was determined. Participants were classified as diabetic if they had previous diagnoses of diabetes, or random blood glucose >200 mg/dl. Diabetic participants were assessed for DR using dilated fundus examination. All data were recorded using the RAAB + DR standardized forms.

Results:

The prevalence of bilateral blindness <3/60 was 3.3% (95% confidence interval [CI]: 2.74 - 3.90). Cataract was the leading cause of blindness (58.6%); followed by posterior segment diseases (20%), which included DR (7; 3.3%). The prevalence of diabetes mellitus (DM) was 22.4%, (95% CI: 21.09 - 23.79), among them; 27.8% had DR. The prevalence of sight-threatening DR was 5.7%.

Conclusion:

The prevalence of DM and the corresponding proportion of DR in this region is lower than that reported in other regions of Saudi Arabia. However, the prevalence of blindness not related to DR is relatively higher than the other studies.The Kingdom of Saudi (KSA) ranks seventh in the global burden of diabetes mellitus (DM), with an estimated prevalence of 23.5% for age groups 20-79 years.1 Ocular complications are quite common among diabetic patients. It is well established that within 15 years of diabetes approximately 2% of diabetics may turn legally blind, and approximately 10% may develop severe visual impairment. Diabetic retinopathy (DR) is one of the serious potential complications. It occurs in approximately 77% of the type 2 diabetics within 10 years of the diabetes onset, and almost in all type 1 diabetics.2 A global review of diabetic retinopathy reported that on average, 34.6% of all diabetic patients have some forms of DR.3 Recent studies in KSA have reported a high prevalence of DR among diabetics in different regions of the country. A recent population based study in Taif,4 in the Western region of KSA reported that 33% of all diabetics have some form of DR; while another hospital based study in the Madinah region reported the same estimate at 36%.5 With this high burden of the disease, the Saudi Ministry of Health (MoH) in collaboration with the Saudi National Prevention of Blindness Committee (NPBC) commissioned more studies to determine the magnitude of the problem in other regions of the vast country. Thus, a population-based survey was conducted to estimate both prevalence and pattern of DR, in addition to the magnitude and causes of blindness and visual impairment in the Jazan district, in the Southwestern region of KSA. Jazan covers an area of 11,670 Km², and has a population of 1,533,496 inhabitants. It lies to the Southwest coast of the Red Sea and is bordered by Yemen to the south. The study adopted the Rapid Assessment for Avoidable Blindness and Diabetic Retinopathy (RAAB+DR) technique, which is a survey methodology developed by the International Centre for Eye Health, London School of Hygiene and Tropical Medicine (ICEH-LSHTM), London, United Kingdom.6 The RAAB+DR is a simple and cost effective cross-sectional community-based survey of persons 50 years and older, that focuses primarily on the prevalence of avoidable blindness. It estimates the prevalence of blindness and visual impairment, their causes, and magnitude of DR in a specific geographical area, usually at the district, or province level. The RAAB+DR methodology has concrete proven reliability and validity.4,7     

Contrast-induced nephropathy after percutaneous coronary interventions in relation to chronic kidney disease and hemodynamic variables

We previously found that contrast-induced nephropathy (CIN) complicating percutaneous coronary intervention adversely affects patients with chronic kidney disease (CKD). Therefore, we further investigated whether the predictors and outcome of CIN after percutaneous coronary intervention differ among patients with versus without CKD. Among 7,230 consecutive patients, CIN (>or=25% or >or=0.5 mg/dl increase in preprocedure serum creatinine 48 hours after the procedure) developed in 381 of 1,980 patients (19.2%) with baseline CKD (estimated glomerular filtration rate [eGFR] <60 ml/min/1.73 m(2)) and in 688 of 5,250 patients (13.1%) without CKD. Decreased eGFRs, periprocedural hypotension, higher contrast media volumes, lower baseline hematocrit, diabetes, pulmonary edema at presentation, intra-aortic balloon pump use, and ejection fraction <40% were the most significant predictors of CIN in patients with CKD. Apart from intra-aortic balloon pump use, predictors of CIN in patients without CKD were the same as mentioned, plus older age and type of contrast media. Regardless of baseline renal function, CIN correlated with longer in-hospital stay and higher rates of in-hospital complications and 1-year mortality compared with patients without CIN. By multivariate analysis, CIN was 1 of the most powerful predictors of 1-year mortality in patients with preexisting CKD (odds ratio 2.37, 95% confidence interval 1.63 to 3.44) or preserved eGFR (odds ratio 1.78; 95% confidence interval 1.22 to 2.60). Thus, regardless of the presence of CKD, baseline characteristics and periprocedural hemodynamic parameters predict CIN, and this complication is associated with worse in-hospital and 1-year outcomes.     

Significance of serum levels of vitamin D and some related minerals in breast cancer patients

Vitamin D and calcium are involved in a wide range of proliferation, apoptosis and cell signaling activities in the body. Suboptimal concentrations may lead to cancer development. The role of phosphate in cancer metabolism is particularly relevant in breast cancer while, magnesium deficiency favors DNA mutations leading to carcinogenesis. Objectives: To determine serum levels of vitamin D, calcium, phosphorus, magnesium, and parathormone in female breast cancer patients and to assess their association with some prognostic factors in breast cancer. Design and methods: This study is done on 98 newly diagnosed female breast cancer patients and 49 age matched apparently healthy female volunteers as controls. Serum samples from all patients and controls were subjected to 25-OH Vit D, calcium, phosphorus, magnesium, and parathormone measurements. Results: In the breast cancer group, the median serum levels of 25-OH Vit D were 15 ng/ml, while it was 21 ng/ml in the control group. Levels of 25-OH Vit D and other tested minerals were significantly lower while calcium:magnesium (Ca:Mg) ratio, and calcium:phosphorus (Ca:P) ratio were significantly higher in the breast cancer group. Significant negative correlation was detected between phosphorus and calcium, ionized calcium , calcium magnesium ratio, and calcium phosphorus ratio. Conclusion: It is not only the deficient levels of Vit D and other related minerals, but the combination of the abnormal levels of all the studied parameters that might contribute to the development of cancer. Further studies with larger number of patient are needed.