RESUMEN
Cutis laxa syndrome is an uncommon connective tissue disorder affecting the major ultrastructure of the skin by progressive loss of elasticity. The results of this syndrome lead to the appearance of premature aging, which might also affect the internal organs. The disorder can be either congenital or acquired. The congenital form consists of autosomal dominant, autosomal recessive, and X-linked recessive patterns. The autosomal recessive pattern is the most common and severe one. Different systemic complications have been described in congenital cutis laxa syndrome, but the most serious and lethal one is cardiopulmonary abnormalities. In this report, we discuss the presentation of congenital cutis laxa syndrome with successful cardiovascular surgical management of multiple valvular heart diseases.
RESUMEN
Background The placenta is a temporary pivotal organ - the gate between the mother and the fetus. It has multiple functions such as nutrient uptake, elimination of waste products, gas exchange, and production of some vital hormones. However, the pregnancy state is a diabetogenic condition caused by insulin resistance, resulting from physiological variations. Gestational diabetes mellitus (GDM) can have an impact on both the mother and the fetus by causing numerous complications. In our research study, we aim to study and compare the quantitative effect of GDM at the microscopical level within the chorionic villi of the placenta of both mothers diagnosed with GDM and healthy mothers as well as the clinical correlation. Methods After applying the inclusion and exclusion criteria, we collected 84 placental samples from February 2017 until May 2017, which were composed of 42 GDM samples and 42 healthy samples. All of these samples have been studied under a light microscope for measuring different parameters. Results We found that some of the measured parameters among diabetic villi were lower than those of healthy villi with a p-value < .05 being significant. These include the surface area of the blood vessels (P = .008), the perimeters of the blood vessels (P = .002), the placental barrier thickness/perimeters of the villous blood vessels ratio (P ≤ .001), the placental barrier thickness/surface area of the blood vessels ratio (P ≤ .001), the number of Hofbauer cells/surface area of the villous ratio (P ≤ .001), the number of the blood vessels/surface area of the villous ratio (P = .001), the surface area of the blood vessels/surface area of the villous ratio (P = .004), and the perimeters of the blood vessels/surface area of the villous ratio (P ≤ .001). These parameters have significant effects on fetal development as well as the mother's status. Conclusions GDM is associated with multiple changes in the placenta. Moreover, these changes can impact the fetoplacental circulation and cause multiple complications for the mother and the fetus. Therefore, identifying pregnant women with GDM and controlling hyperglycemia will improve the outcomes of the pregnancy.
RESUMEN
Glucose-6-phosphate dehydrogenase (G-6-PD) is the major enzyme in the pentose phosphate pathway (PPP). The end products of this pathway are ribose-5-phosphate and nicotinamide adenine dinucleotide phosphate hydrogen (NADPH). G-6-PD deficiency is known to be the most common enzymatic deficiency in red blood cells (RBCs). Genetically, the mode of inheritance is an X-linked recessive disease. The exposure to oxidative stressors will result in hemolytic anemia including fava beans, infections, metabolic conditions such as diabetic ketoacidosis, metabolic acidosis, hyperglycemia, hypoglycemia, and hypothermia. Moreover, surgical stress and certain types of medication are known to lead to hemolytic anemia. Acute hemolytic crisis is a life-threatening situation in patients with G-6-PD deficiency. Therefore, it is extremely important to monitor the patient perioperatively. The authors present this case of successful anesthetic management in a 23-year-old lady with G-6-PD deficiency and a previous history of acute hemolytic anemia undergoing coblation adenoidectomy with septoplasty and turbinectomy.