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Signs and symptoms of this condition typically appear around the age of 3 or 4 months, when babies start to sleep through the night and do not eat as frequently as newborns. Affected infants may have low blood sugar (hypoglycemia), which can lead to seizures. They can also have a buildup of lactic acid in the body (lactic acidosis), high blood levels of a waste product called uric acid (hyperuricemia), and excess amounts of fats in the blood (hyperlipidemia). As they get older, children with GSDI have thin arms and legs and short stature. An enlarged liver may give the appearance of a protruding abdomen. The kidneys may also be enlarged. Affected individuals may also have diarrhea and deposits of cholesterol in the skin (xanthomas).
NTIS refers to a syndrome found in seriously ill or starving patients with low fT3, usually elevated RT3, normal or low TSH, and if prolonged, low fT4. It is found in a high proportion of patients in the ICU setting, and correlates with a poor prognosis if TT4 is <4ug/dl. The patho-physiology includes suppression of TRH release, reducedT3 and T4 turnover, reduction in liver generation of T3, increased formation of RT3, and tissue specific down-regulation of deiodinases, transporters, and TH receptors. Although long debated, tissue TH levels are definitely reduced, and tissue hypothyroidism is presumably present. This is often not clinically evident because of the brief duration, and reduced but not absent tissue levels of TH. Although recognized for nearly 4 decades, interpretation of the syndrome is contested, because of lack of data. Some observes, totally without data, argue that it is a protective response and should not be treated. Other observers (as in this review) present available data suggesting, but not proving, that thyroid hormone replacement is appropriate, not harmful, and may be beneficial. The best form of treatment (TRH,TSH,or T3+T4) and possible accompanying treatments (GHRH, Cortisol, nutrition, insulin) lack consensus. In this review current data are laid out for reader’s review and judgment.
CPAP is a treatment that uses mild air pressure to keep your breathing airways open. It involves using a CPAP machine that includes a mask or other device that fits over your nose or your nose and mouth, straps to position the mask, a tube that connects the mask to the machine’s motor, and a motor that blows air into the tube. CPAP is used to treat sleep-related breathing disorders including sleep apnea. It also may be used to treat preterm infants who have underdeveloped lungs.
Visualization of the larynx by direct or indirect means is referred to as laryngoscopy and is the principal aim during airway management for passage of a tracheal tube. This paper presents a brief background regarding the development and practice of laryngoscopy and examines the equipment and techniques for both direct and indirect methods. Patient evaluation during the airway examination is discussed, as are predictors for difficult intubation. Laryngoscope blade design, newer intubating techniques, and a variety of indirect laryngoscopic technologies are reviewed, as is the learning curve for these techniques and devices.
Gastroparesis -- literally “paralyzed stomach” -- is a serious condition manifested by delayed emptying of stomach contents into the small intestine after a meal. There is no cure for gastroparesis, but treatment can speed gastric emptying and relieve gastrointestinal symptoms such as nausea and vomiting.
A febrile seizure is a convulsion in a child that may be caused by a spike in body temperature, often from an infection. Your child's having a febrile seizure can be alarming, and the few minutes it lasts can seem like an eternity. Febrile seizures represent a unique response of a child's brain to fever, usually the first day of a fever. Fortunately, they're usually harmless and typically don't indicate an ongoing problem. You can help by keeping your child safe during a febrile seizure and by comforting him or her afterward.
Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterised by an exaggerated response of the immune system (a hypersensitivity response) to the fungus Aspergillus (most commonly Aspergillus fumigatus). It occurs most often in patients with asthma or cystic fibrosis.
An antecedent upper respiratory infection is present in 50% of patients. Abdominal pain is a presenting symptom in 1 0-15% of patients. The skin lesions are symmetric, involve dependent parts of the body, and classically progress from an erythematous, macular rash to papular purpura. The joints and kidneys are also commonly involved
Acute intermittent porphyria (AIP) is a rare autosomal dominant metabolic disorder affecting the production of heme, the oxygen-binding prosthetic group of hemoglobin. It is characterized by a deficiency of the enzyme porphobilinogen deaminase.
The cause of HELLP syndrome is unknown, but there are certain factors that may increase your risk of developing it. Preeclampsia is the greatest risk factor. This condition is marked by high blood pressure and swelling, and it typically occurs during the last trimester of pregnancy.
The differential diagnosis for this child's painless hematochezia includes Meckel's diverticulum as well as vascular malformations. Meckel's diverticulum results from a failure of the vitelline duct to obliterate during the first 8 weeks of gestation, leaving behind a blind pouch often containing ectopic gastric tissue. Meckel's diverticulum classically affects children age ~:2 but can also occur in older children or even adults. Young children are more likely to experience painless bleeding due to mucosal irritation from gastric acid; adolescents and adults are more likely to have signs of obstruction. A technetium-99 nuclear scan will identify the diverticulum, which is usually located in the right lower quadrant of the abdomen within 2 feet of the ileocecal valve. Technetium-99 concentrates in the parietal cells of the diverticulum and stomach. The scan is also known as "Meckel's scan" due to its high specificity. A symptomatic Meckel's diverticulum is generally treated with surgical resection.