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Hemophilia A, also called factor VIII (FVIII) deficiency or classic hemophilia, is a genetic disorder caused by missing or defective factor VIII, a clotting protein. Although it is passed down from parents to children, about 1/3 of cases are caused by a spontaneous mutation, a change in a gene. According to the US Centers for Disease Control and Prevention, hemophilia occurs in approximately 1 in 5,000 live births. There are about 20,000 people with hemophilia in the US. All races and ethnic groups are affected. Hemophilia A is four times as common as hemophilia B while more than half of patients with hemophilia A have the severe form of hemophilia.
This is the CT of a 43 year old male patiënt with infected necrotizing pancreatitis that will undergo a VARD procedure; Videoscopic Assisted Retroperitoneal Debridment. Two weeks before this procedure two large bore percutaneous drains were placed in the peripancreatic collection. The patient i...s placed in supine position with the left side 30 degrees elevated. A 5-7 cm subcostal incision is made in the left flank. With help of CT images and by following the percutaneous drain, the subcutaneous tissue and the fascia are dissected and we enter the retroperitoneal peripancreatic collection. First, with a regular suction device any pus encountered is removed. Two long sympathectomy hooks are inserted in order to keep in the incision open. We than insert the zero degree laparoscope. The first necrosis encountered is removed under direct sight with the use of long grasping forceps. Following the percutaneous drain deeper into the cavity, parts of loosely adherent necrotic material are removed. Gently pulling we remove the necrotic tissue. The suction device is helpful in removing any fluid obstructing the view. Complete necrosectomy is not the ultimate aim of this procedure. Only loosely adherent pieces of necrosis are removed thereby keeping the risk of tearing underlying blood vessels to a minimum. In the rare case of extensive bleeding, the retroperitoneal cavity can be easily packed, either awaiting the bleeding to definitely stop or to act as a bridge to angiographic coiling. This patient is now 6 weeks after onset of disease. We always try to postpone surgical intervention, if possible up to 30 days. On the left side of the collection is the percutaneous drain. In this patient the drain had worked well for 2 weeks. When the patient deteriorated again it was decided to perform the VARD procedure. Large pieces of necrotic pancreas can be removed with VARD. This is a big advantage ov VARD over pure endosopic or percutaneous techniques. When all the necrotic tissue is removed we clean the cavity. Two drains are left in situ as a postoperative lavage system. The VARD procedure is performed via a 6 cm incision, which is closed and continuous postoperative lavage started immediately.
An ileostomy is an opening in the belly (abdominal wall) that’s made during surgery. The end of the ileum (the lowest part of the small intestine) is brought through this opening to form a stoma, usually on the lower right side of the abdomen. A Wound Ostomy Continence nurse (WOCN or WOC nurse) or the surgeon will figure out the best location for your stoma. (A WOC nurse is a specially trained registered nurse who takes care of and teaches ostomy patients. This nurse may also be called an ostomy nurse.)
CORRECTION: After review of this video, it is clear that this video is of a baby who is near full term (40 weeks) based on the size. Late trimester "abortions" are defined only to viability of a baby (24 weeks) A 24 week baby is much smaller than this baby shown and by definition this is not a late "abortion" procedure. The proper labeling of this video should be management of a deceased breech baby with "head entrapment" as this was almost certainly a naturally occuring delivery and an OB nightmare (Reviewed by Dr. Frederick Bright)
Classical PKU is an autosomal recessive disorder, caused by mutations in both alleles of the gene for phenylalanine hydroxylase (PAH), found on chromosome 12. In the body, phenylalanine hydroxylase converts the amino acid phenylalanine to tyrosine, another amino acid.
Foot drop is a gait abnormality in which the dropping of the forefoot happens due to weakness, irritation or damage to the common fibular nerve including the sciatic nerve, or paralysis of the muscles in the anterior portion of the lower leg. It is usually a symptom of a greater problem, not a disease in itself.
Pneumoconiosis is a general term given to any lung disease caused by dusts that are breathed in and then deposited deep in the lungs causing damage. Pneumoconiosis is usually considered an occupational lung disease, and includes asbestosis, silicosis and coal workers' pneumoconiosis (CWP), also known as "Black Lung Disease."