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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The megacystis-microcolon-intestinal hypoperistalsis syndrome is a congenital disorder characterized by urinary bladder distension and hypoperistalsis throughout the entire gastrointestinal tract. We present a new case with the typical clinical, radiological, and pathological findings of the syndrome. The diagnosis should be suspected in a patient who present clinically with intestinal obstruction and urinary retention, and confirmed with imaging studies, including abdominal plain films, urinary tract ultrasonography, and contrast studies of the colon and the bladder. The prognosis is generally very poor. Our patient died secondary to sepsis on day 5 of life.
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PMID:Megacystis-microcolon-intestinal hypoperistalsis syndrome. 858 28

Neonatal endocrinology is a diverse topic. Several chapters could be devoted to the endocrinology of fetal transition alone. The next several pages contain a brief overview of some pertinent illnesses. It is intended not to give an absolute map in the care of these patients but to help guide the physician in tailoring an approach for each patient based on current theories and practice parameters. It could also aid in improving the physician's understanding of screening laboratories used to identify those infants at risk of preventable, treatable and potentially disastrous diseases (i.e. congenial hypothyroidism). These metabolic screens are discussed due to their efficacy in the United States. In our experience, depending on the prevalence of a specific disease a few simple procedures allow for an efficient and economic way to reach ill children in a timely fashion. Other topics included in this article were reported based on their common occurrence, the lethality of illnesses if undiagnosed or their unique treatment. In the neonate hypoglycemia, either iatrogenic or secondary to sepsis, a congenital disorder of neisidioblastosis can have severe implications on the development of the CNS if not promptly treated and prevented. Some of these disorders require an experienced endocrinologist or neonatologist to treat and supervise conscientiously (i.e. CAH). However, as most of us know, it is sometimes hard to find such an individual in a community based practice. Therefore, it becomes of paramount value that each of us pays attention to the treatment of these illnesses for the sake of the children we care for.
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PMID:Neonatal endocrinology. 1083 25

Megacystis-microcolon-intestinal hypoperistalsis is a rare congenital disorder characterized by urinary bladder distention and hypoperistalsis throughout the entire gastrointestinal tract. We present a new case with the typical clinical and radiological findings of the syndrome. This diagnosis should be suspected in patients who present with intestinal obstruction and urine retention and should be confirmed with imaging techniques, including abdominal plain films and urinary ultrasonography. Histological findings are non-specific; we found thinning of the intestinal muscle layer and connective tissue proliferation. The prognosis is generally very poor. Our patient died from sepsis at the age of 25 days.
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PMID:[Megabladder-microcolon-intestinal hypoperistalsis syndrome]. 1108 70

Situs inversus (SI) is a rare congenital disorder involving a complete mirror image of the thoracic and abdominal organs. Living donor liver transplantation (LDLT) in SI cases poses particular challenges on account of its technical complexity, and only a few cases have been reported. Here, we present an adult with SI who was managed successfully by LDLT using a left lobe graft. Some technical modifications, including triangulated anastomosis of the hepatic vein, were required but no vascular graft was necessary. Graft function and vascular integrity were excellent throughout the postoperative course, although sepsis secondary to wound infection with methicillin-resistant Staphylococcus aureus developed. In conclusion, LDLT using a left lobe graft is a feasible procedure for patients with SI, even for adults. Therefore, this condition, while rare, should not be a contraindication for LDLT. Meticulous preoperative simulation and planning of the vascular reconstruction are important steps in LDLT for this rare anomaly.
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PMID:Left lobe living donor liver transplantation in an adult patient with situs inversus: technical considerations. 1820 20

Caroli's disease is a rare congenital disorder characterized by cystic dilatation of the large in-trahepatic bile ducts. The most frequent complications due to biliary stasis are cholelithiasis, cholangitis and sepsis as well as an increased risk of cholangiocarcinoma. Patients may have a history of intermittent abdominal pain, pruritus and/or symptoms of cholangitis. It is rarely diagnosed in childhood. A 12-year-old boy with isolated Caroli's disease is described. This child presented at the age of 2 years, with 4 episodes of recurrent bacterial infections. Interestingly he remained asymptomatic for over 10 years, between the second and third episode. During the 4th episode, when he presented with fever and slight abdominal pain, the diagnosis was made on the basis of radiological findings: U/S, CT, MRI and especially with MRCP, in relation with a more typical picture, resembling cholangitis. Since then he has been followed-up systematically for ten years and remains in good clinical condition without further relapses and with unchanged radiological findings. This atypically benign course of Caroli's disease, with intermittent asymptomatic periods, without any treatment, is very rare.
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PMID:Caroli's disease: Description of a case with a benign clinical course. 2471 23

The neonatal foal is in a transitional state from prenatal to postnatal circulation. Healthy newborn foals often have cardiac murmurs and dysrhythmias, which are usually transient and of little clinical significance. The neonatal foal is prone to infection and cardiac trauma. Echocardiography is the main tool used for valuation of the cardiovascular system. With prompt identification and appropriate action, dysrhythmias and other sequel to cardiac trauma can be corrected. With infection, the management and prognosis are driven by concurrent sepsis. Congenital disease represents an interesting diagnostic challenge for the neonatologist, but surgical correction is not appropriate for most equids.
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PMID:The Equine Neonatal Cardiovascular System in Health and Disease. 2661 47

We present the case of a 25-aged woman who consulted due to refractory chronic constipation and fecal incontinence. She had bowel movements every 7-30 days of increased consistency (1-2 Bristol type stools), together with soiling and passive fecal incontinence (Wexner Scale: 12/20). She had undergone surgery shortly after birth for anorectal malformation repair. The colonoscopy and histological study of the rectum were normal. A pelvic magnetic resonance imaging (MRI) was performed which showed a right pararectal mass compressing the rectum without invading it. This mass was compatible with a presacral teratoma or hamartoma. MRI also revealed coccyx agenesis and hypoplasia of the last sacral vertebrae (image 1), all consistent with a Currarino syndrome (CS). The patient received 14 sessions of transcutaneous electrostimulation of the posterior tibial nerve resulting in an increase in bowel movements (every 3 days) and a reduction in fecal incontinence. She was then referred to surgery for presacral mass removal. CS is a congenital disorder characterized by the triad of anorectal malformations, sacral dysgenesis and presacral mass. It is thought to be caused by malformation of the caudal notochord during embryonic development. It is often diagnosed during childhood, although several cases in adults have been reported. This syndrome is associated with a very high rate (95%) of severe chronic and refractory constipation(1). Interestingly, constipation does not appear to be due to mechanical obstruction but to a poorly known intestinal pseudo-obstruction. No defects in the innervation or the musculature of the gut have been identified(2). Complications include ascending meningitis, sepsis, and malignant degeneration of the presacral masses(1). Pelvic and spinal MRI are required for the evaluation of the presacral mass and to rule out communication with the spinal space. Treatment is frequently surgical to prevent complications such as infections and malignant transformation of the presacral masses(3).
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PMID:A CASE OF CURRARINO SYNDROME IN AN ADULT WOMAN PRESENTING WITH REFRACTORY CHRONIC CONSTIPATION. 3320 5