Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 32-year-old HIV-positive black female underwent 70 days of intensive care after she developed acute fatty liver disease of pregnancy. During her hospitalization, multiple invasive procedures were performed on this patient and many residents, staff, nurses, and technicians were exposed to her body secretions. The patient was identified as HIV positive on a routine screen and denied any risk factors for AIDS. This case served as a catalyst to assess current screening efforts in the military for HIV. A questionnaire was distributed to OB/GYN, general surgery, and urology programs throughout the Army, Navy, and Air Force and the results are presented and discussed.
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PMID:HIV screening by the tri-service surgical specialties. 251 81

Diffuse pulmonary uptake of sulfur colloid was seen in a 35-year-old postpartum white female with the presumptive diagnosis of fatty liver disease of pregnancy. As the disease process resolved, the pulmonary uptake of sulfur colloid disappeared. To the authors' knowledge, this scan association has not been previously reported.
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PMID:Diffuse lung uptake of technetium-99m sulfur colloid associated with fatty liver disease of pregnancy. 358 7

With regard to a case of acute fatty liver disease of pregnancy, concerning a primigravida patient, during a twin pregnancy, having survived to a severe form and with two-fetus death in utero. Extreme blood hypocoagulability induced cataclysmic bleeding and threatened the life of the patient who had survived only after the ligature of internal iliac arteries. Nevertheless, one year later, another normal pregnancy was uneventful.
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PMID:[Normal pregnancy after severe acute hepatic steatosis]. 770 62

The acute fatty liver of pregnancy is a specific disease of pregnancy. Its incidence is rare but prognosis is poor in complicated entities, and can compromise the foetal and maternal life. An early diagnosis of the illness and a multidisciplinary management based on uterine evacuation and an intensive medical care can improve the prognosis of the acute fatty liver of pregnancy (A.F.L.P). In hope to contribute at a better approach of this affection, the authors report their experience about 3 cases of A.F.L.P., collected in 1997.
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PMID:[Acute fatty liver of pregnancy. Three case reports]. 1119 Jul 33

Acute fatty liver disease of pregnancy has been recognized as a clinical problem since the 1980s. In the past 8 years, the association of this disease with a genetic inborn error of metabolism in the infant has been recognized. Women who are heterozygous for this disorder are usually asymptomatic until the capacity of their livers to metabolize free fatty acids (FFA) is overwhelmed by a homozygous fetus. The inborn error of metabolism, long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency, may not be immediately recognizable in the infant. Symptoms in the infant are often triggered by an increased long-chain fatty acid load in the diet, or by illness that results in breakdown of endogenous fat. The following case study reviews the clinical pathophysiology of this perinatal health problem and highlights the priorities for the care of infants born to mothers with acute fatty liver disease of pregnancy.
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PMID:Maternal acute fatty liver of pregnancy and the associated risk for long-chain 3-hydroxyacyl-coenzyme a dehydrogenase (LCHAD) deficiency in infants. 1498 77

Diabetes insipidus is an uncommon pathology; its incidence varies from two to six cases in 100,000 pregnancies. It has multiple etiologies and it is classified in central and neurogenic. Patients with diabetes insipidus generally show intense thirst, polyuria, neurologic symptoms and hypernatremia. It does not seem to alter the patient's fertility. Diabetes insipidus is usually associated with pre-eclampsia, HELLP syndrome, and fatty liver disease of pregnancy. This is a report of a case seen at the Hospital General de Cholula, in Puebla, Mexico. A 19 year-old female, with 37.2 weeks of pregnancy, had a history of Langerhans cell histiocytosis since she was four years. Patient was treated with intranasal desmopressin until 2005. She went to an obstetric evaluation; laboratory and cabinet studies were obtained. A healthy 1900 g female was obtained through vaginal delivery, with a 7/9 Apgar score. We should be familiarized with this uncommon pathology because of its association with several obstetric emergencies.
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PMID:[Diabetes insipidus and pregnancy]. 1784 3

Acute fatty liver of pregnancy (AFLP) and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome are serious maternal illnesses occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. AFLP may result from mitochondrial defects in the beta-oxidation of fatty acids, in particular a deficiency of the long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) in the fetus. Clinical findings in AFLP vary and its diagnosis is complicated by a significant overlap in clinical and biochemical features with HELLP syndrome. We report the case of 2 siblings who died, the first one in the neonatal period of asphyxia with multivisceral presentation and the second one from sudden death at 7 months. Autopsy of the latter infant revealed hepatic steatosis associated with cardiomyopathy, which led to suspicion of a fatty acid oxidation deficiency. Mutation analysis demonstrated that both children were homozygous for the common mutation c.1528G>C and the parents were heterozygous for this same mutation. This case demonstrates the importance of screening mothers with acute fatty liver disease of pregnancy and their children at birth for a metabolic disease. This article proposes several metabolic tests for mother and child suspected of having beta-oxidation of a fatty acid disorder.
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PMID:[Acute fatty liver in pregnancy: revealing fetal fatty acid oxidation disorders]. 2232 56

Pregnant and postpartum patients represent a challenge to critical care physicians, as two patients in one have to be cared for and because specific obstetric disorders, not universally covered in formal critical care training, need to be managed. Pregnancy also alters physiologic norms, so that the critical care physician may either fail to recognize a value as abnormal in pregnancy or mistakenly identify as abnormal a value within the normal range for a pregnant woman. In this article, we will review the most frequent obstetric causes of admission of pregnant/postpartum patients to the intensive care unit (hypertensive disease of pregnancy, obstetric hemorrhage, and obstetric sepsis) along with their diagnostic criteria, clinical presentation, and recommended treatment. We will also cover some specific, although less frequent, obstetric disorders, such as acute fatty liver of pregnancy, peripartum cardiomyopathy, and amniotic fluid embolism. Our primary aim is to improve quality of care for these types of patients.
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PMID:Obstetric Disorders in the ICU. 2856 Dec 53

Pregnancy-related acute kidney injury (Pr-AKI) remains a large public health problem, with decreasing incidences in developing countries but seemingly increasing incidences in the United States and Canada. These epidemiologic changes are reflective of the advances in medical and obstetric care, as well as changes in underlying maternal risk factors. The risk factors associated with advanced maternal age, such as hypertension, diabetes, chronic kidney disease, and those associated with reproductive technologies such as multiple gestations, are increasing. Traditional causes of Pr-AKI, such as septic abortions and puerperal sepsis, have been replaced by hypertensive diseases, such as preeclampsia and thrombotic microangiopathies comprising thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS). In this review, we discuss the global impact of Pr-AKI on maternal and fetal outcomes, the predominant etiologies, and key clinical features to distinguish diagnoses, such as preeclampsia/hemolysis elevated liver function test and low platelet (HELLP) syndrome, acute fatty liver disease of pregnancy (AFLP), and other thrombotic microangiopathies. New insights into the pathogenesis of preeclampsia, TTP/aHUS, and AFLP that have unearthed possible therapeutic targets are summarized. We also delve into special consideration needed to give to pyelonephritis and postobstructive causes of Pr-AKI. With each diagnosis, we offer the latest treatment recommendations, such as the positive reports from the use of eculizumab to treat aHUS. In the end, we hope to arm the clinician with the best tools to understand and address this morbid problem that does not seem to be disappearing.
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PMID:Acute Kidney Injury in Pregnancy: The Changing Landscape for the 21st Century. 2972 29

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has presented many diagnostic challenges and uncertainties. Little is known about common pathologies complicating pregnancy and how their behaviour is modified by the presence of SARS-CoV-2. Pregnancy itself can alter the body's response to viral infection, which can cause more severe symptoms. We report the first case of a patient affected with sudden-onset severe pre-eclampsia complicated by acute fatty liver disease of pregnancy, HELLP (haemolysis, elevated liver enzymes and low platelet) syndrome and acute kidney injury following SARS-CoV-2 infection. Although an initial diagnostic dilemma, a multidisciplinary team approach was required to ensure a favourable outcome for both the mother and the baby. Our case report highlights the need for health professionals caring for pregnant women to be aware of the complex interplay between SARS-CoV-2 infection and hypertensive disorders of pregnancy.
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PMID:Severe pre-eclampsia complicated by acute fatty liver disease of pregnancy, HELLP syndrome and acute kidney injury following SARS-CoV-2 infection. 3278 39


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