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Korean Journal of Pediatrics Nov 2016Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the...
Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.
PubMed: 28018454
DOI: 10.3345/kjp.2016.59.11.S84 -
Journal of Clinical Hypertension... Dec 2006Fibromuscular dysplasia is a noninflammatory vascular disease that commonly affects the distal two thirds of the renal artery and branch vessels, but occasionally... (Review)
Review
Fibromuscular dysplasia is a noninflammatory vascular disease that commonly affects the distal two thirds of the renal artery and branch vessels, but occasionally involves other arteries. Progression of stenosis occurs in 16%-38% of renal arteries. Although the etiology is unknown, genetic studies suggest a relationship to the angiotensin-converting enzyme I allele. Thin, young Caucasian women without a family history of hypertension are most commonly affected. An abdominal or flank systolic-diastolic bruit is an important clue for the diagnosis. Most noninvasive screening tests are not sensitive or reproducible to be used to rule out renal artery stenosis, but digital subtraction renal angiography usually confirms the diagnosis. Percutaneous renal artery angioplasty is the treatment of choice, but may not result in normalization of blood pressure if diagnosis is delayed. Since restenosis occurs, continued follow-up is necessary.
Topics: Fibromuscular Dysplasia; Genetic Predisposition to Disease; Humans; Renal Artery
PubMed: 17170616
DOI: 10.1111/j.1524-6175.2006.06021.x -
Nefrologia : Publicacion Oficial de La... 2004First cause of secondary hypertension is renovascular hypertension which presents abdominal bruit in 16 to 20% of cases. This clinical sign is also associated with other... (Review)
Review
First cause of secondary hypertension is renovascular hypertension which presents abdominal bruit in 16 to 20% of cases. This clinical sign is also associated with other vascular disease of the abdomen such as celiac trunk stenosis and/or aneurysms located on the pancreaticoduodenal or gastroduodenal arcs level, with little representation among aneurysm. They usually appear on a context of digestive complications like neoplasias, chronic pancreatitis or gastric obstructions possibly with obstructive icterus, hemorrhage and acute abdomen episodes. Its presentation in other contexts is rare and constitutes a diagnostic challenge. Diagnosis is made by abdominal arteriography which is the best method because you can locate the problem as well as intervene therapeutically with embolization of the aneurysme. We would like to emphasize the importance of a quick diagnosis due to the risk of rupture and the high morbi-mortality associated.
Topics: Aneurysm; Aneurysm, Ruptured; Angioplasty; Arterial Occlusive Diseases; Auscultation; Celiac Artery; Constriction, Pathologic; Early Diagnosis; Embolization, Therapeutic; Female; Headache; Humans; Hypertension, Renovascular; Middle Aged; Radiography; Sound
PubMed: 15219082
DOI: No ID Found -
Jornal Vascular Brasileiro 2023Ischemic gastritis is a rare illness caused by localized or systemic vascular insufficiency. This condition is rarely seen in medical practice due to the vast arterial...
Ischemic gastritis is a rare illness caused by localized or systemic vascular insufficiency. This condition is rarely seen in medical practice due to the vast arterial collateral blood supply to the stomach through the celiac trunk and superior mesenteric artery and also because other etiologies are much more frequent. The classic presentation of chronic ischemia is comprises the triad of postprandial pain, weight loss, and abdominal bruit. Intervention is indicated in symptomatic patients and endovascular treatment is an alternative to surgery in patients with high comorbidity that offers good results. We report a case of a 71-year-old female patient with severe ischemic gastritis with ulcers and bleeding caused by chronic mesenteric ischemia with occlusion of the celiac trunk and inferior mesenteric artery and critical stenosis of the superior mesenteric artery. The diagnosis was confirmed by imaging, and the patient underwent endovascular treatment. This is a rare condition that is difficult to diagnose and treat and a multidisciplinary team is needed for proper management.
PubMed: 37790892
DOI: 10.1590/1677-5449.202300022 -
Journal of Medical Case Reports Oct 2021Thyroid storm is an endocrine emergency and life-threatening condition discovered in 1926. There is no specific laboratory parameter that can differentiate or...
BACKGROUND
Thyroid storm is an endocrine emergency and life-threatening condition discovered in 1926. There is no specific laboratory parameter that can differentiate or distinguish between thyroid storm and primary hyperthyroidism. Diagnosis is made on a clinical scoring system, including the Burch-Wartofsky point scale and Japanese Thyroid Association scoring system. The management is early diagnosis, immediate initiation of anti-thyroid medications, intensive care monitoring, and prevention of multiorgan failure.
CASE PRESENTATION
A 30-year-old Pakistani female presented with complaint of headache, vomiting, and generalized weakness for 3 weeks. She had an episode of seizure-like activity at home, and so was rushed to the emergency department. A detailed thyroid examination revealed a soft, nontender gland with no enlargement or bruit and no exophthalmos. Her thyroid-stimulating hormone was extremely low, with high free triiodothyronine and thyroxine. Thyroglobulin was 425 ng/ml (normal reference range ≤ 55 ng/ml), and thyroid-stimulating hormone receptor antibody was 0.87 IU/L (normal reference range 0-1.75 IU/L). She had high levels of beta-human chorionic gonadotropin hormone on initial presentation. Transvaginal ultrasound showed no intrauterine pregnancy, but an echogenic focus was found adherent to the right ovary with no vascularity. With the chief complaint of headache, she underwent magnetic resonance imaging of the brain that showed multiple scattered hemorrhagic lesions in supratentorial and infratentorial brain parenchyma that were highly suspicious for metastases. Computed tomography scan of the chest, abdomen, and pelvis revealed multiple neoplastic lesions in the lung, liver, spleen, and kidneys. A Tru-Cut liver biopsy showed linear cores of liver tissue with metastatic carcinoma with morphological features consistent with choriocarcinoma. Our patient scored 65 on the Burch-Wartofsky point scale. As per the Japanese Thyroid Association scoring system, our patient met the criteria for a "definite thyroid storm." She had initiated propranolol to achieve adequate control of her heart rate and dexamethasone. Carbimazole was started to control her thyroid function. Her thyroid function after 2 weeks of treatment showed significant improvement. Methotrexate and etoposide were given for choriocarcinoma. She made a good recovery and was discharged home. She will undergo rehabilitation along with ongoing chemotherapy (methotrexate and etoposide weekly till beta-human chorionic gonadotropin levels normalize). Unless her source of beta-human chorionic gonadotropin is carefully under control, she will continue to take anti-thyroid medications.
CONCLUSION
Choriocarcinoma is not only associated with hyperthyroidism but can induce thyroid storm. Beta-human chorionic gonadotropin is directly associated with promoting thyroid dysfunction. Patients with gestational trophoblastic disease should be under close surveillance to prevent thyroid storms.
Topics: Adult; Choriocarcinoma; Chorionic Gonadotropin, beta Subunit, Human; Female; Humans; Pregnancy; Thyroid Crisis; Thyroid Function Tests
PubMed: 34686220
DOI: 10.1186/s13256-021-03123-7 -
Annals of the Royal College of Surgeons... Sep 2015Median arcuate ligament syndrome (MALS) is a rare disorder due to coeliac trunk compression by the median arcuate ligament, resulting in coeliac artery stenosis... (Review)
Review
Median arcuate ligament syndrome (MALS) is a rare disorder due to coeliac trunk compression by the median arcuate ligament, resulting in coeliac artery stenosis characterised by chronic, recurrent abdominal pain. Patients with MALS are often middle-aged females presenting with a triad of postprandial epigastric pain, weight loss and abdominal bruit. It is a diagnosis of exclusion and confirmed by computed tomography or magnetic resonance imaging. Laparoscopic or open surgical decompression are the only treatment options in MALS. We present two cases of MALS treated by laparoscopic decompression as well as a literature review on this treatment.
Topics: Adult; Celiac Artery; Constriction, Pathologic; Decompression, Surgical; Female; Humans; Laparoscopy; Median Arcuate Ligament Syndrome; Tomography, X-Ray Computed
PubMed: 26320770
DOI: 10.1308/rcsann.2015.0025 -
Annals of Surgery Sep 1986Middle aortic syndrome typically occurs as severe hypertension in young patients who have weak or absent femoral pulses and an abdominal bruit. It results from a diffuse...
Middle aortic syndrome typically occurs as severe hypertension in young patients who have weak or absent femoral pulses and an abdominal bruit. It results from a diffuse narrowing of the distal thoracic and abdominal aorta, commonly involving the visceral and renal arteries. The clinical presentation, angiographic assessment, and surgical outcome of 10 patients (mean age: 19.5 years) who underwent one-stage revascularization for middle aortic syndrome were reviewed to determine the effectiveness and durability of one-stage revascularization techniques to relieve these complications. All patients were hypertensive (mean blood pressure: 176 mmHg); six (60%) had severe, poorly controlled hypertension, two of whom had previous failed operations for renovascular hypertension and one who presented with malignant hypertension and acute renal failure. Five patients had disabling myocardial insufficiency, only one of whom had documented coronary artery disease. Four patients had intermittent claudication. Aortography showed variable length high-grade midaortic stenosis, nine had visceral artery involvement, and eight had renal artery involvement. All patients underwent one-stage revascularization by a variety of autogenous and prosthetic techniques. The postoperative recovery was uncomplicated in eight of nine patients and was often associated with dramatic reduction in blood pressure. There was a single death from disruption of the thoracic anastomosis in a patient who had diffuse cystic medial necrosis of the aorta. Arterial biopsy in nine patients indicated evidence for both acquired and congenital origins of the midaortic stenosis. Late follow-up evaluation (mean: 4.1 years) showed normal growth and development, preservation of renal function, and relief of myocardial insufficiency in all patients. Seven patients (77%) are cured of their hypertension, and two (23%) have only mild hypertension. These results indicate that one-stage revascularization of patients with middle aortic syndrome can result in effective and durable relief of these severe life-threatening complications.
Topics: Adolescent; Adult; Aortic Diseases; Arterial Occlusive Diseases; Child; Female; Follow-Up Studies; Humans; Hypertension; Male; Renal Artery; Syndrome
PubMed: 3753060
DOI: 10.1097/00000658-198609000-00012 -
Internal Medicine (Tokyo, Japan) Oct 1992A case of renal artery aneurysm is presented. The patient had no hypertension and no signs of arteriosclerosis obliterans or aortitis syndrome, except for abdominal...
A case of renal artery aneurysm is presented. The patient had no hypertension and no signs of arteriosclerosis obliterans or aortitis syndrome, except for abdominal bruit. A saccular aneurysm, 1 cm in diameter, was demonstrated by two-dimensional and color Doppler ultrasound and documented by angiography. The aneurysm was embolized by a steel coil. The abdominal bruit, though uncommon, is a very important bed-side sign of renal artery aneurysm, if the patient exhibits no arteriosclerosis obliterans or aortitis syndrome. Ultrasound Doppler is very useful in screening for aneurysm.
Topics: Aneurysm; Angiography; Female; Humans; Kidney; Middle Aged; Renal Artery; Ultrasonography
PubMed: 1286231
DOI: 10.2169/internalmedicine.31.1217 -
British Medical Journal Nov 1971The diagnosis of primary cancer of the liver was reviewed in 75 patients. A definitive diagnosis was made during life in 63% and in a further 20% this condition was...
The diagnosis of primary cancer of the liver was reviewed in 75 patients. A definitive diagnosis was made during life in 63% and in a further 20% this condition was suspected though histological confirmation was obtained only at necropsy. The most common presenting complaints were abdominal pain and weight loss and the most frequent findings hepatomegaly and ascites. Less than one-half of the patients were jaundiced and when present it was usually mild. An arterial bruit was heard over the liver in 25% of the patients. A sudden and unexplained deterioration in a patient known to have cirrhosis or haemochromatosis should raise the possibility of a primary hepatic tumour; this occurred in 24% of our patients.Alpha-fetoprotein was found in the serum of 11 out of 18 cases. The presence of a mass in the liver was frequently confirmed by liver scan, portal venography, or hepatic arteriography, but these showed no features diagnostic of a primary tumour. Liver scan also proved useful in localizing the lesion for biopsy purposes. Definitive diagnosis is dependent on the histological demonstration of the features of the tumour. This can frequently be achieved by percutaneous needle biopsy, which was positive in 38 out of 57 patients. Wedge biopsies were positive in a further nine patients.
Topics: Abdomen; Adolescent; Adult; Aged; Alkaline Phosphatase; Angiography; Ascites; Auscultation; Autopsy; Biopsy; Body Weight; Child; Female; Fetal Proteins; Hemochromatosis; Hepatomegaly; Humans; Jaundice; Lipid Metabolism, Inborn Errors; Liver Cirrhosis; Liver Neoplasms; Male; Middle Aged; Pain; Phlebography; Radionuclide Imaging
PubMed: 5124443
DOI: 10.1136/bmj.4.5784.408 -
Radiologia Sep 2013The middle aortic syndrome (MAS) is caused by stenosis of the abdominal aorta, often accompanied by concomitant stenosis of the renal or visceral arteries. Although MAS... (Review)
Review
The middle aortic syndrome (MAS) is caused by stenosis of the abdominal aorta, often accompanied by concomitant stenosis of the renal or visceral arteries. Although MAS is uncommon (0.5-2%), it is an important cause of hypertension in children and adolescents. It is thought to originate in a failure of the two dorsal aortas to fuse during embryological development, and a high percentage of cases are idiopathic. MAS affects the segment of the aorta between the renal arteries in 54% of cases. Clinically, it courses with symptomatic or asymptomatic arterial hypertension. On physical examination, findings include an abdominal bruit, diminished or absent femoral pulses, and a difference between the arterial pressure of the upper and lower limbs. Angiography is the technique of choice, although noninvasive MR angiography and CT angiography have similar diagnostic accuracy. Ultrasonography is the primary screening technique. Medical treatment consists of a combination of different antihypertensives. Surgical treatment can be curative.
Topics: Aorta, Abdominal; Aortic Diseases; Arterial Occlusive Diseases; Child; Child, Preschool; Humans; Male; Syndrome
PubMed: 21724214
DOI: 10.1016/j.rx.2010.12.016