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2.1
Malouf Syndrome
Congestive Cardiomyopathy-Hypergonadotropic Hypogonadism Syndrome

Presentation

Here we present two typical cases of Malouf syndrome. Case: In Figure 1, Twenty-six-year-old women presented to our clinic with a complaint of amenorrhea. She was 165?cm in height and 65?kg in weight. [plexusmd.com]

The phenotype varies and not all features are present in every individual case. [medcraveonline.com]

Here we present two cases of Malouf syndrome and literature review. 1. [hindawi.com]

Entire Body System

  • Precocious Puberty

    Affiliated tissues include heart, testes and hypothalamus, and related phenotypes are ptosis and precocious puberty Disease Ontology : 12 A syndrome characterized by dilated cardiomyopathy and hypergonadotropic hypogonadism that has material basis in [malacards.org]

  • Malnutrition

    Its cause is unknown, but it occurs most frequently in multiparous women older than age 30, particularly those with malnutrition or preeclampsia. [checkorphan.org]

Respiratoric

  • Orthopnea

    At the time of admission the patient exhibited dyspnea, orthopnea and lethargy. [spandidos-publications.com]

Cardiovascular

  • Cardiomegaly

    In these patients, cardiomegaly and heart failure may reverse with treatment, allowing a subsequent normal pregnancy. If cardiomegaly persists despite treatment, the prognosis is poor. [checkorphan.org]

    Chest X-ray showed moderate cardiomegaly with a cardiothoracic ratio of 0.58. On the echocardiogram, left ventricular dilatation was observed with an ejection fraction of 35%. [hindawi.com]

  • Pansystolic Murmur

    On physical examination she had blood pressure (BP) of 90/65 mmHg, raised jugular venous pressure (JVP), crackles over the lung bases, and a pansystolic murmur at the apex. [hindawi.com]

Musculoskeletal

  • Normal Stature

    The first-degree relatives of the patient had experienced normal puberties and were of normal statures. There was no family history of menstrual disorders or dysmorphic features. [spandidos-publications.com]

  • Sloping Shoulders

    Additional signs are chest disorder (as a narrow “keeled breast”, sloping shoulders) and adipose tissue deficit. Micrognathia and sloping shoulders may mimic atypical progeroid phenotype. [medcraveonline.com]

Workup

Axis

  • Left Axis Deviation

    The 12-lead electrocardiogram (ECG) showed sinus tachycardia (heart rate 104 BPM) with left axis deviation, left bundle branch block (QRS duration 124ms), normal corrected QT interval and nonspecific ST-T wave abnormalities. [medcraveonline.com]

Treatment

In these patients, cardiomegaly and heart failure may reverse with treatment, allowing a subsequent normal pregnancy. If cardiomegaly persists despite treatment, the prognosis is poor. [checkorphan.org]

Although there is no specific treatment or cure for DCMHH, there are ways to manage the symptoms. A team of doctors or specialists is often needed to figure out the treatment options for each person. [malacards.org]

Paget’s disease of the bone after treatment with Denosumab: a case report. Bone 2012;50(5):1023–5. (33) Reid IR, Sharma S, Kalluru R, Eagleton C. Treatment of Paget’s Disease of Bone with Denosumab: Case Report and Literature Review. [revista.spr.org.py]

^ "Treatment Options for Hypogonadism". [en.wikipedia.org]

Prognosis

If cardiomegaly persists despite treatment, the prognosis is poor. Dilated cardiomyopathy occurs in 2 of every 100 people and affects all ages and sexes. It’s most common in adult men. [checkorphan.org]

The adult respiratory distress syndrome; definition and prognosis; pathogenesis and treatment. Br J Anaesth 1990; 65: 107–129. Google Scholar | Crossref | Medline 5. Seeper, W., Schneider, U., Kreusler, B. [journals.sagepub.com]

DCM patients with LMNA mutations have poor prognosis due to life-threatening ventricular tachyarrhythmias, progressive heart failure and high risk of sudden cardiac death. [medcraveonline.com]

Etiology

Aortic Dissection: New Frontiers in Diagnosis and Management: Part I: from etiology to diagnostic strategies. Circulation 2003; 108 (5): 628-35. [ Links ] 4. Maraj R, Rerkpattanapipat P, Jacobs L, Makornwattana P, Kotler M. [scielo.cl]

The molecular etiology remains unknown in other indicated cases.1,2,7,8 Detection of such clinical cases indicates other genetic involvement in the pathogenesis of the Malouf syndrome. [medcraveonline.com]

Arthrogryposis (multiple congenital contractures): Diagnostic approach to etiology, classification, genetics, and general principles. European Journal of Medical Genetics, 57(8), 464-472. doi:10.1016/j.ejmg.2014.03.008 Solicitar información [igenomix.es]

Epidemiology

Epidemiology. Dilated Cardiomyopathy: From Genetics to Clinical Management. Springer. pp. 11–16. doi:10.1007/978-3-030-13864-6_2. ISBN 978-3-030-13863-9. PMID 32091720. External links[edit] [en.wikipedia.org]

Pathophysiology

Clinical and pathophysiologic significance. Am Rev Respir Dis 1986; 133: 218–225. Google Scholar | Medline 15. Rinaldo, J.E., Borovetz, H. [journals.sagepub.com]

Prevention

Diabetes and the Metabolic Syndrome Eating for the Prevention and Treatment explains in an easy to understand way, to the health professional and lay person, how important the Metabolic Syndrome is in managing diabetes. [goodreads.com]

This medication may also prevent the harmful effects of stress hormones. Some of the side effects of this medication include dizziness and low blood pressure. [en.wikipedia.org]

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