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Nephrotic Syndrome I : Introduction01:24

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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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Acute Coronary Syndrome I: Introduction01:30

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Acute Coronary Syndrome (ACS) encompasses a spectrum of heart conditions caused by sudden obstruction of coronary arteries, typically resulting from the rupture of an atherosclerotic plaque and subsequent thrombus (blood clot) formation. This obstruction can lead to partial or complete blockage of blood flow, causing varying degrees of myocardial ischemia or infarction.ACS includes the following clinical entities:Unstable Angina (UA)Non-ST-Elevation Myocardial Infarction (NSTEMI)ST-Elevation...
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Irritable Bowel Syndrome I: Introduction01:17

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Irritable Bowel Syndrome (IBS) is characterized by functional disturbances in the gastrointestinal system, presenting a cluster of symptoms without evident structural or biochemical abnormalities. It primarily affects the large intestine and may cause abdominal pain, bloating, excessive gas, diarrhea, constipation, or both.
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Restless Leg Syndrome (RLS), also known as Willis-Ekbom disease, is a neurological disorder characterized by an uncontrollable urge to move the legs due to uncomfortable sensations. These sensations typically occur during periods of rest or inactivity, particularly when lying down or sitting, and can severely disrupt sleep.
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Acute Coronary Syndrome V: Nursing Management01:26

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Nursing Assessment:Nursing management of acute coronary syndrome (ACS) involves taking the patient's history, focusing on primary complaints such as chest pain, dyspnea, and excessive sweating (diaphoresis), as well as other symptoms like back or jaw pain, nausea, vomiting, palpitations, dizziness, and fatigue. The nurse also reviews the patient's history of cardiac events, risk factors such as hypertension, diabetes, smoking, family history, and current medications.In the objective assessment,...
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Nephrotic Syndrome II : Assessment and Medical Management01:26

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IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
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Schopf-Schulz-Passarge Syndrome.

Kinjal D Rambhia1, Vidya Kharkar1, Sunanda Mahajan1

  • 1Department of Dermatology, Seth G.S. Medical College and King Edward Memorial Hospital, Mumbai, Maharashtra, India.

Indian Dermatology Online Journal
|December 4, 2018
PubMed
Summary
This summary is machine-generated.

Schopf-Schulz-Passarge syndrome (SSPS) is a rare genetic disorder. This case study details a patient diagnosed with SSPS, highlighting its characteristic symptoms like skin lesions and dental abnormalities.

Keywords:
Apocrine hidrocystomasSchopf–Schulz–Passarge syndromeectodermal dysplasiakeratoderma

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Area of Science:

  • Dermatology
  • Genetics
  • Rare Diseases

Background:

  • Schopf-Schulz-Passarge syndrome (SSPS) is a rare autosomal recessive ectodermal dysplasia.
  • It presents with a distinct set of clinical features including palmoplantar keratoderma, hypodontia, hypotrichosis, nail dystrophy, and apocrine hidrocystomas.

Observation:

  • A 36-year-old male patient presented with multiple eyelid and periocular apocrine hidrocystomas.
  • Additional symptoms included ichthyosis, palmoplantar keratoderma, hypodontia, nail dystrophy, and hypotrichosis (thin scalp hair).

Findings:

  • Skin biopsy of a periocular lesion confirmed the presence of apocrine hidrocystoma.
  • The histopathological findings, including cystic structures with decapitation secretion, were consistent with the diagnosis.

Implications:

  • This case reinforces the diagnostic criteria for Schopf-Schulz-Passarge syndrome.
  • Accurate diagnosis is crucial for understanding the genetic basis and potential management of this rare ectodermal dysplasia.