NEONATAL CHOLESTASIS

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Learning Objectives
Know the differential diagnosis for neonatal cholestasis.
Understand how to evaluate the neonate with conjugated hyperbilirubinemia.
Know the therapeutic management of neonates with cholestasis.

Neonatal cholestasis is defined as conjugated hyperbilirubinemia developing within the first 90 days of extrauterine life.
Conjugated bilirubin exceeds 1.5 to 2.0 mg/dl.Conjugated bilirubin generally exceeds 20% of the total bilirubin.

Basic distinction is between:
Extrahepatic etiologies
Intrahepatic etiologies

EXTRAHEPATIC ETIOLOGIES
Extrahepatic biliary atresia
Choledochal cyst
Bile duct stenosis
Spontaneous perforation of the bile duct
Cholelithiasis
Inspissated bile/mucus plug
Extrinsic compression of the bile duct

CLINICAL PRESENTATION
Jaundice,Scleral icterus,Hepatomegaly,Acholic stools & Dark urine

EVALUATION

Basic evaluation
 History and physical examination (includes exam of stool color)
 CBC and reticulocyte count
 Electrolytes, BUN, creatinine, calcium, phosphate
 SGOT, SGPT, GGT, alkaline phosphatase
 Total and direct bilirubin
 Total protein, albumin, cholesterol, PT/PTT

Tests for infectious causes
 Indicated cultures of blood, urine, CSF
 TORCH titers, RPR/VDRL
 Urine for CMV
 Hepatitis B and C serology
Ophthalmologic examination

Metabolic work-up
Protein electrophoresis, alpha-1-antitrypsin level and phenotype
Thyroid function tests
Sweat chloride
Urine/serum amino acids
Review results of newborn metabolic screen
Urine reducing substances
Urine bile acids

Radiological evaluation
Ultrasonography
Patient should be NPO to increase likelihood of visualizing the gallbladder
Feeding with exam may demonstrate a functioning gallbladder
Hepatobiliary scintigraphy

Fungal Peritonitis (FP)

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Empiric diagnosis of peritonitis
Pain, fever and a positive dialysate culture are facultative features of peritonitis.
Dialysate culture results are typically not available before 24 hours and are not helpful in initial clinical decision making.
The criteria represent international consensus among adult and pediatric nephrologists.

How frequent is Fungal Peritonitis?
1% of all episodes of peritonitis

What are the predisposing factors for the development of FP?
Prior use of antibiotics to treat bacterial peritonitis or a catheter-related infection.
Nearly 50% of children who developed fungal peritonitis, have no received antibiotics (for peritonitis, ESI, or other reason)  prior to the peritoneal infection.

How long we should treat FP?
The duration of antifungal treatment following catheter removal should be 2 weeks or longer following complete resolution of the clinical symptoms of infection
(or 4 - 6 weeks without catheter removal)

Fungal peritonitis is a rare complication of peritoneal dialysis (it represents 1% of all episodes of peritonitis)
The optimal management  is early catheter removal and the administration of a variety of anti-fungal agents
The mortality rate is not high in the paediatric population
Preservation of the peritoneal membrane and continuation of PD is feasible with early PD catheter removal and appropriate therapy

Esophageal Diseases

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Essential Esophageal Anatomy
The esophagus is 25 cm in length.  The lower 5-7 cm are below the diaphragm
Average distance from incisors to GE junction is 38-40 cm in men.  The distance from the incisors to the cricopharyngeus is 15 cm
Topographically, the esophagus begins at the lower border of C6.  The diaphragmatic hiatus is at T10
The upper 1/3 esophagus is slightly to the left of midline, the middle 1/3 slightly to the right, and the lower 1/3 slightly to the left
The upper 1/3 is composed of striated muscle and is innervated by the vagus and its recurrent branch. The lower 2/3 is composed of smooth muscle and is supplied by the vagus and the intrinsic autonomic nerve plexus

Esophageal Spasm Syndromes
Inadequate LES relaxation
 Achalasia, epiphrenic diverticulum
Uncoordinated esophageal contraction
 Diffuse esophageal spasm (DES)
Hypercontraction
 High-amplitude peristaltic contraction (HAPC, “nutcracker esophagus”), Hypertensive lower esophageal sphincter (HLES)
Hypocontarction
 Ineffective esophageal motility (IEM)

Achalasia
Achalasia is best confirmed by:
A birds beak appearance on barium esophagogram
Aperistalsis of the cervical esophagus
Failure of the LES to relax on swallowing
LES pressure < 5 mmHg
Biopsy proven esophagitis on flexible endososcopy

Treatment of Achalasia
Surgical Myotomy
Transabdominal vs Transthoracic
Dysphagia relief about 90% at 2 years
Recurrent dysphagia within 2 months likely due to incomplete myotomy, torsion of the repair or scarring of the mucosa from cautery
Late-onset dysphagia due to mucosal stricture from reflux, or the latent effects of delayed gastric emptying.  These patients ultimately need gastric or esophageal resection

Pneumatic Dilatation
Disrupts LES muscle fibers and produces relief of symptoms in 50-85% of patients.  However, most patients require multiple dilatations, increasing the risk of perforation (up to 8%).  Long term relief of symptoms in 40-65%

Esophageal Diverticulum
Epiphrenic Diverticulum
Usually pulsion diverticulum located within the distal 10 cm of the thoracic esophagus
Usually right sided
Most are found incidentally, however, the most common symptoms are dysphagia, regurgitation
Barium esophagogram remains the best test for diagnosis
Endoscopy, 24 Hr PH and manometry should be performed
Symptomatic, anatomically dependent and enlarging diverticulum should be surgically repaired
Surgical therapy includes diverticulectomy, myotomy and a partial fundoplication as indicated (Transthoracic or Transabdominal)..

Obstetrical Emergencies

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Postpartum Hemorrhage
Defined as >500 ml blood loss vaginal or >1000 ml blood loss after c-section
PPH - Cause
Think of the 4 T’s:
Tone – decreased uterine tone – most common cause
Trauma – Laceration / Uterine inversion
Tissue – retained placental tissue
Thrombin – depleted coagulation factors

PPH – Retained placenta
Failure to deliver placenta in 30 minutes
Treatment:
Gentle cord traction
Consider injection of 20 units of pitocin in the umbilical vein  (2 ml of pitocin in 20 ml saline)
Manual extraction

27 yr G1P0 is in active labor.  Her pregnancy was uncomplicated.  She was complete at 1300.  At 1415 she delivers an OA Head over an intact perineum.  A “turtle sign” is noted.  You suction the fetal mouth and nose and then assist restitution of the head.  Despite maternal pushing, you are unable to deliver the head over the next minute.

Shoulder Dystocia
Definition:  Delivery in which the anterior shoulder of the baby is impacted against the maternal symphysis pubis and is not deliverable in 60 seconds.
Risk Factors
 Prior shoulder dystocia
 Diabetes
 Prolonged gestation
 Fetal macrosomia
 Maternal obesity

Prevention:
Maintenance of good glycemic control in pregnant diabetic women decreases fetal macrosomia
Elective C-section for fetal macrosomia

Third trimester bleeding
Placenta abruption
Placenta previa
Vasa previa
Uterine rupture

Placental Abruption
Painful third trimester bleeding.
1:120 pregnancies, approx. 1%.
Recurrence rate of 10%.
Port wine stained amniotic fluid.

Placenta Previa
Painless third trimester vaginal bleeding
1:200 pregnancies in 3rd trimester
1:50 grand multiparas,1:1500 nulliparas

Advantages of Intrauterine Insemination
Bypass (Vaginal acidity + cervical mucus hostility)
Deposition of a well prepared sperms as close as possible to the oocytes (Short distance)
Non invasive (like pap smear).
Inexpensive.
Antenatal & perinatal complications (like pregnancies from normal S I)

Disadvantages
1.Multiple pregnancy (>IVF) number of follicles will grow or rupture can not precisely controlled.
2. Infection            Iatrogenic infertility.
3. Psychological (guilt- anger- loss of self esteem)

Indications for IUI
Ejaculatory failure  (Hypospadius- Vaginismus- Impotence- retrograde ej.)
Cervical factor        (mucus hostility-poor mucus)                                     
Male subfertility     (Mild, moderate)
Immunological       (Male sperm a.bs- female antisperm a.b)
Unexplained.
Husband is away from wife for long time (work abroad)
Endometriosis (mild).
Ovulatory (?! Induction + timed sexual I).
Combined non tubal infertility factors.
HIV negative women with processed semen of HIV +ve husband.
Cancer-husband: cryopreservation of semen prior to chemo, radiotherapy  or orchidectomy.

Is IUI and /or COH cost effective for male subfertility compared with IVF?  And for how many cycles?
What is the threshold level for numbers of  motile spermatozoa after sperm preparation beyond which treatment outcome is no longer improved? Or what is the minimum number of motile sperms below which IUI is no longer effective

Update on Infective Endocarditis

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Pathogenesis
Disruption of the endocardial layer as a complication of abnormal blood flow associated with underlying cardiac defect
Bacterium-endothelium interaction with bacterial attachment and invasion of endothelial cells

Underlying valvular abnormality predisposing to infective endocarditis
rheumatic fever:a common cause in the past
mitral valve prolapse:currently represents the most common underlying cardiac abnormality

mitral valve prolapse
risk for infective ednocarditis is increased by 5x-8x
mitral regurgitation increases the risk
leaflet redundancy with myxomatous degeneration is a frequent finding

Coagulase-negative Staphylococci
can produce native-valve endocarditis in mitral valve prolapse
usually subacute, difficult to diagnose, and disregarded as a contaminant
delay in diagnosis and treatment may account for the severe complications
 myocardial abscess formation
 valvular insufficiency requiring valve surgery
 death

Prosthetic Heart Valve
positive blood culture in hospitalized patients with underlying prosthetic valves can be a harbinger of endocarditis
43% patients with nosocomial bacteremia or fungemia had prosthetic valve infection
a serious complication

Polymicrobial Infective Endocarditis clinical features
IV drug use is the predominant risk factor
younger age (mean 36.5 years)
right-sided cardiac involvement in > 60%
streptococci more frequent than S. aureus
mortality rate is 4x higher for pure left-sides vs pure right-sided endocarditis

Diagnostic (Duke) Criteria
Major criteria
 positive blood culture for IE
 evidence of endocardial involvement
Minor criteria
 predisposition (heart condition or IV drug use)
 fever of 100.40F or higher
 vascular or immunologic phenomena
 microbiologic or echocardiographic evidence not meeting major criteria

Scleroderma and Pregnancy

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Scleroderma:It is a term which includes a heterogenous group of limited and systemic conditions causing hardening of the skin.
Systemic sclerosis:It is an extension of the disease process which implies involvement of both skin and other sites, particularly central internal organs.

Scleroderma
Types:
Localized: The localized forms are Morphea and Linear, which affect only the skin (and sometimes the underlying tissues) but do not affect the internal organs
Systemic: The systemic forms of Scleroderma cause fibrosis (scar tissue) to be formed in the skin and/or internal organs. The fibrosis eventually causes the involved skin or organs to harden

Pathogenesis
Uncontrolled and irreversible proliferation of normal connective tissue along with striking vascular changes
Collagen,Proteoglycans,Fibronectin,Laminin
The relationship between these inflammatory cells, their mediators (  ) and subsequent fibrosis may be critical in the initial stages of scleroderma

Clinical  Features:
Fibrous thickening affects skin, muscles, joints, tendons, nervous system and certain internal organs especially esophagus, intestinal tract, lungs and kidneys

Skin (Affected in 90% of cases)   
Initially, the skin is edematous, with vasculitis and often petechial hemorrhages.
Enlarged vessels are frequently present & palpable as Telengectasis
Progressive fibrosis follows
Flexion contractures of arms and Painful flexed claw like hands

Kidneys (60% of Cases)
Glomerular changes resulting from immune complex deposition.
Basement membrane Thickening
Mesangial hypercellularity
Intimal fibrosis of small arterioles

Proper diagnosis of Scleroderma is often long and difficult, since it is a rare disease which few doctors are well-versed in, and in the early stages it may resemble many other connective tissue diseases, such as SLE, Polymyositis, and Rheumatoid Arthritis

Scleroderma & Pregnancy
Will Pregnancy be possible ?
It is possible that  patients with Scleroderma can achieve pregnancy although there is increased sub-fertility in such patients.
Will Pregnancy be complicated ?
Third trimester is the dangerous period with the risks of rapidly developing hypertension, renal failure and of interruption of pregnancy
Reflux esophagitis may increase
Small bowel involvement may cause malabsorption / malnutrition.
Changes of pregnancy may cause increased constipation in already diseased large bowel   

DRUGS IN HEART FAILURE

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Drug management aims to provide symptomatic relief  for the patient while also preventing further deterioration in cardiac function

Diuretics
Effective at treating & controlling fluid retention, but do not prevent disease progression.
If used alone can stimulate renin-angiotensin system & accelerate disease process

Loop Diuretics
Reduce symptoms of congestion by reducing preload
Act on Ascending Loop of Henle to increase the secretion & inhibit reabsoprtion of Na, Chloride and H2O
Long term use lowers B/P by arteriolar vasodilatation
Less likely to cause worsening renal function than Thiazides

Thiazide Diuretics
Prevent Na reabsorption by kidneys
Cause reduced blood volume
Long-term use lowers B/P, by arteriolar vasodilatation

Cautions with Diuretics
Hyponatraemia
Hypokalaemia & low Mg
Hypovolaemia & hypotension
Drug Interaction – reduce hypoglycaemic effect of oral hypoglycaemics > hyperglycaemia
Increased concentration Lithium

ACE Inhibitors
Cornerstone of heart failure management
Blockade of the renin-angiotensin-aldosterone system
Decrease cardiac preload & afterload
Prevention of ventricular remodelling
Approximately 7000 patients evaluated in placebo-controlled clinical trials
Consistent improvement in cardiac function, symptoms and clinical status
Decrease in all-cause mortality by 20-25%
Decrease in combined risk of death and hospitalisation by 20-25%

Beta Blockers
Activation of the sympathetic nervous system is a contributory factor in the pathophysiology of heart failure.
Beta Blockers prevent stimulation of the sympathetic nervous system by inhibiting the action of catecholamines (noradrenaline and adrenaline) at beta-adrenergic receptors.

Effects In Heart Failure
Reduce mortality and morbidity
Improve Left Ventricular function & symptoms
Reduce hospital admissions

Systemic Eye Diseases Part 01

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UVEITIS  AND SYSTEMIC DISEASE
Uveitis can be defined as inflammation of the uvea, the middle, vascular coat of the eye.
Uvea consists of iris, ciliary body and the choroid.The ciliary body contains 2 parts:pars plicata (anterior) and pars planta (posterior).

Causes of anterior uveitis:
1-HLA positive or seronegative group:
           A-Ankylosing spondylitis.
           B-Reiter’s syndrome.
           C-IBD”Inflamatory bowel disease”.
           D-Psoriatic arthritis.
2-Systemic Infections “TB, HIV and syphilis”
3-Sarcoidosis, Beh├žet's disease , JRA.
4-Idiopathic

Symptom of anterior uveitis
 * Redness of the eye
 * Blurred vision
 * Sensitivity to light (photophobia)
 * Dark, floating spots along the visual field
 * Eye pain

Phakomatoses (or "neurocutaneous syndromes") are disorders of central nervous system that additionally result in lesions on the skin and the retina which are:
Neurofibromatosis
  Type I (NF-1) - von Recklinghausen disease
  Type II (NF-2) - bilateral acoustic neuromas
Tuberous sclerosis
von-Hippel-Lindau syndrome
Sturge-Weber syndrome ’’encephalotrigeminal angiomatosis ‘’’

Intraocular lesions in NF-1:Lisch nodules,Retinal astrocytomas,Choroidal naevi ect

Ocular complications frequently occur in rheumatoid arthritis
The lacrimal glands
affected by an inflammatory process with consequent inadequate tear flow.
complains of: dry, gritty, and sore eyes.

Episclera  and sclera coats of the eye,
complain of: red, uncomfortable eye tenderness over the area.
Scleritis is usually much more painful than episcleritis and
the engorged vessels are deeper. If continues, the sclera
may become thin (scleromalacia) the eye may perforate.

RENAL TUBULAR ACIDOSIS

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A 35 year old woman, a nursing home assistant, presents with chronic acidosis that is difficult to manage. Lab evaluation showed Na+ 143, K+ 2.8 Cl- 118, HCO3- 15 BUN 18, Cr 0.7. ABG reveals PH 7.38 Pco2 31, Pao2 100. U/A results were normal with urine ph of 5.0. Urine Na was 40 K 5 and Urine Cl 150.Which disorder best characterizes this patient's syndrome.
  1. Diuretic abuse
  2. Laxative abuse
  3. Distal renal tubular acidosis
  4. Proximal renal tubular acidosis
  5. Type 4 renal tubular acidosis
Renal tubular acidosis (RTA) is applied to a group of transport defects in the reabsorption of bicarbonate (HCO3-), the excretion of hydrogen ion (H+), or both.
The RTA syndromes are characterized by a relatively normal GFR and a metabolic acidosis accompanied by hyperchloremia and a normal plasma anion gap.

OBJECTIVES
Physiology of Renal acidification.
Types of RTA and characteristics
Lab diagnosis of Renal tubular acidosis
Approach to a patient with Renal tubular acidosis

Physiology of Renal Acidification
Kidneys excrete 50-100 meq/day of non carbonic acid generated daily.
This is achieved by H+ secretion  at different levels in the nephron.
The daily acid load cannot be excreted as free H+ ions.
Secreted H+ ions are excreted by binding to either buffers, such as HPO42- and creatinine, or to NH3 to form NH4+.
The extracellular pH is the primary physiologic regulator of net acid excretion.

Renal acid-base homeostasis may be broadly divided into 2 processes
Proximal tubular absorption of HCO3- (Proximal acidification)
Distal Urinary acidification.
 Reabsorption of remaining HCO3-  that escapes proximally.
 Excretion of fixed acids through buffering & Ammonia recycling and excretion of NH4+.

TYPES OF RTA
Proximal RTA (type 2)
 Isolated bicarbonate defect
 Fanconi syndrome
Distal RTA (type 1)
 Classic type
 Hyperkalemic distal RTA
 Hyperkalemic RTA (Type 4)

PROXIMAL Renal tubular acidosis
Proximal RTA (pRTA) is a disorder leading to HCMA secondary to impaired proximal reabsorption of filtered bicarbonate.
Since the proximal tubule is responsible for the reabsorption of 85-90% of filtered HCO3- a defect at this site leads to delivery of large amounts of bicarbonate to the distal tubule.
This leads to bicarbonaturia, kaliuresis and sodium losses.
Thus patients will generally present with hypokalemia and a HCMA.

Cirrhosis of the liver

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Cirrhosis is a common chronic, progressive and diffusive liver disease, caused by one or several agents act repeatedly and persistently.
Histologically, cirrhosis is an irreversible alteration of the liver architecture, consisting of hepatic fibrosis and areas of nodular regeneration

Etiology and pathogenesis
Viral hepatitis
Parasites (schistosomiasis)
Alcoholic liver disease
Cholestasis
Hepatic-Venous outflow obstruction
    Veno-occlusive disease
     Budd-chiari syndrome
     Constrictive pericarditis
     Chronic congestive heart failure
Toxicant and drugs
Metabolic abnormality
Malnutrition
Cryptogenic cirrhosis

Clinical manifestation
Compensated stage                                              Decompensated stage
Fatigue                                                                 Deterioration of liver function
Loss of appetite                                                   Feature of portal hypertention
Anorexia                                                                   Portal-systemic collaterals
Abdominal discomfort                                              Ascites
Abdominal pain                                                        Splenomegaly
Hepatomegaly (slightly or moderately)
Splenomegaly

Ascites
Prominent feature of portal-hypertension
70% of patients are positive
An early sign in presinusoidal portal hypertension
Relative late in intrahepatic portal hypertension
Massive ascites:  abdominal herniae

Complications
 Upper gastrointestinal bleeding
 Hepatic encephalopathy
 Infection
 Hepatorenal syndrome
 Primary liver cancer
 Imbalance of electrolytes and acid-alkaline

Hydrocephalus

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The term hydrocephalus is derived from the Greek words “hydro” meaning water and “cephalus” meaning head.  As the name implies, it is a condition in which the primary characteristic is excessive accumulation of fluid in the brain.  The excessive accumulation of (CSF) cerebrospinal fluid results in an abnormal dilatation of the spaces in the brain called ventricles.  This dilatation causes potentially harmful pressure on the tissue of the brain.
Hydrocephalus may be congenital or acquired.  Congenital hydrocephalus is present at birth, and may be caused be either environmental influences during fetal development or genetic predisposition.  Acquired hydrocephalus develops at the time of birth or at some point afterwards.  This type can affect individuals of all ages and may be caused by injury or disease.
Hydrocephalus is the result when the flow of CSF is disrupted when your body doesn't absorb it properly. CSF provides a number of important functions, including acting as a cushion for protection and transporting nutrients to the brain. There are two main causes; obstructive and non-obstructive.

Obstructive (non-communicating)
This type of hydrocephalus results from an obstruction within the ventricular system of the brain that prevents CSF from flowing or “communicating” within the brain.  The most common type is a narrowing of a channel in the brain that connects two ventricles together.

Non-obstructive (communicating)
This type results from problems with the production or absorption of CSF.  The most common is caused by bleeding into the subarachnoid space in the brain.

Early symptoms (infants)
    * Enlargement of the head
    * Bulging fontanels
    * Sutures are separated
    * Vomiting
Late symptoms
        * Decreased mental function
    * Delayed movements
    * Difficulty feeding
    * Excessive sleepiness
    * Brief, shrill, high-pitched cry
    * Slow growth (0-5 years)

Diagnosis
Tapping with the fingertips on the skull may show abnormal sounds associated with thinning and separation of skull bones.
Scalp veins may appear dilated.
Eyes are depressed.

HYPERTENSIVE EMERGENCIES

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“accelerated hypertension”
 term used to describe individuals with chronic hypertension with associated group 3 Keith-Wagener-Baker retinopathy
“malignant hypertension”
 describe those individuals with group 4 KWB retinopathy changes + papilledema

HPT emergency(crisis):  Is characterized by a severe elevation in BP, complicated by evidence of impending or progressive target/end organ dysfunction
HPT urgency: is a severe elevation in BP without progressive target organ dysfunction

Conditions constituting evidence of end organ dysfunction
Hypertensive encephalopathy
Intracerebral heamorrhage
Stroke
Head trauma
Ischemic heart disease (most common)
 AMI
 Acute LVF with P/oedema
 Unstable angina
Aortic dissection
Eclampsia
Life threatening arterial bleed

Prevalence:
With  progress in anti-hypertensive Rx  – decrease in the lifetime incidence of HPT emergencies from 7% to 1%
Hypertensive crisis more common among elderly and black patients
Studies – HPT related problems amount for 25% of all pt visits to medical section of ED. 33% of these - HTN emergencies.

Etiology
Most common
rapid unexplained rise in BP in pt with chronic essential HPT
most have history of poor treatment/compliance or an abrupt discont of their meds

Other causes
Renal parenchymal disease (80% of sec.causes)
Systemic disorders with renal involvement (SLE)
Renovascular disease (Atheroscleroses/fibromuscular dysplasia)
Endocrine ( phaeochromocytoma/cushing syndrome)
Drugs (cocaine/amphetam/clonidine withdrawal/diet pills)
CNS (trauma or spinal cord disorders – Guillain-Barre
Coarctation of the aorta
Preeclampsia/Eclampsia

Hypertensive encephalopathy
Clinical manifestation of cerebral edema and microhemorrhages seen with dysfunction of cerebral autoregulation
Defined as an acute organic brain syndrome or delirium in the setting of severe hypertension
Not adequately treated – cerebral heamorrhage, coma and death.
BUT with proper treatment – completely reversible.

Acute Coronary Syndrome

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Worldwide Statistics
> 4 million patients are admited with unstable angina and actue MI
> 900,000 patients undergo PTCA with or without stent

Myocardial Ischemia
Spectrum of presentation
Silent ischemia
Exertion-induced angina
Unstable angina
Acute myocardial infarction  

Patients are categorized into 3 groups
Non- cardiac chest pain
Unstable angina
Myocardial infarction

Acute Coronary Syndrome
The spectrum of clinical conditions ranging from:
 Unstable angina
 Non-Q wave MI
 Q-wave MI
Characterized by the common pathophysiology of a disrupted atheroslerotic plaque
Angina at rest (> 20 minutes)
New-onset (<2 months) exertional angina (at least CCSC III in severity)
Recent (< 2 months) acceleration of angina ( increase in severity of at least one CCSC class to at least CCSC class III)

Unstable Angina precipitating factors
Inappropriate tachycardia
 Anemia,fever,hypoxia,tachyarrhythmias,thyrotoxicosis
High afterload
 Aortic valve stenosis,LVH
High preload
 High cardiac output, chamber dilatation
Inotropic state
 Sympathomimetic drugs,cocaine intoxication

Unstable Angina prognostic indicators
Presence of ST-Twave changes with pain
Hemodynamic deterioration
 Pulmonary edema, new mitral regurgitation
 3rd heart sound, hypotension
Other predictors
 Left ventricular dysfunstion extensive CAD, age, comorbid conditions(diabetes mellitus, obstructive pulmonary disease, renal failure, malignancy)

Definition Compartment Syndrome
Condition characterized by raised pressure within a closed space with a potential to cause irreversible damage to the contents of the closed compartment

Aetiology
burns
high pressure injection
trauma
 fractures
 crush (behaves differently)
re-perfusion injuries
medical: coagulation, dialysis, traction
Drug addicts

Differential Diagnosis
Arterial occlusion
Crush syndrome
injury to peripheral nerve

Compartment Syndrome of Hand
occur most often from iatrogenic injuries
arterial-line or IV medications

Hand Compartment Syndrome Physical signs
non specific aching of the hand
disproportionate pain
loss of digital motion & continued swelling
 MP extension and PIP flexion “intrinsic position”
difficult to measure tissue pressure

Forearm Compartment Syndrome
Common after high energy injury
 wrist, forearm fracture
may follow supracondylar fracture of humerus
may result in functionless extremity

Blunt Trauma in the Pregnant Woman

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Trauma occurs in 6-7% of pregnancies in US
Leading nonobstetric cause of  maternal death
Female drivers are more likely to be in a MVA than male drivers

Physiologic Changes in Pregnancy
Pregnant woman can lose 30% (2L) of blood volume before vital signs change
At 30 wks GA the uterus is large enough to compress the great vessels causing
 up to a 30mm Hg drop in systolic BP
 30% drop in stroke volume
A series of 441 pregnant trauma victims with no detectable fetal heart tones showed no fetal survivors.

Results of Large Population Study
Fetal demise prior to 20 weeks gestation not included in this study
Gestational age was the strongest predictor of fetal, neonatal and infant death
What and how severe the trauma was not as strong a predictor as gestational age
Highest risk at <28 weeks gestation
Trauma may cause subclinical, chronic plancenta abruptions
causing insufficient uterine blood supply
Woman involved in a trauma during pregnancy need close monitoring during labor

Fetal Demise
Rate of fetal demise after blunt trauma 3.4-38%
Lead causes
 Placental abruption
 Maternal shock
 Maternal death
1,300-3,900 pregnancies are lost due to trauma each year
Abruption occurs in  40-50% of pregnant woman in severe traumas compared to 1-5% in minor trauma

Uterine Rupture
0.6% of all injuries during pregnancy
Various degrees ranging from seosal hemorrhage to complete avulsion
75% of cases involve the fundus
Fetal mortality approaches 100%
Maternal mortality 10%
 Usually due to other injuries

Preterm Labor
Incidence following trauma is unknow
Estimated to be under 5%
Theory: caused by destabilization of lysosmal enzymes that initiate prostaglandin production
Consider admistering slow-released progesterone for all woman with contracts after trauma

Pathophysiology
Extension of inflammation into peripancreatic tissues
Involve CBD, duodenum, colon, Portal vein, Splenic vein
To be distinguished from compression caused by pseudocysts

Bile duct stricture
Equal incidence in alcoholic & non-alcoholic Chronic Pancreatitis
Higher incidence in CP with inflammatory head mass

Bile duct stricture Complications
Cholangitis 6%-15% of stricture patients
Biliary cirrhosis- 3% to 10%
Liver fibrosis
 Common -up to 73%
 Not evaluated often
 Reduction after decompression

Management options
Endoscopic stenting
Technically possible nearly in all
Risk of stent block & cholangitis
Regular change mandatory with plastic stents
Better results with metallic stents

Results with metallic stents
Most patients with metallic stents will develop recurrent cholangitis or stent obstruction
Chronic inflammation and obstruction may predispose the patient to cholangiocarcinoma

Surgical options
Choledochoduodenostomy
Choledochojejunostomy
Cholecystoduodenostomy
Cholecystojejunostomy
T-tube drainage
Resectional procedures

Surgical procedures have,
Higher initial post procedure risk
Acceptable morbidity in the majority
Near zero mortality
Excellent long term results

Stenting vs surgery
Initial therapy before surgery
Can be the definitive approach for older and morbid patients
Should not be considered as a routine procedure for symptomatic cases

Why we are rushing towards minimally invasive surgery?
Less disruptive to tissues
Patients generally recover faster with less pain
Fewer wound problems
Less scarring

In operative laparoscopies we often use electricity either to cut, desiccate or coagulate;but major catastrophes may arise if non targeted tissue is injured.

Complications
Three factors lead to stray energy burns
Direct coupling
Capacitive coupling
Insulation failure

The low voltage “cut” mode exhibits less capacitive coupling than coag does.
Surgeons must recognize that open circuit activation (electrode not touching tissue) dramatically increases voltage and the possibility of capacitive coupling.
It is desirable to use as low wattage as possible and to limit noncontact activation of the generator.

How to avoid
When possible, place the long edge of the electrode closest to the surgical site and on the same side of the body as the incision if it is a sided procedure.
Choose a well vascularized muscle mass.
Avoid areas of vascular insufficiency, irregular body contours, bony prominences.
Remove excessive hair.
Check equipment before each use
Patient skin is not in contact with metal or, if so, these areas are Insulated.
Solutions are not stored on top of power unit.
Power cord, dispersive pad cord, and cautery pencil cord are carefully placed to avoid possibility of being tripped.
It is recommended that Cords not be wrapped around metal instruments Cords not be bundled together
Use bipolar electrosurgery when appropriate
Select an all metal cannula system as the safest choice. Do not use hybrid cannula systems that mix metal with plastic.
Activate the electrode when touching tissue.
Clean the active tip routinely during surgery to prevent eschar buildup, which can cause tissue to stick and set up resistance to current flow.
Visually inspect instruments throughout each procedure.

Perioperative Management of Patients with Cardiac Rhythm Management Devices
Assure that the electrosurgical receiving plate is positioned so that the current pathway does not pass through or near the cardiac rhythm management devices  (CRMD system)
avoid proximity of the cautery's electrical field to the pulse generator or leads.
use short, intermittent, and irregular bursts at the lowest feasible energy levels.
reconsider the use of a bipolar electrocautery system or ultrasonic (harmonic) scalpel in place of a monopolar electrocautery system, if possible.

Shunt Dysfunction and Infection
Infection
  Skin breakdown over hardware
Mechanical failure
  Undershunting
  Separation of shunt components, fractures, migration of hardware
  Overshunting
  Subdural hematoma

These account for majority of shunt problems

Undershunting
Etiology
Blockage within system
Choroid plexus
Glial adhesions
Build-up of proteinaceous accretions, blood, cells (inflammatory or tumor)
Ventricular end most common site

Disconnection, kinking, or breakage of system
With age, silicone elastomers calcify, break down, & become more rigid & fragile which may promote subcutaneous attachments
Barium impregnation may accelerate process
Tube fracture often occurs near clavicle, likely due to ↑ motion there

Evaluation
History
 Symptoms of active hydrocephalus
 Reason for initial insertion of shunt
 Date & reason of last revision
 Type of hardware
Physical
 Signs of active hydrocephalus
 For children, plot head circumference on graph of normal curves
 Before sutures close, head circumferences crossing growth curves
 Swelling along shunt tubing from CSF dissecting along shunt tract
 Ability of shunt reservoir to pump & refill
  May exacerbate obstruction, esp if shunt is occluded by ependyma due to overshunting initially
In children presenting only w/ N/V, esp those w/ cerebral palsy & feeding G-tubes, R/O GE reflux

Shunt Tap
To obtain CSF specimen
To evaluate shunt function
As temporizing measure to allow function of distally occluded shunt
To inject medication
For catheters placed within tumor cyst (not a true shunt)

Connective Tissue Diseases
Systemic lupus erythematosus
Antiphospholipid antibody syndrome
Ankylosing spondylitis
Rheumatoid arthritis
Scleroderma
Polymyositis and dermatomyositis
Mixed connective tissue disease


Cardiac Diseases in Pts with SLE
Pericardial disease
 Pericarditis
Valvular disease
 Fibrin deposits (Libman-Sacks)
 Fibrous thickening of leaflets and chordae
 Valvular regurgitation and/ or stenosis
 Infective endocarditis
Coronary artery diseases
Myocardial diseae
Hypertrophy

Valvular Disease Associated with SLE
Valve masses or Libman-Sacks vegetation
Leaflet thickening
Valvular regurgitation
Valvular stenosis

Libman-Sacks Vegetation
Cauliflower-like or flat, red multiple spreading masses of 2 – 4 mm in diameter present on the free margins or line of closure of the heart valve

Aortic Root Disease and Valve Disease Associated with Ankylosing Spondylitis
Pathology
 The inflammatory process predominantly of the adventitia and intima of the aortic root results in a fibroblastic reparative response and vascularized fibrous tissue thickening
Aortic valvulitis
 Cusp thickening and retraction
 Thickening of the aorto-mitral junction or subaortic bump
 Proximal aortitis leading to aortic root thickening and dilation
 Aortic and mitral regurgitation..

Cardiac Output
Measures the effectiveness of the heart’s pumping abilities.
CO is defined as the amount of blood that leaves the heart in one minute.
    CO = Stroke Volume (SV) X Heart Rate (HR)
Normal CO: Approximately 4-8 liters/minute
Cardiac Index: CO per square meter of BSA

Stroke Volume (SV)
The amount of blood that leaves the heart with each beat or ventricular contraction.
Not all blood ejected
Normal Adult 70 ml / beat
Ejection Fraction (EF)
The percentage of end-diastole blood actually ejected with each beat or ventricular contraction.
Normal adult 55-70% (healthy heart)

Three factors regulate stroke volume:
Preload - The degree of stretch of the ventricle at the end of diastole.
Contractility - Force of ventricular contraction (systole); inotropy.
Afterload - The amount of resistance the ventricular wall must overcome to eject blood during systole.

Serum Enzymes: Cardiac 
Creatine Phosphokinase  (Total CK / CPK)
 Non-Specific: enzyme elevated with damage to  heart or skeletal muscles and brain tissue.
  Elevates in 4 to 8 hours
  Peaks in 15 to 24 hours
  Returns to normal in 3 to 4 days
Creatine Phosphokinase Isoenzyme (CPK-MB)
 Specific: isoenzyme of CPK; elevated with cardiac muscle damage.
  Elevates in 4 to 8 hours
  Peaks in 15 to 24 hours
  Returns to normal in 3 to 4 days
Myoglobin
 Non-specific: a heme protein found in muscle tissue; elevated with damage to skeletal or cardiac muscle.
  Elevates in 2 to 3 hours
  Peaks 6-9 hours
  Returns to normal 12 hours
Lactic Acid Dehydrogenase (LDH)
 Non-specific: enzyme elevated with damage to many body tissues. (i.e. heart, liver, skeletal muscle, brain and RBC’s); Not frequently used today.

Urinary Incontinence in Older Adults

Posted by e-Medical PPT

Urinary incontinence is defined as the involuntary leakage of urine
Some definitions have required that this be considered a ‘social or hygienic problem’
More widely accepted definitions assume all incontinence fits this criteria
However, the degree of bother to the patient and caregivers is an important consideration

Overactive bladder’ = OAB
Characterized by urinary urgency and frequency
May or may not be associated with incontinence
‘Overactive bladder wet’ =  + leakage of urine
‘Overactive bladder dry’ =  urgency and frequency without leakage

Urinary incontinence (UI) may be chronic (established) or transient (temporary)
This module and the ACOVE Indicators focus primarily on chronic UI

Effects of Urinary Incontinence
Urinary Incontinence has significant negative impact on both overall and health-related quality-of-life (QoL)
Social isolation
Depression
Psychological distress
Increased caregiver burden
Skin inflammation / breakdown
Sleep disturbance
? Increased risk urinary tract infection (UTI)
Limitation or avoidance of sexual activity

Physiology of Micturition
Complex series of coordinated events
Bladder filling and storage of urine
Comprehension of bladder sensations
Physical activity associated with normal voiding
 Mobility to reach toilet facilities
 Transfers on and off toilet
 Ability to adjust clothing appropriately
Voiding process with adequate emptying
Completion of voiding, re-dressing, transfer off of the toilet

Storage of urine
Requires the bladder to fill at low pressures
No unstable bladder contractions
Adequate stretch of bladder detrusor muscle (compliance)
Closed external urinary sphincter
Normal bladder capacity ~ 300 – 500 mL
First sensation of filling ~ 150 – 200 mL
Sensation of need to void ~ 200 – 400 mL...

Management Of Genital Prolapse

Posted by e-Medical PPT

Prolapse/Procidentia is downward decent of uterus &/or vagina (Procidentia is from Latin procidere - to fall)
It is a state of pelvic relaxation due to a disorder of pelvic support structures that is, the endopelvic fascia
It is not a disease but a disabling condition

WEAKNESS OF THE SUPPORTS OF THE UTERUS & VAGINA
Precipitating / Exaggerating / Unmasking Causes -
INCREASED INTRA ABDOMINAL PRESSURE
 Chronic cough
 Chronic Constipation
 Heavy Wt.Lifting / domestic Work
 Obesity, Ascitis
WEAKNESS OF THE SUPPORTS & MUSCLES
 Chronic ill health, malnutrition dysentery, anemia
 Inadequate rest during pureperium
 Menopause

TYPES OF PROLAPSE
Vaginal
Anterior –cystocele & urethrocele
Posterior - Enterocele & Rectocele
Vault Prolapse - a special term applied to the prolapse of upper vagina

Uterine/Utero-vaginal- Acquired or Congenital.
First degree.
Second degree &.
Third degree-(total Prolapse / complete procidentia).
However Procidentia is often used only to denote third degree uterine prolapse.

HOW TO TREAT ?
NON-SURGICAL Methods: -Limited Role
PELVIC FLOOR REHABILITATION (pelvic muscle exercises, galvanic stimulation, physiotherapy, rest in the purperium).
HORMONE REPLACEMENT, both systemic and local.
PESSARY TREATMENT for temporary relief
 During Pregnancy, Pureperium & Lactation
 When Operation is Unsafe due to Extreme Senility/Debility and Diseases
 Preoperatively
 For therapeutic test
SURGICAL TREATMENT: -RECONSTRUCTIVE SURGERY is invariably needed and has to be a COMBINATION OF PROCEDURES to correct the multiple defects.

Epilepsy in Pregnancy

Posted by e-Medical PPT

Effects Of Pregnancy On Epilepsy
1-Seizure frequency may increase: due to:
-Enhanced metabolism & increased drug clearance associated with
pregnancy can result in decreased serum drug concentration.
-Increased volume of distribution of the AED.
-Increased serum binding proteins.
-Decreased or non-compliance with medication.
-Sleep deprivation, hormonal changes of pregnancy (high E), and
associated psychological and emotional stress of pregnancy: all lower 
threshold for seizures.

Effect Of Epilepsy On Pregnancy
Data  on 1st trimester losses, PROM, ante-partum hemorrhage, operative vaginal delivery and CS are inconclusive.
Increased incidence of IUGR, cognitive dysfunction, microcephaly and perinatal mortality (1.2 - 3 times normal).
Increased incidence of congenital malformations.

Effects Of Epilepsy On Fetus And Neonate
1-There is increased risk for infants of epileptic mothers to have epilepsy. The risk of neonatal susceptibility depends on:
Nature of the mother’s seizure disorder.
Genetic factors.
Seizures arises during pregnancy.
Metabolic & toxic consequences of seizures and AEDs.
2-Increase perinatal morbidity.

Specific Syndromes Of Malformations
Fetal Hydantoin Syndrome:
11% of infants exposed will have the syndrome.
There is pre and postnatal growth deficiency, dysmorphic facies and mental retardation.

Facial Valproate Syndrome:
Brachycephaly with high forehead, shallow orbits, small nose, small mouth & low posterior ears.
Long overlapping fingers & toes & hyperconvex nails.
Cleft palate & congenital heart diseases..

Bronchopulmonary Dysplasia(BPD)

Posted by e-Medical PPT

Develops in neonates treated with O2 & PPV .
Originally described by Northway in 1967 using clinical , radiographic & histologic criteria .
Bancalari refined definition using ventilation criteria , O2 requirement @ 28days to keep PaO2>50mmhg & abnormalities in chest x –ray .
Antenatal steroids , early surfactant Rx & gentle modes of ventilation minimize severity of lung injury .

Pathophysiology
It is Multifactorial
Major organ systems involved are lungs & heart
Alveolar stage of lung development - 36wks GA to 18 months post conception
Mechanical ventilation & O2 interferes with alveolar & pulmonary vascular development in preterm mammals .
Severe BPD leads to Pulmonary HT & abnormal pulmonary vascular development .

Stages of Bronchopulmonary Dysplasia
Stage 1 - similar to uncomplicated RDS
Stage 2 - pulmonary parenchymal opacities with bubbly appearance of lungs
Stage 3 & 4 – areas of atelectasis , hyperinflation & fibrous sheaths
Recently CT & MRI of chest – reveals more details of lung injury

Infants with severe BPD have Increased risk of pulmonary morbidity & mortality within the first 2 years of life.

Pulmonary Complications of BPD
Increased resistance & airway reactivity evident in early stages of BPD along with increased FRC .
Severe BPD results in Significant airway obstruction with expiratory flow limitations & further increased FRC secondary to air trapping & hyperinflation

Physiologic Bleeding in Newborn
Vaginal bleeding in response to loss of exposure to maternal estrogen
Usually occurs within first days of life and should have resolved by 2nd week of life

Leukorrhea
Normal physiologic vaginal discharge
Gray white or light yellow in color
Non purulent
Develops early in puberty
Desquamation of vaginal epithelium due to transition to acidic vaginal environment

Labial Adhesions
Partial or complete fusion of labia minora
Usually begins posteriorly and extends upward
Acquired rather than congenital, due to labial irritation/inflammation

Imperforate Hymen
Usually missed in newborn period
Often not picked up until puberty and development of hematocolpos
Presenting symptoms
 Cyclical abdominal pain
 Abdominal distention/mass
 Urinary retention
 Constipation

Vuvlovaginitis
Why are prepubescent children physiologically more susceptible to vulvovaginitis?
Non-estrogenized vulvar and vaginal epithelium is thinner and more susceptible to irritation
Neutral or alkaline ph- good medium for bacterial growth
Vagina of prepubescent child is colonized with 9 different organisms

Complications of laparoscopy

Posted by e-Medical PPT

Operative laparoscopy becomes more widely accepted,
New techniques are being developed and More surgeons are adopting this form of management,
The complication rate can be expected to rise.

The incidence of laparoscopic complications is:1.1% to 5.2% in minor procedures and 2.5% to 6% in major ones

To reduce the prevalence of complications:
Training programmes must include supervision at all levels of development and
There must be a high degree of awareness of the potential risks of laparoscopic surgery.

Complications may be associated with:
The anesthetic
The induction of pneumoperitoneum
Insertion of primary and secondary trocars
Thermal Instruments
Mechanical Instruments
Other associated conditions..

Incidence of Ureteric Injuries
The incidence of ureteric injury varies between 0.1% and 30%, depending on the type of surgery.
1-Obstetric and gynecological surgeries account for approximately 50% of ureteric injuries.
2-Ureteric injuries are less common during vaginal{0.1%} than abdominal hysterectomies1%.
3-Alought prevalence of ureteric injury being higher following gynaecological cancer surgery, it is the benign gynaecological surgery that accounts for most cases.
4-The incidence of all major complications associated with laparoscopy have declined but ureteric injuries have stayed constant at approximately 1 %. 
38% occur during the treatment of endometriosis.

Risk factors for ureteric injuries:
Anatomical risk factors:
Has close attachment to the peritoneum.
Closely related to FGT.
Has variable course.
Not easily seen or palpated.

Pathological risk factors:
1-Congenital anomalies of ureter or kidney.
2-Ureteric displacement by: 
-Uterine size ≥12 weeks 
-Prolapse. 
-Tumor{ ovarian neoplasms}. 
-Cervical or broad ligament swellings.
3-Adhesions:   
-Previous pelvic surgery.   
-Endometriosis.  
-PID
4-Distorted pelvic anatomy.

Sites of Ureteric Injuries:
During laparoscopy the ureter is injured most frequently adjacent to the uterosacral ligaments

MINILAP HYSTERECTOMY

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MINILAPAROTOMY is an established surgical technique long established for female sterilisation.
The same concept is extended to abdominal hysterectomy for benign conditions in selected patients.

PATIENT SELECTION
Average built patient
Mobile uterus less than 12 weeks size
Cervix can be pushed above symphysis pubis
Absence of large adenexal mass

PROCEDURE
Suprapubic transverse incision, 5-6 cm. Long
No pack or retractor
Uterus delivered outside
With fingers or
After fixing with Myomectomy screw / Volsellum or
After Myomectomy
Hysterectomy with or without BSO done by standard procedure
Vagina closed with one or two interrupted sutures
Pelvic & parietal Peritonium not closed
Rectus sheath apposed with continuous suture
Routine & liberal use of diathermy

Mini lap hysterectomy is an easy to learn minimally invasive surgical procedure.
It has a shorter duration of surgery, faster recovery and reduced hospital stay.
The morbidity is very less as general peritoneal insult is avoided.

This patient, aged 35, having had 2 children came to me with a history of a lump in her abdomen. On examination she had a hard mass which was easily palpable up to umbilicus. Diagnosis was easy and it was confirmed by per vaginal examination. She was a school teacher and an operation was convenient to her only after 2 months, during school vacation.
She was medically fit for surgery and we operated her under spinal anesthesia. Hysterectomy was planned and operation, when done systemically it was not as difficult, as initially thought.
Sonography has made it vary easy to detect even small
size fibroid.
It was great tolerance power of the patient to live peacefully, even after knowing that she has so many big fibroids in uterus, requiring operation at the earliest, was appreciated.

Labial Fusion in Gynaecology

Posted by e-Medical PPT

Partial or complete adherence of the labia minora or majora.

Patho-physiology
-Interruption of the dermo-epidermal junction
-combination of factors hypoestrogenaemia +local irritation, inflamm or trauma
-Auto-immune

Labial fusion in children
Incidence
(rare before 3 months & uncommon after age of 5 years)
It is a common ped. Problem (1.8- 3.3%)
Associated with hypoestrogenaemia
It can be a marker of LS or vulval dis or CSA
Treatment:
-Estrogen cream…how long? BUT Sugical     treatment can cause high recurrence, traumatic,     GA
- hygiene, swaps or biopsy (Rare)

D.D. of labial fusion in children
Developmental abnormalities (newborn)
Infection ( Vulvo-vaginitis, pin warms )
Lichen sclerosis
CSA (child sexual abuse)
Hypo-estrogenaemia

Postpartum labial fusion
Extremely rare
Multiple lacerations heals spontaneously
No EB management of multiple lacer.
separation of adhesions
 Estrogen cream
 Amniotic membrane grafting in recurrent cases

Omphalocoele

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Omphalocoele is an Anterior abdominal wall defect at the base of the umbilical cord through which intraabdominal contents are extruded

Gastroschisis is a linear defect of the abdominal wall that permits extrusion of the viscera without involving the umbilicus
It is just lateral to the umbilicus.

Umbilical hernias occur when intestines do return to the abdomen, but later herniate through the umbilicus
Defect in linea alba,subcutaneous tissue and skin covering the protruding bowel
Frequent in premature infants

Clinical Findings (Omphalocoele)
Defect may vary from 2-10 cm
Sac is composed of amnion, Wharton’s jelly and peritoneum
50% have
accompanying liver,spleen, testes/ovary
>50% have associated defects
Location:Epigastric,Central or Hypogastric
Cord attachment is on the sac
Umbilical cord inserts onto the membrane
Large defects can extend from umbilicus to costal margin – contain liver, small and large bowel

Etiology
Result of disturbances in organogenesis during embryonic period (Errors in Midgut Development)
Three theories of Omphalocoele formation
• Persistence of primitive body stalk beyond 12 weeks
• Failure of bowel to return to the abdomen at 10 weeks
• Failure of complete lateral-body fold migration & body wall closure

Associated anomalies
40% have chromosomal abnormalities (Trisomy 13, 18, 21, Turner’s and Klinefelter synd)
45-88% infants have associated malformations
• Cardiovascular, genitourinary, CNS
• Beckwith-Wiedman syndrome
• Pentalogy of Cantrell
Prognosis based on associated anomalies

Discuss Lupus diagnostic criteria and clinical manifestations
Explore Mechanisms of Lupus
Defective apoptosis
IFN-a
TLR signaling
B-cell disregulation
Pathology of autoantibodies
Discuss how research into understanding the mechanisms of SLE is advancing treatment for lupus

Lupus Diagnostic Criteria
1. Malar Rash: Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
2. Discoid rash: Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions
3. Photosensitivity: Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
4. Oral ulcers: Oral or nasopharyngeal ulceration, usually painless, observed by physician
5. Nonerosive Arthritis: Involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion
6. Pleuritis or Pericarditis: a) Pleuritis--convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion OR b) Pericarditis--documented by electrocardigram or rub or evidence of pericardial effusion
7. Renal Disorder: a) Persistent proteinuria > 0.5 grams per day or > than 3+ if quantitation not performed OR b) Cellular casts--may be red cell, hemoglobin, granular, tubular, or mixed
8. Neurologic Disorder: a) Seizures--in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance OR b) Psychosis--in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance
9. Hematologic Disorder: a) Hemolytic anemia--with reticulocytosis OR b) Leukopenia--< 4,000/mm3 on ≥ 2 occasions OR c) Lymphopenia--< 1,500/ mm3 on ≥ 2 occasions OR d) Thrombocytopenia--<100,000/ mm3 in the absence of offending drugs 10. Immunologic Disorder: a) Anti-DNA: antibody to native DNA in abnormal titer OR b) Anti-Sm: presence of antibody to Sm nuclear antigen OR c) Positive finding of antiphospholipid antibodies on: an abnormal serum level of IgG or IgM anticardiolipin antibodies, a positive test result for lupus anticoagulant using a standard method, or a false-positive test result for at least 6 months confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test 11. Positive Antinuclear Antibody: An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs Arthritis Painful joints are #1 symptom in 76-100% of patients With or without signs of inflammation, mild effusions if any Usually involve hands, wrists, knees Can be asymmetric Generalized myalgias particularly deltoids and quads <15% with elevations in CPK Nephritis Very closely linked with prognosis 5- and 10-year survival rates are documented as high as 85% and 73%, respectively (used to be about 0% before immunosuppressants) Diagnosed by presence of proteinuria (>500mg/24 hours)
Watch for symptoms of edema, puffy eyes, frequent urination.
Biopsy helpful to determine whether aggressive treatment is required

Physiologic changes with aging in the urinary tract
Decreased bladder capacity and increased urine production (especially at night)
Decreased voided volume
Decreased estrogen w/menopause leads to thinning of vaginal & urethral mucosa
Decreased lower urinary tract sensory threshold
Problems of urinary storage & emptying
Increase incidence of overflow incontinence from urethral obstruction or stricture
Decreased estrogen levels leads to pH changes in vagina, favoring colonization of E. coli, ↑risk of UTI
Prostatic enlargement can lead to urinary obstruction, increased residual urine & infection

Age-related Risk Factors for UTI
Advanced Age
Fecal incontinence/impaction
Incomplete bladder emptying or neurogenic bladder
Vaginal atrophy/estrogen deficiency
Pelvic prolapse/cystocele
Insufficient fluid intake/dehydration
Indwelling foley catheter or urinary catheterization or instrumentation procedures
Diabetes or immunosuppression
Benign prostatic hypertrophy
Bladder or prostate cancer
Urinary tract obstruction
Spinal cord injury

UTI’s in elderly men are always considered complicated
UTI’s in women are complicated when:
Hx of recurrent UTI
Secondary to structural abnormalities
Catheters
Stones
Urinary retention
Abscess formation or urosepsis
Primary diagnostic and treatment focus in research studies have been related to the elderly female population

UTI in Women
Escherichia coli—gram (-) etiologic agent in ~ 80% of all UTI’s
Research indicates primary source of microbial invasion is retrograde colonization by intestinal pathogens
Other factors influencing colonization: vaginal pH, urethral length, capacity of bacteria to adhere to urothelium..

Posterior Fossa Skull Base Lesions

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Acoustic Neuroma (AN)
Benign schwann cells in collagenous matrix and don’t invade (usually cause symptoms by encroaching)
Usually arise from vestibular (95%) nerve in IAC but if they arise medial then symptoms develop later
95% are unilateral and nonhereditary
Slow growing (0.2-4mm/yr)

Hereditary AN’s
Neurofibromatosis
Type I
Only 5% with AN’s and no bilateral AN’s
Intra and extra-cranial
Appear late
Chromosome 17

Type II
Bilateral AN’s in 96%
Cranial nerve schwannomas
Appear by 2nd decade
Chromosome 22

Signs and symptoms
Sensorineural Hearing Loss,NHL (95%),SSNHL (20%), tinnitus (56%)
Dysequilibrium (50%), vertigo, nystagmus
Facial hypesthesia and loss of corneal reflex
Long tract signs, ataxia
Headaches and nausea
Hitselberger sign

Meningioma
Originate cap cells near arachnoid villi which are more prominent near cranial nerve foramina and venous sinuses.
Grossly appear speckled due to psammoma bodies
25% Cause hyperostosis
Same symptoms as AN but arise from posterior surface of petrous bone so audiometric (75% HL) and vestibular testing is less sensitive.
Only 75% have abnl ABR

Epidermoid
Originates from epithelial rests within temporal bone or CPA.
Stratified squamous epithelial cells lining desquamated keratin
Same symptoms as AN but facial tic and paresis more common
Expand along least resistance so irregular shapes and borders and discovered in 2nd-4th decades.

Vertigo

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Is the dizziness is vertiginous in nature, or more suggestive of near-syncope or non-vestibular dizziness
Is the dizziness is affected by movements of the head, if so it suggests a peripheral vertigo
·a patient with peripheral acute vertigo often prefers to sit upright and not lie down, or prefers to lie still with the unaffected ear undermost; and the patient also prefers to avoid any sudden head movements
·a patient with central vertigo often has a lesser degree of dizziness, which is less affected by head movements and not specifically related to a particular head position
Dizziness – is it vertigo ?
Dizziness and head movement
Vertigo lasting seconds, which is only precipitated by sudden movements of the head (looking up, suddenly twisting the head, suddenly getting up from a supine position or when suddenly rolling over in bed) suggests benign positional vertigo
·a patient with benign positional vertigo may be able to identify a particular movement or position of the head that precipitates vertigo, which usually occurs after a latent period of 10 - 20 seconds
·patients with BPV may complain of non-specific nausea, dysequilibrium and dizziness between attacks

Dizziness – duration assists differential diagnosis
The duration of the vertigo provides useful information
·vertigo lasting seconds suggests benign paroxysmal positional vertigo
·vertigo lasting minutes suggests transient cerebrovascular ischaemia (posterior circulation TIA)
·vertigo lasting hours suggests Meniere's syndrome
·vertigo lasting hours-days suggests vestibular neuronitis or posterior circulation strokes

Dizziness and otological symptoms
Deafness and/or tinnitus suggests peripheral vertigo
·Many patients with vestibular neuronitis have a history of a recent viral illness in the past few weeks. Otological symptoms in a patient with acute vestibular neuronitis suggests acute (serous) labyrinthitis
·Recent severe earache +/- ear discharge +/- fever suggests a middle ear infection and a possible acute (purulent) labyrinthitis
·Recent head trauma, sudden coughing/sneezing + sudden 'pop' in the ear, or recent scuba diving suggests a possible peri-lymphatic fistula (vertiginous symptoms may also be exacerbated by valsalva-type maneuvers or a loud noise)

Acute Abdomen in the ICU Patient

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Acute abdominal pathology that if left untreated will increase patient MORBIDITY & MORTALITY

Physiology
Visceral and parietal peritoneum
Peritoneal fluid normally <50ml
Absorbed via lymphatics in omentum and diaphragmatic peritoneum (30%)
Omentum acts as physiologic “patch” for perforation or infection
Pain – somatic and visceral
Somatic from direct irritation of parietal peritoneum, visceral follows embryologic origin or major splanchnic vessels
Refered pain – ex. Shoulder and phrenic nerve
Pathophysiology
Similar incidence of common diseases as general population plus more unique processes
Post-surgical state
Hypotension and low flow states
Antibiotic therapy (Overgrowth ex. C. diff)
Narcotics
Poor nutrition
Co-morbidities
Trauma

Postoperative Considerations
Bleeding
Anastamotic leak
Fascial Dehiscence
Bowel obstruction
Abscess
Abdominal Compartment Syndrome

Bowel obstruction
Diagnosis often confounded by normal post-op adynamic illeus
Patients on narcotic pain meds
Management per standard protocol
Complete obstruction or nonresolving/ worsening PSBO requires reoperation..

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