Polymyalgia rheumatica

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Polymyalgia rheumatica is a syndrome with pain or stiffness, usually in the neck, shoulders, and hips. The pain can be very sudden, or can occur gradually over a period of time. It may be caused by an inflammatory condition of blood vessels, muscle biopsies however are normal.Patients who have polymyalgia rheumatica may also have temporal arteritis, a potentially dangerous inflammation of blood vessels in the face.
The classic symptoms include:
    * Pain and stiffness (moderate to severe) in the neck, shoulders, and hips, which inhibits activity, especially in the morning/after sleeping.
    * Fatigue and lack of appetite
    * Anemia
    * Mild fever
There is no specific test to diagnose polymyalgia rheumatica. There are many other diseases which cause inflammation and pain in muscles, but there are a few tests that can help narrow down the cause of the pain.
Prednisone is the drug of choice for PMR and treatment duration is frequently greater than one year.

Renal Failure in Multiple Myeloma

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Basic Concepts of Renal Failure
“When 75-80% of renal function is lost, the kidneys lose their ability to regulate the internal environment and all organ systems and physiologic processes are affected by renal failure”
“Uremia or the uremic syndrome refers to the constellation of signs, symptoms and physicochemical changes that occur with either acute or chronic renal failure.”

Some Uremic Changes:
Fluid – electrolytes imbalances
 fluid retention causing edema in legs, abdomen, and face
 hyperkalemia, hypocalcaemia, hyperphosphatemia
Changes in cardiovascular system
 Includes the RAAS due to decrease in renin production interrupting feedback loop
Changes in inflammatory –immune response
 Altered T-cell function
 Altered B-cell number and function
Accumulation of toxins
 Urea
 Creatinine   
 Uric acid   
 Beta 2 microglobulin

What is Myeloma?
Overproduction of  a single immunoglobulin by cancerous plasma cells
Immunoglobulin is “a protein  produced by body’s immune system to help fight infections”
Normally the body make many different types of immunoglobulins (polyclonal)
In Myeloma  the cancerous cells are monoclonal and are usually of no use to the body
These cells are called M-protein and can be detected in urine.
Research is being done to look at the various reasons for  these changes – especially at certain genetic variations  (such as Chromosome 13 deletion)

Renal Failure in Myeloma
Mechanism of inflammation and damage
Light chain cast deposition – tubule obstruction (“myeloma kidney”) by protein deposits
Obstruction of tubules by plasmacytoma
Hypercalcemia, hyperuricemia: both of which can cause inflammation in the tubules due to high concentrations
Renal amyloid: can cause obstruction which will cause inflammation in the glomerulus
Recurrent pyelonephritis chronic recurrent infections that lead to chronic inflammation of the basement membrane of the nephron tubule
May develop RTA2 (Fanconi syndrome)

Vesicoureteral reflux (VUR)

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Vesicoureteral reflux (VUR) is an abnormal movement of urine from the bladder into ureters or kidneys.Vesicoureteral reflux may present before birth as prenatal hydronephrosis, an abnormal widening of the ureter or with a urinary tract infection or acute pyelonephritis.Symptoms such as painful urination or renal colic/flank pain are not symptoms associated with vesicoureteral reflux.Newborns may be lethargic with faltering growth, while infants and young children typically present with pyrexia, dysuria, frequent urination, malodorous urine and GIT symptoms, but only when urinary tract infection is present as the initial presentation of VUR.

Primary VUR
Insufficient submucosal length of the ureter relative to its diameter causes inadequacy of the valvular mechanism. This is precipitated by a congenital defect/lack of longitudinal muscle of the intravesical ureter resulting in an ureterovesicular junction (UVJ) anomaly.

Secondary VUR
In this category the valvular mechanism is intact and healthy to start with but becomes overwhelmed by raised vesicular pressures associated with obstruction, which distorts the ureterovesical junction. The obstructions may be anatomical or functional. Secondary VUR can be further divided into anatomical and functional groups as follows:
Anatomical: Posterior urethral valves; urethral or meatal stenosis.
These causes are treated surgically when possible.
Functional: Bladder instability, neurogenic bladder and non-neurogenic neurogenic bladder Urinary tract infections may cause reflux due to the elevated pressures associated with inflammation.

    * Grade I – reflux into non-dilated ureter
    * Grade II – reflux into the renal pelvis and calyces without dilatation
    * Grade III – mild/moderate dilatation of the ureter, renal pelvis and calyces with minimal blunting of the fornices
    * Grade IV – dilation of the renal pelvis and calyces with moderate ureteral tortuosity
    * Grade V – gross dilatation of the ureter, pelvis and calyces; ureteral tortuosity; loss of papillary impressions

Acute Renal Failure and Cirhosis

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CIRHOSIS
Involves complex Pathophysiologic changes in body.
Ascites-most common complication
Hydraulic and Oncotic pressures determine net capillary pressure and Ascites formation
Portal Pressure >12 mmHg required for Ascites formation
Redution in Systemic Vascular Resistance
Lower mean arterial pressure
Increased cardiac output
Hyperdynamic Circulation
Reduced SVR is more prominent in the Splanchnic circulation- Ascites formation

Mechanisms of Vasodilation in CIRHOSIS
Increased circulation of vasodilators
Vasoactive  intestinal peptide
Substance P
Platelet Activating factor
Glucagon
Praotagladins
Nitric Oxide – most important

Increased NO synthesis is possibly because of,
 Increased endotoxin absorption from GI tract.
 Decreased clearance by liver because of Portasystemic shunts.
 Decreased Reticuloenthelial Cell Function
NO concetration higher in Portal vein than peripheral veins

Consequences of Vasodilation
Increased endogenous Vasoconstrictors.
Sodium retention
Water retention
Renal vasoconstrition

Hepatorenal Syndrome- is a diagnosis of exclusion
Classically charaterised by Oliguria, Benign Urine sediments,Low Urine Sodium, Rise in creatinine

HIP FRACTURES IN THE ELDERLY

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A hip fracture is a femoral fracture that occurs in the proximal end of the femur,near the hip joint.
Hip fracture following a fall is likely to be a pathological fracture. The most common cause of weakness in bone are:
    * Osteoporosis
    * Homocysteine
    * Other metabolic bone diseases such as Paget's disease, osteomalacia, osteopetrosis and osteogenesis imperfecta. Stress fractures may occur in the hip region with metabolic bone disease.
    * Benign or malignant primary bone tumours are rare causes of hip fractures
    * Metastatic cancer deposits in the proximal femur may weaken the bone and cause a pathological hip fracture
* Femoral head fracture denotes a fracture involving the femoral head. This is usually the result of high energy trauma and a dislocation of the hip joint often accompanies this fracture
* Femoral neck fracture (subcapital, or intracapsular fracture)
* Subtrochanteric fracture actually involves the shaft of the femur immediately below the lesser trochanter and may extend down the shaft of the femur.
The classic clinical presentation of a hip fracture is an elderly patient who sustained a low-energy fall and now has pain and is unable to bear weight. On examination, the affected extremity is often shortened and unnaturally, externally rotated compared to the unaffected leg.
X-rays of the affected hip usually make the diagnosis obvious; AP and lateral views should be obtained.
Most hip fractures are treated by orthopedic surgery, which involves implanting an orthosis.For low-grade fractures (Garden types 1 and 2), standard treatment is fixation of the fracture in situ with screws or a sliding screw/plate device.In elderly patients with displaced fractures many surgeons prefer to undertake a Hemiarthroplasty.An intertrochanteric fracture, below the neck of the femur, has a good chance of healing. Treatment involves stabilizing the fracture with a lag screw and plate device to hold the two fragments in position.An intertrochanteric fracture, below the neck of the femur, has a good chance of healing. Treatment involves stabilizing the fracture with a lag screw and plate device to hold the two fragments in position.


Rett Syndrome

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Rett syndrome is a neurodevelopmental disorder of the grey matter of the brain that affects females more commonly than males.Genetically Rett syndrome is caused by mutations in the gene MECP2 located on the X chromosome and can arise sporadically or from germline mutations.Rett syndrome is usually caused (95% or more) by a de novo  mutation in the child, i.e., not inherited from either parent.Brain levels of norepinephrine  are lower in people with Rett syndromeDevelopment is typically normal until 6–18 months, when language and motor milestones regress, purposeful hand use is lost, and acquired deceleration in the rate of head growth (resulting in microcephaly in some) is seen. Hand stereotypes are typical, and breathing irregularities such as hyperventilation, breathholding, or sighing are seen in many. Early on, autistic-like behavior may be seen. The infant with Rett syndrome often avoids detection until 6–18 months, owing to a relatively normal appearance and some developmental progress. However, closer scrutiny reveals disturbance of the normal spontaneous limb and body movements that are thought to be regulated in the brainstem. The brief period of developmental progress is followed by stagnation and regression of previously acquired skills. 
Currently there is no cure for Rett syndrome, but studies have shown that restoring MECP2 function may lead to a cure.


Physiological Jaundice

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What is Jaundice?
A yellow discolouration of the skin, sclera and mucous membrane due to an increase in the serum bilirubin level. This becomes clinically evident when serum bilirubin reaches about 80-100 mol/l.
Physiological jaundice usually
  Peaks 48-72 hours
  Disappears by 1 week
  Does not present before 24 hours
Aetiology of Physiological Jaundice
 Hb in neonate =18-19g/dl and in adult =11-14g/dl
 Breakdown of  excess RBCs (Haemoglobin is a constituent of RBC)
 Hb broken into:
 Globin - a protein that is conserved and utilised
 Haem - cannot be used
     degraded and excreted  

Bilirubin is a product of this degradation.It causes yellow staining of the tissues.The bilirubin first formed is UNCONJUGATED and FAT SOLUBLE.
It cannot be excreted in bile or urine
Unconjugated Bilirubin
 - travels in plasma, bound to albumin.
 - enters the liver cells with the aid of Y &  Z  carrier proteins
 - becomes conjugated with glucoronic acid
The reaction is catalysed by an enzyme Glucuronyl Transferase

Conjugated Bilirubin is  water soluble
 It is excreted through the biliary tree into the gut.
Conjugated  bilirubin is further catabolised by intestinal flora into:
urobilinogen
stercobilin

It forms a major component of bile in faeces. (This gives the characteristic orange colour to faeces.)
A small amount is passed in the urine
Conjugated Bilirubin is unstable
Why?
Due to : 
The relatively alkaline environment of the duodenum and jejunum
Specific enzymes eg beta glucuronidase Converts back into unconjugated bilirubin.

Ascending cholangitis

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Ascending cholangitis or acute cholangitis is an infection of the bile duct,usually caused by bacteria  ascending from its junction with the duodenum . It tends to occur if the bile duct is already partially obstructed by gallstones.Cholangitis can be life-threatening, and is regarded as a medical emergency.A person with cholangitis may complain of abdominal pain (particularly in the right upper quadrant  of the abdomen), fever, rigors and malaise. Some may report jaundice.
Physical examination findings typically include jaundice and right upper quadrant tenderness.Charcot's triad is a set of three common findings in cholangitis: abdominal pain, jaundice, and fever.This was assumed in the past to be present in 50–70% of cases, although more recently the frequency has been reported as 15–20%.
Routine blood tests show features of acute inflammation (raised white blood cell  count and elevated C-reactive protein level), and usually abnormal liver function tests (LFTs). In most cases the LFTs will be consistent with obstruction: raised bilirubin, alkaline phosphatase and γ-glutamyl transpeptidase. In the early stages, however, pressure on the liver cells may be the main feature and the tests will resemble those in hepatitis, with elevations in alanine transaminase and aspartate transaminase.
Bile duct obstruction, which is usually present in acute cholangitis, is generally due to gallstones. 10–30% of cases, however, are due to other causes such as benign stricturing , postoperative damage or an altered structure of the bile ducts such as narrowing at the site of an anastomosis and various tumors (cancer of the bile duct, gallbladder cancer, cancer of the ampulla of Vater, pancreatic cancer or cancer of the duodenum).Cholangitis may also complicate medical procedures involving the bile duct, especially ERCP.

Tuberous Sclerosis

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Tuberous sclerosis is a rare, multi-system genetic disease that causes non-malignant tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin.The physical manifestations of tuberous sclerosis are due to the formation of hamartia  (malformed tissue such as the cortical tubers), hamartomas  (benign growths such as facial angiofibroma and subependymal nodules) and, very rarely, cancerous hamartoblastomas. The effect of these on the brain leads to neurological symptoms such as seizures, developmental delay and behavioral problems.Between 60 and 80% of tuberous sclerosis patients have benign tumors (once thought hamartomatous, but now considered true neoplasms) of the kidneys called angiomyolipomas (AML) frequently causing hematuria. Rhabdomyomas  are benign tumors of striated muscle. A cardiac rhabdomyoma can be discovered using echocardiography  in approximately 50% of people with tuberous sclerosis.
The most common skin abnormalities include:
    * Facial angiofibromas ("adenoma sebaceum"): A rash of reddish spots or bumps, which appear on the nose and cheeks in a butterfly distribution.
    * Periungual fibromas: Also known as Koenen's tumors, these are small fleshy tumors that grow around and under the toenails or fingernails.
    * Hypomelanic macules ("ash leaf spots"): White or lighter patches of skin that may appear anywhere on the body and are caused by a lack of melanin. These are usually the only visible sign of tuberous sclerosis at birth.
    * Shagreen patches: Areas of thick leathery skin that are dimpled like an orange peel, usually found on the lower back or nape of the neck.

Neonatal Sepsis

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Neonatal sepsis specifically refers to the presence of a serious bacterial infection(such as meningitis, pneumonia, pyelonephritis, or gastroenteritis) in the setting of fever. Criteria with regards to hemodynamic compromise or respiratory failure are not useful clinically because these symptoms often do not arise in neonates until death is imminent and unpreventable.It is difficult to clinically exclude sepsis in newborns less than 90 days old that have fever.Except in the case of obvious acute viral bronchiolitis, the current practice in newborns less than 30 days old is to perform a complete workup including complete blood count with differential, blood culture, urinalysis, urine culture, and cerebrospinal fluid(CSF) studies and CSF culture, admit the newborn to the hospital, and treat empirically for serious bacterial infection for at least 48 hours until cultures are demonstrated to show no growth.Attempts have been made to see whether it is possible to risk stratify newborns in order to decide if a newborn can be safely monitored at home without treatment despite having a fever. One such attempt is the Rochester criteria.

Neonatal sepsis screening:
1. DLC showing increased numbers of polymorphs.
2. DLC: band cells > 20%.
3. increased haptoglobins.
4. micro ESR (Erythrocyte Sedimentation Rate) titer > 55mm.
5. gastric aspirate showing > 5 polymorphs per high power field.
6. newborn CSF (CerebroSpinal Fluid) screen: showing increased cells and proteins.
7. suggestive history of chorioamnionitis, PROM (Premature Rupture Of Membranes)

Diagnosis and Treatment of Hyponatremia

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Major Causes of Hyponatremia
EIVF Depletion
True Volume Depletion
CHF or Cirrhosis
SIADH(Syndrome of Inappropriate ADH Release)
Hormone mediated
Adrenal Insufficiency
Hypothyroidism
Pregnancy
Disorders in which ADH levels may be appropriately suppressed
Advanced renal failure
Primary polydipsia
Beer drinker’s potomania
Pseudohyponatremia
High plasma osmolality:  hyperglycemia, mannitol, urea
Normal plasma osmolality:  hyperlipidemia, hyperproteinemia, glycine infusion.

Causes of Hypoosmolality
Volume Depletion
GI, lung or skin losses
Third space sequestration 
Adrenal insufficiency
Renal salt wasting
Cerebral salt wasting
Volume Expansion
CHF, cirrhosis with ascites, nephrotic syndrome
Euvolemic
SIADH, water intoxication, reset osmostat, drugs

Syndrome of Inappropriate ADH Release (Bartter’s Criteria)
Hyponatremia and true hypoosmolality by definition
Euvolemia clinical
Urine less than maximally dilute (urinary osmolality usually > 200 mOsm/kg of H2O)
Normal renal, cardiac, hepatic, adrenal, pituitary, and thyroid function
No history of antidiuretic drugs
No emotional or physical stress
Urinary sodium >20 mEq/litera

Head Trauma and Intra Cranial Pressure

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Head injuries include both injuries to the brain and those to other parts of the head, such as the scalp and skull.Head injuries may be closed or open.A penetrating head injury occurs when an object pierces the skull and breaches the dura mater. Brain injuries may be diffuse, occurring over a wide area, or focal, located in a small, specific area. Brain injury can be at the site of impact, but can also be at the opposite side of the skull due to a contrecoup effect (the impact to the head can cause the brain to move within the skull, causing the brain to impact the interior of the skull opposite the head-impact).
Diffuse axonal injury usually occurs as the result of an acceleration or deceleration motion, not necessarily an impact. Axons are stretched and damaged when parts of the brain of differing density slide over one another.
Intra-axial hemorrhage is cerebral hemorrhage.This category includes intraparenchymal hemorrhage and intraventricular hemorrhage.Intra-axial hemorrhages are more dangerous and harder to treat than extra-axial bleeds.
Extra-axial hemorrhage, bleeding that occurs within the skull but outside of the brain tissue, falls into three subtypes:Epidural hemorrhage,Subdural hemorrhage and Subarachnoid hemorrhage.
A non-contrast CT of the head should be performed immediately in all those who have suffered a moderate or severe head injury.Most head injuries are of a benign nature and require no treatment beyond analgesics and close monitoring for potential complications such as intracranial bleeding. If the brain has been severely damaged by trauma, neurosurgical evaluation may be useful. Treatments may involve controlling elevated intracranial pressure.


Cystic fibrosis

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Cystic fibrosis is a common recessive genetic disease which affects the entire body, causing progressive disability and often early death.CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR). This gene is required to regulate the components of sweat, digestive juices, and mucus. Although most people without CF have two working copies of the CFTR gene, only one is needed to prevent cystic fibrosis. CF develops when neither gene works normally. Therefore, CF is considered an autosomal recessive disease.Approximately 30,000 Americans have CF, making it one of the most common life-shortening inherited diseases in the United States.
The hallmark symptoms of cystic fibrosis are salty tasting skin,poor growth and poor weight gain despite a normal food intake,accumulation of thick, sticky mucus,frequent chest infections and coughing or shortness of breath.Males can be infertile due to congenital absence of the vas deferens.Symptoms often appear in infancy and childhood, such as bowel obstruction due to meconium ileus in newborn babies.
Cystic fibrosis may be diagnosed by many different categories of testing including those such as, newborn screening, sweat testing, or genetic testing.The newborn screen initially measures for raised blood concentration of immunoreactive trypsinogen.Infants with an abnormal newborn screen need a sweat test in order to confirm the CF diagnosis. In many cases, a parent makes the diagnosis because the infant tastes salty.Trypsinogen levels can be increased in individuals who have a single mutated copy of the CFTR gene

Pancreatic Pseudocyst

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A fluid collection contained within a well-defined capsule of fibrous or granulation tissue or a combination of both
Does not possess an epithelial lining
Persists more than 4 weeks
May develop in the setting of acute or chronic pancreatitis
Most common cystic lesions of the pancreas, accounting for 75-80% of such masses
Location
Lesser peritoneal sac in proximity to the pancreas
Large pseudocysts can extend into the paracolic gutters, pelvis, mediastinum, neck or scrotum
May be loculated
Thick fibrous capsule – not a true epithelial lining
Pseudocyst fluid
Similar electrolyte concentrations to plasma
High concentration of amylase, lipase, and enterokinases such as trypsin

Pathophysiology
Pancreatic ductal disruption secondary to
Acute pancreatitis – Necrosis
Chronic pancreatitis – Elevated pancreatic duct pressures from strictures or ductal calculi
Trauma
Ductal obstruction and pancreatic neoplasms.

Movement Disorders

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Wilson’s disease
AR trait caused by mutation in copper-transporting P-type ATPase, causing abnormal copper accumulation in the liver, BG and other organs

Liver disease (40%):  
Subclinical liver disease
95% under age 20                      
Acute hepatitis  
Fulminant hepatitis                                      
Cirrhosis              

              
Neurological Manifestations
Invariably have liver dysfunction
Isolated tremor (50%)
Dysarthria
Drooling
Dysphagia
Gait abnormality, parkinsonian syndrome, generalized dystonic syndrome

Idiopathic Torsion Dystonia (Oppenheim’s Dystonia)
8.2 years (6-9 years)
Normal development, walk normally
Symptoms start in legs in early onset and in arms and cranial structures in later onset
Becomes generalized within one year of onset
Intact mental function & no psychiatric illness
Positive Family History suggesting autosomal dominant inheritance
Course is progressive in early stages followed by Spontaneous stabilization
AD with reduced penetrance
DYT1 gene is responsible for early-onset idiopathic torsion dystonia in Ashkenazi Jewish families, and in one large non-Jewish family
Gene is mapped to chromosome 9q32-34
Glycosaminoglycan (GAG) deletion
Alteration of norepinephrine concentration in several areas of the brainstem

Drug-Induced Movement Disorders
Parkinsonism
Acute dystonia
Tardive dyskinesia
Neuroleptic Malignant Syndrome
Other drug-induced movement disorders

Urinary tract infection (UTI) is common in infants and children
UTI is difficult to recognise
Collecting urine and interpreting laboratory results is not easy
Diagnosis is not always confirmed
UTI in infants and children may have long-term sequelae

Clinical features of UTI
Evaluation of any sick child must include examination of urine
Every young child with unexplained fever should have urine examined
Clinical features often non-specific
Boys seldom get recurrent UTI in absence of urinary tract abnormalities

UTI Diagnosis – Collect urine
Specimen collection
Suprapubic Aspiration
Catheter specimen collection
Clean catch collection
Bag specimen collection
Pad specimen collection

Urine culture
In all children <3 years if clinical suspicion
Definition
  105 CFU/ml of single organism
 On SPA alone any growth of single organism
PS < 105/ml of one organism or a mixed growth = contamination
Urine obtained before start of antibiotics

UTI Treatment
  Neonate
At least 5 days of parenteral therapy
Antibiotic prophylaxis: Trimethoprim
  Infant and older child
“Upper tract “ - 7 days oral
“Lower tract” –3 days oral
Antibiotic prophylaxis for <3 year olds until imaging: Trimethoprim, Nitrofurantoin (not in <3 mo)

Fever and Rash in a 5month old infant

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DIFFERENTIAL DIAGNOSIS
Rubeola (measles virus)
Rubella (rubella virus)
Erythema infectiosum (5th disease; human parvovirus B19)
Roseola (human herpesvirus 6)
Lyme disease (borrelia burgdorferi)
Erythema multiforme (idiopathic)
Secondary syphillis (treponema pallidum)
Meningococcemia (neisseria meningitidis)
Rocky Mountain Spotted Fever (rickettsia rickettsii)
Scarlet Fever (beta-hemolytic streptococcus)
Toxic shock syndrome (staphylococcus aureus)
Kawasaki disease
Varicella (varicella-zoster virus)
Herpes zoster (varicella-zoster virus)
Rickettsialpox (rickettsia akari)
Erythema nodosum (various causes)
Enteroviral infection

Anencephaly

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Anencephaly is a cephalic disorder  that results from a neural tube defect that occurs when the cephalic end of the neural tube fails to close, usually between the 23rd and 26th day of pregnancy, resulting in the absence of a major portion of the brain, skull, and scalp.Children  with this disorder are born without a forebrain, the largest part of the brain consisting mainly of the cerebral hemispheres.The remaining brain tissue is often exposed—not covered by bone or skin.Most babies with this genetic disorder do not survive birth.
Anencephaly can often be diagnosed before birth through an ultrasound examination. The maternal serum alpha-fetoprotein(increased levels suggest a neural tube defect)   and detailed fetal ultrasound can be useful for screening for neural tube defects such as spina bifida or anencephaly.
There is no cure or standard treatment for anencephaly and the prognosis for patients is death.
It is important for women who may become pregnant to get enough folic acid.There is good evidence that folic acid can help reduce the risk of certain birth defects, including anencephaly. Women who are pregnant or planning to become pregnant should take a multivitamin with folic acid every day.


Cataracts in Paediatric patients

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A cataract is a clouding that develops in the crystalline lens of the eye or in its envelope, varying in degree from slight to complete opacity and obstructing the passage of light. Early in the development of age-related cataract the power of the lens may be increased, causing myopia, and Cataracts typically progress slowly to cause vision loss and are potentially blinding if untreated.
As a cataract becomes more opaque, clear vision is compromised. A loss of visual acuity  is noted. Contrast sensitivity is also lost, so that contours, shadows and color vision are less vivid. Veiling glare can be a problem as light is scattered by the cataract into the eye. The affected eye will have an absent red reflex. A contrast sensitivity test should be performed and if a loss in contrast sensitivity is demonstrated an eye specialist consultation is recommended.
Cataracts develop for a variety of reasons, including long-term exposure to ultraviolet light, exposure to radiation, secondary effects of diseases such as diabetes, hypertension and advanced age, or trauma (possibly much earlier); they are usually a result of denaturation of lens protein. Genetic  factors are often a cause of congenital cataracts and positive family history may also play a role in predisposing someone to cataracts at an earlier age.Some drugs can induce cataract development, such as corticosteroids.
It can be associated with systemic conditions
    * Chromosomal disorders
        * 1q21.1 deletion syndrome
        * Alport's syndrome
        * Cri-du-chat syndrome
        * Myotonic dystrophy
        * Patau's syndrome
        * Trisomy 18 (Edward's syndrome)
        * Turner's syndrome
    * Disease of the skin and mucous membranes
        * Atopic dermatitis
    * Metabolic and nutrition diseases
        * Aminoaciduria (Lowe's syndrome)
        * Diabetes mellitus
        * Fabry's disease
        * Galactosemia / galactosemic cataract
    * Infectious diseases
            * Congenital herpes simplex
            * Congenital syphilis
            * Cytomegalic inclusion disease
            * Rubella
When a cataract is sufficiently developed to be removed by surgery, the most effective and common treatment is to make an incision (capsulotomy) into the capsule of the cloudy lens in order to surgically remove the lens. There are two types of eye surgery that can be used to remove cataracts: extra-capsular (extracapsular cataract extraction, or ECCE) and intra-capsular (intracapsular cataract extraction, or ICCE).

Amniocentesis is a medical procedure used in prenatal diagnosis of chromosomal abnormalities and fetal infections,in which a small amount of amniotic fluid, which contains fetal tissues, is extracted from the amnion or amniotic sac surrounding a developing fetus, and the fetal DNA is examined for genetic abnormalities.
With the aid of ultrasound-guidance, a physician punctures the sac in an area away from the fetus and extracts approximately 20 ml of amniotic fluid. After the amniotic fluid is extracted, the fetal cells are separated from the sample. The cells are grown in a culture medium, then fixed and stained. Under a microscope the chromosomes are examined for abnormalities. The most common abnormalities detected are Down syndrome (trisomy 21), Edwards syndrome (trisomy 18), and Turner syndrome (monosomy X). In regard to the fetus, the puncture heals and the amniotic sac replenishes the liquid over the next 24–48 hours.Serious complications can result in miscarriage. Other possible complications include preterm labor and delivery, respiratory distress, postural deformities, fetal trauma and alloimmunisation of the mother.
Chorionic villus sampling(CVS) is a form of prenatal diagnosis to determine chromosomal or genetic disorders in the fetus. It entails getting a sample of the chorionic villus and testing it. CVS usually takes place 10–12 weeks after the last period, earlier than amniocentesis.Risk of miscarriage in CVS in about 0.5 - 1%. Apart from a risk of miscarriage, there is a risk of infection and amniotic fluid leakage. The resulting amniotic fluid leak can develop into a condition known as oligohydramnios  which is low amniotic fluid level. If the resulting oligohydramnios is not treated and the amniotic fluid continues to leak it can result in the baby developing hypoplastic lungs.

Hypospadias

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Hypospadias is a birth defect of the urethra in the male that involves an abnormally placed urinary meatus.Instead of opening at the tip of the glans  of the penis, a hypospadic urethra opens anywhere along a line running from the tip along the ventral aspect of the shaft to the junction of the penis and scrotum or perineum.
Mild hypospadias most often occurs as an isolated birth defect without detectable abnormality of the remainder of the reproductive or endocrine system. However, a minority of infants, especially those with more severe degrees of hypospadias will have additional structural anomalies of the genitourinary tract. Up to 10% of boys with hypospadias have at least one undescended testis, and a similar number have an inguinal hernia. An enlarged prostatic utricle  is common when the hypospadias is severe (scrotal or perineal), and can predispose to urinary tract infections, pseudo-incontinence, or even stone  formation.
The urethral meatus opens on the underside of the glans penis in about 50–75% of cases; these are categorized as first degree hypospadias. Second degree (when the urethra opens on the shaft), and third degree (when the urethra opens on the perineum) occur in up to 20 and 30% of cases respectively.
First degree hypospadias are primarily a cosmetic defect and have little effect on function except for direction of the urinary stream. If uncorrected, a second or third degree hypospadias can make male urination messy, necessitate that it be performed sitting, impair delivery of semen into the vagina (possibly creating problems with fertility), or interfere with erections. In developed countries, most hypospadias are surgically repaired in infancy. Surgical repair of first and second degree hypospadias is nearly always successful in one procedure, usually performed in the first year of life by a pediatric urologist or a plastic surgeon.

Neonatal Hypertension

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Hypertension in newborn infants primarily is of renal origin, although cardiac, endocrine, and pulmonary causes have been described as well.
Renovascular problems that may lead to neonatal hypertension include renal venous thrombosis  and renal artery stenosis secondary to fibromuscular dysplasia. Many infants with FMD may have main renal arteries that appear fairly normal on angiography but demonstrate significant branch vessel disease that can cause severe hypertension.
Numerous congenital renal parenchymal abnormalities can lead to hypertension in the newborn period. For example, patients with autosomal dominant or autosomal recessive polycystic kidney disease (PKD) may present in the newborn period with severe nephromegaly and hypertension.Additional renal parenchymal causes of hypertension in the newborn period include severe acute tubular necrosis, interstitial nephritis, and cortical necrosis.
Therefore, the pathophysiology depends on the organ system involved. For example, hypertension related to renal emboli primarily is a high renin form of hypertension, whereas the hypertension associated with bronchopulmonary dysplasia (BPD) is likely related to hypoxia. Such differences in pathophysiology are very important because they can guide the clinician with respect to evaluation and treatment.
Blood pressure in newborn infants is influenced by various factors, including birthweight, gestational age, and postconceptual age.
In most newborns, hypertension is discovered on routine monitoring of vital signs. Other presentations of neonatal hypertension to be aware of in acutely ill infants include congestive heart failure (CHF) and cardiogenic shock, which are potentially life threatening. Fortunately, these consequences of hypertension gradually resolve with appropriate BP reduction. In the less acutely ill infant, feeding difficulties, unexplained tachypnea, apnea, lethargy, irritability, or seizures may constitute symptoms of unsuspected hypertension. In older infants who have been discharged from the nursery, unexplained irritability or failure to thrive may be the only manifestations of hypertension.

Meningitis
Inflammation of the meninges
Classic triad:Fever,Headache (Severe, frontal), photophobia, n/v Jolt accentuation
Meningismus/altered mental status

Meningeal signs
Kernig sign: one leg with hip flexed, pain in back with extension of knee
Brudzinski sign: flexion of legs and thighs when neck is flexed

Encephalitis
Inflammation of the cerebral cortex
Fever, HA, altered mental status
Key: early mental status changes
More commonly viruses
Obtundation/coma
Behavioral or speech problems, neurological signs, seizures
Meningoencephalitis
Difference from meningitis: less likely fever, more likely personality/behavioral changes

Meningitis in the Elderly
Decreased total  incidence; increased in elderly
Increased prevalence of Listeria (25%)
30-50%:  S. pneumoniae
Less likely Neisseria and Haemophilus
Less likely fever and meningeal signs; more likely neurological symptoms, seizure, coma
More often complicated by pneumonia
Older patients with neurological impairment:  50% mortality

The Lumbar Puncture: Risks
Headache: 10-25%
Typical: appears suddenly upon standing
Decrease CSF pressure with small leak
Decrease risk: small (<20 g) needle, leave patient prone after procedure
Infection (small)
Local bleeding: traumatic tap to epidural hematoma
Brain herniation.....

Seizure Disorders in Pregnancy

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Approximately 1 million women of childbearing age in the United States have seizure disorders. Women with epilepsy appear to have a greater baseline risk of fetal malformations, which is further increased with the use of antiepileptic drugs (AEDs). During pregnancy, the volume of distribution and hepatic metabolism of AEDs are increased. This, along with decreased compliance with AEDs because of concerns about effects on the fetus, leads to an increase in seizure frequency.Generalized tonic-clonic seizures during pregnancy can lead to increased maternal trauma. If the maternal trauma involves the abdomen, a theoretical risk of abruption exists, possibly leading to fetal hypoxia or death. Furthermore, the risk of maternal aspiration can lead to maternal hypoxia, which can also lead to fetal hypoxia.
Specific increases in congenital abnormalities observed in infants born to mothers with epilepsy include a 4-fold increase in cleft lip and palate and a 3- to 4-fold increase in cardiac anomalies. An increase in the rate of neural tube defects is also observed in the offspring of patients with epilepsy who are using carbamazepine or valproic acid. Long-term studies on neurodevelopment show higher rates of abnormal EEG findings, higher rates of developmentally delayed children, and lower intelligence quotient (IQ) scores.Fetal hydantoin syndrome consists of an array of anomalies, including craniofacial anomalies, distal digital hypoplasia, epicanthal folds, hypertelorism, low-set ears, and developmental delay.

Prune belly syndrome

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Prune belly syndrome is a rare, genetic, birth defect affecting about 1 in 40,000 births.About 97% of those affected are male. Prune belly syndrome is a congenital disorder of the urinary system, characterized by a triad of symptoms(A partial or complete lack of abdominal muscles,Undescended testicles in males,Urinary tract abnormality such as unusually large ureters, distended bladder). The syndrome is named for the mass of wrinkled skin that is often present on the abdomens of those with the disorder.

Prune belly syndrome can be diagnosed via ultrasound while a child is still in-utero.An abnormally large abdominal mass is the key indicator, as the abdomen swells with the pressure of accumulated urine. In young children, frequent urinary tract infections often herald prune belly syndrome, as they are normally uncommon. If a problem is suspected, doctors can perform blood tests to check kidney function. Another test that may reveal the syndrome is the voiding cystourethrogram.


Because of the risk of inducing hyperchloraemic acidosis in routine practice, when crystalloid resuscitation or replacement is indicated, balanced salt solutions e.g. Ringer’s lactate/acetate or Hartmann’s solution should replace 0.9% saline, except in cases of hypochloraemia e.g. from vomiting or gastric drainage.
Solutions such as 4%/0.18% dextrose/saline and 5% dextrose are important sources of free water for maintenance, but should be used with caution as excessive amounts may cause dangerous hyponatraemia, especially in children and the elderly. These solutions are not appropriate for resuscitation or replacement therapy except in conditions of significant free water deficit e.g. diabetes insipidus.
To meet maintenance requirements, adult patients should receive sodium 50-100 mmol/day, potassium 40-80 mmol/day in 1.5-2.5 litres of water by the oral, enteral or parenteral route (or a combination of routes). Additional amounts should only be given to correct deficit or continuing losses. Careful monitoring should be undertaken using clinical examination, fluid balance charts, and regular weighing when possible.
In patients without disorders of gastric emptying undergoing elective surgery clear non-particulate oral fluids should not be withheld for more than two hours prior to the induction of anaesthesia...


Obstructive Renal Disorders

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Obstructive uropathy is structural or functional hindrance of normal urine flow, sometimes leading to renal dysfunction (obstructive nephropathy). Symptoms, less likely in chronic obstruction, are pain radiating to the T11 to T12 dermatomes, anuria, nocturia, or polyuria.
Many conditions can cause obstructive uropathy, which may be acute or chronic, partial or complete, and unilateral or bilateral.In children, the most common causes are anatomic abnormalities (including urethral valves or stricture and stenosis at the ureterovesical or ureteropelvic junction).In young adults, the most common cause is a calculus. In older adults, the most common causes are BPH or prostate cancer, retroperitoneal or pelvic tumors, and calculi. Obstruction may occur at any level, from the renal tubules to the external urethral meatus. Proximal to the obstruction, effects may include increased intraluminal pressure, urinary stasis, UTI, or calculus formation.Obstruction is much more common in males, but acquired and congenital urethral strictures and meatal stenosis occur in both males and females. In females, urethral obstruction may occur secondary to a tumor or as a result of stricture formation after radiation therapy, surgery, or urologic instrumentation.
Diagnosis is based on results of bladder catheterization, ultrasonography, CT, cystourethroscopy, cystourethrography, or pyelography, depending on the level of obstruction.

Osteoporosis is a disease of bones that leads to an increased risk of fracture.In osteoporosis the bone mineral density (BMD) is reduced, bone microarchitecture is deteriorating, and the amount and variety of proteins in bone is altered.
Conventional radiography is useful, both by itself and in conjunction with CT or MRI, for detecting complications of osteopenia (reduced bone mass; pre-osteoporosis), such as fractures; for differential diagnosis of osteopenia; or for follow-up examinations in specific clinical settings, such as soft tissue calcifications, secondary hyperparathyroidism, or osteomalacia in renal osteodystrophy. However, radiography is relatively insensitive to detection of early disease and requires a substantial amount of bone loss (about 30%) to be apparent on x-ray images.The main radiographic features of generalized osteoporosis are cortical thinning and increased radiolucency.
Osteomalacia is the softening of the bones due to defective bone mineralization secondary to inadequate amounts of available phosphorus and calcium.Osteomalacia in children is known as rickets, and because of this, use of the term osteomalacia is often restricted to the milder, adult form of the disease.The most common cause of the disease is a deficiency in vitamin D, which is normally obtained from the diet and/or sunlight exposure.Loosers zones or Milkmans pseudofractures are strongly suggestive but not diagnostic of osteomalacia.
Osteosclerosis is an abnormal hardening or increased density of bone on radiographs.Increased radiodensity of bone that may occur with various conditions including renal osteodystrophy, osteopetrosis and pyknodysostosis.
Renal osteodystrophy is a general term used to describe the bony changes associated with chronic renal disease.
Osteopetrosis is a thymic defect which results in generalised osteosclerosis with dense, thick, brittle bones.It is most marked with generalized osteosclerosis, cortical thickening, loss of normal cortico-cancellous differentiation and obliteration of normal trabecular pattern.
Pyknodysostosis is a dysplasia manifest clinically by dwarfism, increased bone fragility and sclerotic bones.It is a syndrome chararacterized by osteosclerosis, short stature, short broad hands

Hirschsprung's disease

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Hirschsprung's disease, or congenital aganglionic megacolon, involves an aganglionic section of bowel that starts at the anus and progresses proximally. The length of bowel that is affected varies but seldom stretches for more than about 30 cm. It arises when ganglion cells in the gut fail to develop and mature correctly. The result is a section of bowel that is essentially paralyzed.According to this new research, Hirschsprung's is caused by the interaction between two proteins encoded by two variant genes. The RET proto-oncogene on chromosome 10 was identified as one of the genes involved. The protein with which RET has to interact in order for Hirschsprung’s disease to develop is termed EDNRB and is encoded by the gene EDNRB located on chromosome 13.
With an incidence of 1/5000 births, the most cited feature is absence of ganglion cells: notably in males, 75% have none in the recto-sigmoid, and 8% with none in the entire colon. The enlarged section of the bowel is found proximally, while the narrowed, aganglionic section is found distally; the absence of ganglion cells results in a persistent over-stimulation of nerves within the affected region, resulting in contraction.
1) Delayed passage of meconium.
2) Abdominal distension.
3) Constipation.

Definitive diagnosis is made by suction biopsy of the distally narrowed segment.Diagnostic techniques involve anorectal manometry,barium enema, and rectal biopsy.
Treatment of Hirschsprung's disease consists of surgical removal (resection) of the abnormal section of the colon, followed by reanastomosis. There used to be two steps typically used to achieve this goal.
The first stage used to be a colostomy.
Later, when the child’s weight, age, and condition is right, a pull-through procedure is performed.

Infantile hypertrophic pyloric stenosis

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Pyloric stenosis(or infantile hypertrophic pyloric stenosis) is a condition that causes severe vomiting in the first few months of life.It is due to hypertrophy of the muscle in the pylorus.It is uncertain whether there is a real congenital narrowing or whether there is a functional hypertrophy of the muscle which develops in the first few weeks of life.
Males are more commonly affected than females. It is also associated with first born males about four times as often, although there exist many more first borns in the poplulation.
Babies with this condition usually present any time in the first weeks to months of life with progressively worsening vomiting. The vomiting is often described as non-bile stained ("non bilious") and "projectile vomiting", because it is more forceful than the usual spittiness (gastro-oesophageal reflux) seen at this age. Some infants present with poor feeding and weight loss, but others demonstrate normal weight gain. Dehydration also can occur causing the baby to cry without having tears, and having less wet or dirty nappies such as going hours or a couple days without having anything.Constant hunger, belching, and colic are other possible signs as the baby is not able to eat properly.
Most cases of pyloric stenosis are diagnosed/confirmed with ultrasound, if available, showing the thickened pylorus.
The definitive treatment of pyloric stenosis is with surgical pyloromyotomy known as Ramstedt's procedure (dividing the muscle of the pylorus to open up the gastric outlet). This is a relatively straightforward surgery that can possibly be done through a single incision (usually 3–4 cm long) or laparoscopically (through several tiny incisions), depending on the surgeon's experience and preference.

Before the child is born the testicle migrates down from high in the abdomen and passes through abdominal wall and groin to take its normal position in the scrotum. Undescended testicles are quite common. They may be present in 4% of boys at birth, and there is an even higher incidence in premature infants. Three-fourths of undescended testicles will descend within the first three months of life.
When a testicle is not in the normal scrotal location several possibilities exist:
    * There may never have been a testicle (congenital absence).
    * The testicle may have atrophied (withered away) before birth due to torsion (twist) or blockage of the testicular blood vessels.
    * The testicle may have descended incompletely and may lie within the inguinal canal (just above the scrotum).
    * The testicle may have not descended properly, but remains within the abdominal cavity.
    * In some children the testes may be found in the groin, but can be brought down into the scrotum during examination. These 'retractile' testicles also will be seen to descend when the child is in the bathtub. Retractile testicles are due to hyperactive muscles that temporarily pull the testicle into the groin. However, retractile testicles are not believed to injure the testicles and require no treatment.

The recommended treatment of the undescended testicle before one year of age. There is evidence that early damage to the germ cells that produce sperm begins at this age. There are two options for treatment. Injections of a hormone, HCG, several times per week over several weeks can produce descent in some children. However, the success rates have been reported to be as low as10%. Also, the results of hormone treatment are less successful in children less than two years of age.
The most effective treatment is surgery, which can be performed as an outpatient. When a testis is felt in the groin area we usually explore the area through a small incision. Most undescended testes are associated with a hernia that must be repaired. After this is done, the testis is brought down into the scrotum and anchored in a space created in the scrotum (orchiopexy).
When a testis is not palpable on physical exam, its location must be determined.It is done by placing a laparoscope through a small incision below the 'belly button' to look in the abdomen at the time of surgery. In those patients found to have testes very high in the abdomen, additional surgery is required to correct the problem. A number of children will be found to have very small abnormal gonads which we will remove (orchiectomy). Most of these children probably had torsion or twisting of the testis on its blood supply prior to birth that led to the small testis. When a boy is left with a single functioning testis it is recommended anchoring it to minimize chances of losing it to torsion later in life.

Embryology of Sexual differentiation

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Sexual differentiation is the process of development of the differences between males and females from an undifferentiated zygote.
Humans have forty-six chromosomes, including two sex chromosomes, XX in females and XY in males. It is obvious that the Y chromosome must carry at least one essential gene which determines testicular formation. A gene in the sex-determining region of the short arm of the Y, now referred to as SRY, has been found to direct production of a protein which binds to DNA, inducing differentiation of cells derived from the genital ridges into testes.
Early in fetal life, germ cells migrate from structures known as yolk sacs to the genital ridge. By week 6, undifferentiated gonads consist of germ cells, supporting cells, and steroidogenic cells.
In a male, SRY and other genes induce differentiation of supporting cells into Sertoli cells and steroidogenic cells into Leydig cells to form testes, which become microscopically identifiable and begin to produce hormones by week 8. Germ cells become spermatogonia.Without SRY, ovaries form during months 2-6. Failure of ovarian development in 45,X girls (Turner syndrome) implies that two functional copies of several Xp and Xq genes are needed. Germ cells become ovarian follicles. Supporting and steroidogenic cells become theca cells and granulosa cells, respectively.
In a male fetus, testes produce steroid and protein hormones essential for internal and external anatomic differentiation. Leydig cells begin to make testosterone by the end of month 2 of gestation. Antimullerian hormone (AMH) is a protein hormone produced by Sertoli cells from the 8th week on. AMH suppresses development of müllerian ducts in males, preventing development of a uterus.

Fetal ovaries produce estradiol, which supports follicular maturation but plays little part in other aspects of prenatal sexual differentiation, as maternal estrogen floods fetuses of both sexes.
Gonads are histologically distinguishable by 6–8 weeks of gestation. A fetus of that age has both mesonephric (wolffian) and paramesonephric (mullerian) ducts. Subsequent development of one set and degeneration of the other depends on the presence or absence of two testicular hormones: testosterone and AMH.Local testosterone causes each wolffian duct to develop into epididymis, vas deferens, and seminal vesicles. Without male testosterone levels, wolffian ducts degenerate and disappear. Müllerian ducts develop into a uterus, fallopian tubes, and upper vagina unless AMH induces degeneration.


Polycystic kidney disease

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In people with polycystic kidney disease, the kidneys become enlarged with multiple cysts that interfere with normal kidney function. This can sometimes lead to renal failure and the need for dialysis or kidney transplantation.There are two major forms of polycystic kidney disease: autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease.
Autosomal dominant polycystic kidney disease (ADPKD) is a common disorder, occurring in approximately 1 in every 400 to 1000 people. Only about one-half of people with ADPKD will be diagnosed during the person's lifetime because the disease is often without symptoms. Autosomal dominant means that there is a 50 percent risk that an affected parent will pass the mutated gene to a child.Approximately 85 percent of families with autosomal dominant polycystic kidney disease (ADPKD) have an abnormality on chromosome 16; these people have PKD1 disease. The remaining 15 percent have a defect that involves a gene on chromosome 4; this is called PKD2 disease.
Autosomal recessive polycystic kidney disease (ARPKD, also called childhood polycystic kidney disease) is typically diagnosed in infancy, although less severe forms may be diagnosed later in childhood or adolescence.
The most common symptoms are pain in the back and the sides—between the ribs and hips—and headaches. The pain can be temporary or persistent, mild or severe.
People with autosomal dominant PKD also can experience the following complications:
    * urinary tract infections—specifically, in the kidney cysts
    * hematuria—blood in the urine
    * liver and pancreatic cysts
    * abnormal heart valves
    * high blood pressure
    * kidney stones
    * Cerebral aneurysm
  It is usually easy to diagnose autosomal dominant polycystic kidney disease (ADPKD) in people who develop flank or abdominal pain and have a family history of ADPKD. An imaging study, such as an ultrasound, magnetic resonance imaging scan (MRI), or CT scan is usually recommended and may reveal large kidneys with multiple cysts on both kidneys. Cysts may also be seen in the liver, pancreas, and spleen.


Septic arthritis

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Septic arthritis is the purulent invasion of a joint by an infectious agent which produces arthritis.People with artificial joints are more at risk than the general population but have slightly different symptoms, are infected with different organisms and require different treatment.Septic arthritis is considered a medical emergency. If untreated, it may destroy the joint in a period of days.
The usual etiology of septic arthritis is bacterial, but viral, mycobacterial, and fungalarthritis occur occasionally.
Bacteria are carried by the bloodstream from an infectious focus elsewhere, introduced by a skin lesion that penetrates the joint, or by extension from adjacent tissue (e.g. bone or bursae bovine tb).
Micro-organisms must reach the synovial membrane of a joint. This can happen in any of the following ways:
    * dissemination of pathogens via the blood, from abscesses or wound infections, or from an unknown focus
    * dissemination from an acute osteomyelitic focus,
    * dissemination from adjacent soft tissue infection,
    * entry via penetrating trauma
    * entry via iatrogenic means
Septic arthritis should be considered whenever one is assessing a patient with joint pain. Usually only one joint is affected (monoarthritis) however in seeding arthritis, several joints can be affected simultaneously; this is especially the case when the infection is caused by staphylococcus or gonococcus bacteria.
The diagnosis of septic arthritis is based on clinical assessment and prompt arthrocentesis. Imaging can sometimes be used to aid in the diagnosis of septic arthritis.
Native X-ray of the joint is neither sensitive nor specific. Ultrasound can detect joint-swelling. MRI findings include: synovial enhancement, perisynovial edema and joint effusion.
Therapy is usually with intravenous antibiotics, analgesia and washout/aspiration of the joint to dryness. Among pediatric patients with an acute hematogenous septic arthritis a short total course of 10 days of antimicrobials is sufficient in uncomplicated cases.


Neonatal Intestinal Obstruction

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Causes of Neonatal Intestinal Obstruction
Colon atresia
Meconium plug
Meconium ileus
Anorectal malformation
Small left colon syndrome
Megacystic-microcolon-intestinal hypoperstalsis syndrome

Colon atresia
Lest common one of the intestinal atresia , the common site is transverse colon
Ischemia in the vascularity of the mesentery necroses of the bowel segment
Polyhydraminos
Contrast enema is diagnostic obstruction site, and distal atrophy , proximal dilatation
Treatment resection of the dilated part and reanastomosis

Meconium plug
Mildest and the most common
Etiology is not clear
No fluid on x-ray
Contrast :diagnostic
Treatment :  rectal stimulation

Meconium ileus
30% of intestinal obstruction, usually present in the first days in life
50% associated with other intestinal problem (volvulus , atresia, perforation )
15% of patients with cystic fibrosis (AR)
P.R: small caliber of rectum, rectum is empty
X-ray: ground- glass appearance
Contrast enema: empty micro colon, entrapment of   meconium in the ileum
50% present with complication (meconium peritonitis, perforation)
X-ray: intraperitonial calcification, free air or very large air_ fluid levels
Treatment : gastrograffin enema administration
Operative evacuation of the obstruction by irrigation

Small left colon syndrome
Rare cause
History of maternal diabetes with abnormal glucose tolerance test in 50%
infants how are hypoglycemic
septic
an association with hypothyroidism, hypermagnesaemia
Ass/e  maternal use of psychotic medication
Dysmotility in the descending colon  


Polyhydramnios

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Polyhydramnios is a medical condition describing an excess of amniotic fluid in the amniotic sac. It is seen in 0.2 to 1.6% of pregnancies.
A single case of polyhydramnios may have one or more causes. About 14% of cases are due to maternal diabetes mellitus, which causes fetal hyperglycemia and resulting polyuria and also rh-isoimmunisation can cause it. About another 20% of cases are associated with fetal anomalies that impair the ability of the fetus to swallow . These anomalies include:
    * gastrointestinal abnormalities such as esophageal atresia, duodenal atresia, facial cleft, neck masses, and tracheoesophageal fistula
    * fetal renal disorders that results in increased urine production during pregnancy, such as in antenatal Bartter syndrome.
    * chromosomal abnormalities such as Down's syndrome and Edwards syndrome 
    * neurological abnormalities such as anencephaly, which impair the swallowing reflex
In a multiple gestation pregnancy, the cause of polyhydramnios usually is twin-twin transfusion syndrome.
During the pregnancy, certain clinical signs may suggest polyhydramnios. In the mother, the physician may observe increased abdominal size out of proportion for her weight gain and gestation age, uterine size that outpaces gestational age, shiny skin with stria (seen mostly in severe polyhydramnios), dyspnea, and chest heaviness. When examining the fetus, faint fetal heart sounds are also an important clinical sign of this condition.
Fetuses with polyhydramnios are at risk for a number of other problems including cord prolapse, placental abruption, premature birth and perinatal death.


Headache and Facial Pain

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There are over 200 types of headache, and the causes range from harmless to life-threatening. The description of the headache, together with findings on neurological examination, determines the need for any further investigations and the most appropriate treatment.
The most common types of headache are the "primary headache disorders", such as tension-type headache and migraine. They have typical features; migraine, for example, tends to be pulsating in character, affecting one side of the head, associated with nausea, disabling in severity, and usually lasts between 3 hours and 3 days. Rarer primary headache disorders are trigeminal neuralgia and cluster headache.
Headaches may be caused by problems elsewhere in the head or neck. Some of these are not harmful, such as cervicogenic headache (pain arising from the neck muscles). Medication overuse headache may occur in those using excessive painkillers for headaches, parodoxically causing worsening headaches.
A number of characteristics make it more likely that the headache is due to potentially dangerous secondary causes; some of these may be life-threatening or cause long-term damage.
"Thunderclap headache" may be the only symptom of subarachnoid hemorrhage.Headache with fever may be caused by meningitis, particularly if there is meningism , and confusion may be indicative of encephalitis.Headache that is worsened by straining or a change in position may be caused by increased pressure in the skull; this is often worse in the morning and associated with vomiting. Raised intracranial pressure may be due to brain tumors, idiopathic intracranial hypertension (IIH, more common in younger overweight women) and occasionally cerebral venous sinus thrombosis.Headache together with weakness in part of the body may indicate a stroke or brain tumor. Headache in older people, particularly when associated with visual symptoms or jaw claudication, may indicate giant cell arteritis (GCA), in which the blood vessel wall is inflamed and obstructs blood flow.Angle closure glaucoma may lead to headache, particularly around the eye, as well as visual abnormalities, nausea, vomiting and a red eye with a dilated pupil.


Hematemesis:vomitting of blood of altered blood(coffee grounds) indicates bleeding proximal to ligament of Treitz
Melena:Tarry stool. Altered (black) blood per rectum (>60ml)
Hematochezia: Bright red or maroon rectal ,bleeding implies bleeding beyond Lig.treitz.

Differentiating Upper from Low GI Bleeding
Hematochezia usually represents a lower GI source bleeding
Upper GI lesion may bleed so briskly that blood doesn`t remain in bowl long enough to become melena
Bleeding lesion distal to treitz Lig.may be either Melena or hematochezia, but never manifests hematemesis

Common cause of upper GI bleeding
Peptic ulcer
Gastropathy (alcohol, aspirin, NSAIDs, stress)
Oesophageal varices
Gastric cancer

Less common cause of upper GI bleeding
Esophageal or intestinal neoplam
Esophagitis; Malloy-weiss tear,
Hemoptysis: Swallowed blood
Anticoagulant fibrinoloytic therapy:
Telangiectases; aneurysm ;vasculitis;Dieulafoy ulcer; AV malformation
Connective tissue disease;
Hemabilia(biliary origin;Crohn`s disease;amyloidosis , hematological diseases


Chronic Obstructive Pulmonary Disease

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Chronic obstructive pulmonary disease (COPD) is the co-occurrence of chronic bronchitis and emphysema, a pair of commonly co-existing diseases of the lungs in which the airways become narrowed.This leads to a limitation of the flow of air to and from the lungs, causing shortness of breath. In clinical practice, COPD is defined by its characteristically low airflow on lung function tests.In contrast to asthma, this limitation is poorly reversible and usually gets progressively worse over time.
COPD is caused by noxious particles or gas, most commonly from tobacco smoking, which triggers an abnormal inflammatory response in the lung.The inflammatory response in the larger airways is known as chronic bronchitis, which is diagnosed clinically when people regularly cough up sputum. In the alveoli, the inflammatory response causes destruction of the tissues of the lung, a process known as emphysema. The natural course of COPD is characterized by occasional sudden worsenings of symptoms called acute exacerbations, most of which are caused by infections or air pollution.
Lung damage and inflammation in the large airways results in chronic bronchitis. Chronic bronchitis is defined in clinical terms as a cough with sputum production on most days for 3 months of a year, for 2 consecutive years.In the airways of the lung, the hallmark of chronic bronchitis is an hyperplasia and hypertrophy of the goblet cells and mucous glands of the airway. As a result, there is more mucus than usual in the airways, contributing to narrowing of the airways and causing a cough with sputum. As chronic bronchitis progresses, there is squamous metaplasia and fibrosis
Lung damage and inflammation of the alveoli results in emphysema. Emphysema is defined as enlargement of the air spaces distal to the terminal bronchioles, with destruction of their walls.The destruction of air space walls reduces the surface area available for the exchange of oxygen and carbon dioxide during breathing. It also reduces the elasticity of the lung itself, which results in a loss of support for the airways that are embedded in the lung. These airways are more likely to collapse causing further limitation to airflow.
The diagnosis of COPD requires lung function tests. Important management strategies are smoking cessation, vaccinations, rehabilitation, and drug therapy.Some patients go on to require long-term oxygen therapy or lung transplantation.


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