Hand Deformities

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Mallet deformity
Deformity in which the fingertip is curled in and cannot straighten itself
Due to injury to extensor digitorium tendons at DIPJ
Forced flexion of the finger when finger is extended:
. Sport Injury: Finger struck by volleyball, basketball or baseball when it is in extension
. Other common mechanisms of injury include forcefully tucking in a bedspread or slipcover or pushing off a sock with extended fingers.

Trigger Finger
Trigger finger is the popular name of stenosing tenosynovitis, a painful condition in which a finger or thumb locks when it is bent (flexed) or straightened (extended).
Due to narrowing of the sheath that surrounds the tendon in the affected finger, or a nodule forms on the tendon.
Trigger finger is often an overuse injury because of repetitive or frequent movement of the fingers (ex. hobbies as playing a musical instrument or crocheting)
Trigger finger may also result from trauma or accident
It is called trigger finger because when the finger unlocks, it pops back suddenly, as if releasing a trigger on a gun.

Boutonniere Deformity
Hyperflexion at the PIP joint with hyperextension at the DIP
Passive extension of the PIP joint is easy.
Flexion deformity of the PIP joint, due to interruption of the central slip of the extensor tendon:
 The lateral bands separate
 The head of the proximal phalanx pops through the gap like a finger through a button hole
 The DIP joint is drawn into hyperextension.
The 3 main etiologies:
  RA and other inflammatory arthritides (most often)
  Mechanical trauma
  Burns and infections

Swan – Neck deformity
the PIP joint is hyper extended . DIP joint is flexed. 
Cause:
Due to injury or inflammation (RA)
Swelling and pain due to inflammation from injury or disease (RA)
Dupuytren contracture
Pathologic condition of the hand in which the fascia of the palm are shortened and thickened


Jaundice

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Bilirubin Metabolism: Pre-Hepatic
Bilirubin is formed in macrophages of the reticuloendothelial system. The initial substrate is predominantly hemaglobin.
Heme group  biliverdin  bilirubin
Bilirubin is insoluble in water and so must be carried by albumin within plasma.
Bilirubin circulates in the blood before uptake by the liver.
Pre-hepatic jaundice = if bilirubin is not taken up by the liver or if it is produced in excess, unconjugated bilirubin is deposited in extra-hepatic tissues.

Bilirubin Metabolism: Hepatic
Bilirubin is taken up into hepatocytes and bound to intracellular proteins.
Bilirubin + UDP glucuronic acid = bilirubin diglucuronide > bilirubin monoglucuronide > UDP
The glucuronide conjugated form of bilirubin is water soluble and is excreted into bile. Excretion occurs into the bile canaliculus by carrier-mediated transport.
Hepatic jaundice = disorders of bilirubin uptake or conjugation

Bilirubin Metabolism: Post-Hepatic
Glucuronide-conjugated bilirubin in bile may be degraded to urobilinogen or partially reabsorbed into plasma.
Urobilinogen pathway:
may be reabsorbed by the gut and returned to the liver
converted to urobilin
reabsorbed into plasma for excretion by kidneys
Conjugated bilirubin pathway:
May be acted upon by bacterial enzymes within the gut to form the bile pigment stercobilinogen. Stercobilinogen may be reabsorbed into plasma for recycling to the liver or for excretion by the kidney, or, it may be oxidized to stercobilin.
Obstructive jaundice = failure of bilirubin to reach the gut, resulting in a reduction in pigment within the stool

DDX: Unconjugated Hyperbilirubinemia
Increased Bilirubin Production
Extravascular hemolysis
Extravasation of blood into tissues
Intravascular hemolysis
Errors in production of red blood cells
Impaired Hepatic Bilirubin Uptake
CHF
Portosystemic shunts
Drug inhibition: rifampin, probenecid
Impaired Bilirubin Conjugation
Gilbert’s disease
Crigler-Najarr syndrome
Neonatal jaundice (this is physiologic)
Hyperthyroidism
Estrogens
Liver diseases (chronic hepatitis, cirrhosis, Wilson’s)...


Pediatric Failure to Thrive

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Failure to Thrive (FTT):
 Weight below the 5th percentile for age and sex
 Weight for age curve falls across two major percentile lines
 A sign that describes a problem rather than a diagnosis
 Usually describes failure to gain wt
 In more severe cases length and head circumference can be affected
 Underlying cause is insufficient usable nutrition to meet the demands for growth
 Approximately 25% of normal children will have a shift down in their wt curve of up to 25%, then follow a normal curve -- this is not failure to thrive

Etiology
Inadequate Caloric Intake
Incorrect preparation of formula
Poor feeding habits (ex: too much juice)
Poverty
Mechanical feeding difficulties (reflux, cleft palate, oromotor dysfunction)
Neglect
 Physicians are strongly encouraged to consider child abuse and neglect in cases of FTT that don’t respond to appropriate interventions

Inadequate absorption
Celiac disease
Cystic fibrosis
Milk allergy
Vitamin deficiency
Biliary Atresia
Necrotizing enterocolitis

Increased metabolism
Hyperthyroidism
Chronic infection
Congenital heart disease
Chronic lung disease

Other considerations
Genetic abnormalities, congenital infections, metabolic disorders (storage diseases, amino acid disorders)

Lab Evaluation
Unless suggested by History and Examination, no routine lab tests recommended initially*
One study of hospitalized pts resulted in only 1.4% of tests being of diagnostic assistance in FTT
If problem persists, could consider:
CBC, U/A, Electrolytes, TSH, ESR, Lead, HIV, Tb
If not improving with adequate diet, consider:
Stool for fat, reducing substances, pathogens 
Celiac antibody testing
CF testing

Management
Goal is “catch-up” weight gain
Most cases can be managed with nutrition intervention and/or feeding behavior modification
General principles:
High Calorie Diet
Close Follow-up
Keep a prospective feeding diary-72 hour
Assure access to WIC, food programs, other community resources...

Urinary Tract Infection in Children

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Risk factors for UTI
  • Poor urine flow
  • Previous proved or suspected UTI
  • Recurrent fever of unknown origin
  • Antenatally diagnosed renal abnormality
  • Family history of vesico-ureteric reflux
  • constipation
  • Dysfunctional voiding
  • Enlarged bladder
  • Abdominal mass
  • Evidence of spinal lesion
  • Poor growth high blood pressure

Urine sampling
A clean catch sample should be obtained
If not possible
 Use non invasive method i.e. Urine collection pad
 Do not use cotton wool balls, gauze or sanitary towels.
If non invasive method not possible
 Use catheter sample or suprapubic aspiration


Indications for urine culture
Diagnosis of acute pyelonephritis/upperUTI
High or intermediate risk of serious illness
Single positive result on dipstick testing
Recurrent UTI
Infection that does not respond to treatment in 24-48hrs
Clinical symptoms and dipstick testing don’t correlate

Localising site of infection
Acute pyelonephritis/upperUTI
Bacteriuria and fever 38’C or higher
Bacteriuria, loin pain/tenderness and fever less than 38’C


Cystitis/lowerUTI
Bacteriuria but no systemic features....

CNS Developmental Anomalies

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Classifications
Neural tube defect
Aqueductal stenosis and hydrocephalus
Chiari malformation
Dandy walker malformation
Craniosynostosis
Arachnoid cyst
Tethered cord syndrome
Split cord malformation

Neural tube defect
1- Neurulation: results in open lesions.
Anterior neuropore: craniorachischisis, anencephaly.
Posterior neuropore: meningomyelocele, meningocele
2- post neurulation: results in skin covered lesions.
Cranial :
   microcephaly, hydrencephaly , holoprocencephaly, lessencephaly, porencephaly, agenesis of corpus callosum

Spinal:
 diastomatomyelia, syringomyelia

Neuronal migration disorders
Agyria
Pachygyria
Hetrotopia
schizencephaly

Spinal dysraphysim
Spina bifida occulta
Congenital absence of spinous process and lamina, no visible meninges or neural tissue
Often incidental finding, no important when it occurs alone
May be as apart of deep seated lesions

Spina bifida cystica
Meningocele :
congenital defect in vertebral arches with cystic distension of meninges, no abnormality of neural tissue

Meningomyelocele:
as above but with structural or functional abnormality of spinal cord or cauda equina
1-2/1000 live birth

Lipo myeloschisis
A subcutaneous lipoma passes via a mid line defect in the lumbodorsal fascia , vertebral neural arch and dura with tethered cord
Is also called cauda equina lipoma
Spinal cord may be split myeloschisis
Dura is dehiscent
May be associated with dermal sinus which may end with epidermoid or dermoid cyst
Causing progressive neurological, urological and orthopaedic dysfunction via tethering of cord or compression
It presents with back mass, skin stigmata, bladder dysfunction, foot deformities or weakness
MRI tool of choice
Surgical treatment is aiming to untethering the cord and debulking the fatty tumour....

Urinary Tract Abnormalities

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Embryology:Intermediate mesoderma: Pronephros, Mesonephros and finally Metanephros
Mesonephros:
 Longitudinal swelling minimal urine production 6-10 weeks
 Mesonephric duct connecting cloaca to kidney
Metanephros
 Mesonephric buds
 Connection of ureteral bud with metanephric blastema induces nephron formation
 Functional by 10 weeks
 
Antenatally detected hydronephrosis
0,5% out of 12 000 antenatal scans revealed fetal malformations
0,25% had genitourinary tract abnormalities

Differential diagnosis
UPJ obstruction
VUR
Primary nonrefluxing megaureter
Ureterocele
Uterovesical junction obstruction
Ectopic ureter
Posterior urethral valves
Megacystitis megaureter
Physiological dilatation
Multicystic dysplastic kidney
Autosomal recessive polycystic kidney disease
Extrophy
Prune belly syndrome

Fetal intervention for hydronephrosis
Types of interventions:
Vesico-amniotic shunts
Fetal cystoscopy and endoscopic valve ablation

Post natal evaluation:
Day 1: Cases with oligohydramnios, urethral obstruction, multicystic renal dyplasia, bilateral moderate-to-severe hydronephrosis or uncertainty of diagnosis
Days 7-10: For mild or unilateral hydronephrosis
Voiding cystourethrography
 Not indicated if normal sonogram post natal
 Value if still post natal hydronephrosis

Infectious diseases and sepsis

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Infectious diseases are the most common group of diseases in the world.
Pandemic infectious diseases can involve a lot of persons including several countries.
Some very dangerous infectious diseases occur as bacterial weapon,For example smallpox, plague, anthrax
Infectious diseases are caused by mycoplasma, rickettsia, viruses, bacteria,fungi, protozoa, parasites.

Virulence is termed extent of  host damage by microorganisms.
Toxicity is termed ability for production and secretion of various toxins.
Invasiveness is termed the microorganism ability to get over host barrier mechanisms,reproduce and spread ability.

Reactions of macroorganism (host) in infectious diseases occur as follow:
Adjustment reactions
Compensative reactions
Defense reactions

Adjustment reactions are conditioned by environment (external factors) when host can not change impaired living conditions.
Compensative reactions are similar to adjustment reactions but connected with host changes.
Defense reactions are an biologic defense of the host.

Infective dose means for pathological origin:
Abscess origin requires 105 coccus
Penetration in blood circulation requires 107 
Sepsis develops with 109 coccus

Sepsis is general severe infectious disease,
caused by permanent or recurrent microorganism admission into blood from nidus of infection.
Sepsis is a polyetiologic, non contagious disease characterized by generalized infection, acyclic clinical course, and special significance of changed reactivity.

Difference from other infectious diseases:
1 polyetiologic causes: pyogenic bacteria,fungi;
2 non contagious,
3 non reproduce in experiment;
4 clinical course is stereotype, it is no depends on etiologic agent;
5 the most common sign of generalized infection
  is metastatic foci at a distance from primary focus;
6 cyclic course is absent;
7 incubation period is absent;
8 immunity is absent;
9 hyperergic reaction is predominance

Clinical definition of sepsis is as follow:
System  inflammatory reaction syndrome
(SIRS) is the response to invasion of difference microorganisms.
It is characterized by two or more SIRS signs.
Hyperthermia, Tachycardia, Tachypnoe, Leukocytosis.

Gram positive microorganisms are infective agent for septicopyemia.
Gram negative microorganisms are infective agent for septicemia or septic shock


Rheumatological emergencies

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Septic Arthritis

Arthritis due to seeding of a joint by a bacteria, mycobacterium or fungus.
Usually by hematogenous spread
Classified as gonococcal or non-gonococcal
Non-gonococcal arthritis is the more potentially destructive form

Gonococcal   
Young, healthy adults
Migratory polyarthralgias/arthritis
Tenosynovitis common
Rare positive Bcx
Positive joint cultures >25%


Non-gonococcal
Young children, elderly, Immunocompromised
Monoarthritis
Tenosynovitis rare
Positive Bcx 40-50%
Positive joint cultures >95%

Giant Cell Arteritis
Vasculitis of medium and large vessels arising from aortic arch
Patients generally over the age of 50; average age is 70
Multinucleated giant cells infiltrate vessels leading to patchy involvement  
Presents with polymyalgia rheumatica in 40% of cases
PMR= symmetrical pain/stiffness in shoulder and pelvic girdle muscles for more than 1 month in a patient age >50 with elevated ESR with rapid response to steroids

Diagnosis of GCA
Diagnose with 3/5 of the following:
Age > 50 years
New headache
Temporal artery abnormality
ESR > 50mm/hr
Abnormal artery biopsy with mononuclear cell infiltrate, granulomatous inflammation,usually with multinucleated giant cells

Scleroderma
Heterogeneous group of disorders with thickened skin as a hallmark
Endothelial disruption and fibroblast proliferation with unknown etiology
Categorized into localized and systemic

Localized Scleroderma
Affecting cutaneous and subcutaneous tissues only
a. Morphea-localized and generalized; patches of sclerotic skin on trunk and limbs
b. Linear- most common in childhood; abnormalities skin and subcutaneous tissues which often follow dermatomal distribution and are found predominantly on one side of the body

Systemic Scleroderma
Skin and internal organ involvement
Limited/CREST
Skin involvement limited to hands, face/neck
pulmonary HTN, GI involvement
Anti-centromere antibody

Diffuse
Extends proximal to MCPs, involves trunk
ILD, CV disease, renal disease, joint deformities, myopathy
Anti-Scl-70


Juvenile idiopathic arthritis

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Juvenile idiopathic arthritis (JIA) is the most common form of persistent arthritis in children. The 3 major types of JIA are oligoarticular JIA, polyarticular JIA and systemic JIA.
Oligoarticular (or pauciarticular) JIA affects 4 or fewer joints in the first 6 months of illness.Patients with oligoarticular JIA are more often ANA positive, when compared to other types of JIA.Accounts for about 50% of JIA cases. Usually involves the knees, ankles, and elbows but smaller joints such as the fingers and toes may also be affected. The hip is not affected unlike polyarticular JIA. It is usually not symmetrical, meaning the affected joints are on one side of the body rather than on both sides simultaneously.
Polyarticular JIA affects 5 or more joints in the first 6 months of disease. This subtype can include the neck and jaw as well as the small joints usually affected. This type of JIA is more common in girls than in boys. Usually the smaller joints are affected in polyarticular JIA, such as the fingers and hands, although weight-bearing joints such as the knees, hips, and ankles may also be affected.
Systemic JIA is characterized by arthritis, fever and a salmon pink rash.Systemic JIA can be challenging to diagnose because the fever and rash come and go.It affects males and females equally, unlike the other two subtypes of JIA.Systemic JIA may have internal organ involvement and lead to serositis (e.g. pericarditis).It is also known as "systemic onset juvenile rheumatoid arthritis".It is sometimes called "adolescent-onset Still's disease", to distinguish it from adult-onset Still's disease. However, there is some evidence that the two conditions are closely related.
Symptoms of JIA are often non-specific initially, and include lethargy, reduced physical activity, and poor appetite.The first manifestation, particularly in young children, may be limping. Children may also become quite ill, presenting with flu-like symptoms that persist. The cardinal clinical feature is persistent swelling of the affected joint(s), which commonly include the knee, ankle, wrist and small joints of the hands and feet. Swelling may be difficult to detect clinically, especially for joints such as those of the spine, sacroiliac joints, shoulder, hip and jaw, where imaging techniques such as ultrasound or MRI are very useful.
Pain is an important feature of JIA, but young children may have difficulty in communicating this symptom. Morning stiffness that improves later in the day is a common feature. Late effects of arthritis include joint contracture (stiff, bent joint) and joint damage. Children with JIA vary in the degree to which they are affected by particular symptoms.


Aspiration Pneumonia

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Misdirection of gastric contents into the larynx resulting from alteration in lower airway defenses such as glottic closure and the cough reflexHalf of all adults aspirate small amounts of oropharyngeal contents in their sleep
Aspiration pneumonia may occur in up to 10% of nursing home residents annually

Predisposing Factors
Decreased consciousness
Alcohol
Drugs
Hepatic failure
CVA
Anesthesia

Esophageal disorders
GERD
Stricture
Tracheoesophageal fistula
Incompetent cardiac sphincter
Protracted vomiting

Disruption of glottic closure
Endotracheal intubation
NG tube
Endoscopy/bronchoscopy
Neuromuscular disorders
Multiple sclerosis
Parkinson’s
Myasthenia gravis

Chemical Pneumonitis
Pathophysiology
Animal models demonstrate that clinically significant pneumonitis results from aspirating at least 1ml/kg of pH<2.5 gastric contents
Fluids that are not harmful to the airway
Water, saline, barium, gastric contents with pH>2.5

Clinical feature
Abrupt onset of dyspnea
Low grade fever
Pink frothy sputum
Diffuse crackles on exam
CXR: diffuse infiltrates

Bacterial Pneumonitis
Clinical features
Much more insidious onset than chemical pneumonitis (days to weeks)
Cough
Fever
Purulent sputum
CXR: Infiltrate frequently in dependent segments

An Approach to Vasculitis

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Definition
Presence of leukocytes in the vessel wall with reactive damage to mural structures
Loss of integrity ___ bleeding
Compromise of lumen ___ ischemia

Primary Vasculitis Syndromes
Wegener's granulomatosis
Churg-Strauss syndrome
Polyarteritis nodosa
Microscopic polyangiitis
Giant cell arteritis
Takayasu's arteritis
Henoch-Schönlein purpura
Idiopathic cutaneous vasculitis
Essential mixed cryoglobulinemia
Behçet's syndrome
Isolated vasculitis of the central nervous system
Cogan's syndrome
Kawasaki disease

Secondary Vasculitis Syndromes
Drug-induced vasculitis
Serum sickness
Vasculitis associated with other primary diseases
 Infection
 Malignancy
 Rheumatic disease

Congenital Adrenal Hyperplasia

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What is CAH?
It is a familial disorder of adrenal steroid biosynthesis with autosomal  recessive mode of inheritance.
The defect is expressed as adrenal enzyme deficiency.
5 major Enzymes deficiency are clinically important
    21-Hydroxylase
    11-b-Hydroxylase
    17-a-Hydroxylase
    3-b-Hsteroid hydrogenese
    20,22 Desmolase deficiency
The enzyme deficiency causes reduction in end-products, accumulation of hormone precursors & increased ACTH production.
The clinical picture reflects the effects of inadequate production of cortisol and aldosterone and the increased production of androgens and steroid metabolites.

21-Hydroxylase Deficiency
Most common type, accounts for >80% of cases.
Incidence is 1:5000 to 1:15000 live birth.
Gene is located on the short arm of chromosome 6 near the C4 locus in close association with HLA genes.
Heterozygous carriers can be detected by ACTH stimulation test.
It is characterized by reduced production of cortisol and aldosterone and increased production of progesterone;17-OH-progesterone, and sex steroids.
The urinary steroid metabolites (17-ketosteroids and pregnanetriol) are elevated above normal levels.
Decreased secretion of aldosterone results in salt loss with hyponatremia and hyperkalemia; plasma renin activity is therefore elevated.
In partial enzyme deficiencies, the aldosterone deficiency is not expressed, and patients remain normonatremic and normokalemic.
The excess androgens causes virilization of girls & ambiguous genitalia & dark scrotum in boys.
2 forms, classic early virilization type with or without salt-losing crisis and non-classic type with late-onset virilization.
Male babies with non salt-losing non-classic type remains asymptomatic till late childhood when they may show signs of sexual precocity.
Because members of the same family may have classic, non-classic & asymptomatic forms, the disorder may be due to allelic variations of the same enzyme.
Mass neonatal screening using filter paper blood sample for 17-OH-Progesterone is used in the USA.

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