Rheumatic fever occurs after a streptococcal infection (usually caused by Group A Beta-Hemolytic Strep (GABHS)).
It is an inflammatory condition that affects the joints, skin, heart and brain.
Major criteria are referred to as Jones criteria
J – Joint involvement which is usually migratory and inflammatory joint involvement that starts in the lower joints and ascends to upper joints
O – (“O” Looks like heart shape) – indicating that patients can develop myocarditis or inflammation of the heart
N – Nodules that are subcutaneous
E – Erythema marginatum which is a rash of ring-like lesions that can start in the trunk or arms. When joined with other rings, it can create a snake-like appearance
S – Sydenham chorea is a late feature which is characterized by jerky, uncontrollable, and purposeless movements resembling twitches
Minor criteria include
C – CRP Increased
A – Arthralgia
F – Fever
E – Elevated ESR
P – Prolonged PR Interval
A – Anamesis
L – Leukocytosis
Diagnosis of rheumatic fever is made after a strep infection (indicated by either throat cultures growing GABHS OR elevated anti-streptolysin O titers in the blood) and:
Two major criteria OR
One major criterion and two minor criteria
1.cleft palate is best repaired
1) Soon after birth B
2) At one month
3) At 6-8 months
4) Between 12-18 months
Cleft lip repair should be done between 3-6 months of age.
2. Intra-osseous access for drugs and fluid administration is recommended for paediatric group up to the age of
1) <one year
2) <4 yeats
3) <6 years
4) Up to 12 years
3. Which of the following is a true statement regarding congenital diaphragmatic hernia (CDH)
1) Common on right side
2) Associated with pulmonary hypoplasia
3) Present with recurrent vomiting at birth
4) Baby benefited with bag mask ventilation
CHD is common on left side by which gastric contents herniate to thoracic cavity , Bag mask ventilation in these babies leads to gastric distension which may further compress the lungs and increase mediastinal shift.
Most Common Site of Primary Bone Tumors
- Chondroblastoma (before physeal closure)
- Osteoclastom/Giant cell tumor (after physeal closure in adults)
- Articular osteochondroma
- Bone cyst
- Osteoclastoma (in children)
- Osteomyelitis mostly starts in metaphysis
- Round cell lesions: Ewing’s sarcoma/Multiple myeloma /Reticulum cell sarcoma
- Osteoid osteoma
- The hallmark feature of rheumatoid arthritis is persistent symmetric polyarthritis (synovitis) of hands and feet.
- The spontaneous onset of excruciating pain, edema, and inflammation in the metatarsalphalangeal joint of the great toe (podagra) is highly suggestive of acute crystal-induced arthritis.
- Podagra is the initial joint manifestation in 50% of gout cases and is eventually involved in 90%.
- RA is a chronic autoimmune multisystem disease having inflammatory arthritis and systemic manifestation.
1. Women (30 to 50 years) are more commonly affected
2. HLA-DR 4 Q is a risk factor for RA.
3. Initial site of disease is synovial membrane
Initiation phase – It is due to non – specific inflammation
Amplification phase – Due to T cell activation
Chronic inflammatory phase – Due to cytokines IL – 1, TNF- alpha (AIPG 2009) and IL – 6
Four of seven criteria are required
1. Morning stiffness – lasting 1 hour before maximal improvement
2. Arthritis of 3 or more joint areas – 14 possible joint areas are right or left PIP MCP, wrist, elbow, knee, ankle and MTP joints
3. Arthritis of hand joints.
4. Symmetrical arthritis.
5. Rheumatoid nodules.
6. Positive Serum rheumatoid factor
7. Radiographic changes – including erosions or unequivocal bong decalcification localized in or most marked adjacent to the involved joint.
Important parasympathetic ganglia in head and neck are :
(i) otic ganglion,
(ii) submandibular ganglion,
(iii) pterygopalatine ganglion
(iv) ciliary ganglion.
Topographically, it is connected to mandibular nerve, while functionally it is related to glossopharyngeal (IX) nerve. Its roots are:-
Sensory root:-By Auriculotemporal nerve.
Sympathetic root:-By sympathetic plexus around middle meningeal artery.
Parasympathetic (secretomotor) root: - This root is by lesser petrosal nerve. Preganglionic fibres begin in inferior salivatory nucleus, pass through glossopharyngeal nerve, then its tympanic branch, tympanic plexus, and the lesser petrosal nerve and relay in otic ganglion.
Postganglionic fibers pass through auriculotemporal nerve and supplies parotid gland.
Motor root :- It is derived from nerve to medial pterygoid which passes unrelayed through ganglion and supplied tensor veli palatini and tensor tympani (Note :- Otic ganglion has a motor root, beside three standard roots of parasympathetic ganglion of head and neck: sensory, sympathetic and parasympathetic).
Functionally, submandibular ganglion is connected to facial nerve, while topographically it is connected to lingual branch ofmandibular nerve. Its roots are :-
Sensory root: - It is from lingual nerve.
Sympathetic root: - It is from sympathetic plexus around facial artery, which contains postganglionic fibers from superior cervical ganglion of sympathetic trunk.
Secretomotor (parasympathetic) root: - Preganglionic fibers arise from superior salivatory nucleus pass through facial nerve then its chorda tympani branch which joins lingual nerve and relay in submandibular ganglion.
Postganglionic fibers supply :-Directly submandibulargland.
Through lingual nerveSublingual salivary gland and glands in oral cavity.
Pterygopalatine Ganglion (Sphenopalatine Ganglion)
It is the largest parasympathetic ganglion, suspended by two roots to maxillary nerve. Functionally, it is related to facial nerve. It is called ganglion of "hay fever". Its roots are:-
Sensory root:-It is from maxillary nerve.
Sympathetic root:-It is sympathetic plexus around internal carotid artery through deep petrosal nerve.
Secretomotor (parasympathetic) root:-Preganglionic fibers arise from lacrimatory nucleus, pass through facial nerve, then to its greater petrosal branch. Greater petrosal nerve unites with deep petrosal nerve (sympathetic fibers) to form nerve to pterygoid canal (Vidian nerve). Fibers reach to pterygopalatine ganglion; only fibers of greater petrosal nerve relay in the ganglion, not of deep petrosal nerve.
Postganglionic fibers supply lacrimal gland and palatal glands, and pharyngeal glands.
Branches of the pterygopalatine ganglion are :-
For lacrimal gland: - Postganglionic fibers pass through zygomatic nerve (branch of maxillary nerve), its zygomaticotemporal division which gives communicating branch to lacrimal nerve for supplying lacrimal gland.
Nasopalatine nerve:-For nasal and palatal glands.
Nasal branches: - For mucous membrane and glands oflateral wall of nasal cavity.
Palatine branches: - One greater palatine and 2-3 lesser palatine branches for glands of soft palate and hard palate.
Orbital branches: - For orbital periosteum.
Pharyngeal branches:-For glands of pharynx.
Topographically, ciliary ganglion is related to nasociliary nerve (a branch of ophthalmic division of trigeminal nerve), but functionally it is related to oculomotor nerve. Its roots are:-
Sensory root:-It is from nasociliary nerve.
Sympathetic root: - It is from plexus around ophthalmic artery.
Parasympathetic root: - It is from a branch to inferior oblique muscle. These fibers arise from Edinger – Westphalnucleus, join oculomotor nerve and then to its branch to inferior oblique to relay in ciliary ganglion.
Postganglionic fibers pass through short ciliary nerves to supply sphincter pupillae and ciliary muscles.
Umbilical cord prolapse
acute, life-threatening emergency for the fetus, in which a part of the umbilical cord lies between the antecedent part of the fetus (mostly head) and the pelvic wall, causing rupture of membranes
Epidemiology: rare (0.5% births)
Etiology: often seen in presentation anomalies (e.g., breech presentation, transverse fetal position), multiple pregnancy, long umbilical cord, or abnormal fetal movement (polyhydramnios, premature birth)
Clinical features: -
- an abrupt change from a previously normal CTG to one with fetal bradycardia or recurrent,
- severe decelerations,
- occuring after the rupture of membranes
Diagnostics: vaginal palpation → thick, pulsating cord is palpable
Treatment: Trendelenburg position; fetus is pushed back into the uterus; immediate tocolysis using β2-mimetics (e.g., fenoterol) → emergency cesarean section
Abetalipoproteinemia: decrease ApoB-48, Apo B-100; pigmentary degeneration of retina, acanthocytes, steatorrhea, cerebellar ataxia.
Acute Fatty Liver of Pregnancy: microvesicular steatosis in the liver, mitochondrial dysfunction in the oxidation of fatty acids leading to an accumulation in hepatocytes
Alkaptonuria: homogentisate oxidase deficiency, increase homogenistic acid, ochronosis, dark blue urine.
AcylCoA Dehydrogenase deficiency (MCAD): fasting hypoglycemia, no ketone bodies, dicarboxilic acidemia.
Bernard Soulier Sd: gp1b deficiency, prolonged bleeding time
Bloom Sd: chromosome 15, Ashkenazi Jews, BLM gene.
Carpenter Sd: craniosynostosis, acrocephaly, craniofacial asymmetry, increased ICP, cutaneous syndactyly, polydactily, mild-profound MR.
Chediak Higashi Sd: Lyst gene mutation, microtubule polymerization defect, no phagolysosome formation, albinism.
Chondrodystrophy: normal-sized trunk and abnormally short limbs and extremities (dwarfism)
Congenital Adrenal Hyperplasia: 17alpha or 21beta or 11 beta hydroxylase deficiency; enlargemente od adrenal glands due to increase ACTH
Congenital Hepatic Fibrosis: hepatic (periporta) fibrosis, irregularly shaped proliferating bile duct, portal hypertension, renal cystic disease.
Cystic Fibrosis: CFTR gene, Phe508, defective Chloride channel, chromosome 7.
Dubin-Johnson Sd: direct hyperBbnemia, cMOAT deficiency, black liver
Endocardial Fibroelastosis: restrictive/infiltrative cardiomyopathy, thick fibroelastic tissue in endocardium of young children, <2yo
Familial Mediterranean Fever: chromosome 16, recurrent autoinflammatory disease, characterized by F°, PMN disfx, sudden attacks pain/inflammation (7 types of attacks (abdominal, joints, chest, scrotal, myalgias, erysipeloid, fever). Complication: AA-amyloidosis
Fanconi Anemia: genetic loss of DNA crosslink repair, often progresses to AML, short stature, ↑incidence of tumors/leukemia, aplastic anemia
Friedreich’s Ataxia: GAA triplet repeat, chromosome 9, neuronal degeneration, progressive gait & limb ataxia, arreflexia, hypertrophic cardiomyopathy, axonal sensory neuropathy, kyphoscoliosis, dysarthria, hand clumsiness, loss of sense of position, impaired vibratory sensation.
Gaucher’s disease: glucocerebrosidase deficiency, glucocerebroside accumulation, femur necrosis, crumpled paper inclusions in macrophages.
Ganzman’s thromboasthenia: gpIIbIIIa deficiency, deficient platelet aggregation.
Hartnup Disease: tryptophan deficiency, leads to niacin deficiency, pellagra-like dermatosis
Hemochromatosis: HFE gene, C282Y MC mutation, chromosome 6, unrestricted reabsorption of Fe+ in SI, iron deposits in organs, bronze diabetes, DM1, malabsorption, cardiomyopathy, joint degeneration, increased iron, ferritin, TIBC. Complications: liver cirrhosis, hepatocelullar carcinoma
Homocystinuria: due to B6 deficiency (defective Cystathionine synthase) or due to B9,B12 deficiency (defective Homocysteine Methyltrasnferase), dislocated lenses (in & down), DVT, stroke, atherosclerosis, MR.
Krabbe's Disease: Galactocerebrosidase deficiency, galactocerebroside accumulation, gobloid cells, optic atrophy, peripheral neuropathy.
Leukocyte Adhesion Defect (LAD): CD-18+ deficiency, omphalitis in newborns, chronic recurrent bacterial infxs, increase WBC count, no abscess or pus formation.
Metachromic Leukodystrophy: Aryl-sulfatase A deficiency, sulfatides accumulation, Demyelination (central & peripheral), Ataxia, Demantia (DAD)
Niemann-Pick Disease: sphingomyelinase deficiency, sphingomyelin accumulation, HSM, cherry-red macula, foam cells.
Phenylketonuria (PKU): phenylalanine hydroxylase deficiency, Phe accumulation, MR, microcephaly, diet low in Phe!!! also in pregnancy, avoid aspartame, musty odor.
Polycystic Kidney Disease (children): ARPKD, rogressive & fatal renal failure, multiple enlarged cysts perpendicualr to renal capsule, association with liver cysts. Bilateral palpable mass.
Rotor Sd: direct hyperBbnemia, cMOAT deficiency, no black liver
Shwaman Diamond Sd: exocrine pancreatic insufficiency (2°MCC in children after CF), bone marrow dysfunction, skeletal abnormalities, short stature.
Situs inversus: assoc w/ Kartagener sd
Sicke Cell Disease and Trait: Hb S, beta globin chain, chromosome 11, position 6, nucleotide codon change (glutamic acid --> valine), vaso-occlusive crisis (pain), autosplenectomy, acute chest pain sd, priapism, hand-foot sd, leg ulcers, aplastic crisis, drepanocytes & Howell-Jolly bodies, hemolytic anemia, jaundice, bone marrow hyperplasia
Tay-Sachs Disease: Hexoaminidase A deficiency, GM2 accumulation, cherry-red macula, onion skin lysosomes.
Thalasemia: alpha (chromosome 16, gene deletion), beta (chromosome 11, point mutation)
Werner Disease: adult progeria
Wilson’s Disease: Chromosome 13, WD gene, ATP7B gene (encondes for Copper transporting ATPase), copper accumulation in liver, brain (putamen), eyes (Descemet membrane - Kayser-Fleischer ring), decreased ceruloplasmin.
Xeroderma Pigmentosa: defective excision endonuclease, no repair of thymine dymers caused by UV radiation, excessive freckling, multiple skin cancers.
TYPES OF TRACHEOSTOMY TUBE
Metallic Tracheostomy Tube
Nonmetallic Tracheostomy Tube
Cuffed Tracheostomy Tubes
Uncuffed Tracheostomy Tubes
Nonmetallic Tracheostomy Tube - Cuffed tubes are used in situation where positive pressure ventilation is required, or when the airway is at risk from aspiration. (In unconscious patient or when patient is on respiration).
Metallic Tracheostomy Tube -Metallic tubes are formed from the alloy of silver, copper and phosphorus .
L: Lungs - Atypical pneumonia.
Relatively nonproductive cough
Pleuritic or non pleuritic chest pain
Confluent or patchy infiltrates on x-ray
Random fact: Interstitial infiltrates aren’t seen often like in other atypical pneumonias.
E: Encephalon - Neurologic abnormalities.
Confusion or changes in mental status
G: Gastrointestinal symptoms.
ION: Na ion decreases.
Hyponatremia (serum sodium level of 131 meq/L)
Chronic superficial glossitis
Chronic irritation -sharp tooth
Ulcer –most common
irregular margins evertededges
Induratedgrowth or mass
Usually age > 50 yrs
Sex both equally
Painless lump or ulcer on tongue
Ankyloglossia-immobility of tongue
Pain –involvement of nerve
Horsenessof voice & dysphagiain posterior 3rd tongue
Lump in neck
Site -common anterior 2/3 near edges
Ulcer papilliferoursor warty, lump fissure
Palpation of posterior 2/3 tongue
Examination of lymph node
Biopsy : margin or excision biopsy
Ultrasound deep LN
CT scan bone invasion & mets
MRI for oral cavity oropharynx