Acute Rheumatic fever

Acute Rheumatic fever:

►RHD is the most common cause of heart disease in children in developing countries

►ARF is mainly a disease of children aged 5–14 years. Initial episodes become less common in older adolescents and young adults and are rare in persons aged >30 years. By contrast, recurrent episodes of ARF remain relatively common in adolescents and young adults. 

►The most common clinical presentation of ARF is polyarthritis and fever. Polyarthritis is present in 60–75% of cases and carditis in 50–60%. 

►Heart Involvement:
Up to 60% of patients with ARF progress to RHD. The endocardium, pericardium, or myocardium may be affected. Valvular damage is the hallmark of rheumatic carditis. 
MC in Acute cases: MR
MC in Chronic cases: MS
MC overall: MR

►ARF almost always affects the large joints—most commonly the knees, ankles, hips, and elbows—and is asymmetric. 

►Sydenham's chorea commonly occurs in the absence of other manifestations, follows a prolonged latent period after group A streptococcal infection, and is found mainly in females. 
Clinical triad of:
Chorea
Hypotonia/weakness
Emotional lability / OCD / ADHD

►Skin Manifestations: The classic rash of ARF is erythema marginatum, It occurs usually on the trunk, sometimes on the limbs, but almost never on the face.

►The most common serologic tests are the anti-streptolysin O (ASO) and anti-DNase B (ADB) titers.

►The mainstay of primary prevention for ARF remains primary prophylaxis (i.e., the timely and complete treatment of group A streptococcal sore throat with antibiotics). If commenced within 9 days of sore throat onset, a course of penicillin (as outlined above for treatment of ARF) will prevent almost all cases of ARF that would otherwise have developed.

Must know about Rheumatoid arthritis (RA)

►Rheumatoid arthritis (RA) is the most common form of chronic inflammatory arthritis and often results in joint damage and physical disability.

►Pleural disease, the most common pulmonary manifestation of RA, may produce pleuritic chest pain and dyspnea, as well as a pleural friction rub and effusion. 

►The earliest involved joints are typically the small joints of the hands and feet. The initial pattern of joint involvement may be monoarticular, oligoarticular (4 joints), or polyarticular (>5 joints), usually in a symmetric distribution. 

►Once the disease process of RA is established, the wrists, metacarpophalangeal (MCP), and proximal interphalangeal (PIP) joints stand out as the most frequently involved joints 

►Inflammation about the ulnar styloid and tenosynovitis of the extensor carpi ulnaris may cause subluxation of the distal ulna, resulting in a "piano-key movement" of the ulnar styloid. 

►Flatfoot deformity is by far the most common hindfoot deformity in rheumatoid arthritis

►One of the most common manifestations of RA in the
hands is tenosynovitis in flexor tendon sheaths; this can
be a major cause of hand weakness.

►The atlantoaxial joint is
prone to subluxation in several directions and is summarized
later.
• The atlas can move: anteriorly on the axis (most
common). > Posteriorly > Vertically
•The earliest and most common symptom of cervical subluxation
is pain radiating up into the occiput.

►RA can be associated with generalized osteopenia
and osteoporosis owing to the effects of drugs
The fibula is the most common fracture site.

►The most frequent site of cardiac involvement in RA is the pericardium. 
Mitral regurgitation is the most common valvular abnormality in RA.

►Rheumatoid vasculitis is seen most commonly in patients with long-standing disease, a positive test for serum RF, and hypocomplementemia; the overall incidence is quite rare, occurring in no more than 1% of cases.

►The most frequently recognized skin lesion in RA is the
rheumatoid nodule. They occur most often on extensor surfaces or pressure points, such as the olecranon process and the proximal ulna, as well as on tendons.
They are subcutaneous and vary in consistency from a soft,
amorphous, entirely mobile mass to a hard, rubbery mass
attached firmly to the periosteum.

►Ocular Keratoconjunctivitis sicca is the most common ophthalmic manifestation of rheumatoid arthritis, with a reported prevalence of 15–25%.

►The most common flexor tendon to rupture in rheumatoid arthritis is FPL rupture, caused by scaphotrapezial synovitis. 

►A normochromic, normocytic anemia often develops in patients with RA and is the most common hematologic abnormality. 

►Felty's syndrome is defined by the clinical triad of neutropenia, splenomegaly, and nodular RA and is seen in less than 1% of patients.

►The most common histopathologic type of lymphoma is a diffuse large B-cell lymphoma.

►The overall mortality rate in RA is two times greater than the general population, with ischemic heart disease being the most common cause of death followed by infection. 

►IgM, IgG, and IgA isotypes of RF occur in sera from patients with RA, although the IgM isotype is the one most frequently measured by commercial laboratories.

►Muscle: In biopsy specimens, atrophy of type II fibers is most
common.

►AZA is most commonly used as a substitute
for MTX when there are contraindications or intolerance
to MTX

Ankylosing spondylitis- Golden Points

►Ankylosing spondylitis is the most frequent seronegative inflammatory spinal disease in adults and the prototype for other members of the spondyloarthropathies.

►The symptoms of the disease are usually first noticed in late adolescence or early adulthood; the median age in Western countries is 23. In 5% of patients, symptoms begin after age 40. 

►Sacroiliitis is often the earliest manifestations of AS. 

►The most important gene in ankylosing spondylitis is HLAB27.
In most populations, it is present in more than 90% of
patients and in less than 10% of the general population.

►Ankylosing spondylitis is the most common association with iritis in young patients, particularly men.

►The most common extraarticular manifestation is acute anterior uveitis, which occurs in 40% of patients and can antedate the spondylitis. 

►Aortic dilatation and aortic regurgitation may be present in approximately 10% of patients. 

►-Ankylosing spondylitis is associated with IgA nephropathy

►Entheseal involvement most frequently occurs at sites
rich in fibrocartilage such as the Achilles tendon.

►The girdle or “root” joints (hips and shoulders) are the most
frequently involved extra-axial joints in AS, and pain in
these areas is the presenting symptom in up to 15% of
patients.

►No laboratory test is diagnostic of AS. In most ethnic groups, HLA-B27 is present in 80–90% of patients. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are often, but not always, elevated. 

►The most common indication for surgery in patients with AS is severe hip joint arthritis, the pain and stiffness of which are usually dramatically relieved by total hip arthroplasty. 

►-Only 5% of those with Ankylosing spondylitis have Inflammatory bowel. But FIFTY percent have mucosal ulcerations!

►Neurologic complications of AS can be caused by fracture,
instability, compression, or inflammation. Traffic accidents
or minor trauma can cause spinal fractures. The C5-C6 or
C6-C7 level is the most commonly involved site.

►The most common pulmonary manifestation of ankylosing spondylitis is ILD (MC upper lobe)

►Radiographically demonstrable sacroiliitis is eventually present in AS. The earliest changes by standard radiography are blurring of the cortical margins of the subchondral bone, followed by erosions and sclerosis. 

►Golimumab is a TNF inhibitor useful for Ankylosing spondylitis. 
This disease is the ONLY time a TNF inhibitor is the FIRST line of therapy

►Methotrexate does not work for the Axial skeleton such as ankylosing spondylitis. it is for peripheral joints such as RA and psoriatic arthritis

Most Common Vertebral Tumors

 •The grade and distribution of primary spinal extradural tumors vary with age. In adults the most common benign primary vertebral tumors of the spine are hemangiomas, accounting for 30% of all primary spine tumors. 

•In adults the most common malignant primary vertebral tumors of the spine plasmacytomas

•In children the most common benign primary vertebral tumors of the spine are osteomas/osteoblastomas

•In children the most common malignant primary vertebral tumors of the spine is Ewing's sarcoma

Most Common Brain Tumors

• Gliomas > Meningiomas > Astrocytomas are the most common type of primary brain tumor in adults. 

• "Meningiomas are diagnosed with increasing frequency as more people undergo neuroimaging studies for various indications. They are now the most common primary brain tumor, accounting for approximately 32% of the total."

• Pilocytic astrocytoma (PA; WHO grade 1) is the most common astrocytic tumor of childhood.

• In children, the most common situation is with cerebellar and optic-pathway pilocytic astrocytoma, whereas in adults, this tends to occur most commonly with hypothalamic pilocytic astrocytoma. 

• Tumors of the lining of the spinal cord and nerve roots predominate (50% to 80% of all spinal tumors); schwannomas and meningiomas are most common, followed by ependymomas.

• The most common brainstem tumor is the pontine glioma, which presents most frequently with cranial nerve VI and VII palsies.

• The CNS is the second most common site of malignancy in children behind leukemia, with the majority of tumors located infratentorially.

• Medulloblastomas are the most common posterior fossa tumors in children.

• Medulloblastomas are the most common malignant brain tumor of childhood

• Germinomas are the most common type, accounting for 33% to 50% of pineal tumors. The peak incidence of germ cell tumors is in the second decade, and few present after the third decade. Gliomas are the next most common pineal region tumor.

• Craniopharyngiomas are the most common nonglial brain tumors in children, occurring primarily in the late first and second decades, although they can present at any age.

Spinal Axis Tumours:

• Of the intradural neoplasms, extramedullary schwannomas and meningiomas are the most common. Schwannomas and meningiomas are normally intradural, but occasionally may present as extradural tumors.

• The most common intramedullary tumors are ependymoma and astrocytoma.

• Briefly:
Most Common Spinal Tumor - Metastasis. 
MC Primary Spinal Tumor - Nerve sheath tumor. 
MC Intramedullary tumor - Ependymoma 
MC site of spinal tumors - Extradural 
MC site of primary spinal tumors - Intradural Extramedullary.

Most commons in Pediatrics

Trisomy 21 (Down's syndrome) patients commonly have a primum ASD > Secundum ASD.
 Isotretinoin (retinoic acid) is very teratogenic with many exposed fetuses developing congenital heart defects. The most common defects are conotruncal and great artery abnormalities, such as transposition of the great arteries and TOF.

VACTERL association:

Vertebral anomalies, Anal atresia, Cardiac defects(VSD), Tracheoesophageal fistula and/or Esophageal atresia, Renal anomalies and Limb defects.

CHARGE syndrome
The letters stand for: coloboma of the eye, heart defects(MC, TOF), atresia of the nasal choanae, retardation of growth and/or development, genital and/or urinary abnormalities, and ear abnormalities and deafness.

Most common cardiac malformation:
It is Bisuspid valve in adults, and VSD in children. It is septal hypertrophy in the newborn.

OVALE physiologically closes soon after birth and anatomically by 3 months. 
DUCTUS physiologically closes by 10 -15 hrs after birth and anatomically by 2- 3 wks.

Most commons in Respiratory System

 In lung cancer, partial deletion of the short arm of chromosome
3 (3p) has been one of the earliest and most common
genetic changes. Chromosome 3p deletion occurs in
almost 100% of SCLC and 90% of NSCLC.
Mutation and more commonly amplification of PIK3CA , which encodes the catalytic
subunit of phosphatidylinositol 3-kinase (PI3K), occur
most commonly in squamous cell carcinomas.


 p53 inactivating mutations are the most common
alterations in cancer, especially lung cancer, where 17p13 frequently demonstrates hemizygous deletion and mutational inactivation in the remaining allele.


 Lung cancer is the most common
cause of brain metastases.


 Patients with persistent
headaches without any structural abnormalities on brain imaging
should undergo spinal fluid evaluation for leptomeningeal
carcinomatosis. A positive cytology is found on an initial lumbar
puncture in 50% to 70% of the patients. In patients with
clinical signs and symptoms suggestive of leptomeningeal metastases,
repeat lumbar puncture is required if the first cytology
evaluation is negative.


 Today, breast cancer is the second most common cause of BM among all solid malignancies, second only to lung cancer; furthermore, it is the most common cause of leptomeningeal carcinomatosis.


 Lung cancer can metastasize to almost
any bone, although the axial skeleton and proximal long bones
are most commonly involved. Bone pain is present in up to
25% of all lung cancer patients at presentation.


 Dermatomyositis and polymyositis are inflammatory conditions characterized by muscle weakness and tenderness, and skin changes, in the case of dermatomyositis.


 Breast and lung cancers are the most common associated malignancies. Most
cases are idiopathic in nature and unrelated to cancer.

Pulsus alternans

Pulsus alternans, in contrast, is defined by beat-to-beat variability of pulse amplitude. It is present only when every other phase I Korotkoff sound is audible as the cuff pressure is lowered slowly, typically in a patient with a regular heart rhythm and independent of the respiratory cycle. In patients with normal heart rates, the finding of pulsus alternans indicates severe left ventricular dysfunction, caused by ischemic or valvular heart disease, long-standing hypertension, or idiopathic dilated cardiomyopathy. In patients with rapid heart rates, Pulsus alternans is of less significance as people with normal heart can develop the finding during paroxysmal tachycardia.

Brain tumors and anticonvulsant drug therapy:

Patients with brain tumors who present with seizures, require anticonvulsant drug therapy. There is no role for prophylactic anticonvulsant drugs in patients who have not had a seizure, thus their use should be restricted to those who have had a convincing ictal event. The agents of choice are those drugs that do not induce the hepatic microsomal enzyme system. These include levetiracetam, topiramate, lamotrigine, valproic acid, or lacosamide. Other drugs such as phenytoin and carbamazepine are used less frequently because they are potent enzyme inducers that can interfere with both glucocorticoid metabolism and the metabolism of chemotherapeutic agents needed to treat the underlying systemic malignancy or the primary brain tumor.