DIAGNOSTIC CRITERIA OF MULTIPLE MYELOMA :

Major:
1.Plasmacytoma
2.Bone marrow plasmacytosis >30% of plasma cells
3.Monoclonal globin spike IgG > 35/L, or IgA >20g/L
4. Light chain excretion on urine electrophoresis > 1g/24hours

Minor:
1.Bone marrow plasmacytosis, 10-30% plasma cells
2.Monoclonal globulin spike, IgG < 35 g/l or IgA <20g/L
3. Lytic bine leaions
4. Normal IgM < 500mg/L, IgA < 1g/L or IgG < 6g/l

Criteria
1. 1 major and 1 minor
or
2. 3 minor that should include 1 and 2 of minor criteria.

Hormones that do not cross placenta

Insulin
PTH
calcitonin

Tetrad of Oligohydramnios

1. Facial clefts
2. IUGR
3. Limb reduction defects
4. Pulmonary hypolasia

Mnemonic:Aspirin: side effects

ASPIRIN:
A : Asthma
S : Salicyalism
P : Peptic ulcer disease/ Phosphorylation-oxidation uncoupling/ PPH/ Platelet disaggregation/ Premature closure of PDA
I : Intestinal blood loss
R : Reye's syndrome
I : Idiosyncracy
N : Noise (tinnitus)

Classification of Sequesterum:

According to shape :
Pencil – Infants
Cylindrical/tubular – Infants
Ring – External fixator
Conical – Amputation stump
Annular – Amputation stump
Coralliform – Perthes’ disease

According to consistency :
Coke like – Tuberculosis
Feathery – Syphilis
Sand like – TB osteomyelitis in metaphysis

According to Color :
Black – Amputation stump , Fungal osteomyelitis
Green – Pseudomonal Osteomyelitis

ITCH you must know.

Winter itch--> Asteotic aczema/xerotic eczema
Swimmer's itch---> bilharziasis/Schitosomiasis
Dhobi's/Jock itch-->Tinea cruris
Ground itch--> Nematode larvae
Barber's Itch--> Sycoses barbae
Itch mite--> Sarcoptes scabiei(scabies)

Baby syndromes

Grey baby syndrome:-

The grey baby syndrome is a rare condition almost exclusively seen in neonates and very young infants receiving high doses of CHLORAMPHENICOL.
Toxic blood levels of chloramphenicol occurs due to :
inadeqaute conjugation of chloramphenicol with glucuronic acid because of inadequate activity of glucuronyl transferase in the newborn liver
decreased renal excretion of the unconjugated chloramphenicol
The infant is cyanosed, is acidotic, has cold peripheries and has the signs of all of marked hypotonia, poor feeding, vomiting, loose stools and a distended abdomen.

Bronze baby syndrome:-
When infants with cholestatic jaundice are exposed to phototherapy they may develop a greyish-brown discoloration of skin.
Probably due to accumulation of porphyrins & other metabolites (formed by action of phototherapy) in plasma of infants who develop cholestasis.Not all infants with cholestasis will develop it.
Dark, grayish brown discoloration of skin
May persist for months
Associated with Conjugated Hyperbilirubinemia.

Carbon baby syndrome:
Universal acquired melanosis is a rare form of hypermelanosis which was synonymously referred to as "Carbon baby".A form of progressive mucocutaneous pigmentation caused by singly dispersed melanosomes within keratinocytes.

Blueberry muffin baby syndrome:
Blueberry muffin baby is a term used to describe neonates whose skin resembles a blueberry muffin (i.e., the skin shows diffuse, dark blue to violaceous purpuric macules and papules). The spots represent dermal hematopoiesis and are a sign of serious systemic disease, most often congenital infection. The congenital infection most commonly associated with this appearance is congenital rubella.

Blue baby syndrome:
Here blue baby implies byblue due to cyanosis.
It can be due to:
cyanotic heart diseases or
methemoglobinemia.

Named Murmurs

1- Uterine Souffle:- Continuous arterial murmur over pregnant uterus
2- Mammary Souffle:- Continuous arterial mu. Over breast during pregnancy/early post partum
3- Duroziez M. :- In AR
4- Carvallo's M. :- Early Systolic M. ( In TR with normal Pul.Artery pressure/In SABE in IV drug users)
5- Graham-Steel M. :- Early Diastolic M. In PulmonarybIncompitance
6- Austin Flint M. :- Mid + late diastolic M. In severe AR (Low pitched which is soft;Dekayed diastolic;Mitral)
7- Carey Coomb's M. :- (Low pitched, Delayed diastolic,Mitral) Short mid diastolic M.seen In Acute Mitral Valvulitis, Acute Rheumatic carditis 8- Siegle's M. :- In AR
9- Gibson's M. :- Train in tunnel M
Continuous M in PDA

Bacteriostatic drugs

Stasis Causes No Total or No complete Though
Massive Elimination

Sulfonamides
Chloramphenicol
Nitrofurantoin
Tetracycline/Tigecycline
Oxazolidinone
Novobiocin Clindamycin
Trimethoprim
Macrolides
Ethambutol

IMPORTANT FUNDOSCOPY FINDINGS

1. CHERRY RED SPOTS :
- TAY SACH'S DISEASE
-NIEMANN PICK'S DISEASE
-CENTRAL RETINAL ARTERY OCCLUSION(CRA0)
-COMOTIO RETINAE( BERLIN'S OEDEMA)
-FABER'S LIPO GRANULOMATOSIS
-METACHROMATIC LEUCODYSTROPHY
-SANDHOFF'S DISEASE
-SIALIDIOSIS
-GAUCHER'S DISEASE
-MULTIPLE SULFATASE DEFICIENCY
-DRUGS (QUININE &DAPSONE)
2. ROTH'S SPOTS
-CANDIDIAL CHORIORETINITIS
-SUB ACUTE BACTERIAL ENDOCARDITIS
-LEUKEMIC DEPOSITS
-ANAEMIC RETINOPATHY
-AIDS
3. GOLDEN RAIN APPEARENCE
-SYNCHYSIS SCINTILLANS
4. DOME OF CHAMPAGNE CORK APPEARANCE
-PAPILLOEDEMA
5. SPLASHED TOMATO APPEARANCE
-ISCHAEMIC CENTRAL RETINAL VEIN OCCLUSION(CRVO)
6. CIRCULAR RING REFLEX
- CENTRAL SEROUS RETINOPPATHY
7. HONEY COMB APP..
-CYSTOID MACULAR EDEMA
8. TOMATO KETCH UP APP..
- STURGE WEBER SYNDROME
9. CANDLE WAX APP..
- SARCOIDOSIS
10. SALT PEPPER APP..
- CONGENITAL SYPHILIS
-RUBELLA
11.BULL'S EYE MACULOPATHY
-CHLOROQUINE TOXICITY
12. MORNING GLORY APP..
-OPTIC DISC COLOBOMA
13. CATTLE TRACK APP..
- CENTRAL RETINAL ARTERY OCCLUSION

Posner-Schlossman Syndrome

Glaucomatocyclitic crisis is a condition with self-limited recurrent episodes of markedly elevated intraocular pressure (IOP) with mild idiopathic anterior chamber inflammation. It is most often classified as secondary inflammatory glaucoma.

In 1948, Posner and Schlossman first recognized glaucomatocyclitic crisis and described the features of this syndrome.[1] For this reason, the entity is often termed Posner-Schlossman syndrome (PSS).

Posner and Schlossman identified the following features[2] :

Recurrent episodes of mild cyclitis
Uniocular involvement
Duration of attack varying from a few hours to several weeks
Signs of a slight decrease in vision, elevated IOP with open angles, corneal edema with a few keratic precipitates, heterochromia with anisocoria, and a large pupil in the affected eye
Normal visual fields
Normal optic disc
Normal IOP and outflow facility, and all provocative tests normal between episodes

Since this original description, other cases attributed to glaucomatocyclitic crisis have been found to deviate from these criteria.[3] For instance, some patients with glaucomatocyclitic crisis have abnormal aqueous humor dynamics and may have an underlying primary open-angle glaucoma (POAG).[4]

Additional features that are now recognized are as follows:

Almost exclusively, this condition affects individuals aged 20-50 years.
Both eyes may be involved at different times but very rarely contemporaneously.[5, 6]
The rise of IOP is out of proportion to the severity of the uveitis, and this rise in IOP precedes the identifiable inflammatory reaction, often by several days.

Kienbock

Kienbock's disease is a disorder of the wrist. It is named for Dr. Robert Kienböck, a radiologist in Vienna, Austria who described osteomalacia of the lunate in 1910.[1]

It is breakdown of the lunate bone, a carpal bone in the wrist that articulates with the radius in the forearm. Specifically, Kienbock's disease is another name for avascular necrosis[2] (death and fracture of bone tissue due to interruption of blood supply) with fragmentation and collapse of the lunate. This has classically been attributed to arterial disruption, but may also occur after events that produce venous congestion with elevated interosseous pressure.

CONDITIONS SHOWING SOFT TISSUE CALCIFICATION AND/OR OSSIFICATION

Generalized conditions 

1. Metabolic disorders With hypercalcaemia
Hyperparathyroidism
Hypervitaminosis D
Idiopathic hypercalcaemia
Milk-alkali syndrome
Without hypercalcaemia
Chronic renal disease with secondary hyperparathyroidism
Hypoparathyroidism
Pseudo- and pseudopseudohypoparathyroidism
Gout
Ochronosis (alkaptonuria)

2. Vascular disorders Arterial and venous
Connective tissue associated with oedema

3. Infection Bacterial
Miliary tuberculosis
Leprosy (nerves)
Parasitic
Cysticercosis
Guinea worm (dracunculosis) Loa-loa
Armillifer armillatus

4. Connective tissue disorders Congenital
Fibrodysplasia ossificans progressiva
Ehlers–Danlos syndrome
Pseudoxanthoma elasticum
Werner's syndrome
Acquired
Calcium pyrophosphate deposition disease (CPPD)
Hydroxyapatite deposition disease (HADD)
Dermatomyositis
Progressive systemic sclerosis (scleroderma)
CREST syndrome (calcification, Raynaud's phenomenon, oesophageal dysmotility, scleroderma, telangiectasia)

5. Miscellaneous Tumoral calcinosis
Renal osteodystrophy
Idiopathic calcinosis universalis
Sarcoidosis with hypercalcaemia

Localized conditions 

1. Soft tissue necrosis Injection sites
Thermal injuries (burns and frost-bite)

2. Trauma Haematoma/subperiosteal
Haematoma/cephalhaematoma
Myositis ossificans
Neurogenic heterotopic ossification

3. Tumours Benign
Haemangioma (phleboliths)
Lipoma
Soft tissue chondroma
Soft tissue aneurysmal bone cyst

Malignant
Synovial sarcoma
Soft tissue osteosarcoma
Soft tissue chondrosarcoma

Some inportant foramina of skull :

Foramen rotundum : Transmits Maxillary division of trigeminal nerve.
Foramen spinosum : Transmits middle meningeal artery
Foramen Transversarium : Transmits vertebral artery
Foramen ovale: Transmits mandibular nerve
Jugular foramen: Transmits IXth , Xth , spinal part of XIth cranial nerve and internal jugular vein.

Onions in Pathology

1. Onion skin appearance in kidney seen in Hyperplastic Arteriosclerosis
2. Onion skin like lesions due to arteritis seen in Lyme’s and SLE
3. Onion skin fibrosis around bile ducts seen in PSC
4. Onion skin pattern of deposition of reactive bone in Ewing's Sarcoma
5. Onion bulb appearance in sural nerves seen in CIDP due to recurrent demyelination and remyelination

Newer drugs for Crohns disease

"ANTI" "F"or "C"rohn's

Adalimumab--> Ada--limu--mab---> TNF inhibitor
Natalizumab--> Nata--lizu--mab---> humanized monoclonal antibody against the cell adhesion molecule α4-integrin
Toclizumab---> Toc--lizu--mabhumanized monoclonal antibody against the interleukin-6 receptor (IL-6R).
Infliximab---> TNF inhibitor

Fontolizumab---> Fonto--lizu--mab---> a humanised anti-interferon γ antibody

Certolizumab---> Certo--lizu--mab---> an anti-TNF-alpha mAb

WORLD Health Day

WORLD Health Day, which is celebrated every 7th of April since 1948 marks the founding anniversary of the World Health Organization.
Every year, a theme is selected to highlight a priority area of worldwide public health concern.
The 2013 World Health Day theme is controlling BLOOD PRESSURE.

Cause of hemorrhagic metastases in the brain

“MATCH” - hemorrhagic metastates

Melanoma
Anaplastic lung Ca
Thyroid Ca
Choriocarcinoma
Hypernephroma (RCCa)

Molecular sub-types of Breast Cancer

Luminal A - ER, PR positive & HER negative
Luminal B - ER, PR & HER positive
Basal - Triple negative
HER 2 enriched - ER, PR negative. HER positive

MELD Score

The MELD score (Model for End stage Liver disease) is a prospectively derived scoring system designed to predict prognosis of patients with liver disease and portal hypertension. It is calculated using three noninvasive variables—the prothrombin time expressed as international normalized ratio (INR), serum bilirubin, and serum creatinine

Differentiate GRAM Positive Cocci !!

Forensic medicine

Superfoetation-an already pregnent woman gets pregnant.

Superfecundation- fertilization of 2 ova from same cycle by two different but closely related sexual acts.

Suppositous child- mother taking someone else's child as her own.

Posthumous child- child born after the father has died.

Bundle branch block

Meniscal tears

AV block

HOLT ORAM SYNDROME

BACKGROUND: Also called as heart-hand syndrome, is an inherited disorder, first described in 1960 by Holt and Oram in a 4 generation family with ASD and thumb abnormalities
GENETICS: Autosomal dominant inheritance with complete penetrance. Due to mutations in TBX5 on long arm of chromosome 12, which is important in development of heart and hand
Clinical features:
Hand defects: Always present. Includes carpal bone fusion, absent radius, absent or triphalangeal thumb

Cardiac defects: Present in 75% of patients. Includes ASD(mc), VSD, conduction defects, AF, anamolous venous return

PROGNOSIS: Depends on severity of cardiac lesions. In mild lesions near normal life expectancy.

This is an image from a Holt Oram syndrome patient showing hand abnormalities

Clinical Features in Patients with Wilson Disease

Hepatic
·         Asymptomatic hepatomegaly
·         Isolated splenomegaly
·         Persistently elevated serum aminotransferase activity (AST, ALT)
·         Fatty liver
·         Acute hepatitis
·         Resembling autoimmune hepatitis
·         Cirrhosis: compensated or decompensated
·         Acute liver failure

Neurological
·         Movement disorders (tremor, involuntary movements)
·         Drooling, dysarthria
·         Rigid dystonia
·         Pseudobulbar palsy
·         Dysautonomia
·         Migraine headaches
·         Insomnia
·         Seizures

Psychiatric
·         Depression
·         Neurotic behaviours
·         Personality changes
·         Psychosis

Other Systems
·         Ocular: Kayser-Fleischer rings, sunflower cataracts
·         Cutaneous: lunulae ceruleae
·         Renal abnormalities: aminoaciduria and nephrolithiasis
·         Skeletal abnormalities: premature osteoporosis and arthritis
·         Cardiomyopathy, dysrhythmias
·         Pancreatitis
·         Hypoparathyroidism
·         Menstrual irregularities; infertility, repeated miscarriages

Image Courtesy: www.eurowilson.org
Text Reference: http://www.wilsonsdisease.org

DISEASES WITH THEIR ENZYME DEFICIENCIES

Criggler-Najjar - UDP-glucuronyl transferase
Von-Gierke’s - Glucose -6- phosphatase
Pompe’s - Acid maltase( Acid α glucosidase)
Mc Ardle’s - Muscle glycogen phosphorylase
Tarui’s disease - Muscle phosphorylase
Niemann-Pick’s - Sphingomyelinase
Farber’s - Ceramidase
Gaucher’s - β-glucosidase
Krabbe’s - β-galactosidase
Tay-Sach’s - Hexosaminidase-A
Phenylketonuria - Phenylalanine hydroxylase
Alkaptonuria - Homogentisate oxidase
Albinism - Tyrosinase
Fabry’s - α-galactosidase
Lesch-Nyhan syndrome - HGPRT
Sandhoff - Hexosaminidase-β
Xeroderma pigmentosum - DNA exinuclease
Sudden infant death syndrome - Medium chain acyl CoA dehydrogenase
Maple syrup urine - Branched chain α-ketoacid dehydrogenase
Acute Intermittent Porphyria- Uroporphyrinogen-1 synthase
Maple syrup urine disease - Alpha ketoacid decarboxylase
Hereditary fructose intolerance Aldolase b
Fructosuria - Fructokinase b
Galctosemia - Galactose-1-phosphate uridyl transferase