TEMPORAL HEADACHE

JAWCLAUDICATION

BLINDNESS IN THE IPSILATERAL EYE DUE TOOPHTHALMIC ARTERY VASCULITIS

MADE BY BIOPSY, WHICH REVEALSINFLAMED VESSEL WALL WITH GIANT CELLSAND INTIMAL FIBROSIS

CORTICOSTEROIDS

ABSENT UPPER EXTREMITY PULSE

DISCREPANCY IN BLOOD PRESSUREBETWEEN THE ARMS (>10 MM HG)

NIGHT SWEATS

ARTHRITIS

MYALGIAS

SKIN NODULES

VISUAL DEFECTS

STROKE

CORTICOSTEROIDS

“-PRIL”—EG, BENAZEPRIL, CAPTOPRIL, ENALAPRIL,LISINOPRIL, RAMIPRIL

MECHANISM

SIDE EFFECTS

“-SARTAN”—EG, AZILSARTAN,CANDESARTAN, EPROSARTAN, IRBESARTAN,LOSARTAN, VALSARTAN

MECHANISM

SIDE EFFECTS

TO REMEMBER THESEHORMONES, USE THEMNEMONIC "MY FLAT PIG"

TO REMEMBER WHICH TYPEOF HORMONE-SECRETINGCELLS FALL INTO WHICHCATEGORY, USE THEMNEMONICS “GPA” AND “BFLAT”:

EVENTUALLY, THE TARGETGLAND RESPONSE IS LIMITEDBY ITS HORMONAL PRODUCTEXERTING NEGATIVEFEEDBACK INHIBITION AT THELEVEL OF THE PITUITARYGLAND OR HYPOTHALAMUS

SIGNS AND SYMPTOMS OFPROLACTINOMA INCLUDE:

ENLARGEMENT OF THEPITUITARY GLAND MAY ALSOLEAD TO COMPRESSION OFTHE OPTIC CHIASM

A PROLACTINOMA MAY HAVEA MASS EFFECT ON THESURROUNDING PITUITARYGLAND, CAUSING GENERALHYPOPITUITARY SYMPTOMS

PROLACTIN INCREASES DOPAMINESECRETION FROMHYPOTHALAMUS

PROLACTIN ALSO INHIBITS GNRH

BECAUSE OF THEPHYSIOLOGICAL ACTION OFDOPAMINE (SUPPRESSINGPROLACTIN), DOPAMINEANTAGONISTS (IE,ANTIPSYCHOTICS) CANCAUSE GALACTORRHEAFROM THIS LOSS OFINHIBITION

THE TREATMENT FORPROLACTINOMA INCLUDESBROMOCRIPTINE ORCABERGOLINE, BOTH OFWHICH ARE DOPAMINEAGONISTS

TRANSSPHENOIDAL SURGICALRESECTION MAY BEINDICATED FOR LARGETUMORS

IF THE EXCESSIVE GHSECRETION OCCURS DURINGCHILDHOOD, PRIOR TOSKELETAL EPIPHYSEALCLOSURE, THE DISEASE STATETHAT RESULTS IS GIGANTISM

IF THE EXCESSIVE GHSECRETION OCCURS DURINGADULTHOOD, AFTEREPIPHYSEAL CLOSURE, THEDISEASE STATE THAT RESULTSIS ACROMEGALY

​GIGANTISM IS CHARACTERIZED BY DISPROPORTIONATELY LONGLIMBS (INCREASED LINEAR BONE GROWTH), WHEREASACROMEGALY PRESENTS AS CONSPICUOUS GROWTH IN THE SKINAND SOFT TISSUES, VISCERA, AND BONES OF THE FACE, HANDS,AND FEET

SIGNS AND SYMPTOMS OFACROMEGALY INCLUDE:

A GROWTH HORMONESUPPRESSION TEST MAY BEPERFORMED TO DETERMINEWHETHER GH PRODUCTION ISSUPPRESSED BY HIGH BLOODSUGAR (INDUCED BYDRINKING A GLUCOSESOLUTION)

SURGERY OR RADIATION

OCTREOTIDE, ASOMATOSTATIN ANALOGUETHAT INHIBITS GH RELEASEFROM THE ANTERIORPITUITARY AND

PEGVISOMANT, A GHRECEPTOR ANTAGONISTWHICH EFFECTIVELY BLOCKSIGF-1 PRODUCTION

THE MAJORITY OF CASES OFHYPOPITUITARISM ARESECONDARY TO DESTRUCTIVEPROCESSES DIRECTLYINVOLVING THE ANTERIORPITUITARY. THIS INCLUDES:

FOR EXAMPLE, A DEFICIENCYOF MSH (SYNTHESIZED FROMA COMMON PRECURSORWITH ACTH) CAN MANIFESTAS PALLOR, DUE TO MSH'SSTIMULATORY EFFECTS ONMELANOCYTES

CORTICOSTEROIDS,

T4 (THYROXINE),

SEX STEROIDS, AND

HUMAN GROWTH HORMONE

HEPATIC GLUCOSE OUTPUT

PROTEIN METABOLISM

FAT METABOLISM

PERIPHERAL LIPOPROTEINMETABOLISM

THE DETRIMENTAL EFFECTSOF AGES INCLUDE:

DIABETIC RETINOPATHY CAN RESULT FROM OSMOTIC DAMAGE TO PERICYTES.SUBSEQUENT MICROANEURYSMS MAY OCCUR IN RETINAL VESSEL,EVENTUALLY LEADING TO RETINAL MICROHEMORRHAGES, RETINAL EXUDATESAND MACULAR EDEMA. OSMOTIC DAMAGE TO THE LENS INCREASES THERISK OF CATARACTS. DIABETICS ARE ALSO AT A HIGHER RISK FORGLAUCOMA

DIABETIC NEUROPATHY IS THE RESULT OF OSMOTIC DAMAGE TO SCHWANN CELLS,WHICH CAN LEAD TO DEMYELINATION AND SENSORIMOTOR PERIPHERAL NEUROPATHY. THE PERIPHERAL NEUROPATHY IS MOST COMMONLY A “STOCKING-GLOVE” DISTALSYMMETRIC POLYNEUROPATHY. THIS LOSS OF SENSATION IN THE DISTAL EXTREMITIESCAN LEAD TO NEUROPATHIC PRESSURE ULCERS, MOST COMMONLY IN THE FEET(DIABETIC FOOT ULCERS)

SYMPTOMS GENERALLYAPPEAR AT GLUCOSE < 70MG/DL

THE SYMPTOMS OF HYPOGLYCEMIA CANREMEMBERED WITH THE MNEMONIC STABAT DIABETES:

1) A FASTING SERUM GLUCOSE LEVEL ≥ 126 MG/DL (7.0MMOL/L) THAT IS MEASURED ON TWO SEPARATEOCCASIONS, IS DIAGNOSTIC FOR DM. FASTING IS DEFINEDAS 8 HOURS WITHOUT CALORIC INTAKE

2) AN ORAL GLUCOSE TOLERANCE TEST MEASURESPOSTPRANDIAL PLASMA GLUCOSE LEVELS, 2 HOURSFOLLOWING A 75-GRAM GLUCOSE BOLUS. PLASMAGLUCOSE LEVELS ≥ 200 MG/DL (11.1 MMOL/L) AREDIAGNOSTIC FOR DM

3) HEMOGLOBIN A1C (HBA1C) IS A MEASURE OF NON-ENZYMATICGLYCATION THAT OCCURS ON THE BETA CHAIN OF THE HEMOGLOBINMOLECULE UPON EXPOSURE TO GLUCOSE IN THE PLASMA. HBA1C ≥ 6.5%IS PART OF THE DIAGNOSTIC CRITERIA FOR DM. THE GOAL FOR LONGTERM MANAGEMENT OF DIABETES IS TO MAINTAIN HBA1C LEVELSBETWEEN 6-7%

IN ADDITION TO THE ABOVE TESTS, IF A RANDOM PLASMAGLUCOSE (RPG) TEST MEASURES 200 MG/DL OR HIGHER, ANDTHE PATIENT IS SHOWING SYMPTOMS OF DIABETES(POLYPHAGIA, POLYDIPSIA, POLYURIA), DIABETES MAY BEDIAGNOSED

T1DM TYPICALLY HAS AN ONSET INCHILDHOOD OR ADOLESCENCE, COMPARED TOT2DM WHICH COMMONLY PRESENTS INADULTHOOD

INSULIN IS THE ESSENTIAL TREATMENT; ORALHYPOGLYCEMICS WILL NOT WORK SINCETHEY REQUIRE FUNCTIONAL BETA ISLETCELLS

GENETIC PREDISPOSITION FOR T1DM ISWEAK, HOWEVER THERE IS ANASSOCIATION WITH HLA-DR3 AND 4

HISTOLOGICALLY, AN ISLET LEUKOCYTEINFILTRATE IS SEEN IN T1DM (BUT NOT INT2DM)

SINCE INSULIN IS A PEPTIDE, IT WOULD BE DEGRADED IN THE GI TRACT IFTAKEN ORALLY. THEREFORE, ROUTINE SUBCUTANEOUS ADMINISTRATION BY APUMP, NEEDLE, OR PEN IS RECOMMENDED (INTRAMUSCULAR INJECTIONCARRIES HIGHER RISK). NOTE THAT INSULIN MAY BE GIVEN IV IN A HOSPITALSETTING IF THE PATIENT IS EXPERIENCING A DIABETIC EMERGENCY (EG, DKA ORHHNK)

GLUCOSE LEVELS MUST BE CHECKEDFREQUENTLY TO AVOID HYPOGLYCEMIA, THEMOST COMMON COMPLICATION OF T1DM

INSULIN-INDUCED HYPOGLYCEMIA CAN BETREATED WITH ORAL SUGAR, ORINTRAMUSCULAR GLUCAGON

INSULIN LISPRO

INSULIN ASPART

INSULIN GLULISINE

SHORT-ACTING INSULIN HAS AN ONSET OF 30 AND 60MINUTES. ITS PEAK EFFECT IS 2-4 HOURS AFTERADMINISTRATION, AND IT HAS A DURATION OF 5-8HOURS

NPH HAS AN ONSET OF 1-3 HOURS, AND APEAK EFFECT AT 8 HOURS. INTERMEDIATE-ACTING INSULIN HAS ADURATION OF 12-16 HOURS

LONG-ACTING INSULINS ALL HAVE AN ONSET OF 1 HOUR,AND A DURATION OF 20-26 HOURS (GLARGINE HAS ALONGER DURATION THAN DETEMIR). NOTE THATLONG-ACTING INSULINS ARE "PEAKLESS"

IN A DIABETIC PATIENT, A COMPLETE LACK OF INSULIN PRODUCTION, OR ARELATIVE INSULIN DEFICIENCY LEADS TO THE USE OF FATS AS ASIGNIFICANT ENERGY SOURCE. KETOSIS IS AN INCREASE IN FATTY ACIDMETABOLISM TO PRODUCE KETONE BODIES, NAMELYΒ-HYDROXYBUTYRATE AND ACETOACETATE, AS AN ALTERNATIVE FUELSOURCE

IN DKA, HYPERGLYCEMIA INDUCES PROFOUND OSMOTIC DIURESIS, WHICHCAUSES WATER AND ELECTROLYTE LOSS (ESPECIALLY POTASSIUM). METABOLICACIDOSIS FORCES HYDROGEN IONS INTO CELLS, WHICH DISPLACES K+ IONSTHAT ARE SUBSEQUENTLY LOST IN THE URINE. THE MNEMONIC "REMEMBER THE3 K'S, OR LOSE AOA" CAN BE USED TO REMEMBER THE CLINICAL HALLMARKS OFDKA:

SEVERE COMPLICATIONS OF DKA INCLUDE HEARTFAILURE AND/OR ARRHYTHMIAS, CEREBRAL EDEMA,AND MUCOR SINUSITIS (A FATAL FUNGALINFECTION)

THE TREATMENT OF DKA INCLUDES NORMAL SALINE FLUIDRESUSCITATION IN ORDER TO CORRECT FLUID DEFICIT (REHYDRATION)AND REDUCE PLASMA OSMOLARITY. POTASSIUM AND INSULIN ARE GIVENALONG WITH GLUCOSE (TO PREVENT THE PATIENT FROM BECOMINGHYPOGLYCEMIC)

THE ONSET OF T2DMUSUALLY OCCURS IN ADULTS,AND OFTEN WITH A STRONGFAMILY HISTORY ANDASSOCIATION WITH MORBIDOBESITY. UNLIKE T1DM,THERE IS NO ASSOCIATIONWITH T2DM AND THE HLASYSTEM

T2DM IS CAUSED BY ACOMBINATION OF GENETICAND ENVIRONMENTALFACTORS, AND HAS BEENASSOCIATED WITH POORDIET, LACK OF EXERCISE, ANDOBESITY

SINCE SOME AMOUNT OF INSULIN IS AVAILABLE IN T2DM, KETOACIDOSIS IS UNLIKELY TOOCCUR —TRUE DKA IS AN UNCOMMON (BUT NOT IMPOSSIBLE) COMPLICATION OFUNCONTROLLED T2DM. HOWEVER, UNCONTROLLED TYPE 2 DIABETICS ARE AT RISK OFDEVELOPING HYPEROSMOLAR HYPERGLYCEMIC NONKETOTIC SYNDROME, A CONDITIONSWHICH RESULTS IN HYPEROSMOLAR INTRAVASCULAR DEHYDRATION THAT, IF LEFTUNTREATED, CAN PROGRESS TO HYPERGLYCEMIC DIABETIC COMA

THE MOST COMMONSYMPTOMS OFHYPERGLYCEMIA INCLUDEPOLYDIPSIA AND POLYURIA

HISTOLOGICALLY, ISLETAMYLOID POLYPEPTIDEDEPOSITS ARE SEEN IN T2DM(BUT NOT IN T1DM)

SULFONYLUREAS

THIAZOLIDINEDIONES

Α-GLUCOSIDASE INHIBITORS

BIGUANIDES

MEGLITINIDES

DIPEPTIDYL PEPTIDASE-4INHIBITORS

AMYLIN ANALOGUES

GLUCAGON-LIKEPOLYPEPTIDE-1 ANALOGS

THE LAST THREE CLASSES LISTED (DAG)ARE NEWER CLASSES OF DIABETESMEDICATION

THERE ARE TWO GENERATIONS OF SULFONYLUREAS. FIRST GENERATIONSULFONYLUREAS HAVE LONGER HALF-LIVES AND INCLUDE CHLORPROPAMIDE ANDTOLBUTAMIDE. SECOND GENERATION SULFONYLUREAS ARE MORE POTENT AND HAVESHORTER HALF-LIVES. THEY INCLUDE GLYBURIDE (THE DOSE OF WHICH MUST BEDECREASED WITH RENAL FAILURE) AND GLIPIZIDE (THE DOSE OF WHICH MUST BEDECREASED WITH LIVER FAILURE)

​OVERADMINISTRATION OFSULFONYLUREAS CAN LEAD TOHYPOGLYCEMIA

SIDE EFFECTS OF THIAZOLIDINEDIONES INCLUDEHEPATOTOXICITY AND CARDIOVASCULAR TOXICITY, WITHABSOLUTE CONTRAINDICATION IN PATIENTS WITH LIVERFAILURE OR CHF

COMMONLY USED THIAZOLIDINEDIONEMEDICATIONS INCLUDE ROSIGLITAZONEAND PIOGLITAZONE

THIS CLASS OF DRUGS ISPOORLY TOLERATED BYPATIENTS. IN EFFECT, THEYARE SIMULATING ADISACCHARIDASE DEFICIENCY(E.G. LACTOSEINTOLERANCE), CAUSINGOSMOTIC DIARRHEA ANDPRESENTING MORE SUGARSTO THE COLONIC FLORA. THE COLONIC FLORADIGESTS THESE SUGARS,RELEASING GAS ANDCAUSING FLATULENCE

TWO COMMONLY USED Α-GLUCOSIDASEINHIBITORS ARE ACARBOSE ANDMIGLITOL

SIDE EFFECTS OF METFORMININCLUDE:

METFORMIN IS THE MOSTIMPORTANT EXAMPLE OF ABIGUANIDE, AND IS THE FIRSTLINE AGENT FOR T2DM

THE MAJOR SIDE EFFECT OFMEGLITINIDES ISHYPOGLYCEMIA (JUST LIKESULFONYLUREAS)

TWO IMPORTANTMEGLITINIDES AREREPAGLINIDE ANDNATEGLINIDE

THESE MEDICATIONS HELP REGULATEPOST-PRANDIAL GLUCOSE FLUCTUATIONSAND DECREASE GLUCAGON LEVELS

LIFESTYLE MODIFICATIONSINCLUDE SMOKINGCESSATION, HEALTHY EATINGHABITS AND ADOPTING ANACTIVE LIFESTYLE

ACE INHIBITORS ARE USEDPROPHYLACTICALLY INPATIENTS WITH DIABETICNEPHROPATHY (REGARDLESSOF BLOOD PRESSURE), DUETO THEIR ABILITY TO DILATETHE NEPHRON’S EFFERENTARTERIOLES, WHICHDECREASES FILTRATIONPRESSURE. THIS DECREASESTHE AMOUNT OF PROTEIN INTHE URINE AND PREVENTS ORSLOWS THE PROGRESSIONOF DIABETES-RELATEDKIDNEY DISEASE

BECAUSE OF THEIMMUNOSUPPRESSIVE EFFECTOF DIABETES, VACCINESSUCH AS PNEUMOVAX ANDAN ANNUAL FLU SHOTSHOULD BE ENCOURAGED INDIABETIC PATIENTS

50% OF CARRIERS OF THEGLUCOKINASE GENEMUTATION (WHICHINCREASES THE GLUCOSETHRESHOLD THAT TRIGGERSINSULIN RELEASE) DEVELOPGESTATIONAL DIABETESMELLITUS.

DIETARY MODIFICATIONSAND EXERCISE SHOULD BEENCOURAGED IN PATIENTSWITH GESTATIONALDIABETES. IF LIFESTYLEMODIFICATION EFFORTS FAIL,INSULIN REPLACEMENT MAYBE NECESSARY

TYPE 2 DIABETES MELLITUS (T2DM) IS MANAGEDBY ORAL HYPOGLYCEMIC MEDICATIONS,ALTHOUGH INSULIN MAY BE ADDED TO AREGIMEN

IN A PATIENT WITH A NORMAL PANCREAS, THERE IS ABASAL LEVEL OF INSULIN THAT CONTROLS FASTING BLOODSUGAR, WITH SURGES OF INSULIN AFTER MEALS TOPREVENT POST-PRANDIAL HYPERGLYCEMIA

IN PATIENTS WITH T1DM, A COMBINATION OFLONG-ACTING (BASAL) INSULIN AND SHORTER-ACTING(POST-PRANDIAL) INSULIN IS USED TO MIMIC NORMALPHYSIOLOGY

THEREFORE, ROUTINE SUBCUTANEOUSADMINISTRATION BY A PUMP, NEEDLE, OR PEN ISRECOMMENDED (INTRAMUSCULAR INJECTION CARRIESHIGHER RISK)

NOTE THAT INSULIN MAY BE GIVEN IV IN AHOSPITAL SETTING IF THE PATIENT ISEXPERIENCING A DIABETIC EMERGENCY (EG, DKAOR HHNK)

INSULIN-INDUCEDHYPOGLYCEMIA CAN BETREATED WITH ORAL SUGAR,OR INTRAMUSCULARGLUCAGON

INSULIN LISPRO

INSULIN ASPART

INSULIN GLULISINE

TO REMEMBER THE RAPID-ACTING INSULINS,USE THE MNEMONIC, "THERE'S NO LAGWITH RAPID-ACTING INSULINS"

THE ONSET OF RAPID-ACTINGINSULIN IS 15 MINUTES

SHORT-ACTING INSULIN IS DIFFERENT FROMRAPID-ACTING INSULIN, SUCH ASGLULISINE, LISPRO AND ASPART

SHORT-ACTING INSULIN HASAN ONSET OF 30 AND 60MINUTES

ITS PEAK EFFECT IS 2-4 HOURS AFTERADMINISTRATION, AND IT HAS A DURATIONOF 5-8 HOURS

NPH IS OFTEN COMBINED WITHRAPID-ACTING AND SHORT-ACTING(REGULAR) INSULIN

NPH HAS AN ONSET OF 1-3HOURS, AND A PEAK EFFECTAT 8 HOURS

INTERMEDIATE-ACTINGINSULIN HAS A DURATION OF12-16 HOURS

THESE INSULINS ARE USED TO HELPCONTROL BASAL LEVELS OFGLUCOSE

IN OTHER WORDS, LONG-ACTING INSULINS ENSURE ASMALL AMOUNT OF INSULIN IS IN THE BLOOD STREAM TOASSIST IN THE MOVEMENT OF GLUCOSE INTO CELLSAROUND THE CLOCK

LONG-ACTING INSULINS ALL HAVE AN ONSET OF 1HOUR, AND A DURATION OF 20-26 HOURS(GLARGINE HAS A LONGER DURATION THANDETEMIR)

NOTE THAT LONG-ACTINGINSULINS ARE "PEAKLESS"

SULFONYLUREAS

THIAZOLIDINEDIONES

Α-GLUCOSIDASE INHIBITORS

BIGUANIDES

MEGLITINIDES

DIPEPTIDYL PEPTIDASE-4INHIBITORS

AMYLIN ANALOGUES

GLUCAGON-LIKEPOLYPEPTIDE-1 ANALOGS

SGLT-2 INHIBITORS

THE LAST THREE CLASSES LISTED (DAGS)ARE NEWER CLASSES OF DIABETESMEDICATION

CARCINOID TUMORS ARE COMPRISED OFNEUROENDOCRINE CELLS WHICH SECRETEVASOACTIVE SUBSTANCES INTO SYSTEMICCIRCULATION

CARCINOID SYNDROME IS STRONGLY ASSOCIATED WITHMETASTATIC DISEASE, BECAUSE THE RESPONSIBLENEOPLASTIC GROWTH MUST SECRETE VASOACTIVESUBSTANCES INTO NON-PORTAL VENOUS CIRCULATION

CARCINOID SYNDROME CAN RARELY BE CAUSED BYLUNG CARCINOID TUMORS, ISLET CELLCARCINOMA, AND MEDULLARY THYROIDCARCINOMA

FLUSHING OF THE SKIN

SECRETORY (WATERY,VOLUMINOUS) DIARRHEA,

ABDOMINAL CRAMPS WITHNAUSEA AND VOMITING

WHEEZING, CAUSED BYBRONCHOCONSTRICTION AND/ORBRONCHOSPASM

TRICUSPID INSUFFICIENCY OR PULMONICVALVE STENOSIS (TIPS)

MADE BY INCREASED URINARY SECRETIONOF 5-HYDROXYINDOLEACETIC ACID (5-HIAA),A DEGRADATION PRODUCT OF SEROTONIN

A SOMATOSTATIN ANALOGUE THAT,AMONG OTHER THINGS, INHIBITS THERELEASE OF SEROTONIN

SURGICAL RESECTION AND CHEMOTHERAPYWITH 5-FLUOROURACIL AND DOXORUBICINARE OTHER TREATMENT OPTIONS

GENERALLY, IN ORDER FOR SYMPTOMS TOOCCUR, THE TUMOR MUST METASTASIZE TOTHE LIVER

UNCOMMONLY, PRIMARY TUMORS IN THEBRONCHUS CAN CAUSE SYMPTOMSWITHOUT METS

CARCINOID HEART DISEASE IS CHARACTERIZED BYPATHOGNOMONIC PLAQUE-LIKE DEPOSITS OF FIBROUSTISSUE IN THE VALVES OF THE RIGHT HEART LEADING TOTRICUSPID VALVE REGURGITATION AND PULMONARY VALVESTENOSIS

<80 = MICROCYTIC, >100 =MACROCYTIC. 80-100 =NORMOCYTIC

THALASSEMIAS

ANEMIA OF CHRONICDISEASE

IRON DEFICIENCY ANEMIA

LEAD POISONING

SIDEROBLASTIC ANEMIAS

THE BEST WAY TODIFFERENTIATE BETWEEN THEMICROCYTIC ANEMIAS IS BYSERUM FERRITIN LEVELS.

VITAMIN B12 DEFICIENCY ISMORE CLOSELY ASSOCIATEDWITH NEUROLOGICSEQUELAE THAN FOLATEDEFICIENCY.

HYPERSEGMENTEDNEUTROPHILS ARE SEEN INBOTH VITAMIN B12 ANDFOLATE DEFICIENCY.

NOTABLE LABORATORYFINDINGS IN MACROCYTICANEMIA CAUSED BY VITAMINB12 OR FOLATE DEFICIENCYINCLUDE:

ANEMIA OF CHRONICDISEASE CAN ALSO CAUSE ANORMOCYTIC ANEMIA.

LEUKEMIAS AND APLASTICANEMIA

ENZYME DEFECTS (G6PDDEFICIENCY)

HEMOGLOBINOPATHIES (HBC,SICKLE CELL), LOOK FORTARGET CELLS

↓ SERUM HAPTOGLOBINSUGGESTS INTRAVASCULARHEMOLYSIS (MORE HBRELEASED INTO SERUM)

↓ HAPTOGLOBIN CANSUGGEST LIVER DISEASE(LIVER CANNOT PRODUCENORMAL LEVELS OFHAPTOGLOBIN)

↑ SERUM LDH CAN SUGGESTHEMOLYSIS, BUT THIS ISNONSPECIFIC (NEED MOREEVIDENCE FOR HEMOLYSIS TOMAKE THE DIAGNOSIS)

THE DAT IS PRIMARILY USEDTO DIAGNOSEIMMUNE-MEDIATEDHEMOLYTIC ANEMIA. THE DATDETECTS RED CELLS THATARE BOUND WITHANTIBODIES ORCOMPLEMENT.

THE IAT IS PRIMARILY USED TOSCREEN FOR BLOOD TYPECOMPATIBILITY PRIOR TOTRANSFUSION. THE IATDETECTS UNBOUNDANTIBODIES AGAINSTCERTAIN TYPES OF RBCANTIGENS.

CONSUMPTION OF PLATELETS INTOSMALL VESSEL MICROTHROMBI

MICROANGIOPATHICHEMOLYTIC ANEMIA

THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP) ISCHARACTERIZED BY THROMBOCYTOPENIA ANDMICROANGIOPATHIC HEMOLYTIC ANEMIA WITH THE TRIAD OFFEVER, TRANSIENT NEUROLOGICAL DEFICITS, AND RENALFAILURE

THE HEMOLYTIC-UREMIC SYNDROME (HUS) ISCHARACTERIZED BY THE SIMULTANEOUS OCCURRENCE OFTHROMBOCYTOPENIA, MICROANGIOPATHIC HEMOLYTICANEMIA, AND ACUTE KIDNEY INJURY

FOLLICULAR LYMPHOMA(MOST COMMON INDOLENTNHL)

MANTLE CELL LYMPHOMA

MARGINAL ZONE LYMPHOMA

BURKITT LYMPHOMA

DIFFUSE LARGE B-CELLLYMPHOMA (MOST COMMONNHL OVERALL)

ADULT T-CELLLYMPHOMA/LEUKEMIA

CUTANEOUS T-CELLLYMPHOMAS (I.E. MYCOSISFUNGOIDES)

DECREASED BONE MARROWPRODUCTION

SEQUESTRATION (USUALLY INTHE SPLEEN)

INCREASED PLATELET CONSUMPTIONOR DESTRUCTION

NOTABLE SIGNS AND SYMPTOMS OF ACUTE ITP INCLUDESUDDEN APPEARANCE OF A PETECHIAL RASH, EASY BRUISING, ORBLEEDING IN AN OTHERWISE HEALTHY CHILD

SPLENOMEGALY AND LYMPHADENOPATHYARE NOT PRESENT

NOTABLE LABORATORY FINDINGS INACUTE ITP INCLUDE:

PROTHROMBIN TIME (PT/INR) AND PARTIAL THROMBOPLASTINTIME (PTT) ARE NORMAL

TREATMENT FOR ACUTE ITP IS RARELY GIVEN (IT IS MOSTLYSELF-LIMITING), EXCEPT IN CASES WHERE THROMBOCYTOPENIA ISSEVERE. CONVENTIONAL DRUG TREATMENT FOR ACUTE ITP IS WITHCORTICOSTEROIDS

CHRONIC ITP OCCURS MOSTOFTEN IN ADULT WOMEN

NOTABLE SIGNS AND SYMPTOMS OF CHRONICITP INCLUDE PETECHIAL RASH ORECCHYMOSES, EASY BRUISING, OR BLEEDING

SPLENOMEGALY AND LYMPHADENOPATHY AREUNCOMMON FINDINGS IN THE IDIOPATHIC(PRIMARY FORM) THEIR PRESENCE SUGGESTS ITPSECONDARY TO A B-CELL NEOPLASM

NOTABLE LABORATORY FINDINGSIN CHRONIC ITP INCLUDE:

PROTHROMBIN TIME (PT/INR) AND PARTIALTHROMBOPLASTIN TIME (PTT) ARE NORMAL

CONVENTIONAL TREATMENTS FORCHRONIC ITP INCLUDE:

TTP IS CAUSED BY DEFICIENCY OF ADAMTS13 (VONWILLEBRAND FACTOR METALLOPROTEASE), WHICHNORMALLY DEGRADES VWF MULTIMERS. THIS ISUSUALLY DUE TO DEVELOPMENT OF ANTI-ADAMTS13AUTOANTIBODIES

THE FORMATION OF MICROTHROMBI IN TTP IS DUE TOACCUMULATION OF LARGE, UNCLEAVED VWF MULTIMERSWHICH ARE THOUGHT TO CONTRIBUTE TO EXCESSIVEPLATELET ADHESION AND AGGREGATION

HUS IS CAUSED BY DAMAGE TO ENDOTHELIALCELLS, LEADING TO PLATELET ADHESIONAND MICROTHROMBI FORMATION

HUS OCCURS MOST OFTEN IN CHILDREN, ESPECIALLY FOLLOWINGGASTROINTESTINAL ILLNESS WITH INFLAMMATORY DIARRHEA HUSIS HIGHLY ASSOCIATED WITH GASTROENTERITIS CAUSED BY THESHIGA-LIKE TOXIN OF ESCHERICHIA COLI O157:H7

ATYPICAL HUS IS ASSOCIATED WITHALTERNATIVE COMPLEMENT PATHWAY INHIBITORDEFICIENCIES. DRUG-INDUCED HUS ISASSOCIATED WITH QUININE AND SOMECHEMOTHERAPY AGENTS

NOTABLE SIGNS AND SYMPTOMS OF TTPAND HUS INCLUDE:

NOTABLE LABORATORY FINDINGS INTTP AND HUS INCLUDE:

PROTHROMBIN TIME (PT/INR) AND PARTIALTHROMBOPLASTIN TIME (PTT) ARE NORMAL

TREATMENT FOR TTP AIMS TO REMOVE AUTOANTIBODIESTO ADAMTS13 AND RESUPPLY THE PLASMA WITH DONORADAMTS13

TREATMENT FOR TYPICAL HUS IS USUALLY SUPPORTIVE.TREATMENT FOR ATYPICAL HUS AIMS TO INHIBITCOMPLEMENT FACTOR ACTIVATION

CONVENTIONAL TREATMENTS FOR TTPAND HUS INCLUDE:

CURRENTLY RECOGNIZEDANTIPHOSPHOLIPID ANTIBODIES INCLUDETHE FOLLOWING:

→ HYPERCOAGULABLE STATE

THESE AUTOANTIBODIES CANINTERFERE WITH PLACENTAFORMATION

THE ATOPIC TRIAD IN CHILDREN ISCHARACTERIZED BY

ALLERGIC RHINITIS PRESENTSWITH

HISTORY OF ALLERGIC RHINITIS ISIMPORTANT IN DISTINGUISHING BETWEENATOPIC AND NON-ATOPIC ASTHMA

CHURG-STRAUSS SYNDROME IS A SMALL VESSELNECROTIZING VASCULITIS ASSOCIATED WITH ALLERGICRHINITIS ALONG WITH ASTHMA, LUNG INFILTRATES, ANDPERIPHERAL EOSINOPHILIA

TREATMENT INCLUDES AVOIDING ALLERGIC TRIGGERS,INTRANASAL CORTICOSTEROIDS (MOST EFFECTIVETREATMENT), ANTIHISTAMINES, LEUKOTRIENEINHIBITORS, IPRATROPIUM BROMIDE, AND CROMOLYN

MOST COMMON CAUSE OFINFECTIOUS RHINITIS ISRHINOVIRUS

SYMPTOMS OF INFECTIOUSRHINITIS INCLUDE

TREATMENT OF INFECTIOUS RHINITIS WITHANTIBIOTICS IS NOT INDICATED. TREATMENTIS SUPPORTIVE, HOWEVER COLDPREPARATIONS SHOULD BE AVOIDED INCHILDREN UNDER THE AGE OF 4

CHOANAL ATRESIA AFFECTSFEMALES TWICE AS OFTEN ASMALES

AS A RESULT OF FAILED CANALIZATION,ABNORMAL BONY (90%) OR SOFT TISSUE(MEMBRANOUS TYPE-10%) IS PRESENT

CHOANAL ATRESIA CAN BE UNILATERAL ORBILATERAL, WITH THE BILATERAL TYPE COMMONLYASSOCIATED WITH OTHER CONGENITALABNORMALITIES

A NEWBORN THAT BECOMES CYANOTICDURING BREASTFEEDING THAT RESOLVESAFTER CRYING IS INDICATIVE OF CHOANALATRESIA

ADENOIDS BEGIN DECREASING IN SIZE BY AGE 5-6, ANDOFTEN DISAPPEAR BY THE TEENAGE YEARS. THEYFUNCTION TO TRAP PATHOGENS AND PRODUCEANTIBODIES TO FIGHT INFECTION

ADENOIDITIS IS TYPICALLY VIRAL INNATURE, BUT CAN ALSO BEBACTERIAL

COMMON VIRAL CAUSES OFADENOIDITIS ARE

COMMON BACTERIAL CAUSESOF ADENOIDITIS ARE

PATIENTS WITH ADENOIDITISCAN PRESENT WITH

TREATMENT INVOLVES

OFTEN THE TONSILS AREREMOVED AT THE SAME TIMEFOR RECURRENT INFECTION

ALLERGIC POLYPS ARE THE MOST COMMON TYPE OFNASAL POLYP. DEVELOPMENT OF ALLERGIC POLYPS IS ANIGE MEDIATED PROCESS, AND EOSINOPHILS ARE SEEN ONPERIPHERAL SMEAR

NASAL POLYPS CAN BE SEEN IN PEDIATRIC PATIENTS WITHCYSTIC FIBROSIS (CF). THE THICK SECRETIONS IN CF CAUSEPOLYPS TO FORM. A SWEAT-CHLORIDE TEST IS INDICATED INCHILDREN WITH NASAL POLYPS TO RULE OUT CF

SIXTY TO SEVENTY PERCENT OF ADULTPATIENTS WHO HAVE A HYPERSENSITIVITYTO ASPIRIN HAVE NASAL POLYPS

THE CLASSIC PATIENT FOR ANANGIOFIBROMA IS AN ADOLESCENT MALEWITH RECURRENT NOSEBLEEDS

PATIENTS TYPICALLY PRESENT WITHUNILATERAL NASAL OBSTRUCTION ANDHISTORY OF RECURRENT NOSEBLEEDS

IMAGING SUCH AS CT AND MRI HELP SHOW THEEXTENT OF THE TUMOR. ANGIOGRAPHY HELPSIDENTIFY WHAT VESSELS THE TUMOR ORIGINATEDFROM

TREATMENT INVOLVES HORMONALTHERAPY, RADIATION, AND/OR SURGICALREMOVAL

THIS CONDITION HAS A HIGHPREVALENCE AMONGSTCHINESE PATIENTS, AND ISMOSTLY FOUND IN MALES

THE THREE SUB-TYPES OFNASOPHARYNGEALCARCINOMA ARE

PRESENTING SYMPTOMSDEPEND ON THE LOCATIONOF THE TUMOR, AND MAYINCLUDE

NASOPHARYNGEALCARCINOMA METASTASIZESTO THE CERVICAL LYMPHNODES IN 70% OF CASES

DIAGNOSIS CAN BE BASEDOFF OF

RADIOTHERAPY IS THE TREATMENT OFCHOICE, AND HAS A 5-YEAR SURVIVALRATE OF 50%

TRANSLATES TO → "WHEN 50%HEMOGLOBIN SATURATION OCCURS ATPO2 > 26.7 MMHG"

WITH A "RIGHT SHIFT" → HEMOGLOBIN HASLESS AFFINITY FOR O2 → FACILITATESUNLOADING OF O2 TO TISSUES

RIGHT SHIFT OCCURS WITH:

CAN BE REMEMBERED WITHMNEMONIC: “CADETs faceRIGHT”

LEFT SHIFT → REFLECTS AN INCREASE INHEMOGLOBIN'S AFFINITY FOR O2 →FACILITATES LOADING OF O2 IN THE LUNGS

OCCURS WITH:

LOCATED BILATERALLY IN THE HYPOGASTRIC REGIONS, POSTERIORTO THE BROAD LIGAMENT OF THE UTERUS

CLINICAL CORRELATE:KRUKENBERG TUMORS

THE CORTEX CONTAINS DEVELOPING FOLLICLES, WHICH UPONRUPTURE WILL BECOME THE CORPUS LUTEUM

THE MEDULLA IS COMPOSED OF CONNECTIVE TISSUE THATCONTAINS THE ARTERIAL VASCULATURE AND VENOUS DRAINAGEOF THE OVARY

OVARIAN ARTERIES (GONADAL ARTERIES), WHICH AREBRANCHES OF THE ABDOMINAL AORTA

ALSO COLLATERAL FLOW FROM THEUTERINE ARTERIES, BRANCHES OF THEINTERNAL ILIAC ARTERIES

AN EARLY SJS COMPLICATION ISSECONDARY BACTERIAL INFECTION

LATER DERMATOLOGIC SEQUELAE INCLUDEIRREGULAR PIGMENTATION, ERUPTIVE NEVI,ABNORMAL REGROWTH OF NAILS, ANDALOPECIA

OPHTHALMOLOGIC COMPLICATIONS INCLUDE DRYEYE, PHOTOPHOBIA, VISUAL IMPAIRMENT, ANDCORNEAL SCARRING THAT CAN LEAD TOBLINDNESS

GENITOURINARY COMPLICATIONS INWOMEN INCLUDE LABIAL AND VAGINALABNORMALITIES AND URINARY RETENTION

LONG-TERM PULMONARY COMPLICATIONSINCLUDE CHRONICBRONCHITIS/BRONCHIOLITIS, BRONCHIECTASIS,AND OBSTRUCTIVE DISORDERS

IT MAY PRESENT YEARS AFTER THE PATIENT HAS LEFT THETROPICAL AREA, AND IS THOUGHT TO BE INFECTIOUS ORTOXIC IN ORIGIN

DIARRHEA

STEATORRHEA

WEIGHT LOSS

FAT-SOLUBLE VITAMINDEFICIENCIES

SERUM FINDINGS, WHICH MAY SHOW A LOW-LEVEL OFFAT-SOLUBLE VITAMINS (A, D, E, K), B12, FOLATE, AND LACKOF ANTI-ENDOMYSIAL, ANTI-GLIADIN, AND ANTI-TISSUETRANSGLUTAMINASE ANTIBODIES

STOOL STUDIES, SHOWINGEXCESS FECAL FAT

HISTOLOGIC FINDINGS ON ENDOSCOPY,REVEALING FLATTENING OF VILLI ANDINFLAMMATION IN THE SMALL BOWEL

ANTIBIOTICS (TETRACYCLINE)

VITAMIN SUPPLEMENTATION(INCLUDING FOLATE)

IMPORTANTLY, UNLIKE IN CELIAC SPRUE, PATIENTS WITHTROPICAL SPRUE HAVE NO RESPONSE TO REMOVAL OFGLUTEN FROM THE DIET

(LOW PROTEIN DIET, THOUGH ONCE CONSIDEREDBENEFICIAL, HAS BEEN SHOWN TO BE DETRIMENTAL IN MOSTPATIENTS AS THEY ARE ALREADY MALNOURISHED ANDREQUIRE SUFFICIENT PROTEIN TO MAINTAIN A HEALTHY BODYWEIGHT)

THE DEFECTIVE CFTR CHANNEL IN CF RESULTS INDECREASED CHLORIDE SECRETION AND INCREASEDREABSORPTION OF SODIUM AND WATER BY EPITHELIALCELLS

COMMON INFECTION-CAUSINGPATHOGENS IN THE LUNGS OF CYSTICFIBROSIS PATIENTS INCLUDE:

IN THE GASTROINTESTINAL TRACT, ABNORMAL BILE ANDPANCREATIC SECRETIONS CAUSE MALDIGESTION ANDMALABSORPTION, INCLUDING FAT SOLUBLE VITAMINDEFICIENCIES (A, D, E, AND K)

MORE THAN 95% OF MEN WITH CYSTIC FIBROSIS AREINFERTILE BECAUSE OF PROBLEMS WITH SPERMTRANSPORT, THOUGH THEY ARE ABLE TO PRODUCENORMAL SPERM

IF THE DISEASE PROGRESSES UNTREATED, PATIENTS MAYEXPERIENCE INCREASED COUGH, TACHYPNEA, DYSPNEA,INCREASED SPUTUM PRODUCTION, MALAISE, ANOREXIA,AND WEIGHT LOSS

PATIENTS WITH CYSTIC FIBROSIS WILLHAVE ELEVATED SWEAT CHLORIDE > 60MEQ/L

ABNORMAL SWEAT CHLORIDE TESTING IS SUFFICIENTTO DIAGNOSE CF IN INFANTS AND IS THEDIAGNOSTIC TEST OF CHOICE FOR NON-INFANTPATIENTS

COMMON PULMONARY CYSTIC FIBROSIS COMPLICATIONSARE HEMOPTYSIS (FREQUENT COUGHING ANDINFLAMMATION LEADS TO THE EROSION OF THE WALLS OFBRONCHIAL ARTERIES), BRONCHIECTASIS, ANDPNEUMOTHORAX

PROGRESSIVE OBSTRUCTIVE LUNG DISEASE AND HYPOXIACAN EVENTUALLY LEAD TO CHRONIC PULMONARYHYPERTENSION AND THEN TO RIGHT HEART FAILURE IN CFPATIENTS

PATIENTS MAY ALSO DEVELOPPANCREATITIS OR DIABETES, DUE TOPANCREATIC DAMAGE CAUSED BY THICK,ABNORMAL SECRETION

TIDAL VOLUME

MINUTE VENTILATION

ALVEOLAR AND ARTERIAL PO2

PREECLAMPSIA

SPONTANEOUS ABORTION

INTRAUTERINE FETAL DEMISE

INTRAUTERINE GROWTHRESTRICTION

INCREASED RISK OF PRETERMDELIVERY

BUT SODIUM LEVELS REMAIN CONSTANTBECAUSE OF THE CONCOMITANT INCREASEIN GFR

ESTROGEN

HUMAN CHORIONICGONADOTROPIN

HUMAN PLACENTALLACTOGEN

NOTE: HPL INCREASESINSULIN RESISTANCECONTRIBUTING TOGESTATIONAL DIABETES

THE MORNING SICKNESSTYPICALLY RESOLVES AROUND14-16 WEEKS' GESTATIONBUT REFLUX PERSISTS

THEY ELICIT STRONG CELLULAR ANDANTIBODY RESPONSES AND CAN CONFERLIFELONG IMMUNITY WITH FEW DOSES

THERE IS A REMOTE CHANCE, HOWEVER,THAT THE MICROBE CAN REVERT TO ITSVIRULENT FORM AND CAUSE THE DISEASE

THEY STIMULATE A WEAKER IMMUNERESPONSE, HOWEVER, AND TAKEADDITIONAL DOSES TO MAINTAIN IMMUNITY

THEY ARE NOT AFFECTED BYCIRCULATING ANTIBODIES UNLIKE THELIVE VACCINES

VACCINE

FIRST DOSE OF HEPATITIS BVACCINE

SECOND DOSE OF HEP BVACCINE CAN BE GIVEN AT 1 OR2 MONTHS

FIRST DOSE OF ROTAVIRUS, DTAP, HIB,PNEUMOCOCCAL CONJUGATE (PCV), ANDINACTIVATED POLIOVIRUS (IPV) VACCINES

SECOND DOSE OF ABOVEVACCINES

THIRD DOSE OF ABOVE VACCINES EXCEPT FORHIB AND CAN BEGIN TO GIVE INFLUENZAVACCINE ANNUALLY IF SEASONALLYAPPROPRIATE

FIRST DOSE OF: MMR,VARICELLA, HEPATITIS A

THIRD DOSE OF HIBVACCINE AND FOURTH DOSEOF PCV

FOURTH DOSE OF DTAP VACCINE

SECOND DOSE OF HEPATITIS A IFAT LEAST 6 MONTHS HAVEELAPSED

CERTAIN HIGH RISK CHILDREN SHOULDRECEIVE MENINGOCOCCAL CONJUGATEVACCINE (MCV4), SINGLE DOSE OF PCV ORPPV

FINAL BOOSTER OF DTAP, FOURTH DOSE OFIPV, SECOND DOSE OF MMR, BOOSTER DOSEOF VARICELLA VACCINE

QUADRIVALENT HPVOFFERED TO BOYS 9-18YEARS

HPV VACCINE SHOULD BE GIVEN TO ALLGIRLS IN THREE DOSES, A SINGLE DOSE OFMCV4

MCV4 IF NOT PREVIOUSLY GIVEN (ESPECIALLYCOLLEGE STUDENTS LIVING IN DORMS) ORA BOOSTER IF GIVEN AT AGE 13-15

PREGNANCY

AGE <2 YEARS OR >50 YEARS

WHEEZING IN PAST 12 MO INA 2–4 YR OLD

ASTHMA OR OTHER LUNG DISEASE

ASPIRINTREATMENT

IMMUNOSUPPRESSION (HIV/AIDS,POST-TRANSPLANT, IFN-ALPHA INHIBITORS,ETC)

MID-PREGNANCY GLANDS ARE COMPETENT TOSECRETE MILK (COLOSTRUM) BUT HIGH LEVELSOF PROGESTERONE PREVENT THIS FROMHAPPENING

PROGESTERONE LEVELS FALL AS MILK ISPRODUCED ON DAYS 2-5 AFTER BIRTH

MOST COMMON TIME FOR THE MOTHERTO EXPERIENCE BREAST ENGORGEMENT IFBREASTS ARE NOT DRAINED PROPERLYDURING NURSING OR MECHANICALPUMPING

OTITIS MEDIA

PNEUMONIA

MENINGITIS

BACTEREMIA

DIARRHEA

URINARY TRACT INFECTION

BOTULISM

NECROTIZING ENTEROCOLITIS

IMMUNOGLOBULINS

COMPLEMENT

INTERFERON

LACTOFERRIN

CLINICAL ASTHMA

ATOPIC DERMATITIS

ECZEMA

TYPE I DIABETES

OBESITY

CELIAC DISEASE

INFLAMMATORY BOWEL DISEASE(ULCERATIVE COLITIS AND CROHNS)

CHILDHOOD LEUKEMIA AND LYMPHOMA

DUE TO POOR POSITIONING OF THEINFANT AT THE BREAST AND POORLATCHING AND REMOVAL

ENGORGEMENT IS RELIEVED BY FREQUENTSUCKLING

FEVER

PHARYNGITIS (SORE THROAT)

“STRAWBERRY TONGUE” — MAY ALSO BESEEN IN KAWASAKI DISEASE

CONFLUENT ERYTHEMATOUS SANDPAPER-LIKE (FINE,BLANCHING) RASH BEGINS ON THE CHEST AND NECK→ SPREADS TO REST OF THE BODY:

MACROLIDES AND ORAL FIRST GENERATIONCEPHALOSPORINS ARE ALTERNATIVES IN PENICILLINALLERGIC PATIENTS

ORTOLANI’S TEST

BARLOW’S TEST

TO DO THIS, A PAVLIK HARNESS IS USED TOMAINTAIN HIP FLEXION AND MILDABDUCTION FOR 1-3 MONTHS

FOR MORE SEVERE DDH, ADDUCTOR MUSCLE TENOTOMY(TENDON RELEASE, CUTTING, OR LENGTHENING) MAY BEINDICATED

OFTEN COMBINED WITH CAPSULARRELEASE AND ADDUCTOR MUSCLETENOTOMIES

SOME PATIENTS WITH SEVERED NEGLECTEDDDH MAY REQUIRE PELVIC OSTEOTOMYWHERE AN ACETABULAR SHELF IS CREATED

POLYARTHRITIS ≥ 5 JOINTS

OLIGOARTHRITIS ≤ 4 JOINTS

SYSTEMIC DISEASE: ARTHRITISWITH RASH AND FEVER

THESE ABNORMALITIES LEAD TO AN INFLAMMATORYSYNOVITIS WITH INCREASED BLOOD FLOW AND EDEMA OFTHE SYNOVIAL TISSUE THIS CAUSES PANNUS FORMATIONAND EROSION OF CARTILAGE AND BONE

JOINT PAIN AND SWELLING

JOINT STIFFNESS IN THEMORNING OR AFTER APERIOD OF IMMOBILITY

FATIGUE IN THE EARLYAFTERNOON

ASYMMETRIC LARGE JOINTINVOLVEMENT

HIGHEST INCIDENCE OFUVEITIS

SYMMETRIC ARTHRITIS OFLARGE AND SMALL JOINTS

CAN PRESENT SIMILAR TOADULT RHEUMATOIDARTHRITIS (NODULES,RHEUMATOID FACTOR)

CAN HAVE INVOLVEMENT OFTHE TEMPOROMANDIBULARJOINT, SPINE, AND HIP

ONSET MAY BE ABRUPT ANDHAVE A HIGH FEVER THATSPIKES 1-2X/DAY

SALMON-COLORED MACULARLESIONS MORE COMMON INTHE TRUNK AND PROXIMALEXTREMITIES

HEPATOMEGALY ANDSPLENOMEGALY ARE PRESENT

CAN INCLUDE SIGNS OFPERICARDITIS, MYOCARDITIS

CBC

ESR AND CRP

RHEUMATOID FACTOR

ANA

FIRST LINE FOR CHILDREN WITH OLIGOARTHRITIS LEADINGTO IMPROVEMENT IN INFLAMMATION AND PAIN ANDADJUNCTIVE THERAPY FOR CHILDREN WITH OTHERSUBTYPES

FIRST LINE FOR CHILDREN WITHPOLYARTICULAR DISEASE AND RESISTANTOLIGOARTICULAR DISEASE

USED IF THERE IS FAILURE OFMETHOTREXATE THERAPY

BREASTFEEDING ISPROTECTIVE

THEY ARE RAPID, INEXPENSIVEAND 80-90% SENSITIVE

IT OFTEN INDICATES THE PRESENCE OF ANOUTLIER WITHIN THE RESULTS

HAS THE TAIL TRAILING OFF TO THE RIGHT

MODE < MEDIAN < MEAN

HAS THE TAIL TRAILING OFF TO THE LEFT

MODE > MEDIAN > MEAN

RESPIRATORY TRACT

BACTERIA STICK TO VISCIDMUCOUS AND AREN’T CLEARED

PANCREAS

INTESTINAL TRACT

→ SKIN TASTE SALTY(POSITIVE SWEAT TEST)

→ MALE INFERTILITY

THIS VARIABLE LOSS OF T CELL-MEDIATEDIMMUNITY MAY RESULT IN RECURRENT VIRALAND FUNGAL INFECTIONS

HYPOCALCEMIA MAYPRESENT AS TETANY

INCLUDING

DIGEORGE ANOMALY CRANIOFACIALCHARACTERISTICS CAN RESULT FROM TERATOGEN(E.G. ALCOHOL) EXPOSURE DURING WEEKS 4-6 OFFETAL DEVELOPMENT

FEATURES OF VELOCARDIOFACIALSYNDROME INCLUDE

BECAUSE VELOCARDIOFACIAL SYNDROME REPRESENTS SIGNIFICANTCLINICAL OVERLAP WITH THE CONGENITAL DEFECTS OF DIGEORGESYNDROME, THE TWO ARE CONSIDERED TO FALL ON THE SPECTRUM OFCHROMOSOME 22Q11.2 DELETION SYNDROME, RATHER THAN ASSEPARATE DISORDERS

THE DELETION CAUSING DIGEORGE SYNDROME HASBEEN MAPPED TO LOCATION 11.2 ON THE LONGARM OF CHROMOSOME 22

HYPERACUTE REJECTION CAN BE AVOIDEDBY TRANSPLANTING ONLY TISSUE WHICH ISHLA OR ABO-COMPATIBLE WITH THEPATIENT

CYTOKINES SUCH AS IFN-YFROM ACTIVATED MEMORYCD4 T CELLS ACTIVATEMACROPHAGES AND ATTACKTISSUE AND VESSELS

THIS TYPE OF REJECTION ISIRREVERSIBLE

THE PARENT’S CELLSRECOGNIZE THE CHILD’S HLAAS FOREIGN (BECAUSE OFTHE CONTRIBUTION OF THEOTHER PARENT) → MOUNTAN ATTACK