22 year old female from pietermaritzburg unwell 52 ... · 22 year old female from pietermaritzburg...
TRANSCRIPT
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Miss K.M.
22 year old female from Pietermaritzburg
Unwell 2/52
• Vomiting “everything she ate”
• Cough production of white sputum
• Increasing shortness of breath
• Fever
• Rigors
• Loose stools, occasional blood stained
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Sistemic enquiry
Weight loss ± 4kg
Palpitations
Headaches
Generalised weakness
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Medical history - nil of note
Surgical history - nil of note
No medication
Family history - nil
Social vegetarian for 13 years
not sexually active
No know allergies
No significant travel history
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CLINICAL EXAMINATION
BP = 68/41 Pulse = 100/min, regular reduced volumeTemp = 37ºCPaleJaundicedDehydratedRespiratory system: moderate respiratory distress
Air entry equal bilaterallyLeft basal crepitation
CVS System: JVP elevatedapex: 5i.c.s.3/6 ESMno peripheral oedema
Abdomen: not distendedsoft2cm hepatomegalyno splenomegaly
CNS: CCS 15/15
Orientated but tirespupils n.a.d.no focal signs? Terminaly neck stiffness
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CONCLUSION
Young vegetarian woman, pale jaundiced, dehydrated, dyspnoeicand with symptoms of heart failure.
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HB = 2,4 Retic = 3,4%
HCT = 6,5
MCV = 121,1
WCC = 1,7
PLAT = 12
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AST = 48 PTTpt = 14,5
LDH = 2320 INR = 1,25TbiL = 80DbiL = 14
ALP = 44GGT = 10ALT = 9
TP = 72ALB = 37UIE 131 / 3,3 / 94 /5,1 / 40
Urine dipstick: + urobilinogen
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COBALAMIN
Complex organometallic compound in which a cobalt atom is situated within a corrin ringCannot be synthesized in the human body and must be supplied in the dietThe only dietary source is animal products: meat and dairy foodsMinimum daily requirement is 2,5mg
A normal individual will become deficient in cobalamin (if absorption were to cease abruptly) in about 3 – 6 years
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FOLIC ACID Pteroylmonoglutamic acidSynthesized by many different plants and bacteriaPrimary dietary source: fruits and vegetables
Some form are labile and may be destroyed by cookingMinimum daily requirement is 50 mg, but this may be increased severalfold during periods of enhances metabolic demand such as pregnancy
Normal individuals have about 5 to 20mg folic acid in various body stores, half in the liver
A deficiency of folate will occur within minutes if dietary intake or intestinal absorption is curtailed
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BIOCHEMICAL CONSIDERATIONS
FolatePrime function of this vitamin is to transfer 1-carbon moieties to various organic compounds
purines dTMP (deoxythymidylate monophostate) methionine
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In human there are 2 metabolically active forms of cobalamin
Ø MethylcobalaminØ Adenosylcobalamin
METHYLCOBALAMINEssential cofactor in the conversion of homocysteine tomethionine (refer to figure 108-2)
Folate trap: in the absence of methylcobalamin tissue folatedeficieny develops megalbolistic hematopoiesis.
NB. Tissue folate stores in Cbl deficiency are substantially reduced despite normal or supranormal serum folate levels.
Also large doses of folate can produce a partial hematologic remission in patients with cobalamin deficiency.
v Decreased methionine production (due to impairment n the conversion of homocysteine to methionine) in cobalamin deficiency results in nervous system damanage and is partially responsible for the neurologic complications of this deficiency.
COBALAMIN
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CLASSIFICATION OF THE MEGALOBLASTIC ANEMIAS
COBALAMIN DEFICIENCYI. INADEQUATE INTAKE: VEGETARIANS RARE
II. MALABSORPTIONA) Inadequate production of I.F.
i) Pernicious anemia ii) Gastrectomy iii) Congenital absence of functional abnormality of I.F
B) DISORDERS OF TERMINAL ILEUMi) Tropical Sprueii) Nontropical sprueiii) Regional enteritisiv) Intestinal resectionv) Neoplasms and granulomatous disorders (rare)
vi) Selective cobalamin malabsorptions(rare)C) COMPETITION FOR COBALAMIN
i) Fish tapewormii) Bacteria: “blind loop” syndrome
D) DRUGS: COMPETITION FOR COBALAMIN
III. OTHERA) Nitrous oxideB) Transcobalamin II deficiency (rare)
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DIAGNOSIS
Clinical features involve:
1. The blood: anemia weaknessvertigopalpitations
anginasymptoms of congestive heart failure
2. The gut: sore tongue (smooth and beefy red)Anorexia with weight lossdiarrhee
3. The CNS: peripheral nerves numbness, paresthesiasweakness, ataxia
spinal cord reflexes diminished or increased
cerebrum Romberg and Babinski may be positive
position and vibration senses are diminished
disturbance of mentation: mild irritable dementia or frank psychosis
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LABORATORY
FBC Significant macrocytosis MCV >100Low reticulocyte countleukocyte countplatelet count decreased
Blood smear:
Ø Anisocytosis and poikilocytosisMacroovalocytes (large, oval fully hemoglobinized erythrocytesNeutrophils show hypersegmentation of the nucleus (a single cell with a nucleus of 6 lobes or more!)
Bone marrow- hypercellular- decreased myeloid/erythroid ratio- abundant stainable iron
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Ø Increased unconjugated bilirubinØ Increased LDH in plasmaØ Serum levels of cobalamin and/ or folateØ RBC folate level = index of folate stressØ Schilling test: - to establish the pathogenesis of
cobalamin deficiencyØ Serum methylmalonic acid level
Serum homocysteine level measure tissue vitamin stores
Ø Both are elevated in cobalamin deficiency Only homocysteine is elevated in folate deficiency
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TREATMENT
Intramuscular cyanocobalamin life long.