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Introduction: Anaemia is the commonest medical disorder to occur in pregnancy. The incidence varies in different countries and depending on: - State of nutrition. - Parasitic infestation (intestinal worms, malaria). - acterial infection (T. ., !T"). - #enetically transmitted disorders of haemoglo$in synthesis. Definition a) Physiologically, anaemia can $e defined as condition in which the o%ygen-carrying capacity of the $lood is decreased. $) Anaemia in pregnancy can $e defined as a haemoglo$ine level less than &'.() gram *dl or a haematocrit less than +'). The normal female haemoglo$in reference range changes from &+ , &( g *dl, in non pregnant state to &'.( , &- g *dl with pregnancy. Physiological changes in pregnancy(the haemodilution of pregnancy) &. plasma volume increase $y (') (increase is greater in twin). -. red $lood cell mass(. /) increases $y +') (needed increase in iron demands). The smaller increase in . / than plasma volume results in a &() dilution effect on normal haemoglo$in levels. (this e%plain the physiologic anaemia). Classification of anaemias &. nutritional anaemias : involve the haeme part of haemoglo$in molecule and include : a. iron deficiency. $. 0olate deficiency. c.1itamin &- deficiency. - .lac2 of production of $lood , haemopoetic, Aplastic varieties. + .inherited anaemia involve the glo$in portion of haemoglo$in molecule and include haemoglo$inopathies and thalassaemias. 3 .$lood loss , haemorrhagic.
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4ultiple pregnancy. /hronic $lood loss- heavy period, haemorrhoids. d) 7ecreased utili;ation e.g. infection 8!T"9. Pathophysiology: 0ormation of . /s occurs in $one marrow from the erythro$last. <ormally the . /s survive in circulation for &-' days, when the are removed $y the reticuloendothelial cells and $ro2en up into haem and
glo$in. "ron is an essential component of haemoglo$in and respiratory en;ymes. The $ody of the normal adult contains appro%imately(+ , (gm). "ngested iron must $e reduced to ferrous $efore it can $e a$sor$ed and the ma=or part of this a$sorption occurs in the upper small intestine .the normal daily inta2e of iron from the diet is (&( , -'mg ),and of this (& , -mg) is a$sor$ed through the intestine. The intestinal mucosa a$sor$s more in iron deficiency (+ , 3gm per day). "ron is transported from the mucosal cells attached to a protein called transferrin, average plasma iron levels associated with transferrin are $etween &&' - &-(g*&''ml.
promormo$last (iron ? copper) polychromatic normo$last reticulocytes lose their nucleus mature erythro$last
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The normal amount of iron lost daily is a$out &mg, and thus the daily loss well $alanced:
7aily iron inta2e in food &( , -'mg A$sorption !pper small intestine a$sor$ed via transferrin (& , -mg). (+ , 3mg)in iron deficiency. plasma "ron with the transferrin (&&' - &-(g).
7uring pregnancy the total e%tra iron re6uired is appro%imately &3''mg. Balance of iron in pregnancy: 4aternal @$ placenta lood loss at delivery :actation (''mg (''mg -''mg -''mg 1400 Therefore e%tra a$sorption occurs from the diet B &'''mg. still supplements are re6uired. Diagnosis: Symptoms: Symptoms of anemia are due to lac2 of @$, resulting in deficient o%ygen carrying power of $lood. The lac2 of iron resulting in reduced formation of tissue en;ymes leading to deficiency tissue utili;ation of o%ygen. The deficient utili;ation of o%ygen com$ined with deficient o%ygen carrying power of $lood result and will depend on the severity and duration of anaemia will include: C :assitude C 0ati6ue C #riddiness C 0ainting C Parasthesia C Tiredness C Dea2ness C :oss of appetite C 7yspnea C Palpitation 8saving due to cessation fetus A of menstruation (3''mg) 9
Signs: Pallor of the mucous mem$ranes, and palmar creases. Smooth and pale tongue due to atrophy of papillae. "n sever cases dependent oedema. Sign of congestive heart failure may $e present. Eoilonychia (spoon shaped). Investigations: Blood: a) .ed $lood indices include : &- 4icrocytic, @ypochromic. -- :ow mean cell volume (4/1). +- :ow mean corpscular haemoglo$in concentration (4/@/). 3- Anisocytosis, poi2ilocytosis. $) Serum iron studies are of minimal value $ecause the normal change of pregnancy mimic finding in iron deficiency anaemia such as: "ncrease total iron $inding capacity(T" /). 7ecrease serum iron and in-ferritin. c) one marrow: aspiration shows decreased iron stores. Implication of anaemia in pregnancy: Mother: &. 4ore prone to a$ortion and premature la$our. -. :ess li2ely to stand AP@ and PP@ of any degree. +. 4ore prone to infection in the puerperium. 3. A cardiac patient suffers from greatly increased dyspnea.
(. .ecovery of an anaemic patients retarded in the postnatal period. F. Peripartum: - increases ris2 of heamodynamic compromise. - increased li2elyhood of transfusion. Baby: (possi$le) &. :ow $irth weight. -. <eonatal anaemia. +. /ognitive impairment. Management: .outine haemoglo$in chec2s should $e carried out on all pregnant patient at $oo2ing -G, +-, +F wee2s and third day postpartum. Prevention: a. A good $alanced diet $efore and during pregnancy include (liver, meat, peas, eggs, appricots, are good sources of iron). $. Prophylactic oral iron in pregnancy. The normal re6uirements of pregnancy are met with a daily supplement of +''mg elemental iron. Hne ta$let of ferrous sulphate (+-(mg) has (F'mg) elemental iron. "f the patient ta2ing well $alanced diet and her @$ within normal no need for iron supplement. reatment: "f iron deficiency anaemia is diagnosed, the treatment will depend on : &. severity of the anaemia. -. duration of pregnancy. +. causes for iron deficiency.
!"B": 4ild anaemia ---@$ $elow &' g*dl. Severe anaemia ---@$ $elow I g*dl. # oral iron: -any ferrous iron salt are effective : ferrous sulphate (+-(mg) orally, administered three times daily will achieve the ma%imum $one marrow response. 0errous gluconate and ferrous fumorate are alternative forms. Treatment should $e continued for (+ J F months) to restore $one marrow iron stores. ##during treatment ma$e sure that: &. The patient gets them. -. She ta2es them. +. See they are effective $y: o "mprove the symptoms. o "ncrease .eticulocytes account. o .ise the haemoglo$in level. Cparental iron: is indicated only if there is no response to oral iron as in the compliance cannot $e secured: o <ot ta2ing oral iron. o /annot tolerate oral iron $ecause it causes gastric upset. o /annot a$sor$ iron from the gut. There is no difference in the rapidity of the $one marrow response whether the iron is given orally or parentally.
a. %ectofer (iron sorbital) : contains ('mg of elemental iron per ml, and is supplied in -ml ampules to $e given daily ".4. .8=ectofar 3ml (-''gm) will raise the @$ level $y &gm*&''ml., so a course of &'--' in=ections re6uired9. $. Inferon (iron de&tran comple&): &ml B ('mg, elemental iron availa$le as -ml ampules. The total re6uirement of iron is calculated $y formula and given as a whole in slow saline intravenous drip after an initial test dose. !o" of mls" 'f inferon re(uired ) *"+ , -t of patient in lbs" , .b/defecint 0 side effect of inferno: :ocal phle$itis. .igor. Sever allergic reaction can occur during treatment. 1aboratory response parameters include: - "ncrease reticulocytes count is the first indicationK it should $e with in + days (-)). - "ncrease @$ * haematocrit is slower ta2ing &3 , -& days. Blood transfusion: is reserved for @$ 3gm), if there is evidence of cardiac decomposition as a result of anaemia then it may $e necessary to give pac2ed cell ( to reduce the load on the heart). 2olic acid: should $e given to all the cases.
@ereditary spherocytosis. Diagnosis: 7ymptoms: - tiredness. - Dea2ness. - :assitude. 7igns: - Pallor of the mucous mem$ranes. - Smooth tongue, sore and red. - Spleen may $e palpa$le in some cases. Investigations: - . / indices include macrocytic . / with high 4/1 L&''fl, A<7 L4/@/. - Peripheral smear , include hypersegmented neutrophils. - Serum folate levels are low. one marrow , megalo$lastic.
8ffects: &. low $irth weight. -. more prone to miscarriage. +. more prone to a$ruption placenta. 3. neural tu$e defects (if the deficiency was present during early em$ryogenesis).
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Management: a. once folate deficiency is diagnosed, treatment with folic acid is re6uired throughout pregnancy and should $e continued for several wee2s postpartum or until the cessation of lactation. $. 0olic acid (& , + mg daily) produce the ma%imum haematologic response and replace stores. c. "n those who is not respond to oral therapy and in severe cases parentral folic acid is recommended (&'mg daily ".4.). 3esponse to treatment: .eticulocyte count , it should increase within + days. The haemoglo$in * haematocrit increase is slower, ta2ing &' , &3 days. Prophyla&is: #enerally unnecessary in communities with reasona$le mi%ed diets, the normal re6uirements of pregnancy are met with a daily supplement of '.3mg folic acid. CCin high ris2 population routine prophyla%is may $e desira$le these include: &) @igh parity. -) 4ultiple pregnancies. +) Previous @*H folate deficiency. 3) Anticonvulsant therapy. () 4ala$sorption disorders, past or present haemoglo$inopathies.
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F) @istory of complications of pregnancy 2nown to $e associated with a high incidence of folate deficiency. C the recommended dose to prevent neural tu$e defects is 3mg* day starting + months $efore pregnancy and during early em$ryogenesis.
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.aemoglobinopathies
@aemoglo$inopathies are inherited disorders of haemoglo$in synthesis affecting the polypeptide chain of the glo$in fraction.
@ AB @ -, 4 he defect of haemoglobin synthesis may be either: A5 9ualitative: when there is su$stitution of an amino acid in the polypeptide chain, as in @ S (sic2le) @ - 4 SF val, in which glutamic radical normally present at position F is replaced $y valine.
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B5 9uantitative: when there is defective production of one type of polypeptide chain and replacement with another, as in thalassemia e.g. -thalassemia (@ $artMs @ 3)
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"na$ility to synthesi;e chain --- -thalassemia. Diagnosis: "s $y haemoglo$in electrophoresis - The difference in electrical charge resulting from different amino acids in the chain. - @aemoglo$in typing should always $e performed in clinically suspicious circumstances including: &. >thnic origin of 2nown high ris2. -. haematocrit N+'). +. low haemoglo$in concentration and haematocrit in spite of full therapy.
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the essential features for .B7 disease are: &. .educed solu$ility at low o%ygen tension. -. .igid and deformed cells which do not easily pass through the microcirculation. +. .educed erythrocyte life span resulting in anaemia. 3. .eticulocytosis. (. one marrow hyperplasia and siderosis. F. folate deficiency. I. megalo$lastosis erythropoiesis. .omo;ygous sic$le cell disease (s<s): - Symptoms are rarely present at $irth $ut developed when @ 0 (-) is replaced $y @ S (- - S).
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- 7eath $y age of ten years is common. 0or those who survive and get pregnant, the maternal and perinatal mortality and mor$idity is high. Clinical features: &- anaemia: - decrease life span. - folate deficiency. - @ F-Ig*dl. - .eticulocytosis. - one marrow hyperplasia. -- 4ultiple infarcts: H$struction of small $lood vessels in the lungs, $one, and cere$ral circulation. +-"ndulent leg ulcer. Crisis: @aemolytic. Throm$otic. Precipitated $y stress, characteri;ed $y shoc2, prostration, and a$dominal pain. 8ffect of pregnancy: Anaemia , sudden deterioration. /risis , more fre6uent, more sever. Pulmonary complication , infection, infarction.
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/ardiac disease , cardiomyopathy, high cardiac output failure. Hther infections especially !T". Throm$oem$olism. "ncreased fetal wastage from a$ortion, preterm la$our, and growth deficiency. Pre-eclampsia and @>::P syndrome. Management: General: Prophylactic folic acid is indicated in all cases. "ron supplementation in some cases. lood transfusion only in severe anaemia.
0ull anti$iotic therapy in infection. In labour: Avoid hypo%ia, dehydration and acidosis. lood transfusion if @ $elow Fg*dl.
.etero;ygous sic$le cell anaemia (A<7) sic$le cell trait: - Pregnancy is usually well tolerated and clinical manifestations are rare.
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- The management of clinical pro$lems and prevention of crisis is as for homo;ygous disease.
halassemia
Thalassemia are the result of failure of production of either and chains and their replacement with other polypeptide chain. They are inherited and occur in $oth homo;ygotic and hetero;ygotic forms. 5thalassemia: thalassemia are found most commonly in S> Asia and are characteri;ed $y ina$ility to synthesi;e chain. "n the ma=ority of cases the chain are replaced $y chain (@ @ .B 3) or $y chain (@ #.omo;ygous( thalassemia ma=or): - rarely survive. - the fetus affected in utero, polyhydramnios, erythro$lastosis, anaemia, hydrops. - clinical picture of haemolytic disease of the new $orn occur. #.etro;ygous ( Thalassemia minor): varying degrees, with a clinical picture similar to that seen in thalassemia. 5thalassemia: artMs @ 3).
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-thalassemia are found most commonly in those of 4editerranean stoc2 and are characteri;ed $y ina$ility to synthesi;e chain whilst chain synthesis is normal. The chain may $e replaced $y additional chain, o chain (@ A-) and *or persistence of chain @ 0. @ 0 is normally the predominant haemoglo$in type at $irth, thalassemia dose not manifest itself until late in infancy or even in adult life. .omo;ygous ( 5thalassemia ma=or): !sually die in second or third decade and few achieve pregnancy. .etero;ygous ( 5thalassemia minor): Tends to develop mild progressive anaemia during pregnancy. Clinical feature: &. Anaemia. -. Oaundice. +. @epatosplenomegally. 3. Stunting growth. (. 0ailure of development of secondary se%ual characters. F. Amenorrhea. I. "nfertility. 8ffects on pregnancy:
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-thalassemia ma=or is rarely encountered in pregnant women usually there are multiple complication and poor prognosis. -thalassemia minor is often first detected during pregnancy as a result of screening of anaemia refractory to treatment with haematonics. Anaemia may $ecome sever and occasionally result in cardiac failure. reatment: 0olate supplementation. "ron should only $e given if iron deficiency is demonstrated. lood transfusion in severe cases.
@us$andMs haemoglo$in type should $e determined. Patients with thalassemia ma=or are transfusion dependent. 7creening: - haemoglo$in electrophoresis. - 7<A techni6ues.
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