Pediatric Flashcards

Question

Ttt of acute asthma Home mgx

Answer 1

Written home plan can reduce the death rate by 70% 1-PEFR :- done every 2-3 times per day ( if PEFR is decreased needs medical intervention & if decreased between 50-80% needs change in medical therapy & if below 50% needs acute intervention 2-inhalation therapy by ventolin inhaler up to 3 time /hour( good response characterized by resolution of symptoms within 1 hour , no further symptoms over next 4 hrs and improvement in PEF peak expiratory flow value to at least 80% ). 3-incomplete response ( persistent symptoms , PEF < 80%) :--needs short course of oral cortico steriod like prednisolone 1-2mg/kg/day for 4 days plus inhaled B agonist therapy . . 4- medical attention:-should be looking for or sought for :1- sever exacerbation & persistent respiratory distress , 2- lack of expected response or lack of sustained improvement after initial therapy 3-further deterioration or high risk factors of asthma morbidity or mortality ..

Answer 2

1-correction of hypoxia , 2- rapid improvement of air way obstruction , 3-prevention of progression or recurrence of symptoms . Intervention or treatment depend on clinical severity on arrival , response to initial therapy, and risk factors associated with increased morbidity and mortality . 1-O2 therapy :-to improve oxygenation which given by mask or nasal prongs 2-3/ min.( hypoxia caused by acute episode of asthma and drugs used in treatment like B-agonist , aminophylin ( as result in VPM through pulmonary vasodilator and increased cardiac output . Mist tent should not be used as resulting in irritation leading to coughing and worsening wheezing . . 2-ventolin or albuterol nebulizer 0.15mglkg every 20 minutes for one hour. Inhaled iprotropium bromide ( given if no significant response is seen within first inhaled B agonist & given every 6 hr in a dose o.25 mg for 6 years of age and 0.5 mg for those 12 years of age 3-may need systemic Cortico.Steriod either orally or I.V 4-in sever case , used intra muscular or subcutaneous adrenalin 0.01ml/kg given once or twice at interval 20 minutes to obtain optimum relief ( 0.05 ml is often effectively in infant and young children ) after one hr ---re-asses :1-if sustained improvement of symptoms , normal physical finding & O2 saturation of more than 92% , PEF of more than 70% for 3 hr home discharge . if discharge , on 1-ventolin inhaler for above 5 years or oral salbutamol 2-.3-7 day course of C.S 3- if no response ( persistent symptoms ) ---asses & hospital admission : ---a- if moderate distress needs ward admission b- if sever distress needs I.C.U

Answer 3

1-moderate to sever exacerbation that not improved within 1-2hr from intensive therapy in emergency ward . 2-has risk factors for morbidity & mortality ( risk factors for sever asthma ) :--A- biological – 1-previous sever asthma exacerbation 2-sever air way obstruction 3- rapid attack 4- sever air way hyper-responsiveness 5-poor response to systemic C.S ⑤ Case form) 6-repeated visit to emergency ward in last 48 hrs * leven in mild 7-2 or more hospital admission of the past year or 3-4 emergency visit in last year . . B-economic & psycho-social :-1- poverty 2- crowding 3-mother less than 20 years 4- poor education 5-family dysfunction Ji & 6- psychopath in parent & children C-environment :- 1- allergen exposure 2-smoking 3-air pollution 4-urban environment

Answer 4

A-admission to I.C.U & doing chart for observation for PR,RR, BP & decrease , send for CBP, S.electrolyte , cardiac monitor & blood gas return to infections & Nat(SIADA) ↳ arrhythmia due hypoxia or drugs analysis ( PO2, PCO2, PH 2 hypokalemia ) kt ↑ if there (due to normal when hypoxia subside. -> to 2 BP in normal hypoxia when hypoxia hypoxia Subside - -> decrease intake of It is due monitor to B-start therapy :- aminophylin) 1- O2 therapy to maintain PO2 70-90% or O2 saturation above 92% response to or TX . drugs return to such as nubiulizer or normal when hypoxia Subside z G ① & 2- correction of dehydration: from insensible water loss , decreased intake of fluid & and duiretic effect of aminophylin ( should be given 2/3 of maintainance ) 3- bronch-dilator by a-continuous or frequent Nebulizer with O2 b- aminophylin( 5mg/kg ) or continuous in a dose 0.75-1.25 mglkg /hr ↳ every hrs c- anti muscurine like atropin sulfate should not used as first line but added with B agonist ( 0.05- 0.1mglkg atropin sulfate in a dose of not more than o.25mg ( S.E like mental confusion , tachycardia ) prednisolone rapid action but mineralo corticoid effect - 4-6 . * * - . dexamethasone effect but iprotropium bromide has fewer S.E than atropin best Steroid asthma rapid action meneralo coid effect 4- C.S ( methyl prednisolone in a dose ofcorte 1-2 mg /kg /every 12 hr for 3-7 days meneralocorticoid -> No & slow action use in -> + no . best 11 Mid 5-mechanical ventilation for sever hypoxia o2 saturation of < 90% 6- sedation is Contra indication . 7-chest x-ray should be done in all patients & repeated as indicated to detect complication . Mettyl prednisolone ↳ L increase patencyof hirways 8-other therapy like Mg sulfate in a dose 25-75 mg/kg with maximum dose 2.5 gm , inhaled heliox ( has adjuvent therapy ) ,

Answer 5

severity of asthma Day with symptom night sym FEV1 1-mild intermittent <2episode per wk <2 per month >80% 2-mild persistent 3-6 episode per wk 3-4 per moth >80% 3-moderate persistent daily symptoms >5night per month 60-80% 4-Sever persistent Continuous Sym. Frequent <60%

Answer 6

Mild intermittent :-- 1-no daily medication needed 2-used short acting inhaled B2 agonist ( salbutamol , albuterol ) as needed ( 1-3 puff every 4 hr ) Mild persistent :- one daily medication like ( low dose inhaled C.S 40micro-gram 1-4 puff per day ), intal ( cromolyn) ,nedocromil & leukotrin antagonist like montolukast , zafirlukast Lipo-oxygenase inhibition like zileuton which given above 12 years Moderate persistent:--either:-A-one daily medication by medium dose inhaled C.S ( 80micro-gram 2-4 puff / twice a day Or B-two daily medication like low to medium dose inhaled c.s + long acting bronch-dilator especially for night time symptoms ( salmetrol ) , sustained released theophylin or long acting B2 agonist Sever persistent :--3 daily medication :- 1- high dose inhaled c.s 80 micro gram puff l twice a day + 2-long acting brncho-dilator( salmetrol )or B2 agonist + 3-oral c.s in a dose 1 mg /kg l day of not exceed 60 mg l day then gradually reduce dose to lowest dose which control symptoms 4- correction comborbid condition & emotional disturbances. Exercise induced asthma :--prevented by :-- 1- inhalation adrenerhic drug immediately before exercise like inhaled albuterol which give protection for 4 hr . OR inhaled salmetrol given halve hr before exercise . 2-inhaled cromolyn or nedocromil shortly before exercise

Answer 7

is a helium/ O2 ( 80/20 or 70/30) mixture that may provide dramatic benefit for emergency department patients with severe asthma exacerbation , it is dense as room air and consequently travel more easily down narrow passage , which lead to quickly decreasing the work of breathing ., . The gas mixture is used to drive the nebulizer ,by better delivery of the inhaled bronchodilator . Briefly , heliox driven nebulizer treatment should have the gas set at rate of 8-10 L/min .and with double usual amount of albuterol .

Answer 8

Severity Low Medium Highly 0-4 years 0.25-0.5 mg 0.5 1mg 4-11 years 0.5 mg 1 mg 2 mg

Answer 9

In mild to moderate distress :-- 1- O2 therapy to achieve o2 saturation ≥ 92% 2- nebulized ventoline 0.15 mg/kg/dose for 5-7 minute / every 20 minute for three time with o2 therapy . or used inhaled ventoline, Albuterol or through metered dose inhaler 3- oral systemic cortico steroid if no immediate response or recently taken oral steroid ( oral prednisolone 1-2 mg/kg ). Severe respiratory distress : 1- O2 therapy 2- high dose ventolin or albuterol with ipratropium through inhaler or nebulizer or metered dose Every 20 minute or continuous for one hour with O2 . 3- oral or I.V or I.M corticosteroid 4- chest x ray 5- may need sub cutaneous adrenaline 6- may need adjuvant therapy (Mg sulfate and heliox )

Answer 10

features folate def 1- absorption in small intestine 2- anemia earlier within 4-7 month of age 3- intrinsic factors no needed absorption 4- FTT( failure to thrive) yes B12 deficiency small intestine later 3-4 years needed for yes strict vegetarian 5- risk factors goat milk , chronic hemolysis drugs ( anti convulsant phenytoin , phenobarb primodin , methotrexate ,TM 6-MCV increased increased 7-MCHC normal normal 8- hypersedmented neutrophile yes yes 9- serum folate decreased normal 10- vit B12 level normal decreased 11- platelets &neutrophil decreased decreased in advance case in advance case

Answer 11

Folic acid def.:--- Folic acid absorbed through out small intestine and human can not synthesis folate & depend on dietary sources including 1/3 from green vegetable & fruit , 1/3 from animal organ like liver and kidney and 1/3 from cereals and grain. - Body O store of folate are limited and Anemia occurs after 2—3 month folate free diet of C|F 1- peak age 4—7 month ( is earlier than IDA) 2-may FTT, chronic diarrhea , irritability. 3- hemorrhage may occur in advance age Causes ;--1-inadeguate intake ( poor nutrition , chronic hemolysis , goat milk ) 2-decrease absorption(Anticonvulsant like phenytoin, phenobarbital, primodin ) 3-drug induced folate metabolism(Methotrexate , Tm/SM, pyrimethamine) 4- cong. Abnormality of folate metabolism Lab. Finding :---- 1- macrocytosis( MCVof more than 100 ) . 2-neutropenia & thrombocytopenia . 3– hypersegmented neutrophil 4- S. folate is decresed & LDH is elevated Treatment ;--Folate 0.5 -1 mg \ day for 3-4 wk until definite hematological response has occurred Maintaince therapy 0.2 mg is adequate Vit. B12 def Is derived from cobalamine in food of animal source( which is commonly meat and fish ) secondary to production by micro-organism (human can not synthesis B12 ) , it is in contrast to folate store , older children and adult have sufficient V.B12 store at least 3-5 years unless infant born to mother with low V.B12 store where clinical sign appeared in first 4-5 month of life . Causes :--- 1- inadequate intake as in strict vegetarian 2- lack of intrinsic factor as in pernicious anemia or gastric surgery . 3- impaired absorption as in dis. of terminal illeum , IBD ,over growth of bacteria , duplication of small intestine 4-absence of vit 12 transport protein . C\F:--Non specific manifestation like weakness, FTT, irritability , pallor, glossitis , vomiting , diarrhea, parasthesia, numbness, development delay. Lab. Finding :--Macrocytosis, hypersegmented neutrophil, neutropenia & thrompocytopenia in advance case , s.iron & folic acid are normal, LDH is elevated Treatment :---1mg paranterally ( increase retic count within 2-4 days Treatment of precipitated factors If neurological involvement --- 1mg of B12 I.M for 2 wks then maintained on dose 1-5 microgram\day

Answer 12

Is most common hematological dis. of infancy & childhood , its frequency related to certain basic aspects of iron metabolism , resulting from lack of sufficient iron for synthesis of Hb . New born infants contains 0.5gm of iron, where in adult contains 5gmof iron ( to normal body (make up this discrepancy needed 1 mg daily as 0.8 absorbed need daily and 0.2 mg to replace shedding of blood cell Note : only absorbed 10% of iron therefore needed daily10 mg 0f iron within food . Etiology :--- 1- inadequate iron intake , cow milk intolerance when younger 1 year of age or toddlers when ingest large amount of cow milk 2- blood loss as in peptic ulcer, polyp, IBD, occult blood, milk allergy , meckles diverticulum. 3- defect in iron absorption as in malabsorption syndrome . C\F:---- 1- pallor . 2- pagophagia . 3-if Hb bellow 5gm\dl developed irritability, anorexia , tachycardia & cardiac dilatation . 4- may associated with impaired alertness & learning Note : IDA is rare before age of 6 month in term infant in the absence of blood loss or before doubling birth weight in preterm infant . . . • Lab. Finding 1-decrease s. iron , increase IBC(iron binding capacity), decrease s. ferritin 2- hypochromic microcytic in blood film. 3- WBC is normal , platelets count is increased ( however , platelets may be decreased in sever IDA). DD:--- 1- other causes of hypochromic microcytic 2-anemia of chronic dis. Treatment :--- 1- treatment of ppt. factors of IDA. 2-oral iron therapy ( 4-6mg\kg of elemental iron for 8wk) Response to iron therapy 24-48 hr: mood change( decrease irritability) 36-48 hr:- bone marrow response, erythroid hyperplasia 48-72 hr : reticulocytosis(peaking at 5-7days ) 4-30 :-days increase Hb level 1-3 :-months repletion of stores 3- blood transfusion :-- is indicated :- a- Hb of less than 4 gm/ dl b-superimposed infection which interfere with response . Prevention of IDA :- 1- bottle fed infant should receive iron containing formula untill12 month of age and exclusive breast infant should receive iron after 6 month of age 2- should introduce iron enrich food at 6 month of age followed by transition to limited amount of cow milk and increased solid food at 1 year 3- adolescent female menstruating need heavy diet enrich with iron .

Answer 13

stoss therapy, vitamin D (300,000-600,000 IU) is administered orally (preferred) or intramuscularly as 2-4 doses over 1 day. Since the doses are observed, stoss therapy is ideal in patients in whom adherence to therapy is questionable. The alternative strategy is daily vitamin D with a minimum dose of 2,000 IU/day for a minimum of 3 mo. Either strategy should be followed by daily vitamin D intake of 400 IU/day if <1 yr old or 600 IU/day if >1 yr old. Children who have symptomatic hypocalcemia might need intravenous (IV) calcium acutely, followed by oral calcium supplements, which typically can be tapered over 2-6 wks. in children who receive adequate dietary calcium. Transient use of IV or oral 1,25-D (calcitriol) is often helpful in reversing hypocalcemia in the acute phase by providing active vitamin D during the delay as supplemental vitamin D is converted to active vitamin D. Prevention: Most cases of nutritional rickets can be prevented by universal administration of 400 IU of vitamin D to infants <1 year old. Older children with risk Fe factors for inadequate intake should receive 600 IU/day. Vitamin D may be administered as a component of a multivitamin or as a vitamin D supplement

Answer 14

Type 1 Children with vitamin D–dependent rickets type 1A, an autosomal recessive disorder, have mutations in the gene encoding renal 1α-hydroxylase, preventing conversion of 25-D into 1,25-D. These patients normally present during the 1st 2 yr of life and can have any of the classic features of rickets, including symptomatic hypocalcemia. They have normal levels of 25-D but low levels of 1,25-D. Vitamin D–dependent rickets type 1B is secondary to a mutation in the gene for a 25-hydroxylase. Patients have low levels of 25-D but normal levels of 1,25-D. Treatment Vitamin D–dependent rickets type 1A responds to long-term treatment with 1,25-D (calcitriol), it is important to ensure adequate intake of calcium. Vitamin D–dependent rickets type 1B may respond to pharmacologic doses of vitamin D2 (3,000 U/day) as a result of alternative enzymes with 25hydroxylase activity or residual activity of the mutant protein. Vitamin D–Dependent Rickets, Type 2 Patients with vitamin D–dependent rickets type 2A have mutations in the gene encoding the vitamin D receptor, preventing a normal physiologic response to 1,25-D. Levels of 1,25-D are extremely elevated in this autosomal recessive disorder. Most patients present during infancy, although rickets in less severely affected patients might not be diagnosed until adulthood. Less severe disease is associated with a partially functional vitamin D receptor. Approximately 50–70% of children have alopecia, which tends to be associated with a more severe form of the disease and can range from alopecia areata to alopecia totalis. Epidermal cysts are a less common manifestation. Vitamin D–dependent rickets type 2B appears to result from overexpression of a hormone response element–binding protein that interferes with the actions of 1,25-D. Alopecia may be present. Treatment Some patients respond to extremely high doses of vitamin D2 (25-D or 1,25-D), especially patients without alopecia, All patients should be given a 3-6 mo trial of high-dose vitamin D and oral calcium. Patients who do not respond to high-dose vitamin D may be treated with long-term IV calcium, with possible transition to very high dose oral calcium supplements. Treatment of patients who do not respond to vitamin D is difficult.

Answer 15

Investigations: S ca normal , S pho decrease , PTH normal or increase , ALP increase , 25 vit D normal , 1,25 vit D relatively decreased. Treatment Patients respond well to a combination of oral phosphorus and 1,25-D (calcitriol). The daily need for phosphorus supplementation is 1-3 g of elemental phosphorus divided into 4 or 5 doses.

Answer 16

1- initiate breast-feeding within 1 hr of birth and four hrs. after s.c. and if there is no contraindication for oral feeding we should start breast feeding, to go into rhythmic sucking. 2- Rooming in: that is to put the baby beside his mother Place the newborn and mother skin-to-skin to satisfied her and decrease the worry of the mother. 3- No need extra water unless the weather is hot. Do not give sterile water, glucose, or formula unless indicated 4- The baby must be exclusively breast fed in the first 4 and 6 months of life 5- 85% of the milk will be finish in the first 5 min. of feeding and in the 2 nd five min. the baby finish all milk, slow feeder infant take about 15-20 min. 6- Feeding at night will lead to prolactin secretion and milk supply. 7- Psychological factor: no factor important than happy and relax state of the mother, and the mother should be fully alert when breast feed her baby. 8- The infant should be fed on demand, but infant who cannot be fed on demand he/she should be fed every 3 hrs. on day and every 4 hrs. at night. 9- Bottle feeding will stop breast feeding because it lead to confusion of the baby between the nipple and the teat of the bottle. 10- Mother's diet should be balanced. Criteria of adequacy of breast feeding 1- The baby is calm, happy and sleep well after feeding. 2- Normal bowel motion, no constipation. 3- Normal urine output. 4- Normal weight gain: 20-25 gm/day. Criteria of inadequate breast feeding 1- the baby is crying most of the time. 2- Long meal time (grasp the breast for a long period). 3- Very short sleep. 4- Loss of weight or failure to gain weight. 5- Constipation. 6- oliguria.

Answer 17

Mastitis Mastitis may follow breast engorgement or a condition called a blocked duct. it occurs in 23% of lactating women and is usually unilateral, manifesting with localized warmth, tenderness, edema, and erythema after the 2nd postdelivery week. Sudden onset of breast pain, myalgia, and fever with fatigue, nausea, vomiting, and headache can also occur. Organisms implicated in mastitis include Staphylococcus aureus, Escherichia coli, group A streptococcus, Haemophilus influenzae, Klebsiella pneumoniae, and Bacteroides spp. Diagnosis is confirmed by physical examination. Oral antibiotics and analgesics, while promoting breast-feeding or emptying of the affected breast, usually resolve the infection. A breast abscess is a less-common complication of mastitis, but it is a more serious infection that requires intravenous antibiotics as well as incision and drainage, along with temporary cessation of feeding from that breast. Causes of blocked duct and mastitis: 1- infrequent or short breast feeding 2- poor draining of part or all the breast due to pressure from clothes, pressure from finger during feeds 3- damaged breast tissue (due to trauma) 4- bacteria allowed entry (due to nipple fissure) 4- hare lip. Treatment: Improve draining of breast by treat the cause like remove the narrow clothes, advise frequent feeding, start feeding on an affected side then on affected side, gentle massage toward nipple, warm compress, if no improve use antibiotics like fluxacillin, erythromycin, complete rest. -Nipple Pain Nipple pain is one of the most common complaints of breast-feeding mothers in the immediate postpartum period. Poor infant positioning and improper latch are the most common reasons for nipple pain beyond the mild discomfort felt early in breast-feeding. If the problem persists and the infant refuses to feed, consideration needs to be given to nipple candidiasis, and both mother and baby should be treated if candidiasis is found. In some cases, especially if accompanied by engorgement, it may be necessary to express milk manually until healing has occurred -Nipple fissure and sore nipple: Causes: improper position or attachment (latch on), or due to Candida infection. Treatment: treat the cause like correct position and attachment, treat Candidiasis for baby and mother, wash breast once and avoid use soap, avoid medicated lotion or ointment, rub hind milk on areola after feed.

Answer 18

A-on mother side: 1- mental and neurological disease of the mother. 2- infections like malaria, septicemia, typhoid fever, T.B., viral hepatitis and CMV infection. 3-secretion of toxic drug: like anticoagulant, antithyroid drugs, cytotoxic drugs. 4-chronic debilitatory disease, like H.F., renal failure, un controlled D.M. B-on baby side: 1- inborn error of metabolism, like galactosemia, phanylketonurea. 2- cleft lip and palate.

Answer 19

It is the 1 st milk produced after delivery continue for 3 days. At 3rd -10 th day of infant life it transform into the transitional milk and finally to mature milk, the amount of colostrum is 15-50 ml/day it is bright lemon in color, more alkaline than mature milk and has more specific gravity, also have anti-infective and laxative effect which is benefit to get rid from mecanium. Content of colostrums: more protein, more mineral, less CHO and fat. Protein 2.7 gm/100ml, fat: 2.9 gm/100ml, lactose 5.3gm/100ml, mineral 0.5 gm/100ml Comparison between human milk and cow's milk Material Protein gm/100ml Fat Carbohydrate (lactose) Calorie Mineral Water Human milk 1 (30% casein, 70 % whey) 4 (6o % unsaturated, 40% saturated) 7 67 Kcl/100ml 0.2% Cow's milk 3.3 (80% casein, 20% whey) 4 (40% unsaturated, 60% saturated) 4.5 67 Kcl/100ml 0.8% The same Vitamins: cow's milk is low in vit. C and D, breast milk contain adequate vit. C and D provided that the mother in good diet and exposed to some light, cow's milk contain more vit. K than human milk, both types contain adequate vit. A and B complex. Contraindicated: Probably save but give with caution: Bacterial content: although human milk is essentially uncontaminated by bacteria, pathogenic organisms in significant number may enter the milk from mastitis. Tubercle, typhoid bacilli, herpes, hepatitis B, rubella, mumps, HIV and CMV may be found at times in the milk of women infected by these organisms. Cow's milk however is good culture for pathogenic bacteria, and many infections are milk borne including streptococcal disease, diphtheria, typhoid fever, salmonellosis and brucellosis, in addition to bacteria that cause G.E and diarrhea. Digestibility: The stomach empties more rapidly after human milk than after whole cow's milk. The curd of cow's milk is reduced in size by boiling and makes less tough and much smaller by evaporation or by add acid and alkali.

Answer 20

Contraindicated: Probably save but give with caution: Bacterial content: although human milk is essentially uncontaminated by bacteria, pathogenic organisms in significant number may enter the milk from mastitis. Tubercle, typhoid bacilli, herpes, hepatitis B, rubella, mumps, HIV and CMV may be found at times in the milk of women infected by these organisms. Cow's milk however is good culture for pathogenic bacteria, and many infections are milk borne including streptococcal disease, diphtheria, typhoid fever, salmonellosis and brucellosis, in addition to bacteria that cause G.E and diarrhea. Digestibility: The stomach empties more rapidly after human milk than after whole cow's milk. The curd of cow's milk is reduced in size by boiling and makes less tough and much smaller by evaporation or by add acid and alkali. drugs and breast feeding Antineoplastic, amphetamine, bromocriptine, cimetidine, chloramphenicol, ergots, heroin, immunosuppressant, iodide, meprobamate, nicotin, Tetracycline, methimazol (antithyriod). Avoid or give with great caution: Aspirin (salicylats), atropine, birth contracaptive pill, cascare, calcifirol, metoclopramide, metronidazole, Phenobarbital, primidine. Anesthetic, Acetaminophen, Aldomet, Chloropromazine, Cadine, Digoxin, Hydralazin, Prednisollon, Theophyllin.

Answer 21

dried milk: prepared by evaporating water from milk to dry for each 100cc of liquid milk is transformed into 12.5gm powder (1:8). a- dried whole milk: fat content is 3.5%. b- dried skim milk: non fat skim milk, fat content o.5%, in half skim milk fat content is 1.5% are available for infant with fat intolerance, should not be used in 1 st 2 y. of life, it's high protein and mineral content may cause sever dehydration. Advantages of dried milk: 1- sterile and highly saluted. 2- composition is constant. 3- can be stored for along period. 5- can be modified to different children. 6- less expensive. Modified formula; 1- low lactose milk 2- lactose free milk e.g. isomil for galactosemia 3- hypo allergic milk (isomil) contain no milk protein, replaced by Soya protein completely 4- phenylalanine free milk (lofenalac) for phenylketonuria 5-Low Na milk for congested heart failure

Answer 22

80-120 kcal/kg/24 h. (110 kcal/kg/24h.), each 30 ml = 1oz = 20 kcal Techniques and preparation: Bottle is rounded not angled, teat is suitable in size, material and flow Sterilization: boiling 5-10 min., or with Na hypochlorid tablet. How to reconstitue.? Ratio 1:8 ; one scoop 4gm reconstitute in 30 ml of water. American scoop is 8gm.

Answer 23

1- should start at the age of 4-6m. 2- weaning should be gradual to prevent G.I.T upset, also sudden weaning cause psychological upset. 3- do not start at summer. 4- should not be start at convalescent period of any disease. 5- replacing on meal for milk feed: any new food should be initially offered once a day with small amount (1-2 teaspoonful) new food are generally best accepted if fairly thin or dilute. 6- it is usually wise to offer the same food daily until the baby become accustomed to it, and not introduce new food more after than every 1-2w. e.g. of food item: a- fruit: washed banana is readily digested and enjoyed by most infants b- vegetables: should be freshly cooked, vegetables are usually added to infant's food by 7m. of age c- eggs: the yolk of the eggs is used initially, egg white should be introduced with caution from allergy. Potato start at the 2 nd 6m. of life, rice, bread added of 6-8m. of life, meat can use by about 6m. of age. Citrus food after 1y., fish after 1y., tomato after 1y. food additives: artificial flavors and colors have been associated with respiratory allergies, urticaria and angioderma.

Answer 24

A. A persistent pattern of inattention and/or hyperactivity/impulsivity that interferes with functioning or development B. Several inattentive or hyperactive/impulsive symptoms were present before 12 yr of age. C. Several inattentive or hyperactive/impulsive symptoms are present in 2 or more settings (e.g., at school [or work] or at home) and is documented independently. D. There is clear evidence of clinically significant impairment in social, academic, or occupational functioning. E. Symptoms do not occur exclusively during the course of schizophrenia, or another psychotic disorder, and are not better accounted for by another mental disorder (e.g., mood disorder, anxiety disorder, dissociative disorder, personality disorder, substance intoxication or withdrawal). Inattention: Six (or more) of the following symptoms of inattention have persisted for ≥6 month’s to a degree that is inconsistent with development level and that negatively impacts directly on social and academic/occupational activities: a. Often fails to give close attention to details or makes careless mistakes in schoolwork, at work, or during other activities (e.g., overlooks or misses details, work is inaccurate). b. Often has difficulty sustaining attention in tasks or play activities. c. Often does not seem to listen when spoken to directly. d. Often does not follow through on instructions and fails to finish schoolwork, chores, or duties in the workplace (not due to oppositional behavior or failure to understand instructions). e. Often has difficulty organizing tasks and activities. f. Often avoids, dislikes, or reluctant to engage in tasks that require sustained mental effort (e.g., schoolwork, is homework). g. Often loses things necessary for tasks or activities (e.g., toys, school assignments, pencils, books, tools). h. Is often easily distracted by extraneous stimuli. i. Is often forgetful in daily activities. Hyperactivity/impulsivity: Six (or more) of the following symptoms of inattention have persisted for ≥6 mo to a degree that is inconsistent with development level and that negatively impacts directly on social and academic/occupational activities. a. Often fidgets with hands or feet or squirms in seat. b. Often leaves seat in classroom or in other situations in which remaining seated is expected. c. Often runs about or climbs excessively in situations in which it is inappropriate (in adolescents or adults, may be limited to subjective feelings of restlessness). d. Often has difficulty playing or engaging in leisure activities quietly. e. Is often “on the go” or often acts as if “driven by a motor.” f. Often talks excessively. Impulsivity. g. Often blurts out answers before questions have been completed. h. Often has difficulty awaiting turn. i. Often interrupts or intrudes on others (e.g., butts into conversations or games).

Answer 25

By 5 yr of age, 90–95% of children are nearly completely continent during the day, and 80–85% are continent at night. Nocturnal enuresis refers to the occurrence of involuntary voiding at night after 5 yr. Enuresis may be primary (estimated 75–90% of children with enuresis; nocturnal urinary control never achieved) or secondary (10–25%; the child was dry at night for at least a few months and then enuresis developed). Overall, 75% of children with enuresis are wet only at night, and 25% are incontinent day and night. This distinction is important, because children with both forms are more likely to have an abnormality of the urinary tract. Monosymptomatic enuresis is more common than polysymptomatic enuresis (associated urgency, hesitancy, frequency, daytime incontinence). 1-supportive measures: • reassure the child and parents that the condition is self-limited and to avoid punitive measures that can affect the child’s psychological development adversely. • Fluid intake should be restricted to 2 oz after 6 or 7 PM. The parents should be certain that the child voids at bedtime. • Avoiding extraneous sugar and caffeine after 5 PM also is beneficial. • If the child snores and the adenoids are enlarged, referral to an otolaryngologist should be considered, because adenoidectomy can cure the enuresis in some cases. • motivational therapy and includes a star chart for dry nights. • Conditioning therapy involves use of a loud auditory or vibratory alarm attached to a moisture sensor in the underwear. The alarm activates when voiding occurs and is intended to awaken children and alert them to void. This form of therapy has a reported success of 30–60%. 2-Pharmacologic therapy: • desmopressin acetate, a synthetic analog of antidiuretic hormone that reduces urine production overnight. This medication is FDA-approved in children and is available as a tablet, with a dosage of 0.2-0.6 mg 2 hr before bedtime. and thus is regarded as second line and is not curative. • For therapy-resistant enuresis or children with symptoms of an overactive bladder, anticholinergic therapy is indicated. Oxybutynin 5 mg at bedtime often is prescribed. • A third-line treatment is imipramine, which is a tricyclic antidepressant. This medication has mild anticholinergic and α-adrenergic effects, reduces the urine output slightly, and also might alter the sleep pattern, Side effects include anxiety, insomnia, and dry mouth, and heart rhythm may be affected. In unsuccessful cases, combining therapies often is effective. Alarm therapy plus desmopressin is more successful than either alone. The combination of oxybutynin chloride and desmopressin is more successful than either alone. Desmopressin and imipramine also may be combined. CAUSES Delayed maturation of the cortical mechanisms that allow voluntary control of the micturition reflex Defective sleep arousal Reduced antidiuretic hormone production at night, resulting in an increased urine output (nocturnal polyuria) Genetic factors, with chromosomes 12 and 13q the likely sites of the gene for enuresis Bladder factors (lack of inhibition, reduced capacity, overactive) Constipation Organic factors, such as urinary tract infection, obstructive uropathy, or sickle cell anemia nephropathy Sleep disorders Sleep-disordered breathing secondary to enlarged adenoids Psychological factors more often implicated in secondary enuresis Family history in enuretic children often positive for enuresis Risk increased with developmental delay, attention-deficit/ hyperactivity disorder, autism spectrum disorders

Answer 26

Wilms tumor, also known as nephroblastoma, is the most common primary malignant renal tumor of childhood. It is the second most common malignant abdominal tumor in childhood after neuroblastoma. The most common sites of metastases are the lungs, regional lymph nodes, and liver. Histologically, the classic Wilms tumor is made up of varying proportions of blastemal, stromal, and epithelial cells. EPIDEMIOLOGY Wilms tumor accounts for 6% of pediatric malignancies and >95% of kidney tumors in children. Approximately 75% of the cases occur in children <5 yr old, with a peak incidence at 2-3 yr. It can arise in one or both kidneys; the incidence of bilateral Wilms tumors is 7%. Most cases are sporadic, but approximately 2% of patients have a family history. In 8–10% of patients, Wilms tumor is observed in the context of hemihypertrophy, aniridia, genitourinary anomalies , and a variety of rare syndromes, including Beckwith-Weidemann syndrome (BWS) and Denys Drash syndrome , fanconi syndrome, trisomy 18. CLINICAL PRESENTATION The most common initial clinical presentation for Wilms tumor is the incidental discovery of an asymptomatic abdominal mass by parents while bathing or clothing an affected child or by a physician during a routine physical examination . Hypertension is present in about 25% of patients at presentation and has been attributed to increased renin activity. Abdominal pain (40%), gross painless hematuria (18%), and constitutional symptoms such as fever, anorexia, and weight loss are other findings at diagnosis. Occasionally, rapid abdominal enlargement and anemia occur because of bleeding into the renal parenchyma or pelvis. Wilms tumor thrombus extends into the inferior vena cava (IVC) in 4–10% of patients and rarely into the right atrium; dislodgment of the intravascular tumor may produce a fatal pulmonary embolism. Patients might also have microcytic anemia from iron deficiency or anemia of chronic disease, polycythemia, elevated platelet count, and acquired deficiency of von Willebrand factor or factor VII deficiency. DIAGNOSIS An abdominal mass in a child should be considered malignant until diagnostic imaging, laboratory findings, and pathology can define its true nature. Imaging studies include plain abdominal radiography, abdominal ultrasonography (US), and CT of the abdomen to define the intrarenal origin of the mass and differentiate it from adrenal masses (e.g., neuroblastoma) and other masses in the abdomen. Abdominal US helps differentiate solid from cystic masses. US with Doppler imaging of renal veins and the IVC is a useful first study to identify Wilms tumor, evaluate the collecting system, and demonstrate tumor thrombi in the renal veins and IVC. CT is useful to define the extent of the disease, integrity of the contralateral kidney, and metastasis .MRI may be helpful in defining an extensive tumor thrombus that extends up to the level of the hepatic veins, or even into the right atrium, and to distinguish Wilms tumor from nephrogenic rests. Chest CT is more sensitive than chest radiography to screen for pulmonary metastasis and is preferably performed before surgery. Regional spread and metastatic lesions can be visualized on positron emission tomography (PET)/CT scanning. The diagnosis is usually made by imaging studies and confirmed by histology at the time of nephrectomy. Although biopsy is a reliable diagnostic tool, it is discouraged since it results in disease upstaging. TREATMENT Children’s Oncology Group (COG) protocols and the International Society of Pediatric Oncology (SIOP) protocols differ in their initial treatment approach. COG advocates upfront surgery prior to initiating treatment, whereas SIOP recommends preoperative chemotherapy. Each approach has advantages and limitations, but they have similar outcomes. Early surgery provides accurate diagnosis and staging and can facilitate risk-adapted therapy. Preoperative chemotherapy can make surgery easier and reduces the risk of intraoperative tumor rupture and hemorrhage. Surgery entails a radical nephrectomy, with meticulous dissection to avoid rupture of the tumor capsule, and lymph node sampling despite the absence of abnormal nodes on preoperative imaging studies or intraoperative assessment. Partial nephrectomy is performed in patients with bilateral disease or those with unilateral Wilms tumor and predisposing syndrome such as the Denys-Drash and WAGR syndromes, to minimize the risk of future renal failure. PROGNOSIS Despite some adverse risk factors that decrease prognosis (metastases, unfavorable histology, recurrent disease, loss of heterozygosity of both 1p and 16q and gain of 1q), most children with Wilms tumor have a very favorable prognosis. Overall survival of children with Wilms tumor exceeds 90%, with some prognostic factors (low stage, favorable histology, young age, low tumor weight) conferring even better outcomes. Wilms tumor tops the list of common pediatric solid tumors in terms of favorable outcome.

Answer 27

A-new born ( until one month of age ) :- 1-foreign material( meconium and amniotic fluid ) 2-bilateral cho-anal atresia 3-cong. Sub-glottic stenosis 4-micrognathia as in pierr Robin syndrome and Diagorgi syndrome . 5-macroglosia as in hypothyrodism &down syndrome 6-cong. Anomalies of the larynx , pharynx & trachea . 7-vocal cord paralysis 8-laryngospasm ( as in intubation and aspiration ) B-infancy ( 1—12 month of age ) 1-laryngomalacia ( commonest ) 2-sub-glotic stenosis 3-rhinitis 5-tongue tumor & ectopic thyroid 4-vascular ring C-Toddler ( 1—3 years ) 1-viral croup ( commonest ) 2-spasmodic croup 3-F.B inhalation. 4-bacteria trachitis 5-tonsil & adenoid hyperatrophy 6-retropharyngial abscess 7-diphteria ( rare ) D-above 3 years :- 1-epiglotitis 3-trauma 5-angio-edema 5-IMN ( infec. Mono nucleosis ) 6-anaphylaxasis 7-peritonsilar abscess in adolescent 2-F.B 8-diphtheria ( rare)

Answer 28

CLINICAL MANIFESTATIONS Laryngomalacia accounts for 45% to 75% of congenital laryngeal anomalies in children with stridor. Stridor is inspiratory, low-pitched, and exacerbated by any exertion: crying, agitation, or feeding and quite by sleeping . The stridor is caused, in part, by decreased laryngeal tone leading to supraglottic collapse during inspiration. Symptoms usually appear within the 1st 2 wk and increase in severity for up to 6 mo, although gradual improvement can begin at any time. Gastroesophageal reflux disease, laryngo-pharyngeal reflux disease, and neurologic disease influence the severity of the disease and thereby the clinical course. DIAGNOSIS 1-The diagnosis is made primarily based on symptoms. The diagnosis is confirmed by outpatient flexible laryngoscopy . 2-When the work of breathing is moderate to severe, airway films and chest radiographs are indicated 3-When dysphagia is suspected, a contrast swallow study and/or a fiberoptic endoscopic evaluation of swallowing (FEES) may be considered. 4-Because 15–60% of infants with laryngomalacia have synchronous airway anomalies, complete bronchoscopy is undertaken for patients with moderate to severe obstruction. TREATMENT 1- Expectant observation is suitable for most infants because most symptoms resolve spontaneously as the child and airway grow. 2-Laryngopharyngeal reflux is managed aggressively with antireflux medications, inhibitors (PPIs). Risk:benefit ratio should be assessed in each patient 3-In 15–20% of patients, symptoms are severe enough to cause progressive respiratory distress, cyanosis, failure to thrive, or cor pulmonale. In these patients, surgical intervention via supraglottoplasty is considered. Supraglottoplasty is 90% successful in relieving upper airway obstruction caused by laryngomalacia..

Answer 29

CLINICAL MANIFESTATIONS Congenital subglottic stenosis is the second most common cause of stridor. The subglottis is the narrowest part of the upper airway in a child and is located in the space extending from the undersurface of the true vocal folds to the inferior margin of the cricoid cartilage. Subglottic stenosis is a narrowing of the subglottic larynx and manifests in the infant with respiratory distress and biphasic or primarily inspiratory stridor. Symptoms often occur with a respiratory tract infection as the edema and thickened secretions of a common cold narrow an already compromised airway leading to recurrent or persistent croup-like symptoms. Biphasic or primarily inspiratory stridor is the typical presenting symptom for congenital subglottic stenosis. The edema and thickened secretions of the common cold further narrow an already marginal airway that leads to croup-like symptoms. DIAGNOSIS The diagnosis made by airway radiographs is confirmed by direct laryngoscopy. During diagnostic laryngoscopy the subglottic larynx is visualized directly and sized objectively using endotracheal tubes . The percentage of stenosis is determined by comparing the size of the patients’ larynx to a standard of laryngeal dimensions based on age. Treatment Stenosis >50% is usually symptomatic and often requires treatment. As with all cases of upper airway obstruction, tracheostomy is avoided when possible. Subglottic stenosis is typically measured using the Myer-Cotton system, with grade I through grade IV subglottic stenosis indicating the severity of narrowing. Dilation and endoscopic laser surgery can be attempted in grade I and II, although they may not be effective because most congenital stenoses are cartilaginous. Anterior cricoid split or laryngotracheal reconstruction with cartilage graft augmentation is typically used in grade III and IV subglottic stenosis.

Answer 30

EPIDEMIOLOGY AND ETIOLOGY Choking is a leading cause of morbidity and mortality among children, especially those younger than 4 yr of age. Most victims of foreign body aspiration are older infants and toddlers ; males have been found to be victims up to 1.7 times more likely than females. Studies show that children younger than 4 yr of age account for 61.7–70% of airway foreign body cases. The most common objects on which children choke are food items (59.5–81% of all choking cases). Nuts, seeds most frequently aspirated food items. Common inorganic objects on which children choke include coins, latex balloons, pins, jewelry, magnets, pen caps, and toys. Globular, compressible, or round objects such as hot dogs, grapes, nuts, balloons, marshmallows, meats, and candies are particularly hazardous due to their ability to completely occlude the airway. Why children more at risk to aspirate a foreign body? 1-because of their developmental vulnerabilities and their underdeveloped ability to swallow food 2- Infants and toddlers often use their mouths to explore their surroundings, and 3-children generally are more likely to be distracted, playing, or ambulatory while eating. >>>Young children are more likely to experience significant blockage by small foreign bodies due to their smaller airway diameter. Mucus and secretions may form a seal around the foreign body, making it more difficult to dislodge by forced air. In addition, the force of air generated by an infant or young child’s cough is less effective in dislodging an airway obstruction. It is recommended that children younger than 5 yr of age should avoid hard candy and chewing gum and that raw fruits and vegetables be cut into small pieces. Other factors, such as developmental delays or disorders causing neurologic or muscular issues, can also put children at higher risk for foreign body aspiration. CLINICAL MANIFESTATIONS depending on the characteristics, duration, and location of the foreign body. The clinical manifestations range from an asymptomatic state to severe respiratory distress. The most serious complication of foreign body aspiration is complete obstruction of the airway, which may be recognized in the conscious child as sudden respiratory distress followed by an inability to speak or cough. There are typically three stages of symptoms that result from aspiration of an object into the airway: 1. Initial event: Paroxysms of coughing, choking, gagging, and possibly airway obstruction occur immediately after aspiration of the foreign body. The child is sometimes able to expel the foreign body during this stage. 2. Asymptomatic interval: The foreign body becomes lodged, reflexes fatigue, and the immediate irritating symptoms subside. The lack of symptoms can be particularly misleading to the provider when a child presents in this stage and accounts for a large percentage of delayed diagnoses and overlooked foreign bodies. A large meta-analysis of more than 30,000 patients showed that diagnosis is delayed more than 25 hr in almost 40% of airway foreign body cases. 3. Complications: Obstruction, erosion, or infection develops, which again directs attention to the presence of a foreign body. In this third stage, complications include fever, cough, hemoptysis, pneumonia, and atelectasis. Acute or chronic complications have been reported in almost 15% of cases of foreign bodies of the airway. DIAGNOSIS History is the most important factor in determining the need for bronchoscopy. A positive history must never be ignored, but a negative history can be misleading. Because nuts and seeds are the most common bronchial foreign bodies, the physician should specifically question the child’s parents about these items, and bronchoscopy essential, including examination of the nose, oral cavity, pharynx, neck, wheezing and asymmetric breath sounds are highly suggestive of foreign body in the airway. In addition to history and physical examination, radiology studies have an important role in diagnosing foreign bodies in the airway. Plain films are typically recommended first, although many foreign bodies are radiolucent (80–96%), and therefore providers often must rely on secondary findings (such as air trapping, asymmetric hyperinflation, obstructive emphysema, atelectasis, mediastinal shift, and consolidation) to indicate suspicion of a foreign body. Expiratory or lateral decubitus films can assist in revealing these suggestive secondary findings. The indication for computed tomography of the chest is currently being explored due to its high sensitivity and specificity, its ability to detect radiolucent objects, and its potential to eliminate the need for an anesthesia and procedure. However, the known risks of radiation must certainly be considered. If there is a high index of suspicion despite negative or inconclusive imaging, bronchoscopy should be performed. TREATMENT A conscious child suspected of having a partial foreign body obstruction should be permitted to cough spontaneously until coughing is no longer effective, respiratory distress and stridor increase, or the child becomes unconscious. If the child becomes unconscious, the child should be gently placed on the ground, supine. The provider should then open the airway with the head-tilt/chin-lift maneuver and attempt mouth-to-mouth ventilation. If ventilation is unsuccessful, the airway is repositioned and ventilation attempted again. If there is still no chest rise, attempts to remove a foreign body are indicated. In an infant <1 yr old, a combination of 5 back blows and 5 chest thrusts is administered . After each cycle of back blows and chest thrusts, the child’s mouth should be visually inspected for the presence of the foreign body. The treatment of choice for airway foreign bodies is prompt endoscopic removal with rigid instruments by a specialist (otolaryngologist or pulmonologist). Bronchoscopy is deferred only until providers have obtained preoperative studies and the patient has been prepared by adequate hydration and emptying of the stomach. Airway foreign bodies are usually removed the same day the diagnosis is first considered. As with any treatment modality, providers must give careful consideration to the risks and benefits of the bronchoscopy procedure when diagnosis is unclear. Potential complications of rigid bronchoscopy;- include bronchospasm, desaturation, bleeding, and airway edema, in addition to the inherent risks of anesthesia.

Answer 31

Pneumothorax is the accumulation of extrapulmonary air within the chest, most commonly from leakage of air from within the lung. Air leaks can be primary or secondary and can be spontaneous, traumatic, iatrogenic, or catamenial. ETIOLOGY AND EPIDEMIOLOGY A primary spontaneous pneumothorax 1-Spontaneous pneumothorax with or without exertion occurs occasionally in teenagers and young adults, most frequently in males who are tall, thin, and thought to have subpleural blebs and Smoking . 2-Individuals with other inherited disorders such as α1-antitrypsin and homocystinuria are also predisposed to pneumothorax. 3-Patients with collagen synthesis defects such as Ehlers-Danlos disease and Marfan syndrome are at increased risk for the development of pneumothorax secondary spontaneous pneumothorax pneumothorax arising as a complication of an underlying lung disorder but without trauma . 1- pneumonia, usually with empyema; In infants with staphylococcal pneumonia, the incidence of pneumothorax is relatively high 2-secondary to pulmonary abscess, gangrene, infarct, rupture of a cyst or an emphysematous bleb (in asthma), or foreign bodies in the lung. 3-It can be found in children hospitalized with asthma exacerbations, and usually resolves without treatment. 4-Pneumothorax is a serious complication in cystic fibrosis. 5-Pneumothorax also occurs in patients with lymphoma or other malignancies, and in graft-versus-host disease with bronchiolitis obliterans Traumatic Non iatrogenic pneumothorax .. External chest or abdominal blunt or penetrating trauma can tear a bronchus or abdominal viscus, with leakage of air into the pleural space. iatrogenic pneumothorax .. can complicate transthoracic needle aspiration, tracheotomy, subclavian line placement, thoracentesis, or trans-bronchial biopsy. It may occur during mechanical or noninvasive ventilation, high-flow nasal cannula therapy, acupuncture, and other diagnostic or therapeutic procedures. Catamenial pneumothorax, an unusual condition that is related to menses, is associated with diaphragmatic defects and pleural blebs. Pneumothorax can be associated with a serous effusion (hydropneu-mothorax), a purulent effusion (pyopneumothorax), or blood (hemo-pneumothorax). Bilateral pneumothorax is rare after the neonatal period but has been reported after lung transplantation and with Mycoplasma pneumoniae infection and tuberculosis. CLINICAL MANIFESTATIONS The onset of pneumothorax is usually abrupt, and the severity of symptoms depends on the extent of the lung collapse and on the amount of preexisting lung disease. Pneumothorax may cause dyspnea, chest pain, and cyanosis. When it occurs in infancy, symptoms and physical signs may be difficult to recognize. Moderate pneumothorax may cause little displacement of the intrathoracic organs and few or no symptoms. Usually, there is respiratory distress, with retractions, markedly decreased breath sounds, and a tympanitic percussion note over the involved hemithorax. . DIAGNOSIS The diagnosis of pneumothorax is usually established by radiographic examination. The amount of air outside the lung varies with time. A radiograph that is taken early shows less lung collapse than one taken later if the leak continues. DIFFERENTIAL DIAGNOSIS Pneumothorax must be differentiated from 1-localized or generalized emphysema, an extensive emphysematous bleb, 2-large pulmonary cavities or other cystic formations 3-diaphragmatic hernia 4-compensatory overexpansion with contralateral atelectasis,. In most cases, chest radiography or CT differentiates among these possibilities. In addition, CT may identify underlying pathology such as blebs. Treatment Therapy varies with the extent of the collapse and the nature and severity of the underlying disease. A small or even moderate-sized pneumothorax in an otherwise normal child may resolve without specific treatment, usually within 1 wk. Administering 100% oxygen may hasten resolution, but patients with chronic hypoxemia should be monitored closely during administration of supplemental oxygen. >>chest tube drainage may be necessary If the pneumothorax is recurrent, secondary, or under tension, or there is more than a small collapse,. >>chemical pleurodesis or surgical thoracotomy is often necessary 1-cystic fibrosis frequently recurs. 2-pneumothorax complicating malignancy does not improve rapidly with observation.

Answer 32

1-there is a shift of mediastinal structures away from the side of the air leak. 2-evidence of circulatory compromise. 3-hearing a “hiss” of rapid exit of air under tension with the insertion of the thoracostomy tube.

Answer 33

ETIOLOGY Pneumomediastinum is typically caused by alveolar rupture which can be due to either a spontaneous or traumatic cause. 1-Primary spontaneous pneumomediastinum can be due to increases in intrathoracic pressure (esophageal perforation), weightlifting, and choking events. 2-secondary spontaneous pneumomediastinum in children younger than age 7 years are lower respiratory tract infections , asthma exacerbations, anorexia nervosa, normal menses, and diabetes mellitus with ketoacidosis. 3-Traumatic causes of pneumomediastinum include both iatrogenic (dental extractions, adenotonsillectomy, high flow nasal cannula therapy, esophageal perforation, and inhalation of helium gas), and non-iatrogenic (inhaled foreign body, penetrating chest trauma, and illicit drug use). CLINICAL MANIFESTATIONS Dyspnea and transient stabbing chest pain that may radiate to the neck are the principal features of pneumomediastinum. Other symptoms may be present like, abdominal pain, cough, chest tightness, facial swelling, choking, tachypnea, fever, stridor, and sore throat. Pneumomediastinum is difficult to detect by physical examination alone. Subcutaneous emphysema is present in the majority of patients. Diagnosis Chest radiography reveals mediastinal air, with a more distinct cardiac border than normal. If a pneumomediastinum is clinically suspected, but is not visualized on a chest x-ray, a chest CT can be performed to provide further radiologic evidence. TREATMENT Treatment is directed primarily at the underlying obstructive pulmonary disease or other precipitating condition. Children who have had pneumomediastinum should be screened for asthma. Analgesics are occasionally needed for chest pain. Children can be observed in the emergency room and discharged if stable. They should be cautioned to avoid heavy lifting and the Valsalva maneuver. Hospital admission with supplemental oxygen administration is more common for patients with secondary pneumomediastinum. Rarely, subcutaneous emphysema can cause sufficient tracheal compression to justify tracheotomy; the tracheotomy also decompresses the mediastinum. COMPLICATIONS Pneumomediastinum is rarely a major problem in older children because the mediastinum can be depressurized by escape of air into the neck or abdomen. In the newborn, however, the rate at which air can leave the mediastinum is limited, and pneumomediastinum can lead to dangerous cardiovascular compromise or pneumothorax

Answer 34

Dry pleurisy ETIOLOGY Dry pleurisy, formerly called plastic pleurisy, may be associated with acute bacterial or viral pulmonary infections or may develop during the course of an acute upper respiratory tract illness. The condition is also associated with tuberculosis and autoimmune diseases such as systemic lupus .erythematosus PATHOLOGY AND PATHOGENESIS The process is usually limited to the visceral pleura, with small .amounts of yellow serous ﬂuid and adhesions between the pleural surfaces CLINICAL MANIFESTATIONS T he primary disease often overshadows signs and symptoms of pleurisy. Pain, the principal symptom, is exaggerated by deep breathing, coughing, and straining. Occasionally, pleural pain is described as a dull ache, which is less likely to vary with breathing. The pain is often localized over the chest wall and is referred to the shoulder or the back. Pain with breathing is responsible for grunting and guarding of respirations, and the child often lies on the aﬀected side in an attempt to decrease respiratory excursions. Early in the illness, a leathery, rough, inspiratory and expiratory friction rub may be audible, but it usually .disappears rapidly LABORATORY FINDINGS Dry pleurisy may be detected on radiographs as a diﬀuse haziness at the pleural surface or a dense, sharply demarcated shadow . The latter ﬁnding may be indistinguishable from small amounts of pleural exudate. Chest radiographic ﬁndings may be normal, but ultrasonography or CT .ﬁndings will be positive DIFFERENTIAL DIAGNOSIS Pleurisy must be distinguished from other diseases, such as epidemic pleurodynia, trauma to the rib cage (rib fracture), lesions of the dorsal root ..ganglia, tumors of the spinal cord, herpes zoster, gallbladder disease TREATMENT .Therapy should be aimed at the underlying disease Serofibrinous pleurisy ETIOLOGY Seroﬁbrinous pleurisy is deﬁned by a ﬁbrinous exudate on the pleural surface and an exudative eﬀusion of serous ﬂuid into the pleural cavity. In general, it is associated with infections of the lung or with inﬂammatory conditions of the abdomen or mediastinum; occasionally, it is found with connective tissue diseases such as lupus erythematosus, periarteritis, and rheumatoid arthritis, and it may be seen with primary or ..metastatic neoplasms of the lung, pleura, or mediastinum PATHOGENESIS Normally, approximately 10 mL of ﬂuid is present in the pleural space, but if formation exceeds clearance, ﬂuid accumulates. Pleural inﬂammation increases the permeability of the plural surface, with increased proteinaceous ﬂuid formation; there may also be some obstruction to lymphatic absorption CLINICAL MANIFESTATIONS Because seroﬁbrinous pleurisy is often preceded by the dry type, early signs and symptoms may be those of dry pleurisy. As ﬂuid accumulates, pleuritic pain may disappear. The patient may become asymptomatic if the eﬀusion remains small, or there may be only signs and symptoms of the underlying disease. Large ﬂuid collections can produce cough, dyspnea, .retractions, tachypnea, orthopnea, or cyanosis Physical ﬁndings depend on the amount of eﬀusion. Dullness may be found on percussion. Breath sounds are decreased or absent, and there is a diminution in tactile fremitus, a shift of the mediastinum away from the aﬀected side, and, occasionally, fullness of the intercostal spaces. If the ﬂuid is not loculated, these signs may shift with changes in position. If extensive pneumonia is present, crackles and rhonchi may also be audible. In infants, physical signs are less deﬁnite, and bronchial breathing may be heard instead of decreased breath sounds LABORATORY FINDINGS Radiographic examination shows a generally homogeneous density obliterating the normal markings of the underlying lung. Small eﬀusions may cause obliteration of only the costophrenic or cardiophrenic angles or a widening of the .interlobar septa DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS Thoracentesis should be performed when pleural ﬂuid is present or is suggested, unless the eﬀusion is small, and the patient has a classic-appearing lobar pneumococcal pneumonia. Thoracentesis can diﬀerentiate seroﬁbrinous pleurisy, empyema, hydrothorax, hemothorax, and .chylothorax TREATMENT Therapy should address the underlying disease. If the eﬀusion is less than 10 mm in size on a chest x-ray, then there is no need for drainage. With a large eﬀusion, draining the ﬂuid makes the patient more comfortable. When a diagnostic thoracentesis is performed, as much ﬂuid as possible should be removed for therapeutic purposes. Rapid removal of ≥1 L of pleural ﬂuid may be associated with the development of reexpansion pulmonary edema. If the underlying disease is adequately treated, further drainage is usually unnecessary, but if suﬃcient ﬂuid reaccumulates to cause respiratory .embarrassment, chest tube drainage should be performed In older children with suspected parapneumonic eﬀusion, tube thoracostomy is considered necessary if the pleural ﬂuid pH is <7.20 or the pleural ﬂuid glucose level is <50 mg/ dL. If the ﬂuid is thick, loculated, or clearly purulent, tube drainage with ﬁbrinolytic therapy or less often video-assisted thoracoscopic surgery (VATS) is indicated

Answer 35

TREATMENT OF MALNUTRITION Principles of Treatment the 10 steps of treatment, which are separated into 2 phases, stabilization and rehabilitation. The aim of the stabilization phase is to repair cellular function, correct Πuid and electrolyte imbalance, restore homeostasis, and prevent death from the interlinked triad of hypoglycemia, hypothermia, and infection. The aim of the rehabilitation phase is to restore wasted tissues (i.e., catch-up growth). Pushing ahead too quickly risks inducing the potentially fatal “refeeding syndrome Emergency Treatment Note that treatment of shock in these children is diΜerent (less rapid, smaller volume, diΜerent Πuid) from treatment of shock in well-nourished children, because shock from dehydration and shock from sepsis often coexist and are diΝcult to diΜerentiate on clinical grounds. Thus the physician must be guided by the response to treatment: children with dehydration respond to intravenous (IV) Πuid, whereas those with septic shock will not respond. Since severely malnourished children can quickly succumb to Πuid overload, they must be monitored closely.

Answer 36

Stabilization the therapeutic directives for stabilization steps 1-7. Giving broadspectrum antibiotics and feeding frequent small amounts of F75 (a specially formulated low-lactose milk with 75 kcal and 0.9 g protein per 100 mL to which potassium, magnesium, and micronutrients are added), will reestablish metabolic control, treat edema, and restore appetite. The parenteral route should be avoided; children who lack appetite should be fed by nasogastric tube, because nutrients delivered within the gut lumen help in its repair. Dehydration status is easily misdiagnosed in severely wasted children, because the usual signs (e.g., slow skin pinch, sunken eyes) may be present even without dehydration. Rehydration must therefore be closely monitored for signs of Πuid overload. Serum electrolyte levels can be misleading because of sodium leaking from the blood into cells and potassium leaking out of cells. Keeping the intake of electrolytes and nutrients constant allows systems to stabilize more quickly than adjusting intake in response to laboratory results.

Answer 37

Rehabilitation The signals for entry to the rehabilitation phase are reduced or minimal edema and return of appetite. A controlled transition over 3 days is recommended to prevent refeeding syndrome. After the transition, unlimited amounts should be given of a highenergy, high-protein milk formula such as F100 (100 kcal and 3 g protein per 100 mL), or a ready-to-use therapeutic food (RUTF), or family foods modiΟed to have comparable energy and protein contents.. To make the transition, for 2 days replace F75 with an equal volume of F100, then increase each successive feed by 10 mL until some feed remains uneaten (usually at about 200 mL/kg/day). After this transition, give 150-220 kcal/kg/day and 4-6 g protein/kg/day, and continue to give potassium, magnesium, and micronutrients. Add iron (3 mg/kg/ day). If breastfed, encourage continued breastfeeding. Children with severe malnutrition have developmental delays, so loving care, structured play, and sensory stimulation during and after treatment are essential to aid recovery of brain function

Answer 38

ETIOLOGY AND EPIDEMIOLOGY CD is an immune-mediated systemic disorder elicited by gluten in wheat and related prolamines from rye and barley in genetically susceptible individuals, and is characterized by the presence of a variable combination of gluten-dependent clinical manifestations, CD–speciΟc antibodies, human leukocyte antigen (HLA)-DQ2 or DQ8 haplotypes, and enteropathy. CD–speciΟc antibodies comprise autoantibodies against TG2 including endomysial antibodies (EMAs), and antibodies against deamidated forms of gliadin peptides. CD is a common disorder with about 1% prevalence of biopsy-proven disease. GENETICS AND PATHOGENESIS A genetic predisposition is suggested by the family aggregation and the concordance in monozygotic twins, which approaches 100%. The strongest association is with HLA-DQ2.5 (1 or 2 copies encoded by DQA1 *05 [for the alpha] and DQB1*02 genes [for the beta chain]) CLINICAL PRESENTATION AND ASSOCIATED DISORDERS Clinical features of CD vary considerably. Intestinal symptoms are more common in children whose disease is diagnosed within the ﬁrst 2 yr of life; failure to thrive, chronic diarrhea, vomiting, abdominal distention, muscle wasting, anorexia, and irritability are present in most cases . Occasionally there is constipation, with cases presenting with intussusception. As the age at presentation of the disease shifts to later in childhood, and with the more extensive use of serologic screening tests, extraintestinal manifestations, without any accompanying digestive symptoms, have increasingly become recognized, aΜecting almost all organs .. One of the most common extraintestinal manifesta-tion of CD is iron-deΟciency anemia, which is usually unresponsive to iron therapy. Osteoporosis may be present; in contrast to adults, it can be reversed by a gluten-free diet, with restoration of normal peak bone densitometric values. Other extraintestinal manifestations include short stature, delayed puberty, arthritis and arthralgia, epilepsy with bilateral occipital calciΟcations, peripheral neuropathies, isolated hypertransaminasemia, dental enamel hypoplasia, and aphthous stomatitis Diagnosis The diagnosis of CD is based on a combination of symptoms, antibodies, HLA status, and duodenal histology. The initial approach to symptomatic patients is to test for anti-TG2 IgA antibodies and for total IgA in serum to exclude IgA deΟciency. If IgA anti-TG2 antibodies are negative, and serum total IgA is normal for age, CD is unlikely to be the cause of the symptoms. If anti-TG2 antibody testing is positive the patients should be referred to a pediatric gastroenterologist for further diagnostic workup, which depends on the serum antibody levels. TREATMENT The only treatment for CD is lifelong strict adherence to a gluten-free diet. This requires a wheat-, barley-, and rye-free diet. Despite evidence that oats are safe for most patients with CD, there is concern regarding the possibility of contamination of oats with gluten during harvesting, milling, and shipping

Answer 39

Hodgkin lymphoma (HL) is a malignant process involving the lymphoreticular system that accounts for 6% of childhood cancers. HL the most common malignancy in adolescence. EPIDEMIOLOGY bimodal age distribution, with peaks at 15-35 yr of age and after 50 yr. the 3rd most common in children <15 yr old. A male predominance is found among young children but lessens with age. Infectious agents may be involved, such as ❶ human herpesvirus 6, ❷cytomegalovirus, and ❸ Epstein-Barr virus (EBV). PATHOGENESIS The Reed-Sternberg (RS) cell, a pathognomonic feature of HL, is a large cell (15-45 μm in diameter) with multiple or multilobulated nuclei. CLINICAL MANIFESTATI ONS Patients typically present with painless, nontender, firm, rubbery, cervical or supraclavicular lymphadenopathy and usually some degree of mediastinal involvement. Clinically detectable hepatosplenomegaly may be encountered. Depending on the extent and location of nodal and extranodal disease, patients may present with symptoms and signs of ❶ airway obstruction (dyspnea, hypoxia, cough), ❷ pleural or pericardial effusion, ❸hepatocellular dysfunction, or ❹bone marrow infiltration (anemia, neutropenia, or thrombocytopenia). ❺ Systemic symptoms, classified as B symptoms (prognostic) , that are considered important in staging are ❶ unexplained fever >38°C (100.4°F), ❷weight loss >10% total body weight over 6 mo, and ❸ drenching night sweats. Less common and not considered of prognostic significance are symptoms of 1 pruritus, lethargy, anorexia, or pain. Patients also exhibit immune system deficiencies that often persist during and after therapy. DIAGNOSIS to identify the presence of a large mediastinal mass before undergoing lymph node biopsy. adequate tissue is obtained, both for light microscopy and for appropriate immunohistochemical and molecular studies. extent includes history, physical examination, and imaging studies, including chest radiograph; CT scans of the neck, chest, abdomen, and pelvis; and positron emission tomography (PET) scan. should include a complete blood cell count (CBC) to identify abnormalities that might suggest marrow involvement; erythrocyte sedimentation rate (ESR); and measurement of serum ferritin, which is of some prognostic significance and, if abnormal at diagnosis, serves as a baseline to evaluate the effects of treatment. This determines “bulk” disease and becomes prognostically significant. Bone marrow aspiration and biopsy should be performed to rule out advanced disease. Prognostic INVESTAGATION : ❶Serum ferritin ❷ LDH ❸Serum copper Lugano Classification for Hodgkin Lymphoma* RELAPSE Most relapses occur within the 1st 3 yr after diagnosis, but relapses as late as 10 yr have been reported. Relapse cannot be predicted accurately with this disease. Poor prognostic features include tumor bulk, stage at diagnosis, extralymphatic disease, and presence of B symptoms. Patients who achieve an initial chemosensitive response but relapse or progress before 12 mo from diagnosis (bad prognosis ) are candidates for myeloablative chemotherapy and autologous stem cell transplantation, with or without radiation therapy. PROGNOSIS With the use of current therapeutic regimens, patients with favorable prognostic factors and early-stage disease have an event-free survival (EFS) of 85–90% and an overall survival (OS) at 5 yr of >95%. Patients with advanced-stage disease have slightly lower EFS (80–85%) and OS (90%), respectively, although OS has approached 100% with doseintense chemotherapy. Prognosis after relapse depends on the time from completion of treatment to recurrence, site of relapse (nodal vs extranodal), and presence of B symptoms at relapse. Patients whose disease relapses >12 mo after chemotherapy alone or combined-modality therapy have the best prognosis.

Answer 40

Non-Hodgkin lymphoma (NHL) accounts for approximately 60% of lymphomas in children and is the 2nd most common malignancy between ages 15 and 35 yr. pediatric NHL is usually high grade. EPIDEMIOLOGY Although most children and adolescents with NHL present with de novo disease, a small number of patients have NHL secondary to specific etiologies, including ❶ inherited or acquired immunodeficiencies (e.g., severe combined immunodeficiency syndrome, Wiskott-Aldrich syndrome), ❷ virus associated malignancy (e.g., HIV, EBV), and ❸as part of genetic syndromes (e.g., ataxia-telangiectasia, Bloom syndrome). PATHOGENES IS Three most prevalent subtypes of childhood and adolescent NHL with different treatment approaches are ❶ lymphoblastic lymphoma (LBL), ❷mature B-cell lymphoma, and 3 ❸ anaplastic large cell lymphoma. Mature Bcell lymphomas comprise 2 main pathologies, Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL). ❷Spinal cord tumors CLINICAL MANIFESTATI ONS The clinical manifestations of childhood and adolescent NHL depend primarily on pathologic subtype and sites of involvement. LBL typically manifests as a symptomatic mediastinal mass and also has a predilection for spreading to the bone marrow, CNS, and testes in males. BL commonly manifests as a diffuse leukemia presentation or massive abdominal (sporadic type) or head and neck (endemic type) tumor and can metastasize to the bone marrow or CNS. Starry-sky pattern DLBCL usually manifests as either an abdominal or a mediastinal primary tumor and, rarely, disseminates to the bone marrow or CNS. ALCL manifests either as a primary cutaneous manifestation (10%) or as systemic disease (90%) with dissemination to liver, spleen, lung, or mediastinum. Bone marrow or CNS disease is rare in ALCL. Site-specific manifestations of NHL include painless, rapid lymph node enlargement; cough or dyspnea with thoracic involvement; superior mediastinal syndrome; ascites, increased abdominal girth or intestinal obstruction with an abdominal mass; nasal congestion, earache, hearing loss, or tonsil enlargement; and localized bone pain. TREATMENT The primary modality of treatment for childhood and adolescent NHL is multiagent systemic chemotherapy and/or immunotherapy with intrathecal chemotherapy.. Surgery is used mainly for diagnosis. Radiation therapy is used only in special circumstances, such as ❶ CNS involvement in LBL or ❷the presence of acute superior mediastinal syndrome or ❸paraplegias. Newly diagnosed patients, especially those with BL or LBL, are at high risk for TLS. These patients require (treatment) ❶ vigorous hydration, ❷frequent electrolyte monitoring,❸and either an xanthine oxidase inhibitor ( allopurinol) or a recombinant urate oxidase ( rasburicase). PROGNOSIS The prognosis is excellent for most forms of childhood and adolescent NHL. Patients with localized disease have a 90–100% survival rate, and those with advanced disease have 80–95% survival.

Answer 41

Superior mediastinal syndrome can occur as a consequence of a large mediastinal mass causing obstruction of blood flow or respiratory airways. can cause cord compression and acute paraplegias requiring emergent radiation therapy. ❸Tumor lysis syndrome (TLS ) can occur from rapid cell turnover, which is especially common in BL. TLS can result in severe metabolic abnormalities, including hyperuricemia, hyperphosphatemia, hyperkalemia, and hypocalcemia. This can rapidly lead to renal insufficiency/failure, as well as cardiac abnormalities, if not aggressively treated. Ttt :aggressive hydration + allopurinol

Answer 42

Mild and moderate dehydration can be treated effectively by ORS, 50 ml/kg of ORS should be given within 4 hours to patient with mild dehydration, and 100 ml/kg of ORS should be given within 4 hours to patient with moderate dehydration, supplementary ORS is given to replace ongoing loss 10 ml/kg for each stool.

Answer 43

For sever dehydration: 1. restore intravascular volume: Normal saline (or ringer lactate): 20 ml/kg over 20 minutes ( repeat until intravascular volume restored). 2. calculate 24 hours water need: This is by addition of deficit water to maintenance water. To calculate deficit water: is by multiplying the percent of dehydration by the body weight ( in grams). Maintenance water: Water is a crucial component of maintenance fluid therapy because of the obligatory daily water losses. These losses are both measurable (urine, stool) and not measurable (insensible losses from the skin and lungs). Failure to replace these losses leads to a child who is thirsty, uncomfortable, and, ultimately, dehydrated. -- Sources of Water Loss Urine: 60% Insensible losses: ∼35% (skin and lungs) Stool: 5% To calculate maintenance water: the first 10 kg of body weight need 100 ml/kg. the second 10 kg body weight need 50 ml/kg. the third 10 kg body weight need 25 ml/kg. e.g. patient his body weight 25 kg need 1000+500+125= 1625 ml. the maximum total fluid per day is 2400 ml, the maximum fluid rate is 100 cc per hour. Urine output is normally the largest cause of water loss. Diseases such as renal failure and SIADH can lead to a decrease in urine volume. The patient with oliguria or anuria has a decreased need for water and electrolytes; continuation of maintenance fluids produces fluid overload. In contrast, postobstructive diuresis, the polyuric phase of acute tubular necrosis, diabetes mellitus, and diabetes insipidus increase urine production. To prevent dehydration, the patient must receive more than standard maintenance fluids when urine output is excessive The approach to decreased or increased urine output is similar . Place patient on insensible fluids (25–40% of maintenance) Replace urine output mL/mL with ½ normal saline 3. select an appropriate fluid: Administer half of the calculated fluid during the first 8 hours (after subtract the bolus from it), and administer the remaining fluid over the next 16 hours. 4. replace the ongoing loss as they occur. Children weighing less than 20-25 kg do best in maintenance fluid with solution contain 1/4 N.S., the glucose in maintenance fluid is 5% dextrose provide 17 k. calories per 100 ml. this is enough to prevent starvation ketoacidosis.

Answer 44

Hyponatremia produce more substantial intravascular volume depletion due to the shift of water from the extracelluler to the intracellular space . as brain cell swell thereis an increase in intracranial pressure and this responsible for most neurological symptoms . Clinical features : Anorexia , emesis ,malaise , headache , confusion , lethargy , seizure and coma hyponatremia also cause muscle cramps andweakness . Treatment : . It is important to avoid rapid correction of hyponatremia , avoid correction of s. Na more than 12 Meq. / day ( because if rapid correction it cause central pontine myelonysis ). So the initial goal is correction of intravascular volume by isotonic solution ( N.S. or ringer lactate ) . most patients with hyponatremic dehydration do well with same strategy that is outlined with isotonic dehydration . D5,1/5 N.S. with 20 Meq/L KCl is usually effective , 1/2 the fluid will be administered over the 1st 8 hours ( K should not be given until the patient voids ) , and Na is monitored to ensure appropriate correction and Na concentration of the fluid is adjusted appropriately , if develop seizer give infusion of 3% saline 12 cc/Kg Hypernatremic dehydration : It is the most dangerous form , hypernatremia can cause serious neurological damage including hemorrhage and thromboses this is to movement of water from intracellular space to intracellular space causing brain cell shrinkage and tearing blood vessels . also this movement of water protect the intravasculer volume thus children with hypernatremia appear less ill than children with a similar degree of dehydration from other types . urine output may be preserved longer and may be less tachy cardia . so unfortunately this lead to brought children to medical attention in delayed and more profound dehydration . Other symptoms of hypernatremia ; irritability , lethargy , fever and hypertonicity . Treatment : To avoid risk of cerebral oedema , s. Na concentration should not be decreased more than 12 Meq/L/day , so we can apply this schedule; 1- restore intra vascular volume ; by I. v. fluid N.S. 20 cc/k.g. over 20 minutes and reassess. 2-determine the time of correction based on the initial Na concentration; S.Na : 145-157 Meq/L : 24 hours 158-170 Meq/L : 48 h. 171-183 Meq/L : 72 h. Typical fluids ; D5,1/4 N.S. (with 20 Meq/l Kcl ) or D5,1/2 N.S. 3- follow up s. Na concentration. 4-adjust fluid based on clinical status ; If s. Na decrease too rapidly; increase Na concentration of i.v. fluid decrease the rate of i.v. fluid. If s. Na decrease too slowly ; decrease Na concentration of the i.v. fluid or increase the rate of fluid. 5- replace the ongoing loss as they occure.

Answer 45

chronic non specific diarrhea.; occur in generally well appearing toddler 1-3 year of age ( toddler diarrhea ) the diarrhea is often brown and watery , at times containing un digested food particles , if the child fluid intake more than 150 cc/kg/ 24 h fluid intake should be reduced to 90 cc/kg/24 h , parent note that patient irritable in the first two days of fluid restriction however this result in decrease stool frequency and volume . if the history suggest the child take more fruit juice reduction in the offending juice correct diarrhea . sorbitol sugar is found in apples , pear can cause diarrhea in toddler , white grape juice is best alternative . if the child fat intake is restricted by the parent the fat intake can increase to 40% of the total calorie . in addition to treat the cause, during treatment of chronic diarrhea should focus on providing adequate nutritional support, also take attention to type and volume of fluid when used in treatment, when use antibiotic therapy in bacterial overgrowth it should be of broad spectrum; such as metronidazole, tetracycline, chloramphenicol and ampicillin and for 2 weeks course, i.v. nutrition and complete bowel rest for secretary diarrhea and lope amide and somatostatin in primary villous atrophy. In Rotavirus-induced severe and protracted diarrhea, oral administration of human immunoglobulins (300 mg/kg) should be considered. Zinc supplementation is an important factor in both prevention and therapy of chronic diarrhea, because it promotes ion absorption, restores epithelial proliferation, and stimulates immune response.

Answer 46

The disaccharidases are located on the brush border membrane surface of small bowel , the most common is lactase deficiency, occasionally congenital deficiency occur . but most often is the result from diffuse acquired lesions of the intestinal epithelium such as infection or celiac In older children and disease . adults late onset genetic lactase deficiency is the most common cause . Clinical manifestation . Watery osmotic diarrhea with stool PH less than 5.6 , contain excess sugar and excoriate the buttocks , patient may bloating and borborygmi. If the Disaccharide involved is a reducing sugar (lactose ) the standard clinitest examination will be 2 + or greater in most cases. Disaccharidase activity can be assayed in mucosal biopsy , breath hydrogen excretion after an oral sugar load is a useful non invasive technique for detecting disaccharide intolerance . Treatment : Consist of removal of milk from the diet , in most cases the elimination need not be total . a lactase preparation is available as a tablet ingested with meal . yogurt contains bacteria that produce lactase enzyme . thus frequently tolerated by lactase deficient individual .

Answer 47

have been identified to cause this rare autosomal recessive congenital disorder . this transporter couples glucose and galactose to the sodium gradient across intestinal and renal brush border . renal tubular epithelium is affected to a lesser degree . Clinical manifestation : Osmotic diarrhea followed ingestion of glucose , breast milk or conventional formula soon after birth because most dietary sugar are polysaccharides or disaccharides with glucose and galactose moieties . the patient may be bloated and if diarrhea persist dehydration and acidosis can be sever , resulting in death , the stool is acidic and contain sugar. the finding of positive reducing substance in a watery stool and slight glycosuria despite low blood sugar level is highly suggestive of Glucose – galactose malabsorption . and it also easily identified by using breath hydrogen test , after ingestion of 0.5 g/kg glucose the breath hydrogen rise to more than 20 PPM . Treatment : Vigorous restriction of glucose and galactose . fructose the only carbohydrate that can be given safely should be added to carbohydrate – free formula in a concentration of 6 – 8% . diarrhea immediately ceases when infants are given such a formula .

Answer 48

It is due to zinc deficiency , early in life the patient experience rash around mucocutaneous junctions ( mouth and anus ) and on the extremities , alopecia , chronic diarrhea and sometime if not treated the patient fail to thrive .serum zinc and alkaline phosphatase are low , intestinal biopsy show Paneth cell inclusion that disappear after treatment. Treatment : Oral zinc sulfate 1-2 mg elemental zinc /kg / 24 h .causes rapid healing of skin lesion and improvement of diarrhea.

Answer 49

It present early in life with watery diarrhea and sometime with streak of blood in the stool the infant may develop eczema , recurrent otitis media , respiratory wheeze and failure to thrive if not treated . Treatment : By avoiding the offending milk , prolong breast feeding decrease the incidence of cows milk protein intolerance . sodium cromoglycate also useful in treatment .

Answer 50

G6PD Defeciency Is X-linked disease , is most important dis. Of hexose mono phosphate pathway , is responsible for 2 clinical syndromes :-a- an episodic H. A ( hemolytic anemia ) induced by infection , drugs , fava bean . b- spontaneous chronic non spherocytic hemolytic anemia . A--- episodic H.A ;--G6PD enzyme Oxidized glutathione NADP ----- NADPH -Glutathione reductase reduced glutathione ( as anti oxidants) Glutathione act to neutralized agent that potentially oxidized either Hb or components of Red cell membrane . If reduced glutathione can not sustained to remove O 2 radicalls generated by oxidants drugs leading to Hb precipitate forming heinz body and Red cell membrane is critically damaged leading to premature destruction or hemolysis . G6PD def.A- may produce spontaneous hemolysis . In preterm baby G6PD def B minus and canton variaties may produce Sever hemolysis even kernicterus Chronic non spherocyteic hemolysis & favism occur on G6PD def B minus ..

Answer 51

Clinical feature recurrent or episodic attack of H.A where clinically appear as pallor & red urine , developed after 24-48hr after ingestion of oxidant properties Agent precipitate hemolysis in patients with G6PD def. :--A- medication :---1- antibiotic like nalidexic acid , chloromphenicol, sulfa., 2- anti malaria 3- others like phenacetin , asperin, vit. K analogue , phenazopyridine . nitrofuradantin B-chemical as benzene , naphthalene , phenylhydrazine . C- illness ;- D.K.A , hepatitis , sepsis . the degree of hemolysis varies with :--1- inciting agent . 2- amount of ingestion 3- the severity of enzyme def. in the patients . If hemolysis is sever – Hb uria , jaundice & Hb is reduced to be life threatening . Favism mean as acute H.A induced by fava bean & thought to more frequent associated with G6PD B minus variant.

Answer 52

1- decreased PCV , 2- IF episode is sever resulting Hb emia & Hb-uria note : most individual with G6PD are asymptomatic & no clinical manifestation unless triggered by infection , drugs and fava bean If haemolysis is sever leading to Hb binding protein called haptoglopin are saturated and free Hb may appeared in the plasma and subsequently in urine . . 3-unstained or supra -vital preparation of RBC reveal Heinz body( Hb ppt) . 4- blood film ;- reveals a few fragmented cell & polychromatophilic ( bluish large RBC ) & the retic count often 5-15%

Answer 53

Diagnosis :-- 1- direct measurements of G6PD level within RBC ( enzyme activity in affected person is 10% of normal or less ) . 2- satisfactory screening test are based on -- :-- a- decoloration of methylene blue . - on reduction of met Hb (measured the reduction of met Hb production after NADP reduction . . - on fluoroscent of NADPH( is direct test to measure the generation of reduction NADPH from NADP and the test is positive if blood spot is failed to show fluoresent under U/V light .. . N .T immediately after hemolysis ---increase retic count & young RBC have significantly higher enzyme activitly than older cell in A- variaty & testing may deferred for a few wks before diagnostically low level of enzyme can be shown . G6PD variants also can be detected by electrophoretic & molecular analysis . Fava bean ( green and dry are induced hemolysis)

Answer 54

Prevention :-- 1- assay for G6PD for those pts are liable for G6PD deficiency as in ethnic group , those with +ve family history of G6PD . 2- avoid triggered factors that induced hemolysis Treatment :-- 1- Admission to hospital . 2- chart for observation ( PR , RR ) . 3- frequent checking of PCV ,retic count . 4- given I.V. fluid in form G/W or saline to induce more diuresis + added NAHCO3 to prevent ppt of Hburia within renal tubules & prevent its damage . 5- if PCV continue to be decreased associated with increased PR, RR , --- more anemia with cardiac compromised needs blood transfusion .

Answer 55

A number of extrinsic agents & disorders may lead to premature destruction of RBC due to presence of antibodies . The hallmark of this disease is a positive coombs tests which detect coated of antibody ( IG ) or component of complements on the RBC surface . erythroblastic fetalis is most important example in pediatric practice. Auto Immune either :----a- warm type b- cold type Warm type :---2 type either :- • 1- transiant form ( account 70-80% of cases , occur between 2-12years & last 3-6 month . 2- prolong or chronic coarse which more frequent in infant & children older 12year , hemolysis continue for months or years .

Answer 56

Auto immune ( warm type ) due to :--a- idiopathic type ( primary ) . B- secondary – 1- lympho proliferative dis. 2-CTD as in SLE • colitis tumor . 3- Chronic infl. Dis as ulcerative • 4-non lymphoid tumor as in ovarian Auto immune ( cold type ) :-- with » cold reactive auto Abs( cryptogenic H.A ) a- idiopathic type . • b- secondary type ---:- • 1-lympho proliferative dis. • 2-infection as in mycoplasma , IMN • • c- drug induced as in :--by Hapten mechanism as pencillin , cephlosporine or Ternary immune complex as (quinidine or quinine ,anti T.B, tetracyclin , insulin ) or True antibody as in methyl dopa , Brufen ,voltarene ,interferone alfa ..

Answer 57

HbS is resulting from genetic mutation where by glutamic acid is replaced by valine at position 6 on β-chain of Hb . This resulting in polymerization of Hb under condition of hypoxia or acidosis . GROUPS OF SCS ( SICKLE CELL SYNDROME ):--- 1-sickle cell trait , 2-Hb SC disease 3-Hb S-β0-thalassemia 4- Hb S- β+ thalassemia. 5- Hbss. HBSS ( SICKLE CELL DIS, OR S. THALASSEMIA) : 1- affected newborn seldom exhibit C/F . 2- H.A. gradually developed over 2-4 months paralleling of replacement of HbF by HbS . 3- infant has abnormal immune function as early as 6 months of age ( splenic dysfunction) . Some children and by 5 years of age have functional asplenia. 4- all patients are at increases risk of infection 5-dactylitis ( hand foot syndrome which symmetrical or unilateral of hand or foot occurring in 50% of children in the second year ). Splenic dysfunction is caused by sickling of RBC within the spleen resulting in inability of spleen to filter microorganism from blood stream . Splenic dysfunction is usually followed eventually by splenic infarction usually by 2-4 years of age .

Answer 58

1- acute painful crisis ( vaso-oclusive crisis ) . 2-splenic sequestration . 3- aplastic crisis . 4-hemolytic crisis Note 1-:no evidence of blood transfusion decrease intensity or duration of episode of painful crises . 2-multiple painful episode requiring hospitalization within one year & pain episode required hospitalization of more than 7 days →it needed evaluation for comorbidities diseases or psycological stress .

Answer 59

IS MEDICAL EMERGENCY that required prompt medical evaluation & delivery of antibiotic because of high risk of bacterial infection & the concomitant high fatality rate when infected . Several clinical strategies have been developed ranging from ; 1- admitted all patients with fever for Intra.venous antibiotic . TO-- 2- administration paranteral 3th generation cephalosporin in out patient setting if no risk factors of occult bacteremia . TO— 3- febrile patients with established risk factors for bacteremia : admission to hospital .

Answer 60

1-severely ill patient . 2- hypotension . 3- poor capillary refilling(more than 4 second ) . 4- temperature of more than 40. 5- WBC of more than 30000 or less than 5000 . 6-platelts count of less than 100 000 . 7- Sever pain . 8- history of pneumococcal sepsis . 9- dehydration . 10- Hb of less than 5gm\dl with Blood culture should be aspirated to all patients with SCA associated fever

Answer 61

1-Infection . 2- acute chest syndrome 3- sever anemia 4-stroke ( 11% as overt stroke , 20% as silent stroke before 18 year) 5-priapism . ( 20% 0f patient between 5-20 year of age ) 6- hyposthenuria 12% . 7-skin ulcer 8-avascular necrosis 9-retinopathy 1-3%

Answer 62

primary prevention Can be accomplished by transcranial doppler (TCD) , Which assess blood velocity in brain blood vesseles (if more than 200 cm\sec--- Transfusion threshold , prophylactic blood transfusion is instituted to maintain Hbs of less than 30% . Secondary prevention ;- is accomplished by blood transfusion to maintain Hbs of less than 30% in first 2 year of stroke & less than 50% thereafter .

Answer 63

1- retic count is increased 5-15%, Hb 5-9gm/dl, normal to increase platelets, WBC 12000—20 000 . 2- blood film :--shows sickle cell, howell jolly body , 3- confirm diagnosis by Hb-electropheresis. SEVERAL SCREENING TESTS AVAILABLE INCLUDE : 1-classic meta-bisulfite sickle cell 2- solubility tests which deoxygenated Hbs precipatated in a aqueous solution 3-hyperactive bone marrow 4-X-ray in vertebral body ( osteoporosis ) and mild expansion of B.M.

Answer 64

A- is directed towards prevention of complication & optimization of health & health coping strategies by :--- 1- all patients should be immunized against pneumococcal , meningococcal & annual adminstration influenza vaccine . 2-pencillin prophylaxis , 3- folic acid 1 mg daily 4- all febrile patients needs aspiration of blood culture & patients with risk factor of bacteremia - needs admission . if well appearing patients & no risk factor – given ceftriaxone &then discharge To be if culture is staph or salmonella , should be evaluated for osteomylitis seen 24hr later for rechecking & results of culture. 5- to understanding of the family ,how to palpate spleen to diagnosis early sequestration crisis . B-avoid the following :- 1- dehydration & acidosis . 2-avoid vascular stasis . 3-cold temperature . C-ttt of complications :- I. pain killer II. blood transfusion in :- acute chest syndrome , stroke , stroke prevention, sever anemia before anesthesia and priapism . -Chronic transfusion therapy used in : 1-still has TCA( transiant cerebral ischemia . 2- avascular necrosis of hip & shoulder III. drug therapy :-- like hydroxy urea :--it is myelosuppressive drug. its aims :- a- decrease severity and rate of crisis of pain to 50% including acute chest syndrome b- decrease blood transfusion to 50% in adult c- decreased hospital stays .and also prevent or slow down damage to your organs . Is safe and well tolerated in children older than 5 years of age . if start in infants :--- ❶may preserve spleenic function , ❷ improve growth pattern , ❸reduce incidence of acute chest syndrome . dose 15-20 mg\kg daily with increase dose every 8 wk of 2.5-5mg|kg ( max. dose is 35 mg |kg ) . theraputic effects required several months follow up of patient on hydroxyurea through measuring CBP( complete blood picture and blood urea stopped treatment if 1-WBC of less than 2500 and 2- platelets of less ( than 100000 ), 3-sever anemia , 4-increased blood urea and liver injury . -L-glutamine ( endari ) :- is indicated to reduce acute complications of sickle cell disease in adult and children aged more than 5 years . Dose < 30 kg :- 5gm twice a day ( packed has 5 gm ) >30 kg : 10 gm / Twice a day Administration : mix immediately before ingestion with 8 oz of cold or room temperature beverage ( water , milk , apple juice ) or 4-5 oz of soft food like yogurt or apple sauce . -Voxelotor ( Oxbryta ) :- is inhibited HbS polymerization ( bind reversibly to Hb stabilizing the oxygenated Hb state and preventing Hb S polymerization by increasing Hb affinity for O2 ) , therefore can inhibit RBC sickling , improve RBC deformability and reduce whole blood viscosity ) . Dose : above 12 years : 1500 mg once daily 4-12 years :-depend on body weight : > 40 kg : 1500 mg once daily 20-40 kg : 900 mg 10-20 : 600 mg once daily Side Effects: ( rare as ):-black tarry stool , chest pain , fever , chill , cough , allergy , face swelling , unusual bleeding or bruising and unusual tiredness . IV. 4- bone marrow transplant

Answer 65

SEQUESTRATION CRISES :- is life threatening condition , with hyper acute drop in the Hb level ( blood volume ) , it is secondary to splenic pooling of the patients RBC and sickling within spleen . It is associated with moderately enlarged spleen plus abdominal pain and retic is elevated . TREATMENT CRISES : 1- chart of observation of pulse rate ,blood pressure & respiratory rate 2- frequent checking size of spleen especially during given blood . 3- if respiratory rate , pulse rate are decreasing and blood pressure increased with normal respiratory rate → should continue on blood , while if patient developed increasing respiratory rate with basal criptation plus increased pulse and decreased blood pressure with decreased size of spleen which mean returing the blood from spleen to circulation and developed pulmonary edema and you should stop blood transfusion . Aplastic crises associated with parvo virus , it is characterized by sever pallor , decreased retic with no abdominal pain , and treatment by blood . Hemolytic crises associated with acute hemolysis due to G6PD def associated with increased retic count , red urine , more jaundice with no abdominal pain

Answer 66

1. frequent or multiple painful episode of crises (of more than 6 attack) or more per year in children or more than 3 episodes per year in adult . . 2. history of acute chest syndrome . 3. sever symptomatic anemia 4. history of stroke or high risk of stroke 5. sever unremitting chronic pain that not control by conservative measures 6. history of other severe vaso occlusive events

Answer 67

Hbs is less than 50% and HbA of more than 50% . Life span is normal & serious complication are very rare. CBP :- within normal range . Hb –electropheresis is diagnostic . Complication include :--- include 1- sudden death during vigorious exercise . 2- splenic infaraction at high attitude . 3- hematuria , hyposthenuria & bacteruria . 4- eye injury as hyphema . Children with sickle cell trait should not have any restriction on activities

Answer 68

THALASSEMIA SYNDROME Thalassemia Syndrome Is genetic disorder in globin chain production Either alpha-Thalassemia. , B- Thalassemia. , Sickle Thalassemia. B-thal. Resulting from deletion or mutation , it is presented on chromosome number 11, while alpha thal. Resulting mainly from deletion , the gene presented on chromosome number 16 . Homozygous b- thalassemia (b- thalassemia major or cooleys anemia ) : CLINICAL FEATURE 1- progressive anemia with profound weakness & cardiac decompensation during the second 6- month of life if not treat ( most infant & children have cardiac decompensation of Hb of 4gm/dl or less .. 2- hepato splenomegaly ( spleen may become so enlarge causing discomfort & even hypersplenism which defined by increased requirement of packed R.B.C. of more than 240 cc/kg/ year . 3-generally fatigue , poor appetite & lethargy are late finding of sever anemia in an infant or child . 4- late finding of disease developed : a- bronze skin ( greenish brown complexion as result from combination of pallor , jaundice and hemosiderosis . b- repeated blood transfusion resulting iron overload LAB FINDING : anemia increased iron absorbion bone marrow ineffective erythropoisis blood transfusion ppt in many organ iron loading 1- anemia :- decrease Hb & elevation of retic count ( of less than 8%) with normal WBC, elevation of unconjugate bilirubin . 2-Hb-electropheresis is diagnostic ( Hb F may reached 98% & A2 2% . 3- if no treatment well can result in B.M. expansion . X-Ray finding as Hair on end appearance . 4- with frequent blood transfusion lead to increase iron in body which measure by S. ferritin . DDX :- A- other causes of hypochromic microcytic anemia ( IDA ,lead & copper poisoning & pyredoxine deficiency. B- anemia with increase retic count ( thalass. Intermedia , SCA,,HbC ,HbS , HbE & auto immune hemolytic anemia ). C- anemia with low retic count .( aplastic anemia as black fan diamond syndrome & fanconi anemia ) D- cong. Spherocytosis .

Answer 69

1- BLOOD TRANSFUSION :- aim of blood transfusion is ❶ to promote general health & well being & ❷ avoid the consequences of ineffective erythropoiesis i.e hyper –transfusion regimen can ❶ correct anemia and ❷ suppress ineffective erythropoiesis , ❸ limiting stimulation of increased absorption of iron →❶ making the bone to heal , ❷ decreased metabolic expenditure , ❸increased growth &❹ limit dietary iron absorption 2- TREATMENT OF IRON OVERLOAD :--- iron overload is measured by 1- s- ferritin 2- liver biopsy supplemented by ferritometry. 3- T2 weighted MRI imaging . 4- Transferrin Saturation Hemosiderosis is prevented by using chelating therapy ( desferioxamine to form desferal –iron complex which excreted by urine & stool & enhance its excretion by vit C Blood transfusion in a dose of 20cc/kg given within 4-6 hr to maintain Hb level in normal range that not result in B.M. expansion i.e pre transfusion is more than 9.5gm/dl –10.5 gm/ dl is good . low transfusion regime use to maintain level of Hb 85-95 gm/dl g not > 12 gm/dl and not <8 gm/dl - give in developing Countries (such as Iraq) due to lack of blood transfusion and lack of therapy to treat iron overbad. but may develop bone marrow changes due to activation of bone marrow. And S .T. increase dose to 100 mg \ kg if : 1-cardiomyopathy . 2- if s. ferritin of more than 2500 nanogram\dl . S .E .:- ❶ ototoxicity , ❷retinal change , ❸bone dysplasia with truncal shortening . Oral iron chelator ( defriprone ) :- is not effective as desferal but more effective in removing cardiac iron . -also -S .E MAY associated with ❶ neutropenia in 3%, ❷arthritis , ❸hepatic fibrosis . now: used oral desferasirox ( Exjade ) which better for compliance , optimum dose is well defined as maintenance dose between 30--< 40 mg/kg/day ( starting dose at 20mg/kg/day and increased dose 5—10 mg/kg every 3-6 month depend on S.ferritin and safety of drug . 3-SPLENECTOMY :- indicated in following :-- 1- hypersplenism 2- thal. Intermedia with falling steady state of height . Before splenectomy ;-- pts should receive pneumo coccal & meningococcal vaccine .& then kept on prophylaxis pencilin therapy . 4- B. M TRANSPLANTATION TO CURE PTS . ( hemapoitic stem cell transplant which most success if pt of less than 15year of age , not excess of iron store or hepatomegally . * Before transplant → give blood transfusion to Suppress bone marrow → give chemotherapy to kill all RBCs in the bone marrow + give prophylaxis antibiotics due to risk of infection.after transplant → give chemotherapy to prevent growth rejection + prophylaxis antibiotic to prevent infection

Answer 70

1-if s. ferritin of more than 1000 nano gram/ dl or 1000 mg/L 2- if s. ferritin is not available , we depend on no. of blood transf. 10—20 time or 3—5 year from onset of blood transfusion . Desferal given by special pump ( S.C.) IN a dose 20-50 mg |kg \day 5 days/week, except patient with Complicated Cases like Cardiomyopathy, arrhythmia, level of ferritin >2500 ng/d! Continuous iv or Sc desferal 100 mg/kg/day

Answer 71

DEPEND ON GENE GELETIONS :- A- deletion of one gene called silent trait ( carrier ) :- has no symptoms and can detected through the rate the α & β synthesis or molecular biological techniques . B- deletion of 2 gene called thalass. Trait :--has mild microcytic anemia ( Barts Hb is 5- 10% ) . C- deletion of 3 gene called Hb-H Dis. :--has marked microcytic anemia and mild hemolysis . it is generally asymptomatic with moderate anemia , mild splenomegally , occasionally jaundice or gall stone . transfusion is not usually required for treatment of anemia . brilliant cresyl blue can stain Hb-H . D- deletion of 4 gene ( Hydrops fetalis ) , severe intrauterine anemia and death unless intra uterine transfusion .. has 80-90% of Barts Hb with Gower 1 , 2 & portland Hb

Answer 72

– non hemolytic febrile reaction . – auto immune hemolytic anemia . – allergic reaction . – delayed transfusion reaction . – anaphylactic reaction .

Answer 73

A- excess erythropoiesis :- 1- bone changes to face . 2-bone :- cortical thinning & risk of fracture 3- spinal cord compression . 4- LAP especially mediastinum & HSM B-iron over-load . C- endocrine failure like short stature , delayed puberty, D.M, hypothyriodism & hypo- parathyridism , d- cardiac complication . e- hepatic involvements . f- chronic hemolysis .– gall stone . g- infection

Answer 74

can cause by WBC sensitivity it is characterized by fever ( other causes should be considered included hemolytic transfusion reaction or administration of a blood with bacterial contamination ) . prevention : by leucoreduction which sharply reduces cytokine accumulation and leucocyte alloimmunisation or through given blood through WBC filter which prevent passing of more than 95% of WBC or if these not available , they give antipyretics

Answer 75

due to plasma protein range its range from mild to severe mild form include urticaria , itching , flushing which generaly mediated by IgE severe reaction such as stridor , bronchospasm , hypotension and other symptoms of anaphylactic may occur especially with IgA deficiency and anti -IgA antibodies Prevention : mild form : by antihistamines or corticosteriod before transfusion . Recurrent reaction can reduced by washing red blood cell to remove the plasma . Patients with IgA deficiency and those with severe reaction may require blood from IgA deficient donors .

Answer 76

begin within minutes or some time within hours of initiation transfusion . it is characterized by abrupt onset of fever , chills , backpain , sense of impending death , dyspnea , Hburia and shocked . It can be caused by error in patients identification or blood typing & compatibility testing Prevention :- 1- used of optimal method for identification the patients and labeling of sample when blood obtained for cross matching . 2-proper linkage of sample to donor unit in blood bank . 3- adherence to standard protocols for screening for antibodies and carrying out the necessary full cross matching of donor unit . 4- use of multiple patient identifiers before blood transfusion . Treatment : 1- stopped blood when suspect the reaction immediately and given intra venous fluid to maintain intra vascular volume 2- diuretic therapy help to preserve renal function 3- if DIC developed may need heparin therapy 4- identification of donor and patient should be checked 5- the blood bank should be alerted to possibility of alloantibodies .

Answer 77

very serious complication that usually but not allways occurs in patients with allo antibodies even red cell from seemingly compatible unit . Hb may fall well below the usual pre transfusion level because of destruction of both donor and recipient red cell Treatment : steroids , immune suppressive drugs & I.V I.G ( intra venous immuno globulin ( little benefit ) and some patients may need rituximab . It is commonly seen to those initiating blood for teenagers and adults with thalassemia intermedia

Answer 78

is common complication , occurring in 10-20 % of thalassemia It is more common in children who begin blood transfusion after 1-3 years of age than those begin transfused earlier . some suggest developing new antibodies after splenectomy Prevention : used extended antigen matched donor blood delayed reaction :-usually occurring after 5-14 days from transfusion and characterised by unexpected anemia , jaundice and malaise it is due antibody which not detected at time of transfusion or new antibodies developed . The sample should be sent for blood bank for detection of antibodies and repeat cross matching of last administered unit

Answer 79

Hb level fall bellow 7—8 gm/dl

Answer 80

1- increase cardiac output & O2 extraction 2- a shunting of blood towards vital organ & tissues. 3-2,3 DPG increases within the RBC leading O2 dissociation curve shifted to right resulting more release of O2 to tissues .

Answer 81

1. Fatigue or tiredness 2. Irritability . 3. Anorexia in anemia in children 4. Pagophagia and anemia in children 5. Skin or mucosal pallor or jaundice 6. Tachycardia and anemia in children 7. Hair loss or weak nails

Answer 82

Anemia Hb &indices Retic count &morphology ---:-1- inadequate response (RPI ) :of < 2% :a- hypochromic microcytic b- normochromic normocytic anemia . c- macrocytic . 2-adequate response : ( RPI ) of > 2% :- 1-Hbpathy 2-Hbss ,SC , S-B thal. 3-enzymopathy as G6PD def. or pyruvate def. 4-membranopathy as cong. Spherocytosis or elliptocytosis 5- extrinsic factors like DIC & HUS ,, Burns ,, wilson dis. ,, Vit E def., abetalipoproteinemia 6-immune H.A as in auto immune , iso immune , drug induced Inadequate response : -Hypochromic microcytic anemia as IDA , thalass. Trait , chronic inflammatory dis. , copper and lead poisoning , sideroblastic anemia . -normochromic normocytic anemia as chronic inflammotory dis. , recent blood loss , malignancy , marrow failure , chronic renal failure & transient erythroplastopenia of childhood . -macrocytic anemia as in folate , Vit 12 def. , marrow failure , hypothyrodism , Down syndrome

Answer 83

Anemia with reticulocytosis - 1- ongoing hemolysis (normal retic count is 1%). :-2- bleeding • Anemia with increase T.S.B:--hemolysis:- doing peripheral blood smear :-- 1-spherocyte as in heriditory spherocytosis , wilson dis. , auto immune H.A . 2-sickle cell ( SCA, S. thalass. ) 3- target cell ( Hb sc ) 4-bite cell / blister cell ( G6PD ) 5- Micro angiopathy H.A (D.I.C., H.U.S ) 6- Nucleated RBC ( B-thalass. ) Note : reticulocyte production index( RPI) : measured by :-observed PCV ------------------- X retic count normal PCV

Answer 84

1- age :; IDA is rare before 6 month in absent of blood loss in term infant and before doubling birth weight in preterm infant . 2- family history : X-linked as in G6PD def . AD : cong spherocytosis , AR in SCA , Thalassemia & fanconi anemia family history of cholycystectomy due to bilirubin stone suggest spherocytosis or other inherited hemolytic anemia 3- nutrition : cow milk : IDA , pica : IDA, strict vegetarian : V12 Def , Goat milk : folate def , cholestasis & malabsorption : Vit E def, plumbism (lead poisoning ) 4- drugs :- G6PD def as in oxidants eg nitrofuradantin , metheprim etc immune mediated hemolysis : as pencillin bone marrow suppresison as chemotherapy phenytoin which increased folate requirement 5- diarrhea :malabsorption of Vit b12 , iron , Vit E milk protein intolerance : induced blood loss intestinal resection : vit B12 def . 6- infection : mcqs Giardia : iron def . intestinal bacterial overgrowth : vit B12 parvo virus : bone marrow suppression malaria : hemolysis, hepatitis : can produce G6PD def , aplastic anemia

Answer 85

SKIN :- 1-hyperpigmentation , café au lait spot : fanconi anemia, neurofibromatosis 2-vitiligo ; Vit B12 def 3-partial oculo cutaneous albinism : Chediak Higashi syndrome 4-jaundice : hemolysis , 5- petechiae & purpura : BM infiltration , HUS , auto immune hemolysis with auto immune thrombocytopenia 6-Erythematous rash :-parvo virus & IMN 7- butterfly rash : SLE , 8-Bruising as in bleeding tendency HEAD :- 1-frontal bossing as thalassemia , chronic subdural hematoma &severe IDA 2-microcephaly : fanconi anemia EYE : 1-microphthalemia : fanconi 2- retinopathy : Hb SS , SC disease 3-optic atrophy : osteopetrosis , 4- K F ring : wilson dis , Mouth:- 1-Glossitis : B12 def , IDA , 2- angular stomatitis : IDA , 3-Cleft lip :Black fan diamond CHEST : 1-shield chest or wide spread nipples : black fan diamond syndrome EXTREMITIES : 1-absent thumb : fanconi , , 2-triphalangeal thumb : Black fan diamond. 3-spoon nail : IDA , 4-dystrophic nail : Dyskeratosis congenita RECTAL : 1-hemorrhoid : portal hypertension , 2-Heme positive stool as in G.I hrr NERVES : 1-irritable and apathy : IDA , 2-PNP : B12, B1 , E , , lead poisoning SHORT STATURE :- fanconi , malnutrition

Answer 86

HEREDITARY PURE RED CELL APLASIA ( BLACKFAN DIAMOND SYN DROME) Is rare condition , is sporadic , although dominant or recessive in 15% ,.& mutation is seen in 25% . Usually become symptomatic in early infancy as profound anemia by 2-6 month of age ( 90% occur in first year of life ). 50% of affected children have congenital anomalies like short stature in 30%, defect in upper extremeties and thumb defect( triphalangeal , cranio facial abnormalities (, cleft palate , macroglossia and cong. Malformtion) in 35-45% ,cardiac and urogenital defect . LAB. FINDING : 1- macrocytosis with no hypersegmented neutrophil . 2- very low retic count . due -to bone marrow suppression. 3-EAD(erythrocyte adenosine deaminase is increased in most pts 4- RBC precursor is markedly reduced . 5- slight elevation of Hb F Sever anemia in the first few months of life. + Sever reticulocytopnea + Congenital anomalies + Normal WBC + normal platelet. = Blackfan Diamond syndrome

Answer 87

DDX: 1- Other causes of low retic count 2- TECCont. DIAGNOSIS DIAGNOSTIC CRITERIA . Age less than 1 year Macrocytic anemia with no other significant cytopenias Reticulocytopenia Normal marrow cellularity with a paucity of erythroid precursors SUPPORTING CRITERIA Major Gene mutation described in classical'' DBA Positive family history (50% genetically transmitted - 50% new mutation) o o Minor o o o o Elevated erythrocyte adenosine deaminase activity Congenital anomalies described in 'classical'' DBA Elevated HbF No evidence of another inherited bone marrow failure syndrome

Answer 88

1- 80% of patients respond initially to steroid of unknown mechanism ( 2mg\kg of prednisolone ). Hb reach to normal within 4-6 wks ( increasing RBC precursor within 1-3 wk of therapy ) . Steriod is not recommended before 6 month of age even some hematologest not given before one year of age . 20% of patients not respond to steroid , it needs blood transfusion PROGNOSIS : 50% are long term responder And 50% who respond initially become resist later & 20% has spontaneous remission . (40% is steriod dependant , 40% transfusion dependant & 20% no need treatment Although it is considered as premalignant syndrome with acute leukemia and mylodysplastic syndrome . .

Answer 89

Is more common acquired red cell aplasia occurring in children( more common than Black fan Diamond syndrome ), IS characterized by :--- 1- occur in previous healthy children between 6 month– 3 years ( mostly above one year of age ) & only 10% after 3 year of age . 2- resulting from transiant immunological suppression of erythropoiesis & often follow viral infection . 3- very low retic count 4- normal ⑧ MCV and HbF 5- virtually all children are recovered within 1—2 month Blood transfusion may be necessary , while cortico steriod with no benefit . Note ;-- any child with presumed TEC who required more than one unit of blood transfusion , you should evaluated for another diagnosis .

Answer 90

Physiological Anemia of infancy Is progressive decline of Hb within first few wks of life & persists for 6-8wk , reaching in full term infant 9-11 gm\ dl at 8-12wk of age , while in preterm infant reaching 79gm\dl by 3-6 wk of age . Several factors are operative:-1- suppression of erythropoeitin production with increasing O2 delivery to tissue. 2- shortened survival of RBC of premature infant 40—60 days instead of 120 days . 3- late hypo-regenerative anemia with absence of reticulocyte. 4- dietary factors like folic acid ( vit.E def. & therapy have no role in this type of anemia ) Treatment 1-Generally required no therapy other than ensuring that diet of the infant contains essential nutrients for normal haematopoiesis especially folic acid & iron 2-anemia in VLBW --- treated by erythropoitin & iron .

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