Clinicopathologic conference arf in a child

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CLINICOPATHOLOGIC CONFERENCE. Conservative management of a child with Acute Renal failure. By : Dr Inayat Ullah PGY-1 Pediatrics Shifa International Hospital Islamabad.

Transcript of Clinicopathologic conference arf in a child

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CLINICOPATHOLOGIC CONFERENCE.Conservative management of a child with Acute Renal failure.

By : Dr Inayat UllahPGY-1 Pediatrics

Shifa International Hospital Islamabad.

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Personal Profile

Name : A.B.C.

Age :1 Year.

Sex : Male

D.O.A : 29-09-2014 2300Hrs

M.O.A : Emergency

Residence : Nowshehra KPK.

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Presenting Complaints

i. VOMITING - 15 DAYS

ii. OLIGUANURIA - 05 DAYS

iii. IRRITABILITY - 02 DAYS

iv. ANURIA - 02 DAYS

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History Of Present Illness

Patient who has been on ATT for the last 5 months which were started due to recurrent diarrheas, fever and failure to thrive.

Presented with

Irritability associated with nil urine output for the last 2 days.

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• The child was also having persistent vomiting not

responding to any medications.• There was no history of skin rash joint pain, RTI or

passing black colored urine. • There is history of weight loss and pallor observed by the

parents.

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Past History

5 months back: presented to a GP for complaints of fever, loose motions, pallor and weight loss who started him on ATT on 28th April 2014.

Follow up: He presented several times to the same physician for same complaints ( G/E, Oral Thrush).

For the last 3 days he remained admitted in a private hospital at Peshawar where he got transfusion of 90ml whole blood, in the meantime his RFT’s came out to be deranged for which he was referred to SIH Islamabad for further management

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Birth History

• SVD at full term with no perinatal complications at home

Cry: immediate. Birth weight not documented but parents says he was healthy

• Vaccinated up to date according to EPI card not available.

• Developmentally normal.

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Family History• Born to consanguineously married parents.

• No family history of such illnesses. All siblings are healthy and alive.

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Examination

• Sick looking with marked pallor, irritable, dehydrated, vitals• R/R - 21/min O2 sats - 92%• H/R - 130/min B.P 100/60 mmHg • Temp – 37 C pulse 122/min• Anthropometric Measures

– Length - 80cm– Weight – 10 kg..

– Head size: 48 cm.

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Systemic Examination

• Resp : B/L clear

• GIT : Abdomen soft, non tender, no palpable flank mass, no Hepatosplenomegaly.

• CVS : S1 + S2 + O

• CNS : GCS 15/15, intact and irritable.

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DIFFERENTIAL DIAGNOSIS

• Acute renal failure. • Urosepsis • Hemolytic Uremic Syndrome.• Thrombotic thrombocytopenic purpura.• Acute transfusion reaction.

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EMERGENCY MANAGEMENT29/09/2014 2300Hrs

Catheterized for monitoring urine output. N/Saline 20ml/kg stat bolus. Inj Furosemide 20mg/kg IV given. PPD given I/D. PRBC’s transfusion 100ml followed by IV Frusemide

(Arranged).

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EMERGENCY MANAGEMENT29/09/2014 2300Hrs

• Treatment plan for floor:

• IV Fluid D51/5N/S @40ml/kg/day insensible fluid loss.• Replace urine output by N/S cc/cc.• Inj Sulzone (Cefoperazone+Sulbactum) 250mg B.I.D.• PRBC’s transfusion 100ml with IV furosemide 20mg

midtranfusion.

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LAB REPORTSNa 136 Hb 5.3

Cl 103 HCT 27.77

K 4.1 WBC 24300

HCO3 15 MCV 83.9

BSR 103 Platelets 465000

BUN/UREA 58/124 Neutrophils 63%

Creatinine 4.74 Lymphos 22%

S.Phosp 5.1 Monos 05%

S.Cholestrol 205

C3 1.25

Alk.phosp 270 Spot urine Creat 72.47

Uric Acid <1 mg/dl Spot urine Na 49

S,Albumin 3.4

FeNa 2.3 %

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Lab continued.URINE R/E

pH 6.0

Sp.Gravity 1.010

WBC/HPF 14-16/hpf

Protien3 plus

RBCS 6-8/hpf

Glucose Trace.

Leukocyte esterase

2 plus

Peripheral Film: Life threatening anemia with few nucleated RBC TLC is raised with differential showing few myeloid precursors. Platelets are normal on film. MP not seen.

USG KUB: Bilateral renal parenchymal disease grade II correlation with RFT suggested.

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01/10/2014

• Pallor improved, had edema • PPD=Negative • B.P 90/60 mmhg• Wt:10.5 kg.• Plan: • D/C IV Fluid.• Replace urine output by 1/2NS

cc for cc• Inj Furosemide 20 mg IV stat

CBC CHEM07

Hb 9.5 (prev 5.3)

Na 131

HCT 27.7 K 4.3

WBC 15000 Cl 105

PLATELETS

514000 HCO3 11

BUN/Urea

58/124

Creatinine

4.3

BSR 76

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02/10/2014.CHEM07

Na 128

K 4.5

Cl 102

HCO3 14

BUN/Urea 65/139

Creatinine 4.77 (prev 4.56)

BSR 65

Blood C/S

Pseudomonas Aeroginosa senitive to (cefoperazone sulbactum)

Urine C/S

No Growth after 48 hours.

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02/10/2014

• Child became irritable with 3 episodes of watery loose stool and abdominal pain since early morning.

• O/E periorbital puffiness and oral ulceration was present no scrotal edema

wt=10.5 kg B.P=90/60mmHg• 24 Hours urinary output 0.3ml/kg/hour.

• Plan • NS 250 ml IV stat• Inj Furosemide 20 mg after NS infusion.• Inj Methyl Prednisolone 300 mg OD over 1 hour for 3 days ( Strict B.P

monitoring during infusion)

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03/10/2014• Loose stool 2 episodes since morning.• Vomiting 1 episode.• O/E Periorbital swelling increased • B.P 95/60 mmHg.• 24 Hour urinary output 0.3ml/kg/hr ( same as yesterday)

• Plan: D/C IV Fluid • Replace urine output cc for cc with ½ NS • Inj Furosemide 20 mg IV stat.• Continue breast feeding

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04/10/2014.• 2 Episodes of loose motion • Oral intake improved • O/E Anasarca present.• Urine output 0.43ml/kg/hr• Wt: 12.1 kg B.P 90/55 mmHg.

• Plan: Furosemide 20mg iv stat.

Replace urine output with ½ NS cc/cc

Na 128

K 5.0

Cl 102

HCO3 13

BUN/Urea 80/171

Creatinine 5.22

Glucose 100

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05/10/2014.

Hb 9.1 Na 127

HCT 25.81 K 4.8

MCV 81.9 Cl 100

PLATELETS 557000 HCO3 09

RETICS 04 BUN/Urea 90/112

Creatinine 5.36

CRP 7.12 g/dl BSR 98

S.Phosp 8.6

S,Ca 6.4

LDH 836

C-ANCA Negative

P-ANCA Negative

ANA Negative

ASMA Negative

Peripheral Film revealed moderate normocytic normochromic anaemia with mild rouleaux formation. TLC normal with differential showing few reactive lymphos. Platelets are adequate no MP seen.

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05/10/2014• 3 Episodes loose motions.• Wt: 13 kg • Urine output 0.36 ml/kg/hr.• B.P 100/60 mmHg.

• Plan:• Inj Furosemide 20 mg iv stat.• Only minimal Breast feed no extra milk/diet/fluid..

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06/10/2014• Generalized edema• Loose motions 2 episode.• Wt : 12.7 kg B.P 100/60 mmHg.• Urine output: 0.3ml/kg/hr.• Plan:• Inf NS 150 ml iv stat.• Replace 100 cc ½ NS after 2 loose stool • Replace urine out cc/cc with ½ NS.

Na 123

K 4.1

Cl 99

HCO3 12

BUN/Urea 102/218

Creat 5.17

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07/10/2014• Loose motion improved.• Afebrile and edema improved.• Wt:12.9 kg B.P 95/60 mmHg• Urine out put 0.73ml/kg/hr.• Plan: D/C IV Sulzone.• NS 200ml iv stat• inj Furosemide 20mg after NS.• Replace urine output cc/cc using ½ NS• Continue mother feed.

Na 121

K 4.1

Cl 98

HCO3 10

BUN/Urea 104/228

Creatinine 5.06

BSR 84

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08/10/2014• Still edematous.• Wt: 13 kg. B.P 100/60 mmHg• Urine out put = 0.85ml/kg/hr.• Plan: • Inj furosemide 20mg iv stat.• Continue mother feed restrict po feed.• Replace urine output cc/cc with ½ NS.

Hb 8.8 Na 115

HCT 24.4 K 4.0

MCV 82.6 Cl 96

PLATELETS

534000

HCO3 08

BUN/Urea

107/228

Retic 3 Creat 5.06

CRP 11.83 BSR 81

LDH 730

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09-11/10/2014

CHEM07 11/10/2014

Na 114

K 3.8

Cl 96

HCO3 06

BUN/Urea 104/222.8

Creat 5.13

BSR 97

CHEM07 10/10/2014

Na 114

K 4.4

Cl 94

HCO3 07

BUN/Urea 109/228

Creat 5.25

BSR 68

CHEM07 09/10/2014

Na 113

K 4.1

Cl 95

HCO3 08

BUN/Urea 107/228

Creatinine 5.12

BSR 81

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09-11/10/2014• On 11th day of admission patient urine output improved

i.e.1.31 ml kg/hr.• B.P Systolic range 95-110 Diastolic 55-75mmHg• Repeated Urine and blood cultures yielded no growth.

• Urinary Catheter was removed and urine bag applied for output monitoring.

• Syp Calcium- P 5cc OD started.USG KUB : Bilateral enlarged swollen kidneys with increased parenchymal echogenecity (grade I-II).

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12/10/2014.

• Still Edematous• Scrotal edema • Wt: 13.6 kg

B.P 100/60mmHg

• Plan: IVFluid NS @35cc/hr.• Syp KCl 13.4mEq/5 cc p/o stat.

Na 117

K 3.5

Cl 96

HCO3 11

BUN/Urea 106/226

Creat 4.99

BSR 87

Ca 5.3

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13/10/2014• Still edematous • scrotal swelling present • Wt: 13.2 kg. B.P100/70 mmHg• Urine output 1.9ml/kg/hr

• Plan: • NS @ 45ml/hr.( rate increased)• Syp kCl 5cc 2 doses at 12 hour interval.

Na 121

K 3.4

Cl 99

HCO3 09

BUN/Urea 96/205

Creat 4.54

BSR 80

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14/10/2014• Edema improved • Oral intake also improved • Urine output 2.05 ml/kg/hr• Loose stool 2 episodes • B.P. 105/70 mmHg

• Plan• 120ml Pedialyte (ORS) after every loose stool..

Na 138

K 5.1

Cl 113

HCO3 08

BUN/Urea 84/179

Creat 3.63

BSR 80

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15-16/10/2014• Remained edematous • Loose stool 6-7 episodes on 15/10 which setteled on 16. • ORS given scrotal support provided for edema.

B.P= 100/70 mmHg

• On 16/10 iv fluid were hold.• Inj Furosemide 20 mg stat given.

Hb 7.4 Na 143

HCT 21.7 K 4.2

WBC 9500 Cl 120

MCV 84.8 HCO3 09

PLATELET 337000 BUN/Urea 60/128

Creat 2.38

BSR 78

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CHEM07 17/10/2014 CBC

Na 142 Hb 7.4

K 4.2 HCT 21.4

CL 117 MCV 84.9

HCO3 10 Platelets 292000

BUN/Urea 50/107 WBC 12000

Craet 1.94

BSR 88

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19/10/2019

• Edema free/Scrotal swelling resolved. • Adequate oral intake/ mother feed.• Adequate urine output

loose stool improved.• Wt: 10.8 kg• B.P 120/75mmHg • Discharged home on Multiviatamins.• Follow-up 27/10/2014.

CHEM07 19/10/2014

CBC

Na 140 Hb 8.1

K 4.2 HCT 24.3

Cl 110 MCV 85

HCO3 14 WBC 10100

BUN/urea

33/70 Platelets

289000

Creat 1.11

BSR 77

LDH 636

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FOLLOW-UP 27/10/2014CHEM07

CBC

Na 139 Hb 8.9

K 4.7 HCT 26

CL 108 MCV 86.1

HCO3 19 WBC 6300

BUN/Urea

20/42 Platelet 677000

Creat 0.54 S.Cholestrol

204

BSR 78 S.Albumin

4.9

URINE R/E

Ph 6.0

Sp.gravity 1.015

Wbc 1-2/hpf

Epi cells 0-1/hpf

Nitrites --

Leukocyte esterase --

Sugar/albumin --

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On Follow up

• Runny nose • Cold flu symptoms.• No fever no vomiting no loose motion

• Symptomatic treatment for URI• Multivitamins given.

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Hemolytic-uremic syndrome

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Definition

• HUS, is a disease characterized by : Microangiopathic Hemolytic anemia Renal failure Low platelet count

• It predominantly, but not exclusively, affects children.

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Types HUS

• Typical HUS D-

• Atypical HUS D+

• HUS due to Complement abnormalities

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ETIOPATHOGENESIS

• Typical/Diarrhea associated/Shiga Toxin

associated HUSEnterohaemorrhagic E. coliShigella dysenteriae type 1Rarely, HUS can occur with E. coli UTI

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CONTI..

• The common serotype of E coli:0157:H7• However, only about 10-15% patients with E. coli

0157:H7 infection will develop HUS• Sources of infection are :

Milk and animal products (incompletely cooked beef, pork, poultry,lamb)

Human feco-oral transmissionVegetables, salads and drinking water may be contaminated

by bacteria shed in animal wastes

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Atypical/Non-Diarrhea Related HUS

Pneumococcal HUS

HUS due to Complement abnormalities

Miscellaneous Causes of HUS / TTPAbnormalities in intracellular vitamin B12 metabolismHIVSystemic lupus erythromatosusMalignanciesRadiation Certain drugs

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Other infections associated with HUS

• Include viruses like :InfluenzaCytomegalovirus Infectious mononucleosis

• Bacteria like: Streptococcii Salmonella

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CONTI…

• The typical pathophysiology involves the shiga-toxin binding to proteins on the surface of glomerular endothelium and inactivating a metalloproteinase called ADAMTS13, which is also involved in the closely related TTP

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CONTI..

• The arterioles, capillaries become obstructed by the resulting complexes of activated platelets which have adhered to endothelium via large multimeric vWF.

• The growing thrombi destroy RBCs as they squeeze

through the narrowed blood vessels, forming schistocytes, or fragments of sheared RBCs.

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CONTI…

• The consumption of platelets as they adhere to the thrombi typically leads to mild or moderate thrombocytopaenia

• However, in comparison to TTP, the kidneys tend to be more severely affected in HUS, and the central nervous system is less commonly affected

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CLINICAL FEATURES

• The commonest clinical presentation of HUS is : Acute pallor Oliguria Diarrhea or dysentery

• It occurs commonly in children between 1-5 years of age

• HUS develops about 5-10 days after onset of diarrhea

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CONTI..

• Hematuria and hypertension are common.• Complications of fluid overload may present with:

Pulmonary edema Hypertensive encephalopathy

• Despite thrombocytopenia, bleeding manifestations are rare • Neurological symptoms like:

Irritability Encephalopathy Seizures

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INVESTIGATIONS

• CBC• Peripheral blood smears• Reticulocyte count• LDH• Bilirubin unconjugated• Cr & BUN• Urine analysis

HemoglobinuriaHematuria Proteinuria

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Schistocytes

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Investigations to Identify Cause

• In patients with diarrhea, the identification of pathogenic EHEC or Shigella is performed by: Stool culture Further serotyping by agglutination or enzyme immunoassay

• Rarely HUS can occur with E. coli UTI: Urine cultures are indicated in non-diarrheal patients

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Conti..

• Bacteriological cultures of body fluids are indicated in suspected pneumococcal disease.SputumCSFBloodPus

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Diagnosis

• Clinically, HUS can be very hard to distinguish from TTP• The laboratory features are almost identical, and not

every case of HUS is preceded by diarrhea• HUS is characterized by the triad of:

Hemolytic anemia ThrombocytopeniaAcute renal failure

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Cont…

– The only distinguishing feature is that in TTP fever and neurological symptoms are often present, but this is not always the case

– The two conditions are sometimes treated as a single entity called TTP/HUS.

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MANAGEMENT

• Supportive Therapy• Antibiotics.• Plasma Therapy• Miscellaneous

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Supportive Therapy

• In all patients, supportive treatment is primary. • Early recognition of disease• Close clinical monitoring of :

Fluid statusBlood pressureDialysis Blood transfusion

• Blood levels of glucose, electrolytes, creatinine and CBC need frequent monitoring

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CONTI..

• With the onset of acute renal failure :Fluid restriction Diuretics No platelets transfusion despite thrombocytopenia.Anticoagulation ,antiplatelets and fibrinolytics

contraindicated.Antibiotics (controvercial)

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Plasma Therapy

• In aHUS due to : complement factor abnormality ADAMTS13 deficiency

• The replacement of the deficient factor with FFP

• Daily plasma infusions (10 to 20 mL/kg/day)

• Plasmapharesis is proposed for those having CNS symptoms with HUS but there is no data on its effectiveness.

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Prognosis

• Less than 5 percent mortality in major medical centers• Half patient require dialysis during acute phase.• 5 percent remain dialysis dependant.• Pneumococcal associated HUS is associated with

increased mortality upto 20 percent.

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KEY MESSAGES

• Good sanitation and maintenance of food hygiene can prevent diarrhea associated HUS.

• Supportive care with early dialysis support remains the cornerstone of management.

• Non-infective atypical HUS should be treated rapidly with plasma therapy.

• Efforts should be made to make an etiological diagnosis in cases of atypical HUS as treatment and prognosis is affected.

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Dialysis

• Indications  Volume overload with evidence of hypertension and/or

pulmonary edema refractory to diuretic therapy  Persistent hyperkalemiaSevere metabolic acidosis unresponsive to medical

managementNeurologic symptoms (altered mental status, seizures)Blood urea nitrogen >100-150 mg/dL (or lower if rapidly rising)Calcium : phosphorus imbalance, with hypocalcemic tetany

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THANKS.