خانم دکتر فاطمه مهتا بصیر
عضو هیئت علمی دانشگاه

فوق تخصص نوزادان

به اتفاق اعضای هیئت علمی

بخش کودکان بیمارستان شهدای تجریش

پاسخ:

تشخيص هاي افتراقي:

خانم دکتر فاطمه مهتا بصیر
عضو هیئت علمی دانشگاه

فوق تخصص نوزادان

به اتفاق اعضای هیئت علمی

بخش کودکان بیمارستان شهدای تجریش

Immune Thrombocytopenic Purpuras &  Pregnancy 

Maternal Disease

~ 1-2 cases / 1000 pregnancies

Platelets count < 20.000 :   treatment needed. if > 50.000 : NVD or C/S

Mother's treatment by IVIG, can not treat fetus.

 10% to 20% fetus are affected. Intra-uterine  fetal hemorrhage have not been reported.

Management of the newborn

Careful follow-up during the first week: after birth platelets count may  continue to decrease.

US imaging of brain if platelets < 50.000

platelets < 20.000 : transfusion and/or IVIG

IC hemorrhage or any other bleeding manifestation : transfusion and IVIG  or glucocorticoid therapy.

Hematuria in Newborn Babies

Causes

Most frequent : A T N  [ perinatal asphyxiation, sepsis, nephrotoxic drugs]

Renal Venous Thrombosis [ gestational diabetes, congenital cyanotic heart diseases, indwelling venous umbilical catheter]

UTI and other causes : trauma, neoplasia , abnormalities of coagulation  and thrombocytopenia

Differential diagnosis

Extraurinary sources ( vaginal, prepuce, perineal, rectal)

Myoglobinuria ( inherited metabolic myopathy , infectious myositis, rhabdomyolysis ) dipstick +

Hemoglobinuria ( erythroblastosis fetalis or other hemolysis)

Pigmenturia ( bile, urate crystal , porphyrin)  dipstick  -

معرفي كيس:

معرفی کیس:اقاي دكترسامعي

رزيدنت بيمارستان شهدا

تشخیص های افتراقی:

اقاي دكتر هادي جعفري تبار

رزيدنت بيمارستان مفيد

خانم دكتر نوشا محمدي

رزيدنت بيمارستان امام حسين

اقاي دكتر هادي جعفري تبار

رزيدنت بيمارستان مفيد

بسم الله الرحمن الرحیم

چهارشنبه 4/8/90

دکترهادی جعفری تبار

Mother ITP=?

Because an ITP-like syndrome can be seen in patients with HIV or hepatitis C infection.

Secondary ITP may be induced by Drugs or occur in patients with Collagen vascular disease .

the diagnosis can be confirmed by appropriate laboratory and radiologic studies

Thrombocytopenia in neonate:

DEFINITION :

Historically, neonatal thrombocytopenia has been defined as a platelet count less than 150,000/microL based upon the definition used in adults, which corresponds to values at or below the 5th percentile. However, healthy preterm and term newborns have counts significantly below this level.

CLASSIFICATION :

Platelet size : large, normal, small

Mode: acquired or congenital

Pathologic mechanism involved

Increased consumption of platelets

Immune thrombocytopenia (Autoimmune, Alloimmune,  Drug-induced Peripheral consumption)

Hypersplenism ( Kasabach-Merritt syndrome, Disseminated intravascular coagulation , Infection, Drug toxicity, Procedure-related, following exchange transfusion )

Miscellaneous (Neonatal cold injury, Von Willebrand disease

Decreased production of platelets

 Congenital thrombocytopenias ,Infiltrative disorders Infections: bacterial, viral, or fungal Drug toxicity

Causes of neonatal thrombocytopenia:

Destructive mechanisms , Immune-mediated thrombocytopenia: ( The most common cause)

Immune (idiopathic) thrombocytopenic purpura 

Neonatal alloimmune thrombocytopenia 

Drug-induced thrombocytopenia (heparin, valporate,…)

Autoimmune disorders (APS , SLE)

Sequestration and trapping  (hypersplenism)

Mechanical destruction(hemodialysis, cardiopulmuonary bypass)

 Platelet activation and consumption(TTP, HUS)

Impaired PLT production:

Infection (EBV, CMV, Varicella, Parvovirus)

Cyanotic heart disease

Bone marrow failure or infiltration (aplastic anemia ,chemotherapy…)

Nutritional deficiencies (Vit B12, Folat, Fe)

Genetic cause:

TAR syndrome 

 Congenital amegakaryocytic thrombocytopenia

Wiskott-Aldrich syndrome 

Giant platelet disorder:( Bernard-Soulier syndrome, gray platelet syndrome)

Miscellaneous :

Phototherapy

Perinatal aspiration syndrome

Persistent pulmonary hypertension

Rhesus alloimmunization

Polycytemia

Metabolic

Post exchange transfusion

Maternal HELLP syndrome

Approach to the thrombocytopenic newborn infant:

Well  Infant:

Maternal History:

History of immune thrombocytopenic purpura or systemic lupus erythematosus?

Previous infant with thrombocytopenia or family history of thrombocytopenic neonate?

Infections during pregnancy?

Drug use during pregnancy?

Maternal platelet count

Decreased, suggests autoimmune

Normal, may be autoimmune or alloimmune process

Neonatal examination:

 Does the child look abnormal?

Thrombocytopenia with absent radii Fanconi anemia

Sick Infant:

Neonatal history:

 Is there a history of perinatal asphyxia?

Does the child have meconium aspiration syndrome or respiratory distress syndrome?

Is there evidence of infection?

Necrotizing enterocolitis Sepsis/disseminated intravascular coagulation

Does the child look abnormal?

 Toxoplasmosis, rubella, cytomegalovirus, herpes simplex Chromosome abnormality

 Is the child small for gestational age?

Could the child have a thrombus?

 Were there indwelling catheters?

Did the child have an intracranial hemorrhage?

Maternal history:

Prenatal or perinatal infection

Preeclampsia, hemolytic anemia, elevated liver enzymes, low platelet count

Abnormal placenta

Neonatal examination :

Head circumference, weight percentile

 Congenital anomalies: radial rays, thumbs, dysmorphic features

 Hepatosplenomegaly

Blueberry muffin rash

Hemangioma

Neonatal laboratory values:

 Abnormalities in the white blood cell count or hematocrit Hyperbilirubinemia or liver function test abnormalitites

Evidence of coagulopathy or DIC

Hematuria:

Hematuria is the medical term for blood in the urine. (presence of more than 5 RBC's in repeated urinary sediments.)

Gross hematuria : means that blood can be seen in the urine with the naked eye because it turns the urine pink, red, or tea-colored. If you see blood in your child's urine, you should call your child's healthcare provider.

Microscopic hematuria : means that the urine is normal in color, but it has an increased number of red blood cells (blood) as seen with a microscope. If your child's urine dipstick shows blood in the urine, the urine should be examined with a microscope to confirm that blood is present.

Causes of Hematuria(gross):

Trauma Causes

Iatrogenic, Self Induced Disorders

Surgical, Procedure Complication

Infectious Disorder (Specific Agent)

Infected organ, Abscesse

Neoplastic Disorders

Allergic, Collagen, Auto-Immune Disorders

Metabolic, Storage Disorders

Biochemical Disorders

Congenital, Developmental Disorders

Hereditary, Familial, Genetic Disorders

Usage, Degenerative, Necrosis, Age Related Disorders

Anatomic, Foreign Body, Structural Disorders

Drugs

Heirarchical Major Groups(Urinary tract disorders )

Reference to Organ System

Poisoning (Specific Agent)

Causes of heme-negative red urine

Medications :

Doxorubicin, Chloroquine ,Deferoxamine, Ibuprofen, Iron, sorbitol Nitrofurantoin, Phenazopyridine, Phenolphthalein, Rifampin,

 Food dyes:

Beets (in selected patients)

Blackberries ,Food coloring

Metabolities:

Bile pigments, Homogentisic acid, Melanin, Methemoglobin ,Porphyrin Tyrosinosis, Urates

Causes in Neonates:

common

Thrombosis (renal vein or artery)

Nephrolithiasis (including hypercalciuria)

Obstruction(hydronephrosis , PUV)

Reflux

Cystic disease

Syphilis(congenital)

meatal or urethral bleeding

Rare

Mesoblastic nephroma

Wilms tumor

Factious causes

sponge kidney

Coagulation abnormalities and hemoglobinopathies

Algorithm for gross or symptomatic microscopic hematuria

Diagnostic algorithm for asymptomatic microscopic hematuria with proteinuria

Algorithm for isolated asymptomatic microscopic hematuria

Thank for attention

خانم دكتر نوشا محمدي

رزيدنت بيمارستان امام حسين

نوزاد 8 روزه با هماچوری

نوزاد پسر 8 روزه ای با شکایت هماچوری واضح مراجعه میکند.

نوزاد حاصل بارداری ترم GA=38w و سزارین میباشد که با آپگار مناسب متولد میگردد.

مادر G2P2Ab0 میباشد و مبتلا به ITP از 4 سال قبل که تحت درمان نامنظم با پردنیزولون و فاموتیدین بوده است.

نوزاد شب قبل از مراجعه 3 نوبت هماچوری واضح داشته است. در صبح روز بستری کمی بیحال بوده ولی علایم شوک و کاهش پرفوزیون محیطی نداشته است.

در معاینه پوست و تنه ایکتریک بوده ولی پتشی و پورپورای قابل ملاحظه نداشته است.

ژنیتالیا طبیعی و ختنه نشده است.

سایر معاینات طبیعی میباشد.

وزن تولد = 2920   وزن هنگام مراجعه = 2950

Lab test, first day:

U/A :  colour= red     SG=1008   PH= 7    Pr= 3+

Glu=  -   Ket=  -    Blood=3+      WBC=2-3          RBC= many     LE=  -

CBC/Diff  :     Hgb=14.1    Hct=38.9  MCV=93.5

MCH=33.9    PLt=49000                                      WBC=11.600   N=48%  l=50%                                                                M=2%-

Bil=12.3      Dir=0.5

Lab TEST, SAME DAY :

U/A :    colour=yellow  app= clear  PH=6.5                 SG=1002   Pr=trace   WBC=5-6  RBC=1-2

bac=  -       U/C=   -

CBC/Diff  :       Hgb=12.4   Hct=33.2  Pt=56000

WBC=14000   N=45%  l=55%                 Ret=1.3%   ESR=5  CRP=3mg/dl

BS=65   BUN=8   Cr=0.3  Na=144  K=5.7 Ca=10.5

  Bil=12.8  Dir=0.4    PT=14   PTT=29  INR=1.2

G6PD=suff

DAY 2 :   U/A :  WBC=5-6   RBC=1-2   

DAY 3 :    U/A :  WBC=2-3    RBC=1-3

Day 4 :     morning=  U/A :   WBC=3-5   RBC=6-8

Blood=+1

Evening=   U/A :    WBC=2-3   RBC=2-3

Blood=+1

DAY 6 :    U/A :  WBC=0-1    RBC=0-1

DAY 7 :    U/A :  WBC=2-3  RBC=3-5  Blood=trace

DAY 10 :   U/A :  PH=7   SG=1010  Pr= -   Glu= -

Bil= -  Crystal=-  Cast= -  Blood= -

App=clear  WBC=0-1   RBC=0-1

Day 1 :           Hgb=12.4                 Plt=50,000

Day 3 :           Hgb=10.9                 Plt=97,000

Day 4 :           Hgb=11.7                 Plt=114,000

Day 5 :           Hgb=10.1                 Plt=97,000

Day 6 :           Hgb=10,5                 Plt=141,000

هماچوری ماکروسکوپیک دو نوبت در بیمارستان مشاهده شد که در هر دو نوبت پلاکت بیمار بیش از 100000 بود.

گرافی توراکوابدومن نرمال بوده است.

سونوگرافی مغز نرمال.

سونوگرافی کلیه در روز سوم بستری: کلیه راست=45mm

کلیه چپ=46mm و ضخامت پارانشیمال طبیعی.شواهدی به نفع سنگ یا ضایعه فضاگیر نداشت.قطر لگنچه در کلیه چپ 32mm AP میباشد که نشاندهنده mild stasis است.

شواهدی به نفع هیدرونفروز در کلیه راست دیده نمیشود.

Diff Diagnosis :

ATN

Cortical necrosis

Vascular disease  (RVT , RAT )

Bleeding & Clotting disorder

Urological Abnormalities

UTI

Glomerular Disease

Tumors   ( Wilms , Neuroblastoma ,Angioma)

Nephrocalcinosis

Trauma