1. Hemorrhagic disease of the newborn can be caused by several inherited and acquired conditions that impair the blood's ability to clot normally, including hemophilia, von Willebrand disease, liver disease, vitamin K deficiency, and others.
2. Vitamin K deficiency bleeding is the most common cause and occurs when newborns do not receive sufficient vitamin K, which is essential for the production of clotting factors. Symptoms can range from mild bruising to life-threatening internal bleeding.
3. Hemophilia is an inherited bleeding disorder where affected newborns are unable to form clots due to deficiencies in specific clotting factors like VIII or IX. Treatment focuses on
Haemorrhagic and Haemolytic of Newborn DiseasesNaqib Bajuri
actually for haemorrhagic newborn diseases, mainly focus of vit K def...the other is for revision n more commonly occur in child n adults....for haemolytic newborn disease, mainly focus on Rh disease n ABO incompatibility.....the other when childhoods
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Definition of neonatal sepsis,type of neonatal sepsis ,early onset neonatal sepsis,late onset neonatal sepsis,Pathophysiology of neonatal sepsis,,sign and symptoms of neonatal sepsis, diagnosis of neonatal sepsis,management of neonatal sepsis, antibiotic used for neonatal sepsis,prevention of neonatal sepsis, prognosis of neonatal sepsis ,and A summary
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One of the most developed cities of India, the city of Chennai is the capital of Tamilnadu and many people from different parts of India come here to earn their bread and butter. Being a metropolitan, the city is filled with towering building and beaches but the sad part as with almost every Indian city
Defecation
Normal defecation begins with movement in the left colon, moving stool toward the anus. When stool reaches the rectum, the distention causes relaxation of the internal sphincter and an awareness of the need to defecate. At the time of defecation, the external sphincter relaxes, and abdominal muscles contract, increasing intrarectal pressure and forcing the stool out
The Valsalva maneuver exerts pressure to expel faeces through a voluntary contraction of the abdominal muscles while maintaining forced expiration against a closed airway. Patients with cardiovascular disease, glaucoma, increased intracranial pressure, or a new surgical wound are at greater risk for cardiac dysrhythmias and elevated blood pressure with the Valsalva maneuver and need to avoid straining to pass the stool.
Normal defecation is painless, resulting in passage of soft, formed stool
CONSTIPATION
Constipation is a symptom, not a disease. Improper diet, reduced fluid intake, lack of exercise, and certain medications can cause constipation. For example, patients receiving opiates for pain after surgery often require a stool softener or laxative to prevent constipation. The signs of constipation include infrequent bowel movements (less than every 3 days), difficulty passing stools, excessive straining, inability to defecate at will, and hard feaces
IMPACTION
Fecal impaction results from unrelieved constipation. It is a collection of hardened feces wedged in the rectum that a person cannot expel. In cases of severe impaction the mass extends up into the sigmoid colon.
DIARRHEA
Diarrhea is an increase in the number of stools and the passage of liquid, unformed feces. It is associated with disorders affecting digestion, absorption, and secretion in the GI tract. Intestinal contents pass through the small and large intestine too quickly to allow for the usual absorption of fluid and nutrients. Irritation within the colon results in increased mucus secretion. As a result, feces become watery, and the patient is unable to control the urge to defecate. Normally an anal bag is safe and effective in long-term treatment of patients with fecal incontinence at home, in hospice, or in the hospital. Fecal incontinence is expensive and a potentially dangerous condition in terms of contamination and risk of skin ulceration
HEMORRHOIDS
Hemorrhoids are dilated, engorged veins in the lining of the rectum. They are either external or internal.
FLATULENCE
As gas accumulates in the lumen of the intestines, the bowel wall stretches and distends (flatulence). It is a common cause of abdominal fullness, pain, and cramping. Normally intestinal gas escapes through the mouth (belching) or the anus (passing of flatus)
FECAL INCONTINENCE
Fecal incontinence is the inability to control passage of feces and gas from the anus. Incontinence harms a patient’s body image
PREPARATION AND GIVING OF LAXATIVESACCORDING TO POTTER AND PERRY,
An enema is the instillation of a solution into the rectum and sig
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4. Introduction
Hemorrhagic disease is a bleeding problem that occurs
in a baby during the first few days of life.
The normal range for the new-born prothrombin
(PT)and partial thromboplastin time (PTT) extend
above than those for a healthy adult.
But there is NO increased risk of bleeding in a
healthy newborn.
5. Neonatal haemostatic system
• At birth, concentrations of the vitamin K dependent
(FII,FVII, FIX, FX) and factors (FXI, FXII) are reduced to
about 50% of normal adult values and are further
reduced in preterm infants (Hemorrhagic Disease of
Newborn).
6. Cont…
Concentrations of the naturally occurring
anticoagulants, antithrombin, protein C, S and Z are
low at birth and as a consequence, both thrombin
generation and thrombin inhibition are reduced in
the newborn period.
• Plasminogen is also low in neonatal period
resulting in a relatively hypofibrinolytic state.
8. COMMON hemorrhagic disease of the
newborn
inherited • Haemophilia A and B
• Von- willebrand disease
• Afibrinogenesis
• Liver disease
• Vit k deficiency
• DIC
• thrombocytopnia
acquired
9. Hemophilia
• Hemophilia is an inherited bleeding disorder.
• Newborn with hemophilia can’t stop bleeding b/c
they don’t have enough clotting factor in blood.
There are many blood clotting factors involved in
forming of clots to stop bleeding.
Two common factors that affect blood clotting are
factor VIII and factor IX.
10. The 3 main forms of hemophilia are:
• Hemophilia A. caused by a lack of the blood clotting
factor VIII. referred as to classic hemophilia /factor
VIII deficiency.
• Hemophilia B. caused by a deficiency of factor IX.
Referred as Christmas disease or factor IX deficiency.
• Hemophilia C. caused by lack of factor XI referred to
factor XI deficiency .
11. causes hemophilia in newborn
Hemophilia types A and B are inherited diseases. They
are passed from parents to children through a gene
on the X chromosome.
• Females have two X chromosomes, while males have
one X and one Y chromosome.
• A female carrier has the hemophilia gene on one of
her X chromosomes.
12. Cont…
• When a hemophilia carrier female is pregnant.
there is a 50/50 chance that hemophilia gene will be
passed on to the baby.
• If the gene is passed on to a son, he will have the
disease but If the gene is passed on to a daughter, she
will be a carrier.
13. The sign and symptoms of hemophilia
Bleeding into the joints.
Bleeding into the skin (which is bruising)
muscle and soft tissue (called a hematoma).
Bleeding of the mouth , nose and gums,
bleeding that is hard to stop after losing a tooth.
Blood found in the urine or stool
Hemophilia C usually doesn’t cause problems ,but
may have increased bleeding after surgery.
14. Diagnosis of hemophilia
The diagnosis of hemophilia is based on patient`s
family history and a physical exam.
Blood tests include
Complete blood count (CBC). It includes RBC, WBC
platlate, hemoglobin and hematocrit
• Clotting factors. levels of each clotting factor.
• Bleeding times. To test the speed that blood clots.
• Genetic or DNA testing. for abnormal genes
15. Hemophilia management
Treatment for hemophilia is aimed to preventing
bleeding complication (mainly head and joint bleeds).
Bleeding in the joint may need surgery or
immobilization. The rehab of the affected joint
include physical therapy and exercise to strengthen
the muscles of affected area.
• Blood transfusions may be needed if major blood
loss has occurred.
16. Cont…
Self-infused factor VIII or IX can allow a newborn
with hemophilia to lead a near normal lifestyle.
Factor VIII concentrates:
• Hemofil M(Baxter)
• Immunate(Baxter)
• Koate-DVI(Bayer)
Factor IX concentrates:
• Immunine(Baxter)
• Aimafix(Kedrion
17. Complications of hemophilia
• hemarthrosis
• inflammation and Long-term joint problems
• Very serious tumor-enlargements, muscle /bone
• Development of antibodies against clotting factors
• Infections from transfusions
• Hematoma
• Spontaneous nosebleed
18. Von Willebrand disease (VWD)
VWD is a genetic disorder caused by missing or
defective Von Willebrand factor (VWF), a clotting
protein. VWF binds factor VIII, a key clotting protein,
and platelets in blood vessel walls, which help form a
platelet plug during the clotting process.
• VWD is most common inherited bleeding disorder
• It is carried on chromosome 12 and occurs equally
in men and women
19.
20. Sign and symptom of VWD
• bleeding from the gums.
• easy bruising.
• prolonged bleeding after cuts or bloodworm.
• frequent and prolonged nosebleeds.
• blood in the stool or urine.
Babies with VWD rarely bleed at birth. Babies with
Type 3 VWD, surgery, including circumcision
• soft tissue/joint bleeding (in more severe forms).
21.
22. Diagnosis of VWD
76% of men with VWD had been diagnosed by age 10,
but 50% of women with VWD were not diagnosed
until after age 12.
• the plasma VWF protein level
•Some subtypes of type II disease are associated
with thrombocytopenia, which may be apparent
during the neonatal period and may result in
bleeding.
23.
24. Liver Disease
• Biliary obstruction impaired vit K absorption
decrease synthesis FII,FVII,FIX and X.
• Severe hepatocellular dz, reduced FV, fibrinogen
& plasminogen activator.
• Dysfunctional fibrinogen (dysfibrinogenaemia)
• Low thrombopoietin production l/t
thrombocytopenia
• Hypersplenism associated with portal HTN l/t
thrombocytopenia
25. Vitamin K Deficiency Bleeding or VKDB
refers to bleeding that occurs as a consequence of
vitamin K deficiency during the first six months of life.
Vitamin K refers to a group of fat-soluble vitamins
that play a role in blood clotting,
bone metabolism, and
regulating blood calcium level
26. Cont…
Coagulation factors II, VII, IX, X and other Gla-proteins
( protein C, protein S, protein Z) also depend on the
presence of vitamin K for their activity.
vitamin K–dependent factors is characterized by
bleeding that tends to be gastrointestinal, nasal,
subgaleal, intracranial, or post-circumcision.
▪
27. Classification
Early-VKDB
▪ Onset: 0-24 hr
▪ Incidence: Rare
▪ Site: Cephalohematoma, Subgaleal, Intracranial,
Gastrointestinal, Umbilicus, Intra-abdominal.
▪ Etiology: Maternal drugs (Phenobarbital, phenytoin,
warfarin, rifampin, isoniazid) that interfere with vit K.
▪ Risk factor: Inherited coagulopathy.
28. CONT…
Classical-VKDB
▪ Onset: 2-7 days
▪ Incidence: ≈2% if infant not given vitamin K.
▪ Site: Gastrointestinal, Ear-nose-throat-mucosal,
Intracranial, Circumcision, Coetaneous, Injection sites.
▪ Etiology: Vitamin K deficiency, Breast-feeding.
29. Conti..
Late-VKDB Onset:1-6 month
▪ Incidence: Dependent on primary disease.
▪ Site: Intracranial, Gastrointestinal, Coetaneous, Ear-
nose-throat mucosal, Injection sites and Thoracic.
▪ Etiology : Cholestasis—malabsorption of vitamin K
(Biliary atresia, cystic fibrosis, hepatitis)
▪ Risk factor : Abetalipoprotein deficiency, Idiopathic
in Asian breastfed infants, Warfarin ingestion.
30. causes vitamin K deficiency
– Minimal transplacental passage of vitamin K
– Limited hepatic storage of vitamin k in newborn
– Low concentration of vitamin k in breast milk
– Absence of the bacterial intestinal flora normally
responsible for the synthesis of vitamin K
not receive a vitamin K shot at birth.
31. presentations of VKDB
Bruises on the baby's head;
External bleeding, especially from the nose or
umbilical cord;
Unnatural skin coloring.
Vomiting blood.
Stool that is dark and sticky.
irritability, convulsions, poor reflexes and poor
feeding.
32. Investigation
▪ Coagulation profile : Prothrombin time (PT),
activated partial thromboplastin time (aPTT),
fibrinogen levels, and a platelet count should be
included in the initial workup for VKDB in a newborn.
– A prolonged PT is usually the first laboratory test
result will be abnormal in VKDB
– Normal aPTT, fibrinogen levels and a platelet count .
– Factor assay.
35. Types of vitamin k
K1: Phylloquinone is predominantly found in green
leafy vegetables, vegetable oils, and dairy products. ...
K2: Menaquinone is synthesized by gut flora.
K3: Menadione is a synthetic, water soluble form that
is no longer used medically because of its ability to
produce hemolytic anemia.
36. Management
The disease may be effectively treated with a slow IV
infusion of 1-5 mg of vitamin K1.
▪ Serious bleeding, particularly in premature infants or
those with liver disease, may require a transfusion of
fresh frozen plasma or whole blood.
37. CONT…
Surgical Care
▪ Normally, VKDB infants do not require surgical care
but in rare cases, an infant may need neurosurgical
evaluation and treatment.
▪ Other conditions, such as those associated with
short bowel syndrome and Hepatobiliary disease may
require surgical evaluation
38. Complication
▪ Intracranial hemorrhage is the primary serious
complication of VKDB
▪ Complications of treatment include anaphylactoid
like reactions during intravenous (IV) vit K admin,
hyperbilirubinemia or hemolytic anemia after high
doses of vitamin K, and hematomas at the site of
injection.
39. Prevention
▪ Early-VKDB : Administrations of vitamin K to infant
at birth or to mother (20 mg) before birth.
▪ Classical-VKDB: Parenteral vitamin K at birth.
▪ Late-VKDB: Parenteral and high-dose oral vitamin K
during periods of malabsorption or cholestasis.
40. Disseminated intravascular coagulation
DIC is an acquired syndrome characterized by
excessive systemic activation of coagulation, resulting
in both hemorrhage and thrombosis. DIC can progress
rapidly into life-threatening multiorgan failure
DIC always occurs as a secondary event, and a
number of prenatal and neonatal problems are
associated with.
42. The laboratory diagnosis of DIC
typical pattern of reduced platelets,
PT, APTT with or without thrombin clotting time),
reduced fibrinogen, and increased D-dimers
Although this pattern is likely to be present in a
neonate with fulminating DIC, findings can vary, and a
number of factors complicate the diagnosis during the
neonatal period.
43. manifestations of DIC in neonate
Bleeding , bruising , low blood pressure, shortness
of breath, confusion.
Managemanet
Treatment by underlyding cause
Fresh frozen plasma (10–15 ml/kg) can be used to
replace haemostatic proteins,
cryoprecipitate (5–10 ml/kg) is a better source of
fibrinogen, which should be kept above 1 g/l
44. Neonatal thrombocytopenia
Thrombocytopenia in neonates is traditionally defined
as a platelet count <150000/mcL
over all incidence of neonatal
thrombocytopenia is (0.7%–0.9%)
In Neonatal Intensive Care Unit (NICU) it is
very high (22%–35%)
48. Immune thrombocytopenia
Immune thrombocytopenia occurs due to passive
transfer of antibodies from the maternal to the fetal
circulation.
Types:
1) Neonatal Alloimmune thrombocytopenia (NAIT)
2) Autoimmune thrombocytopenia
49. Neonatal Alloimmune thrombocytopenia (NAIT)
antibody is produced in the mother against specific
human platelet antigen (HPA) present in the fetus but
absent in the mother.
The antigen is inherited from the father of the fetus.
Early onset severe thrombocytopenia.
combination of severe neonatal thrombocytopenia
with a parenchymal (rather than intraventricular)
intracranial hemorrhage is highly suggestive of NAIT.
50. Diagnosis and management of NAIT
Investigation :
1) Antigen screening (HPA 1,3,5,9,15,4)
2) Brain imaging studies
Management:
1) Suspected NAIT in an unknown pregnancy
2) Known case of NAIT
3)Antenatal mgt of pregnant woman with previous
history of NAIT
51. Management of the neonate with suspected NAIT in an
unknown pregnancy.
1) Random-donor platelet transfusion
2)IVIG (1g/kg/day for 2 days)
3) Antigen-negative platelet transfusion
4) Methylprednisolone (1 mg/kg bid for 3–5 days)
Management of the neonate with known NAIT
Antigen-negative platelet transfusion
Antenatal management of pregnant women with previous
history of NAIT IVIG is given to mother.
55. Clinical
Evaluation Platelets PT PTT Likely diagnosis
Sick D- 1+ 1+ DIC
D- N N
Platelet consumption ( infection,
necrotizing enterocolitis, renal vein
thrombosis )
N 1+ 1+ Liver disease
N N N
Vasculitis (hypoxia, prematurity,
acidosis, hyperosmolality )
Healthy D- N N
ITP, occult infection, thrombosis, B.M.
hypoplasia or infilteration
N 1+ 1+
Hemorrhagic disease of newborn ( vit.
K deficiency )
N N 1+ Hereditary clotting factor deficiencies
N N N
Bleeding due to local factor ( trauma,
anatomic anomalies);
PT = prothrombin time; PTT = partial thromboplastin time; D- = decreased; 1+ = increased; DIC =
disseminated intravascular coagulation; N= normal .
56. References
▪ Nelson Textbook of Pediatrics
▪ Manual of Neonatal Care-Cloherty
▪ oxford handbook of neonatology
▪ Medscape
Clinicalkey