Neonatal hemorrhagic disease is a blood clotting disorder that can affect infants and is caused by a vitamin K deficiency. Vitamin K is relevant for the synthesis of various coagulation factors. To treat the disease, an intravenous substitution of the required vitamin takes place in the infant.
What is neonator hemorrhagic disease?
Blood clotting protects people from infections and excessive blood loss. The central element of coagulation is the so-called coagulation cascade, in which various endogenous and foreign substances are involved. Coagulation disorders can manifest themselves in a tendency to bleed. There is often a genetic cause. See fun-wiki for What is hyperkalemia used for.
On the other hand, an external cause is responsible for the bleeding tendency of patients with Morbus haemorrhagicus neonatorum. The age of manifestation of this pathological phenomenon is infancy. The complex of bleeding-related symptoms is thus referred to as hemorrhagic disease in fetuses and newborns.
Those affected suffer from a so-called haemorrhagic diathesis, which manifests itself either as an abnormally long or abnormally strong tendency to bleed in the event of an injury. Bleeding from an inadequate cause is also sometimes referred to as hemorrhagic diathesis. These three signs may be present at the same time.
Causes
Vitamin K plays an important role in blood clotting. In the human body, the fat-soluble vitamin is involved in the production of various proteins. These proteins are primarily blood coagulation proteins, which are also known as blood coagulation factors. If there is not enough vitamin K in the body, the organism cannot produce enough of the coagulation factors required for clotting.
This connection determines the causal connection of the Morbus haemorrhagicus neonatorum. The primary cause of this clotting disorder is a lack of vitamin K, which prevents the infant’s body from producing enough clotting factors for adequate blood clotting. The vitamin is particularly relevant for coagulation factors II, VII, IX and X.
In addition to malnutrition in the mother during pregnancy, anticonvulsant therapy with drugs such as hydantoin and primidone can lead to such deficiency symptoms in the newborn’s organism. Antibiotic treatments during pregnancy can also be a cause. If the deficiency is not present at birth, the infant is often preceded by parenteral nutrition.
Symptoms, Ailments & Signs
In most cases, neonatal haemorrhagicus disease is present from birth. The phenomenon only rarely occurs due to influences after pregnancy. However, if the vitamin K deficiency is present from birth, it does not have to manifest itself immediately after birth. In most cases, the coagulation disorder manifests itself in a kind of early form at least within the first week of life.
In this early form, the Morbus haemorrhagicus neonatorum becomes noticeable between the third and seventh day of life of the newborn in the form of a cephalhematoma. Other unexplained bruising of the skin can also be signs. For example, the dermal lesions indicate intracranial hemorrhage. In addition, bleeding of the skin and gastrointestinal bleeding can occur. There is no obvious reason for the bleeding.
If neonator haemorrhagic disease is not present at birth, it is usually due to a reduced concentration of vitamin K in breast milk. The late form of the coagulation disorder acquired in this way becomes noticeable in the first three months of life as a tendency to intracranial bleeding.
Diagnosis & course of disease
The diagnosis of Morbus haemorrhagicus neonatorum is often made by the doctor in the first week of life of the baby. Bruising of the skin is decisive for the suspicion of a coagulation disorder. In laboratory diagnostics, a prolonged prothrombin time shows up in the form of an abnormal Quick value.
Both the bleeding time and the partial thromboplastin time are usually found to be normal in the laboratory. The Morbus haemorrhagicus neonatorum must be differentiated from other coagulation disorders. This differential diagnostic delimitation succeeds above all by determining the vitamin K values. The prognosis of the patients is excellent.
Complications
Those affected by neonatal haemorrhagic disease suffer from bruising, which can occur anywhere on the body. The symptoms can often become noticeable a few weeks after birth and are not associated with the effects of violence on the body. They usually occur spontaneously and inexplicably. Bleeding can also occur between the skin.
Those affected suffer from pain, which can be expressed by screaming, especially in children. As a rule, self-healing does not occur, so that treatment of the neonatal haemorrhagic disease by a doctor is necessary in any case. In some cases, the child’s parents also suffer from psychological discomfort, as they are unable to identify the cause of the bruises in the first place.
The treatment of Morbus haemorrhagicus neonatorum takes place in most cases by the addition of vitamins and blood transfusions. There are no further complications. In the worst case, however, it can lead to bleeding in the brain, which can be fatal. For this reason, it is important to identify the source of the bleeding and stop the bleeding to avoid these complications. However, life expectancy is usually not reduced by neonatorum haemorrhagic disease.
When should you go to the doctor?
Morbus haemorrhagicus neonatorum normally only occurs in newborns. Since infants belong to the risk group due to their natural inability to act, parents should show increased vigilance. Newborns are always examined intensively immediately after birth by the obstetricians who are present.
Existing irregularities or abnormalities are noticed and documented. Necessary medical care is taken over by the trained staff in routine processes. The same procedure can be guaranteed for a birth in a birth center or for a home birth with a midwife present. Therefore, parents do not have to take action in these cases.
If a spontaneous delivery takes place without an obstetrician, the mother and child should be transported to the nearest hospital as quickly as possible. If the first symptoms appear a few days after delivery, a doctor is needed. Bruising, bleeding or discoloration of the skin indicate an existing irregularity that needs to be examined and clarified.
Open wounds must be treated sterilely so that no germs can get into the organism. In the worst case, there is an alternative risk of sepsis, which could potentially endanger the life of the child. Circulatory disorders, pain or behavioral problems in the newborn should be presented to a doctor. If there are cardiac arrhythmias, heart palpitations or refusal to eat, a doctor should be consulted immediately.
Treatment & Therapy
Unlike many other coagulation disorders, neonatal haemorrhagic disease can be treated causally. The phenomenon is therefore considered curable and usually requires little more than substitution of the missing vitamin. In most cases, the substitution therapy measure consists of an intravenous substitution.
If the deficiency is not extremely pronounced, the administration of one to two milligrams of the vitamin is sufficient for the causal treatment. Intravenous administration of the vitamin prevents insufficient absorption by the child’s intestinal flora. A blood transfusion is only necessary in extremely rare extreme cases. The values of the blood relevant to coagulation are determined closely during the therapy.
Normally, blood clotting stabilizes within days. If bleeding has occurred in the internal organs, medical intervention may also be necessary to stop the source of the bleeding. A fatal complication would be bleeding in the brain, as it could result in symptoms similar to a stroke. However, brain haemorrhages do not usually occur in the context of neonatal haemorrhagic disease.
Outlook & Forecast
The earlier the existence of Morbus haemolyticus neonatorum is recognized, the better the prognosis and the chances of recovery. In many of the sick children, the disease even subsides without treatment or simply through the use of light therapy. Nevertheless, in a few particularly serious cases, the disease can develop to life-threatening proportions. This is especially the case if there is no suitable therapy. Then there is the possibility that sick children can die from the dreaded complications of the disease.
Without therapy, the constantly progressing haemolysis leads to dangerous hyperbilirubinemia immediately after birth and thus leads to damage to the child through indirect bilirubin. Muscle weakness that particularly occurs in newborns can already indicate the onset of encephalopathy.
With the increasing damage to the brain, those affected develop generalized spasticity and seizures. Respiratory failure and pulmonary hemorrhage are also common. Approximately 25% of the affected fetuses already develop between the 18th and 35th week of pregnancy, due to the anti-D, signs of pronounced anemia with dangerous hemoglobin concentrations below 8 g/dl.
Left untreated, this leads to hypoxia, acidosis, liver damage, and spleen enlargement. This in turn leads to a massive tendency to edema in the diseased fetus. In addition, other complications such as pulmonary edema and pulmonary bleeding can lead to early death.
Prevention
The Morbus haemorrhagicus neonatorum can be prevented. As a preventative measure, vitamin K supplements are routinely given to an infant after birth. The vitamin is administered once between the third and tenth day of life and is repeated around the 28th day of life.
The amount of the substituted vitamin usually corresponds to the recommended two milligrams. Since this preventive measure has long since become standard in hospitals, neonatal haemorrhagic disease now occurs only in rare cases.
Aftercare
In most cases, those affected with Morbus haemorrhagicus neonatorum have no special or direct follow-up measures available. For this reason, early diagnosis of this disease is important in order to avert the occurrence of complications and symptoms in good time. In the case of a desire to have children, the person affected should seek genetic testing and counseling to prevent the disease from reoccurring in the children.
The disease can be treated relatively well with the help of various medications and supplements. The person concerned should always pay attention to the correct dosage and regular intake in order to relieve the symptoms properly and permanently. Regular checks of the internal organs and blood values are also very important in order to identify other complaints at an early stage.
In the case of various treatments or surgical interventions, the person concerned should always inform the doctor about the disease Morbus haemorrhagicus neonatorum so that there are no complications. Further follow-up measures are usually not available to those affected by this disease. Morbus haemorrhagicus neonatorum may reduce the life expectancy of the patient, although no general prediction can be made.
You can do that yourself
Since the bleeding time is significantly prolonged due to the lack of vitamin K, the sick child must be treated with particular care. All nursing activities, such as carrying around, rocking or changing the child should be reduced to a necessary minimum in order not to provoke new bleeding. Strong pressure on a body region or part of the body must be avoided in order not to injure the smallest vessels.
The skin should be checked regularly for so-called petechiae (tiny skin bleeding) or larger bruises. The stool should also be checked for irregularities, such as fresh blood deposits or digested blood (tarry stool). However, the black tar stool in particular can also be confused with ordinary meconium, also known as child spech.
Once the corresponding blood parameters have returned to normal, the newborn can be cared for and touched as usual. It is crucial that the child receives the standard doses of vitamin K, which are administered after birth, on days 3, 10 and 28 of life. Appropriate medical appointments must be made urgently. The pediatrician will also point this out again.
