Morbus haemorrhagicus neonatorum is a disorder of blood clotting that can affect infants and is due to a deficiency of vitamin K. Vitamin K is relevant to the synthesis of various coagulation factors. To treat the disorder, intravenous substitution of the required vitamin takes place in the infant.
What is haemorrhagic neonatal disease?
Blood clotting protects humans from infection and extraordinary blood loss. The central element of coagulation is the so-called coagulation cascade, which involves various endogenous and foreign substances. Coagulation disorders can manifest themselves in a bleeding tendency. Often there is an underlying genetic cause. In contrast, an external cause is responsible for the bleeding tendency of patients with haemorrhagicus neonatorum. The age of manifestation of this pathological phenomenon is infancy. Thus, the complex of bleeding-related symptoms is called hemorrhagic disease in fetuses and neonates. Affected individuals suffer from a condition called hemorrhagic diathesis, which manifests as either abnormally prolonged or abnormally severe bleeding tendencies in the setting of injury. Bleeding from inadequate causes is also sometimes referred to as hemorrhagic diathesis. In some circumstances, these three signs are present simultaneously.
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 clotting proteins, also known as blood clotting factors. Thus, if there is not enough vitamin K in the body, the organism cannot sufficiently produce the clotting factors necessary for clotting. This relationship determines the causal relationship of haemorrhagic neonatal disease. The primary cause of this coagulation disorder is a deficiency of vitamin K, which causes the infant’s body to form too few clotting factors for adequate blood clotting. The vitamin is primarily relevant for clotting factors II, VII, IX and X. In addition to malnutrition of the mother during pregnancy, anticonvulsant therapy with drugs such as hydantoin and primidone can lead to such a deficiency in the organism of the newborn. Antibiotic treatments during pregnancy can also be causative. If the deficiency is not present from birth, it is often preceded by parenteral nutrition of the infant.
Symptoms, complaints, and signs
In most cases, haemorrhagic neonatal disease is present from birth. Only rarely does the phenomenon present due to influences after pregnancy. However, if vitamin K deficiency is present from birth, it need not manifest immediately after birth. In most cases, the coagulation disorder manifests itself at least within the first week of life in a kind of early form. In this early form, haemorrhagic neonatal disease becomes apparent between the third and seventh day of the newborn’s life in the form of a cephalhematoma. Other unexplained bruising of the skin may also be signs. For example, the dermal lesions refer to intracranial hemorrhage. Additionally, bleeding of the skin and gastrointestinal bleeding may present. There is no obvious reason for the bleeding. When haemorrhagicus neonatorum is not present from birth, it is usually due to a decreased concentration of vitamin K in breast milk. The late form of coagulopathy acquired in this way becomes apparent in the first three months of life as an intracranial bleeding tendency.
Diagnosis and course of the disease
The physician often makes the diagnosis of haemorrhagic neonatal disease during the first week of the infant’s life. The key factor in suspecting a coagulation disorder is bruising of the skin. Laboratory diagnostics show a prolonged prothrombin time in the form of an abnormal Quick value. Both the bleeding time and the partial thromboplastin time usually turn out to be normal in laboratory diagnostics. Haemorrhagic neonatal disease must be distinguished from other coagulation disorders. This differential diagnosis can be made primarily by determining vitamin K levels. The prognosis of the patients is excellent.
Complications
As a result of haemorrhagic neonatal disease, affected individuals suffer from bruising that can occur all over the body. The symptoms can often become apparent a few weeks after birth and are not associated with any impact of force on the body. They usually occur spontaneously and inexplicably. Furthermore, bleeding may occur between the skin. The affected person suffers from pain, which may be manifested by crying, especially in children. As a rule, self-healing does not occur, so that treatment of haemorrhagic neonatal disease by a physician 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 bruising in the first place. Treatment of haemorrhagicus neonatorum is usually done by adding vitamins and by blood transfusions. This does not result in further complications. In the worst case, however, bleeding in the brain can occur, which can be fatal. For this reason, the bleeding source must be identified in any case and the bleeding stopped so that these complications do not occur. However, life expectancy is not usually reduced by haemorrhagic neonatal disease.
When should you see a doctor?
Haemorrhagic neonatal disease usually occurs exclusively in newborns. Because infants are part of the at-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 present. Existing irregularities or abnormalities are noticed and documented. Necessary medical care is taken care of by the trained staff in routine procedures. The same procedure can be ensured in the case of a birth in a birth center or a home birth with a midwife present. Therefore, parents do not need to take action in these cases. If a spontaneous delivery takes place without an obstetrician, transport of mother and child to the nearest hospital should be arranged 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 indicates an existing irregularity that must be examined and clarified. Open wounds must be treated sterilely to prevent germs from entering the organism. In the worst case, there is alternatively a threat of sepsis and thus a potential danger to the life of the child. Disturbances of the blood circulation, pain or behavioral abnormalities of the newborn must be presented to a physician. If disturbances of the heart rhythm are present, or if there is palpitations or a refusal to feed, a physician should be consulted immediately.
Treatment and therapy
Unlike many other coagulation disorders, haemorrhagic neonatal disease can be treated causally. Thus, the condition is considered curable and usually does not require much more than substitution of the missing vitamin. In most cases, the substitution therapy consists of intravenous substitution. If the deficiency is not extreme, administration of one to two milligrams of the vitamin is sufficient for causative treatment. Intravenous administration of the vitamin prevents insufficient absorption by the child’s intestinal flora. Blood transfusion is necessary only in extremely rare extreme cases. The coagulation-relevant values of the blood are determined closely during the therapy. Normally, blood coagulation stabilizes within days. If bleeding has occurred in the internal organs, additional medical intervention may be required to stop the source of bleeding. A fatal complication would be bleeding in the brain, as it could result in stroke-like symptoms. However, cerebral hemorrhage does not usually occur in the setting of haemorrhagic neonatal disease.
Outlook and prognosis
The earlier the existence of the disease haemolyticus neonatorum were detected, the better the prognosis and the chances of recovery. In many of the affected children, the disease even resolves without treatment or with the use of light therapy alone. Nevertheless, in a few particularly severe cases, the disease can develop life-threatening proportions. This is especially the case if no suitable therapy is given.Then there is the possibility that sick children may die from the dreaded complications of the disease. Without therapy, the steadily progressing hemolysis, directly after birth, leads to dangerous hyperbilirubinemia and thus causes damage to the child through indirect bilirubin. Particularly occurring muscle weakness in newborns may already indicate the onset of encephalopathy. As the damage to the brain increases, the affected infants develop generalized spasticity and seizures. Often respiratory insufficiency and pulmonary hemorrhage also occur. Approximately 25% of affected fetuses develop signs of marked anemia with dangerous hemoglobin concentrations below 8 g/dL as early as between the 18th and 35th weeks of gestation, due to the anti-D. If left untreated, this leads to hypoxia, acidosis, liver damage and splenomegaly. Which then in turn leads to massive edema tendency in the affected fetuses. In addition, further complications such as pulmonary edema as well as pulmonary hemorrhage can lead to early death.
Prevention
Haemorrhagic neonatal disease can be prevented. As a preventive measure, vitamin K supplements are given to an infant by default after birth. The vitamin is administered once between the third and tenth day of life and repeated around the 28th day of life. In most cases, the amount of vitamin substituted corresponds to the recommended two milligrams. Because this preventive measure has long been standard practice in hospitals, haemorrhagic neonatal disease now occurs only in rare cases.
Follow-up
In most cases, no special or direct measures of aftercare are available to the affected person in haemorrhagic neonatal disease. For this reason, early diagnosis is important in this disease to avert the occurrence of complications and symptoms in time. In the event that a person wishes to have children, he or she should seek genetic testing and counseling to prevent recurrence of the disease in the children. The disease can be treated relatively well with the help of various medications and supplements. The affected person should always pay attention to the correct dosage and also to regular intake in order to alleviate the symptoms properly and permanently. Likewise, regular checks of the internal organs and blood values are very important in order to detect other complaints at an early stage. In the case of various treatments or surgical interventions, the affected person should always inform the doctor about the disease haemorrhagicus neonatorum, so that complications do not arise. Further measures of an aftercare are usually not available to the affected person with this disease. It is possible that haemorrhagicus neonatorum disease reduces the patient’s life expectancy, although no general prediction can be made.
Here’s what you can do yourself
Because the bleeding time is significantly prolonged due to the lack of vitamin K, the sick child must be treated with special care. All nursing activities, such as carrying the child around, rocking him, or changing his diaper, should be reduced to a necessary minimum to avoid provoking new bleeding. Strong pressure on a body region or a body part must be urgently avoided in order not to injure the smallest vessels. The skin should be regularly observed for so-called petechiae (smallest skin bleedings) or larger bruises. The stool should also be checked for irregularities, such as fresh blood deposits or digested blood (tarry stools). However, black tarry stools in particular can also be mistaken for ordinary meconium, also known as puerperal feces. Once the corresponding blood parameters have returned to normal, the newborn can be cared for and touched again as usual. It is crucial that the child receives the standard doses of vitamin K, which are administered after birth, on the 3rd, 10th and 28th day of life. Corresponding medical appointments must be made urgently for this purpose. The pediatrician will also point this out again.
