Author(s) : Dmitrieva Elena Germanovna 1
1 The Clinical Pharmacist , Pharmaceutical Clinic , Russia
Open J Pharma Sci
Article Type : Case Reports
Blueberry Muffin Baby Syndrome (BMB) is a term describing the characteristic widespread bright red to blue - gray spots and papules on the skin of newborns, representing an islet and extramedullary hematopoiesis. It is a Pathomorphology based study, it was found that the lesions are foci of extramedullary hematopoiesis in derme and in the subcutaneous tissue. Subsequently, Cytomegalovirus (CMV) was described in children with both diseases associated with intrauterine infection of the fetus and with hematological, autoimmune and neoplastic pathological processes in newborns. The term CMV was coined to describe a purpuric skin rash in newborns with congenital rubella during an epidemic of infection in the 1960s. in the USA.
A child with congenital rubella sheds the virus for a longer time (up to 20 months). The infection is transmitted by airborne droplets. With a disease of pregnant women, a transplacental transmission route to the embryo and fetus is possible. Children who are not immune to rubella are highly susceptible to infection. In the first months of life, children do not get rubella, as they receive antibodies from their mother. Immunity in a person who has recovered is stable, lifelong. In the case of measles, eye damage (destruction of the cornea, which can lead to loss of vision) is also common, especially among weakened children with a deficiency in vitamin A. And now more seriously - from vaccination, as well as from a disease, if not treated or treated incorrectly, there can be different outcomes.
In young children, the mortality rate from complications reaches 60%. Viral pneumonia should be treated. Antibiotics are not suitable. One of the 1000 cases develop acute encephalitis, which can lead to convulsions, loss of hearing and possible - to mental disability. There is a minimal, but nevertheless, risk of subacute sclerosing panencephalitis, a fatal complication provoked by the replacement of the brain with connective tissue, which can occur several years after measles or rubella against the background of vaccination.
New diagnostic methods in Russia (Immunograms)
First, check the immune status, but it is necessary to do it from the mother of the child - to take blood for antibodies of measles and other childhood infections. You can make Immanograms for children from 3-5 years old. Vaccines, manufactured in the USA from 4 components, cause irreversible disability in children, as it was in Volgograd, Russia.
M P Shaffer et al. gave a list of diseases that are accompanied by WWI
1. Intrauterine infections (toxoplasmosis, gerp esvirusnaya infection, rubella)
2. Other infections (syphilis, parvovirus infection (caused by parvovirus B19)
3. Infection caused by Coxsackie virus B)
5. Haematological disorders (hereditary spherocytosis
6. Hemolytic disease of the newborn (incompatibility blood or ABO Rh)
7. Feto-fetal transfusions
8. Neoplastic processes (congenital leukemia, neuroblastoma, rhabdomyosarcoma congenital, metastatic infiltration innate ennyh malignancies)
9. Histiocytosis (Langerhans cell histiocytosis, lymphocytic gemogistiotsitoz)
11. Neonatal lupus erythematosus.
A combination of blueberry cake syndrome with ?-, ?-, ?-, ?-thalassemia is possible. There is also an association in the literature with type 2 Gaucher disease, as well as with bone demineralization due to primary hyperparathyroidism. In addition to the conditions mentioned above, many congenital vascular lesions can look like VMV. However, few of them are multifocal in nature. Multiple hemangiomas, multifocal lymphangioendotheliomatosis, Bean's syndrome (blue rubber bladder nevus syndrome), and multiple glomangiomas are four conditions that should be considered in the differential diagnosis with IUD . Foci of CMV are clinically manifested by scattered purple spots or infiltrated papules, which are located mainly on the face and neck, trunk. In most cases, the rashes fade and regress within a few weeks (up to 2 months) , in some cases they can leave light brown spots after themselves . Most of the elements appear before birth and can increase during the early neonatal period. In rare cases, a skin rash appears immediately after birth .
Along with general clinical tests, serological tests for the TORCH complex are carried out. Histological examination of the biopsy specimen is carried out when the diagnosis is difficult . Differential diagnosis of skin rashes in newborns is a difficult task, since a wide variety of conditions can be the cause of skin rash in a newborn, such as congenital infections, perinatal anemia caused by hemolytic disease (incompatibility according to the Rh factor, ABO system), hereditary spherocytosis, feto-fetal transfusion syndrome, placental insufficiency, intracranial hemorrhage, neurodegenerative diseases (Aicardi – Gutierrez syndrome), neonatal lupus erythematosus, neoplasms, and autoimmune or hematological disorders [8,9,14,15] .
In the presence of the blueberry muffin syndrome in a newborn, it is necessary to carry out a differential diagnosis not only with the above conditions, but also with such conditions as acute myeloid leukemia, neuroblastoma, as well as possible intrauterine infection [7,16]. In such cases, skin lesions in a newborn child may reflect systemic diseases and require additional research methods, including pathohistological examination of the skin. Treatment and prognosis are determined by the disease that triggered the blueberry cake syndrome.
The newborn boy was taken to the neonatal department of the city hospital 2 hours after home birth. The child was born with rashes on the skin of the neck, lower limbs. Was attached to the breast, the breast was actively sucked. Anthropometric data at birth: body weight 3930 g, length 56 cm, head circumference 35 cm, chest circumference 34 cm. Apgar score 8/9 points. From the anamnesis it is known that the boy was born full-term at 38 weeks, from IV pregnancy, IV birth. Established late registration in the antenatal clinic in the period of 32 weeks. Pregnancy proceeded against the background of hypochromic anemia, there were recurrences of genital herpes in the third trimester, which were accompanied by prolonged low-grade fever. When examining the mother for syphilis, viral hepatitis, HIV infection, the results are negative.
The skin process is represented by scattered single rashes localized on the posterolateral surface of the neck on the right, on the anteromedial surface of the left shoulder, in the region of the arch of the left foot.
Rashes of a non-inflammatory nature, from 7 to 13 mm in diameter, are represented by papules of a purple-cyanotic color, with clear boundaries, hemispherical, rounded outlines, dense consistency. Rashes on the anteromedial surface of the right thigh in the form of a hemorrhagic spot, up to 10 cm (ecchymosis), purple-cyanotic color, with indistinct boundaries (Figure 1-3).
Figure 1: A purple papule on the neck of a newborn, reflecting the process of dermal erythropoiesis in the background chronic congenital measles rubella.
The results of laboratory methods for examining a child on the first day of life.
General blood test: Hb - 183 g/l, Er - 5.13 × 1012 /l, L - 25.9 × 109 /l, eosinophils - 7%, lymphocytes - 11 , 3%, monocytes - 10.3%.
General urine analysis: no deviations.
Biochemical blood test: total protein 57.3 g/l, glucose 2.9 mmol/l, total bilirubin 104.5 ?mol/l, direct bilirubin 0.7 ?mol/l, AST 93 U/l, ALT 36 U/l , C-reactive protein 35 mg /l. The microreaction of blood precipitation to syphilis is negative.
Bacteriological blood culture: no flora detected.
Bacteriological seeding of feces: pathogenic and opportunistic flora was not found.
Urine study by polymerase chain reaction: DNA CMV, HSV-1 and HSV - 2, Chlamydia trachomatis, Mycoplasma genitalium, Ureaplasma - were not detected.
Serological examination of mother and child sera for TORCH-complex in mother and child: antibodies (IgM) to Ch. trachomatis, M. genitalium, Ureaplasma, toxoplasmosis, rubella, cytomegalovirus - not detected, antibodies (IgG) to rubella were detected in the value of 240 IU / ml , to toxoplasmosis in the mother - 0.62 IU / ml, in the child - 0.33 IU / ml, to cytomegalovirus infection - 9.7 IU / ml in the mother and 7.8 IU / ml in the child.
The avidity of IgG antibodies to CMV is 65.1% in the mother and 71.2% in the child.
The avidity of IgG antibodies to rubella in the child was 27.2%.
The data obtained indicate the presence of maternal protective antibodies.
The results of instrumental research methods: Ultrasound diagnostics of the abdominal and retroperitoneal organs revealed a syndrome of hyperechoic renal pyramids, reactive changes in the liver parenchyma.
According to the results of the examination, laboratory and instrumental methods of examination in the department, the diagnosis remained unclear.
A pediatrician treated skin rashes as pyoderma, prescribed a course of antibiotic therapy: Ampicillini 100 mg / kg for 7 days, Amikacini 15 mg / kg for 7 days. In order to prevent the activation of the fungal flora, the child received antimycotic therapy: Fluconazoli 6 mg / kg for 3 days.
The treatment is not correct.
External treatment of skin rash elements: 1% alcohol solution of brilliant green. Due to hyperbilirubinemia, the child received phototherapy.
The diagnosis of “blueberry cake” syndrome was established on the basis of the clinical picture on the 3rd day of the child's life - treatment with Viferon (suppositories).
In the presented clinical observation, it was not possible to establish an obvious causal factor. For the purpose of differential diagnosis, the child was prescribed a histopathological examination of skin rashes, but the child's mother refused to carry out this research method. The child was discharged on the 7th day in satisfactory condition under the supervision of a local pediatrician.
This clinical observation is of interest to both neonatologists and pediatricians and dermatovenerologists. This syndrome is a multidisciplinary pathology and requires timely consultation of narrow specialists. Awareness of doctors about the clinical manifestations of the blueberry muffin syndrome will contribute to timely differential diagnostic search, timely treatment of an infectious disease of a newborn, or refusal of unreasonable therapy, as well as the exclusion of malignant neoplasms in a child.
T Which children sometimes get home before further diagnosis. The child from the described case was examined for oncological reasons, a bone marrow puncture was performed (normal), the parents refused a skin biopsy. Was examined for infectious causes: syphilis, cytomegalovirus, toxoplasmosis, herpes simplex, parvovirus, Coxsackie B2 and hepatitis B and C (negative). Rubella tests were positive for IgM 16.5 IU / ml and IgG 236 IU / ml. The levels of bilirubin and transaminases increased progressively until the 37th day of life, then stopped, from the 47th day the laboratory parameters began to improve, but, unfortunately, the child died from a nosocomial infection on the 57th day of life.
There seemed, that the detected stenosis legochnoy artery. By rasnuha, transferred during pregnancy leads to multiple malformations, and including, a heart defect.
There is a direct connection to the vaccine among mothers of children - chronic measles or to revaya rubella - blueberry muffin syndrome in newborns. Treatment during the neonatal period - Viferon suppositories - from the period 0 - n birth and new therapy.
New treatment regimen
Amizon syrup from 0 to 6 years
Amizon for children increases persistent immunity due to increased concentrations of endogenous interferon (in plasma by 3-4 times), lysozyme and titer of antibodies to pathogens. The drug stimulates cellular immunity by increasing the activity of macrophages and T-lymphocytes. Amizon for children is a strong inducer of endogenous interferon. It can be administered in combination with recombinant interferon preparations. It is also advisable to prescribe the drug Amizon in combination with ascorbic acid. Amizon is not prescribed to patients with intolerance to enisamium iodide and iodine preparations.
For iodine intolerance
You can use Flavoside - Proteflazid, obtained from Soddy pike (Deschampsia cetspitosa L.) and Ground reed (Calamagrostis epigeios L.) Antiviral action is provided by inhibition of specific viral enzymes : reverse transcriptase , DNA polymerase and thymidine kinase. The mechanism of the direct anti-infectious effect of the drug is based on the ability of Proteflazid to inhibit neuraminidase activity, RNA synthesis, and induce the synthesis of endogenous interferon. The drug allows to protect and normalize the indices of local immunity of the mucous membranes of the upper airways (airways) by increasing the level of secretory immunoglobulin A and lactoferrin. After a single oral administration, active synthesis of interferon is observed within 6 days, the peak concentration falls on the 2nd day. The syrup is taken about 20-30 minutes before meals, initially shaking and measuring the dose corresponding to the prescribed treatment regimen with a dosing capacity. Within 1 month. you should take the daily dose, divided into 2 doses, for newborns and children under 1 year old - 1 ml. Viferon from 0 - candles Children under 7 years old, incl. for newborns and premature infants with a gestational age of more than 34 weeks, Viferon is prescribed 150,000 ME, 1 suppository 2 times / day every 12 hours every day for 5 days. According to clinical indications, therapy can be continued. The break between courses is 5 days.
Caution: Vitamin A - when treating with Viferon - you do not need to add Vitamin A! No Aspirin, Paracetamol and T Aylenol!
Pregnant women can take following actions:
A. Emergency prophylaxis is possible when anti- rubella AND mmunoglobulin are administered to contact children and pregnant women. It's better than a vaccin.
Attention! During pregnancy or before it, it is better not to get vaccinated.
B. In Flavozid - from the 1st to the 3rd day - 5 ml 1 time per day
during the 4th day - 8 ml 1 time per day. Pregnant women can take Flavozid syrup on a l e a meal.
C. Otvar elderberry during pregnancy - 1 dessert spoon at a hundred to a Mr boiling water - treating measles and rubella, congenital malformations profilaktiruet. The course is up to 30 days.
D. Imunofan - candles for pregnant women - 1 candle in the anus 1 time in 2 days. The course of treatment is 10 days.
E. Viferon - suppositories for pregnant women - from the 2nd trimester from the 14th week of gestation - 500,000 IU, 1 suppository 2 times a day - after 12 hours daily for 10 days. Then, 4 weeks before childbirth - 150,000 IU, 1 candle 2 times a day, 12 hours later every day. The course of treatment is 5 days. Delivery is indicated from 38 weeks of gestation.
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Corresponding Author: Dmitrieva Elena Germanovna, The Clinical Pharmacist, Pharmaceutical Clinic, Town Ivanovo, 153012, Sacco’s street, Russia. E-mail: CyriX2003@mail.ru
Copyright: © 2021 All copyrights are reserved by Dmitrieva Elena Germanovna, published by Coalesce Research Group. This work is licensed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.