Atypical (severe) combined immunodeficiency (def RAG1).

Republican Scientific Center of Pediatric Oncology, Hematology and Immunology

Cancer (Hematology) department №1, Phone: (017) 265-40-98.

                                                             

Epicrisis №647

Prokopenko Viktoria, born 03.03.2011

Home address: Ukraine, Odessa, st. Lustdorfskaya Road, 29, kv.55.

Was treated in the oncology (hematology) unit № 1 to 02.17 on 04.13.13.

Diagnosis: Atypical (severe) combined immunodeficiency (def RAG1).

Additions: Cytomegalovirus infection. Chronic malnutrition. Condition after the ileum resection, subtotal resection gland appendectomy (07.21.2012). Primary hypothyroidism. Condition after laparotomy (03.15.13).

Blood Type A (II) Rh neg.

The child was admitted with complaints of swollen lymph nodes in the neck and in the groin area, redness, and peeling of the skin, swollen legs, weakness, fever, bloating, cough, retarded weight.

Background: 1^st child. The pregnancy was under a constant threat of termination (fixed treatment repeatedly) in the II trimester of the pregnancy mother revealed a positive PCR for HCV at 21 weeks. Gestation set with diagnosis IUGR hydramnion mother found lg to CMV. Toxoplasma gondii, Chlamydia, with a gain of Ig to CMV in the dynamics, the treatment is not received. Births at 38-39 weeks, weight 2470g, length 47cm. Apgar score of 8 without the complication of BCG delayed saint with malnutrition, hepatitis B is not vaccinated, belly button fell off on the 5th day, the umbilical wound healed without complications. The child was breastfed for up to 8 months. Vaccinated (DTP + polio). In the month I vaccinated with BCG, a month later fester occurred, which was gradually transforming into an ulcer with purulent discharge that would not heal. At 10 months counseled by physiatrician, who prescribed isoniazid for 10 days and rifampicin afterwards.

The child was gaining 1000 grams for the first two months and then 100 grams a month. Her weight reached 9 kg at age one. After that she stopped gaining weight. Psychomotor development was observed until 6 months. According to the child’s mother, Viktoriya developed rush after introduction of baby formulas. An allergic nature has been stubbornly persisting since then. Additionally, there was an increase of temperature to 38°C for 2-3 days, which was accompanied by a constant nasal discharge. At 9 months the child’s body temperature increased to 40°C without catarrh and dyspepsia, which held down during the day then periodically (I once every 2-3 weeks). At 1 year 1 month, the child was hospitalized (04.23-05.04.12) at the local hospital for the purpose of laboratory test of transaminase, which was elevated at that time. PCR for HCV and HBV were found negative. The following diagnosis was established: complication of BCG vaccination in the form of superficial ulcers, hypochromic anemia II stage, protein-energy malnutrition, received standard therapy, intravenous immunoglobulin. 2 days later elevated body temperature was observed again (38°C). Intestinal syndrome. The child was hospitalized at Children's hospital with the diagnosis: Atopic dermatitis, hepatitis of unknown origin as a complication of BCG superficial ulcers, discharged 05.15.12 g. KLA in HB-105, ESR-28 mm / hour at discharge. Due to repetitive increase of the body temperature to 38°C, lower Hb (to 69 gHb/l), rising to 70 cfu mm/h 06.05.12 she was hospitalized again to a local hospital, where she received intravenous Prednsolone, Cefepimum. As a result of this treatment, the t° was normalized. ESR had decreased, Hb remained at the previous level. Released from the hospital on 06.30 (same day Betamethasone injection was received once)

07.07 - t° elevated to 39°C, rash appeared on the abdomen and legs.

07.09 to 10.02.12 – hospitalized with diagnosis: Undifferentiated Connective Tissue Disease (UCTD), Wegener’s Syndrome (?), Primary Immunodeficiency (IDS) (?), Persistent and disseminated BCG-infection, regional lymphadenitis on the left. Moderate hypochromic anemia, protein-energy malnutrition/deficiency. The ileum necrosis (07.21), resection of the ileum area, subtotal resection of the gland appendectomy.

In September 2012 - URTI, rhinosinusitis, purulent conjunctivitis. Received IV therapy, Amikacin, Maxipime, Meropenem, Methylprednisolone 12 mg / day per. The fever persisted at 37.5 ° 37.7 ° C in the mornings, lethargy, drowsiness, flatulence, was examined in the medical center of metabolic deceases from 10.03 to 12.08.12

On 10.05.12 an autopsy in the axillary lymphadenopathy drainage was done. No Connective Tissue Decease was found. Gradual decline in the Glucocorticoids (GCs) is revealed. The child is being examined periodically, including the examinations in the Department of Pediatric Infectious Diseases and Pediatric Immunology NMAPE. The results of research conducted as part of a council chief children immunologist MOH by professor Chernyshova LI and professor Volokha A P.. Assoc. Department of Children's Diseases and Pediatric Immunology NMAPE Bondarenko, AV

Conclusion: Combined immunodeficiency. Morbidity of BCG vaccination in the form of ulcer on the left shoulder with a torpid course and left-hand axillary lymphadenopathy. CMV-infection. Anemia. Catarrhal Proctosigmoiditis. Recommendations have been taken into consideration. During the examination of TORCH infections positive PCR for CMV in the blood were identified. The child received specific therapy with Cyvemene (Ganciclovir), later transitioning to receiving Valaciclovir. During the stay in the hospital, the child was constantly receiving antibacterial products (incl. and TB), antifungal and antiviral therapy. Periodically (interval of 2-3 weeks) received IV immunoglobulin. During the observation period with an interval of 2-3 weeks, the child showed rises in body temperature, which required the administration of immunoglobulin. The child is not gaining weight, stunted, occasionally concerned about pain and bloating 10.26.12 performed colonoscopy: Mucous direct and distal sigmoid colon bright hyperemia dramatically swollen, without destruction foci, vascular pattern is absent; when in contact with the endoscope is not injured. For diagnostic purposes taken multiple sigmoid colon mucosal biopsy without complications.

  

Conclusion: Severe catarrhal proctosigmoiditis. Histological examination of the sigmoid colon biopsy (30.10): Conclusion № 25892/12g.: In a sample biopsy there are intestine tissue fragments, in which in the lamina propria determined pockets of connective tissue and a small lymphocyte - plazmocyte infiltration. Marked proliferation of the glandular lesions enteroepitelium and with the presence of dense oxiphylic discharge on the surface enteroepitelium. Histological examination of the area of ​​the ileum (from 21.07): Conclusion №7220 - 26/12g.: In the preparations defined abscess appendicitis, gangrenous phlegmonous inflammation of the intestine. Histological examination of the lymph node tissue biopsy (05.10) Conclusion № 23739-40: The test material contains small parts loose fibrous and fatty tissue with lymphoid infiltration, karyorhexis sites. Histological examination of the biopsy of the intestine at the Institute of Tuberculosis and Pulmonology FG Janowski (27.11) - There is no data for a specific lesion of the intestine. The patient got IV Vennoimmun 2.5 grams  №3 (03.10-05.10), Bioven Mono 1.25 g №3 (19.10-21.10), 2.0 g Octagam №3 (13.11-15.11), 2.0 g №2 (26.11-27.11) 2.0 07/12/12 c. GCS IV: Methylprednisolone 15 mg / s from 03.10 till 05.10, and 20 mg / s from 06.10 till 09.10, 15 mg / s 10.10 14.10,10 mg / s from 15.10 till 02.11,7,5 mg / s 03.11 on 28.11.12, orally 4 mg, Metypred from 29.11.12. Cymevene 45 mg x 2 times IV from 27.10 to 21.11, Invanz, Ciprofloxacin, Amikacin, Zyvox, Levofloxacin, Fluconazole, Orungal, Isoniazid 0.09 mg x 2  from 05.10 till 23.11, then 80 mg / s from 23.11 , Rifampicin 80 mg / s from 24.11; Ethambutol (0,160. g) from 24.11, Valavir, Biseptol, Tamiflu. Patient was treated in Odessa CSTO from 13.01 till 15.02.13, where the state has deteriorated sharply after the infusion 07.02.13 Bioven Mono, terminal state due to lower hemoglobin to 15 g/l, cardiac arrest. Resuscitation carried out effectively,  packed RBC transfusion was performed. In myelogram from 07.02 - Red germ represented by single normoblasts, erythropoiesis-0, 7%. 15.02 discharged on the demand of parents.

The day of entering the hospital, patient state objectivly: the child had extra low nutrition (1 year 11 months., weight 7.2 kg), small amount of hair, small pointed teeth. The skin is very dry, purplish-red in color, with crusts scratching, big scale peeling, turgor was reduced. Multiple palpable enlarged cervical, axillary and inguinal lymph nodes, max. up to 2 cm. Belly increased in size, very tight. Pasty stools with mucus, 2-3 times a day.

CBC (02.17.13) - HGB 10.2 g/dl; HCT - 29.7, MCV - 91, RBC - 3.25, Ret - 83 ppm, WBC 8.1, EOS 13%, LYMPH - 41%, MONO - 2%, Segmented NEU - 37%, Banded NEU - 7%, ESR - 14 mm/hour

BioChemical Blood Tests (02.17.2013) - Serum Total Protein - 63 g/L, Albumin - 35.5, BUN - 0.9 mg/dL, CRN - 20 mkmol/L, GLU - 3.2 g/L, TBIL - 8.0 mmol/L, ALT - 20 U/L, AST - 23 U/L, LDH - 439 U/L, AMYL - 26 U/L, GGT - 9.0 U/L, TG - 1.43 mmol/L, CRP <1.0, Serum Iron - 18.3, Hp - 148.

Subpopulation of lymphocytes	differentiation markers	physiological range (%)	Contents cells (%)
			18.02.13	26.03.13
T-lymphocytes	CD3+	58-85	92.4	71.9
Active T-lymphocytes	CD3+ HLA-DR+	3-15	71.6	47.2
T-helpers	CD4+ CD8-	30-56	67.3	21.6
T-suppressors	CD4- CD8+	18-45	13.3	25.7
Th/Ts	CD4+/CD8+	0.6-2.3	5.06	0.91
Cytotoxic non T-lymphocytes	CD3- CD8+		0.6	1.7
cortical thymocytes	CD4+ CD8+	0-1	-
В-lymphocytes	CD19+	7-20	5.0	17.6
Natural killer cells	CD16+ CD56+	5-25	2.1	7.6
Natural T-killer cells Active Т-helpers thymic migrants В-memory cells	CD3+ CD16+CD56+ CD4+ CD25+ CD4+ CD31+CD45RA+ CD 19+CD27+IgD-	0-5 3-10 >35 5-15	1.9 2.34 0.4 25.3	9.6 21 0.1 31.2
Leukocyte "gate"	CD45+ CD 14-	>95	99	99.9
Humoral immunity
IgG		7.0-16.0	11,8
IgM		0.4-2.3	1,86
IgА		0.7-4.0	0,72

Conclusion (20/02/13) Neutrophil oxygen-generating function is not compromised. In terms of cellular immunity revealed a sharp decrease in thymic migrants CD4 + CD45RA + CD31 + - 0,49% (at a rate of 35%). Also revealed a dramatic reduction of regulatory T cells CD4 + CD25 + CD127-- 0,8% (rate of from 3 to 10%). In the study of the repertoire of T cell receptor expression infringements TCR Vb5.2, TCR Vbl8, TCR Vb2, TCR Vb11, and T-cell expansion 3 + TCRgamma / delta - 39% (the rate of up to 10%).

Conclusion (30/03/13): Given the results of the immunological studies of peripheral blood (reduction of regulatory T-l, the complete absence of thymic migrants, a high titer of IgE and the ambiguity of the phenotype of T-and B-l and NK, was carried out the molecular genetic study of the ADA gene (result - mutations are not detected), and the gene RAG1 - revealed a homozygous mutation in the coding region of genomic DNA c.256-257delAA, g.368-369delAa, amino acid substitution p.k86fs kl78. Based on the identified genetic and immunological changes the diagnosis: lack of RAG1, atypical ( severe) combined immunodeficiency with the expansion of TCR gamma / delta + T-lymfocyte and the presence of autoimmune disease (autosomal recessive mode of inheritance.) Definition of parents carriage of abnormal alleles of RAG1 - the result is not ready.

Coagulation (20/02/13) aPTT - 28 (31), prothrombin time - 14.7 (14.4), the activity of prothrombin complex - 96% -17 thrombin time (11.3), fibrinogen - 2.0 g / l.

Examination by a neurologist (02/18/13) condition was grave. Naughty. The skin is red with scratching. Head circumference of 45.5 cm Condition after resuscitation 07.02., Terminal condition due to lower hemoglobin of 15 g / l. CHN unremarkable. Expressed malnutrition, skin hangs. Tissue turgor is absent. Decreased muscle tone. Hands reflexes D = S low, legs - no reflexes. Weakly protective reflex. Footing presents . Meningeal symptoms are not detected. Conclusion: Due to the sharp depletion of the child, treatment with anti-TB drugs, lack of reflexes at legs, periodic fever tuberculous meningitis  should be excluded (excluded 19.02). Polyneuropathy, probable degenerative disease of the central nervous system.

Inspection endocrinologist (2/21/13): Physical development is low, disharmonious by weight. Alopecia. Since August gets GCS, blood glucose is normal, normal elektrolitemy. The parents do not have endocrine diseases. In the blood - increasing TSH and decreased FT3. Dz: Exogenous hypercortisolism. Physical developmental delay. Underweight. Primary hypothyroidism (may be because malabsorption).

Revision of histological specimens (resection of the ileum section 21.07.12), the conclusion №1090 - 97 (19/02/13): Fragments of the tissue of the small intestine with areas of necrotic suppurative inflammation, fragments of necrotic tissue.

Virology cell culture (02/19/13) In the isolated cell culture CMV and HSV 1/2 type.

Virology blood 2/21/13: PCR for EBV - neg.

25.02.13: IgM and G to PVB19 -  neg.

25.02.13: PCR for HSV ¹A (received acyclovir IV), HHV - neg., CMV-positive.

26.02.13: AgCMV (pp 65) - neg.

Blood on isohemagglutinins (02/19/13) titer betta At - 1:4.

Blood on IgE (19.02.13): IgE - 55000,0 (normal range 60) IU / ml.

Likvorogramma (19/02/13): glucose - 3, 9, protein-0, 099, cytosine 1\3, mon.-1.

• - a skin biopsy of anterior abdominal wall morphological conclusion № 2029 (02/22/13): The skin with marked acanthosis, weak parakeratosis. In the dermis - expressed  perivascular predominantly globocellular infiltration with single eosinophilic leukocytes. Histologically dermatitis without signs of specificity.

Throat swab (17/02/13): Alpha-hemolytic streptococcus-H-H-, E.cloacae + + + +, C.crusei + +, C.famata + +.

Fecal culture (17/02/13): Pathogenic intestinal flora not found. C.glabrata + + + +, C.crusei 1.

Urine culture for sterility (19.02.13): E.faecalis 10 koE / ml.

Sowing biopsy mucus duodenum(22.02.13): Sphmon.paucimobil. from environs enrichment.

The research activity of adenosine deaminase in R (26.02.13): 19 days after transfusion, 77.7 nmol / mg Hb x hour (control 72.8 + 12.8 mg Nb x hour, n = 28).

The blood on hormones (2/22/13): FT4 -11 4 pmol / L (normal 11-21)

FT3. - 3.09 pmol / L (normal 3,2-7,2)

TSU - 9,38 mkME / ml (normal 0,2-4,2)

AT to TG-15, 71 mIU / mL,

AT to TPO - less than 5 mIU / ml

Prolactin - 165.9 mkME / ml (normal less than 350).

Lymph nodes ultrasound (2/18/13): In the cervical region multiple anterior neck, back neck lymph nodes, max. up to 20x10 mm. Also increased all of peripheral lymph nodes  (max. inguinal right 28x10 mm), with marked hyperplasia, infiltrative changes in the cortex, hilus lymph nodes, increased blood flow in the color Doppler charting mode .

Ultrasound of the abdomen (02/18/13): Study difficult due to the pronounced flatulence. 1 cm increase in liver, spleen 3.5 cm. The intestinal wall change, swollen, infiltrated with increased blood flow in the color Doppler charting mode. Diffuse changes in the liver.

Ultrasound of the heart (02/21/13): SF-36% EF-68%. Rates of overall contractility of left ventricular are suitable. Pericardium - unremarkable.

Electromyography (2/19/13): Severe violations of the motor fibers on small and tibial nerve axonal type.

Сhest X-ray (19/02/13) Available  for visualization lung fields without a clearly defined focal and infiltrative shadows. Pulmonary drawing on both sides of moderately diffuse asymmetrically enriched at the expense of interstitial component, deformed, fuzzy. Changes are expressed in S1, S3, S9-S10 of  right lung. The roots of the lungs are not expanded, low structural. The head of the left root unremarkable. Pleural sinuses are not shaded. Clear aperture is located at the front segments 5-6th ribs. Mediastinum normal size and configuration, in middle position. Сentral venous complex (right) - a projection of the internal jugular vein on the right above the C4. Visible bone tissue of the thoracic cavity without structural changes. Conclusion.: moderately expressed bilateral interstitial lung changes similar to those of permanent change of RI. Сentral venous complex (right) - a projection of the internal jugular vein on the right above the C4.

Cerebral CT (19.02.2013): Pathological space-occupying lesions in the cranial cavity are not revealed. Basal cisterns, the ventricles of the brain, cortical furrows are moderately dilated. Median structures are not displaced. There is a periventricular decrease in the density of white substance of the brain. The sella of normal size and shape. Conclusion: CT-picture of encephalopathy.

FGDS (22.02.13): An area of hyperplasia in the antral mucosa in the greater curvature projection.

Morphological conclusion № 2071-79 (27.02.13): № 1 (mucosa of the duodenum) - small pieces of the mucosa of the duodenum. Villus surface epithelium is partially missing maintaining villous stroma. On other sites - a significant shortening of the villi with crypt hyperplasia (tangential sections can not be excluded).

In the stroma of the mucous membrane of the duodenum - multiple lymphocytes and plasma cells. № 2,3 (antral mucosa) - areas of the mucous membrane of the stomach antrum type with minimal signs of chronic inactive gastritis and beginning fibrosis of the stroma (undefined atrophy). H.pylori (-).

Blood markers for autoimmune diseases (02/19/13)

AFL: a/B2-Glycoprotein Ig M / G - neg. a / Cardiolipin Ig M / G - neg.

ANA-8S - neg.

ANCA: MPO (Ig G) - neg.

PR3 (Ig G) - put.

GBM (Ig G) - neg.

AMA-M2 - in work LKM-1 - in work ASCA IgA / G - neg.

Gliadin IgA / G - neg.

tTg IgA / G - neg.

During the period of stay at hospital - marked improvement of the skin, the skin became normal color without desquamation and peeling. IgE levels during treatment with methylprednisolone  8 mg / day intravenous decreased to 6280.0 IU / ml (06.03.13) to 2296.0 IU / ml (02.04.13).

Abdominal cavity organ US (03/05/13): At the time of examination the intestinal wall is from 1.6 mm to 2.6 mm (border-line values ​​for the age). Overstretched bowel loops filled with liquid contents, active peristalsis. In the upper part there is evident flatulence. Data for the presence of clinically significant amount of free fluid was not obtained.

The negative dynamics of the gastrointestinal tract: an increase in the size of the stomach, the stomach is very tight, apparent venous network on the anterior abdominal wall. The child was first given the mixture based on hydrolysates (Alfarae), and then the pure amino acid (Neocate). Given the negative trend in the defecation 15.03 a purgative enema was given, followed by an ultrasound scan and an abdominal cavity organ picture.

ACO US (15.03.13): After the purgative enema, apparent flatulence. Organs are located fragmentary. The intestinal wall 2.7-3.7 mm, in the right iliac region there is pendulum movement of intestinal contents.To differentiate intestinal impassability against an adhesive illness and a dynamic form.

Radiograph of the abdomen (15.03.13): The lower lung fields with no visible focal and infiltrative shadows. The outer edge-diaphragmatic sinuses are not shaded. Stomach increased in volume. Significantly pneumotisized loops of small bowel and colon. Intestinal wall thickened to 2.5 cm. At mesogaster in the right horizontal fluid level (up to 4.2 cm wide)  is determined. In the subphrenic area to the right there is visualized an enlightenment strip up to 0.15 cm wide due to the presence of free air in the abdominal cavity. The side channels are not differentiated. Conclusion: Given the history X-ray, the picture is similar to enterocolitis, perforation of a hollow organ in the abdomen, dynamic intestinal impassability.

15.03.13 - midline laparotomy. Revision of the abdominal cavity. Transanal bowel intubation. From the transcript of the operation: median laparotomy on the old scar. No effusion in the abdominal. Revealed diffuse imbibition by small-and medium-caliber gas bubbles all over the walls of the colon, crepitus. Mesentery of the entire colon imbibitised by large and very large bubbles of gas. Also determined crepitus. With a detailed examination of all the large and small intestines evident perforation with rupture of the free abdominal cavity and in the mesentery of the intestinal contents were not found. The transanal intubation of intestines with a probe institution to Treitz’s ligament was executed. Effusion for an anaerobic infection taken. Postsurgery diagnosis: Anaerobic total colitis. Pneumomezenterium. Multiple microperforation of the colon. Endoperitonitis. Pneuomoperitoneum.

Treatment in the postoperative period: Metronidazole, Tienam, Vancomycin.
Inoculation of the washings of the abdominal cavity (15.03.13): Klebsiella oxytocae from enrichment medium (sensitive Cefazolin, Cefotaxime, Ceftazidime, Cefepime, Imipenem, Cmikacin, Ciprofloxacin, Tetracycline, Trimethoprim/Sulfometoksazol).
Against postoperative antibacterial therapy the pnevmatization of intestines decreased. However, when added a complex mixture to nutrition enterally - the rapid growth of skin manifestations and  flatulence.
The girl was repeatedly consulted by the main children's gastroenterologist of Ministry of Health of the Republic of Belarus Savanovich I.I. Recommended parenteral nutrition and amino acid mixture "Neocate", to alternate dilution of 100% and 50%, the volume of feeding – depending on tolerance.

20.03.2013 The girl was discussed at the BMT board. The only treatment is to conduct an allogeneic bone marrow transplantation. The search for an unrelated donor was initiated. Currently performing the allo-BMT is impossible because of the infection and nutritional status of the recipient.

The girl was consulted by the deputy director of the clinic, MD Romanova O.N. and  received a 14-day course of antiviral therapy (Ganciclovir 10 mg / kg / day + antiTsMV immunoglobulin number 5), but at the follow-up (21.03.13) PCR for CMV - positive. Due to the lack of  Foskavir and Cidofovir at the hospital the dose of Ganciclovir was increased to 15 mg / kg / day + anti CMV Ig + Octagam 3 g / week twice. In a control test (02.04.13) PCR for CMV - neg.

Blood virology (21.03.13): PCR for CMV - positive.

Control test  (02.04.13) PCR to PMA - neg.

Fecal culture (29.03.13): PIF is not detected. Citrobacter freundii + + +, Morganella morganii + + +, Candida glabrata + + + (sensitive fluconazole, amphotericin, voriconazole).

Urine culture for sterility (02.04.13): Enterobacter cloacae, E.coli 106 KoE / ml. Enterobacter cloacae sensitive nitrofurantoin, amikacin, ciprofloxacin, resistant -augmentin, cotrimoxazole. E.coli sensitive - nitrofurantoin, amikacin, resistant - Ciprofloxacin, augmentin, cotrimoxazole.

ACO US (04.04.13): Evident  pneumatization of the intestines,  the wall in the visible areas is 2.5-3 mm. Free liquid is not determined.

ACO CT. Chest CT (05.04.13): Significant expansion of the lumen of all parts of the colon up to 25-35 mm with a doubling of its contour due to the presence of air layer of 3-4 mm thick. Pathological space-occupying lesions in the abdominal cavity and retroperitoneal space were not revealed. The liver is not enlarged, of homogeneous structure. The pancreas is homogeneous, normal size and position. The kidneys, spleen have no visible changes. Retroperitoneal lymph nodes are not enlarged. Foci of abnormal density in the lung parenchyma are not found. Pattern of diffuse pulmonary is strengthened in all lung fields. In the mediastinum additional formations, pathologically enlarged lymph nodes are not detected.

Lobar and segmental bronchi are passable. The roots of the lungs are not expanded, structural. Axillary lymph nodes are not enlarged. The pleural cavities are free. Soft tissue and chest bone tissue are without structural changes. Conclusion: Signs of colitis, likely with anaerobic flora.

Clearance on endogenous creatinine: 110.6 ml / min x S of 01.04.13, 137,8 ml / min x S of 08.04.13.

Currently, the state of moderate severity, caused by colitis, cachexia. No signs of multiple organ failure. The child is transportable. The last episode of febrile temperature increase on 05.04.2013, No high temperature since 06.04.2013 after the prescription of teicoplanin 70 mg / day and amikin 150 mg / day. Receiving parenteral nutrition, solyumedrol, biseptol, metronidazole, Octagam once every 1-2 weeks, teicoplanin, amikin, diflucan, fungizone, anti-CMV-Ig, L-thyroxine. At sanation of infectious foci, at the nutritional status improving,  with an unrelated donor available re-hospitalization at Belarusian Research Center for Pediatric Oncology, Hematology and Immunology for allo-BMT is possible.

The girl was discharged to continue treatment at the place of residence.

Recommended: - a mixture of "Neocate" for 2-3 weeks, with the positive dynamics - the transition to the mixture on the basis of high protein hydrolysis ("Alfarae");

- parenteral nutrition: kabiven 10 ml / hour permanently, infusion therapy: glucose 10% - 350 ml, glucose 40% -150 ml, NaCl 10% -10.0 ml, KS14% -30.0 ml, MgS04 25% -5.0 ml, Ca glyukonat10 % -5.0 ml, Vamin 18 - 60 ml, the infusion rate - 35 ml / hour; solumedrol 4 mg x 2 times a day intravenously continuously ( IgE control once a month);

Ganciclovir 7.5 mg / kg x 2 times per day by intravenous drip another 3 weeks (total of 5 weeks) at a negative PCR for CMV - switching to the maintenance dose (7.5 mg / kg / day at 1 injection) with positive PCR for CMV - Foskavir or Tsidafavir;

• Diflucan 50 mg x once a day continuously;

•Fungizone  capsules 250 mg  1\2 capsule x 4 times a day until intestines sanation;

• L-thyroxine 25 mcg x once a day daily;

• Metronidazole 250 mg  1 \ 4 tab. x 4 times a day until intestines sanation;

• Biseptol 120 mg x 2 times a day 3 days per week continuously;

• Octagam 2.5-3.0 g once every 2 weeks.

• CVC changing 2 times per week, CVC placed on 21.03.13 (v.subcl.dexttra).

When the negative dynamics of the intestine - Amikin 25 mg / kg / day or Tienam 100 mg / day/ intravenously

There is no quarantine in the department. The patient had no contact with  infectious patients.

The attending physician    Fedorov A.C.

Head of the Department    Kochubinsky D.V.

 

Deputy director of the clinic   Romanova O.N.