Complex cardiac malformation associated with pulmonary hypertension. Surgical treatment

Spitalul Clinic Republican “Timofei Moșneaga”, Secția Chirurgia Malformațiilor cardiace congenitale, Chișinău, Republica Moldova, Al XIII-lea Congres al Asociației Chirurgilor „Nicolae Anestiadi” și al III-lea Congres al Societății de Endoscopie, Chirurgie miniminvazivă și Ultrasonografie ”V.M.Guțu” din Republica MoldovaIntroducere: Malformații cardiace congenitale (MCC) complexe cu șuntare intracardiacă, sunt asociate cu hipertensiune pulmonară (HTP) severă. Iar în lipsa tratamentului chirurgical pot dezvolta rapid Sindromul Eisenmeger. Scopul studiului: Analiza experienței tratamentului chirurgical al anomaliilor MCC complexe. Materiale și metode: În secția chirurgia MCC a SCR din anii 2015 pînă în 2018 au fost operați 51 copii. Grupul de patologii incluse în studiu au fost: Canal atrioventriclar coplet (CAVC) - 30(58,8%) pacienți, Ventricol drept cu cale dubla de iesire (VDCDE)-11(21,5%), Ventricol Unic (VU) - 8(15,%) și Transpoziția de vase magistrale cu DSV (TVM+DSV) - 2(3,9%). Rezultate: Tratamentul chirurgical a inclus corecție etapizată, prima operație fiind cea paleativă “banding de AP” și apoi corecția radicală. Au fost supuși tratamentului chirurgical pe etape 25 (49%), iar corecție radical au suportat din prima 26(51%). Toți copiii cu media PSVD = 54,5mmHg în preoperator. Complicațiile postoperatorii: Pneumonie în 27 cazuri (52,9%), pleurezii în 7 cazuri (13,7%), bloc AV gr.III-2 cazuri. Iar 5(9,8%) cazuri soldate cu deces. Concluzii: Rezultatele operațiilor sunt în strînsă corelație cu vîrsta bolnavului, greutatea acestuia, dereglările hemodinamice, gradul de HTAP și fonul clinic general. Bandingul arterei pulmonare este o metodă cu risc relativ sporit (mortalitate de 5,8%), dar este etapă importantă în managementul pacienţilor cu insuficienţă cardiacă progresivă, hipotrofie şi anomalii asociate. În grupul cu MCC complexe mortalitatea a fost de 9,8%(5 copii) cu toții fiind sub 6kg și vîrsta medie de 4,1l.Introduction: Complex congenital cardiac malformations with intracardiac shunt are associated with advanced of the surgical can rapidly develop Eisenmeger s syndrome. The purpose of the study: was to analyze the experience of surgical treatment of complex congenital heart abnormalities. Materials and Methods: 51 children were operated in the CCM section of SCR in the years 2015 to 2018.The group of pathologies included in the study were: Atrioventricular septal defect (AV-canal)-30 patients, Double Outlet Right Ventricle (DORV)-11 patients, Single Ventricle (SV) - 8 patients, and Transposition of the Great Arteries (TGA) associated with ventricular septal defect-2 patients. Results: Surgical treatment included correction in stages, first was palliative operation ”PA banding” and then radical correction. Twenty-five patients were surgically staget, and 26 patients underwend primary-correction. All children with PSVD mean=54,5mmHg (pressure) in preoperative time. Postoperative complications: Pneumonia in 27 cases (52,9%), pleurisy in 7 cases(13,7%), A-V block in 2 cases and 5 cases(9,8%) of death. Conclusion: The results of the operations are closely correlated with the patient s age, body weight, hemodynamic disturbances, grade of pulmonary hypertension, and general clinical condition. Pulmonary artery banding is a relatively high risk method (5,8% mortality), but is au important step in the managements of patients with progressive heart failure, hypertrophy and associated abnormalities. In the group with complex congenital malformations the mortality was 9,8%,all being below 6kg and the mean age of 4,1 years

Postoperative complications in adult patients with congenital heart malformations and pulmonary hypervolemia

Departamentul de chirurgie cardiovasculară și toracică, IMSP Spitalul Clinic Republican ”Timofei Moșneaga”, Chișinău, Republica Moldova, Al XIII-lea Congres al Asociației Chirurgilor „Nicolae Anestiadi” și al III-lea Congres al Societății de Endoscopie, Chirurgie miniminvazivă și Ultrasonografie ”V.M.Guțu” din Republica MoldovaIntroducere: Incidența în creștere a malformațiilor cardiace congenitale (MCC) și a complicațiilor dezvoltate la maturitate, care înrăutățesc calitatea vieții și prognosticul acestor pacienți prezintă o problemă socio-medicală importantă. Astfel, studiul particularităților tratamentului chirurgical la bolnavii cu MCC și hipervolemie pulmonară (HP) este actual. Material si metode: În lucrare sunt prezentate rezultatele studiului unui lot de 461 pacienți cu vârste cuprinse între 19 și 69 ani, dintre care 310 (67,2 %) au fost femei și 151 (32,8%) au fost bărbați, cu MCC și HP, operați în perioada 1988-2017.Cel mai reprezentativ lot este alcătuit de bolnavii cu defect septal atrial - 369 (80,0%), urmat de defect septal ventricular - 52 (11,3%). Rezultate: Complicațiile pulmonare sunt specifice bolnavilor cu HP. Pneumoniile postoperatorii au fost prezente în 36% și pleureziile - în 23,1% cazuri. Postoperator clasa funcțională (CF) II NYHA s-a înregistrat la 306 (66,4%) pacienți și CF I NYHA - la 120 (26%). 4(8,7%) au rămas în CF IV NYHA, restul fiind în CF III NIHA. Mortalitatea a alcătuit 1,08% (5 pacienți). Concluzii: Tratamentul curativ a VCC este exclusiv chirurgical, de preferinţă până la apariţia hipertensiunii pulmonare severe sau ireversibile. Rezultatele operatorii nu depind de vârsta bolnavului ci de dereglările hemodinamice şi gradul hipertensiunii pulmonare preoperatorii. Modernizarea tehnicilor chirurgicale şi a metodelor de protecţie miocardică au dus la scăderea complicaţiilor şi a mortalităţii post operatorii.Introduction: The increasing incidence of congenital heart malformations (CHM) and complications developed at maturity that aggravate the quality of life and the prognosis of these patients present an important socio-medical problem. Thus, the study of the particularities of surgical treatment in patients with MCC and pulmonary hypervolemia (PH) is a mast. Material and methods: The paper presents the results of a study of a group of 461 patients aged between 19 and 69 years of which 310 (67.2%) were women and 151 (32.8%) were men, with CHM and HP, operated between 1988 and 2017. The most representative group is made up of patients with atrial septal defect - 369 (80.0%), followed by ventricular septal defect - 52 (11.3%). Results: Pulmonary complications are specific to patients with HP. Postoperative pneumonias were present in 36% and pleural effusion - in 23.1% cases. Postoperative functional class (FC) II NYHA was recorded in 306 (66.4%) patients and FC I NYHA - at 120 (26%). 4 (8.7%) remained in FC IV NYHA, the rest being in FC III NIHA. Mortality accounted for 1.08% (5 patients). Conclusions: The curative treatment of CHM is exclusively surgical, preferably until severe or irreversible pulmonary hypertension. Operator results do not depend on the age of the patient but on hemodynamic disturbances and the degree of preoperative pulmonary hypertension. The modernization of surgical techniques and methods of myocardial protection have led to the decrease of postoperative complications and mortality

Cardiovascular syphilis. Syphilitic aortitis. Clinical cases

Secția Chirurgie Malformații Cardiace Congenitale, IMSP Spitalul Clinic Republican ”Timofei Moșneaga”, Chișinău, Republica Moldova, Al XIII-lea Congres al Asociației Chirurgilor „Nicolae Anestiadi” și al III-lea Congres al Societății de Endoscopie, Chirurgie miniminvazivă și Ultrasonografie ”V.M.Guțu” din Republica MoldovaIntroducere: Sifilisul cardiovascular este o formă tardiva a sifilisului visceral, care se manifestă prin mezaortită. În majoritatea cazurilor se complică cu anevrism a aortei , insuficiența valvei aortice și stenozarea ostium arterelor coronare. Material și metode: Prezentăm 3 cazuri de anevrism aortic sifilitic operați în Spitalul Clinic Republican,”Timofei Moșneaga”. Diagnosticul a fost confirmat preoperator prin investigații imagistice (Radiografie, CT vaselor magistrale) și investigații de laboratorserologice, iar postoperator confirmate prin histologia fragmentelor de aorta anevrismatică înlăturate. La toți 3 pacienți s-a intervenit chirurgical – Înlocuirea segmentului aortal anevrismatic, la 2 pacienți a fost nevoie de arrest circulator în condiții de hipotermie. Rezultate: Perioada postoperatorie la 2 pacienți (operați în condiții de arest circulator) complicată cu encefalopatie cu ameliorare treptată. Pacienții detubați în primele 30 de ore după intervenție. Externați la a 14-a zi postoperator. Plăgile cicatrizate per prima. Concluzii: Intervenția chirurgicală este metoda de elecție in tratamentul anevrismelor aortale sifilitice. Depistarea și tratamentul chirurgical al anevrismelor aortale sifilitice reduce numărul complicațiilor severe prin ruptura de anevrism.Introduction: Cardiovascular syphilis is a late form of visceral syphilis, which is manifested by mezaortitis. In most cases, it is complicated by aortic aneurysms, aortic valve insufficiency and coronary artery stenosis. Material and methods: We present 3 cases of aortic aneurysm caused by syphilis hospitalized in the cardiac surgery department. The diagnosis was confirmed preoperatively by imagistic investigation (X-Ray, CT), and serological investigations. Postoperatively confirmed by the histology of the removed aneurysmatic aortic fragment. All patients were surgically intervened by prostheses of the affected aortic segment, in 2 patients there was a need for circulatory arrest under conditions of hypothermia . Results: Post-operative period in 2 patients (operated under circulatory arrest) was complicated with encephalopathy that gradually ceded. Patients were extubated within the first 30 hours after syrgery. Discharged after 14 postoperative days. Primary scarring healing. Conclusions: Surgery is the method of choice in the treatment of aortic aneurysms caused by syphilis. The detection and surgical treatment of aortic aneurysm caused by syphilis reduces the number of severe complications represented by rupture of aneurysm

Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries

Implementing the minimally invasive cardiac surgery in the Republican Clinical Hospital ”Timofei Mosneaga”

Scopul lucrării. Evaluarea perioadei de implementare a intervențiilor cardiace minim invazive, evidențierea primelor diferențe a rezultatelor clinice obținute de către pacient comparativ cu intervenția cardiochirurgicală clasică. Materiale și metode. Conform programului implementării, la etapa obținerii de formare echipei și a tehnicii operatorii, în perioada de debut (noiembrie 2021-iunie 2023) au fost operați prin tehnica minim-invazivă 40 de pacienți. Leziunile cardiace au fost atât din malformațiile cardiace dobândite, cât și cele congenitale, astfel s-au format 5 grupuri de pacienți: 1. Pacienți cu patologie Mitrală izolată – 15 (plastie valvulară/ înlocuire); 2. Pacienți cu patologie Aortică – 12 (înlocuire valvulară); 3. Pacienți cu patologie Mitro- Tricuspidă 8 (plastie valvulară/ înlocuire); 3. Pacienți cu tumoare cardiacă – 3 (mixoame cardiace); 4. Pacienți cu patologie congenitală – 2 (defect de sept atrial, bicuspidie de Aortă). Toți pacienții au fost operați cu circulație extracorporeală cu canulare periferică doar femurală, cu ecocardiografie transesofagiană perioperatorie. Rezultate. Tehnica minim-invazivă în perioada de debut a reușit de aplicat în 18% din volumul total al operațiilor cardiochirurgicale clasice de corecție valvulară, astfel devine accesibilă pentru patologii cardiace complexe. Timpul aflării pacientului în Terapie Intensivă este redus cu 1 zi. Recuperarea postoperatorie variază între 7-10 zile comparativ cu evoluția clasică (12-18 zile). Volumul hemotransfuziei la pacientul cu intervenție cardiacă minim-invazivă este redus cu 40 %. Reintervenție pentru hemostază nu a necesitat nici un pacient. Mortalitatea constituie 0 %. Conversie de la minim invaziv la sternotomie s-a înregistrat la 1 pacient, care constituie 2,5 %. Concluzii. Reducerea traumei operatorii (deoarece nu se efectuează sternotomia), reducerea infecției și sângerării, reducerea hemotransfuziei, reducerea timpului aflării pacientului în staționar devin factorii care fac diferența considerabilă a rezultatului operației cardiace minim-invazive. După obținerea experienței tehnica respectivă devine acceptabilă pentru intervențiile cardiace complexe (multivalulare, DSA+corecție valvulară, tumori+corecție valvulară).Aim of study. Evaluation of the start period of setting up the minimally-invasive cardiac surgery (MICS) program in a multiprofile hospital and emphasising the most important challenges we faced, to be taken into account. Materials and methods. Minimally invasive cardiac interventions started due to implementation of a dedicated project financed by the National Agency for Research and Development. According to the program several steps were planned: team building, gaining knowledge and skills, equipment and disposal acquisition, and patient selection. After rigorous selection, 40 patients with acquired, congenital heart diseases and tumors, operated during the debut period, could be divided in five categories : I : Patients with isolated Mitral pathology -15 cases (plasty/valve replacement); II: Aortic patients -12 cases of valve replacement; III: Patients with Mitral- Tricuspid pathology - 8 cases (plasty/valve replacement); IV: Patients with cardiac myxoma (3 cases); V: Congenital heart disease – 2 patients (bicuspid aorta, atrial septal defect concomitant tricuspid valve regurgitation). All operations were performed with peripheral femoral cannulation under TEE visualization. Short-term and long-term outcomes were studied. Results. Of the total cases of valvular correction in this debut period, minimally invasive operations (MICS) represented 18%. Conversion from MICS to Sternotomy was required for 1 patient (2,5%) without further complications. Mortality was 0%. Mean patients’ stay in ICU was 15h, mean overall stay in hospital was 7-10 days. Conclusions. The established implementing program of MICS proved to be sustainable even for poor settings. The minimallyinvasive technique is applicable in a range of heart valvular pathologies, tumours, congenital defects. Clinical results of the debut period showed increased benefits (reduction of operative trauma, minimal pain, rapid recovery, reduced infectious risk) in all patients. It is of high interest for the implementation of the technique in congenital heart diseases (VSD), but also in ischemic heart disease for minimally invasive myocardial revascularization (MIDCAB)

Table_1_Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021.docx

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.</p

Image_1_Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021.jpeg

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.</p

DataSheet_2_Care of patients with inborn errors of immunity in thirty J Project countries between 2004 and 2021.docx

IntroductionThe J Project (JP) physician education and clinical research collaboration program was started in 2004 and includes by now 32 countries mostly in Eastern and Central Europe (ECE). Until the end of 2021, 344 inborn errors of immunity (IEI)-focused meetings were organized by the JP to raise awareness and facilitate the diagnosis and treatment of patients with IEI.ResultsIn this study, meeting profiles and major diagnostic and treatment parameters were studied. JP center leaders reported patients’ data from 30 countries representing a total population of 506 567 565. Two countries reported patients from JP centers (Konya, Turkey and Cairo University, Egypt). Diagnostic criteria were based on the 2020 update of classification by the IUIS Expert Committee on IEI. The number of JP meetings increased from 6 per year in 2004 and 2005 to 44 and 63 in 2020 and 2021, respectively. The cumulative number of meetings per country varied from 1 to 59 in various countries reflecting partly but not entirely the population of the respective countries. Altogether, 24,879 patients were reported giving an average prevalence of 4.9. Most of the patients had predominantly antibody deficiency (46,32%) followed by patients with combined immunodeficiencies (14.3%). The percentages of patients with bone marrow failure and phenocopies of IEI were less than 1 each. The number of patients was remarkably higher that those reported to the ESID Registry in 13 countries. Immunoglobulin (IgG) substitution was provided to 7,572 patients (5,693 intravenously) and 1,480 patients received hematopoietic stem cell therapy (HSCT). Searching for basic diagnostic parameters revealed the availability of immunochemistry and flow cytometry in 27 and 28 countries, respectively, and targeted gene sequencing and new generation sequencing was available in 21 and 18 countries. The number of IEI centers and experts in the field were 260 and 690, respectively. We found high correlation between the number of IEI centers and patients treated with intravenous IgG (IVIG) (correlation coefficient, cc, 0,916) and with those who were treated with HSCT (cc, 0,905). Similar correlation was found when the number of experts was compared with those treated with HSCT. However, the number of patients treated with subcutaneous Ig (SCIG) only slightly correlated with the number of experts (cc, 0,489) and no correlation was found between the number of centers and patients on SCIG (cc, 0,174).Conclusions1) this is the first study describing major diagnostic and treatment parameters of IEI care in countries of the JP; 2) the data suggest that the JP had tremendous impact on the development of IEI care in ECE; 3) our data help to define major future targets of JP activity in various countries; 4) we suggest that the number of IEI centers and IEI experts closely correlate to the most important treatment parameters; 5) we propose that specialist education among medical professionals plays pivotal role in increasing levels of diagnostics and adequate care of this vulnerable and still highly neglected patient population; 6) this study also provides the basis for further analysis of more specific aspects of IEI care including genetic diagnostics, disease specific prevalence, newborn screening and professional collaboration in JP countries.</p