Biography:

Muhammad Riza Kurniawan is a medical staff in Division of Nephrology, Department of Child Health, Airlangga University-Dr Soetomo Hospital Surabaya Indonesia since 2013. He has his Pediatric Nephrology Training Program in Japan this year

Abstract:

Background : Lupus nephritis is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). The chronic autoimmune disease characterized by multisystem inflammation which often involves the heart, manifest as pericardial effusion. Large pericardial effusions can  cause cardiac tamponade.

Case Report : D, 15 years old boy, came to emergency department with the main complaints of dyspnea, since 7 days before, worsen gradually until one day before admission. From initial physical examination revealed an nervous boy with good nutritional status. The blood pressure was 110/60 mmHg with adequate pulse of 140 beats/minute, respiratory rate of 46 times/minute, temperature 36.9°C. Urine production was equal with 1.3 cc/kg/hour. Head and neck examination anemic and dyspnea were found. He had oral ulcer and frequently appear. Chest examination was symmetrical with retractions, heart sounds were muffled and the apex beat was diffuse and displaced inferiorly. Jugular venous distension was evident. In respiratory examination we found decreased breath sounds at the left lung base. The abdomen was slightly distended and we found hepatomegaly. From initial laboratory examinations the hemoglobin level was 10.0 g/dL, the white blood cells 29.870 per mm3 , The renal function test revealed BUN 22 mg/dL, SC 1.0 mg/dL, and GFR  85.25 mL/minute/1.73 m2. The liver function test resulted SGOT 3253 U/L, SGPT 2330 U/L, and albumin 2.4 g/dl, CRP was 59.38 mg/dL, C3 was 45.2 mg/dl. The others result were within normal limit. From urinalysis revealed erythrocyte 5-10 cell/hpf, leukocyte 5-10 cell/hpf, epithel 1-2/hpf, and proteinuria + 3. The chest X-ray and Echocardiography revealed massive pericardial effusion. The initial treatment included echocardiography guided pericardiocentesis and 400 mL of hemorrhagic pericardial fluid was drained, Tapping pericardiocentesis continue until 3^rd hospitalization. On the 15^th day of hospitalization, the patient was discharged with a good condition.

Biography:

Dr. Vy Vo is a medical nephrologist with expertise in pediatric nephrology such as systemic lupus erythematosus, pediatric chronic kidney disease, glomerular disease. She earned his medical degree and completed her medical residency in pediatric nephrology at Medical University at Ho Chi Minh City, Viet Nam. She is interested in systemic lupus erythemosus and CKD. Working in a biggest department of pediatric dialysis of the Southern of Viet Nam, she is trying to approach patients who need to dialysis, and spend many time to treat and take care of them by her ability as well as always update new knowledge and experience from colleagues. Dr. Vy had completed some CMEs about pediatric dialysis, pediatric kidney transplatation, glomerular disease in children: challenge and update. Furthermore, in her department, she is taking part in a research about: Measuring disease activity of SLE base on SLEIDA in pediatric outpatients

Dr. Vy Vo is a medical nephrologist with expertise in pediatric nephrology such as systemic lupus erythematosus, pediatric chronic kidney disease, glomerular disease. She earned his medical degree and completed her medical residency in pediatric nephrology at Medical University at Ho Chi Minh City, Viet Nam. She is interested in systemic lupus erythemosus and CKD. Working in a biggest department of pediatric dialysis of the Southern of Viet Nam, she is trying to approach patients who need to dialysis, and spend many time to treat and take care of them by her ability as well as always update new knowledge and experience from colleagues. Dr. Vy had completed some CMEs about pediatric dialysis, pediatric kidney transplatation, glomerular disease in children: challenge and update. Furthermore, in her department, she is taking part in a research about: Measuring disease activity of SLE base on SLEIDA in pediatric outpatients

Abstract:

Background: systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can involve many organs. In patients with SLE, there is an association of immunodysfunction due to the disease itself and immunocompromised conditions due to the use of immunosuppressive medications that put the patients susceptible to opportunistic infections. Cytomegalovirus (CMV), has been recognized in recent years to have multi-organ involvement in SLE patients. However, the screening and treatment for CMV infection in SLE patients has not been appropriately done.  Cases: we reported herein three cases of severe CMV disease in pediatric SLE patients. CMV infection was detected after SLE diagnosis, all were female from 12 to 16 years old, gastro-intestinal manifestations in two patients, proliferative nephritis in two, fever and hematologic manifestations in all patients. All patients presented a high viral load in the blood while tissue CMV found in two of three: cerebro-spinal fluid and bone marrow. Two patients received ganciclovir who fully recovered but one patient died with active CMV infection without antiviral treatment. Conclusions: This is the first report on CMV disease in SLE patients in Viet Nam, having changed our look in the management of infectious problem for these patients. 

Key words: CMV infection, lupus, pediatric SLE

INTRODUCTION

Childhood-onset SLE has an incidence of 0.53 to 0.9 per 100,000 children-years and a prevalence of 3.3 to 8.8 per 100,000 children [7]. This prevalence is much higher of 6.3 to 19.3 per 100,000 in some countries of Asia [3]. The manifestations of the disease is often more severe in children than in adults, with a high prevalence of renal involvement occurring in 29%–81% of patients [3]. In the absence of appropriate treatment the child may die from the disease or progress rapidly to renal failure. However, aggressive treatment regimens put the SLE patients susceptible to opportunistic infections that can be severe with sequelae and death. CMV is a widespread viral infection in the healthy population and is also the most found virus in SLE patients [2]. 

Cytomegalovirus (CMV) has been recognized in recent years to have multi-organ manifestations that can resemble active SLE disease and presented as an emerging problem in the past decade [2]. CMV infection relating to SLE can provoke the onset of SLE, exacerbates preexisting SLE, or arise symptoms due to CMV infection itself [12]. However, the screening for CMV infection in SLE patients has not been done universally. We reported herein three cases of severe CMV disease in cSLE at our institution. This is also the first report on CMV disease in SLE patients in Viet Nam.

CASE REPORT

Patient 1: a 16-year-old female patient first developed symptom of SLE in November 2016, starting with fever and polyarthritis of the wrists. Ten days later, she developed anemia and generalized edema. The diagnosis of systemic lupus erythematous has been undertaken according the ACR criteria 1997. The organ involvement composed of hemolytic anemia and diffuse segmental lupus nephritis IVs ( ISN 2004 classification). She was stably discharged after three steroids pulses, prednisone, mycophenolate mofetil (MMF) and chloroquine. On December 2016, she developed a period of diarrhea,fever and generalized convulsions. Lupus activity scoring was negative (normal C3,

C4, anti DsDNA negative). A quantitative PCR CMV was performed indicating 117,000 copies/ ml in blood and 2.65* 10⁵ copies/ml in cerebro-spinal fluid (CSF). This patient was treated with ganciclovir for 21 days then valganciclovir for 3 months. The patient recovered rapidly after 10 days with negativation of blood and CSF CMV.

Patient 2 : a 12-year-old female patient first developed symptoms of severe anemia requiring transfusion before being admitted to our institution. Systemic lupus erythematous was made with the presence of criteria according to the ACR 1997. At the time of diagnosis, she also presented a period of severe septicemia due to Stenotrophomonas maltophilia. She could passed over it with antibiotherapy however she continued to have prolonged fever, malaise and thrombocytopenia. A peripheral PCR CMV showing 100,150 copies/ ml associated with 4.26* 10⁶ copies/ml in the bone marrow. This patient was treated with ganciclovir for 28 days then valganciclovir for two months. Clinically, she recovered rapidly after 10 days. Table 1 showed the amelioration of the blood cell lineages. 

Patient 3: a 14-year-old female patient was diagnosed systemic lupus erythematous with cutaneous involvement and focal nephritis (class III ISN 2004). She was treated with steroids pulses, prednisone, MMF and chloroquine. One month later, she developed a period of bloody diarrhea and prolonged fever. In this course, she also developed a pneumonitis which finally progressed to an acute respiratory distressed syndrome. Neurologically, she presented some convulsions without cerebral hemorrhage and paralysis. She also presented a mild elevation of the transaminases and mild inhibition of the three blood lineages. At this time, a work-up for lupus activity showed negative (normal C3, C4, anti DsDNA negative). A quantitative PCR CMV was performed in the blood showed positive: 15,600 copies/ ml in blood, then 79,800 copies ten days later. Unfortunately, an aggressive immunosuppressive therapy was continued without treatment against CMV. After one month, this patient died in a tableau of multi-organ failure.

DISCUSSION

In pediatric SLE, the results of a meta-analysis demonstrated a high prevalence of neurologic and renal involvement, requiring a more intensive and prolonged immunosuppressive regimen [5]. Such a therapy face these population a high risk of CMV infection [13]. In our series, two of three patients had MMF and high doses of methylprednisolone, which were recognized as the risk factors for CMV infection [9]. However, one patient received only low dose of prednisone and chloroquine (case 3), suggesting other factors relating to this risk. Within two months we could find the presence of CMV disease in all clinically doubted cases. Until 2013, there was only 7 reported cases of CMV infection in pediatric SLE, most of them are non-Caucasian ethnicity [10]. Although there was a strong correlation between CMV infection and asian ethnicity, CMV screening was not routinely performed for lupus patients at our institution. Contrarily, some centers proposed routine and frequent screening by serology even in the absence of symptoms in lupus population [11]. Owing to our restricted conditions, it should be appropriate to base on the clinical symptoms to indicate the blood tests as well as tissue analysis for diagnosis. Clinically, we usually based on the common hematologic symptoms to search for CMV. Additionally, persistent fever despite steroids and antibiotics in SLE should increase our suspicion of CMV. In our series, 2/3 cases presented diarrhea, that was coherent with the literature [13]. These two patients also manifested abdominal crisis but there was no finding of pancreatitis. Pancreatitis might be a complication of CMV infection and can be successfully treated [6]. Two of three patients presented respiratory distress, unfortunately we could not get evidence of CMV in the lung as shown by other authors [1,9]. All of our patients presented a high viral load in the blood. There still be some debate whether the detection of CMV in the blood is sufficient or not for the diagnosis of CMV disease. Similarly, CMV in tissue analysis alone would not necessarily indicate active disease, but symptoms and histopathology can come together to maximize the sensitivity of CMV disease [4]. In practice, should a team working between rheumatologists and infectious specialists be warranted as there are until now no consensus guidelines to treat disease for these population. Practitioners also keep in  mind that an early use of antiviral agents might increase survival in these patients [2]. The patient who died in our series has presented a magnification of the viral load in a period of ten days. Our case might mimic the results from the postmortem studies that showed multiple organ involvement due to disseminated viral infection [9]. 

CONCLUSION

This is the first series of CMV infection in pediatric SLE in a short period of time, indicating the efficacity of treatment with ganciclovir. This report also alerted us to the burden of CMV and prompted us to change our future strategy in the management of infectious problem in this population. 

Conflict of Interest: The authors declare that they have no conflict of interest.

Biography:

Yuan-Hau Lin is a MD, specialist in Nephrology, and a current Ph.D. candidate in the College of Medicine at Taipei Medical University. He is the Secretary-General of the Taiwan Society of Nephrology since 2017. He has one co-author peer review article in PLOS.

Abstract:

Life as we know it does not sustain without water (H₂O). Because of all biochemical reactivity has to take place in water, thermodynamically, if any pathophysiology occurs, water molecule should be the first to response such an event. Prior art, the stable isotopic ratios of hydrogen (d²H) and oxygen (d¹⁸O) in human blood plasma of the end stage renal disease (ESRD) patients are shown to deviate from the normal renal function (NRF) subjects as supposedly the biological isotope homeostasis in human blood plasma should be preserved. Here we study the stable isotopic compositions of water from human blood plasma and from human erythrocyte, collected from a total of 53 ESRD patients and of 19 NRF subjects. A pathological water model established by using the ratio of d²H and d¹⁸O as a state function of thermodynamic entropy shows a plausible singularity of the ESRD. This entropy singularity describes a characteristic water intricacy of the ESRD. In addition, the waters within the erythrocyte and the blood plasma are distinct as regards the stable isotopic compositions of hydrogen and oxygen atoms of water molecule. Of the five NRF subjects who exhibit the ESRD’s entropy singularity are all common with hyperlipidemia. Our in vivo stable isotope water data and the pathological water model indicate that hyperlipidemia is the cause of chronic kidney disease (CKD), and eventually might progress to ESRD. We suggest that people who have hyperlipidemia should not only monitor the cholesterol level but also the stable isotope contents of the body water.

Biography:

Punit Gupta, was Profossser in Government medical college hospital (consider to be the tribal of india), raipur, India. He have done bachelor degree in 2000 I.e MBBS, than master degree in General Medicine in 2003 I.e MD, than Superspeciality in Nephrology I.e DM , With PHD DEGREE . He also awarded the prestigious ISPD SCHOLARSHIP and ASN fellowship. He have awarded with APCN developmental award in Malaysia. And also awarded with many oral n poster presentation National and International. He have presented highest number of papers I.e 31 abstract in single conference under his name till date around 175 paper and abstract is presented by him in different conferences

Abstract:

Aim:. Renal injury in patients of leprosy is due to acute tubular necrosis, drug-induced interstitial nephritis and rarely crescentic glomerulonephritis. Renal injury seems to be common in patients with erythema nodosum leprosum (ENL)

This study is based up on the detection of the renal injury in patient who had taken treatment for Hansen’s disease.

Material & Methods: The study was conducted on 69 patients of treated Hansen’s disease attending OPD & admitted in, Nephrology Unit, Pt.J.N.M.Medical College. All patients underwent general examination, BMI estimation & were subjected to CBC, S. urea, S. creatinine, urine albumin, urine sugar, and echo.

Results:

1. Total number of patient 69
2. 56.52% were males and 43.47% were females.
3. 56.52% of the treated Hansen’s disease patients were having renal injury.
4. Among the patients with proteinuria, 73.91% were males and 26.08% were females.
5. Mean age of the treated Hansen’s disease patients with renal injury is 62.05 ± 10.35 years.
6. Among the treated Hansen’s disease patients with renal injury 56.52% were hypertensive & anaemic.
7. 33.33% were underweight (BMI<18.5 kg/m²) of which 73.91% were males among the treated Hansen’s disease patients with renal injury
8. Among the treated Hansen’s disease patients with proteinuria, hypertension & anaemia, 85.50% had leprosy related deformity (claw hand, trophic ulcer, lost toes/fingers).
9. Anaemia was present in 82.60% of the patients, 56.52% were males and 43.48% were females.
10. 39.13% of the patients were having LVH by electrocardiography of which 77.77% had hypertension in treated Hansen’s disease patients with renal injury.
11. Renal replacement therapy i.e Hemodialysis required in 2.89% patients.
12. Only one death is noted among the patient of Total number of patient 69
13. 56.52% were males and 43.47% were females.
14. 56.52% of the treated Hansen’s disease patients were having renal injury.
15. Among the patients with proteinuria, 73.91% were males and 26.08% were females.
16. Mean age of the treated Hansen’s disease patients with renal injury is 62.05 ± 10.35 years.
17. Among the treated Hansen’s disease patients with renal injury 56.52% were hypertensive & anaemic.
18. 33.33% were underweight (BMI<18.5 kg/m²) of which 73.91% were males among the treated Hansen’s disease patients with renal injury
19. Among the treated Hansen’s disease patients with proteinuria, hypertension & anaemia, 85.50% had leprosy related deformity (claw hand, trophic ulcer, lost toes/fingers).
20. Anaemia was present in 82.60% of the patient of which 56.52% were males and 43.48% were females.
21. 39.13% of the patients were having LVH by electrocardiography of which 77.77% had hypertension in treated Hansen’s disease patients with renal injury.
22. Renal replacement therapy i.e Hemodialysis required in 2.89% patients.
23. Only one death is noted among Hansen disease with renal injury.

Conclusion:

1. Male were more commonly affected by Renal injury among treated Hansen’s disease patient
2. Renal injury was more commonly seen in geriatric age group patients.
3. Renal injury with Hansen disease shows more prevalence of Hypertension and anaemia
4. Treated case of Hansen disease with renal injury shows more leprosy related deformity (claw hand, trophic ulcer, lost toes/fingers) .
5. Most common finding on Echocardiography was Left ventricular hypertrophy seen among the treated Hansen’s disease patients with renal injury.
6. Underweight was more common in male patients of Hansen’s disease with renal injury.