Hemophagocytic lymphohistiocytosis as a rare complication of dengue haemorrhagic fever: a case report.

BACKGROUND: Haemophagocytic lymphohistiocytosis (HLH) is an uncommon systemic inflammatory syndrome that can happen secondary to numerous conditions. It rarely occurs due to dengue infection causing significant mortality and morbidity even with appropriate treatment. The outcome is further poor if the diagnosis of HLH is delayed or left untreated. Therefore, a high degree of clinical suspicion is paramount in diagnosing HLH. CASE PRESENTATION: A 17-year-old Sinhalese boy was admitted to a tertiary care hospital in Sri Lanka with a 4-day history of fever, headache, nausea, vomiting, and diarrhea. He was hemodynamically stable, and the serological investigation confirmed a dengue infection. On the fifth day of fever, he entered the critical phase of dengue infection, confirmed by ultrasound evidence of plasma leaking. However, he had ongoing high fever spikes during the critical phase, and even after the critical phase was over, the fever spikes continued. Simultaneously, hepatosplenomegaly was noticed, and he showed persistent thrombocytopenia, neutropenia, and anemia despite the resolution of the critical phase. Further, the workup revealed a serum ferritin level of > 3000 ng/mL triglyceride level of 314 mg/dL, and the bone marrow biopsy revealed an increased haemophagocytic activity. Secondary HLH was diagnosed on the basis of criteria used in the HLH-2004 trial and successfully managed with intravenous dexamethasone 10 mg/body surface area/day for the first 2 weeks, followed by a tapering regimen over 8 weeks. CONCLUSION: This case emphasizes the need to consider HLH as a potential complication when persistent fever and cytopenias are present after recovering from dengue fever, particularly in patients with unusual clinical features like hepatosplenomegaly. Early recognition and prompt treatment with appropriate immunosuppressive therapy, such as intravenous dexamethasone, can lead to a successful response and good prognosis.

Kounis Syndrome Secondary to Medicine-Induced Hypersensitivity.

INTRODUCTION: Kounis syndrome is the concurrence of an acute coronary syndrome (ACS) caused by coronary vasospasms, acute myocardial infarctions, or stent thromboses in case of allergic or hypersensitivity reactions. Kounis syndrome is mediated by mast cells that interact with macrophages and T-lymphocytes, causing degranulation and inflammation with cytokine release. It is a life-threatening condition that has many trigger factors and is most commonly caused by medicines. Case Presentation. A 71-year-old male was admitted with a fever of five days' duration associated with cellulitis, for which he had been treated with clindamycin and flucloxacillin before admission. He was a diagnosed patient with hypertension and dyslipidemia five years ago. After taking the antibiotics, he had developed generalized itching followed by urticaria suggesting an allergic reaction. Therefore, he was admitted to the hospital. After admission, he developed an ischaemic-type chest pain associated with autonomic symptoms and shortness of breath. An immediate ECG was taken that showed ST-segment depressions in the chest leads V4-V6, confirmed by a repeat ECG. Troponin I was 8 ng/mL. Acute management of ACS was started, and prednisolone 10 mg daily dose was given. After complete recovery, the patient was discharged with aspirin, clopidogrel, atorvastatin, metoprolol, losartan, isosorbide mononitrate, and nicorandil. Prednisolone 10 mg daily dose was given for five days after discharge. CONCLUSION: In immediate hypersensitivity, with persistent cardiovascular instability, Kounis syndrome should be considered, and an electrocardiogram and other appropriate assessments and treatments should be initiated. Prompt management of the allergic reaction and the ACS is vital for a better outcome of Kounis syndrome.

Pulsed Vincristine Therapy in Steroid-Resistant Nephrotic Syndrome.

Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. Methods. Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1.5 mg/m2 in weekly intravenous pulses for 8 weeks along with a tapering steroid regimen of 6 months. All patients were closely followed up for 5 years. Results. Of the 54 patients 39 were males and 15 were females (age range 3.5-11.6 years, median 6.1 years). At the end of the treatment course, 21 patients achieved complete remission while 7 had partial remission and no response was seen in 26 patients. Sustained remission at 6, 12, 24, and 60 months were 15 (27.78%), 11 (20.37%), 9 (16.67%), and 7 (12.96%), respectively. Most side-effects observed were reversible and no serious side-effects were noted during vincristine therapy. Conclusion. Although its therapeutic mechanisms in nephrotic syndrome are still not elucidated, vincristine appears to be a potent alternative that could be considered for treating SRNS.

Efficacy of higher-dose levamisole in maintaining remission in steroid-dependant nephrotic syndrome.

OBJECTIVE: Levamisole (LEV) has been used successfully on an alternate-day regime of 2.5 mg/kg in steroid-dependant nephrotic syndrome (SDNS) to maintain remission. This pilot study was carried out between 2010 and 2015 at a single center in Sri Lanka to evaluate the efficacy of LEV prescribed at 2.5 mg/kg daily, which is double the alternate-day dose. METHODS: Sequential children with SDNS, relapsing more than twice in the preceding 12 months and previously treated with LEV and low-dose alternate-day prednisolone (0.1-0.6 mg/kg) were recruited to the study. This group received LEV (2.5 mg/kg) daily with the same dose of alternate-day prednisolone for 1 year. Urine protein excretion was recorded by parents on a daily basis, and the presence of 3+ proteinuria on 3 consecutive days was considered a relapse. Full blood counts and liver function tests were performed every 3 months to monitor for adverse effects. RESULTS: Sixty-four children were enrolled into the study; six were excluded due to prescription of other immunosuppressive drugs. Median age was 7.9 years; 33 were boys. The number of relapse episodes was 163 [mean per patient 2.8 ± standard deviation (SD) 0.8] in patients on alternate-day LEV and 77 (mean 1.3 ± SD 0.9) for those on daily LEV during the 12-month period of observation. The P value 0.000 (according to the Wilcoxon signed-rank test) was <0.001. No major adverse events were noted. CONCLUSIONS: The prescription of daily LEV is effective and safe for maintaining SDNS remission.

Short courses of daily prednisolone during upper respiratory tract infections reduce relapse frequency in childhood nephrotic syndrome.

BACKGROUND: Relapses of childhood nephrotic syndrome (NS) are frequently precipitated by viral upper respiratory tract infections (URTIs). A review of the literature reveals that in patients with steroid-dependent NS on alternate day corticosteroids, a short course of daily corticosteroid therapy during the course of an URTI may reduce relapse frequency. OBJECTIVE: To assess the effect of a short course of low-dose corticosteroid therapy during the course of an URTI on relapse frequency in patients with steroid-sensitive NS who have not been taking any treatment for a minimum period of 3 months. METHODS: A double-blind placebo-controlled crossover trial was conducted on 48 patients with idiopathic NS who had not been receiving corticosteroid therapy for a minimum of 3 months. Patients were randomized into two groups. Group A received 5 days of daily prednisolone at 0.5 mg/kg at the onset of an URTI while group B received 5 days of placebo. Both groups were followed up for 1 year and the URTI-induced relapse frequency was noted. A crossover was performed during the next year, with group A receiving placebo and group B receiving prednisolone. RESULTS: Thirty-three patients completed the study. In the treatment group, 115 episodes of URTI led to 11 relapses while in the control group 101 episodes of URTI led to 25 relapses. There was no significant difference between the mean number of URTIs between the treatment and control groups. The treatment group had significantly less relapses compared to the control group (p = 0.014). Within the treatment group, 65.6% did not relapse, while the remainder had a single relapse. In contrast, only 40.6% of the control group remained in remission while 40.6% suffered a single relapse and 18.8% had two or more relapses. CONCLUSIONS: Prescribing a short course of daily corticosteroids during an URTI significantly reduces the frequency of URTI-induced relapse in patients with steroid-responsive NS who are off corticosteroid therapy.