Kawasaki Shock Syndrome with Initial Presentation as Neck lymphadenitis: A Case Report

Kawasaki disease (KD) is an acute systemic vasculitis of unknown cause that mainly affects infants and children and can result in coronary artery complications if left untreated. A small subset of KD patients with fever and cervical lymphadenitis has been reported as node-first-presenting KD (NFKD). This type of KD commonly affects the older pediatric population with a more intense inflammatory process. Considering its unusual initial presentation, a delay in diagnosis and treatment increases the risk of coronary artery complications. Herein, we report the case of a 9-year-old female with fever and neck mass that rapidly deteriorated to shock status. A diagnosis of KD was made after the signs and symptoms fulfilled the principal diagnostic criteria. The patient’s heart failure and blood pressure improved dramatically after a single dose of intravenous immunoglobulin. This case reminds us that NFKD could be the initial manifestation of KDSS, which is a potentially fatal condition. We review the literature to identify the overlapping characteristics of NFKD and KDSS, and to highlight the importance of early recognition of atypical KD regardless of age. We conclude that unusually high C-reactive protein, neutrophilia, and thrombocytopenia serve as supplemental laboratory indicators for early identification of KDSS in patients with NFKD.

A Case of Descending Necrotizing Mediastinitis in a Previously Healthy Child

Descending necrotizing mediastinitis (DNM) is a rare complication of oropharyngeal and cervical infection, especially in children. We report a case of DNM secondary to a cervical abscess in a previously healthy 1-year-old boy. The patient presented with redness and swelling of the neck and fever. He was treated with an antimicrobial agent for the diagnosis of cervical lymphadenitis. On the sixth day, a huge mediastinal abscess was found, and he was admitted to the intensive care unit. He was successfully treated with surgical drainage and appropriate antimicrobial therapy. The pus culture isolated multiple bacteria, including methicillin-resistant Staphylococcus aureus (MRSA). Although we did not use an antimicrobial agent covering MRSA, the symptoms and test results improved. Washing with drainage was effective. The patient required multidisciplinary treatment, and we collaborated with specialists in other departments. DNM is a severe disease in which team medical care is needed to provide appropriate treatment.

Effectiveness of Canakinumab Treatment in Colchicine Resistant Familial Mediterranean Fever Cases

Anti-interleukin 1 agents are used successfully in colchicine-resistant or intolerant Familial Mediterranean Fever (FMF) patients. Sixty-five patients with FMF who received canakinumab treatment for at least 6 months due to colchicine resistance or intolerance between 2016 and 2020 in our department were retrospectively analyzed. Canakinumab treatment was given subcutaneously every 4 weeks. After completing monthly canakinumab therapy over 12 months, in patients with complete remission, the dosing interval was extended to every 1.5 months for 6 months, then every 2 months for 6 months, and finally every 3 months for a year. In patients without disease activation, canakinumab treatment was discontinued at the end of 3 years and followed up with colchicine treatment. Patients who had a flare switched to the previous dosing interval. In patients with renal amyloidosis, monthly canakinumab treatment was continued without extending the dose intervals. The mean duration of canakinumab use in our patients was 31.4 ± 10.57 months (6–52 months). The mean age at onset of symptoms was 4.65 ± 3.84 (range, 1–18) years, and the mean age at diagnosis was 5.59 ± 3.9 (range, 4–19) years. Complete remission was achieved in 57 (87.6%) and partial remission in seven (10.7%) patients. One patient was unresponsive to treatment. Canakinumab treatment was discontinued in three patients with complete remission and one patient with drug resistance. Erythrocyte sedimentation rate (ESR) (51.85 ± 15.7 vs. 27.80 ± 13.73 mm/h) and C-reactive protein (CRP) [26 (3-73) vs. 5 (1–48) mg/L] values were compared before and after canakinumab treatment in attack-free periods, a significant decrease was found after canakinumab treatment (p < 0.001, p < 0.001, respectively). Bodyweight Z-scores (respectively −0.80 ± 0.86 vs. −0.49 ± 0.92) were compared, similarly, a statistically significant increase after canakinumab treatment (p < 0.001), but no significant increase in height Z scores (−1.00 ± 0.88 vs. −0.96 ± 0.94) (p = 0.445) was detected. Four patients had FMF-related renal amyloidosis. The decrease in proteinuria with canakinumab treatment was not statistically significant (p = 0.068). Cervical lymphadenitis developed in one and local reactions in two patients. No severe adverse effects requiring discontinuation of canakinumab treatment were observed. Our study showed that canakinumab treatment was highly effective, well-tolerated in pediatric FMF patients, and controlled extension of the canakinumab dose interval was safe.

Role of ultrasound in the diagnosis of cervical tuberculous lymphadenitis in children

Background To describe sonographic characteristics of cervical tuberculous lymphadenitis (CTBL) in children, clinical information, and sonograms of 348 lymph nodes (LNs) from 57 children with CTBL were retrospectively analyzed in this study. Methods We retrospectively reviewed the clinical data and sonograms of 348 LNs from 31 boys and 26 girls with CTBL, who were confirmed by pathology or laboratory examination, at the Hangzhou Red Cross Hospital between June 2014 and December 2020. The age of the children ranged from 1 to 14 years (average 7.1 ± 2.9 years). Results Night sweats, fatigue and loss of appetite were the most common clinical symptoms observed in children with CTBL. Unilateral LN involvements were common. Occasionally, CTBL was found in healthy children with no symptoms. On sonography, the hilus was absent or unclear in all LNs. The short-to-long axis (S/L) ratio was ≥ 0.5, and the edges were unclear in most LNs. Other accompanying findings included necrosis (47.4%), an echogenic thin layer (36.8%), surrounding soft-tissue edema (38.5%), multiple intra-nodal strong echo (28.2%), sinus (22.7%) and abscess formation (6.9%). The Doppler ultrasound showed that the majority of vascularity patterns of CTBL were capsular or peripheral (33.3%). Conclusions Ultrasound is a recommended examination method for children from different age groups with cervical lymphadenitis. The ultrasonic signs of hilus absence, S/L ratio ≥ 0.5, unclear edge, necrosis, echogenic thin layer, strong echoes and capsular or peripheral vascularity may aid in the diagnosis of cervical tuberculous lymphadenitis.

Case Report: A Case of Tuberculosis with Psoriasiform Lichen Scrofulosorum Exhibiting Koebner’s Phenomenon followed by Scrofuloderma

Lichen scrofulosorum is the most common tuberculid in the Indian population and the second most common form of cutaneous tuberculosis. We discuss an uncommon presentation of lichen scrofulosorum, with psoriasiform morphology and koebnerization at tuberculin test site, associated with cervical lymphadenitis in a 17-year-old girl. Although the cutaneous lesions resolved completely after 3 months of antitubercular treatment (ATT), she developed scrofuloderma in the left cervical region at 4 months. In the absence of rifampicin resistance, ATT was continued for another 6 months, with no further evidence of disease activity. This case represents an infrequent occurrence of lichen scrofulosorum followed by scrofuloderma, which necessitated a prolonged course of first-line ATT.

Unusual Presentation of Pustular Eruption in Kawasaki Disease Shock Syndrome: A Case Report

The present study case report described a 46-month-old girl with Kawasaki disease shock syndrome (KDSS) who presented with five days of fevers, unilateral cervical lymphadenitis, pustular eruption, maculopapular rashes, erythema of palms and soles, conjunctivitis, cracked lips, and shock. Laboratory results showed elevated ESR & CRP, leukocytosis, normocytic anemia, and transaminitis. Pustular eruption Gram and Wright stains demonstrated numerous neutrophils. Echocardiogram showed normal results. Fluid resuscitation, broad spectrum antibiotics, inotropic drug, IVIG, and high dose aspirin were given. Diagnosis of Kawasaki disease was supported by clinical and laboratory features at the acute phase, in conjunction with periungual peeling of fingers and toes and thrombocytosis at the subacute phase. The patient made a complete recovery.The present study case showed an unusual pustular eruption in KDSS. Clinicians should consider these presentations to the diagnosis of KDSS and timely prescribed IVIG, to prevent coronary artery aneurysm. Keywords: Pustular eruption; Kawasaki disease shock syndrome

Case Report: BCG-Triggered Hemophagocytic Lymphohistiocytosis in an Infant With X-Linked Recessive Mendelian Susceptibility to Mycobacterial Disease Due to a Variant of Chronic Granulomatous Disease

In the United Arab Emirates, BCG (Bacillus Calmette-Guérin) is administered to all newborns. We present here a young infant with an inborn error of immunity (IEI) who developed fatal adverse events to this live-attenuated vaccine. This male infant received BCG (Serum Institute of India Pvt., Ltd., India) on Day 11 of life. On Day 25, he developed fever, followed by cervical lymphadenitis and bilateral otitis media with fluid drainage. On Day 118, he was admitted with severe hemophagocytic lymphohistiocytosis (HLH), and passed away on Day 145. The diagnostic exome sequencing test identified a hemizygous nonsense variant, NM_000397.3(CYBB):c.676C>T, p.Arg226* (rs137854592). Pathogenic variants of CYBB [cytochrome b(-245), beta subunit; Mendelian Inheritance in Man [MIM] accession code, 300481] are known to cause “immunodeficiency 34, mycobacteriosis, X-linked” (IMD34, MIM#300645) and “chronic granulomatous disease, X-linked” (CGDX, MIM#306400). The natural history of his illness is consistent with “X-linked recessive Mendelian susceptibility to mycobacterial disease (MSMD).” This entity is responsible for his BCG disease and is a likely trigger of his HLH. This disastrous event underlines the importance of developing worldwide policies that target BCG disease prevention, especially in communities with high prevalence of IEI. Moreover, screening for genetic causes of MSMD in the community could pave the way, at least partially, for scale-up of tuberculosis (TB) prevention.

Bauhinia variegate (Kanchnara), An ornamental Plant with significant value in Ayurvedic and Folk Medicinal system

Medicinal plants are important part of traditional medication system. These plants are also the primary source of modern drugs. One such important medicinal plant is Bauhinia variegate (orchid tree) commonly called as Kanchanara in Hindi and Mountain Ebony in English. It belongs to Caesalpiniaceae family. It is one of the most common tree species found throughout the India. This tree is known for its beautiful scented and aesthetic white pinkish flowers. It is traditionally used in many folk cultures around the country for various kind of disorders. It is commonly used plant species in Indian cuisine. Its flower buds are commonly used as vegetables in many regions of India. It holds a significant place in Ayurveda for its curative and healing properties in many diseases especially cervical lymphadenitis, hemorrhage, rectal prolapse, menorrhagia, leukoderma etc. It is used in many Ayurvedic polyherbal formulations as a main ingredient like Kanchanara Guggulu, Ushirasava, Vidangarishta etc. It has diverse nature of phytochemical constituents present in it which are responsible for extraordinary therapeutic properties like anti-microbial, anti-tumor, anti-diabetic, hepatoprotective, immunomodulatory, haemagglutination, anti-oxidant, antigoitrogenic, nephroprotective. The aim of present review is to provide information related to phytochemistry, traditional uses in Ayurveda and folk medicinal system and therapeutic properties of Bauhinia variegate.