Kimura Disease of the Head and Neck Region.

Objective: We analyzed the clinical characteristics and treatment results in surgical patients with a final diagnosis of Kimura disease. Patients and Methods: A total of 8 patients with a final diagnosis of Kimura disease of the head and neck region after surgery from January 2007 to December 2022 were enrolled. Results: The most common symptom was a mass in the head and neck region (n = 7), followed by incidental detection (n = 1). Multiple head and neck lesions were observed except for 1 patient with Kimura disease in the buccal space. Kimura disease could not be diagnosed in 5 patients who underwent preoperative fine-needle aspiration cytology. All patients underwent surgical resection. No major complications after surgery occurred. Six patients received steroids after surgery, with 1 patient undergoing radiotherapy as a side effect of steroid treatment. During the follow-up period, 5 patients relapsed. Of the parameters assessed, only longer symptom duration was statistically significant with relapse of Kimura disease. Conclusion: Clinicians should suspect Kimura disease based on asymptomatic masses, peripheral blood eosinophilia, and imaging examinations and make a definitive diagnosis via surgery.

Characteristics of 18F-FDG PET/CT in patients with Kimura's disease from China.

BACKGROUND: 'Kimura's disease (KD) is a rare chronic inflammatory disorder of unknown etiology and is difficult to diagnose due to poor clinical presentation and imaging features. Few studies on characteristics of 18F-FDG PET/CT of KD have been reported. This study aimed to observe the reliable characteristics and usefulness of 18F-FDG PET/CT for the evaluation of consecutive patients with KD. METHODS: The clinical data and 18F-FDG PET/CT imaging findings of 8 patients with pathologically confirmed KD were reviewed retrospectively.18F-FDG PET/CT images were evaluated visually and semiquantitatively by measuring the maximum standardized uptake value (SUVmax). The correlations between clinical data and 18F-FDG PET/CT features were analyzed by simple linear regression. RESULTS: This study included 7 males and one female ranging in age from 17 to 79 years. The longest diameter of lesions ranged from 0.8 cm to 4.8 cm, and regional or generalized lymphadenopathy was found in all 8 patients with eosinophilia, while subcutaneous masses and salivary gland involvement concurrently were found in 4 patients. 18F-FDG PET/CT revealed that these involved lesions had high 18F-FDG uptake with SUVmax > 2.5 (2.6 to 6.3). Moreover, the margin of the lesions was well defined in 6 cases and ill defined in 2 cases, and homogeneous density and 18F-FDG uptake were both found in all these lesions. There was negative correlation between eosinophils and SUVmax (R2 = 0.538). CONCLUSIONS: Kimura's disease should be considered when 18F-FDG PET/CT is characterized by homogeneous lesions of regional or generalized lymphadenopathy, accompanied with subcutaneous masses and salivary gland involvement concurrently, especially in patients with eosinophilia.

Successful Treatment of Angiolymphoid Hyperplasia with Eosinophilia Associated with Scalp Demodicosis Using Cryotherapy and Topical Metronidazole.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign, vasoproliferative tumour. We report a 25-year-old female patient who reported in 2021 to a dermatology clinic in Rustaq, Oman, with multiple, grouped, erythematous dome-shaped papules and nodules of 6 months duration on the left temporo-occipital region. Biopsy findings were consistent with a diagnosis of ALHE with evidence of Demodex mite infestation in the sebaceous ducts. The patient demonstrated significant improvement following 7 weeks of treatment with multiple cryotherapy sessions and topical application of metronidazole gel. This case suggests that scalp demodicosis may represent a novel trigger for the development of ALHE.

Kimura disease: A rare case in Vietnamese woman.

Kimura disease (KD) is a rare benign chronic inflammatory condition that predominantly affects Asian males. It is characterized by subcutaneous tissue masses in the head and neck region, enlarged lymph nodes, increased blood eosinophilia, and elevated serum total IgE levels. In this report, we describe a rare case of KD in a young Vietnamese female. A 31-year-old Vietnamese woman presented to the hospital with 2 masses in the bilateral cheeks and 1 mass behind the left ear that persisted for 15 years, recurrent skin itching, elevated serum total IgE levels, and increased blood eosinophilia. No medical history of the individual or family was recorded. We performed an excision biopsy of the postauricular mass that revealed follicular hyperplasia with small vessel hyperplasia, diffuse infiltration of eosinophils in lymphoid follicles, and several eosinophilic microabscesses. After a comprehensive review, the final diagnosis for this patient was KD and atopic dermatitis comorbidity. In conclusion, KD is not limited to males, as this report demonstrated. The histopathological examination plays an important role in the diagnosis of KD. This case illustrated the characteristic description of KD and highlights the need for awareness of this rare disease in Asian women.

A case report of minimal change disease associated with Kimura disease complicated by optic neuritis.

Kimura disease (KD) is a rare chronic inflammatory disease that typically presents with soft subcutaneous granulomas in the head and neck regions characterized by elevated blood eosinophils and immunoglobulin E (IgE) level, whose aetiology remains poorly elucidated. Minimal change disease (MCD) has been reported as one of the renal manifestations that KD can present with, indicating that they may share a common pathology. Herein we describe a case of recurrent MCD associated with KD. During a follow-up period of 15 years, MCD recurred three times with increased disease activity of KD as reflected by flares of skin lesions and elevated peripheral eosinophils, and responded well to increased doses of prednisolone and cyclosporin. Notably, visual field defects in his right monocular vision appeared at the time of third recurrence of MCD, leading to the diagnosis of optic neuritis (ON). Optic nerve involvement associated with KD is extremely rare, and this case is noteworthy in that inflammation in the optic nerve was observed at the time of MCD recurrence with increased disease activity of KD, suggesting the existence of a common pathology between KD, MCD, and ON. In patients with KD, an imbalance of T helper (Th) cells with Th2 cells predominating over Th1 cells is observed, which results in hyperIgEemia and eosinophilia. This Th2-predominant immunological status in KD considered to predispose to MCD may also predispose to ON. MCD with a background of Th2-predominant immune state may require attention to the possibility of complication of ON.

Minimally Invasive Surgery for Duodenal Obstructions, 10 Years of Experience in a Single Center.

PURPOSE: Duodenal obstructions are one of the most common causes of upper intestinal obstruction during the neonatal period. Minimally invasive surgical treatment is considered highly complex. We report our experience with 43 patients treated using this method. METHODS: We conducted a retrospective study at our institution from January 2013 to May 2023, including patients classified as having upper intestinal obstructions. All patients received preoperative diagnoses based on clinical findings, radiography, and abdominal ultrasound. Laparoscopic surgery was performed on all patients. RESULTS: We included 43 patients diagnosed with duodenal obstruction (DO) in our study after reviewing the medical records at our hospital for cases meeting this diagnosis. The laparoscopic Kimura procedure was performed on 31 patients (72%), while duodenotomy and anastomosis following the Heineke-Mikulicz principle were performed on 9 patients (21%). In the remaining 3 patients (7%), a side-to-side duodeno-duodeno anastomosis was conducted. Annular pancreas was the most common cause of duodenal obstruction in our series, affecting 21 patients (49%). Type I duodenal atresia was observed in 17 patients (40%), while type III atresia was present in 3 patients (7%), and type II atresia in 2 patients (4%). One case required conversion to open surgery due to concomitant jejunoileal atresia. The only reported complication was partial anastomotic dehiscence, which occurred in two patients (4%). CONCLUSIONS: Minimally invasive surgery (MIS) for managing duodenal obstruction (DO) has proven to be both feasible and safe, yielding comparable outcomes to the traditional open approach; its effectiveness can be significantly enhanced through appropriate training. Furthermore, the growing availability of duodenal atresia simulators offers valuable opportunities to refine laparoscopic skills and apply them effectively. Better outcomes and fewer complications are expected with further experience and an increased number of cases. LEVEL OF EVIDENCE: IV, Case series with no comparison group.

A 23-year-old male patient with Kimura's disease without renal transplantation: a rare case report from Syria.

Introduction and importance: Kimura's disease (KD) is a chronic, nonmalignant inflammatory disorder that primarily affects subcutaneous tissue. It is typically characterized by painless nodules in the head and neck regions, accompanied by elevated eosinophil and serum IgE levels. The purpose of this case study is to elucidate this rare disease, particularly in the Asian region and Syria, and to explore diagnostic and therapeutic methodologies with the objective of mitigating the number of undiagnosed patients suffering from this disease. Case presentation: A 23-year-old male patient presented to the Ear, Nose, and Throat (ENT) Department of the hospital with symptoms that had been initiated 7 months prior. The primary symptoms were pain and swelling in the left preauricular area, followed by subsequent swelling in the right preauricular area the next day. The patient experienced severe, intermittent pain, generalized pruritus, and systemic manifestations, including fever, chills, fatigue, malaise, anorexia, and a weight loss of 20 kg over the course of seven months. A fine-needle aspiration of the left parotid gland revealed the presence of lymphocytes at various maturation stages, with no evidence of abnormal cells. A diagnosis of KD was subsequently confirmed. Clinical discussion: To our knowledge, this case represents the second documented instance of KD in Syria. Furthermore, our case is among the extremely rare instances of KD in a patient without a history of renal transplantation. Conclusion: Further research is essential to ascertain the actual prevalence of this condition and to identify the most effective management strategies.

The complete mitochondrial genome of Heptathela kimurai (Araneae: Heptathelidae).

Heptathela kimurai (Kishida, 1920) is a spider that belongs to the family Heptathelidae which is a basial lineage of spiders. The molecular information of ancestral species belonging to families like Heptathelidae is comparatively limited when compared to spider species from derived families. Here we present the complete mitochondrial genome sequence (mtDNA) of H. kimurai. The sequence was obtained using massively parallel sequencing technology. The circular genome was 14,224 bp in length, and the AT content was 69.53%. The H. kimurai mitochondrial genome contains 13 protein-coding genes (PCGs), 21 tRNA genes, and 2 rRNA genes. The majority of PCGs were found in the heavy strand.

Effectiveness of serological markers of gastric mucosal atrophy in the gastric precancer screening and in cancer prevention.

BACKGROUND: New markers are needed to improve the effectiveness of serological screening for atrophic gastritis. AIM: To develop a cost-effective method for serological screening of atrophic gastritis with a high level of sensitivity. METHODS: Of the 169 patients with atrophic gastritis, selected by the visual endoscopic Kimura-Takemoto method, 165 showed histological mucosal atrophy using the updated Kimura-Takemoto method. All 169 patients were examined for postprandial levels of gastrin-17 (G17) and pepsinogen-1 (PG1) using GastroPanel® (Biohit Plc, Helsinki, Finland). RESULTS: We used the histological standard of five biopsies of the gastric mucosa, in accordance with the Kimura-Takemoto classification system to assess the sensitivity of G17 in detecting gastric mucosal atrophy. We also compared the morpho-functional relationships between the detected histological degree of gastric mucosal atrophy and the serological levels of G17 and PG1, as the markers of atrophic gastritis. The sensitivity of postprandial G17 was 62.2% for serological levels of G17 (range: 0-4 pmol/L) and 100% for serological G17 (range: 0-10 pmol/L) for the detection of monofocal severe atrophic gastritis. No strong correlation was found between the levels of PG1 and degree of histological atrophy determined by the Kimura-Takemoto classification system to identify the severity of mucosal atrophy of the gastric corpus. In the presented clinical case of a 63-year-old man with multifocal atrophic gastritis, there is a pronounced positive long-term dynamics of the serological marker of atrophy - postprandial G17, after five months of rennet replacement therapy. CONCLUSION: Serological screening of multifocal atrophic gastritis by assessment of postprandial G17 is a cost-effective method with high sensitivity. Postprandial G17 is an earlier marker of regression of atrophic gastritis than a morphological examination of a gastric biopsy in accordance with the Sydney system. Therefore, postprandial G17 is recommended for dynamic monitoring of atrophic gastritis after treatment.

Overlap of membranous nephropathy and IgA nephropathy in a patient with Kimura's disease: a case report and literature review.

Introduction: Kimura's disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%-18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD. Case report: A 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080/mm³; eosinophils, 3,200/mm³ (31.7%); serum creatinine, 0.83 mg/dL; and eGFR: 140 mL/min per 1.73 m2. Urinalysis revealed the presence of hematuria and proteinuria and the following results: 24-h proteinuria, 12.9 g; serum albumin, 1.3 g/dL; and elevated IgE level, 750 kU/L. Serologies for hepatitis B, hepatitis C, HIV, and VDRL were all negative. Complement C3 and C4 levels were normal. No monoclonal protein was detected in blood and urine. Parasite infestation was discarded. A biopsy of the cervical lymph node revealed eosinophilic lymphoid hyperplasia, suggesting KD. A kidney biopsy revealed findings consistent with the overlapping of membranous nephropathy with IgA nephropathy. The patient was treated for KD with prednisone 1 mg/kg/d with progressive dose tapering and posterior association of methotrexate 15 mg/week. A renin-angiotensin system inhibitor was prescribed for nephrotic syndrome. The cervical mass regressed, and proteinuria achieved partial remission, with an increase in serum albumin level and normalization of eosinophils and IgE levels. Conclusion: Although uncommon, kidney involvement must be considered in patients with KD. Glomerular diseases are the most frequent form of kidney injury.

Ear lobule reduction using a sub-antitragal groove technique in patients with angiolymphoid hyperplasia with eosinophilia on the earlobe: a case report and literature review.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare condition characterized by nodular lesions predominantly in the head and neck region, often causing discomfort or pain. Treatment remains challenging because of its rarity and the lack of established guidelines. This report presents a case of ALHE affecting the earlobes that was successfully managed using ear lobule reduction surgery and subsequent intralesional steroid injections. A 31-year-old woman with a history of recurrent earlobe masses underwent a partial excision to avoid the loss of the earlobe. Histopathological examination confirmed Kimura disease, a variant of ALHE. Subsequent local methylprednisolone injections effectively controlled the remaining lesions, resulting in significant size reduction without notching. Various treatment modalities have been attempted for this condition; however, recurrence rates remain high. Surgical resection combined with intralesional corticosteroid injections is the preferred approach. In this case, a sub-antitragal groove technique for earlobe reduction was employed to preserve the lateral edge of the ear lobule, minimize the risk of deformity, and achieve a predictable outcome. The sub-antitragal groove technique offers an approach to reduce earlobe size without compromising aesthetics. Further research is required to elucidate the pathogenesis of ALHE and establish standardized treatment protocols for this rare condition.

Kimura's Disease in Temporal Bone: A Case Report.

To publish a rare case of Kimura's Disease in Temporal Bone. A 27 year-old male presenting with history of right ear pain and discharge for 2 months was thoroughly evaluated by clinical evaluation, hematological, radiological and histopathological study. Clinical examination revealed a bulge in posterior-inferior quadrant right side of tympanic membrane. HRCT temporal bone revealed a heterogeneous attenuating focal lesion is noted in the region of right middle ear cavity, mastoid antrum mastoid air cells in continuation with the superior aspect of right jugular foramen with erosions and bone destructions, involving the mastoid air cells and sinus plate. Patient was managed surgically with right side canal wall down mastoidectomy and Type 1 Tympanoplasty. Histopathological examination showed focal ulcerated stratified epithelium, dilated elongated congested blood vessels and hemorrhage. Diagnosis was made as Kimura's disease.

Stubborn Swellings: A Rare Case of Kimura's Disease Presenting as Parotid Swellings.

Kimura disease (KD) is a rare chronic inflammatory disorder characterized by the development of painless subcutaneous nodules, predominantly in the head and neck region. Diagnosis relies on a high index of clinical suspicion and clinicopathological correlation, with core biopsy serving as the gold standard for a definitive diagnosis. While the disease itself is benign, it can cause significant morbidity if left untreated. This case report describes a 48-year-old male who presented with bilateral infraauricular swellings, pruritus, and elevated serum IgE levels along with eosinophilia. Imaging and histopathological correlation confirmed the diagnosis of KD. Combination therapy of corticosteroids and cyclosporine resulted in significant clinical improvement, highlighting the efficacy of the approach while avoiding surgical resection. This case emphasizes the importance of radiologic-pathologic correlation along with the use of serology to effectively diagnose KD, even in atypical presentations.

Laparoscopic antegrade spleen-preserving distal pancreatectomy with conservation of the splenic vessels: a prospective multi-centre case series (with video).

Introduction: Laparoscopic spleen-preserving distal pancreatectomy (LSPDP) with conservation of the splenic artery and vein (Kimura' technique) is considered a technically challenging procedure that requires a high level of expertise in laparoscopic and pancreatic surgery. Methods: A prospective descriptive study on 18 patients with laparoscopic "antegrade" spleen-preserving distal pancreatectomy with Kimura' technique from 2018 to 2023. The perioperative indications, clinical data, intraoperative index, pathological postoperative specimens, postoperative complications, and follow-up results were retrospectively evaluated. Results: The mean age was 39.4±13.3. Only 2 male patients accounted for 11.1%. The average operating time is 171±23 min. The average blood loss is 65.7±43 ml. The average tumor size is 4.1 cm. The average hospitalization is 9.4 days. The rate of pancreatic fistula is 66.7%. There is no case of transferring open surgery or blood transfusion during surgery. The results of pathological after surgery there were eight cases of solid pseudopapillary tumors, four cases of mucinous cystadenoma, six cases of neuroendocrine tumors. Conclusion: Kimura's technique for laparoscopic spleen-preserving distal pancreatectomy is safe and feasible, which can be applied to benign tumors in the body and tail of the pancreas. However, this is a difficult technique in laparoscopic surgery that requires surgeons to have a lot of experience and equipment need to be adequate.

Surgical Treatment of Lithiasis of the Main Pancreatic Duct: A Challenging Case and a Literature Review.

Pancreaticolithiasis represents a rare phenomenon, being superimposed most of the time on a form of chronic pancreatitis of multifactorial etiology. Pancreaticolithiasis is a late complication of the phenomenon of chronic pancreatitis. The reverberant inflammatory process, followed by the fibrotic degeneration of the pancreatic parenchyma, and pancreatic fluid stasis at the ductal level are factors that contribute to the phenomenon of calcium precipitation. This article describes the case of a patient with a diagnosis of pancreaticolithiasis (Wirsung duct lithiasis), a phenomenon superimposed on chronic pancreatitis of ethanolic cause (Rosemont classification). It was decided to perform surgery via the classical approach with the perfection of corporeo-caudal pancreatectomy and preservation of the splenic vessels (Kimura procedure) with pancreatico-jejunal anastomosis on the Roux-en-Y loop. The aim of this study is to identify the best method of treatment for pancreaticolithiasis. To enhance the case and provide a basis for standardization, a literature review was carried out, which included a total of six articles. The results of this study highlight that, currently, the management of symptomatic pancreaticolithiasis encompasses medical therapy (enzyme replacement therapy), interventional therapy (ESWL (extracorporeal shock wave lithotripsy) ± ERCP (endoscopic retrograde cholangiopancreatography), ERCP + sphincterotomy + stent insertion, and POP (oral pancreatoscopy)), and surgical treatment. In conclusion, based on the analysis conducted in this study, the size of the calculi present determines which is the suitable therapeutic care. Unlike stones over 0.5 cm, when surgery is explicitly advised for therapeutic purposes in the absence of endoscopic techniques, stones under 0.5 cm should be treated using endoscopic procedures.

Kimura's Disease in Unusual Anatomical Locations: Clinical and Radiological Characteristics.

Purpose: To explore the clinical and imaging features of rare site Kimura's disease (KD). Methods: Retrospective analysis was conducted on the clinical manifestations, laboratory examinations, and imaging features of five patients with rare site KD. All imaging data, including the location, quantity, size, uniformity, boundary, and enhanced appearance of the lesion were evaluated by two independent radiologists. Results: Of the five patients, four were asymptomatic, and one experienced localized skin itching. Four cases involved subcutaneous nodules in the upper arm, while one was in the inguinal region. The main manifestations were single (three cases) or multiple (two cases) subcutaneous nodules/masses, with three patients accompanied by local lymph node enlargement. Four patients exhibited elevated eosinophil counts in their peripheral blood. Four patients had lesions with vascular flow voids; in three of these, the lesions also showed prominent enhancement. Notably, the lesion in a 5-year-old did not show vascular flow voids but displayed significant enhancement. Additionally, two patients showed edema around the lesions. Conclusion: The presence of solitary or multiple subcutaneous nodules/masses in the upper arm or inguinal area, accompanied by lymph node enlargement, elevated eosinophils in the peripheral blood, and the observation of internal vascular within the lesion, can aid in the diagnosis of KD occurring in uncommon anatomical locations.

Kimura Disease: A Detailed Analysis of Clinical and Radiological Manifestations in a Retrospective Case Series.

Background: Kimura disease (KD) is a rare chronic inflammatory disease that affects mainly young Asian men and is characterized by painless subcutaneous masses, lymphadenopathy, and elevated serum IgE levels. Despite its benign nature, KD poses a diagnostic and therapeutic challenge due to its rarity and clinical variability. Objective: This study aimed to provide a comprehensive analysis of the clinical and radiological features of KD in a retrospective case series, to assess treatment outcomes, and to discuss the implications for diagnosis and management. Methods: We retrospectively analyzed four histologically confirmed cases of KD admitted to Zhejiang Provincial People's Hospital from January 2018 to October 2023. Clinical and radiological data were retrospectively analyzed, and imaging findings were analyzed by two neuroradiologists to determine lesion characteristics and contrast enhancement patterns. Results: Our findings showed that the patients were predominantly male, with a mean age of 43 years and an age range of 13-71 years. All patients presented with painless subcutaneous masses and three of them had peripheral blood eosinophilia and elevated serum IgE levels. Radiographically, the lesions were predominantly ill-defined with heterogeneous enhancement, accompanied by subcutaneous fat atrophy. Complete surgical excision and oral corticosteroids were effective treatments, and no recurrence was noted during follow-up. Conclusion: KD should be considered in the differential diagnosis of painless subcutaneous masses in the head and neck region, especially in the presence of eosinophilia and elevated IgE levels. Our findings contribute to the understanding of KD's clinical and radiological spectrum and highlight the need for long-term follow-up due to the risk of recurrence.

Orbital Kimura disease: maintenance therapy using mycophenolate mofetil.

Kimura disease (KD) is a rare, chronic, inflammatory condition, predominantly found in male patients of Asian ethnicity. It typically presents between 50-60 years of age and usually with bilateral disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) remains the main differential diagnosis, although histological analysis is essential in differentiating from other similarly presenting pathologies. In this case, we present an atypical case of unilateral orbital KD in a middle-aged, Caucasian, male gentleman and no evidence of regional lymphadenopathy along with a literature review of orbital KD and the differential diagnoses, histological features and management modalities available, adding to the sparse literature on the topic. At present, no recognised diagnostic criteria for KD are available, with histopathological analysis through incisional or excisional biopsy being the primary diagnostic method. Complete surgical excision with or without corticosteroid management remains the most common treatment modality although management is shifting to steroid-sparing immunomodulatory therapy. To the best of our knowledge, this is the first case to describe maintenance therapy of KD using mycophenolate mofetil.

Perspective and challenges of mycorrhizal symbiosis in orchid medicinal plants.

The family Orchidaceae is of the most diverse taxon in the plant kingdom, and most of its members are highly valuable herbal medicines. Orchids have a unique mycorrhizal symbiotic relationship with specific fungi for carbohydrate and nutrient supplies in their whole lifecycle. The large-scale cultivation of the medicinal plant Gastodia elata is a successful example of using mycorrhizal symbiotic technology. In this review, we adopted G. elata and Dendrobium officinale as examples to describe the characteristics of orchid mycorrhiza and mycorrhizal benefits for host plants' growth and health (e.g. biotic and abiotic stress and secondary metabolite accumulation). The challenges in applying mycorrhizal technology to the cultivation of orchid medicinal plants in the future were also discussed. This review aims to serve as a theoretical guide for the cultivation of mycorrhizal technology in medicinal orchid plants.