Naoki Kanai

Existence of c-kit Receptor-Positive, Tryptase-Negative, IgE-Negative Cells in Human Allergic Nasal Mucosa: A Candidate for Mast Cell Progenitor

The accumulation of mast cells in the allergic nasal epithelium is well known, yet the mechanism remains unclear. We studied whether there is a candidate for mast cell progenitors in the allergic nasal mucosa tissue removed at the time of surgery. We first confirmed that most mast cells in nasal mucosae of 10 nasal allergic patients had c-kit receptor by immunohistochemistry using the mirror sectioning technique. We then investigated whether c-kit receptor+, tryptase-, IgE- cells existed in nasal mucosae of 15 nasal allergic patients and 15 nonallergic ones using sequential triple immunohistochemistry. We observed the area in which 1,000 to 1,100 tryptase-positive cells (mast cells) existed in both the subepithelial layer and the deep layer of each nasal lamina propria. The epithelial layer above this area was also examined. Some c-kit receptor+, tryptase- cells existed in the nasal mucosae of 11 patients with nasal allergy and of 5 patients with nonallergic rhinitis. From one to four of these cells in the nasal epithelium and subepithelial layer of the 4 allergic patients were IgE-negative. In contrast, no IgE-negative cells existed in the deep layer of allergic nasal mucosae or in any nonallergic nasal mucosae. Our results suggest that mast cell progenitors, hematopoietic progenitor cells or multipotential blood cells exist in the allergic nasal mucosa, and may contribute to the increase of mast cells in the epithelium and subepithelial layer of allergic nasal mucosa.

Six patients with Kawasaki disease showing retropharyngeal low-density areas on computed tomography

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology characterized by fever, skin rash, cervical lymphadenopathy, conjunctivitis, and changes in the mouth and extremities, which predominantly affects young children. The diagnosis of KD is made according to the Japanese diagnostic guideline on the clinical findings. The acute phase symptoms are self-limiting, whereas formation of coronary artery aneurysms could cause acute myocardial infarction in the future. Although several authors have reported cases of KD mimicking retropharyngeal abscess (RPA) and suggested difficulty in differential diagnosis from infectious RPA, each described a single or two cases and often lacked laboratory findings. We present here six Japanese patients with KD showing retropharyngeal low-density area (LDA) on computed tomography (CT) and their laboratory findings.

Apnea in a 2-month-old girl with retropharyngeal abscess.

Sir, Retropharyngeal abscess (RPA) is an acute infectious disease in the space anterior to the prevertebral layer of the deep cervical fascia, and predominantly affects children younger than 5year olds (1). RPA is usually presented with fever, neck mass, neck pain, sore throat, stridor and limitation of neck movement (1). We report an infant with RPA who showed apnea as an early symptom without apparent obstructive lesions. A two-month-old Japanese girl was admitted to our hospital because of cough, nasal obstruction and poor oral intake lasting seven days. She was born to non-consanguineous healthy parents after eventless gestation. There was no history of trauma. On admission, stridor was audible, although there were no abnormal findings in her head, neck, abdomen or extremities on physical examination. Fever transiently developed on the second hospital day. After admission, she was unexpectedly found to be cyanotic without breathing effort. Thereafter, oxygen saturation was around 90% in room air but declined to 80– 85%, which was associated with cyanosis and thoracic motion during sleep or drowsy state, suggesting obstructive or mixed apnea. She recovered from apnea following suctioning of massive saliva from her oropharynx. Laboratory examination demonstrated; white blood cell count 16.81 × 109/L with normal differentiation, haemoglobin 10.2 g/dL, platelet count 895 × 109/L, C-reactive protein 23.6 mg/L. Her liver and renal functions, electrolytes and urinalysis were all within normal ranges. There were no abnormalities on chest and neck roentgenography or brain computed tomography (CT) scan. She was treated by fluid replacement, oxygen supplementation and antibiotics (ampicillin/sulbactam 150 mg/kg/day). No pathogenic bacteria were cultured from her blood or throat. There was no bulging of the pharyngeal wall or swelling of epiglottis on laryngoscopy. The diagnosis of RPA was finally made by enhanced CT scan of her neck (Fig. 1). Both apnea and stridor disappeared by the 10th hospital day without surgical procedures. On the 12th hospital day, RPA was not detected by enhanced CT scan. She was discharged from our hospital after 15 days of hospitalization. Although she suffered from pneumonia 8 months later, there was no clinical or laboratory finding suggesting immunodeficiency or congenital anomaly of her neck or upper airway. In contrast to the previous report on obstructive apnea in chronic RPA (2), there was no apparent obstruction of her upper airway on laryngoscopy or X-ray. Of note was that she recovered from apnea following aspiration of massive saliva from her pharynx. Ingestion of hypochloremic solution such as saliva to the oropharynx stimulates laryngeal chemoreceptors and induces laryngeal constriction and prolonged apnea, namely, airway protective reflex (3). Central apnea often follows obstructive apnea in young infants (3). Given that children with RPA frequently show drooling possibly related to dysphagia (1), accumulation of saliva could have induced the reflex and prolonged apnea particularly in the supine position. Suctioning could clear the salivary fluid from the oropharyngeal and arouse the patient. In conclusion, RPA is a rare but a potentially life-threatening cause of apnea particularly in young infants, even if the size of abscess is not large enough for complete airway obstruction.

Catheter Drainage of Neck Abscess in Children

Image-guided percutaneous drainage has been shown to be a safe and effective alternative to surgery in the management of neck abscess. Two patients, 7-year and 4-month-old girls, were treated with X-ray fluoroscopy and ultrasonography-guided percutaneous 7 Fr trocar-type pigtail catheter drainage and antimicrobial therapy. This method was performed without complications and open surgical drainage was avoided in both cases. One patient had a persistent abscess, but was treated successfully by repeated catheter drainages.Percutaneous drainage may be a useful alternative to surgical drainage in a selected group of patients with localized, smaller abscess without imminent airway obstruction.

A Recurrent Case of Spontaneous Mediastinal Emphysema

Spontaneous mediastinal emphysema is a rare condition that primarily occurs in young, thin, healthy adults. It is rare for cases of spontaneous mediastinal emphysema to recur. Recently, we experienced a recurrent case of spontaneous mediastinal emphysema. A 17-year-old boy came to our hospital complaining of chest pain and dyspnea. His chest computed tomography (CT) showed mediastinal emphysema with cervical emphysema. He was hospitalized and treated with bed rest and antibiotics to prevent infection. Mediastinal air resolved completely after 6 days of hospitalization. Seven weeks following the first episode of spontaneous mediastinal emphysema, the patient was readmitted with a second episode of cervical pain. He was hospitalized again and treated with bed rest and antibiotics to prevent infection. Mediastinal air resolved completely after 4 days of hospitalization. Recurrent spontaneous mediastinal emphysema has been documented only in our patient and 6 others in Japan.

A Case of Castleman's Disease Followed by Peripheral T-cell Lymphoma.

A 57-year-old male was admitted with severe fatigue and systemic lymphadenopathy. Surgical biopsy specimens revealed Castlemans disease of hyaline vascular type. After admission, Guillain-Bane syndrome developed and the ptient was treated with predonisolone and plasma pheresis was given. The patients symptoms and lymphadenopathy responded and disappeared rapidly. Five months later, systemic lymphadenopathy recurred. Surgical biopsy was performed and the histological features were consistent with a diagnosis of peripheral T-cell lymphoma of diffuse mixed type, probably resulting from the progression of the Castlemans disease.

A Neonatal Case of Neuroblastoma in the Neck.

A neonatal boy presented with a painless, smooth, round mass in the left side of his neck at birth. CT, MRI, and echo examinations revealed a solid tumor with calcification occupying the left upper neck. The levels of serum neuron specific enolase (NSE), urine homovanillic acid (HVA), and vanillylmandelic acid (VMA) were elevated, indicating a diagnosis of neuroblastoma. Surgical resection was performed 23 days later because of airway obstruction due to tumor progression. The resected tumor was 43×35×32mm and was diagnosed as a rosette-fibrillary type of neuroblastoma by histopathological analysis. The tumor was of a favorable histological grade and was classified as stage 2A according to the International Neuroblastoma Staging System. After surgery, the patient received systemic chemotherapy of a regimen including cyclophosphamide and vincristine. He is alive and disease free 4 years after diagnosis.

Sjoegren's Syndrome with Pseudolymphoma. A Case Report.

In a 59-year-old female with Sjogrens syndrome, xerostomia and xerophthalmia had started in 1982, and lymphadenopathy of the cervical nodes and sore throat appeared in 1991. Biopsies were taken from the swelling in the nasopharynx and the cervical node. The pathological diagnosis was “pseudolymphoma”. We started chemotherapy with a steroid. The lesions shrank remarkably during one week, but five months later, the swellings enlarged and did not respond to steroid therapy. Biopsy of the nasopharynx was performed again. The pathological diagnosis was “malignant lymphoma”. We started chemotherapy with “CHOP”. The lymphoma disappeared and has not recurred.

Glomus tympanicum tumor; A case report. Benefits of preoprative diagnostic imaging.

The patient was 67-year-old female who complained of pulsatile tinnitus in the right ear. Otoscopy revealed a reddish mass that extended to the annulus.CT, MRI and angiography localized it in the middle ear cavity and distinguished it from the jugular bulb. The tumor was completely resected by posterior hypotympanotomy after embolization of the superior tympanic artery. Absence of tumor recurrence was con-firmed 8 months after surgery. We discuss the benefits of preoperative diagnostic imaging methods.

Adenocarcinoma of sublingual salivary gland; A case report.

A 40-year-old woman complained of a painless mass in the left side of the floor of the mouth. A 2×2cm round, smooth, nontender mass was palpable on the left side of the floor of the mouth; it was fixed to the symphysis of the mandible. No cervical lymph nodes were palpable. T2-weighted MRI scan of the tumor showed smooth margins and a heterogeneous signal. A contrast-enhanced CT scan showed a slightly enhanced mass lesion. Radionuclide scanning with 67Ga showed a hot tumor. Radiography revealed a malignant tumor originating in a minor salivary gland or a sublingual gland. Microscopic examination of the tumor showed adenocarcinoma.Radical neck dissection was combined with resection of the left side of the floor of the mouth and of the internal margin of the mandible. The surgical defect was reconstructed with a rectus abdominis musculocutaneous flap.Postoperative evaluation of mastication and articulation was good. The patient had no evidence of recurrence one year after surgery.