Periodic acid-Schiff Positive Material Research Articles

SECONDARY PULMONARY ALVEOLAR PROTEINOSIS FOLLOWING ALLOGENIC HEMATOPOIETIC CELL TRANSPLANT FOR ACUTE MYELOID LEUKEMIA

SECONDARY PULMONARY ALVEOLAR PROTEINOSIS FOLLOWING ALLOGENIC HEMATOPOIETIC CELL TRANSPLANT FOR ACUTE MYELOID LEUKEMIA

CADASIL and Cavernomas: A Common Mechanism.

CADASIL and Cavernomas: A Common Mechanism.

Effects of Combined Nicotine and Caffeine on the Rat Skeletal Muscles: A Histological and Immunohistochemical Study.

Background:Nicotine and caffeine are pharmacologically active substances that consumed widely in the whole world. Most of the nicotine users also consume caffeine. Smokers tend to drink more coffee than nonsmokers. It is important to characterize these substances with regard to their effects on the histological and immunohistological structure.Objectives:The objective of the study is to assess the impact of combined administration of nicotine and caffeine on histological structure of the skeletal muscle tissue in the adult male Wistar rats.Materials and Methods:Twenty adult male Wistar rats with an average weight of 200–250 g were randomly divided into four equal groups: control, nicotine, caffeine, and combined (nicotine + caffeine). The diaphragm muscle was processed and stained with hematoxylin and eosin (H and E) stain, histochemically by periodic acid–Schiff (PAS) and immunohistochemically by anti-CD68 antibodies.Results:After injected nicotine, thick basement membrane with apparent increase in the positive CD68 macrophages inbetween the diaphragm muscle fibers. After injected caffeine, there was an apparent accumulation of mononuclear cells around some fibers with decrease in the PAS positive fibers. Combined injected (nicotine + caffeine) group, some fibers exhibited deep acidophilic cytoplasm with flat peripheral nuclei and apparent increase of the CD68 positive cells. There was an increase in PAS positive material around fibers appearing as a thick basement membrane.Conclusions:The present study proved that caffeine and nicotine either taken alone or in combination have many negative impacts on the active type of skeletal muscles like diaphragm leading to degenerative changes that may affect their function.

Pulmonary Alveolar Proteinosis After Exposure to Cleaning Fluids

SESSION TITLE: Critical Care 1 SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Sunday, October 29, 2017 at 10:45 AM - 12:00 PM INTRODUCTION: Pulmonary Alveolar Proteinosis (PAP) is a rare disorder characterized by the accumulation of amorphous, periodic acid-Schiff positive material composed of surfactant phospholipids and apoproteins. This can be congenital, primary or secondary. We present a case of secondary PAP in a housecleaner after exposure to cleaning fluids. CASE PRESENTATION: 64 year old woman, with 30 pack smoking years, presented with worsening shortness of breath and dry cough over 6 months. She was initially diagnosed with pneumonia and had partial response to treatment with antibiotics and steroids. Subsequent high resolution computed tomography scan (CT) of the chest revealed bilateral diffuse ground glass infiltrates with intralobular septal thickening with crazy pavement pattern. Further history brought to light her house cleaning occupation using commercial cleaning products. Bronchoscopy revealed the presence of opaque lavage and transbronchial biopsies confirmed alveolated parenchyma with eosinophilic granular material, periodic acid Schiff stain positive foamy exudates. Blood testing for antibodies against Granulocyte Macrophage-Colony Stimulating Factor was negative. The patient was interested in whole lung lavage, however, given her clinical picture and high suspicion for secondary PAP, we recommended a conservative approach with a focus on pulmonary rehabilitation, quitting smoking and avoidance of exposure to cleaning chemicals. At 3 months follow up, her symptoms dramatically improved and a repeat CT chest showed marked resolution of previous findings. DISCUSSION: Secondary PAP develops in association with conditions involving functional impairment or reduced numbers of alveolar macrophages. Such conditions include some hematologic cancers, pharmacologic immunosuppression, inhalation of inorganic dust or toxic fumes1 including silica, paint, wood powder, titanium and welding smoke. Cleaning fluids are commonplace in certain occupations and in domestic life, hence unanswered questions regarding variation in susceptibility after exposure, patient phenotype, level of exposure and constituent chemicals persist and warrant further investigation. Temporal improvement after avoidance of the exposure lends credence to the etiological role played by these agents. CONCLUSIONS: 1Exposure history is paramount when investigating secondary PAP, even outside of the realm of traditional occupational exposures. Reference #1: Trapenll B. et.al, Pulmonary Alveolar Proteinosis N Engl J Med 2003; 349:2527-2539 DISCLOSURE: The following authors have nothing to disclose: Karthika Linga, Minkyung Kwon, Brendon Colaco, Vichaya Arunthari, Neal Patel No Product/Research Disclosure Information

Fibrotic gene expression coexists with alveolar proteinosis in early indium lung

Occupational inhalation of indium compounds can cause the so-called “indium lung disease”. Most affected individuals show pulmonary alveolar proteinosis (PAP) and fibrotic interstitial lung disease. In animal experiments, inhalation of indium tin oxide or indium oxide has been shown to cause lung damage. However, the mechanisms by which indium compounds lead to indium lung disease remain unknown. In this study, we constructed a mouse model of indium lung disease and analyzed gene expression in response to indium exposure. Indium oxide (In2O3, 10 mg/kg, primary particle size <100 nm) was administered intratracheally to C57BL/6 mice (male, 8 weeks of age) twice a week for 8 weeks. Four weeks after the final instillation, histopathological analysis exhibited periodic acid-Schiff positive material in the alveoli, characteristic of PAP. Comprehensive gene expression analysis by RNA-Seq, however, revealed expression of fibrosis-related genes, such as surfactant associated protein D, surfactant associated protein A1, mucin 1, and collagen type I and III, was significantly increased, indicating that fibrotic gene expression progresses in early phase of indium lung. These data supported the latest hypothesis that PAP occurs as an acute phase response and is replaced by fibrosis after long-term latency.

ABL tyrosine kinase inhibitor-induced pulmonary alveolar proteinosis in chronic myeloid leukemia.

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of eosinophilic periodic acid Schiff-positive material in the intra-alveolar and bronchiolar spaces. Tyrosine kinase inhibitors, including imatinib, nilotinib, and dasatinib, have shown excellent efficacy in the treatment of chronic myeloid leukemia (CML). We report a case of acquired PAP in a patient with CML receiving tyrosine kinase inhibitors. A 67-year-old man with CML presented with progressive back pain 5 months after starting imatinib treatment. Acquired PAP was diagnosed based on physical, radiographic, and histopathological findings. The presence of granulocyte-macrophage colony-stimulating autoantibodies suggested that autoimmune mechanisms were involved in the pathogenesis. Interestingly, PAP developed in association with imatinib and dasatinib administration, but not with nilotinib treatment. The patient died of refractory leukemia in lymphoid blast crisis with a newly emerged T315I mutation. Although the incidence is very rare, imatinib and dasatinib associated with PAP development has been reported. Meanwhile, PAP in nilotinib-treated patients has not been reported. Our observation in one patient receiving multiple TKIs suggests that nilotinib may be safer than imatinib or dasatinib in avoiding the development or exacerbation of PAP.

A Rare Cause of Hoarseness of Voice: Lipoid Proteinosis of the Larynx

ABSTRACT Lipoid proteinosis (LP) is a rare genetic disease with autosomal recessive inheritance. It most often involves deposition of periodic acid Schiff positive hyaline material in skin, oral mucosa, larynx and other tissues. But it also involves the central nervous system, lungs, lymph nodes and striated muscles. Hoarseness, small papules on the eyelid border (moniliform blepharosis), enlarged tongue, waxy skin, and diffuse verrucous skin colored or yellowish papules and plaques on traumatized areas and oral mucosa are the most common features leading to the clinical diagnosis of LP. We present the case report of a 12-year-old boy with significant hoarseness, inability to protrude the tongue, beaded papules along the eyelid margins, and scarring of the skin. Of his two sisters, one had the same symptoms but with less clinical severity and the other had no features of LP. How to cite this article Swain SK, Panda M, Patro N, Sahu MC. A Rare Cause of Hoarseness of Voice: Lipoid Proteinosis of the Larynx. Int J Phonosurg Laryngol 2014;4(1):23-26.

Urbach-Wiethe Disease: A Rare Cause of Hoarseness of Voice

ABSTRACTUrbach-Wiethe disease is a rare, autosomal recessive disorder, characterized by infiltration of periodic acid-Schiff positive hyaline material into the skin, oral cavity, larynx and internal organs. The clinical manifestations include hoarseness of voice, beaded papules along the eyelid margins, skin scarring and an inability to protrude the enlarged and thickened tongue. Laryngeal involvement is typical and causes hoarseness of voice. In this paper, we present a case of a middle-aged female with clinical features suggestive of Urbach-Wiethe disease. This entity is of interest to the otolaryngologist as it is a rare cause of hoarseness of voice.How to cite this articleKoganti D. Urbach-Wiethe Disease: A Rare Cause of Hoarseness of Voice. Int J Phonosurg Laryngol 2013;3(2):61-64.

Call for an international survey on therapeutic lavage for pulmonary alveolar proteinosis

To the Editors: Pulmonary alveolar proteinosis (PAP) is a syndrome characterised by accumulation of proteinaceous, periodic acid-Schiff positive material within airspaces due to disruption of surfactant homeostasis [1]. In 95% of cases, PAP is associated with increased serum levels of auto-antibodies neutralising granulocyte-macrophage colony-stimulating factor and, therefore, this form is referred to as “auto-immune PAP” [2]. Irrespective of the underlying cause, the current standard of care for PAP is represented by whole lung lavage (WLL) and, to a lesser extent, bronchoscopic lobar/segmental lavage [3]. WLL has played such a critical role in …

Oral metastasis of alveolar soft-part sarcoma: a case report and review of literature

Alveolar soft-part sarcoma (ASPS) is a rare malignant neoplasm with uncertain histogenesis and with a distinctive morphology. It has been described in the oral cavity, but this is the first report of ASPS metastasizing to the maxillary tuber region. A 27-year-old male patient, who was under chemotherapy treatment for ASPS of the thigh, presented in our dental clinic with a painless and pedunculated nodule on the right tuber maxillae. The nodule was erythematous with smooth and lobular surface, measuring 3 cm in maximum diameter. An incisional biopsy was performed and the diagnosis of metastatic ASPS was made. Histologically, the tumor was characterized by a proliferation of polyhedral cells in pseudoalveolar pattern. Tumor cells were large, showing granular cytoplasm, periodic acid-Schiff positive diastase-resistant intracytoplasmic material, and vesicular nuclei with prominent nucleoli. Unfortunately, the patient died 2 months after the diagnosis of the oral metastasis. Metastases of ASPS to the mouth are very rare and indicate a poor prognosis.

Acute pulmonary alveolar proteinosis due to exposure to cotton dust

Secondary pulmonary alveolar proteinosis (PAP) is rare but may occur in association with malignancy, certain infections, and exposure to inorganic or organic dust and some toxic fumes. This case report describes the second recorded case of PAP due to exposure to cotton dust. A 24-year-old man developed PAP after working as a spinner for eight years without respiratory protection. He was admitted as an emergency patient with very severe dyspnea for four months and cough for several years. Chest X-ray showed bilateral diffuse alveolar consolidation. He died 16 days later, and a diagnosis of acute pulmonary alveolar proteinosis was made at autopsy. The histopathology demonstrated alveoli and respiratory bronchioles filled with characteristic periodic acid Schiff-positive material, which also revealed birefringent bodies of cotton dust under polarized light. Secondary PAP can be fatal and present with acute respiratory failure. The occupational history and characteristic pathology can alert clinicians to the diagnosis.

A case of Noonan syndrome and Whipple’s disease in the same patient

We report the first known case of both Noonan syndrome and Whipple's disease occurring in the same patient. A 36-year-old female with history of Noonan syndrome developed fatigue, anorexia, arthritis of the knees and hands with a diffuse hyperpigmented rash, night sweats, and an unintentional fifteen pound weight loss over 4 mo. Small bowel enteroscopy demonstrated mild edematous yellowish mucosa without friability. Random small bowel biopsies revealed extensive periodic acid-Schiff positive material within the foamy macrophages. She was treated with a 12 mo course of trimethoprim-sulfamethoxazole DS with clinical improvement to baseline status.

Four Different Presentations of a Rare Disease – Whippleʼs Disease

Whipple's disease (WD) is a rare chronic infectious multisystem disease caused by bacterium Tropheryma whipplei. Only about 1000 cases have been reported to date. We describe our experience with 4 cases diagnosed with WD between 2002 and 2007: first 3 cases from Brooklyn, New York and case #4 from Western Massachusetts. Case #1: A 72 year-old (yo) Caucasian male from Spain, living in Brooklyn since 1975, presented with 30 lbs weight loss, pulmonary nodules and retroperitoneal lymphadenopathy. Lymph node biopsy revealed foamy macrophages (FM) containing periodic acid-Schiff (PAS) positive (+) material and negative Ziehl-Neelsen stain (ZN). Retrospectively, he had arthralgia for 10 years and developed intermittent diarrhea, epigastric pain and progressive cognitive decline for the last 6 months. Laboratory data were significant for anemia and evidence of malabsorbtion. On upper endoscopy (EGD) duodenal mucosa had small lymphangiectasia and biopsy was consistent with WD with PAS + FM in lamina propria (LP). Case # 2: A 61 yo Caucasian male from Russia, living in Brooklyn for the last 7 years, with long history of arthralgia was evaluated for iron deficiency anemia. Colonoscopy was unrevealing and EGD showed normal appearing duodenal mucosa with distended villi by FM PAS + and negative ZN on biopsies, consistent with WD. Case #3: A 25 yo African American male presented with diarrhea for 1 year. Colonoscopy with ileoscopy revealed erythema of the terminal ileum mucosa with PAS + FM on biopsies. EGD confirmed the diagnosis of WD with similar findings on duodenal biopsy. Case #4: A 41 yo Caucasian male of Irish descend, with history of seronegative polyarthritis on treatment with high doses of aspirin and intermittent steroids presented with an episode of diarrhea for 4 weeks with negative work-up including colonoscopy. The episode resolved, but subsequently the patient had recurrence of diarrhea and weight loss. On EGD duodenal mucosa appeared flat, pale and biopsies showed PAS + FM in LP suggestive of WD. The diagnosis was confirmed by a PCR assay. All patients were treated with Ceftriaxone for 2 weeks followed by Trimethoprim -sulfamethoxazole for 1 year. Diarrhea and anemia resolved and arthralgia was significant improved in all patients. Patient in case #1 had weight gain and improvement in adenopathy and cognitive function. Discussion: WD has a variety of clinical manifestations and should be considered in patients who present with migratory arthralgias of the large joints, weight loss, diarrhea, and abdominal pain.

Lung cancer may develop subsequently or coincidently with pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by an accumulation of periodic acid-Schiff (PAS) positive lipoproteinaceous material in the alveolar space. It is usually idiopathic, and secondary to hematologic malignancy or some atypical infection. To date, there are only five published case reports of PAP occurring in association with solid organ cancer. We herein report two cases of PAP associated with lung cancer: one, a case of idiopathic PAP with subsequent development of lung cancer, and the other, a case of coexisting lung cancer and PAP. In conclusion, PAP can occur prior to or coincidently with lung cancer.

The Crazy-paving Sign1

The crazy-paving sign was initially recognized in patients who had pulmonary alveolar proteinosis (1). Pulmonary alveolar proteinosis manifests as filling of the alveoli by a proteinaceous material that is positive at periodic acid–Schiff staining and is rich in lipids, in association with an inflammatory response in the adjacent interstitium (3). It is believed that pulmonary alveolar proteinosis results from an abnormality of surfactant production, metabolism, or clearance by type II alveolar cells and macrophages (4). Most cases are idiopathic. Some result from exposure to silica (silicoproteinosis) or occur in association with hematologic disorders, such as lymphoma or leukemia and, occasionally, with human immunodeficiency virus infection (4). The linear network or reticular pattern in the crazy-paving sign is believed to result from a thickening of the interlobular septa, while areas of groundglass opacity result from partial alveolar filling processes (1,5). Interlobular septal thickening can be seen from interstitial fibrosis, but the presence of areas of reticular opacity does not necessarily represent interstitial abnormalities (2). Kang et al (6), using the findings of a single case, suggested that the network in the crazy-paving sign in alveolar proteinosis may result from an accumulation of periodic acid–Schiff-positive material in the airspaces adjacent to the interlobular septa rather than to thickening of the septa. Therefore, when there is a slight increase in the severity of the alveolar filling process at the borders of unit structures such as acini or secondary pulmonary lobules, the networks in the crazy-paving sign can also be caused purely by airspace disease (2). The crazy-paving sign may also be seen in patients with a variety of other diseases (2,7). The differential diagnosis of crazy-paving sign includes Pneumocystis carinii pneumonia, mucinous bronchioloalveolar carcinoma (8), sarcoidosis, lipoid pneumonia (9), adult respiratory distress syndrome (7), and pulmonary hemorrhage syndromes (10). In a prospective study of patients showing the crazy-paving sign, a variety of causes were identified (7). These included P carinii pneumonia, alveolar proteinosis, usual interstitial pneumonia, pulmonary hemorrhage, acute radiation pneumonitis, adult respiratory distress syndrome, and drug-induced pneumonitis. Of these, P carinii pneumonia was most common. Johkoh et al (2) reported 46 patients showing the crazy-paving sign on thin-section CT images, the most common causes included adult respiratory distress syndrome (n 8), bacterial pneumonia (n 7), acute interstitial pneumonia (n 5), and, despite its rarPublished online 10.1148/radiol.2433041835

Histochemical Studies on Structural Changes of Large Conducting Arteries in Male Diabetic Rats

Arab, M.R., Komeili, G.R., Touhidi, A., Sargolzaie, A.F. and Shahraki, M.R. 2006. Histochemical studies on structural changes of large conducting arteries in male diabetic rats. J. Appl. Anim. Res., 30: 145–148. To examine the structural and histochemical changes of aorta and common carotid arteries in male diabetic rats, 63 male Sprague Dawley rats were randomly divided into experimental (n=36) and control (n=27) groups. Diabetes was induced by intraperitoneal injection of 60 mg/kg of streptozotocin in experimental group. There was a significant difference only for periodic acid schiffpositive material in subintimal connective tissue between control and 4 months diabetic groups (P<0.007). Peanut agglutinin staining showed the presence of Galactose IN-Acetyl galactosamine in glycoconjugates of sub-endothelial and adventitial components of studied arteries. It seems that in diabetes, the rate of glycosylation of cell surface and extracellular matrix glycoconjugates changed slowly.

Immuotactoid Glomerulopathy (Fibrillary Glomerulonephritis)

Immunotactoid glomerulopathy first was described in 1977, when Rosenmann and Eliakim (1) reported an unusual glomerular lesion in a 45-yr-old woman who presented with the nephrotic syndrome and renal insufficiency. The electron microscopy was remarkable for organized electron-dense deposits in the form of randomly arranged fibrils that measured 10 nM in diameter. The deposits were associated with mesangial expansion and immune deposits of IgG, IgM, and C3 in a mesangial pattern. Congo red stain of the deposits was negative, and there was no clinical or serologic evidence of a systemic disease. They interpreted the deposits to be “amyloid-like” and speculated that they might represent a “preamyloid” state. In 1980, Schwartz and Lewis (2) reported a case of a 49-yr-old man who presented with the nephrotic syndrome and no evidence of systemic disease and had a similar renal lesion: Immune aggregates were associated with highly organized electron-dense deposits that were composed of microtubules that were arranged in parallel bundles. During 7 yr of follow-up, the patient progressed to renal failure but never demonstrated any clinical or serologic evidence of a systemic disease. To distinguish this lesion from other disorders that have glomerular immune deposits associated with highly organized microtubular or fibrillary structures, such as amyloidosis, cryoglobulinemia, paraproteinemias, and systemic lupus erythematosus (SLE), the term immunotactoid glomerulopathy (ITG) was coined, reflecting the composition (immuno-) and polymeric morphology (tactoid) of the glomerular deposits. In the past 25 yr, >200 cases of ITG have been reported (3–5). Various synonyms have been used to refer to the lesion that is described in these reports, including fibrillary glomerulonephritis (FGN), nonamyloidotic fibrillary glomerulopathy, amyloid-like glomerulopathy, and amyloid stain–negative microfibrillary glomerulopathy, but we believe that they all represent the same or a similar disease process (6–8). The unifying feature in all of the cases …

Adenoid Cystic Carcinoma Arising from the Lower Eyelid

Primary cutaneous adenoid cystic carcinoma is one of the rarest eyelid tumors. A 39-year-old man presented with a small subcutaneous induration in his left lower eyelid close to the lower lacrimal punctum. Under a clinical diagnosis of chalazion, the eyelid induration was extracted surgically via the palpebral conjunctiva. Histopathological examination of the extracted tumor revealed proliferation of basaloid cells with a prominent cribriform pattern containing Alcian blue-positive and periodic acid Schiff-positive material, leading to a diagnosis of adenoid cystic carcinoma. The patient subsequently underwent extended radical excision including the lacrimal sac. No recurrence was observed during 20 months after surgery. Adenoid cystic carcinoma should be considered as a differential diagnosis of eyelid tumors simulating chalazion. Adenoid cystic carcinoma in the eyelid may have a more favorable prognosis than that in the lacrimal gland.

Salzmann's-like nodular degeneration following laser in situ keratomileusis

We report the development of bilateral Salzmann's-like corneal lesions located at the site of laser in situ keratomileusis (LASIK) flap margins in 2 patients. These lesions resulted in ocular surface irritation as well as induction of irregular astigmatism and associated loss of best corrected visual acuity. Both patients had bilateral LASIK before the occurrence of the corneal lesions. A biopsy was performed on 1 nodule. The epithelium overlying this raised bluish-white corneal lesion appeared irregular in thickness, with replacement of Bowman's layer by periodic acid-Schiff positive thickened basement membrane-like material. Underlying this basement membrane was a layer of relatively regular, hypocellular, collagenlike connective tissue, which demonstrated hyalinization on trichrome staining. Based on these 2 cases, it seems possible that the hyposecretion of tears, decreased blink rate, and Dellen effect that often occur following LASIK could produce the corneal irritation needed to induce Salzmann's nodular degeneration.

Madarosis from mitochondriopathy

Madarosis from mitochondriopathy