Hyaline Material Research Articles

Infantile Systemic Hyalinosis: Novel Founder Mutation in the Initiation Codon among "Malis (Farmers)" in Jodhpur.

Infantile systemic hyalinosis (OMIM 236490) is a progressive autosomal recessive disorder characterized by widespread deposition of hyaline material in many tissues leading to multiple subcutaneous skin nodules, gingival hypertrophy and joint contractures. The authors describe five children from four unrelated families, from the "mali (farmer)" community in Jodhpur, with the disorder. All of them had classical clinical features, and four died from severe infections between age of 7 mo to 3 y. Two affected children had the same, but novel mutation in the initiation codon, in homozygous form c.1A>G; p. M1? in capillary morphogenesis protein-2 (CMG2), or ANTXR2 gene on chromosome 4q21.21. The other two parents had the same mutation in heterozygous form. It is likely that this is a founder mutation in this community.

Telepathology a Useful Method in the Diagnosis Digestive Biopsies with Rare Entities. Case Presentation of a Collagenous Gastritis.

Introduction/ Background Collagenous gastritis is a rare entity in current surgical pathology practice. Since the introduction of the term until present, only small series and isolated cases have been reported, generally by high-specialized centers in gastrointestinal pathology. In current practice, the diagnosis of this unusual affection is based on clinical data corroborated with endoscopic and pathological findings. Due to the small number of cases the diagnostic criteria are partly taken from microscopic colitis diagnosis. Thus, it is considered that the association of the appropriate clinical context (stomach pain, watery diarrhea, endoscopic nodular appearance) and histological gastritis changes with collagen deposition of dense material with a 10um minimum variable thickness that comprises capillaries and inflammatory elements. The technical advantage of a digital slide scanner is allowing access to an experienced pathologist in digestive pathology’s opinion and conducting precision measurement. Aims Our presentation brings to attention of pathologist community a rarely diagnosed entity and the advantages of tele-pathology in timely accessing an expert opinion and performing accurate measurements. Methods We received gastric biopsy samples endoscopically taken from a 56-year female who complained of transit disorders, vomiting, epigastric pain that did not improve after IPP treatment. On endoscopy exam a nonspecific aspect with discrete erythema and vaguely nodular appearance of the gastric mucosae was noted. The specimens were paraffin embedded, and serial sections were stained HE, AB-PAS and MGG. The obtained slides were fully scanned and a gastrointestinal pathology expert advice was requested. Subsequently, a Mason’s Trichrome stain and tenascin immunohistochemistry test were performed. The examined section showed gastric mucosal epithelium with scarce intraepithelial lymphocytes (5-6 lymphocytes/100 cells). The surface epithelium had the tendency to roll away from the variable thickened stroma. The glandular architecture and chorion were modified by a nonspecific inflammatory infiltrate. A diagnose of chronic inactive gastritis with intestinal metaplasia areas was propose, and an expert opinion was requested. Digitally scanned sections using the Leica II Aperio slide scanner were taken. Examination of slides was made using the Imagescope12.2 Leica. Micrometeric measurements of the hyaline material varied between 9.5-51um. The review of glass slides on traditional optical microscope was made confirming the diagnosis. Results Collagenous gastritis remains an underestimated condition due to its low incidence and lack of expertise in the field. Hence telepathology becomes a useful tool for requesting a second opinion and precisions measurements.

Pathogenesis of dermonecrosis induced by venom of the spitting cobra, Naja nigricollis: An experimental study in mice

The pathogenesis of dermonecrosis induced by the venom of the African spitting cobra Naja nigricollis was investigated in a mouse model. Intradermal injection of venom induced a macroscopic necrotic lesion. Histological examination revealed early edema of the dermis, followed by blistering, loss of skin appendages and reduction in cellularity. By 24 h, necrosis of the dermis was evident, sections of epidermis were lost, and a fibrinoid hyaline material filled the damaged areas. Abundant inflammatory infiltrate was present in the hypodermis and basal dermis, and there was an increment in the expression of matrix metalloproteinases (MMPs). Thrombi were observed in blood vessels. Abundant cells were present in the dermis by 7 days. By 14 and 28 days, re-epithelization had occurred, collagen was widespread in the dermis, and few skin appendages were present. The RP-HPLC fractions that reproduced the necrotic activity were composed of low molecular mass cytotoxins of the three-finger toxin family and, to a lesser extent, of phospholipases A2 (PLA2). Inhibition of PLA2 of venom by p-bromophenacyl bromide did not reduce the area of necrosis, but modified the appearance of necrotic regions. Depletion of neutrophils and inhibition of venom metalloproteinases and tissue MMPs did not affect dermonecrosis. IgG and F(ab′)2 antivenoms were effective in the neutralization of dermonecrosis when incubated with venom prior to injection. However, when antivenoms were administered immediately after venom injection, dermonecrosis was reduced only to a partial extent, underscoring the difficulties in neutralizing this effect with antivenoms.

PT120 Cardiac Amyloidosis by Transthyretin Gene Mutation Val 122IIe. The First Case Described in Argentina in a No African American Man

Because of its unique anatomic position as the way station for returning venous blood from the small and large intestines and the organ of blood egress back to the heart via the hepatic veins and inferior vena cava, and as the recipient of dual blood flow (portal vein and hepatic artery), there are many lesions that potentially can affect the liver. Lesions affecting the hepatic artery and its branches often produce infarction. The smaller arterioles within portal tracts may be affected by hyaline arteriolosclerosis, relatively newly described in the liver, in which hyaline material is deposited into the arterial wall. The congested liver (congestive hepatopathy) of chronic cardiac disease currently receives major attention from hepatologists because of the possible need for two-organ (heart–liver) transplant. Scoring systems for congestive hepatopathy-related fibrosis are established and discussed in this chapter. Greater attention is being paid to the long-term survivors of Fontan surgery for congenital heart diseases like hypoplastic left heart syndrome; these patients are at an increased risk for hepatocellular carcinoma. The sinusoidal obstruction syndrome (SOS) (formerly termed veno-occlusive disease or VOD) remains an important problem, particularly in individuals undergoing cytoablative therapy with agents such as oxaliplatin.

Urbach-Weithe disease (lipoid proteinosis): A classical presentation.

A 10-year-old female child presented with a history of blistering over face and entire body along with a weak cry since infancy and hoarseness of voice that progressively worsened with age. The blisters would occur in response to minor trauma and resolve with depressed scars. On examination, multiple, atrophic, pock-like scars were noted over the face [Figure 1] as well as extremities and trunk [Figure 2]. Both eyelids showed multiple, well-defined beaded papules along the upper and lower margins (moniliform blepharosis) [Figure 1]. Skin over the extensor aspect of the elbows and knees was thickened and showed a few brownish, thickened plaques [Figure 3]. Upon oral examination, the sublingual frenulum and tongue were found to be infiltrated and thickened with inability to protrude the tongue beyond the oral cavity [Figure 4]. The tongue had a woody hard feel on palpation. The parents denied any history of epilepsy, behavioral disturbances, or similar ailment in any family member. A provisional diagnosis of lipoid proteinosis was made and subsequently confirmed by histopathological examination, which revealed periodic acid schiff (PAS)-positive, diastase-resistant eosinophilic material in dermis and walls of blood vessels [Figure 5]. Parents were counseled regarding the nature and progression of the disease.Figure 1: Multiple beaded papules on upper and lower eyelids in both eyes, with pock-like scars on nose and cheeksFigure 2: Multiple depressed scars over buttocks and thighsFigure 3: Brownish hyperpigmented plaques over both kneesFigure 4: Thickened, woody hard, and fixed tongue in oral cavityFigure 5: PAS-positive, diastase-resistant eosinophillic material deposition in perivascular and periadnexal spaces in dermis (20x)Lipoid proteinosis, also known as Urbach–Wiethe disease or hyalinosis cutis et mucosae, is a rare, autosomal recessive, inherited disorder associated with mutations in the ECM1 gene,[1] which encodes for the glycoprotein extracellular matrix protein 1. About 300 cases have been reported so far in the literature. The disease is characterized by deposition of hyaline material in oral cavity, skin, larynx, central nervous system, and others. It usually presents in infancy as hoarseness and weak cry that progressively worsens with age and may lead to aphonia[2] due to inelasticity of the vocal cords. The woody hard tongue is difficult to move or protrude outside oral cavity.[3] Skin susceptibility to trauma is marked and vesicles/bullae appear in crops, to subsequently heal with acneiform scars. The typical beaded papules along the upper and lower eyelid margins and brownish verrucous plaques over extensor surfaces such as elbows and knees are characteristic of the disease.[4] Poor nail growth, dental anomalies, and intracranial calcification in amygdala and temporal lobes leading to epilepsy or psychiatric disturbances may be seen.[5] Typical skin lesions, hoarseness, and thick immobile tongue usually suffice for clinical diagnosis, although other possible differential diagnoses, such as erythropoietic protoporphyria, amyloidosis, lichen myxedematosus and myxedema with hoarseness need to be carefully ruled out. Histology reveals hyperkeratosis, acanthosis and deposition of characteristic PAS-positive, diastase-resistant eosinophilic hyaline material in the perivascular, periadnexal areas as well as dermis and submucosa. Microlaryngeal surgery for vocal cords, blepharoplasty for eye lesions and dermabrasion, chemical peeling, and CO2 laser therapy for skin lesions may offer some symptomatic relief.[2] Favorable outcome with acitretin and d-penicillamine has been reported by some authors.[67] The overall prognosis of lipoid proteinosis is good, with normal life expectancy and stabilization of the disease in adulthood. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Hyaline Fibromatosis Syndrome: A Rare Inherited Disorder.

Hyaline fibromatosis syndrome (HFS) is rare autosomal recessive disease characterized by the deposition of amorphous hyaline material in skin and visceral organs. It represents a disease spectrum with infantile systemic hyalinosis (ISH) being the severe form and juvenile hyaline fibromatosis (JHF) being the mild form. Dermatologic manifestations include thickened skin, perianal nodules, and facial papules, gingival hyperplasia, large subcutaneous tumors on the scalp, hyperpigmented plaques over the metacarpophalangeal joints and malleoli, and joint contractures. ISH shows a severe visceral involvement, recurrent infections, and early death. We report a case of 2.5-year-old female patient who presented with HFS who had overlapping features of both ISH and JHF. To the best of our knowledge, very few cases of HFS have been reported in Indian literature till date.

Importance of Hyaline Material in Herniated Lumbar Disk Pathology: Predicting the Presence of Hyaline from Preoperative Magnetic Resonance Imaging and its Clinical Significance

Importance of Hyaline Material in Herniated Lumbar Disk Pathology: Predicting the Presence of Hyaline from Preoperative Magnetic Resonance Imaging and its Clinical Significance

Macular Amyloidosis and Epstein-Barr Virus.

Background. Amyloidosis is extracellular precipitation of eosinophilic hyaline material of self-origin with special staining features and fibrillar ultrastructure. Macular amyloidosis is limited to the skin, and several factors have been proposed for its pathogenesis. Detection of Epstein-Barr virus (EBV) DNA in this lesion suggests that this virus can play a role in pathogenesis of this disease. Objective. EBV DNA detection was done on 30 skin samples with a diagnosis of macular amyloidosis and 31 healthy skin samples in the margin of removed melanocytic nevi by using PCR. Results. In patients positive for beta-globin gene in PCR, BLLF1 gene of EBV virus was positive in 23 patients (8 patients in case and 15 patients in the control group). There was no significant difference in presence of EBV DNA between macular amyloidosis (3.8%) and control (23.8%) groups (P = 0.08). Conclusion. The findings of this study showed that EBV is not involved in pathogenesis of macular amyloidosis.

Renal cell carcinoma with t(6;11)(p21.2;q13)/MALAT1-TFEB fusion: a clinical and pathological analysis

To study the clinicopathologic features, immunophenotype, differential diagnosis and prognosis of renal cell carcinoma (RCC) associated with t(6;11)(p21.2;q13)/MALAT1-TFEB gene fusion. A total of 9 cases of such rare tumor were selected for clinicopathologic, immunohistochemical and molecular analysis, with review of literature. The age of the patients ranged from 21 to 42 years (mean=31.3 years). The patients included four men and five women. Histologically, 4 of the 9 cases studied showed classic morphologic features of TFEB RCC, with hyaline material, pigments and psammoma bodies frequently identified. The remaining 5 cases demonstrated uncommon morphology, mimicking perivascular epithelioid cell neoplasm, clear cell RCC, chromophobe RCC or papillary RCC. Immunohistochemical study showed that TFEB and vimentin were positive in all cases. Most of the tumors studied also expressed Ksp-cadherin, E-cadherin, CD117, HMB45, Melan A and Cathepsin K. CKpan showed immunostaining in only 1 case. The staining for TFE3, CD10 and CK7 were all negative. TFEB gene rearrangement was detected in all the 9 cases studied using fluorescence in-situ hybridization. MALAT1-TFEB fusion gene was identified in 2 cases by polymerase chain reaction and direct sequencing. TFEB RCC seemed to be an indolent tumor. During a mean follow-up of 31 months, none developed tumor recurrence, progression, or metastasis. TFEB fusion-associated RCC is a rare neoplasm, tends to occur in young age group and carries an indolent behavior. Diagnosis relies on clinicopathologic findings and immunohistochemical analysis. TFEB break-apart FISH assay is a reliable tool in confirming the diagnosis.

Time, Dose, and Volume Responses in a Mouse Pulmonary Injury Model Following Ablative Irradiation

We aimed to determine the time, dose, and volume responses in a mouse pulmonary injury model following ablative dose focal irradiation (ADFIR) in order to better understand normal lung injury. ADFIR was administered to the left lung of mice using a small animal micro-irradiator. Histopathological evaluation and micro-computed tomography (micro-CT) analyses were performed at 1, 2, 6, and 12 weeks after irradiation. Dose responses were tested at doses of 0-90 Gy in C57BL/6 and C3H/HeJCr mice at 6 weeks after irradiation. The volume effect was evaluated with 1-, 3-, and 5-mm diameter collimators at 1-4 weeks after 90-Gy irradiation. ADFIR caused gross local lung injury of the inflated lung in just 1 week, with extensive hyaline material visible in the irradiated area. The fibrosing process was initiated as early as 2 weeks after irradiation. C3H and C57 mice did not show significant differences in dose response. Six weeks after irradiation, the radiation dose-response curve had a sigmoidal shape, where the lag, log, and stationary phases occurred at <40, 50-70, and >80 Gy, respectively. ADFIR induced substantial volume-dependent structural and functional damage to the lungs, and the volume changes of lung consolidation on micro-CT correlated inversely with lung fibrosis over time. We determined the time, dose, and volume responses in our established small animal model, and found that lung injury was substantially accelerated and phenotypically different from that of prior studies using non-ablative hemi-thorax and complete thorax irradiation schemes.

Hyalinizing Trabecular Tumor of the Thyroid Gland

A 34-year-old woman with a 4-year history of a slowly enlarging thyroid gland underwent a total thyroidectomy. Histologic sections showed multinodular colloid goiter. In addition, a 1.2 centimeter diameter discrete mass with a solid white cut surface was noted within the left lobe.&#x0D; Sections from the left lobe mass show a well-demarcated tumor whose cells are arranged in trabecular and nested growth patterns. (Figure 1) The cells are polygonal to spindly and have ample eosinophilic, slightly granular cytoplasm and oval to angular nuclei that are often grooved. (Figure 2) Hyaline material and a delicate fibrovascular stroma surround the nests and trabeculae, and occasional psammoma bodies are seen. (Figure 3) These features led us to a diagnosis of hyalinizing trabecular tumor.&#x0D; Hyalinizing trabecular tumor (HTT) is a rare thyroid neoplasm of follicular cell derivation.1, 2 The tumor occurs in adults with a wide age range (4th – 7th decades) and a mean age of 47 years. It is more common in females.1 The classic histologic findings are of a solid circumscribed epithelial neoplasm with or without a thin capsule composed of medium to large-sized polygonal to fusiform cells that are arranged in alveolar, trabecular and nested groups. The cells have finely granular, acidophilic, amphophilic or clear cytoplasm. Nuclei often have prominent grooves and small nucleoli. Calcospherites (psammoma bodies) may be present. Colloid is scant or absent. 1, 2, 3&#x0D; &#x0D; Because of overlapping nuclear features, a follicular variant of papillary thyroid carcinoma is a differential diagnosis. Histologic features are usually sufficient to distinguish the entities as a nested-alveolar architecture is rarely a prominent feature of a papillary carcinoma.2 Immunohistochemistry may be of aid in this distinction especially in difficult cases with limited material. Cytokeratin 19 and HBME1 are negative in HTT and are usually positive in papillary thyroid carcinomas.4, 5, 6 Neuroendocrine markers are also negative in HTT and are positive in medullary thyroid carcinomas and paragangliomas.2&#x0D; &#x0D; HTT is of uncertain malignant potential, and a 2008 review of 119 HTTs has shown only one case progressing to malignancy.3 The majority of cases have behaved in a benign fashion and thus may be treated conservatively.1

Treatment of lipoid proteinosis with ablative Er:YAG laser resurfacing

Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis characterized by deposition of amorphous hyaline material in different parts of the body, especially the skin and mucous membranes. Disfiguring lesions predominantly affect facial appearance. There is no curative therapy and treatment options are limited to symptomatic approaches. Facial disfigurement in this disease may have an huge negative effect on the patients' psychology and quality of life. With this regard, the patients may benefit very much from symptomatic treatments. Four patients with LP were treated with Er:YAG laser to ablate disfiguring lesions on the face. Patients were followed up for 14 months to 2 years. We obtained favorable clinical and aesthetic results in all cases with Er:YAG laser treatment and did not observe any recurrences during the follow-up. Depending on our observations Er-YAG laser can be accepted as an effective tool for dermal accumulations and scars of LP with precise ablation capability and favorable esthetic results.

Clinicopathological study of centrally necrotizing carcinoma of the breast.

BackgroundCentrally necrotizing carcinoma of the breast (CNC) represents a newly-identified subset of breast cancer. The clinical and pathological characteristics of this breast cancer subtype are not yet completely understood.MethodsWe assessed the clinicopathological characteristics of 73 cases of CNC and 30 control cases of high-grade infiltrating ductal carcinoma (IDC) with focal necrosis based on light microscopy and immunohistochemical staining for estrogen receptor, progesterone receptor, Cerb-B2/HER2, Ki-67, epidermal growth factor receptor, cytokeratin 5/6, smooth muscle actin, S-100 protein, p63 and CD10.ResultsAll the tumors showed extensive central necrotic or acellular zones with different degrees of fibrotic or hyaline material surrounded by ring-like or ribbon-like residual tumour tissue which were usually high-grade IDCs. The central necrotic zone accounted for at least 30% of the cross-sectional area of the tumor. Thirty-six cases (49.3%) showed a component of ductal carcinoma in situ. The tumorous stroma around the central necrotic zone was accompanied by myxoid matrix formation in 28 cases (40%). Lymphocytic infiltration was present in 53 cases (72.6%). Granulomatous reactions were detected at the periphery of the tumors in 49 cases (67.1%). Immunohistochemistry showed greater expression of basal-like markers (72.2%, 52 cases) than myoepithelial markers (60.6%, 43 cases), both of which were significantly higher than in controls (26.7%, 8 cases) (P < 0.001). According to molecular typing, most CNCs were basal-like subtype (37 cases, 50.7%). Follow-up data were available for 28 patients. Disease progression occurred in 11 patients. The combined rate of recurrence, distant metastasis or death was significantly higher in CNC patients compared with controls (P < 0.05).ConclusionsCNC was associated with distinctive clinicopathologic features mostly characterized as basal-like type. Its high proliferative activity, highly-aggressive biological behavior, and high rates of recurrence and metastasis, suggest that CNC should be classified as a new type of breast carcinoma.

Trichomes related to an unusual method of water retention and protection of the stem apex in an arid zone perennial species.

It is well known that trichomes protect plant organs, and several studies have investigated their role in the adaptation of plants to harsh environments. Recent studies have shown that the production of hydrophilic substances by glandular trichomes and the deposition of this secretion on young organs may facilitate water retention, thus preventing desiccation and favouring organ growth until the plant develops other protective mechanisms. Lychnophora diamantinana is a species endemic to the Brazilian 'campos rupestres' (rocky fields), a region characterized by intense solar radiation and water deficits. This study sought to investigate trichomes and the origin of the substances observed on the stem apices of L. diamantinana. Samples of stem apices, young and expanded leaves were studied using standard techniques, including light microscopy and scanning and transmission electron microscopy. Histochemical tests were used to identify the major groups of metabolites present in the trichomes and the hyaline material deposited on the apices. Non-glandular trichomes and glandular trichomes were observed. The material deposited on the stem apices was hyaline, highly hydrophilic and viscous. This hyaline material primarily consists of carbohydrates that result from the partial degradation of the cell wall of uniseriate trichomes. This degradation occurs at the same time that glandular trichomes secrete terpenoids, phenolic compounds and proteins. These results suggest that the non-glandular trichomes on the leaves of L. diamantinana help protect the young organ, particularly against desiccation, by deposition of highly hydrated substances on the apices. Furthermore, the secretion of glandular trichomes probably repels herbivore and pathogen attacks.

Safety and feasibility of the use of a bevacizumab-methylcellulose mixture as an adjunct to glaucoma surgery: a pilot study.

Bevacizumab, a monoclonal anti-vascular endothelial growth factor antibody, has been suggested as a potential healing therapeutic following glaucoma surgery. Here, we aimed to improve the bioavailability of bevacizumab when used as an adjunct therapy to non-penetrating deep sclerectomy (DS) by using a bevacizumab-methylcellulose mixture (BMM). Ten previously non-operated eyes in ten patients diagnosed with primary open angle glaucoma underwent DS with a subconjunctival injection of 0.3 ml of BMM (bevacizumab 3.75 mg incorporated into 4% methylcellulose) at the surgical site. Bevacizumab release was evaluated in vitro using size-exclusion high performance liquid chromatography (HPLC). Intraocular pressure (IOP), bleb morphology, corneal endothelial cell count (CECC), and complications were evaluated at 6 months after surgery. Using HPLC, bevacizumab was detected in BMM for up to 72 h. Moreover, all surgical blebs remained expanded with hyaline material during the first week. A significant IOP reduction (mean ± SD= -10.3 ± 5.4 mmHg, P<0.001) and diffuse blebs were observed at the final follow-up period. Although CECC was slightly reduced (-7.4%), no complications were observed. In conclusion, bevacizumab was released from BMM, and the use of this innovative mixture yielded good results following DS with no complications. Further studies are required to determine its efficacy prior to establishing BMM as an adjunct treatment for penetrating and non-penetrating glaucoma surgeries.

Importance of Hyaline Material in Herniated Lumbar Disk Pathology: Predicting the Presence of Hyaline from Preoperative Magnetic Resonance Imaging and its Clinical Significance

Importance of Hyaline Material in Herniated Lumbar Disk Pathology: Predicting the Presence of Hyaline from Preoperative Magnetic Resonance Imaging and its Clinical Significance

Importance of Hyaline Material in Herniated Lumbar Disk

Importance of Hyaline Material in Herniated Lumbar Disk

Lipoid proteinosis with oral manifestation in a geriatric patient: A unique case report

Lipoid proteinosis is a very rare, autosomal recessive disorder, characterized by hoarseness of voice, skin scarring, beaded papules along the eyelid margins, and an inability to protrude the enlarged tongue. Infiltration of hyaline material to various organs is found to be responsible for the various manifestations of this disorder. It is mainly prevalent in early infancy and childhood, and is rare in older adults. In this article, we report a 61-year-old patient with the skin and orofacial features of lipoid proteinosis, which is very rarely recorded in literature as far as age and oral manifestation are concerned.

Effect of Semicarbazide on the Testis of Juvenile Male Albino Rat

Background: Semicarbazide (SEM) is an azodicarbonamide byproduct present in a glass jar packaged foods especially baby foods. A relatively high consumption of these products by infants can result in higher exposure compared with other consumers. Objectives: This study was performed to throw light on the effect of SEM on the testis of juvenile male albino rat. Materials and Methods: This study was carried out on 20 juvenile male albino rats divided into the control group, and SEM treated group. Animals of the SEM treated group were administered 40 mg of SEM orally once daily for 30 days. At the end of the experiment, animals were weighed and then sacrificed. The parameters examined were gonadal index, epididymal spermatozoal examination, histological study, immunohistochemical staining for proliferating cell nuclear antigen (PCNA) in addition to ultrastructural study. Morphometric study and statistical analysis of the results were carried out. Results: The gonadal index of SEM treated group showed a significant decrease compared with the control group. Loss of germ cells was confirmed by a significantly low sperm count. SEM induced variable degrees of tubular affection in the form of distorted seminiferous tubules, cellular disorganization, sloughing and cytoplasmic vacuolation. Acidophilic hyaline material, vacuolations, and congested blood vessels were present in most of the interstitial spaces. Immunohistochemically, SEM treatment induced a marked reduction in the number of PCNA immunoreactive cells. An ultrastructural alteration of spermatogenic cells and wide separation in between germ cells were observed. Conclusions: These results suggest that growing animals are more susceptible to gonadal toxicity of SEM. It is better to avoid food products sold in glass jars, especially during the juvenile period.

Lipoid Proteinosis: a case report in two siblings

Lipoid proteinosis was first reported by Urbach and Wiethe in 1929. It is also known as hyalinosis cutis et mucosae or Urbach-Wiethe disease. It is a rare autosomal recessive disorder and characterized by the infiltration of hyaline material in the skin, oral cavity, larynx, and internal organs. Lipid proteinosis presents early in life. Hoarseness develops in infancy. The classic sign is beaded eyelid papules along the lid margin, also known as 'Monilform Blepherosis'. In India about 30 cases have been reported to date. We report the following case because of its rarity in the Indian literature.