Hydroxychloroquine-induced phospholipidosis in a case of SLE: the wolf in zebra clothing.

A 30 year old lady patient of SLE on steroid and hydroxychloroquine therapy presented with lupus nephritis and later developed cardiac symptoms. Her renal biopsy revealed features of Class III lupus nephritis. Also seen was typical lamellated myelinoid material in the glomerulus. The alpha-galactosidase A activity was normal. The clinical morphological and biochemical findings were consistent with Lupus nephritis showing changes of hydroxychloroquine induced phopholipidosis. Electron microscopy along with careful clinical examination and follow up status was instrumental in the diagnosis of the latter.

Microvillous inclusion disease--an ultrastructural diagnosis: with a review of the literature.

Microvillous inclusion disease is a rare disorder of infancy associated with protracted diarrhea. This malady reveals distinct ultrastructural changes. The surface enterocytes of the duodenum show vesicles lined with microvilli and the surface microvilli are poorly formed. The authors present one case of microvillous inclusion disease with a review of the literature.

Ultrastructure of the H. pylori microbe in individuals having macrocytosis and B12 deficiency.

The incidence of Helicobacter pylori gastritis is high in India and the number of individuals with vitamin B12 deficiency is also large. An association has been found between these two factors. It is necessary to determine whether H. pylori infection may be a factor in the causation of B12 deficiency and whether it is associated with any morphological changes on ultrastructural examination. A cohort-based study has been performed, which includes 505 young asymptomatic males. These cases have been investigated for presence of H. pylori and macrocytosis. The study confirms an association between H. pylori infection and B12 deficiency. It is recommended that H. pylori infection be looked for in subjects having macrocytosis of unknown etiology.

Banded collagen in the kidney with special reference to collagenofibrotic glomerulopathy.

BACKGROUND: The normal glomerular basement membrane, composed of type IV collagen, plays an important function in the process of filtration. Rarely, type III, type I, or type V collagen is seen in the glomerulus, resulting in three different types of non-immune mediated glomerulopathies recognized thus far. These are characterized by deposition of banded collagen fibers in the glomerulus. METHODS: The authors reviewed 4934 kidney biopsies submitted over the past 5 years. Five of these revealed the presence of banded collagen in the glomeruli. CONCLUSION: Combined clinical and ultrastructural examination has led to a definitive diagnosis. These diseases exhibit indolent progression and as yet do not have specific treatment.

Non seminomatous mixed germ cell tumor of the testis with predominant component of somatic type malignancy (Rhabdomyosarcoma) - A rare occurrence.

Testicular germ cell tumors (GCT) are a diverse group of neoplasms, broadly divided into seminomatous and non seminomatous types, with varying histomorphology. Mixed germ cell tumors express more than one germ cell component. Somatic type malignancy occurring in testicular GCT is rare. Most often these components present as metastasis, particularly following chemotherapy, rather than primarily involving the testis. We describe a rare case of a young adult with no significant past history, who presented with testicular mass which on histology revealed a non-seminomatous mixed germ cell tumor with additional somatic type malignancy component of a rhabdomyosarcoma.

Clinico-pathological Profile and Outcome of C-3 Glomerulopathy in Indian Children.

INTRODUCTION: There is paucity of data of C3 glomerulopathy in Indian children. METHODS: First Indian pediatric case series where consecutive renal biopsies done over a period of ten years were reviewed to identify those patients who had isolated or predominant C3 deposits on immunofluorescent microscopy, fulfilling the criteria for C-3 glomerulopathy. The clinical, biochemical, serological, histopathological profile, eGFR and the need for renal replacement therapy was analyzed. RESULTS: Eighteen patients, comprising 5.3% (18/298) of all renal biopsies, had C3 glomerulopathy, four with Dense Deposit Disease (DDD) and fourteen with C3 Glomerulonephritis (C3GN) with a median follow-up of 38.2 months. Median age of presentation was 7.45±3.03 years (2.5yrs- 13.5yrs) with nine boys and nine girls. Presentation was nephrotic syndrome in seven (39%), acute nephritic syndrome in three (16.7%), hematuria in five (27.7%) and acute kidney injury in three (16.7%). Median eGFR was 69 ml/min/1.73m2 (8.2-107 ml/min/1.73m2). Hematuria was seen in 16 (88%), proteinuria in 18 (100%) and low C3 in 16 (88%) at the time of presentation. Mesangioproliferative glomerulonephritis was the predominant pattern in DDD while C3GN showed a mix of mesangioproliferative, membranoproliferative, endocapillary and crescentic GN (p = 0.43).Complete or partial remission was seen in seven patients who received long term alternate day steroids alone or with added mycophenolate mofetil. The cumulative patient survival was 70.8%. Kaplan Meir analyses for renal survival without progression to ESRD was 60.2% at one year and 48.1% at five and ten years. CONCLUSION: Interstitial fibrosis and tubular atrophy on renal biopsy was an independent predictor of adverse renal outcome in the cohort (p = 0.013, HR8.1;95% CI -1.6-42).

Desmoplastic non-infantile ganglioglioma: a low-grade tumor, report of two patients.

Desmoplastic infantile gangliogliomas (DIGG) are low-grade (WHO Grade I) tumors of early childhood rarely occurring in older children and young adults. The mainstay of treatment is surgical. We report two older children with DIGG, both showing atypical radiology. One case was treated with radiation. The recurrence treated with only surgical excision showed excellent response to treatment.

Stomach metastasis of breast carcinoma mimicking primary gastric neoplasm on fluorodeoxy glucose-positron emission tomography-computed tomography.

We present fluorodeoxy glucose positron emission tomography-computed tomography (FDG-PET/CT) findings in a case of breast carcinoma. The PET/CT findings in this case were suspicious of second primary neoplasm in the stomach. However, on endoscopic biopsy, the lesion was found to be stomach metastasis of breast carcinoma with estrogen receptor positivity. Stomach is a rare site of breast carcinoma metastasis. Our case suggests that it is difficult to distinguish a stomach metastasis of breast cancer from a primary gastric cancer on the basis of clinical and imaging features. However, this differential diagnosis must be kept in mind and it is important to make such distinction because of its implications on patient management.

Atypical hemolytic uremic syndrome with membranoproliferative glomerulonephritis.

Atypical hemolytic uremic syndrome (aHUS) associated with membranoproliferative glomerulonephritis (MPGN) is an uncommon clinical presentation, especially in children. We report a 8-year-old-boy who presented like aHUS but the kidney biopsy showed MPGN type 1.