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Peutz--Jeghers syndrome (PJS) is characterized by mucocutaneous pigmentation with multiple hamartomatous polyps of the gastrointestinal tract and/or a familial history with autosomal dominant inheritance pattern having incomplete penetrance, with some cases arising from spontaneous mutations. We present this case of a 12-year-old female who presented with jejunojejunal intussusception and on exploration revealed a polypoidal mass around 50 cm from duodenojejunal flexure acting as a lead point. A segmental jejunal resection with anastomosis was performed and histopathology confirmed a solitary Peutz--Jeghers (PJ) hamartomatous polyp. She neither had mucocutaneous pigmentation nor family history of PJS or any other polyps anywhere in the gut on further evaluation by endoscopies. A solitary PJ polyp in the jejunum is a rare entity, and to the best of our knowledge, only around 13 cases have been reported in world literature so far. Regular follow-up is essential in young children, so that future manifestations of the PJS are not missed. [ABSTRACT FROM AUTHOR]

A retroperitoneal microfilarial lymph varix presenting as inguinal swelling is very rare. The clinical presentation of an inguinal lymph varix mimics that of an inguinal hernia. Here, we are reporting a case of bilateral inguinal swelling clinically diagnosed as an inguinal hernia, which eventually turned out to be a large retroperitoneal microfilarial lymph varix mimicking as an inguinal hernia. [ABSTRACT FROM AUTHOR]

Vasodilator Stimulated Phosphoprotein (VASP) is an important cytoskeletal protein, specifically localized to philopodia and lamillopodia. It is expressed in the cardiovascular system including vascular smooth muscle cells (VSMCs). VASP has two important phosphorylation sites- Ser157 and Set239. VASP Ser157 is a preferential phosphorylation site for PKA and PKC whereas VASP Ser239 is phosphorylated by PKG. Recently it was shown that PKC dependent VASP Ser157phosphorylation contributed for VSMC growth. However the isoform of PKC mediating VASP phosphorylation is not known. We hypothesized that PKC mediates VASP phosphorylation by angiotensin II (AngII) and its subsequent translocation to actin stress fibers leading to pathogenic responses in VSMCs. AngII at 100nM concentration phosphorylated VASP in a time dependent manner. Both AngII and PMA stimulated VASP phosphorylation that was inhibited by 2 µM G66983, a PKC inhibitor. AngII-dependent VASP phosphorylation was completely inhibited in cells transfected with adenovirus (10 moi) encoding dominant negative (dn) PKCα but not with dnPKCδ. In addition, AngII treatment for 30 min resulted in redistribution of VASP specifically to the stress fibers. We conclude that PKCα is the predominant isoform required for AngII-dependent VASP phosphorylation and AngII causes change in the pattern of distribution of VASP. This data suggests the importance of VASP in mediating vascular remodeling induced by AngII. [ABSTRACT FROM AUTHOR]

Great attention has been drawn toward the recently defined IgG4 related sclerosing disease, an entity incorporating autoimmune pancreatitis and other organ involvements, hypergammaglobulinemia and deposition of IgG4 in affected tissues. We demonstrate an index case of this disease with multiple organ involvements and an excellent response to corticosteroid treatment. A 69-year-old white man was diagnosed with IgG4 related sclerosing disease with involvement of lungs, pancreas, submandibular glands, lymph nodes, and kidney. In addition, the patient had polyclonal IgG hypergammaglobulinemia and hypocomplementemia. A renal biopsy showed tubulointerstitial nephritis with extensive lymphoplasmacytic infiltrate. Biopsy of the lung showed "inflammatory pseudotumor" with lymphocyte and plasma cell infiltration. IgG4 positive plasma cells were seen in the biopsy of submandibular gland and the kidney. Immuno-staining of the renal biopsy specimen showed vascular deposition of complement split product C4d. Corticosteroid treatment of 2 months showed complete resolution of the disease process. We demonstrate deposition of C4d in the IgG4 related tubulointerstitial nephritis for the first time.