Unusual content of omphalocele: a congenital mature cystic teratoma of falciform ligament of the liver.

Omphalocele is a congenital midline abdominal wall defect that results in herniation of intraabdominal contents covered by a lining of peritoneum and amnion. The severity of the defect often ranges from a minor herniation of the umbilical cord to a significant protrusion that includes large proportions of intestine and liver. We report unusual content of omphalocele that is congenital mature cystic teratoma of falciform ligament of liver which was confirmed by histopathology. This is a very uncommon entity and not described in world literature.

Sutureless skin closure with isoamyl 2-cyanoacrylate in pediatric day-care surgery.

Importance of day-care surgery in pediatric age group is ever increasing and nowadays bulk of surgery is performed as a day-care procedure. Day-care procedure requires short intra-operative period and less postoperative complications. The introduction of tissue adhesive has been received enthusiastically since they may result in equivalent tensile strength-improved cosmetic appearance of the scar and lower infection rate when compared to sutures, staples and adhesive tape. We have used isoamyl 2-cyanoacrylate as a tissue adhesive for skin closure in day-care surgery. Novocryl is ideal for such procedure as it is a sterile, inert, non-toxic, biocompatible and bacteriostatic liquid topical skin adhesive containing isoamyl 2-cyanoacrylate formulation. We applied isoamyl 2-cyanoacrylate glue in 100 patients over skin cut margin after stitching subcutaneous tissue. Skin margin were held together by means of skin hook for 1-2 min. The cosmesis was better as there were no suture marks or lumpiness. There were no major complications in our study. Thus, isoamyl 2-cyanoacrylate can be considered as an alternative to skin suturing in the pediatric day-care surgery.

Gastric teratoma presented as gastric outlet obstruction and melena; report of rare case.

Gastric teratoma is a very rare tumor, accounting for less than 1 % of all teratomas in infants and children. Melena or upper gastrointestinal tract bleeding in newborns and infants is a rare event and is usually caused by a benign lesion. Gastric teratoma has been reported as a cause of gastrointestinal bleeding on a few occasions. As gastric teratomas generally present as a palpable abdominal mass, more aggressive solid masses of childhood must be excluded. We present intramural extension of gastric teratoma presented as symptom of gastric outlet obstruction and melena.

Preduodenal Portal Vein Associated with Duodenal Obstruction of other Etiology: A Case Series.

DuodenalPreduodenal portal vein is a rare and interesting entity which often causes duodenal obstruction. It is also associated with other congenital anomalies. We report here three cases of preduodenal portal vein associated with other anomalies causing duodenal obstruction not related to direct compression by portal vein itself.

Giant splenic lymphangiomatosis in adult: a diagnostic dilemma.

Cystic lymphangiomatosis of the spleen occurs secondary to developmental malformation of the lymphatic system. It is one of the rare entities generally seen in children. It is usually seen in children. We report a case of cystic lymphangiomatosis of the spleen occurring in an adult woman presenting with massive splenomegaly. Total splenectomy was considered to be the treatment of choice for making definitive diagnosis and to exclude the presence of malignancy or other causes of massive splenomegaly.

Purification and characterization of urease from dehusked pigeonpea (Cajanus cajan L) seeds.

Urease has been purified from the dehusked seeds of pigeonpea (Cajanus cajan L.) to apparent electrophoretic homogeneity with approximately 200 fold purification, with a specific activity of 6.24 x10(3) U mg(-1) protein. The enzyme was purified by the sequence of steps, namely, first acetone fractionation, acid step, a second acetone fractionation followed by gel filtration and anion-exchange chromatographies. Single band was observed in both native- and SDS-PAGE. The molecular mass estimated for the native enzyme was 540 kDa whereas subunit values of 90 kDa were determined. Hence, urease is a hexamer of identical subunits. Nickel was observed in the purified enzyme from atomic absorption spectroscopy with approximately 2 nickel ions per enzyme subunit. Both jack bean and soybean ureases are serologically related to pigeonpea urease. The amino acid composition of pigeonpea urease shows high acidic amino acid content. The N-terminal sequence of pigeonpea urease, determined up to the 20th residue, was homologous to that of jack bean and soybean seed ureases. The optimum pH was 7.3 in the pH range 5.0-8.5. Pigeonpea urease shows K(m) for urea of 3.0+/-0.2 mM in 0.05 M Tris-acetate buffer, pH 7.3, at 37 degrees C. The turnover number, k(cat), was observed to be 6.2 x 10(4) s(-1) and k(cat)/K(m) was 2.1 x 10(7) M(-1) s(-1). Pigeonpea urease shows high specificity for its primary substrate urea.

Comparative evaluation of the canal curvature modifications after instrumentation with One Shape rotary and Wave One reciprocating files.

AIMS: This study compared the canal curvature modifications after instrumentation with One Shape (Micro Mega) rotary file and Wave One primary reciprocating file (Dentsply Maillefer, Ballaigues, Switzerland). MATERIALS AND METHODS: Thirty International Organization for Standardization 15, 0.02 taper, Endo Training Blocks (Dentsply Maillefer) were used. In all specimens working length (WL) was established at the reference point 0. Glide path was achieved with Path-File 1, 2 and 3 (Dentsply Maillefer) at the WL. Group 1 were shaped with One Shape file and group 2 with Wave One files. Pre and post-digital images were superimposed, processed with Corel draw Graphic Suite X5 (Corel Corporation, Ottawa, Canada), Adobe Photoshop CS3 (Adobe Systems Inc., San Jose, CA) and Solid works student Edition software (Dassault Systems Solid Works Corp, S.A., Velizy, France). RESULTS: Mean was more for Wave One compared with One Shape. One-way ANOVA and t-test showed a significant difference between One Shape and Wave One at 5% level of significance (P < 0.05). CONCLUSIONS: Canals prepared with Wave One file preserved canal shape, respected the anatomical shape of J-shaped canal and produced a continuously tapered funnel.

Tracheoesophageal fistula with esophageal atresia with absent external ear: a unusual association.

Esophageal atresia with tracheoesophageal fistula (TEF with EA) is a common disease which is associated with many other congenital abnormalities. The most common association is with VACTERL syndrome.. Various types of ear malformations have been reported in CHARGE syndrome associated with EA and TEF. However, absence of external ear has not been described which is being reported in this article. We could not found this association even on extensive search of literature.

Split notochord syndrome associated with dorsal neuroenteric fistula: A rare entity.

Split notochord syndrome (SNS) is an extremely rare congenital malformation associated with anomalies of the vertebral column, gastrointestinal tract and central nervous system. Twenty cases of SNS associated with dorsal enteric fistula have been reported in literature till date. The present report describes a unique case of SNS associated with lumbosacral meningomyelocele, dorsal neuroenteric fistula and dorsal herniation of right kidney along with vessels. The neonate was well managed by excision of enteric fistula, closure of duramater of meningomyelocele and repair of posterior wall hernia after placement of kidney in renal fossa. This kind of entity is uncommon and not been reported earlier.

Therapeutic potential of matrix metalloprotease inhibitors in neuropathic pain.

IMPORTANCE OF THE FIELD: Millions of people suffer from neuropathic pain (NP), but the treatment is empirical and results in transient relief in only a few patients. This is primarily because of the poor understanding of the molecular mechanism underlying NP. Following nerve injury, there is a differential and temporal pattern of MMPs expression that coincides with changes in levels of pro-inflammatory cytokines, suggesting that MMPs not only act as mediators for neuroinflammation but might also be directly involved in pain associated with nerve damage. AREAS COVERED IN THIS REVIEW: The present review describes the different mechanisms of NP. The main focus of the review is to highlight the importance of MMPs in NP and their inhibition as a novel approach for treating NP. WHAT THE READER WILL GAIN: A comprehensive overview of the role of MMPs in the pathogenesis of NP and the potential of MMP inhibition as a therapeutic intervention for NP. TAKE HOME MESSAGE: Targeted therapy using specific MMP inhibitors, siRNAs, peptide inhibitors and monoclonal antibodies can provide a better way of treatment by blocking a single MMP and can reduce the side effects of broad-spectrum MMP inhibitors.

Twin fetus in fetu in a child: a case report and review of the literature.

INTRODUCTION: Fetus in fetu is an extremely rare condition wherein a malformed fetus is found in the abdomen of its twin. This entity is differentiated from teratoma by its embryological origin, its unusual location in the retroperitoneal space, and the presence of vertebral organization with limb buds and well-developed organ systems. The literature cites less than 100 cases worldwide of twin fetus in fetu. CASE PRESENTATION: A two-and-a-half-month-old Asian Indian baby boy had two malformed fetuses in his abdomen. The pre-operative diagnosis was made by performing an ultrasound and a 64-slice computer tomography scan of the baby's abdomen. Two fetoid-like masses were successfully excised from the retroperitoneal area of his abdomen. A macroscopic examination, an X-ray of the specimen after operation, and the histological features observed were suggestive of twin fetus in fetu. CONCLUSION: Fetus in fetu is an extremely rare condition. Before any operation is carried out on a patient, imaging studies should first be conducted to differentiate this condition from teratoma. Surgical excision is a curative procedure, and a macroscopic examination of the sac should be done after twin or multiple fetus in fetu are excised.

Concavo-convex oblique anastomosis technique for jejuno ileal atresia.

AIM: To evaluate the role of end-to-end oblique bowel anastomosis in bowel atresia. METHODS: End-to-end oblique anastomosis was done in 25 neonates of bowel atresia and the results were compared with traditional method of end to back anastomosis in 25 cases. RESULTS: We found less mortality and morbidity (5%) in our technique as compared to end to back technique (20%). CONCLUSIONS: We recommend this technique, as anastomosis is wide with less angulations, flow of effluent is linear, and there is less force exerted over post-anastomotic side wall.

Duplication cyst of pyloroduodenal canal: a rare cause of neonatal gastric outlet obstruction: a case report.

BACKGROUND: A 21 day old male child presented with non bilious vomiting and abdominal mass. CASE PRESENTATION: This case is reported because pyloroduodenal duplication cysts are an extremely rare congenital anomaly, whose clinical presentation often mimics those of hypertrophic pyloric stenosis. Ultrasound examination showed cystic mass at pyloric region and barium study was suggestive of extrinsic mass compressing the pyloric region. A laparotomy, a tense cystic mass was present at the pyloroduodenal junction (PDC) which was resected and end to end anastomosis was done. Patients followed an uneventful recovery and doing well. CONCLUSION: The clinical and radiological analysis can reveal configurational changes consistent with a large extrinsic mass rather than muscular hypertrophy and can lead to accurate preoperative diagnosis.

Prostaglandin E(2) synthase inhibition as a therapeutic target.

BACKGROUND: Most NSAIDs function by inhibiting biosynthesis of PGE(2) by inhibition of COX-1 and/or COX-2. Since COX-1 has a protective function in the gastro-intestinal tract (GIT), non-selective inhibition of both cycloxy genases leads to moderate to severe gastro-intestinal intolerance. Attempts to identify selective inhibitors of COX-2, led to the identification of celecoxib and rofecoxib. However, long-term use of these drugs has serious adverse effects of sudden myocardial infarction and thrombosis. Drug-mediated imbalance in the levels of prostaglandin I(2) (PGI(2)) and thromboxane A(2) (TXA(2)) with a bias towards TXA(2) may be the primary reason for these events. This resulted in the drugs being withdrawn from the market, leaving a need for an effective and safe anti-inflammatory drug. METHODS: Recently, the focus of research has shifted to enzymes downstream of COX in the prosta glandin biosynthetic pathway such as prostaglandin E(2) synthases. Microsomal prostaglandin E(2) synthase-1 (mPGES-1) specifically isomerizes PGH(2) to PGE(2), under inflammatory conditions. In this review, we examine the biology of mPGES-1 and its role in disease. Progress in designing molecules that can selectively inhibit mPGES-1 is reviewed. CONCLUSION: mPGES-1 has the potential to be a target for anti-inflammatory therapy, devoid of adverse GIT and cardiac effects and warrants further investigation.

Noncommunicating isolated enteric duplication cyst in childhood.

Duplications of the alimentary tract are spherical or tubular structures lined by epithelium similar to intestine that are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply and do not communicate with the intestine. There are only 4 cases reported in the English literature-3 in childhood and 1 in an adult, and all are male. We report a case of noncommunicating isolated ileal duplication cyst in a 3-year-old female child that presented as a mass in the right iliac fossa, mimicking an intussusception. The duplication had its own blood supply arising from the terminal ileal mesentery. This report describes the first female patient with this condition and reviews the English literature.

Double coin in esophagus at same location and same alignment - a rare occurrence: a case report.

Coin is the most common foreign body swallowed by pediatric age group. The multiple coin swallowing is extremely rare and very few cases had been reported in English literature. Most of them were present at different site and had different alignment in the esophagus. The location of the coin (trachea vs. esophagus) is commonly determined by the alignment of the coin on radiographic studies. A 4-year-girl was presented to us with history of coin ingestions one day back without any respiratory distress. On radiological study there was suspicion of two coins on same location and alignment. The diagnosis was confirmed after removal. The both coin was removed successfully by esophagoscopy. Unexpected second foreign bodies in pediatric esophageal coin ingestions are rare and it is mandatory to do post operative radiography after removal to exclude duplex coin or tracheal coin. We are presenting this case because of its rarity, difficulty in diagnosis especially when proper history is not available.

A child presented with bilateral congenital constriction ring in lower extremity: a case report.

INTRODUCTION: The congenital constriction ring of lower extremity is very uncommon and rare condition. The actual incidence in general population is not known. In English literature, very few cases are reported time to time as congenital constriction band syndrome associated with musculoskeletal disorder like congenital talipes equino varus. The lesion can involve skin only or goes to deeper structure up to bone, which can lead to gangrene of foot or auto amputation. CASE PRESENTATION: We are presenting a case of bilateral congenital constriction ring in lower limb who presented at age of 4 year without any other associated congenital anomaly, simply managed by Z-plasty, which improves quality of life after physiotherapy. CONCLUSION: Congenital constriction ring of lower limb is extremely rare condition in children. Early diagnosis and management is mandatory, either in single stage or by stage procedure, to prevent auto-amputation of limb and to improve quality of life on feet.

Atresia of ileocecal junction, ileocecal valve: Rare variants of bowel atresia.

Atresia of ileocecal junction and isolated atresia of ileocecal valve are rare types of intestinal atresia with very few reports in literature. We report two such cases. Radiology showed dilated ileal segment and distal micro colon in both the cases. At laparotomy there was atresia of ileocecal junction in the first case and isolated ileocaecal valve atresia with normal ileocecal junction in the other case. Both the babies were managed by ileocolic resection with an end to end anastomosis. The prognosis of ileocecal atresias is satisfactory.

Chronic obstructive pulmonary disease: role of matrix metalloproteases and future challenges of drug therapy.

COPD is a chronic disease of the lung that is characterised by decreased air flow and associated abnormal inflammatory responses of the lungs. A total of 80% of COPD incidences are observed in patients with history of smoking tobacco. The chronic condition of COPD is characterised by airway remodelling, which leads to emphysema and chronic bronchitis. Inflammatory cells of the immune system play a major role in pathophysiology of COPD. High levels of neutrophils, macrophages and CD8(+) T cells have been found in bronchoalveolar lavage samples of COPD patients. Matrix metalloproteases (MMPs), which are secreted by these inflammatory cells, have the enzymatic capacity to cause morphological changes in the lungs and contribute significantly to the COPD state. Increased concentrations of MMP-1, -2, -9, -12 and so on have been found in bronchoalveolar lavage samples of COPD patients compared with non-COPD individuals. COPD is rated as among the top five diseases with high mortality rates and it is estimated that in the next 20 years, the healthcare cost alone for COPD will be US $800 million worldwide. The present drug therapies are neither very efficacious nor cost effective; hence, there is unmet medical need to discover small-molecule drugs for COPD. In this regard, synthetic MMP inhibitors show a great promise for COPD treatment.