Epithelioid trophoblastic tumor: A case series

An epithelioid trophoblastic tumor (ETT) is an extremely rare gestational trophoblastic tumor. Cases of ETT present with abnormal vaginal bleeding in women of reproductive age group with marginally elevated beta human chorionic gonadotrophin (B-hCG) levels. Here, we describe a series of four patients (all were females) including histomorphology, immunoprofiles, and diagnostic difficulty of this rare entity. All cases were in their reproductive age group. The mean pre-treatment hCG level was 665.24 (mIU/mL). Microscopically, all cases had a tumor showing an epithelioid appearance arranged in large nests and sheets. Individual tumor cells were round to polygonal with abundant eosinophilic cytoplasm, with central vesicular nuclei and prominent nucleoli. Areas of hemorrhage, necrosis, and intercellular hyaline-like material deposition were identified in all cases (100%). Immunohistochemically, tumor cells in all cases showed diffuse positivity for AE1/AE3 and p63 (100%). GATA3 was available in one case (25%), which was positive in the tumor cells. In one case (25%), hPL was focally positive, and in one case (25%), it was negative. SALL4 was performed in two cases (50%) and was negative in tumor cells. The mean Ki67 labeling index was 19.2 (range 10–30%). All four patients underwent surgical intervention and were treated with hysterectomy. The mean follow-up in this series was 39.4 months (range 6–70), and all patients are alive to date with a mean survival of 32.8 months (range, 4–67).

Meconium pseudocyst revisited

Meconium peritonitis is defined as a sterile chemical or foreign-body peritonitis that is caused by escape of meconium from the intestinal tract into the peritoneal cavity during the fetal or perinatal period. Although meconium peritonitis is indicative of intrauterine perforation of the intestine, it may occur as early as the 4th to 6th month of INTRA NATAL life and as late as several hours after birth. It can be classified into three pathological variations: fibro-adhesive; cystic and generalized. The cystic type has a meconium filled pseudocyst that may rupture in the peritoneal cavity. Intra-abdominal calcification is pathognomonic for the diagnosis. Here, author reported a classical case of meconium peritonitis with pseudocyst formation, which was treated successfully conservatively.

Primary amoebic meningoencephalitis in an infant

Primary amoebic meningoencephalitis is rare but fatal disease encountered in immunocompetent individuals. Here, we present a case of a previously healthy 8-month-old female child, who presented with features of meningoencephalitis of 2 days' duration. Rapidly moving trophozoites of amoeba were observed in cerebrospinal fluid, which were confirmed to be Naegleria fowleri on polymerase chain reaction. Broad-spectrum antimicrobial therapy with ceftriaxone, vancomycin, amphotericin B and acyclovir was initiated. However, the patient deteriorated and left the hospital against medical advice. The isolation of N. fowleri in this case demands for increased awareness for prompt diagnosis and management in view of its high mortality.

Mephentermine Dependence: Emerging Trend of Substance Abuse and its Challenges

Dependence on Mephentermine, a widely used sympathomimetic pressor agent, is so far not extensively reported. In current report, we describe a male patient consuming extraordinary high amount of Mephentermine who developed psychosis which was successfully treated but relapsed after 5 months. In this background we also tried to highlight this trend of shift from more traditional drugs to new synthetic ones and look into the magnitude of the problem and challenges that lie ahead of us.

Mephentermine Dependence: Emerging Trend of Substance Abuse and its Challenges

Dependence on Mephentermine, a widely used sympathomimetic pressor agent, is so far not extensively reported. In current report, we describe a male patient consuming extraordinary high amount of Mephentermine who developed psychosis which was successfully treated but relapsed after 5 months. In this background we also tried to highlight this trend of shift from more traditional drugs to new synthetic ones and look into the magnitude of the problem and challenges that lie ahead of us.

Congenital lobar emphysema: a rare cause of respiratory distress in a neonate

Congenital lobar emphysema (CLE) is a congenital condition characterized by distension and air trapping of the affected lobe of the lung. It is one of the causes of infantile respiratory distress, which may require surgical resection of affected lobe. Case characteristics: 3-day-old neonate with ventilation refractory respiratory distress. Imaging was suggestive of decreased lung tissue on the right side with ipsilateral mediastinal shift. Intervention/ outcome: Early surgical lobectomy was done to improve lung functions and the child improved dur to early intervention. Message: An early diagnosis with high index of suspicion helps patients with this rare congenital anomaly. Early intervention is the key to good long-term outcome. More awareness about the entity and treatment options available would greatly help improving the outcome and disease burden.

Fatal meningitis by Cryptococcus laurentii in a post‑partum woman: A manifestation of immune reconstitution inflammatory syndrome.

Cryptococcal meningitis in immunocompetent post‑partum women has been rarely reported. Immune restoration during post‑partum period leads to unmasking of many opportunistic infections that may have been acquired during pregnancy but manifest itself in the post‑partum period due to immune reconstitution inflammatory syndrome. This case highlights the importance of considering opportunistic pathogens in immunocompetent patients who may be undergoing immune restoration. We report here a fatal case of post‑partum immunocompetent women who presented with clinical features of meningitis. Prognosis of the cryptococcal meningitis not only depends on the immune status of the patient but also on how early the disease is diagnosed in the course of illness.

Lamotrigine-induced fulminant hepatic failure: an unusual presentation.

Lamotrigine (LTG) is indicated for the management of seizures either alone or in combination with other anticonvulsant agents. Adverse effects with it are usually mild. Less than 1% of subjects show deranged liver function tests during long therapy. Fulminant hepatic failure with LTG is an unusual presentation. We report a fatal case of hepatic failure with LTG monotherapy in a 22-year-old male patient suffering from a seizure disorder. Cases of LTG induced hepatotoxicity should be carefully monitored, particularly serious case of fulminant hepatic failure which should be adequately assessed and reported to determine their exact incidence.

Comparison of tolterodine with standard treatment in pediatric patients with non-neurogenic dysfunctional voiding/over active bladder : A systematic review.

To examine the efficacy, safety and tolerability of tolterodine in children with overactive bladder in comparison with standard treatment i.e. oxybutynin as demonstrated in randomized clinical trials and other studies. A systematic search was done to screen the studies evaluating the effect of tolterodine in children with nonneurogenic overactive bladder. Results of studies were pooled and compared. Efficacy was determined from micturition diaries and dysfunctional voiding symptoms score. Safety and tolerability were assessed from the reported treatment emergent adverse events. A total of six randomized clinical trials and 11 other studies of tolterodine in children with urinary incontinence were included in the present systematic review. The dose of tolterodine used in different settings ranged from ‘0.5 to 8 mg/day’ instead of ‘0.5 to 8 mg/kg per day’ and the duration of studies ranged from 2 weeks to 12 months. Both extended and immediate release preparations of tolterodine were shown to have comparable efficacy and tolterodine proved to have comparable efficacy with better tolerability than oxybutynin in these studies. It can be concluded that tolterodine is efficacious in treatment of urinary incontinence in children. Moreover, its efficacy is comparable to oxybutynin, the most commonly prescribed anticholinergic in this condition, while having better tolerability. Hence, it can be considered as first line therapy for the treatment of urinary incontinence in children.

Clinico-pathological impact of cytogenetic subgroups in B-cell chronic lymphocytic leukemia: Experience from India.

BACKGROUND: The present study of 238 B‑cell Chronic Lymphocytic Leukemia (B‑CLL) patients were undertaken to seek the prevalence and to evaluate clinico‑pathological significance of recurrent genetic abnormalities such as del(13q14.3), trisomy 12, del(11q22.3) (ATM), TP53 deletion, del(6q21) and IgH translocation/deletion. MATERIALS AND METHODS: We applied interphase – fluorescence in situ hybridization (FISH) on total 238 cases of B‑CLL. RESULTS: Our study disclosed 69% of patients with genetic aberrations such as 13q deletion (63%), trisomy 12 (28%), 11q deletion (18%), 6q21 deletion (11%) with comparatively higher frequency of TP53 deletion (22%). Deletion 13q displayed as a most frequent sole abnormality. In group with coexistence of ≥2 aberrations, 13q deletion was a major clone indicating del(13q) as a primary event followed by 11q deletion, TP53 deletion, trisomy 12, 6q deletion as secondary progressive events. In comparison with del(13q), trisomy 12, group with coexistence of ≥2 aberrations associated with poor risk factors such as hyperleukocytosis, advanced stage, and multiple nodes involvement. In a separate study of 116 patients, analysis of IgH abnormalities revealed either partial deletion (24%) or translocation (5%) and were associated with del(13q), trisomy 12, TP53 and ATM deletion. Two of 7 cases had t(14;18), one case had t(8;14), and four cases had other variant IgH translocation t(?;14). CONCLUSION: Detail characterization and clinical impact are necessary to ensure that IgH translocation positive CLL is a distinct pathological entity. Our data suggests that CLL with various cytogenetic subsets, group with coexistence of ≥2 aberrations seems to be a complex cytogenetic subset, needs more attention to understand biological significance and to seek clinical impact for better management of disease.

Systemic mastocytosis with associated acute myeloid leukemia with t (8; 21) (q22; q22).

Systemic mastocytosis with associated clonal hematological nonmast cell lineage disease (SM-AHNMD) is a subtype of mastocytosis associated commonly with myeloid neoplasms, Non-Hodgkin's lymphoma, or other hematological neoplasms. In these conditions, mastocytosis needs to be differentiated from mast cell hyperplasia or mast cell activation states. Neoplastic nature of mastocytosis is proved either by morphology, aberrant immunophenotype, or detection of point mutation at codon-816 of c-kit gene. This is a rare entity, even more so in pediatric population. Herein, we report a case of 14-year-old girl with SM associated with acute myeloid leukemia with maturation with t(8;21). Multifocal dense infiltrate of spindle-shaped mast cells on bone marrow aspirate and biopsy with coexpression of CD2 and CD25 by flow cytometric analysis proved the SM component at the time of diagnosis and persistence at post induction status also.

Hyper-reactio Luteinalis associated with Hypothyroidism

Hyper-reactio luteinalis is a non-neoplastic tumor-like ovarian lesion associated with pregnancy. Most patients are asymptomatic, with the ovarian enlargement being incidentally discovered at the time of cesarean section. We report a case of hyper-reactio luteinalis in a pregnant woman with profound hypothyroidism.

The clinical spectrum of fibrocalculous pancreatic diabetes in north India.

BACKGROUND: Fibrocalculous pancreatic diabetes (FCPD) is a secondary form of diabetes, unique to tropical countries. In earlier reports, patients with FCPD had severe insulin-requiring diabetes, malnutrition and a dismal prognosis. With Improvements in nutrition and medical care, the presentation and prognosis of FCPD may have changed. We report on the clinical profile and prognosis of a cohort of FCPD patients from north India and compare our findings with earlier reports. METHODS: Eighty consecutive FCPD patients who presented to the Diabetes, Gastroenterology and Surgical Gastroenterology services were evaluated for their nutritional status, clinical presentation, beta-cell function (fasting C-peptide) and exocrine function (faecal chymotrypsin). All patients diagnosed between 1994 and 2000 (n = 32) were followed prospectively for weight gain and glycaemic control. RESULTS: Only 55% of FCPD patients had a low body mass index (< 18 kg/m2). At the time of diagnosis of diabetes, only 26 (33%) patients presented with severe insulin-requiring diabetes; these patients were younger [23.7 (8.3) years v. 28.7 (10.6) years, p = 0.04], and had higher haemoglobin A1c [9.7 (3.8)% v. 7.3 (2.6)%, p = 0.005] than those requiring diet control or oral hypoglycaemic agents. FCPD patients had a wide range of fasting serum C-peptide (0.03-0.76 nmol/L). C-peptide was negatively associated with increasing duration of diabetes (r = -0.48, p = 0.001), but there was no correlation with faecal chymotrypsin. On prospective follow up (mean 2.3 years), there was significant improvement in body mass index [19.4 (2.9) kg/m2 v. 17.0 (3.7) kg/m2, p < 0.01] and haemoglobin A,c [6.4 (1.6)% v. 8.0 (3.0)%, p < 0.001]. CONCLUSION: FCPD patients differed from those described in earlier reports in many respects, Including improved nutritional status, a wide range of 3cell function and a more favourable prognosis.

Candidemia in neonatal intensive care unit.

The present study was conducted over a period of 6 months to determine the Candida species causing candidemia in a neonatal intensive care unit and to analyse the risk factors associated with acquisition of significant fungemia. Speciation of the 19 isolated Candida spp was done by the standard techniques. Antimicrobial susceptibility of these isolates was determined by disc diffusion method against Amphotericin B, Fluconazole, Ketoconozole and 5-Flucytosine. Candida glabrata was the most common species involved (42.1%). Other species isolated were C. tropicalis (31.6%). Calbicans (21.1%) and C.parapsilosis (5.2%). All the isolates were sensitive to Amphotericin B. Resistance to other antigungal agents was seen only in C. globrata. Significant candidemia was seen in 14/19 (72.6%) of neonates. Risk factors found to be associated with significant candidemis in these neonates included intake of multiple broad-spectrum antibiotics (p<0.0001), use of total parenteral nutrition (p<0.045) and ventilators (p<0.0001).

Effect of sodium stibogluconate on hepatic mixed function oxidase system and marker enzymes of golden hamsters during Leishmania donovani infection.

During L. donovani infection in golden hamsters, tremendous hepatic damage was observed as apparent from increased activities of glutamate oxaloacetate transaminase, glutamate pyruvate transaminase, succinate dehydrogenase, glucose-6-phosphatase and acid ribonuclease. The levels of cytochrome P-450 and related monooxygenases, viz. aniline hydroxylase and aminopyrine-N-demethylase registered significant decrease in infected animals. Sodium stibogluconate, a standard antileishmanial drug, though caused the removal of parasites from infected tissues, but did not help in the recovery of deranged hepatic markers. The results explain the higher mortality of stibanate treated infected animals as compared to untreated animals infected with L. donovani.

Changes in tubular membrane glycosylation in diabetic, insulin and thyroxin treated rat kidneys.

The effect of insulin, thyroxine and alloxan induced diabetes has been studied on brush border membrane glycosylation in rat kidneys. Expressed on dry weight basis, the membrane protein was elevated in insulin, thyroxine and diabetic membranes compared to the control group. Sialic acid content of the membranes was significantly reduced in insulin and thyroxine injected animals whereas fucose level was unaffected under these conditions. Brush border fucose content was significantly reduced in diabetic animals but sialic acid content was unaffected. Membrane hexose and hexosamines levels were unaltered by hormone treatments, but in diabetic rats hexosamine level was significantly reduced. The binding of radiolabelled UEA, WGA, PNA to purified brush borders corroborated changes in membrane saccharides. These findings suggest the role of hormones in membrane glycosylation of rat kidney tubules.

Presentation and management of Helicobacter pylori infection in childhood.

Helicobacter pylori is responsible for one of the most frequently encountered infectious diseases worldwide. Helicobacter pylori infection can lead to the development of gastritis and peptic ulcer disease. The presence of Helicobacter pylori in the human stomach also represents an increased risk of gastric cancer and gastric lymphoma. Epidemiological data obtained in adults suggest that the actual colonization with Helicobacter pylori is in fact determined by childhood factors. Therefore, the pediatric age group represents the ideal target population for studies concerning the pathogenesis and epidemiology of Helicobacter pylori infection. The present work reflects our experience with regard to the diagnosis, epidemiology and pathogenesis of Helicobacter pylori infection in childhood.