Impact of baseline fluorescent antinuclear antibody positivity on the clinical outcome of patients with primary autoimmune hemolytic anemia

Abstract Introduction Autoimmune haemolytic anaemia (AIHA) is an autoimmune disorder that can present in primary or secondary forms. The literature looking at impact of baseline fluorescent antinuclear antibody (FANA) positivity on outcomes of AIHA patients is infrequent. Objective To study the impact of baseline FANA positivity in patients with primary AIHA. Method A prospective cohort study involving 29 consecutive primary AIHA patients presenting to the Haematology department from 2013 to 2015 was analysed. After recording baseline investigations including fluorescent ANA, all patients were treated as per the standard therapeutic protocols. Clinical remission, disease free survival, relapse, mortality were compared between the FANA positive and FANA Negative AIHA groups. Results Baseline FANA positivity was found in 17 patients (58.62%). Both the groups were comparable in terms of age, sex, Hemoglobin, LDH at presentation, number of lines of treatment needed and duration of follow up. Evan's syndrome was seen in six of FANA positive patients which was statistically significant (0 v/s 6, p= 0.023). FANA positive patients had significantly higher rates of relapse per patient month follow up (1.22 v/s 3.57, p= 0.023) and lower rates of complete response (83.33% v/s 35.29%, p= 0.0118) and relapse free survival at five years. Morbidity and mortality were numerically higher in FANA positive patients. Conclusion Baseline FANA positivity among AIHA patients was found to be associated with lower complete response rates and higher relapse rates with possible higher rates of morbidity. Presence of FANA will give us prognostic value and help us in deciding the treatment options.

Comparing the disinfecting efficacy of pomegranate peel extract oil, Garlic oil, Tulsi leaf oil, and Clove leaf oil with standard autoclaving on dental round burs tested against Enterococcus faecalis: An in vitro study.

Background: The study was conducted to compare and evaluate the disinfecting efficacy of four different herbal oils with standard autoclaving against Enterococcus faecalis (E. faecalis). Fifty round burs were infected with E. faecalis and dipped into the chemical test solution for 10 min. The disinfecting efficacy for each test chemical was checked using culture method on blood agar plate and peptone water test to check for turbidity. Clove leaf oil and Tulsi leaf oil have very good antimicrobial efficacy against E. faecalis which are similar and comparable to that of autoclaving. Aim: This stusy aimed to compare the efficacy of pomegranate peel extract (PPE) oil, Garlic oil, Tulsi leaf oil, and Clove leaf oil with standard autoclaving on dental round burs tested against E. faecalis. Settings and Design: The disinfecting capability of four different experimental oils against autoclave was evaluated on 50 round burs exposed to E faecalis in an In-vitro study. Materials and Methods: A total of 50 round dental burs were exposed to E. faecalis and the disinfecting capability of four different experimental oils against autoclave was evaluated by counting colony-forming units (CFUs) and turbidity test. Statistical Analysis: Statistical analysis was performed using SPSS version 20.0 (IBM), Chicago. The CFUs between the five groups (I, II, III, IV, V) were compared using the Chi-square test. The level of significance was kept at 5%. Results: Clove leaf oil showed the best disinfection efficacy comparable to autoclaving, followed by Tulsi leaf oil with no statistically significant difference between them, whereas garlic oil and PPE oil were significantly less effective against E. faecalis. Conclusion: Clove leaf oil and Tulsi leaf oil can be used for disinfecting dental burs, infected with E. faecalis, as alternatives to autoclaving.

Isolated Cricoid Fracture and Thyroid Hematoma in Blunt Injury of the Neck.

Introduction Blunt trauma to the neck may result in life threatening injuries due to airway compromise. Thyroid Injury in a previously normal gland is rare, so is Isolated Cricoid Fracture. The expanding thyroid hematoma and an unstable larynx compound the effects of compression and lead to sudden deterioration of the patient. Case Report We report the case of young male, who sustained a blunt injury in front of the neck. He developed a swelling in the front of his neck and suddenly deteriorated, needing intubation and airway management. Computed Tomographic imaging of the neck showed thyroid hematoma and fracture of the cricoid ring requiring an emergency hemi thyroidectomy and fixation of the cricoid fracture. Discussion This case brings forth the occurrence of two rare entities, Thyroid hematoma and Isolated Cricoid fracture in the same patient needing expert airway management and exploration.

Long-term outcomes in primary male epispadias.

PURPOSE: The authors present a case series of 30 male patients who presented with primary epispadias between 1989 and 2002 and looked at their long-term outcomes. OBJECTIVES: (1) What procedures and operations did these patients require following their original surgery? (2) What were their outcomes as adults in terms of continence, cosmesis, and sexual function? MATERIALS AND METHODS: Primary male epispadias patients who had gone through transition into Adolescent and Adult Urology services were identified retrospectively from electronic patient records. RESULTS: The authors identified 30 male patients with a median follow-up of 18.5 years. Twenty-four had penopubic epispadias, and six had penile epispadias. All initial surgery took place between 9 and 48 months. Twenty-eight patients needed further surgery over the follow-up period, 26 had surgery for continence, and 12 required revision surgery. At follow-up, 15 patients were continent voiding per urethra, nine patients reported stress leakage with volitional urethral voiding, six patients were using a Mitrofanoff to void, and four of these had an ileocystoplasty. Nineteen patients had documentation on their feelings toward cosmetic outcome; 17 expressed concern. Twenty-one patients had documentation about sexual function; 20 had normal erections with six reporting chordee and nine reporting retrograde ejaculation. No patients were recorded to have fathered any children. DISCUSSION: There are only a few published studies looking at long-term outcomes of genitourinary reconstruction in primary male epispadias and their sexual function in adulthood. The majority of this patient cohort required surgery to improve their continence and had more than one continence procedure. There is limited data on continence outcomes in the literature with small cohorts and rates varying between 40 and 100% at 10-year follow-up. Despite corrective surgery, nearly all the patients were concerned about their genital appearance. Other studies have shown similar outcomes in terms of patient satisfaction and sexual function. The limitations of this work are that the information was gathered retrospectively from the electronic patient record and validated instruments on outcomes were not used. CONCLUSIONS: The study outcomes will be useful for clinicians who are counseling patients and parents regarding what to expect as adults.

Applicability of the Cleveland clinic scoring system for the risk prediction of acute kidney injury after cardiac surgery in a South Asian cohort.

BACKGROUND: Acute kidney injury (AKI) after cardiac surgery is a frequent post-operative complication associated with an increased risk of mortality, morbidity and hospital costs. Preoperative risk scores such as the Cleveland Clinic Scoring Tool (CCST) have been validated in Western population group to identify patients at higher risk of AKI and may facilitate preventive strategies. However, the scoring tool has not been validated systematically in a South Asian cohort. We aimed to evaluate the applicability of the CCST in prediction of AKI after open cardiac surgery in a South-Indian tertiary care center. MATERIALS AND METHODS: A retrospective study of all patients who underwent elective open cardiac surgery over a 4year period from Jan 2012 to Dec 2015 at a single centre were included and relevant details extracted from a comprehensive chart review. The primary outcome was AKI as defined by the Kidney Disease Improving Global Outcomes (KDIGO) criteria. Patients were risk stratified as per the CCST to assess for prediction of AKI into low risk (0-2), intermediate risk (3-5) and high risk (>6) groups. RESULTS: A total of 276 patients underwent open cardiac surgery with mean age of 51.5±13.06 yrs. This included 177 (64.1%) males and 99 females (35.8%). Overall incidence of AKI was 6.88%. Mean age, gender, BMI, preoperative serum creatinine, diabetes mellitus, chronic obstructive pulmonary disease, cardiopulmonary bypass time was similar in patients who developed AKI vs those who did not have AKI postoperatively. The mean CCST scores were 1.6 in those without AKI, 1.5 in stage 1, 3.0 in stage 2 and 3.4 in stage 3 AKI. Higher risk scores predicted greater risk of AKI. A total of 106 patients (38.4%) were on ACE/ARB, 119 patients (43.1%) received beta-blockers, 110 (39.8%) received diuretics while 144(52.1%) had received preoperative statins. Comparison of drug use between the two groups revealed that preoperative use of ACEI/ARB was associated with highest risk of AKI (p=0.006). Mortality rate was also high at 15.7% in those with AKI compared to 3.1% in non-AKI group (p=0.04). CONCLUSION: The modified CCST was valid in risk identification of patients with severe stage of AKI but did not have strong discrimination for early AKI stages. Preoperative statin use did not protect against AKI in our study, however preoperative ARB/ACEI use was significantly associated with occurrence of postoperative AKI.

Linear verrucous hemangioma of the upper limb: a rare case.

Verrucous hemangioma is a rare, congenital vascular malformation of the cutaneous and subcutaneous tissue. It is usually present at birth and gradually increases in size and number with age; occasionally it presents in later adulthood. It has a predilection for the lower extremity and usually presents as warty or hyperkeratotic, bluish and partly confluent papules and plaques. Verrucous hemangioma occurring in a linear pattern is an even more uncommon presentation and very few cases have been reported. We report a boy with verrucous hemangioma localized to the left upper extremity in a linear pattern. We also discuss management.

Native nephrectomy in pediatric transplantation--less is more!

OBJECTIVE: Indications for pre-transplantation native nephrectomy (PTNN) include chronic renal parenchymal infection, proteinuria, intractable hypertension, polycystic kidneys and malignancy. Our aim was to establish the frequency and reasons for PTNN in children undergoing renal transplant at our center. MATERIALS AND METHODS: Children listed for renal transplant between 1998 and 2010 who underwent PTNN were analyzed. Etiology of established renal failure, indication for nephrectomy, stage of chronic kidney disease, laterality, complications, and timing of subsequent transplant were determined. Outcome of children, and that of preserved native kidneys following transplant, was reviewed. RESULTS: 21/203 children listed for transplant (10.3%) underwent PTNN (32 nephrectomies). Indications were drug-resistant proteinuria (6 children), recurrent upper tract urosepsis (6), refractory hypertension (4), malignancy/malignant predisposition (4), concomitant procedure during ureterocystoplasty (1). Median age at nephrectomy was 3.3 years; 86% had impaired renal function at time of (first) nephrectomy. Median time until transplantation following bilateral nephrectomy was 1.7 years. 19/21 children have been transplanted; 17 reached stable graft function. Only 2 children who did not undergo PTNN required nephrectomy post-transplant. CONCLUSION: When malignancies were excluded, PTNN was performed in a minority (8.4%) of children, mainly for proteinuria. This adds great advantage by reducing morbidity. Resulting graft function seems favorable.

Idiopathic calcinosis cutis of nasal dorsum.

A 23-year-old lady presented with a slowly progressing firm mass on the nasal dorsum since 8 months. Her biochemical, haematological and collagen vascular disease screening tests were normal. Radiographs of the nasal bones showed a subcutaneous calcifying lesion with no evidence of nasal bone erosion. A diagnosis of idiopathic calcinosis cutis (ICC) was made. The mass was excised and soft tissue defect was augmented with silicone prosthesis. The histopathology with the haematoxylin and eosin staining and von Kossa stain confirmed the diagnosis of calcinosis cutis. This is an unusual presentation of ICC involving the nasal dorsum requiring surgery and nasal dorsal augmentation with silicone prosthesis.

Synchronous laparoscopic-assisted percutaneous endoscopic gastrostomy and peritoneal dialysis catheter placement is a valid alternative to open surgery.

OBJECTIVE: Gastrostomy feeding is frequently necessary in children receiving chronic peritoneal dialysis (PD). Synchronous laparoscopic-assisted placement of percutaneous endoscopic gastrostomy (PEG) and PD catheter has many potential advantages. This study investigates whether this technique is comparable to open placement. METHODS: The notes of all patients over a 16-year time period were reviewed retrospectively. Peritonitis was defined as the presence of a white blood cell count > 100/mm(3) with at least 50% being polymorphonuclear leukocytes, and infection was defined as the presence of positive peritoneal cultures with peritonitis. RESULTS: Ten patients received primary laparoscopic-assisted PEG and PD catheter insertion (LAP) and 23 patients open gastrostomy and PD catheter (OPEN). PD catheter survival was median 12 months in the LAP group and 17 months in the OPEN group. Peritonitis and infection rates per catheter-year were 0.89 and 0.7 LAP and 0.59 and 0.5 OPEN. The risk of peritonitis and infection was not related to method of placement. CONCLUSIONS: There were no statistically significant differences in outcomes between the two groups. We conclude that laparoscopic-assisted synchronous PD and PEG catheter insertion is safe and effective.

The analgesic effects of the GABAB receptor agonist, baclofen, in a rodent model of functional dyspepsia.

BACKGROUND: The amino acid γ-aminobutyric acid (GABA) is an important modulator of pain but its role in visceral pain syndromes is just beginning to be studied. Our aims were to investigate the effect and mechanism of action of the GABA(B) receptor agonist, baclofen, on gastric hypersensitivity in a validated rat model of functional dyspepsia (FD). METHODS: 10-day-old male rats received 0.2 mL of 0.1% iodoacetamide in 2% sucrose daily by oral gavages for 6 days. Control group received 2% sucrose. At 8-10 weeks rats treated with baclofen (0.3, 1, and 3 mg kg(-1) bw) or saline were tested for behavioral and electromyographic (EMG) visceromotor responses; gastric spinal afferent nerve activity to graded gastric distention and Fos protein expression in dorsal horn of spinal cord segments T8-T10 to noxious gastric distention. KEY RESULTS: Baclofen administration was associated with a significant attenuation of the behavioral and EMG responses (at 1 and 3 mg kg(-1)) and expression of Fos in T8 and T9 segments in neonatal iodoacetamide sensitized rats. However, baclofen administration did not significantly affect splanchnic nerve activity to gastric distention. Baclofen (3 mg kg(-1)) also significantly reduced the expression of spinal Fos in response to gastric distention in control rats to a lesser extent than sensitized rats. CONCLUSIONS & INFERENCES: Baclofen is effective in attenuating pain associated responses in an experimental model of FD and appears to act by central mechanisms. These results provide a basis for clinical trials of this drug in FD patients.

Recognition and treatment of genitourinary complications in paediatric Crohn's disease using Infliximab.

UNLABELLED: Although genitourinary complications of Crohn's are well recognized, available information regarding their clinical course and management is sparse especially in the paediatric population. We report a myriad of urological complications in five paediatric cases, our experience from a tertiary paediatric urological and gastroenterological centre. All children with urological complications had severe Crohn's disease which necessitated the use of immuno-suppressants including Infliximab. Three of four children healed and closed their fistulas after treatment, although failed to avoid future surgery, albeit for other reasons. CONCLUSION: We suggest Infliximab should be considered as a treatment option but in the absence of a common consensus, treatment be tailored to individual cases.

A rare case of skull base metastasis from follicular carcinoma of thyroid.

We are reporting a case of skull base metastasis from follicular carcinoma of thyroid in an adult lady. She presented with the history of a longstanding thyroid swelling and recent onset epistaxis and diplopia. She was evaluated and diagnosed to have follicular carcinoma of thyroid with metastasis to the skull base. She underwent Total Thyroidectomy with modified neck dissection followed by Radioiodine ablation of the metastasis.

Dysplastic kidneys in children - do they grow?

OBJECTIVES: Dysplastic kidneys (DK) are a common cause of chronic kidney disease (CKD). Little is known about their growth or how their sonographic appearance changes. This study aimed to test the hypothesis that DK gain little length, and to identify radiologic trends predictive of CKD. METHODS: Ultrasound scans of children with DK born in 1980-2005 and referred to a single tertiary centre were analysed by a pediatric radiologist. Renal lengths were plotted on standard nomograms and the degree of dysplastic appearance noted. Factors related to DK - bladder outlet obstruction, vesico-ureteric reflux and renal impairment - were noted. RESULTS: Fifty-three children were studied (83 kidneys), of whom 41 were boys; 289 scans were analysed. In 33 children there was associated bladder outlet obstruction or vesico-ureteric reflux. Forty-four DK were noted to fall off their renal length 'centile'. This correlated well with the development of CKD and is statistically significant. Deterioration occurred in 53% of DK; primarily progressive reduction in corticomedullary differentiation. This also correlated well with development of CKD. CONCLUSION: More than half of the DK showed poor growth velocity. This, together with the degree of sonographic abnormality, carries a high predictive value for development of CKD. We recommend diligent serial sonography to follow renal growth and dysplastic appearance in children with DK.

Ellis-van Creveld Syndrome (chondro-ectodermal dysplasia) in two siblings.

Two male siblings aged 12 and 15 years (Figure 1) presented with growth retardation, limb abnormalities and, defective teeth and nail since childhood. There was no history of similar defects in other family members. On examination their height was 114 cm and 130 cm (expected height; 169 cm and 150 cm) respectively. There were hypoplastic and dystrophic nails, and microdontia and hypodontia on dental examination. There were post-axial polydactyly in both the hands and left foot. Skeletal survey revealed short forearms, legs & distal phalanges (Acromesomelia). Cardiac or other organ system defects were not noticed on clinical examination or echocardiogram. Clinical picture was suggestive of a diagnosis of Chondroectodermal dysplasia (Ellis van Creveld syndrome). These cases have been reported for their rarity and for the presence of some rare features of this syndrome.

P2X receptor-mediated visceral hyperalgesia in a rat model of chronic visceral hypersensitivity.

BACKGROUND: Irritable bowel syndrome (IBS) is a common gastrointestinal disorder characterised by abdominal pain and bloating in association with altered bowel movements. Its pathogenesis and the underlying molecular mechanisms of visceral hyperalgesia remain elusive. Recent studies of somatic and other visceral pain models suggest a role for purinergic signalling mediated by the P2X receptor (P2XR) family. AIMS: To examine the role of P2XR signalling in the pathogenesis in a rat model of IBS-like visceral hyperalgesia. METHODS: Visceral hypersensitivity was induced by colonic injection of 0.5% acetic acid (AA) in 10-day-old rats and experiments were conducted at 8 weeks of age. Dorsal root ganglion (DRG) neurons innervating the colon were labelled by injection of DiI (1,1'-dioleyl-3,3,3',3-tetramethylindocarbocyanine methanesulfonate) fluorescence into the colon wall. RESULTS: Visceral hypersensitivity was reversed by TNP-ATP (2'-(or-3')-O-(trinitrophenyl) ATP), a potent P2X1, P2X3 and P2X2/3 receptor antagonist. Rapid application of ATP (20 microM) induced a fast inactivating current in colon-specific DRG neurons from both control and AA-treated rats. There was a twofold increase in the peak ATP responses in neurons from AA-treated rats. These currents were sensitive to TNP-ATP (100 nM). Under current-clamped conditions, ATP evoked a larger membrane depolarisation in neurons from neonatal AA-treated rats than in controls. P2X3R protein expression was significantly enhanced in colon-specific DRGs 8 weeks after neonatal AA treatment. CONCLUSIONS: These data suggest that the large enhancement of P2XR expression and function may contribute to the maintenance of visceral hypersensitivity, thus identifying a specific neurobiological target for the treatment of chronic visceral hyperalgesia.