Surgical nuances in the separation of craniopagus twins - Our experience and a follow up of 15 years.

Craniopagus twins are conjoined twins fused at the cranium. This is the rarest anomaly seen in conjoined twins and craniopagus twins account for 2- 6% of conjoined twins. Conjoined twins are also extremely rare, with the anomaly seen in about 10-20 subjects per billion births. A female preponderance has been noted. Craniopagus twins can be classified into complete or partial, depending on whether or not they have shared dural venous sinuses. They can be further classified into angular or total depending on the alignment of the inter-twin longitudinal axis. Surgical separation of these cases can be an elective procedure or an emergency, mandated by the death of one of the twins. Surgical separation of craniopagus twins is a complex exercise needing detailed evaluation and planning. For the successful management of these twins, a multi-disciplinary approach involving neurosurgeons, plastic surgeons, anesthetists, radiologists, pediatric critical care specialists and ancillary staff is mandatory. We present a case of partially successful elective separation of partial angular craniopagus twins performed in 2002.The surviving twin was managed conservatively for a cerebrospinal fluid leak. The patient subsequently developed a pseudomeningocele, necessitating re-exploration, excision of the gliotic tissue, and repair of the dura and the overlying scalp flap. The patient has a skull defect for which cranioplasty has been deferred. The child is neurologically intact. The post-operative evaluation and the detailed periodic neurological assessment till date (with a follow up of 15 years) have been presented in this study.

Burden and predictors of hypertension in India: results of SEEK (Screening and Early Evaluation of Kidney Disease) study.

BACKGROUND: Hypertension (HTN) is one of the major causes of cardiovascular morbidity and mortality. The objective of the study was to investigate the burden and predictors of HTN in India. METHODS: 6120 subjects participated in the Screening and Early Evaluation of Kidney disease (SEEK), a community-based screening program in 53 camps in 13 representative geographic locations in India. Of these, 5929 had recorded blood pressure (BP) measurements. Potential predictors of HTN were collected using a structured questionnaire for SEEK study. RESULTS: HTN was observed in 43.5% of our cohort. After adjusting for center variation (p < 0.0001), predictors of a higher prevalence of HTN were older age ≥ 40 years (p < 0.0001), BMI of ≥ 23 Kg/M2 (p < 0.0004), larger waist circumference (p < 0.0001), working in sedentary occupation (p < 0.0001), having diabetes mellitus (p < 0.0001), having proteinuria (p < 0.0016), and increased serum creatinine (p < 0.0001). High school/some college education (p = 0.0016), versus less than 9th grade education, was related with lower prevalence of HTN. Of note, proteinuria and CKD were observed in 19% and 23.5% of HTN subjects. About half (54%) of the hypertensive subjects were aware of their hypertension status. CONCLUSIONS: HTN was common in this cohort from India. Older age, BMI ≥ 23 Kg/M2, waist circumference, sedentary occupation, education less, diabetes mellitus, presence of proteinuria, and raised serum creatinine were significant predictors of hypertension. Our data suggest that HTN is a major public health problem in India with low awareness, and requires aggressive community-based screening and education to improve health.

Epidemiology and risk factors of chronic kidney disease in India - results from the SEEK (Screening and Early Evaluation of Kidney Disease) study.

BACKGROUND: There is a rising incidence of chronic kidney disease that is likely to pose major problems for both healthcare and the economy in future years. In India, it has been recently estimated that the age-adjusted incidence rate of ESRD to be 229 per million population (pmp), and >100,000 new patients enter renal replacement programs annually. METHODS: We cross-sectionally screened 6120 Indian subjects from 13 academic and private medical centers all over India. We obtained personal and medical history data through a specifically designed questionnaire. Blood and urine samples were collected. RESULTS: The total cohort included in this analysis is 5588 subjects. The mean ± SD age of all participants was 45.22 ± 15.2 years (range 18-98 years) and 55.1% of them were males and 44.9% were females. The overall prevalence of CKD in the SEEK-India cohort was 17.2% with a mean eGFR of 84.27 ± 76.46 versus 116.94 ± 44.65 mL/min/1.73 m2 in non-CKD group while 79.5% in the CKD group had proteinuria. Prevalence of CKD stages 1, 2, 3, 4 and 5 was 7%, 4.3%, 4.3%, 0.8% and 0.8%, respectively. CONCLUSION: The prevalence of CKD was observed to be 17.2% with ~6% have CKD stage 3 or worse. CKD risk factors were similar to those reported in earlier studies.It should be stressed to all primary care physicians taking care of hypertensive and diabetic patients to screen for early kidney damage. Early intervention may retard the progression of kidney disease. Planning for the preventive health policies and allocation of more resources for the treatment of CKD/ESRD patients are imperative in India.

Collapsing Glomerulopathy Superimposed on Diabetic Nephropathy.

Diabetic nephropathy (DN) is characterized by progressive increase in proteinuria and decline in renal functions. Various forms of nondiabetic kidney disease may be superimposed on DN, which can alter the progression of DN. Collapsing glomerulopathy (CG) may superimpose on DN, and is characterized by rapid worsening of renal failure and has poor prognosis. In our institute, renal biopsies were performed in diabetic patients for increasing proteinuria or worsening renal functions. There were seven cases of CG superimposed on DN. All patients except one had a history of long standing diabetes mellitus. All patients had nephrotic range proteinuria. Four patients had severe renal failure at presentation. Renal biopsy showed CG superimposed on DN. Six patients progressed to end-stage renal disease during follow-up; one patient is in chronic kidney disease-stage 3b. The development of CG contributes to an increased level or new onset proteinuria in DN, and can lead to rapid worsening of renal failure. The diagnosis of CG superimposed on DN is of prognostic significance.

Rare Case Report of De-novo Pauci-immune Necrotizing Crescentic Glomerulonephritis in 1st-week Post-kidney Transplantation.

Early post-transplant de-novo pauci-immune necrotizing crescentic glomerulonephritis occurring within the first week post transplantation has not been reported according to the literature search done by us. Here, we are reporting a 56 years male patient who underwent living spousal donor kidney transplantation for presumed diabetic nephropathy, and developed pauci-immune necrotizing crescentic glomerulonephritis (NCGN) on sixth day post-transplant. Post-renal transplant recurrence of disease in ANCA-associated vasculitis patients occurs within days to months. However, development of de-novo pauci-immune crescentic glomerulonephritis is a rare occurrence. We report this patient who developed probable pauci-immune de-novo NCGN which responded to treatment with plasmapheresis, steroids and cyclophosphamide.

A Case of Hearing Impairment with Renal Dysfunction.

Hearing impairment in a patient with renal failure is an important clue towards etiologic diagnosis of kidney disease. Variety of hereditary diseases, developmental defects, and toxins involve these two organs. However, additional retinopathy is seen in quite a few diseases which include Alport's syndrome and Muckle-Wells syndrome (MWS). We are reporting a case of middle-aged woman with childhood-onset of hearing impairment who presented with renal failure and was diagnosed to have renal amyloidosis on kidney biopsy but without any light chain restriction. During evaluation for live donor kidney transplant, her brother was also found to have hearing impairment and retinopathy however with normal renal function and urinalysis. Genetic testing of both of them was done for panel of mutations related to hereditary amyloidosis which revealed NLRP3 mutation in both. This mutation is characteristic of MWS which can lead to secondary amyloidosis and renal failure.

Conversion from mycophenolate mofetil to enteric-coated mycophenolate sodium in renal transplant recipients with gastrointestinal tract disorders.

OBJECTIVE: The objective of this prospective study was to evaluate the effect of conversion from mycophenolate mofetil (MMF) to enteric-coated mycophenolate sodium (EC-MPS) in renal transplant recipients with gastrointestinal tract (GI) reverse effects using patient-reported outcomes instrument. METHODS: A multicenter, open-label, prospective study was undertaken in renal transplant recipients treated with MMF. In patients experiencing GI tract symptoms, treatment was changed to equimolar EC-MPS (myfortic). At baseline and visit 2 (4-6 weeks after baseline), patients completed the Gastrointestinal Symptom Rating Scale (GSRS) and Gastrointestinal Quality of Life Index (GIQLI), and physicians completed the Overall Treatment Effect (OTE) scale at visit 2. A difference of 0.5 or greater in the OTE score is indicative of clinical relevance. RESULTS: Of 154 patients screened, 118 fulfilled the inclusion or exclusion criteria. Eighty-five men and 33 women with a mean age of 41.6 years participated in this study. Median time since transplantation was 12 months. Mean (SD) dose of MMF reported at baseline was 1209.4 (422.89) mg/d. More than 50% of patients reported MMF-associated nausea, dyspepsia, and abdominal pain. After conversion to an equimolar dose of EC-MPS, patients showed improvement in GI symptoms. This benefit was predominantly observed in patients with moderate to severe symptoms at baseline. On the GSRS, patients reported a significant (P < .05) reduction in symptom burden across all parameters (reflux, 36%; diarrhea, 38%; indigestion, 36%; constipation, 28%; and abdominal pain, 40%). On the GIQLI also, significant (P < .05) improvement was reported (symptoms, 18%; emotional status, 22%; physical functioning, 21%, and use of medical treatment, 18%). On the OTE scale, 84.7% of patients reported improvement in GI symptoms. CONCLUSION: In patients with moderate to severe GI symptoms, changing treatment from MMF to EC-MPS significantly reduces GI-related symptom burden and improves GI-specific quality of life.

Outbreak of Ralstonia mannitolilytica in Hemodialysis Unit: A Case Series.

Ralstonia mannitolilytica is a Gram-negative soil bacteria. It is an emerging opportunistic pathogen in hospital-acquired infections. Maintenance hemodialysis patients at Manipal Hospital Outpatient Haemodialysis unit, Bengaluru, witnessed an outbreak of R. mannitolilytica infection between October 2016 and November 2016. Five patients were infected and one of them presented with infective endocarditis. All patients were successfully treated with antibiotic according to culture and sensitivity pattern. Immediately following the outbreak, environmental sampling was done. The culture from sterile water was positive for R. mannitolilytica growth. The Department of Infection Control ordered for discarding the whole batch of sterile water followed by water treatment with shock chlorination and room disinfection. Following implementation of the same, the outbreak of R. mannitolilytica infection was controlled. R. mannitolilytica infections are hospital acquired, affecting mainly immunocompromised patients. The disease onset and progression is rapid. Early identification of the organism and treatment with appropriate antibiotics is important.

Cerebral salt wasting syndrome in a patient with tuberculous meningitis.

We report a case of a 65 year male with meningitis who had polyuria, severe hyponatremia, volume depletion and very high urinary sodium excretion. He was diagnosed to have cerebral salt wasting syndrome based on clinical and laboratory parameters.

Paraoxonase activity and antibodies to oxidized-LDL in chronic renal failure patients on renal replacement therapy.

Serum paraoxonase (PON1) and antibodies to oxidized-LDL (anti ox-LDL) were measured in chronic renal failure subjects on renal replacement therapy such as hemodialysis (HD) peritoneal dialysis (PD) and transplantation (Txp). Paraoxonase activity was significantly lower in HD and PD group (P<0.001) than in control subjects. In transplant patients, paraoxonase activity was not significantly different from that of controls. Antibodies to ox-LDL was significantly higher in HD, PD and Transplant patients (P<0.0001) compared to control subjects. High titers of antibodies were observed in the HD group compared to the PD and Transplant subjects. A decrease in paraoxonase activity and high titers of Antibodies to ox-LDL in the dialysis group suggest a decreased cardio protective effect of HDL and enhanced risk of premature cardiovascular complications. Whereas in case of transplant subjects, there seems to be restoration of PON1 activity, but elevated levels of anti-oxLDL could still be a potential atherogenic factor. Hence, we propose that estimation of these two parameters can be used as a useful index to measure the cardiac risk in the above patient category.

Primary hyperparathyroidism in a child.

Primary hyperparathyroidism is rare in children. We report a 12-year-old girl who presented with recurrent renal calculi, muscular weakness and inability to walk; was diagnosed to have parathyroid adenoma and underwent parathyroidectomy.

Spectrum of renal involvement in hematolymphoid neoplasms: Renal biopsy findings of 12 cases.

Spectrum of causes for renal dysfunction in patients with hematolymphoid malignancy (excluding plasma cell dyscrasia) is varied. A retrospective evaluation of "native" renal biopsies referred to our institute during the period from January 2010 to December 2013 revealed 12 cases. Age ranged between 7 and 69 (median 54.5) years. All patients were males. The neoplasms included non-Hodgkin lymphoma, chronic lymphocytic leukemia, acute lymphoblastic leukemia, Burkitt's lymphoma, intravascular lymphoma, Hodgkin lymphoma and chronic myeloid leukemia. Proteinuria was noted in 66% of the patients (nephrotic range in 5, subnephrotic range in 3). Renal insufficiency was noted in 100% patients. Malignancy-related kidney injury was noted in 75% of the cases. Renal histology showed lymphomatous infiltration (8), membranoproliferative glomerulonephritis (MPGN) (3), intracapillary monoclonal deposit disease (1) and intravascular lymphoma (1). Distribution of lymphomatous infiltration was diffuse in 50% and focal in 50%. We observed that renal dysfunction was predominantly a direct effect, that is, lymphomatous invasion. Paraneoplastic glomerulopathic changes occur in the form of MPGN. Proteinuria of >2 g/day correlated with glomerular disease.

Efficacy of streptokinase in resistant, relapsing or recurrent CAPD peritonitis.

The efficacy of streptokinase to lyse biofilm in the catheters of CAPD patients with peritonitis was examined in a retrospective review of 10 infusions in 9 patients with difficult to resolve peritonitis. 750,000 units of streptokinase were used. This helped resolve peritonitis in 8 of 10 uses, including two cases due to gram negative bacteria. Staphylococcus was cultured in the other 8 cases. The two episodes which failed to respond were due to Staph epidermidis relapsing peritonitis and required either catheter removal or two additional courses of antibiotics before peritonitis resolved. One infusion was complicated by severe hypotension which was thought to be due to generalized sepsis. The patient had received a previous streptokinase infusion but skin tests for IgE allergy to streptokinase were negative. We conclude that streptokinase is efficacious in the resolution of slow to resolve peritonitis due to either gram positive or gram negative organisms, and potentially saved seven patients from catheter removal.

Infective endocarditis as a cause of fever in hemodialysis patients.

Vascular access infections are common in maintenance hemodialysis patients especially with dual lumen cuffed catheter. Persistent infections may lead to valvular seeding and the development of infective endocarditis. Though antibiotic therapy may often suffice, many patients may require surgical correction which carries a high risk of mortality. However appropriate preoperative therapy may considerably reduce the risk of surgery in maintenance hemodialysis patients.

IgG4 related renal disease: A wolf in sheep's clothing.

IgG4 related disease is a fibro-inflammatory condition with involvement of renal and extra renal organs, characterized by lymphoplasmacytic infiltration with organ dysfunction. We describe three cases of IgG4 related renal disease from a tertiary care hospital in south India.

HLA match likelihoods for Indian patients seeking unrelated donor transplantation grafts: a population-based study.

BACKGROUND: For patients who do not have a suitable human leukocyte antigen (HLA)-matched family donor, unrelated donor registries of adult volunteers and banked umbilical cord blood (UCB) units provide the potential for successful haemopoietic stem-cell transplantation. The size and genetic composition of such registries determines the proportion of patients who will be able to find a suitable match. We aimed to assess the proportion of positive matches for Indian patients. METHODS: Using HLA data from ten existing donor and UCB registries and clinical transplant centres in India, we built population-based genetic models for 14 Indian regions to model Indian registry growth to predict the likelihood of identifying a suitable donor-either an adult donor or UCB-for Indian patients. We computed ranking tables of the top ten haplotypes in each regional group and compared these with four US samples from the National Marrow Donor Program (NMDP) registry. FINDINGS: The mean proportion of individuals who would have a 10/10 adult donor match within India ranged from 14·4% with a registry size of 25 000 to 60·6% with a registry size of 1 000 000. Only when donor registries increased to 250 000 did the match rate within India exceed that found by searching the US-NMDP registry combined with an Indian registry of 25 000 donors. The proportion of matches increased logarithmically with increased registry size (R(2)=0·993). For a UCB registry size of 25 000, 96·4% of individuals would find a 4/6 match; however, only 18·3% would have a 6/6 match. INTERPRETATION: Serial match modelling and follow-up comparisons can identify the relative and progressively greater value of an India-based donor registry and UCB banking network to serve the Indian population. Understanding regional HLA haplotype diversity could guide registry growth and maximise benefit to patients. Similar modelling could guide planning for the needs of other ethnically distinct populations. FUNDING: University of Minnesota and the Indian Council for Medical Research.

Sunitinib induced nephrotic syndrome and thrombotic microangiopathy.

Sunitinib is an oral, multitargeted receptor tyrosine kinase inhibitor of targets such as vascular endothelial growth factor and platelet derived growth factor receptor. It is used for the treatment of metastatic renal cell carcinoma (RCC). Use of sunitinib has been associated with renal dysfunction and nephrotic syndrome. However, simultaneous occurrence of nephrotic syndrome and renal dysfunction in a patient treated with sunitinib is rare. We report a case of metastatic RCC treated with sunitinib for 22 months who presented with nephrotic syndrome and renal dysfunction. Renal biopsy was diagnostic of thrombotic microangiopathy with diffuse effacement of podocytic foot process.