Role of Pediatricians in Early Childhood Nurturing Care Related to Safety and Security.

The five components of nurturing care for early childhood development (good health, adequate nutrition, responsive parenting, early opportunities for learning, and safety and security) are essential for the attainment of the optimal developmental potential of a child, and enabling him/her to become a productive adult. Safety is a state of protection from hazards caused by natural forces or unintentional human error, whereas security comprises of protection from hazards resulting from deliberate, harmful actions or inimical behavior. Unlike the other components, safety and security have been least addressed by health programs in India. The forms of lapses in safety and security in young children include injuries, neglect and maltreatment (physical, emotional, sexual abuse), the magnitude of which is difficult to ascertain in the community. Many mishaps can be avoided by simply understanding child development, taking precautions, and environmental modification. Issues related to safety and security are usually not addressed in routine office practice. Pediatricians have multiple roles and responsibilities. They need to utilize every opportunity to discuss preventive and promotive health care with parents. Converting immunization days to holistic well child visits is an ideal strategy, in which parents can be educated about preventing avoidable injuries and informed about how to keep their children safe and secure. Pediatricians should recognize indicators of volitional injury, neglect and abuse, and be competent in their management. They need to be aware of child rights and the legal protective measures as well as their own their legal obligations. They should network with various agencies involved in child welfare and protection.

Children at Work, Child Labor and Modern Slavery in India: An Overview.

There is a large child work force in India reported to be about 40 million. Child labor is being regarded as a form of modern slavery, as children are forced to work or have no choice to refuse work. Children are employed in a variety of occupations, many of which are hazardous. Exposure to machinery, pesticides, dust in agricultural work and fumes, chemicals, acids, cotton and wool fiber in other forms of work is detrimental to health. A large number are held in bonded servitude. In urban areas, children are employed as domestic helpers and engaged in eateries and auto-repair work. Trafficking and trading of children for work and sexual slavery are also major concerns. Poverty and illiteracy are root causes of child labor, but iniquitous societal attitudes are responsible for abuse and exploitation. Working children are deprived of proper health care and education, and lose their childhood and dignity. Several legal measures exist to prevent child labor and protect them from harm, but are thwarted by the distressing socioeconomic conditions. Although child labor would be difficult to abolish, exploitation can be prevented with concerted efforts of the government agencies, professional bodies and the civil society.

Ureterocele in newborns, infants and children: Ten year prospective study with primary endoscopic deroofing and double J (DJ) stenting.

AIM: To evaluate the success of endoscopic deroofing with double J (DJ) stenting as a primary treatment modality of uncomplicated ureterocele. PATIENTS AND METHODS: All babies with uncomplicated ureterocele referred to us between 2005 and 2015 were to be prospectively recruited into the study without any exception. The preoperative management would include ultrasound KUB on a fixed protocol, micturating cystourethrogram, DTPA scan and MRU (magnetic resonance urography) or IVP (intravenous pyelography). Those who had non-obstructing ureterocele were to be followed conservatively. The indications for operative intervention were to be obstructed ureterocele, bladder outlet obstruction, recurrent UTI or progressive renal scarring. They were then to be taken for cystoscopy in diuretic phase for endoscopic deroofing and DJ stenting with 9F resectoscope. Four weeks later, the DJ stent was to be removed after examining the deroofing in each patient. They were then followed up for future development of symptoms and need for secondary surgery with anatomical and functional imaging as necessary. RESULTS: Forty three consecutive patients (47 ureterocele units) with ureterocele were recruited into the study between 2005 and 2015. Thirty four patients were managed by endoscopic first approach with ureterocele deroofing and DJ stenting. Four babies were asymptomatic and did not show obstructed drainage pattern on DTPA and hence were managed conservatively and kept on follow-up. Four patients underwent primary open surgery and reconstruction because of presentation with complications. ECTOPIC URETEROCELE: There were 17 patients with ectopic ureterocele. Thirteen underwent primary endoscopic deroofing. Three underwent open surgery and one underwent robotic bilateral ureterocele excision and ureteric reimplantation. Eight children out of thirteen are completely asymptomatic after the endoscopic incision. On ultrasound evaluation 8/13 have resolution of the hydronephrosis and 5/13 have stable hydronephrosis with no increase in dilatation. None of these 13 children have required a second endoscopic procedure for inadequate decompression of the ureterocele. None of the 13 patients have any voiding difficulty or incontinence. 2/13 cases with recurrent UTI and persistent symptoms, required secondary surgery. This constituted 15% secondary surgery in the ectopic ureterocele category. ORTHOTOPIC URETEROCELE: There were 21 patients out of 22 with orthotopic ureterocele who underwent endoscopic deroofing. Six out of these 21 patients had episodes of UTI, of which three had resolution of the reflux and two had downgrading of reflux. One child continued to have recurrent UTI and needed a laparoscopic nephroureterectomy. Eighteen children are completely asymptomatic after the initial endoscopic deroofing. The rate of secondary surgery was 4.7% in orthotopic ureterocele group, where nearly 50% have more than five years follow-up. None of our patients who are more than five years, have had any urinary incontinence or voiding dysfunction. All patients remain on follow-up from 6months to 10years (median 5years). CONCLUSION: At this stage of our study with median follow-up of 5years (6months-10years), we suggest that primary endoscopic deroofing with DJ stenting for obstructing ureterocele is the best initial approach for adequate decompression and reducing the rate of secondary surgery.

Management of complicated ureteroceles: Different modalities of treatment and long-term outcome.

The presentation and management of ureterocele has been rarely reported from India and is limited to an odd case report. They can be detected antenatally, may have incidental diagnosis or present with consequences of obstructive uropathy. They always present with secondary complications in adulthood, if the diagnosis and treatment is missed in early years of life. The natural history may be particularly disastrous in cases of bilateral obstructing ureterocele. The complications secondary to obstructive ureterocele can be urinary retention, pyonephrosis, urosepsis, stones and even chronic renal failure. Each of these patients needs an individualized treatment plan. In the period 2003-2013, we managed 36 consecutive patients with varied presentation of this entity. All were managed on the basis of a fixed protocol of investigation and operative intervention. Ultrasound, micturating cystourethrogram, isotope renogram were done preoperatively in all the babies. Those with asymptomatic, unobstructed ureteroceles were left without any intervention. Simple or complex intravesical ureteroceles underwent examination under anesthesia and endoscopic deroofing and DJ stenting for 3 weeks. Of a total of 36 children, 6 were presented with acute complications of ureterocele. They have been managed on an individualized optimum management plan. Their management approach with follow-up is being reported as there is no previous reported series on ureterocele in children from our country.

Robotic augmentation ileocystoplasty with bilateral ureteric reimplantation in a young child with neuropathic bladder.

Neuropathic bladder in children is most commonly secondary to spina bifida. The management starts early in life. The modalities of treatment vary depending on the severity of the symptoms. A proportion of children inspite of adequate medical management need augmentation ileocystoplasty later in life. The open surgery has proven safety and success over many decades. Earlier attempts to perform augmentation cystoplasty by the laparoscopic approach were limited by steep learning curve, long operating times, and technical difficulties in intracorporeal anastomosis. The emergence of robotic technology has revived the interest in minimally invasive approach for complex pediatric urological reconstructions. In the recent times, there has been only one reported case report and small series of pediatric robotic augmentation cystoplasty from Chicago. We report the first minimally invasive robotic reconstruction in a child with neuropathic bladder and early renal decompensation despite appropriate medical treatment, from our country.

Indiana pouch in children: a 15-year experience.

OBJECTIVE: We report our experience with the Indiana pouch (continent urinary reservoir) in 12 consecutive children over the last 15 years and report their follow-up. MATERIAL AND METHODS: Twelve consecutive children, who underwent the continent urinary reservoir procedure in the form of an Indiana pouch, were prospectively enrolled in the study. All consecutive children who were referred to our service with multiple failed surgeries for exstrophy-epispadias repair, cloacal exstrophy, genitourinary rhabdomyosarcoma with residual disease in the trigonal area not amenable to partial cystectomy, and neuropathic bladder with nephrogenic metaplasia were included over the period 1997-2012. All these children were offered the same form of bladder substitution (Indiana pouch) as described by the Indiana group many years ago. Postoperative care was on a fixed protocol, and follow-up details recorded over the years. They were followed up for dry interval with clean intermittent catheterisation, social acceptance, and early and late complications. RESULTS: Out of these 12 patients (7 males and 5 females), eight patients had exstrophy-epispadias with multiple failed operations carried out elsewhere, cloacal exstrophy (2), residual rhabdomyosarcoma in the trigonal area with incontinence following chemotherapy (1), and neuropathic bladder with recurrent diffuse neoplastic polyposis (1). In the follow-up period of 1-15 years (median 24 months) all the patients had a dry interval of 4 h or more with clean intermittent catheterisation. One patient had wound dehiscence during the postoperative period and another required stomal revision 1 year after initial surgery. CONCLUSIONS: The Indiana pouch is a reliable, safe, and effective form of bladder substitution. It can be reconstructed in a wide range of lower urinary tract disorders. In the vast majority of children with multiple failed surgical procedures for exstrophy-epispadias, the Indiana pouch is a safe, reliable, and reproducible procedure to provide a socially acceptable quality of life with a dry interval of 4 h.

Physiological and functional evaluation of the transposed human pylorus as a distal sphincter.

BACKGROUND/AIMS: Studies evaluating the human pylorus as a sphincter are scanty and contradictory. Recently, we have shown technical feasibility of transposing the human pylorus for end-stage fecal incontinence. This unique cohort of patients provided us an opportunity to study the sphincter properties of the pylorus in its ectopic position. METHODS: Antro-pylorus transposition on end sigmoid colostomies (n = 3) and in the perineum (n = 15) was performed for various indications. Antro-pylorus was assessed functionally (digital examination, high resolution spatiotemporal manometry, barium retention studies and colonoscopy) and by imaging (doppler ultrasound, MRI and CT angiography) in its ectopic position. RESULTS: The median resting pressure of pylorus on colostomy was 30 mmHg (range 28-38). In benign group, median resting pressure in perineum was 12.5 mmHg (range 6-44) that increased to 21.5 mmHg (range 12-29) (P = 0.481) and 31 mmHg (range 16-77) (P = 0.034) on first and second follow-up, respectively. In malignant group, median post-operative pressures were 20 mmHg (range 14-36) and 21 mmHg (range 18-44) on first and second follow-up, respectively. A definite tone and gripping sensation were felt in all the patients on digital examination. On distal loopogram, performed through the diverting colostomies, barium was retained proximal to the neo-pyloric valve. Both perineal ultrasound and MRI showed viable transposed graft. CT angiography and color doppler studies confirmed vascular flow in the transposed position. CONCLUSIONS: The human pyloric valve can function as a tonic sphincter when removed from the gastroduodenal continuity.

Disease course in steroid sensitive nephrotic syndrome.

OBJECTIVE: To review the disease course in patients with steroid sensitive nephrotic syndrome (SSNS) and the factors that determine outcome DESIGN: Retrospective, analytical SETTING: Pediatric Nephrology Clinic at referral center in North India PARTICIPANTS/PATIENTS: All patients with SSNS evaluated between 1990 and 2005 INTERVENTION: None MAIN OUTCOME MEASURES: Disease course, in patients with at least 1-yr follow up, was categorized as none or infrequent relapses (IFR), frequent relapses or steroid dependence (FR), and late resistance. Details on complications and therapy with alternative agents were recorded. RESULTS: Records of 2603 patients (74.8% boys) were reviewed. The mean age at onset of illness and at evaluation was 49.7±34.6 and 67.5±37.9 months respectively. The disease course at 1-yr (n=1071) was categorized as IFR in 37.4%, FR in 56.8% and late resistance in 5.9%. During follow up, 224 patients had 249 episodes of serious infections. Alternative medications for frequent relapses (n=501; 46.8%) were chiefly cyclophosphamide and levamisole. Compared to IFR, patients with FR were younger (54.9±36.0 vs. 43.3±31.4 months), fewer had received adequate (=8 weeks) initial treatment (86.8% vs. 81.7%) and had shorter initial remission (7.5±8.6 vs. 3.1±4.8 months) (all P<0.001). At follow up of 56.0±42.6 months, 77.3% patients were in remission or had IFR, and 17.3% had FR. CONCLUSIONS: A high proportion of patients with SSNS show frequent relapses, risk factors for which were an early age at onset, inadequate initial therapy and an early relapse.

Efficacy and safety of treatment with rituximab for difficult steroid-resistant and -dependent nephrotic syndrome: multicentric report.

BACKGROUND AND OBJECTIVES: The treatment of idiopathic nephrotic syndrome is often complicated by a refractory and relapsing course, with risk of drug toxicity and progressive renal failure. We report the efficacy and safety of rituximab in patients with steroid-resistant (SRNS) and steroid-dependent nephrotic syndrome (SDNS) refractory to standard therapy. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This was a cohort study in academic, tertiary care centers in India and the United States. Patients with SRNS or SDNS, not responding to medications or showing calcineurin inhibitor toxicity, treated with two to four doses of intravenous rituximab, and followed ≥12 months were included. Remission was termed as complete, partial, or no response. RESULTS: Thirty-three patients with SRNS (24 initial, 9 late resistance) and 24 with SDNS, with mean ages of 12.7 ± 9.1 and 11.7 ± 2.9 years, respectively, were included. Six months after rituximab therapy, 9 (27.2%) patients with SRNS showed complete remission, 7 (21.2%) had partial remission, and 17 (51.5%) had no response. At 21.5 ± 11.5 months, remission was sustained in 15 (complete: 7, partial: 8) patients. Of 24 patients with SDNS, remission was sustained in 20 (83.3%) at 12 months and in 17 (71%) at follow-up of 16.8 ± 5.9 months. The mean difference in relapses before and 12 months after treatment with rituximab was 3.9 episodes/patient per year. CONCLUSIONS: Therapy with rituximab was safe and effective in inducing and maintaining remission in a significant proportion of patients with difficult SRNS and SDNS.

Intravenous cyclophosphamide in steroid-resistant nephrotic syndrome.

We prospectively examined the effect of treatment with intravenous cyclophosphamide in patients with steroid-resistant nephrotic syndrome. Twenty-four patients (minimal change disease in 11, focal segmental glomerulosclerosis in 9, and mesangioproliferative glomerulonephritis in 4), who did not show remission of proteinuria despite treatment with 8 weeks of oral prednisolone and six intravenous pulses of dexamethasone, were studied. Cyclophosphamide was administered intravenously, at a dose of 750 mg/m(2) once a month for 6 months; therapy with alternate-day prednisolone was continued. The mean (SD) age at treatment was 7.8 (4.0) years; 18 patients had initial resistance and 6 had late resistance. At the end of 6 months treatment, 7 (29.2%) patients each had complete remission (absent proteinuria, normal serum albumin) and partial remission (1-2+ proteinuria, normal serum albumin). Ten (41.6%) patients showed no response to therapy. The mean time to complete or partial remission, after initiation of treatment with cyclophosphamide, was 2.4+/-1.7 months and 2.7+/-1.8 months, respectively. Most responders (85.8% complete and 57.2% partial responders) achieved remission by the third dose of pulse cyclophosphamide. More patients with late resistance (50%) compared with initial resistance (22.2%) achieved complete remission. Partial remission was transient and lasted for a mean duration of 6.4+/-3.5 months. Serious infections were observed during therapy in 5 patients. On long-term follow-up, 5 (20.8%) patients were in remission, while nephrotic-range proteinuria or end-stage renal disease was seen in 17 (70.8%). Findings from the present study suggest that therapy with intravenous cyclophosphamide has limited efficacy in inducing sustained remission in patients with initial corticosteroid resistance. Sustained remission is likely to occur in a significant proportion of patients with late resistance and those with absence of significant tubulointerstitial changes on renal histology.