The Expert Group emphasized that while all patients with SRNS should initially be referred to a pediatric nephrologist for evaluation, the subsequent care might be collaborative involving the primary pediatrician and the nephrologist. Following the diagnosis of SRNS (lack of remission despite treatment with prednisolone at 2 mg/kg/day for 4 weeks), all patients (with initial or late resistance) should undergo a renal biopsy, before instituting specific treatment. Patients with idiopathic SRNS secondary to minimal change disease or focal segmental glomerulosclerosis should receive similar therapy. Effective regimens include treatment with calcineurin inhibitors (tacrolimus, cyclosporine), intra-venous cyclophosphamide or a combination of pulse corticosteroids with oral cyclophosphamide, and tapering doses of alternate day corticosteroids. Supportive management comprises of, when indicated, therapy with angiotensin converting enzyme inhibitors and statins. It is expected that these guidelines shall enable standardization of care for patients with SRNS in the country.
Intestinal Neuronal Dysplasia (IND) is a
motility disorder first described in 1971 by Meier-Ruge associated to
histochemical changes of the bowel wall (hyperplasia of submucous &
myenteric plexus with giant ganglia formation, isolated ganglion
cells in lamina propia and muscularis mucosa, elevation of
in parasympathetic fiber of lamina propia and circular muscle, and
plexus sympathetic hypoplastic innervation), also known as
associated to elevated acetylcholinesterase parasympathetic staining.
condition can occur in an isolated form (either localized to colon or
throughout the bowel), or associated to other diseases such as
(HD), neurofibromatosis, MEN type IIB, and anorectal malformations. It
is estimated that 20-75% of HD cases have IND changes proximal to the
segment. Clinically two different types of isolated IND have been
Type A shows symptoms of abdominal distension, enterocolitis, bloody
intestinal spasticity in imaging studies (Ba Enema) since birth, is
common and associated with hypoplasia of sympathetic nerves. Type B is
more frequent, symptoms are indistinguishable from that of HD, with
constipation, megacolon, and repeated episodes of bowel obstruction.
depends on clinical situation; conservative for minor symptoms until
maturation occurs around the 4th year of life, colostomy and
therapy for life threatening situations.