Childhood Idiopathic Steroid Resistant Nephrotic Syndrome, Different Drugs And Outcome.

Background: The management of steroid resistant nephrotic syndrome (SRNS) is quite difficult in paediatric patients. Not only the remission is difficult but also these patients are at risk of progression to end stage renal disease (ESRD). The goal of treatment is either to achieve complete remission or even partial remission as it is the most important predictor of disease outcome.

Hernia of umbilical cord: report of three unusual cases.

Congenital hernia of umbilical cord is a less frequent entity in newborns and occasionally associated with other maladies. Herein, we report three unusual cases of hernia of umbilical cord. First case was associated with in-utero evisceration of entire small bowel through the presumably ruptured hernia of umbilical cord and other two cases had associated patent vitellointestinal duct (PVID).

Iatrogenic Cushing's syndrome in children presenting at Children's Hospital Lahore using nappy rash ointments.

Objective: To study the characteristics of infants and children presenting with iatrogenic Cushing's Syndrome due to nappy rash ointments.

Methods: The descriptive study was conducted at the Children's Hospital, Lahore, from April to September 2013, and comprised patients presenting with cushingoid features and history of using nappy rash ointments. Patients having Cushing's Syndrome due to causes other than iatrogenic were excluded and so were those taking oral or parenteral steroids due to skin allergy, renal or respiratory disease.

Displacement of Small bowel into the Lesser Sac in a Case of Complete Congenital Pouch Colon.

Postoperative neonatal intestinal obstruction has a myriad of etiology. An operated case of imperforate anus developed intestinal obstruction early postoperatively. At re-operation, missed complete congenital pouch colon with a small perforation and displacement of small bowel into the lesser sac were found.

Simultaneous giant hydatid cysts of brain and liver.

While hepatic hydatid cysts are most common in occurrence, intracranial hydatid cyst is less common. Simultaneous presence of both varieties is quite rare and poses a challenge for management in terms of involvement of multidisciplinary team and multiple interventions. An 8-year boy presented with neurological symptoms of a space occupying lesion.

Multiple singing magnet ingestion leading to pressure necrosis of the small bowel.

Multiple Magnet ingestion may cause a number of sinister complications. We report a case of multiple singing magnets ingestion by a 2-year-old girl resulting in intestinal obstruction and pressure necrosis of the small bowel. A bunch of seven magnets present in the small bowel was removed operatively.

Transorbital penetrating brain injury to frontal lobe by a wheel spoke.

Penetrating brain injury (PBI) is rare and the severest form of head injury with a high morbidity and mortality. A 3.5-year-old girl presented with PBI with a wheel spoke.

Congenital amegakaryocytic thrombocytopenia with multiple physical anomalies in a female neonate.

Congenital Amegakaryocytic Thrombocytopenia (CAMT) is a rare disorder of infancy characterized by isolated thrombocytopenia along with hypoplasia or aplasia of megakaryocytes in the bone marrow. It is caused by c-mpl mutation which disrupts the function of thrombopoietin (TPO) receptor. CAMT in association with physical anomalies is a rare entity with only limited data from single case reports being available.

Appendicular Band Syndrome simulating Appendicular Mass in a Child.

Appendicular band syndrome is an exceedingly rare surgical emergency that may lead to intestinal obstruction and strangulation. We report a case of 2-year-old boy who presented with acute intestinal obstruction with a mass in right iliac fossa (RIF). At exploration, an inflamed appendix had entrapped a loop of terminal ileum leading to its strangulation and gangrene.

Our experience with unusual gastrointestinal tract duplications in infants.

Background: Classical duplications may present along any part of gastrointestinal tract (GIT) from mouth to anus. Atypical or unusual rare varieties of GIT duplications may also occur, but with different anatomical features.

Materials And Methods: We reviewed our 5-year record (February 2008-January 2013) to describe clinical profile of unusual GIT duplications in neonates and small infants.

Colovaginoplasty in a case of mayer-rokitansky-kuster-hauser syndrome.

Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKHS) is characterized by various abnormalities of paramesonephric duct structures; vaginal aplasia being the commonest anomaly in the spectrum. We report a 17-year-old girl; a case of MRKHS with vaginal agenesis. The cervix was present but atretic; uterus, fallopian tubes and ovaries were normal.

Antenatally diagnosed wilms' tumour.

Wilms' tumour (WT) is seldom seen in a neonate and prenatal diagnosis is rare. We present a case of antenatally diagnosed left sided WT with features of hydrops foetalis in a girl baby. Emergency LSCS was done at 34 weeks of gestation for foetal distress.

Congenital hairy polyp of posterior tonsillar pillar.

Congenital hairy polyps are exceedingly rare congenital anomalies. We report a case of congenital hairy polyp arising from posterior tonsillar pillar which was excised with bipolar cautry.

Etiology and genetic factors in clefts of lip and/or palate reported at children's hospital, Lahore, Pakistan.

The etiology of cleft lip (CL) and/or cleft palate (CP) has been extensively studied in industrialized countries and is suggested to be heterogeneous with increasing evidence that both genetic and environmental factors are operating. To evaluate this assertion in a developing country like Pakistan, a case finding cross-sectional study was completed from 1(st) July 2010 to 31(st) May 2011 for 100 cases of CL and/or CP referred to the Genetic Clinic of the Children's Hospital, Lahore, Pakistan. A clinical examination followed by necessary diagnostic work-up was completed for each case.