Clinical Spectrum of Congenital Anomalies of Kidney and Urinary Tract in Children

Objective: To evaluate the clinical spectrum and patterns of clinical presentation incongenital anomalies of kidney and urinary tract. Methods: We enrolled 307 consecutivelypresenting children with congenital anomalies of kidney and urinary tract at the pediatricnephrology clinic. Patients were evaluated clinically, with serum biochemistry, appropriateimaging and radionuclide scans. Results: The most common anomaly was primaryvesicoureteric reflux (VUR) (87, 27.3%), followed by pelviureteral junction obstruction(PUJO) (62,20.1%), multicystic dysplastic kidney (51 16.6%), non-obstructivehydronephrosis (32, 10.4%) and posterior urethral valves (PUV) (23, 7.4%). 247 (80.4%)anomalies had been identified during the antenatal period. Another 33 (10.7%) werediagnosed during evaluation of urinary tract infection, and 21 (6.8%) during evaluation forhypertension at presentation. Obstructive anomalies presented earlier than non-obstructive(7 (3, 22.5) vs 10 (4, 24) mo: (P=0.01)). The median (IQR) ages of presentation for childrenwith PUV (n=23), VUR (n=87) and PUJO (n=62) were 4 (2, 14) mo, 10 (5, 27) mo, and 7 (3,22.5) mo, respectively. Nine (2.9%) children had extrarenal manifestations. Conclusions:The median age at clinical presentation for various subgroups of anomalies indicatesdelayed referral. We emphasize the need for prompt referral in order to initiate appropriatetherapeutic strategies in children with congenital anomalies of kidney and urinary tract

First degree heart block with multi-focal atrial ectopics in an adolescent girl due to hypomagnesemia

Nutritional deficiencies are common in adolescent children and include deficiencies of both micro- and macronutrients. Magnesium is an important mineral that is essential for maintenance of numerous electrophysiological and biochemical processes in the body. We report an adolescent girl who developed an episode of syncope with first degree heart block on electrocardiography and run of multifocal atrial ectopics on 24 h holter monitoring. Serum magnesium was found to be low with decreased urinary magnesium excretion. There were no other electrolyte abnormalities. Structural heart disease was ruled out by a normal echocardiogram. The rhythm changes were attributable to nutritional hypomagnesemia and were promptly reversed on correcting the hypomagnesemia

A new syndrome with overlapping features of Townes-brocks syndrome and single median maxillary central incisor syndrome.

A 14-month-old boy with overlapping features of Townes-Brocks syndrome (TBS) and single median maxillary incisor syndrome (SMMCIS) is being reported with brief review of the above syndromes and possible differential diagnosis.