Pediatric PseudoTumoral Hepatic Tuberculosis. A Great Mimicker!!

Pediatric liver masses are rare and difficult to treat. Common liver masses in children include hepatoblastoma, hemangiomas, liver abscesses, and hydatid disease. Isolated liver tuberculosis (TB) is rare in children and can have variable clinical presentations. We report a child with isolated liver TB masquerading as a liver tumor.

ANCA-associated Vasculitis Presenting as Severe Pulmonary Hypertension and Right Heart Failure.

Microscopic polyangiitis (MPA) is a small vessel multisystemic disorder characterised by necrotising small vessel vasculitis without any immune deposits. Kidney and lung are the predominant organs affected in MPA. Skin, gastrointestinal and neurological findings are also described. Isolated pulmonary manifestations are rare. The authors describe a two-year girl who presented with right heart failure and was subsequently diagnosed as ANCA-associated vasculitis. This case report is intended to sensitise pediatricians to consider systemic vasculitis with pulmonary hemorrhage in children with pulmonary hypertension even in the absence of severe pallor.

Effect of BMI and urinary pH on urolithiasis and its composition.

Urolithiasis is a common urological disease predominantly affecting males. The lifetime risk of urolithiasis varies from 1% to 5% in Asia, 5% to 9% in Europe, 10% to 15% in the USA and 20% to 25% in the middle-east; lowest prevalence is reported from Greenland and Japan. Such differences have been explained on the basis of race, diet and climate factors. Furthermore, changing socio-economic conditions have generated changes in the prevalence, incidence and distribution for age, sex and type of lithiasis in terms of both the site and the chemical as well as the physical composition of the calculi. The aim of our study was to determine the association between body mass index (BMI) and urine pH in patients with urolithiasis and the influence of body size, as reflected by the BMI, on the composition. The study was conducted in the Department of Biochemistry, Maharishi Markandeshwar Institute of Medical Sciences and Research, on urolithiatic patients. The data included patient's age, sex, BMI, urine pH, serum calcium, serum uric acid, serum creatinine and stone composition. Data from 100 patients, 70 men (70%) and 30 women (30%), were analyzed, with 28 patients having normal weight, 38 patients being overweight and 34 patients being obese. The mean age of the patients was 36.58 ± 9.91 years in group I, 40.47 ± 14.48 years in group II and 37.85 ± 12.46 years in group III (P > 0.05). The stone composition was calcium oxalate (CaOx) in 66 patients, calcium phosphate (CaP) in 60 patients, uric acid (UA) in 38 patients, combined calcium oxalate and calcium phosphate in 28 patients and three stones in 10 patients. The urinary pH levels (mean ± SD) were 7.78 ± 1.49 in group I, 7.15 ± 1.11 in group II and 6.29 ± 1.14 in group III patients (P = 0.0001). Urine pH showed a stepwise decrease with increasing BMI (inverse correlation). Urine pH is inversely related to BMI among patients with urolithiasis, as is the occurrence of urate, calcium oxalate and calcium phosphate stones. Similarly, the serum creatinine increased as the BMI and number of stones increased among the study population.

Bell palsy in a neonate with rapid response to oral corticosteroids: a case report.

Idiopathic facial nerve palsy, also known as Bell palsy is rare in the neonatal age group. Other more common causes such as birth trauma; infections, especially otitis media; and congenital malformations need to be excluded. We present here a 4-week-old neonate with Bell palsy who responded rapidly to oral corticosteroids. Such an early presentation of idiopathic facial nerve palsy and use of corticosteroids in neonates is scarcely reported in the literature.

Fatal bronchiolitis obliterans complicating Stevens-Johnson syndrome following treatment with nimesulide: a case report.

Nimesulide is a frequently used non-steroidal anti-inflammatory drug with analgesic and antipyretic effects in children. In view of fatal adverse drug reactions, however, its safety has been questioned. A 5-year-old boy developed Stevens-Johnson syndrome following use of nimesulide which was later complicated by rapidly progressive fatal bronchiolitis obliterans.

Posterior urethral valves: Morphological normalization of posterior urethra after fulguration is a significant factor in prognosis.

AIM: To assess the changes in urethral morphology 3 months post fulguration of posterior urethral valves (PUVs) on micturating cystourethrogram (MCUG) and correlate these changes with the overall clinical status of the patient. MATERIALS AND METHODS: A total of 217 children, managed for PUVs during a period of 6 years in a single surgical unit were prospectively studied. The ratio of the diameters of the prostatic and bulbar urethras (PU/BU) was calculated on the pre- and post-fulguration MCUG films. They were categorized into three groups based on the degree of normalization of posterior urethra (post-fulguration PU/BU ratio). GROUP A: Of the 133 patients, 131 had normal urinary stream and 4 (3%) had nocturnal enuresis. Vesicoureteral reflux (VUR), initially seen in 83 units (31% units), regressed completely at a mean duration of 6 months in 41 units (49%). Of the 152 non-VUR, hydroureteronephrosis (HUN) units, 11 were poorly functioning kidneys. Persistent slow but unobstructed drainage was seen in 23 units (16%) over a period of 1.5-5 years (mean 2.5 years). Group B: All the 11 patients had a normal stream. Four (36.4%) had daytime frequency for a mean duration of 1 year and one (9%) had nocturnal enuresis for 1 year. Grade IV-V VUR was seen in five patients (three bilateral), which regressed completely by 3 months in five units (62.5%). In the non-VUR, HUN patients, slow (but unobstructed) drainage was persistent in two units (14%) at 3 years. Group C: Of the 16 patients, only 5 (31.3%) were asymptomatic. Six patients (nine units) had persistent VUR for 6 months to 3 years. Of the 20 units with HUN, 17 (85%) were persistent at 1-4 years (mean 2 years). Eight patients (50%) required a second fulguration while 3 (18.7%) required urethral dilatation for stricture following which all parameters improved. CONCLUSIONS: Adequacy of fulguration should be assessed by a properly performed MCUG. A postop PU/BU ratio >3 SD (1.92) should alert to an incomplete fulguration or stricture. Patients within normal range ratio have faster recovery of slow draining units, reflux and less voiding dysfunction. There is a strong correlation between incomplete fulguration and persistent slow draining units, uremia, voiding dysfunction and urinary tract infections.

Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate.

Congenital intrahepatic portosystemic venous shunt (IHPSVS) is rare vascular anomaly. We present one case of a 14-month male child who presented with global developmental delay. Child had high ammonia levels with low glutamine and high bile salts on the previous investigations and had history of neonatal seizures since day 13 of life. On admission, serum ammonia levels were elevated to 112micromol/L. Other laboratory investigations including liver and renal function test, and electrolytes were normal. He was, diagnosed to have IHPSVS on the basis of Doppler and CT, and treated by embolization with n-butyl cyanoacrylate (glue). A brief review of diagnostic modalities and endovascular management for the IHPSVS is presented including the present case.

Marfanoid hypermobility syndrome and skeletal abnormalities in a rare case of torsion of wandering spleen.

Wandering spleen is a rare clinical entity characterized by splenic hypermobility resulting from laxity or maldevelopment of supporting splenic ligaments. Its major complication is splenic torsion, which is a potentially fatal surgical emergency. We present a rare case of wandering spleen with torsion and splenic infarction in a patient with marfanoid hypermobility syndrome and vertebral abnormalities.

Rosai-Dorfman disease: unusual cause of diffuse and massive retroperitoneal lymphadenopathy.

Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement. This uncommon entity should also be included in the list of differential diagnoses of diffuse and massive lymphadenopathy, which includes includes infectious/granulomatous conditions, lymphomatous and metastatic disease.

Case report: bilateral multilocular cystic nephromas: a rare occurrence.

We present a rare case of bilateral renal cystic tumours in an 18-month-old female child, which proved to be cystic nephromas on imaging and histopathology. Although unilateral cystic nephromas are known in children; the occurrence of bilateral tumours is rare.

Small bowel tuberculosis: enteroclysis findings.

We describe the radiologic appearances of small bowel tuberculosis as shown by enteroclysis. A total of 265 patients with proven small bowel tuberculosis seen over a period of more than one decade was evaluated. All patients had positive radiologic findings as shown on enteroclysis examination. Of the 265 patients with small bowel tuberculosis, 174 had isolated small bowel involvement with a normal ileocecal region, whereas 91 had associated noncontiguous involvement of the ileocecal region. The most common radiologic finding was the presence of strictures, noted in 62.7% of cases. Most strictures were short, concentric, and smooth in outline. These strictures were solitary or multiple and located mainly in the jejunum. Other radiologic findings were adhesions (21.8%), ulcerations (9.1%), and diffuse thickening of folds (6.4%). Complications noted were in the form of enteroliths, perforations, and fistulae. The radiographic findings of small bowel tuberculosis, although non-specific, may indicate tuberculosis in a high-risk population.

Renal lymphangiomatosis: a rare cause of bilateral nephromegaly.

We report the ultrasound, excretory urography and MR findings in a young child with renal lymphangiomatosis who presented with gradually progressive bilateral flank swelling but who was otherwise asymptomatic. The typical perirenal and parapelvic cysts are visualized as hypoechoic lesions on sonography and hyperintense on T2-weighted HASTE images. T1-weighted image could not delineate the cysts clearly. The renal parenchyma was hyperechoic on sonography, and MRI showed reversal of the normal corticomedullary signal intensity, and confirmed the diagnosis by suggesting the non-parenchymal origin of the cysts.