Diaphragmatic palsy in a 10-month-old boy with infantile tremor syndrome causing respiratory failure with full response to vitamin B12 therapy.

Infantile tremor syndrome (ITS) owing to vitamin B12 deficiency usually presents with tremors, anaemia, pigmentary skin changes, neuro-regression and hypotonia. A 10-month-old boy with ITS and respiratory failure owing to bilateral diaphragmatic palsy who responded to high parenteral doses of vitamin B12 is presented. As far as we are aware, this is the first report of diaphragmatic palsy associated with ITS and vitamin B12 deficiency.

Role of Intrapleural Urokinase in Empyema Thoracis.

OBJECTIVE: To study the role of fibrinolytic therapy in pediatric empyema in relation to duration of hospital stay, need for surgical intervention and survival to discharge. METHODS: Retrospective analysis of case records of children <16 y of age admitted in a tertiary care hospital during January 2013 - December 2017 with diagnosis as empyema thoracis was done. Clinico-laboratory characteristics and the primary and secondary outcomes between the group which received intrapleural urokinase (IPU) and the group which did not (non IPU), were compared. RESULTS: Of the 84 cases, 40 children received IPU. Mean duration of hospital stay in IPU group (17.51 + 4.57 d) was significantly less than non IPU group (24.32 + 10.18 d, CI -10.19 to -3.64, p < 0.001), so was the duration of intercostal drain (ICD) insertion (9.08 + 3.12 d - IPU group vs. 11.20 + 3.95 d - non IPU group, CI -3.68 to -0.50, p < 0.01). No statistically significant difference was found between the groups with regard to need for surgical intervention [IPU - 4 (10%), non IPU - 9 (20.4%), p = 0.23]. There was no mortality or adverse reaction to urokinase in either group. CONCLUSIONS: IPU holds promising results in terms of reduction of hospital stay and duration of ICD insertion. It may be the initial choice of treatment in septated empyema where surgical options are not easily available or cost-effective especially in resource limited settings.

Acute lymphoblastic leukemia presenting as nephromegaly in a child: A rare case report.

Arora SK, Swarnim S, Hemal A, Bidhuri N. Acute lymphoblastic leukemia presenting as nephromegaly in a child: A rare case report. Turk J Pediatr 2019; 61: 97-101. Acute leukemia is the commonest pediatric malignancy with acute lymphoblastic leukemia (ALL) constituting about 75% of all leukemias. ALL commonly presents as fever, bleeding, bone pains, anemia, lymphadenopathy and hepatosplenomegaly. The liver, spleen or lymph nodes are the most common sites of extramedullary involvement in ALL, with renal involvement being relatively uncommon. The usual differential diagnosis of acquired massive bilateral nephromegaly in children includes pyelonephritis, obstructive uropathy, infections such as HIV nephropathy, mucormycosis, glycogen storage diseases, myelofibrosis with extramedullary hematopoiesis, kidney tumors and rarely hematological malignancies like ALL. Here we report a 2 years old child who presented with abdominal distention, low grade fever and constipation. Clinical examination revealed massive bilateral nephromegaly. Preliminary investigations showed severe anemia and slightly elevated WBC counts with presence of reactive changes in lymphocytes along with few atypical cells (9%). Abdominal ultrasonography revealed bilaterally enlarged kidneys which was later confirmed by CT abdomen. He was eventually diagnosed with CALLA positive B cell ALL for which treatment was started in accordance with the International network for cancer treatment and research (INCTR) protocol 02 04. At present, he is on maintenance phase and responding well to the treatment with regression of kidney size to normal on follow up ultrasonogram. Thus, leukemia should be considered in a child presenting with bilateral nephromegaly after exclusion of above mentioned differential diagnosis. Bone marrow aspiration must be done before doing a more invasive investigation like renal biopsy.