Air Gun Injuries in Children - A Near Miss!

Air guns that are employed for protection and entertainment/sporting events can have accidentally harmful consequences. We report three examples of air gun injuries in the pediatric population with injuries, which could be potentially fatal.

Clinical and Genetic Spectrum of Inborn Errors of Immunity in a Tertiary Care Center in Southern India.

OBJECTIVES: To study the incidence, clinical manifestations, and genetic spectrum of primary immunodeficiency diseases (PID)/inborn errors of immunity (IEI) in a tertiary care hospital in Southern India. METHODS: A retrospective analysis of all patients with a clinical suspicion of PID/IEI seen at a tertiary care hospital was performed. All patients had at least one or more warning signs of PID. Serum immunoglobulin levels and other targeted investigations were performed as warranted by the clinical presentation. All families with suspected PID were counseled and offered genetic testing. RESULTS: A total of 225 children were evaluated for PID during the study period of 6 y. Fifty-six of them did not meet the European Society of Immunodeficiencies (ESID) criteria (working definition of clinical diagnosis) and were excluded. An IEI was found in 30/49 (61.2%) patients. The most frequent reason for referral was recurrent/unusual or serious infections (28%), or cytopenia (16%). Group IV diseases of immune dysregulation was the most common category (19%), followed by group III predominant antibody deficiencies in 23/163 (14%), as per the International Union of Immunological Societies (IUIS) classification. CONCLUSIONS: This study highlights the heterogeneity of the present cohort, the underuse of genetic tests, and efforts to provide optimal care for children with possible IEI in this center.

Cervical Thymic Cyst - A Rare Diagnosis.

Cervical thymic cysts are rare cysts which are seen in the first decade of life in males on the left side. The following is a case of a young female with a painless swelling on the right side of the neck. She was evaluated and underwent exploration of the neck and excision of the swelling.

Subacute thyroiditis with airway compromise in a 5-year-old boy.

A 5-year-old boy presented with intermittent fever for 1 month, painful neck swelling associated with dysphagia, hoarseness of voice for 3 weeks and dyspnoea of 1-day duration. On evaluation, he had elevated serum thyroglobulin levels and inflammatory markers. There was a diffuse glandular thyroid enlargement with hypoechoic areas on neck ultrasonography. Fine-needle aspiration cytology was suggestive of subacute thyroiditis (SAT), and MRI of the neck confirmed narrowing of the trachea by the enlarged thyroid. He received steroids to relieve airway compression. Levothyroxine was started. On follow-up, he was symptom-free and euthyroid; steroids and levothyroxine were discontinued. SAT presenting with compression of trachea is rare in children. This highlights the need for identifying the type of thyroiditis to determine treatment modality.

Suboptimal glycemic control among subjects with diabetes mellitus in India: a subset analysis of cross-sectional wave-7 (2016) data from the International Diabetes Management Practices Study (IDMPS).

OBJECTIVE: To assess the real-world management practices of subjects with type 2 diabetes mellitus (T2DM) and type 1 diabetes mellitus (T1DM) in India. METHODS: This cross-sectional study was conducted between 7 March 2016 and 15 May 2016 in India as part of the seventh wave (2016) of the International Diabetes Management Practices Study (IDMPS). Adult subjects with T1DM or T2DM visiting physicians during a 2-week recruitment period were included. RESULTS: A total of 55 physicians included 539 subjects who met eligibility criteria. Of 495 subjects with T2DM, 303 were treated with oral glucose lowering drugs (OGLDs) only, 158 were treated with OGLD + insulin, and 27 received insulin only. Among 44 subjects with T1DM receiving insulin, 13 (29.5%) were also treated with OGLD therapy. The most commonly used insulin regimens were basal alone (69/184; 37.5%) and premixed alone (63/184; 34.2%) in subjects with T2DM, and basal + prandial insulin (24/44; 54.5%) in subjects with T1DM. Proportions of subjects achieving glycemic targets were low [glycated haemoglobin (HbA1c) <7%: T1DM = 7.3% (3/44), T2DM = 25.2% (106/495); as targeted by the treating physician: T1DM = 31.8% (14/44), T2DM = 32.1% (59/185); global target: T1DM = 4.8% (2/42) and T2DM = 1.7% (8/482)]. In subjects with T2DM, HbA1c <7% was noted in 11/22 subjects receiving insulin only and 76/260 receiving only OGLDs. Lack of experience in self-managing insulin dosing, poor diabetes education and failure to titrate insulin dosages were the main reasons for non-achievement of glycemic targets. CONCLUSION: Timely insulinization, education and empowerment of people with diabetes may help improve glycemic control in India.

Congenital Pulmonary Airway Malformation - 19-Year Experience from a Tertiary Care Center in India.

Background: Congenital pulmonary airway malformation (CPAM) is a collection of non-hereditary, developmental anomalies. Our aim was to analyze the histological profiles and prevalence of CPAMs diagnosed in our center. Methods: A retrospective study of all CPAMs diagnosed from January 1999 to May 2018 from a general hospital pathology service was performed. Results: There were 79 cystic lesions encountered in fetuses, neonates, and children, 15 of which were CPAMs {5/2372 (0.21%) autopsies and 10/216026 (0.0046%) surgical resections}. The male:female ratio was 1:1.14. Gestational age of antenatal cases ranged from 22 to 32 weeks, postnatal ages ranged from 7 days to 15 years (mean 2.9 years). The cases were right-sided (8/15;53.3%), left-sided (4/15;26.7%) and bilateral (3/15,20%). Seven (46.7%), 4 (26.7%),3 (20%) and 1 (6.7%) were types 1, 2, 3 and 4, respectively. None of the surgical cases had postoperative mortality or morbidity. Conclusions: Prompt recognition and surgical resectability resulted in normal growth and symptom free survival in our postnatally diagnosed patients. Mortality in antenatally diagnosed fetuses remains high (5/11;45%).

A Rare Case of Fetus within a Fetus.

We present a case of a 45-day-old baby brought to our paediatric outpatient department with complaints of abdominal distension. The prenatal ultrasonographic examination showed a large cystic intrabdominal mass with internal calcifications.

Primary segmental volvulus mimicking ileal atresia.

Neonatal intestinal volvulus in the absence of malrotation is a rare occurrence and rarer still is the intestinal volvulus in absence of any other predisposing factors. Primary segmental volvulus in neonates is very rare entity, which can have catastrophic outcome if not intervened at appropriate time. We report two such cases, which were preoperatively diagnosed as ileal atresia and intraoperatively revealed to be primary segmental volvulus of the ileum.

Duodenal atresia in association with situs inversus abdominus.

We report a rare association of duodenal atresia with situs inversus abdominus in a newborn. The infantogram revealed "reverse double-bubble sign" without dextrocardia. The sonography and echocardiography confirmed the diagnosis of situs inversus abdominus with multiple cardiac anomalies. Laparotomy and a duodenoduodenostomy were carried out.

Mediastinal enteric cyst in a neonate.

Mediastinal enteric cysts are relatively uncommon, and patients tend to present at a later age compared to those with duplications in other areas of alimentary canal. The tendency of enteric cyst to enlarge and produce airway obstruction is sufficient reason for early surgical removal. We report on a case of mediastinal enteric cyst in a neonate with respiratory distress for its early presentation and management. The embryological basis and anatomical issues relating to duplication cysts of the gastrointestinal tract is discussed.

Subcutaneous phycomycosis--fungal infection mimicking a soft tissue tumor: a case report and review of literature.

Subcutaneous phycomycosis is a rare fungal infection of the deeper layers of skin. We describe a representative case. A 10-year-old boy presented with a large swelling on the back of 3 months duration. Biopsy of the lesion confirmed the diagnosis of subcutaneous phycomycosis. There was complete resolution of the lesion after treatment with oral potassium iodide for 3 months.