Clinical correlates to the goniodysgensis among juvenile-onset primary open-angle glaucoma patients

Objectives

To evaluate gonioscopic features and relate them to clinical characteristics in eyes with juvenile-onset primary open-angle glaucoma (JOAG).

Methods

Goniophotographs of unrelated JOAG patients, presenting between 10–40 years of age, were evaluated and compared with 60 healthy subjects in the same age group. Age of onset, family history of glaucoma, highest untreated IOP and visual field defect (mean deviation) were analyzed and correlated with the gonioscopic features among JOAG patients.

Results

Of 126 patients included in the study, 44 (34 %) had a normal open angle (group 1), while 82 (66 %) had developmental anomalies (group 2). Developmental anomalies of the angle were classified as: high iris insertion with or without prominent iris processes (n?=?42), a featureless angle (n?=?30), and those with prominent iris processes alone (n?=?10). There was no difference in age of onset (group 1, 30.5?±?7 years and group 2, 26.3?±?9.6 years) (p?=?0.07) or the untreated IOP at presentation (group 1; 36?±?12.5 mmHg and group 2, 38.8?±?12.3 mmHg; p?=?0.37) between the groups. However, those with angle anomalies presented with a greater visual field defect (MD ?23.5?±?10.5 vs ?14.8?±?13 dB; p?=?0.02) compared to those with normal appearing angle.

Conclusions

While two thirds of JOAG patients present with developmental anomalies of the angle, one third have normal appearing angles. High insertion of the iris is the most common form of gonio dysgenesis observed. Those with angle dysgenesis are more likely to present with severe disease.     

Tuberculosis in acute leukemia: a clinico-hematological profile

We studied 130 consecutive cases of acute leukemia over a 2-year period and identified 9 cases (6.9%) with active tuberculosis (TB). Eight patients with TB had acute myeloid leukemia (AML). Patients with AML were more likely to develop TB as compared to patients with acute lymphoblastic leukemia (ALL) despite the wider use of steroids and radiotherapy in ALL protocols {OR 4.41 (CI 0.53-36.44)}. Only 1 patient died of disseminated TB during post induction neutropenia. All other patients were successfully managed using current anti-tuberculous therapy (ATT). On the whole, TB did not cause any undue delay in chemotherapy and did not flare up during subsequent chemotherapy cycles. However it is not a commonly described infection in acute leukemia and a high index of suspicion is warranted especially in areas endemic for TB.     

Autoimmune hemolytic anemia in India: clinico-hematological spectrum of 79 cases

The clinico-hematological profile and treatment outcome of patients with autoimmune hemolytic anemia (AIHA) were assessed using a retrospective case record analysis. There were 79 (52 primary; 27 secondary) consecutive patients identified with a median age of 30.5 years. The main presenting complaints were pallor (94%), fever (46%), jaundice (51%), bleeding manifestations (10%) and splenomegaly (68%). Jaundice was much more common in primary (63%) as opposed to secondary (26%) disease. The direct antiglobulin test was negative in six patients. Oral prednisolone produced remission in 87.5% patients. Six patients (three children, three adults) relapsed after a median period of 2 months after response. All of these responded to a second course of steroids, in a median period of 14 days. No correlation was found between response and the parameters of age, sex, jaundice, low pretreatment hemoglobin, reticulocyte count, total leucocyte count (TLC), platelet count, subtype of AIHA and hepatosplenomegaly. Relapse correlated with increased duration between the onset of symptoms and treatment (r = 0.996; p = 0.0001).     

Efficacy and safety of anti-D for immune thrombocytopenic purpura in children

This study was conducted in 20 children (16 males) (mean age 9.2 ± 4.34y) with immune thrombocytopenic purpura (ITP) to assess the response to anti-D immunoglobulin. Six patients had newly diagnosed ITP, 6 had persistent ITP and 8 had chronic ITP. The overall response rate was 70% (14/20). The median time to response was 3 days (1–13 days). Response to anti-D was not related to age, sex, severity of bleeding, platelet counts at presentation, ABO blood group, or prior steroid or IVIG response.