Gonadotropin-secreting and thyrotropin-secreting pituitary adenomas: A single-center experience

ABSTRACT Objective: Data regarding rare FPAs from India, a resource limited setting, are limited. We describe a case series of rare FPAs from a single center in western India. Materials and methods: This was a retrospective case record review of patients diagnosed between January 2010 and July 2022. The diagnosis was based on biochemical (inappropriately elevated serum FSH/LH) and pathologic (positive immunostaining for FSH/LH) features in patients with FGA, and elevated serum thyroid hormones and normal/elevated TSH in patients with TSHomas. Results: We identified 11 patients with a total of six FGAs (median age 43.5 years, five men, one FGA cosecreting TSH, median largest dimension 40 mm, range 33-60 mm) and six TSHomas (median age 34.5 years, four women, two TSHomas cosecreting GH, median largest dimension 42.5 mm, range 13-60 mm). Symptoms of sellar mass effects led to pituitary imaging in most patients with FGA. Patients with TSHomas had symptoms of excess hormone secretion (GH/TSH) or sellar mass effects. The TSHomas that cosecreted GH/FSH were larger than those secreting only TSH. Transsphenoidal resection was the most common first-line therapy but significant residual disease was frequent (3 out of 6 FGAs and 4 out of 5 TSHomas). Conclusion: This is the first and second case series of FGAs and TSHomas, respectively, from India. In this study, TSHomas presented at younger age, were larger and had low surgical cure rates.

Gonadotropin-Dependent Precocious Puberty: Single-Center Experience From Western India

Objective: To describe the characteristics of gonadotropin-dependent precocious puberty (GDPP) in Indian children. Methods: Clinical profiles of GDPP (n=78, 61 females) and premature thelarche (n=12) from a single center in Western India were retrospectively studied. Results: Pubertal onset was earlier in boys than girls (29 vs 75 months, respectively; P=0.008). The basal luteinizing hormone (LH) was ?0.3 mIU/mL, except 18% of GDPP girls. At 60 minutes after GnRHa-stimulation, all patients (except one girl) had LH ?5 mIU/mL. The GnRHa-stimulated LH/FSH ratio was ?0.34 at 60 minutes in girls with GDPP unlike premature thelarche. Only one girl had an allergic reaction to long-acting GnRH agonist. Among GnRH agonist-treated girls (n=24), the predicted final adult height was -1.67±1.5 SDS, whereas the attained final height was -0.25±1.48 SDS. Conclusion: We establish the safety and efficacy of long acting GnRH agonist therapy in Indian children with GDPP. The 60-minute stimulated serum LH/FSH of ?0.34 differentiated GDPP from premature thelarche.

Functional parathyroid cystic adenoma: A rare cause of hypercalcemic crisis with primary hyperparathyroidism.

We discuss a case of primary hyperparathyroidism caused by a giant cystic parathyroid adenoma presenting with neck swelling and hypercalcemic crisis. Fine‑needle aspiration cytology of presumed thyroid swelling from one of the two sites aspirated yielded clear fluid but was not attributed to parathyroid pathology. Elevated serum calcium and intact parathormone (iPTH) levels suggested preoperative parathyroid pathology. Ultrasound neck and sestamibi scan for parathyroid localization were not conclusive. Due to resistant hypercalcemia, the patient underwent emergency bilateral neck exploration and excision of the identified left superior parathyroid cyst along with total thyroidectomy. Monitoring of intra‑operative iPTH helped complete removal of hyperfunctioning parathyroid tissue. Histopathological examination confirmed the parathyroid cyst. Cystic parathyroid adenoma should be considered in the differential diagnosis of cystic neck lesions.

Efficacy of Fixed Low Dose Hydroxyurea in Indian Children with Sickle Cell Anemia: A Single Centre Experience.

Introduction: Data on the efficacy of hydroxyurea (HU) in Indian children with sickle cell anaemia (SCA) is limited. Hence, we have evaluated the efficacy of fixed low dose HU in Indian children. Methods: The study cohort consisted of 144 children (<18 years of age) with SCA having severe manifestations (≥3 episodes of vasocclusive crisis or blood transfusions, or having ≥1 episode of acute chest syndrome or cerebrovascular stroke or sequestration crisis) who were started on fixed low dose HU (10 mg/kg/day). They were followed up for two years and monitored for the hematological and clinical efficacy and safety. Results: There was significant increase in the fetal hemoglobin level (HbF%), total hemoglobin and mean corpuscular volume. Vasoocclusive crises, blood transfusions, acute chest syndrome, sequestration crises and hospitalizations decreased significantly. Baseline HbF% had significant positive correlation with HbF% at 24 months. There was significant negative correlation between baseline HbF% and change in HbF% from baseline to 24 months. No significant correlation was found between HbF% at baseline and clinical event rates per year after HU. No major adverse events occurred during the study period. Conclusion: Fixed low dose HU is effective and safe in Indian children with SCA.

Morbidity pattern in hospitalized under five children with sickle cell disease.

Background & objectives: Children with sickle cell disease require more frequent hospital care and younger children (<5 yr of age) are more vulnerable to mortality. There are limited data on the events leading to hospitalizations and death in younger children with sickle cell disease from India. This study was, therefore, undertaken to evaluate the morbidity pattern in hospitalized under five children with sickle cell disease in a tertiary care hospital in Maharashtra, India. Methods: This was a prospective observational study carried out from July 2007 to June 2009. Hospitalized children below five years of age with sickle cell disease were enrolled for the study and evaluated for morbid event/s leading to hospitalization. Haematological indices were noted at baseline (most recent past when patient was not acutely sick) and at the time of hospitalization. Results: Eighty five children with sickle cell disease were hospitalized during the study period. Hospitalization with acute febrile illness (31%) was the most common morbid event followed by severe anaemia (30%) and acute painful events (20%). Majority (62%) of the events occurred between August and October. Forty five patients had foetal haemoglobin (HbF) more than 20 per cent (26.80±4.81%) and morbidity was significantly less in these patients. Interpretation & conclusion: Acute febrile illness was the most common morbid event followed by severe anaemia and acute painful event hospitalized children with sickle cell disease. There was significant seasonal variation with maximum events occurring in the monsoon season.

Sickle Cell Anemia from Central India: A Retrospective Analysis.

Although sickle cell anemia in India is believed to have a mild clinical presentation, few studies report severe disease in many patients from central India. Hence, we have retrospectively studied 316 children with SCA who were followed up for a period of 5.8±5.7 years. There were 55.4 blood transfusions, 43.3 episodes of vaso-occlusive crises requiring hospitalization, and 108.9 hospitalizations per 100 person years. Ninety six (30%) patients had severe disease whereas 74 patients also fulfilled the criteria for hydroxyurea therapy. Significant proportion of children with sickle cell anemia from central India present with severe clinical presentation and require regular medical attention.

Clinico-Epidemiological Profile of Children Hospitalized with Dengue.

Objective To study the clinico-epidemiological profile of children hospitalized with dengue illness. Methods Prospective study of children hospitalized with the diagnosis of dengue illness during from September through November 2006 at a tertiary care centre in Jaipur. Results A total of 948 children including 671 (70.8%) boys and 277 (29.2%) girls were diagnosed to have dengue illness during the outbreak. Two third of children were from urban areas while 6–12 years was the most commonly affected age group (45.8%). 58.3% cases had dengue fever (DF) while 41.7% had DHF (dengue hemorrhagic fever). Dengue fever with bleed (DFB) accounted for 32% of cases. Common constitutional symptoms were vomiting (35.2%), pain abdomen (22.1%) and myalgia (10.1%). Bleeding manifestations were observed in 44.5%of cases.. Positive tourniquet test was the most common manifestation which was seen in 300 cases (31.6%) while in 9.2% cases bleeding was the only manifestation. Epistaxis (25%) was the most common spontaneous bleeding manifestation. Thrombocytopenia was documented in 84%of total cases and bleeding occurred more often in patients with severe thrombocytopenia. Ten children expired with a case fatality rate of 1.1%. Conclusions Children between 6 and 12 yrs were most affected by dengue with larger number of cases from urban areas. DFB cases accounted for almost one third cases of dengue. Epistaxis was the most common spontaneous bleeding manifestation. Bleeding occurs more often in patients with severe thrombocytopenia.