Demographic, Clinical and Biochemical Characteristics of Patients with Fibrocalculous Pancreatic Diabetes in North-East Bangladesh.

Fibrocalculous pancreatic diabetes (FCPD) is a secondary form of diabetes mellitus occurring primarily in tropical countries like Bangladesh and has distinct characteristics. The present study aimed to describe the demographic, clinical and biochemical characteristics of patients with FCPD. This cross-sectional study was conducted at Mymensingh Medical College Hospital, Bangladesh, from January 2019 to December 2021. All patients with FCPD (previously or newly diagnosed) admitted to the inpatient Endocrinology department of the hospital were evaluated. Out of the 15 patients, 73.3% were aged 10-29 years at diagnosis, the male: female ratio was 11:4, rural: urban ratio was 9:6, 20.0% had FCPD in the first-degree family members, 73.3% were underweight, none were overweight/obese or central obese and one of them was hypertensive. Diabetes was uncontrolled in all, with a mean HbA1c of 10.5±1.9%. All but one had low C-peptide and all required insulin to manage diabetes. Although their average (mean or median) lipid parameters were normal, 73.3% of them had dyslipidemia. Among diabetic complications, diabetic nephropathy (66.7%) and neuropathy (66.7%) were more frequent, whereas diabetic retinopathy (6.7%), ischemic heart disease (6.7%) and peripheral vascular disease (6.7%) were less frequently observed; 13.3% had a history of diabetic ketoacidosis. Malnutrition manifested as abnormal skin and hair conditions and anemia was also common in the study subjects. Patients with FCPD are usually young males from rural residences. Microvascular diabetic complications are common, but macrovascular complications and DKA can occur in FCPD.

Noonan Syndrome Presenting with Stunted Growth: A Case Report.

Noonan syndrome is a genetic disorder of autosomal dominant inheritance that prevents normal development in various parts of the body. A spontaneous mutation without any family history may also result in the condition. Noonan syndrome can affect normal growth. Birth weight may be normal, but growth slows over time. The growth spurt usually seen during the teenage years may be delayed, and bone maturity also is delayed. In this case A 13 year's male admitted inpatient Department of Endocrinology, Mymensingh Medical College Hospital in April 2021 with not attaining appropriate height and delayed development of secondary sexual characteristics. His birth weight was normal; gestational and neonatal history was uneventful. He was diagnosed with severe pulmonary stenosis at four years and underwent cardiac surgery at his four and eleven years. He was noted to have growth failure from the age of 9 years onward. He had no family history of such type of disease. On examination, he was short statured, underweight, having an upper: lower segment ratio of 1.05 with an arm span of 126cm. He had craniosynostosis, high arched palate, the thick helix of ears (outer rim), small, upturned nose, depressed broad nose, deeply grooved philtrum, keratosis pilaris of the face and upper arm, slant eyes with proptosis, keloid scar over mid-chest, widely spaced nipple, shield chest, pectus excavatum and cubitus valgus. His sexual maturation score was A1, P1, B1. He had pulmonary stenosis with pulmonary hypertension. He had mild microcytic anemia with normal liver, renal, blood glucose, and calcium profile. His bone age was delayed (9 years), thyroid function was normal. The growth hormone dynamic test after clonidine stimulation was normal. His karyotype was 46XY. We have considered giving recombinant growth hormone therapy to accelerate his height.