Discussion:
Congenital hypopituitarism is a rare condition and refers to absence or reduction in function of two or more hormones produced by pituitary gland. When all pituitary hormones are affected the term panhypopituitarism is used. It can be congenital or acquired. Congenital hypopituitarism is potentially fatal in newborn period but treatable if the diagnosis is made early. Presentation of hypopituitarism can vary in different patients. In our case patient presented with septic shock, persistent hypoglycemia , seizures, hypothermia and jaundice
Blizzard and Albert’s3 first reported Congenital Hypopituitarism in 1956, which described a newborn with microphallus and cryptorchidism, presented with apneic and cyanotic spells in the first few hours of life and died within 24hours.. Our patient presented in the one and half month age hours of life in septic shock with
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In our infant the only clinical clue was microphallus.
There can be many life threatening complications if not treated early. In one case study congenital hypopituitarism presented with renal failure. Patient developed renal failure due to initial cardiovascular compromise related to hypotension4. Sometimes microphalus is only the presentation5. It can also be associated with midline alterations6 .
Congenital hypopituitarism should be followed regularly. It has been suggested these patients have low IQ compared with other normal children7. This case report highlights the importance of prompt and thorough investigation for unexplained hypoglycemia in the newborn. It also emphasises the fact that the association of hypoglycemia and microphallus in the male neonate is presumptive evidence of congenital hypopituitarism. To our knowledge this is the first report of association of late onset congenital hypopituitarism with septic shock and
Premature babies sometimes have apnea. It may happen together with a slow heart rate. Respiratory distress syndrome or RDS is a breathing problem most common in babies born before 34 weeks of pregnancy.” Babies with RDS don’t have a protein called surfactant that keeps small air sacs in the lungs from collapsing. “Intraventricular hemorrhage or IVH is bleeding in the brain. It usually happens near the ventricles in the center of the brain. A ventricles is a space in the brain that’s filled with fluid. Patent ductus ateriosus or PDA is a heart problem that happens in the connection between two major blood vessels near the heart. If the ductus do not close properly after birth, a baby can have breathing problems or heart failure. Heart failure is when enough blood can’t get pumped into the heart causing it to shut down. Necrotizing enter colitis (NEC) is a problem with a baby’s intestines. It causes feeding problems, a swollen belly and diarrhea. It sometimes happens 2 to 3 weeks after a premature bay has been born. Retinopathy of prematurity (ROP) is an abnormal growth of blood vessels in the eye. ROP can lead to vision loss. Jaundice is when a baby's eyes and skin look yellow. A baby has jaundice when his liver isn't fully developed or isn't working well. Anemia is when a baby doesn’t have enough healthy red blood cells to carry oxygen to the rest of the body. Bronchopulmonary dysplasia (BPD) is a lung condition that can develop in premature babies as well as babies who have treatment with a breathing machine. Babies with BPD sometimes develop fluid in the lungs, scarring and lung damage. Premature babies often have trouble fighting off germs because their immune systems are not fully formed. Infections that may affect a premature baby include pneumonia, a lung infection; sepsis, a blood infection; and meningitis, an infection in the fluid around the brain and
pituitary is not creating as much TSH. This would cause the Thyroid to stop producing T4
Benjamin is a 12-4/12 year old boy with Velocardiofacial syndrome, who has been followed for quite some time. Benjamin presented with concerns of micropenis, as well as growth failure. As you can see from the attached penile growth curve Benjamin does in fact have a micropenis with a measurement that is significantly below the normal curve. With respect to his height, he was monitored and fell from 50% to just above 10% on the growth curve. He subsequently underwent growth hormone stimulation testing which he failed with a peak growth hormone response of 2.4ng/ml. Clearly, the failed growth hormone stimulation test and the micropenis are consistent with diagnosis for growth hormone deficiency.”
Some of the progressive signs of respiratory distress exhibited by this infant after birth are; nasal flaring, grunting, coarse breath sounds, increase respiratory rate of 100 and irregular, acrocyanosis, his appearance of being pale, circumoral cyanosis, and him developing jaundice as a result of his increased work of breathing and hypoxia which causes an increase in erythropoietin to be released from the kidneys resulting in polycythemia as a result of low levels of oxygen in the body. Subsequently, as a result of an increase or red blood cell production the body will develop jaundice due to an increase in red blood cell breakdown which causes an increase of bilirubin in the blood to accumulate, therefore, causing jaundice to develop.
On ultrasound there is a live fetus in cephalic presentation. Fetal biometry is consistent with dates. On detailed survey we did note an ONTD that is suspected to be from the lumbar down to the sacral area. There was no skin covering the area and there was no clear evidence of meningomyocele but on transverse imaging of the spine they are significantly splayed. In the intracranial views we did see a lemon and banana sign. The ventricles were upper-normal and prominent, right at 1 cm with dangling choroids.
The Boston Children’s Hospital defines this as a condition where there is too much bilirubin in a baby’s blood and other tissues and fluids.
There have been many changes throughout the years in healthcare. One of the many changes that affect us every day is oxygen toxicity in premature babies and the effects on their eyes. There is a fine line to walk when protecting the function of the brain and preserving vision. The risks have been a concern for healthcare providers for decades. Some people were born at 26 weeks in the early 60’s and suffers from severe retinopathy from over oxygenation. Between 1950 and the mid-1960’s many babies were blinded due to over oxygenation (Millenson, 2015).
overexposed to oxygen for extended periods (Jobe & Kallapur, 2010). When a baby is born
FHI is a clinical and genetical disorder which can be inherited in an autosomal dominant(less common) and autosomal recessive patterns of inheritance. In this cases, the pancreatic beta cells over-produce insulin. The condition is characterized by inadequate suppression of insulin secretion in the presence of severe, recurrent, fasting hypoglycemia (Nestorowicz, et al. n.d.). The clinical manifestations range from life-threatening hypoglycemia presenting in the first days of life to only mildly symptomatic hypoglycemia in an adolescent that may be difficult to identify. The response to medical and surgical therapy also varies between affected individuals. Normally, insulin is secreted in response to the amount of glucose in the
recessive disorder of the exocrine glands. About one in every 2500 Caucasians is affected, and
Hashimoto’s Thyroiditis, also called Hashimoto’s disease is an autoimmune disease in which the body’s immune system attacks the thyroid gland. It was named for the Japanese surgeon who discovered it in 1912 The thyroid is a small butterfly shaped gland in the front of the neck. The thyroid makes hormones called T3 and T4. These hormones regulate metabolism. The thyroid is controlled by hormones of the pituitary gland, which is also called the “master gland.” It is a pea-sized gland located in the base of the brain which, among others, makes thyroid stimulating hormone, or TSH. TSH stimulates the thyroid to make thyroid hormone. With Hashimoto’s disease, the thyroid cells are damaged resulting in the inability to make enough thyroid
rsistence hyperinsulinemic hypoglycemia in infancy (PHHI) is the complex gene disorder and major cause of Hyperinsulinism in infants. It was first identified as a Greek word Nesidioblastosis (islets)in 1938 by George F. Laidlaw. He pointed out those babies who had abnormal or defective pancreas specifically pancreatic beta cell which recognize as producing insulin but some people in 1930s and 1940s found low blood sugar and also noticed an increased level of insulin in the blood . In 1950 Mcquarrie reported that there have been series of children who are having low blood sugar and increase level of insulin and he noted at that time that half of those children were severely brain damage and found that in some communities the death ratio is 1
Primary hyperparathyroidism (PHPT) is an endocrine disorder characterized by autonomous production of parathyroid hormone. The increase in PTH secretion in primary hyperparathyroidism is, in part, due to an elevation in set-point. The increase in the set-point ranging between 15 to 30 percent above that of a normal parathyroid gland is the major determinant of the severity
Furthermore Bath et al have shown that even lower iodine deficiencies can lead to lower cognitive scores in children. The consistency of this topic is as well high. Several other studies have shown that there is a suboptimum cognitive outcome when there is either a mild thyroid hormone deficiency or an iodine deficiency in the mother during pregnancy. The biological plausibility is like the temporality and consistency high. The fetus is dependent on the mother for the thyroid hormones needed for brain and neurodevelopment during the first trimester.